The articulations of Adam's rib with the lower urinary tract

Abstract

Patient 1. A 23-year-old, mentally retarded woman was admitted to the hospital for evaluation of a pelvic mass. Noted to have ambiguous genitalia at birth, the patient was raised as a boy until age 4. At that time, she was hospitalized because of difficulty in urinating. The phallus was intermediate in size; also present were a common urethral-vaginal opening, nearly fused labioscrotal folds, and an inguinal hernia. Laparotomy disclosed a normal-appearing gonad on the left (thought to be an ovary), no gonad on the right, and no uterus, but a white, string-like, apparently solid structure that connected the broad ligaments and was closely applied to a large, hypotonic bladder. The patient had a clitorectomy and enlargement of the urethral orifice and was thereafter raised as a girl. At age 12, she developed marked hirsutism and acne. At age 20, chronic pyelonephritis was diagnosed, and an intravenous pyelogram showed a double collecting system bilaterally, with left hydronephrosis and hydroureters. At that time a firm, movable, midline pelvic mass was noted during two physical examinations, but not on a third, nor was the mass visible on the IVP. The patient was a short, hirsute woman with low-set ears, a low hairline, a broad chest with no breast development, and marked cubitus valgus. Vital signs were normal. There were a male degree and distribution of body hair, some temporal recession, and some mustache hair. A complete blood count and other routine laboratory evaluations were normal, including a serum creatinine level of 0.8 mg/dl and a creatinine clearance of 130 liter/24 hr. Adrenal steroids were normal at