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Key Clinical Message  Pena–Shokeir phenotype is a rare disorder. However, its etiology is incompletely understood. It may be familial or may be due to anoxic–ischemic etiology. Although rare, it can affect one twin in a monoamniotic pregnancy, most likely due to early cord entanglement.

Discordant monoamniotic twins with Pena–Shokeir phenotype