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Rabi Khazim
Youssef Fares
G Muthukumar
Douglas Hedden
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Centre
for Spinal Studies
& Surgery
Queen’s
Medical Centre Nottingham,
NG7 2UH
United
Kingdom
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Correspondence:
Dr. Rabi Khazim
Consultant Orthopaedic and Spinal Surgeon
Southend Hospital
Prittlewell Chase
Westcliff-On-Sea
Essex SSO ORY
England
Fax: (44) 1702 221088
E-mail: rabi@khazim.freeserve.co.uk
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Non-Operative
Management of Atlanto-Axial Rotatory Fixation of More That Four Weeks
Atlanto-axial rotatory fixation (AARF) is an uncommon condition
that occurs primarily in children. The literature suggests that
the prognosis is guarded in cases where treatment has been delayed
for more than four weeks following onset of symptoms. Six children
with AARF were diagnosed 6-9½ weeks following onset of symptoms.
Reduction was achieved in all patients with inpatient pain control
and Halter or Halo traction. All five patients with Type 1 Fielding
and Hawkins AARF had no deformity, no pain and full range of
motion. The patient with Type 3 AARF had persistent ache and
recurrent deformity with 70-80% rotation of 24 months follow-up.
Non-operative treatment for Type 1 Fielding and Hawkins AARF
of more than 4 weeks and less than 3 months duration has a good
chance of achieving and maintaining reduction, good functional
results and no deformity.
Keywords: Atlanto-axial, atlas, axis, sublimation,
fixation and traction.
Atlanto-axial rotatory
fixation (AARF) is an uncommon condition that occurs primarily
in children. The aetiology is poorly understood, although it
has been associated with recent infection or trauma. The management
recommended in the literature has been mostly based on the duration
of symptoms prior to active treatment. Some authors recommend
that cervical traction can be attempted for up to three weeks.
However, the prognosis is guarded and only a few patients will
respond to traction.(5,7,11) We report on the management of
six AARF cases presenting with deformities lasting 6 or more
weeks. |
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Eight cases with acquired torticollis of four or more week’s duration
seen in the Hospital for Sick Children in Toronto from August 1991 to
September 1994 were identified. Two were later diagnosed as secondary
to tumour and were excluded. Six patients were diagnosed as AARF and followed-up
for more than 6 months. There were four females and two males. Average
duration of symptoms before making definitive diagnosis and starting treatment
was 7¼ weeks (range 6-9½ weeks). Average age when active treatment was
started was 7½ years (range from 6½ years to 10½ years).
In four patients, the onset of symptoms was spontaneous. One patient had
associated clavicle fracture and another patient woke up with deformity
after surgery for neck lymph node biopsy, which was negative. None had
a history of infection or other medical diseases. All patients had spasm
and neck pain, mostly on trying to rotate the neck to the side opposite
the torticollis. All patients had a normal neurologic examination. Using
Fielding & Hawkins classification, five patients had Type 1 and one patient
had Type 3.5 All but one had dynamic CT scan to confirm diagnosis (Fig.
1). CT axial, sagittal and coronal reconstruction scans were also helpful
in delineating the deformity (Fig. 2).
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Figure
1 — 7 year and 5-month-old female
sustained a clavicle fracture and presented with 9½ weeks history
of typical Atlanto-Axial Rotatory Fixation, Fielding-Hawkins Type
1 deformity. (a) Plain x-ray of C1-2, open mouth view. (b) Dynamic
CT scan of occiput-axis, with neck rotated to left. (c) With neck
rotated to right. Note that there is no rotation of C1 on C2 on the
left rotation scan; with about 400 rotation of C1 on C2 on the right
rotation scan.
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Figure
2 — CT scan images of C1-2 rotatory
fixation, Fielding & Hawkins Type 3. (a) Axial view; (b) Sagittal
view and (c) Coronal view.
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Figure
3 — At 16 months follow-up, the patient
was symptomatic and had full range of motion in all the planes. (a)
Flexion; (b) Extension; (c) Left rotation and (d) Right rotation.
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All patients were hospitalised and given oral narcotic
analgesics, muscle relaxants (Diazepam) and anti-inflammatory medications
(Naproxen 10-15 mg/kg) as appropriate. To achieve reduction, Halter traction
was used in patients with Fielding Type 1 (5-7 lbs). Halo traction was used
in the patient with Type 3 using increasing weights from 5-9 lbs. For post-reduction
immobilisation, a custom-moulded thermoplast brace was used in two patients,
a SOMI brace in one, while three had no immobilisation. The follow-up was
done until the patient had full range of motion and no pain or deformity
on at least two occasions. The average follow-up was 25 months (range 15
to 30 months).
Reduction was achieved in all patients. The time to reduction was varied;
2 days, 3 days (two patients), 6 days, 3 weeks and 4 weeks. Three of the
six patients had a recurrence of the deformity when the traction was discontinued
and the brace fitted. The patient with Type 3 Fielding & Hawkins had recurrence
after discharge from hospital, despite being immobilised in a brace.
One patient’s deformity recurred when the patient was fitted into a SOMI
brace. The patient was put back in traction for 7 days and then stabilised
in a custom-made brace. In this patient, whose deformity lasted for over
9½ weeks prior to treatment, bracing time was increased gradually over a
two-week period after reduction, with the remaining daytime spent in Halter
traction (Fig. 3). Custom-moulded thermoplast brace was found to provide
the best immobilisation. Bracing was continued for between 4½ to 12 weeks
in three patients. Three patients had no immobilisation. The average hospital
stay was 23 days (range 7-47 days).
At the time of follow-up, five patients had no deformity, no pain and full
range of motion. Only one patient had persistent ache and recurrent deformity
with a limited range of motion, but she had no limitations in her activities.
At 12 months follow-up, she had 80% rotation to left, 20% rotation to right,
full flexion and 50% extension. At 24 months, she had 80% rotation to left
and 70% rotation to right.
In AARF cases, the duration of deformity before treatment is started usually
determines whether non-surgical treatment would be successful. Early diagnosis
of AARF is very important as the prognosis in patients presenting more than
4 weeks has been guarded by most authors.(4,5,8,10,12,14) Despite that,
we still had 6 such cases in a 3 year period.
Fielding & Hawkins reported 15 long-standing cases.(5) In their series of
seventeen patients, the average diagnostic delay was 7½ months, thirteen
of which failed non-operative treatment and underwent fusion. They recommended
fusion for long standing cases (more than 3 months), neural involvement,
anterior displacement more than 4 mm or when adequate conservative treatment
has failed to achieve and maintain correction. Phillips & Hensinger reported
that three of their seven patients with more than 4 weeks duration were
eventually treated with atlanto-axial fusion.(12) Fielding & Hensenberg,
et al. in their two papers recommended that in patients with fixation of
more than 4 weeks duration, cervical traction can be attempted for up to
3 weeks.(5,7) However, the prognosis is guarded and only a few patients
will respond to traction.
There have been few reports of reduction achieved non-operatively. Burkus,
et al, reported successful manipulative reduction in an awake patient 6
weeks following symptoms.(1) Scapinelli reported successful manipulative
reduction under anaesthesia at 3½ months.(13) Both of these patients had
Type 1 Fielding & Hawkins fixation. In Phillips & Hensinger series, four
out of seven patients were treated successfully non-operatively.(12) Six
of their seven patients reduced but two had irreducible recurrence and were
fused. Reduction was not achieved in only one of their patients. We were
able to achieve reduction in all of our six patients. We failed however,
to maintain reduction in the Type 3 Fielding & Hawkins patient. It is not
clear whether post-reduction bracing adds to the success of non-operative
treatment.
Atlanto-axial fusion has been recommended to ensure stability that may be
compromised, especially when anterior displacement is present.(5,12) Coutts
in an experimental work in 1934 found that with an intact transverse ligament,
the atlanto-axial articulation pivots on the eccentrically placed odontoid
and complete bilateral dislocation of the articular process can occur at
approximately 650 of rotation with resultant narrowing of the diameter of
an average spinal canal to 7 mm.(3) With a deficiency of the transverse
ligament allowing 5 mm of anterior displacement of the atlas on the axis,
complete unilateral dislocation can occur at 450 of rotation and narrowing
of the canal diameter to 12 mm.
However, it is not clear whether Type 1 Fielding & Hawkins AARF are unstable.
In these cases, the major stabilising structures of the atlanto-axial complex
(the dens, transverse ligament, lateral masses and ring of the axis) are
all intact. This allows safe manipulative reduction for awake or anaesthetised
patient.(1,9,13) Goddard, et al reported on three Type 1 cases of more than
3 months duration which had irreducible dislocation at the time of surgery,
stating that in-situ fusion would appear merely to perpetuate an already
stable situation.(6) They suggested a two type classification differentiating
between stable subluxation of fixation in the extreme range of motion of
the joint and the unstable dislocation of the lateral mass of C1 on C2.
Only the Type 3 Fielding & Hawkins patients in our series had evidence of
instability and none of our patients had any neurological abnormalities.
Correction of deformity and facial flattening is given as another indication
for surgical treatment. Some authors reported cases of compensatory counter
atlanto-occipital subluxation leading to pseudo reduction with the occiput
and axis pointing in the same direction leaving the axis dislocated at both
levels.(2,11 ) Moore, et al noted that this may create an obvious cosmetic
deformity and may theoretically lead to increased pain and degeneration
in the long term.(9) They reported manipulation under anaesthesia followed
by immobilisation and fusion after open reduction in those cases that failed
closed treatment. Three of their four cases of more than four weeks duration
were fused.
However, the standard recommended surgery has been fusion in-situ.(5,7,12)
This has been reported to result in functional range of motion with a maximal
loss of rotation of 250 in either direction. The mechanism of deformity
correction with in-situ fusion is not clear, although compensatory counter
atlanto-occipital subluxation can be a possible explanation. All Type 1
patients in our study had no deformity; only the Type 3 case had recurrent
deformity that was closely associated with instability.
The hospital stay for our patients averaged 26 days. That may be considered
too long in comparison to a roughly comparable group of six patients in
another series where the average hospital stay was twelve days, although,
two of their six patients needed subsequent arthrodesis and the succeeding
hospital stay was not mentioned.(12) However, non-surgical treatment avoids
the risks and complications of surgery and has good results in the majority
of these cases. We strongly recommend non-operative treatment of Type 1
atlanto-axial rotatory fixation of more than 4 weeks duration as the first
line of treatment. Only if it fails should a fusion be considered.
Although the study size is admittedly small, we believe that patience and
perseverance with non-operative treatment of Type 1 Fielding & Hawkins of
more than 4 weeks and less than 3 months duration has a good chance of achieving
and maintaining reduction, good functional results and no deformity. The
prognosis for other types might not be as good and we cannot recommend non-operative
treatment for Fielding Type 3 cases. The upper limit for delay in diagnosis
leading to a successful non-operative result is not clear.
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| 1. |
Burkus JK, Deponte RJ: Chronic atlanto-axial
rotatory fixation correction by cervical traction. Manipulation
and bracing. J Ped Orthop 1986, 6: 631-633 |
| 2. |
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T, El-Khoury GY: Atlanto-axial rotatory fixation with compensatory
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| 3. |
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| 4. |
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| 5. |
Fielding JW, Hawkins RJ: Atlanto-axial
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| 6. |
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| 7. |
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| 8. |
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| 9. |
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| 10. |
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| 11. |
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| 12. |
Phillips WA, Hensinger RN: The management
of rotatory atlanto-axial subluxation in children. J Bone Surg (Am)
1989, 71-A, 664-667 |
| 13. |
Scapinelli R: Three-dimensional computed
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(Br) 1994, 76-B: 367-370 |
| 14. |
Subach BR, McLaughlin MR, Albright
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