MEDICAL I N T E L L I G E N C E References 1. Taussig, 11. B.: Congenital Malformations of the Ileart. Specific Malfl~rmations. Ed. 2. Cambridge, i l a r v a r d University Press, 1960, Vol. 2. 2. Edwards, J. E.: Anomalies of derivatives of aortic arch system. Mcd. Clio. N. Am., 32:925-9t9, 19t8. 3. (;riswohl, II. E., Jr., and Young, M. D.: Double aortic arch: report of 2 cases and re~iew of literature. I'ediatrics, 4:751-768, 19-19. 4. Altschule, M. D.: Aneur)'sms o f t h e a r c h o f t h e a o r t a due to persistance of a portion of the ductus arteriosus in an adult. Am. I l e a r t J . , 14:113-115, 1937. 5. Graham, E. A.: Aneurysm of ductus arteriosus, with consideration of its importance to thoracic surgeon. Arch. Surg., 41:324-333, 19.10. 6. l'inniger, J. L., and Brown,J. B.: Adrenal lfileochromo c)toma. J. Path. Bart., 61:458--160, 19 t9. 7. Klcinerman, J., Yang, W. M., llackel, D. IL, and Kaurman, N.: Absence of the transverse aortic arch. Arch. l'ath., 65:490-498, 1958. 8. Barger, J. D., Creasman, R. W., and Edwards, J. E.: Bilateral ductus arteriosus associated ",dth interrul~tlon of the aortic arch. Ant. J. Clin. Path., 24: 4.11--14 t, 195-t. 9. Barger, J. I)., Bregman, E. II., arid Edwards, J. E.: Bilateral ducttlS arteriosus with right aortic arch and right-sided descending fiorta. Am. J. Roentgenol., 76: 758-766, 1956. 10. Gould, S. E.: l'ath.logy of the Heart. Ed. 2. Springfield. Illinois, Charles C Thomas, 1960.

ARTERIAL DYSPLASIA WITH RUPTURED BASILAR ARTERY ANEURYSM: REPORT OF A CASE* CllARLES S. H I R S C l l , M.DA" UROS ROESS.MANN, M.I).~.

AND

Abstract

An 11 year old girl died um'xpectedly as a result of spontam'ous subarachnohl hemmthage originati,g from a ruptured giant fusiJbrm aneur~'sm of the basilar arteo'. The aneuo'sm had de~;eloped as a hmg stamling complication ~f arterial fibromuscular dyspla.~ia. This di.wussion describes the light aml electtw~ microscopic findings in the aneuo'sm aml the histologie examination ~f abmmnalities in the mmcular arteries ~f the viscera. Spontaneous r u p t u r e of a giant fusitbrm aneurysln of the basilar artery caused fatal subarachnoid hemorrhage in an I1 year oki girl. She had muhifocal fibromuscular dysplasia of arteries, and the basilar arter)' aneurysm showed the same disease in a far advanced state. *This wo,k was SUl)l)orted iq part b)' U.S. Public I leahh grant NS 05400 from the National Institute of Neurological I)iseascs and Stroke. tAssistant I'rofessor of Forensic I atholo,,y, Case Western P,eserve University, Associate Pathologist and I)cpttty Coroncr, Cu)ahoga Cottnty Coroner's Office, Clevel:md, Ohio. CAsst,ciate l'rofessor ,,f Neuropathology, Case Western Reserve University, Cleveland, Ohio.

Tiffs report documents the unusual pathologic findings of the autopsy and relates these alterations to the pathogenesis of arterial fibromuscular dysplasia. On the m o r n i n g of her death, fin ostensibly healthy, intellectually average, physically active 11 )ear old girl went to school as usual. She behaved normally and made no complaint of illness. After attendance was taken, the pupils went to their first class of the day, which was physical education. She participated in calisthenics for 15 minutes and then comphfined to the teacher that her neck "hurt." She was dismissed from the gymnasium a n d walked u n a t t e n d e d to the school office. Shortly after arrival at the office, she vomited and again complained of pain in her neck. She remained conscious and spokc coherentl)" to a secretary and to a custodian, who drove her home. She walked unassisted from the car on arrival at her house. At home she complained of severe head and neck pain a n d nausea; approximately one hour later she suddenl)" became unresponsive. A police ambulance conveyed her to a nearby hospital. When brought to the emergency room, she was conmtose and apneic; the blood pressure was unobtainable and the pulse was palpable at 80 to 90 beats pcr ininute. A lumbar puncture disclosed bloody cerebrospinal fluid. Despite mechanical ventilation and supportive treatment, she died 90 minutes after arrival at the hospital. T h e decedent's family enfigrated to the United States from hal)" when she was four. I ler mother had pre-eclampsia, but remembers no other disease or complication of pregnancy, labor, or delivery. (The mother had had rubella d u r i n g childhood.) T h e clfild's birth weight was 3 kg. Prior to the onset of the fatal snbarachnoid hemorrhage, the decedent never had been seriously ill. T h e family internist states that her parents and an older sister are in excellent heahh. T h e r e is no family history of vascular or other disease even remotely sinfilar to the decedent's.

AUTOPSY FINDINGS Tile child weighed 44 kg. and measured 150 cm. in length. T h e external examination was unrenmrkable; her skin had a normal texture. Tile viscera were situated normally and showed no congenital deformity or malformation. T h e body cavities were free of hcmorrlmge or cfft, sion. T h e hmgs had a combined weight of 1180 gin. and were unifornfi)' congested and edematous. T h e o t h e r -

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I I U M A N I ' A T I I O I . O ( , Y - - V O L U M E 6, N U M B E R 6

November 1975

Figure I. Arteries dissected from the base of the brain. A ['usifiJrm anet,r)sm of the basilar artery begins at the junction of the normal caliber vertebral arteries. The adventitia over the left (viewer's right) side of the aneur)'sul is h~zmol"rhhgic. T h e o l h e r arteries are no,real.

750

thoracic and a b d o m i n a l o r g a n s s h o w e d no gross abnorlnality. T h e r e was no injury o f the scalp, skull, o r neck. T h e brain weighed I,t 10 gln.; its base was c o v e r e d by fresh s u b a r a c h n o i d h e m o r r h a g e , which e x t c n d c d t h r o u g h the sylvian lissurcs a n d r e a c h e d the lateral aspects o f both cerebral convexities. T h e h e m o r r h a g e origin,tied f i o m a r u p t u r e d a n e u r y s m o f the basilar artery.

T h e basilar artery, which was shifted to tile left o f tile midline, showed inarkcd fusifolun dilatation b e g i n n i n g at the p o i n t w h e r e it o r i g i n a t c d f r o m n o r m a l caliber vertebral arteries. T h e ;.tncurysm was 4.2 cm. l o n g and i.5 cm. in m a x i m a l width; c e p h a l a d to the dilated s e g m e n t , t h e r e was a 1.4 cm. l e n g t h o f n o r m a l basilar artery that was (I.3 cm. wide and b i f u r c a t e d to f o r m the p o s t e r i o r cereln'al a r -

MEDICAL I N T E I . I J ( ; E N C E from the basilar artery, microscopic examination of representative sections from each vessel of the circle of Willis slmwed no pathologic change. ,Microscopic examination of multiple sections from the brain disclosed normal intracerebral blood vessels a n d no cerebral parenchymal abnormality.

teries in the usual fashion (Fig. 1). T h e aneurysmal seglnent of the basilar artery had a patent lumen. Its grayish white wall was firm and 0.2 to 0.3 cm, thick; there was no dissecting intramural henmtoma present. T h e left side and cerebral surface of lhe aneurysm showed a 2.3 cm. area of hemorrlmge in the adventitia. A transmural r u l x u r e was identified in microscopic sections from the area of origin of the left anterior inferior cerebcllar amer)'. T h e bed of the aneurysm was smooth and concave; it produced conslfiCuous deformity of the left side of the base of the pons and ntedulla but no demyelination or other abnormality within their parcnchyma (Fig. 2).* All the arteries forming the circle of Willis and their macroscopic extensions on the surface of the brain were unren~arkable. Aside

L I G H T MICROSCOI'IC CHANGES IN T H E BASII,AR ARTERY "Fhe intima of the basilar artery was markedly tlfickened by loose cellular connective tissue, which had a "myxomatous" appearance in many areas. Nuclei within these latter loci varied from oval to stellate and were separated widely by g r o u n d substance, wlfich stained lightly with eosill, mctachromatically with toluidine blue, and positively with PAS. In a few areas, intimal connective tissue had matured into dense, well organized collagen. T h e intimal fibrosis was associated with degeneration of the internal elastic lamina

*Absence of deleterious pressure effects in these areas is interpreted as bcing'indicativc of a slowly progressive, chronic process.

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