真性半陰陽の1例

Abstract

True hermaphroditism is even rarer, an d the rarest form of all is true lateral form. We have had the good fortune to discover and report a case of true lateral hermaphroditism in detail. The case we are about to report is the fifty-sixth in the literature. It is the seventeenth of the lateral or alternating variety. Patient, aged 15 and reared as a boy, was admitted Dec. 14, 1953, complaining of an undescended right testicle, with malformation of the penis and urethra. We refer to the patient as "he" The body configuration conformed to t h e male type with exception of hair distribution. The external genitalia were unusual in appearance, showing well-developed down-curved phallus and a condition of complate scrotal hypospadia (Fig. 1). In left scrotum normal testicle, epididymis and seminal cord were palpable, although right scrotum was empty. There was no sign of a vaginal opening in perineum, and "he" said "he" had neither ejaculated nor menstruated. The 17-ketosteroid excretion in our case extended from 7.2 to 13.4 mg/24 hrs., it is among the lower values for the normal male in our laboratory. Retrograde pyelogram showed normal renal shadow and no evidence of adrenal tumor. At operation for right undescended testicle, an infantile ovary, small uterus and fallpoian tube with fimbriated end were discovered in groin (Fig. 2, 0, U and T), but no testicle was detected elsewhere. Panhysterectomy and a Denis-Browne type repair for hypospadia was performed successfully. Microscopic examination of sections from the removed ovary demonstrates the presence of numerous primordial follicles and a typical ovarian stroma (Fig. 3). Histological finding of uterus is illustrated in Fig. 4. Subsequently we discovered a vaginal orifice opening into the posterior urethra, and its smear replesented typical vaginal epitheliar pattern (Fig. 5). A left spermatocystogram showed a rudimentary Seminal bladder and simultaneous vaginogram showed bicorna .te vaginal contour (Fig. 7, S and V). A biopsy of the left gonad in the scrotum showed a mature tissue of testicle (Fig. 6). On Feb. 1, 1954, an amputation of vagina was done. Taking the g ross and microscopic features into account, it would seen that there are reasonable grounds for regarding the case as one of true lateral hermaphroditism. (Read at 47th Anual Meeting, Kinki Section of Japan Urological Association, Osaka, Japan, June 6, 1954.)