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1/51. scalp arteriovenous malformation draining into the superior sagittal sinus associated with an intracranial arteriovenous malformation: just a coincidence? Case report.

    OBJECTIVE AND IMPORTANCE: Recent experimental and clinical evidence suggests that hemodynamic changes in the venous system can induce the formation of new arteriovenous malformations (AVMs). In a rat model, increased venous pressure induces the formation of soft tissue and dural AVMs. We report a clinical observation that may support these data. CLINICAL PRESENTATION: A 4-year-old boy with a midline scalp AVM draining into the superior sagittal sinus had an associated intracranial/parenchymal AVM. The cerebral AVM increased venous pressure in the superior sagittal sinus as revealed by angiography. INTERVENTION: The scalp AVM was resected, and the intracranial AVM was treated by use of the gamma knife. CONCLUSION: On the basis of reported experimental data and the morphological and hemodynamic characteristics in this patient's two lesions, we suggest that the scalp AVM might have been induced by hypertension in the superior sagittal sinus. This clinical observation supports the notion suggested by experimental studies that hemodynamic changes can induce the formation of associated AVMs.
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2/51. Circumferential flow reduction during percutaneous embolotherapy of extracranial vascular malformations: the "cookie-cutter" technique.

    We describe a simple, inexpensive, and very effective method of achieving circumferential flow reduction during direct percutaneous cyanoacrylate embolization of a high-flow vascular malformation of the scalp. By using a plastic "cookie cutter" placed over the lesion and applying various degrees of pressure, both venous outflow from and arterial inflow into the lesion were limited. This flow reduction technique improved both the efficacy and safety of the procedure.
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3/51. Pulmonary arteriovenous fistula: mechanical ventilation and hypoxemia.

    Pulmonary arteriovenous malformation (PAVM) is an uncommon congenital anomaly. As PAVM is a direct communication between branches of the pulmonary artery and vein, major disturbances in gas exchange can result. We present a patient with an unsuspected PAVM who came to our institution for drainage of a brain abscess. Arterial blood gas analysis during and after surgery demonstrated a large alveolar-arterial gradient for oxygen in the absence of any obvious pulmonary pathology while the patient was receiving positive pressure ventilation (PPV). Oxygenation improved considerably upon resumption of spontaneous ventilation. A diagnosis of PAVM was made subsequently. We conclude that positive pressure ventilation can worsen right to left shunting in patients with PAVM.
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4/51. Successful surgical ligation under intraoperative portal vein pressure monitoring of a large portosystemic shunt presenting as an intrapulmonary shunt: report of a case.

    We report a rare case of patent ductus venosus (PDV) with collapsed intrahepatic portal branches and an intrapulmonary shunt. Excellent improvement of the intrahepatic portal vein flow was achieved by ligating the large ductus venosus under intraoperative portal vein pressure (PVP) monitoring. A 3-year-old boy being followed up for hypergalactosemia at a local hospital was found to have mild lip cyanosis, exertional dyspnea, clubbed fingers, and mild liver dysfunction with high levels of transaminase and ammonia. cardiac catheterization indicated an intrapulmonary shunt with a ratio of 33%. Abdominal ultrasonography and computed tomography showed remarkable communication between the portal vein and the inferior vena cava. We performed laparotomy and successfully ligated the PDV under PVP monitoring. The PVP did not increase until the catheter was removed 7 days postoperatively. The patient's liver function test results returned to normal within 2 weeks. His serum galactose level was 0 mg/dl after drinking milk, and his SpO2 in room air and exertional dyspnea also improved. He was discharged 18 days after his operation, without any complications. We propose that ligation of a PDV under PVP monitoring could be a treatment of choice, bearing in mind that PDV is associated with collapsed intrahepatic portal branches.
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5/51. Massive pulmonary hemorrhage from dual circulation pulmonary arteriovenous malformations in hereditary hemorrhagic telangiectasia.

    Pulmonary arteriovenous malformations (AVMs) are commonly supplied by the pulmonary arterial system and rarely by the systemic bronchial circulation. The authors outline the case of a young woman with pulmonary AVMs as part of hereditary hemorrhagic telangiectasia with the uncommon presentation of massive hemoptysis. Management of her recurrent, life-threatening pulmonary hemorrhage was complicated by pulmonary AVMs that were supplied by both the pulmonary and systemic bronchial arterial circulatory systems. Transcatheter embolotherapy of the higher pressure bronchial systemic circuit was necessary for acute hemostasis.
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6/51. Unexpected ST segment changes in children--a case report.

    In children, myocardial ischemic changes during anesthesia are a rare event unless there is underlying pathology. The patient in this case report was an apparently healthy child scheduled for adenoidectomy and bilateral tympanostomy. Occurrence of significant ST changes as well as intraoperative and postoperative hypoxemia required further diagnostic work-up. Postoperative echocardiographic findings were suspicious of intrapulmonary right to left shunting. The pulmonary arteriovenous fistula is probably the major pathophysiological factor for the development of hypoxemia and paradoxical air embolism especially during positive pressure ventilation in our patient. Unexpected ST segment changes might also occur in patients with anomalous origin of coronary arteries. Although diagnostic work-up was inconclusive, it is necessary to rule out any underlying pathological process. Further follow-up is also important in order to learn more about these disease states that often have subclinical, but potentially fatal presentation.
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7/51. [Foix-Alajouanine syndrome: case report.]

    In a 52-year-old woman, spinal arteriovenous malformation (AVM) has been associated with what has been known as Foix-Alajouanine syndrome. The pathophysiology of the AV fistula is probably related to increased venous pressure from the AVM plus thrombotic process. The most common initial symptoms are sensory disturbance, pain and leg weakness. Definitive diagnosis of spinal AVMs requires radiographic demonstration of the vascular anomaly. Nevertheless, in this case, suggestive defects of malformations could not be seen, in contrast to the MRI findings and macroscopical and anatomical-pathological lesion. These findings rise our attention, about the need to keep in mind the clinical suspicion of AVM in cases of back pain and motor deficit, and an early surgical conduct in this situation.
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8/51. Central cyanosis due to prominence of the eustachian and thebesian valves.

    A 9-year-old female child with serious central cyanosis was found to have right to left shunt across a large secundum atrial septal defect despite normal right-sided pressures. Preoperative cross-sectional echocardiography suggested the presence of large sinus venosus eustachian and thebesian valves as the mechanism responsible for diversion of the inferior caval and coronary sinus venous return to the left atrium across the interatrial secundum defect. Surgical excision of the unduly prominent sinus venosus valve and patch closure of the atrial septal defect resulted in complete disappearance of the cyanosis and physiological and clinical cure.
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9/51. Ileo-caecal arterio-venous malformation associated with extrahepatic portal hypertension: a case report.

    This paper is a case report describing a boy with down syndrome and a novel combination of multiple vascular anomalies: extrahepatic portal hypertension, an arterio-venous malformation (AVM) at the ileo-caecal junction, and caval/iliac vein anomalies and developing anal bleeding. We considered that the ileo-caecal AVM would be one of the causes of the repeated hematochezia. The patient underwent ileo-caecal resection with the AVM, and anastomosis of the left external iliac vein and the jejunal branch vein because of the stenosis of the superior mesenteric vein (Clatworthy mesocaval shunt). Intraoperative portal pressure measurement at the site of the right colic vein showed a moderate pressure reduction (42.5-31.5 cm H2O). On the fourth month after operation, gastrointestinal fiberscopy showed no existence of esophageal varices. One year after operation, the patient was doing well without bleeding.
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10/51. An arteriovenous malformation masquerading as a carotid-cavernous sinus fistula.

    PURPOSE: To describe a case of an occipital arteriovenous malformation (AVM) presenting with unilateral signs mimicking a carotid-cavernous sinus fistula (CCF). DESIGN: Interventional case report. methods: A 67-year-old normotensive male presented with loss of vision, pain, and proptosis of the right eye. Best-corrected visual acuity was right eye 6/36 and left eye 6/5. Examination of the right eye revealed orbital congestion with arteriolization of the episcleral vessels and an intra-ocular pressure of 44 mm Hg which was refractory to medical treatment. RESULTS: Computed tomography (CT) angiogram and cerebral angiography found an occipital AVM draining into the right sphenoparietal sinus and thereafter the right ophthalmic vein, bypassing the cavernous sinus. Embolization followed by excision of the AVM resulted in recovery of vision, reversal of proptosis, and normalization of intraocular pressure. CONCLUSIONS: Rapid diagnosis and immediate intervention resulted in a rewarding visual recovery despite a persisting left homonymous hemianopia.
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