INTERESTING IMAGE
Klippel-Feil Syndrome With Spinal Dysraphism Diastematomyelia on 18F-NaF Bone PET, CT, and MRI Imaging Emmanuel W. Hudson, MD, and William Makis, MD Abstract: A 49-year-old woman with known Klippel-Feil syndrome had a CT scan for staging of stage IIIA invasive carcinoma of the left breast. She was found to have cervicothoracic spinal dysraphism with diastematomyelia. An 18F-NaF bone PET done as part of the metastatic workup showed the characteristic sagittal bone spur, and MRI demonstrated a complete split of the cervical and upper thoracic spinal cord, previously unknown to the patient. Key Words: diastematomyelia, spinal dysraphism, Klippel-Feil syndrome, F-NaF bone PET
18
(Clin Nucl Med 2016;00: 00–00)
Received for publication October 1, 2015; revision accepted November 26, 2015. From the Department of Diagnostic Imaging, Cross Cancer Institute, Edmonton, Alberta, Canada. Conflicts of interest and sources of funding: none declared. Correspondence to: William Makis MD, Department of Diagnostic Imaging, Cross Cancer Institute, Edmonton, Alberta, T6G 1Z2, Canada. E-mail: [email protected]. Copyright © 2016 Wolters Kluwer Health, Inc. All rights reserved. ISSN: 0363-9762/16/0000–0000 DOI: 10.1097/RLU.0000000000001123
REFERENCES 1. Maroun FB, Jacob JC, Heneghan WD. Diastematomyelia. St. Louis: Warren H.Green, Inc; 1976:1–133. 2. Nagib MG, Maxwell RE, Chou SN. Identification and management of highrisk patients with Klippel-Feil syndrome. J Neurosurg. 1984;61:523–530. 3. Ulmer JL, Elster AD, Ginsberg LE, et al. Klippel-Feil syndrome: CT and MR of congenital and acquired abnormalities of cervical spine and cord. J Comput Assist Tomogr. 1993;17:215–224. 4. Palmers M, Peene P, Massa G. Cervicothoracic diastematomyelia with Klippel-Feil syndrome. Rofo. 2003;175:1579–1581. 5. Zaleska-Dorobisz U, Bladowska J, Biel A, et al. MRI diagnosis of diastematomyelia in a 78 year old woman: case report and literature review. Pol J Radiol. 2010; 75:82–87. 6. Wenger M, Hauswirth CB, Brodhage RP. Undiagnosed adult diastematomyelia associated with neurological symptoms following spinal anaesthesia. Anaesthesia. 2001;56:764–767. 7. Leungh YL, Buxton N. Combined diastematomyelia and hemivertebra: a review of the management at a single centre. J Bone Joint Surg Br. 2005;87B: 1380–1384. 8. David KM, Copp AJ, Stevens JM, et al. Split cervical spinal cord with Klippel-Feil syndrome: seven cases. Brain. 1996;119:1859–1872. 9. Simpson RK, Rose JE. Cervical diastematomyelia. Report of a case and review of a rare congenital anomaly. Arch Neurol. 1987;44:331–334. 10. Thomas BP, Spottswood SE, Wushensky CA. The hot spur sign of diastematomyelia. Clin Nucl Med. 2007;32:292–294.
Clinical Nuclear Medicine • Volume 00, Number 00, Month 2016
Copyright © 2012 American College of Nuclear Medicine
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Hudson and Makis
Clinical Nuclear Medicine • Volume 00, Number 00, Month 2016
FIGURE 1. Axial CT, MRI (3D T2*-weighted spoiled gradient echo sequence) and 18F-NaF PET (dose: 225 MBq, or 6.1 mCi) images demonstrating diastematomyelia (there was a complete split of the cord from the upper cervical spine to the upper thoracic spine) and a sagittal bone spur at the T1 level with associated block vertebra formation. The uptake on 18F-NaF PET was similar to the background bone uptake. The patient was not aware of the split spinal cord and complained of occasional mild neck pain.
FIGURE 2. Coronal CT, MRI (T2-weighted turbo spin-echo sequence), and 18F-NaF PET images demonstrating split in superior thoracic spinal cord. Klippel-Feil syndrome was originally described as patients with a short webbed neck, decreased range of motion in the cervical spine, and a low hairline. Patients with Klippel-Feil syndrome usually present with the disease during childhood, however, can occasionally, as in this case, present later in life. The etiology of Klippel-Feil syndrome and its associated conditions is unknown. The syndrome can present with a variety of other clinical syndromes, including fetal alcohol syndrome, Goldenhar syndrome, and anomalies of the extremities.1–3 Diastematomyelia, also known as a split cord malformation, refers to a type of spinal dysraphism when there is a longitudinal split in the spinal cord either completely or incompletely with or without a fibrous or osseocartilagenous septum. Most cases occur in the lower thoracic and lumbar regions.4–7 Diastematomyelia is very rare in the cervical and upper thoracic regions, with only 32 cases reported in the literature8 and is usually associated with abnormalities of the craniovertebral junction especially with Klippel-Feil syndrome and Chiari type II malformation.9 In 7 cases of Klippel-Feil syndrome, a high cervical cord split was documented with CT, MRI, and CT-myelograms.8 Increased uptake on a 99mTc-MDP bone scan in the sagittal spur in a diastematomyelia patient has been described in 1 case.10 However, 18F-NaF bone PET imaging of diastematomyelia has not been previously described. In our case, the sagittal bone spur did not demonstrate increased 18F-NaF uptake on the bone PET scan.
FIGURE 3. 3D reconstruction images (performed with Terrarecon Aquariusnet Viewer version 4.4) show vertebral fusions from upper cervical spine to upper thoracic spine, as well as the sagittal bone spur and split spinal cord. 2
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© 2016 Wolters Kluwer Health, Inc. All rights reserved.
Copyright © 2012 American College of Nuclear Medicine
Klippel-Feil Syndrome With Spinal Dysraphism Diastematomyelia on 18F-NaF Bone PET, CT, and MRI Imaging Emmanuel W. Hudson, MD, and William Makis, MD Abstract: A 49-year-old woman with known Klippel-Feil syndrome had a CT scan for staging of stage IIIA invasive carcinoma of the left breast. She was found to have cervicothoracic spinal dysraphism with diastematomyelia. An 18F-NaF bone PET done as part of the metastatic workup showed the characteristic sagittal bone spur, and MRI demonstrated a complete split of the cervical and upper thoracic spinal cord, previously unknown to the patient. Key Words: diastematomyelia, spinal dysraphism, Klippel-Feil syndrome, F-NaF bone PET
18
(Clin Nucl Med 2016;00: 00–00)
Received for publication October 1, 2015; revision accepted November 26, 2015. From the Department of Diagnostic Imaging, Cross Cancer Institute, Edmonton, Alberta, Canada. Conflicts of interest and sources of funding: none declared. Correspondence to: William Makis MD, Department of Diagnostic Imaging, Cross Cancer Institute, Edmonton, Alberta, T6G 1Z2, Canada. E-mail: [email protected]. Copyright © 2016 Wolters Kluwer Health, Inc. All rights reserved. ISSN: 0363-9762/16/0000–0000 DOI: 10.1097/RLU.0000000000001123
REFERENCES 1. Maroun FB, Jacob JC, Heneghan WD. Diastematomyelia. St. Louis: Warren H.Green, Inc; 1976:1–133. 2. Nagib MG, Maxwell RE, Chou SN. Identification and management of highrisk patients with Klippel-Feil syndrome. J Neurosurg. 1984;61:523–530. 3. Ulmer JL, Elster AD, Ginsberg LE, et al. Klippel-Feil syndrome: CT and MR of congenital and acquired abnormalities of cervical spine and cord. J Comput Assist Tomogr. 1993;17:215–224. 4. Palmers M, Peene P, Massa G. Cervicothoracic diastematomyelia with Klippel-Feil syndrome. Rofo. 2003;175:1579–1581. 5. Zaleska-Dorobisz U, Bladowska J, Biel A, et al. MRI diagnosis of diastematomyelia in a 78 year old woman: case report and literature review. Pol J Radiol. 2010; 75:82–87. 6. Wenger M, Hauswirth CB, Brodhage RP. Undiagnosed adult diastematomyelia associated with neurological symptoms following spinal anaesthesia. Anaesthesia. 2001;56:764–767. 7. Leungh YL, Buxton N. Combined diastematomyelia and hemivertebra: a review of the management at a single centre. J Bone Joint Surg Br. 2005;87B: 1380–1384. 8. David KM, Copp AJ, Stevens JM, et al. Split cervical spinal cord with Klippel-Feil syndrome: seven cases. Brain. 1996;119:1859–1872. 9. Simpson RK, Rose JE. Cervical diastematomyelia. Report of a case and review of a rare congenital anomaly. Arch Neurol. 1987;44:331–334. 10. Thomas BP, Spottswood SE, Wushensky CA. The hot spur sign of diastematomyelia. Clin Nucl Med. 2007;32:292–294.
Clinical Nuclear Medicine • Volume 00, Number 00, Month 2016
Copyright © 2012 American College of Nuclear Medicine
www.nuclearmed.com
1
Hudson and Makis
Clinical Nuclear Medicine • Volume 00, Number 00, Month 2016
FIGURE 1. Axial CT, MRI (3D T2*-weighted spoiled gradient echo sequence) and 18F-NaF PET (dose: 225 MBq, or 6.1 mCi) images demonstrating diastematomyelia (there was a complete split of the cord from the upper cervical spine to the upper thoracic spine) and a sagittal bone spur at the T1 level with associated block vertebra formation. The uptake on 18F-NaF PET was similar to the background bone uptake. The patient was not aware of the split spinal cord and complained of occasional mild neck pain.
FIGURE 2. Coronal CT, MRI (T2-weighted turbo spin-echo sequence), and 18F-NaF PET images demonstrating split in superior thoracic spinal cord. Klippel-Feil syndrome was originally described as patients with a short webbed neck, decreased range of motion in the cervical spine, and a low hairline. Patients with Klippel-Feil syndrome usually present with the disease during childhood, however, can occasionally, as in this case, present later in life. The etiology of Klippel-Feil syndrome and its associated conditions is unknown. The syndrome can present with a variety of other clinical syndromes, including fetal alcohol syndrome, Goldenhar syndrome, and anomalies of the extremities.1–3 Diastematomyelia, also known as a split cord malformation, refers to a type of spinal dysraphism when there is a longitudinal split in the spinal cord either completely or incompletely with or without a fibrous or osseocartilagenous septum. Most cases occur in the lower thoracic and lumbar regions.4–7 Diastematomyelia is very rare in the cervical and upper thoracic regions, with only 32 cases reported in the literature8 and is usually associated with abnormalities of the craniovertebral junction especially with Klippel-Feil syndrome and Chiari type II malformation.9 In 7 cases of Klippel-Feil syndrome, a high cervical cord split was documented with CT, MRI, and CT-myelograms.8 Increased uptake on a 99mTc-MDP bone scan in the sagittal spur in a diastematomyelia patient has been described in 1 case.10 However, 18F-NaF bone PET imaging of diastematomyelia has not been previously described. In our case, the sagittal bone spur did not demonstrate increased 18F-NaF uptake on the bone PET scan.
FIGURE 3. 3D reconstruction images (performed with Terrarecon Aquariusnet Viewer version 4.4) show vertebral fusions from upper cervical spine to upper thoracic spine, as well as the sagittal bone spur and split spinal cord. 2
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Copyright © 2012 American College of Nuclear Medicine
