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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 15-year-old female with Rett syndrome. B: The patient presented a rigid right thoracic 120°curve.
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Figure 1: A: A 15-year-old female with Rett syndrome. B: The patient presented a rigid right thoracic 120°curve.

Mentions: A 15-year-old female with Rett syndrome was identified and referred to our department for treatment. She presented the typical findings related to this syndrome, as well as a rigid right thoracic curve of 120° with marked kyphosis (Figure 1). Pulmonary function tests could not be performed as a result of her autistic behavior; however arterial blood gases were satisfactory. Surgical treatment was decided and the patient was subjected to combined surgery (anterior and posterior instrumentation and fusion) in a two stage procedure. Due to the severe rigidity of the curve limited correction was achieved. The postoperative course was unremarkable and the patient was discharged 10 days later. At a five year follow up the patient presents a stabilized deformity without clinical findings of further pulmonary deterioration.


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 15-year-old female with Rett syndrome. B: The patient presented a rigid right thoracic 120°curve.
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 1: A: A 15-year-old female with Rett syndrome. B: The patient presented a rigid right thoracic 120°curve.
Mentions: A 15-year-old female with Rett syndrome was identified and referred to our department for treatment. She presented the typical findings related to this syndrome, as well as a rigid right thoracic curve of 120° with marked kyphosis (Figure 1). Pulmonary function tests could not be performed as a result of her autistic behavior; however arterial blood gases were satisfactory. Surgical treatment was decided and the patient was subjected to combined surgery (anterior and posterior instrumentation and fusion) in a two stage procedure. Due to the severe rigidity of the curve limited correction was achieved. The postoperative course was unremarkable and the patient was discharged 10 days later. At a five year follow up the patient presents a stabilized deformity without clinical findings of further pulmonary deterioration.

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