JCU - January 2022 - 68

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Journal of Clinical Urology 15(1)
A telephone call to the patient 6 weeks after his initial
presentation revealed full resolution of symptoms. He suffered
from no sexual dysfunction or impotence and his
pain and venous hardening had resolved.
Discussion
We report a case of spontaneous atraumatic PMD resulting
from a hereditary thrombophilia detected with noninvasive
methods and managed successfully with conservative
medical management. The true incidence of
penile Mondor's disease is unknown, but one case series
reported an incidence of 18 out of 1296 (1.39%) patients in
a sexually transmitted disease clinic over a 12-year period.2
As a result, there are only a handful of care reports on this
topic, with only one other published case of PMD resulting
in a patient with factor V Leiden, which self-resolved with
simple analgesia alone.3 Primary hypercoagulability is,
therefore, an extremely rare cause of this disease and we
herein present the second case known to date of PMD
resulting from an inherited thrombophilia.
The pathogenesis of PMD is unclear but risk factors for
its development are in keeping with Virchow's triad of
vessel damage (typically seen in penile trauma), venous
stasis (encountered with prolonged erections), and hypercoagulability
(malignancy and thrombophilias). Being
homozygous to the factor V Leiden mutation, which is an
inherited resistance to activated protein C, increases the
risk of thromboembolic events by 80- to 100-fold.3 The
importance and frequency of this mutation in patients with
superficial penile vein thrombosis is yet to be determined.
The most commonly described cause in literature, however,
is prolonged and excessive sexual intercourse causing
mechanical trauma to the penis.1
Diagnosis is made on good history taking and thorough
physical examination. Findings on grey-scale ultrasound
include a distended non-compressible superficial
dorsal penile vein with a venous thrombus in situ.4 Color
Doppler confirms the absence of flow within the vein and
the cavernous arteries usually display a low-speed, highresistance
flow pattern.4,5 Magnetic resonance imaging
is not usually performed. While diagnosing PMD, it is
important to consider differentials such as sclerosing
lymphangitis and Peyronie's disease. Sclerosing lymphangitis
is another benign, self-limiting condition of the
penis that presents in sexually active males following
vigorous sexual activity or masturbation affecting the
penile lymphatic system.6 Color Doppler ultrasound is
the key investigation in differentiating PMD from sclerosing
lymphangitis, which does not exhibit the sonographic
findings seen in PMD.6 Clinical examination
alone can differentiate whether the thickening is on the
tunica albuginea.
In many cases, the condition is self-limiting and resolves
within 4-6 weeks as the vessel re-canalizes. Sexual activity
should be avoided during this period. The largest case
series in literature recommends initial medical therapy with
heparin ointment and oral non-steroidal anti-inflammatory
drugs (NSAIDs) with a success rate of 92%.7 Prophylactic
use of topical antibiotics is not recommended.7 Surgical
thrombectomy is reserved for patients refractory to medical
treatment who are symptomatic without flow on the color
Doppler ultrasound after 6 weeks.1,7
Although PMD is a benign condition, there is often
anxiety around sexual dysfunction and it is important to
address this issue when counselling patients. A case series
of 30 patients by Özaken et al. examined the effect PMD
on long-term sexual and erectile function. The authors
found that 28 patients (93%) had full resolution of the
superficial cord-like structure over the dorsum of the penis
at the 1-month follow-up, which persisted in only two
patients.8 In these two patients, this was subsequently significantly
improved on a follow-up visit after 2 months.8
The study also found that there was no significant difference
between the baseline International Index of Erectile
Function scores and the scores at 2 months.8 Sexual dysfunction
and impotence, thus, were not long-term complications
described in this paper.
Due to the rarity of PMD seen in patients with hereditary
thrombophilias and the lack of published data on this
topic, there is no consensus on which patients warrant
screening for thrombophilias and the indications for anticoagulation.
Our suggestion is that patients with idiopathic
PMD, those with widespread thrombophlebitis, those with
a personal or family history of recurrent thromboembolic
events without any clear cause, or those that fail conservative
management raise suspicion for a coagulation disorder
and warrant further investigation. The need for systemic
anticoagulation should be assessed on a case-by-case basis
in consultation with a haematologist.
In conclusion, penile Mondor's disease should be considered
in men with cord-like thickening of their superficial
dorsal penile vein. Once confirmed with Doppler ultrasound,
conservative management with sexual abstinence,
heparin ointments, and oral NSAIDs is recommended as
first-line therapy. Specialist help should be sought when
considering systemic anticoagulation. No long-term sexual
dysfunction has been reported in literature. If medical treatment
is unsuccessful, surgery should be considered.
Conflicting interests
The authors declare that there is no conflict of interest.
Funding
The author(s) received no financial support for the research,
authorship, and/or publication of this article.

JCU - January 2022

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