WOFAPS 2025 8th World Congress of Pediatric Surgery

Introduction: We present a case of a female neonate with prenatal diagnosis of bilateral hydronephrosis and ascites that constituted a diagnostic and therapeutic challenge when an unexpected combination of congenital malformations was detected.

Case presentation: A female neonate with prenatal diagnosis of ascites and bilateral hydronephrosis was referred to us after the clinical examination revealed a rectovestibular fistula and absence of a vaginal orifice. Cystourethrography demonstrated a high urethrovestibular fistula in conjunction with hydrocoplos and high vaginal atresia, while the low-placed rectum was completely separated from the urogenital tract. MRI further revealed the presence of a septate uterus. Preoperative cystoscopy failed to detect any urethral or vesical fistula. The initial operative procedure was posterior sagittal anorectoplasty. Subsequently, via a transabdominal approach the uterus was accessed and incised longitudinally, permitting septal resection and hydrocolpos drainage. Due to hydrocolpos, the large native vagina was mobilized, pulled down and placed posteriorly to the urethra, without need for replacement. The urethrovaginal fistula was excised, leaving the urethra intact. Finally, a transverse colostomy was performed. Postoperatively, hydronephrosis gradually subsided. Three months later, the colostomy was reversed and so far the patient has had an uneventful course.

Conclusion: There is a wide spectrum of cloacal malformations. However, as far as we know this is the first time such a variant is described, posing a challenge for the surgical management. Individualizing the basic principles of cloacal operating treatment into the exact anatomic variant of the patient can yield satisfactory results and an excellent prognosis.