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Objectif : Décrire la prise en charge périopératoire d'une enfant présentant l'association rare d'une myopathie à axes multiples («multiminicore» MMM) et d'une dysplasie ectodermique anhydrotique (DEA).Eléments cliniques : Une fillette de huit ans est admise pour une chirurgie orthopédique programmée des membres inférieurs. La DEA est une dermatose congénitale causée par le développement anormal des tissus d'origine ectodermique; elle entraîne des problèmes de thermorégulation (absence des glandes sudoripares), ainsi que des anomalies dentaires et maxillaires (possible difficulté d'intubation trachéale). La MMM est une myopathie congénitale rare caractérisée par une faiblesse peu ou pas évolutive des muscles proximaux. Elle peut atteindre les muscles respiratoires et être associée à une cardiomyopathie. Cette pathologie est similaire à la myopathie à axe central (central core MCC), qui est reconnue pour être à risque d'hyperthermie maligne (HM). Comme les myorelaxants dépolarisants ni les agents halogénés ne pouvaient être utilisés, nous avons choisi de combiner une anesthésie générale intraveineuse (propofol) à une analgésie péridurale lombaire. Cette association a permis une anesthésie stable, un réveil en douceur et une excellente analgésie per et postopératoire sans complications. La température per et postopératoire a été étroitement surveillée.Conclusion : L'association de ces deux pathologies nécessite de pouvoir faire le diagnostic différentiel rapide entre une hyperthermie due à l'anhydrose et une hyperthermie maligne. La prise en charge anesthésique doit permettre une anesthésie sans agents déclenchants de l'HM et une analgésie postopératoire optimale sans séda-tion résiduelle; d'où l'intérêt de l'association d'une anesthésie iv à une anesthésie loco-régionale, si l'intervention chirurgicale le permet.Purpose : To report the perioperative management of anesthesia and analgesia in a child presenting with the association of multiminicore myopathy (MMM) and anhydrotic ectodermic dysplasia (AED). Clinical features: An eight-year-old girl was admitted for elective orthopedic surgery of the lower limbs. AED is a congenital dermatosis characterized by the absence of sweating and subsequent problems with thermoregulation; in addition, maxillary hypoplasia and abnormal teeth can render intubation difficult. MMM is a rare congenital myopathy characterized by proximal muscle weakness, stable in time or with a slow and progressive evolution. It can involve respiratory muscles and be associated with severe cardiomyopathy. Moreover, MMM shares some characteristics with Central Core Disease which is known to be associated with malignant hyperthermia. Since depolarizing muscle relaxants and halogenated agents could not be used, a combined propofol-based intravenous anesthesia with lumbar epidural analgesia was chosen. This combination provided stable anesthesia, smooth recovery and excellent analgesia during and after the operation, without complications. Temperature was monitored closely during surgery and in the pos...

This preliminary study evaluated two surgical techniques for the treatment of resistant clubfoot: the posteromedial and the subtalar release. Two groups of matched patients with idiopathic clubfoot who underwent surgery between 1983 and 1995 were compared. In group 1 (12 patients, 1 8 feet), posteromedial release was performed. In this group, the results were satisfactory with a tendency to hypocorrection: 4 (22%) feet needed a secondary procedure (average follow-up: 97.8 months). Group 2 (12 patients, 17 feet) underwent complete subtalar release. The results were clinically better for group 2 (average follow-up: 38.8 months). Only 1 (5.9%) patient needed a secondary procedure. These results suggest complete subtalar release yields better postoperative correction than posteromedial release.

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Say-Barber-Biesecker-Young-Simpson's syndrome is one of the Ohdo-like syndromes. It is a very rare congenital condition that is commonly defined by its main clinical features that are blepharophimosis, ptosis, mental retardation, and delayed motor development. They are often associated with skeletal manifestations that are joint laxity, long thumbs and toes, and hypoplastic and/or dislocated patellae. To our knowledge, the available literature does not report any case where attention is drawn to management of skeletal aspect of this specific syndrome, especially surgically. We report 2 cases of SBBYS syndrome with patellar dislocation that we followed for 11 years. One case (with bilateral dislocation) was managed conservatively, and the other (with unilateral dislocation) underwent conservative and surgical treatment. Both had good functional outcome at follow-up. This experience shows that patellar abnormality in this condition can be efficiently addressed conservatively and/or surgically with satisfying results.