Minia University, Egypt
Title: Rapunzel Syndrome in a 7-year-old Egyptian Girl with Primary Biliary Cirrhosis: A case Report and Literature Review
Abdel-Azeem El-Mazary has completed his M.B.B.CH at the age of 24 years and M.D degree at the age of 34 years from Minia University, Egypt. Postdoctoral workshops and training courses in Pediatrics in both of Cairo and Mansoura Universities, Egypt. He is the director of Neonatology Unit Clinic of Minia University Hospital. He is working as Associate Professor in Pediatrics, Pediatric Departement, Minia University. He has published more than 16 papers in reputed journals and has been serving as an editorial board member of (Science Research Journal) and as a reviewer in (European Journal of Clinical Nutrition) .
Bezoars are masses formed by the accumulation of intra luminal non digestible substances that can lead to obstruction of the stomach and the small intestine. The anatomical changes in the gastrointestinal tract are known to cause bezoar formation. The so-called Rapunzel syndrome is the extension of the bezoars down to the duodenum and the jejunum, which is a rare condition. This may occur in subjects with mental retardation and/or psychiatric disorders. We present a 7-year-old girl with primary biliary cirrhosis who was admitted to our department with hematemesis, loss of weight and fatigue complaints along the last 6 months, in whom a giant trichobezoar was identified through endoscopy which completely filled the stomach and duodenum causing partial obstruction and needed surgical interference for removal and psychiatric therapy. We believe that -after MEDLINE search along the last 10 years- this case is the first Egyptian case report of this kind in the literature.
Trichobezoars should be considered as a differential diagnosis in children complaining of recurrent abdominal pain with epigastric mass and progressive loss of weight.Diagnosis can be easily made with radiological interventions and endoscopy. As a case report we present a 7-year-old Egyptian girl with primary biliary cirrhosis and Rapunzel syndrome at very young age.
Keywords: Rapunzel, trichobezoar, biliary, cirrhosis, female, Egyptian.