Anesthetic management of a pregnant woman with Gorham–Stout disease

Gorham–Stout disease is a rare disorder of bone loss and proliferation of lymphatic and vascular tissue (lymphangiomatosis). A 30-year-old nulliparous woman with Gorham–Stout disease presented at 8 weeks of gestation with a fused cervical spine. At 31 weeks she developed basilar invagination and neurological symptoms that were managed with a neck brace. Anesthetic considerations were those of airway compromise, development of severe preeclampsia and Kasabach–Merritt coagulopathy. Elective tracheostomy was declined. She presented two days before a planned cesarean delivery at 35 weeks in preterm labor. A semi-urgent cesarean delivery under spinal anesthetic proceeded uneventfully, with an otolaryngologist present in case a surgical airway was required. Mother and baby were discharged home after three days. Maternal postpartum recovery was complicated by episodes of respiratory compromise and critical bone loss in the cervical spine, necessitating further surgical reinforcement.