SHM Converge Scientific Abstract ePoster Gallery-Severe Disfiguring Scoliosis Causing Restrictive Lung Disease and Pulmonary Hypertension

Pdf Summary

This case describes a 64-year-old man with childhood polio who developed severe kyphoscoliosis, restrictive lung disease, and ultimately WHO Group III pulmonary hypertension. He presented with four months of worsening dyspnea and was found to be profoundly hypoxic and later hypercapnic, with mental status changes requiring brief noninvasive positive pressure ventilation in the ICU. Exam and imaging showed marked thoracic deformity, minimal air movement, cardiomegaly, and dilated pulmonary arteries. Echocardiography demonstrated right atrial and right ventricular dilation with an estimated RV systolic pressure of 80 mmHg, supporting pulmonary hypertension.<br /><br />The discussion explains that post-polio syndrome can cause progressive neuromuscular weakness, including paraspinal muscle weakness and scoliosis. In this patient, the resulting severe chest wall restriction likely led to chronic hypoventilation, hypoxemia, and pulmonary hypertension. The case highlights that musculoskeletal deformities can be an underrecognized cause of restrictive lung disease and secondary pulmonary hypertension.<br /><br />He was treated with antibiotics for possible pneumonia, supportive respiratory care, diuretics, and nocturnal NIPPV, then improved and was discharged on low-flow oxygen. At follow-up three months later, he was doing well on diuretics and had planned cardiology follow-up. The report emphasizes early recognition and screening for pulmonary hypertension in patients with severe scoliosis or other chest wall abnormalities, since timely treatment can improve symptoms and reduce cardiopulmonary complications.

Asset Subtitle

Ryan Keller

Meta Tag

Author List Amy Engler, Christo Mathew, Doris Lin, Ryan Keller

Category Clinical Vignettes

Concept Post-Polio Syndrome

Concept Restrictive Lung Disease

Concept Pulmonary Hypertension

Concept WHO Group III Pulmonary Hypertension

Concept Progressive Weakness

Distinguished Non-Finalist

Presenter Organization Baylor College of Medicine

Presenting Author Ryan Keller

Track Adult

Keywords

post-polio syndrome

kyphoscoliosis

restrictive lung disease

pulmonary hypertension

WHO Group III

chronic hypoventilation

hypoxemia

hypercapnia

noninvasive positive pressure ventilation

chest wall deformity

Post-Polio Syndrome

Restrictive Lung Disease

Pulmonary Hypertension

WHO Group III Pulmonary Hypertension

Progressive Weakness