Annals of Pediatric Surgery, Vol 3, No 3,4, July-October 2007 PP 167-169

Case Report

Rapunzel Syndrome: an Uncommon Problem in Children

Ali Moustafa Ali, Mohamed Hany Hassab
King Fahd medical city, Riyadh, Saudi Arabia

Abstract:
A Trichobezoar represents a mass of accumulated hair within the gastrointestinal tract. Isolated gastric Trichobezoars, those
with extension into the duodenum, and small intestinal Trichobezoars have all been described.1 two cases of Trichobezoars in
a 7.5 and 11years old girls with palpable abdominal masses. The diagnosis was clinically suspected and confirmed by
investigations. Both patients underwent surgical intervention. They had an uneventful recovery. These cases are presented
because of their rarity, unique presentation, and simplicity of management.
Index Word: Trichobezoar, Gastrostomy, Rapunzel syndrome.

INTRODUCTION

T he Rapunzel syndrome was first described by

Vaughn and coauthors in 1968. It is a rare form
of gastric trichobezoar with a tail-like extension into
In both cases abdominal X-rays showed gastric
shadows occupied by radiopaque like mass with
absent gastric bubbles. Barium meal and C.T scan
the duodenum or proximal small intestine.2 Untill (Fig 1), showed large intragastric mass which
2003 only 14 cases of this entity have been reported.3 extending into duodenum.
We report two cases of Rapunzel syndrome. The 1st Gastroscopy was performed in case 2 (Fig 2) and it
one was a 7.5 year old girl, who was admitted to revealed a large ball of hair occupying most of the
Oncology Department with abdominal mass, loss of stomach which could not be removed endoscopically.
appetite for the last week, marked wasting, Body
In both cases the Trichobezoars was removed by
weight was 15 Kg. and some loss in scalp hair. On
gastrotomy (Fig 3), there was a J shaped mass around
examination, there was an epigastric mass occupying
20x15cm, foul smelling, black loose Trichobezoars,
the upper abdomen. The mass was oblong in shape,
weighting 1540 grams in the 1st case. The mass was
firm in consistency, with smooth surface and was
about 2152 grams in 2nd case; with extension into the
mobile up and down.
duodenum and jejunum (Fig 4).
The 2nd is an 11-year old girl, complaining of
The patients had an uneventful recovery, discharged
intermittent colicky abdominal pain since one year
home at the 3rd postoperative day and referred to the
and increased over the last one week. There was no
paediatric behavioural clinic for psychological
history of any psychological problems related to
evaluation and support.
family affairs or parental marital problems. The
patient was stable; with no alopecia. She had hard
non-tender epigastric intra abdominal mass.

Correspondence to: Mohamed Hany Hassab, MD. Senior consultant and Head of paediatric surgery division, King Fahd
Medical City, Riyadh; KSA, Po Box: 365778, Riyadh: 11393. Saudi Arabia, Fax: +966-12889999 – 2463.
Email: mohamed_hany_hassab@hotmail.com
 Ali A& Hassab M.

Fig 1. Abdominal CT scan (with IV & oral contrast) shows Fig 2. Showed a large, greenish black Trichobezoars
large heterogeneous intragastric mass surrounded with occupying most of the stomach.
contrast & extended to the duodenum.

Fig 3. Operative photo. Extension of the bezoars into the Fig 4. Shows Postoperative specimen of Trichobezoar.
duodenum and jejunum is seen.

168 Annals of Pediatric Surgery


DISCUSSION
The Rapunzel syndrome (a trichobezoar with a long tail
extending from the stomach to the small bowel) is an
uncommon disease.4 which can cause small bowel
obstruction, jaundice or even acute pancreatitis.5
De Bakey and Ochsner reviewed 172 cases of
trichobezoar of which 90% were females with age range
from 10 to 19 years. They noted that a palpable
abdominal mass in (87.7%).6 Consistent with their
findings, our two cases were females (7.5 and 11 years
old), and they presented mainly with an abdominal
mass.
Diagnosis may be aided by abdominal plain films,
contrast upper gastrointestinal radiography,
ultrasonography, CT scan, or upper gastrointestinal
endoscopy.5 As in our cases the diagnosis was clinically
suspected, and was confirmed radiologically. The
second case had endoscopy as a trial for non invasive
removal of the trichobezoar.
Larger gastric trichobezoars must be removed either
laparoscopically or by laparotomy.1 Surgery is the
treatment of choice for trichobezoar. 6 The two reported
cases had open gastrostomy.
The two patients were referred to the paediatric
behavioural clinic for psychological evaluation and
169
Ali A. & Hassab M.
support. We recommend psychiatric follow up to
prevent recurrence.
REFERENCES
1. Hoover K, Piotrowski J, St. Pierre K, et al. Simultaneous
gastric and small intestinal trichobezoars—a hairy
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2. Zent RM, Cothren CC, Moore EE, et al. Gastric
Trichobezoar and Rapunzel Syndrome; the American
College of Surgeons. 990,2004.
3. Deevaguntla CR, Prabhakar B, Prasad GR. Rapunzel
Syndrome—A Case Report; JIAPS. 9:33-4,2004.
4. Ventura DE, Herbella FAM, Schettini ST, et al.
Rapunzel syndrome with a fatal outcome in a neglected
child; JPS. 40:1665–7,2005.
5. Jensen AR, Trankiem CT, Steve Lebovitch S, et al.
Gastric outlet obstruction secondary to a large
trichobezoar; JPS. 40:8,2005.
6. Hassan MAG, Grover VK, Sameer H. Trichobezoar
with Gastric Ulcer Perforation - A Case Report; Kuwait
Medical Journal. 35:296-8,2003.
Vol 3, No 3,4, July-October, 2007