Anesthetic Consideration in a Case of Von Hippel–Lindau Disease for Cervical Intramedullary Cyst Excision

Keywords

von Hippel—Lindau - anesthetic consideration - neuromonitoring

Introduction: Von Hippel–Lindau (VHL) disease is an inherited autosomal dominant disorder with predilection to develop characteristic tumors in the cerebellar hemispheres, retina, brain stem, and spinal cord with a prevalence of approximately 1/50,000.

Methodology/Description: A 29-year-old, 55-kg woman presented with tingling and numbness in all four limbs, difficulty in walking, constipation, and urge for micturition. MRI revealed multiple cystic enhancing lesions in cervical and dorsal cord with significant cord edema. Diagnosed with VHL disease, pancreatic cyst, and diabetes mellitus for past 5 years on treatment. Multiple hemangiomas in retina. Operated for right partial nephrectomy 5 years back. Patient had a Glasgow Coma Scale (GCS) of 15/15 preop. Power of 4/5 on right side, 5/5 on left side. Routine hematological, biochemical values, thyroxin, cortisol, chest X-ray, serum electrolytes, calcium and phosphorus levels, 12-lead electrocardiogram, and echocardiogram were normal. Patient was posted for cervical laminectomy and intramedullary cyst excision from C2 to C7 level. Routine monitors were attached, left radial artery was cannulated. Patient induced with inj. midazolam, fentanyl, propofol, and atracurium. Anesthesia maintained with oxygen–air mixture with sevoflurane (<0.5 MAC), dexmedetomidine, and propofol infusion. Intraop neuromonitoring—somatosensory evoked potential (SSEP), motor evoked potential (MEP), bispectral index, neuromuscular monitoring done. MEP—amplitude decreased < 50 intraoperatively with baseline values, while SSEP were maintained. Postoperatively, patient had right upper and lower limb power grade 0/5, which improved significantly by POD-3 to preop level 4/5. Patient was discharged on POD-8.

Conclusion: There is very little literature available for anesthetic management in a VHL disease. Anesthetic management of VHL is by itself a challenge; this case was more challenging due to the presence of cervicodorsal and pancreatic cyst with multiple hemangiomas. The case was successfully managed by vigilant clinical and invasive hemodynamic and neuromonitoring with successful outcome.

• References

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