Malrotation: Symptoms, Types, Causes and Treatment

Intestinal malrotation is a congenital anomaly that can have serious, even life-threatening, consequences if not recognized and treated. While it is often detected and managed in infancy, malrotation can sometimes go unnoticed well into childhood or even adulthood, presenting a diagnostic challenge due to its wide variety of symptoms and forms. In this comprehensive article, we break down malrotation into its key aspects—symptoms, types, causes, and treatment—offering an up-to-date, evidence-based overview for patients, caregivers, and clinicians alike.

Symptoms of Malrotation

Malrotation can present with a strikingly broad spectrum of symptoms, ranging from sudden, severe illness in newborns to vague, chronic complaints in adults. Understanding the variability in symptoms is essential for timely recognition and management.

Age Group	Common Symptoms	Presentation Pattern	Source(s)
Infants	Bilious vomiting, abdominal distention	Acute, rapid onset	1 2 3 4 6
Children	Vomiting, failure to thrive, colicky pain	Intermittent, chronic, acute	2 3 4 13
Adults	Abdominal pain, vomiting, food intolerance	Chronic, vague, acute onset	1 2 5 7
All Ages	Intestinal obstruction, volvulus, ischemia	Acute, life-threatening	1 2 3 4 5

Table 1: Key Symptoms by Age Group and Presentation

Symptom Variability by Age

The age at which malrotation presents has a profound effect on its symptoms:

• Infants usually develop symptoms rapidly, often within the first month of life. The classic sign is bilious vomiting, which may be accompanied by abdominal distention. Left untreated, this can progress to bowel obstruction and ischemia due to volvulus—a twisting of the intestine that cuts off blood supply, representing a surgical emergency 1 2 3 4 6.
• Children may have more subtle, intermittent symptoms. These can include recurrent vomiting (sometimes non-bilious), colicky abdominal pain, failure to thrive, and sometimes signs of malabsorption or diarrhea 2 3 4 13.
• Adults often present with vague, chronic abdominal pain, sometimes associated with vomiting, nausea, or intolerance to certain foods. Acute presentations are possible, especially if volvulus develops, but many adults may have had symptoms for years without a correct diagnosis 1 2 5 7.

Acute versus Chronic Presentations

• Acute presentations: Sudden onset of severe abdominal pain, vomiting (often bilious), signs of bowel obstruction, and occasionally peritonitis or shock. These cases often require immediate surgical intervention due to the risk of bowel ischemia and necrosis 1 2 3 4 5.
• Chronic or intermittent presentations: Recurrent or persistent abdominal pain, intermittent vomiting, poor weight gain, and sometimes nonspecific gastrointestinal complaints. These symptoms can lead to diagnostic delays, especially in older children and adults 1 2 3 5.

Unusual or Atypical Presentations

• Some patients, especially older children and adults, may have symptoms like malabsorption, diarrhea, solid food intolerance, or even biliary obstruction. Rarely, malrotation is discovered incidentally during imaging or surgery for unrelated reasons 3 4 7.

Importance of Symptom Recognition

Recognizing the full spectrum of symptomatic presentations is crucial. Delayed diagnosis increases the risk of complications, including potentially fatal bowel necrosis due to volvulus 2 3 4 5.

Types of Malrotation

Malrotation is not a single anatomical anomaly but a spectrum of rotational abnormalities resulting from errors in embryological development. Understanding the different types helps guide diagnosis and management.

Type	Anatomical Feature	Volvulus Risk	Source(s)
Typical/Classic	Malpositioned duodenojejunal (DJ) flexure, cecum	High	8 14
Nonrotation	Complete failure of midgut rotation	Low/Moderate	8 12
Incomplete Rotation	DJ flexure/cecum malpositioned, broad mesentery	Variable	8 14
Reverse Rotation	Colon posterior to SMA/duodenum	High	9 11
Atypical Malrotation	High/low ligament of Treitz, variable anatomy	Lower	14

Table 2: Main Types of Malrotation and Clinical Relevance

Typical (Classic) Malrotation

• The most common form, with the DJ flexure and cecum abnormally positioned, and a narrow mesenteric base—a key risk factor for midgut volvulus 8 14.
• Ladd’s bands, abnormal peritoneal attachments, may cause duodenal obstruction 1 8.

Nonrotation

• Complete failure of the intestines to rotate, resulting in the small bowel on the right and colon on the left.
• Lower risk of volvulus compared to typical malrotation, but still clinically significant 8 12.

Incomplete Rotation

• The intestines partially rotate, creating abnormal positions of the DJ flexure and cecum.
• The mesentery may be broader, reducing but not eliminating the risk of volvulus 8 14.

Reverse Rotation

• The rarest form (2–4% of cases), where the colon lies posterior to the SMA and duodenum.
• Often presents with obstruction or volvulus and is more commonly seen in women 9 11.

Atypical Malrotation

• Includes variations in the position of the ligament of Treitz (high or low, but left of the midline) and other unusual configurations.
• Generally associated with a lower risk of volvulus and internal hernias, but may have increased surgical morbidity and persistent symptoms postoperatively 14.

Subclinical and Incidentally Discovered Malrotation

• Increasingly, malrotation is found incidentally on imaging, especially with the widespread use of CT scans in adults 7.
• Many of these patients may remain asymptomatic, particularly those with partial or atypical malrotation 7 12.

Causes of Malrotation

Malrotation arises from errors during the complex process of gut rotation and fixation in fetal development. These embryological missteps lead to the various anatomic types of malrotation.

Cause/Mechanism	Description	Timing/Association	Source(s)
Abnormal midgut rotation	Failure/incompletion of 270° counterclockwise rotation	4th–12th gestational week	1 8 10 11
Abnormal mesenteric fixation	Incomplete fixation of mesentery, narrow stalk	Congenital	1 8
Genetic/congenital syndromes	Associated with anomalies (e.g., heterotaxy, BWS)	Congenital	10 11
Ladd bands	Peritoneal bands causing duodenal obstruction	Congenital consequence	1 8 9

Table 3: Causes and Mechanisms Underlying Malrotation

Embryological Development and Rotational Errors

• Normal rotation: During fetal life, the midgut undergoes a 270° counterclockwise rotation around the axis of the superior mesenteric artery (SMA). This brings the intestines into their normal positions and allows for proper fixation of the mesentery 1 8.
• Malrotation: Any interruption or error in this process—whether failure, incomplete, or aberrant rotation—results in abnormal positioning of the bowel and sometimes a narrow mesenteric base, predisposing to volvulus 1 8 10 11.

Abnormal Mesenteric Fixation

• Inadequate fixation of the mesentery to the posterior abdominal wall results in a narrow stalk, which allows for excessive mobility and increased risk of twisting (volvulus) 1 8.

Ladd Bands

• These are abnormal peritoneal attachments that run from the cecum to the right lateral abdominal wall, crossing over and potentially obstructing the duodenum. They are a hallmark of malrotation and a key surgical target 1 8 9.

Genetic and Congenital Associations

• Malrotation is more common in patients with other congenital anomalies or syndromes such as heterotaxy and Beckwith-Wiedemann syndrome (BWS). Up to 60% of patients with malrotation may have associated anomalies 10 11.
• The presence of multiple anomalies may complicate diagnosis and management 10.

When Does Malrotation Occur?

• The critical period is between the 4th and 12th weeks of gestation, when midgut rotation and fixation should occur. Disruptions at different stages lead to the variety of malrotation types 1 8 10 11.

Treatment of Malrotation

Treatment strategies for malrotation are tailored according to age, symptom severity, and anatomical findings. The mainstay of therapy is surgical, but controversies remain, particularly for asymptomatic or incidentally detected cases.

Patient Group	Preferred Treatment	Outcome/Controversy	Source(s)
Symptomatic (any age)	Ladd’s procedure (open/laparoscopic)	High symptom resolution; urgent in volvulus	1 2 3 4 8 13 16
Asymptomatic children	Consider prophylactic Ladd’s	Prevents rare complications; debated	12 13 15
Asymptomatic adults	Observation or selective surgery	Surgery rarely indicated; low volvulus risk	12 15
Volvulus/obstruction	Emergency Ladd’s procedure	Life-saving; sometimes bowel resection required	1 2 3 8 13
Atypical malrotation	Individualized approach	Lower volvulus risk, higher morbidity with surgery	12 14

Table 4: Treatment Approaches and Outcomes

The Ladd's Procedure

The Ladd’s procedure, described in 1936, remains the cornerstone of surgical treatment. It involves:

• Detorsion (untwisting) of any volvulus present
• Division of Ladd’s bands to relieve duodenal obstruction
• Broadening of the mesenteric base to reduce volvulus risk
• Appendectomy (to avoid diagnostic confusion in the future)
• Placement of the small intestine on the right and colon on the left 1 2 3 4 8

Outcomes: The majority of symptomatic patients experience resolution of symptoms following surgery. In infants and children, the procedure is almost always recommended if malrotation is diagnosed 1 2 3 4 13.

Laparoscopic versus Open Approach

• Laparoscopic Ladd’s offers benefits such as shorter hospital stays and fewer adhesions but may have a higher risk of recurrent volvulus compared to the open approach 8 16.
• Both approaches are effective, and choice depends on patient stability and surgeon expertise 16.

Emergency Situations

• If volvulus or bowel ischemia is suspected, emergency surgery is essential to prevent life-threatening necrosis. Occasionally, bowel resection is needed if there is irreversible damage 1 2 3 8 13.

Management of Asymptomatic or Incidental Malrotation

• Children: Many experts advocate elective Ladd’s procedure, especially in younger children, to prevent catastrophic volvulus. However, the risk/benefit balance is debated 12 13 15.
• Adults: Most asymptomatic adults can be managed conservatively with observation, as the risk of volvulus is much lower. Surgery is reserved for those who develop symptoms 12 15.
• Atypical/anatomical variants: If imaging shows a broad-based mesentery and malpositioned duodenum without a narrow mesenteric stalk, observation is reasonable as the risk of volvulus is low 12 14.

Postoperative Considerations and Complications

• Adhesive small bowel obstruction is the most common complication post-Ladd’s, particularly after open surgery 3 16.
• Persistent or recurrent symptoms can occur, especially in patients with atypical or complex malrotation 14.
• Mortality is rare with timely intervention, but delays in diagnosis or treatment increase risk 1 2 3 4 13.

Special Situations

• Congenital heart disease or multiple anomalies: Timing of surgery may be influenced by the need for cardiac or other interventions, requiring a multidisciplinary approach 10 12.
• Recurrent volvulus or persistent symptoms: May necessitate reoperation 16.

Conclusion

Malrotation is a complex congenital anomaly with diverse presentations, anatomical types, and management controversies. Prompt recognition and tailored treatment are vital for optimal patient outcomes.

Main Points:

• Symptoms vary by age: Infants present acutely, while older children and adults may have chronic or subtle symptoms 1 2 3 4 5.
• Anatomical types affect risk: Typical malrotation carries the highest volvulus risk; atypical forms have lower risk 8 14.
• Embryological errors are the root cause: Malrotation arises from abnormal gut rotation and fixation during fetal development 1 8 10 11.
• Surgical treatment is standard for symptomatic patients: The Ladd’s procedure is highly effective, especially in infants and children 1 2 3 4 8 13 16.
• Management of asymptomatic cases is debated: Observation may be appropriate, especially in adults and atypical cases 12 15.
• Complications can be severe but are preventable with timely intervention.

By increasing awareness of malrotation’s varied symptoms and presentations, and applying evidence-based approaches to management, outcomes for patients of all ages can continue to improve.