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368
sepsis is a major determinant of PVF formation [4, 5, 7, 12]. Studies suggest no
difference in the incidence of PVF in inammatory vs. non-inammatory disease.
Furthermore, the severity of the colitis or number of stages in the operation did
not seem to inuence PVF formation. No difference in the incidence of PVF for-
mation was seen in stapled vs. hand-sewn anastomosis [4]. Based upon these data,
there seems to be no preventative measures for PVF formation beyond sound
surgical technique in order to prevent pelvic sepsis, regardless of anastomotic
approach.
Higher Failure Rates forPVF Repair andNeed forPouch Excision
inCrohn’s Disease
Although there seems to be no difference in the incidence of PVF formation in
inammatory vs. non-inammatory disease, there is a clear difference in the healing
of PVFs in these two populations. PVFs in Crohn’s disease patients have a lower
overall healing rate as compared to the non-Crohn’s disease cohort (25% vs. 48%)
[5]. Furthermore, a diagnosis of Crohn’s results in worse treatment outcomes and
higher pouch failure rates [5, 13]. PVFs are noted to occur later in patients with
Crohn’s disease (median of 12months as compared to 8.2months in patients with-
out Crohn’s disease) [12]; prognosis has also been found to be worse when PVFs
occur after ileostomy closure [4].
In summary, approximately 20% of patients have required a pouch excision for
cure [7, 11]. Of the 2% of pouch patients ultimately diagnosed with Crohn’s dis-
ease, the pouch excision rate is approximately 60% as compared to 20% in the
remaining cohort [7].
Recommendations Based onData
Based upon various retrospective and observational data, it is difcult to recom-
mend a rm management algorithm as no single procedure is appropriate for all
PVFs. However, the following recommendations are made based upon the current
literature:
Advise patients that PVFs generally require multiple attempts at closure with local
approaches and ultimately 20% will require pouch excision; an even higher fail-
ure rate is expected in patients with Crohn’s disease. (Strong recommendation
based upon moderate quality evidence)
Fecal diversion should be established prior to attempts at repair of PVFs. (Moderate
recommendation based upon moderate quality evidence)
Local transvaginal or transanal (endo-ileal ap) approaches are appropriate to repair
PVFs that occur below the anastomosis. (Moderate recommendation based upon
moderate quality evidence)
V. V. Lao and S. D. Wexner
369
Muscle interposition ap is appropriate if diversion and local repair fails. (Weak
recommendation based upon low quality evidence)
An abdominal approach is typically appropriate for repair of high PVFs (Moderate
recommendation based upon low quality evidence)

Personal View

PVFs are uncommon and difcult to treat; however, there are several treatment
options available. No single treatment is considered successful or unsuccessful, and
often times multiple operations are needed to close a PVF.Once a PVF is suspected,
the diagnosis is conrmed with a pouchogram and exible sigmoidoscopy. Biopsies
are taken to conrm or exclude Crohn’s disease, as PVF repair in this setting will
fail at a much higher rate; approximately 60% of patients with PVF in the setting of
Crohn’s disease will require pouch excision. Knowing this information will allow
for appropriate expectations and counseling of the patient.
Prior to any procedures to close the PVF, ileal contents should be diverted and, if
needed, debridement and placement of a seton should be undertaken. Diversion for
3–6 months may be considered prior to any further local surgical interventions. This
maneuver helps to control sepsis as well as afford patients symptomatic relief.
Diversion alone can allow for closure of the PVF in certain cases. Any local proce-
dures to close the stula prior to diversion are not advised as failure of the procedure
may preclude use of that procedure after diversion, if and when it fails. Asymptomatic
small stulas would be an exception to this treatment guideline.
The diverting ileostomy should be placed in a loop of small bowel where that
ileostomy can either be subsequently maintained if a redo J-pouch is necessary or
where that point would correspond with an appropriate length pouch which would
reach the anus. In other words, at the time of loop ileostomy construction, always
think about the next potential stage and use a segment of bowel so as not to preclude
construction of a second pouch, if necessary.
After diversion, once local infection is treated and sufcient time has elapsed,
our personal preference is to utilize healthy fresh tissue in the form of the gracilis
muscle as an initial step. If the gracilis muscle fails, consideration could even be
given to a contralateral gracilis interposition. Ultimately, pouch advancement is
reserved as a nal option as transanal pouch advancement is not always possible. It
is our preference to commence pouch advancement in the prone jackknife position,
yet the patient must be advised that if transanal pouch advancement is not possible,
they would be repositioned in the supine modied lithotomy position and prepared
for a combination transabdominal transanal pouch advancement. However, if during
pouch advancement the pouch becomes ischemic and/or damaged beyond salvage,
the fallback position is construction of a second pouch. If a new pouch cannot be
constructed, a permanent ileostomy would be necessary. In these instances, either
the ileostomy is maintained, or the loop ileostomy may be closed and additional
bowel put back into circuit by creating a more distal ileostomy.
40 Management ofIleal Pouch Vaginal Fistulas
370

References

1. Wong WD, Rothenberger DA, Goldberg SM.Ileoanal pouch procedures. Curr Probl Surg.
1985;22(3):1–78.
2. Lolohea S, Lynch AC, Robertson GB, Frizelle FA.Ileal pouch-anal anastomosis-vaginal s-
tula: a review. Dis Colon Rectum. 2005;48(9):1802–10. Review.
3. Tulchinsky H, Hawley PR, Nicholls J.Long-term failure after restorative proctocolectomy for
ulcerative colitis. Ann Surg. 2003;238(2):229–34.
4. Groom JS, Nicholls RJ, Hawley PR, Phillips RK. Pouch-vaginal stula. Br J Surg.
1993;80(7):936–40.
5. Shah NS, Remzi F, Massmann A, Baixauli J, Fazio VW. Management and treatment out-
come of pouch-vaginal stulas following restorative proctocolectomy. Dis Colon Rectum.
2003;46(7):911–7.
6. O'Kelly TJ, Merrett M, Mortensen NJ, Dehn TC, Kettlewell M.Pouch-vaginal stula after
restorative proctocolectomy: aetiology and management. Br J Surg. 1994;81(9):1374–5.
7. Wexner SD, Rothenberger DA, Jensen L, Goldberg SM, Balcos EG, Belliveau P, Bennett BH,
Buls JG, Cohen JM, Kennedy HL, etal. Ileal pouch vaginal stulas: incidence, etiology, and
management. Dis Colon Rectum. 1989;32(6):460–5.
8. Keighley MR, Grobler SP. Fistula complicating restorative proctocolectomy. Br J Surg.
1993;80(8):1065–7.
9. Paye F, Penna C, Chiche L, Tiret E, Frileux P, Parc R.Pouch-related stula following restor-
ative proctocolectomy. Br J Surg. 1996;83(11):1574–7.
10. Lee PY, Fazio VW, Church JM, Hull TL, Eu KW, Lavery IC.Vaginal stula following restor-
ative proctocolectomy. Dis Colon Rectum. 1997;40(7):752–9.
11. Gaertner WB, Witt J, Madoff RD, Mellgren A, Finne CO, Spencer MP. Ileal pouch s-
tulas after restorative proctocolectomy: management and outcomes. Tech Coloproctol.
2014;18(11):1061–6.
12. Tsujinaka S, Ruiz D, Wexner SD, Baig MK, Sands DR, Weiss EG, Nogueras JJ, Efron JE,
Vernava AM 3rd. Surgical management of pouch-vaginal stula after restorative proctocolec-
tomy. J Am Coll Surg. 2006;202(6):912–8.
13. Mallick IH, Hull TL, Remzi FH, Kiran RP.Management and outcome of pouch-vaginal stu-
las after IPAA surgery. Dis Colon Rectum. 2014;57(4):490–6.
14. Burke D, van Laarhoven CJ, Herbst F, Nicholls RJ.Transvaginal repair of pouch-vaginal s-
tula. Br J Surg. 2001;88(2):241–5.
15. Gorne SR, Fichera A, Harris MT, Bauer JJ.Long-term results of salvage surgery for septic
complications after restorative proctocolectomy: does fecal diversion improve outcome? Dis
Colon Rectum. 2003;46(10):1339–44.
16. Zinicola R, Wilkinson KH, Nicholls RJ.Ileal pouch-vaginal stula treated by abdominoanal
advancement of the ileal pouch. Br J Surg. 2003;90(11):1434–5.
17. Johnson PM, O'Connor BI, Cohen Z, McLeod RS.Pouch-vaginal stula after ileal pouch-anal
anastomosis: treatment and outcomes. Dis Colon Rectum. 2005;48(6):1249–53.
V. V. Lao and S. D. Wexner
371
© Springer Nature Switzerland AG 2019
N. Hyman et al. (eds.), Mastery of IBD Surgery,
https://doi.org/10.1007/978-3-030-16755-4_41
J. H. Ashburn (*)
Department of Surgery, Wake Forest University Baptist Medical Center,
Winston-Salem, NC, USA
e-mail: jashburn@wakehealth.edu
41
Management ofIPAA-Associated
Persistent Presacral Sinus
JeanH.Ashburn

Introduction

A persistent presacral sinus tract arising after restorative proctocolectomy with ileal
pouch-anal anastomosis (IPAA) can cause considerable morbidity and is associated
with high rates of pouch failure [1, 2]. These sinuses are blind-ending tracts that
form as a result of a non-healing leak or suture/staple line dehiscence at the pouch-
anal anastomosis. Although many defects remedy themselves with proper drainage
and patience, some chronic tracts may persist and lead to signicant compromise in
pouch function and longevity [3]. A number of interventions have been performed
in patients with IPAA-associated persistent sinus tracts, most of which are in a pos-
terior location, with hopes of achieving either symptom relief or complete closure
of the tract. The optimal approach must consider the specic characteristics of the
persistent sinus tract, and options range from less invasive endoscopic procedures to
more extensive pouch revision and redo of the pouch-anal anastomosis (Table41.1).

Search Strategy

PICO (Population, Intervention, Comparator, Outcome) data were obtained
(Table 41.1). A literature search of EMBASE, MEDLINE, and PubMed was
Table 41.1 PICO table
Patients
Intervention Comparator
Outcome
IPAA patients with
persistent presacral sinus
Unroong of
sinus tract
Recreation of
IPAA
Healing rate of sinus
tract, IPAA loss
372
conducted to identify all English-language publications associated with ulcerative
colitis, ileal pouch-anal anastomosis, and post-operative pouch complications from
1980–2018. The following key search terms were queried: ‘colitis’, ‘ulcerative
colitis’, or ‘inammatory bowel disease’; ‘ileal pouch-anal anastomosis’, ‘IPAA’,
‘J pouch’; ‘presacral sinus’, ‘chronic sinus’, ‘IPAA sinus’, ‘chronic anastomotic
sinus’. Studies were excluded if they considered sinuses other than those related to
the ileoanal anastomosis (i.e. colorectal/coloanal), unless data regarding ileoanal
sinuses could be separated and assessed independently. All reference lists of the
included manuscripts were noted to identify additional publications that were
acceptable for inclusion. Publications from the same institution were carefully
assessed for overlap and only the most recent study was considered in this circum-
stance. Observational, prospective, retrospective, and randomized studies were
considered for the literature assessment.

Results

Despite signicant morbidity of presacral sinus tracts in IPAA surgery, the optimal
management of these persistent sinuses is not well delineated. Reports have not
offered direct comparison among the described techniques to confer superiority of
one approach over the rest. Large randomized studies are lacking and outcomes
from smaller series are varied and limited to retrospective and observational data.
Treatment of diverted, asymptomatic sinus tracts found with a water-soluble con-
trast enema is distinguished from those presenting with symptoms after intestinal
continuity is restored in only a few of the available studies.
A common initial approach to the asymptomatic, diverted presacral sinus is obser-
vation and delay of ileostomy closure. Observational studies evaluating this treat-
ment describe relatively good healing rates with observation and interval reimaging,
followed by ileostomy closure if healing is observed [4]. If sinus tracts persist despite
expectant management, patients are more likely to maintain their ileostomy [5].
When a conservative ‘watch and wait’ strategy is not sufcient to achieve com-
plete healing, several treatments have been reported to surgically correct the presa-
cral sinus tract. The most described method of surgical correction of the IPAA sinus
is unroong, described in 1997 by Whitlow, a method of laying open the sinus tract
by incising the common wall between the tract and pouch body, thus incorporating
the sinus into the lumen of the pouch. All four patients in their initial report of IPAA
sinus who underwent unroong exhibited complete healing within 12months of the
procedure. There were no IPAA failures in this group [6].
Other retrospective reports have reported success of observation with further
intervention for those who do not heal in a timely fashion. One study reported on 22
patients with a persistent sinus tract on contrast imaging after diverted IPAA.Twelve
of these patients healed with observation only, and six patients with persistent tracts
after the observation period underwent successful debridement (unroong), with
successful closure of the tract. Pouch survival was not reported, but two patients
failed treatment and never achieved healing during the study period [7].
J. H. Ashburn
373
One of the largest studies of persistent presacral IPAA tracts reported on 45
patients, with 23 of these patients identied prior to ileostomy closure and 22 after
ileostomy closure. Eight of forty-ve patients underwent unroong initially, with
50% healing. Fourteen of forty-ve patients underwent unroong as an additional
treatment after one of the following; observation, drainage, sinus closure, or diver-
sion. Seven of these fourteen patients achieved complete healing. Of patients under-
going unroong for a persistent sinus, 50% showed complete healing, with healing
increasing to 81% in sinus tracts that were asymptomatic. Fifteen patients (33%) in
the study eventually had pouch failure. Although no patients were initially treated
with pouch redo or revision, three patients underwent pouch redo after a failed ini-
tial treatment. Two patients achieved a successful redo pouch with good function
but one required excision of the redo pouch [8].
Two studies reported on low pelvic anastomotic sinuses, with a few IPAA patients
included in each study. In one study, data related only to IPAA sinuses showed com-
plete healing three out of ve IPAA sinus tracts with observation (two of ve
required maintenance of ileostomy for persistent sinus) [5]. The other similar study
reported on six patients with an IPAA sinus, all of which were able to undergo suc-
cessful ileostomy closure after observation or a combination of observation and
sinus debridement/unroong [9].
Finally, the technique of needle-knife sinusotomy (NKSi) is an endoscopic varia-
tion of surgical unroong that has been more recently described [10–12]. Reports
from the highest volume NKSi center recently reported on 109 patients with pouch
sinus tracts, most of which were symptomatic (93.6%). Complete healing rate was
nearly 50% with symptomatic improvement shown in 77.5%. Over half of the group
required repeat treatments, and 20% of patients experienced sinus-related IPAA
failure [13] (Table41.2).
Recommendations Based OnData
Patients with an asymptomatic IPAA sinus detected prior to ileostomy closure can
be managed initially with a conservative ‘watchful waiting’ strategy in which they
are observed and periodically reimaged with water-soluble enema study to check
for healing of the sinus. Chronic IPAA sinuses that persist after expectant manage-
ment may undergo careful surgical unroong with reasonable hope for healing.
Redo IPAA can be considered for sinus tracts refractory to unroong, or for
patients in whom unroong of sinuses would signicantly interfere with subsequent
pouch revision. (Evidence: low; Recommendation: strong).

Personal View

IPAA sinuses occur as a result of anastomotic separation and sepsis after pouch cre-
ation. If this occurs, patients should undergo exam under anesthesia and transanal
mushroom drain placement every 6–8weeks with downsizing of the drain until the
41 Management ofIPAA-Associated Persistent Presacral Sinus
374
defect heals. Percutaneous drainage should not be used, as this converts the defect
into a stula tract. With proper management, many of these defects will heal over the
course of 9–12months, while some may develop into a presacral sinus tract.
An asymptomatic sinus tract found on routine contrast imaging, prior to loop
ileostomy closure, should be treated initially with a period of watchful waiting, with
interval imaging in a few months to conrm sinus healing prior to ileostomy clo-
sure. Patience is key during this process, as patients are typically feeling well and
eager to experience stoma-free living once again.
Sinuses that persist after watchful waiting, or those presenting with symptoms after
intestinal continuity is restored, can be considered for surgical intervention. Further
endoscopic and radiographic characterization (i.e. pouchoscopy and pelvic MRI) is
helpful here to assess the complexity and length of the sinus tract, and to distinguish
from stulae. Short, supercial sinus tracts should be treated with transanal drain
placement (if sepsis persists) or unroong and debridement of the sinus tract.
Sequential treatments may be necessary, with interim imaging to check for healing.
Small sinuses that are present after the above treatment in a diverted IPAA can be
offered an attempt at ileostomy closure as long as the patient understands the pathol-
ogy and possible outcomes of this approach. Fibrin glue injection has been reported
Table 41.2 Reported literature
Study
Patients (N)
Selection criteria
Treatment/
Healing rate of sinus tract
Pouch
failure rate
Quality of
evidence
Nyam [4] 41
Asymptomatic/
diverted
95% (40/41)
All observation
1/41 Low
van
Koperen
[5]
5
All diverted
60%
3/5 healed with observation
2/5 required ileostomy
40% Low
Whitlow
[6]
4
All diverted
100% (4/4)
All unroong
NA Low
Akbari [7] 22
Asymptomatic/
diverted
95% (20/21)
Observation±debridement
0 Low
Ahmed
Ali [8]
45
25/45
symptomatic
23/45 diverted
60% (27/45)
Observation 65% (23)
Unroong 50% (8)
Drainage 59% (9)
Sinus closure 33% (3)
Diversion 100% (2)
33% Low
Zhou [9] 6
All diverted
100% stoma closure
Observation±debridement/
drainage
NA Low
Lan [13] 109
102/109
symptomatic
49.5% complete
67.8% complete+partial
All NKSi
20% Moderate
Swain [14] 7
Chronic
(5/7 diverted)
100% (7/7)
All debridement + brin glue
NA Low
J. H. Ashburn
375
to be successful [14], but in our experience has not consistently contributed to com-
plete healing.
NKSi can be a successful treatment in carefully selected patients. This should be
limited to short sinus tracts without signs of sepsis, and should be performed by or
in close association with an IPAA surgeon. This treatment is promising, but patients
must understand the limitations and limited follow-up available for NKSi, and have
reasonable expectations for what this approach can achieve. Repeated treatments
may be benecial but can also compromise the posterior pouch wall in a way that
prevents other corrective options if needed.
Some patients with persistent IPAA sinus may be candidates for IPAA revision
or redo. Consideration should be given to those who have not achieved symptom
relief after transanal treatment (i.e. debridement/unroong) or those who demon-
strate a long sinus tract (over 6cm) who have not yet undergone unroong. It is
critically important that attempts to unroof longer tracts be approached with great
caution. Unroong rarely leads to complete healing in these longer tracts and can
cause major damage to the pouch body. This is important to consider because the
ileal pouch can be reused in nearly 2/3 of patients who require revisional pouch
surgery, and disruption of the posterior wall of the pouch during an attempted endo-
scopic intervention may prevent pouch salvage; this, in turn, may make pouch cor-
rection much more challenging or not possible at all [15].

References

1. Wu XR, Ashburn J, Remzi FH, Li Y, Fass H, Shen B.Male gender is associated with a high risk
for chronic antibiotic-refractory pouchitis and ileal pouch anastomotic sinus. J Gastrointest
Surg. 2016;20(3):631–9.
2. Kiely JM, Fazio VW, Remzi FH, Shen B, Kiran RP.Pelvic sepsis after IPAA adversely affects
function of the pouch and quality of life. Dis Colon Rectum. 2012;55(4):387–92.
3. Sagap I, Remzi FH, Hammel JP, Fazio VW. Factors associated with failure in managing
pelvic sepsis after ileal pouch-anal anastomosis (IPAA)—a multivariate analysis. Surgery.
2006;140(4):691–703.
4. Nyam DC, Wolff BG, Dozois RR, Pemberton JH, Mathison SM.Does the presence of a pre-
ileostomy closure asymptomatic pouch-anastomotic sinus tract affect the success of ileal
pouch-anal anastomosis? J Gastrointest Surg. 1997;1(3):274–7.
5. van Koperen PJ, van der Zaag ES, Omloo JM, Slors JF, Bemelman WA.The persisting pre-
sacral sinus after anastomotic leakage following anterior resection or restorative proctocolec-
tomy. Color Dis. 2011;13(1):26–9.
6. Whitlow CB, Opelka FG, Gathright JB Jr, Beck DE. Treatment of colorectal and ileoanal
anastomotic sinuses. Dis Colon Rectum. 1997;40(7):760–3.
7. Akbari RP, Madoff RD, Parker SC, Hagerman G, Minami S, Bullard Dunn KM, Mellgren
AF.Anastomotic sinuses after ileoanal pouch construction: incidence, management, and out-
come. Dis Colon Rectum. 2009;52(3):452–5.
8. Ahmed Ali U, Shen B, Remzi FH, Kiran RP.The management of anastomotic pouch sinus
after IPAA.Dis Colon Rectum. 2012;55(5):541–8.
9. Zhuo C, Trencheva K, Maggiori L, Milsom JW, Sonoda T, Shukla PJ, Vitellaro M, Makino
T, Lee SW.Experience of a specialist centre in the management of anastomotic sinus follow-
ing leaks after low rectal or ileal pouch-anal anastomosis with diverting stoma. Color Dis.
2013;15(11):1429–35.
41 Management ofIPAA-Associated Persistent Presacral Sinus
376
10. Lian L, Geisler D, Shen B.Endoscopic needle knife treatment of chronic presacral sinus at the
anastomosis at an ileal pouch-anal anastomosis. Endoscopy. 2010;42(Suppl 2):E14.
11. Li Y, Shen B.Successful endoscopic needle knife therapy combined with topical doxycycline
injection of chronic sinus at ileal pouch-anal anastomosis. Color Dis. 2012;14(4):e197–9.
12. Wu XR, Wong RCK, Shen B.Endoscopic needle-knife therapy for ileal pouch sinus: a novel
approach for the surgical adverse event (with video). Gastrointest Endosc. 2013;78(6):875–85.
13. Lan N, Shen B. Endoscopic Treatment of Ileal Pouch Sinus. Inamm Bowel Dis.
2018;24(7):1510–9.
14. Swain BT, Ellis CN.Fibrin glue treatment of low rectal and pouch-anal anastomotic sinuses.
Dis Colon Rectum. 2004;47(2):253–5.
15. Remzi FH, Aytac E, Ashburn J, Gu J, Hull TL, Dietz DW, Stocchi L, Church JM, Shen
B.Transabdominal redo ileal pouch surgery for failed restorative proctocolectomy: lessons
learned over 500 patients. Ann Surg. 2015;262(4):675–8.
J. H. Ashburn
377
© Springer Nature Switzerland AG 2019
N. Hyman et al. (eds.), Mastery of IBD Surgery,
https://doi.org/10.1007/978-3-030-16755-4_42
D. Wickramasinghe, MD(Surgery), DM MRCSEd · J. Warusavitarne, B Med, FRACS, PhD (*)
St. Mark’s Hospital, Harrow, UK
e-mail: j.warusavitarne@nhs.net
42
The Management ofPatients
withDysplasia intheAnal Transitional
Zone
DakshithaWickramasinghe andJanindraWarusavitarne

Introduction

Sir Alan Parks in 1978 described the rst case series of proctocolectomy with
ileal- pouch creation [1]. The purpose of the procedure was to remove all disease-
prone mucosa in ulcerative colitis (UC) while maintaining intestinal continuity
[1]. Of the two main surgical methods of forming ileal pouches, the double-sta-
pled (DS) method may have advantages over the hand-sewn (HS) approach
including lower postoperative complications and better functional outcomes [2].
However, an inherent limitation of this technique is the persistence of a length of
anal transitional zone (ATZ), the area between colorectal type mucosa above and
the squamous epithelium below [3]. Because this mucosa is at risk of chronic
inammation and dysplasia [3, 4], there is a long term risk of perianastomotic
malignancy [5]. The management of pouch and ATZ dysplasia take two forms;
minimizing the development of dysplasia and treating established dysplasia. To
minimize dysplasia, the mucosa left after surgery must be minimized and ensu-
ing inammation avoided. The former may be achieved by performing a muco-
sectomy and a HS anastomosis and the latter by treating inammation of the
rectal cuff.