A 5-month-old female infant presented with swelling in the
region of both cheeks extending below the jaws, first
noticed as a peanut sized swelling 3 months earlier. On
examination, there were bright red, bosselated, non tender,
non-fluctuant masses in the area of both parotid glands
involving most of the cheeks, and both submandibular
salivary glands. Overlying skin showed a fine network of
capillaries with excoriation on the left side (Fig. 1, 2).
The infant was born at term, was developmentally normal and
growing well. Ultra-sonography (USG) revealed enlargement of
bilateral parotid, and submandibular salivary glands,
lobular structure with fine echogenic internal septations.
Color Doppler demonstrated numerous blood vessels within the
mass confirming hemangiomas of the glands. Treatment with
oral propranolol was started at 2 mg/kg/day in 3 doses;
however the patient was lost to follow-up.
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Fig.1 Hemangioma involving
left parotid and submandibular glands with
superficial excoriation.
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Fig. 2 Hemangioma affecting
right parotid gland.
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Salivary gland tumors are rare in
children, most common are hemangiomas and the parotid is
most commonly affected. They present as focal lesions or as
part of a segmental distribution involving the V3 mandibular
segment as in our patient. Segmental hemangiomas are more
aggressive, have a prolonged growth phase, higher likelihood
of associated ulceration, airway obstruction, and
soft-tissue and cartilage destruction. Diagnosis is
confirmed by USG, rarely requires MRI. Lesions to be
considered in the differential diagnosis of a parotid
hemangioma include cystic lymphatic malformations (cystic
hygromas). These do not have a solid component as seen by
USG or MRI in PH. Solid lymphatic malformations may extend
beyond the parotid and do not contain prominent blood
vessels. A rhabdomyosarcoma may arise in the head and neck
region, but is rare in infancy, is less vascular and
infiltrates into neighbouring structures. Congenital
infantile fibrosarcoma of the parotid, though very vascular
on Doppler sonography has a non-homogeneous structure. A
solitary infantile myofibromatosis in the parotid region may
show rapid growth followed by involution, however these are
ill defined and not vascular. A sialoblastoma (congenital
carcinoma) is a rare tumor of the salivary glands of infancy
and can be differentiated from a PH on US or MRI because it
is non homogeneous and less vascular.
Most PH involute spontaneously; 10 %
require active management. Recommended therapy includes
intra-lesional corticosteroids for small lesions, systemic
corticosteroids or interferon alfa-2a or -2b for large ones
and more recently oral propranolol. Surgical correction is
recommended in the involuting or involuted phases (preceded
by sclerotherapy).