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Cloacal Exstrophy

Cloacal exstrophy belongs to the category of birth defects known as intestinal defects.

What it is

Cloacal exstrophy is a severe birth defect in which many of the inner abdomen structures are deformed and exposed. Infants who exhibit cloacal exstrophy are born with a portion of their large intestine lying outside of their body. The bladder also lies externally, and is split in half, with each half attached to either side of the large intestine. Male babies with cloacal exstrophy typically exhibit a flat, short penis that is split in half. In females, the clitoris is split in half, and there may be a second vaginal opening.

The deformity affects bowel and urinary control, as well as sexual function. It is a relatively rare birth defect, occurring in one in every 250,000 live births. It can usually be detected using an ultrasound before birth. Cloacal exstrophy often occurs alongside other birth defects such as spina bifida.

Symptoms

  • Abdominal organs, or portions of them, are exposed through an opening in the area of the umbilical cord
  • Bladder is split in half and attached to the large intestine
  • Genitalia are split in half
  • Frequently, intestine is short and anus has not been formed or perforated
  • Often coincides with either major or minor spinal defects

Causes

Clomid: A recent study of major birth defects in the United States demonstrated a link between the use of fertility drug Clomid (clomiphene citrate) and an increased risk of delivering a baby who suffers from cloacal exstrophy. According to this study, pregnant women who took Clomid in the two months prior to conception or the first month of pregnancy had a more than five times increased risk of delivering a baby who suffered from cloacal exstrophy.

Treatment

Treatment requires surgery, and it is done in a serious of stages over a number of years. The number and timing of surgeries are dependent on the child’s individual case. The first stage usually involves inserting the external organs into the abdomen, and attaching the colon to a colostomy pouch so that the child can effectively eliminate stool. When possible, the colon may eventually be attached to the rectum. Later surgeries will focus on reconstructing both the urinary tract and the genitals.

 

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