Microcephalic Newborn with Forehead Nevus Flammeus, Bulging Eyes, and Clenched Fists

Prenatal and Birth Histories

• Mother is an 18-year-old gravida 1 para 0 black woman

• Pregnancy was complicated by intrauterine growth restriction (IUGR) of unclear etiology and nonobstructive polyhydramnios with a normal fetal anatomical survey

• Induced vaginal delivery for IUGR

• Estimated gestational age at birth: 37 weeks

• Prenatal laboratory findings: Group B Streptococcus negative; hepatitis B surface antigen negative; rapid plasma reagin nonreactive; HIV negative; rubella immune; Chlamydia infection during pregnancy treated adequately

• Apgar scores: 2 and 8 at 1 and 5 minutes, respectively; infant initially had no respiratory effort but responded to stimulation and did not require positive-pressure ventilation

• Symmetric small-for-gestational age: Birthweight 1,500 g, length 41 cm, head circumference 32.5 cm (all <3rd percentile)

Presentation

The infant was transferred to the NICU from an outside hospital at 1 month of age because of poor feeding, stridor, and multiple dysmorphic facial features.

Vital Signs–Day 35

• Heart rate: 150 beats/min

• Respiratory rate: 42 breaths/min

• Temperature: 98.8°F (37.1°C)

• Oxygen saturation: 95% on 4 L/min high-flow nasal cannula

Physical Examination–Day 35

• • Growth parameters

  • ∘ Weight: 2.09 kg (<3rd percentile for age)

  • ∘ Length: 41 cm (<3rd percentile for age)

  • ∘ Head circumference: 32.5 cm (<3rd percentile for age)

• • Head

  • ∘ Microcephaly with a triangular shaped head

  • ∘ Bilateral exophthalmos and hypertelorism

  • ∘ Anteverted nares, high arched intact palate

  • ∘ Micrognathia, retrognathia

• • Lungs

  • ∘ Coarse breath sounds with upper airway congestion and inspiratory stridor

  • ∘ Tachypnea and mild subcostal retractions

• • Cardiovascular: Normal S1 and S2, regular …