Rare association of female pseudohermaphroditism, phallic urethra, and posterior cloaca.
We describe a child with the rare clinical entity of female pseudohermaphroditism, accessory phallic urethra, and posterior cloaca who was successfully treated with posterior sagittal anorectovaginourethroplasty. Masculinization was limited to the external genitalia, and no chromosomal, metabolic, or adrenal abnormalities were detected. Associated pathology included bilateral vesicoureteric reflux, a non functioning dysplastic kidney, and bicornuate uterus. The investigation and surgical management of this particularly challenging combination of anomalies is detailed.
Macarthur
Mairi
M
Department of Paediatric Surgery, Royal Aberdeen Children's Hospital, Aberdeen AB25 2ZG, UK.
Mahomed
Anies
A
D000015:Abnormalities, Multiple; D001003:Anal Canal / Q000473:pathology; D002675:Child, Preschool; D002988:Cloaca / Q000002:abnormalities* / Q000601:surgery*; D003251:Constriction, Pathologic; D012734:Disorders of Sex Development / Q000601:surgery*; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007668:Kidney / Q000473:pathology; D014521:Urethra / Q000002:abnormalities* / Q000601:surgery*; D014599:Uterus / Q000002:abnormalities