Introduction
Scrotal lymphedema, a rare entity of lymphedema, is defined as abnormal accumulation
of a protein-rich fluid in the soft-tissue region of the scrotal/penoscrotal territory
coupled with gross deformation of the genitalia[1] because of a derangement of the lymphatic drainage. Lymphedema is classified as
primary (idiopathic) or secondary according to its etiology. Primary lymphedema is
caused by an intrinsic defect of the lymphatic vessels, while secondary lymphedema
may occur after surgery, radiation, tumors, and infections.[2]
Scrotal lymphedema produces mobility and voiding limitations, fatigue, pain, and recurrent
subcutaneous infections because of the difficulty of self-hygiene. It also causes
sexual limitations and social isolation and impairs quality of life.[3]
[4]
[5] Therapy is usually initiated by conservative measures, for example, the complex
decongestive physiotherapy (CDP) first described by Földi, including skin hygiene,
manual lymph drainage, compression bandages, and therapeutic exercises.[6]
[7] However, many patients experience either unsatisfactory results or recurrence even
after minor size reduction.[8]
[9]
Reported methods of surgical reconstruction involve either lymphangioplasty or complete
excision of lymphedematous tissue with local tissue reconstruction in case healthy
skin is available. Other methods of reconstruction will include essentially advancement
flaps, rotational flaps, and split- or even full-thickness free flaps, whose results
in such area exposed to many commensals and uncontrollable hygiene were questionable.[3]
[10]
It has been established that surgical reduction of scrotal lymphedema achieves a significant
improvement in quality of life provided the genitourinary anatomy and physiology remains
unaltered.[11]
In this series of challenging cases, we aimed to share our experience with functional
scrotal reduction without using advancement, rotational, or free flaps while preserving
the structure and function of the penoscrotal region, and demonstrating its impact
on the patient wellness.
Idea
In this retrospective study, we present our experience with huge scrotal lymphedema
cases that were treated with surgical reduction. All patients consented verbally and
formally for photographing their pre- and postoperative conditions and their approval
was submitted in the Institutional Research Board of the Faculty of Medicine, Mansoura
University, under approval number R/18.12.373.
Operative Technique
Our surgical technique was previously described in our published case reports of huge
scrotal lymphedema patients who underwent successful reduction scrotoplasty despite
having a huge-sized scrotum; these cases were included in our case series.[12]
[13] It depended principally on the presence of surrounding healthy scrotal skin that
allowed sufficient flaps opposition and suffice wound healing without involving complex
regional rotational, advancement, or free flaps that rendered the healing questionable.
The penile urethra was secured by a Foley catheter prior to the commencement of the
procedure and remained there postoperatively to facilitate voiding for 3 to 10 days
postoperatively.
Our classic “smilelike” incision ([Fig. 1]; [Supplementary Video S1], Debulking technique for huge scrotal lymphedema management, available in the online
version only) extended typically short below the external inguinal ring from one side
to the other, curving 1 inch below the penoscrotal sulcus until it reached the other
side. The lower incision extended between the exact two previous points but was taken
down and posteriorly to involve much of the scrotal skin to be excised and underlying
lymphedematous tissue.
Fig. 1 Our classic smile incision and creation of the upper and lower flaps from anterior
and lateral views (A, B), and postoperative images after closure of the skin (C, D).
Modification to such incision was undertaken in cases that exhibited significant lymphedematous
tissue in the suprapubic region, which obliterates the penopubic junction and buries
the penis, displacing the perpetual opening downward. In such cases we designed the
anterior flap from the over-hanged skin, excess subcutaneous tissue removed, the dissected
penis with small scrotal skin cuff was transposed proximally to its anatomical site
either brought out through a button hole or after dividing the anterior flap in the
mid-line converting it to a butterfly, the penis with its cuff was repositioned between
the two wings ([Fig. 2]).
Fig. 2 Alternate incision in case of excess lymphedematous suprapubic tissue.
After creation and dissection of the flaps, the testicles and cord components were
carefully dissected ([Fig. 3]) and secured by vessel loops until the time of flap closure. The lymphedematous
tissue was excised principally from the anterior scrotal surface area and was removed
en bloc ([Fig. 4]).
Fig. 3 Dissection and isolation of the testicles and cord in a case with concomitant bilateral
hydrocele.
Fig. 4 En bloc excision of the anterior scrotal skin together with the lymphedematous tissue
after smilelike incision.
The tunica vaginalis was everted routinely. However, in cases with concomitant hydrocele,
it was excised subtotally, thus promoting lymphatic drainage of the scrotal skin via
testicular lymphatics into the nondiseased para-aortic lymph nodes.[14]
The scrotal septum was preserved but thinned out. Then, the flaps were reassessed
and trimmed to provide the least possible size after completion of scrotoplasty. V-excision
was performed from the lower flap if required. Closure was performed by means of a
skin stapler afterward ([Fig. 5]), and draping tapes with scrotal elevation were used to minimize edema.
Fig. 5 Thinning of the scrotal septum and commencement of flap opposition (A), then closing the skin with staples (B), and packing both sides of the scrotum (C).
In the case of coexisting penile lymphedema, the stretched healthy preputial skin
was used to cover the penile shaft; in case of shortage of preputial skin, we covered
the rest of penile shaft with local skin flap ([Fig. 6]).
Fig. 6 A 65-year-old man presented with huge scrotal and penile lymphedema, 20 years ago
after two debulking surgeries, and concomitant left lower limb lymphedema (A). The maximum diameter of the swelling was 92 cm (B). 18 months postoperative follow-up image (C).
Follow-up
During the follow-up period, we encouraged our patients to commit to a self-management
disciplinary program to maintain best outcomes. Our protocol comprised four components:
(1) improving self-hygiene and thorough skin care; (2) self-performed manual lymphatic
drainage; (3) deep breathing exercises (it had a lymphokinetic action); and (4) self-performed
manual intermittent scrotal compression. In our novel method of scrotal compression,
the patient was instructed to lie down on a longitudinally folded linen, half of it
underneath his back and buttocks and the rest of it passing under the patient's lap
underneath the scrotum. The patient himself pulls the folded linen cranially, compressing
the scrotum for 1 to 2 minutes, and then releases it, repeating for 10 times.
Patients had weekly follow-up visits during the first month, then every 3 months until
the first year, and then annually until 3 years of follow-up, where serial circumferential
measurements of the scrotum were taken ([Supplementary Appendix 1], available in the online version only). Assessment of voiding capacity and sexual
functionality (in sexually active individuals) was done in all patients in all visits
through some questions, which the patients answer with yes/no ([Supplementary Appendix 2], available in the online version only). Satisfaction after the operation was assessed
using the Glasgow Benefit Inventory (GBI), which was filled by the patients themselves
after the end of follow-up period, as adopted by some authors previously.[15]
The results were assessed by the GBI, an established questionnaire that assessed how
a procedure had altered the quality of life of the patient. The GBI not only allowed
the calculation of “total” quality of life, as influenced by the intervention, but
also permitted a breakdown of the results into “general subscale,” “social support
subscale,” and “physical health subscale.”
The score was calculated as follows: the sum of the responses is first divided by
the number of questions in the respective subscale to get an average response score.
From the average response score, three was subtracted and the result is multiplied
by 50. This gave a score between −100 and 100, with −100 being the worst possible
change, 0 no change, and 100 the best possible change.[15]
Statistical Analysis
Numerical values were described as mean and standard deviation when normally distributed
and as median and interquartile range if abnormally distributed. Repeated measures
for the entire sample pretreatment, after CDP, after surgery, and in each follow-up
visit were recorded and analyzed using SPSS (version 22.0) according to the appropriate
test.
Results
From January 2014 until July 2018, we performed 18 successful scrotal and penoscrotal
functional reduction surgeries for cases condemned to be hopeless because of the apparently
huge scrotal diameter or the long-standing condition. The ages of our patients ranged
from 14 to 65 years, with a median of 30 years. The maximum scrotal diameter ranged
from 48 to 92 cm with a median of 61 cm preoperatively at the time of presentation.
We had seven cases associated with lower limb lymphedema; two of these were diagnosed
as lymphedema congenita, while five were considered as lymphedema praecox. One patient
had trisomy 21 and was on corticosteroid therapy, one patient had giant cell nevus
syndrome, one patient had Rosai–Dorfman disease, and one patient had secondary lymphedema
that developed postradical cystectomy 6 years before our intervention. Three patients
had concomitant testicular hydrocele and two patients had penile lymphedema as well.
Two patients had recurrent scrotal swelling after previous surgical excision.
Successful reduction from a median size of 61 cm (range, 48–92 cm) to 25 cm (range,
21–29 cm) (p < 0.0001) with total preservation of the genitourinary anatomy was attained immediately
postoperatively. During the follow-up period, which stretched until 72 months postintervention,
the size varied between slight insignificant decreases and increases ([Table 1]). Such significant reduction in size remained until the end of the follow-up period
(median, 26; range: 22–34 cm) (p < 0.0001 between the maximal scrotal diameter preoperatively and 3 years postoperative).
In two patients, the penile skin was grossly damaged, so we used the stretched preputial
skin to cover the penis in one case and a local flap constructed from the hairless
stretched penopubic junction for the second case. The median operative time was 160 minutes
(range, 120–360), and the median total estimated blood loss was 200 mL (range, 120–750).
Table 1
Median, minimum, and maximum of the maximal scrotal preoperatively and throughout
the follow-up period
|
Diameter
|
|
|
Time
|
Median (cm)
|
Minimum
|
Maximum
|
p-Value
|
|
Preoperative
|
61
|
48
|
92
|
<0.0001
|
|
Postoperative
|
25
|
21
|
29
|
|
3 mo
|
29
|
25
|
39
|
|
6 mo
|
28
|
24
|
35
|
|
9 mo
|
28
|
24
|
32
|
|
24 mo
|
28
|
23
|
32
|
|
36 mo
|
26
|
22
|
34
|
Two patients developed wound dehiscence, which caught minor infection in one patient
and was clean in the other. Both were treated with systemic antibiotics besides local
wound care and repeated dressing until healing by secondary intention was achieved.
Two other patients developed minor flap edge necrosis, which also was handled conservatively
under antibiotic coverage until secondary closure was safe.
All patients showed absolutely no problems with micturition. Among those who were
sexually active, all except four had significantly more satisfactory intercourse postoperatively.
Therefore, answers for posed questions in the box ([Supplementary Appendix 2], available in the online version only) were positive for the first 3 questions for
14 patients. All patients experienced no chronic postoperative pain after the surgery
(replied negatively for the fourth question in the box) ([Supplementary Appendix 2], available in the online version only). Scrotal duplex showed testicular vascularity
was unaffected in all cases. The previous factors were assessed in each postoperative
visit by answering the questions in box ([Supplementary Appendix 2], available in the online version only). Scrotal duplex was performed during the
first postoperative visit only.
The GBI was used at the end of the follow-up period to assess the true benefit of
functional scrotal reduction and its impact on the physical wellbeing and the social
support the patient receives after the operation. The analysis of the GBI questionnaire
was performed by members of our team other than those involved in the scrotal reduction
operation. The total score was 55.5 (range, 50–72). It showed that the physical wellbeing
increased by a median of 16.6 points (range, 16–33 points), the social support the
patients received increased by a median of 100 points (range 50–100 points), and the
general wellbeing subscale also showed an increase by a median of 79 (range, 70–83
points) ([Fig. 7]).
Fig. 7 Box and whisker plot for the total, general, physical, and social subscales of the
GBI.
Discussion
The problem of lymphedema affecting the region of the scrotum and penis can lead to
significant harm on the patients' personal hygiene, sexual capability, and desire
to participate and interact with the surrounding environment. Also, faulty medical
practice of treating such cases conservatively despite the absence of an obvious improvement
can lead to significant despair that withholds the patient from seeking proper medical
advice.
We presented 18 challenging cases of scrotal lymphedema that suffered from huge diameters
of the scrotal sac (smallest was 48 cm). The fact that we pose certain conditions
to define functional scrotal reduction surgery is derived from our caution to preserve
the genitourinary functions of the penis and scrotum despite the size. We were never
bailed out to orchidectomy or urinary diversion in any case no matter how deep the
penis was buried or the genitalia were deformed.
The presence of minimal surface area of healthy scrotal skin was mandatory to reconstruct
the scrotum without needing advancement, rotational, or distal split- or full-thickness
skin flaps.[16]
[17]
[18] In our technique, we reconstructed the new scrotal sac mainly using the posterior
flap. This posterior flap resembles the most healthy area in scrotal lymphedema (the
uppermost stretched posterior skin by the hanged swelling with less edema). This condition
was questionable in two cases (one patient with Rosai–Dorfman disease and one patient
with giant cell nevus syndrome) because of the presence of unhealthy velvety scrotal
skin that rendered healing doubtful; however, successful reconstruction was also attained
according to our conditions.
The significant reduction in the size of the scrotum postintervention was maintained
throughout the follow-up period that continued for 3 years. Slight alteration in the
median size in between was insignificant and was attributed to development of postoperative
edema, and its resolution over time until the follow-up period was considered complete.
Our protocol, which was based on self-management discipline, gave satisfactory outcomes
maintained throughout the follow-up period, avoiding slow gradual recurrence. We did
not subscribe compression hosiery or garments (shorts, boxers, pouches, support, or
pads) to any of our patients, thus avoiding the costly, painfully tight garments with
its bad hygienic effect, thus allowing our patients to resume a normal life.
The improvement of the physical, sexual, and voiding capacity was investigated in
each postoperative visit by asking the patient to respond to four questions (whether
he can void freely/whether his sexual performance was improved/whether he can walk
freely/whether he suffered any chronic postoperative pain) and the reply was positive
for all questions for all patients for the first three questions except for four patients
who were sexually inactive. Patients also denied the presence of any chronic regional
postoperative pain after the surgery (question 4). We went further to investigate
the overall benefit from scrotal reduction surgery using the GBI, adopted by some
authors in previous similar work.[5] This also conveyed excellent results on the physical wellbeing, the social support
received, and overall performance. The social support the patients received increased
by 100 points (range, 50–100). Many of them were inclined not to participate in social
activities and were less confident in job opportunities. This has changed radically,
for instance.
The physical wellbeing also improved by a median of 16.6 points (range, 16–33 points),
perhaps because the three questions of physical wellbeing targeted the intake of drugs,
family doctor visits, and frequency of cold infections. This is why, the impact was
not evident as in the social support sector, which was more detailed. The general
wellbeing sensation increased as well by a median of 79 points (range, 70–83 points),
surpassing similar studies using GBI in assessment.
The technique adopted by our lymphedema team was developed over a long period of time
and thorough experience with a huge number of lymphedema cases either in the extremities
or in the scrotum. For example, we adopted the smilelike incision and its butterfly
modification to create flaps that would oppose without the risk of flap ischemia or
necrosis such that minimal postoperative care would be required postintervention.
The appropriate repositioning of the penile shaft in the exact or near-exact anatomical
position is crucial for hygienic voiding. Therefore, the modified butterfly incision
was used in cases where penile shaft would be extremely downward displaced after closure
of the flaps. Also, V-incisions to excise excess redundancy of posterior flap were
used according to the operator discretion whenever needed. The appropriate repositioning
of the penis and its stretching from the surrounding tissue would ensure better voiding
and intercourse functions. According to our experience, surgery remains the gold standard
treatment for the management of huge scrotal lymphedema. Successful preservation of
the genitourinary functions can be attained despite the size in most cases with excellent
cosmesis. Our study is limited by its retrospective nature and the relatively few
numbers of cases, which might have distorted the statistics. However, since we were
dealing with a relatively rare entity and discussing only cases whose maximal scrotal
diameter is approximately 48 cm in the mildest case, such number is acceptable.
