Variants of the Exstrophy Complex: A Single Institution Experience

Abstract

The initial presentation of exstrophy variants can be confusing, often delaying initial treatment. Superior vesical fistulas permit continence without BNR due to an intact urinary sphincter. Variants such as epispadias with bladder prolapse and duplicate or skin covered exstrophy should be closed at birth with standardized techniques to promote bladder growth for later BNR. These cases are faced with the same long-term problems as the classic presentation. Cloacal variants can present with intact anal innervation, allowing a later Pena procedure.

An unusual form of duplicate bladder exstrophy

“…As reported in other series and as our case demonstrates, the initial presentation of an exstrophy variant can be somewhat confusing [5]. Although these exstrophy variants may be mistaken for classic bladder exstrophy, recognition is important because the treatment and prognosis differ greatly.…”

“…There is no urinary communication to the exstrophied component [4]. Superior vesical fistula, sometimes called superior vesical fissure, resembles a vesicostomy or patent urachus and consists of a small communication between the bladder and minimally exstrophic bladder [1,5]. There is a musculoskeletal defect as well.…”

Congenital pouch colon with duplicate bladder exstrophy

“…Coexistence of congenital pouch colon and duplicate bladder exstrophy has not been reported earlier. Duplicate exstrophy refers to the presence of patch of dry, ectopic vesical mucosa on the skin overlying the bladder [7]. This index case represents anteroposterior duplicate bladder exstrophy.…”

Covered exstrophy with ectopic intestinal tissue and bifid phallus: one-stage repair of a complex abnormality

“…Exstrophy variants have been classified into 4 major subgroups: pseudoexstrophy, superior vesical fistula, vesico-intestinal fissure, and duplicate exstrophy. Covered exstrophy is considered as the fifth variant in this spectrum [5] and resembles the classic exstrophy with all of the associated defects, but the bladder is closed and covered by a translucent membrane of skin without muscle or a fascial layer [1]. In addition, an isolated ectopic bowel segment is present on the anterior abdominal wall and attached to the bladder tissue, and it is defined as a sequestration [4].…”