A volvulus is a complete twisting of a loop of intestine around its mesenteric attachment site. This can occur at various locations of the gastrointestinal (GI) tract, including the stomach, small intestine, caecum, transverse colon and sigmoid colon.
Midgut malrotation refers to twisting of the entire midgut about the axis of the superior mesenteric artery (SMA).
A knowledge of embryology is necessary to understand the mechanisms that lead to the development of volvulus and malrotation. At the fourth week of gestation, the GI system is a straight tube centrally located in the abdomen. During the ensuing eight weeks, the midgut rotates and becomes fixed to the posterior abdominal wall. Arrest of development at any stage narrows the mesenteric base and impairs fixation, leaving the bowel at high risk for volvulus.
- Malrotation occurs in approximately 1 in 500 live births. However, the true incidence of malrotation is unknown, since many asymptomatic patients fail to present. Either sex can be affected with the anomaly.
- Approximately 90% of patients with malrotation are diagnosed within the first year of life, 80% of whom are diagnosed within the first month of life.
- Thereafter, it can present at any age and may present in adults of any age.[4, 5, 6]
- Recurrent bowel obstruction in patients with previous abdominal operation for midgut malrotation is mostly due to adhesions but very few reported cases have been due to recurrent volvulus.
Most cases of volvulus associated with malrotation occur in infants and young children. The incidence of malrotation-associated volvulus decreases with age.
It should be remembered that malrotation and volvulus are two distinct entities.
Non-rotation may be asymptomatic and be detected as an incidental finding during GI imaging for some other purpose. Malrotation may cause intermittent symptoms of intestinal obstruction but if a volvulus develops, the obstruction is typically complete. The presenting features will also vary, depending on age.
Bilious vomiting is the key presenting symptom. A child presenting with green or yellow vomiting should be presumed to have volvulus until proved otherwise. Infants presenting in the first 24 hours after birth through the first week of life tend to have more severe obstruction and present with bilious vomiting and feeding intolerance. After the age of 2 months, bilious and non-bilious vomiting occur with equal frequency. Other symptoms may include failure to thrive, anorexia, constipation, bloody stools and intermittent apnoea.
Older children may present more insidiously with cyclical vomiting, recurrent abdominal pain, protein-calorie malnutrition and immunodeficiency. Once intestinal ischaemia develops, pain becomes the most pronounced symptom.
Intestinal malrotation can be a rare cause of chronic abdominal pain in adults. The diagnosis is often missed initially, leading to increased morbidity.
In midgut volvulus associated with malrotation, the entire midgut from the proximal jejunum to the proximal colon may twist around the mesenteric base. Volvulus may lead to intestinal necrosis in several hours without surgical treatment. Rapid-onset and bilious vomiting, leading to metabolic acidosis, lactataemia, oliguria, hypotension and shock with advancing ischaemia.
Careful examination may reveal a palpable abdominal mass in some patients. If ischaemia develops, the presentation is of acute abdomen, with significant abdominal distension, and signs of peritonitis. Blood or sloughed tissue may pass per rectum. Tachycardia, hypovolaemia and septic shock herald the onset of gangrene.
In the acute phase, the differential diagnosis is of acute abdomen/obstruction. Chronic vague abdominal symptoms in older children or adults raise the possibility of a wide range of other symptoms, which should include:
- Annular pancreas
- Duodenal atresia
- Duodenal web
- Gastro-oesophageal reflux
- Henoch-Schönlein purpura
- Hepatitis B
- Hirschsprung's disease
- Hypertrophic pyloric stenosis
- Incarcerated hernia
- Meckel's diverticulum
- Necrotising enterocolitis
- Ovarian torsion
- Peptic ulcer
- Perforated viscus
- Renal stones
- Sickle cell crisis
- Urinary tract infection
The diagnosis is usually made clinically and management should not be delayed in order to obtain the results of laboratory tests.
FBC helps to assess the severity of the illness; a raised white cell count may be found in sepsis or gangrene and a low haemoglobin may suggest venous oozing. Inflammatory markers (eg, CRP) may be normal in the very early stages of volvulus.
Regular U&E monitoring may help to assess the patient's general condition and detect dehydration, sepsis and acidosis:
- Large amounts of fluid can migrate into the bowel lumen and interstitial space and, in such patients, dehydration can occur without diarrhoea and vomiting.
- Hyponatraemia, hyperkalaemia, metabolic acidosis, increased urea and creatinine, hypochloraemia and lactic acidosis can occur in such cases.
In simple malrotation, plain radiographs are frequently normal. Upright, supine and lateral radiographs may be helpful in diagnosing bowel obstruction but may be more contributory in large rather than small bowel obstruction. Radiographs taken several hours apart may be helpful. Dilated small-bowel loops, marked gastric or proximal duodenal dilatation - with or without intestinal gas - and air-fluid levels may be seen. Normal or equivocal results should not delay progress to other tests if the clinical situation is deteriorating.
In midgut volvulus, the classic radiographic finding is a partial duodenal obstruction (dilation of both stomach and proximal duodenum, with a small amount of distal bowel gas). This is known as the 'double bubble' sign. Complete obstruction of the duodenum may also be found; less frequently, a gasless abdomen, ileus or distal small bowel obstruction with multiple dilated loops and air-fluid levels. These are ominous signs.
In malrotation, the duodenojejunal (DJ) junction is misplaced, either at, or to the right of, the midline. Various displacements of lower bowel structures may also be seen.
An upper GI (UGI) contrast series is the investigation par excellence if volvulus is suspected. In a child, the UGI series is performed with a small amount of barium being administered either by bottle or through a nasogastric tube. Various patterns may be observed, including dilation of the proximal duodenum with a 'bird-beak' obstruction and a spiral or corkscrew duodenal configuration. Selected patients may need studies of the lower bowel, using a barium enema.
Other imaging studies
- Ultrasound may reveal a midline abdominal mass in suspected volvulus. Ultrasound and CT scanning can help to confirm malrotation by identifying the position of the mesenteric vessels. CT scan may reveal 'the coffee bean' in sigmoid volvulus.
- The 'whirlpool sign' on colour Doppler may show a whirlpool pattern of flow within the superior mesenteric vein, indicating malrotation with volvulus.
- The low specificity of these tests suggests that, whilst they may be useful screening tools, they are inferior to the UGI contrast series in the acute situation.
Non-surgical treatment for malrotation
- This may be appropriate for older patients with intestinal malrotation who are asymptomatic but they should be warned that volvulus can occur at any time.
- Observation and GI decompression with a nasogastric or orogastric tube should be commenced and a close watch kept for the development of symptoms or signs suggestive of intestinal obstruction.
Because volvulus is such a devastating complication in children, evidence supports operative treatment of asymptomatic malrotation, using Ladd's procedure. This is particularly appropriate for young children with no existing comorbidity, as they recover quickly. There is no similar evidence base to support this approach in adults.
- Ladd's procedure is the treatment of choice in most cases.
- Volvulus is corrected by rotating the small intestine in an anticlockwise direction, the caecum being placed in the left abdomen and the duodenum directed down the right paravertebral gutter. A second-look laparotomy may be used 36 hours later to ensure viability of the remaining bowel.
- A laparoscopic variation of Ladd's procedure has been used in some centres, with the general advantage of decreased adhesions and scarring; however, good visualisation of the entire bowel is necessary.
- Laparoscopy is a particularly suitable investigative procedure for children presenting with acute abdomen. One study found that it produces better cosmesis, less postoperative pain and earlier return of bowel function than laparotomy.
- Complete and persisting midgut volvulus leads to intestinal ischaemia, mucosal necrosis and sepsis. Untreated, perforation, peritonitis and death soon follow.
- Chronic intermittent volvulus may cause malabsorption with constipation interspersed with diarrhoea.
- Postoperatively, the main complication is short-gut syndrome with the consequent problems surrounding parenteral nutrition, ie line sepsis, hepatobiliary dysfunction and growth restriction.
Although malrotation can usually be successfully treated with early diagnosis, sudden catastrophic events, such as intense ischaemia and intestinal necrosis, may occur and may be fatal.
Further reading and references
Mohan P, Ramamoorthy M, Venkataraman J; Clinical vistas: nonrotation of the intestine. CMAJ. 2008 Jul 1179(1):49-50.
Sozen S, Guzel K; Intestinal malrotation in an adult: case report. Ulus Travma Acil Cerrahi Derg. 2012 May18(3):280-2. doi: 10.5505/tjtes.2012.60973.
Kluth D et al; The Embryology of Gut Rotation
Hwang SM, Na YS, Cho Y, et al; Midgut volvulus as a complication of intestinal malrotation in a term pregnancy. Korean J Anesthesiol. 2014 Dec67(Suppl):S98-9. doi: 10.4097/kjae.2014.67.S.S98.
HitenKumar PN, Shah D, Priyanka CB; Unusual presentation of midgut malrotation with incidental nutcracker syndrome in adulthood: case report and literature review. BMJ Case Rep. 2012 Jul 252012. pii: bcr0320126010. doi: 10.1136/bcr-03-2012-6010.
Soker G, Yilmaz C, Karateke F, et al; An unexpected cause of small bowel obstruction in an adult patient: midgut volvulus. BMJ Case Rep. 2014 May 82014. pii: bcr2014205178. doi: 10.1136/bcr-2014-205178.
Haak BW, Bodewitz ST, Kuijper CF, et al; Intestinal malrotation and volvulus in adult life. Int J Surg Case Rep. 20145(5):259-61. doi: 10.1016/j.ijscr.2014.02.013. Epub 2014 Mar 13.
Sheikh F, Balarajah V, Ayantunde AA; Recurrent intestinal volvulus in midgut malrotation causing acute bowel obstruction: A case report. World J Gastrointest Surg. 2013 Mar 275(3):43-6. doi: 10.4240/wjgs.v5.i3.43.
McCollough M, Sharieff GQ; Abdominal surgical emergencies in infants and young children. Emerg Med Clin North Am. 2003 Nov21(4):909-35.
Uba AF, Chirdan LB, Edino ST; Intestinal malrotation: presentation in the older child. Niger J Med. 2005 Jan-Mar
Fu T, Tong WD, He YJ, et al; Surgical management of intestinal malrotation in adults. World J Surg. 2007 Sep31(9):1797-803
Steffensen TS, Gilbert-Barness E, DeStefano KA, et al; Midgut volvulus causing fetal demise in utero. Fetal Pediatr Pathol. 2008 Dec27(4):223-31.
Ameh EA, Nmadu PT; Intestinal volvulus: aetiology, morbidity, and mortality in Nigerian children. Pediatr Surg Int. 2000
Atamanalp SS, Yildirgan MI, Basoglu M, et al; Sigmoid colon volvulus in children: review of 19 cases. Pediatr Surg Int. 2004 Jul
Tackett JJ, Muise ED, Cowles RA; Malrotation: Current strategies navigating the radiologic diagnosis of a surgical emergency. World J Radiol. 2014 Sep 286(9):730-6. doi: 10.4329/wjr.v6.i9.730.
Paslawski M, Gwizdak J, Zlomaniec J; The diagnostic value of different imaging modalities in evaluation of bowel obstruction. Ann Univ Mariae Curie Sklodowska [Med]. 2004
Oh SK, Han BK, Levin TL, et al; Gastric volvulus in children: the twists and turns of an unusual entity. Pediatr Radiol. 2008 Mar38(3):297-304. Epub 2008 Jan 16.
Orzech N, Navarro OM, Langer JC; Is ultrasonography a good screening test for intestinal malrotation? J Pediatr Surg. 2006 May
Salati U, McNeill G, Torreggiani WC, et al; The coffee bean sign in sigmoid volvulus. Radiology. 2011 Feb258(2):651-2.
McVay MR, Kokoska ER, Jackson RJ, et al; Jack Barney Award. The changing spectrum of intestinal malrotation: diagnosis and management. Am J Surg. 2007 Dec194(6):712-7
Malek MM, Burd RS; The optimal management of malrotation diagnosed after infancy: a decision analysis. Am J Surg. 2006 Jan
Matzke GM, Dozois EJ, Larson DW, et al; Surgical management of intestinal malrotation in adults: comparative results for open and laparoscopic Ladd procedures. Surg Endosc. 2005 Oct
Palanivelu C, Rangarajan M, Shetty AR, et al; Intestinal malrotation with midgut volvulus presenting as acute abdomen in children: value of diagnostic and therapeutic laparoscopy. J Laparoendosc Adv Surg Tech A. 2007 Aug17(4):490-2.
Imamoglu M, Cay A, Sarihan H, et al; Rare clinical presentation mode of intestinal malrotation after neonatal period: Malabsorption-like symptoms due to chronic midgut volvulus. Pediatr Int. 2004 Apr
Thakur A, Chiu C, Quiros-Tejeira RE, et al; Morbidity and mortality of short-bowel syndrome in infants with abdominal wall defects. Am Surg. 2002 Jan68(1):75-9.
Lee HC, Pickard SS, Sridhar S, et al; Intestinal malrotation and catastrophic volvulus in infancy. J Emerg Med. 2012 Jul43(1):e49-51. doi: 10.1016/j.jemermed.2011.06.135. Epub 2012 Feb 9.