Anesthetic Management of a Rare Congenital Limb Anomaly with Rh-Negative Pregnancy: A Cesarean Section Case Report

Dr. Shilpa S

Abstract: We present a rare and challenging case of a pregnant woman with tetraphocomelia, an extremely rare congenital limb anomaly characterized by the absence or severe hypoplasia of all four limbs, scheduled for elective cesarean section. The patient was Rh-negative and had a history of previous lower segment cesarean section (LSCS). This unique combination of anatomical deformity, immunohematologic considerations, and obstetric history required a highly individualized multidisciplinary approach to perioperative anesthetic planning. Key considerations included positioning, vascular access, neuraxial anesthesia feasibility, airway management, and intraoperative monitoring. Phocomelia is a rare birth defect that results in significant abnormalities in limb development. It often involves underdeveloped or missing upper limb bones, and the fingers may be fused together. In the most severe cases, known as tetraphocomelia, both the arms and legs are absent, making the hands and feet appear to be directly connected to the body. This condition can be caused by genetic factors or exposure to certain drugs. Genetically, it is usually inherited as an autosomal recessive trait and is linked to various chromosomal abnormalities. It may also arise from spontaneous genetic mutations.

Keywords: tetraphocomelia, congenital limb deformity, cesarean anesthesia, multidisciplinary management, genetic mutation