Amianthoid transformation of costal cartilage matrix in children with pectus excavatum and pectus carinatum.

Alexandr Kurkov,Anatoly Shekhter,Vyacheslav Paukov,Anastasia Frolova,Petr Timashev,Vladimir Plyakin,Svetlana Kotova,Nafisa Fayzullinа,Nikita Kurtak,Anna Guller,Alexey Fayzullin,Lin Han

doi:10.1371/journal.pone.0245159

Abstract

It is unclear if amianthoid transformation (AT) of costal cartilage extracellular matrix (ECM) has an impact on the development of pectus excavatum (PE) and pectus carinatum (PC). AT foci were examined in intrasurgical biopsy specimens of costal cartilages of children (8-17 years old) with PE (n = 12) and PC (n = 12) and in age-matching autopsy control samples (n = 10) using histological and immunohistochemical staining, atomic force and nonlinear optical microscopy, transmission and scanning electron microscopy, morphometry and statistics. AT areas were identified in the costal cartilage ECM in children with normal chest, PE and PC. Each type of the AT areas ("canonical", "intertwined", "fine-fibred" and "intralacunary") had a unique morphological pattern of thickness and alignment of amianthoid fibers (AFs). AFs were formed via lateral aggregation of collagen type II fibrils in the intact ECM. Foci of the AT were observed significantly more frequently in the PE and PC groups. The AT areas had unique quantitative features in each study group. AT is a structurally diverse form of ECM alteration present in healthy and pathological costal cartilage. PE and PC are associated with specific AT disorders.

Highlights

Pectus excavatum (PE, “funnel chest”) and pectus carinatum (PC, “keeled” or “pigeon” chest) are the most frequent anterior chest wall deformations that can lead to cosmetic defects, severe pain and respiratory and cardiovascular system dysfunctions [1,2,3]
amianthoid transformation (AT) areas were identified in the costal cartilage extracellular matrix (ECM) in children with normal chest, pectus excavatum (PE) and PC
amianthoid fibers (AFs) were formed via lateral aggregation of collagen type II fibrils in the intact ECM

Summary

Introduction

Pectus excavatum (PE, “funnel chest”) and pectus carinatum (PC, “keeled” or “pigeon” chest) are the most frequent anterior chest wall deformations that can lead to cosmetic defects, severe pain and respiratory and cardiovascular system dysfunctions [1,2,3]. PE is the most common (0.1–0.3% of all live births [3]) deformation manifesting as a depression of anterior chest wall at the level of 3rd-7th ribs. The majority of PE manifest during the first year of life [4], while PC is usually revealed in adolescence [5]. Both PE and PC are more common in males and progress over time [6]. Peer Review History: PLOS recognizes the benefits of transparency in the peer review process; we enable the publication of all of the content of peer review and author responses alongside final, published articles.

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