Cloacal exstrophy is one of the most complex and severe congenital anomalies affecting a child’s urinary tract, intestinal system, and reproductive organs. It presents at birth with exposed bladder halves and bowel loops due to a failure of normal closure of the lower abdominal wall during fetal development.
The condition requires multi-stage surgical management, and only a few experts in the country, including Dr. Sadashiv Bhole, a senior urologist and pediatric urology specialist in Nagpur, are qualified to lead such highly specialized care.
What is Cloacal Exstrophy?
Cloacal exstrophy, part of the exstrophy-epispadias complex, involves multiple systems and is characterized by:
- Open, split bladder (bladder exstrophy)
- Exposed intestine, often with a visible segment between the bladder halves
- Imperforate anus, meaning the child is born without an anal opening
- Genital malformations, including undescended testicles or split phallus
- Spinal defects, such as spina bifida, which may affect lower limb and bladder function
This condition requires immediate neonatal stabilization and carefully timed reconstructive surgeries.
Causes and Risk Factors
Though the exact cause is unknown, cloacal exstrophy is believed to result from:
- Abnormal development during the 4th to 6th week of gestation
- Genetic factors in rare cases
- Disruption in the formation of the lower abdominal structures and cloacal membrane
It is an extremely rare condition, occurring in about 1 in 200,000 to 400,000 live births.
Symptoms and Presentation
- Visible split bladder and exposed intestines at birth
- Absence of the anal opening (imperforate anus)
- Genital abnormalities including malformed or split penis, bifid scrotum, or ambiguous genitalia
- May be associated with omphalocele, a defect in the abdominal wall where organs protrude
- Presence of spinal deformities like spina bifida
Diagnosis is clinical, made immediately after birth based on the anatomical defects.
Prenatal Diagnosis
Cloacal exstrophy may be suspected during routine fetal anomaly scans, especially if:
- The urinary bladder is not visible on repeated ultrasound exams
- There is a large midline abdominal wall defect
- Additional imaging like fetal MRI can help confirm diagnosis and guide delivery planning
Multidisciplinary Treatment Approach
Neonatal Stabilization
- Careful wrapping of exposed bladder and intestines in sterile dressing
- Intravenous fluids and protection against infection
- Temporary colostomy may be needed to divert feces
Surgical Staging
Treatment is done in phases by a multidisciplinary team involving pediatric urologists, surgeons, orthopedists, and nephrologists.
Phase 1: Initial Repair
- Closure of omphalocele and bladder
- Colostomy creation for bowel management
Phase 2: Bladder and Genital Reconstruction
- Performed by a skilled pediatric urology surgeon
- Reconstructing the bladder neck, urethra, and genitalia
- Preserving or creating functional continence mechanisms
Phase 3: Continence and Functional Recovery
- Bladder augmentation, Mitrofanoff creation, or reimplantation procedures
- Bowel management using ACE (Antegrade Continence Enema) procedures
- Lifelong continence management support
Phase 4: Orthopedic and Neurological Interventions
- Correction of pelvic bone anomalies if needed
- Neurosurgical correction for spinal defects and hydrocephalus
- Urodynamic studies to evaluate long-term bladder function
Long-Term Considerations
- Regular monitoring by a pediatric nephrologist and urologist
- Supportive therapies for mobility, continence training, and psychological development
- In some cases, clean intermittent catheterization (CIC) may be required
- Adolescents may require counseling regarding fertility, puberty, and sexual health
Despite the severity, with skilled intervention, many children lead full, independent lives.
Cloacal exstrophy is a serious yet treatable condition that requires careful surgical planning and lifelong follow-up. With experienced guidance from a specialized pediatric urology clinic, children born with this anomaly can enjoy a healthy, functioning life.
To seek expert evaluation or start a comprehensive treatment plan, consult Dr. Sadashiv Bhole, Nagpur’s leading authority in pediatric reconstructive urology.
