Eur opean Rev iew for Med ical and Pharmacol ogical Sci ences 2016; 20: 4719-4724 Recurrent acute pancreatitis in bowel malrotation G. ALESSANDRI, A. AMODIO 1, L. LANDONI 1, N. DE’ LIGUORI CARINO, C. BASSI 1 HPB Unit, Department of Surgery, Manchester Royal Infirmary Hospital, Central Manchester Foundation Trust, Manchester, UK 1Surgical Department, Pancreas Centre, Hospital of “G.B. Rossi”, University of Verona, Verona, Italy Abstract. – OBJECTIVE: Introduction Recurrent acute pancreatitis is an uncommon diagnosis in teenagers. Excluded alcohol and biliary stones, Midgut malrotation is a not so rare congenital more prevalent aetiologies in these group of abnormality; occurring in one in 500 live birth 1. patients are genetic pancreatitis, pancreatic The majority (approximately 80%) 1,2 of these duct system abnormalities, neoplasia, traumas children develop gastrointestinal (GI) symptoms and congenital abnormalities of the duodenum such as duodenal duplication or diverticulum. in the first month of their life. Midgut malrota - Two reported cases of recurrent pancreatitis tion is described as a variation of the normal associated to midgut malrotation were de - 270° counter clockwise rotation that starts after scribed in English literature. Bowel malrotation the fifth week of pregnancy. Generally, sympto - is a difficult diagnosis in a teenager or a young matic patients present with an obstructive clinical adult and a common delay is documented. picture; vomiting (generally bilious content), in - Ladd’s procedure is largely recognized to be termittent abdominal pain, diarrhoea, constipa - the standard treatment for a symptomatic mal - tion, malabsorption and failure to thrive 3. Midgut rotCaAtioSnE. REPORT: Our Report describes in de - malrotation is always associated with an in - tails a case of recurrent pancreatitis, where a creased risk of bowel volvulus and a related is - late diagnosis of midgut malrotation was ob - chemic gut. tained and an endoscopic management was at - Pancreatitis is an atypical presentation of an tempted. A literature review and an analysis of abnormal intrauterine development of gastro-en - two previously reported cases were performed teric apparatus. Pancreatitis during childhood is to explore a possible aetiopathogenesis of the recurrent acute pancreatitis in patients with generally observed in children with genetic or congenital diseases causing cystic dilatation of midRgEuStU mLTaSlr: otation. 24 months of follow-up showed an bile ducts, maljunction of biliopancreatic ducts, 4-7 asymptomatic patient on a free diet, with a mild pancreas divisum or other HPB malformation . deranged Liver Functional Tests and a normal Causes of pancreatitis in an older age group (up Amylase and Lipase. The recurrence of acute to 25-30) are alcohol, CBD stones, trauma and pancreatitis has not been observed until the congenital abnormalities of the duodenum such preCsOeNntC dLaUyS. IONS: Recurrent episodes of acute as duodenum duplication, intra or extra luminal pancreatitis in young adults, without a history diverticula. Rarely pancreatitis associated with of alcohol abuse or evidence of gallstones, midgut malrotation were documented in the liter - might be an atypical presentation of midgut ature; only two patients with recurrent episode of malrotation and it should be in the differential acute pancreatitis and a diagnosis of bowel mal - diagnosis. In this case, a Ladd’s operation is rotation were reported 8-11 . beneficial and an endoscopic procedure does not obtain advantages. Case Report Key Words: Recurrent acute pancreatitis, Uncommon diagnosis in A 24-years-old woman was referred to our young adult, Bowel malrotation, Ladd’s operation. specialist Hepato-Biliary-Pancreatic (HPB) Cen - tre for recurrent episodes of acute pancreatitis of Corresponding Author: Giorgio Alessandri, MD; e-mail: [email protected] 4719 G. Alessandri, A. Amodio, L. Landoni, N. de’ Liguori Carino, C. Bassi unknown aetiology. Symptoms initially appeared in a fit and well patient 3 years previously; they were characterized by an epigastric and right up - per quadrant colicky abdominal pain, very severe (VAS 10/10), radiated to the right back, with a gradual onset, associated with nausea and several episode of vomiting. On examination, the ab - domen was tender in all upper quadrants with guarding on the right side but no rebound was es - timated. First hospital assessment was in January 2009, blood showed mild deranged amylase (111 UI/L) and Lipase (160 UI/L) with normal Liver Functional Tests (LFTs). Three ultrasound scans Figure 1. (USS) could not show abnormalities. A CT scan The first CT scan performed on the patient (Figure 1) described an uncomplicated oedema - showed the inverted relationship between superior mesen - tous pancreatitis of body and tail of the gland and teric artery and vein, that was not reported. sludge within the gallbladder. The Common bile duct (CBD) was reported not delated, the duode - num and the head of the pancreas were not de - the peritoneum, a remnant of dorsal mesogastri - scribed. A diagnosis of biliary pancreatitis was um. The duodenum was twisted and mild retract - made and an elective laparoscopic cholecystecto - ed upward, the small bowel was situated to the my was performed after a full recovery from the right side of abdomen and the colon was on the episode of acute pancreatitis. The procedure was left. The CBD, the main pancreatic duct (MPD) uneventful. Before the operation, a magnetic res - and the duodenum were anterior to the usual onance cholangiopancreatography (MRCP) was anatomy, more floppy and with an unnatural mo - obtained: “there were no gallstones, not dilata - bility. The pancreaticobiliary junction was de - tion of the CBD nor pancreatic duct” and there scribed as normal. Neither of the CBD and the was not a further description of upper gastroin - MPD were dilated, nor filling defects were ap - testinal (UGI) tract. preciated and a normal pancreatic outflow was During the following year, the patient’s symp - observed after the secretin stimulation. More in - toms relapsed twice with a similar presentation; vestigations were requested and an endoscopic hospital admission and management with simple retrograde cholangiopancreatography (ERCP) analgesia were required. showed: “a prepapillary stricture of the main Eighteen months post-surgery recurrent pancreatic duct without upstream dilatation”. A episode of acute pancreatitis lead to a further ad - pancreatic sphincterotomy was performed during mission. Investigations showed four times elevat - ed amylase and lipase. Several abdominal and a pelvic ultrasounds were requested consecutively, no abnormalities were reported. A new MRCP estimated normal biliary tree and pancreatic duct, a CT scan with contrast showed no bowel ob - struction but a “dysmorphic head of the pancreas and a folded, festooned duodenum” were report - ed. An oesophago-gastro-duodenal endoscopy (OGD) was performed. It described “a consider - able amount of biliary reflux into the stomach and normal first and second portion of duode - num”. An MRCP with secretin (Figure 2) was scheduled in a middle-high volume HPB centre to review the function of the Oddi sphincter. Dur - Figure 2. ing this procedure, a bowel malrotation was de - MRCP with secretin revealed the uncommon pre - scribed. The duodenum and the head of the pan - sentation of the head of pancreas and the MPD (arrows). The creas were not lying on a retroperitoneal space, CBD and MPD were not dilated, nor filling defects were ap - both of them were located between two layers of preciated and a normal pancreatic outflow were observed. 4720 Recurrent acute pancreatitis in bowel malrotation The diagnosis of incomplete bowel rotation, Ladd ligament and an abnormal presentation of the duodenum were done only many years after the episodes of acute pancreatitis. At 9 and 10 years old the child had had two admissions with a diagnosis of idiopathic acute pancreatitis. Cholelithiasis, biochemical and infective aetiolo - gy had been excluded. Several USS had been performed at that time but a diagnosis of malro - tation had not been obtained. The patient, at the age of 17, underwent to an exploration laparoto - my for an acute intestinal obstruction. During the Figure 3. A preoperative contrast follow through showing procedure, it was found a volvulus of the right the bowel presentation. colon secondary to a midgut malrotation. Au - thors just mentioned the attractive hypothesis of a recurrent acute pancreatitis due to a “distortion the ERCP and a plastic stent (4 cm, 5 Fr) left in of the ampulla”, secondary to an uncommon pre - situ for few days. A functional stricture and a bent sentation of the duodenum. duodenum were also confirmed. The second case 11 is a 16 years old girl with a After that procedure, the symptoms did not dis - 6 years history of worsening recurrent acute appear and several episodes of recurrent pancre - pancreatitis. Several investigations were under - atitis were observed after that. Nine months later taken. A CT scan did not show abnormalities of there was a new hospital admission. Amylase ele - the biliary three, pancreas divisum or annular vated at 6000 UI/L with mild deranged LFTs. pancreas. A follow through study showed the USS and MRCP confirmed the uncomplicated presence of bowel malrotation and barium ene - pancreatitis and the ERCP showed a patent ma confirmed it. An ERCP was performed and Wirsung and no stricture on the papilla. The pa - it reported “a distortion of the first and second tient was referred to our centre for recurrent pan - part of the duodenum associated with a mobile creatitis. head of the pancreas. The CBD was mild dilat - Here a new assessment was obtained and all ed but the pancreatic duct was unremarkable performed investigation reviewed. No mutation and there was no abnormality of the pancreatic- of CFTR and SPINK-1 were found on genetic biliary junction”. A laparoscopic Ladd’s proce - tests. A contrast follows through (Figure 3) dure was performed eventually. There was no showed the bowel presentation. It was advocated recurrence of symptoms during the 3 years fol - to be responsible for a functional, intermittent lowing the procedure.