Updates on the Care of Cloacal Exstrophy

Abstract

Cloacal exstrophy is the most severe congenital anomaly of the exstrophy-epispadias complex and is characterized by gastrointestinal, genitourinary, neurospinal, and musculoskeletal malformations. Individualized surgical reconstruction by a multidisciplinary team is required for these complex patients. Not infrequently, patients need staged surgical procedures throughout childhood and adolescence. Following significant improvements in medical care and surgical reconstructive techniques, nearly all patients with cloacal exstrophy now survive, leading to an increased emphasis on quality of life. Increased attention is given to gender identity and the implications of reconstructive decisions. Long-term sequelae of cloacal exstrophy, including functional continence and sexual dysfunction, are recognized, and many patients require ongoing complex care into adulthood.

Keywords: OEIS; bladder; cloacal exstrophy; continence.

Figure 1

Fetal MRI images of a female fetus at 24 weeks gestation with characteristic findings of cloacal exstrophy including large omphalocele (blue arrow), absent urinary bladder, right pelvic kidney with absent left kidney (yellow arrow), left club foot (green arrow), and low spinal cord termination with truncated sacrum and coccyx (red arrow).

Figure 2

Phenotypic characteristics of cloacal exstrophy in a (A) male infant and (B) female infant (Figure 2A adapted from [1]).

Figure 3

Coverage of bladder and cecal plate. (A) Anatomy of cloacal exstrophy; (B) application of transparent adhesive barrier to protect bladder halves and cecal plate and to separate them from the stool emanating from the prolapsed ileum; (C) prolapsed ileum is brought through a hole in the transparent adhesive barrier; (D) application of porous barrier to protect the prolapsed ileum from diaper abrasion.