CASE REPORT – OPEN ACCESS International Journal of Surgery Case Reports 27 (2016) 125–128
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International Journal of Surgery Case Reports journal homepage: www.casereports.com
Extensive ossification of the paraspinal ligaments in a patient with vitamin D-resistant rickets: Case report with literature review Yujiro Hirao ∗, Hirotaka Chikuda, Yasushi Oshima, Yoshitaka Matsubayashi, Sakae Tanaka Department of Orthopaedic Surgery, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo 113-8655, Japan
a r t i c l e
i n f o
Article history: Received 4 June 2016 Received in revised form 22 August 2016 Accepted 24 August 2016 Available online 28 August 2016 Keywords: Spinal ossification Vitamin D-resistant rickets Myelopathy Spinal ankylosis
a b s t r a c t INTRODUCTION: Ectopic ossification of the spinal ligaments is not uncommon in patients with Vitamin D-resistant rickets (VDRR), but the long-term consequences of this condition have not been reported. PRESENTATION OF CASE: The case was a 65-year-old female with VDRR who reported progressive weakness of the upper extremities, difficulty walking, neck pain, and numbness in the left arm. Imaging studies demonstrated cord compression with ectopic ossification at the rim of the occipital bone and OPLL at C1 level. Ankylosis of the whole spine below the C2 vertebra was also noted with preserved mobility only at the craniovertebral junction. DISCUSSION: Our report showed that ectopic ossification of the spinal ligament can result in ankylosis of the entire spine in patients with VDRR. In such patients, the segments with remaining mobility are considered to be at high risk of developing myelopathy due to increased stress at the junction. CONCLUSION: The present case underscores the importance of providing long-term follow-up in VDRR patients presenting with ectopic ossification of the spinal ligaments. In particular, physicians should pay close attention to the possibility of myelopathy in any segments with preserved mobility. © 2016 The Authors. Published by Elsevier Ltd on behalf of IJS Publishing Group Ltd. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
1. Introduction Vitamin D-resistant rickets (VDRR) is a group of metabolic disorders characterized by renal tubular defects in phosphate transport and bone abnormalities resulting in hypophosphatemic rickets or osteomalacia1 . Patients with VDRR may present with deformities of the lower extremities, bone or joint pain, short stature or dental abnormalities [1] and tend to develop calcium deposits at the attachments of tendons or ligaments as they age [1]. It is recognized that ectopic ossifications of the spinal ligaments are not uncommon in VDRR patients, which may result in compression of the spinal cord and subsequent myelopathy [2–5]. Despite this, the long-term consequences of ossified spinal ligaments in VDRR patients have not been well documented. We report a case of VDRR presenting with extensive ossification of the spinal ligaments for which we performed long-term follow-up. 2. Presentation of case The patient was first referred to our hospital because of difficulty in walking at the age of 34, when she was diagnosed with VDDR.
∗ Corresponding author at: Department of Spine and Orthopaedic Surgery, Japanese Red Cross Medical Center, 4-1-22 Hiroo, Shibuya-ku, Tokyo 150–8935, Japan. E-mail address: [email protected] (Y. Hirao).
Later, the diagnosis was genetically confirmed as described by a different research group [6]. The patient had previously undergone T7–T9 laminectomy due to thoracic myelopathy at another hospital at the age of 24, after which her myelopathic symptoms subsided for 7 years. At the age of 34, she underwent a second posterior decompression surgery (T4–T9) for gait disturbance due to thoracic myelopathy after a diagnosis of OPLL and OYL, which resulted in improvement of her symptoms. Since then, the patient has been followed-up on annual basis and remained functionally stable for over 30 years. At the age of 65, she reported weakness of the upper extremities, difficulty walking, neck pain, and numbness in the left arm. She was admitted for further investigation and treatment. On admission, she was 118 cm tall with a marked round back and bowed legs. She was able to walk only short distances supporting herself on a wall. Neurologic examination revealed decreased light touch and pinprick sensation, and motor weakness (3/5 strength) in the distal upper extremities. The grip power was 6 kg in both hands. Tendon reflexes were equivocal with indifferent Babinski sign bilaterally. Plain radiograph showed marked kyphosis of the thoracic spine (T1–T12 angle; 94◦ ) (Fig. 1). Computed tomography demonstrated ankylosis of the whole spine below the C2 vertebra with extensive ossification of the paraspinal ligaments (Fig. 2A, B). In contrast, decreased but preserved mobility (9◦ on flexion and extension) was noted at the craniovertebral junction (CVJ). No overt radiographic instability was found in the atlantoaxial region, with an atlantodental interval of 1 mm. In addition, there was ossification at the
http://dx.doi.org/10.1016/j.ijscr.2016.08.038 2210-2612/© 2016 The Authors. Published by Elsevier Ltd on behalf of IJS Publishing Group Ltd. This is an open access article under the CC BY-NC-ND license (http:// creativecommons.org/licenses/by-nc-nd/4.0/).
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rim of the occipital bone, and OPLL at the C1 level. Magnetic resonance imaging (MRI) revealed spinal cord compression at the levels of both the occipital bone and C1 (Fig. 3). The patient underwent posterior decompression, in which the posterior arch of C1 and the ossified rim of the occipital bone were resected. Deformation of the dural sac was observed at locations corresponding to the resected portions of the rim of the occipital bone and C1 posterior arch, indicating that there had been sustained pressure on the sac. Her postoperative course was uneventful. At the 18-month follow-up visit, the patient was free of pain and numbness. Her grip power had improved to 20 kg in the right and 15 kg in the left hand. She had regained the ability to walk with the support of a cane. 3. Discussion
Fig. 1. Plain lateral radiograph showing marked kyphosis of the thoracic spine.
In this report, we described a case of VDRR associated with extensive ossification of the spinal ligaments followed up for more than 30 years. Ossification of spinal ligaments has been reported in approximately one third of VDRR patients over the age of thirty, and is thought to be linked to both genetic and environmental factors [2,5,6]. In the literature, 23 cases of VDRR patients undergoing surgical treatment for myelopathy have been reported (Table 1) [2,3,7–19]. The majority of the patients are middle-aged (mean age, 44.3 years). In most cases, surgical decompression resulted in favorable outcomes in terms of neurological recovery. However, in reports that consider the implications for spine surgery or changes in the radiological findings, most of the reported followup periods are 2 years or under and the longest period reported is 7 years. In this case, further problems arose 30 years after surgical decompression, a period that has not previously been covered. In the present case, ossification of the spinal ligaments progressed to ankylosis of the entire spine below C2, mimicking ankylosing spondylitis. Given the preserved but minor mobility at the craniovertebral junction, continuing mechanical stress may have been imposed on the stenotic lesion, leading to
Table 1 24 cases of VDRR patients with myelopathy caused by intracanal pathology. Authors, year
Age(yr)/sex
Follow-up duration
Intracanal pathology (level)
Surgery (level)
Dugger and Vandiver [7], 1966 Johnson et al. [8], 1966 Yoshikawa et al. [9], 1968 Highman et al. [10], 1970
60/M 28/F 48/F 28/M 55/M 55/M 50/F 42/M
N/A 2.5 month 6 months 15 months 6 months N/A N/A N/A
bony overgrowth (T11/12) bony overgrowth (T11) OPLL (C3–4) thickened laminae (C2–5) thickened laminae (T6–8) OLF (T5/6, 6/7, 7/8) OLF (C3–5, T7/8) OPLL (C3–5, T7/8), OLF
39/M 49/M 57/M 42/F 32/M 45/M 49/F 48/M 40/F 43/F 54/F 44/F
7 years N/A N/A 6 months 3 months 6 months 3 months 5 years N/A N/A 2 years N/A
OLF (T7–10) OLF (T8/9) OLF (T5–8) degenerative changes (T3–11) OLF (T7–11) CLF (T10/11) OLF (T5/6, 6/7, 7/8, 9/10) OLF (thoracic including T9/10) CLF (C5/6) CLF (cervical) OPLL (C4–6) OPLL (C2/3, C4), calcified disk (C6/7) OPLL (C5–T1, T12/L1), OLF (T8/9, T9/10, T10/11) OPLL (C1/2, C2/3–6/7) OPLL (C2–7) ossification at the rim of the occipital bone, OPLL (C1/2)
laminectomy (T11, 12) laminectomy (T10–L1) laminectomy (C2–5) laminectomy (C2–5) laminectomy (T6–8) laminectomy (T5–8) laminectomy (C3–T1, T7, 8) anterior surgery (C3/4, C4/5), laminectomy (C3–7) laminectomy (T7–11) laminectomy (T8–10) laminectomy (T3–9) laminectomy (T3–11) laminectomy (T7–11) laminectomy (T10, 11) laminectomy (T6–10) laminectomy (T10) laminectomy (C2–6) laminectomy (cervical) laminoplasty (C3–7) anterior surgery (C6/7)
Bradbury et al. [11], 1987
Matsui et al. [3], 1991 Bussiere et al. [12], 1993 Yamamoto and Onofrio [13], 1994 Ballantyne and Findlay [14], 1996 Dunlop and Stirling [15], 1996 Vera et al. [16], 1997 Thomas and Burnet [17], 2000 Velan et al. [5], 2001 Soehle and Casey [4], 2002
laminoplasty (C2–7, T8–11), anterior surgery (T12/L1) Lee et al. [2], 2012 36/F 3 years laminectomy and fusion (C3–7) 32/F 2 years laminoplasty (C2–6) Shiba et al. [19], 2015 resection of both ossified rim 65/F 31 years Present case of the occipital bone and posterior arch of C1 VDRR, Vitamin D-resistant rickets; N/A, data not available; OPLL, ossification of the posterior longitudinal ligament; OLF, ossification of ligamentum flavum; CLF, calcification of ligamentum flav Kawaguchi et al. [18], 2009
44/F
5 years
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Fig. 3. T2-weighted magnetic resonance imagine revealing spinal cord compression at the C1 level by the ossified tissues.
4. Conclusion The present case underscores the importance of continuing the periodic monitoring of VDRR patients presenting with ectopic ossification of the spinal ligaments over a period of decades. Physicians should pay close attention to possible myelopathy occurring at the segment with preserved mobility. Conflict of interest The authors declare that they have no conflict of interest. Funding The present study did not receive funding from any sponsors. Ethical approval For this type of study formal consent is not required. Patient consent Fig. 2. Sagittal reconstruction images of computed tomography demonstrating fusion of the entire spine below C2: Cervical spine (A) and thoracolumbar spine (B).
Informed consent was obtained from all individual participants included in this study. Author contribution
myelopathy. Similar pathomechanisms have been reported in patients with ankylosing spondylitis, presenting as various pathologies at the craniovertebral junction including atlantoaxial subluxation, retroodontoid pannus, basilar invagination, and bone erosion [20]. This study was reported in line with the CARE criteria [21].
Yujiro Hirao: contributed to data collection and writing the manuscript. Hirotaka Chikuda: was the supervisor and contributed to discussion and writing the manuscript. Yasushi Oshima: contributed to discussion and data collection. Yoshitaka Matsubayashi: contributed to discussion and data collection. Sakae Tanaka: contributed to the discussion.
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References [1] R.P. Polisson, S. Martinez, M. Khoury, R.M. Harrel, K.W. Lyles, N. Friedman, J.M. Harrelson, E. Reisner, M.K. Drezner, Calcification of entheses associated with X-linked hypophosphatemic osteomalacia, N. Engl. J. Med 313 (1985) 1–6. [2] S.H. Lee, M.V. Agashe, S.W. Suh, Y.C. Yoon, S.H. Song, J.H. Yang, H. Lee, H.R. Song, Paravertebral ligament ossification in vitamin D-resistant rickets: incidence, clinical significance, and genetic evaluation, Spine Phila Pa 1976 37 (2012) E792–E796. [3] H. Matsui, Y. Katoh, H. Tsuji, Untreated hypophosphatemic vitamin D-resistant rickets with symptomatic ossification of the ligamentum flavum, J. Spinal. Disord. 4 (1991) 110–113. [4] M. Soehle, A.T. Casey, Cervical spinal cord compression attributable to a calcified intervertebral disc in a patient with X-linked hypophosphatemic rickets: case report and review of the literature, Neurosurgery 51 (2002) 239–242. [5] G.J. Velan, B.L. Currier, B.L. Clarke, M. Yaszemski, Ossification of the posterior longitudinal ligament in vitamin D-resistant rickets: case report and review of the literature, Spine Phila Pa 1976 26 (2001) 590–593. [6] T. Saito, Y. Shimizu, M. Hori, M. Taguchi, T. Igarashi, S. Fukumoto, T. Fujita, A patient with hypophosphatemic rickets and ossification of posterior longitudinal ligament caused by a novel homozygous mutation in ENPP1 gene, Bone 49 (2011) 913–916. [7] G.S. Dugger, R.W. Vandiver, Spinal cord compression caused by vitamin D resistant rickets, J. Neurosurg. 25 (1966) 300–303. [8] C.C. Johnston Jr., G.J. Kurlander, D.M. Smith, J.M. Goodman, R.L. Campbell, Familial vitamin D resistant rickets in untreated adult: bony proliferation of neural arches with cord compression, Arch. Intern. Med. 117 (1966) 141–147. [9] S. Yoshikawa, M. Shiba, A. Suzuki, Spinal-cord compression in untreated adult cases of vitamin-D resistant rickets, J. Bone Joint Surg. Am 50 (1968) 743–752. [10] J.H. Highman, P.H. Sanderson, M.M. Sutcliffe, Vitamin-D-resistant osteomalacia as a cause of cord compression, Q. J. Med. 39 (1970) 529–537.
[11] P.G. Bradbury, D.P. Brenton, G.M. Stern, Neurological involvement in X-linked hypophosphataemic rickets, J. Neurol. Neurosurg. Psychiatry 50 (1987) 810–812. [12] J.L. Bussière, J.M. Ristori, L. Miravet, C. Piat, M. Soubrier, T. Bardin, Vitamin-resistant hypophosphatemic rickets and spinal cord compression Apropos of 2 cases, Rev. Rhum. Ed. Fr. 60 (1993) 64–68. [13] Y. Yamamoto, B.M. Onofrio, Spinal canal stenosis with hypophosphatemic vitamin D-resistant rickets: case report, Neurosurgery 35 (512) (1994) 512–515, discussion 514–515. [14] E.S. Ballantyne, G.F. Findlay, Thoracic spinal stenosis in two brothers due to vitamin D-resistant rickets, Eur. Spine J. 5 (1996) 125–127. [15] D.J. Dunlop, A.J. Stirling, Thoracic spinal cord compression caused by hypophosphataemic rickets: a case report and review of the world literature, Eur. Spine J. 5 (1996) 272–274. [16] C.L. Vera, J.K. Cure, W.B. Naso, P.L. Gelven, F. Worsham, B.F. Roof, D. Resnick, C.F. Salinas, J.A. Gross, A. Pacult, Paraplegia due to ossification of ligamenta flava in X-linked hypophosphatemia. A case report, Spine PhilaPa 1976 22 (1997) 710–715. [17] N. Thomas, R. Burnet, Hyperparathyroidism and cervical canal stenosis in twins with hypophosphatemic rickets, Pediatr. Int 42 (2000) 310–312. [18] A. Kawaguchi, K. Miyamoto, K. Wakahara, H. Hosoe, A. Miura, T. Hanamoto, K. Shimizu, Surgical treatment of multiple spinal canal stenoses associated with vitamin D-resistant rickets, J. Clin. Neurosci. 16 (2009) 717–719. [19] M. Shiba, M. Mizuno, K. Kuraishi, H. Suzuki, Cervical ossification of posterior longitudinal ligament in x-linked hypophosphatemic rickets revealing homogeneously increased vertebral bone density, Asian Spine J. 9 (2015) 106–109. [20] G.W. Albert, A.H. Menezes, Ankylosing spondylitis of the craniovertebral junction: a single surgeon’s experience, J. Neurosurg. Spine 14 (2011) 429–436. [21] J. Gagnier, G. Kienle, D.G. Altman, D. Moher, H. Sox, D.S. Riley, the CARE group, The CARE guidelines: consensus-based clinical case report guideline development, J. Clin. Epidemiol. 67 (1) (2013) 46–51.
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Contents lists available at ScienceDirect
International Journal of Surgery Case Reports journal homepage: www.casereports.com
Extensive ossification of the paraspinal ligaments in a patient with vitamin D-resistant rickets: Case report with literature review Yujiro Hirao ∗, Hirotaka Chikuda, Yasushi Oshima, Yoshitaka Matsubayashi, Sakae Tanaka Department of Orthopaedic Surgery, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo 113-8655, Japan
a r t i c l e
i n f o
Article history: Received 4 June 2016 Received in revised form 22 August 2016 Accepted 24 August 2016 Available online 28 August 2016 Keywords: Spinal ossification Vitamin D-resistant rickets Myelopathy Spinal ankylosis
a b s t r a c t INTRODUCTION: Ectopic ossification of the spinal ligaments is not uncommon in patients with Vitamin D-resistant rickets (VDRR), but the long-term consequences of this condition have not been reported. PRESENTATION OF CASE: The case was a 65-year-old female with VDRR who reported progressive weakness of the upper extremities, difficulty walking, neck pain, and numbness in the left arm. Imaging studies demonstrated cord compression with ectopic ossification at the rim of the occipital bone and OPLL at C1 level. Ankylosis of the whole spine below the C2 vertebra was also noted with preserved mobility only at the craniovertebral junction. DISCUSSION: Our report showed that ectopic ossification of the spinal ligament can result in ankylosis of the entire spine in patients with VDRR. In such patients, the segments with remaining mobility are considered to be at high risk of developing myelopathy due to increased stress at the junction. CONCLUSION: The present case underscores the importance of providing long-term follow-up in VDRR patients presenting with ectopic ossification of the spinal ligaments. In particular, physicians should pay close attention to the possibility of myelopathy in any segments with preserved mobility. © 2016 The Authors. Published by Elsevier Ltd on behalf of IJS Publishing Group Ltd. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
1. Introduction Vitamin D-resistant rickets (VDRR) is a group of metabolic disorders characterized by renal tubular defects in phosphate transport and bone abnormalities resulting in hypophosphatemic rickets or osteomalacia1 . Patients with VDRR may present with deformities of the lower extremities, bone or joint pain, short stature or dental abnormalities [1] and tend to develop calcium deposits at the attachments of tendons or ligaments as they age [1]. It is recognized that ectopic ossifications of the spinal ligaments are not uncommon in VDRR patients, which may result in compression of the spinal cord and subsequent myelopathy [2–5]. Despite this, the long-term consequences of ossified spinal ligaments in VDRR patients have not been well documented. We report a case of VDRR presenting with extensive ossification of the spinal ligaments for which we performed long-term follow-up. 2. Presentation of case The patient was first referred to our hospital because of difficulty in walking at the age of 34, when she was diagnosed with VDDR.
∗ Corresponding author at: Department of Spine and Orthopaedic Surgery, Japanese Red Cross Medical Center, 4-1-22 Hiroo, Shibuya-ku, Tokyo 150–8935, Japan. E-mail address: [email protected] (Y. Hirao).
Later, the diagnosis was genetically confirmed as described by a different research group [6]. The patient had previously undergone T7–T9 laminectomy due to thoracic myelopathy at another hospital at the age of 24, after which her myelopathic symptoms subsided for 7 years. At the age of 34, she underwent a second posterior decompression surgery (T4–T9) for gait disturbance due to thoracic myelopathy after a diagnosis of OPLL and OYL, which resulted in improvement of her symptoms. Since then, the patient has been followed-up on annual basis and remained functionally stable for over 30 years. At the age of 65, she reported weakness of the upper extremities, difficulty walking, neck pain, and numbness in the left arm. She was admitted for further investigation and treatment. On admission, she was 118 cm tall with a marked round back and bowed legs. She was able to walk only short distances supporting herself on a wall. Neurologic examination revealed decreased light touch and pinprick sensation, and motor weakness (3/5 strength) in the distal upper extremities. The grip power was 6 kg in both hands. Tendon reflexes were equivocal with indifferent Babinski sign bilaterally. Plain radiograph showed marked kyphosis of the thoracic spine (T1–T12 angle; 94◦ ) (Fig. 1). Computed tomography demonstrated ankylosis of the whole spine below the C2 vertebra with extensive ossification of the paraspinal ligaments (Fig. 2A, B). In contrast, decreased but preserved mobility (9◦ on flexion and extension) was noted at the craniovertebral junction (CVJ). No overt radiographic instability was found in the atlantoaxial region, with an atlantodental interval of 1 mm. In addition, there was ossification at the
http://dx.doi.org/10.1016/j.ijscr.2016.08.038 2210-2612/© 2016 The Authors. Published by Elsevier Ltd on behalf of IJS Publishing Group Ltd. This is an open access article under the CC BY-NC-ND license (http:// creativecommons.org/licenses/by-nc-nd/4.0/).
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rim of the occipital bone, and OPLL at the C1 level. Magnetic resonance imaging (MRI) revealed spinal cord compression at the levels of both the occipital bone and C1 (Fig. 3). The patient underwent posterior decompression, in which the posterior arch of C1 and the ossified rim of the occipital bone were resected. Deformation of the dural sac was observed at locations corresponding to the resected portions of the rim of the occipital bone and C1 posterior arch, indicating that there had been sustained pressure on the sac. Her postoperative course was uneventful. At the 18-month follow-up visit, the patient was free of pain and numbness. Her grip power had improved to 20 kg in the right and 15 kg in the left hand. She had regained the ability to walk with the support of a cane. 3. Discussion
Fig. 1. Plain lateral radiograph showing marked kyphosis of the thoracic spine.
In this report, we described a case of VDRR associated with extensive ossification of the spinal ligaments followed up for more than 30 years. Ossification of spinal ligaments has been reported in approximately one third of VDRR patients over the age of thirty, and is thought to be linked to both genetic and environmental factors [2,5,6]. In the literature, 23 cases of VDRR patients undergoing surgical treatment for myelopathy have been reported (Table 1) [2,3,7–19]. The majority of the patients are middle-aged (mean age, 44.3 years). In most cases, surgical decompression resulted in favorable outcomes in terms of neurological recovery. However, in reports that consider the implications for spine surgery or changes in the radiological findings, most of the reported followup periods are 2 years or under and the longest period reported is 7 years. In this case, further problems arose 30 years after surgical decompression, a period that has not previously been covered. In the present case, ossification of the spinal ligaments progressed to ankylosis of the entire spine below C2, mimicking ankylosing spondylitis. Given the preserved but minor mobility at the craniovertebral junction, continuing mechanical stress may have been imposed on the stenotic lesion, leading to
Table 1 24 cases of VDRR patients with myelopathy caused by intracanal pathology. Authors, year
Age(yr)/sex
Follow-up duration
Intracanal pathology (level)
Surgery (level)
Dugger and Vandiver [7], 1966 Johnson et al. [8], 1966 Yoshikawa et al. [9], 1968 Highman et al. [10], 1970
60/M 28/F 48/F 28/M 55/M 55/M 50/F 42/M
N/A 2.5 month 6 months 15 months 6 months N/A N/A N/A
bony overgrowth (T11/12) bony overgrowth (T11) OPLL (C3–4) thickened laminae (C2–5) thickened laminae (T6–8) OLF (T5/6, 6/7, 7/8) OLF (C3–5, T7/8) OPLL (C3–5, T7/8), OLF
39/M 49/M 57/M 42/F 32/M 45/M 49/F 48/M 40/F 43/F 54/F 44/F
7 years N/A N/A 6 months 3 months 6 months 3 months 5 years N/A N/A 2 years N/A
OLF (T7–10) OLF (T8/9) OLF (T5–8) degenerative changes (T3–11) OLF (T7–11) CLF (T10/11) OLF (T5/6, 6/7, 7/8, 9/10) OLF (thoracic including T9/10) CLF (C5/6) CLF (cervical) OPLL (C4–6) OPLL (C2/3, C4), calcified disk (C6/7) OPLL (C5–T1, T12/L1), OLF (T8/9, T9/10, T10/11) OPLL (C1/2, C2/3–6/7) OPLL (C2–7) ossification at the rim of the occipital bone, OPLL (C1/2)
laminectomy (T11, 12) laminectomy (T10–L1) laminectomy (C2–5) laminectomy (C2–5) laminectomy (T6–8) laminectomy (T5–8) laminectomy (C3–T1, T7, 8) anterior surgery (C3/4, C4/5), laminectomy (C3–7) laminectomy (T7–11) laminectomy (T8–10) laminectomy (T3–9) laminectomy (T3–11) laminectomy (T7–11) laminectomy (T10, 11) laminectomy (T6–10) laminectomy (T10) laminectomy (C2–6) laminectomy (cervical) laminoplasty (C3–7) anterior surgery (C6/7)
Bradbury et al. [11], 1987
Matsui et al. [3], 1991 Bussiere et al. [12], 1993 Yamamoto and Onofrio [13], 1994 Ballantyne and Findlay [14], 1996 Dunlop and Stirling [15], 1996 Vera et al. [16], 1997 Thomas and Burnet [17], 2000 Velan et al. [5], 2001 Soehle and Casey [4], 2002
laminoplasty (C2–7, T8–11), anterior surgery (T12/L1) Lee et al. [2], 2012 36/F 3 years laminectomy and fusion (C3–7) 32/F 2 years laminoplasty (C2–6) Shiba et al. [19], 2015 resection of both ossified rim 65/F 31 years Present case of the occipital bone and posterior arch of C1 VDRR, Vitamin D-resistant rickets; N/A, data not available; OPLL, ossification of the posterior longitudinal ligament; OLF, ossification of ligamentum flavum; CLF, calcification of ligamentum flav Kawaguchi et al. [18], 2009
44/F
5 years
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Fig. 3. T2-weighted magnetic resonance imagine revealing spinal cord compression at the C1 level by the ossified tissues.
4. Conclusion The present case underscores the importance of continuing the periodic monitoring of VDRR patients presenting with ectopic ossification of the spinal ligaments over a period of decades. Physicians should pay close attention to possible myelopathy occurring at the segment with preserved mobility. Conflict of interest The authors declare that they have no conflict of interest. Funding The present study did not receive funding from any sponsors. Ethical approval For this type of study formal consent is not required. Patient consent Fig. 2. Sagittal reconstruction images of computed tomography demonstrating fusion of the entire spine below C2: Cervical spine (A) and thoracolumbar spine (B).
Informed consent was obtained from all individual participants included in this study. Author contribution
myelopathy. Similar pathomechanisms have been reported in patients with ankylosing spondylitis, presenting as various pathologies at the craniovertebral junction including atlantoaxial subluxation, retroodontoid pannus, basilar invagination, and bone erosion [20]. This study was reported in line with the CARE criteria [21].
Yujiro Hirao: contributed to data collection and writing the manuscript. Hirotaka Chikuda: was the supervisor and contributed to discussion and writing the manuscript. Yasushi Oshima: contributed to discussion and data collection. Yoshitaka Matsubayashi: contributed to discussion and data collection. Sakae Tanaka: contributed to the discussion.
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