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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 12-year-old female with Noonan syndrome. Typical webbed neck. Double structural curve with rib deformity.
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Figure 7: A 12-year-old female with Noonan syndrome. Typical webbed neck. Double structural curve with rib deformity.

Mentions: A 12-year-old female with severe thoracic scoliosis secondary to Noonan syndrome was referred to our department for treatment. At the age of 4.5 years she had been subjected to pulmonary valvoplasty with excellent results and a subsequent normal activity level. She had been unsuccessfully treated with a Boston type thoracolumbar brace. When evaluated at our department, the patient presented a rigid right thoracic (T3–T10) 67° curve with significant rib deformity, a left thoracolumbar (T10–L2) 37° curve and thoracic hypokyphosis (Figure 7). After a thorough work-up consisting of hematological, cardiological, endocrinological and anesthesiological evaluation, surgical treatment was decided on, with prophylactic administration of penicillin and postoperative care in an intensive care unit. The postoperative course was uneventful and the patient was discharged on the 9th postoperative day. At a four year follow up (2006) the patient present stable condition without any complications.


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 12-year-old female with Noonan syndrome. Typical webbed neck. Double structural curve with rib deformity.
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 7: A 12-year-old female with Noonan syndrome. Typical webbed neck. Double structural curve with rib deformity.
Mentions: A 12-year-old female with severe thoracic scoliosis secondary to Noonan syndrome was referred to our department for treatment. At the age of 4.5 years she had been subjected to pulmonary valvoplasty with excellent results and a subsequent normal activity level. She had been unsuccessfully treated with a Boston type thoracolumbar brace. When evaluated at our department, the patient presented a rigid right thoracic (T3–T10) 67° curve with significant rib deformity, a left thoracolumbar (T10–L2) 37° curve and thoracic hypokyphosis (Figure 7). After a thorough work-up consisting of hematological, cardiological, endocrinological and anesthesiological evaluation, surgical treatment was decided on, with prophylactic administration of penicillin and postoperative care in an intensive care unit. The postoperative course was uneventful and the patient was discharged on the 9th postoperative day. At a four year follow up (2006) the patient present stable condition without any complications.

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