ACTA NEUROCHIRURGICA Acta Neurochirurgica 57, 257--267 (1981) 9 by Springer-Ver!ag1981 Neurosurgical Clinic of the University of Pavia and Centre for the Study and Medical and Surgical Treatment of Cerebrovascular Diseases, Pavia 1; Paediatric Clinic of the University of Pavia2 and Department of Morbid Anatomy and Histopathotogy, State University of Milan 3, Italy Saccular A n e u r y s m o f I n f a n c y a n d E a r l y C h i l d h o o d Report of a Case By P. Paoletti l, S. Pezzotta 1, M. Scotta ~, and L. Mosca 8 With 4 Figures Summary A case of intracranial saccular aneurysm with intracerebral haematoma occurring in early childhood and presenting with sudden loss of consciousness and right hemiparesis is reported. The aneurysm was located in the opercular portion of the left middle cerebral artery. Surgery, besides removing the intracerebral haematoma, involved clipping and complete removal of the aneurysmal sac. The child made an uneventful recovery, and he is completely safe after 40 months. Microscopic examination of the lesion shows disruption of the normal sequence of the original layers, with widespread inflammatory cells. Keywords: Cerebral aneurysm; infancy; congenital anomaly. Introduction Intracranial arterial aneurysms are rare in childhood. They accounted for 17 of the 1,125 intracranial aneurysms reported by McDonald and Korb 29. Laitinen 2~ observed that 1.3~ of the aneurysms studied were in paediatric patients. Matson 2s reported a series of 15 cases varying from 1 to 17 years in age, while Cambria et al. ~ described a case of congenital saccular aneurysm in a fouryear-old child. Locksley 27 had one case in a group of 2,627 patients, while Breschi et al. s, Thompson and Pribram ~7, Arai et al. 4, Shucart and Wolpert ~!, Becker et al. 5, and Milhorat 3o added one case each. Grode et al. 17 described two cases of middle cerebral artery aneurysms in young babies, while Bolander et al. 6 described the death of an l 1-month-old patient due to the rupture of an anterior com17, 0001-6268/81/0057/0257/8 02.20 258 P. Paoletti et al.: Saccular Aneurysm of Infancy and Early Childhood municating artery aneurysm. Finally Gerosa et al. ~o in 1980 reported 15 cases of cerebral artery aneurysms in children between 8 and 15 years of age. Case Report A 34-month-old boy was admitted to the Paediatric Clinic of the University of Pavia on 6 October 1977. lie presented with a deficit of the left third cranial nerve and right hemiparesis. He had been drowsy for two days Fig. 1. CT scan (6 October 1977) showing a left insulo-parietal haematoma prior to admission. An examination of the fundus oculi revealed that the left disc had blurred edges. CT scan was done immediately, and showed a left insuloparietal haematoma (Fig. 1). A lumbar puncture showed clear CSF with no cells and normal protein and sugar values. Left carotid angiography showed an aneurysm of the opercular portion of the left middle cerebral artery, with a haematoma in the left insular region (Fig. 2). The boy was treated with mannitol, dexamethasone, and tranexamic acid intravenously, and fourteen days later a left temporo-parietal craniotomy was performed. A partially coagulated 40ml haematoma was removed. The aneurysmal sac, which appeared very large and partially thrombosed, was clipped at the neck and removed. The boy was awake after 24 hours of intensive care, but a monoparesis of the right upper limb remained for 15 days. A C T scan was performed 45 days after surgery, and showed a slight ventricular dilatation with no periventricular hypodensity (Fig. 3). Other CT scans were done 4 and 12 months later, and they showed the same vcntricular dilatation. The clinical situation is continuously improving, and the patient's neurological and psychic conditions 40 months after the operation are excellent. Fig. 2. Left carotid angiogram (lateral view) showing an aneurysm in the opercular portion of the left middle cerebral artery Fig. 3. CT scan (5 December 1977) showing a slight ventricle dilatation with no periventricular hypodensity 260 P. Paoletti et al.: Figs. 4 a, b. The surface of the aneurysm, considered in various sections, shows different architecture and pathology: disruption of the normal sequence of the original layers, widespread inflammatory cells, badly organized thrombosis, recent haemorrhages Saccular Aneurysm of Infancy and Early Childhood 261 Microscopic Examination of the Aneurysm The architecture of the original vessel wall appears definitely disturbed. The muscular layer is irregularly thinned and the endothelial covering is frequently disrupted. An incompletely organized thrombus adheres firmly to the inner surface of the aneurysm, while leucocyte nests are widespread among the different structures. On the outer surface of the "berry" a recent blood effusion is detectable. The specific stains for elastic tissue clearly show a complete disappearance of both the internal and external elastic layers with a few randomly distributed residual fibres along the varying thi&ness of the aneurysrnal wall (Figs. 4 a and b). Discussion There are only 25 reported cases of saccular intracranial aneurysm diagnosed during life in children less than 3 years old (Table 1). The sex was given in 24 out of the 25 cases, and only 7 were female. Clinical presentation was subarachnoid haemorrhage (SAH) due to aneurysm rupture in only 14 out of 25 patients. In three cases it was due to head injury, in three other cases to hydrocephalus, in one case to macrocephalus, in three cases to seizures, and in six cases to motor deficits. The aneurysm in the 24 cases fully described was located 13 times at the level of the middle cerebral artery (MCA) or its branches, 7 times at the level of the internal carotid artery (ICA), anterior carotid artery (ACA), or anterior communicating artery (ACoA) and their branches, twice at the level of the posterior cerebral artery (PCA), and only twice in the posterior fossa (basilar artery and posterior inferior cerebellar artery). Surgery was performed 21 times in the 25 cases described. Results were excellent or good in 16 cases, fair in 3 cases, and there were two deaths (0.5%). The youngest patient to be operated on was a four-week-old girl reported by Jones and Sheaburn in 1961 s0. The largest intracranial aneurysm was reported by Ventureyra et aI. in 1980 40. It was a giant globoid aneurysm, 8 X 9 cm in size, arising from an anomalous posterior cerebral artery. Histology was examined in only seven cases. Nineteen other cases were discovered at autopsy in children less than three years old (Table 2). The sex was given in 16 cases out of 19, and 7 were female. The youngest infant was 64 hours old, and a 42 mm embryo with a basilar aneurysm was also reported. The clinical presentation was SAH in five cases, hydrocephalus with posterior fossa or third ventricle mass in two cases, hydrocephalus alone in 1 case, seizures in two cases, head injury in two cases, and motor deficits in two cases. The aneurysm location was infratentorial in 8 cases out of 19, at the level of PCA in 3 cases, at the level of ICA, ACA, ACoA and their branches in 5 cases, and at the level of M C A (peripheral branch) P. call (L) M C A (L) M C A (R) M C A (L) ACoA DNP BA Amacher and Drake 2 Amacher and Drake 2 Arai et al. 8 Arai et al. 4 Becket et al. 5 Bolander et al. 6 Breschi et al. 8 Ferry et aI, t3 Garcia-Chavez and Moossy 15 M C A (L) Grode et al. 17 M C A (L) Grode et al. 1~ M C A (g) Location of aneurysm * Authors M F F M M M F M M M M Sex 5 months 7 weeks 3 months 3 months 4 months 24 months 26 months 11 months 12 months 6 months 20 months Age SAH, hydrocephalus, L. hemiparesis, ophthalmoplegia SAH SAH SAH SAH SAH, L. hemiparesis right hemiparesis SAH head injury head injury, coma seizures Clinical presentation Table I. E a r l y C h i l d h o o d A r t e r i a l A n e u r y s m s D i a g n o s e d D u r i n g L i f e R. hemiparesis excellent excellent good fair no surgery, died of respiratory arrest good excellent no surgery died 2 days after SAH hydrocephalus, mild deficits no surgery, died at 5 months Surgical outcome yes --- yes yes --- -- -- Histology bO .~ g~ o Po M C A (R) M C A (R) A C h A (R) PCA P I C A (L) M C A (R) P. call (R) A C A (L) M C A (L) I C A (L) I C A (R) M C A (R) P C A (L) Kimbell et al. 21 Lapras 2~ Lemmen and Schneider ~5 Matson 28 Matson 2s Milhorat Th. H. 30 Morelli e t al. 31 Paoletti et aI. (this paper) Shucart and Wolpert 34 Thompson and P r i b r a m 37 Vapalathi e t al. 89 Ventureyra e t al. ao DNP F F M M F M M M M M M M F 5 months 3 months 6 months 34 months 6 months 1 month 4 months 16 months 12 months 23 months 11 months 8 months 16 months 4 weeks DNP good excellent died excellent excellent excellent died minimal deficits excellent SAH, R. hemiparesis excellent diabetes insipidis excellent ophthahnoplegia, no surgery L. hemiparesis SAH, seizures excellent macrocephalus, anemia excellent SAH, R. hemiparesis SAH, sudden coma DNP SAH headache, vomiting hydrocephalus head injury, S A H seizures, S A H yes -- yes --- -- -- -- --- yes -- yes * K e y to table: A C A = anterior cerebral artery, A C h A = anterior choroidal artery, A C o A = anterior communicating artery, B A = basilar artery, C o f W = circle o f Willis, D N P = data not presented, I C A = internal carotid artery, L = left, M C A = middle cerebral artery, P C A = posterior cerebral artery, P I C A = posterior inferior cerebellar artery, P. call = pericallosal artery, R = right, V - vertebral M C A (L) H a m m i l l and Carter 18 Jones and Sheaburn z0 ga tO O O t'x5 g~ .q o .q ), p~ BA BA ICA (?) V (R) ACoA BA (bifurcation) ACA (L) P I C A (L) BA (bifurcation) P I C A (?) PCA (L) MCA (?) P C A (?) C of W MCA (L) MCA (?) ICA (?) PCA (?) BA Alpers i Bremer 7 Devadiga et aI. i0 Dial and Maurer H Fearnsides 12 Forster and A1pers 14 Garcia-Chaves and Moossy 1.~ Jane 29 Lee and Kandal124 Lemmen and Schneider 25 Lipper et al. 26 McDonald and Korb 2~ Newcomb and Munns 32 Newcomb and Munns 32 Pool and Potts 33 Talbot as Thompson and Pribram 37 Thompson et al. 36 Valle and Cavalcanti 33 * Key to table: See Table 1. Location of aneurysm * Authors F DNP M M DNP M F M F M F M M M DNP M F F F Sex Clinical presentation 2 months DNP embryo (42 mm) D N P 14 months seizures, hemiparesis, ophthalmoplegia 33 months head injury, SAH 19 months DNP 3 months seizures, sudden coma 2 months SAH 12 months hydrocephalus, posterior fossa mass 13 days DNP 8 months hydrocephalus, posterior, 3rd ventricle mass 19 days SAH 18 months DNP 64 hours SAH 23 days head injury, SAH 11 months hydrocephalus 18 months DNP 9 months ophthalmoplegia, quadriplegia 12 months DNP 2 months DNP Age Table 2. Early Childhood Arterial Aneurysrns Discovered at Autopsy yes yes ---- yes yes yes yes -yes -yes -- Histology t~ ~ o ila 4~ Saccular Aneurysm of Infancy and Early Childhood 265 MCA and its branches in 3 other cases. Histological examination was performed in 8 cases out of 19. In conclusion, it appears that the incidence of saccular intracranial aneurysm in infancy is very low, because only 44 cases have been described in 65 years (from the report of Fearnsides in 1916 to date). SAH was present in only 19 cases out of the 35 fully described (54.30/0, while an intracerebral haematoma was present in 12 cases (34.3~ Aneurysm in this age group occurs in unusual locations compared to those in late childhood and in adults. Peripheral branches of the MCA and ACA and the bifurcation of the ICA are frequent sites, but infratentoriai aneurysms were also present 10 times in the 43 cases described (23.3~ Acknowledgements We are grateful to Mrs. N. Gnes for preparing the manuscript. References 1. Alpers, B. J., Aneurysms of the circle of Willis: Morphological and clinical observations. In: Intracranial aneurysms and subarachnoid hemorrhage, pp. 5--24 (Fields, W. S., et aI., eds.). Springfield, Ii1.: Ch. C Thomas. 1965. 2. Amacher, A. L., Drake, C. 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