RAPUNZEL SYNDROME IN A 15-YEAR-OLD INDIAN GIRL WITH AUTOIMMUNE HEPTITIS: A CASE REPORT AND LITERATURE REVIEW

Dr Priynka Ranga, Dr Pardaman Singh, Dr R. K. Goel, Dr Deepak Gehlan, Dr Kamal Mehta, Dr Parteek Tendon

Abstract


Introduction:

Bezoars are masses formed by the accumulation of intra-luminalnon-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.

Case presentation:

We present a 15-year-old female who was admitted to our department with history of pain in abdomen, loss of weight, recurrent vomiting,amenorrhea, easyfatigability for last 6 months. For which patient underwent investigations in which patient was havingclinical picture  of malnutrition with normal level of LH,FSH,prolactin, thyroid profile and patient USG abdomen revealed coarse hepatic echotexture.

Patient was Further found to be positive for ANTI LKM -1 (3 TIMES OF NORMAL VALVE) and diagnosis of autoimmune hepatitis was made and was put on immunosuppressant. CECT abdomen was ordered which reaveled a giant trichobezoarcompletely filling the stomach and duodenum causing partial obstruction ,needing surgical interference for removal and psychiatric therapy.

We believe that this is a rare case  presentation of Rapunzel syndrome in a 15 years old young female with Autoimmune liver disease and Trichotillomania . The link between Autoimmune liver diseases and neuropsychiatry disorders is still poorly understood and this case report may help us to understand the link between the two disorders.

Conclusions:

Trichobezoars should be considered as a differential diagnosis in children complaining of recurrent abdominal pain,vomiting with epigastric mass and progressive weight loss. The link between autoimmune liver diseases and neuropsychiatry disorders is still poorly understood and this case may help us to understand the link between the two disorders. It is an original case report of interest to physician and psychiatry specialists.


Keywords


RAPUNZEL SYNDROME, Bezoars, gastrointestinal

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References


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