DOI: http://dx.doi.org/10.18203/2349-2902.isj20214394

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

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

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References


Vaughan ED Jr, Sawyers JL, Scott HW Jr. The Rapunzel syndrome. An unusual complication of intestinal bezoar. Surgery. 1968;63(2):339-43.

Grimm Brothers: Rapunzel. Translated by Godwin-Jones R. Richmond, Virginia Commonwealth University Department of Foreign Languages. 1994-1999.

Naik S, Gupta V, Naik S, Rangole A, Chaudhary AK, Jain P et al. Rapunzel syndrome reviewed and redefined. Dig Surg. 2007;24(3):157-61.

Sanders MK. Bezoars: From Mystical Charms to Medical and Nutritional Management. Practical Gastroenterolo. 2004:28;37-50.

Pace AM, Fearne C. Trichobezoar in a 13-year-old male: A case report and review of literature. Malta Med J. 2003;15:39-40.

Deslypere JP, Praet M, Verdonk G. An unusual case of the trichobezoar: the Rapunzel syndrome. Am J Gastroenterol. 1982;77(7):467-70.

Schlosser S, Black DW, Blum N, Goldstein RB. The demography, phenomenology, and family history of 22 persons with compulsive hair pulling. Ann Clin Psychiatry. 1994;6(3):147-52.

Western C, Bokhari S, Gould S. Rapunzel syndrome: a case report and review. J Gastrointest Surg. 2008;12(9):1612-4.

Bhatia MS, Singhal PK, Rastogi V, Dhar NK, Nigam VR, Taneja SB. Clinical profile of trichotillomania. J Indian Med Assoc. 1991;89(5):137-9.

Naik S, Gupta V, Naik S, Rangole A, Chaudhary AK, Jain P, Sharma AK. Rapunzel syndrome reviewed and redefined. Dig Surg. 2007;24(3):157-61.

Kwong WT, Kalmaz D. A modern form of Rapunzel syndrome: trichobezoar composed of synthetic hair extensions. Clin Gastroenterol Hepatol. 2014;12(5):A33-4.

Wang Z, Cao F, Liu D, Fang Y, Li F. The diagnosis and treatment of Rapunzel syndrome. Acta Radiol Open. 2016;5(11):2058460115627660.

Ullah W, Saleem K, Ahmad E, Anwer F. Rapunzel syndrome: a rare cause of hypoproteinaemia and review of literature. BMJ Case Rep. 2016;2016:bcr2016216600.

Sah DE, Koo J, Price VH. Trichotillomania. Dermatol Ther. 2008;21(1):13-21.

Newman B, Girdany BR. Gastric trichobezoars-sonographic and computed tomographic appearance. Pediatr Radiol. 1990;20(7):526-7.

West WM, Duncan ND. CT appearances of the Rapunzel syndrome: an unusual form of bezoar and gastrointestinal obstruction. Pediatr Radiol. 1998;28(5):315-6.

Wang YG, Seitz U, Li ZL, Soehendra N, Qiao XA. Endoscopic management of huge bezoars. Endoscopy. 1998;30(4):371-4.

Gonuguntla V, Joshi DD. Rapunzel syndrome: a comprehensive review of an unusual case of trichobezoar. Clin Med Res. 2009;7(3):99-102.

Soufi M, Benamr S, Belhassan M, Massrouri R, Ouazzani H, Chad B. Giant trichobezoar of duodenojejunal flexure: a rare entity. Saudi J Gastroenterol. 2010;16(3):215-7.

Gorter RR, Kneepkens CM, Mattens EC, Aronson DC, Heij HA. Management of trichobezoar: case report and literature review. Pediatr Surg Int. 2010;26(5):457-63.

Fallon SC, Slater BJ, Larimer EL, Brandt ML, Lopez ME. The surgical management of Rapunzel syndrome: a case series and literature review. J Pediatr Surg. 2013;48(4):830-4.

Tudor EC, Clark MC. Laparoscopic-assisted removal of gastric trichobezoar; a novel technique to reduce operative complications and time. J Pediatr Surg. 2013;48(3):e13-5.

Franklin ME, Edson AL, Ledley DA, Cahill SP. Behavior therapy for pediatric trichotillomania: a randomized controlled trial. J Am Acad Child Adolesc Psychiatry. 2011;50(8):763-71.