Abstract
Cloacal exstrophy (CE) is a rare condition, and the preservation of a short hindgut
is crucial for growth, fluid-electrolyte balance, and bowel management. Herein, we
present the case of an infant with concurrent anomalies and the challenges faced during
the preservation of both hindguts. A preterm male infant, born at 34 weeks weighing
2 kg, was diagnosed with CE. The first stage of CE repair was performed at 3 months
of age, involving the separation and tubularization of the cecal plate and the joining
of the two hemi-bladders. During the procedure, hindgut duplication was noted. Each
hindgut had a short mesentery with far-separated, tiny blind ends (7 and 10 cm in
length), each directed toward opposite sides of the pelvis and measuring 8 Fr in caliber.
Due to the risk of compromising the blood supply during rotation and mobilization
of one hindgut, the decision was made to exteriorize each hindgut as end stomas. Both
stomas began functioning as expected. However, a colo-cutaneous fistula (connecting
the cecum to the midline surgical wound) developed and was managed conservatively
for 8 weeks. During this period, despite the functioning left stoma, the right stoma
was stenosed, and the fistula persisted. The infant underwent a second laparotomy
for fistula repair and reconstruction of both hindguts. This was successfully managed
by creating a single-lumen end stoma, preserving the entire length of the hindguts
with a wider caliber. In conclusion, complex cases of CE are uncommon, and unexpected
operative findings should always be anticipated. Reconstructing both hindguts into
a single lumen during the initial procedure can be challenging but is achievable with
time and careful effort. Preservation of both hindgut ends should be prioritized.
However, long-term outcomes remain uncertain due to the rarity of this presentation.
Keywords
hindgut - intestinal duplication - cloacal exstrophy
