Congenital Anomalies of the Trunk and Upper Extremity

Chapter 31 Congenital Anomalies of the Trunk and Upper Extremity



Derek M. Kelly




CONGENITAL ELEVATION OF THE SCAPULA (SPRENGEL DEFORMITY) 


CONGENITAL MUSCULAR TORTICOLLIS 


CONGENITAL PSEUDARTHROSIS OF THE CLAVICLE 


CONGENITAL DISLOCATION OF THE RADIAL HEAD 


CONGENITAL PSEUDARTHROSIS OF THE RADIUS 


CONGENITAL PSEUDARTHROSIS OF THE ULNA 


CONGENITAL RADIOULNAR SYNOSTOSIS 


This chapter discusses congenital elevation of the scapula, congenital torticollis, and congenital pseudarthrosis of the clavicle, radius, and ulna. Congenital anomalies of the hand and certain other anomalies of the forearm are discussed in Chapter 79. Congenital conditions of the spine are discussed in Chapters 40, 41, and 44.



Congenital Elevation of the Scapula (sprengel Deformity)


In Sprengel deformity, the scapula lies more superiorly than it should in relation to the thoracic cage and usually is hypoplastic and misshapen. Other congenital anomalies may be present, such as cervical ribs, malformations of ribs, and anomalies of the cervical vertebrae (Klippel-Feil syndrome); rarely, one or more scapular muscles are partly or completely absent. The severity of the functional impairment typically is related to the severity of the deformity. If the deformity is mild, the scapula is only slightly elevated and is a bit smaller than normal and its motion is only mildly limited; however, if the deformity is severe, the scapula is very small and can be so elevated that it almost touches the occiput. The patient’s head often is deviated toward the affected side. In about one third of patients, an extra ossicle, the omovertebral bone, is present; this is a rhomboidal plaque of cartilage and bone lying in a strong fascial sheath that extends from the superior angle of the scapula to the spinous process, lamina, or transverse process of one or more lower cervical vertebrae. A similar osseous structure has also been reported extending from the medial border of the scapula to the occiput. Sometimes a well-developed joint is found between the omovertebral bone and the scapula; sometimes it is attached to the scapula by fibrous tissue only. A solid osseous ridge between the spinous processes and the scapula is rare.


In a morphometric analysis using three-dimensional CT, Cho et al. found that most of the affected scapulae in 15 patients with Sprengel deformity had a characteristic shape, with a decrease in the height-to-width ratio. An inverse relationship was found between scapular rotation and superior displacement; no significant difference was found in glenoid version. Cho et al. suggested that the point of tethering of the omovertebral connection, when present, may determine the shape, rotation, and superior displacement of the scapula and that three-dimensional CT can be helpful in delineating the deformity and planning scapuloplasty.


If deformity and impairment are mild, no treatment is indicated; if they are more severe, surgery may be indicated, depending on the age of the patient and the severity of any associated deformities. Because the deformity is more than just simple scapular elevation, the results of surgical treatment of Sprengel deformity can vary. The long-term function of the shoulder and cosmetic appearance must be carefully measured against the surgical risk and natural history of the deformity. A 26-year review of 22 patients with Sprengel deformity treated by either observation or surgical repair suggested that surgically treated patients had almost 40 degrees more abduction than their nonsurgical counterparts, with a subjective improvement in cosmesis.


An operation to bring the scapula inferiorly to near its normal position is ideally attempted soon after 3 years of age, because the operation becomes more difficult as the child grows. In older children, an attempt to bring the scapula inferiorly to its normal level can injure the brachial plexus.


Numerous operations have been described to correct Sprengel deformity. Green described surgical release of muscles from the scapula along with excision of the supraspinatus portion of the scapula and any omovertebral bone. The scapula is moved inferiorly to a more normal position, and the muscles are reattached. Other modifications include suturing the scapula into a pocket in the latissimus dorsi after rotating the scapula and moving it caudad to a more normal position, and avoiding dissection of the serratus anterior muscle so that mobilization is begun immediately postoperatively.


Woodward described transfer of the origin of the trapezius muscle to a more inferior position on the spinous processes. Greitemann, Rondhuis, and Karbowski recommended the Woodward procedure for patients with impaired function; for patients with only cosmetic problems, resection of part of the superior angle of the scapula was preferred. They suggested that better results are obtained with the Woodward procedure because (1) the muscles are incised farther from the scapula, which lowers the risk of formation of a scar-keloid that may fix the scapula in poor position; (2) a larger mobilization is possible; and (3) the postoperative scar is not as thick as with Green’s procedure. Borges et al. added excision of the prominent superomedial border of the scapula to the Woodward procedure. We generally prefer the Woodard procedure (see later) (Fig. 31-1).


image

FIGURE 31-1 A, Sprengel deformity (left sided) in 5-year-old boy. B, Posteroanterior radiograph shows congenital elevation of left scapula. C, Posteroanterior radiograph after Woodward procedure.


In an effort to improve function of the shoulder and the cosmetic appearance, Mears developed a procedure that includes partial resection of the scapula, removal of any omovertebral communication, and release of the long head of the triceps from the scapula. In the eight patients in whom this technique was used, average flexion improved from 100 to 175 degrees and abduction improved from 90 to 150 degrees. In two patients, hypertrophic scars formed at the curvilinear incision; this problem was eliminated by the use of a transverse incision in subsequent patients. Mears observed that a contracture of the long head of the triceps seems to represent a significant inhibition to full abduction in patients with Sprengel deformity and that release of this contracture allows increased abduction. Early postoperative active and active-assisted motion exercises of the shoulder are used to improve function.


Brachial plexus palsy is the most severe complication of surgery for Sprengel deformity. The scapula in this deformity is hypoplastic compared with the normal scapula. During surgery, attention should be directed to placing the spine of the scapula at the same level as that on the opposite side, rather than aligning exactly the inferior angles of the scapulae. To avoid brachial plexus palsy, several authors recommended morcellization of the clavicle on the ipsilateral side as a first step in the operative treatment of Sprengel deformity. This is not a routine part of surgical treatment but is recommended in severe deformity or in children who show signs of brachial plexus palsy after surgical correction. Others have suggested the use of intraoperative somatosensory evoked potentials to monitor brachial plexus function during surgical correction.





Woodward Operation




Technique 31-1




image Place the patient prone on the operating table, and prepare and drape both shoulders so that the involved shoulder girdle and the arm can be manipulated and the uninvolved scapula can be inspected in its normal position.


image Make a midline incision from the spinous process of the first cervical vertebra distally to that of the ninth thoracic vertebra (Fig. 31-2A). Undermine the skin and subcutaneous tissues laterally to the medial border of the scapula.


image Identify the lateral border of the trapezius in the distal end of the incision, and by blunt dissection separate it from the underlying latissimus dorsi muscle.


image By sharp dissection, free the fascial sheath of origin of the trapezius from the spinous processes.


image Identify the origins of the rhomboideus major and minor muscles, and by sharp dissection free them from the spinous processes.


image Free the rhomboids and the superior part of the trapezius from the muscles of the chest wall anterior to them.


image Retract the freed sheet of muscles laterally to expose any omovertebral bone or fibrous bands attached to the superior angle of the scapula.


image By extraperiosteal dissection, excise any omovertebral bone, or if the bone is absent, excise any fibrous band or contracted levator scapulae; avoid injuring the spinal accessory nerve, the nerves to the rhomboids, and the transverse cervical artery.


image If the supraspinous part of the scapula is deformed, resect it along with its periosteum; this releases the levator scapulae (if not already excised), allowing the shoulder girdle to move more freely (Fig. 31-2B).


image Divide transversely the remaining narrow attachment of the trapezius at the level of the fourth cervical vertebra.


image Displace the scapula along with the attached sheet of muscles distally until its spine lies at the same level as that of the opposite scapula (Fig. 31-2C).


image While holding the scapula in this position, reattach the aponeuroses of the trapezius and rhomboids to the spinous processes at a more inferior level.


image In the distal part of the incision, create a fold in the origin of the trapezius and either excise the excess tissue or incise the fold and overlap and suture in place the resultant free edges.


image

FIGURE 31-2 Woodward operation for congenital elevation of scapula. A, Elevation of scapula, extensive origin of trapezius, and skin incision are shown. B, Skin has been incised in midline. Origins of trapezius and of rhomboideus major and minor have been freed from spinous processes, and these muscles have been retracted laterally. Levator scapulae, any omovertebral bone, and any deformed superior angle of scapula are to be excised. C, Remaining narrow attachment of trapezius superiorly has been divided at level of C4. Scapula and attached sheet of muscles have been displaced inferiorly, and aponeuroses of trapezius and rhomboids have been reattached to spinous processes at more inferior level. A redundant fold of trapezius aponeurosis is formed inferiorly. Fold of trapezius aponeurosis has been incised, and resultant free edges have been overlapped and sutured in place. Free superior edge of trapezius also has been sutured. SEE TECHNIQUE 31-1.


(Modified from Woodward JW: Congenital elevation of the scapula: correction by release and transplantation of muscle origins: a preliminary report, J Bone Joint Surg 43A:219, 1961.)



Postoperative Care

A Velpeau bandage is applied and is worn for about 2 weeks. Active and passive range-of-motion exercises are begun.



Morcellization of the Clavicle




Technique 31-2




image Make a straight incision over the clavicle extending from 1.5 cm lateral to the sternoclavicular joint to 1.5 cm medial to the acromioclavicular joint.


image Expose the clavicle subperiosteally.


image Divide the bone 2 cm from each end, remove it, and cut it into small pieces (morcellize).


image Replace the pieces in the periosteal tube, and close the tube with interrupted sutures.


image Close the subcutaneous tissues and skin in a routine manner.



Congenital Muscular Torticollis


Congenital muscular torticollis (CMT) is caused by fibromatosis within the sternocleidomastoid muscle. A mass either is palpable at birth or becomes so, usually during the first 2 weeks. Congenital muscular torticollis is more common on the right side than on the left side. It may involve the muscle diffusely, but more often it is localized near the clavicular attachment of the muscle. The mass attains maximal size within 1 or 2 months and may remain the same size or become smaller; usually, it diminishes and disappears within 1 year. If it fails to disappear, the muscle becomes permanently fibrotic and contracted and causes torticollis, which also is permanent unless treated (Fig. 31-3).


image

FIGURE 31-3 Congenital torticollis in 14-month-old boy.


Although CMT has been recognized for centuries, its cause remains unclear. Clinical studies have shown that infants with CMT are more often the product of a difficult delivery and have an increased incidence of associated musculoskeletal disorders, such as metatarsus adductus, developmental dysplasia of the hip, and talipes equinovarus. There is a reported incidence of congenital dislocation of the hip or dysplasia of the acetabulum ranging from 7% to 20% in children with CMT. Careful hip screening and, if necessary, ultrasound evaluation are indicated.


Various hypotheses of the cause of CMT include malposition of the fetus in utero, birth trauma, infection, and vascular injury. Davids, Wenger, and Mubarak found that MRI of 10 infants with CMT showed signals in the sternocleidomastoid muscle similar to signals observed in the forearm and leg after compartment syndrome. Further investigation included cadaver dissections and injection studies that defined the sternocleidomastoid muscle compartment; pressure measurements of three patients with CMT that confirmed the presence of this compartment in vivo; and clinical review of 48 children with CMT that showed a relationship between birth position and the side affected by contracture. These findings led the authors to postulate that CMT may represent the sequela of an intrauterine or perinatal compartment syndrome (Fig. 31-4).


image

FIGURE 31-4 Pathophysiology of congenital muscular torticollis proposed by Davids, Wenger, and Mubarak, who suggested that congenital muscular torticollis may represent the sequela of intrauterine or perinatal compartment syndrome.


A palpable nodule is typically present in the affect sternocleidomastoid muscle at birth or within the first few weeks of life. The patient may also have associated plagiocephaly and facial asymmetry. The presence of the characteristic fibrotic nodule typically confirms the diagnosis, rendering further radiographic evaluation unnecessary in most cases. When the diagnosis remains in doubt, cervical spine radiographs are appropriate. Finally, some authors have advocated ultrasonography for the evaluation and management of congenital muscular torticollis.


When CMT is seen in early infancy, it is impossible to tell whether the mass causing it will disappear spontaneously. Lin and Chou reported that ultrasonography was useful in predicting which infants would require surgical treatment. Those patients in whom fibrotic change was limited to only the lower third of the sternocleidomastoid muscle recovered without surgery, whereas 35% of patients with whole muscle involvement required surgical release.


Only conservative treatment is indicated during infancy. The parents should be instructed to stretch the sternocleidomastoid muscle by manipulating the infant’s head manually. The child’s chin is rotated toward the shoulder on the side of the affected sternocleidomastoid muscle while the head is tilted toward the opposite shoulder. Excising the lesion during early infancy is unjustified; surgery should be delayed until evolution of the fibromatosis is complete, and then, if necessary, the muscle can be released at one or both ends. CMT typically resolves with a home stretching program during the first year of life. However, Canale et al. found that CMT did not resolve spontaneously if it persisted beyond the age of 1 year. Children who were treated during the first year of life had better results than children treated later, and an exercise program was more likely to be successful if the restriction of motion was less than 30 degrees and there was no facial asymmetry or if the facial asymmetry was noted only by the examiner. Nonoperative therapy after age 1 year was rarely successful. Regardless of the type of treatment, established facial asymmetry and limitation of motion of more than 30 degrees at the beginning of treatment usually precluded a good result.


Any permanent torticollis slowly becomes worse during growth. The head becomes inclined toward the affected side and the face toward the opposite side. If the deformity is severe, the ipsilateral shoulder becomes elevated and the frontooccipital diameter of the skull may become less than normal. Such severe deformity could and should be prevented by surgery during early childhood. Ideally, surgery is performed just before school age so that sufficient growth remains for remodeling of facial asymmetry while giving enough time for the growth of the structures to make surgical dissection and release easier. Many patients are first seen only after the deformities have become fixed, and the remaining growth potential is insufficient to correct them (Fig. 31-5). Nevertheless, many authors have suggested that surgical release in older children can be successful and should be attempted even if the child presents later. The clinical results are significantly less successful in children who have finished growth than in children who still have growth remaining; however, most patients have marked improvement in neck motion and head tilt, with satisfactory functional and cosmetic results.


image

FIGURE 31-5 Untreated torticollis (right) in 19-year-old man; note limited rotation and plagiocephaly.


Several operations have been devised to release the sternocleidomastoid muscle at the clavicle. Unipolar release of the muscle distally is appropriate for mild deformity. Bipolar release proximally and distally may be indicated for moderate and severe torticollis. Endoscopic release of the sternocleidomastoid muscle has been described, with suggested advantages of precise division of the muscle fibers, preservation of the neurovascular structures, and an inconspicuous scar; we have no experience with this technique and no large series have been reported.





Unipolar Release


Open unipolar tenotomy of the sternocleidomastoid muscle could be followed by tethering of the scar to the deep structures, reattachment of the clavicular head or the sternal head of the sternocleidomastoid muscle, loss of contour of the muscle, failure to correct the tilt of the head, or failure of facial asymmetry to correct. Tethering of the scar to the deep structures is more common in younger patients; therefore, the operation should be postponed until after 4 years of age.




Technique 31-3




image Make an incision 5 cm long just superior to and parallel to the medial end of the clavicle (Fig. 31-6), and deepen it to the tendons of the sternal and clavicular attachments of the sternocleidomastoid muscle.


image Incise the tendon sheath longitudinally, and pass a hemostat or other blunt instrument posterior to the tendons.


image By traction on the hemostat, draw the tendons outside the wound and superior and inferior to the hemostat; clamp them and resect 2.5 cm of their inferior ends. If it is contracted, divide the platysma muscle and adjacent fascia.


image With the child’s head turned toward the affected side and the chin depressed, explore the wound digitally for any remaining bands of contracted muscle or fascia; and if any are found, divide them under direct vision until the deformity can, if possible, be overcorrected.


image If after this procedure overcorrection is not possible, make a small transverse incision inferior to the mastoid process and carefully divide the muscle near the bone. Avoid damaging the spinal accessory nerve.


image Close the wound, and apply a bulky dressing that holds the head in the overcorrected position.


image

FIGURE 31-6 Unipolar release for torticollis. Note line of skin incision.



Postoperative Care

At 1 week postoperatively, physical therapy, including manual stretching of the neck to maintain the overcorrected position, is begun. Manual stretching should be continued three times daily for 3 to 6 months; the use of plaster casts or braces usually is unnecessary (Fig. 31-7).


image

FIGURE 31-7 Seven-year-old boy with left congenital muscular torticollis. A, Before unipolar supraclavicular release. B, After unipolar release; note scar superior to clavicle in transverse line of skin crease. SEE TECHNIQUE 31-3.

Only gold members can continue reading. Log In or Register to continue

Related posts:

Benign/Aggressive Tumors of Bone Soft Tissue Tumors Magnetic Resonance Imaging in Orthopaedics Surgical Techniques Compartment Syndromes and Volkmann Contracture Hand Infections

Stay updated, free articles. Join our Telegram channel
Tags:
Jun 5, 2016 | Posted by in ORTHOPEDIC | Comments Off on Congenital Anomalies of the Trunk and Upper Extremity

Full access? Get Clinical Tree
Get Clinical Tree app for offline access