Adult HD in Jamaica

Original article doi:10.1111/j.1463-1318.2009.02174.x
Adult Hirschsprung’s disease in Jamaica: operative treatment
and outcome
N. D. Duncan, J. Plummer, S. E. Dundas, A. Martin and A. H. McDonald
Department of Surgery, Radiology, Anaesthesia and Intensive Care, University of the West Indies, Mona, Jamaica
Received 25 June 2009; accepted 21 October 2009; Accepted Article online 21 December 2009
Abstract
Aim This study reviewed the operative management and
outcome of patients with adult Hirschsprung’s disease
treated at the University Hospital of the West Indies,
Mona, Jamaica.
Method The case notes for 11 patients with adult
Hirschsprung’s disease, treated operatively between
January 1986 and December 2007, were reviewed and
data on preoperative diagnosis, operative procedures and
postoperative complications were retrieved.
Results Diagnosis was by open rectal biopsy in nine
patients and by rectal suction biopsy in two patients. The
time taken for preoperative bowel preparation ranged
from 10 to 35 days, and colostomy was required in three
patients to facilitate bowel cleansing. The Soave and
Swenson procedures were used in six and five patients,
respectively, leading to anastomotic stricture in one
patient treated using the Soave procedure and to leakage
in two patients treated using the Swenson procedure. All
procedures took longer than 300 min, and 300–800 ml
of blood or plasma was transfused.
Conclusion Therapeutic procedures for adult Hirsch-
sprung’s disease may result in life-threatening anastamot-
ic complications. A protective colostomy is recommended
when Swenson’s procedure is used.
Keywords Adult Hirschsprung’s disease, anastomotic
leakage
Introduction
Hirschsprung’s disease is usually associated with infancy
[1]. Ganglion cells are absent from both submucous and
myenteric nerve plexuses of the terminal bowel, causing
peristalsis failure and resulting in functional intestinal
obstruction [2]. Patients develop massive colonic disten-
sion with air and faeces, and are prone to life-threatening
enterocolitis [3,4].
The first report of Hirschsprung’s disease in an adult is
attributed to Rosin, who in 1950 described the disease in
a 54-year-old physician [5]. We traced approximately 300
cases of adult Hirschsprung’s disease in the medical
literature up to 2006 [6]. A diagnosis of adult Hirsch-
sprung’s disease is made if a patient with colorectal
aganglionosis is older than the arbitrary age of 10 years at
the time of diagnosis [6–8]. Publications on adult
Hirschsprung’s disease have been in the form of case
reports [6,7,9], case series [10–13] and retrospective
studies [14,15]. Patients with adult Hirschsprung’s
disease are usually laxative-dependent and have a lifelong
history of constipation [7–9,14,15]. With few exceptions
[16,17], these patients have a short or an ultra-short
segment of aganglionosis [6,7,9].
The three surgical procedures employed to treat adult
Hirschsprung’s disease (the Soave procedure, the Swen-
son procedure and the Duhamel procedure) are the same
as used for classic Hirschsprung’s disease in infancy [18–
21]. All three involve the resection of histologically
confirmed aganglionic bowel but differ in the mode of
reconstruction. The Soave procedure involves removal of
the rectal mucosa while retaining the muscular cuff, and a
ganlionic segment of colon is anastomosed to the mucosa
of the anal canal [18,19,22,23]. In the Swenson proce-
dure, the aganglionic rectum is resected and ganglionic
colon is anastomosed full thickness to the anal canal [20].
The Duhamel procedure involves a side-to-side colorectal
anastomosis after drawing the ganglionic colon retro-
rectally through a window created in the posterior upper
anal canal [21]. The surgeon’s preference has been the
main determining factor in the operation a patient
receives [24].
Correspondence to: N. D. Duncan, Department of Surgery, Radiology,
Anaesthesia and Intensive Care, University Hospital of the West Indies, Mona,
Kingston 7, Jamaica.
E-mail: [email protected]
Ó 2011 The Authors
454 Colorectal Disease Ó 2011 The Association of Coloproctology of Great Britain and Ireland. 13, 454–458
This report reviews the management of 11 cases of
adult Hirschsprung’s disease treated at the University
Hospital of the West Indies (UHWI), Mona. This
institution is the premier tertiary referral facility in
Jamaica, which has a population of 2.6 million.
Method
The case notes of 11 patients diagnosed with adult
Hirschsprung’s disease and treated at the UHWI, Mona,
between 1986 and 2008 were reviewed. Patients > 18
years of age were managed jointly by paediatric and adult
surgical teams, while only paediatric surgeons managed
younger patients. Paediatric surgeons co-managed adult
patients because they had greater experience with proce-
dures for Hirschsprung’s disease.
The age and gender of patients, radiological investi-
gations, duration of clinical symptoms and presence or
absence of a stoma prior to corrective surgery were
recorded. The rectal biopsy technique used to make the
diagnosis in each patient was also recorded.
The bowel was cleared of faeces before surgery by
enemas and whole-gut irrigation with normal saline via a
nasogastric tube, and by manual removal of faeces from
the rectum where necessary. In three patients with a
colostomy, irrigation of the bowel via this route was also
undertaken.
In each patient, the definitive procedure chosen, the
operative time and the volume of blood products
transfused were recorded. Operative times were recorded
to the nearest 10 min, commencing from the induction
of anaesthesia to reversal on the operating table.
Antibiotic therapy was given for 24 h perioperatively as
cover against aerobes or anaerobes. All patients had a
nasogastric tube and a urethral catheter.
An open operative procedure was performed in each
case. Patients were treated with either ‘Boley’s’ modifi-
cation of Soave’s procedure [22,23] or by the standard
Swenson operation [20]. None underwent the Duhamel
procedure [21]. A colo-anal anastamosis was fashioned
using interrupted vicryl or silk sutures. No proximal
diverting stoma was formed. The three patients with a
previous colostomy underwent closure of the stoma at
the same time. All patients therefore had a one-stage
(eight patients) or a two-stage (three patients) ‘pull-
through’ procedure. Those treated using Soave’s proce-
dure had closed suction tube drainage of the de-epithel-
ialized rectal cuff [19]. Trans-anastamotic tube drains
were placed in all patients to assist postoperative gaseous
decompression of the colon [19].
This study was conducted with the formal approval of
the Ethics Board, Faculty of Medical Sciences, University
of the West Indies, Mona.
Results
The 11 patients (12–38 years of age) included eight male
patients. The operative details and clinical outcomes are
summarized in Table 1. All had a history of constipation
and laxative dependency dating back to infancy. The five
patients younger than 18 years were included in the study
because the traditional definition of adult Hirschsprung’s
disease includes patients of 10 years of age and older [6–
8]. They had shorter periods between defecation
(< 4 weeks) compared with patients over 18 years of
age who defecated sometimes as infrequently as every
12 weeks.
An initial abdominal X-ray indicated massive faecal
loading, causing left hemi-diaphragmatic elevation in all
patients. A barium enema in three patients showed a
Table 1 Perioperative details of patients
with adult Hirschsprung’s disease.
Patient
no.
Age ⁄
gender Biopsy Stoma Operation
Time
(min)
Blood product
transfusion
(ml)
Postoperative
hospital
stay (days)
1 12M O No Soave 420 300 PC, 300 P 5
2 13M RSB No Swenson 420 0 6
3 14M RSB No Swenson 300 390 P 27 + 60
4 20M O No Soave 300 500 WB 7
5 24M O Yes Swenson 300 0 10
6 27M O No Soave 300 600 WB 10
7 28M O Yes Soave 420 300 PC, 800 P 10
8 18M O Yes Swenson 360 300 WB 23 + 5
9 12F O No Soave 300 500 WB 10
10 13F RSB + O No Swenson 360 300 PC 8
11 38F O No Soave 330 0 5
F, female; M, male; O, open biopsy; PC, packed cells; P, plasma; RSB, rectal suction
biopsy; WB, whole blood.
N. D. Duncan et al. Adult Hirschsprung’s disease in Jamaica
Ó 2011 The Authors
Colorectal Disease Ó 2011 The Association of Coloproctology of Great Britain and Ireland. 13, 454–458 455
transition zone between the ganglionic and aganglionic
bowel (see Fig. 1). Hirschsprung’s disease was diag-
nosed by open biopsy of the rectum 1–2 cm above the
pectinate line [1] in nine patients and by rectal suction
biopsy in two patients [25,26]. In one patient, rectal
suction biopsy provided an inadequate specimen but
diagnosis was determined on repeat biopsy using the
open technique.
The period of time taken for preoperative bowel
cleansing ranged from 10 to 35 days. Attempts at bowel
cleansing failed in two (patients 5 and 7) of the three
patients who already had a colostomy owing to the
presence of a faecal bolus (Fig. 2).
Six patients underwent Soave’s procedure and five
underwent Swenson’s procedure. The operative times
ranged from 300 to 420 min (the average operative times
were 360 min for the Swenson procedure and 348 min
for the Soave procedure). Nine patients were transfused
with 300–800 ml of packed cells or plasma during
surgery but none became hypotensive because of blood
loss.
Postoperative morbidity occurred in three patients,
two of whom (patients 3 and 8) developed life-threaten-
ing morbidity. Patient 3, a 14-year-old adolescent male,
developed anastomotic dehiscence and faecal peritonitis
and was managed by diverting transverse loop colostomy
and a revision Swenson’s procedure 3 months later.
Patient 8, an 18-year-old man also developed an anasta-
motic dehiscence after Swenson’s procedure. A revision
Swenson’s procedure was undertaken 6 months later.
Non-life-threatening postoperative morbidity occurred in
patient 7 who developed a tight anastamotic stricture
following a Soave procedure. This resolved after a
6-month period of dilatation.
The range of follow up was 1–10 years. At the time of
writing, all patients had good faecal continence with a
stool frequency of two to five times per week and had
gained weight.
Discussion
This is the first report from the West Indies on adult
Hirschsprung’s disease, where only few referral centres
undertake surgical correction of this condition. There is
no Hirschsprung’s disease registry in the West Indies, and
this report outlines only the Jamaican experience with
adult Hirschsprung’s disease between 1986 and 2008.
The gender distribution of the patients (nine male
patients and three female patients) in this study is
consistent with other reports on adult Hirschsprung’s
disease, which refer to a 4:1 male/female ratio [7,9,14].
Although a lifelong history of constipation and
abdominal discomfort was elicited from all patients
[13–15], and left hemi-diaphragmatic elevation was
evident on abdominal radiographs [8], variations in
clinical presentation may occur in adult Hirschsprung’s
disease. Constipation may commence, not in childhood
but in late adulthood [7]. Massive faecal overload may
lead not only to abdominal problems but also to thoracic
consequences, including splaying of the lower ribs,
mediastinal deviation, pulmonary atelectasis and pneu-
monia [8,9]. A visible transition zone following admin-
istration of a barium enema, which is a classic feature of
Hirschsprung’s disease [1], may be absent in ultra-short-
segment adult Hirschsprung’s disease with megarectum
[8,9].
The fact that rectal suction biopsy produced an
inadequate specimen in one of three patients biopsied is
in keeping with the view that this technique may not be
ideal for older patients [1,27]. Croffie et al., in a review,
concluded that rectal suction biopsy gave the best results
before 3 years of age [28].
Figure 2 Radiograph showing a calcified faecal bolus in the
sigmoid colon.
Figure 1 Barium enema examination showing the rectosigmoid
transition zone.
Adult Hirschsprung’s disease in Jamaica N. D. Duncan et al.
Ó 2011 The Authors
456 Colorectal Disease Ó 2011 The Association of Coloproctology of Great Britain and Ireland. 13, 454–458
Fairgrieve [13], in 1963, recommended a two-stage
procedure as the safest operative approach for the
treatment of adult Hirschsprung’s disease. He based this
recommendation on the fact that chronic faecal overload
caused massive colonic dilatation and bowel disparity,
resulting in difficulty in forming the anastomosis. Frozen-
section biopsy and colostomy were recommended as the
first stage, followed by abdomino-anal ‘pull through’
some months after. A time interval between operations
was advised to allow the colon to return to its normal
diameter. Two-stage procedures, however, are not with-
out risk because a colostomy does not guarantee
successful faecal clearance [9]. A colostomy sited specif-
ically for bowel cleansing in 3 of 11 patients failed to
achieve this objective in two of the patients and, as a
consequence, an extended bowel resection that included
the faecal bolus had to be undertaken in these two
patients. The resection of faecally impacted colon in adult
Hirschsprung’s disease was highlighted by Miyamoto
et al., who resorted to left hemicolectomy 40 days after
transverse colostomy because of the inability to clear the
faeces [7].
The two operations employed in this study (Soave’s
procedure and Swenson’s procedure) were time consum-
ing, as both took longer than 5 h. Blood loss was
significant in 8 of 11 patients who were transfused with
300–800 ml of blood during surgery. The risks associ-
ated with the procedures (pelvic nerve injury and
anastamosis leakage in the Swenson procedure and
anastamotic stricture in the Soave procedure) are well
documented [1,8,24] and surgeons have warned of the
added difficulty posed by the adult patient [2,8,13]. Two
of the 11 patients developed anastamotic dehiscence and
faecal peritonitis following Swenson’s procedure and one
developed a troublesome stricture after a Soave opera-
tion. It can be argued that a protective colostomy with a
definitive ‘pull-through’ procedure might have prevented
the life-threatening complication of anastamotic dehis-
cence [8] but such complications are not rare in adult
Hirschsprung’s disease, having been reported, by Myam-
oto et al., to occur in 28.6%, 19.3% and 7.4% of patients
Swenson’s, Soave’s and Duhamel’s procedure, respec-
tively [7].
Duhamel’s operation is regarded by many authors to
be the preferred procedure as the operative morbidity is
lower than that associated with Soave’s and Swenson’s
procedures [7,8,14,15]. This is because only limited
pelvic dissection is necessary to complete the procedure,
reducing the risk of pelvic nerve injury and involving less
pelvic dissection. Furthermore, the use of a GIA
TM
stapler (Covidien, Mansfield, Massachusetts, USA) facil-
itates the side-to-side colorectal anastamosis [1,7]. This
approach was not employed in Jamaica because local
surgeons were more familiar with the Soave and Swenson
procedures. Despite increasing reliance on minimally
invasive laparoscopic [29] and trans-anal pull-through
techniques [30] for Hirschsprung’s disease in children,
we found no reports advocating this approach for adult
patients with Hirschsprung’s disease. The clear need for
less-invasive options, however, has led some surgeons to
use anorectal myectomy alone or in combination with
anterior resection [11]. Anorectal myectomy involves
resecting a longitudinal strip of aganglionic rectal muscle,
thereby alleviating the obstructive effect of internal
sphincter spasm [1]. It must be emphasized, however,
that anorectal myectomy is contraindicated in all patients
except for those with a very short segment (< 5 cm) of
aganglionosis [1,11].
This retrospective study is the first to outline adult
Hirschsprung’s disease at a West Indian referral institu-
tion. Our finding that Swenson’s procedure in particular
was associated with significant life-threatening morbidity
reinforces the present perceptions on the risks of this
procedure in adult Hirschsprung’s disease. This review
also highlights the management of the duration of the
operation. It is possible that the absence of a protective
colostomy might have contributed to life-threatening
anastomotic complications. We therefore recommend its
use when performing the Swenson’s procedure in Adult
Hirschprung’s disease.
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458 Colorectal Disease Ó 2011 The Association of Coloproctology of Great Britain and Ireland. 13, 454–458
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