Organised description about hirschprung's disease

1. Dr. Punjan Bikram Thapa
Resident
Radiology and Imaging
Phase – B
BSMMU

2. months
-abdominal distension for 1
weeks
-Vomitting since 3 days
-Failure to thrive
OE :
GC- fair, Vitals : normal
Abdomen: distended , tender

6. Introduction:
Affects approximately 1:5000-
8000 live births
Accounts for 15-20% of cases of
neonatal bowel obstruction
80 % present with first 6
weeks in life.

7. Embroyology:
caudal direction.
These cells reach the rectum by
12 weeks and commence the
intramural migration from
Auerbach’s ( myenteric ) plexsus
to the submucosal plexsus.
Hirschprung’s disease:
is caused by abnormal neural

8. Clinical Presentation:
present with abdominal
distension, bilious vomiting or
enterocolitis
Associated with:
 intestinal atresia, malrotation
Down’syndrome

9. Classification:
short segment disease: ~75%
rectal and distal sigmoid colonic involvement only
long segment: ~15%
typically extends to splenic flexure/ transverse colon
total colonic
aganglionosis: ~7.5% (range 2-
13%)
also known as Zuezler- Wilson syndrome
occasional extension of aganglionosis into the small bowel
ultrashort segment disease
3-4 cm of internal anal sphincter only
controversial entity

10. Imaging Modalities:
Plain radiograpy
Fluroscopy
Ultrasound
CT Scan
Biopsy
Rectal Manometry

11. Radiographic Features:
can determined
to distal bowel
obstruction.
An empty
rectum is
common finding
Classic finding is
a dilated proximal
colon with the
aganglionic cone
Plain X-ray abdomen showing a
dilated proximal sigmoid
colon with a smaller distal sigmoid
with relatively little rectal gas in a
neonate with Hirschsprung’s disease

12. Fluroscopy:
caliber occur, with
the dilated, normal
colon above and
the narrowed,
aganglionic colon
below
Abnormal ,
irregular
contraction of
aganglionic
segment. Barium enema showing reduced caliber of
the rectum followed by a transition zone to
an enlarged caliber sigmoid.

13. The diagram (lateral view) depicts the varied appearance of the
transition zone in the rectum in Hirschsprung’s disease.
A. In the very young, the transition zone may be cone shaped,
with the caliber imperceptibly decreasing as it goes
from the sigmoid colon to the rectum.
B and C. A discrete change in caliber is more typical, with the
radiologic transition zone more clearly defined in C.

14. Hirsprung ‘s disease.
Barium enema showing
transition in mid
descending colon
Barium enema showing
reduced caliber and length
of large bowel, with no clear
transition zone
-Total colonic aganglionosis
A reduced caliber
rectum and dilated
large –bowel loops
with and irregular
mucosal contour
(dyskinesia)

15. diagnose HD.
• compares ratio of
rectal diameter and
is considered
abnormal, if the
sigmoid colon is
more dilated than
the rectum
•R/S index< 1 – in
Hirschprung’s
Preoperative BEs in patients with
TCA.
a Ileocecal valve reflux, reflux of
contrast material into the terminal
ileum (arrow).
b Question-mark-shape colon,
described as a rounded and shortened
contour of hepatic and splenic flexures
with short appearing colon (arrows)

16. Ultrasound :
Dilated loops of
colon with
obstruction is
usually not
detected prior to
25 weeks
gestation
 help in
determining
dynamic and a
Prenatal ultrasound finding of a
fetus with Hirschsprung’s disease
at 34 weeks.
A dilated bowel measuring 2 cm in
diameter (star) is followed by a
narrowing bowel (arrowheads).

17. USG shows
-dilated loops of colon (peripheral location, lack of peristalsis, haustral markings or pelvic
location)
-Increased abdominal circumference
Polyhydramnios.

18. and the transition
zones but also to
definitively
exclude other
diseases which
can also
cause chronic
constipation.
Preoperative
planning of
CT scan in sagittal
reformatted images shows
ultrashort transition zone
(red arrow) (TZ length was
1 mm, and mean TZ ratio
was 1.2)atio was 1.2).

19. 2 years old male infant presented with constipation.
Scout anteroposterior image of abdomen showed abdominal gaseous
distension.
MDCT in sagittal reformatted shows long segment TZ
(above sigmoid colon) (red arrow).
MDCT in oblique coronal and axial images showed the high level of
transition zone (red arrow) (TZ length was 9 mm, and mean TZ ratio was
2.7).

20. cells are present
Performed by rectal suction
biopsy or full thickness biopsy
Must be taken above anal verge
Rectal Manometry:
In patient with normal barium

21. Differential diagnosis
neonatal bowel wall to bacteria
•usually develops 2-3 days
following birth
•incidence is ~1 in 1000 births
Clinical presentation:
poor feeding
bile-stained vomitus

22. normal polygonal
gas shape
bowel wall
edema
with thumb
printing
pneumatosis
intestinalis
.
The bubbly appearance of the abdomen is
caused by air within the bowel wall.
In some segments, the intramural air
clearly parallels the lumen; in other
segments, it is seen as a circular pattern

23. the vascular
state
-hypervascular (viable but engorged
in early stage)
-hypovascular (infarcted in a later
stage)
intramural gas
manifesting as
hyperechoic
foci within the
bowel wall
Transverse abdominal sonogram of a 9-day-
old preterm baby shows intramural air in the
descending colon (box and magnified image
on the right) and peritoneal free air (arrows)
suggesting bowel perforation.
Transverse sonogram of the liver of a 4-day old
preterm baby shows multiple hyperechoic foci
(arrows), some with posterior acoustic shadowing,
consistent with portal venous gas.

24. Water-soluble enema depicts an area of
minor narrowing in the
mid transverse colon (straight arrow) and a
more severely narrowed
segment at the hepatic flexure (curved
arrows)- Colonic stricture from necrotizing
enterocolitis.
A large amount of free peritoneal air
outlines the falciform ligament (black
arrows). The air has also given the entire
right upper quadrant an unusual lucent
appearance. The inner and
outer walls (white arrows) of multiple
bowel loops are visible, another sign of
free peritoneal air
-Necrotizing enterocolitis with
perforation: the football sign.

25. Differential Diagnosis:
sudden painless enlargement of
the proximal colon accompanied
by distension.
Related to decreased
parasympathetic activity
 Risk factor :Trauma, post-
surgery, infection, electrolyte
imbalance

26. Fluroscopy :
A single
contrast/water-
soluble enema
demonstrates the
absence of any
mechanical
obstruction
CT – scan:
Xray shows distension is
pancolonic and extends to
the rectum, suggestive of
acute colonic
pseudoobstruction

27. FigA,B—31-year-old woman, Axial and coronal contrast-enhanced CT images show
markedly distended transverse colon with large amount of fecal material. Transitional
zone (arrow,) is at splenic flexure and has no obstructive lesions.
FigC —71-year-old woman with
Axial contrast-enhanced CT image
obtained when colitis was present
shows relatively long segmental
wall thickening with mucosal fold
thickening in transverse colon.
Increased vascularity (arrows) of
marginal vessels is evident.

28. mental retardation,
HD, or cystic
fibrosis; or
aerophagia.
usually between 7
and 10 years
abdominal pain
vomiting
accompanying
Abdominal
Transverse color
Doppler sonogram of an epigastric
mass shows the whirlpool sign
consisting of the SMA (arrow) and
SMV (arrowhead ) twisted and
draped clockwise around the
mesentery
Differential Diagnosis

29. include:
coffee bean
sign
Frimann-Dahl
sign - three
dense lines
converge
towards the site
of obstruction

30. lacking haustra forming a closed-
loop obstruction
whirl sign: twisting of the
mesentery and mesenteric
vessels
bird beak sign: if rectal contrast
has been administered
X-marks-the-spot sign: crossing
loops of bowel

31. Fig: 3D VR colonscopy RA RP and transparency views shows
marked distension of the sigmoid colon with its apex directed to the
right upper quadrant of the abdomen with smooth tapering and beaking
of its ends forming a closed loop and giving the coffee bean
appearance.
Bird's beak appearance of the rectum at the site of the sigmoid twist is
noted.

32. Treatment for HD:
Adminstration of isotonic enema
Adminstration of stool softner
Low residue diet
Surgical :
The surgery involves two steps
1.Temporary Colostomy
2.Definitive Surgery : after about
year colostomy is closed
A.Sweson

33. • THANK YOU FOR
PATIENCE