Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content + At a Glance Download Section PDF Listen ++ Aphalangia of the hands and feet with hemivertebrae, including pulmonary hypoplasia, congenital heart anomalies (ventricular septal defect), and dysgenesis of the urogenital tract and rectum. Death usually occurs during the first year of life. + Synonym Download Section PDF Listen ++ Aphalangia with Hemivertebrae; Aphalangia of the Hands and Feet, Hemivertebrae, and Visceral Malformations. + Incidence and Genetic Inheritance Download Section PDF Listen ++ Only a few cases have been reported in the literature. Autosomal recessive. + Clinical Aspects Download Section PDF Listen ++ Aphalangia of the hands and feet, hemivertebrae, pulmonary hypoplasia, ventricular septal defect, and dysgenesis of urogenital tract and rectum. May die of respiratory insufficiency in early infancy. + Anesthetic Considerations Download Section PDF Listen ++ Assess respiratory function (chest radiograph, arterial blood gases) and cardiovascular system (echocardiography). May have hypoplastic lungs requiring intraoperative and postoperative ventilation. Patient may have ventricular septal defects, so endocarditis prophylaxis is required. Left-to-right intracardiac shunting leads to pulmonary hypertension. Abnormalities of the spine may make extradural and intrathecal anesthesia difficult to perform. + References Download Section PDF Listen ++Johnson VP, Munson DP: A new syndrome of aphalangia, hemivertebrae, and urogenital-intestinal dysgenesis. Clin Genet 38:346, 1990. [PubMed: 2282714]