Sagittal Approach Research Articles

Feasibility of perineal sagittal approaches in patients without anorectal malformations

Perineal sagittal approaches (posterior sagittal anorectoplasty and anterior and posterior sagittal transanorectal approaches) allow complete anatomic exposure of the perineum and lower pelvis. Moreover, they reduce the risk of damaging important structures because the incision is led in the midline. Therefore, many surgeons have used these approaches to treat diseases other than anorectal malformations (ARM), including intestinal dysganglionosis, trauma, pseudohermaphroditism, presacral mass, and rectal duplication. The aim of this study was to describe a small series of patients operated on via these approaches at Gaslini Children's Hospital over a 5-year period. We retrospectively evaluated 10 patients consecutively operated on via a perineal sagittal approach, with or without sphincteric structure involvement, between January 1997 and December 2001. All of these patients were without ARM. Indications included retrorectal abscesses (two), iatrogenic anal canal stenosis (one), postinflammatory anal canal stenosis (one), internal anal sphincter neurogenic achalasia (one), female pseudohermaphroditism (one), benign sacrococcygeal teratomas (two), malignant sacrococcygeal teratoma (one), and perineal rhabdomyosarcoma (one). Protective colostomy was used in four patients. The parameters that we analysed included technical details, possible complications, perineal cosmetic appearance, and outcome. No complications were experienced. The postoperative cosmetic perineal appearance was excellent in all patients, and continence, when assessed, was always considered satisfactory. All tumours underwent complete gross resection. However, one patient with malignant sacrococcygeal teratoma died as a result of the malignant process 2 years after surgery. Although our study was carried out on a small series of patients, it confirmed that perineal sagittal approaches can be used not only for ARM but also for other conditions involving perirectal pouches, presacral space, and urogenital structures, as these approaches are safe and provide excellent cosmetic results as well as satisfactory functional outcome. Although tumours can be treated via these approaches, outcome remains related to the nature and malignancy of the disease itself.

Removal of a deeply impacted mandibular third molar through a sagittal split ramus osteotomy approach

We describe the case of a 48-year-old man who, after a 5-year history of recurrent infection and intermittent trismus associated with a deeply impacted lower right third molar tooth, presented to the accident and emergency department with severely limited mouth opening, extensive facial swelling and pyrexia. The lower right third molar was later removed successfully through a sagittal split ramus osteotomy approach. This case shows that the sagittal split osteotomy continues to have a valuable role in the removal of deeply impacted lower third molars, particularly when they are in close proximity to the inferior alveolar nerve.

THE ANTERIOR SAGITTAL TRANSRECTAL APPROACH FOR TRAUMATIC URETHROVAGINAL FISTULA CLOSURE

The anterior sagittal transrectal approach (ASTRA) de- rived from the posterior sagittal anorectoplasty introduced by deVries and Pena in 1982 and was primarily developed for the treatment of anorectal malformations.1 We report the management of a recurrent traumatic urethrovaginal fistula using this technique. CASE REPORT A 10-year-old girl presented with a recurrent posttrau- matic fistula after a mine explosion in Afghanistan. Follow- ing the injury primary closure was performed twice, initially via a combined abdominovaginal procedure, and a secondary transvaginal closure was performed. Two years after the reconstructions the patient presented with recurrent urinary tract infections in combination with urge incontinence and additional permanent urinary drib- bling. Voiding cystourethrogram showed a recurrent fistula between the proximal urethra and the proximal third of the vagina (fig. 1, A). Additional urodynamic investigation re- vealed a bladder capacity of 110 ml, unstable detrusor activ- ity with intravesical pressure to 80 cm H2O and complete bladder emptying. An unimpaired upper urinary tract was confirmed by ultrasonography and excretory urography. Ure- throcystoscopy demonstrated an intact distal and mid ure- thra, while a wide proximal fistula to the vagina close to the bladder neck was detected. In this portion the vagina ap- peared uninjured and a normal cervix uteri was identified. However, on vaginoscopy no cervix could be found, indicating a separation of the vagina by the previous trauma (fig. 2, A). Based on these endoscopic findings, the ASTRA procedure was selected for fistula closure. Standard mechanical bowel preparation was performed for 2 days preoperatively with senna. With the patient in the prone position a midline incision was made from the anus to the vaginal introitus. The anterior rectal and posterior vag- inal walls were incised in the midline to the level of the peritoneal reflection. Following exposure of the dorsal vagi- nal wall a cicatricial obliteration of the mid vaginal portion with separation of the vagina in 2 parts was observed (fig. 2, B). The fistula was located at the proximal third of the vagina, and, therefore, could not be identified on vagino- scopy. Excision of the fistula and lateral mobilization of the surrounding tissue were performed, and the urethra was longitudinally sutured over a 10Fr catheter with interrupted sutures. A subcutaneous Martius flap was prepared out of the right labium and used as a secondary layer to cover the urethra. The vagina was repaired posterior to the Martius flap. Following the procedure a 10Fr suprapubic tube cystos- tomy was used for 4 weeks. Neither drains nor a protective colostomy was needed. Perioperative antibiotic treatment in- cluded metronidazole and cefuroxime, and was continued for 9 days after surgery. After urinary drainage for 4 weeks voiding cystourethro- gram confirmed complete fistula closure (fig. 1, B). At 3-month followup the girl was continent and voided without residual urine. Persistent urgency is treated with anticholin- ergics. DISCUSSION Urethrovaginal fistula in childhood is a rare condition, and, therefore, an evolving field. Following trauma the fis- tula in girls mainly occurs in the proximal part of the urethra

Anorectal malformations in females—facts for the gynaecologist

Anorectal malformations in females are characterized by an abnormal position of the anal orifice, anterior to the normal site. In such children, there is a close association between the anorectum and the genitourinary systems, with possible gynaecologic and obstetric implications. The malformations include a spectrum of defects, ranging from the least severe, where the anus opens on the perineum, to the most severe—the common cloaca. Here the urinary, genital and alimentary systems open into a common orifice. There is a high incidence of genital and urinary anomalies associated with the cloaca. At times, the vagina may have to be reconstructed. The surgery of anorectal anomalies is usually done through the posterior sagittal approach. This approach allows for good visualization and access to the anorectal and genital region. Frequently, it is possible to observe, but not correct the associated genital anomalies, which are usually asymptomatic in childhood. However, on attaining menarche, such anomalies may be manifest. Also, these anomalies may be of significance at the time of conception and childbirth.

The posterior sagittal trans-sphincteric approach

The posterior sagittal, transphincteric approach to treat different pelvic problems has been known since last century. Although some surgeons have embraced it and have enthusiastically advocated it s use, it has never become an overly popular technique. The purpose of this study is to evaluate the advantages and disadvantages of the approach, both from an historical perspective and from the authors experience. The international literature on the subject was reviewed since 1877 up to the present date. A retrospective evaluation of the authors experience was conducted, and the results reviewed. Specific attention was paid to the final result obtained in the treatment of the original condition, surgical complications and the effect of the surgical approach on bowel and urinary control. The experiential review included 114 cases. They were divided into two groups. A included 85 patients who underwent a posterior sagittal transphincteric approach that included 49 cases of Hirschsprung s disease (primary 21, secondary 28), 15 presacral masses; 10 rectaltumors; 7 acquired recto-genito-urinary fistulae; and 4 cases of idiopathic rectal prolapse. Group B included 29 patients who underwent a posterior sagittal trans-anorectal approach, in which the anterior wall of the rectum and the sphincter was divided as well.. This group included 12 cases of urogenital sinuses; 8 acquired urethral stricture or atresia after trauma; and 9 posterior urethral masses. Post-operative bowel control was normal all cases except in those patients whose basic condition had resulted in fecal incontinence, or who had sustained an irreversible injury prior tothe operation. Urinary control was normal except in cases with pre-operative incontinence. Complications included recurrence of recto-genitourinary fistulae in 3 cases, recto-cutaneous fistula in 3 Hirschsprung s patients and 2 partial wound dehisences. The posterior sagittal trans-sphincteric approach represents a useful technical alternative. It seems to be particularly useful to treat complications after surgery for Hirschsprung s disease, pre-sacral masses, acquired recto-genito-urinary fistula and idiopathic rectal prolapse. The transanorectal approach provides excellent exposure to the posterior urethra and vagina. Boweland urinary control are not compromised.

Endorectal pull-through with posterior sagittal approach to the repair of postoperative rectourethral and rectovaginal fistula

Background/purpose: Rectourethral (RUF) or rectovaginal fistula (RVF) is a troublesome complication after anorectal surgery because of dense adhesions around the fistula. The authors applied a new technique for the redo surgery. Methods: Case 1 is Hirschsprung’s disease in a 1-year-old boy who underwent modified Duhamel’s procedure and had RUF. Case 2 is rectovestibular fistula in an 11-year-old girl who had anterior sagittal anorectoplasty complicated by RVF. Case 3 is multiple urogenital anomalies including rectovesical fistula in a 4-year-old boy in whom transvesical repair was unsuccessful. The colon was mobilized as far as possible at laparotomy. The rectum was opened via a posterior sagittal approach leaving 1 cm of the anal canal. Extended endorectal mucosectomy was performed to the dentate line, and the fistula was closed from inside of the rectum. The remaining mucosal cuff was everted out of the anus and the intact colon was pulled through the rectum and anastomosed to the cuff extraanally. Results: The postoperative contrast enema showed no recurrent fistula, and defecation was not impaired. Conclusions: Endorectal pull-through of the intact colon can spare troublesome mobilization of the fistula and can prevent the recurrence of fistula. Rectal incision via a posterior sagittal approach provides a direct view of the fistula.

Successful resection of prostatic rhabdomyosarcoma by the posterior sagittal approach combined with preoperative chemotherapy

The authors used a posterior sagittal approach to successfully remove a huge prostatic rhabdomyosarcoma from a 3-year-old boy after he had received intensive chemotherapy. This procedure enables safe removal of the prostatic rhabdomyosarcoma under direct vision and with minimal damage to other pelvic organs.

New developments in anal surgery: Congenital ano-rectal malformations

Congenital ano-rectal malformations present with a wide spectrum of severity. Some patients have relatively benign malformations that require a straightforward surgical repair, and the patient has an excellent prognosis in terms of bowel control. Other patients may have far more substantial defects. The surgical repair for these types of malformations is more complex, and the prognosis for urinary and bowel control is variable, primarily depending on the severity of the defect. Through the examination of a large series of patients, we have come to understand far more about the condition known as imperforate anus (IA) than has been understood in the past. Because of the excellent exposure that is achieved with the posterior sagittal approach, a better understanding of the actual anatomy that exists in these patients has been realized. This has led to a new classification system, based on the actual anatomy, with technical and prognostic ramifications. It has become clear that the pressure augmented distal colostogram is the most important test for planning the definitive repair in those patients that cannot undergo primary repair in the newborn period. A push toward newborn primary repair has been seen, and some authors have reported this approach in patients with more complex malformations. The posterior sagittal approach in male patients has been refined, and the vast majority of these patients can now be repaired without a laparotomy. In female patients, those with a vestibular fistula can be repaired in the newborn period without a colostomy, although the repair is technically demanding. By far the most significant technical advance is the development of the maneuver known as total urogenital mobilization. This maneuver, used during the repair of cloacas, has significant decreased the total operative time and postoperative complication rate associated with this operation. Finally, the use of minimally invasive techniques has been applied to these conditions. © 2003 Elsevier Inc. All rights reserved.

Anal canal duplication in infants

Background/Purpose: Anal canal duplication (ACD) is the most distal and the least frequent digestive duplication. A review of the English-language literature found 15 cases reported in the pediatric age group. Methods: A retrospective chart review was performed for our experience from 1999 to 2001 with 6 patients who presented with a midline postanal opening. Results: All of 6 patients were girls and included one set of twins. Patients were from 3 to 9 months of age at the time of diagnosis (mean, 4.5 months). The anal canal duplication was delineated clearly by contrast study of the tract. All were tubular structures, 10 to 12 mm in length. None of the patients had any other associated anomalies. Five of 6 patients underwent operation between the ages of 3 and 8 months (mean, 5.4 months). Excision of the ACD was accomplished through the posterior sagittal approach. The orifice of the ACD, measuring 1 to 2 mm in diameter located just behind anus, directed to the lumen of the anal canal by keeping in the midline and ended blindly 5 mm above the dentate line without luminal communication. Histology findings showed a squamous epithelium with smooth muscle bundles in 2 cases and pseudostratified columnar epithelium with focally squamous epithelial lining and adjacent smooth muscle bundles in 3 cases. The postoperative courses were uneventful with satisfactory anal function. One patient has not yet undergone operation and has been well on outpatient follow-up. Conclusions: ACD is a congenital developmental lesion located in the midline posterior to the anus presenting as a tubular structure without communication with the anal canal, usually discovered in early infancy, and characteristically predominant in girls. The authors recommend that all ACDs, regardness of size and length, should be removed surgically to restore the normal anatomy and to avoid delayed presentation of infection such as perianal abscess or fistula formation. J Pediatr Surg 38:758-762. © 2003 Elsevier Inc. All rights reserved.

The posterior sagittal trans-sphincteric and trans-rectal approaches.

The posterior sagittal, transphincteric approach to treat different pelvic problems has been known since the last century. Although some surgeons have embraced it and have enthusiastically advocated it's use, it has never become an overly popular technique. The purpose of this study is to evaluate the advantages and disadvantages of the approach, both from a historical perspective and from the authors' experience. The international literature on the subject was reviewed since 1877 up to the present date. A retrospective evaluation of the authors' experience was conducted. Specific attention was paid to the final result obtained in the treatment of the original condition, surgical complications and the effect of the surgical approach. on bowel and urinary control. The review included 114 patients divided into two groups. Group A included 85 patients who underwent a posterior sagittal trans-sphincteric approach. Group B included 29 patients who underwent a posterior sagittal transanorectal approach, in which the anterior wall of the rectum and the sphincter were divided. Postoperative bowel control was normal in all patients except in those whose basic condition had resulted in fecal incontinence, or who had sustained an irreversible injury prior to the operation. Urinary control was normal except in cases with pre-operative incontinence. Complications included recurrence of rectogenito-urinary fistulae in 3 cases, rectocutaneous fistula in 3 patients with Hirschsprung's disease, and 2 partial wound dehisences. The posterior sagittal trans-sphincteric approach represents a useful technical alternative. It seems to be particularly useful to treat complications after surgery for Hirschsprung's disease, presacral masses, acquired rectogenito-urinary fistulae and idiopathic rectal prolapse. The transanorectal approach provides excellent exposure to the posterior urethra and vagina. Bowel and urinary control are not compromised.

‘High’ anorectal malformation in boys: Need for clarity of definition and management

Posterior sagittal anorectoplasty is purported to be the approach of choice for the treatment of both intermediate and high variety of anorectal malformations. The authors describe their devastating experiences with 2 cases of high variety of anorectal malformations in which defects were repaired thorough the posterior sagittal approach. In the first case, because of the high level of the rectal pouch, complete urethrovesical disconnection was done along with inadvertent mobilization and pull-through of urinary bladder. The patient subsequently required major reconstructive procedures. In another case, the bladder was partially mobilized before the mistake was realized; the high-lying rectum was later identified, and a pull-through was performed without any ill consequences. The authors infer that only the patients with rectal pouches ending caudal to the third sacral vertebra should have their definitive surgery performed through posterior sagittal approach. They suggest that PS3 line should be the watershed in management of anorectal malformation in boys. J Pediatr Surg 37:1637-1639. Copyright 2002, Elsevier Science (USA). All rights reserved.

Combined abdominal and posterior sagittal approach for redo pull-through operation in Hirschsprung's disease

Background/Purpose: In Hirschsprung's disease (HD) redo pull-through (PT) is indicated for anastomotic complications and retained aganglionosis after a previous operation. Duhamel or Swenson method is used commonly for redo operations. The pelvic dissection may be difficult, especially in Swenson's type of operation, because of fibrosis resulting from previous surgery or its complications. To overcome this, the authors used a combined abdominal and posterior sagittal approach to perform redo pull-through of Swenson's type in 4 children. Methods: Four boys (2.5 to 12 years) underwent redo pull-through for failed endorectal pull through (n = 2), persistent symptoms after 2 myectomies (n = 1) and late anastomotic disruption after Swenson's PT (n = 1). Abdominal dissection was done first to mobilize colon and resect aganglionic segment as far as the mid pelvis. The mobilized ganglionic colon was tacked to the pelvic rectal stump, hemostasis checked, and the abdomen closed. The lower pelvic dissection was done through the posterior sagittal route, under direct vision. The remainder of diseased rectum was excised, and the pull-through colon was retrieved and anastomosed to the anal stump. No covering colostomy was done. Results: A rectocutaneous fistula developed in one patient, which healed spontaneously. All patients had increased stool frequency in the early postoperative period but improved with time. All patients have attained normal voluntary bowel actions, but one child has infrequent minor soiling. There was no anastomotic narrowing in any case. Conclusions: Posterior sagittal approach is a useful alternative in difficult redo pull-through surgery. It offers excellent exposure, precise dissection, and direct anastomosis. There are minimal chances of complications, and continence is retained. J Pediatr Surg 37:1156-1159. Copyright 2002, Elsevier Science (USA). All rights reserved.

The posterior sagittal transrectal approach (York-Mason) for repair of recto-urinary fistulas secondary to lower urinary tract surgery: our experience

The posterior sagittal transrectal approach (York-Mason) for repair of recto-urinary fistulas secondary to lower urinary tract surgery: our experience

Pena Operation as a Redo Procedure for Anorectal Malformation

【The aim of this study was to evaluate the posterior sagittal anorectoplasty (PSARP) as a re-do operation in patients who failed initial repair of anorectal malformation. Nine patients (4 boys and 5 girls) who had previous failed surgery for anorectal malformation underwent secondary operations through posterior sagittal approach. The main reasons of surgery were constipation (n=3) and persistent anatomical derangement in spite of previous correction surgery (n=6). In addition to constipation, the former group (n=3) had various anatomical defects, and the latter group (n=6), of course, had constipation in some degrees. Patients ranged in age from 2 to 19 years (median 3 years) with only one over the age of 6 years. The primary procedures included PSARP (n=8) and anoplasty (n=1). The rectum was mobilized from surrounding structures through posterior sagittal approach and anatomical defects were corrected. The rectum underwent reconstruction, which involved relocation of the rectum and anus within the limits of the intact muscle complex. Patients underwent follow-up for periods ranging from 6 to 77 months (mean 37 months) after surgery. Anatomical corrections of all the defects were successfully fulfilled in 9 patients. All the patients were satisfied with the functional results after redo-PSARP compared with the preoperative defecatory function. This study suggests that (1) some of the patients with troublesome constipation may have anatomical defects, prominent or hidden, (2) surgeons should suspect the possibility of anatomical defect as the cause of incontinence and (3) preoperative thorough investigation to reveal the anatomical defects should be included in estimating patients with severe incontinence after previous surgery and planning the correction for failed previous surgery as well.】

Dorsal free graft urethroplasty for urethral stricture by ventral sagittal urethrotomy approach

Objectives. To explore the feasibility of applying a dorsal free graft to treat urethral stricture by the ventral sagittal urethrotomy approach without mobilizing the urethra. Methods. Twelve patients with long or multiple strictures of the anterior urethra were treated by a dorsal free full-thickness preputial or buccal mucosa graft. The urethra was not separated from the corporal bodies and was opened in the midline over the stricture. The floor of the urethra was incised, and an elliptical raw area was created over the tunica on which a free full-thickness graft of preputial or buccal mucosa was secured. The urethra was retubularized in one stage. Results. After a follow-up of 8 to 40 months, one recurrence developed and required dilation. Conclusions. The ventral sagittal urethrotomy approach for dorsal free graft urethroplasty is not only feasible and successful, but is easy to perform.

Long-Term Follow-Up Results of the Treatment of High and Intermediate Anorectal Malformations Using a Modified Technique of Posterior Sagittal Anorectoplasty

The aim of this study was to evaluate the long-term follow-up result of a modified technique in the operation for high and intermediate anorectal malformations. The operation was performed by a posterior sagittal approach with three modifications: The external sphincter complex was not opened on the posterior side, the rectal pouch was not tapered, the dissection was performed outside the rectal pouch. From January 1984 to December 1992, 70 cases were operated on including 55 boys and 15 girls. There was one postoperative death during the first 48 hours due to malignant hyperthermia and one late death due to an unrelated disease. A follow-up result was obtained in 48 of 68 survivors (71 %) with follow-up ranging from 13 months to 10 years 11 months (mean: 72 months). According to the modified Wingspread criteria, 2 patients (4 %) had very good anorectal function, 26 children (54 %) had good anorectal function, 18 children (38 %) had fair anorectal function, and only two children (4 %) had poor anorectal function. All children had urinary continence and all boys had a normal erection. The long-term follow-up result was satisfactory. Two important factors affecting the anorectal function were the type of malformations and the thickness of the external sphincter complex.

Surgical treatment of the Müllerian duct remnants

Background: Persistent Müllerian duct tissue in male individuals may result in an enlarged prostatic utricle (utricular cysts and utricle) or a Müllerian duct cysts, either distinctively or synonymously. In intersex patients Müllerian duct remnants (MDR) are an usual occurrence. Surgical excision is the definitive treatment of symptomatic remnants, as well as during the reconstruction of intersexual genitalia. Many approaches have been described. The authors review their experience in intersex patients. Methods: From 1986 to 1999, the authors treated 111 patients with intersex disorders. The records of 47 patients raised as boys with MDR were reviewed. Based on the symptoms and the size of the remnants, in 32 patients the structures were removed. In 13 patients extirpation was done by perineal approach, in 9 by transperitoneal approach, and in the remaining 9 patients the combined abdominal and perineal approach were undertaken. In one patient the large prostatic utricle was extirpated by a posterior sagittal pararectal approach. Perineal approach was mainly used in younger asymptomatic children, with the prostatic utricle disclosed incidentally during genitography because of intersex disorders. Operation was performed only in cases in which the prostatic utricle was observed by cystoscopy or identified by Fogarty balloon catheter introduction into the prostatic utricle. In older patients these structures were discovered frequently after failed urethroplasty, or after symptoms of urinary infection, urinary retention, or epididymitis. We elected to use the transperitoneal approach based on the extension of these structures into the pelvis. The average age of patients at the time of surgery was 8.6 years, with a range of 1 to 30 years. Results: There were no rectal or bladder injuries during surgery. An older patient had temporary impotence after abdomino-perineal extirpation. The lack of ejaculation, seen in 5 patients, was related to frequent intra-utricular termination of the vas deferens. Posterior sagittal pararectal approach certainly enabled complete exposure and exact visualization of all structures, with considerably decreased bleeding. If gonadal biopsy or gonadectomy were necessary, the transperitoneal approach could not be avoided. Conclusions: Surgical treatment of MDR in intersex patients varies according to the size of the utricle, and a double approach is often necessary. A high degree of success may be achieved with minimal morbidity. J Pediatr Surg 36:870-876. Copyright © 2001 by W.B. Saunders Company.

Lipoblastoma

Background/Purpose: Lipoblastoma is a rare benign mesenchymal tumor of embryonal fat that occurs almost exclusively in infants and children. This study was done to determine the clinical and pathologic characteristics of lipoblastoma. Methods: Nine cases of pathologically proven lipoblastoma from 1979 to 1997 were reviewed. There were 6 boys and 3 girls ranging in age from 3 months to 29 months. Results: A soft tissue mass was the chief complaint in 7 patients, abdominal distension in 1 patient with a retroperitoneal mass, and defecation difficulty in 1 patient with a perirectal mass. In 4 patients, tumors occurred on the back. Other tumor location includes the neck, scrotum, retroperitoneum, perirectal area, and buttock in 1 patient each. Lesions measured 2.3 to 19.5 cm. Complete excision was done in 8 patients. One perirectal tumor was removed by both the posterior sagittal approach and the intraabdominal approach but incompletely resected. Two tumors located on the back recurred with intraspinal extension 12 months and 18 months after resection. Second resection and second resection with laminectomy were done. Leg pain and urinary incontinence developed in 1 patient but improved on conservative treatment. Conclusions: Lipoblastoma is a benign neoplasm but can frequently recur (25%) in spite of complete excision. Lipoblastomas occurring on the back had a high recurrence rate (50%) and associated with intraspinal extension. J Pediatr Surg 36:905-907. Copyright © 2001 by W.B. Saunders Company.

Urological management of cloacal anomalies.

A cloacal anomaly results from incomplete urorectal division and is frequently associated with genitourinary abnormalities. Experience of the urological reconstruction of this entity is reported. Nine girls with cloacal malformation have been treated at the Osaka Medical Center in the past 9 years. Seven patients were detected by prenatal ultrasonography as having: megacystis (two patients); hydronephrosis (two patients); or hydrometrocolpos (three patients). Two patients underwent prenatal shunt placement between the enlarged bladder and the amniotic space. For neonatal urinary diversion, four patients received cystostomy. Six of seven patients with associated hydrocolpos required intermittent catheterization to decompress an enlarged vagina. Vesicoureteral reflux was detected in 12 ureters of seven patients. Antireflux surgery was indicated in four patients before definitive repair. Definitive reconstruction was performed on eight patients. The posterior sagittal approach was used in all patients. Vaginal reconstruction was done utilizing a perineal skin flap (one patient), a tubularized vaginal flap (three patients), distal rectum (three patients) and total urogenital mobilization (one patient). Postoperatively, urethrovaginal fistula was created in one patient and complete occlusion was seen in one patient. The anatomical variety of this entity determines the management options from in utero. It involves not only the creation of three perineal orifices, but also a continent, catheterizable urethra under the stabilization of renal function. Accomplishment of the definitive repair requires the combined expertise of experienced pediatric surgeons and pediatric urologists.

SURGICAL TREATMENT OF CONGENITAL ADRENAL HYPERPLASIA

Unraveling of the genetics of CAH offers the possibility of earlier detection and prenatal treatment or, alternatively, blastocyst embryo selection and eventually in utero gene therapy. Endocrine, surgical, and anesthesia management after birth have improved, leading to a better outcome for these patients. In the authors' experience, early one-stage reconstructive surgery, although demanding, allows one to use all available tissue. Once mastered, the repair is actually technically easier than vaginal pull-through surgery in the adolescent. Patients go through childhood with a body image that is more concordant with normal. Neither the child nor the parents must suffer the anticipation of a major operative intervention at puberty that can cause great emotional stress and that may be more difficult. The authors have encountered situations in late adolescence in which it has been impossible to separate the urogenital sinus from below. Under these circumstances, one can consider a posterior sagittal approach in which the rectum is bivalved to allow one to approach the vagina from below in an attempt to separate it safely from the urethra and to mobilize it to the perineum. It is also feasible to consider fashioning a segment of sigmoid colon as a neovagina, realizing that mucosal drainage needs to be managed daily. The authors have also encountered the rare 46,XX patient raised as a male and committed to the male role. In these cases, the patient can be offered gonadectomy, followed by staged complex hypospadias repair, and surgery to remove Müllerian structures and, if possible, to preserve the vas, followed by prepenile scrotal repair and insertion of testicular prostheses. Children with CAH require a lifetime of care with surgical approaches that are age appropriate. These patients can lead a full and productive life. It is the physician's responsibility to make certain that these children reach their full potential with the least number of interventions, which should be designed and optimized to produce the best possible outcome.