What is Midgut Volvulus?
A volvulus is a medical condition where the intestines twist around themselves. This can happen at any age, but it’s more commonly found in babies and young children. A volvulus can cause a blockage, interrupting the intestines’ blood supply.
This often happens due to a birth defect known as intestinal malrotation, but it can also occur in any part of the intestine without this condition being present. Intestinal malrotation can increase the risk of a baby developing a specific type of volvulus, called a midgut volvulus, within the first few weeks after birth. A midgut volvulus usually involves a blockage of blood vessels in the membrane connected to the intestines, also known as the intestinal mesentery, especially if intestinal malrotation is present.
What Causes Midgut Volvulus?
When a baby is developing in the womb, their intestines form in three parts: the foregut, midgut, and hindgut. Between the 4th and 6th weeks of pregnancy, the midgut part of the intestines undergoes normal rotation. The intestines develop so fast at this time that they can’t all fit inside the abdomen. As a result, a part of the intestines moves out into the umbilical cord.
Between the 8th and 10th week of pregnancy, these intestines move back into the abdomen. As they do this, they rotate 270 degrees in a counter-clockwise direction. This rotation occurs around the main blood supply to the midgut, known as the superior mesenteric artery.
The fourth part of the duodenum, the upper section of the small intestine, and the beginning part of the jejunum, another part of the small intestine, form a shape that looks like a “C”. This happens with the development of a particular structure called the ligament of Treitz, and this structure and the “C” it forms are positioned above the superior mesenteric artery.
The cecal part of the intestine, which connects the small intestine to the large intestine, then becomes fixed to the back inside wall of the abdomen, above the superior mesenteric artery. This is placed diagonally from around the level of the T12 vertebra, which is near the bottom of the spine between your shoulder blades, to the right of the L5 vertebra in your lower back.
Risk Factors and Frequency for Midgut Volvulus
Anomalies of rotation and fixation, refer to abnormal development or placement of certain organs. These conditions occur twice as often in males compared to females. These anomalies can be categorized into four types:
- Non-rotation
- Incomplete rotation
- Reverse rotation
- Anomalous fixation of the mesentery.
Signs and Symptoms of Midgut Volvulus
Midgut volvulus is a medical condition that usually happens within the first few weeks after birth, but can occur at any age. The main symptom is green (bilious) vomiting. In babies, it may appear suddenly along with signs like a bloated upper belly, belly tenderness, a fast decrease in blood pressure and health, and blood in the stool. These could signify a twisted intestine (volvulus) due to misplaced intestines (intestinal malrotation). If not promptly treated, it can cause severe issues leading to a drop in blood circulation. Advanced stages of the illness might show signs of inflammation of the belly lining due to interruption of blood supply to the intestines, including redness and swelling of the belly skin. The symptoms may not show up strongly at the beginning, so staying alert about this condition is crucial.
In older kids and adults, the most common symptom is belly pain. It can start suddenly over hours or days, or it can come and go over weeks, months, or even years. Other potential symptoms may include nothing and on—or regularly repeating—vomiting, chronic diarrhea, malabsorption, or failure to thrive.
- Green (bilious) vomiting
- A bloated upper belly
- Belly tenderness
- A fast decrease in blood pressure and health
- Blood in the stool
- Sudden or long-term belly pain
- On and off vomiting
- Chronic diarrhea
- Malabsorption
- Failure to thrive
Testing for Midgut Volvulus
When a newborn is suspected of having a medical condition, the baby might not always show clear signs of being sick. This is why it’s crucial for the doctors to use imaging tests to examine the baby’s body internally. One common imaging test used is the abdominal X-ray, which typically shows little gas in the intestine and few air-fluid levels for babies with this suspected condition.
If these signs are present, doctors will immediately work to stabilize the baby by providing them with sufficient fluids to ensure proper blood flow and urine output. Once the child is stable, surgery can be considered. A type of minimally invasive surgery called laparoscopy is often an option.
If the child is stable and there is no evidence of a serious complication known as peritonitis (inflammation of the lining of the abdomen), an upper gastrointestinal series, which is a series of X-rays of the upper digestive system, can be performed. This X-ray will often reveal a malrotation, meaning that the intestine did not rotate into the right position as it formed. Specifically, the location of the duodenojejunal junction, where the stomach empties into the small intestine, shifts from its usual position to the right side of the spine. This can result in various complications like volvulus (twisting of the intestine) or duodenal obstruction (blockage in the small intestine), both of which can be identified by unusual patterns like the “corkscrew effect” on the X-ray.
A barium enema, which is a type of X-ray of the bowel, may show an abnormal position of the cecum, a part of the large intestine, but this sign can be unreliable, especially in a small baby. A more reliable method could be an ultrasound of the abdomen, which can also reveal midgut volvulus, another type of intestinal twisting. Certain unusual positions of blood vessels and a sign known as whirlpool sign (vessels swirling around the base of the mesentery, which is the tissue that attaches the intestines to the back wall of the abdomen) can help doctors confirm this diagnosis.
Treatment Options for Midgut Volvulus
When a condition called volvulus, which is a twisting of the intestines, is suspected, it’s crucial to get surgical treatment as soon as possible. This is important to prevent or reverse a lack of blood supply to the twist, which could be harmful. The surgeon untwists the intestines in the opposite direction that it twisted – much like the phrase “turning back the hands of time” suggests.
The Ladd’s procedure, named after its creator Dr. William Edward Ladd, is often done following the untwisting of the intestines. This surgical procedure doesn’t fix the original cause of the twisting but rather prevents it from happening again. The surgeon does this by cutting bands of tissues between the cecum (part of the large intestine), the lateral abdominal wall, the duodenum (beginning of the small intestine) and the terminal ileum (end of the small intestine). This allows a major artery, the superior mesenteric artery, to relax and prevents recurrent twisting of the intestine. During this procedure, the appendix is also generally removed to prevent confusion in future medical imaging.
Traditionally, an open Ladd’s operation (a larger incision in abdomen) is performed. However, increasingly surgeons are performing it laparoscopically which means smaller incisions. This modern method is associated with faster recovery and shorter hospital stays. When a patient presents advanced blood supply deprivation, a simpler reduction of the volvulus is performed without the Ladd procedure. The surgeon would then recheck after 24 to 36 hours for any damage to the blood supply of the intestines.
In some cases when a portion of the intestine has been damaged extensively, the surgeon may use a transparent plastic silo to regularly monitor the intestine and plan for further surgery. If a part of the intestine is dead, it might need to be removed, but conservatively so as to maintain enough length for feeding and to prevent a condition known as short-gut syndrome. Early diagnosis and treatment dramatically improves the outcome. Delay can not only lead to death, but may also result in a shortage of useable intestine, requiring a transplant.
What else can Midgut Volvulus be?
When a baby has midgut volvulus, doctors need to consider a number of other conditions that could be causing the symptoms. These could include:
- A bowel obstruction in a newborn infant
- A birth defect known as a ‘congenital band’
- Intestinal or stomach twisting, also called ‘intestinal volvulus’
- A serious intestinal condition known as ‘necrotizing enterocolitis’
- A dangerous infection called ‘neonatal sepsis’
- A condition causing a blockage in the small intestine, called ‘pediatric duodenal atresia’
- Acid reflux in infants, also known as ‘pediatric gastroesophageal reflux’
Possible Complications When Diagnosed with Midgut Volvulus
If the diagnosis or surgical treatment of mesenteric ischemia is delayed, it can result in gangrene or decay, often affecting most of the small intestine. In such cases, the decayed part of the intestine usually needs to be removed, a process known as resection. This could result in short bowel syndrome. Another commonly reported complication is the development of small bowel obstruction due to adhesions, which are bands of scar-like tissue.
Common Complications:
- Gangrene in the small intestine.
- Resection of decayed intestinal parts, leading to short bowel syndrome.
- Small bowel obstruction due to adhesions.
Recovery from Midgut Volvulus
Even though there’s no bowel opening included in the surgery, some patients might experience a delay before they’re able to eat or drink normally again. This is often seen in patients who have significant swelling in their stomach or duodenum (the first part of the small intestine), patients whose intestines aren’t moving or contracting properly, or patients who’ve had diseased portions of their intestines removed and sewn back together.
In these scenarios, a nasogastric tube (a tube that passes through the nose and down to the stomach) can be really useful for helping to relieve pressure in the bowel. If the patient needs to fast for an extended period due to a significant portion of the small bowel being removed, then doctors might consider total parenteral nutrition (this is when nutrients are provided directly into the bloodstream).
Kids who weren’t growing as they should before the surgery might also need to be closely monitored after they leave the hospital to ensure healthy growth is back on track.
