CASE REPORT A. K. Bangroo Æ Shashi Tiwari Æ Ramji Khetri Mohit Sahni Congenital pouch colon with prune belly syndrome and megalourethra Accepted: 24 January 2005 / Published online: 3 May 2005 Ó Springer-Verlag 2005 Abstract Congenital pouch colon (CPC), an unusual high anorectal malformation in which a varying length of colon is replaced by a dilated pouch, has appeared in a number of case reports and series published in the English literature. There is only one case report of its association with prune belly syndrome (also known as triad syndrome, Eagle—Barrett syndrome, and abdom- inal muscle deficiency syndrome). We report the description and management of this rare association in a male neonate with CPC, prune belly syndrome, and congenital megalourethra. Keywords Congenital pouch colon Æ Prune belly syndrome Æ Megalourethra Introduction Prune belly syndrome (PBS) is a well-documented triad of abdominal wall deficiency, urinary tract anomalies, and cryptorchidism [1]. Skeletal, cardiac, and gastroin- testinal tract abnormalities have been reported in association with this syndrome [2]. Errors of fixation and rotation are the most common gastrointestinal tract anomalies associated with it [3, 4]. Although some cases of anorectal agenesis with PBS are on record [5, 6], the English literature so far contains only one case of pouch colon in association with PBS [7]. Recently we observed a very rare association of pouch colon, megalourethra, and PBS in a full-term male neonate born to a multip- arous mother. Case report A term male neonate, born via normal vaginal delivery to Indian parents after an uneventful pregnancy, was brought to our hospital at 20 h of life because of an absent anus and passing of meconium-stained urine. The baby was pink and active, with a birth weight of 2.8 kg. His abdomen was flabby, with visible loops of intestine and visible peristaltic movement. Two lumps were seen, one over the left hypochondrium and another over the hypogastrium. The lump over the left hypochondrium was 5 and 4 cm in its maximum horizontal and vertical dimensions, respectively. It was cystic in consistency, transilluminant, and ballotable. The lump over the hypogastrium was 4 cm in its maximum horizontal dimension; its lower pole was not felt; and its upper pole was 6 cm from the symphysis pubis. This swelling was firm and well defined, and it decreased in size when pressure was applied over it, with the passage of a small amount of urine. Neither testis was palpable in the scrotum, which was ill-formed. A scaphoid urethral diverticulum was present anteriorly in the penile urethra (Fig. 1). The anal opening was absent, and the median raphe was prominent. No perineal bulge was felt, and sacral pieces were complete. An invertogram (Fig. 2) showed one large air-fluid level covering more than 50% of the width of the abdomen, suggestive of a high rectal anomaly with a pouch colon. Ultrasonography of the abdomen showed a large ventral hernia with dilated bowel loops and bilateral grossly hydronephrotic kid- A. K. Bangroo (&) Department of Pediatric Surgery, 1.3 Administration Block, St. Stephen’s Hospital, Tis Hazari, Delhi, 110054, India E-mail: abangroo@yahoo.com Fax: +91-11-23932412 S. Tiwari Department of Surgery, St. Stephen’s Hospital, Tis Hazari, Delhi, 110054, India R. Khetri Department of Pediatric Surgery, No.4.1 Doctors Hostel, St. Stephen’s Hospital, Tis Hazari, Delhi, 110054, India M. Sahni Department of Pediatrics, No.2.4 Doctors Hostel, St. Stephen’s Hospital, Tis Hazari, Delhi, 110054, India Pediatr Surg Int (2005) 21: 474–477 DOI 10.1007/s00383-005-1439-0