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Show Journal of Clinical Neuro- ophthalmology 9( 1): 33- 36, 1989. © 1989 Raven Press, Ltd., New York Acute Oculomotor Nerve Palsy in Childhood Is Arteriography Necessary? Eugene B. Gabianelli, B. A., Terence G. Klingele, M. D., and Ronald M. Burde, M. D. In the past, angiography was performed in all patients as part of the initial workup for isolated oculomotor paralysis, except patients older than 40 years with pupillary sparing. The pupil- sparing group was not subjected to angiography because of a low probability of cerebral aneurysm. It is believed that the case reported here constitutes the lower age limit ( 14 years) for documented, isolated oculomotor paralysis due to aneurysm. It is recommended that an angiogram not be a necessary part of the workup of patients 10 years old or younger. Key Words: Angiography- Oculomotor nerve paralysis- Children. From the Departments of Ophthalmology ( E. B. G., T. G. K., RMB) and Neurology and Neurological Surgery ( R. M. B.), Washington University School of Medicine, St. Louis, Missouri. Address correspondence and reprint requests to Ronald M. Burde, M. D., Department of Ophthalmology, Box 8096, 660 South Euclid Avenue, St. Louis, MO 63110, U. S. A. 33 In the patient with headache and acute oculomotor nerve palsy, aneurysm of the posterior communicating artery is a major consideration. For this reason, cerebral arteriography is often performed on such patients, even if computed tomographic ( CT) and magnetic resonance imaging ( MRI) scans are normal, to exclude the possibility of an aneurysm. If the patient is over 40 years old and the pupil is spared, the cause is likely to be small vessel disease of the oculomotor nerve trunk, and arteriography is not necessary. In patients over age 40 with pupillary involvement and in all patients under the age of 40, angiography or MRI is usually performed to rule out aneurysm ( Table 1) ( 1). In children, however, cerebral aneurysms in general and posterior communicating artery aneurysms in particular are rare. Several authors have questioned the need for arteriography if high resolution CT is normal ( 2- 4). The risks of arteriography are low, though not absent, and in young children, especially if MRI is available, arteriography may not be indicated for initial workup of oculomotor palsy. We believe the following case is unique and may help establish the lower age limit for risk of oculomotor nerve palsy caused by aneurysm of the posterior communicating artery. CASE REPORT A 14- year- old girl came to the neuro- ophthalmology service complaining of headache and a drooping left eyelid that she had had for 3 months. Her gestation, birth, and childhood development had been completely normal. Her problems had begun about 3 months before admission to the hospital in January, 1978, at 14V2 years of age, with the development of severe, constant, left frontal headache which was not alleviated by aspirin. About 6 weeks before her admission, the left eye- 34 E. B. GABIANELLI ET AL. DISCUSSION TABLE 1. Initial management of acquired isolated oculomotor nerve palsy without signs of subarachnoid hemorrhage Although aneurysm is responsible for - 20% of isolated oculomotor paralysis in adults, and is thus a major diagnostic concern, aneurysm is a much less common cause ( 3%) of oculomotor paralysis in children ( Table 2). In considering the need for angiogram or magnetic resonance imaging ( MRl) in lid began to droop. Ptosis was progressive until the left eye was completely shut 1 month before admission. Two weeks prior to admission, diplopia was noticed when the left eyelid was manually raised. The patient believed that the vision was normal from the left eye when the lid was raised. No other symptoms were noticed by the patient, and she denied having a stiff neck. There was no history of diabetes, trauma, infection, migraine, or malignancy. The family history was negative for diabetes, visual problems, and migraine. Examination on April 10, 1978, revealed a visual acuity of 20/ 20 in both eyes. A complete left ptosis was observed. The left pupil was dilated and fixed at 7 mm in diameter, and the left eye was deviated laterally and inferiorly in primary gaze. Ductions were full in the right eye, but elevation, depression, and adduction were severely limited in the left eye. The result of neurologic examination was otherwise normal, without signs of subarachnoid hemorrhage. A second- generation computed tomographic ( CT) scan, performed on April 12, 1978, was negative. On April 13 a transfemoral, three- vessel cerebral angiogram showed a lobulated aneurysm at the junction of the left internal carotid and posterior communicating arteries. The patient underwent a metal clip ligation of the aneurysm through a left frontotemporal craniotomy on April 14, 1978. Following surgery, she awakened and was alert. The oculomotor paralysis showed slight improvement by April 21, and the patient was discharged 1 week after surgery, receiving diphenylhydantoin, 100 mg three times daily. TABLE 2. Causes of isolated oculomotor palsy From references 1, 9, 15, and 16. cases of children with oculomotor paralysis that is isolated or associated with minimal symptomatology, one must examine the incidence of aneurysm in childhood. Cerebral aneurysms are rare in children. Patel and Richardson ( 5) reviewed 3,000 patients with ruptured intracranial aneurysm: 58 were younger than 19 years, and only four were younger than 10 years. Laitinen ( 6) reviewed 1,175 cases of cerebral aneurysm with only 35 patients under age 15 and one under age 10. Matson ( 7) reported 13 patients under 19 years old, five of whom were under 10 years old. The Cooperative Study on Intracranial Aneurysms ( 8) looked at 2,627 patients: 42 were under age 20, and only seven were under age 10. Clinically latent cerebral aneurysms are found in ~ 8% of autopsies of adults, but none are reported in children ( 5). Of all 6,802 aneurysms in these series ( 5,6,8), only 12 ( 0.2%) were in children under the age of 10 years. When cerebral aneurysms do occur in childhood, they almost always present with subarachnoid hemorrhage. In Matson's series ( 7), of 13 patients under 20 years old, 12 presented with subarachnoid hemorrhage. All five younger than 10 years old presented with signs of subarachnoid hemorrhage. Thus, it is obvious that childhood aneurysms presenting as third nerve palsies are exceedingly rare. Review of six large series ( 9- 14) of aneurysm- induced oculomotor paralysis revealed no reported cases in patients under age 20 years ( Table 3). None of the 16 patients under age 10 studied by Matson ( 7), Patel and Richardson ( 5.), or Locksley ( 8) had isolated ophthalmoplegia. We reviewed series ( 9,15,16) of isolated acquired oculomotor paralysis in patients younger than 20 years and found only two of 40 ( 5%) cases due to aneurysm ( Table 4). To our knowledge, these two cases reported by Miller ( 15) are the only ones, other than the one reported here, of acquired oculomo- Children « 20 years) Adults Cause Percent Cause Percent Congenital 45 Aneurysm 20 Trauma 20 Vasculopathic 20 Tumor 10 Trauma 10" 1.,- 15 Inflammatory 10 Tumor 10" 1.,- 15 Infectious and 5 Infectious and 5 postinfectious postinfectious Ophthalmoplegic 5 Miscellaneous 30 migraine Cryptogenic ~ 3 Aneurysm 3 Pupil spared Blood pressure 2- h postprandial blood glucose > 40 years Pupil involved CT scan of head Cerebrospinal fluid studies Cerebral angiogram < 40 years CT, computed tomography. CT scan of head Cerebrospinal fluid studies Cerebral angiogram JClin Neuro- ophthalmol. Vol. 9. No. 1. 1989 ARTERIOGRAPHY IN ACUTE OCULOMOTOR NERVE PALSY 35 TABLE 3. Age incidence of aneurysm- induced oculomotor paralysis Reference Green et al. ( 9) Dailey et al. ( 10) Botterell et al. ( 11) Cogan and Mount ( 12) Cantu et al. ( 13) Raja ( 14) Total No. of cases 38 30 40 39 26 42 121 No. < age 20 years oooooo o TABLE 5. Initial management of acquired oculomotor nerve palsy without sign of subarachnoid hemorrhage > 40 years < 10 years 10- 40 years Pupil involved Pupil spared CT scan MRI scan MRI scan Blood pressure of head of head of head 2- h postprandial MRI scan CSF studies CSF studies blood glucose of head Cerebral Cerebral CSF studies angiography angiography CT, computed tomography; MRI, magnetic resonance imaging; CSF, Cerebrospinal fluid. TABLE 4. Incidence of aneurysm- induced third nerve palsy in children tor paralysis caused by aneurysm in patients under 20 years. Miller's two patients; aged 16 and 17, both presented with oculomotor palsy, severe headache, and stiff neck. Angiograms revealed aneurysms of the posterior communicating artery in both cases. Our patient, 14 years old, presented with oculomotor palsy and headache but without stiff neck. The diagnosis of posterior communicating artery aneurysm was obtained by angiogram in this case as well. The rarity of aneurysm- induced third nerve palsies in children reflects not only the rarity of aneurysms in children but the rarity of posterior communicating artery aneurysms as well. The three largest series ( 5,7,17) of childhood aneurysm report a total of 27 cases in patients under age 10, only one of which is a posterior communicating artery aneurysm. Although Burde et al. ( 1) recommend arteriography for all patients under the age of 40 years with acquired oculomotor palsies, the absence of reported cases of isolated oculomotor nerve palsy due to aneurysm in children leads us to believe that arteriography is unnecessary in patients under age 10 if the symptoms and signs of subarachnoid hemorrhage are absent or high- resolution CT scan or adequate MRI scan is normal ( Table 5). Both MRI and CT scanning offer noninvasive means of identifying mass lesions, with the former allowing a more specific etiologic diagnosis if the aneurysm is of sufficient size to be imaged (~ 1O mm) ( 18- 20). Utilization of both these modalities in Reference Miller ( 15) Harley ( 16) Green et al. ( 9) Total Acquired isolated third nerve palsies « age 20 years) 17 17 6 40 Number due to aneurysm 2oo 2 children can be carried out with minimal sedation in comparison to that needed for angiography. Thus, both the noninvasiveness of the techniques and the type of sedation required avoid much of the morbidity and possible mortality associated with arteriography. However, if signs and symptoms of subarachnoid hemorrhage are present, angiography is indicated. Acknowledgment: Supported in part by an unrestricted grant from Research To Prevent Blindness, Inc. ( Department of Ophthalmology). REFERENCES 1. Burde RM, Savino PI, Trobe JD. Clinical decisions in neuroophthalmology. 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Arch Ophthalmol 1964; 72: 154- 67. 10. Dailey EI, Holloway JA, Murto RE, Schlezinger NS. Evaluation of ocular signs and symptoms in cerebral aneurysms. Arch Ophthalmol 1964; 71: 463- 74. 11. Botterell E, Lloyd LA, Hoffman HJ. Oculomotor palsy due to supraclinoid internal carotid artery Berry aneurysm. Am JOphthalmoI1962; 54: 609- 16. 12. Cogan 0, Mount H. Intracranial aneurysms causing ophthalmoplegia. Arch OphthalmoI1963; 70: 757- 71. 13. Cantu RC, Sanders T, Hepler S. Effects of carotid ligation on aneurysm- induced oculomotor palsy. J Neurosurg 1969; 31: 528- 32. 14. Raja I. Aneurysm- induced third nerve palsy. J Neurosurg 1972; 36: 538- 51. J Clin Neuro- ophthalmol, Vol. 9, No. 1, 1989 36 E. B. GABIANELLI ET AL. 15. Miller NR. Solitary oculomotor nerve palsy in childhood. Am I OphthalmoI1977; 83: 106-- 11. 16. Harley RD. Paralytic strabismus in children. Etiologic incidence and management of the third, fourth, and sixth nerve palsies. Ophthalmology 1980; 87: 24- 43. 17. 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