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Copyright © 2007 Via Medica 277 Intestinal malrotation is a developmental anomaly affecting the position andperitoneal attachments of the small and large bowels during organogenesis infoetal life. It has been defined as absent or incomplete rotation and fixation ofthe embryonic gut around the superior mesenteric artery. In the present paper,we review the definition, history, embryology/aetiology, epidemiology, symp-toms and signs, diagnosis and treatment of intestinal malformations. Moreover,Key words: malrotation, embryology, intestine, organogenesisKey words: malrotation, embryology, intestine, organogenesisHISTORYMalrotation was first noted by Mall in 1898 [1].There are pre-20th century reports of malrotation. In century reports of malrotation. Insic article on the treatment of malrotation, introduc-ing his surgical approach, Ladds procedureŽ, whichremains the cornerstone of practice today.EMBRYOLOGY/AETIOLOGYNormal gut rotation places the two ends of theintestinal tract, namely the proximal duodenojeju-nal loop and the distal caecocolic loop, simultaneous-ly in their proper positions [18]. In this process, which brought to you by CORE View metadata, citation and similar papers at core.ac.uk provided by Via Medica Journals takes place from the 5 to the 12 gestational weeks, gestational wee

ks,EPIDEMIOLOGYIntestinal malrotation occurs at a rate of 1 in 500live births. Male predominance exists in neonatalpresentations at a male-to-female ratio of 2:1. Upto 40% of patients with malrotation present withinthe first week of life, 50% up to one month of age and75% by the age of one year. The remaining 25% ofpatients present later, even into adult life. There is nosexual bias among patients older than one year [14].SYMPTOMS AND SIGNSThe antenatal diagnosis of malrotation can besuggested by identification of its complications, suchas bowel dilatation, ascites or meconium peritoni-tis. Malrotation can be associated with a narrowmesenteric base, an anatomical arrangement pre-disposing to potentially fatal mid-gut volvulus andinfarction [8], which are the main complications ofmalrotation. After birth therefore the diagnosis isusually established on the basis of abdominal painand bilious vomiting secondary to bowel obstruc-tion. Although bilious vomiting is the presentingsymptom among almost all patients diagnosed inthe neonatal period, clinical features of older patients(infants or children) include chronic or recurrentcolicky abdominal pain, intermittent constipation,colicky abdominal pain, intermittent constipation,obstruction, intestinal obstruction, gastrointestinalbleeding, peritonitis a

nd even septic shock.Malrotation can cause internal herniation, it usu-ally has a chronic picture, while its physical exami-nation findings can be unremarkable, and diagnosisis made by radiological studies and the index of sus-picion only. Left mesentericoparietal hernias maycause haematochezia, haemorrhoids and dilatedanterior abdominal veins which are related to venousobstruction. If the bowel of the patient is obstruct-ed at the time of presentation, abdominal tender-ness and guarding may be present, and a soft glob-ular mass may be palpated at the location of thehernia [14].Intestinal malrotation in an adult may present bythe subsets of acute intestinal ischaemia includingmesenteric arterial occlusion, venous thrombosis andnon-occlusive mesenteric ischaemia [4, 17].The attribution of symptoms and signs to malro-tation is further confounded by the presence of dys-motility in many of these patients, who will main-motility in many of these patients, who will main-Malrotation may occur in association with otherMalrotation may occur in association with otherDIAGNOSISClinical investigation hinges on the upper gas-trointestinal contrast study, while some experts alsoadvocate a routine contrast enema to determine theposition of the caecum if it is not seen on the fol-low-through films. The upper gas

trointestinal con-trast study derives most of its diagnostic informa-tion by defining the position of the duodenojejunal(D…J) flexure with respect to the pylorus and verte-bral column. Other findings, such as volvulus, duode- ure is inferior to the pylorus, unless the stomach isure is inferior to the pylorus, unless the stomach issmall bowel typically resides in the right abdomenand the colon and caecum are often on the left. Thecontrast enema is less reliable in identifying malro-„plain abdominal X-ray film. This may show theplain abdominal X-ray film. This may show thebly pneumatosis intestinalis may be observed if may be observed if„ultrasonography. This has been shown to haveultrasonography. This has been shown to have„computed tomography (CT) scanning. This is notomputed tomography (CT) scanning. This is not„air enema [12];„scintigraphy [11].ration, evisceration of the intestines is essential tocal or duodenal attachment to the posterior abdom- trointestinal obstruction are shown in Table 1.„Case 2: with Downs syndrome, ambiguous gen-„Case 11: with congenital heart disease (mitral re-„Case 13: with congenital heart disease (atrial sep-„Case 18: congenital heart disease (patent ductus„Case 26: imperforated anus, ectopic kidney andsurgical procedures and intraoperative final diag- FindingsFr

equencySexMale17 (56.7%)Female13 (43.3%)AgeNeonate17 (56.7%)Infant7 (23.3%)Child (preschool age)6 (20%)Bilious vomiting16 (53.3%)Non-bilious vomiting5 (16.7%)Abdominal pain5 (16.7%)Faecal vomiting2 (6.7%)Abdominal distension2 (6.7%)Multiple gross anomaly1 (3.3%) Prolonged icterus1 (3.3%) Type of imaging studiesNumberReported findingsNumber of cases Plain abdominal X-ray films27Gastric or duodenalPresent22 (81.5%)Absent5 (18.5%)Distal duodenal airNormal13 (48.1%)Decreased4 (14.8%)Absent10 (37.0%) Reported double bubble sign4 (14.8%)14Bowel obstructionComplete6 (42.9%)Partial4 (28.6%)None4 (28.6%)Displacement of bowelPresent4 (28.6%)Absent10 (71.4%)Cecum malpositionPresent6 (42.9%) Absent8 (57.1%)Barium enema8Caecum malpositionPresent4 (50%) Absent4 (50%) The peritoneal bands, which have a compressive effect on the duodenum, are becoming detached (malrotations showing distension of the stomach together with centuryagnoses of the subjects reported are of potential use1.Aiken JJ, Oldham KT (2005) Malrotation. In: Ashcraft KW,2.Anatol TI (1992) Intestinal malrotation in Trinidad. J R3.Beaudoin S, Mathiot-Gavarin A, Gouizi G, Bargy F4.Cassart M, Massez A, Lingier P, Absil AS, Donner C,5.Chou CK, Chang JM, Tsai TC, Mak CW, How CC (1995)6.Cieri MV, Arnold GL, Torfs CP (1999) Malrotation in7.Devane SP

, Coombes R, Smith VV, Bisset WM, and Final diagnosisNumber of casesSurgical procedureNumber of casesPure malrotation16 (53.3%)Ladds procedure13 (43.3%)Ladds procedure without appendectomy3 (10%)Malrotation with mid-gut volvulus10 (33.3%)Ladds procedure and derotating volvulus6 (20%)Ladds procedure, bowel resection4 (13.3%)Malrotation and duodenal atresia2 (6.7%)Ladds procedure and duodenojejeunostomy2 (6.7%)Malrotation with Meckels diverticulum1 (3.3%)Ladds procedure, diverticulectomy1 (3.3%)and duodenal atresiaand duodenojejeunostomy Malrotation and biliary atresia1 (3.3%)1 (3.3%)8.Dilley AV, Pereira J, Shi ECP, Adams S, Kern IB, Currie B,9.Erez I, Reish O, Kovalivker M, Lazar L, Raz A, Katz S10.Howell CG, Vozza F, Shaw S, Robinson M, Srouji MN,11.Kovanlikaya A, Miller JH, Williams HT (1996) Malrota-12.Lobo E, Daneman A, Fields JM, Keller MS, Alton DJ,13.Matsuki M, Narabayashi I, Inoue Y, Yamasaki K (1997)14.Parish A,15.Potts SR, Thomas PS, Garstin WIH, McGoldrick J (1985)The duodenal triangle: a plain film sign of midgut16.Smith VL, Long F, Nwomeh BC (2006) Monozygotic17.Svab J, Rathous I, Klofanda J, Vyborny J, Kotrlikova E,Vitkova I, Povysil C (2005) Intestinal ischaemia „ conse-18.Touloukian RJ, Smith EI (1998) Disorders of rotation19.Weinberger E, Winters WD, Liddell RM, Rosenbaum