Severe hypertension in an adolescent girl

rectal wall distention and anoxia. Although constipated, our patient was only significantly distended at 2 days and 2 months of age, making it difficult to account for the loss of ganglion ceils on this basis. In fact, constipation probably was the result of an initial absence (or early disappearance) of ganglion cells from the sigmoid. Although paucity of ganglion cells in the rectal wall may cause a clinical picture similar to that of Hirschsprung disease, ganglion cells in our patient were readily apparent on acetylcholinesterase staining of the initial rectal biopsy. A viral etiology is possible, although there were no suggestive inflammatory changes noted on the initial biopsy. There was also no history of exposure to neurotoxic agents capable of causing ganglion cell degeneration. After consideration of several possible mechanisms, we believe that acquired distal aganglionosis in this case may have resulted from an inapparent hypoxic event. R E F E R E N C E S