Br. J. Surg. Vol. 67 (1980) 436438 Printed in Great Britain

Results following surgery for Hirschsprung's disease: a review of three operations with a reference to neorectal capacity OMAR K H A N A N D H A R O L D H . N I X O N *

SUMMARY The results of operations on a group of 62 patients from those operated for Hirschsprungk disease in a 5-year- period have been assessed. The bowel function and control, also the physical development of the child, have been examined following operation by the Swenson. Duhamel and sutured Soave techniques. The best results were achieved by the Duhamel retrorectal pullthrough operation, although the diyerences were not great.

Late deaths due to an unrecognized enterocolitis remain a problem.

THERE are few reports in the surgical literature compar- ing the results of the three main types of operation for this congenital large bowel condition (1, 2). The clinical, functional and developmental results of patients oper- ated o n by the transanal prolapse and resection tech- nique of Swenson (3), retrorectal pullthrough of Duhamel, Lester Martin modification (4) and the endorectal mucosal stripping operation of Soave (5) are compared (Fig. 1).

were reviewed at least 5 years following their operation--17 girls and 45 boys. These cases were selected to fulfill the following criteria: investigated preoperatively by a barium enema, anorectal manometry and rectal biopsy; operated upon not less than 5 years before by the senior author (H. H. N.); continued to be reviewed here ( 1 5 were lost to follow-up) (Table I).

The barium enema examination was performed on the unprepared bowel. This was diagnostic in 34 patients and suggestive of a low large bowel obstruction in 10. In the remaining 18 cases the X-ray appearances were not helpful, including 7 patients who had the enema performed elsewhere. Anorectal manometry was diagnostic in 33 cases. These tests were performed, without an anaesthetic, using apparatus described by Lawson and Nixon (10) and the interpretation was based on their criteria. Ketamine anaesthesia in toddlers undergoing manometry would have improved these results just as further barium examinations may have increased their accuracy. However, this seemed unnecessary since the diag- nosis was made on the Bodian submucosal biopsy. The histological specimens were examined by the normal haemat- oxylin and eosin staining techniques and also by the cholines- terase histochemical method of Karnovsky and Roots ( I I).

When the diagnosis of Hirschsprung's disease was confirmed

Swenson type Original Duhamel Endorectal pullthrough (Simonsen. Soave. Nixon)

Fig. 1. The operative procedures.

The assessment of faecal continence is difficult in this every patient in this series had a preliminary loop colostomy, relatively young age group. Several authors have followed in due course by a definitive pullthrough procedure attempted t o determine factors in anal continence to with colostomy cover. The colostomy was not closed until give an evaluation by anorectal pressure contrast studies of the distal gut showed sound healing, usually

2 or 3 weeks later. Five years following operation the patients returned for their studies and by radiological methods (6-9).

annual check-up, whereupon the clinical state, development and anal canal and the activity of the voluntary and bowel control were recorded. Anorectal manometry was sphincter in response to neorectal distension have been performed to determine the activity of the gut and sphincters made, including assessment of rectal capacity. and to measure the capacity of the neorectum.

Measurements Of the resting pressure in the

Patients and methods From 1967 to 1972, 107 cases were operated upon for Hirschsprung's disease at this hospital in'one unit: 62 children

*The Hospital for Sick Children, Great Ormond Street, London WCI. Requests for reprints to: H. H. Nixon.

Surgical treatment of Hirschsprung’s disease 437

I0 30 50 70

0

0 .

0 I Du 10 hamel 30

0

0

._ 50 70

- 1 1 , . ,

Soave

10 30 50 70 Resting pressure (cmH,O)

Fig. 2. Neorectal capacity and resting pressures (lines denote limits of accepted normal range).

Table I: DETAILS OF 62 CASES OF HIRSCHSPRUNG’S DISEASE FOLLOWED FOR 5 YEARS AFTER OPERATION

Swenson Duhamel Soave

1 . No. of patients 21 21 20 2. Condition diagnosed at (age range) 2 mth-9 yr 2 mth-8 yr 2 mth-7 yr 3. Average age at resection 9 mth 6 mth 6 mth 4. Diagnosis confirmed by

X-ray Positive 8 14 12 Suggestive 3 3 4 Not diagnostic 10 4 4

Positive 11 10 12 Suggestive 2 5 5 Not diagnostic 7 5 3

Anorectal physiology

5. Length of resected gut

6. Enterocolitis 10 7 3 7. Late deaths from enterocolitis 3 2 I 8. Immediate postoperative deaths 0 0 0 9. Constipation 13 6 I

10. Obstructive symptoms 7 3 2

13. GU problems 0 0 0

Mean 12 cm lOcm 16.5 cm Range 5-29 cm 65-28 cm 10-24 cm

Every 3rd day Every day > I motion/day H 6 10 8 I 1. Frequency of stoolin 12. Fully potty trained 2, yr after op.*

14. Patients with residual problems 7 with soiling None 8 with loose motions 15. Heightlweight development (mean centile) 55/45 60150 70150

* Others took longer.

Results (Table I ) In this group of patients, 20 developed signs and There was a steady improvement in function and symptoms of enterocolitis after surgery and unfortu- bowel control with the passage of time. The older nately 6 died (one 8 years postoperatively) since their children achieved a more satisfactory degree of control condition was not recognized but treated elsewhere as than the younger age group, but should the disease have gastroenteritis. None of these patients who died had been recognized only later in life, a persistent chronic evidence of residual Hirschsprung’s disease clinically or constipation sometimes delayed development of normal at post-mortem examination. Enterocolitis may appear function due to the secondary changes in the retained in the otherwise healthy child postoperatively. ganglionic bowel. Twenty-four of the 56 infants

The clinical assessment of the resultant continence achieved completely normal bowel control within 2 % correlated with the radiological and manometric years of the operation and according to their parents findings. were ‘potty trained’ in this time, having good control

438 0. Khan and H. H. Nixon

over defaecation with knowledge of presence of stool in the rectum.

These patients are amongst those noted in Fig. 2 whose neorectal capacity and resting pressure in the anal canal were within our accepted normal range (rectal capacity 150-200 ml and resting pressure 30-40 cmH,O). It will be seen that the rectal capacity and resting pressure varied considerably in all three groups of patients.

Thirteen of the 19 patients available for review after the Duhamel procedure had a normal capacity neorec- tum with a normal consistency and control of bowel motions.

With the Swenson procedure 11 of the 18 surviving patients had a larger capacity rectum. This appeared to be related to a chronic constipation secondary to a rectal inertia. The constipation responded to aperients and bowel training.

Eleven of the 19 patients who had a Soave procedure had a small capacity neorectum likely to be responsible for the frequent bowel motions. This frequency usually responded to loperamide.

Uncomplicated recovery also influenced continence, since the 3 children who developed pelvic sepsis had impaired bowel function and delayed achievement of the same degree of control. By covering the pullthrough procedure with a colostomy, the more serious compli- cations of anastomotic breakdown have been averted.

Enuresis was not a problem in any of the patients, they all had full bladder control by the time they were potty trained.

The length of gut resected also appeared to influence bowel control as the greater the length of the resection the more fluid was the consistency of the bowel motion and the greater the incidence of soiling.

The average heights and weights of the 62 children show that these children were above the 50th percentile in height, but were below the 50th percentile in weight. None of these children had reached puberty.

Discussion This is a survey of operations performed for Hirschsprung’s disease in one unit at this hospital. There are many operations, and each has many vari- ations in technique, but the most satisfying results achieved in this series followed the Duhamel operation, though the differences between the operations were marginal. The Duhamel is also the operation of choice for this condition in adults at St Mark’s Hospital using the Lester Martin modification (2).

Some of the operations have fallen into disrepute, especially in the hands of the inexperienced surgeon. To quote Swenson (12), ‘If a well-trained surgeon may observe the details of the operation and then persevere, good results may be obtained’.

The Swenson procedure has been criticized for com- plications, not only of constipation and poor control but problems associated with damage to the pelvic splanchnic nerves and to the genitourinary system, since to resect the aganglionic segment completely one has to perform a very low resection. However, no damage is caused to the pelvic splanchnic nerves by a correctly performed Swenson operation as shown by an earlier series from this hospital (1 3). Such complications are the result of incorrect dissection or of infection from anastomotic leakage in the absence of a protective colostomy.

The Soave procedure has been criticized for rectal strictures. If the outer muscle cuff is devascularized this may occur but is avoidable by correct technique preserv- ing the superior rectal artery. The need for dilatations of the ‘anastomosis’ is avoided by substituting the suture technique for the original non-suture method (14, 15).

In the Duhamel operation, the anterior half, although aganglionic, has sensation, while the posterior gan- glionic segment is peristaltic, which enables the patient to defaecate normally. This appears theoretically to be a more physiological operation.

Diarrhoea may be of no significance but diarrhoea with abdominal distension indicates a visit to a doctor for investigation to exclude or abort an attack of enterocolitis. Enterocolitis may affect children who never had the condition before or after surgery: it can unexpectedly and suddenly appear several years after operation-in one instance in this series as long as 8 years after a successful operation with normal health in the intervening years.

Acknowledgements We are grateful to the Editorial Committee of the Archives of Disease in Childhood for permission to reproduce Fig. 1 from ‘Hirschsprung’s disease’ by H. H. Nixon (1964; 39 109-15).

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