Ectrodactyly, ectodermal dysplasia, macular degeneration syndrome: a further contribution.

EEM syndrome is a rare condition characterised by ectodermal dysplasia, ectrodactyly and macular dystrophy. Additional abnormalities such as alopecia, cataract, absent eyebrows, and oligodontia may occur. We report two brothers and a sister born to consanguineous parents with EEM syndrome. EEM syndrome differs from other ectrodactly syndromes by the characteristic findings in the ocular fundus showing extensive retinochoroidal atrophy with diffuse retinal pigmentation and mild arteriolar attenuation at the posterior pole. In contrast to other ectrodactyly syndromes autosomal recessive inheritance is most likely.

Ilizarov external fixation for severely comminuted supracondylar and intercondylar fractures of the distal femur.

Our aim was to determine the clinical effectiveness and safety of Ilizarov external fixation for the acute treatment of severely comminuted extra-articular and intercondylar fractures of the distal femur. A total of 14 consecutive patients with complex fractures was treated. There were three type-A3, two type-C2 and nine type-C3 fractures according to the AO/ASIF system. The mean follow-up was 14 months. Most fractures (13) united primarily at a mean of 16 weeks. One patient with a type-IIIA open fracture had infection and nonunion. The mean range of flexion of the knee at the final follow-up was 105 degrees (35 to 130). We conclude that, in the treatment of comminuted fractures of the distal femur, the Ilizarov fixator is safe and effective in providing stability and allowing early rehabilitation.

Selective restoration of motor function in the ulnar nerve by transfer of the anterior interosseous nerve. An anatomical feasibility study.

BACKGROUND: Proximal ulnar-nerve lesions have an unfavorable prognosis. The goal of the present study was to evaluate the feasibility of selective restoration of motor function of the ulnar nerve by the transfer of the anterior interosseous nerve or one of its branches to the motor branch of the ulnar nerve. METHODS: Ten cadaveric arms were used in the present study. The ulnar nerve and its motor and sensory branches as well as the anterior interosseous nerve and its branches were dissected. The widths of the motor branch of the ulnar nerve and the anterior interosseous nerve and its motor branches as well as the relevant distances from the points of divergence were measured. The axons were counted, and the distances from the end of the main anterior interosseous nerve, its motor branches, and the motor branch of the ulnar nerve to the level of the dorsal sensory branch of the ulnar nerve were measured. RESULTS: Our results indicate that the length, width, and number of axons of the branch of the anterior interosseous nerve to the pronator quadratus make it suitable for transfer to the motor branch of the ulnar nerve. The use of the main anterior interosseous nerve or its motor branches to the flexor pollicis longus and the flexor digitorum profundus is less feasible because of the need to graft a long segment and the longer distance from the level of transfer to the motor end points. CONCLUSIONS: The findings of the present study confirm the feasibility of motor-nerve transfer for reconstruction after an injury of the ulnar nerve. Nerve-grafting would be needed for injuries distal to the level of the dorsal sensory branch of the ulnar nerve.

Osteoid osteoma of the carpal bones.

Osteoid osteoma is a benign bone tumor that rarely localizes in the hand or the carpal bones. We report two cases of osteoid osteoma localized in two different carpal bones. Unremitting wrist pain was a major clinical symptom. Surgical treatment including excision of the nidus was dramatically curative. In young patients, osteoid osteoma should be considered in the differential diagnosis of chronic wrist pain.

Preliminary traction as a single determinant of avascular necrosis in developmental dislocation of the hip.

The role of preliminary traction before closed reduction in the treatment of developmental dysplasia of the hip has been questioned by many authors lately. However, the studies advocating or opposing the use of this treatment modality include several other parameters besides traction. Thus, it is unclear whether the affection is the result of preliminary traction or concurrent variables such as the "human position." This study aimed to put forward the effect of preliminary traction as a single determinant of avascular necrosis. We had two groups of patients who had developmental dislocation of the hip. The first group consisted of 52 patients treated with preliminary traction before closed reduction and the other group comprised 40 patients treated with closed reduction without preliminary traction. Both groups were similar in age, gender, side, level of dislocation, and method and duration of immobilization. Three patients from the first group developed avascular necrosis, which was not statistically significant. Preliminary traction did not affect the rate of avascular necrosis.