Rapunzel’s Syndrome – A Rare Phenomenon

Introduction

Rapunzel Syndrome is a rare phenomenon describing a trichobezoar which extends through the stomach into the small intestine or even colon. It is named after the fairy tale of the Grimm brothers and was first reported under that name in 1968 (Vaughan ED et al., Surgery 1968; 63: 339–343), whereas Rolleston describes already in 1924 a similar case of a girl who died from a “hair ball of the stomach” in 1917 (Rolleston JD, Proc R Soc Med. 1924; 17 (Sect Study Dis Child): 5–8). He himself cited a first recorded case of Trichobezoar from Baudamant in 1779 (Baudamant M, Hist Soc Roy Med 1777–1779; 2: 262–263).

From 1968–2006 27 cases were reported world-wide, with only one case in a male patient (Naik S et al., Digestive Surgery 2007; 24: 157–161). Since then more and more case reports have followed, with 53 publications listed in PubMed from 2007 by now (PubMed: “Rapunzel Syndrome case report” 14.05.2014). Most of the cases affect patients between 13 and 20 years of age (Gonuguntia V et al., Clin Med Res 2009; 7: 99–102), often in the course of psychiatric disorders. A history of trichotillomania and trichotillophagia is often neglected. The swallowed hairs, which are resistant to digestion due to their smooth surface, accumulate in the stomach and form a growing mass. This condition may remain asymptomatic for years. Besides of unspecific abdominal symptoms like vomiting, nausea, loss of weight and malnutrition it may in later stages also cause life-threatening complications like bowel-obstruction, -perforation or peritonitis (Gorter RR et al., Pediatr Surg Int 2010; 26: 457–463). Therefore an early diagnosis is crucial. Diagnostic procedures may include labaratory tests, ultrasound, barium contrast study, abdominal x-ray, CT-scan, MRI and endoscopy. Gold standard of the therapy remains the open surgical removal, as endoscopic and laparoscopic approaches are most often not successful. Moreover the open surgery allows the examination of the whole bowel system in order to determine satellites (Gorter RR et al., Pediatr Surg Int 2010; 26: 457–463). Medical treatment and enzymatic degradation was also reported as ineffetive (Jensen AR et al., J Pediatr Surg 2005; 40: 1364–1365, Coulter R et al., South Med J. 2005; 98: 1042–1044).