Introduction
Materials and methods
Inclusion criteria for occipital-cervical fusion stabilization surgery
Results
Pre-operative findings
Symptom/problem | % Pre-surgery | % Post-surgery | %With improvement in frequency post-surgerya | % With worsening of frequency post-surgerya | p valueb | % With onset post-surgeryc |
---|---|---|---|---|---|---|
Headaches | 100% | 95% (19/20) | 85% (17/20) | 0 | < 0.001 | 0 |
Fatigue | 100% | 100% | 30% (6/20) | 15% (3/20) | NS | 0 |
Dizziness | 100% | 95% (19/20) | 70% (14/20) | 10% (2/20) | < 0.0007 | 0 |
Muscle pain | 95% (19/20) | 95% (19/20) | 36.8% (7/19) | 10.5% (2/19) | NS | 0 |
Upper extremity weakness | 90% (18/20) | 85% (17/20) | 61.1% (11/18) | 22.2% (4/18) | NS | 0 |
Joint pain | 85% (17/20) | 85% (17/20) | 29.4% (5/17) | 11.8% (2/17) | NS | 0 |
Neck pain | 85% (17/20) | 90% (18/20) | 70.6% (12/17) | 5.9% (1/17) | NS | 33.3% (1/3) |
Balance problems | 85% (17/20) | 85% (17/20) | 82.4% (14/17) | 5.9% (1/17) | < 0.0001 | 0 |
Night awakenings | 85% (17/20) | 85% (17/20) | 23.5% (4/17) | 11.8% (2/17) | NS | 0 |
Memory problems | 80% (16/20) | 80% (16/20) | 68.9% (11/16) | 0 | < 0.002 | 0 |
Walking problems | 80% (16/20) | 70% (14/20) | 68.9% (11/16) | 6.3% (1/16) | < 0.002 | 0 |
Upper extremity numbness | 75% (15/20) | 85% (17/20) | 73.3% (11/15) | 6.7% (1/15) | NS | 40% (2/5) |
Hands and feet turning cold | 75% (15/20) | 70% (14/20) | 26.75% (4/15) | 6.7% (1/15) | NS | 0 |
Lower extremity numbness | 75% (15/20) | 70% (14/20) | 60% (9/15) | 13.3% (2/15) | NS | 0 |
Visual problems | 75% (15/20) | 80% (16/20) | 53.3% (8/15) | 13.3% (2/15) | NS | 20% (1/5) |
Lower extremity weakness | 65% (13/20) | 70% (14/20) | 69.2% (9/13) | 15.4% (2/13) | NS | 14.3% (1/7) |
Vertigo | 65% (13/20) | 50% (10/20) | 92.3% (12/13) | 0 | < 0.0006 | 0 |
Hearing problems | 65% (13/20) | 65% (13/20) | 61.5% (8/13) | 15.4% (2/13) | NS (0.053) | 14.3% (1/7) |
Speech problems | 60% (12/20) | 55% (11/20) | 80% (8/12) | 8.3% (1/12) | < 0.03 | 0 |
Frequent daytime urination (>every 2 h) | 60% (12/20) | 45% (9/20) | 41.7% (5/12) | 0 | < 0.02 | 0 |
GERD | 55% (11/20) | 55% (11/20) | 36.4% (4/11) | 0 | NS | 11.1% (1/9) |
Swallowing/choking problems | 55% (11/20) | 55% (11/20) | 63.4% (7/11) | 18.2% (2/11) | NS | 22.2% (2/9) |
Nocturia (> twice a night) | 55% (11/20) | 55% (11/20) | 27.3% (3/11) | 9.1% (1/11) | NS | 11.1% (1/9) |
IBS | 50% (10/20) | 50% (10/20) | 30% (3/10) | 0 | NS | 0 |
Tremors | 40% (8/20) | 40% (8/20) | 87.5% (7/8) | 0 | NS | 0 |
Fainting | 35% (7/20) | 25% (5/20) | 85.7% (6/7) | 0 | NS | 14.3% (1/7) |
Numbness in back | 30% (6/20) | 40% (8/20) | 66.7% (4/6) | 0 | NS | 14.3% (2/14) |
Sleep apnea | 25% (5/20) | 25% (5/20) | 20% (1/5) | 0 | NS | 0 |
Patient satisfaction
Postoperative findings
Area of pain | Pre-surgery | 2 year post-surgery | p value | 5 year post-surgery | p value |
---|---|---|---|---|---|
Headaches | 8.10 | 4.35 | < 0.0001 | 5.75 | < 0.00002 |
Neck | 6.45 | 4.05 | < 0.002 | 4.7 | NS (< .091) |
Joints | 5.30 | 4.60 | NS | 3.70 | < 0.018 |
Muscles | 5.95 | 4.70 | < 0.009 | 4.45 | NS (< .069) |
Normal before surgerya | Normal after surgery | Improvement after surgeryb | No change in abnormal findingb | Worsening after surgeryc | |
---|---|---|---|---|---|
Strength | |||||
Deltoids | 15/19 (78.9%) | 18/19 (94.7%) | 4/4 (100%) | 0 | 1/15 (6.7%) |
Biceps | 15/19 (78.9%) | 18/19 (94.7%) | 4/4 (100%) | 0 | 1/15 (6.7%) |
Triceps | 12/19 (63.2%) | 17/19 (89.5%) | 7/7 (100%) | 0 | 2/12 (16.7%) |
Grips | 13/19 (68.4%) | 17/19 (89.5%) | 6/6 (100%) | 0 | 2/13 (15.4%) |
Quads | 11/19 (57.9%) | 16/19 (84.2%) | 7/8 (87.5%) | 1/8 (12.5%) | 2/11 (18.2%) |
Hamstrings | 12/19 (63.2%) | 15/19 (78.9%) | 6/7 (85.7%) | 1/7 (14.3%) | 2/12 (16.7%) |
Iliopsoas | 10/19 (52.6%) | 16/19 (84.2%) | 9/9 (100%) | 0 | 2/10 (20.0%) |
Reflexes | |||||
Biceps | 12/18 (66.7%) | 14/18 (77.8%) | 4/6 (66.7%) | 2/6 (33.3%) | 2/12 (16.7%) |
Triceps | 13/18 (72.2%) | 12/18 (66.7%) | 2/5 (40.0%) | 3/5 (60.0%) | 3/13 (23.1%) |
Patella | 10/18 (55.5%) | 12/18 (66.7%) | 5/8 (62.5%) | 3/8 (37.5%) | 3/10 (30.0%) |
Achilles | 12/18 (66.7%) | 12/18 (66.7%) | 4/6 (66.7%) | 2/6 (33.3%) | 3/12 (25.0%) |
Other | |||||
Heel to toe | 10/15 (66.7%) | 13/15 (86.7%) | 4/5 (80.0%) | 1/5 (20.0%) | 1/10 (10.0%) |
Finger to nose | 14/14 (100.0%) | 14/14 (100.0%) | NA | NA | 0 |
Rapid alternating movements | 13/13 (100.0%) | 12/13 (92.3%) | NA | NA | 1/13 (7.7%) |
Romberg | 13/16 (81.3%) | 14/16 (87.5%) | 2/3 (66.7%) | 1/3 (33.3%) | 1/13 (7.7%) |
Sensation to vibration | 10/10 (100%) | 10/10 (100%) | 0 | NA | 0 |
Sensation to pinprick | 7/16 (43.8%) | 9/16 (56.25%) | 5/9 (55.6%) | 4/9 (44.4%) | 3/7 (42.9%) |
Absence of tremor | 19/19 (100%) | 16/19 (84.2%) | NA | NA | 3/19 (15.8%) |
Patient | CXA pre-opa | CXA post-op | Grabb-Oakes pre-opb | Grabb-Oakes post-op | HHM pre-opc | HHM post-opc |
---|---|---|---|---|---|---|
1 | 131 | 150 | 8 | 6 | ||
2 | 135 | 151 | 7.5 | 8 | ||
3 | 130 | 146 | 0.1 | |||
4 | 131 | 141 | 8.5 | 7.4 | 1 | |
5 | 115 | 143 | 12 | 5.2 | 9.2 | 0.1 |
6 | 124 | 142 | 12 | 7.4 | 1 | |
7 | 120 | 152 | 8.8 | 5 | 9.1 | 1 |
8 | 128 | 146 | 10 | 7.7 | ||
9 | 130 | 149 | 10 | 8 | 4.3 | |
10 | 124 | 149 | 9.9 | 5.4 | 1 | |
11 | 132 | 143 | 8 | 7.4 | 3.2 | 1.4 |
12 | 128 | 156 | 7.6 | 4.6 | 2 | |
13 | 130 | 162 | 0 | |||
14 | 130 | 146 | 9 | 6.6 | 2.9 | 2 |
15 | 115 | 150 | 8.8 | 7 | 0 | |
16 | 130 | 146 | 7.9 | 6.7 | 1 | |
17 | 131 | 152 | 7.9 | 6 | 0.4 | |
18 | 128 | 140 | 9.6 | 7 | ||
19 | 126 | 146 | 9.5 | 7.6 | 1 | |
20 | 131 | 143 | 9.5 | 7.1 | 1 |
Symptom/problem | % Pre-surgery | % Post-surgery | % With improvement in frequency post-surgery* | % With worsening of frequency post-surgerya | p valueb | % With onset post-surgeryc |
---|---|---|---|---|---|---|
Headaches | 100% | 100% | 65% (13/20) | 0 | < 0.0001 | 0 |
Dizziness | 100% | 85%(17/20) | 75%(15/20) | 15%(3/20) | < 0.02 | 0 |
Balance problems | 85% (17/20) | 75%(15/20) | 58.8%(10/17) | 11.8%(2/17) | < 0.008 | 0 |
Memory problems | 80% (16/20) | 80%(16/20) | 25%(4/16) | 12.5%(2/16) | NS (< .1) | 0 |
Walking problems | 80% (16/20) | 50% (10/20) | 68.8% (11/16) | 6.3%(1/16) | < 0.003 | 25%(1/4) |
Vertigo | 65% (13/20) | 55%(11/20) | 84.6%(11/13) | 0 | NS(.074) | 28.6%(2/7) |
Speech problems | 60% (12/20) | 35%(7/20) | 66.7% (8/12) | 0 | < 0.006 | 0 |
Frequent daytime urination (>every 2 h) | 60% (12/20) | 35%(7/20) | 66.7% (8/12) | 0 | < 0.04 | 25%(2/8) |
Functional outcome
Patient # | Age at surgery | Gender | Karnofsky pre-op | Karnofsky 2 years post-op | Karnofsky 5 years post-op | Current work/school status | Present illnesses/contributing factors |
---|---|---|---|---|---|---|---|
1 | 18 | F | 50 | 80 | 80 | In school, doing research | 2015 hardware removal and fusion augmentation |
2 | 11 | F | 30 | 50 | 70 | Online school, part time | EDS issues-chronic pain, difficulty walking (WC for long dist), t-spine pinching, l-spine popping and sliding, both spasming, further surgical procedures between 2012 and 2014 including untethering of spinal cord, ACDF C3-C5 and C5-C6, LP and hardware revision C2-C3 |
3 | 17 | F | 80 | 90 | 90 | In school/ waitressing | MVA 4–5 months ago with C7 fx, shoulder pain |
4 | 17 | F | 40 | 80 | 60 | Not in school | EDS issues, intracranial HTN, GI issues, recently failed lumbar shunt, (score goes to 80 when shunt is working), Had previous Chiai Decompression and duroplasty in 8/10 and 5/11; 4 shunt revisions 2013–2015, Also had ACDF C4-C5 and fusion T6-T11 in 2015; fusion revision T8-L4 12/6/16, fusion C2-T1 12/27/16, had immune reaction to bone dust with vascular swelling, volunteers at mom’s school when able. |
5 | 20 | F | 50 | 80 | 70 | Not in school or working | Had decompression in 5/09; EDS issues-dystonias/ dislocations, fatigue, pain all over/ back and leg pain, more dislocations and subluxations, slipped disk in back ~ 5×/week, fiancé helps with shopping, driving > 30 min, can do most ADL’s needs help with heavier tasks |
6 | 20 | F | 50 | 40 | 40 | Fully disabled/bed bound | EDS issues- severe dislocations, inc. ICP, cervical medullary syndrome, POTS, dysautonomias, J-tube, gastroparesis, clotting disorder, 5 clots incl. R internal jugular, migraines, intractable aura, GERD, constipation, dec. cog function, Patient had tethered cord procedure 11/2011, has moderate cognitive impairment, in house nursing/palliative care |
7 | 43 | F | 60 | 70 | 80 | Part time job | Fatigue, pain, arms/leg joints- is able to care for son |
8 | 34 | F | 50 | 50 | 70 | disabled | Had 2004 suboccipital decompression and previous TC procedure, EDS issues- IIH, Adrenal insuf, OA, MCAD, scoliosis, interstitial cystitis, 2013 tethered cord procedure, had hardware removal with augmentation of fusion 2015 with improved POTS, headaches are better but continue to keep her from working, she believes they may be related to IIH |
9 | 17 | F | 60 | 80 | 90 | Full time job | Still has blackout/dysautonomia issues and severe pain 1-2x yearly, had 2014 hardware revision, routine PT helps her function at higher level |
10 | 28 | F | 50 | 60 | 70 | disabled | Pituitary adenoma, failed hip surgery, possible eagle syndrome, tremors, adrenal insufficiency, acromegaly, daily H/A, dislocations, 4/14 hardware removal/fusion augmentation; fusion C2-T1 arthrex ligament augment 1/10/17, can do self care |
11 | 18 | F | 80 | 60 | 90 | Full time Student | Symptoms improved after stopping diazepam, had 2014 hardware removal with augmentation fusion, credits surgery and PT/life balance |
12 | 17 | F | 30 | 90 | 80 | Working part time | Headaches, joint pain elbows, knees, urinary problems, had MVA in July now 22 weeks pregnant (2/22), can do house work and self care |
13 | 12 | F | 80 | 90 | 90 | Student | Getting straight A’s, taking dance classes. Has 3–4 classes a semester, has found that school plus work is too much in that it increases fatigue/headaches and other symptoms |
14 | 36 | F | 60 | 70 | 70 | Disabled | Pressure, LP shunt placement 2013, revision 10/15, tethered cord procedure in 2012, hardware revision in 2013, ACDF C3-C4 2014, fusion C4-T1 2015. PANDAS, POTS, ICH, June surgery/does self care but needs help with heavier chores, shopping, driving more than locally-no highway driving, has headache, instability, shoulder blade pain, 10/16 fusion C2-T1 |
15 | 19 | M | 70 | 90 | 80 | Student, part time | Pain and neuropsych symptoms, 2010 TC and LP shunt Takin 1 class at community college, lives at home |
16 | 18 | F | 80 | 80 | 90 | Working from home/part time work | Had Chiari decompression procedures 3x in 2010,2012 hardware revision and 2015 fusion augment/ chronic H/A thinks she will need pain management for full time work, 3/7/17 TC |
17 | 26 | F | 50 | 50 | 70 | Disabled | Pain and fatigue from EDS, unrelated to surgeries, can function ~ 90–120 min QD, dysautonomias, hypothyroidism, Raynauds, on ssdi, uses adaptive equipment |
18 | 31 | F | 60 | 50 | 50 | Disabled | Had ACDF C5-C6 6/2016, before that had severe H/A, trouble walking, joint pain, hip problems, neck pain further down upper back/arms/shoulders. Needs assistance with prepping food and bathing |
19 | 53 | F | 60 | 90 | 80 | Retired, thinks she could work part time otherwise | Superior mesenteric artery syndrome “nutcracker syndrome”, L renal vein compression, pneumonia, vascular digestive issues, had SMA transposition (1–2 yr. recovery), was posted for hardware revision with augment fusion occ-c1/c2 in April 17, surgery has not happened as of May 17. Does self-care |
20 | 17 | F | 60 | 80 | 60 | New born, unable to work | Had a decompression in 10/07, TC in 8/09; EDS issues- Lumbar shunt,, inc. ICP, c-spine pain, PT helps, 27 wks pregnant as of 9/16- needs help with different ADLs based on pain/energy, has lumbar shunt pressure issues that have to wait to be addressed postnatally, walks ~ 20 min, WC after that, PT helps, 2014 LP and hardware removal, augmentation of fusion, has 10wk old as of 2/22, needs help with basic housework |
Radiological outcomes
Complications of surgery
Discussion
Ehlers-Danlos syndrome
Ligamentous laxity at the craniocervical junction
Neurological deficit has been attributed to ligamentous laxity at the craniocervical junction
The cervical medullary syndrome
Radiological metrics in the diagnosis of basilar invagination and CCI
Non-operative management of patients with craniocervical instability due to hereditary connective tissue disorder
Indications for surgery
Surgical open reduction
Reduction, fusion/stabilization appears to improve pain and neurological deficit
Co-morbid conditions in this population that confounded the outcome
Complications of surgery
Concerns about adjacent segment degeneration
Before CVF | After CVF | P value | |
---|---|---|---|
All surgeries | 30% (6/30) range 0–3, avg. 0.5 | 60%(12/20) range 0–9, avg. 1.7 | <0.04 |
Chiari decompression | 25% (5/20)a | 0 | <0.05 |
TCR | 15% (3/20) | 25%(5/20) | NS |
LP shunt | 5% (1/20) | 15% (3/20) | NS |
Hardware removal with fusion augment | 0 | 40%(8/20) | <0.002 |
Fusion at other level | 0 | 25% (5/20) | <0.04 |
ACDF | 0 | 20%(4/20)a | NS |
C2-T1 fusion | 0 | 15%(3/20) | NS |
Thoracic Fusion | 0 | 10%(2/20) | NS |
Hardware revision, other level | 0 | 10% (2/20) | NS |
Shunt revision | 0 | 10% (2/20)a | NS |
Lumbar puncture | 0 | 10% (2/20) | NS |