A 36-Year-Old Female With Congenital Contractural Arachnodactyly and Pectus Excavatum Requiring Fourth-Time Redo Surgical Correction
Autor: | Rachel S. Dada, Alper Toker, Jeremiah W. Hayanga, Mir Ali Abbas Khan, Heather K. Hayanga |
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Jazyk: | angličtina |
Rok vydání: | 2021 |
Předmět: |
Marfan syndrome
beal's syndrome medicine.medical_specialty pectus excavatum macromolecular substances Nuss procedure cardiac anesthesia congenital contractural arachnodactyly Pectus excavatum nuss procedure Anesthesiology medicine echocardiography Restrictive lung disease Congenital contractural arachnodactyly Kyphoscoliosis Rib cage cardiac physiology business.industry fungi General Engineering medicine.disease thoracic surgery Surgery Cardiothoracic surgery Cardiac/Thoracic/Vascular Surgery cardiovascular system Anatomy business |
Zdroj: | Cureus |
ISSN: | 2168-8184 |
Popis: | Congenital contractural arachnodactyly (CCA) is a rare connective tissue disorder that has several phenotypic similarities to Marfan syndrome. Among the phenotypic characteristics of patients with CCA, severe kyphoscoliosis and thoracic cage abnormalities are commonly reported. In this case report, we describe a patient with coexisting CCA and severe pectus excavatum requiring multiple surgical repairs. The impact severe scoliosis and pectus excavatum in isolation have on cardiopulmonary anatomy and physiology can be significant, and their effects can be profound concomitantly. These defects have the propensity of causing restrictive lung disease and external cardiac compression. |
Databáze: | OpenAIRE |
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