Keywords
extramammary Paget's disease - squamous cell carcinoma - vulvar reconstruction
Introduction
Complex vulvar defects are challenging owing to their three-dimensional nature and
proximity to the vaginal, urethral, and anal orifices. Our previous series outlined
the subunit principle of vulvar reconstruction, where a maximum of two flaps were
used.[1] This series highlighted that any reconstruction plan must provide external coverage
and inner lining, and avoid disruption of critical structures. The purpose of this
paper is to demonstrate the utility of three or more flaps to reconstruct more challenging
defects. We define complex defects as those which are bilateral, involving both vaginal
and anal orifices, and requiring three or more flaps to achieve a functional and aesthetically
acceptable outcome.
Cases
Four patients who underwent reconstruction for extensive vulvar defects from extramammary
Paget's disease (EMPD; n = 3) and squamous cell carcinoma (n = 1) resections were studied ([Table 1]). The defects' sizes ranged from 108 to 157 cm2; all involved the vaginal wall, anal canal, and perineum. We define the perineum
as the patch of skin between the vulva and anus. The data collected were anonymized
and recorded with informed consent from the patients. Patients' satisfaction after
surgery was assessed using a questionnaire ([Appendix 1] [available in the online version only]), which was adapted from gynecological and
urological sources.[2]
[3]
[4] The flaps included the gluteal fold VY advancement flap as the primary workhorse
flap,[5]
[6] gracilis muscle flap, gracilis medial thigh VY advancement flap, mons pubis rotation
flap, and buttock VY advancement flap ([Fig. 1]). There were no major flap complications. The average follow-up period was 7 years.
Two patients had superficial infections that responded to topical antifungals and
intravenous antibiotics. The results of the patient satisfaction questionnaire are
shown in [Table 2].
Table 1
Patient demographic data and outcomes
|
Case number
|
Age (years)
|
Diagnosis
|
Defect type
|
Defect size (cm2)
|
Type of reconstruction
|
Colostomy
|
Complications
|
|
1
|
64
|
EMPD
|
Bilateral, involving mons pubis, vulva, vagina, and anal verge
|
157
|
Bilateral gluteal fold VY advancement flaps, bilateral gracilis muscle flaps, and
SCIP flap
|
Yes
|
Fungal infection (treated topically)
|
|
2
|
40
|
EMPD
|
Bilateral, involving vulva, introitus, and anal verge
|
108
|
Bilateral gluteal fold VY advancement flaps and pudendal thigh flap
|
Yes
|
Fungal infection (treated topically)
|
|
3
|
43
|
SCC
|
Bilateral, involving vulva, introitus, vagina, and anal canal
|
123
|
Bilateral gluteal fold VY advancement flaps and buttock VY advancement flap
|
Yes
|
Bacterial infection (treated with antibiotics)
|
|
4
|
60
|
EMPD
|
Bilateral, involving mons pubis, vulva, introitus, vagina, anal verge, and inner thigh
|
124
|
Bilateral gluteal fold VY advancement flaps, mons pubis rotation flap, and medial
thigh VY advancement flap
|
No
|
Nil
|
Abbreviations: EMPD, extramammary Paget's disease; SCC, squamous cell carcinoma; SCIP,
superficial circumflex iliac perforator.
Fig. 1 Types of local flaps used in complex vulvar defects. (1) Gluteal fold/buttock VY
advancement flaps. (2) Gracilis muscle flaps. (3) Medial thigh VY advancement flap.
(4) Pudendal thigh flap. (5) Superficial circumflex iliac perforator flap.
Table 2
Patient satisfaction outcomes
|
Case number
|
Micturition
|
Defecation
|
Sexual function
|
Vaginal orifice
|
Scarring/Preservation of the vulvar subunits
|
|
1
|
Smooth, undeviated stream
|
Normal fecal caliber
|
Not applicable
|
Mildly constricted
|
Satisfied
|
|
2
|
Smooth, undeviated stream
|
Normal fecal caliber
|
Not applicable
|
Normal opening
|
Satisfied
|
|
3
|
Smooth, undeviated stream
|
Normal fecal caliber
|
Intercourse without lubrication
|
Normal opening
|
Satisfied
|
|
4
|
Smooth, undeviated stream
|
Normal fecal caliber
|
Not applicable
|
Normal opening
|
Satisfied
|
Case 1
A 64-year-old patient presented with EMPD involving the mons pubis, vulva, vagina,
and anal verge circumferentially ([Fig. 2]). Five flaps were used in two stages to reconstruct the defect. Firstly, bilateral
gracilis muscle flaps were mobilized to line the introitus and create a partition
between the anus and the vagina. The muscle flaps were skin-grafted. Secondly, bilateral
gluteal fold VY advancement flaps were used to reconstruct the perianal skin and the
lower two-thirds of the vulva. The mons defect was temporarily covered with topical
negative pressure wound therapy (NPWT) and prepped for closure at a second stage.
Two weeks later, an extended super-thin superficial circumflex iliac perforator (SCIP)
flap was raised to cover the mons pubis and the upper vulva bilaterally. Urinary and
fecal diversion by means of a urinary catheter and loop colostomy kept the wounds
clean. A month later, flap and scar revision were performed under local anesthesia.
The colostomy was reversed after 3 months. Evaluation of her perineal function at
2 years showed smooth and undeviated urinary passage, normal fecal caliber, a smaller
introitus, and no problems with feminine hygiene. The patient was satisfied with her
physical and functional outcomes.
Fig. 2 Patient of case 1. (A) Defect after EMPD resection. (B) The right gracilis muscle flap was mobilized. The left gracilis muscle was transposed
medially to the defect. (C) The paired gracilis muscle flaps lining the introitus and vagina, before the application
of skin grafts. The muscle flaps were sutured together at 6 o'clock to create the
anovaginal septum. (D) Paired gluteal fold VY advancement flaps were mobilized to reconstruct the perianal
skin and lower vulva. (E) A left SCIP flap was used to reconstruct the mons and the upper vulva. (F) Perineal view, 2 years postoperatively. (G) Buttock view, 2 years postoperatively. EMPD, extramammary Paget's disease; SCIP,
superficial circumflex iliac perforator.
Case 2
A 40-year-old, obese patient presented with bilateral EMPD involving the vulva, introitus,
and anal verge circumferentially ([Fig. 3]). Following wide resection, three flaps were used in two stages to reconstruct the
defects. First, the perianal skin and lower vulva were bilaterally reconstructed using
paired VY advancement gluteal fold flaps. Then, a pedicled super-thin pudendal thigh
flap was raised to cover the residual left vulva defect since the left gluteal fold
flap could not reach the mid-vulva due to its adiposity.[7] She developed a postoperative cutaneous candidal infection, which responded to topical
antifungals. As in case 1, she had a urinary catheter and loop colostomy to maintain
hygiene. Evaluation at 6 months showed normal perineal function. She remained virgo
intacta.
Fig. 3 Patient of case 2. (A) EMPD involving the vulva and perianal skin bilaterally. (B) Paired gluteal fold VY advancement flaps were mobilized. (C) A pudendal thigh flap was used for the residual vulvar defect. (D) Result 6 months postoperatively. EMPD, extramammary Paget's disease.
Case 3
A 43-year-old female presented with high-grade squamous intraepithelial carcinoma
bilaterally, involving the vulva, introitus, vagina, and anal canal ([Fig. 4]). She was sexually active. Furthermore, she had cervical and vaginal intraepithelial
neoplasia III due to human papillomavirus 16. Tumor resection constituted laser ablation
of cervical and vaginal lesions, a lower third vulvectomy that included the posterior
vaginal wall, and circumferential excision of perianal skin including anal mucosa
below the dentate line. Reconstruction was achieved in two stages. Firstly, the lower
vulva and posterior vaginal wall were reconstructed using bilateral gluteal fold VY
advancement flaps, which were crucial in recreating a partition between the vagina
and anus. The anus was temporarily dressed with topical NPWT and the diverting colostomy
kept it clean. Three weeks later, a right buttock VY advancement flap was used to
resurface the anus. The folded “horns” of the advancement flap covered two-thirds
of the anal canal, and the remaining third was left to heal by secondary intention.
Her wounds were superficially infected with Escherichia coli and Enterococcus faecalis, which responded to systemic antibiotics. The colostomy was reversed 3 weeks after
her anal canal had completely healed. Evaluation at 7 years showed normal perineal
function. She had resumed sexual activity and was satisfied with her physical and
functional outcomes.
Fig. 4 Patient of case 3. (A) Defect after resection of squamous cell carcinoma in situ. (B) Bilateral gluteal fold VY advancement flaps were mobilized to reconstruct the middle
and lower vulva, and the vaginal wall. (C) A right buttock VY advancement flap was used to line the anal canal. (D) Result 7 years postoperatively. The dilator shows patency of the introitus and vagina.
Case 4
A 60-year-old female presented with bilateral EMPD involving the mons pubis, vulva,
introitus, vagina, anal verge, and the inner thigh ([Fig. 5]). Following a wide resection, three flaps were used to reconstruct the defects.
Initially, bilateral gluteal fold advancement flaps were used to reconstruct the lower
vulva, and a mons skin rotation flap was mobilized to close the wound superiorly.
Postoperatively, her wounds healed uneventfully, although her right margins were positive
for EMPD. Six months later, she underwent a repeat resection over the right neo-vulva
and coverage with a right gracilis medial thigh VY advancement flap. Evaluation 19
years postoperatively indicated normal perineal function. The patient was satisfied
with her physical and functional outcomes.
Fig. 5 Patient of case 4. (A) Defect after resection of EMPD. Gluteal fold flaps were marked as shown. “X” denoted
the loudest audible perforator signal detected by the Doppler probe. (B) Paired gluteal fold flaps and a mons pubis rotation flap completely cover the defects.
(C) A gracilis medial thigh VY advancement flap was subsequently used for EMPD recurrence.
(1) Gluteal fold flap; (2) mons rotation flap; and (3) gracilis medial thigh VY advancement
flap. (D) Result 19 years postoperatively, showing a centrally located and patent urethral
meatus. The vagina was patent and hidden. EMPD, extramammary Paget's disease.
Discussion
The basis for our approach was to recreate native anatomical boundaries. When the
defect extends from the vulva to the anus, the perineum must be reconstructed to avoid
merging of the orifices. Otherwise, there would be no separation of excretory passages
and vaginal secretions. Thus, simply approximating skin to close a defect was avoided.
From a lithotomy view, the vulva could be divided into three segments based on flap
options[8]: the upper third, consisting of the mons pubis and the upper labia; the middle third,
consisting of the labia proper; and the lower third, consisting of the lower vulva
and perineum. The first segment was covered by the lower abdominal rotation flap or
medial thigh VY advancement flap, while the last two segments were covered by gluteal
fold flaps.[5]
[6] An adjacent area, constituting the perianal skin and anal canal, was covered using
gluteal fold and buttock VY advancement flaps.
Case 1 was challenging because it necessitated the reconstruction of an anovaginal
septum. The approximated gracilis muscle flaps in a “sphincter configuration” were
useful for this purpose ([Fig. 2C]). The flaps were overlaid with skin grafts to line the vaginal wall. Paired gluteal
fold VY flaps were then advanced over it to reconstruct the perianal skin, perineum,
and lower vulva. A fifth flap from the left groin was raised to reconstruct the mons
pubis and line the periurethral area. Attention was specifically directed towards
preserving the periurethral lining to prevent scarring around the urethra. Although
the patient was not sexually active, preservation of the introitus was necessary for
maintaining vaginal hygiene. In case 2, apart from the paired gluteal fold flaps,
an additional pudendal thigh flap was mobilized to preserve the bi-crescentic fullness
of the labia majora. Sexual function took precedence for case 3, thus the paired gluteal
fold flaps were prioritized for vulva and vagina wall reconstruction. After flap healing,
the anus was reconstructed with a buttock advancement flap. Case 4 illustrated the
utility of the medial thigh VY advancement flap in EMPD recurrence, since bilateral
gluteal fold flaps were expended in the first operation.
Cho et al.[9] described the “three-directional” local flap approach for reconstruction of an extensive
perineal defect using a pedicled gracilis myocutaneous flap for the deeper and bulkier
portion of the defect, and other fasciocutaneous flaps for the thinner and shallower
portions. Additionally, they highlighted advantages of using multiple flaps, such
as being less prone to infection, and providing padding and pliability - qualities
not seen with skin grafting. Single large flaps, including the anterolateral thigh
flap[10] and deep inferior epigastric perforator flap,[11] have been used for such cases, but they are bulky and do little to mimic natural
contours.
Topical NPWT was used liberally, as it promoted flap adherence to the wound bed. Achieving
a seal was not difficult because of fecal and urinary diversion. This way, raw wounds
were never left exposed as they can be easily colonized. Other nursing adjuncts included
cage cradle nursing with an incorporated ventilation fan and in-bed off-loading exercises
to prevent pressure injury.
In the rehabilitative period, the patients were taught perineal hygiene using a mirror,
since sensory feedback is altered after surgery. Self-digital dilatation of the introitus
and anus was initiated to promote tissue pliability. Reassurance was given that sexual
activity could be resumed, aided by lubrication, after complete wound healing (case
3). From 3 to 6 months postoperatively, preparation for colostomy reversal entailed
biofeedback exercises to improve coordination and relaxation of the anal sphincter.[12]
Conclusion
In this multi-flap approach, paired gluteal fold flaps were our workhorse flaps. Additional
flaps were employed as necessary, with the aim to maximize functional and aesthetic
outcomes.
