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266
E. K. Johnson et al.
Local anesthetic is inltrated into the area to
be excised and the incision is made down to the
level of the subcutaneous fat. The overlying skin
is excised taking care to leave the subcutaneous
fat in place. A ap is then raised across the midline out to the opposite safe zone border. The
thickness of this ap should approximate that of
a breast ap that would be created during a mastectomy in order to ensure its viability. When
creating the ap down toward the distal portion
of the incision (near the anus), the ap should be
thicker to prevent dimple formation near the
anus. Any disease related debris or granulation
tissue should be gently debrided with a surgical
sponge and irrigation with saline should be performed. Any remaining “cyst wall” or tissue
contracture can be divided into squares with a
scalpel or electrocautery device. The subcutaneous tissue is then closed in layers with an absorbable suture. The supercial layers are
reapproximated in layers, lastly with a subcuticular suture. In some cases, even though there has
not been a large volume of tissue excision, there
will be a very deep wound. This occurs in those
with particularly deep natal clefts. While evidence is lacking, the authors would encourage
the use of a closed suction drain in this setting.
We have anecdotally seen some failures in this
setting when a drain is not used. The drain should
be left in place until the output is 20mL or less
for 2 consecutive days.
A 2011 study compared the results of the cleft
lift procedure to wide excision and packing in 70
patients [9]. A total of 97% of patients undergoing cleft lift healed completely while only 73% of
wide excision patients healed. Three of nine
patients with chronic wounds underwent subsequent cleft lift with a 100% success rate.
Recurrence was noted in 2.5% of cleft lifts and in
20% of wide excisions. Others have shown similar success in rates of healing with the cleft lift
procedure as compared to wide excision and
packing and excision and primary midline closure [10]. This technique has also been compared
to the Limberg ap in a randomized prospective
fashion [11]. Short term outcomes of 122 patients
were analyzed and revealed that those undergoing the cleft lift had shorter operative durations,
less excised tissue weight, improved pain scores,
and fewer physical limitations on postoperative
day 10. There were no differences in healing,
complications, or early recurrences.
This technique is certainly easier to perform,
takes less time, and removes less tissue than the
more complex ap procedures such as the rhomboid ap. It results in attening of the natal cleft
which is a desired goal. Unfortunately, not every
patient with PD is a candidate for this procedure.
Individuals with complex recurrent disease and
large open wounds may not be ideal candidates,
and may require more extensive ap procedures.
Disease that is very close to the anus may cause
difculty with this technique, though if open
wounds are able to be moved off the midline,
they may still heal.
The authors have noted some early wound
dehiscences in some cases treated with this technique (Fig.15.5). As with any ap procedure, it is
important to limit the patient’s activity for several
weeks to allow for healing. A traumatic wound
disruption can easily occur. Also noted is the
relatively short healing time when these wounds
Fig. 15.5 Wound dehiscences after cleft lift procedure
15 Pilonidal Disease
267
do separate—again very likely related to the fact
that the wound is off the midline (perhaps just
barely off midline—and the cleft is now at). If
an early wound separation does occur, just simply treat the patient as you would for any open
wound. One can expect healing in 2–6 weeks
with proper hygiene and wound care. We would
also recommend avoidance of any ap procedure in an active smoker or other patient with
modiable risk factors for poor wound healing.
These procedures are elective and should be performed in patients whose conditions have been
optimized.
Rhomboid/Limberg Flap
The rhomboid ap is a useful but more complex
procedure that can be used in any setting of PD,
but is typically reserved for more severe cases.
The procedure involves wide excision of a rhombus shaped area of tissue encompassing all disease in the midline (Fig.15.6). While most will
excise tissue down to the level of the post-sacral
fascia, this is not entirely necessary. Excision
down to a depth one would use in an unroong
procedure is acceptable. It must be ensured
however that the thickness of the mobilized lipocutaneous ap approximates the thickness of the
tissue that is excised. This technique works well
in the setting of complex recurrent disease. The
planned incision is marked, and after disease
excision the ap is raised with electrocautery. It
is recommended to handle the ap gently during
mobilization. It is important to take care to under-
mine the areas adjacent to the ap so that the
most tension-free closure can be obtained. Once
the ap is completely mobilized, it is centrally
anchored to the post-sacral tissues with an
absorbable suture. A closed-suction drain is
placed and a layered closure takes place using
absorbable suture. The skin can be closed using a
variety of techniques, none of which has proven
to be superior. Some surgeons will cover the nal
closure with glue to create a water-tight seal
(Fig.15.7). A modication of this procedure was
created in order to keep the caudal point of the
incision away from the anus.
The drain can be left in place for 48h or until
it has produced 20mL or less daily for 2 consecutive days. The patient should avoid any strenuous
activity for 2–4 weeks. It is not uncommon for
these wounds to separate slightly in one or two
areas over the ensuing 2weeks (Fig.15.8). These
open wounds require some minor wound care and
is usually well tolerated. Occasionally it will take
4–8weeks for the wound to completely heal. In
some cases, the disease spans a very large area
over the sacrum extending from the perianal area
Fig. 15.6 Rhomboid/Lindberg ap Fig. 15.7 Wound closure after rhomboid ap
268
Fig. 15.8 Wound separation after rhomboid ap
for a long distance cephalad. Surgeons may be
uncomfortable with creating such a large area of
excision and ap in this setting. When this trepidation is present, the technique can still be used
but may be modied. The most difcult area in
which to achieve healing is the caudal midline. An
excision can be performed, and ap created such
that the caudal midline is covered leaving an open
wound cephalad. The remaining wound can be
managed in a variety of ways, but the use of a
negative pressure wound therapy device makes
this management easy (Fig.15.9). This device can
be used in the standard fashion until the remaining wound is small enough to manage using standard dressings. The area will typically heal
quickly, and does not impair the ap in any way.
Potential surgical site related postoperative
complications include wound dehiscence, ap
necrosis, hematoma, wound infection, and
seroma. These complications occur at rates of
4%, 0–2%, 1%, 3–5%, and 3% respectively [12,
13]. Recurrence can be seen in approximately 4%
[13]. Several series have compared outcomes
associated with the Limberg ap (LF), modied
Limberg ap (MLF), and excision with primary
midline closure [14, 15, 52, 53]. The evidence
indicates that the LF or MLF is associated with
faster return to work, lower rates of surgical site
infection, lower recurrence, and lower rates of
wound dehiscence. Comparisons of the MLF, LF,
and Karydakis ap show similar superiority for
the LF and MLF [16, 17], while others have
shown equivalence [18].
E. K. Johnson et al.
Fig. 15.9 Negative pressure wound device in pilonidal
wound
Disease Recurrence
There are several known risk factors that predispose to the occurrence of PD, and many authors
have attempted to investigate factors that may
predict disease recurrence. Familial history of
disease, increased sinus number, larger cavity
diameter, and primary wound closure have been
shown to be associated with higher rates of recurrence [54]. Interestingly, tobacco smoking and
body mass index >25 have NOT been shown to
increase recurrence [55]. As stated above, the
authors recommend optimization of modiable
risk factors for surgical site occurrence, such as
smoking cessation and glycemic control prior to
performing these elective procedures, despite a
lack of robust evidence supporting this in pilonidal disease. Recurrence has been shown to be
lower in patients who undergo surgical incision
and drainage prior to denitive surgery as compared to patients who have spontaneous abscess
rupture [56]. This nding could be related to low
15 Pilonidal Disease
269
grade ongoing sepsis secondary to incomplete
drainage in those who undergo denitive surgery,
though we have no evidence to support this
directly. Along these lines, surgery performed in
the “after-hours” and potentially emergent setting has been associated with higher recurrence
rates [57]. Many publications that report on
recurrence are criticized secondary to a lack of
long-term follow up. A study of German military
members was able to demonstrate recurrence
rates that were 22% higher than previously
reported through collection of data over a longer
period of follow up [58]. Recurrences up to
20years after surgery were seen, and they recommended that studies investigating long-term outcomes should have at least 5years of follow up.
Conclusion
Pilonidal disease is a chronic inammatory
process that can present with a wide spectrum
of severity, but invariably leads to disability in
affected individuals and results in a substan-
tial decrease in their quality of life. While
treatment of this disease process can be
viewed as less “glamourous” than many oth-
ers, it most certainly results in a grateful
patient. Pilonidal disease is quite common and
any general or colorectal surgeon can be
expected to care for a number of affected indi-
viduals. In order to ensure optimal treatment
and outcomes, it is critical to tailor recom-
mendations to the severity of disease, anatomy
of disease, and our patient’s expectations of
risks and expected outcomes.
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Perianal Hidradenitis Suppurativa
EmilySteinhagen andMichaelF.McGee
16
Abbreviations
CD Crohn’s disease
HS Hidradenitis suppurativa
SCC Squamous cell carcinoma
VAC Vacuum assisted closure
Introduction
Hidradenitis suppurativa (HS) is a chronic
inammatory skin disorder characterized by
recurrent, chronic, and inammatory lesions of
terminal hair follicles in the axillae, scalp, groin,
and perineum. The pathophysiology of HS is
multifactorial, encompassing genetic, immunologic, hormonal, and infectious factors. Rather
than an isolated dermatologic condition, emerging evidence suggests HS is primarily a systemic
E. Steinhagen
University Hospitals, Cleveland, OH, USA
e-mail: Emily.Steinhagen@uhhospitals.org
M. F. McGee (*)
Department of Surgery, Division of Gastrointestinal
and Oncologic Surgery, Section of Colon and Rectal
Surgery, Feinberg School of Medicine, Northwestern
University, Chicago, IL, USA
e-mail: mmcgee1@nm.org
inammatory disease with secondary skin manifestations [1]. Compared with other dermatologic
diseases, HS profoundly impacts patient quality
of life and can inict signicant psychosocial
morbidity requiring coordinated care between
primary care physicians, dermatologists, and surgeons [2].
Epidemiology andRisk Factors
Approximately 1–4% of the population is
affected by HS [3], with a female predominance
of approximately 3:1 in most series. Typically,
HS begins after puberty [4]. HS is more commonly seen in black/African descent patients,
and it has been suggested that race/ethnicity
may impact disease severity [5]. HS patients
demonstrate higher rates of smoking, obesity,
dyslipidemia, diabetes, and metabolic syndrome
compared to healthy controls [6]. Hypertension,
thyroid disorders, psychiatric disorders, arthropathies, and polycystic ovary syndrome are also
associated with HS [3]. There is a relatively high
incidence of concomitant HS and Crohn’s disease (CD). A 2010 survey suggests up to 17%
of CD patients report a history of axillary and/
or inguinal “recurrent painful boils” suggestive
of HS [7].
© Springer International Publishing AG, part of Springer Nature 2019
D. E. Beck et al. (eds.), Fundamentals of Anorectal Surgery,
https://doi.org/10.1007/978-3-319-65966-4_16
273
274
E. Steinhagen and M. F. McGee
Pathogenesis
HS is hypothesized to originate with occlusion
of the terminal follicular acrofundibuulum of
the folliculopilosebaceous unit, leading to recurrent apocrinitis that manifests as cysts, draining
sinuses, and subsequent scar formation [8].
Aberrant immunity may play a role in HS
as it is associated with other immune-mediated
diseases such as Crohn’s disease and pyoderma
gangrenosum. Increases in peri-lesional immune
cells and TNF-α mRNA precede the clinical
development of HS [9–11]. Pro-inammatory
cytokines IL-1 and IL-17, also involved in CD
and psoriasis, have also been implicated [9, 12,
13]. T-cell deregulation has been implicated both
in the pathogenesis of CD and HS.Furthermore
the efcacy of anti-TNF-α agents for both CD
and HS suggests each entity shares, at minimum,
partially overlapping signaling pathways.
Approximately one-third of HS patients have
a familial form of HS felt to pass through an autosomal dominant pattern [14]. HS has been associated with mutation of the PSENEN gene which
encodes a subunit of the γ-secretase protein; however, mutations affecting the secretase protein are
present in only 15% of patients [15–17].
primarily based on clinical ndings, biopsy is
usually not performed. When evaluating suspected HS, differential considerations should
include cutaneous infections (e.g., furuncles,
lymphogranuloma venereum, and dermoid/epidermoid cysts), lymphadenopathy, lymphoma,
soft tissue malignancy, CD, and tuberculosis.
Crohn’s Disease andHS
There is often a ne and blurry line between
HS and perianal CD.The cumulative risk of an
inammatory bowel disease (IBD) patient developing HS is approximately 1 and 3% at 10 and
30years; and the overall risk of developing HS
in IBD is nine times that of the general population [18]. Since perianal CD and HS may be
indistinguishable, evaluation and management
of perianal disease requires experience with both
entities and the anorectum. Suspicion of perianal CD or HS with associated gastrointestinal
symptoms merits endoscopic evaluation of the
entire ileocolon. Exam under anesthesia is often
required to exclude and manage anal stula.
Imaging
Bacteria
Not surprisingly, skin ora is frequently implicated in HS, however the role of causation versus colonization versus contamination is unclear.
Staphylococcus aureus is the most commonly
implicated organism in HS. Coagulase negative staphylococci, streptococci viridans, beta-
hemolytic streptococci, and enterococci make
up the majority of other bacteria involved in
HS. Commonly, skin swab cultures of infected
HS lesions reveal normal skin ora and may not
reect subcutaneous pathogens, which may misguide initial antimicrobial treatment.
Dierential Diagnosis
HS is diagnosed clinically upon appearance,
location, and chronicity. Since HS is diagnosed
Clinical assessment, based on palpation and visual
inspection, guides treatment for the majority of
HS patients; however, peri-lesional inammation, induration, and scarring may overestimate
the burden of HS. Imaging has been selectively
used by some groups to characterize the true
nature and extent of HS involvement. Imaging
may be used pre-operatively to develop a surgical
strategy by mapping subcutaneous inammation,
sinuses, and stulae [19]. Duplexed ultrasound
permits visualization of the skin and subcutaneous structures and characteristic peripheral
hypervascularity surrounding uid collections
and stulae in HS. Imaging proponents report
that routine use of duplexed ultrasound altered
management in over 80% of patients initially
assessed clinically, with the majority of clinical examinations underestimating disease severity [20]. Imaging may be useful in the perianal
region where HS may be confused with CD, or
16 Perianal Hidradenitis Suppurativa
275
in cases where both are present and need to be
distinguished. MRI, CT, stulography, and endoanal ultrasound (US) may all be useful to assess
the location and origin of stulas and sinus tracts.
Medical Treatment
Antibiotics
Spartan high-quality evidence supports the use
of antibiotics to treat HS absent infection or
cellulitis. Tetracycline, clindamycin, and other
combinations of systemic antibiotics are initially
useful when there are widespread areas of infection. Topical clindamycin 1% solution decreased
the number of abscesses, inammatory nodules,
and pustules in HS patients [21]. A pilot study
revealed a decrease in HS severity when patients
were treated with a 6week course of ertapenem,
and continued to improve when transitioned to a
“consolidation” regimen of rifampicin/moxioxacin/metronidazole for 6weeks [22]. This strategy may be used to effectively downstage disease
burden to facilitate a more focused surgery.
Steroids
Intralesional corticosteroid injections have been
used to treat acute ares and persistent nodules
with a clinical response within 2–3 days [23].
While somewhat effective, intra-lesional steroid
injections are relatively contraindicated when
local super-infection is suspected, and injections
may cause skin pigment changes and telangiectasia. Systemic corticosteroid “bursts” with
a rapid taper can be used for acute HS ares,
but long- term use of systemic steroids is not
recommended.
Anti-TNF Agents
Anti-TNF-α agents can effectively treat
HS. Iniximab has been shown to decrease HS
severity by over 50% and improve quality of life
in HS patients [24]. Similarly, adalimumab effectively decreased HS disease severity in 80% of a
154 patient trial [25]. Etanercept showed some
promise in small cohort studies [26, 27], but a
randomized double blind trial showed no difference after 12weeks of treatment [28]. Currently,
adalimumab is the only FDA approved TNF-α
agent for treating HS.Notably, however, the efcacy of anti TNF-α agents in patients with HS
and concomitant inammatory disorders may
be decreased when compared to those with HS
alone [29]. As a result, patients with HS and concomitant inammatory disease frequently require
dose escalations [30].
Surgical Treatment
Surgery offers both acute temporization of HS
via incision and drainage and long-term cure via
wide local excision of affected skin. Wide local
surgical excision is the most effective treatment
for severe or advanced HS and in many cases,
offers the only hope at cure [4, 31]. Consequently,
some have advocated early and aggressive surgical treatment of HS, given the relative futility of
medical treatments [32]. For many patients suffering with intractable HS, the punishment of a
sometimes large surgery and protracted recovery
is worth the crime of chronic suffering inicted
by a chronic and otherwise non-curable disease.
Incision and drainage is commonly performed
to relieve pain and control active infection. Since
drainage alone does not address the underlying
inammatory process, incised HS lesions tend
to recur. Surgical un-roong implies opening a
lesion more fully to remove keratinous debris on
the lesion oor [33, 34]. Un-roong is typically
guided by probing sinus openings to explore the
extent of the lesion(s). The roof of each structure
is excised to allow debridement of granuloma and
purulence whereby exposing the epithelialized
lesion base, and the wound heals secondarily.
Un-roong is generally recommended for limited
and focal disease as it may spare more tissue than
wide local excision [35].
Wide local excision removes the entirety of
active and at-risk HS tissue, not just the actively
infected areas (Fig. 16.1). The breadth of excision is determined by the size of the lesion and
the distance between adjacent lesions; while the
276
E. Steinhagen and M. F. McGee
a
b
Fig. 16.1 (a) Pre-operative perianal, inguinal, and scrotal
hidradenitis suppurativa. (b) Following wide local excision. Photos courtesy of Randolph M.Steinhagen, MD
depth is to the portions of subcutaneous fat deep
to all inammatory tracts. Since there is no benet to extra-wide margins, 1cm margins are adequate [32]. Wide or radical excisions should be
performed cautiously in areas with neurovascular
structures such as the axilla and inguinal regions.
Un-roong is acceptable in areas where excision
is not feasible due to anatomic constraints, such
as the perigenital skin.
After limited excision, HS may recur in
43% of patients with a disease-free interval of
11months. HS recurrence following wide excision was 27% after 20 months [36]. In a large
series of 182 patients undergoing 229 excisions,
60% of patients had resolution of HS at 1 year
follow up, but, 32% noted interval development
of lesser severe lesions at the same or adjacent
site. It should be noted that in the same study,
nearly 9% of patients experienced no improvement in symptoms despite wide excision, likely
owing to the difculty in treating this entity [37].
Excisions can leave large wounds that must be
either closed, reconstructed with tissue transfer techniques, or allowed to heal secondarily.
Appropriate pre-operative planning, often in
conjunction with a plastic surgeon, can assist
with determining an effective strategy for wound
management.
Following excision, primary closure may be
employed for small defects that approximate
well with no tension. When possible it may confer faster healing, and improved cosmetic and
functional outcomes; however high recurrence
and infection rates render primary closure a
suboptimal treatment in most cases. Moreover,
primary closure should be avoided in high tension or inamed areas—which is often present in
most cases of chronic HS.Wounds can be closed
loosely to allow for outow of drainage as necessary or packing with wicks between stitches
or staples can be employed [32]. Meta-analysis
demonstrated the recurrence rates for wide excision, local excision, and un-roong to be 13%,
22%, and 27%, respectively [38].
Healing by secondary intention will occur
in even the largest wounds but can be a time
and resource consuming process for patients.
Secondarily intended healing occurs through
wound granulation, contraction, and epithelialization. Following several weeks to months, the
nal scar from secondary healing is typically
considerably smaller than the initial defect [39].
In areas of joint mobility, such wound and scar
shrinkage rarely can cause joint contractures. The
use of vacuum assisted closure (VAC) devices has
been described for large, open wounds after excision for HS. VAC therapy is postulated to shorten
the time to wound closure, decrease bacterial
counts, control wound uid losses, and promote
granulation tissue growth. However, VAC placement may often be challenged with HS since the
inguinal, axillary, and perianal regions may be
extraordinarily difcult to sustain an adequate
dressing seal.
Skin grafts are an option for coverage of larger
defects. Split-thickness, meshed grafts are commonly used. The benets include low risks for
serious complication and the functional outcome