Persistent primitive hypoglossal artery aneurysm--case report

The aneurysm arising from a persistent primitive hypoglossal artery (PHA) is rare, and only 13 such cases have been reported in literature. We present a 62-year-old woman with an aneurysm of PHA at its junction with the basilar artery. The patient consulted our hospital for a transient loss of consciousness and headaches. No neurological deficit was found, but MRI and MRA showed an aneurysm of the vertebrobasilar junction. Cerebral angiogram after admission showed the aneurysm of PHA at its junction with the basilar artery. Perspective 3D-CTA and 3D-T2 weighted MR images were composed to simulate the condition and aneurysmal surgery via the transcondylar approach was carried out. The aneurysm was successfully clipped and the patient was discharged with no neurological deficits. Perspective 3 D-CTA and MRI simulation were very useful for this operation.     

Persistent primitive hypoglossal artery associated with cerebral aneurysm and cervical internal carotid artery stenosis--case report.

A 71-year-old female had vertigo attacks once or twice a day secondary to vertebrobasilar insufficiency. Left carotid angiography revealed persistent primitive hypoglossal artery (PPHA) associated with a large internal carotid artery (ICA) aneurysm and severe stenosis of the ICA. The bilateral vertebral arteries were hypoplastic. The basilar artery was opacified via the PPHA but not via vertebral arteries. Clipping of the aneurysm was performed first because the risk of rupture of the aneurysm was not negligible. One month after clipping, carotid endarterectomy using a T-shaped shunt system was successfully performed. The postoperative course was uneventful and the vertebrobasilar ischemic attacks did not recur. Left carotid angiography demonstrated complete obliteration of the aneurysm and disappearance of the carotid artery stenosis. Low ICA flow (70 ml/min) and low stump pressure of the PPHA (25 mmHg) strongly suggested low perfusion of the posterior circulation. Carotid endarterectomy may be essential for augmentation of the posterior circulation in patients with PPHA associated with ICA stenosis.     

Large arteriovenous malformation associated with persistent primitive hypoglossal artery--case report.

A rare case of large arteriovenous malformation (AVM) with persistent primitive hypoglossal artery in a 43-year-old male was treated by staged embolization, followed by total removal. AVM associated with carotid-basilar anastomosis is quite rare, but the incidence of AVM in patients with carotid-basilar anastomosis is high. AVM associated with persistent carotid-basilar anastomosis has no distinguishing features compared with ordinary AVM, but participation of the posterior circulation as a feeder is characteristic.     

Persistent primitive hypoglossal artery associated with Arnold-Chiari type I malformation

The case of a 49-year-old woman with persistent primitive hypoglossal artery associated with Arnold-Chiari type I malformation is reported. The hypoglossal artery was located on the left side. The left vertebral artery was hypoplastic, the right vertebral artery could not be visualized angiographically. The clinical significance of a persistent primitive hypoglossal artery is discussed.     

Anomalous internal carotid artery associated with ipsilateral cerebral arteriovenous malformation--case report.

A 15-year-old girl presented with an extremely rare anomalous origin of the internal carotid artery (ICA) from the common carotid artery, associated with an arteriovenous malformation in the brain and aberrant course of the ICA in the temporal bone. These anomalies, which were found on the same side, may be due to a maldevelopment of the vascular network including the aortic arches.     

Persistent primitive hypoglossal artery associated with proximal posterior inferior cerebellar artery aneurysm

BACKGROUND: A persistent primitive hypoglossal artery (PPHA) is a rare anomaly. The association of PPHA with intracranial aneurysms of the artery has also been rarely reported. We surgically treated a case of PPHA associated with a ruptured saccular aneurysm at the proximal posterior inferior cerebellar artery (PICA). CASE DESCRIPTION: The patient was admitted because of subarachnoid hemorrhaging. Angiography and three-dimensional computed tomography (CT) angiography (3D-CTA) demonstrated a left PPHA entering the posterior fossa through the left large hypoglossal canal. The left vertebral artery was absent. A saccular aneurysm was found at the junction of the PPHA and the proximal PICA. 3D-CTA showed not only the aneurysm itself but also the anatomical relationship between the aneurysm and the surrounding structures. Therefore, 3D-CTA was very useful in planning the surgery. The neck of the aneurysm was clipped through a far lateral approach associated with a C1 laminectomy, because this case had a large posterior condylar emissary vein and the aneurysm was located just posteroinferior to the hypoglossal canal. CONCLUSIONS: A case of PPHA associated with an aneurysm at the proximal PICA is reported. This case not only had a large hypoglossal canal but also had a huge posterior condylar emissary vein in the large posterior condylar canal. Anomalous structures associated with PPHA are also discussed. Finally, 3D-CTA proved to be very useful in planning the optimal surgical modality around the lateral portion of the foramen magnum.     

Persistent primitive hypoglossal artery aneurysms--report of two cases.

The authors present two patients with subarachnoid hemorrhage caused by ruptured intracranial saccular aneurysms of the persistent primitive hypoglossal artery. A standard unilateral suboccipital approach in one patient resulted in incomplete neck clipping since the operative field was restricted by a protruding jugular tubercle. Successful aneurysmal neck clipping was achieved in the second patient via a unilateral-transcondylar-suboccipital approach with resection of the jugular tubercle and rim of the foramen magnum.     

Persistent primitive trigeminal artery associated with brain cavernoma. Case report

Among cases of embryonic carotid-basilar anastomosis which may persist after birth, persistent trigeminal artery is the most common. It has been associated with a wide variety of intracranial abnormalities. We are unaware of any other reported association with cavernoma. We report a young woman who experienced seizures following spontaneous abortion. A CT scan disclosed a right frontal hematoma. MRI revealed a cavernoma associated with a persistent trigeminal artery. The cavernoma was removed through a frontal approach. The aim of the present case is to report another type of lesion fortuitously associated with a persistent trigeminal artery.     

Persistent primitive trigeminal artery aneurysm associated with cerebellar hemangioblastoma. Case report

A 72-year-old man presented with a cerebellar vermian tumor manifesting as headaches and vertigo. Angiography disclosed a vascular tumor fed by the superior cerebellar artery and an aneurysm of a primitive trigeminal artery. The patient underwent right occipital craniotomy to remove the highly vascular tumor via an occipital transtentorial approach. Association of a cerebral aneurysm with a hemangioblastoma has been reported previously in only five cases. Only three aneurysms were located on the feeding artery. The aneurysm in this case was not on the feeding artery. Simple coincidence might account for this case.     

Moyamoya disease with persistent primitive hypoglossal artery. Case report

A 44-year-old female developed aphasia and visual disturbance of the left eye. Four-vessel angiography showed occlusion of the extra- and intracranial portions of the right internal carotid artery, severe stenosis of the left internal carotid fork, bilateral moyamoya vessels, and a persistent primitive hypoglossal artery on the left side. Measurement of cerebral blood flow by 133xe inhalation revealed decreased flow and no response to acetazolamide loading in the left cerebral hemisphere. The patient underwent superficial temporal artery-middle cerebral artery anastomosis, first on the left and then on the right side. There have been no other reported cases of moyamoya disease associated with a persistent primitive hypoglossal artery. Although the cause of moyamoya disease remains to be established, it is generally believed that stenotic changes of the carotid fork cause the development of moyamoya vessels as collateral pathways. According to this theory, there is no significant etiological relationship between moyamoya disease and persistent primitive hypoglossal artery. However, persistent primitive hypoglossal artery is compatible with moyamoya disease in terms of providing a collateral pathway via the vertebrobasilar system.     

Persistent primitive hypoglossal artery with retrograde flow from the vertebrobasilar system: a case report

The persistent primitive hypoglossal artery (PPHA) is one of the pairs of arterial connections that exist in the human embryo between the developing anterior and posterior circulation. Normally the PPHA arises from the cervical internal carotid artery (ICA) and passes through the hypoglossal canal to join the caudal basilar artery (BA). In most cases the vertebral arteries (VA) are either hypoplastic or aplastic and the posterior communicating arteries (PComA) are absent; thus, the main supply to the posterior circulation comes from the internal carotid via the PPHA in an antegrade fashion. Atherosclerotic plaques in the ICA and PPHA present with ischemic symptoms of both the carotid and vertebrobasilar systems. We report a case of a 53-year-old female who presented with a transient episode of left lower extremity numbness and weakness. Work-up with computed tomography (CT) and magnetic resonance imaging (MRI) showed a small watershed infarct in the right middle cerebral artery (MCA)/posterior cerebral artery (PCA) territory. Diagnostic angiography revealed severe proximal stenosis of the cervical ICA and presence of a PPHA just above the stenosis with retrograde filling from the vertebrobasilar junction to the distal cervical ICA. The patient underwent a carotid endarterectomy with intraoperative EEG monitoring. Intraoperative blood flow measurements were made before and after endarterectomy showing evidence of reversal of blood flow to a normal antegrade fashion. The postoperative angiogram showed resolution of the right ICA stenosis and persistence of the PHA. To our knowledge this is the first case report of a PPHA exhibiting reversal of blood flow from the posterior into the anterior circulation. Awareness of this embryological anomaly and its interaction with acquired atherosclerotic disease will minimize misinterpretation of vascular diagnostic studies.     

Persistent hypoglossal artery.

During embryological development, the hypoglossal artery serves as a transient anastomosis between the internal carotid artery and the vertebro-basilar system. This artery occasionally persists into adult life. Two cases of persistent hypoglossal artery we have recently encountered are reported here. The embryology, diagnosis and potential problems for carotid surgery are discussed.     

Persistent hypoglossal artery in combination with multifocal arteriovenous malformations of the brain: case report

The persistence of embryonic cerebral vessels in the adult is not a frequent occurrence, neither is the presence of multifocal arteriovenous malformations (AVMs) of the brain. The most commonly reported type of persistent carotid-basilar anastomosis is the primitive trigeminal artery, followed by the primitive hypoglossal artery (PHA). In this report, a 30-year-old, right-handed woman hospitalized because of subarachnoid hemorrhage and harboring an intracerebral-intraventricular hematoma resulting from the rupture of one of two independent AVMs of the left cerebral hemisphere, was found also to have a right persistent PHA. One AVM was intraventricular and had ruptured; the other was subcortical, intact in the parietal lobe. The PHA originated as a large anomalous branch of the right internal carotid artery in the neck and joined the basilar artery after entering the posterior fossa through the ipsilateral anterior condyloid foramen, which was enlarged. At craniotomy, the two AVMs were successfully excised with the aid of microsurgical technique. These two independently rare conditions, namely, multifocal cerebral AVMs and persistent PHA, warrant our desire to report this case.     

Basilar bifurcation aneurysms associated with persistent primitive hypoglossal artery

We report two cases of ruptured basilar bifurcation aneurysm associated with a persistent primitive hypoglossal artery. Angiograms revealed low-positioned aneurysms; in both cases bilateral vertebral arteries and posterior communicating arteries were hypoplastic or aplastic. Both aneurysms were successfully clipped via subtemporal transtentorial approach through the craniotomy ipsilateral to the side of the primitive hypoglossal artery. The ipsilateral craniotomy and exposure of the cervical carotid artery were helpful for obtaining the proximal control of the basilar artery needed to perform the clipping procedure with safety.     

Klippel-Trenaunay-Weber syndrome with spinal arteriovenous malformation--case report

A 28-year-old female with Klippel-Trenaunay-Weber syndrome associated with an arteriovenous malformation (AVM) of the spinal cord is reported. She was admitted to our hospital with a 4-month history of steadily progressive weakness and dysesthesia of the legs. A nevus flammeus, varices, hypertrophy and elongation of the left leg had been present since her infancy. These symptoms progressed and she became unable to walk and pass water by herself. Myelography disclosed a spinal AVM extending from Th11 to L2. Angiography confirmed the presence of stretched and tortuous vessels as well as an arteriovenous shunt in the left leg. Magnetic resonance imaging showed a high-intensity area extending from Th10 to L2. Following laminectomy from Th10 to L2 and resection of the AVM, her motor and bladder functions worsened. However, 6 months later, her motor function improved to the preoperative state and the bladder dysfunction disappeared. The coexistence of Klippel-Trenaunay-Weber syndrome with spinal AVM, considered to be rare, is discussed, and the pertinent literature is reviewed. The case presented here is the first to have been surgically treated in Japan.     

Posterior inferior cerebellar artery aneurysm associated with persistent primitive hypoglossal artery

A 49-year-old woman presented with subarachnoid hemorrhage (SAH) from an aneurysm associated with a persistent primitive hypoglossal artery (PPHA) manifesting as sudden onset of headache, but without neurological deficits. Conventional computed tomography (CT) of her head showed no abnormality but lumbar tap indicated SAH. Three-dimensional (3D)-CT angiography showed a PPHA originating from the internal carotid artery and an aneurysm of the posterior inferior cerebellar artery at the junction with the remnant hypoplastic vertebral artery. 3D-CT angiography was essentially useful for presurgical planning to determine the extent of craniotomy and the space for possible temporary clipping, and confirmed the diagnosis of aneurysmal SAH. The aneurysm was clipped and she returned to her job 4 weeks later. Cerebral angiography is the golden standard technique to diagnose PPHA, but 3D-CT angiography can be recommended for presurgical evaluation, especially in patients with complex and anomalous anatomical structures.     

A case report: persistent primitive hypoglossal artery aneurysm

The authors describe a case of persistent primitive hypoglossal artery aneurysm in a 42-year-old woman who had complained of headache, mainly in the occiput, for 5 days prior to admission. Because of a sudden exacerbation of the headache associated with vomiting, she was hospitalized on July 31, 1988. On admission, a cranial computed tomography scan demonstrated a high density lesion in the basal cisterns which suggested subarachnoid hemorrhage (SAH). Right carotid angiography revealed a persistent primitive hypoglossal artery and an aneurysm arising from this artery at the junction of the posterior inferior cerebellar artery. Bilateral vertebral arteries were shown to be hypoplastic. This was followed by a right suboccipital craniectomy on the 6th hospital day at which time a neck clipping was made. Her postoperative course was uneventful. On discharge on August 22, she was ambulatory and had no neurological deficit except for a mild hoarseness which developed after surgery. Well over one hundred cases of persistent primitive hypoglossal artery aneurysm have been reported. However, as far as we could discern, there have been only 9 cases of persistent primitive hypoglossal artery aneurysm including this present case. Most of the cases had SAH due to the rupture of these aneurysms with favorable outcome after the surgery. In addition, some embryological considerations were made.     

Persistent trigeminal artery associated with brainstem infarct--case report.

A 47-year-old male presented with a sudden onset of right hemiparesis and numbness of the left face. Magnetic resonance imaging demonstrated a lacunar infarct in the ventral pons. Cerebral angiography demonstrated a persistent trigeminal artery (PTA) anastomosing the left internal carotid artery to the distal basilar artery. Bilateral vertebral arteries and the basilar artery below the PTA junction were extremely hypoplastic. The bilateral posterior communicating arteries were embryonic. The posterior fossa circulation was almost independent from the circle of Willis. The poor vascular supply to the posterior fossa probably caused the brainstem infarct.