Authors: Kato (Araki) Y et al.
Source: Cureus 17(11): e96942, November 2025. DOI: 10.7759/cureus.96942.
Summary:
This case report describes the successful anesthetic management of a cesarean section in a 29-year-old parturient with Von Hippel-Lindau (VHL) disease, a rare autosomal-dominant disorder associated with cerebellar and spinal hemangioblastomas, retinal lesions, pancreatic cysts, renal cell carcinoma, and possible pheochromocytoma. The patient had multiple CNS lesions, including a cerebellar hemangioblastoma and extensive syringomyelia from C2 to T12, as well as pancreatic cysts and a small renal tumor.
Given the risk of hemangioblastoma rupture and intracranial or intraspinal pressure shifts, the anesthetic plan required careful consideration. The patient was fully counseled on three delivery-anesthetic options: vaginal delivery with epidural analgesia, cesarean section with epidural anesthesia, or cesarean section under general anesthesia. Both neuraxial techniques carried theoretical risks due to spinal lesions and the possibility of dural puncture causing intracranial pressure changes. General anesthesia posed its own concerns regarding hypertensive responses during intubation that might precipitate bleeding within CNS tumors.
At 36+4 weeks, she developed increasing contractions and proceeded to an emergency cesarean section. General anesthesia was induced using propofol, remifentanil, fentanyl, and rocuronium, with pre-intubation remifentanil given to blunt the hypertensive response. Intubation was smooth, hemodynamics remained stable, and the neonate was delivered three minutes after incision with Apgar scores of 7 and 9. Maintenance anesthesia used propofol and remifentanil with additional fentanyl. She was extubated safely, had an uncomplicated postoperative course, and was discharged with her newborn on postoperative day six.
The authors emphasize that management of VHL parturients must be individualized. Although epidural anesthesia has been used safely in other VHL cases, general anesthesia may provide better control of hemodynamic changes and avoid dural puncture risks. Pre-delivery anesthetic consultation, multidisciplinary planning, and patient-centered decision-making are essential to safe outcomes.
What You Should Know:
• VHL parturients are high risk because CNS hemangioblastomas can bleed with hemodynamic surges or intracranial pressure changes.
• Neuraxial anesthesia may be acceptable if no spinal lesions are present, but dural puncture could theoretically trigger complications.
• General anesthesia avoids dural puncture but risks hypertensive spikes, making smooth induction and tight BP control essential.
• Pre-delivery anesthetic evaluation is critical; the choice of anesthesia should be based on anatomy, symptoms, and fully informed patient preference.
• In this case, general anesthesia was chosen and successfully delivered with stable hemodynamics and good maternal–fetal outcomes.
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