Fibromuscular Dysplasia of the Coronary Arteries: A Case Report and Review of the Literature.

Fibromuscular dysplasia is a nonatherosclerotic and non-inflammatory vascular disease with primary lesion of renal and internal carotid arteries. We present a neonatal case of fibromuscular dysplasia who died on the second day of life. The newborn suffered from fibromuscular dysplasia of the coronary arteries and a congenital heart defect. The interesting feature of this case was the formation of aneurysms of the coronary arteries with pulmonary atresia. This case demonstrates a casuistically rare form of association between fibromuscular dysplasia of the coronary arteries and pulmonary artery atresia.

[Vascular pathology in the aspect of sudden death in young adults and connective tissue dysplasia: anatomical, physiological, and morphological parallels]. / Patologiia sosudov v aspekte vnezapnoi smerti lits molodogo vozrasta i displaziia soedinitel'noi tkani: anatomo-fiziologicheskie i morfologicheskie paralleli.

Vascular pathology in young adults has during the last 10 years been diagnosed more often during forensic medical examination of sudden death. Major morphological alterations are revealed in cerebral vessels, coronary vessels, and at the level of the ascending portion of the aorta. Generally, in the young age there is no stenosing atherosclerosis inducing vascular lesions and the development of complications. It was determined that connective tissue dysplasia is pathology wherein weakness of the vascular wall is genetically preconditioned, thus promoting formation of vascular aneurysms and rupture of the latter under conditions of provoking factors such as going in for sports, physical loads, and psychoemotional stress.

An 8-month-old boy with congenital fibromuscular dysplasia presenting with shock caused by sudden renal hemorrhage.

Fibromuscular dysplasia (FMD) is a non-atheromatous, non-inflammatory, multifocal segmental angiopathy. FMD is the most common cause of pediatric renovascular hypertension. Aneurysmal formation of the main renal artery and distal branches is a rare complication of FMD in infancy. We report an 8-month-old boy with FMD presenting with shock caused by sudden renal hemorrhage that necessitated removal of one kidney. A diagnosis of renovascular hypertension resulting from intimal type FMD with aneurysmal formation was made on the basis of the presence of hypertension, elevation of PRA and aldosterone activity, pathological findings and the results of renal angiography. Our findings suggest that it is therefore necessary to consider FMD with aneurysmal formation as a possible cause of hypertension and renal hemorrhage in infants.

Congenital fibromuscular dysplasia involving multivessels in an infant with fatal outcome.

UNLABELLED: We report the unusual case of a 2-month-old boy with systemic fibromuscular dysplasia (FMD). He presented with congenital renovascular hypertension due to stenosis of the right renal artery, and later developed renal infarction on the contralateral side resulting in renal failure. The boy subsequently died of intracranial haemorrhage at the age of 14 months. During the course, hemiconvulsion caused by a Moyamoya disease-like vascular lesion was noted. Stenotic lesions of both the abdominal aorta and its branches were also revealed by angiography. Post-mortem examination confirmed that the coronary, splenic and mesenteric arteries were also affected and their histological findings were compatible with FMD. To our knowledge, this is the first congenital case of FMD demonstrating a rapidly progressive course resulting in a fatal outcome. In this case, multivessels in both intracranial and extracranial arteries were involved. CONCLUSION: Our case suggests that the nature of fibromuscular dysplasia is congenital in origin and its aetiology, at least in some cases, is a systemic abnormality of vascular development.

Estudio patológico de la displasia y el adosamiento valvulares en la anomalía de Ebstein / Histologic study of the tricuspid leaflets in Ebstein's anomaly

Se estudiaron cuarenta corazones con anomalía de Ebstein de la válvula tricúspide con el propósito de correlacionar el adosamiento y la displasia valvulares, la atrialización del ventrículo derecho y la fibrosis de pared ventricular derecha. Se encontró una correlación positiva entre ellos. La atrialización ventricular derecha fue directamente proporcional al grado de adosamiento valvar. La correlación en la severidad entre el adosamiento y la displasia fue positiva en el 45 por ciento de nuestro material. La fibrosis del ventrículo derecho fue muy frecuente cuando existió permeabilidad de la válvula pulmonar (90 por ciento), siendo menor (10 por ciento) cuando existió atresia de esta válvula. El daño estructural en la pared ventricular derecha fue directamente proporcional al grado de adosamiento valvar cuando existió permeabilidad de la válvula pulmonar (90 por ciento). En estos corazones el anillo anatómico de la válvula tricúspide siempre estuvo dilatado, mientras que en el grupo con atresia pulmonar, dicha dilatación sólo ocurrió en el 10 por ciento. Cuando existió discordancia atrioventricular el tamaño del anillo anatómico, el grado de adosamiento y displasia valvulares, así como la fibrosis ventricular fueron menos acentuados que en los especímenes con concordancia atrioventricular. La anomalía de Ebstein es una cardiopatía caracterizada por alteración en la histodiferenciación del ventrículo derecho y de la válvula tricúspide que deriva en gran parte de él

Fibromuscular dysplasia and thrombosed aneurysm of the popliteal artery in a child.

A case is reported of bilateral congenital popliteal aneurysms in a 10-year-old girl. Spontaneous thrombosis of the left aneurysm resulted in acute ischemia of the left leg below the knee, and the aneurysm was bypassed with an autogenous saphenous vein graft. Elective resection of the right aneurysm revealed fibromuscular dysplasia, of the medial hyperplasia type, as the cause of the aneurysm. One year after operation, the grafts remained patent, and the patient was asymptomatic.

Fibromuscular dysplasia and the brain. II. Current concept of the disease.

Eleven hundred cases from the literature of fibromuscular dysplasia (FMD) are reviewed including 300 cases with aortocranial lesions. The male-female ratio is 1:2, and the prevalence seems increased among Caucasians. The clinical diagnosis of FMD is made by angiography, ten years earlier in patients with hypertension (mean age 39 years) than in those with cerebrovascular symptoms (mean age 50 years). Segmental dysplastic lesions are found mainly in primary aortic branches. All age groups may be affected and follow-up studies give evidence for stationary as well as slowly progressive lesions. A multifactorial hypothesis of etiology is presented: congenital minor lesions of tunica medial might predispose to aneurysms and to an abnormal fibroproliferative response to mechanical or circulatory stimuli. The association of FMD and intracranial aneurysmal disease in females is discussed. Inheritance as a dominant trait with reduced penetrance in males is suspected. Current aspects on morphology, symptomatology and clinical management are presented.