Verrucous Venous Malformation and Overgrowth: About Two Cases

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*Corresponding author: María Velayos López, Servicio Cirugía
Pediátrica, Hospital La Paz, Paseo de la Castellana, 261, Madrid,
Spain, Tel: +34-652376654
Accepted: May 12, 2021
Published online: May 14, 2021
Citaon: Velayos M, Estefanía-Fernández K, Sarmiento-Caldas
MC, et al. (2021) Verrucous Venous Malformaon and Over-
growth: About Two Cases. Arch of Pedia Surg 5(1):100-103
Open Access | Page 100 |
Vol 5 | Issue 1 | Pages 100-103
Copyright: © 2021 Velayos M, et al. This is an open-access arcle distributed under the terms
of the Creave Commons Aribuon License, which permits unrestricted use, distribuon, and
reproducon in any medium, provided the original author and source are credited.
Archives of Pediatric Surgery
ISSN: 2643-5721
SCHOLARS.DIRECT
DOI: 10.36959/472/363
Verrucous Venous Malformation and Overgrowth:
About Two Cases
Velayos M1, Estefanía-Fernández K1, Sarmiento-Caldas MC1, Moralla-Lapeña L1, Triana P1, Díaz M1,
Rodriguez Laguna L2, Marnez González V2 and López-Guérrez JC1
1Department of Pediatric Surgery, Hospital Universitario La Paz, Madrid Spain
2Department of Genecs, Hospital Universitario La Paz, Madrid, Spain
Verrucous venous malformaons (VVM), previously
known as verrucous hemangiomas, are rare congenital vas-
cular anomalies that inially appear as at blue-red lesions
that progressively evolve into very characterisc wart and hy-
perkeratoc forms. They mainly aect the lower extremies
and, unlike angiokeratoma, they extend into the deep dermis,
subcutaneous ssue and even reach the fascia [1,2]. Couto, et
al. found the somac mutaon MAP3K3 in paents with VVM
[3]. Treatment is dicult in large size cases and oen requires
a muldisciplinary approach including laser, surgery and
more recently pharmacological therapy with rapamycin, as
described by Zhang, et al. in their retrospecve cohort [4,5].
Dierent paerns of presentaon of VVM have been de-
scribed: Linear, focal, segmental or mixed and, associaons
such as the segmental paern associated with brain caverno-
mas, has been reported by Feldmeyer, et al. in 2014 [6]. On
the other hand, there are numerous overgrowth disorders
associated with vascular anomalies among which the PROS
spectrum represents the best known group [7]. This group of
syndromes has a phenotype associated with a well-dened
mutaon and, in the case of the PROS spectrum, where the
PIKC3CA alteraon is the cause, inhibitory therapy with drugs
such as rapamycin or alpelisib is a targeted treatment line.
This has been the movaon for numerous invesgaons to
be able to associate mutaons to characterisc phenotypes
and thus dene specic inhibitory therapies for each type of
disorder.
In this context, we believe it is appropriate to report two
cases of VVM that associate overgrowth of the aected ipsi-
lateral limb. The rst case is a VVM of the lower right limb in a
4-year-old girl with lower limb dismetry 2 cm. It presented as
a reddish-blue, warty and hyperkeratoc lesion that respond-
ed favorably to treatment with rapamycin, reducing its colour
and volume. However, it was observed that the associated
limb overgrowth did not remit with this drug and currently
Research Article
Keywords
Verrucous venous malformaon, Verrucous hemangioma, Rapamycin, Overgrowth, MAP3K3
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updates
forces the paent to wear a shoe li (Figure 1). The second
case corresponds to an VVM in an 8-year-old girl. She pre-
sented a bluish, warty and hyperkeratoc lesion on the back
of her right thigh accompanied by at perilesional areas of
the same color. In turn, the plantar region and the back of the
right foot were aected by a lesion similar to the appearance
of the perilesional lesions already described, accompanied
by macrodactyly and macropodia of the fourth and h toes
(Figure 2). It was treated by surgical removal and the genec
test showed posivity for the MAP3K3 mutaon.
Up to our knowledge this is the rst report of VVM and
MAP3K3 mutaon associated to overgrowth.
Funding Sources
None declared.
Conict of Interest
The authors have no conicts of interest relevant to this
arcle to disclose.
Consent Statement
Obtained.

Citaon: Velayos M, Estefanía-Fernández K, Sarmiento-Caldas MC, et al. (2021) Verrucous Venous Malformaon and Overgrowth: About Two
Cases. Arch of Pedia Surg 5(1):100-103
Velayos et al. Arch of Pedia Surg 2021, 5(1):100-103 Open Access | Page 101 |
Figure 1: First paent case with VVM of the lower right limb Photographs A, B and C show the evoluon of the injury aer treatment
with rapamycin. Picture D shows the lower limb dismetry 2 cm.
Figure 2: Second paent case with VVM lesion on the popliteal aspect of her right lower limb accompanied by at perilesional areas
of the same color. The plantar region and the back of the right foot accompanied by macrodactyly and macropodia of the fourth and
h toes.

Citaon: Velayos M, Estefanía-Fernández K, Sarmiento-Caldas MC, et al. (2021) Verrucous Venous Malformaon and Overgrowth: About Two
Cases. Arch of Pedia Surg 5(1):100-103
Velayos et al. Arch of Pedia Surg 2021, 5(1):100-103 Open Access | Page 102 |
5. Leavens J, Worswick S, Kim GH, et al. (2019) Verrucous venous
malformaon. Dermatol Online J 25: 0-4.
6. Feldmeyer L, Baumann-Vogel H, Tournierlasserve E, et al. (2014)
Hyperkeratoc cutaneous vascular malformaon associated
with familial cerebral cavernous malformaons (FCCM) with
Krit1/Ccm1 mutaon. Eur J Dermatology 24: 255-257.
7. Kim M, Keppler-Noreuil, Jonathan J, et al. (2017) PIK3CA-related
overgrowth spectrum (PROS): Diagnosc and tesng eligibility
criteria, dierenal diagnosis, and evaluaon. Physiol Behav
176: 139-148.
References
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Copyright: © 2021 Velayos M, et al. This is an open-access arcle distributed under the terms
of the Creave Commons Aribuon License, which permits unrestricted use, distribuon, and
reproducon in any medium, provided the original author and source are credited.
SCHOLARS.DIRECT
DOI: 10.36959/472/363