The World Federation of Interventional and Therapeutic Neuroradiology.
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Humans↗
Biomedical subjects
G Rodesch
Publications and source records attributed to G Rodesch.
[Pupil-sparing painful oculomotor neuropathy due to a carotico-cavernous aneurysm].
INTRODUCTION: In third cranial nerve palsy, the lack of mydriasis evokes a noncompressive mechanism. Case report. We report a case of a slightly painful, pure extrinsic third-nerve palsy, complete except for the partial ptosis secondary to the compression by an intracavernous carotid aneurysm. Percutaneous endovascular embolization was followed by complete regression of the palsy within 4 weeks. The pupil may have been spared by the mechanism of compression after the separation of the pupillomotor and extrinsic fibers or the ischemia of the third nerve by its arterial trunk lesion. CONCLUSION: This case report underlines that brain radiological explorations are necessary in some third-nerve pupil-sparing palsies.
Aged↗
Hereditary hemorrhagic telangiectasia in children: endovascular treatment of neurovascular malformations: results in 31 patients.
Hereditary hemorrhagic telangiectasia (HHT) is a heterogeneous disease that can present with a variety of clinical manifestations. The neurovascular complications of this disease, especially in children, may be potentially devastating. The purpose of this article was to review the therapeutic results of endovascular treatment of neurovascular malformations in children. A total of 31 patients under the age of 16 were included in this retrospective analysis. All children were treated in a single center. Twenty children presented with 28 arteriovenous (AV) fistulae, including seven children with spinal AV fistulae and 14 children with cerebral AV fistulae (one child had both a spinal and cerebral fistulae). Eleven children had small nidus-type AV malformations. All embolizations were performed employing superselective glue injection. Follow-up ranged between 3 and 168 months (mean 66 months). A total of 115 feeding vessels were embolized in 81 single sessions, resulting in a mean overall occlusion rate of the malformation of 77.4% (ranging from 30 to 100%). Two of 31 patients (6.5%) died as a direct complication of the embolization procedure; two patients (6.5%) had a persistent new neurological deficit; eight patients (26.7%) were clinically unchanged following the procedure; in 13 patients (41.9%) an amelioration of symptoms but no cure could be achieved; and six patients (19.4%) were completely asymptomatic following the endovascular procedure. In the surviving patients morphological complete occlusion was possible in twelve patients (38.7%); therapy is still not completed in six patients. Since the natural history of neurovascular manifestations of HHT in children is associated with high morbidity and mortality, therapeutic intervention is mandatory. In most instances a morphological target can be identified; therefore, even partial and staged treatment can be performed. Our results demonstrate that in 27/31 patients these targeted interventions resulted in stabilizing the disease, ameliorating the symptoms or even complete resolution. The endovascular approach employing glue as the embolizing agent represents a safe and efficient way to control the neurovascular phenotypes of HHT.
Adolescent↗
Neurovascular phenotypes in hereditary haemorrhagic telangiectasia patients according to age. Review of 50 consecutive patients aged 1 day-60 years.
Hereditary haemorrhagic telangiectasia (HHT) is inherited as an autosomal dominant trait with varying penetrance and expressivity. Some of the most devastating consequences of this disease result from cerebral vascular malformations that manifest themselves in either arteriovenous fistulae (AVF), small nidus-type arteriovenous malformations (AVM) or micro-AVMs with a nidus less than 1 cm in size. The purpose of this study was to compare the phenotypes of CNS-manifestations of HHT with the age of the patient. The charts and angiographic films of 50 patients diagnosed with HHT according to the Curaçao criteria were retrospectively evaluated concerning age of onset of symptoms, or, if not applicable of first consultation. The files were reviewed for clinical presentation, family and personal history, while the patients' angiograms were analysed with respect to the number of lesions (single and multiple), the location (superficial supratentorial, deep supratentorial, infratentorial, and spinal), and type of lesion (fistulous AVM, nidus-type AVM, and micro-AVM). A total of 75 central nervous system manifestations of HHT were found. Lesions included seven spinal cord AVFs that were all present in the paediatric age group (mean age: 2.2 years), 34 cerebral AV fistulae, all but two affected patients were less than 6 years (mean age 3.0). Sixteen nidus type AVMs (mean age: 23.1 years) and 18 micro-AVMs (mean age: 31.8 years) were found. HHT displays an age-related penetrance of clinical manifestations. Since members of the same family can present with completely different phenotypes of this disease there seems to be no relationship between the type of mutation and the phenotype of the disease. Since there seems to be a continuum of vascular abnormalities (from large fistulous areas to small AVMs and micro-AVMs) associated with HHT, the most likely determinating factor of the HHT phenotype is the timing of the revealing event in relation to the maturity of the vessel. Presumably, the trigger of the quiescent genetical abnormality transforms a "dormant" disease into a morphologically and therefore clinically detectable one by impairing a specific vessel segment at a specific (more or less vulnerable) period of time. The nature of this triggering event is, however, as of yet unclear.
Adolescent↗
Supratentorial cerebral arteriovenous fistulas (AVFs) in children: review of 41 cases with 63 non choroidal single-hole AVFs.
PURPOSE: In this article we explore the various aspects of the supratentorial Single-Hole AVFs (ST AVFs) in children, focusing on their clinical features, angio-architecture, treatment indications and the role and results of endovascular management. MATERIALS AND METHODS: Among 1565 cases of brain AVMs seen at our neurovascular center, 620 cases were seen in the pediatric age group (<or= to 15 year-old) and 303/620 were non-Galenic brain AVMs. Among 52 brain AVFs found in children, we reviewed 41 pediatric patients with supratentorial single-hole cerebral AVFs (M/F=28/13) with a total of 63 AVFs. Mean age at presentation was 24 months. Four cases had their lesion diagnosed in utero without particular prognostic significance. 17 cases were multifocal with a total of 39 AVFs. The most common clinical presentation leading to diagnosis was cardiac sufficiency in (31.7%), epilepsy (24.4%), macrocrania (14.6%). Venous ectasia (87.8%) and pial venous stenosis (41.5%) were the most frequent angiographic features. There were 11 cases of Hemorrhagic Heriditary Telangiectasia (HHT1) (including two suspicious cases) among the 41 patients (26.8%) and among those 4 cases had single AVFs and 7 cases had multiple AVFs. RESULTS: Thirty-five children were treated in our institution: 34/35 by embolization alone, 1/35 by a combination of embolization and radiosurgery. 32/35 patients were treated with glue alone, 3/35 with coils (2/3 with glue also). A total of 57 separate lesions were treated. On clinical follow up (from 1985-2002, mean 4.2 years), 88.6% of patients proved to be either asymptomatic, improved from previous clinical symptoms or stabilized. 40% of patients had their lesion(s) already completely excluded. Post operative mortality was 5.6% (2/35), permanent neurological morbidity 3% (1/33). Twenty-six of these 33 patients (78.6%) were neurologically normal. 5/33 patients continue to have neurological symptoms or retardation. 2/33 patients died during follow up (1 ischemic stroke from Pulmonary AVF, 1 death despite partial embolisation). CONCLUSION: Cerebral arteriovenous fistulae are a rare disease, but not infrequently seen in neonates and infants with AVMs. In one fourth of these patients HHT is suspected to be present. The AVFs are always superficial and fed by pial (cortical) arteries. They seldom reveal a hemorrhagic event. They are similar to those encountered in the posterior fossa or spinal cord. Endovascular treatment using NBCA was the treatment modality chosen resulting in a high rate of success and allowing children to grow up normally with no hemorrhages on follow up and no new symptom other than those already present on admission.
Adolescent↗
Posterior cranial fossa single-hole arteriovenous fistulae in children: 14 consecutive cases.
We report 14 consecutive children with 23 posterior cranial fossa arteriovenous fistula (AVF); six had multifocal lesions, involving the supratentorial brain in three and the spinal cord in one. There were two boys and four girls with a family history compatible with hereditary haemorrhagic telangiectasia. The diagnosis was made in infancy in eight cases and in a further six before the age of 12 years; mean age at diagnosis was 3.5 years. The male-to-female ratio was 1.8:1. Presenting features were macrocrania in four cases, haemorrhage or headache in three and nonhaemorrhagic neurological deficits or and cardiac overload in two. Dominant supply to the symptomatic fistula arose from the posterior inferior cerebellar artery in five cases, anterior inferior cerebellar artery in two and the upper basilar artery system in seven. All children were primarily treated by transarterial embolisation. We treated thirteen children (93%) by transarterial embolisation alone; one older child with a history of haemorrhage also underwent radiosurgery. We obtained 100% exclusion of the fistula(e) in six children, 95-80% in five, 80-50% in one and <50% in one. Of the incompletely treated cases, three had conservative management, and two with 80% and one with 60% reduction of their lesion are scheduled for elective treatment; two partially treated case died. There was no morbidity due to the endovascular procedures. Follow-up since referral is 6 months-10 years (mean 4.5 years). Ten children are neurologically normal, two have persistent (pre-existing) neurological deficits and two are dead.
Arteriovenous Fistula↗
Angio-architecture of spinal cord arteriovenous shunts at presentation. Clinical correlations in adults and children. The Bicêtre experience on 155 consecutive patients seen between 1981-1999.
OBJECTIVE: To analyse possible relationships between the symptoms and signs created by intradural spinal cord arteriovenous shunts (SCAVSs) and their angio-architecture. METHODS: The clinical and radiological files of 155 patients, both adults and children, seen between 1981 and 1999 with a SCAVS were retrospectively reviewed. SCAVSs were divided into nidus type (small superficial or large intramedullary), and fistulas (micro (mAVFs)- or macrofistulas (MAVFs)). The angio-architectural points studied were arterial stenosis, proximal arterial aneurysm, distal arterial aneurysm, arteriovenous fistulas, pial venous drainage and/or reflux, venous ectasias, venous stenosis, venous thrombosis, false aneurysms. Correlations between architecture, location, age groups and symptoms and signs were made. FINDINGS: A male predominance was noted in both adults and children. Most of the lesions were located at the thoracic level, but hemorrhage occurred as the presenting event predominantly in cervical localizations. 30 patients (20 AVMs; 4 mAVFs, 6 MAVFs) were children. 5 of the 6 MAVFs found in children were associated with Hereditary Hemorrhagic Telengiectasia (HHT1). Twenty one children (70%) had bled. Spontaneous total or subtotal recovery was the rule in 15 of those who had bled (72%) but early recurrent hemorrhages occurred in 2. Among 125 adults 56 presented with hemorrhage (45%). The proportions of those who recovered spontaneously (71%) or rebled (3,6%) in the same year after the initial episode were very similar to children. All other adults presented with acute or progressive non-hemorrhagic episodes. There was no significant difference in the angio-architecture between hemorrhagic and non hemorrhagic SCAVS, except for that occurrence of pseudo-aneurysms. Hemorrhage in SCAVSs may not be exclusively due to haemodynamic factors. Venous congestion was responsible for progressive symptoms. Acute deficits unrelated to bleeds were due to intralesional thrombosis or hemodynamic changes. INTERPRETATION: The short term prognosis of hemorrhagic SCAVSs is good, and there is no need for emergency treatment. MRI delineates the cord and makes the diagnosis of the lesion but angiography remains the gold standard for analysis of the vasculature. The angio-architecture reflects the ageing of the lesion but there is not a precise correlation between angio-architecture and clinical symptoms, except for pseudoaneurysms which relate to the hemorrhagic portion of the lesion.
Adolescent↗
PHACES syndrome: a review of eight previously unreported cases with late arterial occlusions.
PHACE and PHACES are acronyms for a syndrome of variable expression comprising posterior cranial fossa malformations, facial haemangiomas, arterial anomalies, aortic coarctation and other cardiac disorders, ocular abnormalities and stenotic arterial disease. We review five girls and three boys aged 1 month-14 years with disorders from this spectrum. Six had large facial haemangiomas but recent reports suggest that small haemangiomas may occur; hence our inclusion of two possible cases. We also focus on the recently recognised feature of progressive intracranial arterial occlusions, present in four of our patients, later than previously recognised, from 4 to 14 years of age. We suggest that many elements of this disorder could reflect an abnormality of cell proliferation and apoptosis.
Adolescent↗
Late spinal dislocation after treatment of spinal arteriovenous malformation. A case of Charcot spinal arthropathy.
Neuropathic arthropathy of the spine is a destructive condition of the spine which is secondary to a loss of the protective proprioceptive reflexes. In the majority of cases, it occurs in patients who have suffered from traumatic medullary lesions and is responsible for destruction of the vertebral bodies and considerable spinal deformity. We report a case of neurogenic lumbar arthropathy in a patient with a spinal arteriovenous malformation. This vascular lesion caused considerable disturbances of proprioception. The course was favorable with regard to the deformity after correction and fusion by posterior approach.
Adult↗
Vein of Galen aneurysm malformation (VGAM): closing the management loop.
We report a case where an antenatal scan showed a cerebral vessel dilatation consistent with a vein of Galen malformation (VGAM). After delivery the diagnosis was confirmed on MRI scan. The infant's condition was stable in relation to the two important clinical indicators, mainly not in congestive cardiac failure and no hydrocephalus. The vein of Galen assessment score as used in Hopital Bicetre was 20/21. The scoring system is based on a multiorgan assessment including the five parameters cardiac, cerebral, respiratory, hepatic and renal (Table 1). A score of < 8 indicates no treatment, 8-12 means immediate intervention while a score > 12 delays intervention to circa six months of age. At four months the head circumference accelerated with hydroceaphalus on scans and the infant became clinically symptomatic. Urgent embolisation was performed. The procedure uses N-butylcyanoacrylate (NBCA) glue as the embolisation agent, which in this case was inserted through the left posteriomedial choroidal artery. Complete obliteration was achieved. The infant subsequently required insertion of a ventriculoperitoneal shunt for the hydrocephalus component. The infant has had no recurrence. He remains well on objective examination he has mild developmental delay. In summary this case highlights the value of antenatal diagnosis,the VGAM assessment score to determine the optimal time of intervention and the effectiveness of embolisation.
Adhesives↗
Facial hemangioma and cerebral corticovascular dysplasia: a syndrome associated with epilepsy.
The authors performed imaging studies in two children with epilepsy and congenital facial hemangioma. The first patient had dysplastic pericallosal arteries and frontal polymicrogyria. In the second patient, dysplastic arteries and dysplastic cortex lined the interhemispheric fissure, the dysplastic cortex bridging across the midline, which resulted in holoprosencephaly. Abnormal cortical development may underlie epilepsy in children with facial hemangioma.
Adolescent↗
Spinal cord arteriovenous shunts: from imaging to management.
Spinal cord arteriovenous shunts (SCAVSs) are either fistulas or niduses that can be separated in four different groups according to their localization and relationship to the dura. Paraspinal AVSs are located outside the spine and are responsible for neurological symptoms because of cord compression by ertatic veins, venous congestion or arterial steal. Epidural shunts are located in the epidural space and drain in epidural veins with secondary intradural congestion. Dural shunts are embedded in the dura, produce a cord venous myelopathy after draining through veins that either pierce the dura far from a nerve root or accompany a nerve root. Intradural shunts affect the cord, the roots or the filum. Additionally, they can be classified according to their potential relationships with genetics, vascular biological features and angiogenesis into genetic hereditary lesions (hereditary hemorrhagic telangiectasia), genetic non-hereditary lesions (multiple lesions with metameric links) and single lesions (AVMs or micro AVFs). MRI and MRA are able to visualise SCAVS early after the onset of clinical symptoms. The type of shunt and its localization may remain difficult to be precise. Angiography remains the gold standard for analysis of the anatomical, morphological and architectural features necessary for therapeutic decisions in both paediatric and adult populations. In our series, embolisation is chosen in first intention whatever the type of shunt responsible for the clinical symptoms and glue is preferably used. In paraspinal, dural or epidural arteriovenous shunts, the goal of treatment should be complete closure of the shunt. A complete cure by embolization is rather easily achieved in paraspinal lesions. Failure of endovascular therapy in dural or epidural shunts must bring the patient to surgery. The prognosis of most intradural shunts seems better than previously thought, even after haemorrhage. In intradural spinal cord arteriovenous shunts, embolisation targeted towards the portions of the malformation felt to be responsible for the symptoms (venous congestion) or pointing to the point of rupture (false aneurysms) of the malformation, allows restoration of a new hemodynamic equilibrium between the malformation and the cord itself. Such targeted treatment offers long-term stabilisation or improvement to patients suffering from SCAVSs and good protection against (re) haemorrhages, with an acceptable morbidity. Cure of the shunt is not imperative to obtain these satisfactory outcomes. The clinical results obtained by such management compare favourably with those obtained by neurosurgery.
Angiography↗
Cerebrofacial vascular disease.
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Abnormalities, Multiple↗
Severe cardiac failure in newborns with VGAM. Prognosis significance of hemodynamic parameters in neonates presenting with severe heart failure owing to vein of Galen arteriovenous malformation.
OBJECTIVE: Neonatal vein of Galen malformation complicated by severe cardiac failure is a rare disease. The purpose was to assess the outcome of this life-threatening malformation and identify hemodynamic prognostic factors. DESIGN: Retrospective study. PATIENTS: Twenty-four newborns with cardiac failure requiring mechanical ventilation were consecutively admitted from 1986 to 2000. INTERVENTIONS: Cardiovascular evaluation including echocardiogram was performed in all cases. Eighteen transarterial shunt occlusions with glue were applied by the same team of three physicians. MEASUREMENTS AND RESULTS: Twelve babies survived and underwent one endovascular session at least (median age 20 days) with a mean 63 months follow-up. Embolization was not performed in 6 of the 12 nonsurvivors because of severe brain damage or profound hypotension Cardiogenic shock occurred in all nonsurvivors, but also in one long-term survivor (p<0.0001). Echocardiogram showed signs of right ventricular failure, most often in the dead babies (p=0.005). The pulmonary systemic arterial pressure ratio was significantly higher in the nonsurvivor group (p=0.031), and it decreased significantly after the first embolization only in patients who survived (p=0.01). Patent ductus arteriosus and a diastolic aortic reversed-flow were present in all nonsurvivors in contrast to 30% of the long-term survivors (p=0.003 ). There was no difference in the left ventricular contractility and mean cardiac output between the two groups. CONCLUSIONS: The outcome of vein of Galen malformation complicated by severe cardiac failure requiring mechanical ventilation remains poor. Neonatal embolization seems to be beneficial only in babies without suprasystemic pulmonary hypertension.
Arteriovenous Malformations↗
Effect of partial targeted N-butyl-cyano-acrylate embolization in brain AVM.
BACKGROUND: The management of cerebral arteriovenous malformations needs effective treatments. So far, no study has shown that partial targeted embolization treatment (PTET) reduces the risk of intracranial hemorrhage with respect to the natural history of the malformation. METHODS: The pre-treatment and post-treatment-initialization hemorrhage incidences of neuro-interventional patients were compared. Two hundred fifteen patient years from 519 patients were used to observe the short term course of the untreated disease. Five hundred patient years from 326 patients were used to observe hemorrhage after the start of treatment. The Kaplan-Meier estimator of hemorrhage free time under treatment was compared with results in the literature. Confounding influences resulting from selection processes or the disease parameters were studied. RESULTS: The yearly hemorrhage incidence rate of all untreated patients was observed as 0.089 (95% CI [0.053, 0.138]). This rate was 0.052 (95% CI [0.019, 0.114]) in the subgroup of patients who underwent PTET later. In the same group the observed annual rate after the start of PTET was 0.036 (95% CI [0.021, 0.057]). Crawford's results about intracranial hemorrhage during the natural course show the lowest risk values compared to other published studies [3]. There was a significant difference between the Crawford's reference data and the ICH incidence after the start of PTET in the neuro-interventional population (p=0.037). The morbidity risk in treated patients was 5.3% for a transitory and 2% for a persisting neurological deficit. Mortality results were compared with those of Crawford. CONCLUSION: The neuro-interventional patients under study show a lower hemorrhage risk than the population studied by Crawford. A significant superiority with respect to hemorrhage risk is established two years after the start of the PTET treatment.
Adolescent↗
[Natural evolution of an aneurysm of the V4 segment of the vertebral artery into a giant aneurysm. A case report].
We report the case of a 78-year-old female with an aneurysm of the V4 segment of the vertebral artery and a six-year follow-up without any surgical or endovascular treatment. The natural evolution of a small aneurysm into a giant aneurysm is rarely observed. We discuss the pathophysiology of the development and growth of such aneurysms through a review of the literature.
Aged↗
Intracranial haemorrhage within the first two years of life.
BACKGROUND: Spontaneous intracranial haemorrhage is not common in infants, with differences from adults in both aetiology and severity. The infantile CNS is more vulnerable because of incomplete hydrovenous maturation. We analyzed infantile intracranial haemorrhage mainly caused by structural brain lesions and discuss specific aetiologies with regard to haemodynamic characteristics. SUBJECTS AND METHODS: We reviewed 20 infants less than 2 years of age from a total of 328 neonates and infants with intracranial vascular lesions seen in our institution since 1985. Associated or causative lesions were arteriovenous malformation (AVM) in 6, dural sinus malformation (DSM) in 4, arteriovenous fistula (AVF) in 3, aneurysm in 2, developmental venous anomaly (DVA) in 1, vein of Galen malformation (VGAM) in 1, and others in 3. The locations of haematomas were intracerebral (ICH) in 8, combined ICH and intraventricular haemorrhage (IVH) in 5, IVH alone in 5, subarachnoid haemorrhage (SAH) in 1, and combined SDH and ICH in 1. FINDINGS: Three patterns of haemorrhage were noted in high-flow vascular lesions such as AVM or AVF (n=9); haemorrhage at the site of nidus or fistula corresponding to nidal pseudoaneurysm in 4, regional venous hypertension with pial venous reflux in 3, global venous infarction causing multifocal haemorrhage in distant brain areas in 2. Aneurysmal bleeds were caused by dissecting aneurysms at the level of dural penetration of cranial vessels. One infant had haemorrhage near a DVA without evidence of cavernous malformation suggesting the possibility of venous ischaemia. IVH was associated with shunt operations in 4 infants with DSM, and SDH followed by ICH in a infant with VGAM. INTERPRETATION: Spontaneous intracranial haemorrhage in infants and neonates is rare; it is associated with specific lesions which show some differences from their adult counterparts. The vein-related causes of hemorrhage are largely the pathophysiologic characteristics in this age group. Absence of hemorrhage in VGAM is remarkable in addition to occurrence of most hemorrhages after shunting.
Arteriovenous Fistula↗
Gamma knife outcome models as a reference standard in the embolisation of cerebral arteriovenous malformations.
BACKGROUND: We sought to utilise outcome models from Gamma Knife radiosurgery (GKRS) to cerebral arteriovenous malformations (AVM) as a reference standard in assessing the clinical outcome of embolisation, thus comparing the outcomes of two different management alternatives, in the same patients. METHODS: 87 consecutive patients with 88 AVM were admitted during 1997-1999 for initial embolisation of an AVM. The clinical outcomes were recorded prospectively. Angiography under stereotactic conditions with measurement of AVM volume was performed before and after embolisation. GKRS outcome models were used to predict obliteration rate, complication rate and risk of haemorrhage before and after embolisation. The clinical outcome of embolisation followed by predicted outcome of adjunct GKRS was then compared with the predicted outcome of GKRS as the only treatment. FINDINGS: Eight patients were subjected to microcatheterisation but not to embolisation. By the end of the study period, embolisation had been terminated in 55 patients out of 80 (69%). The predicted outcome of GKRS alone was 58 obliterations and 12 complications while that of the combined management was 58 obliterations and 15 complications. The difference was not significant on the p < 0.1 level. INTERPRETATION: Volume measurement from angiography and outcome models from Gamma Knife radiosurgery are useful as a reference standard in the management of AVM. Absolute volume reduction from embolisation is most prominent for AVM > 10 ml and thus facilitates subsequent radiosurgery. For AVM < or = 10 ml, GKRS as the only treatment can be an alternative to primary embolisation, particularly if no significant volume reduction or obviously beneficial effect of targeted embolisation is expected. Further prospective studies are needed to identify subgroups in which one treatment has advantages over the other.
Adolescent↗