Ulnar Deviation And Wrist Subluxation

synostosis

Ulna anlage present Forearm pronated

Progressive ulnar deviation and bowing common

Type II is most common type of ulnar clubhand.

Adapted from Dobyns JH, Wood VE, Bayne LG. Congenital hand deformities. In: Green DP, ed. Operative Hand Surgery, 3rded. New York: Churchill-Livingstone; 1993:251—548.

Type II is most common type of ulnar clubhand.

Adapted from Dobyns JH, Wood VE, Bayne LG. Congenital hand deformities. In: Green DP, ed. Operative Hand Surgery, 3rded. New York: Churchill-Livingstone; 1993:251—548.

a definable cause. Ulnar deficiency is an uncommon anomaly, and the ratio between radial and ulnar deficiency ranges from 4:1 to 10:1. There is a spectrum of ulnar deficiency with varying degrees of ulna absence, elbow instability, and ulnar deviation of the hand. The classification system is based on the amount of ulna remaining and the degree of deformity (Table 77—1). The typical deformity is a shortened forearm with a bowed radius and ulnar deviation of the hand, with digital anomalies. The elbow usually has restricted motion, and its stability is related to the presence and size of the proximal ulna. The deficient length of the ulna increases the load borne by the radius, and the radial head may subluxate or dislocate. In addition, in a type IV ulnar clubhand the radial head is fused to the humerus. The wrist and hand deformities most commonly involve the ulnar carpus (pisiform, hamate, tri-quetrum) and ulnar rays. However, radial-sided deficiencies (e.g., thumb hypoplasia) can also occur in ulnar deficiency.

The hypoplastic or absent ulna may be replaced by an unossified anlage that connects the remaining proximal ulna or humerus to the ulnar carpus and/or distal radius. This structure has no growth potential and may act as a tether to cause progressive bowing of the radius and ulnar deviation during growth. The ulnar anlage is most commonly present in type II and IV ulnar deficiency, but its incidence and constancy as deforming forces are controversial.

Nonsurgical Management

The initial treatment of ulnar deficiency is nonoperative with stretching and splinting. The parents are instructed on passive stretching of the wrist at each diaper change. A splint is fabricated to improve the alignment of the limb and prevent further ulnar deviation of the wrist. This splint is worn at nighttime so as not to inhibit daily use of the arm or interfere with the development of extremity function.

Children with congenital anomalies can adapt remarkably well to their deformities. They develop compensatory maneuvers to accomplish both simple and intricate functional tasks. The treating physician should be cautious when considering surgical intervention for anomalies like ulnar deficiency. Ample time should be spent with the child and family discussing what activities can and cannot be performed. The child should be observed performing activities, and the compensatory motion that has developed over time should be noted. The proposed surgery should not interfere with these established patterns of function in favor of cosmesis. In addition, adaptive equipment can be used to accomplish a variety of tasks and should be considered prior to surgery. When contemplating surgery, realistic goals should be discussed with the family and defined prior to the procedure.

Surgical Management

Type I ulnar deficiency does not usually require treatment as there is minimal bowing of the radius and the elbow is stable. Types II, III, and IV may require treatment to address the elbow, forearm, and wrist.

The treatment of the forearm and wrist deformity is controversial. If progressive radial bowing and ulnar deviation occur in a type II or IV ulnar deficiency, then resection of the ulnar anlage at 6 months of age is indicated. However, prophylactic excision of the anlage is no longer recommended. Loss of the ulna causes increased load borne by the radius, which can yield radial bowing and/or radial head subluxation or dislocation. Radial head excision is indicated if the radial head becomes symptomatic, although we prefer to wait until skeletal maturity. If the elbow is sta-

Radial Head Dislocation Ulnar Deformity
Figure 77—4. Clinicalphotograph of a 7-year-old girl with type IV ulnar clubhand with hand-on-flank deformity.

ble, then simple excision is performed. However, with a short ulna and unstable elbow, a creation of a forearm may be the preferred treatment with placement of the radius on the remaining proximal ulna. However, this procedure eliminates any forearm rotation and may hamper function.

In a type IV deformity, the pronated hand is often facing backward and resting on the flank (hand-on-flank deformity) (Fig. 77-4). This places the hand in a poor position for function. Corrective osteotomy to rotate the forearm and realign the elbow in a forward position is indicated. The radiohumeral synostosis is approached through a lateral incision, with protection of the radial nerve. The apex of the deformity is identified and a biplane corrective osteotomy is performed using a closing wedge to place the hand in front of the trunk, the forearm in mid-rotation, and the elbow in flexion.

The hand deformities are the major predictor of function and require individualized treatment. The goal for hand function is to achieve grasp, release, and pinch. The required surgery is dictated by the extent of the deformity, especially because both radial and ulnar anomalies can be present. Ulnar-sided syndactyly is common, and separation with web space reconstruction is commonly performed. In addition, thumb hypoplasia may be present, which limits prehensile activities. Mild thumb hypoplasia can be treated by tendon transfers, to augment function. However, severe cases require ablation and pollicization of an adjacent digit.

Suggested Readings

Blair WF, Shurr DG, Buckwalter JA. Functional status in ulnar deficiency. JPediatr Orthop 1983;3:37-40.

Broudy AS, Smith RJ. Deformities of the hand and wrist with ulnar deficiency. J Hand Surg [Am] 1979;4:304-315.

Cole RJ, Manske PR. Classification of ulnar deficiency according to the thumb and first web space. J Hand Surg [Am] 1997;22A:479-488.

Dobyns JH, Wood VE, Bayne LG. Congenital hand deformities. In: Green DP, ed. Operative Hand Surgery, 3rd ed. New York: Churchill-Livingstone; 1993:251-548.

Flatt AE. The Care of Congenital Hand Anomalies, 2nd ed. St. Louis: Quality Medical Publishing; 1994:411-424.

Johnson J, Omer GE. Congenital ulnar deficiency. Natural history and therapeutic implications. Hand Clin 1985;1:499-510.

Kozin SH, Thoder JJ. Congenital anomalies of the upper extremity. In: Baratz ME, Watson AD, ImbrigliaJE, eds. Orthopaedic Surgery: The Essentials. New York: Thieme; 1999:657-673.

Marcus NA, Omer GE. Carpal deviation in congenital ulnar deficiency. J Bone Joint Surg [Am] 1984;66A:1003-1007.

Miller JK, Wenner SM, Kruger LM. Ulnar deficiency. J Hand Surg [Am] 1986;11A: 822-829.

Schmidt CC, Neufield SK. Ulnar ray deficiency. Hand Clin 1998;14:65-76.

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Responses

  • ANJA
    Is ulnar drift deformity progressive?
    8 years ago
  • reginald
    Where is your radial head in your elbow?
    8 years ago
  • posco
    WHAT IS Ulnar deviation and wrist subluxation?
    7 years ago
  • jo headstrong
    When to do surgery for ulnar deviation?
    3 years ago
  • ryan
    How to realign ulnar deviation?
    2 months ago

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