Dehydrated hereditary stomatocytosis may be associated with transient ascites or hydrops in the perinatal period. The cause for the fluid accumulation is unclear. The severity of anemia alone is an insufficient explanation.
The neonate may present with:
(1) unexplained ascites or hydrops
(2) evidence of hemolytic anemia (classic stomatocytes may be rare)
(3) a family history of hemolytic anemia or a similarly affected sibling
(4) in vitro pseudohyperkalemia
(5) splenomegaly
(6) negative workup for other causes of anemia
The fluid accumulation is transient. Ascites fluid may need to be drained either in utero or after delivery to allow for adequate respiration.
Splenectomy must be avoided because the procedure is associated with fatal thrombemboli. in patients with hereditary stomatocytosis.
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