Case Report of a Skip Segment Hirschsprung’s Disease: A Real Phenomenon

Abstract

Introduction and importance
Hirschsprung’s disease is a congenital anomaly that results from an incomplete craniocaudal migration and maturation of intestinal ganglion progenitor cells leading to distal intestinal aganglionosis. Skip segment Hirschsprung’s disease is an extremely rare phenomenon. We report a case involving only the small bowel with confirmed colonic ganglionosis.

Case presentation
A case report of a 14-month-old with a skipped segment involving the distal 50 cm of the small bowel associated with colonic ganglionosis is presented. A current review of the literature is discussed.

Clinical discussion
Our patient had persistent obstructive symptoms despite undergoing a technically good, ganglionic pull-through operation at an outside institution. A laparoscopic-assisted pull-through might have documented a small bowel wall diameter discrepancy.

Conclusion
Although rare, skip segment Hirschsprung’s disease is a real phenomenon that paediatric surgeons should be aware of and could involve small and large bowels.

Full text

CASE
REPORT
–
OPEN
ACCESS
International
Journal
of
Surgery
Case
Reports
80
(2021)
105630
Contents
lists
available
at
ScienceDirect
International
Journal
of
Surgery
Case
Reports
jou
rn
al
homepage:
www.casereports.com
Case
report
of
a
skip
segment
Hirschsprung’s
disease:
A
real
phenomenon
Yousef
El-Goharya,
Clare
Skerrittb,
Vinay
Prasadc,
Ihab
Halaweishd,
Richard
J.
Woodd,∗
aDepartment
of
Paediatric
Surgery,
Leeds
Children’s
Hospital,
Leeds,
UK
bDepartment
of
Paediatric
Surgery,
Bristol
Royal
Hospital
for
Children,
Bristol,
UK
cDepartment
of
Pathology
and
Laboratory
Medicine,
Nationwide
Children’s
Hospital,
Columbus,
OH,
USA
dDepartment
of
Pediatric
Colorectal
and
Pelvic
Reconstructive
Surgery,
Nationwide
Children’s
Hospital,
700
Children’s
Drive,
Columbus,
OH,
USA
a
r
t
i
c
l
e
i
n
f
o
Article
history:
Received
16
December
2020
Received
in
revised
form
4
February
2021
Accepted
4
February
2021
Available
online
9
February
2021
Keywords:
Hirschsprung’s
disease
Skip
segment
Aganglionosis
Case
report
a
b
s
t
r
a
c
t
INTRODUCTION
AND
IMPORTANCE:
Hirschsprung’s
disease
is
a
congenital
anomaly
that
results
from
an
incomplete
craniocaudal
migration
and
maturation
of
intestinal
ganglion
progenitor
cells
leading
to
distal
intestinal
aganglionosis.
Skip
segment
Hirschsprung’s
disease
is
an
extremely
rare
phenomenon.
We
report
a
case
involving
only
the
small
bowel
with
confirmed
colonic
ganglionosis.
CASE
PRESENTATION:
A
case
report
of
a
14-month-old
with
a
skipped
segment
involving
the
distal
50
cm
of
the
small
bowel
associated
with
colonic
ganglionosis
is
presented.
A
current
review
of
the
literature
is
discussed.
CLINICAL
DISCUSSION:
Our
patient
had
persistent
obstructive
symptoms
despite
undergoing
a
technically
good,
ganglionic
pull-through
operation
at
an
outside
institution.
A
laparoscopic-assisted
pull-through
might
have
documented
a
small
bowel
wall
diameter
discrepancy.
CONCLUSION:
Although
rare,
skip
segment
Hirschsprung’s
disease
is
a
real
phenomenon
that
paediatric
surgeons
should
be
aware
of
and
could
involve
small
and
large
bowels.
©
2021
The
Authors.
Published
by
Elsevier
Ltd
on
behalf
of
IJS
Publishing
Group
Ltd.
This
is
an
open
access
article
under
the
CC
BY-NC-ND
license
(http://creativecommons.org/licenses/by-nc-nd/4.0/).
1.
Introduction
Hirschsprung’s
disease
is
a
congenital
anomaly
that
manifests
as
functional
intestinal
obstruction
due
to
the
failure
of
cranio-
caudal
migration
and
maturation
of
intestinal
ganglion
progenitor
cells,
leading
to
distal
intestinal
aganglionosis
[1].
Depending
on
the
level
of
arrested
caudal
migration,
a
patient
may
manifest
with
a
short
segment,
long
segment,
total
colonic,
or
total
intestinal
aganglionosis.
In
all
cases,
the
congenital
absence
of
ganglion
cells
starts
in
the
distal
rectum
and
extends
proximally.
A
rare
entity
has
been
described
in
the
literature
of
patients
with
Hirschsprung’s
disease
presenting
with
a
skipped
segment
[2].
This
goes
against
the
embryological
theory
of
arrested
craniocaudal
migration
of
neural
crest-derived
cells.
O’Donnell
et
al.
described
in
their
sys-
tematic
review
24
cases
of
segmental
Hirschsprung’s
disease,
all
of
which
involved
the
colon
[2].
We
describe
a
rare
case
of
segmental
Hirschsprung’s
disease
involving
the
distal
50
cm
of
small
bowel
with
confirmed
colonic
ganglionosis.
∗Corresponding
author
at:
Department
of
Pediatric
Colorectal
and
Pelvic
Recon-
structive
Surgery,
Nationwide
Children’s
Hospital,
611
E.
Livingston
Ave.,
Columbus,
OH,
43205,
USA.
E-mail
address:
richard.wood@nationwidechildrens.org
(R.J.
Wood).
2.
Presentation
of
case
A
14-month-old
boy
with
no
relevant
family
or
drug
history
underwent
a
trans-anal
only
Soave
pull-through
at
an
outside
insti-
tution
for
a
reported
short
segment
Hirschsprung’s
disease
when
he
was
two
weeks
of
age.
The
patient
was
doing
well
until
he
was
4-months
old
when
he
developed
an
episode
of
enterocoli-
tis.
Subsequently,
the
patient
had
eight
enterocolitis
episodes,
all
requiring
hospital
admissions
despite
undergoing
daily
rectal
irri-
gation
and
receiving
four
separate
internal
anal
sphincter
Botox
injections.
He
took
45
mg
of
Senna
daily;
however,
he
had
per-
sistent
obstructive
symptoms
and
abdominal
distention.
He
was
referred
to
our
centre
for
a
second
opinion
regarding
his
ongo-
ing
abdominal
distention
and
recurrent
enterocolitis.
A
scout
film
during
the
contrast
enema
revealed
several
dilated
small
bowel
loops
with
evidence
of
faecalization
concerning
for
partial
small
bowel
obstruction
(Fig.
1A).
However,
despite
evidence
of
small
bowel
dilatation
on
the
scout
imaging,
the
contrast
refluxed
into
non-dilated
terminal
ileum
suggesting
an
obstruction
caused
by
an
internal
hernia
more
proximally
(Fig.
2B).
The
next
day,
he
was
taken
to
the
operating
room
by
the
senior
author
for
a
presumed
internal
hernia
since
his
original
operation
was
a
trans-anal
only
approach.
An
examination
under
anesthesia
of
the
anus
revealed
an
intact
dentate
line
with
the
anastomosis
visible,
but
no
evi-
https://doi.org/10.1016/j.ijscr.2021.02.016
2210-2612/©
2021
The
Authors.
Published
by
Elsevier
Ltd
on
behalf
of
IJS
Publishing
Group
Ltd.
This
is
an
open
access
article
under
the
CC
BY-NC-ND
license
(http://
creativecommons.org/licenses/by-nc-nd/4.0/).

CASE
REPORT
–
OPEN
ACCESS
Y.
El-Gohary,
C.
Skerritt,
V.
Prasad
et
al.
International
Journal
of
Surgery
Case
Reports
80
(2021)
105630
Fig.
1.
(A)
Scout
film
with
several
dilated
small
bowel
loops
with
evidence
of
faecalization
concerning
for
partial
small
bowel
obstruction.
(B)
Contrast
enema
with
contrast
filling
normal
caliber
“question
mark-like”
colon
and
terminal
ileum.
Fig.
2.
Exploratory
laparotomy
revealing
aganglionic
distal
50
cm
of
the
small
bowel
(*)
decompressed
with
dilated
proximal
small
bowel
(+).
The
caecum
(x)
and
colon
(#)
were
all
ganglionic.
dence
of
a
stricture.
We
proceeded
with
a
diagnostic
laparoscopy
and
did
not
find
evidence
of
an
internal
hernia
to
account
for
the
dif-
fuse
proximal
small
bowel
dilation.
We
converted
to
an
exploratory
laparotomy
through
a
lower
midline
incision.
The
terminal
50
cm
of
small
bowel
was
decompressed
(Fig.
2*)
and
identified
a
tran-
sition
zone
proximally
to
a
more
dilated
small
bowel
without
any
clear
aetiology
(Fig.
2+).
Full-thickness
biopsies
were
initially
taken
from
the
terminal
ileum
(Fig.
2*)
and
caecum
(Fig.
2x).
Gan-
glion
cells
were
present
in
the
caecum,
but
the
terminal
ileum
was
aganglionic.
This
prompted
us
to
take
full-thickness
biop-
sies
at
10,
15,
20,
25,
45,
and
50
cm
from
the
ileocaecal
valve
(Fig.
2*).
All
were
aganglionic
except
for
the
dilated
small
bowel
measured
at
50
cm
from
the
ileocecal
valve
(Fig.
3).
Biopsies
of
the
hepatic
flexure,
descending
colon,
and
rectum
confirming
gan-
glionic
bowel
(Fig.
3).
The
patient
was
diverted
with
a
divided
ileostomy
and
mucous
fistula.
The
patient
was
then
reversed
six
months
later
with
the
aganglionic
bowel
resected
and
an
ileo-
colic
anastomosis
performed.
He
was
followed
in
the
surgical
outpatients
and
is
now
thriving
with
no
further
episodes
of
ente-
rocolitis.
3.
Discussion
We
present
a
segmental
Hirschsprung’s
disease
case
involving
only
the
small
bowel,
with
confirmed
colonic
ganglionosis.
To
the
best
of
our
knowledge,
there
have
been
40
reported
cases
of
skip
segment
Hirschsprung’s
disease
reported
in
the
literature,
the
vast
majority
of
which
involve
the
colon
(Table
1)
[2–6].
While
the
dis-
ease
is
believed
to
result
from
arrested
craniocaudal
migration
of
neural
crest-derived
cells,
these
cases
go
against
the
classic
embry-
ological
explanation.
In
O’Donnell
et
al.
systematic
review
of
24
cases
of
segmental
Hirschsprung’s
disease,
all
of
the
cases
involved
only
the
colon,
with
22
out
of
24
cases
being
total
colonic
agan-
glionosis
[2].
It
has
been
theorized
the
skip
lesion
results
from
extramural
migration
of
neuroblasts
across
the
mesenteric
bor-
der
and
into
the
colon,
thereby
ending
up
ahead
of
the
wavefront
2

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Y.
El-Gohary,
C.
Skerritt,
V.
Prasad
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105630
Fig.
3.
Haematoxylin
and
Eosin
staining
of
the
full-thickness
biopsies
of
different
parts
of
the
intestine.
3

CASE
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El-Gohary,
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V.
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105630
Table
1
Summary
of
all
cases
of
skip
segment
Hirschsprung’s
disease
reported
in
the
literature
from
1954
to
2020
[7–28].
TCA:
Total
colonic
aganglionosis;
TIA:
Total
intestinal
aganglionosis;
TI:
terminal
ileum;
DJF:
Duodenal
jejunal
flexure.
Reference
Year
Sex
Number
of
patients
Location
of
skip
segment
Keefer
1954
M
1
Rectosigmoid
with
skip
in
sigmoid
sprinz
1961
F
1
TCA
except
skip
in
transverse
colon
Mac
Iver
1972
M
1
Rectosigmoid
with
skip
in
sigmoid
Martin
1979
M
1
TCA
except
skip
in
transverse
colon
De
Chadarevian
1982
M
1
TCA
except
skip
in
transverse
colon
Yunis
1983
M
5
TCA
except
skip
in
transverse
colon
M
TCA
except
skip
in
transverse
colon
M
TCA
except
skip
in
transverse
colon
M
TCA
except
skip
in
transverse
colon
M
TCA
except
skip
in
ascending
colon
Taguchi
1983
M
1
TCA
except
skip
in
ascending
colon
Seldenrijk
1986
M
2
TCA
with
multiple
skips
F
TCA
with
multiple
skips
Anderson
1986
M
1
TCA
except
skip
in
ascending
colon
Kapur
1995
F
3
TCA
except
skip
in
ascending
colon
F
TCA
except
skip
in
caecum
F
TCA
except
skip
in
ascending
colon
Yang
2005
M
3
TCA
with
multiple
skips
M
TCA
with
multiple
skips
M
TCA
with
multiple
skips
Ziad
2006
M
2
TCA
except
skip
in
transverse
colon
F
TCA
except
skip
in
caecum
Oshio
2008
M
1
TCA
except
skip
in
ascending
colon
Puri
2010
M
1
TCA
except
skip
in
transverse
colon
castle
2011
M
1
Aganglionosis
terminal
8
cm
of
TI
and
TCA
except
skip
in
transverse
colon
and
caecum
Doi
2011
F
1
TCA
except
skip
in
transverse
colon
Burjonrappa
2012
M
1
TCA
except
skip
in
caecum
Skelly
2012
M
1
TIA
except
stomach,
10
and
45
cm
distal
to
DJF
moore 2013
M
2Skip
in
right
and
ascending
colon
FSkip
in
ascending
colon
and
appendix
Erten
2014
M
1
TCA
except
skip
in
ascending
colon
and
2
cm
aganglionic
ileum
6
cm
from
TI
RAGUNATH
2014
M
1
TCA
with
skip
in
ascending
colon
and
hepatic
flexure
Gross
2015
M
1
Skip
in
transverse
and
descending
colon
Ruiz
2016
M
1
TCA
except
skip
in
caecum
and
ascending
colon
COE
2016
M
2
Distal
rectal
skip
segment
Alfawaz
2017
M
1
Skip
in
proximal
sigmoid
Shenoy
2019
M
1
Distal
rectal
skip
segment
Yu 2019 M2Skip
in
sigmoid
M
Skip
in
transverse
colon
El-Gohary
2020
M
1
Aganglionosis
terminal
50
cm
of
TI
resulting
in
the
skip
segment
[2].
With
41
reported
cases
in
the
literature,
including
our
case,
skip
segment
Hirschsprung’s
disease
must
be
considered
a
real
phenomenon
(Table
1).
We
suspect
there
are
probably
several
unreported
cases
in
the
world
with
segmen-
tal
Hirschsprung’s
disease
that
paeditric
surgeons
have
dismissed
since
it
goes
against
the
dogma
of
arrested
craniocaudal
migra-
tion.
Our
case
is
unique
because
the
aganglionic
segment
involves
only
the
distal
small
bowel
with
confirmed
colonic
gangliono-
sis.
Since
the
original
pull-through
operation
was
done
via
a
trans-
anal
only
approach,
this
might
have
led
the
surgeon
to
miss
two
transition
points,
which
could
have
been
identified
laparoscopi-
cally.
We
recommend
against
a
trans-anal
only
approach
for
any
pull-through
procedure
to
document
the
transition
zone
and
offer
the
patient
the
correct
surgery
the
first
time.
This
case
illustrates
how
careful
mapping
of
bowel
via
multiple
biopsies
can
yield
the
maximal
amount
of
bowel
possible
for
a
pull-through
and
avoid
the
necessity
of
multiple
operations.
It
is
clear
from
the
41
cases
of
the
skip
segment
variant
of
Hirschsprung’s
disease
that
there
is
a
strong
male
predilection
with
an
almost
5:1
male-to-female
ratio
and
strong
preponder-
ance
in
total
colonic
aganglionosis.
However,
a
question
mark
remains
(Fig.
1B)
over
the
embryological
explanation
of
skip
seg-
ment
Hirschsprung’s
disease.
Until
further
studies
are
done,
it
will
remain
an
enigma.
4.
Conclusion
Trans-anal
only
pull-through
is
ill
advisable.
Although
rare,
skip
segment
Hirschsprung’s
disease
is
a
real
phenomenon
that
paedi-
atric
surgeons
should
be
aware
of
and
could
involve
small
and
large
bowels.
This
manuscript
was
written
in
line
with
the
SCARE
2020
Guide-
line
[29].
Declaration
of
Competing
Interest
The
authors
report
no
declarations
of
interest.
Funding
The
authors
declare
they
have
received
no
funding
for
the
prepa-
ration
of
this
document.
Ethical
approval
Ethical
approval
is
exempt
at
our
institution.
4

CASE
REPORT
–
OPEN
ACCESS
Y.
El-Gohary,
C.
Skerritt,
V.
Prasad
et
al.
International
Journal
of
Surgery
Case
Reports
80
(2021)
105630
Consent
Written
informed
consent
was
obtained
from
the
patient
for
publication
of
this
case
report
and
accompanying
images.
A
copy
of
the
written
consent
is
available
for
review
by
the
Editor-in-Chief
of
this
journal
on
request.
Authors’
contribution
Study
conception
and
design:
Richard
J
Wood.
Data
acquisition:
Yousef
El-Gohary,
Vinay
Shah.
Analysis
and
data
interpretation:
Yousef
El-Gohary,
Clare
Skerritt,
Vinay
Shah,
Ihab
Halaweish,
Richard
J
Wood.
Drafting
of
the
manuscript:
Yousef
El-Gohary,
Clare
Skerritt.
Critical
revision:
Clare
Skerritt,
Vinay
Shah,
Ihab
Halaweish,
Richard
J
Wood.
Registration
of
research
studies
Not
Applicable.
Guarantor
Richard
J
Wood.
Provenance
and
peer
review
Not
commissioned,
externally
peer-reviewed.
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http://dx.doi.org/10.1097/MOP.0b013e328360c2a0.
[2]
A.M.
O’Donnell,
P.
Puri,
Skip
segment
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disease:
a
systematic
review,
Pediatr.
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