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Letters to the Editor

Indian Pediatrics 1998; 35:682-683

Rapunzel Syndrome


The bezoars commonly encountered in infancy and early childhood are lactobezoars; phytobezoars, trichobezoars and trichophytobezoars. These are usually encountered after 5 years of age(1). An unusual case of phytobezoar in a fourteen month old female child is reported.

She presented with a 3 day history of abdominal distension and bilious vomiting. This was preceded by diarrhea for a week, which was treated with anti-cholinergics elsewhere. She was malnourished, lethargic, dehydrated and anemic. The abdomen was distended but there was no local or rebound tenderness. Two ill-defined masses having restricted mobility, one in left flank and another in the right paraumbilical region, could be felt. Occasional tinkling could be heard on auscultating the abdomen. The rectal examination was normal.

Hemoglobin at admission was 8 g/ dl. Blood biochemistry was normal. Upright abdominal roentgenogram was inconclusive. Keeping intussusception in mind as a probable clinical diagnosis, an ultrasound guided saline enema was done; this showed a persistent hyperechoic elongated mass in the ascending colon, that could be retrogradely traced into the terminal ileum. To clarify the nature of mass, a barium enema was later done, this revealed barium entangled in a tail-like projection. No definite preoperative diagnosis could be made inspite of all the above investigations.

After adequate resuscitation a laparotomy was performed which revealed extensively necrotic gut chocked with phytobezoar comprising of coir fibers. Multiple perforations confined to the mesenteric border of small bowel were found sealed off by adherence to mesentery or other loops of bowel. Minimal manipulation of bowel led to spontaneous opening of these perforations resulting in gross contamination of the peritoneal cavity. The bezoar was removed piece meal through multiple perforated sites. The part of phytobezoar present in the stomach was removed through an anterior gastrostomy made at right angle to the long axis of stomach. Extensive resection of necrotic jejuno-ileum was done. After resection, 15 cm of proximal jejunum, 20 cm of proximal ileum and 5 cm of terminal ileum were noted to be viable. The middle segment of small bowel was. arranged in anti-peristaltic manner and anastomoses performed. Peritoneal toilet was done.

Post-operatively, on requisitioning, the mother revealed history of coir fibre (Moonj) ingestion. The post-operative period was uneventful and the child was discharged on the tenth post-operative day. She was lost to follow up only to be seen after a month in a debilitated, moribund state. She succumbed to septicemia after another five days.

An unusual form of long bezoar extending from stomach to ileocaecal valve or beyond it has been described as Rapunzel Syndrome(2). The long, tail like extension of the bezoar causes contraction and construction of long segment of bowel as a result of peristaltic activity(2). The hyper- active peristalsis causes the bowel to be drawn tightly on the intraluminal obstructing material. Because of the inequality in the length of bowel and mesentery, there is unequal pressure along the mesenteric border of the bowel. As the bowel becomes move compressed, pressure necrosis may develop along the mesenteric aspect of the bowel wall at points of maximum tension(2). The other complications include internal fistulae(3), obstructive jaundice(4), megaloblastic anemia(5), protein-losing enteropathy(5) and short bowel syndrome(6).
 

Y.K. Sarin,
In-charge,
Pediatric Surgery Unit,

Department of Surgery,
Christian Medical College and Hospital,
Ludhiana
141008,
India.

 

References


1. Grosfeld JL, Schreiner RL, Franken EA. The changing pattern of gastrointestinal bezoars in infants and children. Surgery 1980; 88: 425-432.

2. Vaughan ED, Sawyers JL, Scott WH. The Rapunzel syndrome: An unusual complication of intestinal bezoar. Surgery 1968; 63:
339-343.

3. Jaiswal K, Sharma RK, Gupta RL, Malhotra A. Gastrojejunal fistula - A complication of recurrent trichobezoar. Indian J Surg 1995; 57: 43-45.

4. Schreiber H, Filston He. Obstructive jaundice due to gastric trichobezoar. J Pediatr Surg 1976; 11: 103-104.

5. Bernstein LH, Gutstein S, Eflon G, Wagle A, Graham E. Trichobezoar: An unusual case of megaloblastic anaemia and hypoproteinemia in childhood. Am J Dig Dis 1973; 18: 67-71.

6. Schwartz MZ, Maeda K. Short bowel syndrome in infants and children. Pediatr Clin N Am 1985; 32: 1265-1279.
 

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