Fatemeh Elham Mahjoub - Department of Pathology, Bahrami Children Hospital Affiliated to Tehran University of Medical Sciences, Tehran, Iran.
Mehri Najafi Sani - Pediatric Gastro- Enterology And Nutrition Research Center, Tehran University of Medical Sciences, Tehran, Iran.
Nakisa Niknejad - Department of pathology, Besat Hospital, Hamedan University of Medical Sciences, Hamedan, Iran
Shahsanam Gheibi - Associate Professor in Pediatric Gastroenterology, Children Medical Center, Urumiah University of Medical Sciences, Urumiah, Iran.

Introduction:Pancreas divisum is a frequent congenital abnormality of the pancreas. The diagnosis may be delayed due to non-specific manifestations. Recently, Magnetic resonance chol angio-pancreatography (MRCP) has been proposed as the gold standard for definitive diagnosis of such anomalies. Association of pancreas divisum with gall bladder agenesis is also reported. Most cases presented with recurrent pancreatitis. Hereby we wanted to present a 12 year old boy with pancreas divisum and gall bladder agenesis who was treated with wrong impression of autoimmune hepatitis for 2.5 years.Case presentation:A 12 year old boy referred with history of abdominal pain, nausea and vomiting started three years ago. Elevated liver enzymes and elevated gamma glutamyl transferase (GGT) were detected and liver biopsy was performed for him. Pathologic examination in another center was reported as chronic hepatitis (Histology activity index (HAI) grade: 6/18 and stage: 1/6). He underwent treatment with diagnosis of autoimmune hepatitis but liver enzymes were fluctuating despite treatment in a Yo Yo pattern. Paraffin blocks and slides were sent to our center for second look. Reassessment of slides revealed no significant portal fibro- inflammatory process (only mild infiltration of lymphomononuclear cells in few portal areas with no significant interface hepatitis). The patient had an abdominal sonography early in childhood which revealed agenesis of gall bladder and was confirmed in recent sonography. Also MRCP was performed and pancreas divisum was detected. Treatment of auto immune hepatitis was discontinued and he was referred to surgeon for possible corrective surgery. Discussion:Pancreas divisum is a rather common congenital anomaly and can be seen with gall bladder agenesis. The above mentioned combination mostly present with recurrent acute pancreatitis or chronic pancreatitis. To our knowledge this is the first case with these anomalies presenting with elevated liver enzymes in a Yo Yo pattern who was treated with diagnosis of auto immune hepatitis for a long time.

Pancreas Divisum, Gallbladder Agenesis, Magnetic Resonance Cholangiopancreatography