Results After Posterior Sagittai Anorectoplasty: A New Approach to High Imperforate Anus John L. Cahill, MD, Seattle, Washington Dennis L. Christie, MD, Seattle, Washington
T h e infant with anorectal agenesis has been subject to a variety of surgical procedures for reconstruction with equally variable surgical resulLs. When the level of rectal atresia lies above the origin of the levator ani musculature, the pubococcygeal line of Stephens, the defect is classified as a high imperforat~ anus [1]. T h e rectal pouch m a y end blindly or result in a fistula to the rectoprostatic urethra in the male patient. Much less commonly, in the female patient, the fistula terminates in the u p p e r vagina. In the past, exploration for a high rectal pouch through a limited perineal incision has resulted in a poor outcome due to damage to the nerve and muscle structures as well as the genitourinary tract. Stephens' sacroperineal and Kiesewetter's sacroabdominoperineal operation, recognizing the components of the levator ani, the puborectalis, and the pubococcygeus muscle sling added a new dimension to the identification of the structures and more accurate placement of the newly constructed rectum [2]. These operations, however, still carry a high incidence of both childhood incontinence and postoperative prolapse. Recently, de Vries and Fefia [3, 4], based on autopsy sections of fetuses and newborn infants, and studies of serially sectioned h u m a n embryos, have focused attention on the importance of the external sphincter muscle. Utilizing a posterior sagittal approach, the external sphincteric musculature is identified by use of an electrostimulator. T h e operative exposure allows meticulous dissection with preservation of sphincter e]ements and avoidance of injury to the nerves and genital structures. T h e rectourethral fistula m a y be accurately dissected and closed, and the terminal bowel, tapered and reconstructed in its proper relationship to the levator ani and external sphincter muscles. Early reports, after this procedure have been encouraging and have led us to a d o p t this approach for patients with high rectal atresia. From Departments of Surgeryand Pediatrics (Gastro6nterology), The Chll. dren's Orthopedic Pk~plt~l and ~Ir~l Center and ~ Division of Pediatric Gastroenterology, The Mason Clinic, Seattle, Washington. Requests for reprints should be addressed to John L. Cahill, MO, Department of Surgery, Children's Orthopedic Hospftal and Medical Center, 4800 Sand Point Way Northeast, Seattle, Washington 98105. Presented at the 71st Annual Meeting of the North Pacific Surgtca! Assocletton, Seattle, Washington. November 9 and 10, 1984.
Vo~me140,May 1985
Material and Methods Six patients, all male with high imperforate anus, underwent posterior sagittal anorectoplasty after initial colostomy in the period from 1982 to 1984. A rectoprostatic urethral fistula was present in each patient, and all except one patient had rmrmal saerococcygeal development. One patient, with only four sacral segments, has an associated mild unilateral hydronephrosis. All patients were initially evaluated with pelvic ultrasonography and subsequently underwent a divided sigmoid colostomy while newborns. Urologic workup included an intravenous pyelogram and voiding cystourethrogram. In addition, a contrast study of the distal rectum and fistula was carried out. Posterior sagittal anorectoplasty was performed at age 3 months to I year. Operation: A urethral catheter is used in the male patient before operation. The patient is placed in "the prone jackknife position, and transcutaneous electrostimulation with the bipolar muscle stimulator is used to detei"mine the appropriate anal site by observing the nature of the contractions of the sphincteric musculature. A midsagittal incision, utilizing electrostimulation of the external sphincter, is made between the sacrum and the median raphe. The coccyx is sagittally split after dividing the insertion of the deep external sphincter into the lateral halves. The levator muscle is split in the midline, and the incision is carried down through the endopelvic fascia to the longitudinal smooth muscle coat of the terminal bowel. The bowel is then mobilized by sharp dissection from the lateral levator muscle fibers and opened just dorsally to expose the rectourethral fistula. The submucosa is dissected, leaving the muscular coats of the terminal bowel on the prostatic capsule to avoid damage to the nerves and genital structures. The fistula is transsected and closed with absorbable suture. Above the prostatic area, the full thickness of the bowel wall is mobilized to a position just at the level of the pelvic peritoneal reflection. If the terminal bowel is dilated and hypertrophic, the bowel is tapered by wedge resection of a dorsal segment of the wall. The bowel is then reconstructed in two layers with absorbable 5-0 suture and the smooth muscle coat with a 5-0 monofilament nonabsorbable suture. Adequate length is obtained so that the bowel tube can be placed in its proper relationship with the split striated muscle complex at the perineal body. The levator musculature is sutured to the bowel wall laterally and dorsally, and the external sphincter fibers in a circumferential fashion at several levels. The bowel is trimmed so that there is a good skin approximation without redundancy. The anal opening is made to allow the initial passage of a no. 12
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Cahill and Christie
TABLE I
Posterior Saglttai Anorectoplasty: Postoperative Clinical Data
Age at Patient 1 2 3 4 5 6
Operation 1 11 3 4 3 6
yr me me me me me
Age at Follow-Up 3 yr 6 2 yr 8 2 yr I yr 7 1 yr 3 10 me
me one rno me
T A B L E Ii
Posterior S a g l t t a i A n o r e c t o p l a s t y : Manometric Data Mean
Bowel Status Continent (2 yr) Continent (18 me) Continent (2 yr) 1 - 2 stools/d 8 stools/d 4 - 5 stools/d
Patient
Sensation
Canal Pressure (rnm Hg)
1 2 3 4 5
+ -I+ + -
40 30 40 36 16
Anal inhibitory Reflex
Continence
+ + + + +
+ + + - ( 1 yr 7 me) - (1 yr 3 me)
+ = present; - = absent.
Hegar's dilator. If adequate length cannot be attained, the perineal wound is packed loosely and closed. The patient is turned over for an abdominal operation. The rectum and sigmoid colon are mobilized and passed down with the aid of a previously placed catheter into the perineal wound and the abdomen is closed. The patient is turned and the perineal procedure is finished. The Foley catheter is left in place for 1 week postoperatively. The perineal sutures are removed at 2 weeks and gentle anal dilatation is begun. Colostomy ch)sure is performed 6 to 8 weeks after anorectoplasty and gentle rectal dilatations are continued for 3 months. Patients are seen at 3 month intervals thereafter for the first year after closure of a colostomy, and anorectal manometry is performed at I year intervals until continence is present. Manometry is carried out with a pneumohydraulic capillary pump and a constant perfusion catheter with a balloon tip and eight pressure channels. Results
Five patients were recons*~ructed entirely with a perineal approach. One patient required a combined a b d o m i n a l and perineal procedure. All six p a t i e n t s h a d their colostomies closed a n d were followed for 4 m o n t h s to 21/2 years (Table I). T h r e e patients obtained continence between the age of 18 months and 2 years. One patient was nearly continent at 11/2years of age and a n o t h e r ( P a t i e n t 5) was still having eight stools per d a y at age 1 year 3 months. T h e youngest p a t i e n t {Patient 6) was 4 m o n t h s postoperative a t follow-up and had a normal stool p a t t e r n for his age. N o n e of the patients had a p r o b l e m with urinary dysfunction or infection postoperatively and none have manifest constipation or obstructive symptoms. Anorectal m a n o m e t r y was performed on five patients a t least 1 year postoperative (Table II). Four had normal rectal sensation as d e t e r m i n e d by balloon inflation. T h e m e a n anal canal pressure ranged between 30 and 40 m m H g in these patients. One pat i e n t ( P a t i e n t 5) who lacked sensation had a m e a n anal canal pressure of 16 m m Hg. T h e anal inhibitory reflex was found to be present in all five patients who underwent m a n o m e t r y , suggesting t h a t the internal sphincter was present. I t was difficult to assess external sphincter function since electrostimulation was n o t carried out.
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Comments
Surgical t r e a t m e n t of anorectal anomalies has advanced considerably over the past 2 decades after the reclassifications as proposed by S t e p h e n s and S m i t h [5] which divide anorectal anomalies into low translevator defects, in which a local perineal approach m a y be applied, and those at i n t e r m e d i a t e and s u p r a l e v a t o r levels in which the t e r m i n a l bowel ends at a level of the lowest ossification point of the ischiun~'or at the pubococcygeal level. T h e recognition of the important role of the pubal rectalis muscle in continence has i m p r o v e d functional results after a variety of operative procedures. T h e preference of Stephens and Smith for the sacral perineal approach which allows visualization of the pubal rectalis portion of the levator ani musculature ha~s been modified by Kiesewetter [6] to a s a c r o a b d o m i n o p e r i n e a l operation which focuses attention upon the importance of the external sphincters as well as preservation of the puborectalis musculature. Unfortunately, this procedure, as well as the combined pullthrough Rehbein [7] operation, involved a blind pullthrough procedure in the retrourethral area of the external sphincteric m u s c u l a t u r e in the creation of a subcutaneous anal site. Analysis of the long-term results in patients treated by these operations reveals a still unsatisfactory degree of continence a n d has led to a search for other operative n~ethods. de Vries and Pefia have reintroduced the posterior sagittal a p p r o a c h which was originally described a b o u t 150 years ago [8]. T h e i r a n a t o m i c a n d embryologic studies have shown t h a t the external sphincteric m u s c u l a t u r e extending f r o m the perin e u m to the coccyx or s a c r u m is p r e s e n t in all patients, including those whose bowel terminates above the pubococcygeal line. T h e a p p r o a c h allows visualization of the musculature of the external sphincter and its division into a thin superficial s u b c u t a n e o u s sphincter t h a t does not insert on the coccyx and a deep external sphincter t h a t has significant bulk and inserts on the coccyx. I n t e r p o s e d striated muscle fibers between the external sphincter a n d the levator ani t h a t insert on the pubu8 forms a striated muscle
The American Journal of ~ r y
I~ew Approach to High Imperforate Anus
complex that can be reconstructed to the terminal bowel in the creation of an anal canal by first tapering the bowel and suturing this newly constructed anus to the perineum. Their reported combined experience with this procedure numbers over 100 cases, and follow-up results have been encouraging, although many patients have yet to have their colostomies closed. Lateral x-ray contrast studies have been carried out. The radiologic assessment requires cooperation and is not applicable to the very young patient. It does allow for assessment of motility of the distal bowel. Anorectal manometric studies have been useful in comparing normal patients with patients after operative procedures. Manometric criteria for continence have been developed by Scharli [9]. The anorectal canal pressure depends on the actions of the external and internal sphincters as well as the pubal rectalis muscle sling. In the healthy child; this pressure is between 25 and 35 mm Hg. Motor function of the sphincters can be determined by studying the anorectal pressure profile as well as the various reflexes, especially relaxation of the internal sphincter when it is distended with a balloon on the tip of the pressure recording catheter. Scharli has reported that resting canal pressures between 15 and 25 mm Hg are compatible with only partial continence and that under 15 mm Hg, incontinence occurs. Three patients in the present study with continence have resting anal canal pressures of 30 to 40 mm Hg recorded. Five patients have evidence of internal sphincter function as shown by a positive anal inhibitory reflex. Other factors that may affect continence include rectal sensitivity in the wall of the anorectum, stretch receptors in the puborectalis muscles, and perianal skin sensation. Serial postoperative evaluation of patients noting decreased frequency and increased consistency of the stool, the appearance of the sensation of fullness, and the urge to defecate indicate approaching continence. Clinical examination of the rectum estimating reflex activity of the external sphincter, tested by stroking the perianal skin, and digital examination of the anorectum, estimating the tone of the external sphincter and the sensitivity of the muscle, have been
Volume 149, M~y 1985
found to be useful. These factors together with objective manometric data form the basis of evaluation of results.
Summary Six patients, '~ t~ 12 months of age have undergone posterior sagittal anorectoplasty for high imperforate anus. A rectourethral fistula was present in all patients. The operative procedure performed was the method of de Vries and Pefia, utilizing etectrostimulation and identification of the external sphincter muscles. One patient required an abdominal operation as well as the perineal approach. There were no complications. Serial assessment was carried out over a period of 4 months to 2]/uyears. Normal continence was achieved in three patients. Anorectal manometry was performed in five patients. Four ef five patients had normal rectal sensation and normal mean anal canal pressures. These results suggest that this procedure is applicable to the young infant with high imperforate anus, and a satisfactory result can be anticipated. References 1. Stephens FD. Congenital imperforate rectum, recto-urethral and rectovaginal fistulae. Aust NZ J Surg 1953;22:161-72. 2. Ktesewetter WB, Turner CR. Continence after surgery for imperforate anus: a critical analysis and preliminary experience with sacropertneal pullthrough. Ann Surg 1963;158:498511. 3. de Vries PA, Per~a A. Posterior sagittal anorectoplasty. J Pedtatr Suvg 1982;17:638-43. 4. Pef',a A, de Vries PA. Posterior sagittal anorectoplasty: Impocl~lt technical considerations and new applications. J Pediatr Surg 1982;17:791-811. 5. Stephens FD, Smith ED. Anorectal malformations in children. Chicago: Yearbook Medical, 1971:139. 6. Ktesewetter WB. Imperforate anus: I1 The rationale and technic of the sacroabdominoperlneal operation. J Pediatr Surg 1967;2:106-10. 7. Rehbein F. Imperforate anus: experiences with abdomino-perIneal and abdomtnosacro-perineal pullthrough procedures. J Pediatr Surg 1987;2:99-105. 8. de Vrtes PA. The surgery of anorectal anomalies: Its evolution, with evaluations of procedures. Curr Probl Surg 1984;21: 44-5. 9. Scharli AF. Analysis of anal incontinence. Prog Pediatr Surg 1984;17:93-104.
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