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Case 2: Rapidly Growing Neck Mass in an Extremely Preterm Infant with Pulmonary Hypertension.

A male infant is born at a gestational age of 27 weeks, 4 days via cesarean delivery to a 27-year-old gravida 2, para 1 woman with severe preeclampsia (treated with intravenous magnesium and nifedipine). The pregnancy had been complicated by systemic lupus erythematosus (treated with prednisone and plaquenil) and type 2 diabetes (treated with insulin and aspirin). Second-trimester prenatal ultrasonography at 25 weeks’ gestation reveals intrauterine growth restriction with an estimated fetal weight less than the 3rd percentile. At delivery, the infant's Apgar scores are 5 and 8 at 1 and 5 minutes, respectively. He receives positive pressure ventilation, makes a transition to continuous positive airway pressure (CPAP) of 5 cm H2O, and is transported to the NICU.

During his NICU stay, the infant develops severe bronchopulmonary dysplasia with pulmonary hypertension, apnea and anemia of prematurity, retinopathy of prematurity, failure to thrive, and postnatal growth restriction. Forty-four days after birth, he starts treatment with sildenafil 1 mg/kg per dose every 8 hours for pulmonary hypertension. Fifty-eight days after birth, soft, nonblanching skin-colored nodules develop in both axillae and supraclavicular fossae, but are more prominent on the right side (Fig 1).



Figure 1. 
Photograph of the infant demonstrating a right-sided neck mass (arrow). This …