Twenty-Five

Years’ Experience

With Hirschsprung’s

Disease

By Preston Foster, George Cowan, and Earle L. Wrenn, Jr Chicago,

Illinois and Memphis,

9 Sixty-three patients with biopsy-proven Hirschsprung’s disease were diagnosed at Le8onheur Children’s Medical Center, Memphis, TN between 1955 and 1980. Fifty-eight of these patients had pull-through procedures performed by three similarly trained pediatric surgeons. The follow-up was 100 percent, averaging 8 years from initial diagnosis. Demographics, surgical procedures performed, and complications are reviewed. Significant findings are (1) anastomotic strictures occurred most frequently when the level of aganglionosis was at the sigmoid colon; (2) postoperative encopresis was most likely when the endorectal pull-through procedures were performed before the age of 10 months; and (3) with selective use of colostomies or enterostomies performed prior to the pullthrough procedure, the incidence of enterocolitis was low, with 0% mortality. On the basis of these findings, we recommend that, when the most proximal level of aganglionosis is the sigmoid colon, it is important to critically inspect the angulation of mesenteric blood vessels and viability of the splenic flexure pull-through colon segment to prevent ischemia and therefore anastomotic strictures. The endorectal pull-through procedure should be delayed until after 10 months of age. Infants with Hirschsprung’s disease should have a colostomy or enterostomy prior to a pull-through procedure. Patients diagnosed at 10 months of age or more, who have not had earlier bouts of enterocolitis, are not low percentile weight, and are without signs of severe obstruction, are candidates for pullthrough without a prior fecal diversion procedure. Q 1990 by WA Saunders Company. INDEX WORDS: Hirschsprung’s disease: aganglionic megacolon.

F

OLLOWING Hirschsprung’s thorough clinical description of congenital megacolon,’ Tiffin et al’s histological confirmation of associated aganglionosis,* and Swenson’s and Bill’s development of the pull-through procedure,3 thousands of infants and children have been cured of congenital megacolon. Modifications by Duhamel,4 Soave,’ and Rehbein6 have been instituted to decrease complications and improve functional results. Despite these advances, surgical treatment of Hirschsprung’s disease can result in wound infection, anastomotic leakage, anastomotic strictures, chronic encopresis, and enterocolitis. Experience with these problems will be reviewed along with possible causes and preventive measures. MATERIALS AND METHODS

Hospital records of 7 1patients with biopsy-proven Hirschsprung’s disease, from 1955 to 1980, were reviewed for demographics, procedures performed, and complications. Eight patients who had pull-through procedures performed at other institutions were exJournal of

Pediatric Surgery,

Vol 25,

No 5 (May), 1990: pp 531-534

Tennessee

eluded. Of the remaining 63 patients, 58 underwent a pull-through procedure. These 58 patients will be reviewed in further discussion of complications and management. Follow-up was 100%. with a median follow-up of 8 years. When a pull-through procedure was performed prior to 10 months of age, it was defined as “early.” The traditional and recommended age for performance of a pull-through procedure for Hirschsprung’s disease is 10 to 15 months.’ An anastomotic stricture was defined as any symptomatic narrowing of the anastomosis that required dilatation or revision. In all Duhamel cases, obliteration of the rectal septum was performed initially with the Gross crushing clamp. Any patient who had a sudden onset of diarrhea with abdominal distention, fever, and leukocytosis was considered to have enterocolitis. Chronic postoperative encopresis was defined as continuous, uncontrollable soiling of underclothing present for more than 2 years postoperatively. RESULTS

There was a total of 63 patients, 48 males (76.2%) and 15 females (23.8%). There were 51 white patients (80.1%) 11 black patients (17.4%), and one oriental patient (1.5%). The most common proximal level of aganglionosis was the rectosigmoid (43.2%). The second most common level was the sigmoid colon (20.9%), followed by small bowel aganglionosis (10.3%) and the rectum (8.6%) (Fig 1). Three Swenson procedures were performed prior to 1960. Since then, 43 Duhamel procedures have been performed. Twelve patients had endorectal pullthrough procedures, 10 with the Boley modification,8 and two as described by Soave.’ The use of colostomies or enterostomies prior to pull-through procedures was selective by age at initial presentation. The majority of patients diagnosed after 10 months of age underwent a pull-through without a prior colostomy or enterostomy (Fig 2). Most patients diagnosed prior to 10 months of age had an initial colostomy or enterostomy performed. Pull-through procedures were usually performed between 1 and 2 years of age (Fig 3). Pull-through

From the Department of General Surgery, Rush-Presbyterian-St Luke’s Medical Center, Chicago IL; and the Departments of General Surgery and Pediatric Surgery, University of Tennessee Medical School at Memphis, and Lebonheur Children’s Medical Center, Memphis, TN. Date accepted: April 7.1989. Address reprint requests to Preston Foster, MD, Department of General Surgery, Rush-Presbyterian& Luke’s Medical Center, 1753 W Congress Pkwy, Chicago, IL 60612. @ 1990 by W.B. Saunders Company. 0022-3468/90/2505-0015%03.00/0 531

532

FOSTER, COWAN, AND WRENN

12

-I

Descending Colon (8.6%) Upper Sigmoid

Sigmoid

Total COlOll

(20.8%)

Rectostgmoid

(43.2%)

Rectum (6.6%) Low Rectum Ultrashort

(1.7%) (1.7%)

Fig 1. Level of aganglionosis of patients undergoing pullthrough procedures. Each dot represents one patient’s most proximal level of aganglionosis.

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4

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6 10 12 14 16 16 20 22 24 Years-j

AGE

procedures were rarely performed on children 10 months of age or younger. Each of the five patients in whom a pull-through was performed before the age of 3 months had aganglionosis to the level of the ileum or jejunum. A few patients had pull-through procedures performed between 2 and 23 years of age. This older group consists of patients who were diagnosed late or initially refused operation. The incisional infection rate was 1.7% for all pullthrough procedures. One of the 12 patients who had an endorectal pull-through procedure developed an incisional infection. None of the patients undergoing the Duhamel or Swenson procedures developed an incisional infection.

0 SUBGROUP OF PATiENTS WITH LONG SEGMENT AGANGLIONOSIS

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Fig 2. Age at the time of diagnosis of aganglionosis. (Upper graph) patients with no colostomy or enterostomy performed before a pull-through procedure. (Lower graph1 patients with colostomy or enterostomy performed before a pull-through procedure. Note the selective use of colostomies and enterostomies prior to pull-through procedures as related to age at diagnosis.

Fig 3.

Age at the time of pull-through procedure.

Two anastomotic dehiscences occurred (3.4%). One followed an endorectal pull-through and the other occurred following a Swenson procedure. Each of these complications was recognized early postoperatively. A diverting colostomy was performed in each case and the pelvic abscesses drained, with no mortality. Twelve patients (21%) developed an anastomotic stricture. Nine had a Duhamel procedure and three had an endorectal pull-through procedure. All strictures resolved following one or two reapplications of the Gross clamp to the septum for the Duhamel procedures, or rectal dilatation of the stenoses for endorectal pull-through or Swenson procedures. Patients with aganglionosis at the level of the sigmoid colon developed a significantly greater number of anastomotic strictures (P < .05) (Fig 4). No other level of agangliosis was significantly associated with anastomotic strictures. Enterocolitis occurred prior to a pull-through procedure in six of 58 patients (10.3%), and followed in four of 58 patients (6.8%), with no deaths. No patient had both preoperative and postoperative enterocolitis. The incidence of enterocolitis either prior to or following a pull-through procedure was not associated with any particular level of aganglionosis (P -C .l) (Fig 5). Of the 58 patients who underwent a pull-through procedure, seven (12%) had chronic encopresis. All of these patients became asymptomatic 3 to 7 years postoperatively. The incidence of chronic encopresis was high in patients who underwent the endorectal pull-through procedure. Of 12 patients undergoing endorectal pullthrough procedure, five (42%) had chronic encopresis. Four of four patients (100%) who had an endorectal pull-through before aged 10 months had chronic encopresis. In constrast, only one of the eight patients

533

HIRSCHSPRUNG’S DISEASE

Rectosigmoid

(43.2%)

Rectum(8.6%) Low Rectum Ultrashort

(1.7%) (1.7%)

Fig 4. Level of aganglionoais and the incidence of anaatomotic strictures. Black dots represent patients not developing anastomotic strictures: open circles represent patients with enastomotic strictures. Chi-square analysis of a 2 x 2 contingency table of the strictured and nonstrictured subgroups, versus the level of aganglionosis, the sigmoid colon, and all other segments is significant (P < .Ol).

(12%) who had this procedure performed after the age of 10 months had chronic encopresis (P < .05) (Fig 6). Five of 63 patients died of multiple congenital neurological and cardiac abnormalities before a pullthrough procedure for Hirschsprung’s disease could be performed. Of the 58 operative patients, two with small bowel aganglionosis died after a pull-through procedure. There were no deaths in the patients who had sigmoid or more distal aganglionosis who underwent a pull-through procedure. DISCUSSION

Enterocolitis continues to be the major cause of mortality in patients with Hirschsprung’s disease. This

Descending Colon (8.6%) I

Total COkX I5

Rectosigmoid

143.2%)

(8.6%) Low Rectum

(1.7%) (1.7%)

Fig 5. Level of aganglionosis and incidence of enterocolitis in patients undergoing pull-through procedures. Black dots represent patients without enterocolitis: open circles represent patients with enterocolitis before a pull-through procedure (n = 6); and stars represent patients with entercolitis after a pull-through procedure (n = 4). Chi-square analysis shows no significant diierence in the incidence of snterocolitis either prior to or following a pull-through procedure. as related to the level of aganlionosis when grouped as (1) the rectum and distally, (2) the rectosigmoid. (31 the sigmoid and descending colon. and 14) the transverse colon and proximally.

Fig 6. Incidence of chronic encopresis as related to age of the patient at the time of the pull-through procedure and the procedure performed. Note: All four of the patients Cith endorectal pull-through procedures performed prior to 10 months of age had chronic encopresis. In contrast, only one of the eight patients undergoing the endorectal pull-through procedure after 10 months of age suffered this complication (P < .05).

complication can be averted by early diagnosis and relief of obstruction.g A recent study reported an 18% incidence of enterocolitis appearing prior to any surgical procedure. These patients had a 30% mortality. The authors also reported a 15% incidence of postoperative enterocolitis, with a 2% mortality.9 Most of the deaths occurred in patients who remained undiagnosed until after 3 months of age and presented with this complication.g When the diagnosis of Hirschsprung’s disease is made prior to 10 months of age, three possible modes of management may be appropriate. (1) Immediate pullthrough; (2) diverting enterostomy followed by pullthrough; or (3) removal of impaction and routine enemas followed by pull-through at a later date. We favor the second mode of management (Fig 2), which has a relatively low incidence of enterocolitis (10.3%) prior to pull-through, and no mortality. Patients who present at more than 10 months of age, without severe signs of obstruction, without prior enterocolitis, and at an acceptable percentile weight for age, are candidates for conservative decompression (ie, removal of impaction and routine enemas) followed expeditiously by a pull-through procedure (Fig 2). This selective approach, using age and clinical findings, is associated with a low incidence of enterocolitis both prior to and following pull-through procedures. The incidence of enterocolitis both prior to and following pull-through procedures was not associated with any particular level of aganglionosis (Fig 5). This is in agreement with a recent series that addresses this relationship. lo In contrast, a large collected series reported a statistically higher incidence of enterocolitis in those patients with long segment disease, ie, aganglionosis proximal to the left colon9 Because the level of aganglionosis is only one of many factors that might

534

FOSTER, COWAN, AND WRENN

predispose the patient to enterocolitis, the review of this single factor alone might produce this apparent disagreement. Anastomotic strictures can be related to poor blood supply to the pull-through segment of colon.” The high incidence of strictures in patients with sigmoid colon aganglionosis may be related to the mobilization of the splenic flexure as the pull-through segment in the rectum. Ischemia to this segment may be produced by the precarious blood supply of the splenic flexure, aggravated by the marked angulation of the colonic vessels in anastomosis of the pull-through segment in the rectum. Careful evaluation of the vasculature, including vessel angulation and briskness of bleeding from the cut end of the colon, is especially recommended when the splenic flexure is the segment for pull-through. Patients who underwent the endorectal pull-through and those who had this procedure at an age less than 10 months, had a higher incidence of chronic encopresis. (Fig 6) In contrast, only one patient who underwent an endorectal pull-through procedures after the age of 10 months had this complication (Fig 6). Although we did not use the endorectal pull-through procedure as often as the Duhamel procedure, there was no greater incidence of encopresis early in our series than later, Patients undergoing endorectal pullthrough procedures prior to 10 months of age did not experience a significant increase in other related complications, such as anastomotic strictures, infections, or enterocolitis. These findings somewhat dispel pure inexperience with this particular procedure as the cause of this association of chronic encopresis with the

early performance of the endorectal pull-through procedure. A high incidence of chronic encopresis following endorectal pull-through procedures has been described.‘* In this study, eight of 11 patients had continued soiling, with a median follow-up of 32 months. In contrast, a recent series comparing the endorectal pull-through with the Duhamel procedure described no specific problem with encopresis, no matter the age at which either procedure was performed.13 In a recent series in which 20 infants had endorectal pull-through procedures, no episodes of chronic encopresis were noted.14 The relationship between the early performance of endorectal pull-through procedures and chronic encopresis is not clear. “Overflow” fecal soiling has been related to a continued spastic internal anal sphincter following an endorectal pull-through procedure.i5 The endorectal pull-through is the only pull-through procedure that does not involve direct weakening of the internal anal sphincter. In contrast, the Swenson and Duhamel operations involve at least a partial dividing or weakening of the sphincter. This functional obstruction could contribute to the incidence of chronic encopresis in these patients, yet there were no other symptoms of obstruction or a higher incidence of enterocolitis. An increase incidence of diarrhea following the endorectal pull-through has been noted.“j Possibly, the decreased consistency of the stools might make fecal continence more difficult to achieve. Although no specific explanation for this finding is forthcoming, we cannot recommend early performance of endorectal pull-through procedures because of the high incidence of chronic encopresis in our series.

REFERENCES 1. Hirschsprung H: Stuhlthragheit Neuageborener infogle von Dilatation und Hypertrophie des Colons. Jahrbuchfur Kinderheilkunde 27: l-7,1888 2. Tiffin ME, Chandler LR, Faber HK: Localized absence of the ganglionic cell of the myenteric plexus in congenital megacolon. Am J Dis Child 59:1071-l’O82, 1940 3. Swenson 0, Bill AH: Resection of rectum and rectosigmoid with preservation of sphincter for benign spastic lesions producing megacolon. Surgery 242 12-220, 1948 4. Duhamel B: Retrorectal and transanal pull-through procedure for the treatment of Hirschsprung’s disease. Dis Colon Rectum 7:455-458, 1964 5. Soave F: Hirschsprung’s disease: A new surgical technique. Arch Dis Child 39: 116- 124, 1964 6. Rehbein F: Intraabdominelle resktion oder Rektosigmoidektomie bei der Hirschsprung’schen Krankheit. Chirurg 29:336, 1958 7. Peter G, Jones: The timing of surgical procedures, in: Clinical Pediatric Surgery: Diagnosis and Management (ed 2). New York, NY, Blackwell, 1976, pp 17-25 8. Boley SJ: New modification of the surgical treatment of Hirschsprung’s disease. Surgery 56:1015-1017,1964 9. Kleinhaus S, Boley SJ, Sheran M, et al: Hirschsprung’s

disease: A survey of the members of the surgical section of the American Academy Pediatrics. J Pediatr Surg 14:588-597, 1979 10. Nixon HH: Surgical treatment of Hirschsprung’s disease, in Holschneider AM (ed): Hirschsprung’s disease. New York, NY, Thelme-Stratton, 1982, pp 103- 113 11. Swenson 0, Idriss FS: Excision of rectal stricture with end-to-end anastomosis. Arch Surg 93:54-58, 1966 12. Postuma R, Cockery JJ: Abnormalities of function and fecal water following the modified Soave operation for Hirschsprung’s disease. Prog Pediatr Surg 101141-154, 1977 13. Soper RT, Figueroa DR: Surgical treatment of Hirschsprung’s disease: Comparison of modification of the Duhamel and Soave operation. J Pediatr Surg 6:761-766, 1971 14. So HB, Schwartz DL, Becker JM, et al: Endorectal “pullthrough” without preliminary colostomy in neonates with Hirschsprung’s disease. J Pediatr Surg 151470-471,198O 15. Clausen EG, Davies OG: Late complications of the Swenson pull-through operation for Hirschsprung’s disease. Am J Surg 106:372-380, 1963 16. Harrison MW, Deitz DM, Campbell JR, Campbell TJ: Diagnosis and management of Hirschsprung’s disease: A 25 year perspective. Am J Surg 152:49-54, 1986

Twenty-five years' experience with Hirschsprung's disease.

Sixty-three patients with biopsy-proven Hirschsprung's disease were diagnosed at LeBonheur Children's Medical Center, Memphis, TN between 1955 and 198...
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