Pitfalls

• Although some authors have not recommended wrist reconstruction in these patients, it is possible to achieve elbow flexion through triceps lengthening and capsulectomy. Pins should be cut off beneath the skin to allow more prolonged retention, and ulnar osteotomy may be required in cases of severe bowing.

Diagnosis

Radial Clubhead

This patient shows complete absence of radius with the frequently associated findings of an elbow contracture combined with complete absence of the thumb. The diagnosis is radial longitudinal deficiency.

Radial longitudinal deficiency is seen in ~1 in 50,000 births. This condition is seen more often in males than females and is bilateral in almost 50% of the cases. Classification of this deformity is as follows: type I, short distal radius; type II, hypoplastic radius; type III, partial absence of radius; type IV, complete absence of radius. The type IV deficiency is the most common. Radial longitudinal deficiency occurs frequently in association with other malformations involving the cardiac, gastrointestinal, pulmonary, genitourinary, neurologic, and skeletal systems. Syndromes that involve radial longitudinal deficiency include Fanconi's anemia, the thrombocytopenia absent radius syndrome, the Holt-Oram syndrome, and the VATER association (vertebral defects, imperforate anus, tracheoesophageal fistula, and radial and renal dysplasia).

In this disorder, the preaxial muscles along the radial aspect of the forearm are severely affected or absent. A common flexor/extensor carpi radialis may be found and provides a significant deforming force. Although the ulnar artery is present, the radial artery is usually absent. The radial nerve is also absent with an anomalous branch of the median nerve often found to be a tethering structure when surgery is performed.

Surgical Management

At age 6 months the patient underwent surgery for correction of the elbow contracture as well as wrist deformity. With the patient supine on the operating table and the arm on a short-arm board, a general anesthetic was administered. A sterile tourniquet was utilized with elevation to 200 mm Hg.

The elbow was approached through a straight midline incision directly over the triceps. The ulnar nerve was identified and retracted. The triceps was Z lengthened using a step-cut technique and a posterior elbow capsulectomy was performed. This allowed passive motion of the elbow to 120 degrees of flexion. The triceps was then reapproximated using nonabsorbable sutures.

The wrist was approached through a gently curving incision, which allowed exposure of the distal ulna. A capsulotomy was performed to allow reduction of the carpal bones over the end of the ulna under direct vision. Radical radial soft tissue release was required. The nonossified lunate could be reduced on the end of the ulna without pressure. A conjoined flexor and extensor carpi radialis tendon was identified and was detached from its insertion into the radial soft tissues and transferred to the ulnar side of the wrist, where it was sewn into the extensor carpi ulnaris tendon. A single 2.0-mm Steinmann pin was advanced retrograde through the end of the ulna and out the ulnar shaft. It was then advanced antegrade into the index metacarpal to achieve radialization. The Steinmann pin was cut off and advanced beneath the skin but left prominent to allow for removal at a future date (Fig. 76—2).

Postoperative Management

The patient was placed in a long-arm cast with the elbow at 120 degrees of flexion. The cast was extended the entire length of the forearm to include the hand. Immobilization was continued for 3 months using a thermomold splint.

Radialization Wrist
Figure 76—2. (A) Postradialization anteroposterior radiograph. (B) Lateralpostradialization radiograph.

Alternative Methods of Management

Treatment of radial longitudinal deficiency may require multiple procedures. In the case presented, elbow release was combined with corrective surgery at the wrist and pollicization was performed 1 year later. Centralization is performed by aligning the third metacarpal axis with the long axis of the ulna and utilizing internal fixation for stability. In the example given, radialization was performed and may allow a more aggressive correction of the wrist deformity. In more severe cases or in patients presenting at a later age, a partial carpectomy may be required to allow reduction of the carpus onto the end of the ulna. In all cases, excessive pressure must be avoided at the level of the distal ulna to prevent growth arrest.

Some authors have advocated the use of soft tissue stretching techniques using external fixators prior to correction with either radialization or centralization. This can be of great benefit in allowing easier correction.

Although recent reports have shown the promise of vascularized epiphyseal plate and metatarsophalangeal joint transfer, the long-term growth potential of this transferred tissue is not known.

Complications

Complications related to reconstruction for radial longitudinal deficiency include infection, pin migration or breakage, recurrence ofdeformity, and a resultant shortened fore-

What Radial Tunnel SyndromeRadial Tunnel Syndrome

Figure 76—4. (A) Recurrence of radial deviation in patient 2 years postsurgery. (B) Flexion deformity recurrence in same patient.

arm. The internal fixation pin should be left in as long as possible to prevent deformity recurrence; however, frequently this pin may migrate either through the ulna proximally or metacarpal distally, causing skin irritation and eventual breakdown (Fig. 76—3).

With eventual pin removal, there may be recurrence of deformity both in the flexion and radial deviation planes (Fig. 76-4). Although this is noted clinically and radiographically, it seldom recurs to an extent requiring revision surgery. Long-term studies show that both treated and nontreated cases of radial longitudinal deficiency have a significantly short forearm with the ulna measuring 40 to 60% of the un-involved side. Excessive pressure on the distal ulna at the time of centralization/ radialization may lead to early closure of the ulnar epiphyseal plate, resulting in excessive amounts of ulnar shortening.

Further reconstructive procedures such as pollicization are deferred until at least 1 year of age and may be combined with internal fixation removal.

Suggested Readings

Bayne LG, Klug MS. Long-term review of the surgical treatment of radial deficiencies. J Hand Surg [Am] 1987;12A:169-179.

Buck-Gramcko D. Radialization as a new treatment for radial club hand. J Hand Surg [Am] 1985;10A:964-968.

Kessler I. Centralization of the radial club hand by gradual distraction. J Hand Surg [Br] 1989;l4B:37-42.

Lamb DW. Radial club hand. A continuing study of sixty-eight patients with one hundred and seventeen club hands. J Bone Joint Surg [Am] 1977;59A:1-13.

Lamb DW, Scott H, Lam WL, Gillespie WJ, Hooper G. Operative correction of radial club hand. A long-term follow-up centralization of the hand on the ulna. J Hand Surg 1997;22B:533-536.

Riordan DC. Congenital absence of the radius, a 15-year follow up. J Bone Joint Surg [Am] 1963;45A:1783.

Watson HK, Beebe RD, Cruz NI. A centralization procedure for radial club hand. J Hand Surg [Am] 1984;9A:54l-547.

Essentials of Human Physiology

Essentials of Human Physiology

This ebook provides an introductory explanation of the workings of the human body, with an effort to draw connections between the body systems and explain their interdependencies. A framework for the book is homeostasis and how the body maintains balance within each system. This is intended as a first introduction to physiology for a college-level course.

Get My Free Ebook


Post a comment