A FEMALE INFANT was delivered vaginally at 36 weeks' gestation to a 32-year-old gravida 2, para 1 mother. At birth, a subcutaneous mass measuring 6 × 5 × 2.5 cm in diameter was noted in the left parieto-occipital region of the scalp. A computed tomographic scan of the head showed a soft tissue subcutaneous dense lesion with calcification consistent with large hemangioma (Figure 1). Owing to ulceration of skin overlying the hemangioma, the lesion was surgically removed. Blood loss required multiple transfusions during surgery. The patient developed hypotension, asystolic rhythm, and died despite vigorous resuscitation efforts. A surgical specimen showed proliferation of blood-filled, thin-walled, endothelial-lined immature small vessels separated by connective tissue (Figure 2). At autopsy there were diffuse petechial hemorrhages on the front chest and both upper arms, ranging from 0.1 to 0.3 cm. Both lungs had diffuse petechial hemorrhages. The parenchyma was purple-red, firm, and uniform. Sections revealed collapsed alveoli and small airways with moderate to severe congestion and mild thickening of the septae. Most of the small vessels contained basophilic granular material, which was positive for factor VIII immunohistochemical staining (Figure 3 and Figure 4). However, Carstair stain for fibrin was negative in the lung specimen. Platelet microthrombi were occasionally seen in the small vessels of the intestine and heart, while no thrombi were seen in the sections of skin, kidney, liver, or adrenal glands.
Guo Z, Chandra S. Pathological Case of the Month. Arch Pediatr Adolesc Med. 2001;155(6):737-738. doi:10.1001/archpedi.155.6.737