Intestinal Malrotation 

Updated: Dec 19, 2018
Author: Denis D Bensard, MD, FACS, FAAP; Chief Editor: Carmen Cuffari, MD 

Overview

Background

During normal abdominal development, the 3 divisions of the GI tract (ie, foregut, midgut, hindgut) herniate out from the abdominal cavity, where they then undergo a 270º counterclockwise rotation around the superior mesenteric vessels. Following this rotation, the bowels return to the abdominal cavity, with fixation of the duodenojejunal loop to the left of the midline and the cecum in the right lower quadrant.

Intestinal malrotation, also known as intestinal nonrotation or incomplete rotation, refers to any variation in this rotation and fixation of the GI tract during development. Interruption of typical intestinal rotation and fixation during fetal development can occur at a wide range of locations; this leads to various acute and chronic presentations of disease. The most common type found in pediatric patients is incomplete rotation predisposing to midgut volvulus, requiring emergent operative intervention.[1, 2]

The first reports of intestinal malrotation were based on surgical and autopsy findings and occurred prior to 1900; however, the first description of the embryologic process of intestinal rotation and fixation was not published until 1898.[3] In 1923, Dott was the first to describe the relationship between embryologic intestinal rotation and surgical treatment.[4] In 1936, William E. Ladd wrote the classic article on treatment of malrotation. His surgical approach, now known as the Ladd procedure, remains the cornerstone of practice today.[5]

Pathophysiology

Intestinal malrotation occurs due to disruption of the normal embryologic development of the bowel. Understanding of normal abdominal development aids in the understanding of the etiology of the clinical findings seen with malrotation.

  • Normal embryologic development of the alimentary tract:
    • The alimentary tract develops from the embryologic foregut, midgut, and hindgut. Normal rotation takes place around the superior mesenteric artery (SMA) as the axis. It is described by referring to 2 ends of the alimentary canal, the proximal duodenojejunal loop and the distal cecocolic loop, and is usually divided into 3 stages. Both loops make a total of 270° in rotation during normal development. Both loops start in a vertical plane parallel to the SMA and end in a horizontal plane. See the image below.
Normal rotation of the intestines during developme Normal rotation of the intestines during development. The superior mesenteric artery (SMA) is the axis. The duodenojejunal loop (red arrow) begins superior to the SMA, and the cecocolic loop (green arrow) begins inferior to the SMA.

 

See the list below:

  • Stage I occurs between 5-10 weeks' gestation. It is the period of physiologic herniation of the bowel into the base of the umbilical cord. The duodenojejunal loop begins superior to the SMA at a 90° position and rotates 180° in a counterclockwise direction. At 180°, the loop is to the anatomical right of the SMA, and by 270°, it is beneath the SMA. The cecocolic loop begins beneath the SMA at 270°. It rotates 90° in a counterclockwise manner and ends at the anatomical left of the SMA at a 0° position. Both loops maintain these positions until the bowel returns to the abdominal cavity. Also during this period, the midgut lengthens along the SMA, and, as rotation continues, a broad pedicle is formed at the base of the mesentery. This broad base protects against midgut volvulus.
  • Stage II occurs at 10 weeks' gestation, the period when the bowel returns to the abdominal cavity. As it return s, the duodenojejunal loop rotates an additional 90° to end at the anatomical left of the SMA, the 0° position. The cecocolic loop turns 180° more as it reenters the abdominal cavity. This turn places it to the anatomical right of the SMA, a 180° position.
  • Stage III lasts from 11 weeks' gestation until term. It involves the descent of the cecum to the right lower quadrant and fixation of the mesenteries.

Nonrotation:

  • Arrest in development at stage I results in nonrotation. Subsequently, the duodenojejunal junction does not lie inferior and to the left of the SMA, and the cecum does not lie in the right lower quadrant. The mesentery in turn forms a narrow base as the gut lengthens on the SMA without rotation, and this narrow base is prone to clockwise twisting leading to midgut volvulus. The width of the base of the mesentery is different in each patient, and not every patient with nonrotation develops midgut volvulus.

Incomplete rotation:

  • Stage II arrest results in incomplete rotation and is most likely to result in duodenal obstruction. Typically, peritoneal bands running from the misplaced cecum to the mesentery compress the third portion of the duodenum. Depending on how much rotation was completed prior to arrest, the mesenteric base may be narrow and, again, midgut volvulus can occur. Internal herniations may also occur with incomplete rotation if the duodenojejunal loop does not rotate but the cecocolic loop does rotate. This may trap most of the small bowel in the mesentery of the large bowel, creating a right mesocolic (paraduodenal) hernia.

Incomplete fixation:

  • Potential hernia pouches form when the mesentery of the right and left colon and the duodenum do not become fixed to the retroperitoneum. If the descending mesocolon between the inferior mesenteric vein and the posterior parietal attachment remains unfixed, the small intestine may push out through the unsupported area as it migrates to the left upper quadrant. This creates a left mesocolic hernia with possible entrapment and strangulation of the bowel. If the cecum remains unfixed, volvulus of the terminal ileum, cecum, and proximal ascending colon may occur.

Epidemiology

Frequency

United States

Intestinal malrotation occurs in between 1 in 200 and 1 in 500 live births.[6, 7] However, most patients with malrotation are asymptomatic, with symptomatic malrotation occurring in only 1 in 6000 live births.[8] Symptoms and diagnosis may occur at any age, with some reports of prenatal diagnosis of intestinal malrotation.[9]

Malrotation may occur as an isolated anomaly or in association with other congenital anomalies; 30-62% of children with malrotation have an associated congenital anomaly. All children with diaphragmatic hernia, gastroschisis, and omphalocele have intestinal malrotation by definition. Additionally, malrotation is seen in approximately 17% of patients with duodenal atresia and 33% of patients with jejunoileal atresia.[10, 11]

Mortality/Morbidity

Data from recent series reveal that mortality rates in adults and children operated on for intestinal malrotation range from 0-14%. Higher mortality rates are seen in cases with acute onset of midgut volvulus, delayed diagnosis, or the presence of intestinal necrosis.[12, 13, 14, 15, 1, 16] Children with other associated anomalies also have higher overall mortality rates. A report of 25 years' experience demonstrated congenital cardiovascular disease in 27.1% of patients with intestinal malrotation; those patients had a morbidity rate of 61.1% after intestinal malrotation surgery.[17]

Sex

Male predominance is observed in neonatal presentations at a male-to-female ratio of 2:1. No sexual predilection is observed in patients older than 1 year.

Age

Traditional teaching suggests that as many as 40% of patients with malrotation present within the first week of life, 50% in the first month, and 75% in the first year. However, more recent series have shown that malrotation is increasingly identified in adults. A series of 170 patients with intestinal malrotation diagnosed at a single institution between 1992-2009 found that 31% were infants, 21% were aged 1-18 years, and the remaining 48% were adults.[18] Although unusual, there are reports of adults presenting with total small bowel volvulus due to malrotation in adults.[19] A second series found that 42% of patients with a new diagnosis of malrotation were adults.[20]  Reports have even documented congenital malrotation presenting during pregnancy.[21, 22, 23]

 

Presentation

History

Intestinal malrotation can present as either an acute or chronic process. Additionally, various types of rotational defects are recognized. The history of present illness varies depending on these different factors.

Acute midgut volvulus

See the list below:

  • Usually occurs during the first year of life

  • Sudden onset of bilious emesis

  • Diffuse abdominal pain out of proportion to physical examination

Chronic midgut volvulus

See the list below:

  • Chronic midgut volvulus is due to intermittent or partial twisting that results in lymphatic and venous obstruction.

  • The most common symptoms are recurrent abdominal pain and malabsorption syndrome.[24]

  • Further history taking among older patients with acute midgut volvulus may reveal presence of missed diagnosis of chronic midgut volvulus.

  • Other clinical features include recurrent bouts of diarrhea alternating with constipation, intolerance of solid food, obstructive jaundice, and gastroesophageal reflux.[25]

Acute duodenal obstruction

See the list below:

  • This anomaly is usually recognized in infants and is due to compression or kinking of the duodenum by peritoneal bands (Ladd bands).

  • Patients present with forceful vomiting, which may or may not be bile-stained, depending on the location of the obstruction with respect to the entrance of the common bile duct (ampulla of Vater).

Chronic duodenal obstruction

See the list below:

  • The typical age at diagnosis ranges from infancy to preschool-age.

  • The most common symptom is vomiting, which is usually bilious.

  • Patients may also have failure to thrive and intermittent abdominal pain (frequently diagnosed as colic).

Internal herniation

See the list below:

  • Internal herniation usually causes chronic symptoms.

  • Patients have recurrent abdominal pain, which may progress from intermittent to constant.

  • They experience vomiting as well as constipation at times.

  • They are often diagnosed with psychosocial problems.

Physical

Physical examination findings may vary depending on the type of rotational defect as well as whether the symptoms are chronic or acute in onset.

Acute midgut volvulus

See the list below:

  • This is usually associated with abdominal distention with diffuse tenderness and guarding on examination.

  • Prolonged volvulus leads to vascular compromise, which can cause intraluminal bleeding evidenced by melena and/or hematemesis.

  • Worsening intestinal ischemia can lead to signs of shock including poor perfusion, decreased urine output, hypotension, elevated lactate, and base deficit.

  • Other signs of intestinal ischemia include peritonitis and discoloration of the skin.

Chronic midgut volvulus

See the list below:

  • Physical examination findings vary depending on whether the volvulus is present at the time of examination

  • If partial or complete volvulus is present at the time of examination, the patient may have signs and symptoms equivalent to those of acute midgut volvulus.

  • Patients usually have some degree of abdominal pain and distention.

Acute duodenal obstruction

See the list below:

  • Abdominal distention and gastric waves may be present.

  • Passage of meconium or stool can be present.

  • These patients usually do not have signs of peritonitis or shock, unless volvulus is also present distal to the obstruction.

Chronic duodenal obstruction

See the list below:

  • Physical examination findings may be completely normal at the time of presentation.

  • Abdominal distention and tenderness may be present.

  • Diagnosis is usually made by history and a high level of clinical suspicion; physical examination findings are very unreliable.

Internal herniation

See the list below:

  • Physical examination findings can be unremarkable; diagnosis is made by a high index of clinical suspicion and radiologic studies.

  • Patients with left mesentericoparietal hernias may have findings related to venous obstruction, such as hematochezia, hemorrhoids, and dilated anterior abdominal veins.

  • If acute obstruction is present at the time of presentation, guarding and tenderness may be present and a globular mass may be palpated at the location of the hernia.

Causes

See Pathophysiology.

 

DDx

Differential Diagnoses

 

Workup

Laboratory Studies

The following studies may help facilitate the diagnosis and guide treatment in patients with suspected intestinal malrotation:

CBC count

An elevated or decreased WBC count may indicate systemic inflammation and/or sepsis.

Thrombocytopenia may indicate a platelet consumptive process (eg, necrotizing enterocolitis); additionally, platelets are an acute phase reactant with thrombocytosis indicating systemic inflammation.

A decreased hemoglobin/hematocrit gives evidence of blood loss, possibly through GI bleeding.

Arterial, capillary, or venous blood gas and lactate

Metabolic acidosis provides evidence for ongoing ischemia as observed with necrotizing enterocolitis or strangulated bowel (volvulus). This is demonstrated on serum laboratory studies with an elevated lactate and/or base deficit

Blood chemistries

Patients with volvulus and resultant bowel ischemia can develop severe metabolic derangements, which should be corrected, if possible, prior to operative intervention.

Ongoing sodium, chloride, and bicarbonate losses can occur through suctioned GI secretions.

Hyperkalemia may occur secondary to metabolic acidosis and hemolysis.

Urinalysis and urine culture

These may be useful to rule out other infectious causes if the differential remains unclear.

Type and screen

A current type and screen must be available because these patients often require emergent operative intervention which may or may not require blood transfusion.

Prothrombin time (PT) and activated partial thromboplastin time (aPTT)

These should be obtained in patients with sepsis, if ongoing blood loss is a concern and prior to operative intervention.

Imaging Studies

The following imaging studies may be helpful in clarifying the diagnosis:

Plain abdominal radiography

Plain radiography has limited use for defining intestinal obstruction. The classic pattern for duodenal obstruction, if present, is the double-bubble sign produced by an enlarged stomach and proximal duodenum with little gas in the remainder of the bowel. Distended bowel loops with or without pneumatosis intestinalis may be seen. If pneumoperitoneum is suspected, a left lateral decubitus film can be obtained to better visualize this process

Upper GI series

Upper GI series is the criterion standard to diagnose intestinal malrotation, with a sensitivity of 93-100%. However, upper GI but should only be obtained in patients who are hemodynamically stable.[26, 27, 28]

Normal rotation is present if the duodenal C-loop crosses the midline and places the duodenojejunal junction to the left of the spine at a level equivalent or superior to the pylorus. If the contrast ends abruptly or tapers in a corkscrew pattern, midgut volvulus or some other form of proximal obstruction may be present. Barium is the contrast of choice in patients who are stable or have chronic symptoms

Contrast studies may not be possible in patients who are actively vomiting or are otherwise unstable and need immediate surgical exploration. Water-soluble agents should be used if the study must be performed prior to emergent operative intervention. See the image below.

In this upper GI series with abnormal results, the In this upper GI series with abnormal results, the duodenum does not cross the midline, and the small bowel is present only in the right side of the abdomen.

In this upper GI series with abnormal results, the duodenum does not cross the midline, and the small bowel is present only in the right side of the abdomen.

Lower GI series (contrast enema)

Occasionally, upper GI series may fail to define the location of the duodenojejunal junction, in which case, a contrast enema may be helpful to identify the location of the cecum.

A normally placed cecum does not unequivocally rule out intestinal malrotation and clinical judgment must be exercised. See the image below.

These 2 lower GI series show the cecum (arrows) in These 2 lower GI series show the cecum (arrows) in the right upper quadrant, indicative of malrotation.

Ultrasonography

Ultrasonography is quickly becoming a useful imaging modality in infants and children with abdominal pain and has been used to accurately diagnose intestinal malrotation with or without midgut volvulus.[29, 30, 31]

In the hands of experienced ultrasonographers, ultrasonography has been shown to have false positive rate of 0% and a false negative rate of 0-30%.[32, 33]

Findings indicative of malrotation include inversion of the superior mesenteric artery (SMA) and the superior mesenteric vein (SMV) and a retroperitoneal position of the duodenum after fluid bolus via a nasogastric tube.[32]

Other diagnostic findings include fixed midline bowel loops and duodenal dilation with distal tapering. The finding of the SMV coiled around the SMA is suggestive of intestinal volvulus The presence of ascites and thickened bowel wall were not found to be statistically significant predictors of malrotation with midgut volvulus

CT scanning

CT scanning is not well developed for diagnosing malrotation and midgut volvulus among patients with acute onset of symptoms. However, CT scan may have more utility in diagnosing midgut volvulus among patients with chronic presentations.[34]

Scattered case reports of its use are noted, but it is not recommended as the principal diagnostic tool.

Procedures

The following procedures may be helpful:

NG tube insertion

Insert an NG tube in all patients with bilious emesis and suspected malrotation.

Adjust the NG tube to low intermittent suction in order to decompress the bowel proximal to any obstruction that may be present.

Central venous catheter placement

Most patients require long-term intravenous access after surgery, especially if midgut volvulus is present.

Because intravenous nutrition is likely to be necessary, central line access is preferable over peripheral access so that parenteral nutrition can be delivered.

 

Treatment

Medical Care

Medical care of intestinal malrotation is directed toward stabilizing the patient.

Where malrotation with volvulus or obstruction is suspected, seek immediate pediatric surgical consultation. Maintain patients on nothing by mouth (NPO) and adjust NG or orogastric tube to low intermittent suction. Correct fluid and electrolyte deficits. All patients require intravenous resuscitation with physiologic salt solution. Administer broad-spectrum antibiotics prior to surgery, if possible.

If a patient has signs of shock, administer appropriate fluids, blood products, and vasopressor medications to improve hypotension. Dopamine is often used as first-line therapy because of its possible effects to increase splanchnic blood flow. Dopamine can be started at an infusion rate of 3 mcg/kg/min intravenously (IV) and continued postoperatively even if the patient is not hypotensive.

Most patients require long-term intravenous access after surgery, particularly if midgut volvulus is present. Additionally, intravenous nutrition is likely to be necessary. For this reason, central venous catheters should be placed in most patients.

If the patient is unstable, do not delay surgical intervention for upper GI and laboratory studies. Quick surgical intervention, not prolonged medical management, is associated with the best results if midgut volvulus is suspected.

Surgical Care

The Ladd procedure remains the cornerstone of surgical treatment for malrotation today. Prior to William Ladd's publication in 1936, surgical treatment for malrotation with or without volvulus had a mortality rate higher than 90%. In fact, at Ladd's own institution, the mortality rate was 100% before the development of his new technique. A classic Ladd procedure is described as reduction of volvulus (if present), division of mesenteric bands, placement of small bowel on the right and large bowel on the left of the abdomen, and appendectomy. Published reports for laparoscopic Ladd procedure are now appearing in the literature as well.[35, 36]

Midgut volvulus

If midgut volvulus is present, the entire small intestine along with the transverse colon is delivered out of the abdominal incision, where the volvulus can be reduced. Because the volvulus usually twists in a clockwise direction, reduction is accomplished by twisting in a counterclockwise direction. Complete detorsion usually requires 2-3 twists of the bowel. After the blood supply has been restored by detorsion, the surgeon must make a decision about viability of the involved bowel. The outcome is better when no gangrenous bowel is present or when a small, localized gangrenous segment is present, which can be resected and a primary anastomosis performed.

If multiple areas of questionable viability are present, many surgeons choose to leave the areas and perform a second-look operation in 12-24 hours if the patient is not showing clinical recovery. See the image below.

This patient had malrotation with midgut volvulus. This patient had malrotation with midgut volvulus. The gut is darkened in color because of ischemia.

Grossly necrotic bowel should be resected. Primary anastomosis versus diversion of the fecal stream with a proximal ostomy should be performed at the surgeon’s discretion. Three principles should be used to guide decision making:

  • The highest priority is to preserve the maximum length of intestine required for survival.

  • Questionably viable bowel should not be anastomosed.

  • Resection of the entire small bowel require life-long parenteral nutrition or small bowel transplant.[37]

Duodenal obstruction

After the volvulus is reduced or if no volvulus was present, identify any extrinsic obstruction to the duodenum.

If peritoneal bands crossing the duodenum are found, ligate them with careful attention to protecting the superior mesenteric vessels. The bands may also obstruct the ileum or the jejunum and sometimes run to the gallbladder and liver.

Extrinsic obstruction may also be due to the cecum, colon, or superior mesenteric artery (SMA), impinging on the duodenum; relief is obtained by placing the cecum with its mesentery in the left upper quadrant and exposing the anterior duodenum through its entire length. After extrinsic obstruction has been relieved, determine that no intrinsic obstruction exists by passing an NG tube through the duodenum.

Appendectomy

Appendectomy is performed during operation for malrotation for 2 reasons[38] :

  • The normal anatomical placement of the appendix is disrupted when the cecum is placed in the left upper quadrant, making the diagnosis of future appendicitis challenging.
  • Dissection of the peritoneal bands causes damage to the appendiceal vessels.

Laparoscopy

Use of a laparoscopic approach to the correction of malrotation is feasible (level III, level IV evidence) but long-term outcome data is lacking. An open approach is thought to create adhesions, thereby reducing the risk of recurrent volvulus. Critics of the laparoscopic approach cite that laparoscopy is associated with minimal adhesion formation and inadequate widening of the mesentery and thus may not afford the patient comparable long-term benefit.[39, 40, 41]

Laparoscopy has been used to repair malrotation in clinically stable patients with or without signs of duodenal obstruction without midgut volvulus.

The Ladd procedure, including widening of the mesenteric base and dissection of peritoneal bands, has been performed successfully using a laparoscopic approach and has resulted in shorter hospital length of stay.

Laparoscopic Ladd procedure is now accepted as an initial approach to surgical correction, with recent reports showing superior short-term results including shorter term to full diet without any increase in operative duration.[42, 43, 44, 45]

Some authors have reported on the success of single-incision laparoscopic Ladd procedure for intestinal malrotation without volvulus in adults.[46]

A study reported that in children aged 6 months or older with suspicion of intestinal malrotation but not presenting with an acute abdomen or hemodynamically instability, laparoscopy should be considered as a first approach to diagnose and subsequently treat intestinal malrotation.[47]

 

Heterotaxy

Patients with heterotaxy syndromes have increased rates of abnormalities of intestinal rotation. The approach to the management of these rotational abnormalities is controversial.[48]

Some authors advocate for screening of all children with an upper GI study and elective correction of any rotational abnormality.[48] However, a retrospective review of 29 children who underwent Ladd procedure revealed that the complication rate was 57% among patients with heterotaxy who were otherwise asymptomatic, compared with only 9% in the symptomatic, nonheterotaxy group.[49] These authors recommend that asymptomatic children be managed conservatively with operative intervention reserved for those children who become symptomatic. A systematic review by Cullis et al found that out of 414 asymptomatic patients that had no screening for intestinal rotation, one patient developed malrotation symptoms which was confirmed by laparotomy.[50]

Consultations

Consultations with the following specialists may be helpful:

  • Pediatric surgeon: The only definitive treatment for malrotation is surgical in nature

  • Dietary/intravenous nutrition team: In hospitals where available, include a nutritionist in the medical team to monitor total parenteral nutrition to ensure optimal nutrition. Children who undergo extensive small bowel resection require long-term nutritional support and benefit from early consultation with the nutrition team.

  • Pediatric gastroenterologist: Consultation with a gastroenterologist should be considered early in the postoperative course, particularly in those children who have undergone extensive small bowel resection due to the need for long-term parenteral nutrition and the resulting cholestasis in this population.

  • Physical therapist: A physical therapist can help with range of motion exercises and strength conditioning while patients are bedridden. Frequent repositioning also helps prevent decubitus pressure ulcers and head molding in infants.

  • Occupational therapist: Occupational therapists can also assist with splinting of extremities that contain central lines to prevent contractures as well as range of motion exercises.

  • Speech therapist: A speech therapist can help with oral stimulation while patients are not being fed. This stimulation can prevent feeding aversion and poor coordination when oral feeds are restarted.

Diet

Feeding strategies are discussed under Further Inpatient Care.

 

Follow-up

Further Outpatient Care

Long-term management of growth and nutrition

Patients with short-bowel syndrome are at high risk for failure to thrive. These infants require frequent monitoring of growth parameters during the immediate postoperative period to ensure adequate weight, length, and head circumference gains.

Patients may develop iron, folate, and vitamin B-12 deficiencies due to malabsorption depending on how much bowel is resected.

Outpatient care should be individualized, depending on each patient’s operative and postoperative course.

Development considerations

Children with prolonged hospitalization may experience developmental delays and require aggressive physical, occupational, and speech therapy.

Infants can develop poor truncal control due to prolonged periods in the supine position while hospitalized, additionally they may develop contractures in the extremities, or feeding aversion due to prolonged periods with NG tubes in place and taking nothing by mouth.

Developmental delays should be monitored in both inpatient and outpatient settings and should be intervened upon as early as possible.[51]

Further Inpatient Care

Immediate postoperative care in patients with intestinal malrotation includes the following:

  • Intravascular volume status should be monitored postoperatively. Patients are typically hypovolemic due to operative blood loss, third spacing of fluids in the edematous bowel, and fluid and electrolyte losses from nasogastric tube decompression. Fluid resuscitation should occur with physiologic salt solution and blood products as needed.

  • Broad-spectrum antibiotics should be continued at the discretion of the surgeon.

  • NG tube decompression should be continued until bowel function returns to normal. Patients with midgut volvulus have a longer time to return of bowel function than those without volvulus; additionally, infants also experience a longer time to return of bowel function than do older children.

Nutrition in the postoperative period is as follows:

  • In the immediate postoperative period, the time to return of bowel function depends on the duration of obstruction and extent of bowel compromise.[37]

  • Most patients require total parenteral nutrition until full oral feedings can be reestablished and should have a central venous catheter placed.

  • Patients may also require intravenous infusion of amino acid solutions to achieve positive nitrogen balance.

  • Adequate nutrition is essential to ensure wound healing and protect from bacterial overgrowth.

  • Infants who are malnourished have longer recovery time.

  • Patients should receive full nutritional support with parenteral nutrition until they are able to consume at least 50% of daily caloric requirement. Parenteral nutrition can then be weaned slowly as full enteral intake is achieved.

Strategies to improve nutrition and considerations for short bowel syndrome are as follows:

  • Enteral feeding can be initiated with elemental formula. In the initial postoperative period, absorptive surface area and enzyme activity are decreased. Volume and concentration of feeds can be advanced as absorptive capacity increases.

  • Infants who undergo extensive small bowel resection are at risk of developing short bowel syndrome. These infants may require long-term parenteral nutrition until the remaining bowel is able to adapt and undergo compensatory growth. In these children, feeding should be initiated with small amounts of enteral nutrition to encourage adaptation of the bowel and provide nutrition for the mucosa. Gastrostomy tube placement may be helpful in this situation and should be assessed on a case-by-case basis.[37]

Transfer

All children suspected of having malrotation with or without midgut volvulus should be transferred to a facility with pediatric surgical support. Furthermore, any child with bilious emesis is assumed to have a surgical problem until proven otherwise.

Complications

Complications include the following:

  • Short-bowel syndrome: Short-bowel syndrome is the most common complication of midgut volvulus. These patients have longer delays to recovery of bowel motility and function. They are at high risk for malabsorption and can require long-term parenteral nutrition. Furthermore, these patients have more complications from treatment and longer hospital stays than patients with malrotation without volvulus.

  • Infection: Wound infections and sepsis can occur in the immediate postoperative period, requiring extended treatment with intravenous antibiotics. Additionally, central venous catheters have the potential to become infected causing bacteremia and/or sepsis

  • Surgical complications: Postoperative and surgical complications are more likely to occur in those patients with acute symptoms than those with chronic symptoms.[14] One review reported an overall complication rate of 8.7% (14 of 161) following Ladd procedure.[52] Complications reported include adhesive small bowel obstruction in 6% with 5 requiring reoperation (3%), and 1 patient developed recurrent volvulus (1%). A second review showed comparable rates of recurrent volvulus (2%, 1 of 57) and reoperation for adhesive small bowel obstruction (2%, 1 of 57).[53] Other series have reported lower rates of recurrent volvulus, 0.4% in one series of 441 patients, and 0.6% in a series of 159 patients who underwent Ladd's procedure.[37]

  • Persistent GI symptoms: In the same series of 57 patients, 13 had persistent (>6 mo) GI symptoms, including constipation (6), intractable diarrhea (1), abdominal pain (2), vomiting (3), and feeding difficulties (1) following Ladd procedure.[53]

  • Mortality: Death occurs due to peritonitis, late nutritional complications, or catheter-related sepsis. Rates are increased among children younger than one year. Following Ladd procedure, mortality rates reported in the literature are as low as 2%.[52] However, if more than 75% of the bowel is necrotic, mortality is as high as 65%.[37]

Prognosis

Prognosis is dependent on the presence of ischemic or necrotic bowel, the amount of bowel resected, and the age of the child.  In general, older children have improved morbidity and mortality compared to infants. The presence of midgut volvulus is associated with prolonged hospital length of stay.  Long-term prognosis is dependent on how much bowel is resected and the development of short-bowel syndrome.

 

Questions & Answers

Overview

What is intestinal malrotation?

What is the pathophysiology of intestinal malrotation?

What is the prevalence of intestinal malrotation?

What is the mortality and morbidity associated with intestinal malrotation?

What are the sexual predilections of intestinal malrotation?

Which age groups have the highest prevalence of intestinal malrotation?

Presentation

Which clinical history findings are characteristic of acute midgut volvulus?

Which clinical history findings are characteristic of chronic midgut volvulus?

Which clinical history findings are characteristic of acute duodenal obstruction?

Which clinical history findings are characteristic of chronic duodenal obstruction?

Which clinical history findings are characteristic of internal intestinal herniation?

Which factors determine the clinical presentation of intestinal malrotation?

Which physical findings are characteristic of acute midgut volvulus?

Which physical findings are characteristic of chronic midgut volvulus?

Which physical findings are characteristic of acute duodenal obstruction?

Which physical findings are characteristic of chronic duodenal obstruction?

Which physical findings are characteristic of internal intestinal herniation?

Which factors affect the physical findings of intestinal malrotation?

DDX

What are the differential diagnoses for Intestinal Malrotation?

Workup

What is the role of CBC count in the workup of intestinal malrotation?

What is the role of serum lab tests in the workup of intestinal malrotation?

What is the role of blood chemistries in the workup of intestinal malrotation?

What is the role of urinalysis and urine culture in the workup of intestinal malrotation?

What is the role of blood typing and screening in the workup of intestinal malrotation?

What is the role of PT and aPTT in the workup of intestinal malrotation?

What is the role of plain radiography in the workup of intestinal malrotation?

What is the role of an upper GI series in the workup of intestinal malrotation?

What is the role of contrast enema in the workup of intestinal malrotation?

What is the role of ultrasonography in the workup of intestinal malrotation?

What is the role of CT scanning in the workup of intestinal malrotation?

What is the role of an NG tube in the workup of intestinal malrotation?

What is the role of central venous catheter placement in the workup of intestinal malrotation?

Treatment

How is intestinal malrotation treated?

What is the role of surgery in the treatment of intestinal malrotation treated?

What is the surgical treatment for midgut volvulus?

What is the surgical treatment for duodenal obstruction?

What is the role of appendectomy in the treatment of intestinal malrotation?

What is the role of laparoscopy in the treatment of intestinal malrotation?

How is intestinal malrotation treated in patients with heterotaxy syndromes?

Which specialist consultations are beneficial to patients with intestinal malrotation?

Follow-up

What is included in the long-term monitoring of patients with intestinal malrotation?

How are developmental delays treated in children with intestinal malrotation?

What is included in immediate postoperative care following surgery for intestinal malrotation?

How is malnutrition prevented following surgery for intestinal malrotation?

When is patient transfer considered for the treatment of intestinal malrotation?

What are the possible complications of intestinal malrotation?

What is the prognosis of intestinal malrotation?