23rd Annual Pain Medicine Meeting 2024

Title: Postdural Puncture Headache in a Patient with Thoracic Arachnoid Web with known Joint Hypermobility: Considerations and Cautions

Authors: Krish T. Bharat MD, Patricia Maina MD, Navdeep Manhas MD, James Gutcho MD, Anusha Amaravathi MD, John Horton MD

Background:

Patients with connective tissue disorders can present unique challenges in their management and procedural complications due to tissue fragility.
Postdural puncture headaches (PDPH) are positional bilateral headache associated with nausea and dizziness.
Due to the formation of fragile meningeal diverticula or simple dural rents, there is an increased risk of PDPH in patients with connective tissue disorders.
The current gold standard, Epidural Blood Patches (EBPs) are unfortunately invasive and can be associated with worse outcomes in such patients.
This case highlights the need for less invasive diagnostic and therapeutic care in this population.

Case Description:

This case report presents a rare and complex scenario involving a 48-year-old female patient with a history of joint hypermobility syndrome.
Initially presenting with acute on chronic low back pain accompanied by new right lower extremity weakness and radicular symptoms, and a band-like mid-thoracic pain.
Diagnostic investigations revealed a L5-S1 radiculopathy and the unexpected finding of a thoracic arachnoid web.
During her rehabilitation, while undergoing a diagnostic thoracic spine myelogram (TSG), she experienced PDPH.
Unfortunately, conservative measures including rest, hydration, caffeine, brief course of medications did not provide much relief.

Diagnostics and Results:

Lumbar MRI revealed mild bilateral foraminal encroachment without significant stenosis, and EMG confirmed L5-S1 radiculopathy.
Initial conservative treatments (bed rest, hydration, caffeine) for patient's PDPH provided not much relief, and medications (tylenol, gabapentin, opiates) were used with limited success.
A sphenopalatine ganglion block offered brief mild improvement, but was generally well tolerated and did briefly promote improved therapy tolerance and function.
Due to persistent symptoms, an epidural blood patch (EBP) was performed 9 days later, which was unfortunately poor tolerated but ultimately did provide mild relief.
Given her medical history, there was concern for worsening radiculitis, and arachnoiditis from a more invasive option such as EBP.

Discussion:

Patients with connective tissue disorders, such as Ehlers-Danlos Syndrome (EDS), have an increased risk of PDPH due to fragile meningeal diverticula or dural rents.
Hypermobility disorders with tissue fragility are associated with spontaneous dural rupture and related headaches.
Arachnoid webs/cysts are rare but spinal meningeal cysts may be linked to connective tissue disorders.
Preliminary studies have shown EBPs, while usually well tolerated, may be poorly tolerated in this population and increase risks such as meningeal irritation, and worsening radiculopathy.
Certain European Anesthesia guidelines recommend avoiding neuraxial procedures in EDS patients, highlighting the need for alternative approaches.

Conclusion:

Further research is needed to explore the relationship between connective tissue disorders and poor EBP outcomes.
Less invasive methods, such as Greater Occipital Nerve Blocks and Sphenopalatine Ganglion Blocks, should be considered before EBPs in managing PDPH in these patients.
Emphasizing less invasive diagnostic and therapeutic procedures is crucial when treating patients with connective tissue disorders.

References:

Schievink, W.I., Gordon, O.K. and Tourje, J. (2004) Connective Tissue Disorders with Spontaneous Spinal Cerebrospinal Fluid Leaks and Intracranial Hypotension: A Prospective Study. Neurosurgery, 54, 65-70.
Wiesmann, T., Castori, M., Malfait, F., & Wulf, H. (2014). Recommendations for anesthesia and perioperative management in patients with Ehlers-Danlos syndrome(s). Orphanet journal of rare diseases, 9, 109.
Henderson Sr, F. C., Austin, C., Benzel, E., Bolognese, P., Ellenbogen, R., Francomano, C. A., ... & Voermans, N. C. (2017, March). Neurological and spinal manifestations of the Ehlers–Danlos syndromes. In American Journal of Medical Genetics Part C: Seminars in Medical Genetics (Vol. 175, No. 1, pp. 195-211).
Zetlaoui, P. J., Buchheit, T., & Benhamou, D. (2022). Epidural blood patch: A narrative review. AnaesthesiaCritical Care & Pain Medicine, 41(5), 101138.
Azzi, A. et al. (2022). Correlates of PDPH and efficacy of different treatment options. British journal of pain, 16(2), 228–236.

POSTDURAL PUNCTURE HEADACHE IN A PATIENT WITH THORACIC ARACHNOID WEB AND KNOWN JOINT HYPERMOBILITY: CONSIDERATIONS AND CAUTIONS