Abboy et al. (2008) described a brother and sister, both of whom died at less than 1 hour of age, with recurrent nonimmune hydrops fetalis of unknown etiology (see 236750). At birth, both individuals were noted to have ... Abboy et al. (2008) described a brother and sister, both of whom died at less than 1 hour of age, with recurrent nonimmune hydrops fetalis of unknown etiology (see 236750). At birth, both individuals were noted to have marked edema of the entire body, slightly low set ears, small nose, mild micrognathia, small chest, camptodactyly, single transverse palmar creases, rhizomelic shortening of the upper extremities, slight ankle varus deformity bilaterally, and bilateral humeral fractures. Radiographs and autopsies on both patients showed diffuse soft tissue edema, gracile bones (with poor columnization of chondrocytes), especially in the upper extremities and ribs, and bilateral humeral fractures. Both sibs also had extreme pulmonary hypoplasia, hypoplastic adrenal glands, absence of germ cells, polyhydramnios, and normal or near normal fetal movements.