Rapunzel syndrome: The unsuspected culprit

Abstract

Trichobezoar is a rare intriguing disorder in which swallowed hairs accumulates in the stomach. Being indigestible and slippery, it could not be propulsed and becomes entrapped within the stomach. Large amounts can thus accumulate over the years forming a hair ball. Rapunzel syndrome is a variant where hair accumulation reaches the small gut and beyond in some cases. Although the syndrome has been known for many years, only 24 cases have been reported in the literature and the discovery of a new case is always surprising. In this report, we present two cases discovered within a period of three months. One of them was pregnant and had small bowel intussusception and perforation, a very rare combination. We hereby add two more cases to the literature. To our knowledge, this is the first report on two cases of Rapunzel syndrome, the diagnosis of which demands a high index of suspicion.

Rapunzel Syndrome With Reverse Intussusception Managed by Laparoscopy: Case Report

“…3 Usually there are no symptoms until the trichobezoars reaches a substantial size. 4 An indentable abdominal mass is the commonest presentation, 5 with other features like alopecia circumscripta and signs of gastric outlet obstruction. 4 Gastric bezoar formation occurs in patients with altered gastric physiology, impaired gastric emptying, reduced acid production, or all of these together.…”

“…CECT scan has a high accuracy rate and differentiates it from any neoplasms . 5,6 CECT scan shows a well-circumscribed ovoid intraluminal lesion, composed of concentric whorls of different densities with pockets of air enmeshed within it, appearing in the stomach region. Beyond the lesion, the bowel collapses.…”

Rapunzel syndrome — a case report

“…Mucus covering the bezoar provides a glistening shiny surface that prevents propulsion by peristalsis through the duodenum [5]. The bezoar becomes entrapped within the mucosal folds, where it then becomes enmeshed [9].…”

“…Diagnostic modalities include ultrasound study, CT scanning, and upper endoscopy [9]. On conventional radiography, a gastric bezoar is seen as a mass of opaque soft tissue in a swollen stomach.…”

Nephrotic syndrome in a female patient with Rapunzel syndrome

“…ДПК -двенадцатиперстная кишка КТ -компьютерная томография НС -нефротический синдром СОЭ -скорость оседания эритроцитов СР -синдром Рапунцель ме желудочно-кишечных симптомов (боли в животе, тошнота, рвота, снижение аппетита, быстрое насыщение) ряд авторов ука-зывают на другие проявления, связанные с трихобезоаром: ане-мию [2, 4-6, 14, 17], в том числе мегалобластную [2], гипоальбу-минемию [2,5,6], лейкоцитоз [6], гипотрофию [17], фронтальное облысение [4], аллергические проявления [3]. Описаны случаи трихобезоара, сочетающегося с другими заболеваниями: серпо-видноклеточной анемией [18], целиакией [19], болезнью Мене-трие [20], бронхолегочной дисплазией и двусторонней нейросен-сорной тугоухостью [2].…”