Arthrogryposis multiplex congenita (AMC) is a rare disease with multiple joint contractures. It is widely believed that bilaterally dislocated hips should not be reduced since movement is satisfactory and open reduction has had poor results. Since 1977 we have performed a new method of open reduction using an extensive anterolateral approach on ten hips in five children with AMC. The mean age at surgery was 31.5 months (17 to 64) and the mean follow-up was 11.8 years (3.8 to 19.5). At the final follow-up all children walked without crutches or canes. Two managed independently, one required a long leg brace and two had short leg braces because of knee and/or foot problems. The clinical results were good in eight hips and fair in two and on the Severin classification seven hips were rated as good (group I or group II). We recommend the extensive anterolateral approach for unilateral or bilateral dislocation of the hip in children with arthrogryposis or developmental dislocation of the hip.

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Thirty-eight patients with muscular torticollis were treated by a partial resection of the sternocleidomastoid muscle between 1970 and 1981. Clinical evaluations were performed on 35 of these patients who were followed up for 10 years (average of 11.3 years). Of 31 patients under 5 years of age at operation, good results according to Canale's criteria were obtained in 28 (90%). In four patients aged 6 years or more at operation, three showed good results. Postoperatively, massive cotton bandaging was applied with the neck in the neutral position for 3 weeks. A plaster cast, brace, or physiotherapy involving active motion training was not necessary.

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We treated 120 children between the ages of 12 and 31 months with 137 developmental dislocations of the hip and reviewed them at a mean follow-up of 14 years. We had used two-directional arthrography of all hips before reduction to evaluate the anterior, superior, and posterior portions of the limbus. Of the 137 hips, 64 had no interposed limbus in the AP view of the arthrogram, but 45 of these had an interposed anterior or posterior portion of the limbus. The hips with good stability and no interposed limbus in either AP or lateral arthrograms had excellent results by closed methods; in the other cases the results were less satisfactory. Our findings suggest that hips suitable for management by closed reduction can be identified by two-directional arthrography. Hips shown to have an interposed limbus are best managed by open reduction.

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SUMMARY The authors report the second case of a female with typical Lesch‐Nyhan syndrome. She exhibited athetoid movement, self‐mutilation, mental retardation and spasticity. Laboratory investigations revealed hyperuricaemia, hyperuricosuria and decreased erythrocyte hypoxanthine guanine phosphoribosyl transferase activity. She has normal female external genitalia and karyotype. Her parents are non‐consanǵuineous and there is no family member with gout, nephropathy or any psychoneurological disorder. To prevent self‐stimulation, it was necessary to fix the patient's upper extremities to the backrest of her wheelchair. The authors also describe an apparatus that limits elbow flexion. RÉSUMÉ Syndrome de Lesch‐Nyhan chez une fille Les auteurs rapportent le second cas d'un syndrome de Lesch‐Nyhan typique chez une fille. Elle présentait des mouvements athétosiques, une auto‐mutilation, un retard mental et une spasticité. Les recherches de laboratoire précisaient une hyperuricémie, une hyperuricosurie et une activité diminuée pour la transférase hypoxanthine guanine phosphoribosyl érythrocytaire. Elle avait un caryotype et des organes génitaux externes normaux de fille. Ses parents n'étaient pas consanguins et il n'y avait aucun membre de la famille avec une goutte, une néphropathie ou un trouble psychoneurologique. II fut nécessaire de fixer les bras de la fillette au dos du fauteuil roulant pour prévenir l'auto‐mutilation. Les auteurs décrìvent également un appareillage de limitation de la flexion des coudes. ZUSAMMENFASSUNG Eine Patientin mit Lesch‐Nyhan Syndrom Die Autoren berichten über eine zweite Patientin mit einem typischen Lesch‐Nyhan Syndrom. Sie zeigte athetotische Bewegungen, Selbstverstümmelung, geistige Retardierung und Spastik. Die Laboruntersuchungen ergaben Hyperurikämie, Hyperurikosurie und einen Mangel an Hypoxanthin‐Guanin‐phosporibosyl‐transferase. Sie hat ein normales äußeres Genitale und einen weiblichen Karyotyp. Ihre Eltern sind blutsverwandt und kein Familienmitglied leidet an Gicht, Nephropathie oder einer psycho‐neurologischen Erkrankung. Um die Selbstverstümmelung zu verhindern, war es notwending, die Arme der Patienten an der Rückenlehne das Rollstuhls zu fixieren. Die Autoren beschreiben außerden eine Vorrichtung, mit der die Beugung des Unterarms eingeschränkt wird. RESUMEN Enferma con síndrome de Lesch‐Nyhan Los autores aportan el segundo caso de una hembra con un típico síndrome de Lesch‐Nyhan. Tenía movimientos atetoides, automutilaciones, retraso mental y espasticidad. Las investigaciones de laboratorio revelaron una hiperuricemia, hiperuricosuria y una disminución en los eritrocitos de la actividad de la hipoxantina guanina fosforibosil transferasa. Tenía unos genitales externos normales y un cariotipo normal. Sus padres no eran consanguíneos y nadie en su familia padecia de gota, nefropatía ni ninguna alteración psiconeurológica. Para prevenir la áutomutilación, fue necesario fijar las extremidades superiores de la paciente al respaldo de su silla de ruedas. Los autores también de...