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Imperforate anus
Imperforate Anus II. The Rationale and Technic of the Sacroabdominoperineal Operation
By WILLIAM B. KIESEWETTER N A PREVIOUS COMMUNICATION 1 we reported on the surgical anat.omy of imperforate anus. There are 4 areas in which some practical application of these observations can be made in high type III cases that need a pull-through procedure. These are the maximal use of puborectalis capabilities, the effective employment of the external sphincter muscle, the minimal disturbance of pelvic sensation, and the best use of perianal skin. The puborectalis muscle is the only strong muscle left for continence in imperforate anus, and there are indications that there may be some sensory fibers in it as well. It is most important, therefore, that it be carefully preserved and utilized. It is our feeling that this can best be done by a posterior, sacral approach to the operative area as the initial part of the operative procedure. As suggested by Stephens, 2 this enables one to localize the puborectalis sling, and to begin the dissection from below the sling if the blind pouch is above the pubococcygeal line. Should the blind pouch be below the pubococcygeal line, the pubococcygeus portion of the levator sling is split in the midline and the puborectalis tunnel developed from above. It is our feeling that this preliminary localization of the desired pull-through tunnel can be done without disarticulating the coccyx, and that by so doing, one may lessen any possible damage done to that area. The sacral approach is simply one of accurately delineating an area through which a subsequent pull-through will be done within the external sphincter and puborectalis muscles. Out of the previous sensory studies 1 came the distinct impression that the preservation of sensation is best with the least amount of dissection in the pelvis. This can be best accomplished by the use of the pull-through technic of Romualdi, 3 Rehbein 4 and Soave, ~ who use a seromuscular sleeve as part of the tunnel for pull-through by denuding the mucosa from the distal 8-10 cm. of bowel. In so doing, the nerve endings of sensation may be damaged, but the sensory factors that may be in the seromuscular coat and surrounding musculature are preserved as are the pelvic nerves. This denuding of mucosa also permits good visualization and closure of a rectourinary or high rectovaginal fistula, should such be present. Finally, if there is some ingrowth of dermal nerve fibers for a centimeter or two above the mucocutaneous line; it seems wise to make some type of cruciate incision in the skin and to interdigitate it with the pulled through bowel. Most of the protruding mucosa which so often results from conventional pullthroughs is eliminated. Mucocutaneous stricture is also less likely to form postoperatively.
I
From The Surgical Clinic of the Children's Hospital of Pittsburgh and The Department of Surgery of the University of Pittsburgh School of Medicine. WILLIAM B. KIESEWETTER,M.D.: Professor of Pediatric Surgery, University of Pittsburgh, School of Medicine; Surgeon-in-Chief, Children's Hospital of Pittsburgh. 106 JOURNAL OF PEDIATRICSURGERY,VOL. 2, No. 2 (APRIL), 1967
IMPERFORATE ANUS: SACROABDOMINOPERINEALOPERATION
~07
OPERATIVE APPROACH
Neonatal Care In those patients who will require an abdominoperineal pull-through to correct their abnormality, a colostomy should be instituted as soon as the diagnosis is made in the newborn period. The most effective colostomy in our hands has been the divided left transverse colostomy with the distal stoma higher than the proximal. Such a divided colostomy lessens the chance of fecal material from the proximal bowel entering the distal bowel. When the abdomen is open, a catheter should be placed in the distal limb of the colostomy and the meconium irrigated out to prevent inspissation and reduce the chance of urinary tract infect i o n in those patients with a fistula. Layer by layer arterial silk sutures are placed joining the peritoneum, fascia and skin to the divided segments of bowel. A skin bridge of at least 2 inches between the stomas should be created. Appropriate urinary studies should be undertaken to rule out any major urinary tract anomalies. A distal colostomy loop barium study should be done for the purpose of estimating the distance from the blind bowel to the skin and defining its relationship to the puboeoccygeal line. At the same time, indigo carmine can be given with the barium to ascertain or confirm the presence of a rectourinary fistula.
Sacral Portion of Definitive Procedure The time of election for definitive therapy is 6 to 12 months of age. Mechanical cleansing of the distal loop of the colostomy is the only bowel preparation employed. A large Levine tube is put down the distal colostomy to help in identifying the end of the blind pouch through the subsequent sacral incision. The patient is placed in the face-down position after having had a metal urethral sound inserted. The buttocks are strapped apart. The incision itself is made from the tip of the coccyx to 1-2 cm. from the projected anal opening. The coccyx is left untouched, and a midline dissection is carried out with central division of the levator sling if the puborectalis muscle is below the coccyx. The level of the puborectalis will be determined by the position of the blind rectum in the distal loop barium study. It will lie just beyond the lower-most portion of that structure. If the descent of the bowel has proceeded beyond the coccyx, the pubococeygeal portion of the ]evator sling will have to be divided in the midline to look down from above on the blind bowel and the puboreetalis muscle. If the blind bowel is at or above the tip of the coccyx, the puborectalis sling will be found at the tip of the coccyx, by dissection in the midline from below. These relationships can be readily seen in Figure 1A. The puborectalis is gently dilated to make a canal about 2-4 cm. in diameter. A crueiate skin incision is made over the external sphincter (Fig. 1B). The skin is separated from the muscle and preserved in toto. An opening is made in the middle of the muscle and it is gradually dilated with Hegar dilators until large enough to accept the prospective pull-through segment (usually 2-4 cm. in diameter). The previously made purorectalis tunnel and external sphincter opening are joined and dilated with Hegar dilators in such fashion as not to tear the muscles. A 1/2 inch empty Penrose drain is placed in the tunnel up to the level of the blind pouch (Fig. 1B). We emphasize that an empty Penrose drain should be utilized so that a Hegar dilator can be inserted through it at a later stage in the procedure. The posterior incision is closed so as to carefully rejoin any pubococeygeal and anococcygeal fibers that may have been divided in the course of the dissection.
Abdominal Portion of Procedure With the child in the supine position, a left paramedian or left lower quadrant hockev stick incision is made. No pelvic dissection is carried on outside of the bowel. A small amount of saline is injected beneath the seromuscular coat of the bowel just above the peritoneal reflection so as to leave a distal pouch of approximately 8-10 cm. The seromuscular coat is incised vertically through the saline injection and a 2--4 cm. wide band of mucous membrane is dissected free from the seromuscular coat in circumferential fashion. It is
108
WILLIAM B. KIESEWETTER
)) :OLON
)R A N I
Fig. 1B Fig. 1A Fig. 1 A . - - A view of the anatomy as seen through the sacral approach. The blind terminal bowel is seen below the coccyx. A sound is in the urethra and a hemostat outlines the puborectalis sling looking down from above through the divided puboeoccygeal fibers of the levator ant. Fig. l B , - - A n empty Penrose drain is in the new external sphincter-puborectalis canal.
preferable to leave the bowel intact in the course of this dissection, for it makes it easier to separate the seromuscular and mucosal coats. These 2 layers are then separately divided, and the mucosa is cored out in its entirety as shown in Figure 2A. This removal of mucosa should include its attachment to the urinary tract or high vagina if a rectourinary or high rectovaginal fistula is present. The fistula should be divided and oversewn with fine catgut. The projected pull-through segment of rectosigmoid is then prepared so as to provide adequate length to reach the skin without tension. This should be done by dividing vascular attachments centrally in the mesentery and preserving the marginal arcades. A Hegar dilator is inserted through the hollow portion of the perineal Penrose drain until it is felt at the bottom of the seromuscular blind pouch (Fig. 2B). A blind pouch incision is made on the Hegar dilator and the incision enlarged until it is sufficient to accommodate the prospective pull-through segment. The Penrose drain itself is used only as a guide for the insertion of grasping forceps which can be placed on the drain from the outside and drawn up into the seromuscular cuff. We have used 2 Allis clamps for this purpose. It is preferable not to sew the pull-through segment of bowel to the Penrose drain, because in the pullthrough the stitches may tear out and the localization of the puborectalis tunnel lost. Grasping the rectosigmoid segment with the Ali~s clamps, this howe[ is brought onto the perineum without tearing the puborectalis tunnel or the external sphincter circle (Fig. 3A). If the external sphincter opening is too small, it is desirable to enlarge it by cleanly dividing the muscle in its anterior portion. The bowel should be under no tension prior to interdigitation of the pull-through with the quadrant skin flaps. Cutting out V-portions in the full thickness of the bowel facilitates placing the skin tips as far up in the reconstructed anal canal as possible (Fig. 3C). The only stitches used in the perineum are for close approximation of the interdigitated mncocutaneous junction. The seromuscular sleeve should be drained through a stab wound in the abdominal wall The sleeve itself should be attached loosely to the bowel with a few stitches. Reperitoneali-
IMPERFORATE
ANUS:
SACROABDOMINOPERINEAL
109
OPERATION
DR OSE AIN)
Fig. 2A Fig. 2B
Fig. 2 A . - - T h e m u c o s a is b e i n g d i s s e c t e d a w a y f r o m t h e seromuscular coat of t h e d i s t a l 8--10 cm. of b l i n d r e c t u m . Fig. 2 B . - - A H e g a r d i l a t o r is i n d e n t i n g t h e s e r o m u s e u l a r sleeve.
in
position
within
the
Penrose
guide
and
zation of the abdominal cavity should be carried out before closure of the abdominal wound in layers.
Postoperative Care The seromuscular sleeve drain should be removed in 3 to 5 days. Gentle dilatation with Hegar dilators should not be started until at least 14 days postoperatively. It is desirable to close the colostomy as soon as an adequate anorectal channel has been established. The fecal stream is the best dilator. Such colostomy closure can generally b e accomplished by the end of the third postoperative week. Antibiotics are not used pre- or postoperatively without specific indication. SUMMARIO
IN INTERLINGUA
In un serie de 9 disseetiones post morte de specimens de ano imperforate nos ha notate que le sphincter externe esseva semper presente, que ]e musculo puborectal esseva al linea pubococcygee sed n u n q u a m supra illo, e que le musculo puborectal se trovava tanto infra le linea pubococcygee como le intestino cec. Prolongate observationes catamnestic in 12 patientes operate ha revelate que le sensation esseva intacte quando le intestino distal non habeva essite dissecate, que certe reactiones sensori esseva presente in le muscu]o puborectal per se, e que sensation se extendeva in omne casos per 1 a 2 cm in alto como un increscentia a b l e linea mucocutanee. NOTE A cin6 of this procedure is available from the Film Library, Davis and Geek Company, Danbury, Connecticut.
110
W I L L I A M B, K I E S E W E T T E R
// "I S '~[~llI Jjill |
q
~.l,/
A
IB
G
Fig. 3 . - - A , cruciate skin incision on the perineum. B, the rectosigmoid within the undivided circle of the external sphincter. C, interdigitation of proctodeal skin and pull-through.
REFERENCES 1. Kiesewetter, W. B., and Nixon, H. H.: Imperforate anus. I. Its surgical anatomy. J. Pediat. Surg, 2:000, 1967. 2. Stepheus, F. B.: Imperforate rectum: a new surgical technique. Med. J. Australia 2:202, 1953. 3. Romualdi, P.: Treatment of some particular difllcult cases of anus prostaticus by means of abdomino-perineal intra-
rectal operation. Rev. Chir. Pediat. 111:27, 1961. 4. Rehbein, F.: Operation for anal and rectal atresia with recto-urethral fistula. Chirurg. 30:417-18, 1959. 5. Soave, F.: Hirschsprung's disease: a new surgical technique. Arch. Dis. Child. 39:116, 1964.
By WILLIAM B. KIESEWETTER N A PREVIOUS COMMUNICATION 1 we reported on the surgical anat.omy of imperforate anus. There are 4 areas in which some practical application of these observations can be made in high type III cases that need a pull-through procedure. These are the maximal use of puborectalis capabilities, the effective employment of the external sphincter muscle, the minimal disturbance of pelvic sensation, and the best use of perianal skin. The puborectalis muscle is the only strong muscle left for continence in imperforate anus, and there are indications that there may be some sensory fibers in it as well. It is most important, therefore, that it be carefully preserved and utilized. It is our feeling that this can best be done by a posterior, sacral approach to the operative area as the initial part of the operative procedure. As suggested by Stephens, 2 this enables one to localize the puborectalis sling, and to begin the dissection from below the sling if the blind pouch is above the pubococcygeal line. Should the blind pouch be below the pubococcygeal line, the pubococcygeus portion of the levator sling is split in the midline and the puborectalis tunnel developed from above. It is our feeling that this preliminary localization of the desired pull-through tunnel can be done without disarticulating the coccyx, and that by so doing, one may lessen any possible damage done to that area. The sacral approach is simply one of accurately delineating an area through which a subsequent pull-through will be done within the external sphincter and puborectalis muscles. Out of the previous sensory studies 1 came the distinct impression that the preservation of sensation is best with the least amount of dissection in the pelvis. This can be best accomplished by the use of the pull-through technic of Romualdi, 3 Rehbein 4 and Soave, ~ who use a seromuscular sleeve as part of the tunnel for pull-through by denuding the mucosa from the distal 8-10 cm. of bowel. In so doing, the nerve endings of sensation may be damaged, but the sensory factors that may be in the seromuscular coat and surrounding musculature are preserved as are the pelvic nerves. This denuding of mucosa also permits good visualization and closure of a rectourinary or high rectovaginal fistula, should such be present. Finally, if there is some ingrowth of dermal nerve fibers for a centimeter or two above the mucocutaneous line; it seems wise to make some type of cruciate incision in the skin and to interdigitate it with the pulled through bowel. Most of the protruding mucosa which so often results from conventional pullthroughs is eliminated. Mucocutaneous stricture is also less likely to form postoperatively.
I
From The Surgical Clinic of the Children's Hospital of Pittsburgh and The Department of Surgery of the University of Pittsburgh School of Medicine. WILLIAM B. KIESEWETTER,M.D.: Professor of Pediatric Surgery, University of Pittsburgh, School of Medicine; Surgeon-in-Chief, Children's Hospital of Pittsburgh. 106 JOURNAL OF PEDIATRICSURGERY,VOL. 2, No. 2 (APRIL), 1967
IMPERFORATE ANUS: SACROABDOMINOPERINEALOPERATION
~07
OPERATIVE APPROACH
Neonatal Care In those patients who will require an abdominoperineal pull-through to correct their abnormality, a colostomy should be instituted as soon as the diagnosis is made in the newborn period. The most effective colostomy in our hands has been the divided left transverse colostomy with the distal stoma higher than the proximal. Such a divided colostomy lessens the chance of fecal material from the proximal bowel entering the distal bowel. When the abdomen is open, a catheter should be placed in the distal limb of the colostomy and the meconium irrigated out to prevent inspissation and reduce the chance of urinary tract infect i o n in those patients with a fistula. Layer by layer arterial silk sutures are placed joining the peritoneum, fascia and skin to the divided segments of bowel. A skin bridge of at least 2 inches between the stomas should be created. Appropriate urinary studies should be undertaken to rule out any major urinary tract anomalies. A distal colostomy loop barium study should be done for the purpose of estimating the distance from the blind bowel to the skin and defining its relationship to the puboeoccygeal line. At the same time, indigo carmine can be given with the barium to ascertain or confirm the presence of a rectourinary fistula.
Sacral Portion of Definitive Procedure The time of election for definitive therapy is 6 to 12 months of age. Mechanical cleansing of the distal loop of the colostomy is the only bowel preparation employed. A large Levine tube is put down the distal colostomy to help in identifying the end of the blind pouch through the subsequent sacral incision. The patient is placed in the face-down position after having had a metal urethral sound inserted. The buttocks are strapped apart. The incision itself is made from the tip of the coccyx to 1-2 cm. from the projected anal opening. The coccyx is left untouched, and a midline dissection is carried out with central division of the levator sling if the puborectalis muscle is below the coccyx. The level of the puborectalis will be determined by the position of the blind rectum in the distal loop barium study. It will lie just beyond the lower-most portion of that structure. If the descent of the bowel has proceeded beyond the coccyx, the pubococeygeal portion of the ]evator sling will have to be divided in the midline to look down from above on the blind bowel and the puboreetalis muscle. If the blind bowel is at or above the tip of the coccyx, the puborectalis sling will be found at the tip of the coccyx, by dissection in the midline from below. These relationships can be readily seen in Figure 1A. The puborectalis is gently dilated to make a canal about 2-4 cm. in diameter. A crueiate skin incision is made over the external sphincter (Fig. 1B). The skin is separated from the muscle and preserved in toto. An opening is made in the middle of the muscle and it is gradually dilated with Hegar dilators until large enough to accept the prospective pull-through segment (usually 2-4 cm. in diameter). The previously made purorectalis tunnel and external sphincter opening are joined and dilated with Hegar dilators in such fashion as not to tear the muscles. A 1/2 inch empty Penrose drain is placed in the tunnel up to the level of the blind pouch (Fig. 1B). We emphasize that an empty Penrose drain should be utilized so that a Hegar dilator can be inserted through it at a later stage in the procedure. The posterior incision is closed so as to carefully rejoin any pubococeygeal and anococcygeal fibers that may have been divided in the course of the dissection.
Abdominal Portion of Procedure With the child in the supine position, a left paramedian or left lower quadrant hockev stick incision is made. No pelvic dissection is carried on outside of the bowel. A small amount of saline is injected beneath the seromuscular coat of the bowel just above the peritoneal reflection so as to leave a distal pouch of approximately 8-10 cm. The seromuscular coat is incised vertically through the saline injection and a 2--4 cm. wide band of mucous membrane is dissected free from the seromuscular coat in circumferential fashion. It is
108
WILLIAM B. KIESEWETTER
)) :OLON
)R A N I
Fig. 1B Fig. 1A Fig. 1 A . - - A view of the anatomy as seen through the sacral approach. The blind terminal bowel is seen below the coccyx. A sound is in the urethra and a hemostat outlines the puborectalis sling looking down from above through the divided puboeoccygeal fibers of the levator ant. Fig. l B , - - A n empty Penrose drain is in the new external sphincter-puborectalis canal.
preferable to leave the bowel intact in the course of this dissection, for it makes it easier to separate the seromuscular and mucosal coats. These 2 layers are then separately divided, and the mucosa is cored out in its entirety as shown in Figure 2A. This removal of mucosa should include its attachment to the urinary tract or high vagina if a rectourinary or high rectovaginal fistula is present. The fistula should be divided and oversewn with fine catgut. The projected pull-through segment of rectosigmoid is then prepared so as to provide adequate length to reach the skin without tension. This should be done by dividing vascular attachments centrally in the mesentery and preserving the marginal arcades. A Hegar dilator is inserted through the hollow portion of the perineal Penrose drain until it is felt at the bottom of the seromuscular blind pouch (Fig. 2B). A blind pouch incision is made on the Hegar dilator and the incision enlarged until it is sufficient to accommodate the prospective pull-through segment. The Penrose drain itself is used only as a guide for the insertion of grasping forceps which can be placed on the drain from the outside and drawn up into the seromuscular cuff. We have used 2 Allis clamps for this purpose. It is preferable not to sew the pull-through segment of bowel to the Penrose drain, because in the pullthrough the stitches may tear out and the localization of the puborectalis tunnel lost. Grasping the rectosigmoid segment with the Ali~s clamps, this howe[ is brought onto the perineum without tearing the puborectalis tunnel or the external sphincter circle (Fig. 3A). If the external sphincter opening is too small, it is desirable to enlarge it by cleanly dividing the muscle in its anterior portion. The bowel should be under no tension prior to interdigitation of the pull-through with the quadrant skin flaps. Cutting out V-portions in the full thickness of the bowel facilitates placing the skin tips as far up in the reconstructed anal canal as possible (Fig. 3C). The only stitches used in the perineum are for close approximation of the interdigitated mncocutaneous junction. The seromuscular sleeve should be drained through a stab wound in the abdominal wall The sleeve itself should be attached loosely to the bowel with a few stitches. Reperitoneali-
IMPERFORATE
ANUS:
SACROABDOMINOPERINEAL
109
OPERATION
DR OSE AIN)
Fig. 2A Fig. 2B
Fig. 2 A . - - T h e m u c o s a is b e i n g d i s s e c t e d a w a y f r o m t h e seromuscular coat of t h e d i s t a l 8--10 cm. of b l i n d r e c t u m . Fig. 2 B . - - A H e g a r d i l a t o r is i n d e n t i n g t h e s e r o m u s e u l a r sleeve.
in
position
within
the
Penrose
guide
and
zation of the abdominal cavity should be carried out before closure of the abdominal wound in layers.
Postoperative Care The seromuscular sleeve drain should be removed in 3 to 5 days. Gentle dilatation with Hegar dilators should not be started until at least 14 days postoperatively. It is desirable to close the colostomy as soon as an adequate anorectal channel has been established. The fecal stream is the best dilator. Such colostomy closure can generally b e accomplished by the end of the third postoperative week. Antibiotics are not used pre- or postoperatively without specific indication. SUMMARIO
IN INTERLINGUA
In un serie de 9 disseetiones post morte de specimens de ano imperforate nos ha notate que le sphincter externe esseva semper presente, que ]e musculo puborectal esseva al linea pubococcygee sed n u n q u a m supra illo, e que le musculo puborectal se trovava tanto infra le linea pubococcygee como le intestino cec. Prolongate observationes catamnestic in 12 patientes operate ha revelate que le sensation esseva intacte quando le intestino distal non habeva essite dissecate, que certe reactiones sensori esseva presente in le muscu]o puborectal per se, e que sensation se extendeva in omne casos per 1 a 2 cm in alto como un increscentia a b l e linea mucocutanee. NOTE A cin6 of this procedure is available from the Film Library, Davis and Geek Company, Danbury, Connecticut.
110
W I L L I A M B, K I E S E W E T T E R
// "I S '~[~llI Jjill |
q
~.l,/
A
IB
G
Fig. 3 . - - A , cruciate skin incision on the perineum. B, the rectosigmoid within the undivided circle of the external sphincter. C, interdigitation of proctodeal skin and pull-through.
REFERENCES 1. Kiesewetter, W. B., and Nixon, H. H.: Imperforate anus. I. Its surgical anatomy. J. Pediat. Surg, 2:000, 1967. 2. Stepheus, F. B.: Imperforate rectum: a new surgical technique. Med. J. Australia 2:202, 1953. 3. Romualdi, P.: Treatment of some particular difllcult cases of anus prostaticus by means of abdomino-perineal intra-
rectal operation. Rev. Chir. Pediat. 111:27, 1961. 4. Rehbein, F.: Operation for anal and rectal atresia with recto-urethral fistula. Chirurg. 30:417-18, 1959. 5. Soave, F.: Hirschsprung's disease: a new surgical technique. Arch. Dis. Child. 39:116, 1964.