# Anaesthetic Management of Neurofibromatosis of an Unknown Subtype for Emergency Caesarean Section

## TL;DR

This paper discusses the safe use of spinal anesthesia in a pregnant woman with undiagnosed neurofibromatosis during an emergency caesarean section.

## Contribution

It presents a case demonstrating that spinal anesthesia can be safely used in unclassified neurofibromatosis when imaging is unavailable.

## Key findings

- Spinal anesthesia was safely administered without neurological or cardiovascular complications.
- Phenylephrine boluses effectively managed spinal-induced hypotension.
- The case supports individualized clinical judgment over routine exclusion of neuraxial techniques in such scenarios.

## Abstract

Neurofibromatosis is a heterogeneous group of inherited neurocutaneous disorders with variable multisystem involvement and important implications for obstetric anaesthesia. Anaesthetic concerns include potential airway involvement, occult spinal or intracranial tumours, cardiovascular instability, and the association with catecholamine-secreting tumours. These factors often lead to a preference for general anaesthesia, despite well-recognised maternal risks associated with general anaesthesia in obstetric practice. Decision-making is further complicated when neurofibromatosis is previously undiagnosed and unclassified, and when preoperative imaging is unavailable. We report the case of a 33-year-old gravida 3 para 2 woman at term gestation who required emergency caesarean delivery for fetal distress after declining a trial of vaginal birth following a previous caesarean section. She had extensive cutaneous neurofibromas but no prior diagnosis, genetic classification, or radiological evaluation. The subtype of neurofibromatosis was therefore unknown at presentation. Clinical assessment revealed no neurological symptoms, no focal neurological deficits, no features suggestive of raised intracranial pressure, no clinical evidence of airway or mediastinal involvement, and no history suggestive of catecholamine excess. The case occurred in a resource-limited setting, where urgent neuroimaging was not available. Following senior anaesthetic assessment and informed consent, spinal anaesthesia was selected after careful consideration of the relative risks of neuraxial versus general anaesthesia in the context of diagnostic uncertainty and obstetric urgency. Intrathecal hyperbaric bupivacaine with fentanyl was administered, producing an adequate surgical block. Anticipated spinal-induced hypotension was managed with titrated phenylephrine boluses, and haemodynamic stability was maintained throughout the procedure. The caesarean section and postoperative course were uneventful, with no neurological or cardiovascular complications. This case highlights the importance of phenotype-based clinical risk stratification when managing parturients with suspected but unclassified neurofibromatosis. In the absence of neurological deficits or features suggestive of raised intracranial pressure, neuraxial anaesthesia may be a reasonable option even when imaging is unavailable. This approach may be particularly relevant in emergency obstetric scenarios and resource-limited settings, where delays associated with investigation may increase maternal and fetal risk. The case adds to limited evidence supporting judicious use of spinal anaesthesia in selected parturients with suspected neurofibromatosis and underscores the need for individualized clinical judgement rather than routine exclusion of neuraxial techniques.