Authors: Kramchi Y et al.
Cureus, June 7, 2026.
Summary
This case report describes the emergency anesthetic management of a 42-year-old woman at 40 weeks and two days of pregnancy who was found to have previously undiagnosed neurofibromatosis type 1 immediately before an emergency cesarean section.
The cesarean delivery was requested because of suspected uterine rupture based on severe uterine tenderness and hypertonicity. The urgency of the situation did not allow time for neuroimaging or a complete neurologic and cardiovascular evaluation.
Physical examination revealed widespread café-au-lait macules, hundreds of cutaneous neurofibromas, and large bilateral breast and nipple-areolar masses considered consistent with possible plexiform neurofibromas. Dermatology agreed that the clinical findings met diagnostic criteria for neurofibromatosis type 1.
The diagnosis created several important anesthetic concerns. Neurofibromas may involve the tongue, pharynx, larynx, or supraglottic airway and cause unexpected difficult ventilation or intubation. Clinically silent spinal or intracranial tumors may also make neuraxial anesthesia hazardous.
Additional concerns included possible renovascular hypertension, occult pheochromocytoma, respiratory disease, and an unpredictable response to neuromuscular blocking medications.
Because no spinal or intracranial imaging was available, the team selected general anesthesia rather than spinal or epidural anesthesia. This was considered the most defensible option in the emergency circumstances, although neither general nor neuraxial anesthesia is automatically safe in patients with neurofibromatosis type 1.
Airway examination showed:
• Mallampati class III
• Thyromental distance of 6 cm
• Normal mouth opening
• Normal neck movement
• No visible oral lesions
However, a normal oral examination could not exclude deeper laryngeal or supraglottic neurofibromas. A complete difficult-airway setup was therefore prepared, including video laryngoscopy, supraglottic airway devices, fiberoptic equipment, and surgical airway capability.
After three minutes of preoxygenation, rapid-sequence induction was performed with:
• Propofol 120 mg
• Rocuronium 50 mg
Direct laryngoscopy produced a Cormack-Lehane grade I view. No glottic or supraglottic neurofibromas were seen, and the patient was intubated without difficulty using a 7-mm cuffed endotracheal tube.
Anesthesia was maintained with isoflurane in an oxygen-air mixture. After delivery and cord clamping, fentanyl and oxytocin were administered.
The cesarean section lasted approximately 45 minutes. The suspected uterine rupture was not found, and the uterine wall was intact. Estimated blood loss was less than 500 mL.
The patient remained hemodynamically stable throughout the procedure without vasopressors or antihypertensive medications.
A healthy female infant weighing 3,100 g was delivered with Apgar scores of 9, 10, and 10 at one, five, and 10 minutes.
Emergence and extubation were uncomplicated. The patient remained stable in the recovery unit and was later transferred to the obstetric ward. Referrals were arranged for neurology, dermatology, medical genetics, and further systemic evaluation.
What You Should Know
Neurofibromatosis type 1 may be recognized for the first time during an emergency through careful physical examination.
Neither general nor neuraxial anesthesia is inherently safe in these patients.
Neuraxial anesthesia may be risky when spinal tumors, intracranial lesions, or elevated intracranial pressure have not been excluded.
General anesthesia carries the possibility of an unexpectedly difficult airway because internal neurofibromas may be clinically silent.
A normal oral examination does not exclude laryngeal or supraglottic disease.
The uncomplicated intubation in this patient should not reduce the need for complete difficult-airway preparation in other patients with neurofibromatosis type 1.
Patients should also be assessed for hypertension, pheochromocytoma, renal artery stenosis, spinal lesions, intracranial tumors, respiratory disease, and possible malignant transformation of plexiform neurofibromas.
Quantitative neuromuscular monitoring is strongly recommended because responses to neuromuscular blocking medications may be unpredictable. Monitoring was unavailable in this case, which was an important limitation.
Rocuronium with sugammadex immediately available may be used for rapid-sequence induction, but sugammadex availability does not replace quantitative neuromuscular monitoring.
This case demonstrates that emergency general anesthesia can be performed successfully in a patient with previously undiagnosed neurofibromatosis type 1 when the disease is recognized quickly and comprehensive airway preparation is completed.
Thank you to Cureus for allowing us to summarize this article.