Malrotation of the bowel, or intestinal malrotation, is a birth defect that causes imperfect development of the digestive tract during fetal growth in utero. As the intestine in a fetus grows from its beginning in the form of a straight tube into the various, complex components of the digestive system, the intestine must fully rotate around the superior mesenteric artery during the stage where it returns to the abdominal cavity. When the intestine fails to correctly rotate, it is known as malrotation. This condition also affects the proper positioning of the end of the small intestine (the cecum). Subsequent tissue growth is then also affected, leading to a condition called Ladd’s bands. Ladd’s bands are rings of tissue between the cecum and wall of the small intestine that can obstruct the passage of food through the bowel, thereby impeding digestive processes — a potentially life-threatening complication. Depending on which stage the normal pattern for intestinal development is stopped or disrupted, different types of intestinal malrotation can occur which vary in their severity: nonrotation, incomplete rotation, and incomplete fixation. If, in the latter stages of intestinal growth, portions of the developing digestive tract fail to fuse in the right places in the right way, small spaces may be left through which the intestine can slip and become choked.
Infants born with intestinal malrotation may be in danger of intestinal blockage from Ladd’s bands or from a volvulus — a twisting of the intestine that can cause an obstruction in the passage of food and damage to other parts of the digestive system by impeding blood circulation to those areas. When a volvulus causes a critical disruption in the flow of blood, a surgical emergency may exist. If the volvulus is not corrected, portions of the small intestines can become necrotic and die within hours of the onset of the volvulus.
One out of every five hundred infants is born with some form of intestinal malrotation. Approximately three-quarters of cases are diagnosed within the first year after birth. Twice as many boys as girls tend to show symptoms within the first month to one year after birth; though, both sexes are afflicted by symptoms of malrotation equally after one year of age.
Symptoms of malrotation do not normally appear until complications — some kind of intestinal obstruction or volvulus arise. Not all children with intestinal malrotation will develop complications due to a intestine malrotation, but those who do are usually infants under one year of age. The symptoms depend on the severity and type of malrotation, and on the age of the child.
Common symptoms of intestinal obstruction (because of twisting or from blockage by Ladd’s bands, or other reasons) include, but are not limited to the list below. Individual children may present with any one of a number of symptoms from the list but may react differently depending on severity of the symptom and demeanor of the child.
Professional medical help should be sought immediately if a child is:
- Showing signs of a pattern of acute abdominal cramping that lasts up to a few minutes, then stops and repeats several minutes later.
- Vigorous crying and pulling up the legs,
- Swollen, tender-to-the-touch abdomen
- Green or yellow vomit (bile – a digestive fluid), or vomit that looks like feces (an early sign of malrotation)
- Bloody stools
- Children suffering from malrotation, volvulus, or intestinal obstruction may also display any number of the following symptoms excluding or in addition to those above:
- Rapid heart rate
- Irritability, or crying that cannot be appeased
- Not urinating (due to dehydration from malabsorption of food)
- Lethargy and other behavior uncommon to the child’s disposition
- Extremely irregular stools
- Poor appetite
- Pale in color
Bilious vomiting in an infant with abdominal pain or symptoms should prompt immediate investigation and abdominal imaging by medical personnel.
Accurate description of the symptoms is crucial in correct diagnosis of intestinal malrotation and volvulus. A faster diagnosis may be made if the medical professional is aware of any known congenital defects that the child has, since malrotation happens more frequently in conjunction with conditions such as:
- Other gastrointestinal defects or diseases
- Hernias, especially on the diaphragm
- Cardiac diseases
- Diseases or defects of other organs, especially the spleen or liver
Once an intestinal blockage is presumed, a medical professional should immediately order at least one of several internal imaging procedures — nonsurgical methods of permitting a detailed examination of the functioning of the internal organs, such as an ultrasound, a CT scan, contrast enema, or X-ray. Blood typing and screening should also be performed in expectation of possible emergency surgery. Additional laboratory work-ups are also usually performed, including urinalysis and blood analysis.
Treatment depends on the severity of the malrotation and the general health and condition of the child. A number of different medications and procedures are used to stabilize a child with a suspected intestinal malrotation, but a surgical procedure is almost always eventually necessary. For most cases involving intestinal obstruction, dehydration and infection are prevented through administration of intravenous fluids, and gas build-up in the stomach is relieved and prevented through insertion of a nasogastric tube (small tube inserted through the nose, down the throat to the stomach).
Since volvulus cuts off the blood flow to the intestine, it is a life-threatening condition and must be corrected through surgery without delay. The most common and successful technique for rectifying volvulus and intestinal malrotation is the Ladd procedure: straightening of the intestine, division of Ladd’s bands, placing the small intestine in the right side of the abdomen and the large intestine on the left side, and removal of the appendix. When malrotation occurs, the appendix is located on the left side of the abdomen instead of the right, thus an appendectomy is performed as a preemptive procedure to alleviate the risk of misdiagnosis in any future case of appendicitis. Once the intestine is untwisted, blood flow can be restored; however, if blood supply to the bowels has been cut off for too long, damage can occur, and another surgical procedure is performed within two days to verify the health of the intestine. When permanent damage has occurred, that section of the intestine has to be removed. Although corrective procedures for volvulus, intestinal malrotation, and intestinal obstruction work very well, subsequent intestinal obstruction may happen due to build-up of scar tissue from surgical wounds, but only in rare cases does volvulus reoccur.
The recovery process depends mainly on the rapidity of proper diagnosis, but also on the overall health of the child, a child’s age when symptoms developed, and amount of intestine that sustained damage (if any), among numerous other factors. Children with other congenital defects may be required to maintain a regimen of medication that may include antibiotics, anti-clotting agents, or hypertension medication. When too little intestine is present to process adequate nutrients out of the food — a condition called short-bowel syndrome — children may be placed on a strict nutritional plan or need intravenous nutritional support. Ultimately, children with short gut syndrome may require an intestinal transplant.
Delay in Treatment
Any unnecessary delay in the diagnosis or treatment of malrotation and volvulus can lead to significant and severe injuries to the infant. If a child has lost all or part of his or her small intestines and/or ileocecal valve due to malrotation, the medical records should be gathered and reviewed by an attorney with experience handling medical negligence cases involving bowel malrotation or subsequent volvulus.
Read more about malrotation in newborn babies.