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Sibs with the polyasplenia developmental field defect

✍ Scribed by Toriello, Helga V. ;Kokx, Nicholas ;Higgins, James V. ;Hofman, Ronald ;Waterman, Donald F. ;Opitz, John M. ;Reynolds, James F.

Publisher
John Wiley and Sons
Year
1986
Tongue
English
Weight
442 KB
Volume
25
Category
Article
ISSN
0148-7299

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✦ Synopsis

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.

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