Rapunzel Syndrome
Dr. Tirth Patel
Rapunzel Syndrome
A case came to emergency department in Sola Civil hospital. Details as follows!
Introduction:
An unusual form of bezoar extending from the stomach to the small intestine or beyond which leads to obstruction or perforation has been described as Rapunzel syndrome, which is named after the long-haired girl Rapunzel in the fairy tale by the Brothers Grimm. Trichobezoar is a hair ball in alimentary canal, mostly seen in mentally retarded females, which may be attributed to the traditional long hair. Patient usually has tendency of trichophagia and trichotillomania. Patient may either present with simple abdominal pain or acute abdominal pain with symptoms of persistent vomiting, anorexia, weight loss and various motility disorders. Patient can be evaluated by clinical and radiological examination like X-rays, CT-scan, USG, and GI scopy. The treatment consists of removal of mass of hair only or resection of gangrenous bowel with anastomosis and post-op psychiatric counselling is must.

Case Study:
•We present a case of 15-year-old girl came in emergency department with symptoms of abdominal pain, recurrent vomiting, abdominal distension and absolute constipation during the last 5 days.
•P/H: anorexia, weight loss, anemia and trichophagia
•O/E, abdomen shows generalized tenderness, guarding and distention. Bowel sounds were exaggerated. Rectum examination revealed an empty rectum.
•Pt is Conscious with tachycardia BP: 100/70 mm Hg

Investigation:
•Blood sample was sent to laboratory.
•The X-ray showed multiple air fluid levels without any air under diaphragm.
•On USG: Dilated small bowel with air and fluid level within it. Showing to and fro movement suggesting of acute small bowel obstruction.

Management:
•After optimizing the patient for GA, Emergency Exploratory Laparotomy was performed through midline incision; which revealed per op dilated small bowel loop with gangrenous portion of 25 cm ileum including ileocecal junction with mass inside, and various skip lesions on it.
•A total 41 inches Gangrenous bowel with skip lesions was resected en mass involving ileocecal junction, caecum with appendix, part of ascending colon containing trichobezoar and end to end ileo-ascending colonic anastomosis was done in four layer using silk sutures. Abdominal drain was kept and removed on post op 6th day when outcome decreased to 50 ml only.
•The post-surgery follow-up was unremarkable. The patient was referred for psychiatric counselling.

Conclusion:
•Trichobezoar, an under-diagnosed entity, particularly considered in young female patient having psychological disturbances and H/o of trichophagia, with acute abdominal symptoms. Patient should be evaluated thoroughly by utilizing various modality to come to a diagnosis. Management of the case is not only surgical but post-operative psychological assessment and counselling is must to prevent recurrence.

Affiliations:
Dr. Hemang Panchal
Assistant Professor of Surgery
GMERS Medical College, Sola

Dr. Aashish Desai
Associate Professor of Surgery
GMERS Medical College, Sola
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Dr. T●●●h P●●●l
Dr. T●●●h P●●●l General Medicine
How can i help u Ketan sir ??
Sep 14, 2016Like
S●●●●●●●l G●●●l
S●●●●●●●l G●●●l General Medicine
Nice
Sep 15, 2016Like
M●●●●i B●●●●r
M●●●●i B●●●●r General Medicine
Nyc????
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