Cloacal exstrophy
Cloacal exstrophy (EC) is a severe birth defect wherein much of the abdominal organs (the bladder and intestines) are exposed. It often causes the splitting of the bladder, genitalia, and the anus. It is sometimes called OEIS complex.
| Cloacal exstrophy | |
|---|---|
| Other names | Omphalocele-cloacal exstrophy-imperforate anus-spinal defect syndrome |
| Specialty | Medical genetics |
| Complications | Limb deformities, open neural tube defects |
| Treatment | Surgical intervention |
Diagnostic tests can include ultrasound, voiding cystourethrogram (VCUG), intravenous pyelogram (IVP), nuclear renogram, computerized axial tomography (CT scan), and magnetic resonance imaging (MRI). Cloacal exstrophy is a rare birth defect, present in 1/200,000 pregnancies and 1/400,000 live births. It is associated with a defect of the ventral body wall and can be caused by inhibited mesodermal migration. The defect can often be comorbid with spinal bifida and kidney abnormalities. Many individuals who are born with XY cloacal exstrophy are assigned female at birth due to the appearance of the external genitalia. Performing genital surgery on intersex infants for cosmetic purposes is considered a human rights violation by the World Health Organization.