Trisomy 18 may present features, which overlap with Pena-Shokeir syndrome, in particular craniofacial, limbs and intrathoracic abnormalities. Non lethal forms of arthrogryposis present the same set of findings, except for the pulmonary hypoplasia 4 08D0C9EA79F9BACE118C8200AA004BA90B02000000080000000E0000005F005200650066003400330039003900330030003400320031000000 . Pena-Shokeir is a lethal condition. A significant number of the affected fetuses are born prematurely. Among the survivors, the majority dies within few weeks of life 5 08D0C9EA79F9BACE118C8200AA004BA90B02000000080000000E0000005F005200650066003400330039003900330030003600310033000000 .
Pulmonary complication is the main cause of death.
Termination of pregnancy can be offered before viability. Standard prenatal care should be changed throughout the pregnancy only for maternal indications. Pena-Shokeir phenotype in Smith s recognizable patterns of human malformation. WB Saunders Company Philadelphia 1997, 174-175. Moore D.C. Prenatal sonographic diagnosis of Pena-Shokeir syndrome type I, or fetal akinesia deformation sequence.