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Cervical spine surgery in patients with diastrophic dysplasia: Case report with long-term follow-up.

Jasiewicz B, Potaczek T, Duda S, Tęsiorowski M - J Craniovertebr Junction Spine (2015 Oct-Dec)

Bottom Line: The effects of the correction remained stable for respectively 6 and 10 years of the follow-up period.The unique follow-up confirms that this type of intervention leads to an effective and long lasting results.Significant cervical kyphosis in patients suffering from DTD may be treated surgically using anterior approach even in young children with a favorable and lasting results.

View Article: PubMed Central - PubMed

Affiliation: Department of Orthopaedic Surgery and Rehabilitation, Faculty of Medicine, Jagiellonian University, Zakopane, Poland.

ABSTRACT
Cervical kyphosis in diastrophic dysplasia (DTD) is a very dangerous deformity which may lead to compression of neural structures resulting in tetraplegia or even. Treatment of this deformity is usually surgical, but no long-term follow-up studies are presented in the literature. Authors present a case of two children with DTD who underwent anterior corpectomy due to severe cervical kyphosis. The kyphotic deformity was corrected and the normal spinal canal width was restored. The effects of the correction remained stable for respectively 6 and 10 years of the follow-up period. The unique follow-up confirms that this type of intervention leads to an effective and long lasting results. Significant cervical kyphosis in patients suffering from DTD may be treated surgically using anterior approach even in young children with a favorable and lasting results.

No MeSH data available.


Related in: MedlinePlus

Case 1, male. Follow-up computed tomography scan of the cervical spine with visible smooth shape of the vertebral canal
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Figure 2: Case 1, male. Follow-up computed tomography scan of the cervical spine with visible smooth shape of the vertebral canal

Mentions: The patient, Caucasian male, with genetically confirmed DTD, presented at the age of six due to progressing spinal deformity and increasing walking problems. Medical history included previous hip and foot surgeries. The clinical picture included Hitchhiker's thumb, facial dimorphism, knee joint contractures, hip dislocations, clubfeet, and scoliosis. Spinal films showed a 70° right-sided thoracic scoliosis with 26° kyphosis. Hypoplasia of C5 vertebral body with a 10 mm anterior dislocation of C4 vertebra was present [Figure 1]. Segmental C4/C5 kyphosis equalled 40° and the entire cervical spine exhibited 20° of kyphosis. The width of the spinal canal at this level was 5.7 mm in the sagittal plane with spinal cord modelling, but with no intramedullary changes. Due to difficulties with walking associated with critical stenosis at the level of the defect and confirmed progression of cervical kyphosis surgery was recommended. Partial corpectomy of C5 was performed with reposition of C4/C5 dislocation and anterior C4-C6 fixation with mesh cage and plate. No intraoperative complications were noted. The soft collar was recommended for 6 weeks. Surgery restored the correct sagittal cervical spine profile to 34° of lordosis and segmental C4/C5 angulation to 10° lordosis. In the following years, the patient underwent surgery for scoliosis, hip osteotomy and forefoot reconstruction. Ten years after cervical spine surgery C4/C5 segmental lordosis was 10°, C1-C7 lordosis 51°. Smooth outlines of the vertebral canal walls in the cervical spine were present in a follow-up computer tomography scan [Figure 2]. The patient remained an independent, self-sufficient walker.


Cervical spine surgery in patients with diastrophic dysplasia: Case report with long-term follow-up.

Jasiewicz B, Potaczek T, Duda S, Tęsiorowski M - J Craniovertebr Junction Spine (2015 Oct-Dec)

Case 1, male. Follow-up computed tomography scan of the cervical spine with visible smooth shape of the vertebral canal
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4660502&req=5

Figure 2: Case 1, male. Follow-up computed tomography scan of the cervical spine with visible smooth shape of the vertebral canal
Mentions: The patient, Caucasian male, with genetically confirmed DTD, presented at the age of six due to progressing spinal deformity and increasing walking problems. Medical history included previous hip and foot surgeries. The clinical picture included Hitchhiker's thumb, facial dimorphism, knee joint contractures, hip dislocations, clubfeet, and scoliosis. Spinal films showed a 70° right-sided thoracic scoliosis with 26° kyphosis. Hypoplasia of C5 vertebral body with a 10 mm anterior dislocation of C4 vertebra was present [Figure 1]. Segmental C4/C5 kyphosis equalled 40° and the entire cervical spine exhibited 20° of kyphosis. The width of the spinal canal at this level was 5.7 mm in the sagittal plane with spinal cord modelling, but with no intramedullary changes. Due to difficulties with walking associated with critical stenosis at the level of the defect and confirmed progression of cervical kyphosis surgery was recommended. Partial corpectomy of C5 was performed with reposition of C4/C5 dislocation and anterior C4-C6 fixation with mesh cage and plate. No intraoperative complications were noted. The soft collar was recommended for 6 weeks. Surgery restored the correct sagittal cervical spine profile to 34° of lordosis and segmental C4/C5 angulation to 10° lordosis. In the following years, the patient underwent surgery for scoliosis, hip osteotomy and forefoot reconstruction. Ten years after cervical spine surgery C4/C5 segmental lordosis was 10°, C1-C7 lordosis 51°. Smooth outlines of the vertebral canal walls in the cervical spine were present in a follow-up computer tomography scan [Figure 2]. The patient remained an independent, self-sufficient walker.

Bottom Line: The effects of the correction remained stable for respectively 6 and 10 years of the follow-up period.The unique follow-up confirms that this type of intervention leads to an effective and long lasting results.Significant cervical kyphosis in patients suffering from DTD may be treated surgically using anterior approach even in young children with a favorable and lasting results.

View Article: PubMed Central - PubMed

Affiliation: Department of Orthopaedic Surgery and Rehabilitation, Faculty of Medicine, Jagiellonian University, Zakopane, Poland.

ABSTRACT
Cervical kyphosis in diastrophic dysplasia (DTD) is a very dangerous deformity which may lead to compression of neural structures resulting in tetraplegia or even. Treatment of this deformity is usually surgical, but no long-term follow-up studies are presented in the literature. Authors present a case of two children with DTD who underwent anterior corpectomy due to severe cervical kyphosis. The kyphotic deformity was corrected and the normal spinal canal width was restored. The effects of the correction remained stable for respectively 6 and 10 years of the follow-up period. The unique follow-up confirms that this type of intervention leads to an effective and long lasting results. Significant cervical kyphosis in patients suffering from DTD may be treated surgically using anterior approach even in young children with a favorable and lasting results.

No MeSH data available.


Related in: MedlinePlus