Clinical Pearl: Spina Bifida C1-2 – Physical Therapy Approach to Management of Associated Headaches and Neck Pain

Spina Bifida Occulta is a relatively mild form of neural tube defect in comparison to its counterparts meningocele, myelomeningocele, and closed neural tube defects. Eubanks et al indicates a 12% occurrence of Spina Bifida Occulta in the United States, while the National Institute of Neurological Disorders and Stroke rates this within a range of 10-20% of the population. Individuals afflicted with Spina Bifida Occulta could live a lifetime without the knowledge that their system is affected, as this form of the disorder generally does not present with symptoms but rather is diagnosed secondarily after trauma requires radiological examination of the spine. Therefore, Spina Bifida Occulta rarely has a direct correlation to dysfunction, but may inherently alter the stability or function of the affected segment or segments if impacted by external stimuli.

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Von Torklus and Gehweiler verified the formation of the cervical spine begins in the 2nd intrauterine month of gestation while complete ossification does not occur until the age of 10. There are innumerable steps in this complicated and fascinating process that can be impacted, leading to spina bifida, anencephaly, and chiari malformation. It is thought that Folic Acid, a B vitamin, may play a role in successful completion of this cascade of events.

The following case report will highlight a patient who presented with migraine variant headaches with residual tension component that had known C1-C2 Spina Bifida Occulta, and will outline the Physical Therapy approach to treatment of associated chronic symptoms.

History and Subjective Reports

An otherwise medically uncomplicated 55-year-old female presented for Physical Therapy evaluation with reports of mixed migraine and tension headaches that were initially diagnosed at age five. Patient was afflicted for several years when symptoms became dormant in her teens and generally remained so until approximately 1993 when the patient was then 33 years old in correlation with pregnancy. At the time of return, the patient does not recall any trauma, rather waking abruptly with severe neck pain and headache, subsequently provoking nausea and vomiting. This had recurred 3-4 times per year, but eventually settled on daily cervicogenic headaches with more notable migraine-type symptoms at least several days per week. Subjectively, the patient depicted the symptoms as, feels like somebody is taking a pliers to the top of my head and ripping it apart. There was also notable orbital pressure, superior molar pain bilaterally, and occasional posterior cranial pool of liquid sensation that was otherwise vetted out by her physicians.

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While in the midst of an exacerbation, patient took 800 mg of Ibuprofen, applied black eye patches, and got fresh air that typically calmed the more potent and otherwise debilitating symptoms. This patient described her olfactory sense as overactive and found shiny colors such as red and orange to be provoking of her symptoms. She had intermittent fullness in the right ear without tinnitus. Her blood pressure tended to be low, but without immediate hypotensive diagnosis. With the exception of post botulinum toxin injection, the patient did not indicate any upper extremity radicular or somatic referred pain.

Prior Treatment

  • Botulinum Toxin Injection ( 2008 )
    – Procedure: 100 units of Botulinum Toxin Type A diluted in 1 mL of normal Saline injected into the Left Trapezius in a grid fashion via a 25-gauge 2-inch needle.
    – Response: Patient reported left upper extremity pain and paralysis that lasted for several weeks with full recovery post.
  • Left levator scapula, left rhomboid and posterior thoracic trapezius Trigger Point Injections ( 2009 initial with subsequent repeats PRN ).
    – Procedure: 2 mL with 6.6 mg Triamcinolone and a 2-to-1 mixture of 0.5% bupicavaine and 1% lidocaine with patient receiving 40 mg of triamcinolone via a 25-gauge needle.
    – Response: Patient reported short-term reduction in headaches and cervical spine tension requiring PRN use.
  • Acupuncture and Chiropractic ( 2005 ) Patient reported no sustained benefit.
  • Deep Tissue Massage completed weekly since 2005 moderate, short-term benefit.
  • Repeat of Chiropractic treatment ( 2015) with Cervical Spine Manipulative therapy approach (upper cervical spine included) Patient reported initial benefit with significant exacerbation of symptoms within 3 weeks of 3x/week treatments. Specific technique unknown.

Radiographic Examination

  • MRI Cervical Spine 2005
    – MRI completed from Foramen Magnum to T3 without contrast.
    – Minimal bulging of the annulus fibrosus at the C6 interspaces without central canal spinal stenosis or spinal cord compression.
    – Small focal disk protrusion extended to left of the midline at the C5 interspace did not result in spinal cord or nerve root compression.
    – The Cervical Spinal Cord looked normal.
    – Minor developmental anomaly, hypoplastic left lamina of C2 with spina bifida.
  • Non-contrast CT Cervical Spine 2008
    – Congenital anomalies of the C1 and C2 vertebrae.
    – Congenital nonunion of the posterior arch of C1. The remainder of the C1 vertebrae is normal.
    – Mild hypoplasia of the left C2 lamina.
    – Congenital non-fusion of the posterior arch of the C2 vertebra.
    – Minimal narrowing of the AP diameter of the central canal at the C2 level.
    – Bifid spinous process of C2 the left segment of the spinous process was free floating, that is, not joined to the left lamina or right aspect of the spinous process.
    – Probable mild hypoplasia of the lamina bilaterally at the C3 level.
    – Neural foramina and central canal were patent.

Objective Findings Physical Therapy Evaluation, May 2015

  • Right AA mobility limited 50% upon bilateral comparison
  • OA hypomobility present bilaterally
  • Cervical Spine Side-bending: Right 19 degrees, Left 25 degrees
  • Cervical Spine Flexion limited 40 degrees with anterior shear noted at C4-5
  • Cervical Spine Extension WNL with shear present at C4-5, lacked 50% if not allowed to progress into unstable range
  • Cranial Nerve assessment intact
  • ULTT WNL bilaterally
  • Hypotonicity Right RCPM, IOC
  • Hypertonicity Left RCPM, IOC, bilateral SOC, bilateral SCM, Upper trapezius, and levator scapulae right-left
  • Headache symptoms provoked with tactile compression to medial trigger point of upper trapezius and superior obliquus capitis bilaterally
  • Mild provocation of upper molar pain with tactile compression of bilateral masseter, but primarily bilateral temporalis
  • Moderate limits C3-4 facet/down-glide mobility with hypermobility C5-7 with ERSR C3-5
  • Forward Head, elevated and protracted bilateral scapulae with component of hypertoncity from pectoralis musculature
  • UE strength WNL to MMT, hesitation with lattisimus and lower trapezius testing
  • Ligamentous, tectoral membrane negative
  • Moderate restriction cephelad to caudad cervicothoracic fascia

Physical Therapy Diagnosis

Patient presented with impairments to upper cervical spine mobility as well as restriction to gross planes, facet, and segmental mobility with reduced proprioceptive control noted at C4-5 with flexion and extension. Neuromyofascial pain and restriction present throughout the cervicothoracic spine with headache symptoms provoked with compression to bilateral upper trapezius and superior obliquus capitis. There was also immediate referral noted with tactile compression of bilateral temporalis musculature to known molar patterning. Patient did appear to have intact ligamentous structures, no notable alteration to upper extremity neural mobility and presented without upper extremity radicular symptoms.

Initial Treatment

  • Functional Dry Needling ( FDN ) to bilateral upper trapezius ( medial trigger point at C5/6), superior obliquus capitis, and cervical multifidus C4-5. This was completed with direct, point stimulation at 10 Hz to bilateral upper trapezius and superior obliquus capitis.
  • ERSR facet MET at C4
  • MET to promote right AA mobility
  • Supine, VOR eye reflex movements for low impact, proprioceptive training to cervical spine musculature was completed for neuromuscular re-education.
  • Educated patient on appropriate alterations to seated, work postures to reduce mechanical stress to the affected structures.
  • Began discussion of deep breathing techniques for relaxation.

Follow-up Visit completed two weeks following evaluation

  • Subjective: Headaches were resolved for one week with minimal return subsequently. Range of motion was improved, most noted with driving. Patient indicated concentration to complete tasks at work was greatly improved secondary to significant reduction in headaches and pain status.
  • Objective:
    – Right Cervical Spine Side-bending 40 degree ( 19 upon evaluation )
    – Left Cervical Spine Side-bending 34 degree ( 25 degree upon evaluation )
    – Unable to provoke headache referral upon tactile compression of medial trigger point of bilateral upper trapezius, increased tone/headache referral midsubstance
    – Right AA mobility 25% upon bilateral comparison ( 50% upon evaluation )
  • Treatment:
    – Supine and seated deep neck  flex-or activation with focus to segmental stabilization with patient implemented tactile cue at C4-5.
    – Gaze reflex for re-education with target at the wall. Patient to begin with rotation and progress with flexion/extension.
    – FDN to bilateral mid-substance upper trapezius and C3-T1 multifidus( multifidus indwelling with ES 130 for 10 minutes )
    – Cross friction massage right proximal SCM
    – Grade II-III right mid-cervical down-glide mobilization
    – Tactile cue C3-4, C4-5 with supine deep neck flex-or activation

Outcomes

  • Improved upper cervical spine and gross planes, most notably side-bending
  • Reduced frequency and intensity of headache symptoms leading to improved concentration on work tasks
  • Improved myofascial restrictions and resolved upper trapezius headache referral to midline points as well as superior obliquus capitis
  • At follow-up, patient was provided contacts for continued treatment in Minneapolis, as she was relocating for work the week following her last visit.

Conclusion

During the short duration that the patient was evaluated and able to participate in treatment including manual therapy, neuromuscular re-education principles, and self care techniques, she was able to demonstrate gains both objectively and subjectively in what had previously been very longstanding and debilitating symptoms. Although there were apparent neuromyofascial and musculoskeletal restrictions about the cervical spine, it does not allow for direct correlation to primary impact from the known C1-C2 spina bifida occulta. As with many patients afflicted with Spina Bifida Occulta, this patient was unaware of this anomaly until radiological examination was requested secondary to unchanging and debilitating migraine-type symptoms. Due to the relative rare occurrence of upper cervical Spina Bifida Occulta, with most research indicating a 90% occurrence within the lumbar spine and only 2-4% impacting the posterior arch in the upper cervical levels, there is little for comparison on presenting symptoms of both migraine and tension headaches. In isolation of direct causation, the patient was demonstrating positive gains with only two treatment sessions. In the same light, due to restricted treatment timeframe, a conclusion on prolonged impact is unknown.

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