11/27/2007 Case 2: Subendothelial vacuolization in choroid plexus


Clinical History:

A 25-day-old full-term female born to a 28-year-old woman presented after prolonged rupture of membranes and positive maternal culture for group B Streptococcus. The infant was floppy at birth with APGAR scores of 5, 6, and 9 at 1, 5, and 10 minutes, respectively. Workup was started in the NICU and ampicillin/gentamicin was started.
Echocardiogram showed cardiomegaly with cardiac dysfunction and she was started on dobutamine, milrinone drip and Lasix. Repeat sepsis evaluation was done and antibiotics were changed to vancomycin/ampicillin and meropenem. TORCH work up, metabolic evaluation and karyotype were normal. At birth, blood pressure was
51/34. By day 5 of life, it ranged from 96-105/60. She was put on sodium nitroprusside, esmolol, labetalol drip, milrinone drip, Lasix and hydralazine. She failed to respond and phenoxybenzamine was added. She was on Actigall for hyperbilirubinemia. On abdominal sonogram, a complex, cystic mass was seen in the right adrenal. This was
interpreted as an evolving adrenal hemorrhage. CT scan of the abdomen confirmed this finding and showed possible vascular compromise to the right kidney. Pertinent lab data: dopamine 1520 pg/mL, epinephrine 316 pg/mL, norepinephrine 4484 pg/mL, plasma renin activity 121.67 ng/mL/hr. Plasma metanephrine was elevated. Significant physical exam findings included: severe jaundice, distended abdomen with hepatomegaly. A hard palpable mass on the anterior axillary margin of the left arm was suspicious for metastatic neuroblastoma. No neuroblastoma was present in the biopsy. The infant became hypoxic and bradycardic. She developed asystole and died on her twenty-fifth day of life.

Diagnostic Notes:

The e-mailed photomicrograph showed choroid plexus of the autopsy brain. Subendothelial vascular wall cells, only in choroid plexus and not in vessels of other tissues, were large and vacuolated. Other brain findings were hypoxic including in the pons and subiculum as well as in the, hippocampal CA-1 region. The question arose as to the nature of the subendothelial foamy cells in the vessels in the choroid plexus stroma. Choroid plexus epithelium is well preserved, so artifact was questionable but not ruled out. Whipple's disease had also been considered. Other stains were considered by the group but based on the current findings of subendothelial foamy cells in the vessels in the choroid plexus stroma no specific diagnosis was offered.

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