Priapism associated with Niemann-Pick disease in a 15-year-old boy.

A 15-year-old boy presented with priapism of 46 h duration. There was no known cause of priapism detected in him. During evaluation, biopsy of the lymph node and bone marrow aspiration detected patient to be having Niemann-Pick disease. He was managed with cavernous aspiration, saline irrigation, and intracavernosal phenylephrine injection. Although priapism is associated with many known diseases, about half of the cases are of idiopathic origin. We are reporting the first case of priapism associated with Niemann-Pick disease in the literature.

Female epispadias.

Isolated female epispadias without bladder exstrophy is an extremely rare congenital anomaly. The symptoms of female epispadias are primary urinary incontinence and abnormal anatomical features. A 7-year-old girl presented with partial incontinence of urine. On physical examination, bifid clitoris and labia minora were seen. The vagina and hymen were normal. Voiding cystourethrogram showed no reflux. With the diagnosis of isolated female epispadias, single stage reconstruction of the urethra, labia minora and clitoris was performed.

Congenital Lobar emphysema.

Congenital lobar emphysema is a rare but important clinical entity which may come to the paediatrician as an acute respiratory emergency. This is defined as a post-natal over-distension of one or more lobes of a histologically normal lung. To the best of our knowledge, this condition has not been reported in Indian Medical Literature to-date. Not that the actual incidence of Congenital Lobar Emphysema has changed materially but increased clinical awareness of the possibility of the diagnosis and the widespread use of roentgenography in infants has clearly resulted in frequent recognition of this condition. The plain x-ray study is so characteristic that an immediate diagnosis can be made for further line of management.