Evolving methods in genetic epidemiology. IV. Approaches to non-Mendelian inheritance

Dissecting aneurysm of the basilar artery is a rare but increasingly recognized entity, with a frequently fatal or morbid outcome. Unlike the well established proximal occlusion and trapping approaches to vertebral artery dissections, surgical intervention for basilar lesions has been limited to wrapping techniques for arterial wall reinforcement. We report a case of midbasilar dissecting aneurysm successfully treated by clipping the proximal basilar artery below the level of the anterior inferior cerebellar arteries, allowing retrograde flow via the posterior communicating arteries to provide continued basilar perfusion. With the growing recognition of basilar dissection and pseudoaneurysm formation there is a need for improved therapeutic options. We suggest that definitive treatment can be achieved using the principle of proximal occlusion and flow reversal, and review the pertinent literature on basilar artery dissection.     

Development of a dissecting aneurysm on the vertebral artery immediately after occlusion of the contralateral vertebral artery: a case report

A 49 year old female presented with subarachnoid hemorrhage due to a ruptured dissecting aneurysm on the left vertebral artery (VA). Following an occlusion test, we performed proximal occlusion of the left VA with detachable balloons. However, a dissecting aneurysm on the right VA developed three weeks later. After an occlusion test had showed no change in cerebral blood flow, auditory brain stem response, or neurological status, proximal occlusion of the right VA was performed. The patient has returned to normal life without neurological deficits. Bilateral dissecting aneurysms of the VA are quite common, but de novo VA dissecting aneurysms or enlargement of such aneurysms after occlusion of contralateral VA are rare. This case suggests that hemodynamics stress may be a causal factor in the development of VA dissecting aneurysms. Careful pre- and post-operative neuroradiological examination of the contralateral VA are required in patients undergoing VA occlusion for dissecting aneurysms.     

Rotational vertebral artery occlusion: a mechanism of vertebrobasilar insufficiency

OBJECTIVE: Symptomatic dynamic changes in blood flow secondary to vertebral artery compression with rotational head motion are evaluated in a series of patients as a cause for posterior circulation transient ischemic attacks. These cases are classic examples of rotational vertebral artery occlusion and allow for the discussion of the anatomic basis, angiographic features, and treatment options. ILLUSTRATIVE CASES: In our series, symptoms of vertebrobasilar insufficiency were reproducible with rotational head movement. Compression of the vertebral artery was demonstrated angiographically. The correct site of occlusion of the vertebral artery was apparent only by dynamic angiography with progressive head rotation. All of the patients presented in the illustrative cases had occlusion at the C2 level; however, one patient had been previously misdiagnosed and another had an additional site of occlusion. The anatomic course of the vertebral artery is described in addition to the sites of rotational occlusion. CONCLUSION: Rotational vertebral occlusion is an important cause of vertebrobasilar symptoms, which may lead to permanent neurological deficit if left undiagnosed. Dynamic angiography is the established method of diagnosis. Great care must be taken to avoid misdiagnosing the site of occlusion or missing a second occlusive site. For this reason, it is crucial to have a thorough understanding of the anatomic course of the vertebral artery and the muscular and tendinous insertions, which may cause rotational occlusion. The decision for treatment must be based on the site of occlusion as well as the assessment of the patient as a surgical candidate. A review of the literature reveals that surgical treatment is effective and must be considered to avoid further morbidity.     

Endosaccular occlusion of basilar artery bifurcation aneurysms using electrically detachable coils

Thirteen patients with basilar artery bifurcation aneurysms, treated by electrothrombosis using electrically detachable coils, are presented. Nine of them presented after hemorrhage, two with mass effect, and two were found coincidentally with other ruptured aneurysms. Selection for endovascular therapy was based on the following criteria: 1) poor clinical condition (Hunt and Hess III-V); 2) high surgical risk; 3) age and poor medical condition; 4) morphological features (small necked aneurysms). With endovascular Guglielmi detachable coils aneurysm occlusion ranging from 70-100% was achieved in all cases. All five small necked and two large broad necked aneurysms were totally occluded. Two large aneurysms had a 95% occlusion and two other large aneurysms were 90% occluded. In another patient with a large broad based aneurysm only an 80% occlusion was achieved, because of tortuosity of the vertebrobasilar system. Our last patient, who presented as grade V clinically, was partially treated with a 70% aneurysmal occlusion. The clinical results were excellent in 10 and good in 2. The only poor outcome was seen in the grade V patient. There was no morbidity or mortality related to therapy. The only complication was an asymptomatic dissecting aneurysm at the origin of the vertebral artery. Angiographic follow up time ranged from 6 to 20 months with a mean of 9 months. Four patients were treated too recently to have their angiographic follow up at 6 months. Two patients were lost to follow up. Clinical follow up ranged from 1 to 17 months with a mean of 8.9 months. The analysis of our cases clearly shows that aneurysms, which were densely packed with coils, especially if small necked, were less likely to be reperfused and showed a longlasting stable result. Large broad based aneurysms were more likely to be reopened by blood flow after the first procedure, especially if loosely filled with coils, and needed up to 3 interventions to achieve a satisfying result, whereas later in the series a high percentage rate of occlusion was seen after the first procedure. We consider now also a less than 100% occlusion acceptable, because most of the aneurysms will rupture at the dome, which was occluded in all our cases. We conclude, that this new endovascular method is a viable alternative in the treatment of posterior circulation aneurysms with a high surgical risk, in old patients and those in poor clinical and medical condition.     

Endovascular stent treatment of cervical internal carotid artery aneurysms with parent vessel preservation

BACKGROUND: Aneurysms involving the cervical portion of the internal carotid artery (ICA) frequently result from prior trauma or dissection. CASE DESCRIPTIONS: Two patients are reported with cervical internal carotid artery aneurysms. In both cases, disease involving the contralateral ICA precluded safe treatment of the aneurysms by ICA occlusion. Endovascular stents placed across the diseased portion of the artery resulted in thrombosis of the aneurysm with preservation of the parent artery. CONCLUSION: Endovascular stent placement should be considered for treatment of aneurysms involving the cervical ICA when preservation of the parent vessel is necessary.     

Brain lipid peroxidation and antioxidant levels in fetal lambs 72 hours after asphyxia by partial umbilical cord occlusion

Giant middle cerebral artery (MCA) trifurcation aneurysms that cannot be excluded directly can be treated by flow inversion achieved by creation of an extracranial-intracranial bypass distal to the aneurysm, followed by occlusion of the parent vessel proximal to the aneurysm. As opposed to surgical occlusion, endovascular occlusion avoids dissection of the aneurysm area, and the site of occlusion can be chosen according to the flow distribution demonstrated on angiography performed during test occlusions. Two patients with giant aneurysms of the MCA trifurcation benefited from flow inversion treatment. Forty-eight hours after an MCA-superficial temporal artery bypass had been created, the M1 segment was occluded by inserting a coil in the first patient and the internal carotid artery was occluded with balloons in the second patient (there was no communicating artery in the latter case). Both occlusions were performed immediately after a clinical test of occlusion tolerance. The patients were clinically intact during the postoperative course. Follow-up angiography performed 11 and 4 months, respectively, after vessel occlusion showed that the aneurysm occlusion was stable.     

Testosterone sulphation and glucuronidation in the human liver: interindividual variability

We present a case with the traumatic extracranial internal carotid artery dissecting aneurysm. A 21-year-old man was involved in a motorcycle accident, resulting in multiple injuries but no apparent head and neck injuries. Head CT was normal on his admission. He was discharged from his local hospital 3 weeks after the accident without any neurological deficits. Five weeks after the accident, he suddenly presented with a motor aphasia and a right hemiparesis. CT and MRI showed infarctions in the left para-Sylvian and the left angular areas. Angiography showed a left extracranial carotid artery dissecting aneurysm at the level of C1 vertebral arch. The patient was initially managed by an anticoagulant agent, but he suffered from another transient ischemic attack due to distal embolism from the aneurysm. Balloon occlusion test of the left ICA was performed under monitoring EEG, SEP. Mean stump pressure (MSP) revealed 60 mmHg. and MSP/Mean systematic blood pressure revealed 67%. We judged that the left ICA ligation was a safe method to treat this patient, however, considering the patient's age and the side of the lesion, left STA-MICA bypass and ligation of the left ICA were carried out in one stage. Postoperatively, the patient did not show any cerebral ischemic complications and angiography showed disappearance of the aneurysm and patency of the bypass. The left MCA territories were filled well by cross circulation and the bypass.     

A large vertebrobasilar junction aneurysm grown at the proximal end of basilar artery fenestration--usefulness of balloon occlusion test with 99mTc-HMPAO SPECT under induced hypotension and consideration in therapeutic strategies

A 48-year-old lady suffered a transient loss of consciousness. CT and MRI revealed a large vascular lesion compressing the left lower pons. Angiography revealed a large aneurysm at vertebro-basilar junction, dome of which projected anteriorly and left to midline. Her previous vertebral angiogram taken 10 years ago when she suffered a subarachnoid hemorrhage from the left MCA aneurysm, had showed a fenestration of lower basilar artery without apparent aneurysm. Bilateral super-selective vertebral angiograms revealed that the aneurysm arose at the proximal end of the fenestration, and vertebrobasilar junction was incorporated into the aneurysm indicating broad neck aneurysm. The left posterior communicating artery was well developed. Balloon test occlusion (BTO) of bilateral vertebral artery was performed under normotension and induced hypotension. 99mHM-PAO SPECT was used to examine cerebral blood flow (CBF) during hypotensive BTO. The patient tolerated the test and CBF imaging showed insignificant sight decrease in bilateral cerebellar hemispheres. Exploration of the aneurysm was carried out by the right far lateral suboccipital approach. Bilateral vertebral arteries and the right segment of the basilar artery fenestration were identified. Neck clipping of the aneurysm with reconstruction of the parent vessels were tried with fenestrate clip. However, narrow operative field and large dome of the aneurysm made it hard to identify the left segment of the fenestration. Neck clipping was given up and clipping of bilateral vertebral arteries were performed distal to posterior inferior cerebellar artery with three body clippings. The patient showed moderate postoperative left lower nerve palsy, which was gradually improved in several weeks. Follow-up angiography revealed no opacification of the aneurysm.(ABSTRACT TRUNCATED AT 250 WORDS)     

Extralobar pulmonary sequestration presenting as a mediastinal malignancy

OBJECTIVE: We intended to characterize the CT patterns of hemorrhage associated with ruptured posterior inferior cerebellar artery (PICA) aneurysms. MATERIALS AND METHODS: CT scans of 44 cases of angiographically confirmed ruptured saccular PICA aneurysms (4) aneurysms at the junction of the vertebral artery and the PICA and three distal PICA aneurysms) were retrospectively reviewed. All scans had been obtained within 2 days of the subarachnoid hemorrhage (SAH) (day 0 [less than 24 hr], 35 patients; day 1, eight patients; day 2, one patient). Presence or absence of hemorrhage in specific subarachnoid, intraventricular, and intraparenchymal locations was noted, as were the presence and degree of hydrocephalus. RESULTS: Posterior fossa SAH was present in 95% of cases. Isolated posterior fossa SAH was present in 30% of cases, but in no case was isolated supratentorial SAH present. Supratentorial SAH was present in 70% of cases. SAH involving the sylvian fissure or the interhemispheric region was present in 25% and 23% of cases, respectively. SAH along the convexity was present in 2% of cases. Intraventricular hemorrhage (IVH) with or without associated SAH was seen in 95% of cases, whereas isolated IVH was seen in 5% of cases. Hydrocephalus was present in 95% of cases and was moderate to marked in 70%. Both IVH and hydrocephalus were present in 93% of cases. CONCLUSION: Ruptured PICA aneurysms almost always coexist with hydrocephalus and IVH, as seen in 93% of cases, and almost never coexist with SAH along the convexity. The most common pattern of hemorrhage associated with such aneurysms includes IVH and posterior fossa hemorrhage. Extensive supratentorial SAH, in conjunction with posterior fossa SAH, is a common finding in patients with ruptured PICA aneurysms. SAH isolated to the posterior fossa is present in a sizeable minority of cases.     

Aneurysm of the major vessels in neurofibromatosis

The case of a patient who presented with a ruptured aneurysm of the brachial artery and type I neurofibromatosis is presented. Angiography revealed a ruptured aneurysm of the brachial artery in the middle of the upper arm. Repair of the artery with autogenous vein grafting was impossible due to the extremely brittle brachial artery and accompanying veins. The blood supply distal to the aneurysm was secured by collaterals, and the aneurysm, including a relatively long portion of the brachial artery and veins adjacent to the aneurysm, was resected. The patient died of massive hemorrhage from the subclavian artery of the involved side 9 days postoperatively. Histological and immunohistological examinations of the tissues involved in the ruptured aneurysm were conducted. The resected brachial artery and veins were surrounded by hypertrophied tissue which tested positive for S-100 protein and negative for desmin and action. These findings suggest that the origin of the proliferating tissue was not mesodermal dysplasia, but neurofibroma occurring near or in the vessels. A ruptured aneurysm in a patient with neurofibromatosis should not be treated with reconstruction of the vessels. The treatment of choice is surgical or endovascular occlusion of the vessels involved.     

Intravascular stent and endovascular coil placement for a ruptured fusiform aneurysm of the basilar artery. Case report and review of the literature

The authors demonstrate the technical feasibility of using intravascular stents in conjunction with electrolytically detachable coils (Guglielmi detachable coils [GDCs]) for treatment of fusiform, broad-based, acutely ruptured intracranial aneurysms and review the literature on endovascular approaches to ruptured aneurysms and cerebral stent placement. A 77-year-old man presented with an acute subarachnoid hemorrhage of the posterior fossa. A fusiform aneurysm with a broad-based neck measuring 12 mm and involving the distal vertebral artery (VA) and proximal third of the basilar artery (BA) was demonstrated on cerebral angiography. The aneurysm was judged to be inoperable. Six days later a repeated hemorrhage occurred. A 15-mm-long intravascular stent was placed across the base of the aneurysm in the BA and expanded to 4 mm to act as a bridging scaffold to create a neck. A microcatheter was then guided through the interstices of the stent into the body and dome of the aneurysm, and GDCs were deposited for occlusion. The arteriogram obtained after stent placement demonstrated occlusion of the main dome and body of the aneurysm. The coils were stably positioned and held in place by the stent across the distal VA and BA fusiform aneurysm. Excellent blood flow to the distal BA and posterior cerebral artery was maintained through the stent. There were no new brainstem ischemic events attributable to the procedure. No rebleeding from the aneurysm had occurred by the 10.5-month follow-up evaluation, and the patient has experienced significant neurological improvement. Certain types of intracranial fusiform aneurysms may now be treated by combining intravascular stent and GDC placement for aneurysm occlusion via an endovascular approach. This is the first known clinical application of this novel approach in a ruptured cerebral aneurysm.     

Medullary infarction due to vertebral dissecting aneurysm in a patient with Beh?et's disease]

A 65-year-old man with Beh?et's disease was admitted to our hospital because of an acute left occipital headache and truncal ataxia. He had been treated with prednisolone since the age of 40. MRI showed a hyperintense lesion on T2-weighted images on the left of the medulla oblongata. Left vertebral angiogram showed findings consistent with a dissection at the V4 segment of the artery. After admission, the patient showed marked improvement and was discharged 1 month later without neurological deficits. This is a very rare case of medullary infarction due to vertebral dissecting aneurysm in a patient with Beh?et's disease.     

Dissecting aneurysm of the extracranial vertebral artery causing TIA: a case report

Spontaneous dissecting aneurysm of the extracranial vertebral artery is not frequent. We report a case of dissecting aneurysm of the left extracranial vertebral artery causing TIA. A 44-year-old man was admitted to our hospital with severe occipitalgia of sudden onset on Dec. 21, 1995. On admission, he presented with left hemiparesis and left hemidysesthesia. CT scan and SPECT showed no abnormal findings. His neurological deficits completely improved on the following day. Initial cerebral angiography performed on Dec. 27, 1995 showed pearl and string sign at V3 segment and irregular stenosis at V2 segment suggesting a dissecting aneurysm. We treated conservatively. Repeated angiography performed four weeks after the onset showed resolution of the stenosic lesion and disappearance of the aneurysm. We discussed diagnosis and etiology and treatment of dissecting aneurysm of the extracranial vertebral artery.     

Histological patterns of atherosclerotic plaques in unstable angina patients vary according to clinical presentation

Aneurysms of the cervical vertebral artery (VA) are uncommon; they are often caused by trauma or spontaneous dissection. A fusiform aneurysm without evidence of atherosclerosis or dissection has not been reported previously. A 46-year-old man presented with a pontine infarct. Imaging revealed a fusiform aneurysm of the left VA at the C5-6 level, with occlusion of the basilar artery. Associated minor anomalies included fusion of the vertebral bodies of C5 and C6, cervical rib and platybasia. The left VA arose directly from the aortic arch and entered the transverse foramen at the C4 level. Hyperextension and left lateral flexion of the neck caused kinking of the VA proximal to the aneurysm. Turbulent flow in the aneurysm lumen was noted on angiography.     

A case of middle cerebral artery occlusion caused by dissecting aneurysm

We reported a case of middle cerebral artery occlusion caused by a dissecting aneurysm that was successfully treated by intra-arterial fibrinolysis. A 38-year-old man suddenly developed left hemiparesis and became confused. He was transferred to our hospital one hour and 27 minutes after the ictus. Right carotid angiogram (CAG) revealed aneurysmal dilatation of the horizontal portion of the middle cerebral artery and occlusion of the anterior trunk. Twelve million IU of tissue plasminogen activator was injected to fibrinolyze the thrombus of the occlusion site through a microcatheter. After this, the patient became alert and hemiparesis disappeared. It took three hours and 30 minutes to gain recanalization after the ictus. Right CAG obtained the next day demonstrated the patency of the anterior trunk and the characteristic finding of the dissecting aneurysm viz "double lumen" as a result of fibrinolysis of the thrombus in the false lumen. Right CAG obtained two weeks later demonstrated "string and pearl sign" instead of the "double lumen" as a result of partial thrombosis in the false lumen. Follow-up CAG obtained nine months after the ictus demonstrated marked reduction of the aneurysmal size as the result of progressing thrombosis of the false lumen. The characteristic angiographic findings of the dissecting aneurysm such as "double lumen" and "string and pearl sign" seemed to be mostly influenced by the status of the thrombus in the false lumen. In case of major cerebral arterial occlusion caused by the embolus or thrombus from the dissecting aneurysm, intra-arterial fibrinolysis seems to be a possible treatment modality, even though it is accompanied by the risk of giving rise to subarachnoid hemorrhage.     

Concurrent aneurysm rupture and thrombosis of high grade internal carotid artery stenosis: report of two cases

BACKGROUND: The simultaneous presentation of aneurysmal subarachnoid hemorrhage and thrombosis of a high-grade internal carotid artery stenosis is rare, and their management raises several treatment dilemmas. METHODS: Two such patients with ruptured aneurysms are presented: one with high-grade internal carotid artery stenosis that progressed to occlusion and one with acute internal carotid artery occlusion. RESULTS: Both patients were treated with craniotomy for clipping of the ruptured aneurysm followed by carotid thromboendarterectomy. CONCLUSIONS: We advocate urgent surgical treatment of both lesions, dealing with the most symptomatic lesion first. These two cases demonstrate the importance of reestablishing blood flow in patients with an acutely thrombosed carotid artery.     

Utility and feasibility of assessing myocardial mechanics after bypass

The authors present the case of a 34-year-old man struck over the left mastoid region by a hockey puck, who suffered a fatal rupture of a left vertebral artery berry aneurysm. He became apneic within seconds of the injury and had no brainstem reflex within minutes. The postmortem examination showed massive subarachnoid hemorrhage in the posterior fossa and the remnants of a berry aneurysm near the intradural origin of the left vertebral artery, 11 mm proximal to the posterior inferior cerebellar artery. Rupture of a saccular aneurysm as a result of head trauma is rare. This is the first reported case of a posterior circulation aneurysm rupture as a result of head trauma.     

Concurrent subarachnoid hemorrhage due to ruptured aneurysm and hypertensive intracerebral hemorrhage--case report

A 64-year-old female presented with hypertensive thalamic hemorrhage concurrent with subarachnoid hemorrhage (SAH) due to a ruptured aneurysm manifesting as sudden onset of right hemiparesis followed by severe headache. The aneurysm was located in the basilar artery at the origin of the superior cerebellar artery, remote from the thalamic hematoma. The aneurysm was clipped 3 weeks after SAH. She was discharged with slight right hemiparesis. The method and timing of surgery for such patients depend on hematoma size, location of the aneurysm and hematoma, and neurological status. The intracerebral hemorrhage remote from the ruptured aneurysm should be treated initially if necessary, and the aneurysm clipped after the brain swelling has reduced.     

Circulatory arrest with profound hypothermia during the surgical treatment of large internal carotid artery aneurysm--case report

A 43-year-old male presented with a cerebral aneurysm manifesting as right facial paresthesia, without neurological deficit. Angiography revealed a large aneurysm (22 mm) of the left internal carotid artery. Intravascular treatment using placement of a detachable coil was attempted, but the coil did not stay in the aneurysmal cavity and the procedure was abandoned. The patient did not tolerate the transient balloon occlusion test of the left internal carotid artery. Therefore, the aneurysm was clipped through an open craniotomy with profound hypothermia (20 degrees C) with cardiac arrest (24 minutes). The aneurysmal dome was collapsed, allowing easy dissection of the posterior communicating artery. The closed chest method was used during the extracorporeal cardiopulmonary bypass. Postoperative angiography revealed complete neck clipping with preservation of carotid blood flow. The patient recovered well and resumed his employment. Circulatory arrest with hypothermia provides several benefits for the surgical treatment of large and giant aneurysms.     

Postoperative arteriography in surgery for intracranial aneurysm. Prospective study in a consecutive series of 267 operated aneurysms

OBJECTIVES: We present the results of a prospective study of post-operative angiographic data in a consecutive series of 267 intracranial aneurysms (in 217 patients) operated on by the same surgeon (M.S.). MATERIAL AND METHODS: All patients underwent a preoperative as well as a postoperative angiographic control. Besides, an analysis of the operative reports was done in order to correlate the surgical and angiographic data. RESULTS: Out of 267 operated aneurysms, 257 (96%) were located in the anterior circulation, and 10 (4%) in the posterior circulation. The surgical clipping was considered incomplete in 17 aneurysms (6.3%). Aneurysmal remnants were classified in 5 grades; grade I: remnant less than 50% of the neck size; grade II: remnant more than 50% of the neck size; grade III: remnant of a multilobed aneurysmal sac; grade IV: residual sac less than 75% of the aneurysmal size; and grade V: residual sac more than 75% of the aneurysmal size. The analysis of the operative reports helped in understanding the favoring factors of incomplete clipping: large neck and/or huge sized sac, insufficient exposure and dissection of the neck. The presence of a collateral artery with a large infundibulum in the vicinity of the neck-implantation zone on the parent artery. CONCLUSIONS: In most cases the surgeon can easily control peroperatively under the microscope, after puncture-evacuation of the sac, the watertightness of clipping and the absence of any residual neck or sac of the aneurysm. Therefore the remaining place for a postoperative arteriography can be limited to those cases when the surgeon has some doubt concerning the perfection of clipping, as well as for giant and/or "difficult" aneurysms. A re-operation or a complementary endovascular treatment can be discussed for remnants in graded III, IV or V. Knowledge concerning the percentages of aneurysm with neck remnant only and of aneurysms with sac remnant obtained by surgery is interesting at the present time when endovascular treatment is becoming popular. In our series they amounted at 4.1% and 2.2%, respectively. These percentages are those of a series comprising all types of aneurysms. Needless to say, that the percentage of incomplete occlusion will be less if only the aneurysms with small-sized neck were taken into account.