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An Operative Technique for the Management of Certain Urogenital Defects of Pseudohermaphroditism
THE JOURI\"AL OF UROLOGY
Vol. 84, N'o, 2, August 1960 Printed 1·n U.S.A.
AN OPERATIVE TECHNIQUE FOR THE MANAGEMENT OF CERTAIN UROGENITAL DEFECTS OF PSEUDOHERYIAPHRODITISM IAN J'vL THOMPSON*
AXD
RALPH STRAFFO~t
From the University of Michigan School of 1\Ieclicine, Department of Urology, _A_nn Arbor, 2\Iich.
Congenital aclrC'nocortical hyperplasia, productive of female pseudohermaphroditism, should be easily recognizC'cl in view of tlw widespread dissemination of the characteristic features of the anomaly. Should any uncertainty exist regarding appropriate sex categorization for a child in the neonatal period, 17-ketosteroid and sex chromatin studies will permit prompt assignment of the proper sex for rearing and the institution of suitable suppressive or replacement therapy for the endocrinologic or electrolyte discrepancies of the adrenocortical pseudohermaphroclitc. The disastrous sociopsychologic consequences of changing the sex for rearing after the first year of life have been amply demonstrated, and the unrecognized adrenocortical female pseudohermaphroditc in later years is and usually should remain a male. Since the external and internal genitalia, except for the hypertrophied phallus, are female, serious and rather extcnsive rehabilitative procedures are necessitated to permit the child to mature in the male role erroneously assigned to him at birth. In June 1959, a 9-year-old child was admitted to the Cniversity Hospital for clarification of a possible error in the original sex for rearing assignment. ThC' patient's general appearance was that of a well-developed 9-year-old boy. Psychologic tcsting indicated that the psychosexual orientation was entirely male. The patient's family had been aware that at birth, there had been doubt as to the true gender of the child, but since a male infant had been more desirable, the birth certificate had been changed from Linda Marie to Linden and a male role decided upon. The presence of perinea! hypospadias and the necessity of sitting to void had been accepted by the child ,Yithout too great psychological hardRead at annual meeting of North Central Section of American Urologicnl Association, Inc., Chicago, October i-10, 1959. * Present address: The Missouri University Medical Center, Columbia, Mo. t Present address: The Cleveland Clinic, Cleveland 6, Ohio.
ship, clue possibly to even greater adjustments mandated by the many discordant marital situations he had encountered at home. A few months prior to rntering the University Hospital, an appendectomy had been performed elsewhere and a uterus and ovaries encountered. At the surgeon's insistence, further investigation was then initiated. The sex chromatin study was positive, indicating generic femaleness, and the 17-ketosteroid excretion 26 mg. in 24 hours, greatly in excess of normal. Further examinations were carried out in reference to urogenital architecture and the dynamics of urine transport. The voiding pattern and urinary control were normal. The urine was sterile. Endoscopic evaluations outlined a normal bladder and female type of urethra. There was a most capacious vagina, obviously a result of distention from the urinary stream. There was an exceptionally well-developed phallus and fused labial folds of such sizP as to be easily mistaken for scrotal compartments. The perinea! opening simulated a typical hypospadiac meatus in that position. The upper urinary tracts were normal, as were electrolyte and blood studies. The general physical examination was not remarkable. The bone age according to Todd's atlas was reported as 14 years. Since the child and his family were so resolutely moti vatecl and oriented toward a continuation of his male role in society, it was deemed appropriate to carry out ablation of the female organs of reproduction, and to embark upon reconstruction of the external genitalia in order that a phallic urethral meatus might be obtained. In view of the capaciousness of the vagina, utilization of a portion of this structure to form the extension of the urethra onto the phallus was envisioned. At operation, the uterus, tubes and ovaries were removed and the dilated vagina freed from the posterior aspect of the bladder and the anterior aspect of the rectum. vVhen the junction of the anterior vaginal wall and the most distal extent of the urethra was reached the vagina was bisected along its lateral bordprs and
406
PSEUDOHER:\BPHRODITIS1VI
FIG. l. Antcrim vaginal \\'all /hp outlined for use as 11rethral extension
F1G. 2. A, labial folds divided. Vaginal flap emerges from perirn~al opening 1n·epa1·n.tm~· to fon11:1Jion of urethrnl tube around indwelling eatheter. Hooded prepnce has been freed from dorsum of ph:.dl11i. B, ne\\' urethral tnbe has been formed and buried under rennited labial folds. Foreskin buUonholed iu manner ol' ~esbit. C, Nesbit <'hordee repair. Preputial skin transposed to ventral surface of phallus. D, completion of chordee repair and approximation of preputial skin to site of termination of vaginal tube fla.p.
the posterior half completely ('Xciscd. The anterior half was then swung on the hinge of its junction with the urethra, ancl brought out through the perinea I hypospadiac: opening (fig. l). Sutures were plac('d in such a fashion as to join the lateral cut margin of the vaginal mucosa to the roof of the introital area to form the beginning of the urethral reconstru('tion. The length of vaginal nurnculature availrthk was m1ffieient to reach
nearly to the frerrnlar :tn•a of the phallus. Th,, labial folds had been di \·id eel in the mirllirn· and the margins of the vaginal tube ,,ere sutured to the subcutaneous tissue: in this area (fig. 2, . I). standard Nesbit type of chordee repair 1rn.-< performed and the foreskin could lw rc·adilv approximated to the vagina.J tube strip on thf' ventral surface. The labial folds \H'l'(' then n· unikd over the tube fornwd from the
408
I. M. THOMPSON AND R. STRAFFON
flap, and the new urethral meatus was joined to the preputial skin which had been brought to the ventral surface by the buttonhole repair of the chordee (fig. 2, B, C, D). A suprapubic tube was employed for temporary urinary diversion. The postoperative course was uneventful and when the suprapubic tube was removed after 16 days, normal voiding ensued. To the present time, the child has had no micturitional difficulty and with suitable antibacterial therapy, the urine has become sterile. An operation to carry the urethra to the tip of the phallus will be undertaken within the coming year.
It is unfortunate that so serious an error in management occurred in this child, forcing the substitution of an extensive, reconstructive, surgical procedure for hormone suppression and clitoridectomy. These unfortunate patients will undoubtedly be encountered less frequently in the future, but should circumstances dictate the need for so profound an alteration in the architecture of the external and internal genitalia, the use of the vagina for reconstruction of the perinea! and penile urethra appears to afford a simple and suitable surgical solution.
Vol. 84, N'o, 2, August 1960 Printed 1·n U.S.A.
AN OPERATIVE TECHNIQUE FOR THE MANAGEMENT OF CERTAIN UROGENITAL DEFECTS OF PSEUDOHERYIAPHRODITISM IAN J'vL THOMPSON*
AXD
RALPH STRAFFO~t
From the University of Michigan School of 1\Ieclicine, Department of Urology, _A_nn Arbor, 2\Iich.
Congenital aclrC'nocortical hyperplasia, productive of female pseudohermaphroditism, should be easily recognizC'cl in view of tlw widespread dissemination of the characteristic features of the anomaly. Should any uncertainty exist regarding appropriate sex categorization for a child in the neonatal period, 17-ketosteroid and sex chromatin studies will permit prompt assignment of the proper sex for rearing and the institution of suitable suppressive or replacement therapy for the endocrinologic or electrolyte discrepancies of the adrenocortical pseudohermaphroclitc. The disastrous sociopsychologic consequences of changing the sex for rearing after the first year of life have been amply demonstrated, and the unrecognized adrenocortical female pseudohermaphroditc in later years is and usually should remain a male. Since the external and internal genitalia, except for the hypertrophied phallus, are female, serious and rather extcnsive rehabilitative procedures are necessitated to permit the child to mature in the male role erroneously assigned to him at birth. In June 1959, a 9-year-old child was admitted to the Cniversity Hospital for clarification of a possible error in the original sex for rearing assignment. ThC' patient's general appearance was that of a well-developed 9-year-old boy. Psychologic tcsting indicated that the psychosexual orientation was entirely male. The patient's family had been aware that at birth, there had been doubt as to the true gender of the child, but since a male infant had been more desirable, the birth certificate had been changed from Linda Marie to Linden and a male role decided upon. The presence of perinea! hypospadias and the necessity of sitting to void had been accepted by the child ,Yithout too great psychological hardRead at annual meeting of North Central Section of American Urologicnl Association, Inc., Chicago, October i-10, 1959. * Present address: The Missouri University Medical Center, Columbia, Mo. t Present address: The Cleveland Clinic, Cleveland 6, Ohio.
ship, clue possibly to even greater adjustments mandated by the many discordant marital situations he had encountered at home. A few months prior to rntering the University Hospital, an appendectomy had been performed elsewhere and a uterus and ovaries encountered. At the surgeon's insistence, further investigation was then initiated. The sex chromatin study was positive, indicating generic femaleness, and the 17-ketosteroid excretion 26 mg. in 24 hours, greatly in excess of normal. Further examinations were carried out in reference to urogenital architecture and the dynamics of urine transport. The voiding pattern and urinary control were normal. The urine was sterile. Endoscopic evaluations outlined a normal bladder and female type of urethra. There was a most capacious vagina, obviously a result of distention from the urinary stream. There was an exceptionally well-developed phallus and fused labial folds of such sizP as to be easily mistaken for scrotal compartments. The perinea! opening simulated a typical hypospadiac meatus in that position. The upper urinary tracts were normal, as were electrolyte and blood studies. The general physical examination was not remarkable. The bone age according to Todd's atlas was reported as 14 years. Since the child and his family were so resolutely moti vatecl and oriented toward a continuation of his male role in society, it was deemed appropriate to carry out ablation of the female organs of reproduction, and to embark upon reconstruction of the external genitalia in order that a phallic urethral meatus might be obtained. In view of the capaciousness of the vagina, utilization of a portion of this structure to form the extension of the urethra onto the phallus was envisioned. At operation, the uterus, tubes and ovaries were removed and the dilated vagina freed from the posterior aspect of the bladder and the anterior aspect of the rectum. vVhen the junction of the anterior vaginal wall and the most distal extent of the urethra was reached the vagina was bisected along its lateral bordprs and
406
PSEUDOHER:\BPHRODITIS1VI
FIG. l. Antcrim vaginal \\'all /hp outlined for use as 11rethral extension
F1G. 2. A, labial folds divided. Vaginal flap emerges from perirn~al opening 1n·epa1·n.tm~· to fon11:1Jion of urethrnl tube around indwelling eatheter. Hooded prepnce has been freed from dorsum of ph:.dl11i. B, ne\\' urethral tnbe has been formed and buried under rennited labial folds. Foreskin buUonholed iu manner ol' ~esbit. C, Nesbit <'hordee repair. Preputial skin transposed to ventral surface of phallus. D, completion of chordee repair and approximation of preputial skin to site of termination of vaginal tube fla.p.
the posterior half completely ('Xciscd. The anterior half was then swung on the hinge of its junction with the urethra, ancl brought out through the perinea I hypospadiac: opening (fig. l). Sutures were plac('d in such a fashion as to join the lateral cut margin of the vaginal mucosa to the roof of the introital area to form the beginning of the urethral reconstru('tion. The length of vaginal nurnculature availrthk was m1ffieient to reach
nearly to the frerrnlar :tn•a of the phallus. Th,, labial folds had been di \·id eel in the mirllirn· and the margins of the vaginal tube ,,ere sutured to the subcutaneous tissue: in this area (fig. 2, . I). standard Nesbit type of chordee repair 1rn.-< performed and the foreskin could lw rc·adilv approximated to the vagina.J tube strip on thf' ventral surface. The labial folds \H'l'(' then n· unikd over the tube fornwd from the
408
I. M. THOMPSON AND R. STRAFFON
flap, and the new urethral meatus was joined to the preputial skin which had been brought to the ventral surface by the buttonhole repair of the chordee (fig. 2, B, C, D). A suprapubic tube was employed for temporary urinary diversion. The postoperative course was uneventful and when the suprapubic tube was removed after 16 days, normal voiding ensued. To the present time, the child has had no micturitional difficulty and with suitable antibacterial therapy, the urine has become sterile. An operation to carry the urethra to the tip of the phallus will be undertaken within the coming year.
It is unfortunate that so serious an error in management occurred in this child, forcing the substitution of an extensive, reconstructive, surgical procedure for hormone suppression and clitoridectomy. These unfortunate patients will undoubtedly be encountered less frequently in the future, but should circumstances dictate the need for so profound an alteration in the architecture of the external and internal genitalia, the use of the vagina for reconstruction of the perinea! and penile urethra appears to afford a simple and suitable surgical solution.