From the Division of Obstetric Anesthesia and.
Department of Obstetrics and Gynecology, Center for Perinatal Care, Child Health and Development, Kitasato University Hospital, Sagamihara City, Japan.
A A Pract. 2021 Feb 11;15(2):e01400. doi: 10.1213/XAA.0000000000001400.
Jeune syndrome, also known as asphyxiating thoracic dystrophy, is a rare form of autosomal recessive skeletal dysplasia. Respiratory distress due to thoracic and lung dysplasia is the primary complication associated with this disorder in neonates. Women with Jeune syndrome seldom conceive and give birth, as only a few survive until adulthood. Herein, we report the world's first case of a cesarean delivery under spinal anesthesia in a pregnant woman with Jeune syndrome with a history of chest wall reconstruction and spinal fusion surgeries.
Jeune 综合征,也称致死性骨发育不良,是一种罕见的常染色体隐性遗传性骨骼发育不良。新生儿因胸肺部发育不良导致呼吸困难是这种疾病的主要并发症。Jeune 综合征患者很少怀孕生育,因为只有少数患者能存活至成年。在此,我们报道了首例 Jeune 综合征孕妇行剖宫产的病例,该孕妇曾有过胸壁重建和脊柱融合手术史,采用了椎管内麻醉。