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CHAPTER 48 The Pediatric Head and Neck 1657

prenatally, with 90% presenting by age 2 years. Clinically, the

skin is usually normal but may have a bluish hue, puckering, or

tiny vesicles. 163 hese lesions can be difuse or focal, tend to

invade multiple anatomic facial planes, and will rapidly enlarge

if complicated by hemorrhage or infection. Hypertrophy and

intraosseous invasion of adjacent bone, usually the mandible,

can occur.

About 75% of lymphatic lesions occur in the neck, and most

originate within the posterior triangle or oral cavity, with lesions

occurring on the let side twice as oten as on the right side. 43,48,82

Of infants with lymphatic malformations, 3% to 10% have airway

compromise from respiratory obstruction or mediastinal extension.

47,157 Although most children with lymphatic malformation

have a normal karyotype, several syndromes are associated, such

as Turner, Down, Noonan, Roberts, and trisomy 13 and 18. 144

On ultrasound, most lymphatic malformations are multiloculated

cystic masses with septa of variable thickness and solid

components 47 (Fig. 48.49, Video 48.4). Fluid-luid levels or

echogenic debris can result from superimposed hemorrhage or

infection. MRI provides optimum tissue characterization

and lesion delineation before surgical intervention. 168 Surgical

resection is the treatment of choice but can be diicult if the

lesion invades neural, vascular, and muscular structures; therefore

sclerotherapy, radiofrequency ablation, and laser therapy are

other options. 48,168 Other malformations involving the lymphatic

system include generalized lymphatic anomaly, Gorham-Stout

disease, channel-type lymphatic malformation, primary lymphedema,

and others. 162

Venous malformations are sot, compressible lesions that

may distend with increased venous pressure (Valsalva maneuver).

About 40% of these lesions occur in the head and neck, and

many invade multiple fascial planes and involve osseous structures.

163 hese lesions, appearing clinically as sot, bluish,

compressible masses, consist of thin-walled, dilated, dysplastic,

small and large venous channels deicient in smooth muscle. 43

Rapid enlargement may occur secondary to phlebothrombosis.

Multiple venous malformations are seen in Mafucci syndrome

and blue rubber bleb nevus syndrome, cerebral cavernous

malformation, and glomuvenous malformation. 162,169 Phleboliths

are present in 16% of cases and can help conirm the

diagnosis. Ultrasound demonstrates hypoechoic lesions with

heterogeneous architecture. Acoustic shadowing secondary to

phleboliths and luid-luid levels may be identiied 48 (Fig. 48.50).

On Doppler sonography, venous low-velocity low is identiied.

Treatment includes elastic compression, sclerotherapy, and surgical

excision.

Arteriovenous malformations (AVMs) represent collections

of a nidus of tortuous abnormal, thin-walled vessels connecting

dilated feeding arteries to draining veins. hese lesions may be

centered in cutaneous, muscular, or intraosseous structures.

FIG. 48.49 Lymphatic Malformation. Lymphatic malformation in

a 5-year-old child with long-standing left neck mass. Ultrasound shows

a multilocular cystic lesion with a luid-luid level in one of the locules,

indicating internal hemorrhage. See also Video 48.4.

A

B

FIG. 48.50 Venous Malformation. (A) Side-by-side gray-scale and color Doppler. Masseter muscle is enlarged and heterogeneous with

hypoechoic areas and a hyperechoic focus that shadows (arrowhead), consistent with phlebolith. Color Doppler demonstrates mild increase in

vascularity. (B) Axial T2-weighted fat-saturated magnetic resonance image demonstrates high-signal lesion iniltrating the masseter muscle and

parapharyngeal space. Dark foci are consistent with phleboliths (arrows).

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