International Surgery Journal | November 2021 | Vol 8 | Issue 11 Page 3463 International Surgery Journal Manchikanti D et al. Int Surg J. 2021 Nov;8(11):3463-3466 http://www.ijsurgery.com pISSN 2349-3305 | eISSN 2349-2902 Case Report Rapunzel syndrome: an uncommon tale of a long hairy tail - case report and review of literature Dinesh Manchikanti, Manisha Aggarwal, Shaji Thomas*, Ashish Arsia, Rahul Pusuluri, Sanjay Kumar INTRODUCTION Trichobezoar, which was first described by Baudomont in 1779, is a rare clinical entity in which a ball of hair accumulates within the alimentary tract. It is more common to find this problem in young patients with psychiatric issues, more frequently in girls than boys. 1 When the tail of the trichobezoar extends into the small intestine, this condition is given the name - Rapunzel syndrome (RS) because of the resemblance of this tail to the hair of Rapunzel, a Grimm Brothers fairy tale princess locked up inside a tower, who uses her long, golden hair to permit her young prince to climb up to her window and rescue her. 2 RS was first described in the literature by Vaughan et al in which a dense compact mass of hair (trichobezoar) was found in stomach with extension into intestine through duodenum in patients with a history of psychiatric disord er. 1 The presentation of RS spans a wide spectrum, varying from an asymptomatic state to lump abdomen, gastrointestinal ulceration, obstruction or even perforation in young psychiatric patients. 3 ABSTRACT Trichobezoar is a rare clinical entity in which a ball of hair accumulates within the alimentary tract. When the tail of the trichobezoar extends into the small intestine, this condition is called Rapunzel syndrome (RS). A 14-year-old female presented with pain abdomen and vomiting for 2 weeks, and a history of trichotillomania and trichophagia, and an epigastric lump. A contrast enhanced computerized tomography (CECT) of the abdomen showed a grossly distended stomach with a heterogeneous mass containing trapped air with underlying normal mucosa suggestive of trichobezoar, with its tail extending into the proximal jejunum suggestive of RS. During laparotomy, a giant trichobezoar was seen in the stomach with its tail extending beyond the duodenum into the proximal jejunum. The entire specimen was delivered out intact. On follow up, she has no surgical complications, and was on behaviour therapy. Trichobezoars form when ingested hair strands become retained in the folds of the gastric mucosa and becomes entangled, forming a ball too large to exit the stomach. Trichotillomania and trichophagia are seen in many of these patients. Patients present with abdominal pain, vomiting, gastric outlet obstruction, and an epigastric mass. In the case of RS, complete removal without breakage and distal migration is important. RS should be considered as a differential diagnosis in a young girl with abdominal pain, vomiting, anaemia and upper abdominal lump. Early diagnosis prevents complications. Surgical removal is treatment of choice. Trichobezoar often coexists with psychiatric illness. Psychiatric evaluation, counselling and treatment are helpful in preventing recurrence. Keywords: RS, Trichobezoar, Trichotillomania, Trichophagia Department of Surgery, Lady Hardinge Medical College and Dr RML Hospital, New Delhi, India Received: 25 September 2021 Accepted: 19 October 2021 *Correspondence: Dr. Shaji Thomas, E-mail: drshajithomas@yahoo.com Copyright: © the author(s), publisher and licensee Medip Academy. This is an open-access article distributed under the terms of the Creative Commons Attribution Non-Commercial License, which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. DOI: https://dx.doi.org/10.18203/2349-2902.isj20214394