Marfan's Disease and Erdheim's cystic medionecrosis. A study of their pathology

Differentiation between Marfan's disease, or its forme fruste counterpart and Erdheim's cystic medionecrosis can be made in cases of aortic dilatation or dissection. This study examined the morphological features at light microscopy of aortic tissue, removed at surgery in 10 patients with...

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Bibliographic Details
Published in:European heart journal 1991-01, Vol.12 (1), p.83-87
Main Authors: TROTTER, S. E., OLSEN, E. G. J.
Format: Article
Language:English
Subjects:
Adolescent
Adult
Aged
Aged, 80 and over
Aorta - pathology
aortic dilatation
Aortic Diseases - diagnosis
Aortic Diseases - pathology
Aortic dissection
Child
Child, Preschool
Cysts
Diagnosis, Differential
Elastic Tissue - pathology
Erdheim's cystic medionecrosis
Female
Humans
Infant
Infant, Newborn
Male
Marfan Syndrome - diagnosis
Marfan Syndrome - pathology
Marfan Syndrome - surgery
Marian's disease
Middle Aged
Necrosis
Phenotype
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Summary:Differentiation between Marfan's disease, or its forme fruste counterpart and Erdheim's cystic medionecrosis can be made in cases of aortic dilatation or dissection. This study examined the morphological features at light microscopy of aortic tissue, removed at surgery in 10 patients with Marfan's disease. A separate group consisted of 24 patients who did not express the complete Marfan phenotype but had family members who displayed the full and typical features of the disease. This group represented the so-called ‘forme fruste’ variant. These two groups were compared with 12 patients in whom a histopathological diagnosis of Erdheim's cystic medionecrosis had been made. Finally normal aortic tissue from postmortem subjects were made available representing controls. In each case the degree of elastic fragmentation and cystic change of the aortic media was assessed and tabulated. The results indicate that severe fragmentation of elastic is similarly identifiable in both Marfan and Marfan forme fruste groups and that differentiation is easy pathologically from subjects with the comparatively modest fragmentation seen in Erdheim's cystic medionecrosis. Although cystic change was encountered in all groups, its severity tended to parallel the age of the subject, and we consider from our study that Erdheim's cystic medionecrosis represents no more than an exaggerated age-related change.
ISSN:0195-668X
1522-9645
DOI:10.1093/oxfordjournals.eurheartj.a059830