Diagnosis and management of patients with posterior hypospadias

Abstract

Normal male sexual development comprises three sequential steps: the development of genetic, gonadal and somatic sex. The secretion and action of three fetal testicular hormones are necessary: mullerian inhibiting substance, testosterone, 5α-dihydrotestosterone. The latter transforms the urogenital sinus, the urogenital tubercle and genital swellings into the prostate and external genitalia. Based on these endocrinological aspects of somatic differentiation, a classification of male pseudohermaphroditism is established. Exact and immediate diagnosis in patients with ambiguity of external genitalia should be followed by early gender assignment and adequate operative therapy. After deciding to treat a patient in the male direction, preoperative penile stimulation, either by local application or systemic therapy with androgens is of great importance to suppress the risk of complications and unsatisfactory results. Then a one-stage repair or multistage technique can be carried out to correct the penile deformity and construct a neourethra. We prefer a staged approach and report our results and experience in the last three decades. If the patient is raised as a female, genitoplasty and, later on, vaginoplasty is performed, the gonads are removed and replacement hormone therapy is initiated.