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

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.

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Journal
Cureus
Published
2026-02-19
DOI
https://doi.org/10.7759/cureus.103898
Primary Topic
Lysosomal Storage Disorders Research
Type
article
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article

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

Panagiota Panagiotou, Paraskevi Mavridou, Christos Exarchos, Pinelopi Kitsakou et al.
Cureus
Lysosomal Storage Disorders Research
article

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

Panagiota Panagiotou, Paraskevi Mavridou, Christos Exarchos, Pinelopi Kitsakou, Chrysoula Antigoni Koutsi
article en

Abstract

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.

CureusVol. 18(2)
General Hospital of Ioannina G. Hatzikosta (GR)
Good health and well-being
Openalex Percentile: Top 49%
Lysosomal Storage Disorders Research
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Spinal Anesthesia in a Respiratory-Compromised Patient With Fabry Disease Undergoing Umbilical Hernia Repair: A Case Report — Panagiota Panagiotou, Paraskevi Mavridou, et al. · Cureus (2026) | TGRS Research Map | TGRS