Pediatric Cloacal Exstrophy

Cloacal exstrophy is a congenital condition affecting the lower abdominal wall, bladder, intestines and reproductive organs. It is extremely rare, occurring in approximately 1 in every 200,000 to 400,000 live births. In this complex birth defect, several abdominal and pelvic structures fail to close and develop normally during fetal development.

At birth, infants with cloacal exstrophy typically have:

• The bladder split and exposed on the outside of the abdomen (bladder exstrophy)
• An exposed segment of intestine between the two halves of the bladder (usually the cecum and ileum)
• An omphalocele (a defect at the belly button, where abdominal organs protrude into a membranous sac)
• Separation of the pelvic bones
• Imperforate anus (absence of a normal anal opening)
• Abnormalities of the genitals, uterus and/or vagina

It affects both boys and girls, and it requires staged surgeries and lifelong medical care.

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Infants with cloacal exstrophy present with a visible defect at birth. Symptoms and signs vary but may include:

• Bladder plates visible and open on the abdominal wall
• No visible anal opening
• Genital abnormalities (such as a bifid scrotum in boys or underdeveloped vagina in girls)
• Difficulty passing stool or urine
• Umbilical defect (omphalocele)
• Separation of the pubic bones (diastasis)
• Associated spinal anomalies

Because cloacal exstrophy affects multiple organ systems, it often requires coordination among pediatric surgeons, urologists and neonatologists shortly after birth.

The exact cause of cloacal exstrophy is unknown, but it occurs very early in fetal development when the lower abdominal wall and cloacal membrane fail to form and close properly. This disruption results in the external exposure of abdominal and pelvic organs.

While the condition usually occurs sporadically, meaning it most often appears unexpectedly in a child with no family history of the disorder, it may be associated with genetic or environmental factors in some cases. It is not typically inherited and does not usually recur in families. However, prenatal detection may be possible through detailed ultrasound imaging in the second trimester.

Treatment for cloacal exstrophy is complex and involves staged surgical reconstruction over time. The goal is to reconstruct the bladder, bowel and genital structures while preserving kidney function, promoting continence and optimizing quality of life.

At Children’s Memorial Hermann Hospital, our Comprehensive Congenital Colorectal Program brings together a multidisciplinary team of affiliated pediatric surgeons, urologists, orthopedic surgeons, gynecologists, nephrologists, neurosurgeons and rehabilitation specialists to manage care at each step.

Initial Management (Newborn Period)

• Stabilization – Managing fluid balance, protecting exposed organs and evaluating overall health
• Surgical repair of omphalocele, if present
• Creation of a colostomy or ileostomy – To allow stool to pass
• Bladder and pelvic reconstruction (staged) – Typically performed in several steps during infancy and early childhood

Reconstructive Surgeries

• Bladder closure and pelvic osteotomy – Performed to reconstruct the bladder and bring the pubic bones closer together for stability
• Pull-through surgery – Reconnects the colon to a newly constructed anus
• Genitourinary reconstruction – Tailored to the child’s anatomy and may include creation or revision of a vagina, phalloplasty or clitoroplasty, and urethral reconstruction

Long-term Support

• Bowel management program – To support fecal continence through medications, enemas or other therapies
• Urinary continence support – Some children may require catheterization or surgical interventions
• Hormonal and reproductive counseling – Especially important during puberty
• Physical and occupational therapy – To support development, mobility and independence

Children with cloacal exstrophy often face lifelong challenges, but with specialized care and a dedicated multidisciplinary team, many go on to thrive. Long-term outcomes depend on the severity of the initial defect, the success of reconstructive surgeries and the presence of associated anomalies.

Long-term considerations may include:

• Bladder and bowel incontinence or need for ongoing management
• Kidney function monitoring and protection
• Sexual development and reproductive health concerns
• Emotional and psychological support
• Transition to adult care for urological and gynecologic follow-up

Our affiliated multidisciplinary team supports patients and families through each stage of growth, with a focus on function, independence and overall well-being.

At Children’s Memorial Hermann Hospital, we provide compassionate, personalized care for children with bladder exstrophy and related urologic conditions. Through the Comprehensive Congenital Colorectal Program, families have access to the latest surgical techniques, advanced bowel/bladder management and coordinated, long-term support.

Through our affiliation with UTHealth Houston¹, we bring together a skilled multidisciplinary team with deep experience in treating colorectal conditions. Every child’s treatment plan is personalized to their anatomy, needs and long-term goals from infancy through adolescence and beyond.

If you have any questions, use the online tool below to help us connect with you. To refer a patient or schedule an appointment, please contact our clinic using the information below.

¹ Affiliated physicians evaluate patients at UT Physicians clinic locations and perform all inpatient procedures and treatments at Children’s Memorial Hermann Hospital.