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Home , Intestinal malrotation, Pancreas divisum, Volvulus

Eur opean Rev iew for Med ical and Pharmacol ogical Sci ences 2016; 20: 4719-4724 Recurrent acute 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, Centre, Hospital of “G.B. Rossi”, University of Verona, Verona, Italy

Abstract. – OBJECTIVE: Introduction Recurrent is an uncommon diagnosis in teenagers. Excluded alcohol and biliary stones, 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 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 . 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; (generally bilious content), in - Ladd’s procedure is largely recognized to be termittent , diarrhoea, constipa - the standard treatment for a symptomatic mal - tion, and 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 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 ducts, maljunction of biliopancreatic ducts, 4-7 asymptomatic patient on a free diet, with a mild 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 , 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 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, 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 (CBD) was reported not delated, the duode - num and the head of the pancreas were not de - the , 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 and the colon was on the episode of acute pancreatitis. The procedure was left. The CBD, the main (MPD) uneventful. Before the operation, a magnetic res - and the duodenum were anterior to the usual onance cholangiopancreatography (MRCP) was , 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. obstruction of the duodenum. Thus, the increas - ing pressure inside the bowel leads to recurrence of symptoms. The mobility of the head of the pancreas was considered responsible for the functional obstruction to the pancreatic outlet. A Ladd’s procedure was scheduled and performed for the patient. A contrast follow through (Figure 4) was obtained one month after the procedure. No recurrence of symptoms has been observed after 24 months of follow-up.

Discussion

In literature, only two cases were reported with an association between midgut malrotation 8-11 Figure 4. and recurrent episodes of acute pancreatitis . A contrast follow through obtained one month 8 The first case described is a retrospective hy - after Ladd’s procedure. Arrows point at the duodenopexy pothesis that remains without objective proof. performed.

4721 G. Alessandri, A. Amodio, L. Landoni, N. de’ Liguori Carino, C. Bassi

Sasaki et al 11 probed a possible aetiology with the latter part of the duodenum were anterior to several investigation. Symptoms were related to the retroperitoneal plan, with a degree of mobili - an unusual feature of the duodenum and pancre - ty. This increased the possibility of an obstruc - atic head, twisted and retracted upward. This un - tion of the MPD as reported by Sasaki et al 11 . usual UGI presentation was assumed to be re - The duodenum was closely related to a malrotated sponsible for a functional, intermittent pancreatic and ascending colon. They were attached duct obstruction and consequently recurrent pan - to the right side of the abdomen by peritoneal creatitis. Findings obtained in that patient by a bands (Ladd’s ligament) including part of the duo - follow through study and an ERCP supported the denum. In this patient a pancreatic sphincterotomy hypothesis. The lack of recurrence of symptoms and a stent did not settle the symptoms, showing during the following 3 years of follow-up sup - that pancreatic duct obstruction was not related to ported authors opinion. a “distortion of the Ampulla of Vater”, as men - It is well documented as an inflammation of the tioned by Kirby et al 8. The particular presentation pancreas can be obtained in an experimental way of the proximal midgut instead might be advocat - on mice, blocking the proximal part of the small ed as responsible of a variable duodenal obstruc - bowel, distally to the papilla of Vater and before it, tion. This type of , with an inter - increasing the intraduodenal local pressure 12 . mittent kinking of the pancreatic duct, might be The non-specific presentation of our patient considered in our patient as a possible contribu - causes difficulty in establishing a diagnosis. After tive aetiopathogenic mechanism of the recurrent the first episode of abdominal pain, she was man - acute pancreatitis. aged as a biliary pancreatitis 13 . A conservative In all these cases there was a delay in the diag - management of pancreatitis and an elective la - nosis of midgut malrotation. Spigland et al 10 re - paroscopic cholecystectomy were p erformed 14,15 . ported generally the delay of 20 months to reach Recurrence of symptoms were carefully investi - the diagnosis of malrotation in teenager. In our gated by MRCP, CT abdomen, USS and a MRCP case, symptoms initially started in January 2009 with secretin (Figure 2). Eventually, an ERCP and the abnormal bowel presentation was detect - was scheduled. It reported: “a prepapillary stric - ed in September 2010. Numerous USS, MRI and ture of the main pancreatic duct without up - CT scans were performed. stream dilatation and normal outflow, in a patient Reassessing all available imaging of our pa - with bowel malrotation”. According to this diag - tient obtained before the diagnosis, it was possi - nosis, a pancreatic sphincterotomy was per - ble to observe typical features of incomplete ro - formed, but recurrences were observed again. tation of gut. US, CT and MRI are not the pre - When the patient arrived in our centre, a simple ferred modalities to make a diagnosis of malrota - UGI series (Figure 3) was requested and the case tion. Careful evaluation of all bowel and the in - discussed in a multi-disciplinary meeting. All verted relationship between superior mesenteric available imagines were reassessed. A midgut artery and vein 16-18 (Figure 1) can help to under - malrotation with a Ladd’s ligament was con - stand the anatomy. The gold start investigation to firmed and judged to be responsible for recurrent assess the first part of the GI tract is the contrast pancreatitis. Intraoperative findings supported follow through study. There are three main radio - our impression. In patient assessed in our centre, logical signs to detect an UGI malrotation: (1) there were no signs of biliary obstruction. There abnormal position of the duodenojejunal junc - was no abnormality in the main pancreatic duct, tion; (2) spiral, “corkscrew” or Z-shaped course that was no dilated and without any irregularity of duodenum and proximal ; (3) a je - of the wall. The unusual presentation of the duo - junum located in the right quadrants. In some pa - denum and the small bowel were remarkable. tients, a contrast enema might be useful to con - The duodenojejunal junction was not found in firm the diagnosis, as it can show the large bowel the conventional position. The duodenum had an located on the left side of the abdomen. irregular twisted course, located in the right up - In our case and in the case described by Sasaki per quadrant of the abdomen and with a Z shape et al, the Ladd’s operation was performed elective - (Figure 3). Peritoneal bands entrapped the de - ly, which is widely recognized as the procedure of scending and transverse portion of the duodenum choice to correct a bowel malrotation. Several au - (Ladd’s bands), responsible for its fixity to the thors advocate a surgical correction in all patient retroperitoneal space, showing a variable degree with a diagnosis of malformation 19,20 , regardless of bowel obstruction. The pancreatic head and the presence of symptoms. A significant percent -

4722 Recurrent acute pancreatitis in bowel malrotation

FILSTON HC, K IRKS DR. age of patients dies or suffers substantial morbidi - 2) Malrotation--the ubiquitous ty due to loss of gut, secondary to a complicating anomaly. J Pediatr Surg 1981; 16: 614-620. 1-21 HOWELL CG, V OZZA F, S HAW S, R OBINSON M, S ROUJI volvulus and ischemic gut . Asymptomatic pa - 3) MN, K RASNA I, Z IEGLER MM tients with a bowel malrotation will always have . Malrotation, malnutri - 22-23 tion, and ischemic bowel disease. J Pediatr Surg an increased risk of volvulus . 1982; 17: 469-473. Our patient underwent an endoscopic pancre - TASHIRO S, I MAIZUMI T, O HKAWA H, O KADA A, K ATOH T, 4) atic sphincterotomy before surgery. Recurrence KAWAHARADA Y, S HIMADA H, T AKAMATSU H, M IYAKE H, TODANI T of symptoms followed in several occasions. In ; Committee for Registration of the our institute, an open Ladd’s procedure was per - Japanese Study Group on Pancreaticobiliary formed. During the there was a con - Maljunction. Pancreaticobiliary maljunction: retro - firmation of the diagnosis. Ladd’s band was in - spective and nationwide survey in Japan. J Hepa - tobiliary Pancreat Surg 2003; 10: 345-351. cised, the duodenum was carefully mobilized and KAMISAWA T, M ATSUKAWA M, A MEMIYA K, T U Y, E GAWA 5) N, O KAMOTO A, A IZAWA S. manipulated straight. A duodenopexy was creat - Pancreatitis associated ed and the duodenum was relocated to the right with pancreaticobiliary maljunction. Hepatogas - side of the abdomen in a non-twisted position, as troenterology 2003; 50: 1665-1668. 24 NAKAMURA T, O KADA A, H IGAKI J, T OJO H, O KAMOTO described by Bax and Van der Zee . The appen - 6) M. dectomy was not conducted and broadening of Pancreaticobiliary maljunction-associated the was deemed not necessary 25,26 . The pancreatitis: an experimental study on the activa - surgical procedure was based on intraoperative tion of pancreatic phospholipase A2. World J 27 Surg 1996; 20: 543-550. finding; a “complete not rotation” pattern , the SHUKRI N, W ASA M, H ASEGAWA T, O KADA A. 7) Diagnos - cecum located on the right lower quadrant and a tic significance of pancreas divisum in early life. general intra-abdominal assessment suggested a Eur J Pediatr Surg 2000; 10: 12-16. broad mesentery with a low risk for complicating KIRBY CP, F REEMAN JK, F ORD WD, D AVIDSON GP, F UR - 22,23 8) NESS ME. volvulus after duodenopexy . Malrotation with recurrent volvulus pre - senting with , pruritus, and pancreati - tis. Pediatr Surg Int 2000; 16: 130-131. SPITZ L, O RR JD, H ARRIES JT. Conclusions 9) Obstructive jaundice secondary to chronic midgut volvulus. Arch Dis Child 1983; 58: 383-385. SPIGLAND N, B RANDT ML, Y AZBECK S. Congenital abnormalities have to be consid - 10) Malrotation pre - ered as a possible etiology of recurrent acute senting beyond the neonatal period. J Pediatr pancreatitis in a teenager, when the abuse of al - Surg 1990; 25: 1139-1142. cohol and the diagnosis of gallstones are exclud - SASAKI T, S OH H, K IMURA T, H ASEGAWA T, O KADA A, 11) FUKUZAWA M. ed. The gold standard to make a diagnosis of Recurrent acute pancreatitis caused midgut malrotation remain the gastrografin fol - by malrotation of the intestine and effective treat - low through study. However, a careful assess - ment with laparoscopic Ladd’s procedure. Pediatr Surg Int 2005; 21: 994-996. ment of the presentation of the small bowel and DICKSON AP, F OULIS AK, I MRIE CW. 12) Histology and the relationship between the mesenteric vessels bacteriology of closed duodenal loop models of can be used in all other cross section studies to experimental acute pancreatitis in the rat. Diges - obtain the diagnosis. A symptomatic patient with tion 1986; 34: 15-21. OJETTI V, M IGNECO A, M ANNO A, V ERBO A, R IZZO G, midgut malrotation needs a Ladd’s procedure. 13) GENTILONI SILVERI N Recurrent acute pancreatitis can be included in . Management of acute pancre - the clinical picture of a symptomatic patient with atitis in emergency. Eur Rev Med Pharmacol Sci 2005; 9: 133-140. midgut malrotation. LATERZA L, S CALDAFERRI F, B RUNO G, A GNES A, B OŠKOS - 14) KI I, I ANIRO G, G ERARDI V, O JETTI V, A LFIERI S, G ASBAR - RINI A . Pancreatic function assessment. Eur Rev –C–o–n––f–li–c–t– –o–f– I–n––te– –r-e– s–t– Med Pharmacol Sci 2013; 17(2 Suppl): 65-71. FIOCCA F, S ANTAGATI A, C ECI V, D ONATELLI G, P ASQUALI - 15) The Authors declare that they have no conflict of interests. NI MJ, M ORETTI MG, S PERANZA V, D I GIULI M, M INERVI - NI S, S PORTELLI G, G IRI S . ERCP and acute pancreati - tis. Eur Rev Med Pharmacol Sci 2002; 6: 13-17. References WEINBERGER E, W INTERS WD, L IDDELL RM, R OSENBAUM 16) DM, K RAUTER D. Sonographic diagnosis of intesti - TORRES AM, Z IEGLER MM. nal malrotation in infants: importance of the rela - 1) Malrotation of the intes - tive positions of the superior mesenteric vein and tine. World J Surg 1993; 17: 326-331. artery. AJR Am J Roentgenol 1992; 159: 825-828.

4723 G. Alessandri, A. Amodio, L. Landoni, N. de’ Liguori Carino, C. Bassi

DUFOUR D, D ELAET MH, D ASSONVILLE M, C ADRANEL S, LONG FR, K RAMER SS, M ARKOWITZ RI, T AYLOR GE. 17) PERLMUTTER N. 22) Ra - Midgut malrotation, the reliability of diographic patterns of in sonographic diagnosis. Pediatr Radiol 1992; 22: children. Radiographics 1996; 16: 547-556. SCHEY WL, D ONALDSON JS, S TY JR. 21-23. 23) Malrotation of NICHOLS DM, L I DK. 18) Superior mesenteric vein rota - bowel: variable patterns with different surgical tion: a CT sign of midgut malrotation. AJR Am J considerations. J Pediatr Surg 1993; 28: 96-101. BAX NM, VAN DER ZEE DC. Roentgenol 1983; 141: 707-708. 24) Laparoscopic treatment PRASIL P, F LAGEOLE H, S HAW KS, N GUYEN LT, Y OUSSEF of intestinal malrotation in children. Surg Endosc 19) S, L ABERGE JM. Should malrotation in children be 1998; 12: 1314-1316. LADD WE. treated differently according to age? J Pediatr 25) Surgical diseases of the alimentary Surg 2000; 35: 756-758. tract in infants. N Engl J Med 1936; 215: 705-708. POWELL DM, O THERSEN B, S MITH CD. LADD WE, G ROSS RE 20) Malrotation of 26) . Intestinal obstruction resulting the intestines in children: the effect of age on pre - from malrotation of the intestines and colon in ab - sentation and therapy. J Pediatr Surg 1989; 24: dominal surgery of infancy and childhood, Chap 5. 777-780. WB Saunders, Philadelphia, 1941; pp. 53-70. SKANDALAKIS JE, G RAY SW. STROUSE PJ. 21) The small intestines. The 27) Disorders of intestinal rotation and fix - Embryology for surgeons, 2nd ed. Williams and ation (“malrotation”). Pediatr Radiol 2004; 34 : Wilkins, Baltimore, 1994; pp. 184-241. 837-851.

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