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PLASTIC BRONCHITIS - IS IT A SYNDROMIC DISEASE?

LIDIJA SRKOČ MAJČICA ; General Hospital Zabok and Hospital of Croatian Veterans, Department of Pediatrics, Zagreb, Croatia
DOROTEA BARTONIČEK ; University Hospital Centre Zagreb, Department of Pediatric Cardiology, Zagreb, Croatia
DRAŽEN BELINA ; Department of Cardial Surgery, University Hospital Centre Zagreb, Zagreb, Croatia
SVEN SEIWERTH ; Department of Pathology, University of Zagreb School of Medicine, Zagreb, Croatia
IVAN MALČIĆ ; University Hospital Centre Zagreb, Department of Pediatric Cardiology, Zagreb, Croatia

Abstract

Plastic or cast bronchitis is a very rare and severe condition that occurs as a late complication with increasing frequency in children after the establishment of the Fontan’s circulation. The case study presents an 11-year-old boy with an initial diagnosis of hypoplastic left heart syndrome (HLHS) who developed plastic bronchitis features with typical clinical, macroscopic and microscopic fi ndings. Possible etiological factors are considered in the disease pathogenesis, from some of the borderline original criteria (hypoplasia of large pulmonary blood vessels) to numerous operating and interventional procedures. Regardless of the fact that there is no unique etiological factor, the disease is marked by an underlying disorder in the lymphatic drainage. Possible etiological factors are evaluated in the paper, with extensive analysis of a possible lymphatic fl ow disorder in cardiopulmonary relationship with Fontan’s circulation. Furthermore, other possible late complications with Fontan’s circulation are presented, as well as a comprehensive and successful therapeutic approach to the plastic bronchitis syndrome.

Keywords

Fontan’s circulation; late complications; plastic bronchitis; children; etiology; treatment

Hrčak ID:

245740

URI

https://hrcak.srce.hr/245740

Publication date:

6.11.2020.

Article data in other languages: croatian

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