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Contents lists available at ScienceDirect

Digestive and Liver Disease

journal homepage: www.elsevier.com/locate/dld

Alimentary Tract

Cone-like resection, stulectomy and mucosal rectal sleeve partial

endorectal pull-through in paediatric Crohns disease with perianal
complex stula
Girolamo Mattioli a,b, , Luca Pio a,b , Serena Arrigo a , Alessio Pini Prato a ,
Giovanni Montobbio a , Nicola Massimo Disma a , Arrigo Barabino a
a
b

Giannina Gaslini Institute, Genoa, Italy

DINOGMI, University of Genoa, Italy

a r t i c l e

i n f o

Article history:
Received 24 February 2015
Accepted 6 May 2015
Available online xxx
Keywords:
Complex stula
Cone-like resection
Paediatric
Perianal Crohns

a b s t r a c t
Background: Perianal abscesses and stulae have been reported in approximately 15% of patients with
paediatric Crohns disease and they are associated with poor quality of life. Several surgical techniques
were proposed for the treatment of perianal Crohns disease, characterized by an elevated incidence of
failure, incontinence, and relapse.
Aim of our study was to present the technical details and results of our surgical technique in case of
recurrent, persistent, complex perianal ano-rectal destroying Crohns disease not responding to medical
treatment.
Methods: Data of patients who underwent surgical treatment (cone-like resection, stulectomy, sphincter
reconstruction, endorectal advancement sleeve aps like in Soave endorectal pull-through) for complicated high-level trans, inter or suprasphincteric stulae between January 2009 and June 2014 were
retrospectively reviewed.
Results: 20 surgical procedures were performed in 11 patients (males 72.7%) with transsphincteric (n = 5),
intersphincteric (n = 4) and suprasphincteric (n = 2) stulae. Three patients needed a second treatment.
Two patients needed more than 2 surgeries and one temporary colostomy. No patient presented anal
incontinence at 15 months median follow-up.
Conclusions: Although several procedures may be required to obtain a complete remission of perianal
lesions, in our series the proposed surgical technique seemed effective and safe, preserving anal continence in all treated cases and reducing the need of faecal diversion.
2015 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved.

1. Introduction
Paediatric Crohns disease (PCD) accounts for 2025% of total
diagnosis of this inammatory bowel disease, with an incidence
ranging from 0.2 to 9.5:100.000 children in the United States.
Perianal disease has been reported in 815% of paediatric
patients [1,2].
Complex stula can have transsphincteric, suprasphincteric,
intrasphincteric and extrasphincteric perianal localization [3] and
represents a challenge for paediatric surgeons.
In recent years, magnetic resonance imaging (MRI) has become
an important instrument to evaluate complex stula severity and

pelvic anatomy, providing indications for the correct type of surgical intervention. However, many false negative results and poor
sensitivity of this tool have been reported [4,5].
Different approaches have been described for the treatment of
complex stula in children, including simple drainage, mobilization
of tissue aps, seton placement, stulotomy, anus-sparing proctocolectomy, and defunctioning ileostomy [68]. However, the risk
of complications remained high, with more than one procedure for
recurrence in 2950% of cases [810].
Aim of this study was to report on the surgical technique used
in our institution, describing surgical details and main results.
2. Materials and methods
2.1. Study population

Corresponding author at: Paediatric Surgery Unit, Istituto Giannina Gaslini, Largo
G. Gaslini 5, 16147 Genoa, Italy. Tel.: +39 010 56362217; fax: +39 010 3075092.
E-mail address: girolamomattioli@ospedale-gaslini.ge.it (G. Mattioli).

All patients with diagnosis of perianal PCD admitted to our

Institute for complex stula (dened according to Bell criteria)

http://dx.doi.org/10.1016/j.dld.2015.05.003
1590-8658/ 2015 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved.

Please cite this article in press as: Mattioli G, et al. Cone-like resection, stulectomy and mucosal rectal sleeve partial endorectal pullthrough in paediatric Crohns disease with perianal complex stula. Dig Liver Dis (2015), http://dx.doi.org/10.1016/j.dld.2015.05.003

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with recurrent/persistent, anorectal involvement not responding

to medical treatment, between January 2009 and June 2014 were
prospectively included in a database.
Patients with simple stula (subcutaneous stula) were
excluded from the study.
This study was performed according to national ethical guidelines and informed consent was obtained for surgical treatment and
data collection from parents or guardians. Data including demographics, previous surgical and medical treatments, surgical details
of interventions, and clinical follow-up were retrospectively analyzed.
All patients were studied with pre-operative MRI. All patients
were continent before surgical procedures.

2.2. Cone-like resection technique (CLR)

Peri-operative antibiotic prophylaxis with metronidazole was
administered. Patients were placed in the lithotomy position under
general anaesthesia without preoperative bowel preparation. A
probe was inserted through the stula to measure the distance from
its internal opening. The stula tract was completely mobilized enbloc with the granulation tissue reaching the normal fatty tissue
near the pelvic oor. A cone-like excision of skin and perianal tissue was performed with the cone base in the perineum including
the anal canal if affected, and the cone apex in the rectal wall where
the stula opened (Figs. 1 and 2).
Exposure of levator ani was needed to completely remove the
affected tissue, including also rectal wall and anal sphincters if
involved.
The second step of this surgical approach was to recreate the
anal canal. The rectal sleeve was prepared proximally to the internal opening of the rectum. We used Soave endorectal pull-through
(ERPT), pulling the normal rectal mucosa to the anal skin and suturing the sphincters to recreate anal ring normal shape. The perianal
skin was always left open in order to reduce infection risks. No more
than two areas were treated simultaneously (Figs. 2 and 3).

Fig. 1. Illustration of cone-like resection and rectal sleeve. IS, internal sphincter;
ES, external sphincter; IF, intersphincteric stula; TF, transsphincteric stula; SS,
suprasphinteric stula; R, rectum; RS, rectal sleeve; CLFR, cone-like stulectomy
resection.

2.3. Endpoints
The primary endpoint was clinical recurrence, dened symptomatic recurrence requiring surgical treatment.
Secondary endpoints were denition of 30-day post-operative
complication rate using Clavien-Dindo classication [11], assessment of post-operative faecal incontinence using Yamataka score

Fig. 2. The chronic Crohns granulation tissue involves skin, fatty tissue, anal sphincter and rectal wall. In selected cases, when there is recurrence despite adequate medical
treatment, therefore the inamed tissue should be completely removed before complete destruction of sphincter activity due to risk of sepsis. The blue arrow indicates the
previously placed seton.

Please cite this article in press as: Mattioli G, et al. Cone-like resection, stulectomy and mucosal rectal sleeve partial endorectal pullthrough in paediatric Crohns disease with perianal complex stula. Dig Liver Dis (2015), http://dx.doi.org/10.1016/j.dld.2015.05.003

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Table 1
Overall patient population characteristics (n = 11).
Characteristic
Males
Median age (years)
Transsphincteric stula
Intersphincteric stula
Suprasphincteric stula
Median PDAI
Previous surgery
Seton
Abscess drainage
Fistulotomy
Median operative time (min)
Median hospital stay (days)
Median FLACC score
Median number of procedures
Complications

N (%)
8 (72.7)
12 (range 519)
5 (45.4%)
4 (36.4%)
2 (18.2%)
5 (range 312)
4 (36.4%)
6 (54.5%)
1 (9.1%)
40 (range: 2080)
4 (range: 37)
0
1 (range: 15)
0

PDAI, Perineal Disease Activity Index; FLACC, Faces, Legs, Activity, Cry and Consolability score.
Fig. 3. Cone-like resection includes removal of ano-rectal canal and perianal tissue (skin and subcutaneous) macroscopically involved by Crohns granulomatosis
reaching the normal muscle of the perineum (levator ani, blue arrow). The second
step includes restoration of normal continuity of the ano-rectal canal and sphincter
activity (yellow arrow). The proximal rectal mucosa is pulled down to the perianal
skin. The anal ring is recreated by suturing the normal muscle. The skin is left open.
The aspect of resection is cone-like, the apex is in the rectum and the base is the
skin of the perineum.

[12], and analysis of post-operative pain using Faces, Legs, Activity,

Cry, and Consolability (FLACC) score [13].
Follow-up was performed with clinical evaluation.
2.4. Statistical analysis
Continuous variables are reported as means and standard deviation or median and range and were compared using Students t
tests; categorical variables are reported as n (%) and were compared
using Chi-squared tests or Fishers test. Possible risk factors as age
at presentation, type of previous surgical treatment, pre-operative
or post-operative medical treatment were analyzed with statistical
signicance dened as p < 0.05.
3. Results
From January 2009 to June 2014, 11 patients were treated (72.7%
males, median age at surgery 12 years, range: 519 years).
In two patients perianal disease appeared after diagnosis of
Crohns disease (after 1 and 8 years), during immunosuppressive therapy (azathioprine) and on mesalazine. In the other cases,
Crohns disease was diagnosed during the evaluation of perianal
disease. Location was ileocaecal (n = 7), ileocolic (n = 3), and panenteric (n = 1).
At preoperative endoscopy, rectal inammation was present
only in 2 cases (18.2%). One girl, previously followed for syndromic
diarrhoea by home parenteral nutrition, presented with perianal
rectal Crohns-like disease involvement. Median Perineal Disease
Activity Index (PDAI) was 5 (range: 312).
Diagnosis and classication of perineal disease were made by
the surgeon with evaluation under anaesthesia with pelvic MRI.
Four subjects were initially treated at another hospital (3 with
simple drainage and seton placement, 1 with stulotomy). One
patient presented a gluteus abscess and drainage with seton placement was performed along with prolonged antibiotic treatment.
After abscess resolution, complex stula persisted. The other
6 patients underwent simple drainage before CLR (54.5%). In 2
cases, patients were receiving medical treatment with biologics,

azathioprine and thalidomide (18.2%); in the other 9 cases only

antibiotics were administered.
The locations of the stulae are shown in Table 1; transsphincteric location was present in 5 patients (45%).
Median surgery duration was 40 min (range 2080 min) and
median hospital stay was 4 days (range 37 days; Table 1).
Anti-TNF therapy was started in all patients within 10 days postoperatively, for a minimum of 12 months. No signicant adverse
effects were observed. Clinical and endoscopic follow-up was performed, post-operative MRI was performed in complex cases with
more than two recurrences requiring repeated surgery or to rule out
abscess. Step down to thiopurines was performed only in patients
with sustained clinical and endoscopic remission. Median followup was 15 months (range 1156 months).
A total of 20 CLRs with rectal sleeve were performed. In 6 cases
complete remission was obtained after the rst operation (54.5%).
The remaining 5 required subsequent surgeries for relapses or new
localizations. Three patients needed a second intervention, one a
third procedure. One patient needed ve treatments (Supplementary Table S1) and required colostomy for the recurrence of complex
stula, despite biological treatment. Colostomy was closed after 12
months and the patient underwent clinical follow-up.
The median number of procedures to obtain stula healing was
1 (range: 15).
Evidence of wound healing by second intention was provided
in the rst month of follow-up (Fig. 4), and at the end of surgical
treatment all eleven patients healed with complete restitutio ad
integrum. No other minor recurrences were observed.
Age at surgery, absence of previous surgical treatments, type of
medical treatment before surgical procedure were not statistically
related with recurrence. Post-operative pain was easily controlled
with elastomeric pump (chirocaine plus clonidine) for the rst two
days and with non-steroidal anti-inammatory drugs on day three
every 8 h. Daily FLACC score was 0 for all patients.
No major complications nor anal incontinence were observed
(Table 1).
4. Discussion
Complex stula is a debilitating condition for paediatric
patients. Several treatments have been proposed, however the risk
of recurrence remains high with a long history of medical and surgical procedures.
Very few studies on major surgical treatments for paediatric
complex stula are available in the literature. Current NASPHGAN
guidelines [6] recommend surgeons not to perform advancement

Please cite this article in press as: Mattioli G, et al. Cone-like resection, stulectomy and mucosal rectal sleeve partial endorectal pullthrough in paediatric Crohns disease with perianal complex stula. Dig Liver Dis (2015), http://dx.doi.org/10.1016/j.dld.2015.05.003

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Fig. 4. Healing by second intention with normal stool retention, sensory function,
and continence. The cosmetic aspect is not objectively evaluated but scar retraction
is limited and anal shape is normal.

aps or major surgery for high risk of failure, suggesting colostomy

or ileostomy in case of severe or recurrent perianal Crohns disease, especially in case of refractory infectious complications (such
as recurrent abscess). Fistulectomy and other major surgical procedures did not gain popularity in the treatment of complex stula
for the risk of sphincter injury and incontinence.
In our opinion, it is better to completely remove perianal chronic
inammatory tissue, obviously only in case of persistent, recurrent
complex disease not responding to medical treatment.
The purpose of this study was to use cone-like resection to reach
complete removal of granulation tissue (stulectomy) and recreate
the ano-rectal canal. Sphincteric activity was restored using ERPT
with rectal sleeve as main surgical technique in complex stula
in order to reach primary healing, low recurrence rate, and minimal risk of sphincter injury when sphincteric section was necessary
to remove all inammatory tissue. ERPT allows the restoration of
nearly normal perineal shape and limited scar retractions.
In our series of 11 patients with complex stula, cone-like
stulectomy with rectal sleeve was a safe and well-tolerated procedure. Fistula healing rate was 54.5% with no case of faecal
incontinence after the rst surgical procedure and 100% after maximum 5 procedures.
In accordance to Arroyo et al. [14] our series conrms that stulectomy with sphincteric surgery is a procedure with limited risk
of faecal incontinence.
The role of faecal diversion for complex stula remains unclear
in the literature. In adult patients, the reported incidence is about
31% [15], while in children the incidence of faecal diversion was
reported in few articles, and in the largest series, 23% of cases had
defunctioning ileostomy with 38% of stoma-related complications
[7].
Though faecal diversion is an accepted major invasive treatment for complex stula, in addition to stoma complications the
childs quality of life must also be considered. In adult patients some
studies on quality of life of have been published [1618], to our
knowledge, there is only one reported study on this topic in the
paediatric population [19].
In our series, CLR was used as rst major surgical procedure
and faecal diversion was associated with stulectomy only in one
patient (9%) with recurrent complex stula and high risk of sepsis.
The introduction of biologic agents has dramatically changed the
therapeutic strategy for IBD in children. The rst evidence-based
practical guidelines on medical management in paediatric-onset

Crohns disease have recently been published. Among the recommended biologics, anti-TNF agents are the treatment of choice in
active perianal stulising disease in combination with appropriate
surgical intervention [20].
Combined anti-TNF therapy and surgery showed improved
healing and lower recurrence of stulae compared with surgery
alone in paediatric patients [21]. Combined treatment was also successful in adult patients with faster and prolonger stula healing as
described by Sciaudone et al. [22].
Hukkinen reported a 70% healing rate in 13 patients and setons
were kept for 8 months [23]. In our series, all patients recovered from complex stula and wounds healed by second intention
within one month.
NASPHGAN guidelines also suggested seton placement for treatment of paediatric complex stula but as reported by Langer et al.
[6,7], in some cases up to 7 placements with multiple anaesthesia procedures can be required. Furthermore, reported healing rate
reported is low.
CLR is characterized by a low number of surgical interventions
and consequently of anaesthesia procedures in children. CLR also
allows an easy post-operative pain management: morphine is not
required and patients may be discharged only with non-steroidal
anti-inammatory drugs.
In our experience, CLR is a safe and well-tolerated technique
with high primary healing and low recurrence rates, without risk of
sphincter injury and faecal incontinence when performed by experienced surgeons. In some cases multiple procedures are required,
and adequate medical treatment with biologics is needed to consolidate remission. CLR could be considered as a primary major
surgical technique in children with complex stula, in association
with biologics, reserving enterostomy only for very difcult nonresponder cases.
However, as reported by Pellino et al. [24], other surgical treatments like brin glue or adipose tissue-derived stem cell injection
have shown promising preliminary results and further studies are
required to improve surgical outcomes for the treatment of complex stula.
Conict of interest
None declared.
Acknowledgements
We thank Anna Capurro for her help in revising the manuscript.
Appendix A. Supplementary data
Supplementary data associated with this article can be found, in
the online version, at http://dx.doi.org/10.1016/j.dld.2015.05.003
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Please cite this article in press as: Mattioli G, et al. Cone-like resection, stulectomy and mucosal rectal sleeve partial endorectal pullthrough in paediatric Crohns disease with perianal complex stula. Dig Liver Dis (2015), http://dx.doi.org/10.1016/j.dld.2015.05.003