Case report shows successful duodenal gastrointestinal stromal tumor resection in a patient with neurofibromatosis type 1, indicating effective anesthesia management strategies.
Introduction: Neurofibromatosis type 1 (NF1), also known as Von Recklinghausen disease, is a rare autosomal dominant neurocutaneous disorder. It is clinically characterized by multiple café au lait macules, intertriginous freckling, multiple cutaneous neurofibromas and learning disability or behavior problems. About half of the people with Neurofibromatosis type 1 (NF1) develop internal plexiform neurofibromas and may remain undiagnosed. Other organ systems can also be affected. Choosing the appropriate anesthesia for patients with neurofibromatosis is a unique challenge. Case Presentation: This case report presents the perioperative management of a 54-years-old male diagnosed with the challenging condition of neurofibromatosis type 1 (NF1) who underwent surgery for resection of a duodenal gastrointestinal stromal tumor (GIST). To mitigate the risks associated with a possible difficult airway and neuraxial anesthesia, an awake videolaryngoscopy before induction was done, and a bilateral Transversus Abdominis Plane (TAP) block was performed for pain management. The operation proceeded uneventfully, recovery was without complications and with satisfactory postoperative pain control. The patient reported minimal discomfort and did not require additional analgesics during the recovery period. He was discharged home in good general condition on the fifth postoperative day. Conclusion: Optimal anesthetic care in NF1 is inherently patient-specific, driven by systematic assessment of organ involvement and anticipation of perioperative complications.
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Mechkaroska et al. (2025) studied this question.
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