Surgical outcome of jejunum-jejunum intussusception secondary to Rapunzel syndrome: a case report

Abstract

BackgroundAdult intestinal intussusception is a rare condition caused by the mechanical disruption of bowel motility. A bezoar is defined as indigestible material inside the gastrointestinal tract that develops into a trapped mass; the most frequent bezoar is a trichobezoar. When a trichobezoar extends into the small intestine it is defined as Rapunzel’s syndrome. Literature describing complications related to this pathology remains scarce.Case presentationA 16-year-old Mexican girl presented to our emergency room with acute abdomen and a presumptive diagnosis of intestinal obstruction. Computed tomography was suggestive of intussusception. Surgery confirmed a jejunal-jejunal intussusception with a mass within the gastric cavity extending into her small intestine, corresponding to a trichobezoar. A manual intussusception reduction and a gastrotomy with extraction of the trichobezoar were performed.ConclusionsWe present a case of a jejunum intussusception as a complication of Rapunzel syndrome. Our patient had a favorable outcome after surgical intervention with a manual intussusception reduction, with retrograde displacement of the trichobezoar into the gastric lumen, and a complete extraction through a gastrostomy. Follow-up included psychiatric evaluation.

An Unusual Case of Primary Ileal Trichobezoar Causing Intussusception

“…Onset of abdominal pain can vary from 12 months prior [ 17 ] to the day before hospitalization [ 27 ]. Obstructive symptoms, such as constipation, obstipation, and distention, are present in some cases [ 15 , 16 , 21 , 23 , 28 , 32 , 37 ], but diarrhea is also possible [ 18 , 27 ]. A physical exam may be helpful in determining the location of the trichobezoar, as patients experiencing ‘Rapunzel syndrome’ often had a palpable abdominal mass [ 13 , 14 , 17 – 21 , 25 , 26 , 28 , 37 ], while patients without ‘Rapunzel syndrome’ did not [ 27 , 32 ].…”

“…Imaging of ‘Rapunzel syndrome’ is helpful in diagnosis, as the gastric trichobezoar accrues a large mass before the tail reaches a sufficient size to cause intussusception. This results in the CT scan finding of a heterogeneous mass occupying a dilated gastric chamber, described as a ‘beehive pattern’ by Bolivar-Rodriguez et al [ 15 ]. Abdominal radiography and ultrasound have also been used to successfully diagnose ‘Rapunzel syndrome’, but CT is preferred due to its higher specificity [ 39 ].…”

Síndrome de Rapunzel asociado a obstrucción intestinal y hemorragia de vías digestivas altas: reporte de caso y revisión de literatura

“…Los fragmentos del bezoar se pueden presionar hacia el intestino delgado o extraer por la boca, siempre proporcionando un sobretubo para facilitar los pases frecuentes del endoscopio y propendiendo a la protección de la vía aérea. La fragmentación y la extracción endoscópicas proporcionan resultados favorables en el 85-90% de los bezoares gástricos pequeños, con las mejores tasas de efectividad en casos de combinación con terapia enzimática proteolítica [22][23][24][25][26] .…”

Intussusception of the small intestine – a rare cause of gastrointestinal obstruction in adults – case report

“…In adults, the cause is usually known; there is often a lead point (LP) of the intussusception [1]. It may be a small intestine polyp, a tumor: a submucosal lipoma, mucous cyst (mucocele), overly long stump after appendectomy, postoperative suture line, or Meckel's diverticulum, but also cystic fibrosis, Crohn's disease, posttraumatic hematoma, or trichobezoar [1,2,3].…”