Clinical Pediatrics
2015, Vol. 54(7) 700–702
© The Author(s) 2015
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DOI: 10.1177/0009922815569208
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Resident Round
Case Report
An 8-year-old, otherwise healthy girl presents with a
2-month history of focal swelling in her right nasal root
area. The swelling improves when she sits upright and
worsens with supine positioning, facial movements, and
physical activity. The mother reports prominent veins on
her nose since birth and smaller superficial vessels on
her eyelids and chin. She has diminished visual acuity, a
“popping” sensation in the corner of her right eye, and
nasal congestion with tenderness. Because of significant
pain caused by the nasal swelling and improperly fitting
eyeglasses, she experienced difficulties in school and
began homeschooling. She was unable to play softball
because of inability to see without her eyeglasses. After
a prior sinus computed tomography scan revealed an
ethmoid sinus infection, she was treated with antibiotics.
Her fever and tenderness resolved but the swelling was
unchanged. There is no history of nasal trauma, relevant
family history, or other areas of similar swelling.
Examination revealed an ill-defined, nonpulsatile,
nontender, reducible subcutaneous mass with surround-
ing soft tissue edema on the right aspect of the nasal
root. Mild yellowish discoloration of the nasal bridge
with 2 overlying dilated veins were noted, but no pig-
ment, nodularity, or epidermal changes were present.
The mass and edema are most visible when in a
Trendelenburg position and bulge with Valsalva maneu-
vers (Figure 1A). The fullness decreases quickly on sit-
ting upright (Figure 1B).
Clinical Course
After being seen by 17 physicians with workup including
sinus computed tomography, brain magnetic resonance
imaging (MRI), magnetic resonance angiography/venog-
raphy, nasal endoscopy, chest radiography, and blood-
work, a Doppler ultrasound and MRI were performed.
Doppler ultrasound over the right nasal bridge showed a
small soft tissue mass containing numerous dilated slow
flow veins. Axial T2-weighted MRI at the level of the
upper nares and ethmoid sinus revealed a 1.7 × 0.7 × 0.6
cm high signal, homogenously enhancing irregular soft
tissue mass in the subcutaneous region closely associated
with the right angular vein (Figure 2). Incidental ethmoid
mucosal edema was present, and the adjacent nasal bone
was dehiscent, suggesting a slowly growing chronic
lesion. Surgical excision was considered but, because of
proximity to the eye and possible cosmetic morbidity,
percutaneous sclerotherapy was performed instead and
swelling rapidly resolved. Digital subtraction venogram
during direct needle puncture of the malformation showed
venous outflow in a medial periorbital vein. The venous
egress was compressed in order to avoid optic or intracra-
nial effects of sclerotherapy agent. There were no compli-
cations following sclerotherapy, and no other treatment
was given. At the 2-month posttreatment follow-up, the
venous malformation (VM) remained asymptomatic and
the patient’s eyeglasses were able to fit, allowing her to
return to school and return to playing softball. No addi-
tional follow-up was needed at this time, but the possibil-
ity of recurrence during puberty, pregnancy, or due to
trauma was explained to the patient’s mother.
Diagnosis
Nasal root venous malformation.
Discussion
Patients with VMs often experience high rates of misdi-
agnosis because of challenges in distinguishing VMs
from other vascular anomalies. Classification of vascu-
lar abnormalities can be based on both vascular kinetics
and clinical aspects.
1
Two major classifications include
vascular tumors, which are caused by endothelial hyper-
plasia and vascular malformations, which are due to
dysmorphogenesis rather than abnormal endothelial
growth.
1
Hemangiomas are the most common type of
vascular tumor, occurring on the skin of 4% to 10% of
infants.
2
Because of their prevalence, VMs are fre-
quently misdiagnosed as hemangiomas despite biologic
569208CPJ XX X 10.1177/0009922815569208Clinical PediatricsVuong et al
research-article 2015
1
University of North Carolina at Chapel Hill, Chapel Hill, NC, USA
Corresponding Author:
Kim T. Vuong, 9925 Park Walk West, Charlotte, NC 28269, USA.
Email: kim_vuong@med.unc.edu
Nasal Root Venous Malformation
Kim T. Vuong
1
, Katherine M. Varman, MD
1
,
Joseph Stavas, MD
1
, and Dean S. Morrell, MD
1