Management of cloacal exstrophy: Experience from tertiary hospital, Tanzania. Case series.

Abstract

Introduction and importance: Cloacal exstrophy (CE) is defined as a complex anomaly that affects the urogenital and intestinal tracts. It is the most serious form of anomaly that is described within the so-called exstrophy-epispadias complex. These malformations usually present a challenge in the management of particular conditions, as most of these forms require multiple surgeries, resulting in the use of multidisciplinary approaches, including reconstructive urologists, pediatric surgeons, orthopedic surgeons, endocrinologists, pediatricians, psychologists and nutritionists. Additionally, these patients present with ambiguous genitalia, which is another aspect that needs to be taken into consideration during the management of this condition.

Case presentation: The first patient, a baby who was 8 days of life and referred from a peripheral hospital, presented with classic features of cloaca exstrophy. He underwent first-stage cloacal exstrophy repair. The intraoperative findings included a bi-halved bladder and phallus, and the ureters were not appreciated, but there was continuous urine leakage from the bi-halved bladder and no uterus or ovaries. Poorly formed cecum, cecal-cutaneous fistula with an everted part of the terminal ileum protruding outside (mucosa-out), no transverse, no descending colon, collapsing small bowel, left undescended testis in the inguinal region, and right abdominal undescended testis. He first underwent surgery, which involved ileostomy, omphalocele closure and proper bladder exstrophy construction. The second patient, a 6-day-old female, had a similar presentation and physical findings as the first patient did, except that she had elephantoid trunk deformity with a cecal fistula, bifid clitoris, two cervical orifices, and two uteri completely separated with ovaries. Mobilization of the hindgut, closure of the cecal fistula, and proper bladder exstrophy after repair of the posterior wall were performed. The third patient was a 10-day-old female, similar to the second patient, but this patient presented with a left leg deformity with wide diastasis. In this case, the urinary bladder was not bivalved, and the cecal fistula had perforated just below the posterior wall of the urinary bladder. A mild omphalocele, bifid clitoris and vagina, one cervical orifice, and two uteri completely separated, with ovaries observed. Mobilization of the hindgut, closure of the cecal fistula, and proper bladder exstrophy after repair of the posterior wall were performed. The postoperative period was uneventful.

Clinical discussion: Surgical management of cloacal exstrophy is typically undertaken in the newborn period (48-72 h) as a combined effort between pediatric surgery and urology. In the setting of associated spinal dysraphism, neurosurgical consultation and closure should be undertaken as soon as the infant becomes medically stable. Early operation minimizes bacterial colonization of exposed viscera and may decrease the need for pelvic osteotomy. The goals of treatment include securing the abdominal wall and bladder closure, preserving renal function, preventing short bowel syndrome, creating functional and cosmetically acceptable genitalia, and attaining acceptable urinary and fecal continence.

Conclusion: Cloacal exstrophy remains a rare and complex congenital anomaly characterized by an array of anatomical defects affecting multiple organ systems. With respect to the approach of this congenital malformation, it is therefore important that these individuals and their families remain under the care of a multidisciplinary team of providers who can offer medical care, counseling and life-long follow-up.