Cureus. 2026 Jun 7;18(6):e110387. doi: 10.7759/cureus.110387. eCollection 2026 Jun.
ABSTRACT
Neurofibromatosis type 1 (NF1) is an autosomal dominant multisystem disorder that presents significant challenges to the anesthesiologist, particularly in the obstetric setting. The presence of neurofibromas along the airway, potential spinal and intracranial tumors, cardiovascular instability, and altered pharmacological responses collectively complicate anesthetic planning. We report the case of a 42-year-old primigravida at 40 weeks and two days of gestation with previously undiagnosed NF1, admitted for emergency cesarean section under the clinical suspicion of uterine rupture. General examination revealed extensive cutaneous lesions and bilateral breast masses consistent with cutaneous and plexiform neurofibromas. Airway assessment identified a Mallampati grade III classification. Given the strong clinical suspicion of NF1, the absence of prior neuroimaging, and the emergency nature of the procedure, general anesthesia was chosen over neuraxial anesthesia. Rapid sequence induction was performed with propofol and rocuronium, with full difficult airway equipment immediately available. Direct laryngoscopy yielded a Cormack-Lehane grade I view with no glottic lesions identified, and orotracheal intubation was accomplished without difficulty. Anesthesia was maintained with isoflurane in an oxygen/air mixture (fraction of inspired oxygen (FiO2) 0.4). The procedure lasted 45 minutes, hemodynamic stability was maintained throughout without vasopressor support, and a healthy female neonate was delivered with Apgar scores of 9, 10, and 10 at one, five, and 10 minutes. Emergence from anesthesia was smooth and uneventful. This case highlights the importance of systematic clinical recognition of NF1 at admission, thorough airway evaluation, appropriate anesthetic decision-making in the absence of neuroimaging, and the critical role of anticipatory preparation in emergency obstetric scenarios involving undiagnosed multisystem disease.
PMID:42416955 | PMC:PMC13338750 | DOI:10.7759/cureus.110387