Journal of Pediatric Surgery 49 (2014) 1647–1651
Contents lists available at ScienceDirect
Journal of Pediatric Surgery
journal homepage: www.elsevier.com/locate/jpedsurg
Male genital lymphedema: clinical features and management in 25
pediatric patients☆,☆☆
Carolyn C. Schook a, c, Ann M. Kulungowski b, c, Arin K. Greene a, c, Steven J. Fishman b, c,⁎
a
b
c
Department of Plastic and Oral Surgery, Boston Children's Hospital, Boston, MA 02115, USA
Department of Surgery, Boston Children's Hospital, Boston, MA 02115, USA
Vascular Anomalies Center, Boston Children's Hospital, Harvard Medical School, Boston, MA 02115, USA
a r t i c l e
i n f o
Article history:
Received 13 February 2014
Received in revised form 26 May 2014
Accepted 29 May 2014
Key words:
Genital
Lymphedema
Swelling
Lymphatic malformation
Vascular anomaly
a b s t r a c t
Purpose: Genital lymphedema in the pediatric population is poorly understood. The purpose of this study was
to determine the epidemiology, morbidity, and treatment outcomes for males with genital lymphedema.
Materials and methods: Male patients with genital lymphedema evaluated at our vascular anomalies center
between 1995 and 2011 were reviewed. Etiology, age-of-onset, location, morbidity, and treatment were
analyzed.
Results: Of the 3889 patients with vascular anomalies, 25 (0.6%) had genital lymphedema: 92% (23/25) with
primary and 24.0% (6/25) with familial/syndromic lymphedema. For primary disease, the mean age-of-onset
was 4.5 ± 6.3 years with 60.9% (14/23) presenting in infancy, 13.0% (3/23) in childhood, and 26.1% (6/23) in
adolescence. Combined penoscrotal lymphedema was identified in 72.0% (18/25) of patients; 19 children
(76.0%) had concomitant lower extremity involvement. The most common complication was cellulitis
(24.0%). Surgical contouring was performed in 44.0% (11/25) of patients. Patients with operative intervention
and follow-up (n = 6) had sustained improvement after a median of 4.2 years (range: 0.3–11.0).
Conclusions: Lymphedema of the male genitalia is typically idiopathic. Most patients develop swelling in
infancy but can present in adolescence and occasionally childhood. The penis and scrotum are usually both
involved and concurrent lower-extremity swelling is common. Surgical debulking can improve symptoms
and appearance.
© 2014 Elsevier Inc. All rights reserveed.
Lymphedema is chronic swelling of tissue owing to poor
lymphatic function from either anomalous lymphatic development
of (primary) or injury to (secondary) lymphatic nodes or vessels.
Affected tissue initially swells from accumulation of subcutaneous
lymph; resultant inflammation stimulates adipose deposition and
fibrosis causing further enlargement over time [1]. Primary
lymphedema is uncommon; it affects 1.2 per 100,000 persons less
than 20 years of age [2]. Secondary lymphedema affects the
majority (90%) of patients, principally the lower extremities of
adults, owing to infection or treatment for malignancy [3]. In
developing countries, filarial infestation is the most common cause
of genital lymphedema [3]. The prevalence of primary genital
lymphedema is unknown. Complications of lymphedema include
infection, functional disability, and chronic cutaneous changes.
Disfigurement from lymphedema, particularly of the genitalia, can
cause considerable psychosocial distress.
☆ Statement of financial interest: No financial support or benefits were given to the
authors from any source that is related to the scientific work reported in this article.
☆☆ Lists of products used: No products or devices were used in this article.
⁎ Corresponding author at: Department of Surgery, Boston Children's Hospital, 300
Longwood Ave. Boston, MA 02115. Tel.: +1 617 355 3040; fax: +1 617 730 0752.
E-mail address: steven.fishman@childrens.harvard.edu (S.J. Fishman).
http://dx.doi.org/10.1016/j.jpedsurg.2014.05.031
0022-3468/© 2014 Elsevier Inc. All rights reserveed.
Genital lymphedema in the pediatric population is not only
infrequent but also poorly understood. Previous reports on genital
lymphedema have been limited by small numbers of patients [4–12]
and/or are comprised largely of adults [13–19]. Conflicting opinions
on the utility of compression therapy [10,14,16,20] and timing of
operative interventions (early versus post-pubertal) exist
[4,7,10,14,16]. The purpose of this study was to determine the
epidemiology, morbidity, and treatment outcome for males with
genital lymphedema in an effort to improve patient counseling and
offer management options for practitioners.
1. Materials and methods
After approval by the Committee on Clinical Investigation at
Boston Children's Hospital, the Vascular Anomalies Center database
was queried for all male patients with “lymphedema” of the penis
and/or scrotum who presented to our center between January 1995
and January 2011. Records were retrospectively reviewed for
referring diagnosis, age at presentation and intervention, anatomic
location, morbidity, treatment modalities, and outcome. Diagnosis
was made by clinical history, physical examination, and/or imaging
studies. Age-of-onset was categorized based on physiological age:
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C.C. Schook et al. / Journal of Pediatric Surgery 49 (2014) 1647–1651
infancy (b 12 months), childhood (1–9), and adolescence (10–21 years)
[21–23]. Data are presented as mean ± standard deviation.
Excisional debulking is utilized in our center for lymphedema of
the penis and scrotum. The procedures were performed by two
surgeons in the following manner. The genitalia are prepared and
draped widely and a urinary catheter of the largest size that can be
easily inserted is placed. For penile lymphedema, a circumferential
incision is made in the penile skin several millimeters proximal to the
corona of the glans penis, using the scar from previous circumcision, if
relevant. The skin of the shaft of the penis is then dissected just below
the dermis circumferentially to the base. The entirety of the shaft skin
is unfurled proximally using blunt, sharp, and/or cautery dissection.
The underlying lymphedematous adipose tissue is then dissected off
Buck's fascia circumferentially. Care is taken to avoid injury to the
skin, neurovascular bundles, or urethra. Once the lymphedematous
tissue is removed, the redundant skin is advanced back down the
penile shaft. Excess circumference of skin is resected to leave just
enough to comfortably wrap the penile shaft. When possible, the least
abnormal skin is saved for penile coverage, discarding as much of the
skin containing vesicles, dimples, or fibrosis. The skin is approximated
to create a longitudinal scar at the ventral midline. The excess length
of skin is excised to facilitate reapproximation at the distal precoronal incisional location. A small round closed-suction drain is
placed under the skin flap, positioned dorsally away from the urethra
and brought out in a suprapubic position. Interrupted intradermal
absorbable sutures followed by a running subcuticular suture are used
for closure. A penile block with 0.25% bupivacaine is performed prior
to dressing the wound. The penis is gently wrapped with circumferential gauze, leaving the urinary catheter in place for 24 hours.
For scrotal lymphedema, a lenticular incision is made vertically
encompassing the median raphe or transversely at the most
dependent portion of the scrotum. The testes and spermatic cord
structures are identified and protected. A full thickness wedge of
scrotal tissue, encompassing skin, adipose tissue and dartos is excised.
If hydroceles are present, the tunica vaginalis is similarly excised. The
excess scrotal septum is resected and the testes are secured with nonabsorbable sutures to the reduced septum. Scrotoplasty is performed
to create a new midline raphe or transverse closure parallel to the
rugae. In cases of extreme scrotal enlargement, additional redundant
tissue is resected from the lateral aspect of each hemi-scrotum and
closed in a V-Y advancement fashion. Closure is accomplished with
multiple layers of absorbable suture in the tunica vaginalis, dartos,
subcutaneous tissue, and skin. Post-operative scrotal elevation is
employed for 1 week and patients are encouraged to wear a
compressive undergarment thereafter.
2. Results
Of the 3889 male patients with a vascular anomaly, 25 (0.6%) had
lymphedema of the genitalia. Primary lymphedema occurred in 92.0%
(23/25) of patients and six children (24.0%) had syndromic or familial
lymphedema [Noonan syndrome (n = 2), lymphedema–distichiasis
(n = 2), and Milroy disease (n = 2)] (Table 1). Two patients (8.0%)
Table 1
Characteristics of genital lymphedema in 25 male pediatric patients.
Etiology
Age of onset
Location
Primary
Familial/syndromic
Secondary
Infancy
Childhood
Adolescence
Genital
Combined penile and scrotal
Isolated scrotal
Isolated penile
Associated lower extremity involvement
92.0%
24.0%
8.0%
60.9%
13.0%
26.1%
(23/25)
(6/25)
(2/25)
(14/23)
(3/23)
(6/23)
72.0%
24.0%
4.0%
76.0%
(18/25)
(6/25)
(1/25)
(19/25)
had secondary lymphedema from operative intervention for undescended testes (n = 1) or non-infectious granulomatous disease
(n = 1). Average age of onset was 4.5 ± 6.3 years (range: 0 to 15).
Fourteen (60.9%) patients presented in infancy. Childhood onset
occurred in 13.0% (3/23) of patients; the remaining 26.1% (6/23)
presented during adolescence. The referring diagnosis was accurate in
64.0% (16/25) of patients; the most common erroneous diagnosis was
capillary-lymphatico-venous malformation (Klippel-Trenaunay syndrome). Diagnosis was made by history and physical examination in
almost all patients. No adjunctive imaging studies were performed in
8.0% (2/25) of children. MRI [72.0% (18/25)] and/or lymphoscintigraphy [36.0% (9/25)] were reviewed when available; of these studies,
most were obtained at prior institutions or primarily for evaluation of
non-genital anomalies. Pathologic specimens were reviewed in
addition to imaging and clinical evaluation in nine (36.0%) children.
Penoscrotal lymphedema was identified in 72.0% (n = 18) of patients.
Isolated scrotal and penile disease was found in 24.0% (n = 6) and 4.0%
(n = 1) of patients, respectively. Concomitant involvement of the lower
extremities occurred in 76.0% (n = 19) of children. Genitourinary
symptoms were observed in 60.0% (n = 15) of patients and included
hematuria [12% (n = 3)], altered urinary mechanics [8.0% (n = 2)], and
phimosis [8.0% (n = 2)] (Table 2). Additional morbidities included
cellulitis [24.0% (n = 6)], lymphorrhea [24.0% (n = 6)], and skin changes
[12% (n = 3)].
Treatment was administered to 72.0% (18/25) of patients; seven
(28.0%) children were observed (Table 3). Compression therapy was
prescribed to 12 patients (48.0%). Of the patients receiving compression therapy, four (33.3%) later received surgical debulking. Contouring to improve symptoms and/or the appearance of the genitalia was
performed in 44.0% (n = 11) of patients with a mean of 1.5 ± 1.0
procedures per patient. One patient underwent concomitant debulking of a lymphedematous lower extremity. Initial operative intervention occurred at average 13.9 ± 8.0 years of age. Post-operative
complications (n = 2) included the appearance of granulomatous
tissue along a penile shaft suture line that improved with time and a
superficial wound infection treated with antibiotics. Post-operative
follow-up was available for 54.5% (6/11) of patients with surgical
procedures at a median of 4.2 years (range: 0.3–11.0). All patients
with operative treatment and follow-up had sustained improvement.
3. Discussion
Genital lymphedema is an uncommon but troubling cause of
deformity of the male genitalia. The epidemiology and morbidity are
unclear in part owing to the rarity of the condition. Furthermore,
diagnostic criteria, timing of therapies, and surgical techniques have
been debated. To our knowledge, this is the largest series of male
pediatric patients with genital lymphedema.
The etiology of genital lymphedema in children in the United
States is predominately idiopathic. While most patients develop
symptoms in infancy or early childhood, the diagnosis is often delayed
for several years. Lymphedema is most commonly confused with
other vascular anomalies, particularly if the lower extremities are also
affected. An accurate diagnosis is imperative in providing appropriate
Table 2
Complications of genital lymphedema in 25 male pediatric patients.
Cellulitis
Lymphorrhea
Skin changes
Hematuria
Pain
Bleeding
Altered urinary mechanics
Chylous reflux
Phimosis
Dysuria
24.0%
24.0%
12.0%
12.0%
8.0%
8.0%
8.0%
8.0%
8.0%
4.0%
(6/25)
(6/25)
(3/25)
(3/25)
(2/25)
(2/25)
(2/25)
(2/25)
(2/25)
(1/25)
C.C. Schook et al. / Journal of Pediatric Surgery 49 (2014) 1647–1651
Table 3
Treatment of genital lymphedema in 25 male pediatric patients.
Observation only
Compression therapy
Contouring/debulking
Mean number of procedures
Mean age at first intervention
Follow-up available
Median years of follow-up
Sustained improvement
Other
Hydrocele repair/aspiration
Orchidopexy
Circumcision
Urethral meatotomy
28.0% (7/25)
48.0% (12/25)
44.0% (11/25)
1.5 ± 1.0
13.9 ± 8.0 years
54.5% (6/11)
4.2 (range: 0.3–11.0) years
100.0% (6/6)
56.0% (14/25)
36.0% (9/25)
16.0% (4/25)
8.0% (2/25)
4.0% (1/25)
counseling and treatment. History and physical examination are the
primary mode of diagnosis, followed by imaging in limited cases.
Initial evaluation of male genital lymphedema includes a precise
history to rule-out secondary causes such as trauma, infection, prior
radiation therapy, or surgery. Non-infectious granulomatous disease
has been linked with lymphedema in children, affecting one patient in
our series, and is likely related to an inflammatory process (e.g.
Crohn's disease) [24,25]. Family history and extraneous physical
findings should be evaluated as lymphedema occasionally can be
syndromic or inherited. Milroy disease is lower extremity lymphedema at birth with a family history of the condition; Meige disease is
similar but presents in adolescence [26,27]. Lymphedema in both Milroy
and Meige disease can extend to the genitalia. Noonan syndrome,
diagnosed either by clinical evaluation or genetic testing, consists of
short stature, webbed neck, low-set ears, and cardiac anomalies;
lymphedema is a secondary finding [28]. Lymphedema can also occur
with distichiasis (an extra row of eyelashes arising from the meibomian
glands) [29]. To our knowledge, no syndromic or familial form of
lymphedema has been linked uniquely to the genitalia.
In all forms of lymphedema, the lower extremities are often
concurrently involved, although isolated genital disease does occur.
The scrotum is involved in almost all patients. Combined penoscrotal
disease is the most common presentation. Isolated penile enlargement is rare although disease limited to the scrotum is more common.
Pitting edema and minimal cutaneous changes are present in early
disease; non-pitting overgrowth will occur over time from adipose
deposition and fibrosis.
History and physical examination are rarely equivocal. Nevertheless,
some of our patients had confirmatory lymphoscintigraphy prior to
referral. Delayed transport and/or dermal backflow of radiolabeled
colloid indicate lymphatic dysfunction with 92% sensitivity and 100%
specificity [30]. Lymphoscintigraphy can be performed either by scrotal
injection when isolated genital disease occurs or injection of the foot if
the lower limbs are involved. If lateralization of labeled colloid is less clear
1649
with an extremity lymphoscintigram, a dedicated genital examination
may be indicated. Ultrasonography is less specific and CT entails radiation
to the testes. MRI can be particularly useful to distinguish other vascular
anomalies, such as venous or lymphatic malformation, from lymphedema, and can also be of value in operative planning. Biopsy should be
utilized only if malignancy or other conditions, such as non-infectious
granulomatous disease, are suspected.
Once the diagnosis of lymphedema is made, symptomatic treatment
should be initiated. Lymphedema can adversely influence a patient's
quality of life both functionally and emotionally. Our study indicated
that forty-percent of patients will have no symptoms beyond
enlargement of the genitalia; however, the psychosocial consequences
of disfigurement should not be trivialized particularly in a developing
child. Emotional health should be screened in all patients; a formal
psychiatric assessment may be indicated in some children.
For those patients with additional somatic symptoms, cellulitis,
lymphorrhea, and skin changes were most common. Cellulitis is a
potentially life-threatening consequence of lymphedema. It was
observed in a quarter of our patients. The presence of cellulitis
mandates prompt treatment with oral or intravenous antibiotics
depending upon severity. A patient experiencing three or more
episodes of cellulitis per year may benefit from a prophylactic lowdose regimen of antibiotics. Infection prevention includes daily
washing of the genitalia and application of a moisturizing agent to
prevent desiccation and breakdown of the skin.
The primary goal of conservative lymphedema treatment is to
minimize stagnant lymph accumulation to prevent progression and
improve contour. Pharmacologic treatment has thus far been limited.
Preliminary investigations, suggest that angiogenesis and vasculogenesis play no role in the expansion of lymphedema; therefore,
angiogenic inhibitors are not indicated [31]. Compression therapy
with stockings or fitted garments is the mainstay of non-operative
treatment in patients with lower extremity lymphedema [32].
Because of the shape and location of genital lymphedema, wrapping
and stocking material can be difficult to apply and may require
ingenuity on behalf of the patient and family. Applying fitted
compression stockings to a growing infant is often impractical.
Many patients utilize over-the-counter elastic shorts. Some patients
also place a wedge under the pelvis to elevate the genitalia at night to
improve lymphatic return. The merits of compression and elevation
therapy in genital lymphedema are mixed and should be weighed
against inconvenience to the patient [4,7]. However, in our experience, as well as others, compression therapy is a useful tool to
maintain and even reduce genital size when tolerated [16]. Postoperatively, penile wrapping is particularly effective at minimizing
recurrent swelling.
Surgical treatment of genital lymphedema is indicated for patients
with significant morbidity who have failed non-operative management. In our center, operative indications include excessive size,
Fig. 1. Operative debulking of male genital lymphedema. (Left) Patient at 12 years of age with swelling of the penis and scrotum. Patient has had lymphedema since birth; surgical
intervention was delayed until puberty was completed. (Middle) Intraoperative appearance of debulking procedure. (Right) Three month follow-up demonstrates an improved contour.
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C.C. Schook et al. / Journal of Pediatric Surgery 49 (2014) 1647–1651
Fig. 2. Surgical debulking of male scrotal lyphedema. He had previously undergone contour resection of the penile shaft. The scrotum is large and pendulous. (Left) Pre-operative
appearance of scrotum with area of resection marked. (Middle) Intraoperative appearance of debulking procedure. (Right) Follow-up appearance of scrotum.
functional impairment, and/or disfigurement (Fig. 1). The optimal
operative technique and timing remain controversial. There are two
surgical approaches to lymphedema: physiological or excisional.
Physiologic procedures, which include local flap transfers and
lymphaticovenous anastomoses, have shown inconsistent benefits
in the treatment of extremity lymphedema. In the genitalia, these
procedures mostly have been abandoned owing to inefficacy.
Excisional procedures remove lymphedematous tissue with or
without excision of the overlying skin. We favor excisional intervention at our center for both penile and scrotal debulking (Fig. 2). There
are alternative surgical techniques for debulking the penis and
scrotum largely related to placement of the incisions, such as a
transverse incision at the base of the penis or scrotum [33]. That
approach has the advantage of avoiding scars on the penile shaft.
However, with that incision, excess skin circumference cannot be
removed from the penis. We prefer to remove as much redundant skin
as possible to minimize re-enlargement. The small amount of residual
subcutaneous tissue can thicken over time. Thus, removing excess
skin leaves less subcutis to contribute to future girth regrowth. The
key to removing excess lymphedematous tissue particularly along the
penile shaft is to maintain sufficient vascular attachment both
proximally and distally to avoid wound ischemia. A ventrally located
incision in the scrotum has been reported to result in an unsatisfactory result. If there is re-growth of lymphedematous tissue on either
side of the scar, the scrotum may assume a labia-like appearance
[34,35]. We exercise care in reconstructing the scrotal septum with no
tension and did not observe this outcome in our patients. Minimization and thoughtful placement of incisions are crucial as lymphedematous tissue is prone to wound break down and weeping. Because
excisional procedures do not treat the underlying pathology, postoperative compression is still required after resection to prevent the
re-accumulation of lymph and ongoing deposition of adipose tissue.
Multiple staged serial excision procedures may be necessary over the
patient's lifetime owing to recurrence.
The optimal timing of the genital debulking procedures is
controversial. Some argue for early intervention to minimize the
physical and emotional strain on a pediatric patient [13]. In our center,
we prefer to delay surgical intervention until pubertal changes to
avoid overcorrection as well as to facilitate the procedure by operating
on larger structures. In extreme cases, conservative early debulking
can be performed with the expectation of potential additional
intervention when the child is older (Fig. 3). Some patients with
genital lymphedema will have a concurrent hydrocele, undescended
testes, and/or chylous reflux. These conditions can be addressed at the
time of the debulking. Conversely, older patients with these additional
diagnoses but moderate lymphedema may desire debulking at the
time of their correction when no intervention for the lymphedema
would otherwise be undertaken. Like any procedure in the pediatric
population, the exact timing of intervention should be weighed
considering the extent of deformity, functional impairment, and
psychological disturbance. All patients and their families should be
properly counseled regarding their expectations. Disfigurement can
be greatly improved, but the genitalia will likely never appear
completely normal.
There are several limitations to our study. Most important is its
retrospective nature. Additionally, many patients were initially
managed at outside institutions and referred to our center. This
allows for gaps in the medical record and fewer surgical patients
returning for follow-up.
4. Conclusions
Lymphedema of the male genitalia is typically idiopathic and
usually concomitantly involves the lower extremities. Most patients
experience swelling in infancy and early childhood; the remaining
patients present in adolescence. Psychosocial morbidity is common.
Infection and lymphatic leak may occur in as many as a quarter of
patients. Compression therapy may limit worsening or improve
Fig. 3. Operative reconstruction of male genital lymphedema. Because of distortion of penile tissue, skin changes, and phimosis, conservative debulking with penile reconstruction by
adjacent tissue advancement flap closure was performed on 17 month old patient with distichiasis–lymphedema. Right inguinal orchidopexy was also performed for undescended
testis. (Left) Pre-operative penile deformity. (Middle) One week post-resection. (Right) Seven month follow-up demonstrating sustained improvement.
C.C. Schook et al. / Journal of Pediatric Surgery 49 (2014) 1647–1651
swelling in early disease but can be difficult to implement. Contour
resection can decrease bulk, improve appearance, and minimize
psychosocial impairment. Optimally, operative intervention is postponed until after pubertal changes to the genitalia occur to avoid
overcorrection and facilitate the procedure because of enlarged
structures. When necessary, patients with significant overgrowth or
other genitourinary symptoms can have conservative debulking in
childhood. Regardless of initial age at intervention, serial excisions
may be necessary for sustained improvement because of recurrence.
Acknowledgments
Dr. Kulungowski was supported by the Stuart and Jane Weitzman
Fellowship in Vascular Anomalies.
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