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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 27-year-old female with faciohumeroscapular muscular dystrophy. Marked non-structural hyperlordosis. Upright position achieved only by forward pelvic tilt.
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Figure 2: A 27-year-old female with faciohumeroscapular muscular dystrophy. Marked non-structural hyperlordosis. Upright position achieved only by forward pelvic tilt.

Mentions: A 27-year-old female with facioscapulohumeral muscular dystrophy was referred to our department for evaluation of an extreme lumbar hyperlordosis, which made ambulation difficult. The patient presented the typical apathetic face, severe weakness of shoulder girdle and upper arm muscles, an extreme lumbar hyperlordosis (120°) and pelvic tilt when standing, as well as a mild double structural scoliotic curve (25°). The hyperlordosis was completely corrected in the supine position. The patient was able to support the upper body and stand only by pressing down on the posterior aspect of her pelvis with both her hands (Figure 2). She was able to sit in a satisfactory position without support. The hyperlordosis was considered secondary to severe pelvic tilt due to inadequacy of the gluteus maximus to maintain hip extension. Loss of support by trunk muscles as a primary cause was ruled out because of satisfactory sitting position. Correction with instrumentation would result in further anterior decompensation. Surgical intervention and orthosis were decided against, for as long as standing remains possible and sitting position without support is satisfactory. At a five year follow up the patient has lost the ability to stay erect, she is using a wheel chair maintaining a satisfactory trunk balance and we believe that surgical treatment is not indicated.


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 27-year-old female with faciohumeroscapular muscular dystrophy. Marked non-structural hyperlordosis. Upright position achieved only by forward pelvic tilt.
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 2: A 27-year-old female with faciohumeroscapular muscular dystrophy. Marked non-structural hyperlordosis. Upright position achieved only by forward pelvic tilt.
Mentions: A 27-year-old female with facioscapulohumeral muscular dystrophy was referred to our department for evaluation of an extreme lumbar hyperlordosis, which made ambulation difficult. The patient presented the typical apathetic face, severe weakness of shoulder girdle and upper arm muscles, an extreme lumbar hyperlordosis (120°) and pelvic tilt when standing, as well as a mild double structural scoliotic curve (25°). The hyperlordosis was completely corrected in the supine position. The patient was able to support the upper body and stand only by pressing down on the posterior aspect of her pelvis with both her hands (Figure 2). She was able to sit in a satisfactory position without support. The hyperlordosis was considered secondary to severe pelvic tilt due to inadequacy of the gluteus maximus to maintain hip extension. Loss of support by trunk muscles as a primary cause was ruled out because of satisfactory sitting position. Correction with instrumentation would result in further anterior decompensation. Surgical intervention and orthosis were decided against, for as long as standing remains possible and sitting position without support is satisfactory. At a five year follow up the patient has lost the ability to stay erect, she is using a wheel chair maintaining a satisfactory trunk balance and we believe that surgical treatment is not indicated.

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