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In utero sonographic detection of hand and foot deformities.

The in utero sonographic detection of some of the more subtle anomalies of the hand and foot is now possible with the improved resolution of the newer ultrasound equipment. Nine case reports of fetal hand and/or foot deformities are presented, with 12 total lesions. The deformities include clinodactyly, symbrachydactyly, talipomanus, talipes, and "rockerbottom" foot. The sonographic appearance of each of these is discussed.

Abnormalities, Multiple↗

The lupus hand: a new surgical approach.

Three patients with lupus erythematosus developed severe hand deformities which, over the course of a year, went on to fixed volar subluxation of the proximal phalanges with some ulnar drift. The flexion deformity at the metacarpophalangeal joints of nearly 90 degrees was not correctable passively, and the palmar skin became macerated. The articular cartilage of the metacarpal heads and proximal phalanges was well preserved and replacement arthroplasty was not justified. Contracture not only in the intrinsics but also in the long flexors was not relieved by operation on the soft tissues. A step-cut metacarpal shortening did correct the remaining intrinsic and long flexor contracture, corrected the volar dislocation, restored full metacarpophalangeal extension, and maintained a full range of flexion. Position of the bones was maintained by intramedullary Steinmann pins and crossed Kirschner wires and, in one patient, by a small screw. No recurrences developed in the follow up period of 18 to 96 months.

Arthritis↗

Congenital metacarpotalar syndrome.

Three children were presented having similar facies, musculoskeletal anomalies and a common ethnic background. All had dislocated hips, scoliosis, congenital vertical tali and hand deformities. There was a very small web space between the first and second metacarpals, extension deformities at the wrist, flexion deformities at the metacarpophalangeal joints, and at one or more proximal interphalangeal joints. In addition, 2 of the 3 youngsters had marked crowding of the teeth and a small maxillary arch. All laboratory studies were normal. The most alarming finding was the poor wound healing which averaged 48%. The wound complication rate at Shriner's Hospital in San Francisco during the identical period of time these children were treated ranged from 1.2% to 1.8%. It is believed that this represents a new syndrome, as similar cases could not be found in the literature. The orthopedist may treat these anomalies in the standard fashion but should keep in mind the propensity for poor wound healing in these patients. The common ethnic background was that of Spanish-American or Chicago families.

Abnormalities, Multiple↗

Surgical management of the hand in Freeman-Sheldon syndrome.

Freeman-Sheldon syndrome is a rare form of distal arthrogryposis characterized by craniofacial anomalies, a rheumatoid-appearing hand, and pedal deformities. The hand deformities include ulnar deviation of the fingers, camptodactyly, first web space contracture, and hypoplasia of the thumb. Because of clinical variability and rarity, there is no standard management protocol. The authors have developed a systematic method of management of the hand in Freeman-Sheldon syndrome using principles commonly applied to other complex hand problems. In 17 years, the authors have evaluated nine patients. Of 18 hands evaluated, the authors have operated on 15. Good results were achieved in five hands and fair results in 10. There were no cases of useless hands. The authors have been most pleased with the results after crossed intrinsic transfers, centralization of extensor tendons, and intrinsic release of the thumb. Although physical deformities remain, functional adaptation is generally good.

Abnormalities, Multiple↗

Triphalangeal thumbs.

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Congenital Abnormalities↗

Deforming arthritis of the hands in polymyositis.

Arthritis of the hands with erosions, periosteal calcification, and interphalangeal thumb joint instability was seen in 6 patients with polymyositis. "Overlap" features such as Raynaud's phenomenon, positive LE clot test, and positive antinuclear antibody test were present, but clinically the primary disease was clearly polymyositis. This rather unusual constellation of roentgenographic findings strongly suggests the possibility of polymyositis.

Adult↗

Familial granulomatous synovitis, uveitis, and cranial neuropathies.

A family is presented that had what is believed to be a previously undescribed syndrome of granulomatous synovitis, bilateral recurrent uveitis, and cranial neuropathies. Affected members included the proband, his brother, father, and probably the decreased paternal grandmother. Disease onset was in childhood. Each had symmetric, boggy polysynovitis of the hands and wrists, resulting in nearly identical boutonniere deformities. Hand radiography in the proband and his brother revealed no erosions or joint destruction despite more than 20 years of disease. Synovectomy specimens in the proband and his brother showed granulomatous inflammation with giant cells. Recurrent, nongranulomatous, acute iridocyclitis with visual impairment afflicted the proband, brother, and father. Apparently corticosteroid-responsive bilateral neurosensory hearing loss occurred in the proband, and a transient sixth cranial nerve palsy in his brother. All members of the family were antinuclear antibody-, rheumatoid factor-, and HLA-B27-negative. Serum angiotensin-converting enzyme levels were within normal limits in all family members. The inheritance pattern of this syndrome is most consistent with an autosomal dominant mode.

Acute Disease↗