Alberto Peña

Posterior sagittal anorectoplasty: Important technical considerations and new applications

Posterior sagittal anorectoplasty (PSARP) is a new technique for the repair of high anorectal malformations. It is based upon complete exposure of the anorectal region by means of a median sagittal incision that runs from the sacrum to the anal dimple, cutting through all muscle structures behind the rectum. It was learned through this procedure that the external sphincter is a functionally useful prominent structure. No puborectalis sling, as such, could be identified. It was possible, however, to recognize a muscle continuity from the skin to the sacral insertion of the levator ani. Since it is impossible to pull the generally ectatic rectum through without destroying the muscle structures present, the rectum must be tapered to allow suturing the muscle behind it. In all these anomalies, the rectum and urethra (or vagina) are very closely joined, sharing a common wall, and their separation calls for extensive exposure. A number of technical details clarified in the course of applying the procedure in 54 patients are fully discussed. This approach also proved to be very successful in the management of two patients with rectocloacal fistula, two with rectal atresia and two with stenosis. Colostomy has been closed in 27 patients and fecal continence may be described as excellent, except in those patients with severe sacral anomalies, and unquestionably superior to that obtained by us with other techniques.

Total urogenital mobilization—An easier way to repair cloacas☆

The surgical treatment of persistent cloaca is a serious challenge. The operation is technically difficult and the final results for urinary and fecal function are far from excellent. The repair of a cloaca includes, among other maneuvers, the separation of the vagina from the urinary tract. This step is a serious technical challenge and is very time consuming. Devascularization of these structures is the main source of complications such as urethro-vaginal fistula, vaginal stricture, and acquired vaginal atresia. To avoid these complications and to facilitate the cloacal repair, a new technical variation using total urogenital mobilization was performed in 11 patients. In this procedure, after the rectum is separated from the vagina, both the urethra and the vagina are mobilized together as a single unit. The surgical time spent during the reconstruction was reduced by approximately 70%. All patients recovered well from the operations and have been followed up for 1 to 14 months. The blood supply of the vagina and urethra in all cases remained excellent. No patient developed urethrovaginal fistula, vaginal stricture, or acquired vaginal atresia. The cosmetic appearance in these patients is superior to the one achieved with previous techniques. Although this maneuver may not render better urinary or fecal control, the urethra is more accessible for catheterization. These preliminary results suggest that the total urogenital mobilization maneuver provides a definite technical advance in the repair of cloaca malformations.

Bowel management for fecal incontinence in patients with anorectal malformations

BACKGROUND/PURPOSE Fecal incontinence is common in patients operated on for anorectal malformations. Treatment with enemas, laxatives, and medications are often given by clinicians in an indiscriminate manner and without a demonstrated benefit. A systematic diagnostic approach and bowel management program was developed for patients suffering from fecal incontinence, and a retrospective analysis of the results is presented. METHODS Three hundred forty-eight patients were seen in consultation for fecal incontinence after repair of imperforate anus at other institutions. Clinical and radiological evaluation helped determine different types of patients. Group I consisted of 147 patients who were considered candidates for reoperation and forms the basis of a future report. Group II included 172 patients who had no potential for bowel control and were therefore candidates for bowel management. These patients fell into two categories; group IIA included 44 patients with incontinence and constipation. The bowel management involved the use of daily large enemas only. Group IIB included 128 patients with incontinence and a tendency to diarrhea. Group III consisted of 29 patients who had pseudoincontinence. They had an original defect with good prognosis, good sphincters, good sacrum, and a well-located rectum. They suffered from severe constipation, megasigmoid, chronic fecal impaction, and overflow pseudoincontinence and were treated with laxatives or sigmoid resection. RESULTS Bowel management was successful in 93% of patients in the constipation group (IIA) and 88% in the diarrhea group (IIB). Ninety-seven percent of patients in group III became fecally continent. CONCLUSION Bowel management consisting of enemas, laxatives, and medications is successful when administered in an organized manner. It is vital to determine the type of fecal incontinence from which the patients suffer and to target their treatment accordingly.

Posterior sagittal anorectoplasty: Results in the management of 332 cases of anorectal, malformations

This is a retrospective evaluation of the functional results obtained in 332 patients who underwent posterior sagittal anorectoplasty (PSARP) for the treatment of an anorectal malformation. All patients were operated on by the same person between September 1980 and June 1987. One hundred sixty-one patients were excluded: younger than 3 years, mentally retarded, colostomy still open, deaths, lost to follow-up, and having complex malformations. Patients were grouped according to their potential for continence. Voluntary bowel movements were considered one of the best indicators of fecal continence and were found among patients with a normal sacrum in 77% of those with rectourethral fistula and 30% of those with a vesical fistula. Except in 1 case, all patients with a normal sacrum and low malformation, vestibular fistula, anorectal agenesis without fistula, persistent cloaca, and atresia or stenosis had voluntary bowel movements. Different degress of soiling were present in patients with a normal sacrum including 20% with vestibular fistula, 30% with no fistula, 25% with atresia or stenosis, 61% with rectourethral fistula, 75% with cloacas, 50% with vesical fistulae, and 0% low malformations. Different degrees of constipation were found in patients with a normal sacrum including 50% with low malformations, 70% with vestibular fistulae, 55% with no fistula, 25% with atresia or stenosis, 30% with urethral fistulae, 75% with cloacas, and 50% with vesical fistulae. In contrast, only 20% of patients with more than 3 sacral vertebrae missing achieved voluntary bowel movements; 60% suffered constant soiling, 20% constipation, and 60% urinary incontinence. All evaluations were done without medical management. The average age of patients with voluntary bowel movements was 4.5 years; for patients without voluntary bowel movements it was 4 years. Patients with a normal sacrum and fecal incontinence operated upon elsewhere underwent secondary PSARP, achieving “marked improvement” in 45% of cases, “some improvement” in 37%, and “no improvement” in 18%. In contrast, those with an abnormal sacrum achieved 20%, 30%, and 50% respectively in each of the above-mentioned categories.

Continent appendicostomy in the bowel management of fecally incontinent children

BACKGROUND Fecal incontinence is common in children who have anorectal malformations, Hirschsprungs Disease, and spina bifida and can negatively impact their emotional and social development. Enemas have been used as an artificial way to keep children clean and to improve their quality of life. This method is unpleasant for many children, particularly when they reach adolescence. Malone in 1990 described an alternative method in which the appendix is used as a conduit to administer an antegrade enema. METHODS The authors describe their experience with this new procedure, modified by them, and used in 20 patients. In the original procedure, the base of the appendix is divided, inverted, and reimplanted into the cecum with an antireflux technique. The authors simplify this by plicating the cecum around the appendix to create a one-way valve mechanism but leaving the appendix in its original position. The authors also mobilize the cecum and exteriorize the appendix at the umbilicus to create an inconspicuous stoma. If the native appendix is absent a neoappendix was created from a flap of cecum. RESULTS Nineteen of 20 patients (95%) are now completely clean 24 hours a day. Stricture of the stoma occurred in two patients and required revision. Leakage at the appendicostomy site occurred in three patients, and two required a tighter plication. CONCLUSIONS The technique is used to change the route of enema administration, and is only used in patients for whom bowel management with rectal enemas has been proven successful. The appendix must be preserved whenever possible in patients at risk for fecal incontinence.

The tethered spinal cord in patients with anorectal malformations

The aims of this study were to find the prevalence of tethered cord in patients with anorectal malformations; to determine if the presence of tethered cord relates to the severity of the anorectal defect, and to certain symptoms, signs, radiologic findings, and associated anomalies; and finally to determine whether tethered cord impacted on a patients functional prognosis and whether surgical untethering improved the patient. The authors studied 934 patients with anorectal malformations, 111 of whom had magnetic resonance imaging (MRI) of the spine. We compared patients with and without tethered cord by using parametric and nonparametric statistical tests. Tethered cord occurred in 24% of the patients. The prevalence varied according to the type of anorectal defect from 43% in the complex group to 11% in patients with rectovestibular fistula. Patients with tethered cord had a lateral sacral ratio lower than that of patients without tethered cord (0.410 versus 0.702). Tethered cord was present in 90% of patients with myelodysplasia, 60% of patients with a presacral mass, 57% of patients with sacral hemivertebrae, and 56% of patients with a single kidney. The greater number of associated anomalies a patient had, the greater the risk of having tethered cord (P < .05 for all differences). The authors noted differences between patients with and without tethered cord in the presence of voluntary bowel movements (46% versus 70%), fecal soiling (91% versus 63%), constipation (21% versus 43%), and urinary incontinence (86% versus 42%). The data indicate that patients with tethered cord have a worse functional prognosis than patients without tethered cord. However, the incontinence in our patients was also predictable based on the type of anorectal defect and the character of the sacrum irrespective of the presence of tethered cord. Eighteen patients underwent surgical untethering of the cord, and none had any significant change in bowel or urinary function postoperatively. No patient with tethered cord experienced incontinence that could be attributed to the cord defect alone. This study suggests that tethered cord occurs more frequently in patients with severe anorectal defects, sacral hypodevelopment, myelodysplasia, presacral mass, sacral hemivertebrae, or a single kidney, or in those with an anorectal defect with poor functional prognosis. At present no solid evidence supports the concept that tethered cord by itself affects the functional prognosis of patients with anorectal malformations. Also, there is no good evidence demonstrating that surgical untethering improves the prognosis.

The surgical management of persistent cloaca: results in 54 patients treated with a posterior sagittal approach.

This report describes the authors personal experience in the surgical treatment of 54 patients. The approach is called posterior sagittal ano recto vagino urethroplasty (PSARVUP). Forty patients underwent a primary procedure, and 14 a secondary operation. The anatomic variations found were multiple, integrating a wide spectrum of defects. The posterior sagittal approach proved to be a good initial approach, and permitted complete repair of the defect in 47 patients. Seven patients required a laparotomy in addition. The length of the common channel varied from 0.5 to 7 cm. Common channels longer than 3 cm usually required some technical alternative to replace the vagina. In at least 34 cases, the vagina was reconstructed primarily without any additional technical manoeuvres. Different degrees of vaginal and uterine septation were found in 25 of 50 cases. Hydrocolpos was an associated defect in 14 of 49 patients. Sixty-eight percent of the patients had an important associated urological defect. Twenty-six patients were clinically evaluated without medical management, twenty-one of whom had voluntary bowel movements by the age of 3 years, but most of them had minor episodes of soiling. Nineteen patients had a normal sacrum, and five had urinary incontinence that was successfully managed by intermittent catheterization. Seven patients had a very abnormal sacrum, and five of them had urinary incontinence. Twenty patients underwent a late postoperative vaginoscopy, 14 of whom showed an adequate introitus and vagina, whereas five had different degrees of narrowing of the introitus. Six patients had a urethrovaginal fistula. One ureter was accidentally divided and one vagina had complete ischaemic necrosis.

Transanorectal approach for the treatment of urogenital sinus: Preliminary report

The treatment of the urogenital sinus with normal rectum still represents a challenge. A perineal approach with or without a skin flap seems to be effective for those patients with a low implantation of the vagina. However, in patients with a high vaginal implantation, this treatment frequently fails to provide a good, functional vagina due to a narrow, strictured vaginal opening. Based on previous experience in the treatment of more than 80 patients with a persistent cloaca, a posterior sagittal transanorectal approach with a protective colostomy was performed in three patients with urogenital sinus and normal rectum. The pelvis was approached through a midsagittal posterior incision; the coccyx was split and the entire anorectal sphincteric mechanism was divided in the midline. The rectum was bivalved in the midline including both posterior and anterior rectal walls. This provided excellent exposure to the urogenital sinus. The vagina was then fully separated from the urogenital sinus (as described in cases of persistent cloacas), and then mobilized and sutured to the perineum. The rectum and sphincteric mechanism were meticulously reconstructed. A midline incision assures the preservation of anorectal innervation, and provides excellent exposure to the pelvis. Anal dilatations are not necessary to maintain a patent and supple anorectal opening because the rectum has two suture lines, one in front of the other. After the colostomy was closed, all patients had appropriate bowel control for their age; two of them are fully continent for urine and the third one still has a suprapubic cystostomy tube waiting for a repair of an additional urethral malformation.

Treatment of fecal incontinence with a comprehensive bowel management program

PURPOSE Many articles describe the antegrade continence enemas (ACEs), but few refer to a bowel management program. A successful ACE may not help a patient without such management. Valuable lessons were learned by implementation of bowel management in 495 fecally incontinent patients. METHODS We previously reported 201 patients. Thereafter, another 294 patients participated in our program. On the basis of a contrast enema and symptoms, they were divided as follows: (a) 220 constipated patients and (b) 74 patients with tendency toward diarrhea. Colonic stool was monitored with abdominal radiographs, modifying the management according to the patients response and radiologic findings. For constipated patients, the emphasis was on using large enemas. For patients with tendency toward diarrhea, we used small enemas, a constipating diet, loperamide, and pectin. Diagnoses included anorectal malformation (223), Hirschsprungs (36), spina bifida (12), and miscellaneous (23). RESULTS The management was successful in 279 patients (95%)-higher in constipated patients (98%) and less successful in patients with tendency toward diarrhea (84%). CONCLUSIONS The key to a successful bowel management program rests in tailoring the type of enema, medication, and diet to the specific type of colon. The best way to determine the effect of an enema is with an abdominal film. The ACE procedures should be recommended only after successful bowel management.

Megasigmoid: A source of pseudoincontinence in children with repaired anorectal malformations

Three children with a history of anorectal malformation repairs were referred to the authors for evaluation and management of fecal incontinence. Their ages ranged from 5 to 7 years. On examination, all the children had fecal impaction and localized dilatation of the rectosigmoid colon. Medical treatment was tried but failed to control the symptoms, and the patients frequently had to be hospitalized for disimpaction. To correct this problem, the authors resected the dilated sigmoid colon, anastomosing the nondilated descending colon to the rectal ampulla, which was preserved to serve as a reservoir. Postoperatively, constipation was cured in all patients. In addition the patients became fecally continent postoperatively, which was an unexpected bonus. The authors believe that localized dilatation of the rectosigmoid should always be considered whenever a child is having intractable constipation after repair of an anorectal malformation and that sigmoid resection may be considered as a therapeutic alternative. Segmental dilatation of the sigmoid colon may be a source of fecal pseudoincontinence and, therefore, should be ruled out when the surgeon is evaluating patients with fecal incontinence.