Sibs with the polyasplenia developmental field defect

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Abstract
We report on two brothers each with absent spleen, multiple cardiac defects, and varying degrees of situs inversus. One brother also had omphalocele; the other also had annular pancreas. These findings are consistent with a diagnosis of polyasplenia, a complex developmental field defect affecting visceral and cardiac situs, with associated spleen and heart defects. We also discuss familial cases from a developmental field perspective.