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[Sonographic image of a form of arthrogryposis multiplex congenita].

AMC is a collective term for a group of sporadic or inherent diseases characterised by multiple non-progressive congenital joint contractures. The report demonstrates the sonographic findings of a neural form of AMC at 38 weeks' gestation. Aetiology, pathogenesis and clinical findings of this malformation are discussed.

Adolescent↗

[Clinical aspects and treatment of arthrogryposis multiplex congenita. Lower extremity].

This is a report about 25 patients of 28 AMC patients with affected lower limbs. From distal to proximal the joints are less attacked. The club-foot is most frequent. It nearly always needs operative treatment. The best results can be obtained by bony operations (exstirpation of the talus, lateral resection of the cuboid, subtalar arthrodesis). In knee joints the frequent flexion-gryposis is functionaly disturbing. It can be eliminated in most of the cases only by operation. The supracondylar extension-osteotomy has to be rejected in children in favour of a dorsal arthrolysis. Flexion-gryposis preponderate in hip joints. Conservative treatment is most often successful, when flexion is not supported by flexion-gryposis of the knee. Frog like contractures are very obstinate and need operative treatment. There exist different opinions whether open reduction of bilateral complete luxation of the hip is necessary or not. Our 3 patients with complete luxation of both hips had a satisfying function of hips after false external rotation was eliminated by osteotomy of the femur. Unilateral luxation of a hip needs open reduction even if a live in sitting position has to be expected. Our own experience is compared with reports of other authors and guiding principles for treatment are presented.

Arthrogryposis↗

Lethal autosomal recessive arthrogryposis multiplex congenita with whistling face and calcifications of the nervous system.

Three children from a sibship of four had congenital contractures, scarce facial expressions, central nervous system dysfunction, and early death. Extensive deposits of calcium compounds were found at postmortem examination of the nervous system and of skeletal muscle. The disorder in these sibs is presumably inherited as an autosomal recessive trait. The metabolic basis for the calcium deposition has yet to be discovered.

Arthrogryposis↗