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Case Reports
. 2020 Mar 4:8:82.
doi: 10.3389/fped.2020.00082. eCollection 2020.

An Unusual Case of Hypoproteinemia in Childhood: Keep in Mind Trichobezoar

Mariangela Stinco  1 , Alessandra Montemaggi  1 , Bruno Noccioli  2 , Massimo Resti  1 , Salvatore Grosso  3 , Sandra Trapani  1
Affiliations
Case Reports

An Unusual Case of Hypoproteinemia in Childhood: Keep in Mind Trichobezoar

Mariangela Stinco et al. Front Pediatr. .

Abstract

Protein-losing enteropathy (PLE) is a rare condition characterized by protein loss through the gastrointestinal tract, leading to hypo-proteinemia. Patients may be asymptomatic or present with variety of complications of hypoproteinemia (e.g., oedema, ascites, pleural, and cardial effusions). We describe a case report of a young girl suffering from behavioral disorder since childhood who presented with generalized oedema, hypoproteinaemia, and microcytic hypochromic anemia. In addition, the girl had an intervention for jejunal atresia and intestinal malrotation in her past medical history. Upper gastrointestinal endoscopy revealed a trichobezoar extending from stomach into the small bowel, thus classified as Rapunzel Syndrome (RS), causing mechanical obstruction of intestinal lumen and intestinal lymphatic drainage resulting in a protein-losing enteropathy (PLE). Trichobezoar was successfully removed by a surgical laparotomy resulting in resolution of symptoms and normalization of biochemical parameters. Possibly, previous surgery might have had an influence on intestinal dysmotility and trichobezoar formation. PLE is a very rare presenting symptom of RS, developing as result of intestinal obstruction caused by large trichobezoars. RS has to be considered in patients, especially adolescents, suffering from behavior disorder as trichotillomania and trichophagia. Surgical removal and nutritional supplementation are the gold treatment of large trichobezoar.

Keywords: Rapunzel Syndrome; hypoproteinemia; oedema; protein-losing enteropathy; trichobezoar.

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Figures

Figure 1
Figure 1
Gastrointestinal endoscopy: a large tricobezoar occupying lumen of distal duodenum.
Figure 2
Figure 2
Large intra-luminal solid mass (tricobezoar) excised from the duodenum.
See this image and copyright information in PMC

References

    1. Naik S, Gupta V, Naik S, Rangole A, Chaudhary AK, Jain P, et al. . Rapunzel syndrome reviewed and redefined. Dig Surg. (2007) 24:157–61. 10.1159/000102098 - DOI - PubMed
    1. Novak AB, Zupančič Ž, Plut D, Gvardijančič D, Homan M. Rapunzel syndrome: a rare form of trichobezoar in the stomach with some extension into the small intestine. Acta Dermatovenerol Alp Pannonica Adriat. (2018) 27:155–7. 10.15570/actaapa.2018.32 - DOI - PubMed
    1. Braamskamp MJAM, Dolman KM, Tabbers MM. Clinical practice: protein-losing enteropathy in children. Eur J Pediatr. (2010) 169:1179–85. 10.1007/s00431-010-1235-2 - DOI - PMC - PubMed
    1. Vignes S, Bellanger J. Primary intestinal lymphangiectasia (Waldmann's disease). Rev Med Interne. (2018) 39:580–5. 10.1016/j.revmed.2017.07.009 - DOI - PubMed
    1. Cannalire G, Conti L, Celoni M, Grassi C, Cella A, Bensi G, et al. . Rapunzel syndrome: an infrequent cause of severe iron deficiency anemia and abdominal pain presenting to the pediatric emergency department. BMC Pediatr. (2018) 18:125. 10.1186/s12887-018-1097-8 - DOI - PMC - PubMed

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