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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 (

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We report a case of symptomatic symmetrical, bilateral absence of the cavernous internal carotid (ICA) and transdural vertebral artery (VA) segments with formation of a rete mirabile. There have been similar reports in Asian patients; ours is the first in the Western population. A 29- year-old woman presented with recurrent temporary blindness and an episode of minor subarachnoid haemorrhage. Angiography demonstrated absence of the C5 and C6 segments of the ICA and the C1/2 segments of the VA bilaterally, with typical carotid rete mirabile formation to reconstitute the distal flow. Comparative anatomy and embryology show retia in several species and point to their secondary origin. The anomalies of the ICA cannot be called agenesis but rather absence since they are likely to result from perinatal disappearance of the artery rather its failure to develop.

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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.

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SUMMARY: Cerebral arteriovenous malformations (CAVM) can be associated with Hereditary Haemorrhagic Telangiectasia (HHT), a dominantly inherited vascular disorder with variable penetrance and expressivity. The presentation and angiographic features were analysed retrospectively. The purpose is to point to special groups of AVM patients within the overall CAVMs and to discuss the issue of screening. We reviewed 34 cases of HHT-related CAVM from the data bank in Bicêtre from 1985-2003. In Spinal cord AVM (SCAVM) there were 194 patients with 5 HHT. HHT was diagnosed when at least two criteria were met; cutaneous telangiectasia, epistaxis, visceral AVMs, angiographic findings of AVF and first degree family history. Intracranial haemorrhage was the presenting symptom in 8.8% and the risk of haemorrhage in the natural history was 0.7% per year. The commonest angiographic features in adults are nidus(81.8%) and multiplicity(45.5%), while in the paediatric group venous ectasia and giant pouches(91.3%), AVF(69.6%) and multiplicity( 52.2%). In spinal cord lesions macrofistulas are demonstrated in 83% of HHT with no multiplicity. HHT-related CAVMs present as multiple lesions, cortical in location, micro AVMs or AVF. HHT in SCAVM is expressed as single macro AVF, especially in the paediatric group. AVF in children are highly suggestive of HHT. We do not recommend screening in HHT adult patients for CAVM, while in the paediatric population, screening could be recommended at six months of age for cerebrospinal localization. These patients should be screened for Pulmonary AVF, which needs to be treated in priority.

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UNLABELLED: Abstract: Background and Purpose. Dural Arteriovenous Shunt (DAVS) in children include Dural sinus malformation (DSM), infantile and adult types. They are rare and seldom reported. Our purpose was to highlight the angiographic features of the DSM sub group for prognosis of clinical evolution and outcome and to lay guidelines for management. METHODS: From a dedicated neurovascular data bank, there were 52 cases of arteriovenous dural shunts in children from 1985 to 2003. Of these, there were 30 patients with DSM, which we analysed the various angioarchitecture, presentation and neurological outcome. Children clinical status was evaluated and scored at admission and follow up. Results. There was an overall male dominance of 2:1. Antenatal diagnosis was obtained in 8/30 (26.7%) cases. Mean age of diagnosis was 5 months. Mean age at first consultation was 8.7 months. No patient was diagnosed during childhood. The most common clinical presentations were macrocrania 76.7%, seizures 23.3% and mental retardation 23.3%. In 14/30 (35.7%) of the patients, the therapeutic decision was to manage conservatively; in 5/14 (30.7%) with predictable favourable evolution and in 9/14 (64.3%) with irreversible poor neurological outcome. In the remaining 16/30 (53.3%) patients, endovascular treatment was performed. In 12/16 (75.0%) patients the neurological outcome was good, 3/16 (18.8%) patients had unfavourable evolution despite embolization. There was no morbidity mortality related to the procedures themselves. 1/16 (6.3%) patient was lost to follow-up. Overall 12/29 (45.8%) patients had an unfavourable neurological outcome with 11 patients dead and 1 with severe neurological deficit. In the surviving group of children, 17/18 (94.4%) have a good neurological outcome; in 10/18 (55.5%) the lesion is morphologically excluded. Conclusion. DSM is rare disease with high mortality. They usually proceed to either total or partial spontaneous thrombosis before the age of 2 thus compromising normal cerebral venous drainage. DSM away from the torcular, good cavernous sinus, cavernous capture of sylvian veins, absence of pial veins, straight sinus or superior sagital sinus (SSS) reflux and absence of jugular bulb dysmaturation represent factors of good prognosis. Such patients will highly benefit for endovascular treatment. In partial endovascular approach the aim being is to separate the brain drainage from DSM drainage. This will be achieved by the transarterial approach to the associated mural arterio-venous shunts (AVS) and by disconnecting the pial reflux by transvenous route.

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SUMMARY: We describe the case of a three-year-old Danish boy born at term by Cesarean due to cardiac insufficiency in the last trimestre of intra-uterine life for which he is being treated with cardiac drugs. At birth, he was noted to have bilateral ocular malformations on the upper eyelids, and diffuse scalp lipoma and alopecia. Due to the retarded growth (score - 3D) he had a MRI and angiography which demonstrated a high flow fistula on the basilar tip artery and another one on the MCA branch treated by endovascluar approach. Intracranial arachnoid cysts, dysplastic cortex, ventricular enlargement and lipoma were noted too, establishing the diagnosis of Encephalocraniocutaneous Lipomatosis Syndrome, a rare disease, especially in bilateral presentation. To our knowledge this is the first observation of ECCL associated with intracranial pial arteriovenous fistulas.

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SUMMARY: The purpose of the paper is the follow-up of embolised intradural saccular Arterial Aneurysms (AA), excluding giant, dissecting, inflammatory, fusiform or AA associated to BVAM. Since its introduction in 1991, the Guglielmi Detachable Coil has offered protection against aneurysmal rebleeding in the critical few days and months after SAH regardless of the grade. A number of questions remain: is complete angiographic obliteration necessary at first embolisation? What duration of clinical / angiographic follow-up (FU) is required to ensure the risk of haemorrhage has been eliminated? What is the long-term protection against rebleeding? One hundred and two patients with 160 intradural saccular AA embolised before april 1997 were selected for this study. They had at least 5-yrs clinical FU, of which 22 patients had a mid- term (3 years) and 45 patients had a 5-year or more angiographic FU (mean 67,7 months per patient). Twenty-eight embolised AAs with 100% occlusion at 1 year, remained unchanged on the 5-year angiograms. A further 14 patients with complete occlusion at 1 year showed persisting complete occlusion on angiogram at 3-years FU, which in our series means that complete occlusion after the first year post-embolisation implies that the aneurysm will remain completely occluded. All secondary spontaneous thromboses (27.6% of cases), occurred during the first year pos- embolisation. In six patients with subtotal or partial occlusion no change was seen for three consecutive years of FU; none showed later change at 5-year angiography. Below 80% occlusion our series does not provide enough information but we consider the situation instable. No mortality related to the procedure was observed in the unruptured AA group.No bleeding or re-bleeding has occurred since the beginning of our experience (1993) in saccular AA treated by GDC-Coil. Coil-embolisation of properly selected patients is effective in protecting against bleeding or re-bleeding at short and long-term with stable morphological results provided a strict follow-up control is established at short term.

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SUMMARY: This is an unusual case report of an infant, who initially presented with a facial haemangioma and was later diagnosed to have a dural sinus malformation (DSM) involving the torcula. The DSM increased in size lateralising to the right transverse sinus at three months of age. Postnatal enlargement of the dural sinus has not been described before suggesting a delay in the maturation of the dural sinus which normally would occur antenatally. There was a further association with a complex developmental venous anomaly (DVA) draining the right cerebral hemisphere into the deep cerebral vein and multiple cavernous malformations. The DVA was not clearly demonstrated at age one month but was more obvious at age three months. This would be the first reported case of DSM associated with a DVA. Increasing venous hypertension probably contributed to the poor opacification of the DVA on follow-up angiography at age six months and to the haemorrhagic changes within the cavernomas on magnetic resonance imaging (MRI). The therapeutic goal was to correct venous hypertension by partially embolising the dural shunts to remodel the cerebral vasculature and preserve the patent sinus. The treatment strategy and possible link between the complex disease entities presented in this infant are discussed. Despite these attemps, the lesion continued to grow compressing the posterior fossa structures. The infant died at nine months of age.

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S-Adenosylhomocysteine (AdoHcy) hydrolase catalyzes the reversible hydrolysis of AdoHcy to adenosine (Ado) and homocysteine (Hcy), playing an essential role in modulating the cellular Hcy levels and regulating activities of a host of methyltransferases in eukaryotic cells. This enzyme exists in an open conformation (active site unoccupied) and a closed conformation (active site occupied with substrate or inhibitor) [Turner, M. A., Yang, X., Yin, D., Kuczera, K., Borchardt, R. T., and Howell, P. L. (2000) Cell Biochem. Biophys. 33, 101-125]. To investigate the binding of natural substrates during catalysis, the computational docking program AutoDock (with confirming calculations using CHARMM) was used to predict the binding modes of various substrates or inhibitors with the closed and open forms of AdoHcy hydrolase. The results have revealed that the interaction between a substrate and the open form of the enzyme is nonspecific, whereas the binding of the substrate in the closed form is highly specific with the adenine moiety of a substrate as the main recognition factor. Residues Thr57, Glu59, Glu156, Gln181, Lys186, Asp190, Met351, and His35 are involved in substrate binding, which is consistent with the crystal structure. His55 in the docked model appears to participate in the elimination of water from Ado through the interaction with the 5'-OH group of Ado. In the same reaction, Asp131 removes a proton from the 4' position of the substrate after the oxidation-reduction reaction in the enzyme. To identify the residues that bind the Hcy moiety, AdoHcy was docked to the closed form of AdoHcy hydrolase. The Hcy tail is predicted to interact with His55, Cys79, Asn80, Asp131, Asp134, and Leu344 in a strained conformation, which may lower the reaction barrier and enhance the catalysis rate.

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OBJECTIVE: Impacted foreign bodies in the trachea and esophagus are common. In the esophagus, these are usually bones, while for the trachea, it is commonly some form of seed or nut. The incidence of impacted dental prostheses is not highlighted in the literature. They usually have a definite history of ingestion, frequently during trauma, seizures, or sleep. Prompt management in a safe and effective manner is required if significant morbidity, and even mortality, is to be avoided. METHOD: The records of all patients who presented to the University Hospital in Kuala Lumpur with a definite history of foreign-body ingestion in the form of a dental prosthesis were reviewed, and their respective radiographs reassessed. RESULTS: There were 21 patients with impacted dental prostheses from a total of 200 patients who had impacted foreign bodies (11.5%). Indirect laryngoscopy detected five of these dentures, and in only seven were the dentures seen on plain radiography (33%)-all of which had metal wires attached. Of the 21 patients, 16 had the foreign bodies removed endoscopically; 2 could not be removed and were allowed to pass through the gastrointestinal tract under close monitoring; and 3 whose dental prostheses were not detected and who subsequently passed the foreign bodies. CONCLUSIONS: Impacted dental prostheses appear to constitute a significant proportion of impacted foreign bodies in the orolaryngopharynx. The majority are radiolucent. In the presence of positive history or/and symptoms, endoscopic examination and removal is suggested if possible to reduce morbidity. No serious complications have been seen.

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