Secondary Thoracic Insufficiency Syndrome in None Ambulatory Myelodisplastic Children.
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Transcript of Secondary Thoracic Insufficiency Syndrome in None Ambulatory Myelodisplastic Children.
Secondary Thoracic Insufficiency Syndrome in None Ambulatory Myelodisplastic Children
Authors
Norman Ramirez MD Pablo Valentin BS Frances M. Rodriguez BS/ RT Francisco Jaume MD Axel Velez MD Ivan Iriarte MD John Flynn MD ( Deceased)
Introduction None ambulatory children with myelodysplasia develop
progressive neuromuscular scoliosis at an early age.
This early onset scoliosis affect lung growth and normal respiration.
They develop Secondary Thoracic Insufficiency Syndrome due the diaphragms is forced cephalad by the collapsing spinal deformity and short trunk.
The natural history of Secondary TIS has never been described
Purpose
To evaluate the spine and chest anatomy of early onset scoliosis in myelodysplastic none ambulatory patients.
Determine how the chest wall mechanics and lung function are affected
Describe the natural history of Secondary TIS
Methods and Material 31 Myelodisplastic None Ambulatory patients were
evaluated
Demographic History and physical examination Radiographs CT scans Pulmonary function test Arterial blood gases Echocardiogram
Results 15 male/ 16 female Two patients currently deceased due to respiratory
failures Average age : 14.6 years ( range : 5 – 27 years) Body Mass Index : 18 ( range : 8 to 33 ) Respiratory problems: Snoring :
17 pts
Mouth breathing: 21 pts
Cessant Breathing: 9 pts
Insomnia : 7 pts
Somnolence: 7 pts
Radiographs
Scoliosis Level : Mostly T8 to Pelvis
Cobb Angle: AP
Supine: 30°
Sitting : 40°
Sitting push up: 29°
Lateral : Kyphosis Lordosis
Sitting : 35° 75°
Radiographs
Space available for the Lung (SAL) : 85%
Thoracic Spine Height : Sitting 18 cm
Sitting Push up 19 cm
Supine traction 20 cm
Lumbar Spine Height: Sitting 9 cm
Sitting Push up 12 cm
Supine traction 10 cm
Ct Scan
Thoracic Spine Height: 19 cm Expected: 28 cm Lumbar Spine Height: 11 cm Expected: 18 cm
Lung Volume 1376 cm3
Expected Lung Volume ( Age/Gender) 3195 cm3
Lung Volume/ Expected Lung Volume: 43 %
Pulmonary Function Test
Forced Vital Capacity %: 56
Forced Expiratory Volume 1 %: 55
FEV1/ FVC Ratio: 112
Blood gases/ Echocardiogram
Blood Gases : PO2 : 99
PCO2: 35
HCO3: 25
Echocardiogram: No evidence of pulmonary hypertension
Conclusions This the first study to validate the entity named
Secondary TIS
In none ambulatory myelodysplastic patients the progressive spine deformity causes Secondary TIS
At early age the respiratory dysfunction can be difficult to detect clinically
The most important factor was the sagittal spine deformity.