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Rare causes of scoliosis and spine deformity: experience and particular features.

Soultanis KC, Payatakes AH, Chouliaras VT, Mandellos GC, Pyrovolou NE, Pliarchopoulou FM, Soucacos PN - Scoliosis (2007)

Bottom Line: Surgery was avoided in 3 patients.This study illustrates the fact that different disorders are related with curves with different characteristics, different accompanying problems and possible complications.Investigation and understanding of the underlying pathology is an essential part of the clinical evaluation and preoperative work-up, as clinical experience at any specific center is limited.

View Article: PubMed Central - HTML - PubMed

Affiliation: 1st Department of Orthopaedic Surgery, School of Medicine, University of Athens, "Attikon" Hospital, Rimini 1 Haidari 12462, Athens, Greece. ksoultanis@otenet.gr.

ABSTRACT

Background: Spine deformity can be idiopathic (more than 80% of cases), neuromuscular, congenital or neurofibromatosis-related. However, there are many disorders that may also be involved. We present our experience treating patients with scoliosis or other spine deformities related to rare clinical entities.

Methods: A retrospective study of the records of a school-screening study in North-West Greece was performed, covering a 10-year period (1992-2002). The records were searched for patients with deformities related to rare disorders. These patients were reviewed as regards to characteristics of underlying disorder and spine deformity, treatment and results, complications, intraoperative and anaesthesiologic difficulties particular to each case.

Results: In 13 cases, the spine deformity presented in relation to rare disorders. The underlying disorder was rare neurological disease in 2 cases (Rett syndrome, progressive hemidystonia), muscular disorders (facioscapulohumeral muscular dystrophy, arthrogryposis) in 2 patients, osteogenesis imperfecta in 2 cases, Marfan syndrome, osteopetrosis tarda, spondyloepiphyseal dysplasia congenita, cleidocranial dysplasia and Noonan syndrome in 1 case each. In 2 cases scoliosis was related to other congenital anomalies (phocomelia, blindness). Nine of these patients were surgically treated. Surgery was avoided in 3 patients.

Conclusion: This study illustrates the fact that different disorders are related with curves with different characteristics, different accompanying problems and possible complications. Investigation and understanding of the underlying pathology is an essential part of the clinical evaluation and preoperative work-up, as clinical experience at any specific center is limited.

No MeSH data available.


Related in: MedlinePlus

A: A 17-year-old male with osteopetrosis tarda. Typical cranial deformity, thoracic scoliosis. B: Radiological findings. Note the "thick" quality of bones. C: Excellent result 1.5 years postoperatively.
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Figure 6: A: A 17-year-old male with osteopetrosis tarda. Typical cranial deformity, thoracic scoliosis. B: Radiological findings. Note the "thick" quality of bones. C: Excellent result 1.5 years postoperatively.

Mentions: A 17-year-old male with osteopetrosis tarda was referred to our department for treatment. He had typical cranial deformity, short stature, hearing disability, and a history of multiple operations for correction of cleft palate and dental anomalies (Figure 6A). Radiologic examination revealed typical disease characteristics, as well as a right thoracic (T4–T12) 45° curve with lower lumbar spina bifida (Figure 6B). Complete blood count and chemistry were normal. The patient was subjected to posterior T4–L1 instrumentation and fusion resulting in excellent cosmetic result (Figure 6C). Intubation was difficult due to local anatomy, but there were no intraoperative complications as a result of the brittle vertebrae. No mechanical failure or infection has presented in a follow-up of 8 years.


Rare causes of scoliosis and spine deformity: experience and particular features.

Soultanis KC, Payatakes AH, Chouliaras VT, Mandellos GC, Pyrovolou NE, Pliarchopoulou FM, Soucacos PN - Scoliosis (2007)

A: A 17-year-old male with osteopetrosis tarda. Typical cranial deformity, thoracic scoliosis. B: Radiological findings. Note the "thick" quality of bones. C: Excellent result 1.5 years postoperatively.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2164934&req=5

Figure 6: A: A 17-year-old male with osteopetrosis tarda. Typical cranial deformity, thoracic scoliosis. B: Radiological findings. Note the "thick" quality of bones. C: Excellent result 1.5 years postoperatively.
Mentions: A 17-year-old male with osteopetrosis tarda was referred to our department for treatment. He had typical cranial deformity, short stature, hearing disability, and a history of multiple operations for correction of cleft palate and dental anomalies (Figure 6A). Radiologic examination revealed typical disease characteristics, as well as a right thoracic (T4–T12) 45° curve with lower lumbar spina bifida (Figure 6B). Complete blood count and chemistry were normal. The patient was subjected to posterior T4–L1 instrumentation and fusion resulting in excellent cosmetic result (Figure 6C). Intubation was difficult due to local anatomy, but there were no intraoperative complications as a result of the brittle vertebrae. No mechanical failure or infection has presented in a follow-up of 8 years.

Bottom Line: Surgery was avoided in 3 patients.This study illustrates the fact that different disorders are related with curves with different characteristics, different accompanying problems and possible complications.Investigation and understanding of the underlying pathology is an essential part of the clinical evaluation and preoperative work-up, as clinical experience at any specific center is limited.

View Article: PubMed Central - HTML - PubMed

Affiliation: 1st Department of Orthopaedic Surgery, School of Medicine, University of Athens, "Attikon" Hospital, Rimini 1 Haidari 12462, Athens, Greece. ksoultanis@otenet.gr.

ABSTRACT

Background: Spine deformity can be idiopathic (more than 80% of cases), neuromuscular, congenital or neurofibromatosis-related. However, there are many disorders that may also be involved. We present our experience treating patients with scoliosis or other spine deformities related to rare clinical entities.

Methods: A retrospective study of the records of a school-screening study in North-West Greece was performed, covering a 10-year period (1992-2002). The records were searched for patients with deformities related to rare disorders. These patients were reviewed as regards to characteristics of underlying disorder and spine deformity, treatment and results, complications, intraoperative and anaesthesiologic difficulties particular to each case.

Results: In 13 cases, the spine deformity presented in relation to rare disorders. The underlying disorder was rare neurological disease in 2 cases (Rett syndrome, progressive hemidystonia), muscular disorders (facioscapulohumeral muscular dystrophy, arthrogryposis) in 2 patients, osteogenesis imperfecta in 2 cases, Marfan syndrome, osteopetrosis tarda, spondyloepiphyseal dysplasia congenita, cleidocranial dysplasia and Noonan syndrome in 1 case each. In 2 cases scoliosis was related to other congenital anomalies (phocomelia, blindness). Nine of these patients were surgically treated. Surgery was avoided in 3 patients.

Conclusion: This study illustrates the fact that different disorders are related with curves with different characteristics, different accompanying problems and possible complications. Investigation and understanding of the underlying pathology is an essential part of the clinical evaluation and preoperative work-up, as clinical experience at any specific center is limited.

No MeSH data available.


Related in: MedlinePlus