The document discusses the problematic Soave cuff in Hirschsprung's disease. It describes how the residual muscular cuff from the Soave procedure can cause obstructive symptoms like constipation or enterocolitis. The study examined 17 patients who underwent redo surgery for symptoms caused by an obstructing Soave cuff. All patients had transanal dissection and excision of the cuff, with some also having resection of proximally dilated colon. After surgery, patients' obstructive symptoms improved significantly.

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Jurnal reading
The Problematic Soave Cuff in Hirschsprung
Disease : Manifestations and treatment
Belinda H. Dickie, Keith M. Webb, Balgopal Eradi, Marc A. Levitt
Colorectal Center for Children Cincinnati Children's Hospital Medical Center, 3333 Burnet Avenue ML 20
23, Cincinnati, OH 45229, U
Journal of Pediatric Surgery 49 (2014) 77–81
Azis Aimaduddin AI
Pembimbing : dr. Suwardi. SpB, SpBA

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Introduction
• Hirschsprung’s disease (HD) is
characterised by an absence of ganglio
n cells in the distal bowel and extendi
ng proximally for varying distances.
• The absence of ganglion cells has been
attributed to failure of migration of ne
ural crest cells.

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1. abdominal distention
2. 95% - failure to pass meconium in first 24 h
ours life
3. Bilious vomiting
• Older children - constipation, failure to thrive
• 10-15% - severe diarrhea alternating w/ con
stipation—enterocolitis of Hirschsprung’s dise
ase

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Surgical operation
• Swenson Procedure (1948)
• Duhamel Procedure (1960)
• Soave Procedure (1963)
• Rehbein
• transanal technique
• the addition of laparoscopy to all of th
ese procedures

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SOAVE PROCEDUR
The Soave procedure was developed specifically
to protect The surrounding structures, outside
of the rectal wall, from damage, by
performing an endorectal dissection, leaving a
muscular cuff behind.
The procedure was initially performed
transabdominally, but was
later adapted to a transanal approach

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SOAVE PROCEDUR

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In Soave procedure, we have identified a group of
children who have recurrent
obstructive symptoms. manifested by
- recurrent enterocolitis,
- Severe Constipation
-overflow incontinence
The reason to explain
these symptoms, is an obstructing muscular cuff . This
is defined by the rectal cuff from the residual muscular
sleeve causing a narrowing around the pull through
and subsequently a functional obstruction.

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METHODS
Inclusi
- Soave cuff problem only
- with or without proximally dilated
Colon
Exclusi
- anastamotic stricture,
- transition zone
- aganglionic segment
- pathological or anatomical reason

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36 patients identified from our database who
presented to our Center with symptoms of obstruction after
an initial
Soave pullthrough in whom we performed a redo procedure.
17 of the patients had an obstructing Soave cuff as their
only reason for their symptoms (identified preoperatively by
clinical
exam, contrast enema and postoperative confirmation from
pathology).
19 other patients who had a Soave cuff plus an
additional anatomic or pathologic issue were excluded from
this analysis and have been described elsewhere

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PRESENTATION of PATIENT
recurent enterocolitis 10
severe constipation 6
overflow incontinence 2
chronic abdominal
distention 2
failure to trive 1
distal narrowing 1

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CONTRAS ENEMA
SOAVE Cuff 9
Prominent presacral
space (A) 4
Distal narrowing ( B ) 4
A B

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EXAMINATION
Palpable Cuff 14
Destroyed dentata 2
RECTAL BIOPSI
Normal biopsi dan no
hypertrophic nerve 17
Abnormal biopsi 2

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Redo surgery was offered to the patients because of symptoms
and findings on clinical exam. It was explained that the only
anatomic reason identifiable to account for the symptoms was
a problematic Soave cuff.
All patients underwent a transanal dissection around the
pullthrough colon. This plane defined the space between the
previous pullthrough segment and the previous retained
muscular cuff.
6 patients also had a laparotomy.
1 patient, because of an absent dentate line and concern for
continence pre and post-operativelyalso had a Malone
appendicostomy done as part of the procedure.
During the transanal dissection, the muscular cuff was
palpated, visualized, and excised.
4 of the patients had removal of the muscular cuff only.
The remaining patients had resection of
proximally dilated colon (mean length 7.2 cm, range of 3–30
cm).

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OPERATIF
POST
OPERATIF
Need irrigation for enterocolitis or
abdominal distension 10 3
voluntary bowel movements. 9 5
Severe constipation and impactions 6 NONE

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Discussion
• the Soave modification left a long aganglionic
muscular sleeve,which can extend from the p
eritoneal reflection down to the anal canal
• the residual sleeve, where the normal colon i
s pulled through, can remain in :
- contracted state
- a compressive action
- Influence peristalsis in the normal colon

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• Obstructive symptoms can occur in 11%–42%
of patients after undergoing a pullthrough pro
cedure for Hirschsprung disease.
• Residual Soave cuff as a culprit in the etiology
of the obstructive symptoms
• The Soave procedure have in fact modified
their technique to limit the amount of residual
aganglionic segment (the cuff) to 1–2 cm from
the beginning of their dissection ( “Soaveson”
)
• Soave cuffs will prevent the development of ob
structive symptoms. In our Center we use the
Swenson approach to avoid this problem

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• A rectal cuff on clinical exam or on a contrast
• enema should be suspected in the algorithm of
working up a patient who is not doing well follo
wing a Soave pullthrough
• Some of these patients may be the ones diagno
sed with internal sphincter achalasia and treate
d with Botox . Because the Botox temporarily
relieves the spastic muscular cuff, the patients
also experience temporary relief of their sympt
oms.
• The effectiveness of a posterior myectomy vari
es with the underlying symptoms. 60% of child
ren with chronic constipation andn 75% of child
ren with recurrent enterocolitis, following a pull
-through procedure had improved symptoms af
ter a myectomy.

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• 27 of the 32 patients had a previous Soave a
nd one could postulate that the myectomy is
breaking an obstructing Soave cuff. We avoid
this procedure in our practice, as the risk of
the myectomy is potential permanent injury t
o the sphincter resulting in fecal incontinence

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• The patient with a potential obstructing Soav
e cuff, if suspected, this can often be identifi
ed on clinical exam and contrast enema. We
do not routinely use manometry in our institu
tion, (anorectal or colonic in this situation)
but this could potentially be a useful adjunct
in the work up and follow-up after a redo pull
through.
• Our management, in these case is to proceed
with
- a redo transanal pullthrough
- remove the muscular cuff
- potentially any proximally dilated colon

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• Although the cuff may have been split at the original
surgery, fibrosis around the retained muscular cuff
forming an obstructing ring or rolling up of the cuff ma
y occur.
Dividing and removing the cuff relieve the distal
obstruction and resolve their symptoms.
In patients with an obstructing cuff and proximal dilat
ed colon, we feel that the dilation is due to the functio
nal obstruction caused by the muscular cuff.
Because the redo surgery in these cases includes
resection of the cuff and the proximally dilated colon,
improvement in symptoms could be due to
the removal of both anatomical issues.