Kozlowski-Warren-Fisher Syndrome

At a glance

It is a lethal malformative syndrome characterized by cloverleaf skull and bone dysplasia in stillborn.

Synonym

Cloverleaf Skull Generalized Bone Dysplasia Syndrome.

Incidence

One reported case is reported in the literature.

Genetic inheritance

Male infant stillborn at 35 weeks’ gestation. The mode of genetic inheritance remains unknown.

Clinical aspects

Cloverleaf deformity of the skull, with extremely broad anterior fontanelle, frontal, and coronal sutures, small posterior fossa and foramen magnum, and gross hydrocephaly. Midfacial hypoplasia with shallow orbits, a narrow chest with long, narrow lungs, normal heart except for a large foramen ovale with a fenestrated, diaphanous flap were further findings. The limbs were short with the proximal segment being most severely affected. The abdominal contents were morphologically normal, but there were widespread petechial hemorrhages of the organs.

Anesthetic considerations

There is no anesthetic implication in this lethal medical condition at birth.

References