Abstract
Cystic fibrosis (CF) is a genetic disorder that primarily affects the respiratory and gastrointestinal systems, often leading to significant perioperative challenges due to compromised lung function, recurrent infections, and chronic respiratory failure. Managing anesthesia in patients with CF requires careful consideration, particularly because of the increased risk of respiratory complications with general anesthesia (GA). Neuraxial anesthesia, such as spinal anesthesia, presents an alternative that can reduce the likelihood of postoperative pulmonary issues, including respiratory depression, hypoxemia, and atelectasis. However, spinal anesthesia is not without risk, particularly in the presence of coagulation abnormalities, which must be considered. We present the case of a 26-year-old male with severe CF, complicated by chronic respiratory failure and recurrent infections, who presented with acute appendicitis. The patient, with a history of bronchiectasis and chronic colonization by multidrug-resistant organisms, had stable but significantly impaired respiratory function, with a forced expiratory volume in one second (FEV1) of 1.62 L (38% predicted). He had a history of multiple hospital admissions for exacerbations of lung disease, none of which required mechanical ventilation or intensive care, with the most recent admission occurring 13 months prior to this event. Preoperative coagulation studies revealed an elevated international normalized ratio (INR) of 1.52 (normal range 0.8-1.2) and an activated partial thromboplastin time (APTT) of 38.1 seconds (normal <29.4 seconds), for which the patient received vitamin K without improvement. Despite these abnormalities, a thorough preoperative assessment and multidisciplinary discussion led to the decision to proceed with spinal anesthesia, carefully weighing the risks and benefits. An initial spinal anesthetic attempt with 2.0 mL of hyperbaric bupivacaine 0.5% (10 mg) and 2.5 mcg of sufentanil was administered at the L3-L4 interspace using a 27G Whitacre needle. Despite confirmed cerebrospinal fluid flow before injection, no sensory or motor block occurred, requiring an additional spinal injection. A second dose of 2.0 mL of hyperbaric bupivacaine 0.5% was administered at the L2-L3 interspace without sufentanil. A satisfactory sensory block to the T6 level was eventually obtained, allowing the appendectomy to proceed without intraoperative complications. Postoperatively, the patient was closely monitored in a high-dependency unit. He maintained stable respiratory function and experienced a smooth recovery, with minimal opioid use to avoid respiratory depression. The patient was discharged on the fifth postoperative day without respiratory or other anesthesia-related complications. This case highlights the importance of individualized care in CF patients undergoing surgery. Neuraxial anesthesia, when carefully planned and executed, can offer a safer alternative to GA by minimizing respiratory risks. However, the presence of coagulation abnormalities requires a detailed risk-benefit analysis, multidisciplinary collaboration, and vigilant intraoperative and postoperative care to ensure patient safety and optimize outcomes.