johnson–munson syndrome johnson–munson syndrome aphalangy, hemivertebrae and urogenital-intestinal dysgenesis is an extremely rare syndrome, described only in three siblings. it associates hypoplasia or aplasia of phalanges of hands and feet, hemivertebrae and various urogenital and/or intestinal abnormalities. intrafamilial variability is important as one sister had lethal abnormalities (potter sequence and pulmonary hypoplasia), while her affected brother was in good health with normal psychomotor development at 6 months of age. prognosis seems to depend mainly on the severity of visceral malformations. etiology and inheritance remain unknown. johnson–munson syndromeother namesaphalangy-hemivertebrae-urogenital-intestinal dysgenesis syndrome