Disorders of Sexual Development and Cloacal Malformations

Renal and urinary issues

  • Children with DSD require continued multidisciplinary follow-up that includes urologists and endocrinologists.
  • Cloacal anomalies are rare and occur over a broad spectrum, with a reported incidence of 1 in 50,000 live births.
  • Vaginoplasty is necessary to achieve future sexual function.
  • More than half of patients who undergo vaginoplasty as part of their primary reconstruction still require a second vaginal reconstruction later in life.
  • Based on the high incidence of subsequent vaginal stenosis and the need for additional interventions, some paediatric urologists advocate delaying vaginoplasty until patients are old enough to make decisions about their own care.

Reasons for follow-up

Cancer formation has been reported in the remnant mullerian structures, gonads, adrenal glands, and neovagina.

Surveillance should be considered throughout the patient’s lifetime.

Any bloody discharge or postcoital bleeding should prompt further evaluation.

Transitional urology
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