Cloacal Exstrophy: A Case Report and Literature Review

Abstract

Cloacal exstrophy is an extremely rare congenital malformation resulting in an exstrophy of the urinary, intestinal, and genital organs and associated with anomalies of other organ systems. We present a complicated case of cloacal exstrophy and the recent progress in the management of this probably most complicated anomaly in pediatric urology and surgery.

Omphalocele, exstrophy of cloaca, imperforate anus, and spinal defect complex, multiple major reconstructive surgeries needed

“…Some considered that patient survival and optimal management of this anomaly reflect the maturity of pediatric surgery and urology and pediatric health care in general. [ 6 ]…”

“…Over the past 30 years, the focus has shifted from the survival, to improving patient outcomes and ensuring the optimum quality of life. [ 6 ]…”

“…Gender assignment is an important and debatable issue; historically, a genetically male infant with a phallus of inadequate size for reconstruction was often assigned to female gender, performing early orchidectomy with subsequent hormone replacement at puberty. [ 6 ] The studies reported unclear conclusions on the possible psychosocial and behavioral outcomes of gender reassignment of genotypic males being raised as females. [ 7 ] At the same time, advancement in the surgical techniques for phallic reconstruction, many are advocating for assigning gender according to karyotype.…”

Vulvoplasty in New South Wales, 2001–2013: a population‐based record linkage study

“…Pardo et al 2006 (61) Laser 55 3 patients had minimal suture dehiscence that did not require further suture. Munhoz et al 2006 (7) Inferior wedge resection and superior pedicle flap reconstruction 21 5 complications: including distal flap necrosis in 1 patient, small local haematoma in 1 patient, superficial infection in 1 patient and wound dehiscence between flap and the resected area in 2 patients. Rezai et al 2007 (28) De-epithelialised reduction (n=50) Running W-shaped resection (n=50) 100 3 patients suffered post-operative infection resolved with antibiotics.…”

“…Cloacal exstrophy (21) * A severe birth defect wherein much of the abdominal organs (the bladder and intestines) are exposed. It often causes the splitting of both male and female genitalia (specifically, the penis and clitoris respectively), and the anus is occasionally sealed.…”