ART OF MEDICINE 2017

 

MALIGNANT HYPERTENSION AND HZELLYA-ERDGEYM'S SYNDROME

 

M.M. Bagriy, L.O. Popovych, L.O. Poberezhna

             SHEI “Ivano-Frankivsk National Medical University” Pathology and Forensic Medicine Department, Ivano-Frankivsk, Ukraine.

 

Abstract

Summary. Hypertension is one of the main factors that contribute to the degeneration of the middle membrane of the aorta as like a disruption of collagen, smooth myocytes and elastic fibers with the development of  the dissection of aorta. Hypertension appears  approximately in  84% of patients with aortic dissection. Provoking factors for the dissection of aorta are hypertensive crisis, intense physical activity, pregnancy, chest trauma, iatrogenic factors. In the modern classifications malignant hypertension is not isolated, but the term is used to refer the  severe fast-progressive flow of arterial hypertension with the characteristic changes in the ocular fundus. Malignant course can have  primary (up to 3% of cases) and secondary (symptomatic) arterial hypertensions (20-25% of cases). As primary hypertension occurs much more often than  secondary, it becomes  the main source of malignant hypertension.

Among the causes of  the dissection of aorta by far in patients of  the working age (30-50 years) determine Hzellya-Erdheim’s syndrome – the dissection of aorta due to its medianecrosis with no signs of atherosclerotic lesion of  the vessels wall. In recent years medianecrosis of aorta  became the  “leader” among   nosological forms of the dissection of aorta in young people,  having outstripped by the number of cases of Non-specific aortoarteritis and Marfan’s syndrome.

The article presents the fatal case of  Hzellya-Erdheim’s syndrome  with the total lesion of aorta. During the autopsy the main pathological changes were noted  from aorta, heart and kidneys.

The whole space of aorta (from the ascending department to the  abdominal department inclusively) is  with the  signs of separation of its wall; stratified space is filled with cherry clots. Histopathologically in the middle membrane of the ascending aorta are presented degenerative changes in elastic fibers  with insignificant  petrification, in the middle membrane  are observed single lymphocytes, the adventitia around vasa vasorum includes slight lymphocytic-macrophage infiltration;  on the boarders of  external and medium membrane  are observed sharply expressed degenerative changes in the walls  and the areas of the dissection, in thickness of which are erythrocytes. Slightly thickened intima by mucoid swelling with single lymphocytes.

Pathohistologically  large and medium-sized branches of the renal artery with sharply narrowed  gap due to the  thickened intima as a result of  the mucoid oedema in large branches  and hyperplasia of connective tissue in the middle ones. Arched  and interlobular arteries are also with  sharply narrowed gap via the connective tissue, muscular- fibrous hyperplasia. Internal elastic membrane with signs of multiplication. In some interlobular arteries is observed circular intimal hyperplasia by the  type  similar to “proliferative endarteritis”. In  the gap of some arterioles are observed  thrombotic masses. Glomeruli with small features of decreasing, with thickened glomerular basement membranes  (ischemic glomerulopathy). Huge part of glomeruli  is globally sclerosed. Tubules are atrophic, interstitial  sclerosis.

Thereby, the lesion of aorta by the presented Hzellya-Erdgeym's syndrome  manifested  by its dissection type I with intrafocal  longitudinal massive hemorrhage. The cause of death was massive hemopericardium that arose as a result of breaking the outer wall of the aorta. Trigger factor for aortic dissection was malignant hypertension. Cardiomegaly with severe left ventricular hypertrophy (3.0 cm), thrombosis of the major branches of the renal artery, interlobular arteries and arterioles, endothelial proliferation of interlobular arteries and arterioles, presence of intimal insudative changes of blood vessels of the kidneys indicate  about the  malignant hypertension. Hypertension has led to the development of  hypertensive nephropathy, which clinically manifested as end-stage CKD. Hypertension also led to the early development of atherosclerotic changes, also  including  these changes in  the aorta.

 

Key words: malignant hypertension, hypertensive nephropathy, Hzellya-Erdgeym's syndrome, medianecrosis of aorta, diseases of aorta.

 

Full text: PDF (Rus)

References
  1. Безродная Л.В. Современные подходы к лечению злокачественной гипертензии / Л.В. Безродная, Е.П. Свищенко // Medicus Amicus : Медицинская газета. – 2006. – №2. – С. 8 – 9.
  2. Вергун А.Р. Синдром Гзеля-Ердгейма: розшарування аорти внаслідок її медіанекрозу / А.Р.Вергун // Укр. мед. часопис. – 2001. – №2 (22). – С. 124 – 126.
  3. Зербино Д.Д. Расслаивающие аневризмы аорты: клинические маски, особенности дифференциальной диагностики / Д.Д. Зербино, Ю.И. Кузык // Клин. мед. – 2002. – №5. – С. 58 – 61.
  4. Зербіно Д. Д. Хвороби аорти: класифікація, диференційна діагностика /  Д. Д. Зербіно // Львівський медичний часопис. – 2008. – №1 – 2. – С. 55 – 63.
  5. Зербіно Д.Д. Розшаровуюча аневризма при медіанекрозі аорти (синдром Гзеля-Ердгейма): відкриття етіології / Д.Д. Зербіно, Ю.І. Кузик // Практична ангіологія. − 2012. − № 3 – 4 (52 – 53). − С. 33 – 35.
  6. Осовська Н.Ю. Особливості аневризми аорти у пацієнтів різного віку / Н.Ю. Осовська // Вісник Вінницького національного медичного університету. − 2013. − Т.17, №2. − С. 482 – 489.