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Fetal Chronic Idiopathic Pseudo-obstruction

Fetal chronic idiopathic pseudo-obstruction (CIP) is a disorder in which part of the intestines function poorly as if they are obstructed, but there is no mechanical cause for the dysfunction. The muscles in the gastrointestinal tract are not able to move the food content through the body making it difficult for the baby to receive adequate nutrition through conventional means. When tests are performed, no blockage is found. One of the most severe forms of CIP is megacystis microcolon intestinal hypoperistalsis (MMIH), which is characterized by an enlarged bladder, a small colon and a dilated small intestine.

Diagnosis

Fetal CIP can be suggested by ultrasound (sonogram) examination prior to birth. Evaluation of the bowels is part of the routine ultrasound examination done by many obstetricians as part of their routine prenatal care around the 20th week of pregnancy. If the bladder and stomach appear to be enlarged and hydronephrosis is suspected, it may be a sign that MMIH is present. New research shows that an amniocentesis test (in which an obstetrician draws out some of the amniotic fluid from the abdomen) and a cystocentesis (in which urine is taken from the baby's bladder in the womb for analysis) may be able to confirm the diagnosis of MMIH.

Treatment

If CIP is diagnosed before birth, the staff at Lurie Children’s Institute for Fetal Health will counsel the parents and prepare them for what to expect when their baby is born. Plans are made to assure that the baby is delivered in a hospital that has a neonatal intensive care unit (NICU) and a neonatologist present at the delivery. If MMIH or CIP is present, it may be necessary for the baby to receive total parenteral nutrition (TPN) to help meet his/her nutritional needs.

TPN is a special mixture of glucose, protein, fat, vitamins and minerals that are given through an IV into the veins. Many people call it intravenous feedings. TPN provides the nutrients needed when the baby cannot eat or absorb the nutrients from foods. The TPN solution is usually infused continuously over several hours of the day.

There are many variations of this disease, so treatment depends on which part of the intestines are affected and to what degree they are affected. Some children may undergo decompression, which brings a portion of the bowel to the surface, allowing elimination through a tube inserted into the stomach. In some instances, portions of the intestine that are not functioning may be removed in order to utilize the functional portions to the maximum extent. Additionally, if a child is diagnosed with MMIH, they may require urological surgery early in the newborn period for associated urological problems including hydronephrosis or dilatation of the ureters.

Long-term Effects

The long-term outlook for these children depends on the severity of their diagnosis. At one end of the spectrum, some children undergo surgery early on in childhood and go on to lead normal lives without the need for TPN. Some children may be able to eat normally during the day but continue their TPN feedings at night. Because this can be done at home, these children can lead relatively normal lives. If children begin to have complications with TPN, they may need an intestinal transplant. Intestinal transplantation is used only with a limited subset of patients who fail all conservative attempts and are at risk of death from worsening complications of TPN.

Programs

Lurie Children's has one of the first multidisciplinary intestinal failure and transplantation programs dedicated entirely to the care of children. The number of transplants performed and the success rates of these transplants continues to climb. The director of the Intestinal Transplantation and Rehabilitation Program has performed 11 transplants since 2004. Children who undergo transplantation and survive the first six months have a good outlook for long-term survival.