Resumen

CARMONA DE UZCATEGUI, María Luisa; CHACIN, Belkys; RAMIREZ, Liliana  y  MORENO, Franklin. Osteogénesis imperfecta tipo II: Estudio de dos casos. Rev Obstet Ginecol Venez [online]. 2010, vol.70, n.2, pp.116-121. ISSN 0048-7732.

We present two clinical cases of fetuses with osteogenesis imperfecta type II, products of preterm vaginal deliveries, both females, without vital signs and congenital malformations evident in the skeletal system. Macroscopical study shows in both fetuses soft and crushed skull, collapse of the thoracoabdominal region, shortening of the limbs and external female genitalia. Pulmonary hypoplasia and intestinal malrotation are evident at the dissection. Radiological screening shows total absence of ossification for the first fetus and only rudimentary points for the base of the skull, scapula, humerus, a forearm bone, the ilium, femur and leg bon for the second fetus. Current treatment available is far from being successful and is limited to orthopedic therapy and administration of bisphosphonates. Transplantation of mesenchymal cells and their transformation into osteogenic cells, opens new perspectives on the management of the disease.

Palabras clave : Osteogenesis imperfecta; Ossification; Mesenchymal cells.