Association of Pediatric Rapunzel Syndrome with ProteinLosing Enteropathy

Johanna R Kaufman, Michelle Solomon and Seema Khan

Department of Graduate Medical Education, Children’s National Medical Center, USA George Washington School of Medicine, USA Kaiser MidAtlantic Permanente Medical Group, USA Division of Gastroenterology, Hepatology and Nutrition, Children’s National Medical Center, USA

^*Correspondance to: Johanna R Kaufman 

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Abstract:

Rapunzel Syndrome describes the rare presentation of a trichobezoar with extension of ingested hairs through the pylorus into the small intestine. In this report, we describe the case of a 4-yearold girl presenting with anasarca found to have hypoalbuminemia, severe malnutrition and Protein-Losing Enteropathy (PLE). Further questioning revealed a history of trichotillomania and trichophagia. An upper GI series demonstrated findings of a trichobezoar extending into the proximal duodenum, consistent with Rapunzel Syndrome. An exploratory laparotomy with anterior gastrotomy removed an extensive trichobezoar. The patient subsequently tolerated advancement of diet with improvement in total serum protein levels. This signifies a rare presentation of PLE in the setting of Rapunzel Syndrome, as well as the youngest reported case of Rapunzel Syndrome to occur in the United States.

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Cite the Article:

Kaufman JR, Solomon M, Khan S. Association of Pediatric Rapunzel Syndrome with Protein-Losing Enteropathy. Ann Clin Case Rep. 2020; 5: 1853.