Scandinavian Journal of Surgery 100: 243249, 2011
Modern Treatment of Hirschsprungs disease
A. Gunnarsdttir1, 2, 3, T. Wester1, 2
1 Section of Pediatric Surgery, Astrid Lindgren Childrens Hospital, Karolinska University Hospital,
Stockholm, Sweden
2 Department of Womens and Childrens Health, Karolinska Institutet, Stockholm, Sweden
3 Section of Pediatric Surgery, The Childrens Hospital, Landspitali, University Hospital, Reykavik, Iceland
Key words: Hirschsprungs disease; transanal endorectal pull-through; laparoscopically assisted
pull-through; constipation; soiling; enterocolitis; quality of life
Definition, history, and etiology
Hirschsprungs disease (HD) is a congenital gastro-
intestinal motility disorder characterized by an ab-
sence of neuronal cell bodies in both myenteric and
submucous plexuses in the intestinal wall (1). HD is
named after Harald Hirschsprung, a pediatrician at
Queen Louise Childrens Hospital in Copenhagen,
Denmark, who described 2 cases of children with
megacolon in 1887 (2). It was though not until the
beginning of the twentieth century that Tittel noted
the absence of ganglion cells in the distal colon of a
child with HD (3). This aganglionosis, pathogno-
monic for HD, always starts distally and is limited to
the rectum and the sigmoid colon in 8085% of the
cases. Less frequently, the aganglionosis involves the
entire colon with or without a part of the small intes-
tine. Functionally, this results in a sustained contrac-
tion of the aganglionic segment causing obstructive
symptoms. The etiology of HD is heterogeneous and
not fully understood. During embryonic develop-
ment, the neural crest cells migrate caudally where
they differentiate amongst other cell types to gangli-
onic cells of the enteric nervous system. Studies of
mice suggest that there is a delay or arrest in this
migration, which results in the neural crest cells fail-
ing to reach their correct positions in the distal intes-
tine (4, 5). Other studies have indicated that neural
crest cells fail to survive, proliferate, or differentiate
due to abnormalities within their microenvironment
after the migration has occurred (610).
Correspondence:
Tomas Wester M.D.
Department of Pediatric Surgery
Karolinska University Hospital
SE - 17176 Stockholm
Sweden
Email: tomas.wester@karolinska.se
HD occurs in about 1/5000 live born babies and is
more common in boys than girls (4:1) (11). This to-
gether with the fact that about 30% of patients with
HD have other abnormalities suggests a genetic etiol-
ogy for HD (11, 12). Different animal and human
studies have identified various gene defects in HD
(13). The most common mutations are located to the
RET proto-oncogene on chromosome 10, accounting
for approximately 20% of sporadic cases of HD and
about 50% of familial cases (1416).
Clinical presentation and diagnostic
work-up
The patients with HD are most often diagnosed in the
neonatal period (17). The clinical presentation is dis-
tended abdomen, delayed passage of meconium and
vomiting. Older children more often present with
chronic constipation, which usually starts already
during the breastfeeding period. Most children who
present later have short-segment aganglionosis. Ap-
proximately 10% of patients with HD present with
enterocolitis, with fever, abdominal distension, ab-
dominal pain, and in the most severe cases, septice-
mia. This may be a life-threatening condition (1).
Plain abdominal radiographs usually show dilated
bowel loops. Contrast enema is the preferred first
diagnostic procedure and shows a transition zone
between the normal (often dilated) and the narrow
aganglionic bowel in about 7090% of the cases (18,
19). For definite diagnosis a rectal biopsy is needed
for histological evaluation. The absence of ganglionic
cells in the myenteric and submucous plexuses and
the finding of hypertrophic nerve trunks are pathog-
nomonic for HD. This is attained traditionally with a
full-thickness biopsy or more commonly nowadays,
with a submucosal rectal suction biopsy. Anorectal
manometry may aid in the diagnosis where the
244 A. Gunnarsdttir, T. Wester
r ectoanal inhibitory reflex is absent in children with
HD (20).
Operative management
Transabdominal operations
Surgical management for HD aims at removing the
aganglionic bowel and reconstructing the intestinal
tract by bringing the normally innervated bowel
down to the anus while preserving normal sphincter
function. Swenson and Bill were the first to describe
an operation for HD by removing the aganglionic
bowel with a pull-through in 1948 (21). Traditionally,
Swensons procedure was done in a three-stage man-
ner with diverting colostomy prior to the reconstruc-
tive surgery. Duhamel, Soave, and Rehbein have also
described techniques for reconstruction of HD, mod-
ifications of which have been widely used (2124).
The surgical approach changed gradually from
three-stage procedures to one-stage pull-through
without colostomy in the 1980s. This has turned out
to be as favorable as the multistage procedures with
benefits for the patients and reduction in health
care costs due to shorter and fewer hospital stays
(25, 26).
There has been a continuous development over the
years of both operative techniques and diagnostic
tools used for HD, striving towards minimal invasive
procedures for the benefit of the patients, without
compromising the results and diagnostic accuracy on
the way. The term minimal invasive surgery is used
for any procedure that is less invasive for the patient
than traditional open surgery. It most often involves
laparoscopy, endoscopy or computer-assisted surgery
and usually implies less surgical trauma for the pa-
tient, faster recovery and shorter hospital stay. It also
includes superior cosmetic results for the patient. Be-
cause of the small size of our pediatric patients, com-
bined methods are often chosen, i.e. laparoscopic as-
sisted surgery. The development of these techniques
has changed the surgical management of HD consid-
Fig. 1a. Expose the anal canal. Fig. 1b. Incise the mucosa 5 mm
above the dentate line. Dissect be-
tween the submucosa and the cir-
cular muscle layer.
erably during the last decade and they are dominat-
ing the modern treatment of HD today.The main op-
erative approaches used today are the total transanal
endorectal pull-through (TERPT) (2729) and the
laparoscopic assisted pull-through (30, 31).
Total transanal endorectal pull-through
(TERPT)
The transanal endorectal pull-through is carried out
with a circumferential incision in the rectal mucosa
about 5 mm above the dentate line, to establish a
submucosal plane. The dissection is then carried out
in that plane for variable length leaving an agangli-
onic muscular cuff behind, minimizing the risk of
injury to pelvic structures. The original description of
the TERPT method included leaving an approxi-
mately 6 cm long muscular cuff, which was split with
a longitudinal myectomy in the posterior wall (27, 28,
32). During the last years, reports of TERPT opera-
tions using a shorter muscular cuff without myec-
tomy have been shown to be just as beneficial (3336).
After the appropriate length is achieved, the rectal
muscular wall is divided circumferentially and the
full thickness of rectum mobilized out through the
anus, dividing the small vascular pedicles along the
rectum and colon (Fig 1). A full thickness biopsy is
taken from macroscopically normal ganglionic colon
for frozen section to determine the resection level of
the colon before suturing the final anastomosis. Some
surgeons prefer to take biopsies from the sigmoid
colon through a small infra-umbilical incision to con-
firm the level of the transition zone before starting
the transanal dissection (37).
Since first described in 1998, the TERPT has be-
come widely used because of many advantages. It
does not require laparotomy or laparoscopy and is
especially useful for aganglionosis confined to the
rectosigmoid area (38). The risks of intra-abdominal
contamination and adhesion formation are minimized
and the procedure reduces the risk of damaging the
pelvic structures. It is also less expensive and usually
includes faster recovery time postoperatively. The
Fig. 1c. Cut the muscular layers Fig. 1d. Mobilize until normo-
once above the pelvis floor. Con- ganglionic colon is reached.
tinue the full-thickness dissection
to mobilize the rectum and sig-
moid colon.
 Modern treatment of Hirschsprungs disease
patients can be discharged from the hospital 23 days
after surgery. TERPT results in excellent cosmetic ap-
pearance of the abdominal wall with no visible scar-
ring without compromising the bowel function
(2729, 3235, 3841).
The TERPT method has, however, its limitations
and is mostly recommended for patients with agan-
glionosis limited to the rectosigmoid colon. With a
longer or undefined aganglionic segment, a combina-
tion of a laparoscopic assisted pull-through or an
infra-umbilical mini-laparotomy for segmental biopsy
is recommended (36, 41, 42).
Laparoscopic assisted pull-through
The first report of primary laparoscopic-assisted en-
dorectal pull-through for HD was published by
Georgeson et al in 1995 (30). The procedure is done
by inserting a 45 mm 30 scope in the right upper
quadrant just below the liver edge after gaining a 12
cm H2O pneumoperitoneum with a Varess needle in
the umbilicus. Two 45 mm trocars are then inserted,
one in the lower right quadrant and one in the left
upper quadrant of the abdomen. Sometimes an ad-
ditional trocar is needed supra-pubically for better
traction of the colon during the laparoscopic dissec-
tion of the rectum (30). After full mobilization of the
aganglionic colon and rectum the procedure contin-
ues from the perineum with transanal dissection of
the rectal mucosa in a similar way as described above
for the TERPT method. The main advantages of the
laparoscopic approach are the possibility to take the
seromuscular biopsies for early identification of the
ganglionic normal colon. It also provides better mo-
bilization and dissection of the distal aganglionic
colon and rectum under direct vision, thereby mini-
mizing the transanal dissection. This might be an
important factor as it minimizes the dilatation of the
anal canal often needed in the TERPT procedure (30,
42). The laparoscopic approach has, as the TERPT
method, shown faster recovery time with shorter
hospital stay compared to the open methods. It also
has shown better cosmetic results, less perioperative
complications and superior functional results
(4244).
Functional outcome
Long-term obstructive symptoms, such as constipa-
tion, abdominal distension, and enterocolitis are com-
mon after procedures for HD. The most common
reasons for persistent obstruction are mechanical ob-
struction, recurrent or residual aganglionosis, motil-
ity disorders of proximal bowel, internal sphincter
achalasia, and functional stool-holding behavior (45).
The patient with severe obstructive symptoms should
be evaluated with clinical examination to rule out
stricture, rectal suction biopsies to exclude residual
aganglionosis, and contrast enema to assess dilata-
tion of the proximal colon (45). Postoperative entero-
colitis has been reported to occur in 042% of the
patients after one-stage pull-through procedures (46,
245
47). The higher incidence reflects a wider definition
of Hirschsprung-associated enterocolitis, but also an
increased awareness of the condition. Soiling has
been a concern in the discussion of the TERPT be-
cause stretching of the sphincters during the proce-
dure may potentially result in permanent sphincter
injuries. However, studies have shown that the mano-
metric sphincter pressures are similar after transanal
and transabdominal surgery and also that the mano-
metric findings are similar before and after a trans
anal procedure (48, 49). Another concern that has
been raised is that the anal canal may be damaged if
the mucosal incision is done too low, injuring the
dentate line. This could impair discrimination be-
tween gas, fluid and solid stools and result in soiling
after transanal pull-through (50).
The overall outcome in patients with HD appears
to be similar after transabdominal procedures, lap-
aroscopically assisted pull-through, and TERPT.
There is a lack of both adequately controlled long-
term follow up studies and prospective controlled
studies. In adolescence and adulthood many studies
have shown that the bowel function is impaired to a
variable degree (5155).The bowel function has been
considered to improve with time after open Duhamel
procedure (56).On the other hand, more recently, it
was shown that the bowel function deteriorates with
increasing age (53).
After laparoscopically assisted endorectal pull-
through with a transanal dissection, Georgeson
showed a low incidence of early complications (42).
Most patients were too young to assess continence,
but 18 of 20 older patients were reported to be conti-
nent. Langer et al reported 141 patients who had un-
dergone transanal one-stage pull-through with few
early complications (39). Eighty-one of those patients
had normal bowel function at follow up. Elhalaby et
al. reported the outcome in 149 patients after trans
anal pull-through and found perianal excoriations in
almost one third of the patients (32). Six of the pa-
tients had recurrent constipation and 7 of 42 patients
older than 3 years had soiling. Comparing transab-
dominal Soave Boley procedure with laparoscopically
assisted pull-through with a transanal dissection,
Mattioli et al showed that the complication rate was
similar between both groups, although the laparo-
scopically operated group had shorter hospital stay
(57). None of the patients older than 4 years of age
had fecal incontinence. Several studies have com-
pared total TERPT with transabdominal procedures
and found similar complication rate and short-term
functional outcome between both groups, although
the hospital stay was shorter after TERPT (35, 41, 58).
On the other hand, El-Sawaf et al reported better con-
tinence score for open transabdominal endorectal
pull-through than for TERPT, although the stool pat-
tern and enterocolitis score was better in the TERPT
group (59).
Comparing laparoscopically assisted and total
TERPT, both done with transanal endorectal dissec-
tion, Stensrud et al found similar high incidence of
soiling in both groups (60). Fifty-four percent of the
patients in the transanal group and 58% of patients
in the laparoscopically assisted group reported soil-
246 A. Gunnarsdttir, T. Wester
ing at follow up at the median age of 5.7 and 10.1
years respectively (60).
In a multicenter study from five large North Amer-
ican institutions patients operated with transanal dis-
section (total transanal or laparoscopically assisted)
and transabdominal technique (open or laparoscopic)
were compared. The transabdominal approach re-
sulted in more complications than the transanal tech-
niques. Nineteen percent of the patients in the trans-
abdominal group had more than one late complica-
tion compared to 4 % of patients in the transanal
group. The transabdominal group had more episodes
of enterocolitis and greater number of daily bowel
movements. However, there were no differences be-
tween both groups for continence and stooling pat-
tern scores (61).
Botulinum toxin injection
Mild obstructive symptoms can often be managed by
dietary measures, laxatives or enemas. Occasionally
the symptoms are more severe with recurrent bouts
of enterocolitis and repeated admissions to the hos-
pital. Some children need rectal stimulation or irriga-
tion to empty stools. If investigations do not reveal
any plausible reasons for the obstruction, the symp-
toms are often caused by internal sphincter achalasia,
which could be an indication for intra-sphincteric in-
jection of botulinum toxin. This method was first de-
scribed by Langer et al in 1997 (62). Botulinum toxin
A is injected into the internal sphincter under general
anesthesia. The administered doses vary widely, from
15 to 120 units, between different reports (63, 64). The
injection of botulinum toxin frequently has to be re-
peated, usually after 34 months. An early prospec-
tive study showed excellent results (63). The outcome
has been less favorable in other studies (65). A recent
study reported that 80% of the patients responded to
the first injection but 69% required a second injection.
The number of admissions to hospital for obstructive
symptoms decreased significantly (64). Chumpitazi
et al reviewed a group of patients referred for defeca-
tion disorders after surgery for HD. Sixty-nine per-
cent of their patients received botulinum toxin injec-
tions. A short-term response was shown in 89.2% of
the patients (66).
Myectomy
In patients who do respond to botulinum toxin, but
do not want to continue with repeated injections, my-
ectomy is an option. Wildhaber et al reported the
outcome after posterior myectomy or myotomy in 32
patients with obstructive symptoms after pull-
through. The operation was performed at a mean
time of 3.1 years after the pull-through and the aver-
age time to follow up was 8.6 years. The response rate
depended on the indication to do the myectomy. Sev-
enty-five percent of the patients with recurrent en-
terocolitis became symptom-free and 60% of patients
with chronic constipation responded to the myec-
tomy/myotomy. On the other hand, only 17 % of
those with residual aganglionosis and constipation
improved (67).
Redo pull-through
Rarely patients with persistent obstructive symp-
toms, enterocolitis or soiling may benefit from a re-
peat pull-through procedure. The indications are
residual or acquired aganglionosis, severe strictures,
and dysfunctional, sometimes severely dilated, prox-
imal bowel. Conservative measures should have been
attempted before considering a redo pull-through.
The patients should undergo investigations with rec-
tal biopsies and contrast enema. Different techniques
have been proposed for the redo procedure depend-
ing on the previous operation the patient had and
also the preference of the surgeon. Langer reported
9 cases, 8 of who had a Duhamel procedure (68).
Three of the patients had a normal stool pattern at
follow up, whereas four had persistent obstructive
symptoms, one had soiling, and one a stool-holding
behavior. Teitelbaum et al. reported the experience
with 26 redo pull-through procedures (69). Fourteen
patients were operated on with an endorectal pull-
through, 9 with a Duhamel, and 3 with a Swenson
procedure. At follow up all neurologically intact pa-
tients older than 3 years of age were continent, ex-
cept for two who had daily soiling. Redo pull-
through operations may be technically very difficult
and demanding due to scarring after previous op-
erations or pelvic sepsis. However, in carefully se-
lected cases, the reports indicate that the outcome
can be good or excellent (68, 69).
Quality of life
Quality of life (QoL) is difficult to measure with no
real consensus regarding a gold standard definition
of QoL. There are many factors affecting individual
QoL and it has become an important endpoint in
evaluating results of medical treatment for chronic
diseases. Most questionnaires for QoL rely on self
judgment of health and are dependent on different
internal and external factors such as employment,
education, leisure time, social belonging, physical
and mental health which differ greatly and are valued
differently between individuals.
Measurements of QoL should be multi-dimensional
and include at least three broad domains that can be
affected by ones disease or treatment, including
physical, mental and social functioning (70). One en-
counters problem when valuing different studies re-
garding QoL for patients with HD in the heterogene-
ity of the questionnaires used (70). It is therefore im-
perative to use validated questionnaires when esti-
mating QoL for comparison of results. For adults, the
SF-36 health survey is a well established generic
health survey and has been used in several studies
for adults with HD which found that general QoL of
adults operated for HD in childhood were overall
good (7072). Hartman et al showed that adults oper-
ated for HD scored significantly lower on the physi-
cal health scale (PCS) compared to control group,
whereas Gunnarsdttir et al found that adults with
HD scored higher than expected for role-physical
function (RP) and PCS; this was found particularly
 Modern treatment of Hirschsprungs disease
noted in men, who also had higher mean scores for
physical function (PF) and bodily pain (BP) than ex-
pected. This supports Hartmans conclusion that it is
not the physical symptoms that mostly affect the QoL
of adult HD patients (71). Gender subdivision, how-
ever, showed that the QoL outcome for women with
HD was poorer than for men in all subgroups and
women scored significantly lower in general- (GH)
and mental health (MH) compared to the control
group (71, 72).
Most follow-up studies after pull-through opera-
tions for HD focus on functional results and early
complications after the pull-through operation in
children with HD are well known as previously
shown (1, 73, 74). Follow-up studies for HD are gen-
erally limited to 515 years postoperatively and indi-
cate an improvement in bowel symptoms by the time
the children reach adolescence and this improvement
up to adolescence has also been noted in the general
QoL of patients with HD (52, 7378).
Bowel function alone is though not necessarily a
good indicator of QoL for adult individuals with HD
(52, 79, 80). On the contrary, Hartman et al showed in
their study of 320 patients operated on for anorectal
malformations and HD that generic QoL is particu-
larly affected by psychosocial function but not phys-
ical symptoms, such as fecal incontinence and consti-
pation (71). In children with HD, however, Mills et al
found that fecal continence was an important predic-
tor of overall QoL (78).
When looking at disease-specific functioning and
QoL the diversity of questionnaires used varies
greatly between studies and between different age
groups. Gastrointestinal Quality of Life Index (GIQLI)
is a 36-item instrument concerning gastrointestinal
disease-related symptoms, physical status and psy-
chosocial dysfunction in adults and has been gaining
popularity for usage in adults born with congenital
diseases in the gastrointestinal tract (53, 72, 81). These
studies show that adults operated for HD have no
significant difference between the patient group and
the age- and gender-matched control group for the
total GIQLI score (53, 72). They, however, differed in
subgroups focusing on large bowel function with in-
creased problem related to fecal soiling, constipation
and social problems related to bowel function (53,
72).
In summary, from the available studies it seems
that bowel function is better in adolescence than in
younger children but adolescents tend to report more
psychosocial QoL problems (70). Adults operated for
HD have more functional problems with bowel move-
ment compared to healthy control groups without
affecting their general QoL, possibly indicating that
most of the operated patients had managed to cope
with these symptoms as adults.
Management of total colonic
aganglionosis
Total colonic aganglionosis (TCA) occurs in 215% of
patients with aganglionosis. The entire colon is agan-
glionic and various lengths of the small bowel may
247
also be involved. TCA has been described to differ
clinically, radiologically, and histologically from rec-
tosigmoid Hirschsprungs disease, which may lead
to misdiagnosis (82).
TCA has been associated with a higher mortality
and morbidity than short segment disease. Several
surgical methods have been proposed for TCA, such
as the Martin-Duhamel procedure, Duhamel proce-
dure, Swensons procedure, and endorectal pull-
through with and without a J-pouch. A recent system-
atic review concluded that no single method is supe-
rior to the others with respect to mortality, morbidity,
enterocolitis or functional outcome. An increased
morbidity was shown in those who underwent a
Martin-Duhamel operation using the descending co-
lon (83). The Martin-Duhamel procedure has previ-
ously been associated with a 75% incidence of entero-
colitis (84).
Early complications include anastomotic leakage,
anal strictures, and perineal excoriations. Late com-
plications are frequent bowel movements, inconti-
nence, soiling, and recurrent enterocolitis. A few re-
cent studies have reported enterocolitis to occur post-
operatively in as many as approximately 55% of the
patients (85, 86). In their meta-analysis, Marquez et
al showed that the mean incidence of enterocolitis
was 22% (only seven of the studies reported the inci-
dence of enterocolitis) (83). Occasional patients need
anal dilatations, myectomy, or botulinum toxin injec-
tions to treat recurrent enterocolitis (86).Soiling is
very common and occurs in 3848% of the cases (86,
87). Fecal incontinence is also common, particularly
during night-time, and has been reported in one third
of the patients (87). A considerable number of pa-
tients end up with a permanent ileostomy (84, 86).
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Received: September 22, 2011