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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: Preoperative photo of a 16-year-old boy with arthrogryposis. B: Preoperative x-ray reveals a rigid curve of 91 degrees. C: Postoperative photo of the same patient shows partial correction and improved trunk balance. D: Postoperative x-ray shows partial correction of the curve that manages to control further pulmonary deterioration.
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Figure 3: A: Preoperative photo of a 16-year-old boy with arthrogryposis. B: Preoperative x-ray reveals a rigid curve of 91 degrees. C: Postoperative photo of the same patient shows partial correction and improved trunk balance. D: Postoperative x-ray shows partial correction of the curve that manages to control further pulmonary deterioration.

Mentions: A 16-year-old male presented with arthrogryposis and a right thoracic curve of 91 degrees (Figure 3A, 3B). Pulmonary function proved severely deteriorated as spirometry tests recorded FVC 25%, so after careful preoperative evaluation surgical treatment was decided. Anterior surgery was avoided due to poor respiratory function of the patient. Posterior spinal fusion was performed with partial correction of the curve in order to prevent further progression (Figure 3C, 3D). Postoperative hospitalization in intensive care unit was done for one week. The rest postoperative course was uneventful but with long hospitalization regarding the pulmonary functions of the patient. At a 3 year follow up the patient presents a stable condition with improvement of pulmonary function (FVC 30%) and absence of any complication.


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: Preoperative photo of a 16-year-old boy with arthrogryposis. B: Preoperative x-ray reveals a rigid curve of 91 degrees. C: Postoperative photo of the same patient shows partial correction and improved trunk balance. D: Postoperative x-ray shows partial correction of the curve that manages to control further pulmonary deterioration.
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 3: A: Preoperative photo of a 16-year-old boy with arthrogryposis. B: Preoperative x-ray reveals a rigid curve of 91 degrees. C: Postoperative photo of the same patient shows partial correction and improved trunk balance. D: Postoperative x-ray shows partial correction of the curve that manages to control further pulmonary deterioration.
Mentions: A 16-year-old male presented with arthrogryposis and a right thoracic curve of 91 degrees (Figure 3A, 3B). Pulmonary function proved severely deteriorated as spirometry tests recorded FVC 25%, so after careful preoperative evaluation surgical treatment was decided. Anterior surgery was avoided due to poor respiratory function of the patient. Posterior spinal fusion was performed with partial correction of the curve in order to prevent further progression (Figure 3C, 3D). Postoperative hospitalization in intensive care unit was done for one week. The rest postoperative course was uneventful but with long hospitalization regarding the pulmonary functions of the patient. At a 3 year follow up the patient presents a stable condition with improvement of pulmonary function (FVC 30%) and absence of any complication.

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