Carotid Arterial Elastic Hyperplasia in aNewbornBY JOEL A. THOMPSON, M.D., MARGARET L. GRUNNET, M.D., AND

ROBERT E. ANDERSON, M.D.

Abstract:CarotidA rterialElasticHyperplasiain a Newborn

• Cerebrovascular insufficiency in infancy and childhood is well documented and has a diverseand often unknown etiology. Reported here is a polycythemic, microcephalic infant girl of 43weeks' gestation with bilateral cerebral infarction occurring in the perinatal period. Infarctionwas the result of bilateral carotid artery stenosis produced by massive reduplication of the inter-nal elastic lamina. Review of the literature failed to reveal any reports of similar arterial lesions.The angiographical picture was also unique.

The etiology of this unusual defect is not known, but we believe the problem was congenitalperhaps due to an intrauterine infectious process.

Additional Key Words cerebral infarction congenital vascular disease

• Numerous reports of cerebrovascular insufficiencyin infancy and childhood have been recorded, and thisproblem has been the subject of comprehensive evalua-tion by the Joint Committee for Stroke Facilities in itsrecent report.1 The etiology of congenitalcerebrovascular insufficiency is obscure in many cases,since mortality rates are relatively low and tissuespecimens, therefore, are usually unavailable.

The following is a report of bilateral cerebral in-farction in a polycythemic newborn infant withstenosis of the carotid arteries resulting from massivereduplication of the internal elastic lamina.

Case ReportThe patient was a 2,830 gm, 43-week gestation, infant girl(small for gestational age), who was the product of an un-complicated pregnancy and delivery. The 33-year-oldmother had two living children and four prior miscarriages.There was no maternal history of infection or ingestion ofteratogenic agents during the pregnancy.

The infant was in no acute distress at birth, althoughmeconium staining of the skin and fingernails was noted.Within five minutes of birth the patient developedgeneralized tonic-clonic seizures, which were controlled withintramuscular phenobarbital (5 mg per kilogram). A bulgingfontanelle was noted. The initial head circumference (OFC)was 32.2 cm, greater than two standard deviations below themean for an infant girl of 43 weeks' gestation. No focalneurological deficit was present.

At one day of age the fontanelle was still bulging, andintermittent, generalized seizure activity persisted. The

From the Departments of Neurology, Pathology, and Radiology,University of Utah College of Medicine, 50 North Medical Drive,Salt Lake City, Utah 84132.

This study was supported in part by National Institutes ofHealth Training Grants 2 T1-NB55O3 and 2 T1-NB53O9, and byNINDS Special Fellowship 5 Fl l-NS-2477 NSRA, as well as TheEleanor Roosevelt Cancer Foundation Research Institute.

patient was then admitted to the University of Utah MedicalCenter. Physical examination revealed a temperature of 98°F, respirations of 50 per minute, and a pulse of 120 perminute. The head transilluminated normally. The right pupilwas dilated and fixed to light, and there was a left sixth nervepalsy. The latter findings were interpreted as signs oftranstentorial uncal herniation. Hyperactive, symmetricaldeep tendon reflexes and intermittent decorticate posturingwere noted as well.

On admission the hematocrit was 75, polycythemic fora child of this age. Skull x-rays revealed diastasis of thesutures but no intracranial calcification. Serum calcium,phosphorus, glucose and electrolytes, urine amino acidscreen, serum immunoglobulin-M, chest x-ray, karyotype,and cultures of the blood, cerebrospinal fluid and urine werenormal.

Seizure activity was controlled with intramuscularphenobarbital. Increased intracranial pressure was treatedwith dexamethasone 2 mg I.V. every eight hours. Subduraland ventricular taps were performed in an attempt to deter-mine the cause of the increased intracranial pressure. Thesubdural taps were negative, and the ventricular systemcould not be entered. Eleven hours after admission (two daysof age) the patient had an episode of bradycardia and apneaand was promptly resuscitated. At that time bilateral fixedand dilated pupils were noted, as well as intermittentdecerebrate posturing. Mannitol (1.5 gm per kilogram) wasgiven without apparent improvement, and the patient wasprepared for cerebral angiography.

A right carotid angiogram (fig. 1) revealed a stenotic le-sion in the right external carotid artery a few millimetersbeyond the carotid bifurcation, and an overall decrease incaliber of the right internal carotid with multiple discreteand diffuse stenotic lesions. The right ophthalmic arteryfilled well, but virtually no dye extended beyond the rightcarotid siphon. An aortic arch study revealed multiplesimilar stenotic lesions in the left internal carotid artery,minimal flow to the anterior circulation, and moderatelydecreased flow in the posterior circulation. An intravenouspyelogram was normal.

The patient died at two and one-half days of age.

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Right carotid angiogram demonstrating multiple stenotic regions in the right internal and external carotidarteries (arrows).

Pathological ExaminationThe brain was that of a term infant. There were bilateralrecent infarcts in the distributions of both the middle andanterior cerebral arteries, as well as cerebral edema withevidence of transtentorial herniation. The area supplied bythe posterior circulation was relatively well preserved.Microscopically these infarcts contained pericellularvacuolization, spongy change and petechiae, with noevidence of an inflammatory reaction. The left cerebellarhemisphere contained a vascular malformation formed of acollection of large, thin-walled vessels, but was otherwisenormal. The brain stem was intact.

The walls of all four carotid arteries in the neck weremarkedly thickened. Stenosis was estimated at 70% to 80%.Microscopically there was dramatic reduplication of the in-ternal elastic lamina, with no increase in smooth muscle cellsor adventitia (fig. 2). No thrombi were seen. Carotid arteriesabove the siphon had normal walls grossly. Vessels withinthe brain and the rest of the body were normal on gross andmicroscopic examination.

DiscussionA number of pathological processes may lead to in-sufficiency of the cerebral circulation in infancy andchildhood. These include arteriosclerosis,2 arteritis ofinfectious or collagen vascular origin,* embolismassociated with congenital or rheumatic heart dis-ease,4 subacute bacterial endocarditis,' sickle celldisease,6 thrombosis associated with congenital heartdisease,' trauma3 or infection.7 The etiology in themajority of cases is unknown.

Harvey and Alvord,2 in a classification ofcerebral arterial lesions associated with "acutehemiplegia of childhood," cite several examples ofcerebral arterial dysplasia including intimalhyperplasia,8 medial hypoplasia or atrophy,9 andelastic hypoplasia or atrophy.10 Fibromusculardysplasia and Moyamoya disease also have beendescribed as causes of cerebral ischemia, but we have

393 Slrolt., Vol 6. Ju/y-Auguit 1975

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CAKOTID ARTIRIAL [LASTIC HYPIRPLASIA IN A NIWBORN

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Cross section of the right internal carotid artery, demonstrating dramatic reduplication of the internalelastic lamina, X 250.

found no reports of dramatic reduplication of the in-ternal elastic lamina of the carotid arteries resulting instenosis such as was seen in the case reported here.Search of the literature also reveals no similar-appearing arteriographical abnormalities of thecarotid arteries. The lesion is unlike that seen infibromuscular dysplasia.10"14

Our patient presents an intriguing combination ofcerebral arterial dysplasia, microcephaly andpolycythemia in a postmature infant, small for hergestational age. We speculate that a primary carotidartery dysplasia caused intrauterine cerebral ischemiaand resulted in microcephaly.

Both histological and radiographical evidencesupports the conclusion that carotid artery stenosis(70% to 80%) resulted in a significant decrease incerebral blood flow. Polycythemia may have con-tributed to cerebral ischemia. Stasis of blood due toincreased viscosity has been reported to produceseizure activity and focal neurological deficits such ashemiplegia in infants without arterial stenosis.16 Theobserved increase in intracranial pressure in this

patient was the result of cerebral edema accom-panying massive bilateral cerebral infarction.

The etiology of the complex constellation of find-ings in this patient is not known. She wasmicrocephalic and demonstrated intrauterine growthretardation, both possible stigmata of intrauterine in-fection. Her mother did have four prior miscarriages,suggesting an adverse intrauterine environment orgenetic defect. The patient's karyotype was that of anormal female, and the urine amino acid analysisshowed no evidence of homocystinuria as a cause ofthe arterial disorder. These results, however, wouldnot rule out a genetic defect:

References1. Gold AP, Challenor YB, Gilles FH, et al: IX. Strokes in

children. Parts 1 and 2. Stroke 4:833-894, 1007-1052, 19732. Harvey FH, Alvord EC Jr: Juvenile cerebral arteriosclerosis

and other arteriopathies of childhood. Six autopsied cases.Acta Neurol Scand 48:479-509, 1972

3. Banker BQ: Cerebral vascular disease of infancy andchildhood. I. Occlusive vascular disease. J Neuropath ExpNeurol 20:127-140, 1960

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4. Byers RK, McLean WT: Etiology and course of certainhemiplegias with aphasia in childhood. Pediatrics 29:376-383, 1962

5. Murphy F, Shillito J Jr: Avoidance of false angiographiclocalizotion of the site of internal carotid artery occlusion. JNeurosurg 16:24-31, 1959

6. Mymin D: Carotid thrombosis in childhood. Arch Dis Child35:515-518, 1960

7. Litchfield HR: Carotid artery thrombosis complicatingretropharyngeal abscess. Arch Pediat 55:36-41, 1936

8. Fisher CM: Early-life carotid artery occlusion associated withlate intracranial hemorrhage: Observations on the ischemicpathogenesis of mantle sclerosis. Lab Invest 8:680-700,1959

9. Wolman L: Cerebral dissecting aneurysms. Brain 82:276-291, 1959

10. Klassen AC, Sung HH, Stadlen EM: Histologic changes in thecerebral arteries with changing age. J Neuropath ExpNeurol 27:607-623, 1968

11. Hilal SK, Solomon GE, Gold AP, et al: Primary cerebralarterial occlusive disease in children. I. Acute acquiredhemiplegia. Radiology 99:71-86, 1971

12. Andersen PE: Fibromuscular hyperplasia in children. ActaRadiol (Diagn) 10:90-96, 1970

13. Taveras JM, Poser CM: Roentgenologic aspects of cerebralangiography in children. Amer J Roentgen 82:371-381,1959

14. Shillito J: Carotid arteritis: A cause of hemiplegia inchildhood. J Neurosurg 21:540-551, 1964

15. Gatti RA, et ah Neonatal polycythemia with transientcyanosis and cardiorespiratory abnormalities. J Pediat69:1063, 1966

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JOEL A. THOMPSON, MARGARET L. GRUNNET and ROBERT E. ANDERSONCarotid Arterial Elastic Hyperplasia in a Newborn

Print ISSN: 0039-2499. Online ISSN: 1524-4628 Copyright © 1975 American Heart Association, Inc. All rights reserved.

is published by the American Heart Association, 7272 Greenville Avenue, Dallas, TX 75231Stroke doi: 10.1161/01.STR.6.4.391

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