Intestinal Malrotation (Volvulus)

Intestinal malrotation is a congenital condition that affects the position of the intestines.

When the intestines form during fetal development, they coil and rotate into the proper position and attach to the abdominal wall. If the intestines do not coil and rotate properly, it is called intestinal malrotation.

Intestinal malrotation can cause a problem called volvulus, where the intestines twist in ways they should not. This can cause blockages that prevent stool from passing. Volvulus can also cut off blood and oxygen flow, potentially resulting in death of some or all of the intestines.

Learn more about intestinal malrotation and volvulus, including its definition, causes, symptoms, diagnosis, treatment, and potential risks and complications.

What is intestinal malrotation?

Intestinal malrotation occurs when the intestines do not rotate correctly during fetal development, resulting in abnormal positioning. This can lead to volvulus (twisting of the intestines), which can obstruct blood flow and cause bowel obstruction. Although malrotation often presents in infancy, it can sometimes remain asymptomatic until later in life.

Causes of intestinal malrotation

Intestinal malrotation occurs during embryonic development, before a baby is born.

During fetal development, the intestines normally undergo a complex process of rotation and fixation within the abdomen. Malrotation occurs when this process is incomplete or abnormal.

Symptoms of intestinal malrotation

Signs of malrotation and volvulus can vary, but they often include frequent vominiting and belly pain.

Symptoms of malrotation in infants

• Bilious vomiting: Green or yellow vomit, a key sign of bowel obstruction.
• Abdominal pain and distension: Visible swelling of the abdomen and discomfort.
• Feeding intolerance: Difficulty in feeding, reduced appetite, and weight loss.
• Lethargy: Unusual tiredness and lack of energy.
• Bloody stools: Presence of blood in stool, indicating possible bowel ischemia.

Symptoms of malrotation in older children and adults

• Intermittent abdominal pain: Recurring episodes of pain, often associated with bowel obstruction.
• Nausea and vomiting: Persistent nausea and vomiting that can be bilious.
• Malabsorption and growth issues: Poor nutrient absorption due to improper bowel alignment.
• Weight loss: Unintended weight loss due to malabsorption or digestive issues.

Diagnosis of intestinal malrotation

To diagnose intestinal malrotation, a health care provider may use tests including:

• Symptom assessment: Detailed inquiry about the presence, onset, and severity of symptoms such as vomiting, pain, and feeding intolerance.
• Physical examination: Examination of the abdomen to detect signs of distension, tenderness, and bowel sounds.
• Abdominal X-ray: Initial imaging to look for signs of bowel obstruction, volvulus, or abnormal gas patterns.
• Upper gastrointestinal (GI) series: A specialized X-ray using contrast dye to visualize the position and movement of the intestines, often showing the “corkscrew” or “bird’s beak” appearance of volvulus.
• Ultrasound: Non-invasive imaging to assess the position of the intestines and detect volvulus, looking for the “whirlpool sign.”
• CT scan: Detailed cross-sectional imaging to provide comprehensive views of abdominal organs and detect malrotation and volvulus.
• Barium enema: An imaging test where barium contrast is introduced through the rectum to visualize the colon and lower intestines, helping to confirm malrotation.

Treatment of intestinal malrotation

Volvulus is a serious medical emergency that requires immediate surgical intervention to untwist the intestines and restore blood flow, preventing bowel necrosis.

Surgical treatment for intestinal malrotation may include:

• Ladd’s procedure: A surgery to release abnormal bands of tissue, untwist the intestines, and position the intestines to minimize future twisting.
• Kareem’s procedure: A surgery to rotate the intestines to their proper position and connect them to the abdominal wall.

Post-operative care

• Monitoring: Close monitoring in a health care facility for recovery and to manage any complications.
• Pain management: Administration of pain relief medications to ensure comfort during recovery.
• Nutrition: Gradual reintroduction of feedings, starting with clear liquids and advancing as tolerated under medical supervision.
• Wound care: Instructions on keeping the surgical area clean and allowing it to heal properly.

Long-term care

• Follow-up appointments: Routine follow-up with the surgical team to monitor recovery, assess bowel function, and address any complications.
• Nutritional support: Continued assessment and management of nutritional status, especially in cases of malabsorption.
• Developmental monitoring: Ensuring appropriate growth and development in infants and children through regular pediatric check-ups.

Potential risks and complications

Complications of untreated malrotation

• Volvulus: Twisting of the intestines leading to bowel obstruction, ischemia, and necrosis.
• Intestinal ischemia: Reduced blood flow to bowel segments, potentially leading to tissue death and perforation.
• Peritonitis: Inflammation of the abdominal lining due to bowel perforation, causing severe infection and sepsis.

Intestinal malrotation is a serious congenital condition that requires prompt diagnosis and treatment to prevent life-threatening complications. Understanding the causes, symptoms, diagnostic methods, and treatment options is essential for effective management. If your child is experiencing symptoms of intestinal malrotation or has a known diagnosis, consulting with a health care provider will help develop a comprehensive treatment plan tailored to your specific needs and circumstances.