Neurofibromatosis Surgery in Singapore

Several clinical scenarios may prompt consideration of surgery:

• Progressive neurological deficit may represent an indication. When a tumour compresses a nerve to the point of causing weakness, numbness, or loss of function, early intervention may help prevent permanent damage. The window for nerve recovery narrows as compression duration increases.
• Intractable pain that impairs daily function is another common indication. Neurofibromas can cause localised pain from nerve irritation or referred pain along nerve distribution patterns. When conservative measures—medication, physical therapy, positioning modifications—fail to provide adequate relief, surgical removal may address the pain source directly.
• Rapid tumour growth warrants attention. Whilst neurofibromas typically grow slowly, a sudden size increase may indicate transformation towards a more aggressive tumour type, necessitating prompt surgical assessment.
• Cosmetic and psychosocial burden should not be dismissed. Visible tumours, particularly on the face or hands, can significantly affect quality of life, social interactions, and psychological well-being. Surgical removal in these cases addresses legitimate health concerns.

Surgery is not appropriate for all patients. Situations where surgery may not be recommended include:

• Stable, asymptomatic tumours that pose no functional threat
• Extensive plexiform tumours where removal would cause unacceptable nerve damage
• Significant medical conditions that increase surgical risk disproportionately
• Patient preference for continued observation when clinically appropriate

The principle of individualised care means that two patients with similar tumours may receive different recommendations based on their complete clinical picture.

Neurofibroma surgeries are typically performed under general anaesthesia, ensuring patient comfort and immobility during delicate dissection. For smaller, superficial tumours, local anaesthesia with sedation may be appropriate.

Patient positioning is determined by tumour location:

• Tumours on the trunk may require prone (face-down) or lateral (side-lying) positioning
• Extremity tumours typically allow supine positioning with the affected limb prepared and draped separately
• Head and neck tumours may require specialised positioning with head stabilisation

Proper positioning aims to provide surgical access whilst maintaining patient safety—avoiding nerve compression points and ensuring adequate ventilation.

The surgical phase begins with careful incision planning. Incisions are designed to provide adequate exposure whilst respecting aesthetic considerations and avoiding unnecessary tissue disruption.

Once the tumour is visualised, dissection proceeds along the tumour capsule. Microsurgical technique—using magnification and fine instruments—allows identification of individual nerve fascicles entering and exiting the tumour. Where possible, these fascicles are preserved; where tumour infiltration makes preservation impossible, the surgeon must balance complete removal against functional loss.

Applying his training in microsurgery, Dr Goh employs meticulous dissection techniques that prioritise neural preservation whilst achieving safe tumour removal—recognising that preserving nerve function often matters more to patients than achieving completely clear margins.

Intraoperative nerve monitoring may be employed for tumours involving motor nerves. Electrical stimulation identifies functional nerve tissue, allowing the surgeon to distinguish viable nerve from tumour and adjust the dissection accordingly.

Wound closure depends on the extent of tissue removed and the anatomical location. Simple closures are possible when adequate tissue remains. Larger defects may require:

• Local tissue rearrangement (flaps) to close wounds without tension
• Skin grafting for surface coverage
• Microsurgical reconstruction for complex defects requiring tissue transfer from distant sites

The removal of neurofibromas often involves intricate work around nerve pathways and the skin, which is why the expertise of a plastic surgeon is vital. Unlike general excision, plastic surgery focuses on form and function—using reconstructive microsurgery to repair defects left behind by the tumour.

This may include local tissue rearrangement, skin grafting, or microsurgical flaps to restore natural contours and preserve movement, particularly in the face or extremities.

Active surveillance (watchful waiting with regular monitoring) remains appropriate for many patients. Stable, asymptomatic tumours that do not threaten neurological function can be safely observed with periodic clinical examination and imaging.

Surgery may become preferable when:

• Surveillance reveals progressive growth
• Symptoms develop or worsen
• Tumour characteristics suggest transformation risk

The decision to transition from observation to surgery is rarely urgent. Patients typically have time to consider options, seek second opinions, and prepare mentally and practically for surgical intervention.

Choosing surveillance initially does not preclude surgery later. Many patients are monitored for years before circumstances change sufficiently to warrant surgical consideration.

Medical therapies for neurofibromatosis have historically been limited, but recent developments offer new options for certain patients.

MEK inhibitors (a class of targeted therapy) have shown effectiveness in shrinking some plexiform neurofibromas, particularly in children. These medications may reduce tumour size enough to defer or avoid surgery, or may shrink tumours to make subsequent surgery safer.

Medical therapy does not replace surgery for all patients. Tumours causing acute neurological compromise typically require surgical decompression regardless of potential response to medication. Additionally, not all tumour types respond equally to available medications.

The choice between medical and surgical management—or their combination—requires professional guidance based on tumour characteristics, patient age, and treatment goals.