Transcript Hirschsprung’s Disease Options for Surgical Correction

Hirschsprung’s Disease: Options
for Surgical Correction
George W. Holcomb, III, M.D., MBA
Children’s Mercy Hospital
Kansas City, Missouri
Hirschsprung’s Disease
Principles
• Barium enema for suspected disease
• Anorectal biopsy for confirmation of diagnosis
• Selection of operation based on extent of
disease – need biopsy of ganglionated colon
Hirschsprung’s Disease
Historical Approach
• Colostomy at diagnosis
• Pullthrough 6 - 12 months later
• Duhamel
• Soave
• Swenson
Hirschsprung’s Disease
Current Surgical Options
One-stage
Two-stage
Swenson
Swenson
Duhamel
Duhamel
Soave
Soave
Laparoscopic-assisted
(Laparoscopic-assisted)
Transanal
(Transanal)
Hirschsprung’s Disease
• One stage operation for rectosigmoid
disease is current preferred approach
• Laparoscopic-assisted or transanal
approach best suited for typical
rectosigmoid disease
• Unclear (in my mind) whether one or two
stage procedure is best for disease
proximal to splenic flexure
Hirschsprung’s Disease
• Initial transabdominal biopsy with
frozen section is
important in
determining what
surgical option is best
for each patient
• Biopsy can be
performed through
umbilicus easily
Hirschsprung’s Disease
Extent of Disease
Optimal Surgical Option
Rectosigmoid
Laparoscopic-assisted or
transanal
Descending colon
Laparoscopic-assisted
Transverse colon
Two-stage approach
Rt colon; ileum
Two-stage approach
Ileal pullthrough
(? Laparoscopic)
Hirschsprung’s Disease
Personal Approach
• Laparoscopic directed biopsy
thru umbilicus
• For rectosigmoid disease,
laparoscopic assisted
pullthrough
•
Tension free relocation of
ganglionic colon to anus
•
Already in abdomen for
laparoscopic directed biopsy
Patient Positioning
• Baby across O.R.
table
• Circumferential, full
body prep
• Urinary catheter
Port Positioning
Port placement
5 mm umbilical
5 mm RUQ
5 mm RLQ
3 mm (optional) LUQ
Technique
Ligation of mesenteric vessels
Technique
Mobilization of rectum
Technique
Mobilization of rectum
Technique
Mobilization of left colon
Laparoscopic Pullthrough
• Laparoscopic
mobilization of
recto-sigmoid
vasculature
• Extracorporeal
endorectral dissection
Transanal Dissection
Transanal Dissection
Transanal Dissection
Technique
Completed pullthrough
Laparoscopic Pullthrough
Primary Procedure - No Colostomy
Transanal Approach
• Best suited for low rectosigmoid
disease
• Concern is tension on
vasculature as it is brought to
anus
• Appropriate technique if
transabdominal biopsy is
performed to define extent of
disease
• If biopsy done, why not mobilize
laparoscopically?
• If no biopsy, must be ready to do
transverse colon or ileal
pullthrough in newborn period
Hirschsprung’s Disease
Primary Pullthrough
• Obviates need for colostomy care
• Second operation and hospitalization not
required
• Normal bowel function established early
• Reduced cost of care
Children’s Mercy Hospital
Results
2000 - 2001
15 Pts
Primary Lap
Pullthrough
Colostomy + Open
Pullthrough
Mean
Age (Wk)
Wt(kg)
7
36
(1 - 138)
6.41
(2.7 - 11.5)
8
27
(12 - 54)
7.24
(5.3 - 8.1)
# Pts
Children’s Mercy Hospital
Results
Total Postop
Hospitalization(D)
Primary Lap
Pullthrough
Colostomy +
Open Pullthrough
Total
Cost ($)
3.7
(3-7)
$38,489
(21,040 - 71,034)
3.4 + 3.5
(2-19) (2-4)
$70,858
(20,087 - 165,642)
Hirschsprung’s Disease
Primary Pullthrough
Open
+ transanal
Laparoscopy
+ transanal
Transanal
approach alone
• 1990’s
• Allows
biopsy
• Good
results
• Allows
biopsy
• Mobilization
of rectum,
sigmoid,
left colon
• Good early
results
• No ABD
exploration
•Pt. selection very
important
• Good early
results
Postoperative Care
• Biggest problem is enterocolitis
Postoperative Management
• Anorectal dilations
•
Begin 3rd week, Hegar 8 BID
•
Advance to Hegar 14 QD, then QOD, then
Q3D, up to Q week
•
Flagyl/anorectal irrigations if develop
enterocolitis
• Personal Experience (50 cases/7 yrs)
•
All well except 2 with colostomy (one closed)
Summary
• Functional results following pullthrough are
probably similar b/w approaches
• Patient selection important for primary
pullthrough regardless of approach
• Laparoscopic one stage approach affords good
results, has less total hospitalization and is less
costly than two stage approach
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