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Recurrence of Rapunzel Syndrome

2010; Elsevier BV; Volume: 157; Issue: 2 Linguagem: Inglês

10.1016/j.jpeds.2010.02.019

1097-6833

Sheela Raikar, Prateek Wali, Seema A. Khan,

Surgical Sutures and Adhesives

A 12-year-old African-American girl presented with a 3-day history of severe, intermittent epigastric abdominal pain and vomiting. Her medical history was significant for depression and a previous laparotomy at age 4 for "eating Barbie doll's hair." On physical examination, the patient was noted to have short-cropped hair. An abdominal examination was positive for generalized tenderness and guarding. Laboratory data revealed an elevated white blood cell count with a left shift and elevated platelet count. A plain radiograph of the abdomen was suggestive of a partial small bowel obstruction. A computed tomography (CT) scan of the abdomen (Figure 1) revealed a mass in the stomach extending through the pylorus into the proximal small bowel. Upper endoscopy revealed a large trichobezoar extending from the stomach (Figure 2; available at www.jpeds.com) into the pylorus (Figure 3; available at www.jpeds.com) beyond the second part of the duodenum. After multiple attempts to remove the trichobezoar through endoscopy failed, an upper midline laparatomy was performed. An extensive trichobezoar, which formed a cast of the stomach and duodenum, was removed (Figure 4). The patient had an uncomplicated postoperative course and was discharged to home with outpatient psychiatric follow-up. Trichobezoars result from trichophagia with associated trichotillomania. Only an estimated 1% of patients with trichophagia develop a trichobezoar.1Frey A. McKee M. King R. Martin A. Hair apparent: Rapunzel syndrome.Am J Psychiatry. 2005; 162: 242-248Crossref PubMed Scopus (83) Google Scholar, 2Gonuguntla V. Joshi D. Rapunzel syndrome: a comprehensive review of an unusual case of trichobezoar.Clin Med Res. 2009; 7: 99-102Crossref PubMed Scopus (106) Google Scholar Rapunzel syndrome is a rare form of trichobezoar, seen mainly in adolescent girls, with the tail extending through the pylorus into the small intestine. Patients may have a history of severe halitosis and patchy alopecia. Common presenting features include abdominal pain, nausea, vomiting, obstruction, and peritonitis. Complications may include failure to thrive, chronic anemia, ulcer formation, perforation, pancreatitis, and intussusception.3Western C. Bokhari S. Gould S. Rapunzel syndrome: a case report and review.J Gastrointest Surg. 2008; 12: 1612-1614Crossref PubMed Scopus (21) Google Scholar The extent of the trichobezoar may be determined by imaging studies, such as CT or magnetic resonance imaging, before intervention. Endoscopic removal is limited to phytobezoars, lactobezoars, and small gastric trichobezoars. In Rapunzel syndrome, endoscopic removal is generally unsuccessful because of the bezoar's large dimensions, firmness, and extension into the small intestine. Open laparotomy or laparoscopy remains the standard definitive treatment.2Gonuguntla V. Joshi D. Rapunzel syndrome: a comprehensive review of an unusual case of trichobezoar.Clin Med Res. 2009; 7: 99-102Crossref PubMed Scopus (106) Google Scholar, 4Alsafwah S. Alzein M. Small bowel obstruction due to trichobezoar: role of upper endoscopy in diagnosis.Gastrointest Endosc. 2000; 52: 784-786Abstract Full Text Full Text PDF PubMed Scopus (51) Google Scholar Psychiatric evaluation and follow-up are essential to help prevent recurrence. References available at www.jpeds.com