The PAGOD syndrome refers to a rare combination of congenital malformations associated with secondary regression of midline structures including the gonadal anlage.


Inheritance: autosomal recessive


An affected patient will be phenotypically female with 46 chromosomes but may be XX or XY.



(1) P: pulmonary hypoplasia with hypoplasia of the accompanying pulmonary artery

(2) AG: agonadism

(3) O: omphalocele and/or diaphragmatic defect

(4) D: dextrocardia


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