Chapter Outline
Pathology
Course and Prognosis
Treatment Overview
Management of the Hip
Management of the Knee
Management of Specific Deformities of the Foot and Ankle
Management of the Trunk
Management of the Shoulder
Management of the Elbow
Management of the Forearm
Poliomyelitis is an acute infectious disease caused by a group of neurotropic viruses that initially invade the gastrointestinal and respiratory tracts and subsequently spread to the central nervous system (CNS) through the hematogenous route. The poliomyelitis virus has a special affinity for the anterior horn cells of the spinal cord and for certain motor nuclei of the brainstem. These cells undergo necrosis, which results in loss of innervation of the motor units that they supply.
The first description of paralytic poliomyelitis was given by Underwood in 1789.
Infection may be caused by type I, II, or III poliomyelitis virus. No cross-immunity exists among the various types of poliovirus; thus infection may recur in the same individual. Poliovirus is a member of the enterovirus group, which includes coxsackievirus and the echoviruses. Paralytic disease that is clinically and pathologically indistinguishable from poliomyelitis can be produced by various other enteroviruses. These viruses may be isolated by tissue culture.
In the past, poliomyelitis was an epidemic disease in the summer and fall months, with sporadic cases occurring throughout winter and spring. The development and widespread use of a prophylactic vaccine greatly reduced the incidence of poliomyelitis; however, sporadic cases still do occur, and continued rehabilitation of patients who have had the disease remains a concern of the orthopaedic surgeon.
More recently, attention has focused on cases of poliomyelitis caused by oral vaccine, so-called vaccine-associated paralytic poliomyelitis. The risk of contracting poliomyelitis from the vaccine remains extremely low, with a rate of 1 case per 2.5 million doses. Between 1980 and 1989, 80 such cases were reported in the United States. During the same period no cases resulted from wild virus, and 5 cases of imported disease were reported. People at risk for contracting vaccine-associated disease were infants receiving their first dose, persons in contact with vaccine recipients who were not vaccinated, and immunologically compromised people. Because of this rare but devastating occurrence, the Centers for Disease Control and Prevention (CDC) recommended that children be inoculated first with inactivated vaccine, followed by oral attenuated vaccine. In 2006 the CDC reported that vaccine-associated paralytic polio cases had been eliminated in the United States after this change in vaccine administration; the last such case was reported in 1999. It is important to differentiate vaccine-related polio from Guillain-Barré syndrome. The latter has been reported to occur more frequently during oral polio vaccination campaigns.
The effort to eliminate poliomyelitis worldwide continues. Great credit goes to several organizations for their work, including Rotary International, the Bill and Melinda Gates Foundation, and the World Health Organization. Much progress has been made, as noted in 2011 that between 2009 and 2010 the rate of polio in Nigeria and India had been reduced by 95%. The authors stated that this represented “real opportunities in India and Nigeria, setting the stage for polio to follow smallpox into the history books.” In a 2012 report, however, these recent gains were reported to be offset by continued endemicity in Pakistan and Afghanistan where large outbreaks had occurred. Major issues are the identification of asymptomatic viral carriers and the difficulty of environmental screening, which even involves the screening of sewage. Polio can be contracted by unvaccinated adults traveling to countries where polio is still endemic.
Another manifestation of poliomyelitis that has received considerable attention is the occurrence of postpolio syndrome. This syndrome is characterized by increasing muscle weakness, fatigue, pain, and loss of function in individuals who contracted poliomyelitis 20 or more years earlier. Perry and associates showed that this condition represents a chronic overuse syndrome, with overstressed muscles “wearing out.” Another theory of the origin of this phenomenon is the failure of axonal sprouts that formed during the healing process after poliomyelitis. The prevalence is higher in women than in men, and the syndrome is more likely in those who contracted polio at an early age. A discussion of the management of this problem, however, is beyond the scope of a text on pediatric orthopaedics.
This chapter deals with general principles of the management of paralytic deformities of the musculoskeletal system that result from poliomyelitis. These principles are applicable not only to the treatment of poliomyelitis but also to the management of similar problems of flaccid paralysis secondary to other causes. For a detailed account of the disease and its medical aspects of management, the reader is referred to the voluminous literature on the subject.
Pathology
Poliovirus has a definite predilection for the anterior horn cells of the spinal cord and for certain motor nuclei in the brainstem. The lumbar and cervical enlargements of the spinal cord are the most commonly affected. The damaging action on the motoneurons may be direct, occurring through the toxic effects of the virus, or indirect, occurring through ischemia, edema, and hemorrhage in the neurons’ supportive glial tissue.
The motoneurons swell and the Nissl substance in their cytoplasm undergoes chromatolysis. An inflammatory reaction ensues, with infiltration of polymorphonuclear and mononuclear cells into the gray matter, particularly the perivascular areas. The necrotic bodies are subsequently replaced by scar tissue.
Involvement of the anterior horn cells varies from minimal injury, with temporary inhibition of metabolic activity and rapid recovery, to complete and irrevocable destruction.
Paralysis is of the flaccid type, with the individual motor units following the “all-or-none” law, because the virus affects the anterior horn cells rather than the muscle. The percentage of motor units destroyed varies, and the resultant muscle weakness is proportionate to the number of motor units that are lost. For example, a muscle with “poor” muscle strength will have 20% of its motor units functioning, whereas a muscle with “good” motor strength will have 80% of its motor units functioning. These remaining functional motor units are called guiding neuromuscular units and are of particular importance in retaining patterns of motion of the individual muscles or muscle groups during the recovery stage. The recovery of muscle power primarily depends on restitution of the anterior horn cells of the spinal cord that have been damaged but not destroyed.
Course and Prognosis
The course of the disease is subdivided into acute, convalescent, and chronic phases. The acute phase, which lasts from 5 to 10 days, is the period of acute illness when paralysis may occur. It is further subdivided into the preparalytic phase and the paralytic phase. The acute phase is ordinarily thought to end when the patient has had no fever for 48 hours.
The convalescent phase encompasses the 16-month period after the acute phase. During this time a varying degree of spontaneous recovery in muscle power takes place. This phase is also further subdivided into the sensitive phase (lasting from 2 weeks to several months), characterized by hypersensitivity of muscles, which are tender and “in spasm,” and the insensitive phase, in which the muscles are no longer sensitive but are still in the period of recovery.
The chronic or residual phase is the final stage of the disease after recovery of muscle power has taken place. It encompasses the rest of the patient’s life after termination of the convalescent period.
Immediately after onset it is difficult to make an accurate prognosis regarding the rate and extent of spontaneous recovery. It is best to assume that the involved muscles will recover until the subsequent course of the disease demonstrates otherwise. Muscle recovery is most marked in the first 3 to 6 months, and the potential for recovery ceases approximately 16 to 18 months after onset.
The two primary factors to consider in the prognosis are the severity of the initial paralysis and the diffuseness of its regional distribution. If total paralysis of a muscle persists beyond the second month, severe motor cell destruction is indicated, and the likelihood of any significant return of function is poor. If the initial paralysis is partial, the prognosis is better.
The condition of the neighboring muscles is another consideration. A weakened muscle surrounded by completely paralyzed muscles has less chance of recovery than does a muscle of corresponding power that is surrounded by strong muscles. Muscle spasm, contracture of antagonist muscle groups, deformity, and inadequate early treatment are other factors that may interfere with recovery of muscle function.
Treatment Overview
Management of poliomyelitis varies with the stage of the disease and the severity and extent of paralysis. Treatment in the acute febrile stage is primarily the domain of the pediatrician or internist, and patients are admitted to infectious disease hospitals or to isolation units of general hospitals. Care of the musculoskeletal system, however, is important from the first day of the disease. It is imperative that the orthopaedic surgeon be consulted to examine a patient with a suspected case of poliomyelitis before lumbar puncture is performed. The surgeon should be responsible for all orders concerning management of the musculoskeletal system. The pediatrician is responsible for general care of the patient, especially any problems of respiratory system and bulbar involvement, should they develop. Once afebrile for 18 hours (i.e., after termination of the acute stage), the patient should be transferred to the service of the orthopaedic surgeon, who assumes the dominant role. Such delineation in addition to continuity of supervision is mandatory because it stimulates early attention to deforming tendencies and prevents their development.
Acute Phase
During the initial, febrile phase of the disease, the primary concerns of the orthopaedic surgeon are the comfort of the patient and the prevention of deformity. It is best to place the patient on complete bed rest and restrict physical activities to a minimum. The patient is irritable and apprehensive. It is important to reassure the patient and allay fears.
General medical measures consist of the administration of a varied diet with relatively high fluid intake, attention to urinary retention and bladder paralysis, prevention of constipation and fecal impaction, and provision of analgesia for pain. Opiates and other medications that have a depressing action on the CNS should not be given in the presence of impending paralysis of the muscles of respiration.
A detailed determination of the severity and extent of muscle paralysis is not warranted during this febrile period. By gentle handling of the limbs and trunk, however, the clinician can make an approximate assessment of the degree and distribution of the motor weakness without much distress to the patient. This initial muscle examination has diagnostic and therapeutic implications. It also provides the necessary information to prevent the development of potential deformities consequent to paralysis.
Ordinarily, paralysis develops 2 or 3 days after the onset of fever and increases in severity for several days. Progressive involvement ceases only after the elevated temperature returns to normal. Characteristically, the paralysis in poliomyelitis is asymmetric. In the presence of symmetric paralysis of the limbs and trunk, a paralytic disease other than poliomyelitis should be considered. In a large epidemic the care of patients will be much simplified if those with paralysis are separated from those without paralysis.
Management of Respiratory Involvement
Patients with bulbar and respiratory involvement require specialized intensive care. An early appraisal of the distribution and extent of paralysis will help detect muscle weakness in certain areas that should alert the clinician to the possible development of such distressing complications. For example, a patient who cannot lift his or her head because of paralysis of the anterior neck muscles or one who has a nasal intonation of the voice, difficulty swallowing, and weakness of the facial muscles should be watched carefully for bulbar involvement. Prompt diagnosis and treatment are essential to keep the patient’s airways open because the condition may be fatal. Aspiration of unswallowed secretions is a definite danger. The foot of the bed is elevated and the patient is placed in a prone or lateral position. Frequent suction or postural drainage is usually required. Occasionally, tracheostomy may be necessary.
Another anatomic area that should be observed for muscle weakness is the shoulder girdle. The nerve supply to the deltoid muscle is provided by the fifth cervical root; it is adjacent to the fourth cervical root, which innervates the diaphragm. Consequently, progressive paralysis of the deltoid muscle is usually followed by paralysis of the intercostal muscles and the diaphragm. An increased rate of breathing, use of accessory muscles of respiration, and restlessness, anxiety, and disorientation are signs that should alert the physician to the possible need for a mechanical respirator. Paralysis of the diaphragm is easily detected on fluoroscopy. Abdominal muscle weakness is determined by asking the patient to lift the head and shoulder or the lower limbs. Asymmetry of power is indicated by the Beevor sign, which is a shift of the umbilicus toward the stronger muscles.
Prevention of Deformity
Patient positioning should provide correct anatomic alignment of the limbs and proper posture of the trunk. The aim is to prevent the development of deformities. The bed should give adequate support and should not sag. A firm foam rubber mattress is preferable. Bed boards should be placed beneath the mattress and should be hinged to permit sitting in the later convalescent period. A padded footboard is used to maintain the ankles and feet in neutral position when the patient is lying supine or prone. Pulling the end of the mattress about 10 cm away from the footboard provides an interspace in which the heels are allowed to fall. Periods in the supine position should be alternated with periods in the prone position. The prone position is important for maintenance of good muscle tone of the gluteus maximus and erector spinae muscles.
When the patient is lying supine, the knees should be held in slight flexion with padded rolls under them and behind the proximal ends of the tibiae to prevent genu recurvatum and posterior subluxation of the tibiae. A slightly flexed position of the knees relaxes the sensitive hamstrings. Excessive flexion of the knees, however, should be avoided. Sandbags or rolled pads are placed on the lateral sides of the thighs and legs to prevent external rotation deformity of the lower limbs. Intermittent use of rolls between the scapulae will prevent forward hunching of the shoulders.
The limbs should not be maintained in rigidly fixed positions. Several times a day the joints are carried passively through their range of motion; this will help relieve muscle pain. Overstretching of the muscles, however, should be avoided. The patient should be handled as gently as possible. Passive motion of the joints of a limb is imperative to prevent stiffness and myostatic contractures. At times, during severe spasm of the hip flexors, hamstrings, and gastrocnemius, the sensitivity and pain of muscles are so great that anatomic alignment cannot be assumed without excessive discomfort.
Management of Muscle Spasm
“Muscle spasm,” a principal manifestation of poliomyelitis in its early stages, is characterized by protective contraction of the muscles to prevent a potentially painful movement. “Muscle resistance to stretch” is more descriptive of this reflex guarding action of the muscles, which resembles the muscle spasm associated with painful phenomena such as hamstring spasm in synovitis of the knee. True spasticity and signs of upper motoneuron involvement are absent. The exact cause of the muscle pain and sensitivity is unknown. Most probably these manifestations are the result of inflammatory changes in the posterior ganglia and meninges. Other possible causes are lesions in the reticular substance and lesions of the internuncial neurons in which inhibitory fibers to the anterior horn cells are affected.
The degree of muscle pain and sensitivity varies considerably. Some muscle discomfort is usually present in the preparalytic period. Nerve traction tests, such as those of Lasègue and Kernig, increase muscle spasm and pain. Spontaneous severe pain is rare but occasionally seen in adult patients. The important consideration is that the painful strong muscles tend to shorten during the sensitive phase; if these muscles are maintained in their shortened position, myostatic contracture and fixed deformity will develop.
In the acute and sensitive phase of convalescence, application of moist heat relieves the sensitivity of the muscles and alleviates discomfort. Physiologically, heat increases local temperature and enhances blood flow to the muscle. It has no specific therapeutic effect on the course of the paralysis and actual recovery of involved nerve cells. Heat is more beneficial if it is applied intermittently for short periods.
In the acute phase, to minimize handling of the patient, a lay-on wool pack is used. It consists of three layers, one of wool blanket material (wrung out of boiling water by passing it twice through a wringer) and one of waterproof material, which in turn is covered by an outer layer of wool blanket. The number of these packs and the duration of their use are individualized according to the intensity of pain and spasm. In general, two moist heat packs are applied during a 20-minute period. Continuous and overzealous use of heat should be avoided because it can be tiring and harmful to the patient. Moist heat is best used before physical therapy in the acute phase to assist in developing greater range of joint motion and to facilitate the performance of active exercises. Warm tub baths are substituted for the lay-on packs within a few days after the patient’s temperature has returned to normal and when the patient’s general condition permits. The buoyant effect of water makes it easier for the weakened muscles to move. Active exercises in water in the acute phase should be closely supervised so that the patient does not substitute stronger muscles for the weaker ones. Again, the patient’s comfort is the primary consideration. The temperature of the tub baths should be approximately 100° F, and the total period of immersion in the tub should not exceed 20 minutes. In cases of extensive paralysis, overhead cranes may be used to lower the patient directly into the tub from the stretcher.
Convalescent Phase
The objectives of treatment during the convalescent stage are (1) attainment of maximum recovery in individual muscles, (2) restoration and maintenance of normal range of joint motion, (3) prevention of deformities and correction of deformities if they occur, and (4) achievement of the best possible physiologic status of the neuromusculoskeletal system.
Management of Muscle Spasm and Prevention of Deformity
In the early part of the convalescent stage, because muscle sensitivity and spasm are still present, the use of hot packs is continued for the comfort of the patient. Passive exercises are performed four to six times a day to prevent the development of contractural deformity. When joint motion is limited, gentle passive stretching exercises are added to the therapy program. This exercise regimen should not cause the patient discomfort; however, the threshold of pain may be very low in an apprehensive, sensitive person. A firm but sympathetic attitude by the therapist is important, and the patient should be encouraged more each time to gain a greater degree of motion. Tendencies toward deformity should be observed, such as external rotation and abduction of the hips, plantar flexion of the feet, or adduction of the shoulders. Passive stretching exercises should be directed toward preventing and correcting deformity.
Muscle Examination
Several days after onset of the convalescent stage, a complete muscle examination should be performed. Ordinarily, it is done in stages to avoid fatiguing or disturbing the patient. This initial motor assessment provides a basis for comparison with subsequent examinations, and it also serves as a guide to the therapy regimen that is to be instituted. The rate and extent of muscle recovery are determined by repeating these muscle tests periodically—monthly during the first 4 months, bimonthly during the following 8 months, and then quarterly during the second year of the disease. The prognostic value of the serial muscle tests is evident: when a muscle exhibits little or no improvement in power during a 3-month period, it is unlikely that it will recover or gain strength of functional significance. In such a case the patient should be fitted with appropriate orthotic support and allowed greater activity. In contrast, a muscle that shows steady improvement has a good possibility of recovering to a functional level; hence it is unwise to apply an above-knee orthosis to this weak limb and permit the patient to walk.
Preserving and Restoring Neuromuscular Function
In management of the convalescent stage of poliomyelitis, the following aspects of neuromuscular function must be considered.
Patterns of Motor Activity
Limb motions are complex and are not the result of isolated contraction of a single muscle. The functions of many muscles are integrated and coordinated in the execution of a movement and are controlled by the automatic reflexes of the CNS. In dorsiflexion of the ankle, for example, the anterior tibial muscle, the toe extensors, and the peroneus tertius are the prime movers that execute the desired movement, whereas the triceps surae and the toe flexors are the antagonist muscles that become relaxed because of the reciprocal innervation of the agonist and antagonist muscles. The synergist and fixation muscles also contract while the prime mover acts.
In the presence of muscle weakness, the tendency is to use a strong group of muscles that can perform the action more easily and readily, thus excluding the weaker muscles from the pattern of motor activity. A muscle that has been temporarily paralyzed will be left out of the pattern of motion permanently if other muscles substitute for its action during the period of its recovery. In the convalescent stage, these muscular substitutions and abnormal patterns of motor activity should be avoided.
Some neuromuscular units often remain intact in the paralyzed muscles; they act as “guiding contractile units,” and in the performance of active exercises, these functioning neuromuscular units should be used to guide the body part in execution of normal motion.
For example, in reeducation of a poor anterior tibial muscle, the ankle joint is first passively dorsiflexed through its full arc of motion to stretch any contracture of the triceps surae muscle. The limb is then placed in a side-lying position to eliminate the force of gravity, and the ankle joint is again passively dorsiflexed in some inversion through its full range. The therapist assists the patient to localize the action of the anterior tibial muscle and emphasizes that substitution by the toe extensors and peroneus tertius muscle should be avoided.
Next the patient is asked to produce an active, sustained contraction of the anterior tibial throughout its full arc of motion, first with and then without assistance. As the muscle becomes stronger, the limb is placed in the supine position to make the muscle work against gravity, and gradually increasing manual resistance is applied. The active exercises are graduated on the basis of performance. Muscles that are overworked lose strength.
In poliomyelitis, reciprocal innervation between agonist and antagonist muscles is often disturbed, with resultant loss of synergistic muscular action and the normal pattern of motor activity.
Fatigue
A paralyzed muscle is easily fatigued. This is readily shown by its rapid loss of power and inability to function after several effective contractions. Forcing a weak muscle beyond its point of maximal action does not increase its strength; on the contrary, it inhibits recovery of the paralyzed muscle. It is important to observe the level of functional activity of a weak muscle so that it is not forced to exceed its capability.
Contractural Deformity and Progressive Loss of Function
Flaccid paralysis is the chief cause of functional loss. Muscular action is also inhibited by pain, sensitivity, and spasm. When a muscle is maintained in a shortened position for a prolonged period, myostatic contracture develops. Muscle imbalance and increased stress secondary to abnormal patterns of activity are other factors producing deformity. Growth is an important consideration in the management of poliomyelitis in children. The contour of bony structures is influenced by paralysis and dynamic muscle imbalance. For example, when the triceps surae muscle is weak and the ankle dorsiflexors are of normal motor strength, a progressive calcaneus deformity of the hindfoot results. If the child is permitted to walk without support and protection, the loss of power of the triceps surae muscle will be greater because the muscle is working against gravity. Figure 37-1 shows the vicious cycle of factors causing progressive loss of function in poliomyelitis.
In the asensitive stage, proper alignment of the limbs and full range of joint motion must be restored and maintained. Passive stretching exercises are performed vigorously. In the presence of muscle imbalance and a tendency to develop contracture, bivalved casts should be used at night to maintain the part in correct position. When a deformity is fixed, wedging casts or traction may be applied.
Active exercises are performed to integrate recovering motor units into the normal pattern of motion; their primary objective is not to produce hypertrophy of muscles that are already functioning normally. Hydrotherapy and active exercises in a pool are prescribed for patients with extensive paralysis. Motion of the hips, shoulders, and trunk is greatly facilitated in the pool because the buoyant effect of the water facilitates coordinated motion of the parts. Strict supervision by the therapist is mandatory, however, to prevent substitution of strong muscles for those that are weak. Excessive exercises and overwork should be avoided. Patients with extensive paralysis are initially instructed to ambulate in the pool; when adequate control of the trunk and lower limbs is achieved, this is no longer necessary. Standing balance should be developed first, followed by walking with the help of crutches. The gait pattern should be a reciprocal four-point gait, with the amount of body weight borne depending on the degree of paralysis. The physical therapist assists in locomotion so that abnormal mechanisms do not develop. During the convalescent period, use of an orthosis should be kept to a minimum because it increases the workload on the paralytic levels and tends to produce abnormal gait patterns. In severe paralysis of the lower limbs and trunk, however, locomotion may be impossible without the support of an adequate orthosis. General activities of the patient are gradually increased. During the first few minutes of locomotion the gait may be effective, but with fatigue it may become poor. Random, purposeless activity should be discouraged.
Chronic Phase
The goals of treatment in the residual stage are to enable the patient to attain maximal function and to obtain the greatest amount of productive activity despite residual weakness. With continued growth and use of the limb, progressive deformities that will ultimately cause loss of function may develop. Hence an equally important task during the chronic stage is to prevent deformities and to correct them, should they develop. The residual stage is a dynamic, not a static, period. Much can be done to improve the functional capacity of the patient.
Physical Therapy
In the residual stage the physical therapy regimen is directed toward (1) increasing the motor strength of muscles by active hypertrophy exercises, (2) preventing or correcting deformity by passive stretching exercises, and (3) achieving maximum functional activity.
Active Hypertrophy Exercises
Active hypertrophy exercises are performed primarily for the benefit of marginal muscles, to elevate or maintain their functional level. (There is little to be gained by exercising zero or “trace” muscles that remain unchanged after 18 months, and the same is true of muscles that have a “good” or “normal” rating.) For example, when the anterior tibial and toe extensor muscles are “fair” in motor strength and the triceps surae muscles are “normal,” active exercises of the ankle dorsiflexors should be performed to maintain them at the antigravity functional level. Progressive resistance exercises entail the use of activity graded in proportion to the strength of the involved muscles. These exercises are recommended in the residual stage of poliomyelitis to increase the strength and improve the endurance of such individual muscles or groups of muscles as a “fair” quadriceps or triceps surae or a “fair plus” hip abductor muscle to maximum capacity. Whether progressive resistive exercises are of any permanent value when the motor strength of a muscle is less than “fair minus” is doubtful; a “poor” quadriceps muscle cannot, through hypertrophy exercises, be improved to “fair” strength so that it can lift the leg against gravity. Correction of flexion deformity of the knee, however, may provide added strength by eliminating the need for the quadriceps muscle to work against deformity.
Passive Stretching Exercises
Prevention of contractural deformity is much simpler than correction of such deformity. When a limb is continuously maintained in one position, contracture and fixed deformity develop as a result of the effects of gravity and dynamic imbalance of muscles. An ankle joint held in plantar flexion because of weak dorsiflexors and a strong triceps surae is susceptible to the development of progressive equinus deformity if the ankle is not passively stretched into dorsiflexion every day. The calf muscles should also be passively stretched to prevent the development of equinus deformity; this is implemented by the use of a bivalved night cast to hold the foot out of equinus and in neutral position. Passive stretching exercises should be performed gently several times a day. In the presence of muscle imbalance, however, these exercises are not adequate to prevent deformity, and other measures should be undertaken, such as the use of a removable bivalved long-leg night cast to hold the foot in neutral position and wearing of a below-knee dorsiflexion-assist spring orthosis during the day.
Functional Training
The purpose of a functional training program is to enable the patient to overcome the handicaps imposed by the physical disability. The residual deficit in function varies, depending on the extent and severity of paralysis. The needs of a growing child progressively change. In the residual stage the patient is taught how to use all available muscles to accomplish a task successfully. This is in contrast to the convalescent stage, when the patient is not allowed to substitute stronger muscles for weaker ones. For example, when the anterior tibial is “poor” in motor strength in the convalescent stage, the child is not permitted to use the toe extensors to dorsiflex the foot when active exercises are performed with the anterior tibial. In the chronic stage, however, if anterior tibial function is still “poor,” the child is taught how to dorsiflex the foot by using the toe extensors and peroneal muscles.
At times the activity of stronger muscles is suppressed to prevent the development of deformity. For example, an individual with “normal” sartorius, biceps femoris, and peroneal muscles but “poor” iliopsoas, medial hamstring, and anterior tibial muscles walks with a marked external rotation deformity of the foot and leg. It is important to supervise such a patient’s gait and teach the patient to suppress the eversion power of the peroneals and the externally rotating power of the biceps femoris and the sartorius to prevent the development of an external rotation deformity of the lower limb.
To teach a child merely to walk with crutches and orthoses is not satisfactory. The child should be instructed in activities of daily living, such as how to get in and out of chairs, open doors, and enter an automobile.
Orthoses and Other Apparatus
Use of an apparatus may be necessary during the asensitive period of the convalescent stage and the residual stage of poliomyelitis. The primary objectives of the orthosis are to (1) support the patient and enable the patient to walk and increase functional activity, (2) protect a weak muscle from overstretching, (3) augment the action of weak muscles or substitute for muscles completely lost, (4) prevent deformity and malposition, and (5) correct deformity by stretching certain groups of muscles that have been contracted. The support, substitution, and corrective mechanisms may be combined in a single apparatus. In general, dynamic splinting is more desirable than static splinting.
General Principles
Certain general principles should be followed regarding the use of an apparatus in patients with poliomyelitis. Whenever satisfactory recovery of function is expected, an orthosis should be used with caution on the lower limbs because it is likely to produce an abnormal gait pattern. Thus during the early convalescent period, use of an orthosis should be deferred until after maximum recovery of muscle function has taken place. Locomotion without an orthosis but with the support of crutches should be attempted to stimulate active muscular function through the exercise of walking. Use of an orthosis should not, however, be postponed if deformities appear likely to develop from the stress of weight bearing. The needs of each patient are different, and the use of a lower limb orthosis depends on the severity of the muscle weakness and the degree of dynamic imbalance of the muscles. If the patient has extensive paralysis of the lower limbs, use of an orthosis may be the only means of achieving stance and locomotion.
In general, use of an orthosis should be as minimal as the condition permits. For example, if a patient with paralysis of both lower limbs were to be fitted with two above-knee orthoses, he or she would also need to use two crutches to walk. If the patient were to use two crutches, he or she could do as well with an above-knee orthosis on one leg only because only minimal motor strength is required of the other leg to walk without an orthosis. During the stance phase on the leg without the orthotic support, the tripod base is completed by the two crutches; the knee is stabilized by being locked in hyperextension. “Fair” motor strength in the ankle dorsiflexors and hip flexor muscles allows clearance of the lower limb in the swing phase.
It is imperative to explain to the patient the reasons for using an orthosis. The patient should understand clearly that wearing the orthosis will help in the early convalescent stage of the disease and that the orthosis may be discarded later, after training or reconstructive surgery. For example, the use of a dorsiflexion-assist below-knee orthosis may be unnecessary after a successful anterior transfer of the peroneal tendons, or an opponens splint may be discarded after a satisfactory opponens tendon transfer. In addition, when the child becomes an adult, he or she may no longer need an above-knee orthosis to prevent genu recurvatum.
The continued use of an orthosis should be reevaluated regularly. Before advising that use of an orthosis be discontinued, the clinician should be certain that no possibility for the development of progressive deformities exists and that the level and quality of functional performance will not deteriorate.
Specific Applications
Lower Extremity.
When the toe extensor and anterior tibial muscles are paralyzed and the triceps surae muscle is normal, a dorsiflexion-assist spring orthosis (which acts as an active substitute for the weak ankle dorsiflexors) is preferable to a below-knee caliper orthosis with a posterior stop that prevents plantar flexion of the ankle beyond neutral position. In paralysis of the gastrocnemius and soleus muscles, a plantar flexion–assist spring below-knee orthosis with a dorsiflexion stop at neutral position is prescribed ( Fig. 37-2 ). In the presence of a flail ankle and foot, a double-action ankle joint (both plantar flexion–assist and dorsiflexion-assist) is provided, and a varus or valgus T -strap is added to the shoe as necessary. In addition, inside or outside wedges are prescribed for the shoe depending on the deformity of the foot.
When the muscles controlling the knee are paralyzed, an above-knee orthosis with a drop-lock knee joint is prescribed. This type of orthosis provides knee stability for walking and can be unlocked during sitting. If genu recurvatum results from paralysis of the triceps surae in the presence of some strength of the quadriceps femoris, it can be controlled by an above-knee orthosis with a free knee joint constructed so that complete extension of the orthosis at the knee is prevented. Proper positioning of the thigh and calf bands also checks genu recurvatum. Genu varum or knock-knee pads are added as necessary. When flexion deformity of the knee is present as a result of dynamic imbalance between the hamstrings and quadriceps femoris muscles, a well-padded anterior knee component is prescribed. An Engen extension knee orthosis is worn at night to correct flexion deformity of the knee.
Hip.
If the muscles controlling the hip are weak, stability of the hip joint can be provided by an ischial weight-bearing thigh socket; crutches are used if necessary. Rotational alignment of the lower limbs is obtained by the addition of rotation straps or twisters. Ordinarily the patient walks better without a pelvic band and drop-lock hips; however, in a young child with gluteus maximus paralysis, these devices may be used temporarily for balance. Frequently the spine also requires support. When upright posture is resumed, the abdominal muscles overstretch, and severe lumbar lordosis and paralytic scoliosis develop. Any asymmetric involvement of the abdominal and trunk musculature should always be carefully noted. An abdominal corset support with metal stays often serves to control abdominal muscle paralysis. If the trunk extensors are weak, a spinal orthosis with an abdominal corset is provided. If the spine is unstable and collapsing, it may be supported by a molded plastic body jacket constructed from a plaster-of-Paris cast made while the patient is standing, with traction provided by a head sling. In paralytic scoliosis, usually a Milwaukee brace is worn, provided that lower limb paralysis is not extensive and wearing such an appliance does not prevent ambulation. In these instances the Milwaukee brace is used intermittently during periods of recumbency or sitting, or both.
Upper Extremity.
In the upper limb the paralyzed shoulder muscles, particularly the deltoid, are best protected from the effects of gravity with a sling, which allows functional use of the forearm and hand. During the initial period of 6 to 8 weeks an abduction shoulder splint may be worn at night and during part of the day to prevent overstretching of the deltoid muscle, particularly when the patient has associated paralytic subluxation or dislocation of the shoulder joint. A cock-up wrist splint is used when the wrist extensors are paralyzed, and an opponens splint is used when the opponens of the thumb is weak. When the intrinsic muscles of the hand are paralyzed, hyperextension of the metacarpophalangeal joints is prevented by a knuckle-bender dynamic splint.
Surgical Treatment
A multitude of operative procedures can be performed both for the correction of paralytic deformities and for the total physical rehabilitation of a child with poliomyelitis. These procedures may include fasciotomy, capsulotomy, tendon transfers, osteotomy, and arthrodesis. Leg length inequality commonly occurs in poliomyelitis as a result of shortening in the paralyzed leg.
Principles of Tendon Transfer
Tendon transfer entails shifting the insertion of a muscle from its normal attachment to another site to replace the active muscular action that was lost by paralysis and to restore dynamic muscle balance. The procedure was originally described by Nicoladoni in 1882. Many surgeons have devised various types of tendon transfers and established their usefulness. Lange, Velpeau, Vulpius, Codivilla, Mayer, Biesalski, Goldthwait, Ober, Steindler, Bunnell, and Green are some who may be mentioned. * The term tendon transplantation should not be used interchangeably with tendon transfer because the two are not synonymous. Tendon transplantation refers to the excision of a tendon and its use as a free graft. In muscle transplantation both the origin and the insertion of a muscle are detached, and the entire muscle with its intact neurovascular supply is transplanted to a completely new site.
* References .
The basic principles of tendon transfers have been outlined by Green and are listed here.- 1.
The muscle to be transferred must have adequate motor strength to carry out the new function. As a rule, the motor rating of the muscle should be good or normal to warrant transfer. The function that the transferred muscle is intended to perform is another consideration. In the lower limb, for example, in the presence of footdrop, anterior transfer of the peroneus longus is adequate to produce effective ankle dorsiflexion, whereas in calcaneus limp, posterior transfer of the peroneus longus alone to the os calcis is not sufficient to substitute for action of the gastrocnemius-soleus, and the additional action of two or three motors such as the flexor digitorum communis and the anterior tibial muscles is required. Ordinarily, one grade of motor power is lost after a muscle is transferred.
- 2.
The range of motion of muscles on contraction is an important consideration. This range must be similar to that of the muscles for which they are being substituted; furthermore, whenever muscles are transferred in combination, their range of contraction should not differ significantly. The transfer of antagonistic muscles is not ordinarily as effective as the transfer of muscles having similar function or corollary activity. However, with meticulous postoperative care, antagonistic muscles may be transferred effectively with good results. Posterior transfer of the anterior tibial to the os calcis and transfer of the hamstring muscles to the patella are common examples of such antagonistic transfers.
- 3.
In choosing the muscles for transfer, the surgeon must weigh the loss of original function that will result from the tendon transfer against the gains to be obtained. For example, in the presence of hip flexor weakness, the hamstring muscles should not be transferred to the patella for quadriceps paralysis because loss of active knee flexion added to the lack of hip flexion will be a greater disability. Whenever possible, muscle balance must be restored. Ideally, a deforming muscle force must be shifted so that it substitutes for an essential weakness. In the foot and ankle, for example, the muscles of inversion and eversion and those of plantar flexion and dorsiflexion should be balanced. A common pitfall is transfer of the peroneus longus muscle posteriorly to the os calcis in the presence of a strong anterior tibial muscle. Normally, the anterior tibial muscle dorsiflexes the first metatarsal and the peroneus longus opposes this action. With posterior transfer of the peroneus longus, the unopposed anterior tibial gradually causes the first metatarsal to ride up and produces a dorsal bunion. Thus the peroneus longus should not be transferred to the os calcis unless the anterior tibial is shifted from its insertion on the first metatarsal to the midline of the foot.
- 4.
The joints on which the transferred muscle is to act should have functional range of motion. All contractural deformity should be corrected by wedging casts or soft tissue release before tendon transfer. An anterior transfer for footdrop, for example, should not be performed in the presence of equinus deformity of the ankle.
- 5.
A smooth gliding channel with adequate space must be provided for excursion of the tendon in its new location. The paratenon and synovial sheath are preserved over the tendon surface during dissection. It is preferable to pass the tendon beneath the deep fascia through tissues that permit free gliding rather than subcutaneously. A wide portion of the intermuscular septum is excised whenever muscles are passed from one muscle compartment to another. Sufficient space should be provided for the tendon so that adhesions will not form. An Ober tendon passer of appropriate size should be used to redirect the tendon to its new insertion; the tendon passer spreads the tissues and prevents binding.
- 6.
The neurovascular supply of the transferred muscle must not be damaged while transferring the tendon. The surgeon must be careful to avoid denervating the muscle while freeing it for redirection. When the tendon is pulled up from the distal wound into the proximal incision, traction should not be applied to the origin of the muscle. Stretching of the motor nerve can be prevented by using a double-hand technique: the proximal segment of the tendon is held steady with a moist sponge while traction is applied on its distal segment with another sponge. Acute angulation or torsion of the neurovascular bundle is another cause of injury. Gentle handling is imperative to preserve innervation and function of the transferred muscle.
- 7.
In rerouting of the tendon, a straight line of contraction must be provided between the origin of the muscle and its new insertion. Angular courses and passages over pulley systems should be avoided. To allow adequate freeing of the muscle toward its origin, the incision over the belly of the muscle must be long and proximally located.
- 8.
The tendon should be reattached to its new site under sufficient tension so that the transferred muscle will have a maximal range of contraction. The transferred muscle should be tested during the operation to ensure that it will hold the part in optimal position. In the lower limb, where weight-bearing forces are involved, the tendon is ordinarily attached to bone, whereas in the upper limb it is sutured to the tendon. An important technical detail is scarification of the distal segment of the tendon that is to be anchored to a bone or tendon; this is achieved by excising the sheath and paratenon and “roughening” the tendon by scraping and crosshatching it with a knife. To diminish any tension on the tendon while it is healing, the position of immobilization in a cast should allow the transferred tendon to be in a relaxed attitude. For example, when the flexor carpi ulnaris is transferred to the extensor carpi radialis longus, the tension on the tendon should be sufficient to hold the wrist in 30 degrees of dorsiflexion. However, when the cast is applied, the wrist is immobilized in the overcorrected position of 45 to 50 degrees of dorsiflexion.
Postoperative Care and Training
Postoperative care and training are fundamental to achieving a good result. The following principles, given by Green, should be followed meticulously.
The age of the patient at the time of tendon transfer is an important preoperative consideration. The child should be old enough, preferably older than 4 years, to cooperate in training of the transfer. A delay in tendon transfer in the presence of muscle imbalance leads to progressive deformity. Usually, conservative measures should be undertaken to control deforming factors, but early surgery may be indicated when a delay in tendon transfer would result in increasing structural deformity. A common example is the rapid development of progressive calcaneus deformity of the foot with paralysis of the gastrocnemius-soleus muscles and strong ankle dorsiflexors. An early posterior transfer prevents the development of a deformed foot.
Support of the part in an overcorrected position should be continued until the muscle has assumed full function and there is no tendency for the deformity to recur. A bivalved cast or an orthosis holds the transferred tendon in a relaxed position.
It is best to teach the patient preoperatively to localize active contraction in the muscle to be transferred. Active exercises are continued postoperatively as soon as the reaction to surgery and pain have subsided. The surgeon should assist the physical therapist during the initial exercises. When tendon transfer is combined with arthrodesis, muscle reeducation is delayed until adequate bony union has taken place.
The patient is instructed to contract the transferred muscle voluntarily by moving the part through the arc of motion that was the original normal action of the muscle while the therapist manually guides the part to move in the direction that is intended to be provided by the transfer. For example, when the peroneus longus muscle is transferred anteriorly to the base of the second metatarsal, the active motion called for is eversion in combination with guided dorsiflexion, or if the anterior tibial muscle has been transferred posteriorly to the os calcis, active inversion is combined with guided plantar flexion of the ankle. In anterior transfer of the hamstrings to the patella for quadriceps femoris paralysis, the patient is placed in a side-lying position and asked to extend the hip actively (using the hamstrings) as the knee is guided into extension. If the flexor carpi ulnaris has been transferred to the extensor carpi radialis longus, the wrist is gently guided into extension as the patient turns it in the ulnar direction. With one hand the therapist should palpate the belly and tendon of the transferred muscle to ensure its contraction. In the beginning the exercises are performed in the bivalved cast. Motion of the concerned joint is executed slowly, steadily, and smoothly through as full a range as possible. Soon the limb is taken out of the cast and is properly positioned, and measures are taken to prevent stretching of the tendon out of its resting position.
Occasionally the patient is unable to contract the transferred muscle actively and has difficulty “finding” it. To enable the patient to use the transfer actively and to help in acquiring the feeling desired, the therapist may exert gentle mild tension on the transferred tendon, have the patient shift positions during attempts at active contraction, or advise the patient in the use of corollary motions. If difficulty finding the transfer persists after 2 weeks, electrical stimulation may be used to initiate contraction as the patient attempts to use the muscle. After a few sessions the patient begins to feel the transfer and to contract it voluntarily.
As soon as the patient is able to contract the transferred muscle actively, exercises in the direction of the original action of the muscle are discontinued and only motions in the new function provided by the transfer are performed.
When poor motor strength develops in the transferred muscle—that is, it can carry the part through the full range of motion with gravity eliminated—the physical therapist instructs one of the parents to perform the exercises with the child. The exercise regimen is supervised by the physical therapist and the surgeon, who check it at weekly or biweekly intervals.
Initially the limb should be retained in the bivalved cast for support, except during the exercise periods. As soon as the motor strength of the transfer becomes “fair,” use of a bivalved cast during the day is gradually discontinued. Controlled activities are permitted to develop function. These activities are permitted sooner in the upper than in the lower limb. The age and dependability of the patient are other considerations. Resistive exercises to develop power are begun whenever the transfer has a normal range of action and is “fair” in strength. It is also important to exercise the antagonistic muscles to prevent disuse atrophy.
The next stage of training is incorporation of the transfer into the new functional pattern. This is particularly important in the lower limb, in which the muscles are concerned with gait. For example, the action of a peroneus transfer may be good in that it can dorsiflex the ankle through a full range and take moderate resistance, yet during locomotion, voluntary control over the transfer may be lost and the patient may walk with a footdrop gait. The transition to walking requires diligent supervision. Of particular importance is the use of crutches, which protect the limb from undue strain and at the same time allow the patient to be taught to use the transfer and to become accustomed to it. First the patient is asked to take a single step, and the therapist ensures that the muscle contracts and dorsiflexes the ankle. As soon as the transfer functions throughout all the phases of a single step, the walking periods are gradually increased until the normal gait pattern becomes a conditioned reflex.
Orthoses should be used in the postoperative period judiciously and for specific reasons. Orthotic support protects the part and allows early activity. This is indicated particularly when paralysis is extensive, as in myelomeningocele. In a posterior transfer to the os calcis, for example, a plantar flexion–assist orthosis with a dorsiflexion stop at right angles and crutches may be used to assist in developing function in the transfers and prevent stretching. However, standing and walking exercises are also performed without the brace to stimulate function in the transfer. Prolonged use of a bivalved night cast is important to prevent the development of a contractural deformity that would oppose the action of the transfer (e.g., equinus deformity of the ankle in the setting of anterior transfer for dorsiflexion).
From the beginning, daily stretching exercises should be a part of the exercise regimen. Stretching and night support are continued over a long period until full strength has developed in the muscle and balanced function is observed between agonistic and antagonistic muscles with no tendency for recurrence of the original deformity. In fact, the use of stretching and active exercises should be a simple rule of daily living.
Arthrodesis to provide stability and correct osseous deformity may be indicated, particularly in the foot. However, if dynamic balance is established before the development of structural deformity, arthrodesis may be unnecessary. When it is necessary to combine arthrodesis with tendon transfer, muscle reeducation must be delayed until adequate bony union has taken place.
Management of the Hip
Soft Tissue Contracture
The common deformity of the hip secondary to soft tissue contracture consists of flexion, abduction, and external rotation. Several factors must be considered in its pathogenesis. During the acute and convalescent stages of poliomyelitis, the patient lies supine in the so-called frog-leg attitude with the hips flexed, abducted, and externally rotated; the knees are flexed, and the feet are in equinovarus posture. This position is assumed because of spasm of the hamstrings, hip flexors, tensor fasciae latae, and hip abductor muscles and because of the force of gravity acting on the flail lower limbs. Maintenance of the lower limbs in malposture results in permanent shortening of the soft tissues. Contracture of the intermuscular septa and enveloping fasciae occurs first. This can easily be observed at surgery. On sectioning of the contracted fasciae that cover normal muscle fibers and retraction of the cut edges of the fascia 2 to 3 cm, the underlying muscle tissue is found to be in a relaxed condition when it is elevated with tissue forceps. Partially paralyzed muscle becomes shortened because of contracture of the involved fibrosed muscle fibers scattered throughout the normal muscle tissue. Adaptive shortening of normal muscle occurs later. Structural bony deformity develops with growth in the presence of soft tissue contracture and dynamic muscle imbalance.
The iliotibial band (or tract) is the thickened lateral portion of the fascia lata; it is located along the entire lateral aspect of the thigh and extends from the greater trochanteric region to below the knee. Superiorly, the iliotibial band is attached to the iliac crest by three prongs: a middle one through the aponeurosis over the gluteus medius, an anterior one through the tensor fasciae latae, and a posterior one through the gluteus maximus ( Fig. 37-3 ).
Throughout its extent on the lateral aspect of the thigh, the iliotibial tract is continuous on its deep surface with the lateral intermuscular septum, through which it is firmly attached to the linea aspera on the posterior aspect of the femur. At the knee joint level, fascial expansions from the anterior border of the iliotibial tract join expansions that emanate from the quadriceps muscle to form the lateral patellar retinaculum. The lower end of the iliotibial band is attached to the lateral condyle of the tibia and the head of the fibula. Proximally the iliotibial band is located in a plane that is anterior and lateral to the axis of the hip joint, whereas distally in a normal limb the iliotibial tract inserts on the tibia in front of the axis of the knee joint. Irwin stated, however, that the lower part of the iliotibial tract lies in a plane posterior and lateral to the axis of the knee joint.
Contracture of the iliotibial band may contribute directly or indirectly to development of the deformities described in the following subsections.
Lower Limb
Flexion, Abduction, and External Rotation Contracture of the Hip
The shortened iliotibial band, which is in a plane anterior and lateral to the hip joint, draws the femur into flexion and abduction at the hip, with the pelvis as the fixed point. External rotation deformity results from maintenance of the malposture of the frog-leg position. The related muscles—the tensor fasciae latae, reflected head of the rectus femoris, sartorius, and external rotators of the hip—will undergo myostatic contracture if the fascial contracture is not corrected. The fixed soft tissue contracture causes anteversion of the proximal end of the femur.
Flexion and Valgus Deformity of the Knee and External Torsion of the Tibia
The iliotibial band crosses lateral to the axis of the knee. When it is contracted, a force is exerted on the lateral aspect of the joint and the tibia is gradually abducted on the femur. Its deforming action resembles that of a taut string on the concavity of an archer’s bow. Irwin proposed that flexion deformity of the knee develops as a result of the location of the band in a plane posterior to the axis of motion of the knee joint. However, subsequent studies did not support this observation. The short head of the biceps takes its origin in part from the intermuscular septum, which in turn is attached to the iliotibial band. Flexion deformity of the knee develops as a result of spasm and subsequent myostatic contracture of the short head of the biceps. Prolonged maintenance of the knee in flexion causes contracture of the patellar retinacula and soft tissues behind the knee.
External Torsion of the Tibia and Subluxation of the Knee Joint
The pull of the laterally located iliotibial band and the short head of the biceps femoris gradually rotates the tibia and fibula externally on the femur. When the contracture is not controlled, the deforming forces cause posterolateral subluxation of the knee with displacement of the fibular head into the popliteal space.
Positional Pes Varus
Positional pes varus results from an ill-fitted orthosis that fails to compensate for the external tibial torsion. The axes of the knee and ankle joints do not occupy the same horizontal plane in external torsion of the tibia. When an above-knee orthosis manufactured with these joints in the same horizontal plane is fitted to a limb with external tibial torsion, the appliance forces the foot into varus position so that the ankle is in line with the knee joint. Initially, the varus deformity is purely functional (the foot assumes normal alignment when the lateral upright of the orthosis is allowed to rotate externally on the thigh); it later becomes fixed because of permanent shortening of the soft tissues and adaptive osseous changes in the tarsal bones.
Pelvis and Trunk
Pelvic Deformity, Lumbar Scoliosis, and Subluxation of the Contralateral Hip
In abduction deformity of the hip secondary to contracture of the iliotibial band, the pelvis is level with or at a right angle to the vertical axis of the trunk as long as the affected hip is maintained in abduction; however, when it is brought parallel to the vertical axis of the body in the weight-bearing position, the pelvis is forced to assume an oblique position. This pelvic obliquity results from contracture below the iliac crest. Lumbosacral scoliosis, convex to the low side of the pelvis, simultaneously develops. The contralateral hip subluxates.
Exaggerated Lumbar Lordosis
Exaggerated lumbar lordosis is produced by bilateral flexion contracture of the hips. It is a compensatory response to the increased pelvic inclination when the trunk assumes an upright position.
Pelvic Obliquity
Fixed pelvic obliquity is a common deformity after poliomyelitis and may be caused by suprapelvic, intrapelvic, or infrapelvic abnormalities. In an extensive study conducted in Korean patients, pelvic obliquity was classified into two major types and four subtypes relative to the resultant scoliosis. In major type I, the pelvis is lower on the short-leg side and we recommended ipsilateral abductor fasciotomy and, at times, contralateral lumbodorsal fasciotomy to correct the deformity. In type II deformities, the pelvis is high on the short-limb side as a result of adduction contracture of the ipsilateral hip, abduction contracture of the contralateral hip, or ipsilateral lumbodorsal fascial contracture.
Treatment
Bivalved Casts
Static malpostural deformities of the lower limbs in the acute and subacute stages of poliomyelitis can be prevented by the use of bivalved casts to maintain the joints in neutral position. A horizontal bar in the posterior half of the cast or a rotational strap controls malrotation at the hips. The knees should be in slight flexion to prevent genu recurvatum. Passive exercises are performed to maintain full range of joint motion.
Passive Stretching Exercises
Minimal contracture of the iliotibial band can be corrected by passive stretching exercises, which follow the same steps as in the Ober test. These exercises can also be performed with the patient supine and the hip that is to be stretched hanging over the edge of the bed. In an older patient the iliotibial band can be stretched by the following exercise: the patient should stand sideways approximately 2 feet away from the wall with the hip that is to be stretched placed facing it. With the feet on the ground and the legs together, the hip is brought toward the wall to the count of 10 and is then returned to the original position. This exercise should be performed for 20 repetitions, 3 times a day.
Ober and Yount Fasciotomies
When the iliotibial band is contracted to such a degree that fixed deformity at the hip and knee with tilting of the pelvis has resulted, correction cannot be obtained by manipulative stretching or application of a series of plaster casts. The pelvis cannot be locked securely enough to permit stretching forces to be exerted on the shortened iliotibial band; instead, the pelvis is tilted into an oblique and hyperextended position, thereby stretching the lateral and anterior abdominal muscles on the side of the contracture.
Surgical intervention is the only way to correct the deformity. The shortened soft tissues must be sectioned proximally as well as distally by combining the Ober fasciotomy with the Yount procedure. As stated previously, the primary cause of the deformities is contracture of the intermuscular septa, the enveloping fascia, and the fibrosed muscular tissue in the partially involved muscles. Normal muscle tissue should not be divided.
Both lower limbs and hips are prepared and draped in sterile fashion. The Ober fasciotomy is performed through an incision that starts at the junction of the posterior and middle thirds of the iliac crest and then extends distally to the anterior superior iliac spine, where it swings posterolaterally for a distance of 10 cm. The wound flaps are mobilized to expose the sartorius, rectus femoris, tensor fasciae femoris, and gluteus medius and minimus muscles. The enveloping fascia of these muscles, the intermuscular septa, the intervening fibrosed muscular tissue, and the iliotibial band are sectioned as far back as the greater trochanter. The Ober and Thomas tests are performed to determine by palpation the occurrence of any tight bands, which are divided if present. Normal muscle tissue and the anterior capsule of the hip joint should not be divided. The contracted fibers of the Bigelow ligament can be released without entering the hip joint.
The Yount procedure consists of excision of a segment of the iliotibial band and the lateral intermuscular septum in the distal part of the thigh. A midlateral longitudinal incision is made beginning immediately above the knee joint line and extending cephalad for a distance of 10 cm. The subcutaneous tissue is divided and the wound flaps are mobilized by blunt dissection to expose the anterolateral aspect of the thigh in its distal fourth. Next a 7-cm block of the iliotibial band, the fascia lata covering the vastus lateralis muscle, and the lateral intermuscular septum are excised. It is important to divide the lateral intermuscular septum down to the femur. If it is contracted and contributing to flexion deformity of the knee, the lateral patellar retinaculum is also divided.
In severe cases with lateral rotatory subluxation of the knee, the biceps femoris muscle is lengthened by the fractional method, with extreme care taken not to injure the common peroneal nerve ( Plate 37-1 ). This lengthening can be performed through the same incision. An attempt at reduction is then made by forcibly extending and internally rotating the knee. Often, Z -lengthening of the fibular collateral ligament is necessary to achieve reduction.
Both the hip and the thigh wounds are closed routinely. Bilateral long-leg casts are applied with the knees held in full extension. Metal rings are anchored to the cast on both its anterior and posterior aspects so that the patient can be placed in suspension traction. One set of rings is placed in the distal fourth of the leg and another set in the proximal fourth. Rotational straps can be added to the plaster cast if necessary. The patient is placed on two or three half-mattresses so that the lower limbs can hang free at the edge of the mattress and the hips can be hyperextended or flexed by suspension ( Fig. 37-4 ). An infant or small child can be placed on a bent, hyperextended Bradford frame to achieve the same result. The opposite lower limb is flexed at the hip to obliterate the lumbar lordosis. The affected limb is gradually hyperextended, adducted, and internally rotated at the hip to stretch out all remaining contractural deformity. The same position of the hips can be achieved with the patient prone or supine. In patients with bilateral cases the hips are alternated several times a day. Manipulative stretching exercises are performed three times a day. Meticulous observation of circulation and sensation in the toes is imperative, especially if excessive shortening of neurovascular structures was observed at surgery.
In patients with myelomeningocele who have impaired sensation, stretching by the method described may cause pressure sores. The atrophied bones of these children may also be fractured easily by vigorous manipulations or stretching procedures.
Passive stretching by the suspension-traction method is continued for 3 weeks. As the child grows, with progressive longitudinal growth of the femur, contracture of the iliotibial band will recur unless passive stretching exercises and proper positioning of the joints in bivalved casts are continued during periods of growth.
Gluteus Medius Paralysis
When the hip abductor muscles are paralyzed, the trunk sways toward the affected side, and the contralateral side of the pelvis drops during the weight-bearing phase of gait.
Iliopsoas Muscle Transfer for Lateral Stability of the Hip
Lateral stability of the hip joint is best achieved by transferring the iliopsoas muscle from the lesser trochanter to the greater trochanter ( Plate 37-2 ). I commonly perform the Sharrard modification of the Mustard iliopsoas transfer, which involves making the hole in the ilium as far posteriorly as the nerve supply to the iliacus will allow. The importance of using a nerve stimulator while transferring the iliopsoas muscle cannot be overemphasized. The hip should be protected with crutches until the transferred iliopsoas is “fair plus” or “good” in motor strength and the Trendelenburg test is negative. During this period the patient should sleep in a bivalved hip spica cast to maintain the hip in 40 to 60 degrees of abduction. Active hip abduction exercises should be performed diligently, with the child graduating from the supine position to side lying against gravity and then to a standing Trendelenburg position.
External Oblique Muscle Transfer for Hip Abduction
The external oblique abdominal muscle can be used to restore hip abduction power. Lowman used part of the external oblique muscle and attached it to the greater trochanter with a strip of fascia lata. Thomas and colleagues transferred the entire muscle belly of the external oblique. The remaining abdominal muscles (rectus abdominis, internal oblique, and transverse muscles) maintain integrity of the abdominal wall. I have had no personal experience with external oblique muscle transfer for paralysis of the hip abductors. Physiologically, the procedure is sound; for details of operative technique, the reader is referred to the original article. In addition, the tensor fasciae latae muscle may be transferred posteriorly on the iliac crest to increase hip abduction strength.
Gluteus Maximus Paralysis
Instability of the hip and exaggerated lumbar lordosis result from paralysis of the gluteus maximus muscle. In gait, the trunk lurches backward when body weight is borne on the affected side. When the hip flexor muscles are of normal strength, increasing flexion deformity of the hip develops.
For motor evaluation of the gluteus maximus muscle, the patient is placed prone with the lower limbs hanging off the examining table. The knee is in flexion to eliminate action of the hamstrings. The patient is asked to extend the hip against gravity and manual resistance. This position also allows the examiner to evaluate the degree of flexion deformity of the hip when it is extended passively. If unable to lift the thigh against gravity, the patient is placed in a side-lying position to eliminate the force of gravity. Any abduction contracture is best determined by the Ober test because the degree of hip abduction noted on maximal extension of the hip in prone position is not as accurate.
In gluteus maximus paralysis, stability of the pelvis may be achieved by adding posterior gluteal crisscross straps between the pelvic band and the thigh band of the above-knee orthosis. An alternative method is to discard the pelvic band and fit an ischial weight-bearing quadrilateral socket to the upper thigh segment of the orthosis. Often, however, the additional support of one or two crutches is required.
Muscle transfers to restore gluteus maximus function are not always successful and should be undertaken only after considerable deliberation. Lange transferred the erector spinae muscle to the greater trochanter and used silk sutures to obtain length. Ober and Hey Groves used a strip of fascia lata to attach the erector spinae muscle to the greater trochanter.
The technique of Ober was further improved by Barr, who used a wide strip of fascia lata, including the iliotibial tract and tensor fasciae latae muscle ( Fig. 37-5 ). Contractures about the hip, such as fascia and tight intermuscular septa, are released, particularly those that are anterior and lateral to the hip joint. Complete mobilization of the iliotibial tract in addition to shift of its pull laterally to the greater trochanter removes a major deforming force. Release of contracted investing fascia about the shortened erector spinae muscle permits rotation of the pelvis to a nearly normal position and diminishes the severity of fixed lumbar lordosis.
Malrotation of the limb is prevented and corrected by transfer of the insertion of the tensor fasciae latae into the greater trochanter. Stability of the hip is provided if there is power in the erector spinae and tensor fasciae latae muscles, which act in conjunction as a digastric muscle transfer. The operation does not significantly improve the extensor or abductor power of the hip but appears to produce a more dynamic fasciodesis. Stance and gait are improved by relief of hip flexion contracture, stabilization of the hip, and relief of lumbar lordosis.
The operative technique, as advocated by Barr in 1964, is as follows.
Surgical Technique (Barr)
The patient, under general anesthesia, is placed in the lateral position with both limbs flexed 90 degrees at the hip and knee; the affected limb is uppermost, abducted, and resting on pillows. The skin of the lumbar region, buttock, and limb is prepared from the ribs to the midcalf. The operative field is draped so that the limb can be moved freely. The incision in the thigh begins just anterior to the head of the fibula and ends proximally just distal to the anterior superior iliac spine passing over the greater trochanter. The iliotibial band is exposed throughout its full length and breadth and is divided transversely at the level of the distal pole of the patella. A stout silk suture is passed through its free end, and as wide a strip of fascia as can be obtained is dissected upward and preserved as the tendon of insertion of the tensor fasciae latae muscle. Beginning at the trochanteric level, the dissection is carried toward the anterior superior iliac spine while mobilizing the distal half of the tensor fasciae latae muscle and preserving its neurovascular bundle. The intermuscular septa and other contracted fascial structures at the knee and anterior to the hip are divided as necessary while an assistant holds the hip and knee in as much extension as possible. The sartorius and rectus muscles are tenotomized if they are contracted and totally paralyzed. The iliopsoas tendon, if need be, may be divided at its insertion but should be transposed to a more proximal and anterior position in the intertrochanteric region. The anterior capsule of the hip may also be divided through the same incision if it prevents extension of the hip. The neurovascular bundle is preserved.
Subperiosteal anchorage of the fascial strip to the femur is accomplished by making two parallel longitudinal incisions, usually 5 to 6 cm long, through the origin of the vastus lateralis and the periosteum, one on the anterolateral and the other on the posterolateral aspect of the femur just below the greater trochanter, and tunneling beneath the periosteum to join the two incisions. The strip of fascia is then passed through the tunnel and secured to the periosteum by silk sutures. This must be done with the hip held in as much extension as possible, without putting undue force on the tissues and while maintaining slight abduction and neutral position with regard to rotation.
The lumbar incision is approximately 15 cm long. It is made parallel to and 5 to 8 cm lateral to the line of the spinous processes of the fourth and fifth lumbar and first sacral vertebrae. The inferior end of the incision is located medial to and about 5 cm distal to the posterior superior iliac spine. The incision is deepened through the lumbodorsal fascia, which is reflected to expose the underlying erector spinae muscle. By blunt dissection along a vertical line, the lateral two thirds of this muscle mass is mobilized and freed from the medial third, which is left attached to the adjacent spinous processes and laminae. The mobilized muscle is freed by sharp dissection from its origin to the ilium and sacrum. Because the nerve and blood supply to this muscle is segmental and enters from its ventral surface, it may be necessary to sacrifice one or two of the most distal neurovascular bundles to mobilize a 10-cm length of muscle mass.
By means of a long tendon carrier, the free end of the fascia lata is passed within the gluteal muscle compartment and enters the lumbar incision just medial to the posterior superior iliac spine. The tunnel at its point of emergence is carefully dilated by the surgeon’s finger so that the fascia can glide freely. The gliding deep surface of the fascia should be placed as it lies ventrally. With the hip held in extension, the fascia is attached, under moderate tension, to the free end of the mobilized erector spinae muscle. This is best done by laying the ventral surface of the muscle on the subcutaneous surface of the fascial strip, passing the suture in the end of the fascia through the muscle as far proximally as possible, and then fixing the edges of the fascia to the edges of the muscle flap by a series of interrupted sutures. The distal end of the muscle is thus covered on its deep surface by the fascia lata. The lumbar incision is closed in layers; it is usually possible to close the lumbodorsal fascia partially over the transplant. The thigh incision is closed in routine manner. No attempt should be made to close the defect in the fascia of the thigh. After the application of sterile dressings, the extremity is immobilized by elastic bandages and long plaster splints that extend from the ribs to the toes. The hip is immobilized in as much extension as can be obtained comfortably. No attempt is made to correct the hip flexion contracture completely at this time.
Technique for Correction of Remaining Contractures in Poliomyelitic Deformities
After 10 days to 2 weeks, when the incisions have healed, the remaining contractures are gradually stretched out. The lumbar spine and the opposite lower extremity are immobilized in a spica with that hip in sufficient flexion to obliterate the lumbar lordosis. A separate toe-to-groin cast is applied to the affected limb with the knee preferably in almost complete extension. With the patient supine the affected limb in its plaster cast is suspended from an overhead frame. The contracture can then be stretched gradually and completely by lowering the limb incrementally each day until the hip comes into hyperextension. During this procedure circulation and sensation in the toes should be watched carefully, especially if excessive shortening of the femoral vessels and nerves was observed at surgery.
If a knee flexion deformity is present, it may be corrected simultaneously by wedging the cast.
As a rule the deformity is satisfactorily corrected in 2 to 3 weeks. The apparatus is then removed and assistive muscle reeducation exercises are begun with the patient in recumbency. Underwater exercises are of value. A bivalved long spica to hold the hip in the corrected position should be worn at night for several months. Walking with crutches is permitted as soon as the transplant functions satisfactorily, usually approximately 6 weeks postoperatively. Many patients require bilateral transplants and should undergo surgery in two stages 4 to 6 weeks apart. Careful gait training is essential if the best results are to be obtained.
Hogshead and Ponseti found formation of an erector spinae flap in myelomeningocele to be difficult. The procedure was bloody and the ramifications of the meningocele sac were inadvertently entered; this resulted in troublesome drainage of cerebrospinal fluid through the wound. Because, in their experience, erector spinae transfer did not provide active power of hip extension or abduction, these surgeons recommended attachment of the distal end of the fascia lata band to the freed lumbodorsal fascia at the level of the third or fourth lumbar vertebra ( Fig. 37-6 ). They termed the operative procedure fascia lata transfer to the erector spinae. The route of the transfer should be subfascial, and its direction from the greater trochanter to the region of the posterior superior iliac spine should be as far posterior as possible.
Caution should be exercised during anterior release of soft tissue contractures of the hip. Every effort should be made to preserve viable muscles and their nerve and blood supply. To prevent anterior dislocation of the femoral head, the anterior capsule of the hip should not be sectioned. When contracture of the anterior capsule is fixed and it limits extension of the hip, the anterior capsule should be released.
In the Sharrard modification of the Mustard operation, a hole is made in the posterior part of the ilium, and the iliacus muscle is sutured to the lateral surface of the ilium (see Plate 37-2 ). The operation was designed to provide power of hip extension, as well as hip abduction. Unfortunately, the motor nerve supply of the iliacus muscle is frequently distal in its location, thus limiting the degree of posterior positioning of the iliac hole. In my experience, the Sharrard iliopsoas transfer has not been successful in providing active power of hip extension against gravity in the presence of complete paralysis of the gluteus maximus muscle. When the hamstring muscles are normal in motor strength and the gluteus maximus is only partially paralyzed, this procedure restores functional strength of hip extension and provides substantial improvement in gait.
Paralytic Dislocation of the Hip
Hip dislocation in poliomyelitis is an acquired deformity caused by flaccid paralysis and the resulting muscular imbalance that develops. In a young child when the gluteus maximus and medius muscles are paralyzed and the hip flexors and adductors are of normal strength, eventual luxation of the hip is almost inevitable. Loss of hip abductor power causes retardation of growth from the greater trochanteric apophysis. Disparity of relative growth from the capital femoral epiphysis and the greater trochanteric apophysis causes increasing valgus deformity of the femoral neck. In severe cases the angle between the neck and shaft of the femur increases to 180 degrees. Excessive anteversion of the femoral neck may also develop. When the angle between the femoral neck and the horizontal plane of the pelvis approaches 90 degrees, the hip joint becomes mechanically unstable. Under the forces of body weight, the capsule gradually becomes lax and the femoral head rides out of the acetabulum. The empty acetabulum retains adequate depth for several years after paralytic dislocation. With lack of concentric pressure of the femoral head in the acetabulum, however, progressive shallowness and obliquity of the acetabular roof develop. Thus factors in the pathogenesis of true paralytic dislocation are muscle imbalance, coxa valga, and laxity of the capsule. In treatment, it is important to remember that coxa valga precedes subluxation and shallowness of the acetabulum.
Acquired hip dislocation does not usually occur in a totally flail lower limb, particularly if the patient has been walking with the support of an orthosis. If inadequately treated, however, abduction-flexion–external rotation contracture may develop in the flail hip as a result of shortening of the iliotibial band. When the lower limbs are aligned parallel to the vertical axis of the body in the weight-bearing position, the pelvis is forced into an oblique position. The contralateral hip—the one on the high side of the pelvis—is in a markedly functional valgus position and eventually becomes dislocated. Pelvic obliquity may also result from the foregoing factors; another cause may be severe structural scoliosis in the suprapelvic region. This type of scoliosis should be distinguished from the positional scoliosis that is produced by pelvic obliquity as a result of contractural deformities below the pelvis.
Surgical Treatment
A review by Lau and associates of surgical treatment of paralytic dislocations of the hip in poliomyelitis patients demonstrated that the keys to successful reduction are restoration of muscle balance, correction of the femoral neck-shaft angle, correction of anteversion, and restoration of acetabular coverage. We also emphasize the importance of posterior acetabular coverage.
Muscle Transfer
Dynamic balance about the hip is restored by appropriate muscle transfers. If the age at onset of paralysis and muscle imbalance is younger than 2 years, iliopsoas transfer to restore power of hip abduction is performed when the child is 4 or 5 years of age. If the coxa valga deformity is less than 150 degrees, a preliminary varization osteotomy is unnecessary; the valgus deformity will correct itself with growth once hip abductor power is restored. If the coxa valga deformity is greater than 150 degrees, it is best to correct the deformity and obtain a femoral neck-shaft angle of 110 degrees before iliopsoas transfer.
If at the time of paralysis the patient is older than 2 years, iliopsoas transfer may be postponed and the stability of the hip monitored periodically with radiographs. When the coxa valga exceeds 160 degrees and the femoral head starts to subluxate laterally, varization osteotomy is performed. In patients younger than 6 years, the femoral neck-shaft angle is reduced to 105 degrees; in older patients the angle is corrected to 125 degrees. Often, if dynamic muscle imbalance persists, valgus deformity will recur with growth. The procedure should be followed in 6 months to a year by transfer of the iliopsoas.
Varization Osteotomy
The operative technique of varization osteotomy follows the same principles as those of valgus osteotomy, as described in Chapter 35 . Any adduction contracture of the hip should be passively stretched and corrected by split Russell traction, with the hips gradually brought into wide abduction. Adductor myotomy of the hip should be avoided whenever possible. The anterolateral surface of the subtrochanteric region of the femur is subperiosteally exposed, as described in Plate 37-3 . The line of osteotomy is shaped like a modified dome with a lateral buttress of cortical bone in the proximal segment to lock the upper end of the distal segment while the femoral shaft is adducted. This procedure is the reverse of valgus osteotomy. Rotational malalignment can be corrected at the same time. I use Crow pins or threaded Steinmann pins and a Roger Anderson apparatus to fix the fragments together. Other surgeons may use a bone plate with four screws, a blade plate, or two staples. It is a matter of personal preference and depends on past experience. Blundell Jones exposed the trochanteric region of the proximal end of the femur posterolaterally with the patient in the prone position and corrected the valgus deformity by excising a wedge of bone with its base medially.
When the hip is completely dislocated, the hip joint capsule is stretched out and lax. Paralytic hip dislocation is easily reduced. In the beginning the femoral head can be relocated into the acetabulum by simple abduction of the hip. Later, however, soft tissue contracture may develop, and an initial period of skin or skeletal traction is then indicated. Prolonged immobilization of the hip in a spica cast after reduction is not recommended. Once the cast is removed, the dislocation will recur. The use of a solid hip spica cast does not correct the etiologic factors, and it has the additional disadvantage of causing disuse atrophy of muscles and bone. To stimulate normal proximal femoral growth, weight bearing should be restored as soon as possible.
Reefing and repair of the capsule are essential. These procedures are described and illustrated in Plate 16-3 . An iliopsoas transfer is performed at the same time to restore power of hip abduction and muscle balance about the hip. If the acetabulum is shallow and maldirected, the procedure may be combined with a Salter innominate osteotomy.
Arthrodesis of the Hip
Fusion of the hip in poliomyelitis may increase the ability to walk and eliminate the need for orthotic support. The procedure does have serious disadvantages, however, which should be carefully considered. Sharp and colleagues reported a series of 16 hip fusions performed in children for paralysis caused by poliomyelitis. The number of fractures (8 of the femur and 1 of the tibia) was high. In addition, there were 3 cases of pseudarthrosis and 1 of slipped capital femoral epiphysis. In 3 patients the hip was fused and subsequently required correction by femoral osteotomy. One patient had marked limitation of knee motion after prolonged immobilization in the cast; in another, amputation was indicated because of excessive shortening of the limb.
A stiff hip burdens the spine and knee with abnormal stress and strain. Thus ligamentous instability of the knee, progressive lumbosacral scoliosis, and trunk instability secondary to extensive paralysis of the abdominal muscles are absolute contraindications to hip fusion in poliomyelitis. A functional quadriceps femoris is desirable but not absolutely necessary, as long as the patient has no flexion deformity of the knee and stability of the foot and ankle is provided by a strong triceps surae muscle or by pantalar arthrodesis in a 15-degree equinus position. Stability of the flail knee is achieved as body weight falls on the ball of the foot, forces the heel onto the ground, and drives the knee into hyperextension ( Fig. 37-7 ).
Hallock in 1942, 1950, and 1958 reported an enlarging series of hip fusions performed in patients with flail lower limbs as a result of poliomyelitis. At first the procedure was used only in patients with painful arthritic subluxation or dislocation of the hip or when previous reconstructive operations such as open reduction, shelf stabilization, or muscle transfers had failed. Hallock later extended his indications to include several individuals with severe hip lurch from extensive hip muscle paralysis without dislocation. He reported gratifying results: the arthrodesis relieved pain, achieved stability, and decreased the limp. Hallock recommended that optimum position of fusion to be 35 degrees of flexion, neutral rotation, and a neutral abduction-adduction position, except in female patients or when considerable shortening is present, in which case 10 or 15 degrees of abduction is advised for biologic reasons and to compensate in some measure for the inequality of leg length.
When marked shortening of the flail limb makes equalization impractical, hip fusion should not be performed. The age of the patient is another consideration; it is imperative that the patient be mature enough to understand the disadvantages of a stiff hip. Hip fusion in a patient with a paralytic flail lower limb is controversial and should be considered only after thorough and meticulous assessment of the patient.
Management of the Knee
Quadriceps Femoris Paralysis
The quadriceps is commonly affected by poliomyelitis. When the patients have slight genu recurvatum with adequate strength of the triceps surae and hamstring muscles, the knee is stabilized by locking it in hyperextension ( Fig. 37-8 ). Patients so treated are able to walk satisfactorily. During the stance phase of gait, quadriceps weakness is compensated for by tilting the trunk and center of gravity of the body forward. The only functional disabilities are difficulty climbing steps and running. In the presence of knee flexion deformity, however, the knee joint becomes unstable because it cannot be locked in hyperextension.
Muscle Transfer
Indications and Contraindications
Several muscles have been transferred to restore knee extension power, namely, the biceps femoris, semitendinosus, sartorius, tensor fasciae latae, and adductor longus. † When the hamstring muscles are normal, they can be transferred anteriorly to the patella and ligamentum patellae to provide extension and stability of the knee. This procedure is advised when instability of the knee interferes with ordinary walking or when the patient will be able to dispense with an orthosis after such a transfer. Each case, however, must be considered individually. When the hip flexors are less than “fair” in motor strength, anterior transfer of the hamstrings is absolutely contraindicated. After surgery the patient will be unable to clear the limb from the floor, and consequently the disability will be greater. The triceps surae muscle must be at least “fair” in strength; if not, with loss of all dynamic posterior knee support, marked genu recurvatum will develop. It is preferable to have adequate strength of the gluteus maximus and hip abductor muscles. Before tendon transfer, any flexion contracture of the knee and equinus deformity of the ankle should be fully corrected by wedging casts. The mechanics of the patellofemoral articulation should be normal. Any significant malalignment of the lower limb, such as marked genu valgum, should also be corrected preoperatively.
† References .
Transfer of both the biceps femoris and the semitendinosus muscle is the procedure of choice. The strength of the tensor fasciae latae and sartorius muscles is not sufficient to substitute for the quadriceps. In an electromyographic study of 21 patients with paralysis of the lower limb secondary to poliomyelitis in whom 39 muscle transfers for quadriceps paralysis were performed, Sutherland and associates reported the following results: 10 to 14 hamstring transfers achieved conversion from swing phase to stance phase activity (roughly comparable to that of the normal quadriceps femoris), and 2 of 11 sartorius transfers and 4 of 12 tensor fasciae latae transfers achieved stance phase activity.Surgical Technique
The operative technique of transfer of the biceps femoris and semitendinosus muscles, as described by Crego and Fischer and Schwartzmann and Crego, is as follows ( Fig. 37-9 ). The patient is placed supine with a large sandbag under the ipsilateral hip so that the patient is tilted 45 degrees to the opposite side and the knee to be operated on is in semiflexion. A longitudinal incision is made over the posterolateral aspect of the thigh, starting immediately above the head of the fibula and extending proximally to terminate at the junction of the proximal and middle thirds of the thigh. The subcutaneous tissue and deep fascia are incised in line with the skin incision. The common peroneal nerve, located posteromedial to the biceps tendon, is identified and gently retracted posteriorly with moist umbilical tape. The biceps femoris tendon is dissected free of its surrounding soft tissues and retracted anterolaterally. At its point of attachment to the fibular head the lateral collateral ligament adheres to the biceps tendon; great caution must be exercised to protect and not divide it. Next the biceps tendon is detached from its insertion on the head of the fibula. Using sharp and blunt dissection, the surgeon frees the muscle bodies of the short and long heads of the biceps muscle proximally as high as possible while taking care to preserve their nerve and blood supply. The new direction of the line of pull of the transfer must be as nearly vertical as possible; if the transferred tendons run horizontally, the muscles will pull the patella in a posterior direction.
Next a transverse incision is made over the anterior aspect of the knee, centered over the distal third of the patella. The subcutaneous tissue and deep fascia are divided. The wound flaps are undermined to expose the patella and patellar tendon. During a large Ober tendon transfer, a wide subcutaneous tunnel is made extending from the patella incision to the incision on the lateral aspect of the thigh. A 10- to 15-cm-long segment of the intermuscular septum and the iliotibial band is excised to allow free gliding of the transferred muscle belly.
The sandbag is next removed and placed under the opposite hip so that the patient is positioned semilaterally, turned to the ipsilateral side. A longitudinal incision is made over the posteromedial aspect of the thigh, beginning 3 cm proximal to the popliteal crease and extending to the junction of the middle and proximal thirds of the thigh. The subcutaneous tissue and deep fascia are divided. The semitendinosus tendon is isolated, and through a separate small incision over the anteromedial aspect of the proximal part of the leg it is detached from its insertion on the tibia. It is easy to identify the semitendinosus tendon in the distal leg wound by pulling on it in the proximal thigh wound; anatomically at its insertion the semitendinosus tendon is located posterior to the sartorius tendon and inferior to the tendon of the gracilis. The semitendinosus tendon is then delivered into the proximal wound and dissected free to the middle third of the thigh. Through a wide subcutaneous tunnel from the anterior transverse knee incision to the posteromedial thigh incision, the semitendinosus tendon is rerouted and delivered into the prepatellar area. Again the deep fascia is widely incised to avoid angulation and to permit free gliding of the semitendinosus tendon.
Next the prepatellar bursa is reflected and retracted to one side and an I -shaped incision is made through the quadriceps tendon and periosteum over the anterior surface of the patella. These tissues are stripped and reflected medially and laterally. With a -in drill, oblique longitudinal tunnels are made through the patella, starting at the superolateral and superomedial poles of the patella and emerging on each side of the patellar tendon. The tunnels are enlarged with progressively increasing sizes of hand drills and curets. The operator must be careful not to damage the articular surface of the patella.
With braided silk whip sutures on their ends, the biceps femoris tendon and the semitendinosus tendon are each pulled through their respective tunnels in the patella and sutured to the patellar tendon under tension. Additional interrupted sutures are placed proximally and distally to fix the biceps and semitendinosus tendons to the rectus femoris and patellar tendons. The soft tissues are sutured over the anterior aspect of the patella and the wounds are closed. A long-leg cast that holds the knee in neutral position but not in hyperextension is applied.
Postoperative Care and Functional Training
Meticulous postoperative care is important to obtain a satisfactory result. Tension on the transferred hamstring is prevented by avoiding flexion of the hip. The patient is kept supine in bed for 3 weeks, and it should be strongly emphasized to personnel that the patient is not to sit.
Functional training of the transfer is begun 3 to 5 days after surgery or as soon as the patient is comfortable. The patient is placed on his or her side to eliminate the force of gravity. The knee and hip are slightly flexed, and the patient is asked to extend the hip and knee. Active contraction of the hamstrings as knee extensors is initiated by having the patient execute his or her former action of hip extension. Active guided knee extension exercises are then performed from starting positions of greater knee flexion and decreasing hip flexion; the patient should soon be encouraged to divorce the two movements of knee and hip extension. The active exercise of knee extension is performed with the hip in the partially flexed position of the normal pattern of locomotion without, however, extending the hip.
The function of the antagonistic muscles should not be ignored. Active knee flexion exercises are carried out (through a limited range initially) while making sure that the transferred muscle is not used for both extensor and flexor functions.
As soon as the transferred muscle is “fair minus” in motor strength, the patient, while still supine in bed, is asked to go slowly through the motions of walking: namely, ankle-foot dorsiflexion and hip flexion, followed by knee extension (using the transfer), hip extension, and ankle plantar flexion. The same exercises are performed standing, first in parallel bars and then in crutches. During the stance phase, hyperextension of the knee should be avoided. A bivalved cast is worn at night for 8 to 12 months to prevent stretching of the transferred muscles. Orthotic devices to support the knee are not usually necessary unless their use is indicated for control of the foot and ankle.
Complications
Genu recurvatum is a not infrequent complication; it occurs in 10% to 20% of reported cases and is a natural consequence of an operation in which the hamstring muscles that normally provide dynamic support to the knee posteriorly are removed and transferred anteriorly. Other factors contributing to its pathogenesis are (1) pes equinus, (2) selection of patients with inadequate (less than “fair”) strength in the triceps surae muscle, (3) immobilization of the knee in hyperextension in the postoperative period, (4) lack of an adequate and diligent postoperative exercise regimen with resultant failure to develop active knee flexion against gravity, and (5) improper use of orthotic support after surgery. The development of genu recurvatum can be minimized if the preceding factors are circumvented.
Lateral instability of the knee often results from inadvertent operative division of the tibial or fibular collateral ligaments while detaching the semitendinosus and biceps tendons from their insertion.
Lateral dislocation of the patella commonly occurs when the biceps femoris alone is transferred. This complication can be prevented by transfer of both the biceps and the semitendinosus muscles.
Failure of transfer may result from denervation of muscles during proximal dissection, inadequate postoperative training, or binding down of the transfer by adhesions in sharp angular pathways to the patella.
Flexion Deformity of the Knee
Contracture of the iliotibial band secondary to the static forces of malposture of a flail lower limb causes flexion contracture of the knee, along with flexion-abduction–external rotation deformity of the hip, genu valgum, and external tibial torsion. This deformity is preventable and, if minimal, can be corrected by passive exercises and wedging casts. When it is marked, an Ober-Yount open surgical release of the contracted iliotibial band is required.
Flexion contracture of the knee may also result from a dynamic imbalance between the quadriceps femoris and hamstring muscles ( Fig. 37-10 ). As stated previously, when flexion deformity of the knee is present, paralysis of the quadriceps muscle cannot be compensated for by locking the knee in hyperextension, and the knee is then unstable. Thus it is imperative that the knee flexion deformity be fully corrected.
It is important to understand the pathomechanics of a knee that has become fixed in flexion. In a normal knee the last 5 degrees of extension is accompanied by medial rotation of the femur on the tibia, a movement that tightens the collateral ligaments and oblique posterior ligament, thus locking the knee in extension. Because the axis of knee motion passes not through the joint line but through the upper attachments of the collateral ligaments, the tibial plateau must glide forward on the femoral condyles. In fixed flexion deformity of the knee this normal gliding movement does not take place; instead, a simple rocking motion occurs. When the knee is forced into extension, the tibia subluxates posteriorly, and the knee joint becomes incongruous and painful. When correcting a fixed flexion deformity of the knee it is important to preserve joint congruity by pulling the tibial plateau forward on the femoral condyles. This is accomplished by applying skeletal traction through a pin in the proximal end of the tibia after section of the contracted iliotibial band and patellar retinacular expansions that are usually adherent to the joint capsule and that obliterate the lateral recesses. Posterior capsulotomy of the knee is not usually required.
Supracondylar osteotomy may be indicated in patients with a marked fixed flexion deformity and structural bony changes in the femoral condyles. Osteotomy is also indicated to align the lower limb when significant genu valgum persists after correction of soft tissue contracture.
Asirvatham and associates warned against the use of a proximal tibial extension and medial rotation osteotomy to correct knee flexion contracture and tibial lateral rotation deformity simultaneously. Recurrence of contracture, genu recurvatum, and peroneal palsy complicated the outcome.
Genu Recurvatum
Hyperextension of the knee in poliomyelitis may develop as a result of stretching of the soft tissues in the back of the knee, or it may be caused by structural bony changes, with depression and downward sloping of the anterior portion of the tibial plateau.
Genu Recurvatum Caused by Stretching of the Soft Tissues in the Back of the Knee
Genu recurvatum can occur in patients with extensive paralysis of the lower limb with marked weakness of the hamstrings, triceps surae, and quadriceps femoris muscles ( Fig. 37-11 ). There is frequently calcaneus deformity of the foot. With continued weight bearing, the hamstring and triceps surae muscles and the capsule and ligaments in the posterior aspect of the knee stretch and elongate. The degree of genu recurvatum rapidly increases with loss of the support normally provided by the muscles and ligaments. The functional disability is usually great; an above-knee orthosis with a posterior knee strap is often required to support the knee.
Heyman recommended the use of peroneal tendons to construct posterior check ligaments for preventing hyperextension of the knee. When the patient has associated excessive lateral instability of the knee, the collateral ligaments are also reinforced. The tendons are passed through drill holes placed superior to the epiphyseal plate at the lower end of the femur and inferior to the epiphyseal plate of the upper end of the tibia, thus avoiding any injury to the epiphyseal plate. The tendons are firmly anchored with the knee in 30 degrees of flexion. An above-knee cast is worn for 6 weeks. The knee is then further protected for 3 months in an above-knee orthosis that limits extension of the knee to 5 degrees less than neutral. In a long-term follow-up note, Heyman reported complete and lasting correction in five cases, with extension of the knee limited to a point just short of neutral. In my experience, however, under the force of body weight, the tendons and shortened soft tissues eventually become stretched and the deformity recurs. I recommend the Heyman tenodesis operation for genu recurvatum in a patient younger than 10 years, in whom osseous structural changes in the tibial plateau have not yet taken place. To prevent deformity from recurring until skeletal growth has been completed, the patient sleeps in a bivalved night cast that holds the knee in 40 degrees of flexion. For walking, the knee is held in 5 to 10 degrees of flexion in an above-knee orthosis.
Genu Recurvatum Resulting From Ankle Equinus and Hamstring Weakness
This type of genu recurvatum develops in patients with an equinus deformity of the ankle with normal triceps surae and hamstring muscles but a weak quadriceps femoris muscle. The paralyzed quadriceps muscle is unable to lock the knee in neutral extension, and on heel strike the proximal end of the tibia is forced into hyperextension with limited dorsiflexion of the ankle. With continued walking and the stress of weight bearing, the anterior portion of the tibial plateau becomes depressed and is tilted inferiorly. The bony deformity is corrected either by open-up wedge osteotomy or by close-up wedge osteotomy of the proximal end of the tibia. The procedure is usually performed at the subcondylar level distal to the proximal tibial tubercle. It is best to delay surgery until skeletal growth is completed. The technique described by Irwin is simple and satisfactory ( Fig. 37-12, A and B ). A modified dome-shaped osteotomy achieves the same result ( Fig. 37-12, C and D ).
We, however, prefer an open-up wedge osteotomy ( Fig. 37-12, E and F ). The operative technique is as follows. A curved transverse incision is made across the anterior aspect of the leg, centered 1.5 cm distal to the proximal tibial tubercle. The lateral limb of the incision is continued proximally to terminate immediately superior and posterior to the upper end of the fibula. Subcutaneous tissue and fasciae are divided in line with the skin incision, and the wound flaps are mobilized and retracted. First, the neck and 2 cm of the proximal shaft of the fibula are exposed extraperiosteally. Meticulous attention must be paid to avoiding damage to the common peroneal nerve and proximal fibular epiphyseal plate (if open). With drill holes and a sharp thin osteotome, a simple short oblique osteotomy of the proximal shaft of the fibula is performed. Often it is desirable to excise a wedge of bone from the proximal end of the fibula with its base facing posteriorly.
Next a T -shaped incision is made in the periosteum over the anteromedial surface of the proximal end of the tibia. The growing apophysis of the proximal tibial tubercle and the upper epiphyseal plate of the tibia should not be disturbed by stripping the periosteum. The level of osteotomy is immediately distal to the proximal tibial tubercle; its line is marked with a starter, and then holes are drilled through the anteromedial and lateral cortices, with the posterior cortex of the tibia left intact.
Three large, threaded Steinmann pins are chosen, and their fit in the Roger Anderson apparatus is double-checked. Starting from the medial side, the first threaded Steinmann pin is placed transversely through the distal portion of the proximal fragment. The pin should just engage in the lateral cortex of the tibia (thereby avoiding injury to the common peroneal nerve), and it should be more posterior in position, away from the proximal tibial tubercle. The second and third Steinmann pins are placed transversely through the distal fragment of the tibia 5 and 10 cm, respectively, distal to the osteotomy site. The tibia is then divided with an osteotome, leaving the posterior cortex intact. When the proximal tibial fragment is kept in maximal hyperextension by forward pull on the first Steinmann pin and by manual pressure on the anterior surface of the knee and distal part of the thigh, the leg and the distal segment of the tibia are forced posteriorly and a wedge-shaped defect is created at the osteotomy site with its base facing anteriorly. A lamina spreader may be used effectively to open up the wedge.
Osteotomes of different widths are placed into the osteotomy site to determine the size of the iliac bone graft wedges, which are taken in routine manner with both cortices intact. It is best to obtain radiographs with the proper osteotome placed at the osteotomy site to double-check the correction. The degree of angulation at the osteotomy site should be approximately 10 degrees greater than that of the genu recurvatum, and the longitudinal axis of the distal fragment of the tibia should be parallel to that of the femur. The proximal tibial fragment should be in hyperextension.
Next two iliac bone graft wedges are placed at the osteotomy site (one is medial, the other lateral to the tibial crest); they are locked in place with an impactor. The surrounding spaces are firmly packed with bone graft chips. The lateral bars of the Roger Anderson apparatus are tightened to provide additional stability to the osteotomy site. The correction obtained is then rechecked with radiographs. The wound is closed in the usual manner. The Roger Anderson apparatus is padded with petrolatum gauze to prevent its incorporation into the cast. An above-knee cast is applied with the knee in extension.
Mehta and Mukherjee reported successful use of a femoral osteotomy to correct genu recurvatum, with flattening of the femoral condyles. Deformity recurred in only one case.
Flail Knee
A flail knee is unstable ( Fig. 37-13 ). For weight bearing it requires the support of an above-knee orthosis with a drop-lock knee. With such an orthosis, the patient is able to flex the knee while sitting. Arthrodesis of the knee should not be performed in children; it is best postponed until adulthood, when the patient is mature enough to understand and assess the advantages and disadvantages of a fused stiff knee. In patients with unilateral involvement I do not recommend arthrodesis of the knee, especially if they have associated muscle weakness of the hip and foot. When both lower limbs are paralyzed, however, one limb can be supported in an above-knee orthosis and the other knee stabilized by fusion, provided the hip has normal musculature and the foot is fixed in a slightly equinus posture. The technical details of arthrodesis of the knee are described in the literature.
In a large series of patients, Men and colleagues reported good results with soft tissue releases, extension osteotomies of the femur, and a patellar bone block for genu recurvatum.
Management of Specific Deformities of the Foot and Ankle
Paralysis of the muscles acting on the foot may result in various deformities and functional disability of the foot, depending on the particular muscle or muscles involved and the strength of the remaining musculature.
Normal Physiology
Stability of the foot depends on several factors: the contour of the bones and the articular surfaces, the integrity of the ligamentous and capsular support, and the motor strength of the muscles. The combined mobility of the foot and ankle is equal to that of a universal joint. Motions of the ankle, subtalar, and midtarsal joints are related to each other. In inversion of the hindfoot, for example, the os calcis is displaced forward, and adduction and inversion of the forefoot are produced; when the hindfoot is everted, the os calcis moves backward and the forefoot is abducted and everted. When the ankle joint is plantar flexed, the hindfoot inverts, whereas in dorsiflexion of the ankle, the hindfoot everts. The foot is most stable in eversion and dorsiflexion and least stable in equinus position and inversion.
The muscles that produce plantar flexion are the gastrocnemius-soleus, flexor hallucis longus, flexor digitorum longus, peroneus longus, peroneus brevis, and posterior tibial. Dorsiflexor muscles are the anterior tibial, extensor hallucis longus, extensor digitorum communis, and peroneus tertius. The muscles that produce inversion are the posterior tibial, flexor hallucis longus, and anterior tibial; the evertors of the foot are the peroneus brevis, peroneus tertius, extensor digitorum communis, and extensor hallucis longus. The muscles that plantar flex the ankle and foot provide the force for forward propulsion of the body during locomotion. The dorsiflexor muscle group clears the foot during the swing phase of gait.
Approximately two thirds of the total musculature of the leg is constituted by the triceps surae, one of the strongest muscles in the body. It acts on the foot as a first-class lever with the ankle joint as a fulcrum. The working capacity of the triceps surae is 6.5 kg/m, whereas that of the dorsiflexors of the ankle joint is only 1.4 kg/m, or a relative ratio of 4 : 1. This gross discrepancy in muscle mass between the plantar flexors and dorsiflexors of the ankle is the result of developmental and mechanical factors. The strength of the calf muscles is a necessary antigravitational force against the elevated center of gravity of the body in the upright posture. Moreover, because the center of gravity of the human body falls anterior to the ankle joint, the triceps surae must counteract a strong rotatory component in ankle dorsiflexion. The muscles that provide lateral stability to the foot in plantar flexion are the posterior tibial and peroneals, whereas in dorsiflexion, lateral stability is provided by the action of the anterior tibial and extensor digitorum communis.
Treatment of Muscle Imbalance
Muscle imbalance produces progressive deformity. This deformity is flexible in the beginning, but with skeletal growth, fixed soft tissue and structural osseous deformity will develop. The deformities of the foot and loss of function produced by muscle imbalance are predictable. The dynamic imbalance from paralysis of the major muscle groups, the resultant deformity, and its treatment are presented in Table 37-1 .