- Email: [email protected]
Cervical involvement in Morquio syndrome
Accepted Manuscript Cervical involvement in Morquio syndrome Ayla Buyukkaya, Mehmet Ali Özel, Ömer Onbas PII:
S1529-9430(15)00622-1
DOI:
10.1016/j.spinee.2015.06.035
Reference:
SPINEE 56382
To appear in:
The Spine Journal
Received Date: 20 May 2015 Revised Date:
4 June 2015
Accepted Date: 12 June 2015
Please cite this article as: Buyukkaya A, Özel MA, Onbas Ö, Cervical involvement in Morquio syndrome, The Spine Journal (2015), doi: 10.1016/j.spinee.2015.06.035. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
ACCEPTED MANUSCRIPT Cervical involvement in Morquio syndrome
RI PT
Ayla Buyukkaya1, Mehmet Ali Özel 2 , Ömer Onbas 2
Duzce Ataturk Goverment Hospital, Department of Radiology
2
Duzce University, School of Medicine, Department of Radiology
SC
1
Contributed by Buyukkaya A.
M AN U
Running title; Morquio syndrome and cervical involvement
[email protected] Tel: 90 380 514 3638
AC C
EP
TE D
Department of Radiology, Duzce Ataturk Government Hospital, Duzce, Turkey
1
ACCEPTED MANUSCRIPT
Cervical involvement in Morquio syndrome
2
Morquio syndrome is a rare autosomal recessive skeletal dysplasia characterized by
3
altered mucopolysaccharide metabolism. Typical cervical vertebra involvement drew our
4
attention in an 11-year-old girl with Morquio syndrome. In patients with Morquio syndrome,
5
narrowing of the soft tissue gap can be observed in the vertebrae posteriorly, due to
6
glycosaminoglycan accumulation (1). In our case, canal narrowing and cervical cord
7
compression were observed due to thickening of the posterior vertebra at the craniovertebral
8
junction. Mild hydrocephalus was seen on cranial MRI and was thought to be associated with
9
the canal narrowing at the C1 level. In addition to the other findings, a fusion defect was seen
SC
in the C1 vertebra anteriorly and posteriorly (Figure 1).
M AN U
10
RI PT
1
11
Referance
13
1: McKay SD, Al-Omari A, Tomlinson LA, Dormans JP. Review of cervical spine anomalies
14
in genetic syndromes. Spine (Phila Pa 1976). 2012 Mar 1;37(5):E269-77.
15 16
TE D
12
Figure 1: Sagittal T2-weighted image (A) shows dural thickening posteriorly at the C1 level
18
due to metabolic accumulation (*); at this level, channel narrowing and cord compression are
19
seen. A fissure line on the posterior side of the C1 vertebra (arrow) are seen on, coronal (C)
20
and axial (D) computed tomography scan.
AC C
21
EP
17
AC C
EP
TE D
M AN U
SC
RI PT
ACCEPTED MANUSCRIPT
S1529-9430(15)00622-1
DOI:
10.1016/j.spinee.2015.06.035
Reference:
SPINEE 56382
To appear in:
The Spine Journal
Received Date: 20 May 2015 Revised Date:
4 June 2015
Accepted Date: 12 June 2015
Please cite this article as: Buyukkaya A, Özel MA, Onbas Ö, Cervical involvement in Morquio syndrome, The Spine Journal (2015), doi: 10.1016/j.spinee.2015.06.035. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
ACCEPTED MANUSCRIPT Cervical involvement in Morquio syndrome
RI PT
Ayla Buyukkaya1, Mehmet Ali Özel 2 , Ömer Onbas 2
Duzce Ataturk Goverment Hospital, Department of Radiology
2
Duzce University, School of Medicine, Department of Radiology
SC
1
Contributed by Buyukkaya A.
M AN U
Running title; Morquio syndrome and cervical involvement
[email protected] Tel: 90 380 514 3638
AC C
EP
TE D
Department of Radiology, Duzce Ataturk Government Hospital, Duzce, Turkey
1
ACCEPTED MANUSCRIPT
Cervical involvement in Morquio syndrome
2
Morquio syndrome is a rare autosomal recessive skeletal dysplasia characterized by
3
altered mucopolysaccharide metabolism. Typical cervical vertebra involvement drew our
4
attention in an 11-year-old girl with Morquio syndrome. In patients with Morquio syndrome,
5
narrowing of the soft tissue gap can be observed in the vertebrae posteriorly, due to
6
glycosaminoglycan accumulation (1). In our case, canal narrowing and cervical cord
7
compression were observed due to thickening of the posterior vertebra at the craniovertebral
8
junction. Mild hydrocephalus was seen on cranial MRI and was thought to be associated with
9
the canal narrowing at the C1 level. In addition to the other findings, a fusion defect was seen
SC
in the C1 vertebra anteriorly and posteriorly (Figure 1).
M AN U
10
RI PT
1
11
Referance
13
1: McKay SD, Al-Omari A, Tomlinson LA, Dormans JP. Review of cervical spine anomalies
14
in genetic syndromes. Spine (Phila Pa 1976). 2012 Mar 1;37(5):E269-77.
15 16
TE D
12
Figure 1: Sagittal T2-weighted image (A) shows dural thickening posteriorly at the C1 level
18
due to metabolic accumulation (*); at this level, channel narrowing and cord compression are
19
seen. A fissure line on the posterior side of the C1 vertebra (arrow) are seen on, coronal (C)
20
and axial (D) computed tomography scan.
AC C
21
EP
17
AC C
EP
TE D
M AN U
SC
RI PT
ACCEPTED MANUSCRIPT