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Persistent cloaco is the most severe type of anorectal malformation encountered in children. Patients with cloacal anomalies have a high incidence of associated anomalies most commonly: urinary tract and spinal. Persistent cloaca remains a difficult reconstructive challenge but it is now possible to anatomically correct the defect with surgery in the majority of patients. This review discusses embryology, prenatal diagnosis, neonatal physical and radiological findings. A summary of early management and investigation is provided and the commonest surgical reconstruction techniques are discussed. The main goals of surgical reconstruction are the achievement of bowel and bladder control for the child and normal sexual function in adult life. Although the majority of cloaca patients can achieve faecal and urinary continence with the surgical reconstructive procedures performed today, many require additional/multiple urological procedures to achieve continence, treat bladder dysfunction and to protect renal function. One half of patients will develop renal failure, so regular and life long surveillance is mandatory. Due to the high number of associated gynaecological problems all patients should be assessed by a paediatric gynaecologist at puberty.

Patients with cloacal malformation have a high incidence of innate bladder dysfunction. However, surgical reconstruction by total urogenital mobilization can cause further deterioration of bladder function, particularly in the group with a long common channel. Urodynamic assessment is necessary to detect bladder dysfunction in these patients.

Cloacal anomalies can be diagnosed prenatally and should be considered in any female fetus presenting with bilateral hydronephrosis, a poorly visualized bladder and a cystic lesion arising from the pelvis. Prenatal diagnosis allows time for parental counselling and planning of the delivery at a centre equipped with neonatal intensive-care and paediatric surgical facilities.

Patients born with persistent cloaca have a high incidence of gynecological problems at the onset of menses and in early adult life. Therefore, it is necessary to reassess these girls at early puberty by ultrasound/magnetic resonance imaging and vaginoscopy. Additional surgery may then be necessary to create a vagina for menstruation and sexual intercourse.

The majority of patients born with a cloacal malformation can achieve social fecal and urinary control, although in 46% additional reconstructive surgery was required. The presence of a good bladder neck, short common channel, a normal sacrum and 2 normal kidneys indicates improved prognosis for urinary continence. Achieving normal voiding continence in a child with a cloacal malformation still represents an enormous surgical challenge.

Renal impairment causes significant morbidity and mortality in patients with a cloacal malformation. These patients need a complete nephrourological assessment in the neonatal period due to the high incidence of urinary tract and sacral anomalies as well as careful post-reconstruction followup.