J Neurosurg 47:619-622, 1977
Bilateral persistent trigeminal arteries Case report
EUGENE F. BINET, M . D . , AND RONALD F. YOUNG, M . D .
Departments of Radiology and Neurosurgery, State University Hospital of the Upstate Medical Center, Syracuse, New York
A case is presented of bilateral persistent trigeminal arteries associated with reticulum cell sarcoma of the cerebellum. KEY WORDS 9 bilateral trigeminai arteries carotid-basilar anastomosis
9
HE primitive trigeminal artery is 1956.1 Karasawa, et al., 2 reported a case of a the most common of the three well left primitive otic artery and a right primitive known persistent carotid-basilar anas- trigeminal artery occurring in a patient with tomoses. First described anatomically by multiple cerebrovascular anomalies. In their Quain in 1844, 7 this presegmental vessel rep- extensive review of this subject, Wollresents the persistence of a primitive connec- schlaeger and Wollschlaeger 9,1~ found no intion between longitudinal parallel arteries stance of bilateral persistent primitive trigemipresent along the surface of the hindbrain of nal arteries reported in the English literature. the 4 mm embryo, and the internal carotid The following case report demonstrates the artery. In normally developing embryos these occurrence of bilateral persistent trigeminal longitudinal vessels fuse into the basilar arteries in association with a primary reticartery. The function of the primitive ulum cell sarcoma of the cerebellum. trigeminal artery is replaced by the posterior communicating artery and the vertebral Case Report arteries? Sutton 8 first described the angiographic appearance of a persistent trigeminal A 60-year-old right-handed woman was artery in 1950. The calculated "angiographic first seen with complaints of headache, occurrence" of this anomaly is approximately vomiting, and unsteady gait of 3-month dura0.1% to 0.2%. 4 tion. She noted markedly decreased hearing In the material reviewed by Krayenbfihl in the left ear, and had become unable to walk and Ya~argil, 3 no example of bilateral per- without assistance. sistence of trigeminal arteries was found. The Examination. Positive findings on incidence of persistence of more than one neurological testing included bilateral primitive artery is unknown. In 1922, Oertel e papilledema, coarse horizontal nystagmus on described a patient with persistent trigeminal lateral gaze bilaterally, left sensorineural and hypoglossal arteries. One case of bilateral hearing loss, marked ataxia of gait, decreased persistent primitive trigeminal arteries was rapid alternating movements and ataxia on reported in the Italian literature by Brea in finger-to-nose, and heel-to-shin testing on the
T
J. Neurosurg. / Volume 47 / October, 1977
619
E. F. Binet and R. F. Young
FIG. 1. Computerized tomographic study with enhancement. Note the highly vascular multinodular mass lying within the left cerebellar hemisphere. Cerebellar edema is present. The fourth ventricle is compressed and displaced to the right. The lateral ventricles are enlarged. left. Motor and sensory examination were normal. No pathological reflexes were present. Results of laboratory examinations including routine complete blood count and urinalysis, bone marrow biopsy, nuclear medicine liver and spleen imaging, gastro-
intestinal series, and barium enema were normal. Radiological Studies. A computerized tomographic study showed a highly vascular, multinodular mass lying within the left cerebellar hemisphere associated with significant adjacent cerebellar edema. The fourth ventricle was compressed and shifted to the right. The lateral ventricles were dilated (Fig. 1). At transfemoral angiography the right vertebral artery could not be identified. The left vertebral artery was very small, measuring approximately 2 m m in diameter. Selective injection into the root of the left subclavian artery demonstrated a snaall left posterior inferior cerebellar artery which was displaced inferiorly and to the right (Fig. 2). No tumor stain could be identified. A left common carotid artery injection revealed a left persistent trigeminal artery (Fig. 3). The contrast material refluxed into the posterior circulation with good visualization of both posterior cerebral arteries, both superior cerebellar arteries, and the dome of the basilar artery. A selective right internal carotid angiogram revealed the presence of a right trigeminal artery (Fig. 4). The contrast medium also refluxed into the left trigeminal artery. In addition, there was good visualization of both posterior cerebral and superior cerebellar arteries, the entire basilar artery,
FIG. 2. Left subclavian angiogram, arterial phase, anteroposterior (left) and lateral (right) projections. The left vertebral artery is hypoplastic. The left posterior inferior cerebellar artery is displaced inferiorly and to the right. 620
J. Neurosurg. / Volume 4 7 / October, 1977
Bilateral persistent trigeminal arteries
FIG. 3. Left carotid angiogram, arterial phase, anteroposterior (left) and lateral (right) projections. A left persistent trigeminal artery is present (small arrowheads). There is good visualization of the basilar and the right and left posterior cerebral and superior cerebellar arteries.
FXG. 4. Right carotid angiogram, arterial phase, anteroposterior (left) and lateral (right) projections. A right persistent trigeminal artery is present (small arrowheads). The persistent left trigeminal artery, is also identified (open arrow). There is good visualization of the vertebrobasilar system. The posterior inferior cerebellar artery is displaced medially and inferiorly.
J. Neurosurg. / Volume 47 / October, 1977
621
E. F. Binet and R. F. Young both vertebral arteries, and the proximal portion of the left posterior inferior cerebellar artery. Again, although displacement of this vessel was evident, no tumor stain was observed. Operation. A left suboccipital craniotomy was performed with partial excision of a firm left cerebellar hemisphere mass invading through the subarachnoid space into the dura. A review of the material submitted for microscopic analysis showed parenchymal infiltration and leptomeningeal spread of tumor composed of a pleomorphic array of lymphoid cells resembling malignant histiocytes. Reticulum stain demonstrated an increase of perivascular connective tissue fibers. Final diagnosis was lymphoproliferative disorder consistent with reticulum cell sarcoma of microglial origin. Postoperative Course. Postoperatively, the patient's neurological state was markedly improved. Her headache and vomiting ceased, and she was able to walk unassisted. She underwent a course of 4000 rads of radiotherapy to the whole brain with a booster dose of 1200 rads to the posterior fossa) At follow-up e x a m i n a t i o n 10 months postoperatively, her neurological state had remained normal except for mild hearing loss in the left ear and nystagmus on lateral gaze. Discussion
This case of bilateral persistent trigeminal arteries is of theoretical interest since it is the first report in the English language literature of a bilateral persistence of this carotidbasilar anastomosis. It is also of practical importance. Injection of contrast material into the vertebral arteries in the presence of bilateral persistent trigeminal arteries will result in poor filling of the posterior fossa circulation and an angiogram of suboptimal quality. This effect would likely be greater with a bilateral than a unilateral anomaly since the flow of blood from the carotid to the basilar system should be greater in the bilateral type. In addition, the basilar artery from its junction with the anomalous artery to its origin may be hypoplastic, and one or both vertebral arteries may also be hypoplastic? In the case reported here the
622
right vertebral artery could not be identified and the left was hypoplastic, preventing a selective injection of contrast material. Thus, as in this case, an injection of contrast material into the carotid artery may be necessary to obtain a satisfactory posterior fossa angiogram. Persistent carotid basilar anastomoses have been demonstrated in association with many neurological diseases and its association in this case with a reticulum cell sarcoma of the cerebellum is coincidental. References
1. Brea GB: Sulla Persistenza Della Anastomosi Carotido Basilare. Sistema Nervosa 8:17-23, 1956 2. Karasawa J, Terano M, Nishikawa M, et al: [A case of bilateral persistent carotid-basilar anastomoses (primitive otic artery and primitive trigeminal artery) with multiple cerebrovascular anomalies.] Brain Nerve (Tokyo) 24:91-98, 1972 (Jap) 3. Krayenb/ihl HA, Yasargil MG: Cerebral Angiography, ed 2. Philadelphia: JB Lippincott, 1969 4. Lie TA: Congenital Anomalies of the Carotid Arteries. Amsterdam: Excerpta Medica, 1968 5. Littman P, Wang CC: Reticulum cell sarcoma of the brain. A review of the literature and a study of 19 cases. Cancer 35:1412-1420, 1975 6. Oertel O: Ober die persistenz embryonaler Verbindungen zwishen der A. carotis interna und der A. vertebralis cerebralis. Dtsch Med Wochenschr 48:1264, 1922 7. Quain R: The Anatomy of the Arteries of the Human Body. London: Taylor and Walton, 1844 8. Sutton D: Anomalous carotid-basilar anastomosis. Br J Radiol 23:617-619, 1950 9. Wollschlaeger G, Wolischlaeger PB: The circle of Willis, in Newton TH, Potts DG (eds): Radiology of the Skull and Brain, Volume 2,
Book 2. St. Louis: CV Mosby, 1974, pp 1171-1201 10. Wollschlaeger G, Wollschlaeger PB: Personal communication, 1975
Address reprint requests to: Ronald F. Young, M.D., Division of Neurosurgery, Harbor General Hospital, 1000 West Carson Street, Torrans, California 90502.
J. Neurosurg. / Volume 47 / October, 1977
Bilateral persistent trigeminal arteries Case report
EUGENE F. BINET, M . D . , AND RONALD F. YOUNG, M . D .
Departments of Radiology and Neurosurgery, State University Hospital of the Upstate Medical Center, Syracuse, New York
A case is presented of bilateral persistent trigeminal arteries associated with reticulum cell sarcoma of the cerebellum. KEY WORDS 9 bilateral trigeminai arteries carotid-basilar anastomosis
9
HE primitive trigeminal artery is 1956.1 Karasawa, et al., 2 reported a case of a the most common of the three well left primitive otic artery and a right primitive known persistent carotid-basilar anas- trigeminal artery occurring in a patient with tomoses. First described anatomically by multiple cerebrovascular anomalies. In their Quain in 1844, 7 this presegmental vessel rep- extensive review of this subject, Wollresents the persistence of a primitive connec- schlaeger and Wollschlaeger 9,1~ found no intion between longitudinal parallel arteries stance of bilateral persistent primitive trigemipresent along the surface of the hindbrain of nal arteries reported in the English literature. the 4 mm embryo, and the internal carotid The following case report demonstrates the artery. In normally developing embryos these occurrence of bilateral persistent trigeminal longitudinal vessels fuse into the basilar arteries in association with a primary reticartery. The function of the primitive ulum cell sarcoma of the cerebellum. trigeminal artery is replaced by the posterior communicating artery and the vertebral Case Report arteries? Sutton 8 first described the angiographic appearance of a persistent trigeminal A 60-year-old right-handed woman was artery in 1950. The calculated "angiographic first seen with complaints of headache, occurrence" of this anomaly is approximately vomiting, and unsteady gait of 3-month dura0.1% to 0.2%. 4 tion. She noted markedly decreased hearing In the material reviewed by Krayenbfihl in the left ear, and had become unable to walk and Ya~argil, 3 no example of bilateral per- without assistance. sistence of trigeminal arteries was found. The Examination. Positive findings on incidence of persistence of more than one neurological testing included bilateral primitive artery is unknown. In 1922, Oertel e papilledema, coarse horizontal nystagmus on described a patient with persistent trigeminal lateral gaze bilaterally, left sensorineural and hypoglossal arteries. One case of bilateral hearing loss, marked ataxia of gait, decreased persistent primitive trigeminal arteries was rapid alternating movements and ataxia on reported in the Italian literature by Brea in finger-to-nose, and heel-to-shin testing on the
T
J. Neurosurg. / Volume 47 / October, 1977
619
E. F. Binet and R. F. Young
FIG. 1. Computerized tomographic study with enhancement. Note the highly vascular multinodular mass lying within the left cerebellar hemisphere. Cerebellar edema is present. The fourth ventricle is compressed and displaced to the right. The lateral ventricles are enlarged. left. Motor and sensory examination were normal. No pathological reflexes were present. Results of laboratory examinations including routine complete blood count and urinalysis, bone marrow biopsy, nuclear medicine liver and spleen imaging, gastro-
intestinal series, and barium enema were normal. Radiological Studies. A computerized tomographic study showed a highly vascular, multinodular mass lying within the left cerebellar hemisphere associated with significant adjacent cerebellar edema. The fourth ventricle was compressed and shifted to the right. The lateral ventricles were dilated (Fig. 1). At transfemoral angiography the right vertebral artery could not be identified. The left vertebral artery was very small, measuring approximately 2 m m in diameter. Selective injection into the root of the left subclavian artery demonstrated a snaall left posterior inferior cerebellar artery which was displaced inferiorly and to the right (Fig. 2). No tumor stain could be identified. A left common carotid artery injection revealed a left persistent trigeminal artery (Fig. 3). The contrast material refluxed into the posterior circulation with good visualization of both posterior cerebral arteries, both superior cerebellar arteries, and the dome of the basilar artery. A selective right internal carotid angiogram revealed the presence of a right trigeminal artery (Fig. 4). The contrast medium also refluxed into the left trigeminal artery. In addition, there was good visualization of both posterior cerebral and superior cerebellar arteries, the entire basilar artery,
FIG. 2. Left subclavian angiogram, arterial phase, anteroposterior (left) and lateral (right) projections. The left vertebral artery is hypoplastic. The left posterior inferior cerebellar artery is displaced inferiorly and to the right. 620
J. Neurosurg. / Volume 4 7 / October, 1977
Bilateral persistent trigeminal arteries
FIG. 3. Left carotid angiogram, arterial phase, anteroposterior (left) and lateral (right) projections. A left persistent trigeminal artery is present (small arrowheads). There is good visualization of the basilar and the right and left posterior cerebral and superior cerebellar arteries.
FXG. 4. Right carotid angiogram, arterial phase, anteroposterior (left) and lateral (right) projections. A right persistent trigeminal artery is present (small arrowheads). The persistent left trigeminal artery, is also identified (open arrow). There is good visualization of the vertebrobasilar system. The posterior inferior cerebellar artery is displaced medially and inferiorly.
J. Neurosurg. / Volume 47 / October, 1977
621
E. F. Binet and R. F. Young both vertebral arteries, and the proximal portion of the left posterior inferior cerebellar artery. Again, although displacement of this vessel was evident, no tumor stain was observed. Operation. A left suboccipital craniotomy was performed with partial excision of a firm left cerebellar hemisphere mass invading through the subarachnoid space into the dura. A review of the material submitted for microscopic analysis showed parenchymal infiltration and leptomeningeal spread of tumor composed of a pleomorphic array of lymphoid cells resembling malignant histiocytes. Reticulum stain demonstrated an increase of perivascular connective tissue fibers. Final diagnosis was lymphoproliferative disorder consistent with reticulum cell sarcoma of microglial origin. Postoperative Course. Postoperatively, the patient's neurological state was markedly improved. Her headache and vomiting ceased, and she was able to walk unassisted. She underwent a course of 4000 rads of radiotherapy to the whole brain with a booster dose of 1200 rads to the posterior fossa) At follow-up e x a m i n a t i o n 10 months postoperatively, her neurological state had remained normal except for mild hearing loss in the left ear and nystagmus on lateral gaze. Discussion
This case of bilateral persistent trigeminal arteries is of theoretical interest since it is the first report in the English language literature of a bilateral persistence of this carotidbasilar anastomosis. It is also of practical importance. Injection of contrast material into the vertebral arteries in the presence of bilateral persistent trigeminal arteries will result in poor filling of the posterior fossa circulation and an angiogram of suboptimal quality. This effect would likely be greater with a bilateral than a unilateral anomaly since the flow of blood from the carotid to the basilar system should be greater in the bilateral type. In addition, the basilar artery from its junction with the anomalous artery to its origin may be hypoplastic, and one or both vertebral arteries may also be hypoplastic? In the case reported here the
622
right vertebral artery could not be identified and the left was hypoplastic, preventing a selective injection of contrast material. Thus, as in this case, an injection of contrast material into the carotid artery may be necessary to obtain a satisfactory posterior fossa angiogram. Persistent carotid basilar anastomoses have been demonstrated in association with many neurological diseases and its association in this case with a reticulum cell sarcoma of the cerebellum is coincidental. References
1. Brea GB: Sulla Persistenza Della Anastomosi Carotido Basilare. Sistema Nervosa 8:17-23, 1956 2. Karasawa J, Terano M, Nishikawa M, et al: [A case of bilateral persistent carotid-basilar anastomoses (primitive otic artery and primitive trigeminal artery) with multiple cerebrovascular anomalies.] Brain Nerve (Tokyo) 24:91-98, 1972 (Jap) 3. Krayenb/ihl HA, Yasargil MG: Cerebral Angiography, ed 2. Philadelphia: JB Lippincott, 1969 4. Lie TA: Congenital Anomalies of the Carotid Arteries. Amsterdam: Excerpta Medica, 1968 5. Littman P, Wang CC: Reticulum cell sarcoma of the brain. A review of the literature and a study of 19 cases. Cancer 35:1412-1420, 1975 6. Oertel O: Ober die persistenz embryonaler Verbindungen zwishen der A. carotis interna und der A. vertebralis cerebralis. Dtsch Med Wochenschr 48:1264, 1922 7. Quain R: The Anatomy of the Arteries of the Human Body. London: Taylor and Walton, 1844 8. Sutton D: Anomalous carotid-basilar anastomosis. Br J Radiol 23:617-619, 1950 9. Wollschlaeger G, Wolischlaeger PB: The circle of Willis, in Newton TH, Potts DG (eds): Radiology of the Skull and Brain, Volume 2,
Book 2. St. Louis: CV Mosby, 1974, pp 1171-1201 10. Wollschlaeger G, Wollschlaeger PB: Personal communication, 1975
Address reprint requests to: Ronald F. Young, M.D., Division of Neurosurgery, Harbor General Hospital, 1000 West Carson Street, Torrans, California 90502.
J. Neurosurg. / Volume 47 / October, 1977
