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Anterior sagittal transanorectal approach to the urogenital sinus in adrenogenital syndrome: Preliminary report
Anterior
Sagittal Transanorectal Approach to the Urogenital Sinus in Adrenogenital Syndrome: Preliminary Report By Remigio
Dbmini,
Fabio Rossi, Pier Luca Ceccarelli, Bologna, Italy
l In the female adrenogenital syndrome, the treatment of the urogenital sinus with high implanted vagina still presents a surgical challenge. The conventional technique (perineal vaginal pull-through) has been plagued by a high incidence of vaginal stenosis. A posterior sagittal transanorectal approach was proposed as an alternative to obtain an excellent exposure and thus an adequate mobilization of the vagina. But bivalving a normal rectum and anus has the potential for compromising bowel control and represents a bold maneuver, and the rectum and sphincteric mechanism must be meticulously reconstructed. In the original procedure a protective colostomy must be performed before the operation. To reduce these disadvantages, the authors made the following modifications: sagittal incision of only the anterior rectal wall (Anterior Sagittal Transanorectal Approach-ASTRA) and protective colostomy at the same time as the operation. In this way, maintaining the same excellent exposure and reducing the number of operations from three to two, we operated on 10 girls with adrenogenital syndrome: 4 with high, 3 with intermediate urogenital sinus, and 3 who had previously undergone vaginal pull-through but experienced a vaginal retraction with severe stenosis. At the time of surgery four patients were under 1 year (mean, 9.25 months) and six were from 2 to 11 years of age (mean, 6.5 years). After closing the colostomy, all patients were continent in stools and urine, and the vagina looked normal. The authors suggest using this modified approach as an alternative to the conventional operation and for those patients in whom other techniques have failed. Copyright o 1997 by W.B. Saunders Company INDEX WORDS: tal approach.
Urogenital
sinus,
vaginoplasty,
transanorec-
T
HE ADRENOGENITAL SYNDROME in 46,Xx girls results in a variable degree of virilization externally with persistence of the urogenital sinus. The timing and modality of the surgical repair is determined by the point at which the vagina joins the urethra. Historically, in low-ending vagina, feminizing genitoplasty by cut-back or flap vaginoplasty was performed in the first months of life.’ Instead, in high-ending vagina, staged repair was preferred with early genitoplasty, followed by vaginal pull-through at 2 to 4 years of age.2 Recently, however, others have proposed a one-stage early surgical reconstruction.3-5 For this group of patients,
From fhe Division of Pediatric Surgery, Depamnent of Pediatrics, Utziversity of Bologna, Bologna. Italy. Address reprint requests to Remigio Dbmmr, Department of Pediatrics, Diviston of Pedzatric Surgery. Via Massarenti 11, 40138 Bologna, Italy. Copyright 0 1997 by WB. Saunders Company 0022-3468/97/3205-0013$03.00/O 714
and Roberto
De Castro
stenosis of the vaginal orifice can be a late complication. Therefore, referring to Pefia’s procedure,6,7 we modified his original technique, splitting only the anterior rectum to obtain the same excellent exposure, an adequate mobilization of the vagina, and an easier reconstruction of the rectum. Here we report our preliminary experience with 10 girls treated in the last 2 years. MATERIALS
AND METHODS
From March 1993 to January 1995 we performed the ASTRA technique on 10 girls with adrenogenital syndrome. All the patients had an anatomic evaluation of their urogenital sinus based on physical examination, retrograde gemtogram, and cysto-vaginoscopy. Four patients, three of whom were under 1 year and 1 patient who was 5 years old, had a true high vagina that entered in the urethra proximal to the external sphincter; three patients had an intermediate confluence of the vagina with a urogenital sinus longer than 2 to 3 cm, as measured via cystoscopy; and three patients had a vaginal retraction with severe stenosis secondary to a previous flap vaginoplasty. At the time of surgery four patients were under 1 year (mean, 9.25 months) and six patients were from 2 to 11 years of age (mean, 6.5 years). The patient is placed in a knee-chest position and a ribbon gauze impregnated wrth betadine is packed into the rectum to prevent any contamination. A midline sagittal mcrsion IS made from the anterior margin of the anus to the single perineal orifice of the urogenital sinus with a needle tip cautery. The incision is deepened through the perineal body and only the anterior anorectal wall is opened until the urogemtal sinus is exposed (Fig 1A). The incision in the rectum may be extended as high as necessary. The critical part of this operation is the dissection of the antertor vagmal wall from the urethra because they share a common wall (Fig IB). After that, the procedure is the same as for the repair of a cloaca. Once the vagina has been separated from the urethra and bladder neck up to the peritoneal reflection and circumferentially, the distal neourethra is reconstructed. suturing the walls of the previous urogenital sinus in two layers around a Foley catheter. After a sign&ant mobilization of the vagina. the distal narrow fistulous part is resected and the vaginal orifice is then sutured to the perineal skin immediately behind the urethral orifice (Fig 1C). Finally the anterior rectal wall is reconstructed in two layers with the perineal body (Fig 1D). The patient is then turned onto the lithotomy position, and a protective colostomy is performed. The genitoplasty is performed after 1 month. when the colostomy is closed. Clitoroplasty is started with a circumferential mcision around the glans, degloving the clitoris. Subsequently the resection of the corpora is made from the corona to just beyond their bifurcation, preserving a dorsal strip of tumca albuginea, which contams the neurovascular bundles8 When necessary, the glans IS reduced by excising a wedge of ttssue and then suturing it to the corporal stumps. The phallic skm is divided in the midline dorsally and remodeled into labia minora. The redundant labioscrotal tissue is reduced and brought posteriorly to create a normal-appearing Iabra majora.
RESULTS
At the last follow-up in February 1996 the patients ranged in age from 12 months to 34 months (mean, 22.2 JoufnalofPediatric
Surgery,
Vol32,
No 5 (May),
1997: pp 714-716
ADRENOGENITAL
SYNDROME
715
Fig 1. (A) Sagittal view of a urogenital sinus (UGS) with high implanted vagina proximally to the external sphincter (ES). The arrow shows the sagittal incision limited to the anterior rectal wall IASTRA). (B) The UGS is opened on its posterior wall in the midline, exposing both the urethra and vagina. The dissection initially is performed through a common wall (arrow). (C) The vagina is fully mobilized and the distal narrow fistulous part is resected (dotted line). (D) Sagittal view of the complete repair.
months). Eight patients, at present over 3 years of age, were completely continent of stools and urine, and two under 3 years of age showed normal habits for their age. In all of the patients a urethral catheter passed without resistance and during digital examination the anal canal was noted to be supple. Vaginal dilatation was effected without anesthesia, only to confirm the patency. The vaginal orifice was supple and accepted without resistance a Hegar dilator of appropriate size for the age (Hegar no. S-10 in patients under 3 years; Hegar no. 12-16 in patients over 3 years). A long dilation program was never necessary. The cosmetic appearance was considered good in all cases, except for one patient with atrophy of the clitoris, secondary to a previous operation. DISCUSSION
The surgical treatment of the high urogenital sinus in adrenogenital syndrome is still a serious challenge. The goals of this surgery are to join the vagina to the perineum without tension, to avoid injury to the urethra, and preserve the integrity of the external urethral sphincter and an acceptable cosmetic external appearance. Hendren2 in 1969 proposed the staged repair by perineal approach, which is still the most frequently applied,8.9
even though alternative procedures have been proposed, including early one-stage perineal clitorovaginoplasty,3-5 or transvesical,“,12 parasacraL2 and abdominoperineal approaches,i3J4 suggesting that the ideal procedure has not been found. In this surgery the critical point is presented by the exposure of the surgical field for a technically demanding operation. The perineal approach allows limited mobilization of the vagina, and the vaginal pull-through must be completed with the use of skin flaps. After this type of vaginoplasty the main problem is stenosis at the suture lines, which usually results in midvaginal stenosis, but this complication is also often found in low vaginoplasty. l5 As with many other defects in the urogenital sinus there is a spectrum, and some so-called low forms are intermediate for which simple flap vaginoplasty will not be enough. In this way, attempts to repair a urogenital sinus with a high or intermediate vaginal implantation by the perineal approach can result in future vaginal stenosis.rO In these cases even those suture lines that seem fairly external at the end of the operation seem to retract inward postoperatively. It has been stated previously that half of the patients with congenital adrenal hyperplasia needed a secondary vaginoplasty.i3 Recently Bailez et all6 reported
DOMINI
716
that 78.5% of salt-losing patients required two or more procedures to achieve a normal vaginal opening. To prevent this complication it is necessary to dilate routinely the vaginal orifice until the patient becomes sexually active, but this practice is found rather distasteful both for the parents and for the patients. Moreover, repeated mechanical dilations of a strictured anastomosis are often unsuccessful. To prevent this complication the best method is to try to bring the vagina to the perineum with a tension-free anastomosis, if possible, and not the perineum to the vagina. Pefia et aP7 recently proposed a posterior sagittal approach, splitting the posterior and anterior rectal walls with division of the entire muscle mechanism. This provides a wide exposure and exact visualization of all structures, which allows an optimal mobilization of the vagina, making a dilation program unnecessary. But to bivalve the normal anorectum is a bold maneuver that can interfere with bowel control, and a protective temporary colostomy is required before the main repair. To reduce these disadvantages, we made some modifications. We split in the midline only the anterior rectal wall, maintaining the same excellent exposure, saving the levator anus and the posterior part of muscle complex and external sphincter.r7 The protective colostomy is made at the same time as the operation, reducing the number of operations from three to two. With current methods of bowel preparation it is possible to use this approach without a diverting colostomy, but the only sure way to eliminate infection is to perform a
ET AL
protective colostomy. An infection in this area may produce very serious complications such as fistula formation and fecal incontinence. Moreover, this approach allows a complete anatomic reconstruction of the urethra, avoiding female hypospadia or an intravaginal urethral meatus that can occur with perineal repair3.18 Also if the importance of avoiding injury to the external urinary sphincter is stressed,*.13.15according to Pefia’s anatomical view.lg we believe it can be divided in the sagittal midline without interfering with urinary continence. Finally the vaginal orifice, which often results with an inverted perineal flap as an isolated hole in the perineum, is located in a more physiological positionlo.‘* Even though the follow-up is too short to make definitive statements, we believe that this modified and simplified procedure should be considered as a useful alternative to the standard perineal approach and for those patients for whom other techniques have failed. ADDENDUM
Since the manuscript was submitted, we successfully used the anterior sagittal transanorectal approach without a protective colostomy in a patient with mixed gonadal dysgenesis. Our decision not to create a colostomy was based on the assumption that a patient must be subjected to a strict bowel preparation and to postoperative total parenteral nutrition for 10 days. At present, our aim is to perform the ASTRA for the correction of urogenital sinus as a one-stage procedure.
REFERENCES 1. Hendren WH, Donahoe PK: The correction of congenital abnormalities of the vagina and perineum. J Pediatr Surg 15:751-763, 1980 2. He&en WH, Crawford JD: Adrenogenital syndrome: The anatomy of the anomaly and its repair. Some new concepts. .I Pediatr Surg
of vaginal anomalies and their surgical correction. J Pediatr Surg 30:271-276, 1995 11. Passerini GG: Anew l-stage procedure for clitorovaginoplasty in severely masculimzed female pseudohermaphrodites. J Urol 142:565-
4:49-58, 1969 3. Gonzales
568.1989 12. Parrot
R, Fernandes ET: Single-stage feminization genitoplasty. J Urol 143:776-778, 1990 4. Donahoe PK, Gustafson ML: Early one-stage surgical reconstruction of the extremely high vagina in patients with congenital adrenal hyperplasia. J Pediatr Surg 29:352-358, 1994 5. De Jong TPVM. Boemers TML: Neonatal management of female intersex by clitorovaginoplasty. J Urol 154:830-832, 1995 6. Petia A, Filmer B, Bonilla E, et al: Transanorectal approach for the treatment of urogenital sinus: Preliminary report. J Pediatr Surg 27:681-685, 1992 7. Peiia A, Bonilla E, Mendez M, et al: The posterior sagittal approach: Further pediatric applications. Pediatr Surg Int 7:274-278, 1992 8. Kogan SJ, Smey P. Levitt SB: Subtunical total reduction clitoroplasty: A safe modification of existing technique. J Urol 130:776-778. 1983 9. Hendren WH, Atala A: Repair of the high vagina in girls with severely masculinized anatomy from the adrenogenital syndrome. J Pediatr Surg 30:91-94, 1995 10. Powell DM, Newman KD, Randolph J: A proposed classificatron
agement
TS, Woodard JR: Abdominoperitoneal of the high, short vagina in the adrenogenital
146:647-648,1991 13. Hendren
approach to mansyndrome. J Urol
WH. Donahoe PK: The correction of congenital abnormalities of the vagma and perineum. J Pediatr Surg 15:75 l-763, 1980 14. Hecker WC: Operatrve correction of intersexual genitals in chtldren. Prog Pedtatr Surg 17:21-31. 1984 15. Donahoe PK, Hendren WH: Perineal reconstruction in ambiguous genitalia infants ratsed as females. Ann Surg 200:363-372, 1984 16 Bailez MM, Gearhart JP, Mrgeon C, et al: Vaginal reconstruction after initial constructton of the external genitalia in girls with sahwasting adrenal hyperplasia. J Urol 148:680-682, 1992 17 Gecelter L: Transanorectal approach to the postertor urethra and bladder neck. J Urol 109:1011-1016. 1973 18. Dumanian GA, Donahoe PK: Bilateral rotated buttock flaps for vaginal atresia m severely masculinized females with adrenogemtal syndrome. Plast Recontr Surg 90:487-491, 1992 19. Petia A: The surgical management of persistent cloacas. Results in 54 patients treated with the posterior sagittal approach. J Pediatr Surg 24:590-598,
1989
Sagittal Transanorectal Approach to the Urogenital Sinus in Adrenogenital Syndrome: Preliminary Report By Remigio
Dbmini,
Fabio Rossi, Pier Luca Ceccarelli, Bologna, Italy
l In the female adrenogenital syndrome, the treatment of the urogenital sinus with high implanted vagina still presents a surgical challenge. The conventional technique (perineal vaginal pull-through) has been plagued by a high incidence of vaginal stenosis. A posterior sagittal transanorectal approach was proposed as an alternative to obtain an excellent exposure and thus an adequate mobilization of the vagina. But bivalving a normal rectum and anus has the potential for compromising bowel control and represents a bold maneuver, and the rectum and sphincteric mechanism must be meticulously reconstructed. In the original procedure a protective colostomy must be performed before the operation. To reduce these disadvantages, the authors made the following modifications: sagittal incision of only the anterior rectal wall (Anterior Sagittal Transanorectal Approach-ASTRA) and protective colostomy at the same time as the operation. In this way, maintaining the same excellent exposure and reducing the number of operations from three to two, we operated on 10 girls with adrenogenital syndrome: 4 with high, 3 with intermediate urogenital sinus, and 3 who had previously undergone vaginal pull-through but experienced a vaginal retraction with severe stenosis. At the time of surgery four patients were under 1 year (mean, 9.25 months) and six were from 2 to 11 years of age (mean, 6.5 years). After closing the colostomy, all patients were continent in stools and urine, and the vagina looked normal. The authors suggest using this modified approach as an alternative to the conventional operation and for those patients in whom other techniques have failed. Copyright o 1997 by W.B. Saunders Company INDEX WORDS: tal approach.
Urogenital
sinus,
vaginoplasty,
transanorec-
T
HE ADRENOGENITAL SYNDROME in 46,Xx girls results in a variable degree of virilization externally with persistence of the urogenital sinus. The timing and modality of the surgical repair is determined by the point at which the vagina joins the urethra. Historically, in low-ending vagina, feminizing genitoplasty by cut-back or flap vaginoplasty was performed in the first months of life.’ Instead, in high-ending vagina, staged repair was preferred with early genitoplasty, followed by vaginal pull-through at 2 to 4 years of age.2 Recently, however, others have proposed a one-stage early surgical reconstruction.3-5 For this group of patients,
From fhe Division of Pediatric Surgery, Depamnent of Pediatrics, Utziversity of Bologna, Bologna. Italy. Address reprint requests to Remigio Dbmmr, Department of Pediatrics, Diviston of Pedzatric Surgery. Via Massarenti 11, 40138 Bologna, Italy. Copyright 0 1997 by WB. Saunders Company 0022-3468/97/3205-0013$03.00/O 714
and Roberto
De Castro
stenosis of the vaginal orifice can be a late complication. Therefore, referring to Pefia’s procedure,6,7 we modified his original technique, splitting only the anterior rectum to obtain the same excellent exposure, an adequate mobilization of the vagina, and an easier reconstruction of the rectum. Here we report our preliminary experience with 10 girls treated in the last 2 years. MATERIALS
AND METHODS
From March 1993 to January 1995 we performed the ASTRA technique on 10 girls with adrenogenital syndrome. All the patients had an anatomic evaluation of their urogenital sinus based on physical examination, retrograde gemtogram, and cysto-vaginoscopy. Four patients, three of whom were under 1 year and 1 patient who was 5 years old, had a true high vagina that entered in the urethra proximal to the external sphincter; three patients had an intermediate confluence of the vagina with a urogenital sinus longer than 2 to 3 cm, as measured via cystoscopy; and three patients had a vaginal retraction with severe stenosis secondary to a previous flap vaginoplasty. At the time of surgery four patients were under 1 year (mean, 9.25 months) and six patients were from 2 to 11 years of age (mean, 6.5 years). The patient is placed in a knee-chest position and a ribbon gauze impregnated wrth betadine is packed into the rectum to prevent any contamination. A midline sagittal mcrsion IS made from the anterior margin of the anus to the single perineal orifice of the urogenital sinus with a needle tip cautery. The incision is deepened through the perineal body and only the anterior anorectal wall is opened until the urogemtal sinus is exposed (Fig 1A). The incision in the rectum may be extended as high as necessary. The critical part of this operation is the dissection of the antertor vagmal wall from the urethra because they share a common wall (Fig IB). After that, the procedure is the same as for the repair of a cloaca. Once the vagina has been separated from the urethra and bladder neck up to the peritoneal reflection and circumferentially, the distal neourethra is reconstructed. suturing the walls of the previous urogenital sinus in two layers around a Foley catheter. After a sign&ant mobilization of the vagina. the distal narrow fistulous part is resected and the vaginal orifice is then sutured to the perineal skin immediately behind the urethral orifice (Fig 1C). Finally the anterior rectal wall is reconstructed in two layers with the perineal body (Fig 1D). The patient is then turned onto the lithotomy position, and a protective colostomy is performed. The genitoplasty is performed after 1 month. when the colostomy is closed. Clitoroplasty is started with a circumferential mcision around the glans, degloving the clitoris. Subsequently the resection of the corpora is made from the corona to just beyond their bifurcation, preserving a dorsal strip of tumca albuginea, which contams the neurovascular bundles8 When necessary, the glans IS reduced by excising a wedge of ttssue and then suturing it to the corporal stumps. The phallic skm is divided in the midline dorsally and remodeled into labia minora. The redundant labioscrotal tissue is reduced and brought posteriorly to create a normal-appearing Iabra majora.
RESULTS
At the last follow-up in February 1996 the patients ranged in age from 12 months to 34 months (mean, 22.2 JoufnalofPediatric
Surgery,
Vol32,
No 5 (May),
1997: pp 714-716
ADRENOGENITAL
SYNDROME
715
Fig 1. (A) Sagittal view of a urogenital sinus (UGS) with high implanted vagina proximally to the external sphincter (ES). The arrow shows the sagittal incision limited to the anterior rectal wall IASTRA). (B) The UGS is opened on its posterior wall in the midline, exposing both the urethra and vagina. The dissection initially is performed through a common wall (arrow). (C) The vagina is fully mobilized and the distal narrow fistulous part is resected (dotted line). (D) Sagittal view of the complete repair.
months). Eight patients, at present over 3 years of age, were completely continent of stools and urine, and two under 3 years of age showed normal habits for their age. In all of the patients a urethral catheter passed without resistance and during digital examination the anal canal was noted to be supple. Vaginal dilatation was effected without anesthesia, only to confirm the patency. The vaginal orifice was supple and accepted without resistance a Hegar dilator of appropriate size for the age (Hegar no. S-10 in patients under 3 years; Hegar no. 12-16 in patients over 3 years). A long dilation program was never necessary. The cosmetic appearance was considered good in all cases, except for one patient with atrophy of the clitoris, secondary to a previous operation. DISCUSSION
The surgical treatment of the high urogenital sinus in adrenogenital syndrome is still a serious challenge. The goals of this surgery are to join the vagina to the perineum without tension, to avoid injury to the urethra, and preserve the integrity of the external urethral sphincter and an acceptable cosmetic external appearance. Hendren2 in 1969 proposed the staged repair by perineal approach, which is still the most frequently applied,8.9
even though alternative procedures have been proposed, including early one-stage perineal clitorovaginoplasty,3-5 or transvesical,“,12 parasacraL2 and abdominoperineal approaches,i3J4 suggesting that the ideal procedure has not been found. In this surgery the critical point is presented by the exposure of the surgical field for a technically demanding operation. The perineal approach allows limited mobilization of the vagina, and the vaginal pull-through must be completed with the use of skin flaps. After this type of vaginoplasty the main problem is stenosis at the suture lines, which usually results in midvaginal stenosis, but this complication is also often found in low vaginoplasty. l5 As with many other defects in the urogenital sinus there is a spectrum, and some so-called low forms are intermediate for which simple flap vaginoplasty will not be enough. In this way, attempts to repair a urogenital sinus with a high or intermediate vaginal implantation by the perineal approach can result in future vaginal stenosis.rO In these cases even those suture lines that seem fairly external at the end of the operation seem to retract inward postoperatively. It has been stated previously that half of the patients with congenital adrenal hyperplasia needed a secondary vaginoplasty.i3 Recently Bailez et all6 reported
DOMINI
716
that 78.5% of salt-losing patients required two or more procedures to achieve a normal vaginal opening. To prevent this complication it is necessary to dilate routinely the vaginal orifice until the patient becomes sexually active, but this practice is found rather distasteful both for the parents and for the patients. Moreover, repeated mechanical dilations of a strictured anastomosis are often unsuccessful. To prevent this complication the best method is to try to bring the vagina to the perineum with a tension-free anastomosis, if possible, and not the perineum to the vagina. Pefia et aP7 recently proposed a posterior sagittal approach, splitting the posterior and anterior rectal walls with division of the entire muscle mechanism. This provides a wide exposure and exact visualization of all structures, which allows an optimal mobilization of the vagina, making a dilation program unnecessary. But to bivalve the normal anorectum is a bold maneuver that can interfere with bowel control, and a protective temporary colostomy is required before the main repair. To reduce these disadvantages, we made some modifications. We split in the midline only the anterior rectal wall, maintaining the same excellent exposure, saving the levator anus and the posterior part of muscle complex and external sphincter.r7 The protective colostomy is made at the same time as the operation, reducing the number of operations from three to two. With current methods of bowel preparation it is possible to use this approach without a diverting colostomy, but the only sure way to eliminate infection is to perform a
ET AL
protective colostomy. An infection in this area may produce very serious complications such as fistula formation and fecal incontinence. Moreover, this approach allows a complete anatomic reconstruction of the urethra, avoiding female hypospadia or an intravaginal urethral meatus that can occur with perineal repair3.18 Also if the importance of avoiding injury to the external urinary sphincter is stressed,*.13.15according to Pefia’s anatomical view.lg we believe it can be divided in the sagittal midline without interfering with urinary continence. Finally the vaginal orifice, which often results with an inverted perineal flap as an isolated hole in the perineum, is located in a more physiological positionlo.‘* Even though the follow-up is too short to make definitive statements, we believe that this modified and simplified procedure should be considered as a useful alternative to the standard perineal approach and for those patients for whom other techniques have failed. ADDENDUM
Since the manuscript was submitted, we successfully used the anterior sagittal transanorectal approach without a protective colostomy in a patient with mixed gonadal dysgenesis. Our decision not to create a colostomy was based on the assumption that a patient must be subjected to a strict bowel preparation and to postoperative total parenteral nutrition for 10 days. At present, our aim is to perform the ASTRA for the correction of urogenital sinus as a one-stage procedure.
REFERENCES 1. Hendren WH, Donahoe PK: The correction of congenital abnormalities of the vagina and perineum. J Pediatr Surg 15:751-763, 1980 2. He&en WH, Crawford JD: Adrenogenital syndrome: The anatomy of the anomaly and its repair. Some new concepts. .I Pediatr Surg
of vaginal anomalies and their surgical correction. J Pediatr Surg 30:271-276, 1995 11. Passerini GG: Anew l-stage procedure for clitorovaginoplasty in severely masculimzed female pseudohermaphrodites. J Urol 142:565-
4:49-58, 1969 3. Gonzales
568.1989 12. Parrot
R, Fernandes ET: Single-stage feminization genitoplasty. J Urol 143:776-778, 1990 4. Donahoe PK, Gustafson ML: Early one-stage surgical reconstruction of the extremely high vagina in patients with congenital adrenal hyperplasia. J Pediatr Surg 29:352-358, 1994 5. De Jong TPVM. Boemers TML: Neonatal management of female intersex by clitorovaginoplasty. J Urol 154:830-832, 1995 6. Petia A, Filmer B, Bonilla E, et al: Transanorectal approach for the treatment of urogenital sinus: Preliminary report. J Pediatr Surg 27:681-685, 1992 7. Peiia A, Bonilla E, Mendez M, et al: The posterior sagittal approach: Further pediatric applications. Pediatr Surg Int 7:274-278, 1992 8. Kogan SJ, Smey P. Levitt SB: Subtunical total reduction clitoroplasty: A safe modification of existing technique. J Urol 130:776-778. 1983 9. Hendren WH, Atala A: Repair of the high vagina in girls with severely masculinized anatomy from the adrenogenital syndrome. J Pediatr Surg 30:91-94, 1995 10. Powell DM, Newman KD, Randolph J: A proposed classificatron
agement
TS, Woodard JR: Abdominoperitoneal of the high, short vagina in the adrenogenital
146:647-648,1991 13. Hendren
approach to mansyndrome. J Urol
WH. Donahoe PK: The correction of congenital abnormalities of the vagma and perineum. J Pediatr Surg 15:75 l-763, 1980 14. Hecker WC: Operatrve correction of intersexual genitals in chtldren. Prog Pedtatr Surg 17:21-31. 1984 15. Donahoe PK, Hendren WH: Perineal reconstruction in ambiguous genitalia infants ratsed as females. Ann Surg 200:363-372, 1984 16 Bailez MM, Gearhart JP, Mrgeon C, et al: Vaginal reconstruction after initial constructton of the external genitalia in girls with sahwasting adrenal hyperplasia. J Urol 148:680-682, 1992 17 Gecelter L: Transanorectal approach to the postertor urethra and bladder neck. J Urol 109:1011-1016. 1973 18. Dumanian GA, Donahoe PK: Bilateral rotated buttock flaps for vaginal atresia m severely masculinized females with adrenogemtal syndrome. Plast Recontr Surg 90:487-491, 1992 19. Petia A: The surgical management of persistent cloacas. Results in 54 patients treated with the posterior sagittal approach. J Pediatr Surg 24:590-598,
1989