Grand Rounds
Multifocal Vascular Tumors and Fetal Hydrops

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Clinical Discussion

Fetal hydrops is defined as the accumulation of fluid in at least 2 fetal compartments, including the subcutaneous, pleural, peritoneal, and/or pericardial space. Nonimmune hydrops fetalis accounts for the majority of cases of fetal hydrops. It affects 1:1500 to 1:3750 deliveries and has a perinatal mortality rate of 55%-98%, depending upon the etiology.2 The etiologies for nonimmune fetal hydrops are numerous, although some of the more common causes include aneuploidy, congenital infection

Pathologic Findings

A skin biopsy from a lesion on the left upper arm was performed on day of life 2. Sections demonstrated a slightly atrophic epidermis and a vascular proliferation composed of small and slit-like vascular channels lined by plump endothelial cells with occasional mitoses in the superficial dermis (Figure 2, A). The moderately cellular intervening stroma contained dermal dendrocytes and occasional mast cells. The vascular endothelial cells strongly expressed GLUT-1, and D2-40 expression, a marker

Clinical Course

On the basis of the clinical and pathologic findings, the infant was diagnosed with IH involving the skin, liver, and brain. The infant was empirically started on propranolol and methylprednisolone within 24 hours after birth. After histologic confirmation of the diagnosis, methylprednisolone was increased to 2 mg/kg/dose intravenously every 12 hours and propranolol dosing was adjusted to 0.2 mg/kg/dose intravenously every 8 hours to approximate oral doses within the range of 3-5 mg/kg/day.

A

Discussion

The terms “disseminated” or diffuse neonatal hemangiomatosis (DNH) describe patients with the onset of numerous IH in the neonatal period with generalized involvement of the skin and potential involvement of other organs. Glick et al11 reviewed DNH, citing difficulty with this nomenclature, and advocated using the term multifocal IH (diagnosed by GLUT-1 expression) with or without extracutaneous involvement with as a better descriptor for this clinical presentation. Cited mortality figures for

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The authors declare no conflicts of interest.

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