What is the best approach to manage a patient with a T5-T6 syrinx (thoracic spine syrinx) causing nipple pain?

Management of T5-T6 Syrinx Causing Nipple Pain

A T5-T6 thoracic syrinx causing nipple pain requires neurosurgical evaluation for definitive treatment, as the nipple pain represents neuropathic referred pain from irritation of the T3-T5 intercostal nerves that supply the breast and nipple, and surgical intervention targeting the underlying syrinx is the only treatment that addresses the root cause.

Understanding the Pathophysiology

The nipple pain in this case is referred neuropathic pain rather than a primary breast problem. The nerve supply to the breast and nipple comes from the anterolateral and anteromedial branches of the intercostal nerves from T3 to T5, and irritation anywhere along their course can lead to breast or nipple pain 1. A T5-T6 syrinx directly affects these nerve roots, causing dermatomal pain that manifests as nipple discomfort.

This is not a breast pathology requiring mammography or breast imaging—the ACR guidelines on breast pain specifically identify spinal nerve root syndrome as an extramammary cause of perceived breast/nipple pain 1.

Immediate Diagnostic Steps

Obtain Complete Spine MRI

• Image the entire spine, not just the T5-T6 region, as syringes frequently extend beyond the initially suspected area 2
• Include brain imaging to evaluate for Chiari malformation, the most common underlying cause of syringomyelia (present in 25-50% of cases) 2
• Use comprehensive MRI sequences: T1-weighted, T2-weighted, FLAIR, T2*-weighted gradient echo, pre- and post-contrast T1-weighted, and high-resolution heavily T2-weighted 3D sequences 2

Assess for Associated Conditions

• Evaluate for Chiari malformation at the craniocervical junction 2, 3
• Look for evidence of spinal arachnoiditis, trauma, or intramedullary tumors 3, 4
• Document the complete extent of neurological symptoms beyond nipple pain (motor weakness, sensory deficits, bowel/bladder dysfunction) 3

Definitive Treatment: Neurosurgical Intervention

Primary Surgical Approach

For Chiari-associated syringomyelia (most common):

• Posterior fossa decompression with or without duraplasty is first-line treatment 2
• The goal is to re-establish physiological CSF flow in the subarachnoid spaces 3
• Cerebellar tonsil reduction may be performed during decompression to improve syrinx and symptoms 2

For non-Chiari syringomyelia:

• Treat the underlying cause (arachnoidolysis for adhesions, tumor resection if tumor-associated) 3, 4
• Shunting procedures (syringoperitoneal, syringosubarachnoid) may be considered as primary or secondary treatment 4

Post-Surgical Monitoring

• Allow 6-12 months for syrinx reduction after initial surgery before considering additional intervention 2, 5
• Symptom resolution and syrinx resolution do not correlate directly—nipple pain may persist despite radiographic improvement, or vice versa 5, 6
• If no radiographic improvement after 6-12 months and symptoms persist or worsen, additional neurosurgical intervention is indicated 2, 5

Symptomatic Pain Management During Surgical Planning or Recovery

While awaiting surgery or during the post-operative monitoring period, neuropathic pain medications are appropriate:

First-Line Neuropathic Pain Agents

• Secondary-amine tricyclic antidepressants (nortriptyline or desipramine): Start low, titrate slowly, administer at bedtime 1
• Calcium channel α2-δ ligands (gabapentin or pregabalin) 1
• SSNRIs (duloxetine or venlafaxine) for peripheral neuropathic pain 1

Interventional Options for Refractory Pain

• Intercostal nerve blocks targeting T3-T5 dermatomes 7
• Pulsed radiofrequency ablation of affected intercostal nerves 7
• Spinal cord stimulator trial for severe, treatment-resistant cases 7

Medication Trial Duration

• Allow 6-8 weeks for an adequate trial of TCAs, including 2 weeks at the highest tolerated dose 1
• If partial relief, add a second first-line medication from a different class 1
• If no relief after adequate trials, consider second-line agents or pain specialist referral 1

Critical Pitfalls to Avoid

Do not pursue breast-focused workup (mammography, breast ultrasound, breast MRI) unless there are additional breast-specific symptoms or findings on examination, as this represents referred spinal pain 1.

Do not delay neurosurgical referral for symptomatic syringomyelia, as the natural history is typically one of gradual, stepwise neurological deterioration over many years 3.

Do not rush to reoperation if symptoms persist immediately post-surgery—the Congress of Neurological Surgeons specifically recommends waiting 6-12 months for syrinx reduction before considering additional intervention 2, 5.

Do not assume symptom severity correlates with syrinx size—research shows no significant relationship between syrinx dimensions and degree of disability or symptom distribution 6.