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
Jazyk: angličtina
Rok vydání: 2021
Předmět:
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