3 Decades HD

Hirschsprung's Disease: Three Decades' Experience at a Single Institution

BV M.D. Klein and A.l. Philippart

Defro ft, Michigan

Experience with 250 patients treated for Hirschsprung's disease over the past three decades shows several interesting features. Only 58% had rectosigmoid disease. Twenty-six percent had long segment disease and 12% had total colon aganglionosis. This distribution did not change significantly over the decades. The male:female ratio was 3.3 in all groups. but dropped to 2.2 with total colon disease. The mean age at diagnosis decreased from 18.8 months during the 1960’s to 2.6 months in the 1980. The mean age at which the pullthrough procedure was performed also dropped from 34 months to 15 months. Twenty-three patients died: 17 in the 1960’s and three in each of the succeeding decades. The Swenson procedure was predominant during the 1980’s. but was only 2% of the pull-through In the 1980’s. The endorectal pullthrough increased from 22% in the 1960’s' to 71%. in the 1980’s. In the 1970’s and 1980’s the Duhamel accounted for 28% of the pull-through. with a mean follow-up of 51 months. the mean number of episodes of post pullthrough Enterocolitis per patient was 0.53. This increase from 0.44 in the 1980., to 0.52 In the 1970’s. to 0.67 In the 1980’s. The Incidence of post-pullthrough Hirschsprung’s Enterocolitis increased somewhat with the length of the bowel involved, and was greatest with the Duhmel (0.95 episodes per patient. less with the Swensen (0.60. and least with endorectal pullthrough (0.38 In contrast, the incidence of complications other than Enterocolitis were greatest with the Swenson 20% less with the endorectal pullthrough (17%) and least with the Duhamel 13% The number of patients treated has not varied greatly over the decades. Prediagnosis Enterocolitis is no longer as prevalent. and thus not as great a danger as it was before 1960 but post--pullthrough Enterocolitis still causes significant morbidity. and may be related to the type of pullthrough performed. and the length of bowel involved. Long-segment disease was much more common in this series than previously reported and the mortality with this entity has not been great.

INDEX WORDS: Hirschsprung's disease; megacolon. aganglionic.

Hirschsprung’s disease continues to be a common, if not the most common, cause of Intestional obstruction in the newborn. While we ~t)continue to speak of it as one disease, there are many ways in which longer segment (LSH) and total colon aganglionosisis (TCH) differ from the more common rectosigmoid (RSH) involvement. We also observed that not all the characteristics of Hirschsprung's disease which were so clear in the 1960's were evident in the l990's. For these reasons we reviewed the whole of Hirschsprung's disease at the Children's Hospital of Michigan in Detroit (CHM) from January 1960 through June 1991.

MATERIALS AND METHODS

Several means were used to identify patients with Hirschsprung’s disease including the medical records computer system. depart-met of pathology index records, the neon atology data base, and a prospective index of patients with Hirschsprung's disease main-tained by a former chief of Surgery. All available hospital and office or clinic records were analyzed and extracted. A total of 250 patients were found to hsve a definitive diagnosis of Hirschsprung's disease. In 246 the diagnosis was made at CHM. In reporting results only those with definite information either positive or negative are included so the number of patients in each analysis varies to exclude those with missing data. Medical records are such that in some cases a pathology report and operative report may be available with no other hospital chart. Thus, we have data on that patient for the type of operation and a definitive diagnosis, but no follow-up data or information on how the patient presented.

Data were reviewed after extraction. coded onto data sheets. and analyzed using the MIDAS mainframe computer software pro-gram. Data were analyzed to investigate what changes might have occurred over the roughly three decades of the 1960's (100 patients), 1970's (85), and 1980's (65) as well as what differences there might be among patients with RSH (144 patients, 58%), I,SH (65,26%), and TCH (29, 12%).

RESULTS

Birth History

Males were 77% (192) of all patients and the male:female ratio was 3.5 for RSH and fell to 2.2 for TCH (LSH was 3.0). Only 17 patients (8%) were premature by either weight or gestational age. Twenty-four percent (n = 179) of all mothers gave a history of some complication of pregnancy This was greatest (50%) among prematures and increased in each decade from 13% in the 1960s to 39% in the 1980s. It was also greater in those with TCH (63% in the 1980's), but not in those with LSH.

Eighteen percent had another congenital anomaly (44/238). This was greater in the 1980s (27%) and especially greater among premature (47%). Eleven percent (19/176) had respiratory distress at birth as evidenced by a need for oxygen or a ventilator (six patients). This increased to 17% in the 19805 and was much higher in children with TCH (LSH children were no different than those with RSH).

Diagnoses

Mean age at diagnosis was 10 months and was much less for premature (3.9 months). Age at diagnosis also decreased with each decade from 18.8 to 5.5 to 2.6 months. It was least (1.0 months) with TCH. The time between first recognizable symptoms and contact with a physician (mean, 60 days) also decreased over the decades from 103 to 18 days. It was much less in those with TCH (2.5 days).

Eighty percent had constipation as one of their presenting symptoms, somewhat fewer in TCH (65%). Vomiting was a presenting symptom in 58%, some-what greater in TCH (73%). Malnutrition or an appearance of spindly arms and legs was unusual (5%). Abdominal distention was present at diagnosis in 80% of all patients in all decades no matter how long the segment involved. Eighty percent of the patients had the diagnosis made with the first barium enema (201 patients had barium enema reports available), but it was diagnostic in only 55% of those with TCH. Ml but two patients had the diagnosis confirmed by rectal biopsy, and suction biopsies were used increasingly in the 19705 (19%) and 19805 (35%). Still, 85% had full-thickness rectal biopsies.

Colostomy

Enterocolitis (EC) was unusual prior to colostomy, a mean of 0.089 episodes per patient (n = 237). Overall it was greater in TCH, but in the 1980's it was highest in RSH (0.158) and lowest in TCII (0.091). The number of hospital admissions prior to colostomy was also greater in the 1980's (2.0 v 1.5 in the 1960s) and in TCH (2.9 overall v 1.6 for RSH and LSH). EC also occurred after the colostomy and before the pullthrough (PT), 0.125 episodes per patient.

PullThrough

The mean age at which the PT was done decreased over the decades from 34 months to 19 to 15 and was not different for TCH. In the 1960s most patients had Swenson procedures (SWPT) (70%), 20% had endo-rectal pullthrough (ERPT), and 10% had the Duhamel procedure (DUPTS). The variation in procedures could be clearly related to the practice of individual surgeons. By the 19805 only one SWPT was. done while there were 35 ERPTS and 13 DUPTS. Of 19 patients with TCH, 12 (74%) had ERPTS, 4 had DUPTS, and 3 had SWPTS. There was no difference in operating room time for the different operations for the length of the involved bowel. It is interesting that the mean operating time increased & #189; hour each decade to 4½ hours in the19805 Ten percent to 12% of all PTs had complications, and this did not vary with length of segment or decade. There was a difference in the rate of complications, however, depending on which type of PT was done. complications followed the SWPT in 20% of patents, the ER~ in 17%, and the DUPT in only 13%. This finding that the DUPT has somewhat more EC and fewer complications continues in the last decade when the 5WPT is no longer a consideration. In the 1980s the DUPT had a complication rate The ERFI· had a 20% complication raw, and an EC ~ of 031 considering only TCH, the incidence of was slightly less the DU~ (1.0) than with the ~ (0.7).Hospital stay at time of PT also did not vary with the length of the segment involved, but did decrease over the decades from 18.7 to 17.4 to 10.5 days.

Follow-Up Data on the clinical course following the PT are available for 218 patients with a mean length of follow-up of 51 months. The mean number of hospital admissions after the PT was 1.25 for all patients and seemed to increase with the decades (1.07, 1.10, 1.76). Overall it was not significantly greater for TCII(1.32)The mean number of episodes of EC increased with each decade (0.44,0.52,0.67) and was greatest In patients after DUPT (Table 1). It was also much greater in prematures (1.4) and in patients with TCH(0.96).Mortality has decreased over the decades (17 patients, 19%; 3 patients, 5%; and 3 patients, 6%), but remains an issue. It did not vary with length of bowel segment involved (RSH 8%, LSH 12%, TCH 10%).

Table 1. Frequency of Enterocolitis following Different Pullthrough Procedures
Length of Bowel involved	Swenson	Duhamel	Endorectal Pullthrough	All Pullthrough’s
Rectosigmoid	0.51	1.0	0.21	0.46
Long Segment	0.63	0.88	0.39	0.54
Total Colon	1.62	0.7	1.0	0.96
All patients	0.6	0.95	0.38	0.53

NOTE. Data given as mean number of episodes per patient.

Table 2 Comparison of RSH, LSH and TCH to show that LSH is more like RSH than it is Like TCH

Variable	Recosigmoid	Long Segment	Total Colon
Males (%)	78	75	12
Associated anomalies (%)	23	7	21
Respiratory distress at birth (%)	9	6	32
Birth weight (kg)	3.41	3.43	3.1
Complications of pregnancy (%)	25	12	45
Age at diagnosis (mos)	11	12	1.6
Time from symptom to diagnosis (d)	67	87	2.5
Constipation at presentation (%)	60	28	12
Vomiting at presentation (%)	52	59	73
EC prior to colostomy (Episodes/Patient)	0.9	0.8	0.15
EC after colostomy, prior to PT (Episodes/Patient)	0.1	0.1	0.39
Hospital stay at PT (D)	14.8	16.4	25
EC after PT (Episodes/Patient)	0.46	0.54	0.96

Abbreviations: EC, Enterocolitis PT Pullthrough

DISCUSSION

TCH in this series increased from 7% of patients in the 196Os to 20% in the 1980s. The overall incidence of 13% is similar to other series. Morbidity in this group was certainly higher than in RSH and LSH, but mortality was no different.

Prematurely is still unusual, but the number with associated anomalies is higher than previously reported. Like most other centers we more often make the diagnosis at a younger age, and less frequently miss the diagnosis of TCH. An interesting feature of the data in this study is the remarkable difference between LSH and TCH. LSH is much more like RSH in many respects than it is like TCH (Table 2). Thus these entities should be sorted and identified in evaluating treatments.

The awareness of the importance of the early diagnosis and treatment of Hirschsprung's disease in order to prevent the significant incidence of death from EC was emphasized by Kleinhaus et al from a review of the experience of the members of the Surgical Section of the American Academy of Pediatrics. In general we have relied on the barium enema because of its use in differential diagnosis as more patients present earlier as intestinal obstruction. In the older patient with constipation we have been disappointed in the rectal suction biopsy as the mucosa is usually quite thick and we cannot obtain submucosa. In addition there tends to be more bleeding in suction biopsy from the inflamed and congested mucosa of the child with chronic constipation.

The finding that the ERFT is associated with less EC then the DUPT has been Noted elsewhere and disputed, Data, especially follow-up data, on our current clinical practice are not available. Most patients at CHM, however, have an ERPT for RSH or LSH Those with TCH are not more likely to have a DUPT. Since a greater portion of our recently Hirschsprung’s disease patients have TCH we have found that stool rate and EC with ERPT for TCH greater then we would like. Despite the decreased number of episodes of EC with the ERFT, some surgeons continue to use the DUPT because of a decreased incidence of other postoperative complications such as stenosis and its applicability to longer segment disease.

REFERENCES

1. Kleinhaus S. , Boley SJ. Sheran M. et al: Hirschsprung's disease. A survey of the members of the Surgical Section of the American Academy of Pediatrics. J Pediatric Surg 14: 588-597 1979

1. Candy TG, Modified Duhamel procedure for the Treatment of Hirschsprung’s disease in infancy and childhood Review of 41 consecutive cases. J pediatric surgery 17:773-778 1982

Discussion

D. Tuggle (Oklahoma City, OK): Dr Klein, this timely paper concerning close to 300 patients treated for Hirschsprung's disease over 30 years at a single institution serves to drive home several points. You and your colleagues have provided us with a yardstick against which to measure our results.

Long-term follow-up of the children we care for is an important part of what we do. This paper demonstrates that children with Hirschsprung's disease can over a decade have a reduction in morbidity and

mortality probably due to refinements in surgical technique and postoperative care, but the incidence does not approach zero.

These data also suggest that certain types of pull-through may have fewer operative problems but an increased incidence of Enterocolitis. Also, the distribution of aganglionosis in the patients in Detroit is different than that previously reported and may reflect a more accurate distribution of the length of aganglionosis. I have several questions for you:

First, it has been suggested that neonates could undergo a definitive pull-through procedure for Hirschsprung's disease. Did any neonatal patient have definitive pull-through in this series? And, specifically, are you for or against a neonatal pull-through?

Were there any deaths due to Enterocolitis prior to surgery in your series?

Have any of your patients been noted to have neuronal dysplasia? Also, you did not mention ultra-short-segment Hirschsprung's in this group of patients. Specifically, how would you manage ultra short segment Hirschsprung's disease?

And finally, we have recently considered a total colectomy and a J pouch ileoanal pull-through for total colonic Hirschsprung's. Do you think that would be a reasonable alternative from your experience?

M. Klein (response): We have done some pullthroughs for newborns recently although there are none included in this series because their follow-up is too short. Our average follow-up here was about 5 years. It's interesting that it takes a few people like Dr So to show us the way. We've had success so far in the few patients that we've done.

We haven't had a death due to Enterocolitis prior to colostomy or pull-through, but some of our deaths were due to Enterocolitis after the pull-through, more frequently in the total colon patients. Our overall mortality was about 5%.

Neuronal intestinal dysplasia is a concept we still find difficult. We probably would call those patients idiopathic pseudoobstruction or megacolon microcystis. It seems to me that the more times I review the slides of the ones known as NID, the more times the pathologist finally changes and says, "Oh, no, that's just Hirschsprung's disease."

Ultrashort Hirschsprung's disease I haven't personally seen. Arvin Philippart tells me he doesn't think he's seen it.

In terms of the "J" pouch. I have been impressed that many people do an endorectal pull-through for total colon disease and get excellent results I really think it has to do with the age of the patient. The. younger the patient, the more I'd be happy to do a straight endorectal pullthrough The older the patient, the more I would treat him like someone with ulcerative colitis and use a "J” pouch.

From the Department of Sw~~r, Wayne State University School of Medicine, and the Children's Hospital of Michigan,Dietrott, ML

Presented at the 1992 Annaul Meeting of the Section on Stirgeiy of the Antetican Academy of Ped~itncs, San Francisco, Cat'f'omia, October 9-11, 199Z

Aadeess ~q~rttt requests 10 Michael D. Kfrtn, MD, Department of Surery, Chitilnen 'S Ho'pital of Michigan 3901 Bea~lein Blvd, Detroit, MI 48201.

Copyright 01993 ly WB. Satendete Company

0022.3468193/2810.0014$03.OOl0

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of Pediarric Surgery, Vol 29, No 10(October. 1993: pp 1291-1294)

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