Radioulnar synostosis in Williams-Beuren syndrome: a component manifestation.
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The object of this study was to identify the best approach for exposing the radioulnar interosseous membrane while protecting the posterior interosseous n. (PIN). Twenty paired upper limbs were used to obtain measurements of the PIN and expose the distal two-thirds of the interosseous membrane in the forearm through a dorsoradial approach. The length of the PIN from the radial head to the arcade of Frohse (AF) was 26.5 +/- 1.6 mm in males and 25.3 +/- 1.1 mm in females. The length of the PIN between the radial head and the point where the PIN exits from the supinator was 66.7 +/- 4.7 mm in males and 64.0 +/- 2.5 mm in females. The length of the PIN covered by the supinator was 44.0 +/- 0.5 mm in males and 37.0 +/- 0.5 mm in females. The distance between the point where the PIN exits from the supinator and the lateral margin of the radius was 15.0 +/- 0.9 mm in males and 14.5 +/- 0.9 mm in females. The distance between the exit point of the PIN from the supinator and the lateral margin of the ulna was 18.2 +/- 0.6 mm in males and 17.9 +/- 0.7 mm in females. The distance from the point where the most lateral branch of the PIN entered the abductor pollicis longus (APL) to the lateral margin of the radius was 3.5 +/- 0.5 mm. In 20 cadaveric upper limb, the middle and distal portions of the interosseous membrane were exposed through the interval between the extensor carpi radialis brevis (ECRB) and extensor digitorum communis (EDC) m., after the origin of the extensor pollicis brevis (EPB), and extensor pollicis longus (EPL) mm. had been elevated from the lateral margin of the radius. The present study suggests that using dorsoradial approach facilitates exposure of the middle and distal portions of the interosseous membrane.
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Until recently the problem of painful, symptomatic arthritis of the wrist secondary to congenitally incomplete separation of carpal bones has been infrequently recognized. Five patients with either excessive stress loading or trauma had eight symptomatic wrists with congenitally incomplete separation of the triquetral-lunate joint. Three of these patients had bilateral symptoms. Six of the wrists had been treated by a limited wrist arthrodesis of the triquetral-lunate joint resulting in asymptomatic wrists and improved range of motion. It appears that patients with this congenital condition poorly tolerate stress loading or trauma secondary to deficient intra-articular cartilage formation resulting in a clinical and anatomic state similar to degenerative arthritis. We suggest a limited wrist arthrodesis as definitive treatment for symptomatic congenitally incomplete separation of the triquetral-lunate joint, with possible application in incomplete separation of the other intercarpal joints.
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BACKGROUND: The hypoglossia-hypodactylia syndrome, a combination of limb hypogenesis and micrognathia is exceptionally associated with glossopalatine ankylosis and cleft palate. CASE REPORT: A twin girl from monochorionic pregnancy had hypoglossia and micrognathia with anterior maxillo-mandibular fusion, glossopalatine ankylosis and cleft palate. Her left foot was amputated distal to the talus and calcaneous; her right foot had only one phallange. The second phallange of the second finger was hypoplastic on her right hand. There was no abnormality on left hand. Her sister was unaffected. The maxillo-mandibular fusion was divided on second day. The infant was able to suckle on the tenth day. Episodes of airway obstruction occurred on cardiorespiratory recording and the patient was further monitored at home. CONCLUSION: This case suggests that the hemodynamic disorders due to multiple vascular connections from monochorionic pregnancies may have induced ischemic lesions of hypoglossia-hypodactylia. Other malformations and signs of brain stem dysfunction should be detected.