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| Year : 2004 | Volume
: 9
| Issue : 1 | Page : 39-41 |
Warrdenburg syndrome type 2 associated with total colonic aganglionosis : a case report
KVJ Rao, K Kameswari, DS Rao, SS Kumar, C Rajasekhar, MK Naik
Dept. of Pediatric Surgery, Andhra Medical College, Visakhapatnam, AP
Correspondence Address:
KVJ Rao
Dept. of Pediatric Surgery, Andhra Medical College, Visakhapatnam, AP
 Source of Support: None, Conflict of Interest: None  | Check |
ABSTRACT: A 14-day old male baby presented with abdominal distension, constipation since birth and abnormal pigmentation. The perineum was normal. Plain X-ray abdomen in erect posture showed small bowel obstruction, Barium Enema did not show any transition zone, At laparotomy there was a transition zone in the ileum approximately 20 cm from the ileo-cecal junction and dilatation of the proximal intestine. Multiple seromuscular biopsies from the ileum, colon, rectum and appendix were taken; all were negativefor ganglion cells. Loop ileostomy was performed in the right lower quadrant of the abdomen.
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