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Atlanto
axial dislocations are rare yet devastating to the patients unfortunate enough
to have them. AADs result in symptoms ranging from respiratory depression and
CO2 narcosis to quadripariesis and incontinence. Thus management
strategies are very important to ensure 2 essential requirements: that the
compression at the Cervico-Medullary Junction is released, and stability of the
Cranio-Vertebral Junction is ensured.
We
present a patient with improper correction of an AAD who presented with
inadequate decompression with adequate stabilization, posteriorly with a
Gallies fusion. The patient had no symptomatic relief despite aggressive
physiotherapy. Imaging revealed good bony fusion but severe compression on the
cord by a large jutting odontoid. The odontoid was removed trans orally and the
patient was subjected to rehabilitation therapy. She improved well and is
ambulant without support now.
The
report highlights the need to address both aspects of cranio-vertebral junction
pathology when confronted with a case as mentioned. Simply stabilizing the
C1-C2 joint or C0-C2 joint without adequate neurological decompression is NOT
enough. Tailoring the treatment to meet the needs of the patient is essential
for proper patient recovery and satisfaction.
INTRODUCTION
Atlanto-Axial Dislocation is rare, yet
devastating to its sufferers. Congenital AAD is even more infrequent, arising
out of insufficient maturation and development of the branchial arches and
occipital and cervical somites [1-4]. Other causes of AAD such as inflammatory
diseases, trauma and bone disorders are more common and increasing in
incidence. The primary problem with an AAD is the compression on the
Cervico-Medullary Junction, critical to vegetative functions such as
Respiration, Cardiac activity and consciousness [3-5]. Time and again,
mortality and severe morbidity has been demonstrated due to dysfunction of the
CMJ alone [3-5] Yet reams of literature have been published focusing on the
dangers of instability at the CVJ. This has led to a disproportionate emphasis
on fusion of the CVJ rather than decompression of the CMJ [5]. The result
although an improvement from before, leads to little improvement and long
standing damage to the medulla and cervical cord leading to sometimes
irreversible damage to the neural elements of the CMJ. It is therefore critical
to examine both aspects carefully before embarking on a surgical strategy for
the patient. Such a case is discussed below.
CASE
REPORT
A 62 years old lady was admitted with spastic
Quadripariesis (2/5) and urinary urge incontinence. Her neurological status had
worsened following a trivial fall 10 days ago. She had presented with the same
problem 10 years ago when she was seen in 1994. She was diagnosed with
congenital Atlanto-Axial Dislocation (AAD) and subjected to an
Occipito-Cervical Fusion with a posterior stainless steel wiring and bone graft
construct. Post-surgery her spasticity was the same although her neck pain was
much better. She was subjected to rigorous physiotherapy but too significant
improvement. She was, before her fall, ambulant with support and able to
perform her activities of daily living with
On arrival in the OPD, she was bedridden with
spastic quadripariesis Power grade 2/5 with spasticity of 4(modified Ashworth’s
score). Her lower cranial nerves were normal allowing her to swallow and speak
normally. She had no radicular pain, but complained of paraesthesia over all 4
limbs all the time.
On investigating her situation, her X-ray of
the cervical spine and CVJ (Figure 1)
showed good bony fusion with the stainless steel wire in situ posteriorly.
Dynamic X-rays showed no instability, but Basilar Invagination was seen. A CT
scan of the cervical spine and CVJ confirmed the findings of the X-ray. This
showed Basilar Invagination (BI) and an increased Atlanto-Dens Interval (ADI) (Figure 2).
Hence it was clear that the fusion done 10
years ago did only convert a mobile AAD into a fixed AAD. There was no
correction of the BI either which had led to a fusion of the C0-C1, C2 complex
in a compromised position. The next part of our investigation was to probe the
extent of damage sustained by the Cervico-Medullary Junction (CMJ) and Spinal
Cord. This proved difficult, as the stainless steel wire prevented us from
getting an MRI of the CVJ done. Due to the long-standing disease and wrong
surgery done in the past, we concluded that the MRI was a priority, hence the
stainless steel wire was surgically removed (as fusion had occurred, its
function in situ was void) and the
patient was then subjected to an MRI of the CMJ and Cervical spine.
The MRI showed massive cord compression at
the CVJ with thinning of the CMJ and myelomalacia changes. No Chiari, hydrocephalus,
syrinx or other abnormalities were seen (Figure
3). The dire nature of the compression, primarily due to the AAD and the BI
which had not been managed appropriately before, placed us in a dilemma
regarding the high risk, the decompression now entailed. Resultant cord edema,
could lead to catastrophic respiratory paralysis and prolonged ICU and
Ventilator dependency, not to mention quadriplegia, and death. To be doubly
sure of the stability of the CVJ, another dynamic X-ray was taken which showed
good stability (Figure 4).
The options before us were:
1. Foramen
Magnum Decompression (FMD)
2. Trans-Oral
Odontoidectomy
We proceeded with the latter option, as the
primary cause of compression was the malpositioned odontoid process of C2.
After a thorough work up, the patient was
placed supine after nasopharyngeal intubation was done and mouth opened with a
Boyle-Davis Mouth gag. The posterior pharyngeal wall was incised and dissected
under C-ram guidance to reveal the anterior arch of C1 and the Odontoid. Both
were drilled to expose the dura of the CMJ. Haemostasis was achieved, before
closure was done with soluble sutures.
Post op the patient was mobilized immediately
and she showed significant decrease in spasticity. Gradual physiotherapy and
rehab care showed excellent recovery. She now is ambulant without support, and
performs all activities of daily living freely. She is also active in the
kitchen and performs her daily chores well (something that was impossible
pre-op) and lives a happy functional life.
DISCUSSION
Craniovertebral Instability is a difficult
problem to address. No two patients are the same. Tailoring the management to
each individual’s needs is important to ensure good recovery and patient
satisfaction [5-7]. The most important aspects of CVJ disorders are that, there
are 2 main issues that need attention.
Instability [1-11]
The improperly fused elements of the CVJ lead
to instability in weight bearing of the head. Such a problem is significant and
alarming, which can lead to significant morbidity. However, CVJ instability is
not seen in all disorders. Its severity and the need to intervene, needs to be
assessed with respect to the pathology of the disease and the cause of the
symptoms of the patient. Blindly fusing all CVJ abnormalities will result in
patients like the one discussed, where fusion and excellent stability have
resulted in no improvement in functional status.
Neurological
decompression [4-6,8,11]
The crux of all procedures, aimed at
resolving disease at the CVJ, eventually involves reduction of compression
(either bony, ligamentous or produced by external agents such as pannus or pus)
on the CMJ. Without adequate neurological decompression, there will be no
symptomatic relief for the patient and hence no recovery. It is therefore
imperative to understand the nature of the disease and the cause of the
symptoms of the patient.
Deciding upon the right management strategy
requires many stages of deduction to be performed. These include [7-12]:
1. Neurological history:
These help deduce the causative pathology and thereby point in the direction of
the plausible problem. For example, traumatic CVJ dislocations will usually
have a predominant component of instability rather than compression while
rheumatoid arthritis and inflammatory conditions, will lead to pannus formation
and predominantly compressive pathology. A good grasp of the causative etiology
and the pathological progression helps decide on surgery, and adjuvant therapy
[10-12].
2. Examination: The presence of
a Myelopathy definitely implies serious neurological compression, making its
release a priority [12-14].
3. Presence of abnormal anatomy: The
existence of a BI, AAD or other related abnormalities implies reduction to
normal anatomical position along with adequate decompression [6,8,11,12].
4. Instability and neurological compression
seen on neuro-radiology: The need for a dynamic study of the CVJ
is obvious as it detects instability best of all. In certain complicated
anatomical situations, CT scan in flexion and extension is helpful to identify
the chief offending pathology. An MRI however is essential to detect the status
of the CMJ and the cervical cord. No manipulation of the CVJ and CMJ must be
done without an MRI. Failure to do this can result in catastrophic destruction
of a delicate and vital structure [3,5,8,9,11,13,14].
5. Presence of previous surgical implants
and their implications to management: As discussed in
the case report, the presence of the inter laminar fusion wire along with
posterior fusion changed the situation completely. Although trans-oral
odontoidectomy is frowned upon nowadays, due to a sturdy posterior fusion being
present, there was no need to re-stabilize the joint. Hence only decompression
was required. The stainless-steel wire caused us much grief, as an MRI of the
CMJ was impossible with it in situ. Due to the essential nature of the MRI, it
was decided to surgically remove it and then take an MRI before planning
definitive management. Such a strategy, although cumbersome and long-winded,
will eventually point the correct direction to take, and hence is worth the
trouble to both patient and surgeon.
The algorithm for management is well known
and is hence not addressed here. Posterior fusion with adequate decompression
is the treatment of choice here. The choice of C0-C1-C2 fusion or just C1-C2
fusion depends upon the extent and nature of the instability. Decompresive
procedures include laminectomies and Foramen magnum decompressions. These again
are to be decided upon studying the MRI and matching the compression to the
symptoms experienced by the patents as cause and effect.
Lastly, postoperative physiotherapy is
perhaps more important than surgery itself [14]. Rehabilitation experts are the
wizards who make noteworthy improvements in the lives of the patients. Surgery
alone is useless if not combined with proper rehab support. As with our
patient, it was long-term, persistent and assiduous rehab care that led to the
excellent results experienced by our patients [14]. This underlines the need of
a team approach to these complex conditions, where Neurosurgeons, nurses,
physiotherapists, anesthetists, radiologists and technicians combine to give
the patient the best result possible.
SUMMARY AND
CONCLUSION
Pathologies of the CVJ are complex and do NOT
have a 1 size fits all solution. A thorough understanding of the situation
coupled with adequate emphasis to BOTH neurological decompression AS WELL AS
instability gives the best result overall, and help avoid difficult and
inconvenient redo surgeries.
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