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Persistent cloaca and phallic urethra
1Department of Urology, Long Island Jewish Medical Center, New Hyde Park, New York.
Insights
This study reviewed 54 children with persistent cloaca, finding frequent urinary tract anomalies and rare cases of masculinized genitalia. The posterior sagittal approach proved effective for surgical repair.
Area of Science:
- Pediatric Surgery
- Congenital Malformations
- Urology
Background:
- Persistent cloaca is a rare congenital anomaly requiring complex surgical management.
- Understanding associated anomalies is crucial for comprehensive patient care.
- This study focuses on a cohort of 54 children treated over 8 years.
Observation:
- The most frequent associated anomalies involved the urinary tract, including renal agenesis, dysplasia, vesicoureteral reflux, and megaureter.
- Four patients presented with a rare cloacal opening at the tip of a pseudophallus.
- Two patients also had an accessory urethra, indicating significant external genitalia masculinization.
Findings:
- Virilization was limited to the genitalia, with no detected adrenal, metabolic, or chromosomal abnormalities.
- Patients with persistent cloaca exhibit a higher likelihood of complex congenital malformations compared to isolated cloaca.
- The posterior sagittal approach was effectively utilized for the surgical repair of these complex cases.
Implications:
- Highlights the significant association between persistent cloaca and complex urinary tract malformations.
- Emphasizes the need for thorough evaluation of associated anomalies in patients with persistent cloaca.
- Demonstrates the efficacy of the posterior sagittal approach in managing these challenging congenital conditions.
Abstract:
During the last 8 years 54 children with a persistent cloaca have been treated by one of us (A. P.). The most common associated anomalies involved the urinary tract, with renal agenesis, renal dysplasia, vesicoureteral reflux and megaureter encountered most frequently. Four patients demonstrated the rare entity of an opening at the tip of a pseudophallus and 2 also had an accessory urethra in association with the cloacal malformation. These children exhibited some form of masculinization of the external genitalia with the accessory urethra or cloacal channel assuming a phallic position in an enlarged clitoris. No adrenal, metabolic or chromosomal abnormalities were detected and virilization was limited to the genitalia. These cases illustrate the greater predilection of these patients to more complex congenital malformations than patients with an isolated cloaca. The posterior sagittal approach has been used effectively for repair of these congenital malformations.