title: Sibs with the polyasplenia developmental field defect.
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.
authors.affiliation: Blodgett Memorial Medical Center, Genetics/Birth Defects/Neurology Clinic, Grand Rapids, MI 49506.
authors.forename: H V
authors.initials: HV
authors.lastname: Toriello
authors.forename: N
authors.initials: N
authors.lastname: Kokx
authors.forename: J V
authors.initials: JV
authors.lastname: Higgins
authors.forename: R
authors.initials: R
authors.lastname: Hofman
authors.forename: D F
authors.initials: DF
authors.lastname: Waterman
mesh_terms: D000015:Abnormalities, Multiple / Q000235:genetics*; D006330:Heart Defects, Congenital / Q000235:genetics; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D013154:Spleen / Q000002:abnormalities*