What is the treatment for EPS?

Many patients will need an operation to free the bowel from the encapsulating membrane. Most hospitals do not have experience in surgically treating EPS and so will refer their patients to one of two nationally designated hospitals who do have surgical experience with this condition. The two centres in the UK are in Cambridge and Manchester.

If a patient is experiencing frequent vomiting, sometimes even after every drink, then they need to come into hospital. By passing a naso-gastric tube (tube through the nose into the stomach) we can keep the stomach empty. This should stop the vomiting and prevent a dangerous complication called aspiration, when stomach contents are inhaled into the lungs during a vomiting episode, resulting in pneumonia.

Because patients with EPS become malnourished and lose a lot of weight, it is important to improve their nutritional status before the operation. As preparation for surgery it is usually necessary to feed patients with a special intravenous feed called parenteral nutrition (PN) for 1 or 2 weeks so that they are as fit as possible. This type of nutrition is necessary because patients cannot eat enough to maintain their weight and take in the necessary calories and nutrients without vomiting. PN is given as an inpatient, either in Cambridge or in a hospital closer to home. Patients need to have a line inserted into one of the large neck veins and may have up to 2 litres of PN infused every 24 hours. They will need daily dialysis to remove the excess fluid. If someone has a functioning kidney transplant they will be able to pass out the extra fluid as urine. 

Possible complications

There are several possible complications resulting from surgery:

Stoma

If the fibrotic tissue is very difficult to remove and the bowel wall is very weak, it is possible that the bowel may tear. If this happens the surgeons will need to make an ileostomy or stoma, where the bowel is brought out on to the surface of the abdomen. A stoma looks like a small spout and is similar in colour and texture to the inside of the mouth. It does not hurt or have any feeling. The bowel contents (faeces) will drain out into a bag which is securely stuck down to the skin around the stoma. It is usually possible to reverse the stoma after a few months so the patient will be able to go to the toilet and pass faeces again as normal. About 1 in 10 patients (10%) will require a stoma.

Infection

Some patients will have infected fluid or tissue in the abdomen before they come into hospital, and some patients may develop infection after the operation. Patients are given antibiotics to help prevent infection, but sometimes the infections are resistant to common antibiotics, especially if the patient has been given a lot of antibiotics in the past. Most of the time infections can be treated with a range of antibiotics.  Sometimes patients get a chest infection or an infection in their blood (septicaemia).

Re-operations

About 1 in 3 patients will have to go back to theatre to have more operations. This may be for bleeding, infection or a dressing change to the abdominal wound.

Benefits of the surgery

If the surgery is successful, patients may well be able to eat normally again and will not need further PN treatment. They will not experience nausea and vomiting, should no longer have pain and their weight should return to normal, although this may take some months. However, even after a successful operation some patients may still experience tummy rumbles or have to modify their diet such as eating smaller meals more frequently. If an ileostomy was formed at the time of the EPS surgery we will arrange another admission for an operation to join the bowel up again. Some patients may also be fit enough to go on to have a kidney transplant.

How long do people stay in hospital?

The recovery time after surgery for EPS is very variable. Having good nutrition by having PN first does help. The average total length of stay in hospital is around 4 - 5 weeks; 1 – 2 weeks before the operation and about 3 weeks after the operation. Some people are discharged after 1 – 2 weeks, but others will have to stay longer, occasionally several weeks.