Malrotation and Volvulus

What is malrotation?

Malrotation is an abnormality which affects the bowel and develops while an unborn baby is still in the womb; it is often mentioned alongside volvulus. Volvulus is a complication of malrotation, which occurs when the bowel is twisted and the blood supply to part of the bowel is consequently cut off. Malrotation occurs when the bowel does not develop normally. At the beginning of a pregnancy the bowel is a long tube-like straight organ, which coils up to fit in the limited space available after around the 10th week of pregnancy. If the bowel is not coiled in the normal position, this is known as malrotation.

Around 60 percent of cases of malrotation are diagnosed in infants under the age of 1 month. Malrotation can affect boys and girls but boys are more likely to be diagnosed during infancy. Malrotation affects around 1 in every 2,500 to 3,000 children born in the UK.

What causes malrotation?

The cause of malrotation is not fully understood, only that it occurs when the bowel does not develop normally in an unborn baby. The bowel starts life as a tube-like structure but coils to fit in a small space at the age of 10 weeks. If the bowel is not coiled properly, this is called malrotation. Malrotation is not caused by complications during pregnancy.

How is malrotation diagnosed?

X-rays can diagnose malrotation but additional tests, including barium swallow and an ultrasound scan, may also be carried out. The barium X-ray allows doctors to see detailed images of the barium going through the digestion process and will highlight the affected area of the bowel.

Symptoms of malrotation

In some cases malrotation does not cause symptoms and some people are not diagnosed with the condition, since they never experience any problems and do not realise the bowel is not coiled properly. In some cases adhesions, which are ribbons of tissue, can develop and block off part of the small intestine, known as the duodenum, which can make it difficult to digest food. Vomiting bile is often a symptom of malrotation. Bile is a yellow-green colour liquid, which is made by the liver and stored in the gallbladder

In many cases the symptoms of volvulus, a complication of malrotation, leads to the diagnosis. Symptoms of volvulus include:

• Sudden episodes of crying.
• Pulling the legs to the chest (this helps to ease pain).
• Stomach cramps.
• Not passing faeces.
• Vomiting (caused by the inability to digest food properly).

Complications of malrotation

If malrotation is not treated it can cause a baby to suffer from dehydration, which can be serious and potentially life-threatening. Symptoms of dehydration include:

• Tiredness and a lack of energy.
• Urinating less frequently.
• Sunken fontanel (the soft spot on the skull).

How is malrotation treated?

Malrotation is usually treated by surgery, which is carried out under general anaesthetic. The operation involves straightening out the affected section of the bowel and coiling it back into the correct position. This operation is known as Ladd’s procedure. In many cases the surgeon will also remove the appendix, as it is not essential for good health and is usually positioned on the wrong side in babies with malrotation.

If the bowel is not fully healthy the surgeon will remove parts in which the tissue is dead. If a large amount has to be removed they will need to create a stoma, which is an opening to allow the body to dispose of waste. Like all surgical procedures there is a risk of complications, but surgeons are highly trained and the risk is minute. The benefits of surgery far outweigh the risks and your surgeon will explain how the procedure works and what risks are involved before the operation.

It is important for malrotation and volvulus to be treated as quickly as possible if an individual is experiencing problems caused by adhesions, since parts of the bowel can die if the blood supply is cut off and there is a risk of infection. Dehydration can also become serious very quickly in young children.

After the operation children will be required to stay in hospital until they are feeding well and starting to put weight on. This can take quite a long time but will vary according to the child. During the recovery period the child will be given pain relief and fed intravenously.

What is the outlook for children with malrotation?

The outlook is positive for children who are diagnosed and treated early and the majority of cases are diagnosed within the first 12 months. If a large part of the bowel has been damaged and subsequently removed, this will impact on a child’s daily life and they may need long-term treatment.