Hirschsprung’s disease (HD) is rare in adults and it is thus often undiagnosed or misdiagnosed. Through this series of 12 patients we try to study the clinical characteristics of this pathology, to define its diagnostic clues and to assess the different therapeutic approaches. Definitive diagnosis is established on histology of specimens from the rectum and colon. The disease involved the rectum and the sigmoid colon in 2 patients and was confined to the rectum, in the 10 others. Treatment was in all cases surgical consisting of recto-colic resection associated with coloanal anastomosis and a protective right lateral ileostomy. We conclude that Hirschsprung’s disease is rare in adults but by no means exceptional. It should be considered in young adults with a history of chronic constipation. Diagnosis is first of all clinical. When barium enema appearances are pathognomonic we needn’t resort to histology to confirm the diagnosis. Anorectal manometry does not usually show RAIR. Current primary treatment of HD diagnosed in adults consists mainly of surgical resection.

1. Hirschsprung’s disease in adults: Clinical and therapeutic features
IRJS
Hirschsprung’s disease in adults: Clinical and
therapeutic features
Haithem Zaafouri¹*, Skander Mrad¹, Manel Mabrouk², Noomen Haoues¹, Mariam Bel Hadj
Salah³, Rabii Noomene¹, Ahmed Bouhafa¹, Anis Ben Maamer¹, Abderraouf Cherif¹
1*
Department of general surgery Habib Thameur hospital Tunis, Tunisia.
2
Department of internal medicine Razi hospital Tunis, Tunisia.
3
Department of Pathological Anatomy and Cytology Habib Thameur hospital Tunis, Tunisia.
Hirschsprung’s disease (HD) is rare in adults and it is thus often undiagnosed or
misdiagnosed. Through this series of 12 patients we try to study the clinical characteristics
of this pathology, to define its diagnostic clues and to assess the different therapeutic
approaches.
Definitive diagnosis is established on histology of specimens from the rectum and colon.
The disease involved the rectum and the sigmoid colon in 2 patients and was confined to
the rectum, in the 10 others.
Treatment was in all cases surgical consisting of recto-colic resection associated with
coloanal anastomosis and a protective right lateral ileostomy.
We conclude that Hirschsprung’s disease is rare in adults but by no means exceptional. It
should be considered in young adults with a history of chronic constipation. Diagnosis is
first of all clinical. When barium enema appearances are pathognomonic we needn’t resort
to histology to confirm the diagnosis. Anorectal manometry does not usually show RAIR.
Current primary treatment of HD diagnosed in adults consists mainly of surgical resection.
Key words: Hirschsprung, rectoanal inhibitory reflex, intestinal obstruction, coloanal anastomosis, Duhamel.
INTRODUCTION
Hirschsprung’s disease (HD) is a rare pathology
affecting about 1 in 5000 births. It is usually diagnosed
in the newborn period; it is the most frequent cause of
bowel obstruction in children. A diagnosis of adult HD is
made when the patient is older than the age of 10 years
(Miyamoto M 2005) at the time of diagnosis. This
disease is often undiagnosed, but its real incidence is
estimated at 2% (Grove K 2009). More than 550 cases
of adult HD have been reported in the English literature
since 1950 (Doodnath R, 2010).
Surgical treatment of HD consists in resecting or
excluding the aganglionic zone in the digestive tube
and preserving normal anal sphincter function.
Several procedures have been described in the
literature. The choice of the procedure depends on the
postoperative outcome and on the experience of the
surgical team. Prognosis is usually good and the results
are habitually satisfactory after surgery.
Objective
Management of adult HD poses a double problem:
- Diagnosis is not easy given the non-specific
presentation of the disease which may manifest in
advanced years (up to 73 years of age in some
reported cases).
- Treatment remains surgical but should the same
procedures as used for HD in infancy be employed? In
other words, there’s some question as to whether to
use the same procedures as used for HD in infancy or
to employ more adapted techniques.
Corresponding author: Dr Haithem Zaafouri,
Assistant professor, Department of general surgery
Habib Thameur hospital Tunis, Tunisia. Email:
zaafouri.haithem@hotmail.fr, Tel: +21697348839
International Research Journal of Surgery
Vol. 2(1), pp. 009-017, March, 2015. © www.premierpublishers.org. ISSN: 2326-7352x
Research Article

2. Hirschsprung’s disease in adults: Clinical and therapeutic features
Zaafouri et al. 009
Table 1. Clinical assessment of incontinence (Holschneider 1983)
Frequency of bowel movements - normal(1-2/ daily)
- often(3-5/daily)
- very often
2
1
0
Consistency of stools - normal
- soft
- loose
2
1
0
Soiling - absence
- stress incontinence diarrhea
- permanent
2
1
0
Anorectal sensitivity - positive
- incomplete
- absent
2
1
0
Ability to hold on - minutes
- seconds
- impossible
2
1
0
Discrimination between stools and gas - normal
- incomplete
- absent
2
1
0
Need for treatment (enemas, medication) - no
- occasional
- always
2
1
0
 Normal intestinal function
 Good result: good bowel control, minor discomfort in social life
 Fair result: regular discomfort in social life
 Poor result: in case of fecal incontinence or permanent soiling
Intestinal function:
 Normal: 14 points
 Good (good bowel control, minor discomfort in social life): 10-13 points
 Fair(regular discomfort in social life): 5-9 points
 Poor(total rectal incontinence): 0-4 points
The aim of this work is to assess the diagnostic and
therapeutic approaches adopted in our department and
to compare them to data from the literature.
MATERIAL AND METHODS
This is a descriptive retrospective study of hospital
records of patients admitted to the department of
general surgery of Habib Thameur Hospital in Tunis
from January 1, 2000 to December 31, 2013. The study
which involved 12 cases, focused on the clinical
presentation, diagnostic procedures, treatment given
and course of the disease.
In order to assess the functional outcome, we collected
all the relevant information obtained during follow-up
visits or by telephone conversation. The findings were
assessed according to Holschneider’s score
(Holschneider AM 1996) (Table 1).
We noted the daily frequency of bowel movements, the
consistency of stools, the possibility to discriminate
between stools and gas, the possibility to hold on in
case of call of nature and if there were any soilings
while specifying the circumstances of their occurrence
in case of stress or concomitant diarrhea.
We also noted if the patients had taken any laxatives or
on the contrary medications to slow down the bowel
movements. During the follow-up visits we tried to
assess the anorectal sensitivity and the sphincter tone.
RESULTS
The study involved 10 men and 2 women aged 31 on
average (range 20-50 years) who had presented, in
most cases, with an obstructive syndrome. In fact, 8
patients (66%) hadn’t passed stools or gas for 24
hours, two patients (16%) had presented to the
emergency department with a peritoneal syndrome,
and the two other patients (16%) had been treated in
the gastroenterology department for chronic
constipation and were referred to our department for
further investigations and surgery after confirmation of
the diagnosis of HD.
History-taking revealed chronic constipation and
manifestations of sub-obstructive syndrome since
childhood in 6 of our patients (50%).
Physical examination showed:
 Important asymmetrical abdominal distension, a
tympanic percussion note and a fecaloma on digital
rectal examination in the eight patients, who presented
with acute intestinal obstruction.
 A temperature of 38.5C, generalized guarding
with painful mobilization of the pouch of Douglas on
digital rectal examination in the two patients admitted to
the emergency department for a peritoneal syndrome.
Plain X-ray abdomen examination was performed in all
patients (Fig 1 and 2); results are shown in Table 2.
Barium enema was performed in 2 of our patients only.

3. Hirschsprung’s disease in adults: Clinical and therapeutic features
Int. Res. J Surg. 010
Table 2. Findings of plain X-ray abdomen examination
Radiologic findings Number N Percentage %
Absence of pelvic aeration 10 83%
Bowel distention 10 83%
Hydro-aeric levels 8 66%
fecaloma 6 50%
pneumoperitoneum 1 8%
Figure 1 and 2. Appearance of a fecaloma and of distended colon on different plain X-ray radiographs of the abdomen
It showed the transition zone and thus determined the
level of the aganglionosis (Fig 3).
Abdominal CT scan was performed in the patients who
had presented to the emergency department with an
obstructive syndrome (n=8) to confirm the organic
nature of the obstruction, determine its site and above
all rule out a tumoral origin of the obstruction which is
more common at this age in our patients.
Plain X-ray radiographs obtained in our patients
revealed an important abdominal distention without an
obvious obstacle proximal to a fecaloma.
Anorectal manometry was performed in 3 patients (25%
of cases) aged 20, 22 and 29, respectively. Recto-anal
inhibitory reflex RAIR) was absent in the three cases.
Biopsies were practiced in all patients: endoscopic
biopsy in 10 patients and open rectal biopsy in 2
patients. The absence of ganglion cells and hyperplasia
of the myenteric nerve plexus (nerve nets) were
confirmed in all cases.
Histologic examinations revealed that it was a short
rectal form in 10 patients (83% of cases) and a recto-
sigmoid form in 2 patients (17% of cases).
Expectant treatment was prescribed to patients rushed
to hospital and in whom the diagnosis of HD hadn’t
been made yet.
Left lateral colostomies (9 by open surgery and one by
laparoscopy) were performed in the patients with an
obstructive syndrome.
As for the two patients with a peritoneal syndrome, a
peritoneal lavage was carried out with resection of the
perforated colonic segment and colostomy, using the
Bouilly-Volkmann procedure in one patient and the
Hartmann procedure in the other.
Definitive treatment was proposed as a first intention
treatment to the two patients referred by the
gastroenterology department, and as a second
intention treatment to the rest of patients who had had
derivation colostomies (3 months on average after
lateral colostomy and 6 months on average in the
patients who had had acute peritonitis).
In each patient, surgery consisted of a colorectal
resection involving the affected zone and the dilated
colonic segment with a coloanal anastomosis on a J-
shaped colic reservoir protected by a right lateral

4. Hirschsprung’s disease in adults: Clinical and therapeutic features
Zaafouri et al. 011
Figure 3. Barium enema radiograph obtained in a 20
year-old patient showing the level of the aganglionosis
Table 3. Follow-up and functionnal outcome according to Holschneider’s score in 6 of our patients
patients sex Age (years) Follow-up time (months) Holschneider’s score
1 M 30 18 6
2 F 46 12 5
3 M 28 24 6
4 M 20 36 11
5 F 33 18 8
6 M 22 36 12
ileostomy. Restoration of continuity was achieved 4
months postoperatively on average. There were no
deaths in our series. There were two cases of coloanal
anastomosis which required repeated dilatations under
general anesthesia before restoration of continuity
achieved 5 months and 11 months after surgery,
respectively.
Soiling was noted in a patient who was referred to a
specialized center for functional rehabilitation. Eight
months later, the results were considered satisfactory.
Mean follow-up time was 24 months. We managed to
call six of the twelve patients. Four of them considered
their functional outcome fair; whereas the two others
considered it good according to Holschneider’s score
(Table 3).
DISCUSSION
Through the study of these 12 cases, the authors
underline the characteristics of adult Hirschsprung’s
disease which, in spite of its rarity, poses diagnostic
and therapeutic problems:
 The absence of specific symptoms and the
regular use of laxatives and even of cleansing enemas
account for the high frequency of acute bowel
obstruction as an initial complaint.
 Two therapeutic approaches were adopted in
the management of our patients: The first approach
consisted of a two-stage surgical operation comprising
a colostomy in its first phase, for bowel cleaning by
removal of the fecaloma, followed, a few months later
by surgical resection. The second approach was based
on a single-stage operation in which the resection,
restoration of continuity and coloanal anastomosis were
all performed during the same intervention.
 After a follow-up period of 24 months, 66% of
the patients we got in touch with considered their
intestinal functional outcome “fair” and 34% of them
considered it “good”. Even though Duhamel’s operation
is the most practiced procedure in the world for the
management of adult HD, we think, after this study, that
colorectal resection with coloanal anastomosis when
they are performed by experienced surgeons, give in
the long run excellent results with a low morbidity and

5. Hirschsprung’s disease in adults: Clinical and therapeutic features
Int. Res. J Surg. 012
Table 4. Examples of published cases of adult HD
Years Authors Number of cases References
1950-1979 15 authors 27 [5-8] [10-20]
1985 Elliott 39 [21]
1990 Starling 8 [22]
1995 Wu 5 [23]
2005 Miyamoto 1 [1]
2010 Vorobyov 90 [24]
2011 Duncan 11 [25]
no surgery-related mortality.
Adult HD was first described by Rosin in 1950 (Rosin
JD 1950). Since then, several other cases have been
reported in the literature: Hiatt in 1951 (Hiatt RB 1951),
Kempton in 1954 (Kempton JJ 1954), Lee in 1956 (Lee
CM Jr 1956) and others (Rehbein F 1966; Marshall WH
1962; State D 1963; Madsen CM 1964; Briggs HC
1971; McGarity 1974; Horovitz IL 1974; Metzger PP
1978; Lesser PB 1979; Swenson O 1957; Maglietta ED
1960; Myers MB 1966). About 550 cases have been
reported in the English literature since 1950 (Doodnath
R, 2010) consisting mainly of case reports and of some
retrospective studies (Table 4).
The incidence of adult HD is still unknown, though
grover announces that it can be as high as 2% of the
population (Kunal G 2009).
Some authors (Parc R 1991) think that there are no
cases of adult HD (cases of HD of adult onset) but
there are only cases which go undiagnosed or
misdiagnosed until adulthood.
The very good body care given to children by their
mothers and which consists mainly of regular cleansing
enemas, allows these HD patients to reach adulthood
before having to undergo surgery.
Some other authors (Miyamoto M 2005; Barnes PR
1986) have reported cases of HD in patients aged over
30 without a history of constipation or of other relevant
symptoms and signs in their childhood.
Rich (Rich AJ 1983) reported a case of newly
diagnosed HD in a 74-year-old patient.
A diagnosis of adult HD is made if a patient (with
colorectal aganglionosis) is older than the arbitrary age
of 10 years at the time of diagnosis (Miyamoto M 2005;
Barnes PR 1986; Fairgrieve J 1963; Mc Cready RA
1980). As with HD presenting in the neonatal period,
adult HD is predominant among males (Table 5).
Like in our series, a history of chronic constipation was
reported by most patients in the different studies (Elliot
MS 1985; Fairgrieve J 1963; Todd IP 1977). Habitually,
it was a major constipation that required laxatives and
often enemas and whose course was interrupted by
unexplained episodes of remission or on the contrary
by an intestinal obstruction that may have necessitated
a laparotomy and even a colectomy. All of Duncan’s
(Duncan N D 2011) and Wang’s (Lin Wang 2014)
patients suffered from unexplored chronic constipation.
Mean duration between two defecations for Duncan’s
patients was 3 to 4 weeks, with an extreme of 12
weeks.
Acute intestinal obstruction was the most frequent chief
complaint of our patients with 66% of cases against
1.8% of cases in the meta-analysis by Doodnath
(Doodnath R 2010). Habitually, it was a lower
obstruction caused by a fecaloma. There were,
however, other cases where the obstruction was due to
a volvulus of the sigmoid colon (5 cases) or to a
volvulus of the transverse colon (1 patient). Alagumuthu
(M Alagumuthu 2011) reported in 2011 three new
cases of adult HD revealed by a volvulus of the sigmoid
colon.
Severe abdominal distension caused by a fecaloma
may lead, in the absence of urgent and adequate
management, to urinary complications such as acute
urinary retention which in turn may lead to the
diagnosis of HD like in the case reported by
Loganathan in 2013 (P Loganathan 2013), or to chest
and respiratory tract complications such as mediastinal
deviation, pulmonary atelectasis or pneumonia
(Metzger PP 1978; Crocker NL 1991).
Barium enema examination reveals a typical funnel-
shaped transition zone (Teitelbaum DH 2006) and
permits thus to determine the level of the aganglionic
segment (Starling JR 1986). This zone may, however,
be absent in the very short forms (less than 5cm) of HD
(Crocker NL 1991; Keighley MRB 1993). Kim (Kim HJ
2008) was unable to detect this radiologic feature in
20% of his patients. The second sign is the retention of
the product of contrast that we can visualize on late
radiographs. Barium enema led to the diagnosis in
84.3% of Vorobyov’s patients (Vorobyov G I 2010).
All of Kim’s patients (Kim HJ 2008) had benefited from
abdominal CT scans that permitted to exclude the other
differential diagnoses and to detect the radiologic
transition zone (the level of the aganglionosis) in each
patient. Abdominal CT scan examinations were

6. Hirschsprung’s disease in adults: Clinical and therapeutic features
Zaafouri et al. 013
Table 5. sex ratio of HD in adults
Year Authors Sex ratio M/F References
1980 McCready 4 (40/10) [31]
1985 Elliott 2 (26/13) [21]
1990 Starling ∞ (8/0) [22]
1995 Wu 4 (4/1) [23]
1995 Kim 1.75 (7/4) [32]
2010 Vorobyov 2.6 (65/25) [24]
2011 Duncan 2.6 (8/3) [25]
practiced in our patients who had presented to the
emergency department with an obstructive syndrome
mainly to rule out a tumoral cause which is more
common at this age. Histologic examinations of the
different operative specimens led to the same
conclusion as the CT scans, i.e. the findings revealed
by both methods totally agreed as to the level of the
aganglionosis.
Anorectal manometry is not a specific diagnostic
procedure of HD: RAIR is absent in idiopathic
megacolon (Barnes PR 1986) and may be present in
very short aganglionoses limited to the anal canal
(Penninckx F 1975; Yoshioka K 1987).
Histologic confirmation was obtained prior to surgery for
all our patients by open rectal biopsy (17% of cases) or
endoscopic biopsy (83 of cases). In Duncan’s series
(Duncan N D 2011), however, the diagnosis of HD was
confirmed by open rectal biopsy in 10 patients out of 11
(90.9% of cases). Rectal suction biopsy provided an
inadequate specimen whose results couldn’t be fully
exploited in a third of patients in some series
(Teitelbaum DH 2006; Alizai NK 1998). Croffie [46]
concluded that this technique gives better results when
it is used in children less than 3 years old.
Histological data provided by biopsies from HD patients
determine the form of the disease which can be ultra
short, rectal, rectosigmoid or totally colonic. Resection
of the aganglionic zone depends on the level of the
aganglionosis. Myers (Myers MB 1966) was the first to
describe in 1966 a case of HD in a 37-year-old man in
whom the aganglionosis and hyperplasia of the nerve
nets involved all the colon.
In our series there was a marked predominance of the
rectal form (83% against 17% for the recto-sigmoid
form). Our results are in accordance with those
published by Doodnath in 2010 (Doodnath R, 2010). In
fact, out of 390 patients, 79.8% of them had lesions that
were confined to the rectum against 12.5% with recto-
sigmoid lesions.
Fairgrieve (Fairgrieve J, 1963) suggested in 1963 an
early therapeutic approach for the management of adult
HD. This approach was based on a two-stage operation
in which the first stage consisted in performing a
colostomy to facilitate bowel cleaning by evacuating the
fecaloma, and the second stage, carried out a few
months later, was devoted to surgical resection and
coloanal anastomosis. The author defended his
strategy arguing that a time-interval between operations
is necessary to allow the initially dilated colon to return
to its normal diameter after colic derivation and to the
surgeon to form the anastomosis without any bowel
disparity. However, initial colostomy does not totally
guarantee successful fecal clearance. In fact, of the 3
patients from Duncan’s series (Duncan N D 2011) who
had undergone colostomy for bowel cleaning, two of
them had to be operated on without totally evacuating
the fecaloma.
A second therapeutic approach that was adopted by
several surgeons, was based on a single-stage
operation-i.e. the patient is brought to the operating
theatre only once. This is the case with Vorobyov
(Vorobyov G I 2010) who chose this approach for
67.8% of his patients.
Two-stage surgery is reserved, according to its
defenders, for patients who have initially undergone
emergency surgery for acute intestinal obstruction or
for perforated colic peritonitis. This was the case for
83% of our patients and 32.2% of Vorobyov’s patients
(Vorobyov G I 2010).
Surgical treatment of HD consists in resecting or
excluding the aganglionic zone in the digestive tube
while avoiding nerve injury and preserving normal anal
sphincter function.
The choice of the procedure depends not only on
morbid-mortality and on its functional outcome, but also
on the habits and experience of the team of surgeons
and other care givers. The difference in terms of results
between the different procedures is not significant and
should be considered cautiously given the small
number of published cases of adult HD and the
absence of prospective randomized and comparative
studies.
The Duhamel procedure is the most practiced
technique in the world: according to a British review
study published in 2010 (Doodnath R, 2010), a total of
231 patients (47.2%) among 490 patients who had
undergone surgery were operated on by the Duhamel
procedure. This technique does not require resection of

7. Hirschsprung’s disease in adults: Clinical and therapeutic features
Int. Res. J Surg. 014
Table 6. Postoperative complications occurring in 490 adult
HD patients between 1950 and 2010
Complications Number
(n=)
Percentage
%
Anastomotic structure 1 0.2
Anastomotic dehiscence 19 3.9
Pelvic abscess 1 0.2
Perineal abscess 1 0.2
Pre-sacral abscess 3 0.6
Bleeding requiring transfusion 2 0.4
Necrosis or retraction of colon 3 0.6
Enterocolitis 5 1.0
Pulmonary embolism 1 0.2
Impotence 3 0.6
Anastomotic fistula 5 1.0
the aganglionic rectum; the rectum is only excluded and
thus the patient is spared any extensive pelvic
dissection that may damage the pelvic sensory nerves.
For these reasons, several authors (Miyamoto M 2005;
Elliot MS 1985; Todd IP 1977) think that it is the
procedure that is associated with the lowest rate of
postoperative complications. As we said earlier, the
choice of the technique often depends on the habits
and experience of the team of surgeons. Like Duncan
(Duncan N D 2011), we didn’t in our department use
this procedure for our patients.
Wang (Lin Wang 2014) published in January 2014 the
long-term results obtained over 44 months and the
quality of life of 59 patients operated on at “Jinling”
Hospital (Ning Li 2013). The technique adopted, called
“Jinling technique”, consisted in performing a subtotal
colectomy with a modified Duhamel procedure. The
different stages of this technique are as follows: a
subtotal colectomy associated with an appendicectomy,
an end-to-side colorectal mechanical anastomosis on
the posterior wall of the rectum at 2cm from the
pectinate line and a side-to-side colorectal mechanical
anastomosis over 5 to 6 cm forming a kind of colorectal
pouch. Forty-two percent of the patients from this series
were operated on by laparoscopy.
The distinctive technical feature of the Soave procedure
is that it can entirely be performed by lower approach.
According to data from the literature (Kleinhaus S 1979)
it is associated with the highest rate of postoperative
complications (anastomotic stricture, for example).
Myectomy that was first described in 1966 by Lynn
(Lynn HB 1966) and practiced in Mayo Clinc, is mainly
reserved for the short or recto-sigmoid forms, but can
also be used in combination with laparoscopy for the
treatment of more extensive forms. Its advantage over
other procedures is that it can be limited to a perineal
approach.
In 1952, State (State D 1952) described the anterior
resection of rectum and colectomy as therapeutic
possibilities in the management of HD in 3 patients
aged between 10 and 21. In our department, we have
chosen, out of habit, to perform a colorectal resection
and to fashion a coloanal anastomosis with a–shaped
reservoir in all our patients. The rate of specific
morbidity was low and the functional outcome was
rather satisfactory.
Post-operative complications in adult HD patients were
mostly minor. Non-specific complications or specific
ones such as anastomotic dehiscence, anastomotic
stricture, pelvic abscess, anastomotic fistula, retraction
or necrosis of colon may occur. Here we have in the
following Table 6 a summary of the postoperative
complications occurring in 490 adult HD patients
between 1950 and 2010.
In Table 7, we sum up to postoperative complications
occurring in 229 adult HD patients reported in the
English literature up to 2005.
Eighty-two percent of Vorobyov’s patients (Vorobyov G
I 2010) considered their sphincter functional outcomes
good, 15% of them thought they were satisfactory and
only 3% found them poor. The author concluded that,
based on statistically significant data (p<0.05) the long-
term functional outcome after a Duhamel procedure
depended on the extension of the aganglionic zone and
on the extent of dilatation proximal to it: the more

8. Hirschsprung’s disease in adults: Clinical and therapeutic features
Zaafouri et al. 015
Table 7. Postoperative complications in adult HD patients
Techniques
Number of cases Postoperative complications
major minor
Duhamel 87 6.9% 5.7%
Swenson 35 28.6% 5.7%
Soave 31 19.4% 9.7%
Myectomy 35 2.9% 2.9%
LAR/myectomy 8 0% 0%
LAR/colectomy 33 12.1% 6.1%
Table 8. Functional outcome after surgery in adult HD patients
Techniques
Number of cases Functional outcome
“Good” “Fair” “Poor”
Duhamel 87 80.5% 19.5% 0%
Swenson 35 85.7% 0% 14.3%
Soave 31 77.4% 6.5% 16.1%
Myectomy 35 45.7% 5.7% 48.6%
LAR/myectomy 8 100% 0% 0%
LAR/colectomy 33 78.8% 6.1% 15.2%
confined to the rectum the lesions are and the dilatation
limited to the sigmoid, the more satisfied the patient is.
In Wang’s series (Lin Wang 2014), 11 patients had 17
major postoperative complications such as anastomotic
dehiscence, rectal incontinence, intestinal obstruction,
sexual or urinary complications. Statistically significant
data revealed that the patients who had had this
procedure were satisfied with the functional outcome in
97% to 98% of cases (evaluation made by the scores of
Wexner, BFS and GIQLI).
We sum up in Table 8 the functional outcomes
occurring after surgery in 229 adult HD patients
published in the English literature up to 2005.
It is clear that the Duhamel procedure is the most
practiced by surgeons all over the world, that it is
associated with the lowest rate of postoperative
complications and at the same time with the best long-
term outcomes.
CONCLUSION
Adult Hirschsprung’s disease is rare but by no means
exceptional. It should be considered in every young
patient presenting with a history of long-standing
constipation. Diagnosis is first of all clinical. Barium
enema radiographs may be pathognomonic and spare
the patient further investigations to confirm the
diagnosis Recto-anal inhibitory reflex is usually absent
on manometry.
Primary treatment of adult HD is surgical. The choice of
the procedure depends not only on morbid-mortality
and on its functional outcome, but also on the habits
and experience of the team of surgeons and other care
givers.
Even though the Duhamel procedure is the most
practiced in the world for the treatment of HD in adults,
we believe after this study that colo-anal resection with

9. Hirschsprung’s disease in adults: Clinical and therapeutic features
Int. Res. J Surg. 016
coloanal anastomosis gives in the long-run satisfactory
and even excellent results with no mortality and a low
morbidity rate, provided that the operation is performed
by an experienced team.
AUTHORSHIP
Conception and design of study: Zaafouri H, Ben
Maamer A
Acquisition of data: Mrad S, Mabrouk M
Data analysis and or interpretation: Haoues N, Bel Hadj
Salah M
Drafting of manuscript: Zaafouri H and Noomene R
Approval of final version of manuscript: Bouhafa A and
Cherif A
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Accepted 19 February, 2015.
Citation: Zaafouri H, Mrad S, Mabrouk M, Haoues N,
Salah MBH, Noomene R, Ahmed Bouhafa, Anis Ben
Maamer and Abderraouf Cherif (2015). Hirschsprung’s
disease in adults: Clinical and therapeutic features.
International Research Journal of Surgery, 2(1): 009-
017.
Copyright: © 2015 Zaafouri et al. This is an open-
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