Fibromuscular dysplasia in a 2 years old child – casuistics

Česká verzia

Authors: J. Surgošová ¹; M. Kolníková ¹; Ľ. Podracká ²; T. Dallos ²; S. Jakešová ³; P. Talarčík ⁴
Authors place of work: Klinika detskej neurológie LFUK, NÚDCH, Bratislava, Slovensko ¹; Pediatrická klinika LFUK, NÚDCH, Bratislava, Slovensko ²; Rádiologické oddelenie NÚDCH, Bratislava, Slovensko ³; Cythopatos, s. r. o., Bratislava, Slovensko ⁴
Published in the journal: Čes-slov Pediat 2019; 74 (3): 161-167.
Category:

Summary

Fibromuscular dysplasia is an idiopathic, non-inflammatory disease of small and middle-sized arteries at which the focal rebuilding of vessel walls eventuates into their stenoses, oclusias, dissections or aneurysms. The disease may affect virtually every arterial basin. In adult population it occurs mostly in the group of young females and affects mostly the renal basin.

The authors present a case of a child with serious perinatal anamnesis that developed a clinical appearance of quickly progressing ischemic affected CNS. It was accompanied by uncorrectable hypertension, stenotic affection of renal, carotidal and vertebral basin and very probably splanchnic and extremital arteries. The disease of the child had malign course and ended fatally. That is not typical in adult population, however it occurs in child population.

In adult population it is a rare, nevertheless well described disease. The rareness and specifics of the disease in kids might point to it being a separate unit despite identical histopathological findings.

Keywords:

fibromuscular dysplasia – ischemic stroke – arterial stenosis – hypertension – string of beads – cutis marmorata – hemihypotrophy

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