residual aganglionosis after pull-through operation

7/27/2019 Residual Aganglionosis After Pull-through Operation

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Residual aganglionosis after pull-through operationfor Hirschsprungs disease: a systematic review and

meta-analysis

Florian Friedmacher , Prem Puri

Pediatr Surg Int (2011) 27:10531057


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Introduction


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Pull-through (PT)operations forHirschsprungs

disease(HD) generally

have satisfactoryoutcome

Some continue to have

disturbances of bowelfunction after definitive

operation :

constipation,enterocolitis and

recurrentobstructivesymptoms .


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Majority with residual bowelproblems

managed by non-surgicaltreatment : laxatives,

enemas orintrasphincteric

botulinum toxin injection

Few patients with persistentabdominal distension,

constipation or enterocolitisdue to postoperativestricture or retainedaganglionic segment

require a redo PToperation


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Purpose of the study

meta-analysis designed to

determine incidence and

outcome of residualaganglionosis(RA) in patients withHD following PT operation


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Methods


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PubMed and MEDLINE databases

All studies that reported cases of patients withHD who had undergone redo PT operations for

RA or transition-zone bowel (TZB)

Between 1985 and 2011.


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Search terms

residualaganglionosis

transition-zone bowel

redo and

repeat pull-through

reoperation

Hirschsprungsdisease


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Additional cases

The reference lists fromretrieved articles were

reviewed

All published studies and

abstracts presented atvarious meetings evaluated.


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Only publications

containing all the relevantdetails were included in

the literature review


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Type of study

Gender

Recurrent bowel symptoms Histological findings on repeat rectal biopsy

Patients age at initial PT and redo PToperation

Type of surgical procedures performed

Postoperative follow-up with recurrentsymptoms.

Detailedinformation

recorded


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Publications not

giving adequateclinical data of

patients wereexcluded


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Results


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Between 1985 and 2011

29 publishedarticles

24 articles(82.8%) fromsingle centers

5 (17.2%) frommulticenter

studies


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555 patients with HDunderwent redo PT


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193 (34.8%)demonstrated abnormalhistological findings on

repeat rectal biopsy.

144 (74.6%) revealed RA 49 (25.4%) TZB


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Of these 193 patients

persistent abdominal distension/constipationn=135

recurrent episodes of enterocolitisn=45

Histological evidence of RA in the resectedproximal margin of the pulled-through bowel

n=13


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Patients gender

reported in 135(69.9%)

patients.

A male-to-female ratio of3.5:1 was observed


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Initial PT procedure

Documented in 143 (74.0%) patients

Soave procedure n=82

Duhamel procedure n=24 Rehbein procedure n=15

Swenson procedure n=14

Transanal endorectal PT (TERPT) n= 7

Posterior sagittal approach n= 1

50 patients not reported.


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Redo PT procedure

Documented in 143 (74.1%)

Duhamel procedure n=57

TERPT n=40

Soave procedure n=35

Swenson procedure n=10

Posterior sagittal approach n= 1

Type of redo procedure not reported in 50 pts


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Age

Patients age at redo PT operation

Documented in 108 (56.0%) patients Mean of 4.4 years (range 4 months17 years).

Time between initial PT and redo PT operation

reported in 74 (38.3%) patients mean of 2.8 years (range 6 months8 years).


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Follow-up

available in 134 (69.4%) patients

mean follow-up time : 4.1 years (3 m 23 yrs)

Of the 134 patients,

99 (73.9%) pts normal bowel habits after redo PT operation.

19 pts persistent/intermittent constipation with occasionalsoiling

16 patients had recurrent enterocolitis with or withoutperianal excoriation.


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Most of the patients were fecallycontinent and had normal bowel

movements except for occasional soiling .

No significant difference in functional

outcome between the various redo PTprocedures


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discussion


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Several studies : no statistically

significant difference in thefunctional outcome with respectto bowel function between the

various PT procedures to treat HD


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For a successful PT , it is

essential that all aganglionicbowel is resected and bowelwith normal innervation isanastomosed to the anus


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193 (34.8%) demonstrated

abnormal histological findingson repeat rectal biopsy.

144 (74.6%) revealed RA49 (25.4%) TZB


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Of these 193 patients

persistent abdominal distension/constipationn=135

recurrent episodes of enterocolitis

n=45

Histological evidence of RA in the resectedproximal margin of the pulled-through boweln=13


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meta-analysis reveals

RA and TZB : underlying

causes of persistent bowelsymptoms in one-third of

all patients requiring redoPT operation.


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Redo PT operation for RA orTZB :

potentially preventable by accurateidentification of the proximal margin of

the aganglionic bowel and transition-zone by an experiencedhistopathologist


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During frozen section analysis at the time ofthe initial PT operation:

the pathologist must confirm normal ganglion cells andabsence of nerve trunks at the site of the plannedanastomosis

Major problem with the intraoperative frozen

section biopsies :

can indicate the presence of ganglion cells withoutdifferentiating between hypo- and dysganglionosis


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Shayan K et al (2004): Reliability of intraoperativefrozen sections in the management of Hirschsprungs

disease, J Pediatr Surg

3% of 304 children who had intraoperative frozen section analysisduring PT operation showed a discrepancy between the frozensection diagnosis and the final pathological diagnosis.

The use of rapid technique of acetylcholinesterasestaining may help overcome this problem


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To prevent pulling-through the

transition-zone for anastomosis resecting several cm above the

proximal ganglionic bowel identified

by the pathologist during frozensections.


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Recurrent bowel problems after PT

Constipation after PT operation in vast majority

non-operative methods :laxatives and enemas

Postoperative enterocolitis

rectal irrigation with or without metronidazole prophylaxis

persistent constipation, abdominal distension or recurrentepisodes of enterocolitis

a full thickness rectal biopsy indicated to rule out RA or TZB


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presence of RA or TZB

resection of this section of bowelmay cure the patients of theirrecurrent symptoms.

a redo PT is generally recommendedfor surgical management of RA


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the choice of procedure far fromobvious.

type of previous failed procedure

level of anastomosis

rectal blood supply and presence of fibrosis or inflammation in the

perirectal pouches must be considered


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In the present meta-

analysis, most patientswith HD had normal bowel

function after redo PToperation


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references


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Repeated pull-through surgery for complicatedHirschsprung's disease--principles derived from

clinical experience.

Schweizer P, Berger S, Schweizer M, HolschneiderAM, Beck O

J Pediatr Surg. 2007 Mar;42(3):536-43


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Methods

17 pts with HD aged 2 to 9 years

Surgical revision indicated by incomplete resection of the transition zone in16 patients, anastomotic strictures in 9 patients, and fistulas in 2 patients.

All 17 patients Redo Duhamel PT

Median follow-up 9 years (range, 1-23 years).


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CONCLUSIONS

predominant cause for persistent or recurrentobstructive symptoms after initial pull-throughprocedure : incomplete resection of the

transition zone.

Redo Duhamel pull-through procedure is able toprovide the definitive solution to the problem.


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Redo pull-through in Hirschsprung's [corrected]disease for obstructive symptoms due to residual

aganglionosis and transition zone bowel.

Lawal TA, Chatoorgoon K, Collins MH, Coe A, Pena A,Levitt MA.

J Pediatr Surg. 2011 Feb;46(2):342-7


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METHODS

93 pts with HD with recurrent

problems after PT All required reoperations

25 had residual aganglionosis/transition-

zone histology : the only indication forredo in 16 children.


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RESULTS

Rectal biopsy: hypertrophic nerves (n = 16), absentganglion cells (n = 6), and normal ganglion cells (n = 10).

Original frozen-section biopsy only sampled theseromuscular layer in 3 children, leading to misdiagnosis.

In all cases, obstructive symptoms were resolved, and nopatient had recurrent enterocolitis.


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CONCLUSIONS

Patients' post pull-through with recurrent obstructivesymptoms may have residual aganglionosis or transition-zone bowel.

Reoperation can result in the resolution of thesesymptoms.

A full-thickness biopsy at the time of the initial pull-through to include the mucosa and submucosa mayincrease the possibility of identifying hypertrophic nerves


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Reoperation for Hirschsprung disease: pathology of theresected problematic distal pull-throughCoe A, Collins MH, Lawal T, Louden E, Levitt MA, Pena APediatr Dev Pathol. 2012 Jan-Feb;15(1):30-8

histopathology of pull-through bowel segments resected because of poor postoperativeoutcome from 30 patients

MC indication for reoperation: constipation/obstruction

Transition zone (bowel with at least two nerves 40 m diameter per 400 high-powerfield, and ganglion cells) or aganglionic bowel (bowel with at least two nerves 40 m per

high-power field diameter, but without ganglion cells) was found in 19/30 (63%) resections.


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Thank you

residual aganglionosis after pull-through operation

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