Child's Brain 5: 166-173 (1979) The Results of Operating upon Cerebral Aneurysms and Angiomas in Children and Adolescents II. Cerebral Angiomas A.Loren Amacher, Charles G. Drake and Lada Hovind Departments of Paediatrics and Clinical Neurological Sciences, University of Western Ontario, London, Ont. Key Words. Angiomas, cerebral • Children • Surgical treatment • Results Abstract. Symptomatic angiomas o f the brain, brain stem or cerebellum are un­ common in all age groups. They usually present by subarachnoid and intraparenchymatous hemorrhage, but they may act as expanding mass lesions, as causes of ingavescent neurological deficit (?‘steal phenomenon'), as epilepticogenic foci, or as a source of relentless headache. We have operated upon 20 patients, 18 years of age or less, with symptomatic angiomas. There has been no surgical mortality, and no patient has been made worse by operation. Introduction Symptomatic angiomas are uncommon at any age. In the young, they are an important differential diagnosis in subarachnoid hemorrhage or intraparenchymal hematoma. They may present, less commonly, as mass lesions, epilepticogenic foci, causes of transient ischaemic attacks or progressive neurological deficit, or as a cause of severe migraine-like headache [1]. It is the purpose of this paper to discuss the results of operating upon angiomas in young patients. Clinical Material Downloaded by: Stockholm University Library 130.237.165.40 - 11/19/2018 4:21:42 PM All young patients operated upon in this center, with an angiographic and patho­ logical diagnosis o f intracranial angiomas are included. The clinical data are summarized in table I. Table I. Clinical data, angiomas in the young Sex Age Presentation Site Operation Postoperative angiogram Result Comment 1 N.S. F 9 It. cerebellum excision not done excellent see text 2 K.J. F 14 it. parietal excision obliterated excellent small ICH 3 R.D. 4 C.K. M M 3.5 0.75 headache, IICP (mass lesion) SAH numbness rt. leg. incont. seizure, SAH seizures excellent fair small ICH retarded, seizures reduced M M 1 13 obliterated obliterated excellent excellent 7 J.P. M 17 Skull erosion SAH ( x 5) stupor, postfossa signs SAFI 4 years prev. rt. brain stem signs excision hemispherectomy (3 opns) excision excision not done obliterated 5 T.D. 6 N.S. rt. rolandic It. cerebral, diffuse rt. parietal rt. CPA rt. brain stem, surface excision obliterated good 8 C.P. 9 B. 13. 10 M.G. II J.F. M F F M 18 5 17 14 SAH ( x 2) SAH + ICH SAH + ICH SAH It. ICH rt. trigone It. frontal rt. occipital rt. parietal 12 J.C. F II SAH + ICH rt. parietal excision excision excision 1. partial excision 2. excision excision obliterated excellent obliterated excellent obliterated excellent residual, larger good excellent obliterated small residual excellent 13 G.K. 14 A.B. 15 L.G. 16 C.S. 17 R.E. 18 C.M. 19 J.C. M F F F M F M 3 9 18 18 17 18 6 obliterated residual obliterated obliterated not done obliterated no change good excellent excellent excellent no change excellent large ICH no change see text M 17 It. parietal corpus, call. rt. occipital quad, plate It. rolandic rt. frontal rt. cerebellum brain stem rt. cerebellum excision excision excision excision exploration excision clip feeders 20 J.F. seizure SAH SAH SAH parinauds TIA 'steal' SAH + ICH SAH brain stem signs SAH + ICH excision obliterated excellent clot in angle slow resolution of rt. cranial nerve signs and It. hemiparesis ventricular clot frontal ICH large ICH see text The Results of Operating upon Cerebral Aneurysms Case cerebellar ICH SAH = Subarachnoid hemorrhage: ICH = intracerebral hematoma; IICP = increased intracranial pressure; TIA = transient ischemicattack. Note: Since submission, a further 12 cases have been operated upon, without mortality or increased morbidity, ages 5-16 years. Downloaded by: Stockholm University Library 130.237.165.40 - 11/19/2018 4:21:42 PM 10 years, no recurrence slow, no fits see text inferior surface see text Amaeher/Drake/Hovind 168 (1) Symptomatology: Of the 20 patients. 15 had experienced intracranial bleeding. Of these, 8 had preoperative clinical indications o f intraparenchymal clots, confirmed at operation, while a further 3 had small, asymptomatic clots. In I case (7. J.P.), the SAH had occurred 4 years previously, whereas ongoing symptoms were due to brain stem com­ pression. In 2 cases, seizures were the principal clinical manifestation. In I case each, headache of increased intracranial pressure, skull erosion, and transient ischaemic attacks prompted operation. (2) Sex and age: There was no sex predcliction. 4 patients presented at 0-4 years. 4 at 5-9 years, 4 at 10-14 years, and 8 at 15-18 years. 3) Sites: 14/20 lesions were supratentorial. Five were parietal, two were frontal, two occipital, two rolandic, one intraventricular, one in the corpus callosum, and one was pan-hemispheric. Three lesions were in the cerebellum, two on the brain stem, and one in the cerebello­ pontine angle. (4) Case reports: Certain interesting and important features of angiomas and their surgical treatment can best be illustrated by case summaries. Case / A 9-year-old girl was admitted because of increasingly severe headache for several weeks, accompanied by papilledema and split coronal sutures. Preoperative angiography and computerized tomography suggested a left cerebellar mass, showed a ‘cut off' vessel, and hydrocephalus (fig. 1). At a left suboccipital craniotomy, a thrombosed venous angioma was encountered and excised. Postoperatively, her headache disappeared, and the hydrocephalus subsided. Fig. 1. Vertebral angiogram, lateral (left) and Towne's views. Note the 'cut-off' artery (arrows). Downloaded by: Stockholm University Library 130.237.165.40 - 11/19/2018 4:21:42 PM Comment. It is well to remember (hat not all angiomas are diagnosed preoperatively, and that one should be prepared for anything until the pathology is established. The Results of Operating upon Cerebral Aneurysms 169 Fig. 2. Carotid angiograms, lateral views. Preoperative view (top) o f angioma: note the large anterior cerebral feeder. Postoperative (bottom) view: note normal size of anterior cerebral artery, enlarged middle cerebral feeder, and previously unseen angioma component (arrow). Downloaded by: Stockholm University Library 130.237.165.40 - 11/19/2018 4:21:42 PM Case 11 This 14-year-old boy was struck down by a sudden subarachnoid and right parietal hemorrhage that produced brief coma and a left hemiparesis. He recovered quickly. Angiography showed an angioma o f the right parietal lobe (fig. 2). The lesion was removed, but postoperative angiography revealed residual angioma, enlargement of the previously normal middle cerebral artery, and a previously unseen deeper component to the lesion (fig. 2). 6 months later, an angiogram revealed even further growth of the residual angioma. This time, complete excision was accomplished, and proven by postoperative angiography. All feeders returned to normal calibre. Amachcr/Drake/Hovind 170 Comment. This case illustrates: (I) incompletely excised angiomas may enlarge again: (2) when the lesion is incompletely removed, feeding arteries may persist in their enlarged state; (3) the full extent of an angioma may not be visualized, even by complete angio­ graphy; (4) postoperative angiography is important ir. the assessment o f results of surgery for angioma, and (5) once an angioma is obliterated, feeding arteries return to normal size. Case 14 A.B., a 9-year-old girl, was awakened from sleep by a severe apical headache, accompanied by vomiting. Computerized tomography showed clotted blood in the pineal region, and in the left lateral ventricle. Angiography demonstrated a fine-vessel angioma of the corpus callosum, posteriorly (fig. 3). The lesion was approached between the hemi­ spheres. The part above and within the corpus callosum was removed, but a small portion extending posteriorly, under the corpus callosum splenium and onto the left fornix, was left in place. Residual angioma was confirmed by angiography. Comment. Previous experience would suggest that this residual angioma may regrow and rebleed [l|. Repeat angiography is planned at intervals. If enlargement or further hemorrhage occur, total excision may be attempted, in spite of a rather hazardous location. Not all residuals of small volume cause further trouble. In case 12, repeat angio­ graphy has indicated no further growth, and she remains very well. Fig. 3. Lateral vertebral (left) and lateral carotid (right) angiograms, showing fine vessel angioma (arrows) feeding from both circulations, and draining through the galenic system (open arrow). Downloaded by: Stockholm University Library 130.237.165.40 - 11/19/2018 4:21:42 PM Case 16 An 18-year-old girl, C.S., developed a sudden headache, followed by a left hemiparesis, a Parinaud’s Syndrome, and mild pupillary asymmetry. An angioma was seen at angiography, fed largely from the superior cerebellar arteries and draining to the vein of Galen (fig. 4). There was mild hydrocephalus. The lesion appeared to be in the region of 171 Fig. 4. Vertebral angiogram, lateral (left) and Towne’s (right) views of angioma in quadrigeminal cistern and draining into Galenic system. Fig. 5. Vertebral angiogram, lateral. Huge angioma occupying right cerebellum. Downloaded by: Stockholm University Library 130.237.165.40 - 11/19/2018 4:21:42 PM The Results o f Operating upon Cerebral Aneurysms Amachcr/Drake/Hovind 172 the quadrigeminal cistern and plate. Through a supraoerebellar approach, the angioma was excised from the quadrigeminal plate. The patient returned to a normal neurological state. Comment. Angiomas may be removed from difficult areas, by microsurgical technique, with safety. In this case, the dilatation of the vein of Galen w'as secondary to a nearby angioma, and does not qualify as a vein of Galen aneurysm [2], Fig. 6. Vertebral angiogram, lateral, postoperative. Note the occlusive clips, and the unchanged size of the angioma. Downloaded by: Stockholm University Library 130.237.165.40 - 11/19/2018 4:21:42 PM Case 19 This 6-year-old boy was rendered unconscious and apneic by a subarachnoid hemor­ rhage. Coma persisted for 4 days. He slowly improved, but was left with a right hemiparesis, bilateral arm intention tremor, and right ataxia. A bruit could be heard over right frontal and occipital areas. The angiogram showed a huge angioma, with large feeders from su­ perior and posterior inferior cerebellar arteries (fig. 5). occupying the right cerebellum. A combined right suboccipital and subtemporal approach was used. Excision was deemed impossible. Clips were placed on the right superior and posterior inferior cerebellar arteries, close to the lesion. The subsequent angiogram showed the angioma to be unchanged, with copious alternate blood supply (fig. 6). The boy has shown slow improvement in neuro­ logical function. The Results of Operating upon Cerebral Aneurysms 173 Comment. This and previous experiences [I] indicate that the simple clipping of feeding vessels to angiomas is ineffectual as treatment. Unheralded and unseen feeders simply open up. to maintain the arterial inflow. Results The results are summarized in table I. An excellent result indicates return to presymptomatic status. A good result indicates persistence of mild neurological or intellectual deficit, present preoperatively. The points to be emphasized are: (1) there was no surgical mortality in 23 procedures done on 20 patients; (2) no patient was made worse by operation: (3) postoperative angiography was done 18 times on 17 patients, confirming surgical obliteration in 14 procedures, residual angioma in 4, and (4) to date, recurrent hemorrhage has not occurred from residual angiomas; but. at least 2 patients had suffered at least one repeat hemorrhage prior to operation: thus, we do not consider patients with residual angiomas safe from future catastrophe [I], Conclusions Symptomatic angiomas are unusual in young people. They can be surgically obliterated with a very low mortality. Postoperative angiography is important in the assessment of surgical therapy, and, in the follow-up of patients with residual angioma. References 2 Amacher, A.L.; Allcock, J.M., and Drake, C.G.: Cerebral angiomas. The sequelae of surgical treatment. J. Neurosurg. 37: 571-575 (1972). Amacher, A.L. and Shillito, J., jr.: The syndromes and surgery of aneurysms of the great vein o f Galen. J. Neurosurg. 39: 89-98 (1973). A. Loren Amacher, Victoria Hospital. 375 South Street, London. Ont. N6A 4G5 (Canada) Downloaded by: Stockholm University Library 130.237.165.40 - 11/19/2018 4:21:42 PM 1