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Limb malformation involving syndactyly, median clefts of the hands and feet, and aplasia and/or hypoplasia of the phalanges, metacarpals, and metatarsals.

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Split-Hand and Split-Foot with Mandibular Hypoplasia; Acrorenal-Uterine-Mandibular Syndrome.

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Extremely rare congenital abnormality of unknown cause. Autosomal recessive.

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At birth, the diagnosis is suspected on the clinical aspect, oligohydramnios, and intrauterine growth retardation. Features include severe ectrodactyly (split-hand, split-foot malformation) associated with genital and visceral abnormalities (uterus didelphys or unicornis, vaginal anomalies, polycystic kidney disease or hypoplastic kidneys, diaphragmatic hernia, absent lung lobes, lung segmentation defects). Scoliosis, pectus carinatum, and abnormal number of ribs and vertebral size have been reported. Severe mandibular hypoplasia and low-set ears may be additional features.

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The presence of severe mandibular hypoplasia may indicate difficult airway management; difficult tracheal intubation must be anticipated. Electrolytes and fluid intake must be appropriately balanced in patients with renal insufficiency. Spontaneous ventilation should be maintained until the airway is secured. The dosage of drugs with predominantly renal excretion must be reduced according to the degree of renal insufficiency.

Evans JA, Phillips S, Reed M, et al: Severe acro-renal-uterine-mandibular syndrome. Am J Med Genet 93:67, 2000.  [PubMed: 10861684]

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