Advances in medical and surgical care over the past 20 years have resulted in children who formerly would have died at birth or in infancy now surviving into adulthood, many with significant, permanent physical disabilities, including those due to cerebral palsy (CP). Increased awareness of these problems is needed by adult health care providers, who will be providing direct care to these individuals, and also by pediatric providers, who may be able to anticipate and prevent some of the long-term problems. This article reviews the common medical-surgical problems and their management in adults with CP. In addition the life experience of a 44 year-old with CP is described from a personal perspective.
Advances in medical and surgical care over the past 20 years have resulted in children who formerly would have died at birth or in infancy now surviving into adulthood, many with significant, permanent physical disabilities, including those due to cerebral palsy (CP). Increased awareness of these problems is needed by adult health care providers, who will be providing direct care to these individuals, and also by pediatric providers, who may be able to anticipate and prevent some of the long-term problems. The primary condition of CP, by definition, is non-progressive over time. Secondary conditions are those that develop as a result of the primary condition and include entities such as soft tissue contractures, degenerative arthritis, and equinovalgus foot deformities. These conditions can be prevented with early diagnosis and appropriate intervention before problematic sequelae. Associated conditions are those that occur with increased prevalence in individuals with CP, such as visual or auditory impairment, learning disability, and gastroesophageal reflux. These conditions are not necessarily preventable, but their impact may be lessened by early diagnosis and intervention during the developmental years. Comorbid conditions are those unrelated to the primary disability and appear with a similar frequency whether one has CP or not (eg, diabetes, hypertension). In the authors’ experience, too often medical care providers blame the primary condition for just about all the symptoms and problems that can develop in the adult with CP. Symptoms such as leg pain, discomfort in the lower back region, and headaches are too often misattributed to the underlying condition of CP, giving no further pursuit to more specific and definitive diagnosis. For example, a person with CP presenting with a headache may be erroneously told that “all people with CP develop headaches at some point in time,” with no additional diagnostics being offered. Strauss and colleagues in reviewing the public health record for the State of California reported an up to 9 times higher risk of brain cancer in people with CP both young and old. As with any evaluation of an individual presenting with medical or surgical symptoms, the main initial goal should be to establish a correct diagnosis. This aim will be less frequently achieved if all loss of function and medical symptoms in individuals with CP are too easily attributed to the primary condition of CP.
Adults with CP, along with other individuals maturing with developmental conditions, are living longer, associated with improvements in medical and surgical care for all. Estimates of the number of adults with CP in the United States have ranged between 400,000 and 500,000, depending on the defined age of an adult. With aging comes an increase in the incidence of many conditions that can result in pain and significant loss of function over time. In addition to a possible higher risk of brain cancer in people with CP, Strauss and colleagues also reported a 3 times higher risk of breast cancer and up to 4 times increased risk of cardiovascular death. Most of this higher risk is thought secondary to inadequate medical screening in the adult with CP. Inadequate screening in part relates to lack of education of the medical provider, undersized and inaccessible medical examination rooms and equipment, and not enough time being allotted to the provider for adequate history taking and physical evaluation. Communication barriers are especially significant for those adults who are nonverbal, require augmentative communication devices, or have expression of pain that is not recognized by the busy clinician. It is not uncommon to see adults with CP surviving well past 60 years of age and maintaining a functional lifestyle, with or without caregiver assistance. Higher survival has been found in those adults with increased functional levels, ambulatory and with mat mobility, and in individuals with gastrostomy-tube feeding. Rimmer was one of the first investigators to report that regular exercise improves functional status, decreases the level of required assistance, and reduces the incidence of secondary conditions in people with disability. Heller and colleagues subsequently reported that exercise participation and frequency depended mostly on the care provider’s attitude; if the care provider felt that exercise was important to the individual with physical disability, then exercise occurred.
A major premise in the care for the adult with CP is that major functional deterioration is almost always secondary to something other than the primary condition. Multiple diagnoses always need to be considered. It is not uncommon to have an adult with CP who also develops multiple sclerosis, Parkinson disease, Alzheimer disease, depression, cerebrovascular accident, or other associated or comorbid conditions. In the primary author’s opinion, primitive reflexes, as well as a gradual trend toward more dystonia, may be more noticeable with aging in this population.
Adults with cerebral palsy: common medical-surgical problems and their management
Spasticity
Spasticity persists beyond growth and development as a common threat to optimal function in the adult with CP. Botulinum toxin continues to have a significant role in management, relaxing hypertonic muscles for functional gain in the absence of fixed contractures. Botulinum toxin can be particularly helpful for those individuals with dystonia, either proximal or distal, in relieving painful spasms, improving vertical posture, and maximizing functional gain. Adults with CP may benefit from intrathecal baclofen, more commonly but not exclusively those of nonambulatory status. It is not uncommon to see catheter tip placements as high as the mid-cervical spine in individuals with more dystonia or upper extremity involvement. Medical management of spasticity is addressed in further detail in the article by Damiano and colleagues in this issue. As noted by those investigators, most of the literature on the use of antispasticity medications in children is extrapolated from research on adult populations.
Orthopedic
Spine
Scoliosis can be present in up to 60% of adults with CP, and is particularly likely in those with nonambulatory status with spastic quadriparesis. Progression with aging can occur at approximately 1° per year and should be monitored carefully over time. Progression can be accompanied by loss of function. Pain, when it occurs, is often associated with thoracolumbar soft tissue strain on the convex side and degenerative changes in facet joints on the concave side. Custom-molded seating or postural thoracolumbar orthoses can provide relief, along with other conservative measures such as episodic physical therapy and nonsteroidal anti-inflammatory drugs (NSAIDs). Scoliosis-related pain can be a new experience for the adult with CP, as scoliosis in children with CP is generally without pain.
Spondylolysis is thought to be an acquired condition related to a stress fracture through the pars interarticularis resulting from repetitive hyperextension. The prevalence of spondylolysis has been estimated at 4.4% at 6 years of age, increasing to the adult rate of 6% at 14 years of age. With one exception, a defect in the pars interarticularis has never been identified at birth. Spondylolisthesis can be associated with spondylolysis, the development of which is felt to be infrequent after the age of 6 years in able-bodied children. Reports in the literature have identified spondylolysis in weight-bearing adults with CP with an estimated prevalence between 21% and 30% in patients with or without dystonia. The prevalence may be higher in individuals of status post selective posterior rhizotomy and with associated increased anterior pelvic tilt. In a series of 143 patients who had never walked, in which the condition of CP was predominant, no case of spondylolysis or spondylolisthesis was detected radiographically. Dystonic movements in the lumbosacral spine, particularly into extension and axial rotation, appear to be contributing to the higher incidence of spondylolysis in patients with CP. It is not uncommon in the primary author’s experience to see adults with CP having chronic back pain followed by their primary care providers for years. The back pain has usually been attributed to their primary condition of CP, believed to be expected and usual, and requiring no further diagnostic evaluation. Simple radiographs of the lumbar spine, including an oblique view, often reveal spondylolysis with low-grade spondylolisthesis, not uncommonly improving with basic conservative care. Symptoms typically improve within 6 months of treatment, including pelvic stabilization exercises, core strengthening, activity limitations, NSAIDs, and episodic use of a lumbosacral corset when helpful. Efforts to minimize significant anterior pelvic tilt in weight-bearing children may be helpful in preventing these potential stress fractures later in life, particularly in those undergoing selective posterior rhizotomy or aggressive hamstring lengthening, especially in the presence of tight hip flexor muscles. Botulinum toxin injections into painful dystonic lumbar paraspinal muscles may be helpful in minimizing some of the extension and axial rotation that can contribute to future spondylolysis. Gait aids and appropriate intermittent use of power mobility may provide additional protective factors. Careful monitoring through serial radiographs of the lumbar spine in those individuals with increased risk can allow early detection and intervention as appropriate. Surgical options including segmental fusion in the presence of failed conservative intervention, and any neurologic compromise should be used when necessary. Efforts to minimize toe walking should be provided, with use of appropriate orthoses when indicated. Symptomatic prestress fractures of the pars interarticularis also need to be considered and nuclear medicine bone scans may assist diagnosis. Medical history should include a review of any falls or injuries to the lumbar and pelvic regions, as more distant traumatic etiological factors may not be considered relevant by the individual at the time of medical evaluation.
Cervical stenosis has been found to occur with much higher incidence in adults with CP and athetosis than in normal controls. Harada and colleagues studied 180 patients with CP and athetosis in comparison with 417 control subjects, and found an 8-fold increased frequency of early cervical disc degeneration and a 6- to 8-times increased frequency of listhetic instability in the mid-cervical spine in the individuals with athetoid CP. The combination of disc degeneration and listhetic instability with narrowed spinal canal was felt to predispose these individuals to rapid progressive loss of function and devastating neurologic deficit. Functional deterioration was also noted by Ando and Ueda in approximately 35% of adults with CP, with a higher frequency among those with involuntary movements of the head and neck. Symptoms can include neck pain with loss of ambulation, progressive hypertonicity, and loss of bladder control and upper extremity function, occurring sometimes over a 6- to 18-month period. Additional studies focused on adults with CP and athetosis have associated higher incidence of cervical spondylosis and myelopathy, with dystonic head and neck postures.
Serial magnetic resonance imaging scans every 2 years in individuals with higher risk, beginning in young adulthood, may facilitate early identification of cervical spondylosis and stenosis, allowing for more pro-active intervention and prevention of sequelae. Botulinum toxin injections may be helpful in minimizing cervical dystonia, particularly excessive movements into extension and axial rotation, along with improving posture and facilitating the fit of orthotic devices. Medications for control of dystonia should be considered, including intrathecal baclofen therapy in carefully selected individuals. More calm environments and use of sensory biofeedback techniques and stress reduction strategies may also be helpful in reducing some regional dystonia. The primary author can recall a patient who, when flying alone in her glider plane, was completely relieved of all her dystonic symptoms until touchdown, when the ground support staff would come to her assistance. Cervical discomfort of any sort should be taken seriously in this population, as it may be the only prodrome recognizable before more devastating neurologic compromise. Serial neurologic examinations adapted for individuals with CP are also encouraged. Reproducible voluntary motor functions measured over time, along with a clinically reproducible spasticity measure, are suggested. Close monitoring of bowel and bladder functions for any changes, such as frequency, urgency, retention, and incontinence are not to be neglected. If conservative care fails, surgical decompression of the stenotic cervical canal may be required. A trend toward an anterior approach with interbody fusion and posterior wiring has been noted in the literature. High risks of such surgery include regional dystonia postoperatively in the surgical zone, potential for aspiration, bleeding, and limited options for use of immobilization devices. Nonetheless, cervical stenosis associated with serious functional loss over time seems to be rapidly progressive in this population of patients with dystonic CP. Surgical intervention, despite the high risks, seems warranted when conservative care has failed to maintain function and comfort. Early identification and intervention should prevent potentially catastrophic sequelae of cervical stenosis in adults with CP and cervical dystonia, or at least minimize the surgical intervention required.
Hip
Hip displacement occurs in approximately 1% of patients with spastic hemiplegia, 5% with diplegia, and up to 55% in those with quadriplegia. Pain with degenerative arthritis and joint space incongruity can occur in at least 50% of individuals with CP having dislocated hips or pseudo-acetabulum formation over time. This problem is of particular concern in individuals having weight-bearing function in the lower extremities. Weight bearing can be limited, but important to the person functionally with standing pivot transfers, standing table usage on a regular basis, or during household or community ambulation or crawling. Pain and osteoarthritic changes can result in loss of functional weight bearing and mobility that is progressive over time. Early identification of hip dysplasia and intervention in the younger child should lead to prevention of significant hip subluxation/dislocation and pseudo-acetabulum formation in many individuals. Nonetheless, the painful arthritic hip, with or without dysplasia, in the adult with CP is not uncommon. Intra-articular injections with long-acting steroid and anesthetic can provide relief in the dysplastic, dislocated, or osteoarthritic hip for 6 months or longer. This procedure is often done under fluoroscopy, with previous arthrogram to identify needle placement, to assure optimal drug placement and disbursement throughout the painful bony interfaces. These injections can be combined with phenol injections to the obturator nerves to improve hip abduction and shift articulating surfaces to those with more cartilaginous cover. Periarticular botulinum toxin to address the painful adult hip can relieve additional tension myalgias and spasms, adding further relief. Total hip arthroplasties have been reported as safe and effective for selected individuals with CP having severe degenerative arthritis and pseudo-acetabulum formation. Long-term follow-up studies have shown 94% pain relief and improved function over time even when operated on at a relatively young age of 30 years after hip arthroplasty. Wear and tear seems to be minimal, which may relate to fewer steps per day and over time in the adult with CP. Proximal femoral resection-interposition arthroplasty may be helpful in individuals having no weight-bearing function in the lower extremities. This is often a secondary procedure when more conservative care, such as intra-articular steroid, Botox, or phenol injections have not provided sufficient relief. The question of whether crawling is used for functional household mobility should be answered before surgical intervention, as most individuals will not offer this information on their own. The primary author has observed an individual of nonambulatory status but able to crawl within his home, with a painful dislocated osteoarthritic hip. This individual, having never been asked about crawling, had a proximal femoral resection performed at an outside institution. This operation eliminated his ability to crawl, resulting in the need to move out of his home and enter institutional care. The need to question adult individuals with CP regarding crawling behavior cannot be overemphasized. Self-injurious behavior must also be assessed pre- and postoperatively, as individuals can scratch their surgical incisions and disrupt traction units and immobilization devices if this problem is not carefully managed. Pain control needs to be carefully assessed, especially in those individuals with limited communication skills and variations of expression. Nonetheless, end-stage hip disease in functional weight-bearing adults with CP is virtually certain to result in loss of gait and mobility. In this scenario, total hip arthroplasties may be appropriate despite documented inherent risks and complications.
Knee
Patella alta is a relatively uncommon condition in ambulatory adults with CP, especially with spastic diplegia. It is commonly associated with anterior knee pain in pre-adolescence or adolescence, with progression over time. An Insall ratio generally greater than 1 is observed on lateral radiographs. The Insall ratio is determined by dividing the length of the patellar tendon (measured from the posterior surface of the lower pole of the patella to its insertion on top of the tibial tubercle) by the greatest diagonal length of the patella with the knee in 30° of flexion. The ratio should be approximately 1 with less than 20% variation. The condition is commonly seen with crouch gait, limiting ambulation distance and contributing to further biomechanical and lever arm dysfunction on gait analysis. Stress fractures may occur at the inferior pole of the patella, with palpable tenderness, requiring excision in the failure of conservative care. Subluxations and dislocations of the patella are additional complications.
Medical and surgical efforts to minimize crouch gait during the developmental and pre-adolescent years help prevent problems in adults with CP. These procedures include maximizing the knee-ankle-foot extension couple along with hamstring, quadriceps, and hip flexor stretching and strengthening muscles involved in weight bearing and gait. Excessive tightness of the rectus femoris muscle, in particular, can contribute to the develop and persistence of patella alta. In the primary author’s opinion, more focus on quadriceps stretching in children with CP may be helpful in minimizing patella alta in adulthood. Increased prone-lying exercises and abdominal strengthening, minimizing anterior pelvic tilt, should additionally be beneficial. The young and middle-aged adult may benefit from patellar taping techniques to keep the patella more midline and tracking within the trochlear groove. Insall and colleagues noted that clinical outcomes seemed to correlate more with patellar congruence than with severity of chondromalacia at the time of operation. Neoprene patellar tracking orthoses may provide additional reduction of symptoms in this regard. Intra-articular injections with a longer-acting steroid and anesthetic can provide more immediate relief, sometimes lasting 6 months or longer. The primary author has also used botulinum toxin A injections to the distal quadriceps mechanism, helping to relax somewhat the muscle adjacent to the superior patella. Injections are followed by a myofascial technique to lower the patella to a more inferior position closer to the center and midline of the knee joint. Physical therapy and NSAIDs can be of additional help as part of an overall conservative care program. With the failure of conservative care in the more skeletally mature individual with CP and progressive crouch gait, more aggressive surgical options should be considered. Such options can include multilevel operative interventions to correct femoral and tibial torsion, equinovalgus foot deformities along with distal femoral extension wedge osteotomies, patellar and tibial tubercle advancements, hamstring lengthening, and rectus femoris transfers. Close monitoring of patella position over the developmental years, including a focus on preventative strategies as discussed earlier, may well prevent symptomatic patella alta later in life and the need for more aggressive multilevel orthopedic surgery.
Neurogenic Bladder
Neurogenic bladder does exist in CP and can be seen in up to 15% of the population. It seems to be more common in those with bilateral involvement, with an equal prevalence in males and females. Symptoms of frequency and urgency are most common, often associated with a hyperreflexic bladder of smaller volume. Anticholinergic medications can often relieve symptoms and provide continence in the absence of urinary retention. In the primary author’s experience, the Mitrofanoff procedure (appendicovesical conduit), with or without bladder augmentation, can provide continence and social confidence in carefully selected individuals with urinary retention, eliminating the need for indwelling catheters. For urinary retention secondary to detrusor sphincter dysynergia, the primary author has also successfully injected Botulinum toxin A into the pelvic floor musculature, relieving hypertonicity and outflow obstruction, sometimes repeated as an office procedure every 4 to 6 months. This procedure can facilitate urinary drainage in the absence of catheterizations. Upper tract pathology (hydronephrosis or vesicoureteral reflux) is rare in the adult with CP in the absence of urinary retention. Medical providers should be asking more questions regarding bladder function in individuals with CP across the lifespan. This topic is often not raised by the patient, family, or other care providers, possibly secondary to a false assumption that urinary incontinence is just “part of living” with the primary condition of CP.
The conditions that have been discussed here are not uncommon in the adult with CP and should be anticipated by the specialty medical provider. Additional conditions specific to the adult with CP are becoming more recognized, but further discussion at this time is beyond the scope of this article. It is critical that the reader recognize that medical providers must not attribute every ache, pain, or loss of function presenting in the adult with CP to the primary condition of CP. Additional diagnostic inquiry is necessary as often secondary, associated, or comorbid conditions may be present and may be amenable to intervention. Practitioners focusing on children with CP need increased awareness of these problems in adults as interventions during childhood and adolescents may be helpful in prevention.
Identifying adult specialty providers and clinics can be difficult and resources vary geographically. There are few adult providers who have pursued training in conditions of childhood onset and few pediatric providers who are knowledgeable about the secondary, associated, or comorbid conditions manifesting in adulthood. Perhaps teaming of interested and capable pediatric and adult providers will facilitate the transition of youth with CP to adult providers. Networks and data banks of adult providers and clinics are being formed through organizations like the American Academy of Cerebral Palsy and Developmental Medicine and United Cerebral Palsy to help facilitate adult care and outcomes.
Related posts:
New Clinical and Research Trends in Lower Extremity Management for Ambulatory Children with Cerebral Palsy
Bone Density in Cerebral Palsy
Access to Employment and Economic Independence in Cerebral Palsy
Clinical Applications of Outcome Tools in Ambulatory Children with Cerebral Palsy
Understanding Function and Other Outcomes in Cerebral Palsy
Establishing Access to Technology: An Evaluation and Intervention Model to Increase the Participation of Children with Cerebral Palsy
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