Spinal Anesthesia in a Respiratory-Compromised Patient With Fabry Disease Undergoing Umbilical Hernia Repair: A Case...

Fabry disease (FD) is a rare X-linked lysosomal storage disorder associated with multisystemic involvement, making anesthetic management challenging due to potential cardiomyopathy, renal insufficiency, and airway difficulties. We present the case of a 42-year-old female patient with FD and significant respiratory comorbidities, including active smoking and moderate-to-severe airflow obstruction (FEV1: 50% predicted), alongside a body mass index (BMI) of 32.3 kg/m², presenting for umbilical hernia repair. Given the high risk of perioperative respiratory complications, spinal anesthesia was selected. A subarachnoid block performed at L2-L3 achieved a T6 sensory level. The patient maintained hemodynamic stability without vasopressor support and experienced no respiratory or neurological complications. This case contributes to the scarce literature on neuraxial anesthesia in FD, demonstrating that spinal anesthesia can be safely extended to abdominal wall procedures requiring higher dermatomal levels (T6), provided that hemodynamic monitoring is rigorous.