[Pulmonary dysfunction patterns in patients with Marfan and Marfanoid syndrome associated with scoliosis and the influencing factors].
OBJECTIVE
To investigate the impairment pattern and the influencing factors of pulmonary function in patients with Marfan and Marfanoid syndrome associated scoliosis (MS).


METHODS
In this retrospective study, totally 25 MS patients (aged 11 - 20 years, 11 boys and 14 girls) who received posterior instrumentation and fusion (Group A) and 38 adolescent idiopathic scoliosis (AIS) patients (Group B) (aged 10 - 19 years, 11 boys and 27 girls) were included from February 1998 to September 2007. The curve pattern was matched in both groups. The preoperative pulmonary function test (PFTs) were compared in two groups. And the parameters influencing the preoperative pulmonary function were analyzed in group A.


RESULTS
In Group A, the Cobb angle of thoracic curve was negatively correlated with the percentage of predicted pulmonary volumes (VC%, FVC% and FEV1%) (r = -0.514, -0.503, -0.464, P < 0.05). And the reduction of lung function parameters (VC%, FVC%, FEV1% and MMEF%) was more severe in Group A than in Group B with compared magnitude of thoracic curve (P < 0.05). In Group A, the extent of impairment of pulmonary function in patients with the number of vertebrae involved ≥ 8 were more severe than those involved < 8 vertebrae (P < 0.05). However, there was no significant difference of deterioration of lung function between the higher apex (T₄₋₈) subgroup and lower apex (T₉₋₁₂) subgroup. And no correlation was found between thoracic kyphosis and the degrees of impairment of respiration function.


CONCLUSIONS
Patients with MS have mixed ventilation dysfunction, which is more severe than AIS patients with matched age and Cobb angle. The pulmonary dysfunction in MS patients can be influenced by the severity of thoracic curve and the number of involved vertebrae.
Wang
Bin
B
Department of Orthopaedic Surgery, the Affiliated Drum Tower Hospital of Nanjing University Medical School, Nanjing 210008, China.
Cao
Xing-bing
XB
Qiu
Yong
Y
Qian
Bang-ping
BP
Sun
Xu
X
Huang
Ai-bing
AB
Zhu
Ze-zhang
ZZ
Yu
Yang
Y
Zhu
Feng
F
Ma
Wei-wei
WW
D000293:Adolescent; D002648:Child; D005260:Female; D006801:Humans; D008168:Lung / Q000503:physiopathology*; D008297:Male; D008382:Marfan Syndrome / Q000150:complications / Q000503:physiopathology*; D012129:Respiratory Function Tests; D012189:Retrospective Studies; D012600:Scoliosis / Q000150:complications / Q000503:physiopathology*; D055815:Young Adult