1/34. Spinal intradural perimedullary arteriovenous fistula with varix in infant.A rare occurrence of type IV spinal arteriovenous malformation (intradural perimedullary arteriovenous fistula) is described in an 18-month-old boy initially misdiagnosed with guillain-barre syndrome. An intramedullary mixed-intensity mass lesion at Th1 was demonstrated by magnetic resonance imaging together with flow voids over the dorsal aspect of the swollen spinal cord. angiography demonstrated an intradural perimedullary arteriovenous fistula including an intraparenchymal vascular pocket. After partial embolisation of the posterior spinal arteries through the left intercostal-radicular artery, the arteriovenous fistula was removed completely together with an organised haematoma. The fistula directly opened into a vascular pocket, which was confirmed pathologically to be a varix. The postoperative course was uneventful, and the patient resumed ambulation within 4 months. The case, subclassifiable as a type IVb spinal perimedullary AVF, was unique given its location and the patient's age at presentation.- - - - - - - - - - ranking = 1keywords = arteriovenous malformation, malformation (Clic here for more details about this article) |
2/34. Congestive brain oedema associated with a pial arteriovenous malformation with impaired venous drainage.We describe two patients with an unruptured pial AVM accompanied by significant brain oedema at initial presentation. In both cases, the primary drainer was a cortical vein showing varicose dilatation. in which venous congestion was indicated by magnetic resonance imaging (MRI). The restriction of venous drainage presumably caused venous hypertension in the surrounding brain, leading to the brain oedema and neurological symptoms. brain oedema can develop in patients with an unruptured AVM by venous congestion following spontaneous thrombosis of venous components. Varicosity in a major cortical draining vein and a small nidus are the possible lesions predisposing this fairly rare condition for unruptured AVMs.- - - - - - - - - - ranking = 4keywords = arteriovenous malformation, malformation (Clic here for more details about this article) |
3/34. Orofacial findings in the Klippel-Trenaunay syndrome.The Klippel-Trenaunay syndrome is a triad of congenital anomalies characterised by haemangiomas, varicosities, and unilateral bony and soft tissue hypertrophy. hypertrophy usually affects one distal limb, but trunk or face may be affected. Cutaneous haemangiomas (nevus flammeus) of varying extent and irregular contour are often present in the hypertrophic regions. Varicosities may also be part of the vascular lesions of the syndrome. Orofacial manifestations include facial asymmetry, jaw enlargement, and malocclusions as well as premature tooth eruption. Two cases of the Klippel-Trenaunay syndrome are presented here. Both of these show the typical hemifacial hypertrophy and premature eruption of teeth on the affected side. In the first case only the left mandibular region was affected. In contrast, in the second there was hypertrophy of the whole left side of the body including upper and lower jaws. This boy also suffers from congenital ideokinetic retardation, while the first was otherwise normal. Both cases differ from previously reported cases of the Klippel-Trenaunay syndrome in lacking any prominent facial nevus flammeus. In the first case there was also malformation of the crown of the first permanent molar on the affected side that has not been described previously.- - - - - - - - - - ranking = 0.055401710842243keywords = malformation (Clic here for more details about this article) |
4/34. Superior vena cava varix incriminating obstructive total anomalous pulmonary venous connection in a 42-day-old infant.We report a 42-day-old infant, who presented shortness of breath and remarkable cyanosis postprandially, with heterotaxy syndrome and complex congenital cardiovascular malformations. We deemed that the occurrence of the varix of the superior vena cava (SVC) may be related to the obstructive total anomalous pulmonary venous connection (TAPVC) with turbulent jet via the stenotic orifice to the wall of the SVC. Intravenous prostaglandin E(1) unmasks obstruction of the TAPVC in our patient. We correlated the clinical features with the radioimaging findings of the plain chest film, the echocardiography, the angiocardiography, and the electron beam computed tomography (CT) of the chest, and ascribed the morphogenesis of the varix of the SVC to obstructive TAPVC in this infant.- - - - - - - - - - ranking = 0.055401710842243keywords = malformation (Clic here for more details about this article) |
5/34. Klippel-Trenaunay syndrome with a life-threatening thromboembolic event.Klippel-Trenaunay syndrome is a congenital disorder characterised by the triad of cutaneous vascular nevi, soft tissue or bony hypertrophy, and varicose veins or venous malformations involving one or more extremities. An incidence of venous thromboembolism of up to 22% has been reported in this disorder. Also reported but rare is the development of trophic changes. Herein, we report the case of a male with Klippel-Trenaunay syndrome, deep vein thrombosis, venous ulceration, and death due to recurrent pulmonary embolism.- - - - - - - - - - ranking = 0.055401710842243keywords = malformation (Clic here for more details about this article) |
6/34. Colonic arteriovenous malformation in a child misinterpreted as an idiopathic colonic varicosis on angiography: remarks on current classification of childhood intestinal vascular malformations.A case of lower gastrointestinal hemorrhage in a child caused by an arteriovenous malformation (AVM) of the colon is presented. On diagnostic angiography, the lesion was misinterpretated as an idiopathic colonic varicosis because none of the characteristic features of an AVM were present. The role of angiography and shortcomings in nomenclature and classification of intestinal vascular anomalies in childhood are discussed.- - - - - - - - - - ranking = 5.221606843369keywords = arteriovenous malformation, malformation (Clic here for more details about this article) |
7/34. Rendu-Osler-Weber disease with a giant intracerebral varix secondary to a high-flow pial AVF: case report.BACKGROUND: Intracranial varices are rare and most are associated with vein of Galen arteriovenous malformations (AVM) or fistulas (AVF). DESCRIPTION: A 43-year-old left-handed man presented with right hemihypesthesia and spastic gait. Neuroradiological examination revealed a spinal AVF and a giant intracerebral varix associated with a high-flow pial AVF. He had recurrent episodes of nasal bleeding, which were also confirmed in his mother's medical history, and telangiectases in the tip of his tongue and fingers. He was diagnosed with Rendu-Osler-Weber disease. After resection of the spinal AVF that produced his symptoms, we surgically exposed and obliterated the giant varix and AVF under intra- and postoperative hypotension and mild barbiturate therapy. The arteriovenous shunt was completely obliterated without hyperperfusion of the surrounding brain. CONCLUSION: This is an extremely rare case of Rendu-Osler-Weber disease with a giant intracerebral varix secondary to a high-flow pial AVF that did not involve the vein of Galen.- - - - - - - - - - ranking = 1keywords = arteriovenous malformation, malformation (Clic here for more details about this article) |
8/34. Orbital varices, cranial defects, and encephaloceles: an unrecognized association.PURPOSE: To highlight an apparently unreported association between orbital varices and malformations of the cranial skeleton with or without abnormalities of the underlying cerebral parenchyma. DESIGN: Retrospective, noncomparative review of case notes and radiologic imaging. PARTICIPANTS: Three hundred ten patients attending the Orbital Clinic at Moorfields eye Hospital, london, with a diagnosis of low-pressure, low-flow orbital venous anomalies. methods: All available orbital imaging for patients with orbital venous anomalies was examined. For those with defects of the cranial base, the radiologic characteristics were noted and the clinical details were reviewed. MAIN OUTCOME MEASURES: The presence and type of orbital roof or medial wall defects and associated nasal or other cranial anomalies. RESULTS: Imaging was adequate for review in 222 of 310 patients (72%), and anomalies of the neighboring cranium or cerebral structure were found in 10 of 222 patients (4.5%). In the group with cranial anomalies, the proportion of men (7/10; 70%) did not significantly differ from that in the group with varices but without cranial anomalies (93/212; 44%; P = 0.19, Fisher exact test). Orbital varices were associated with 3 types of cranial anomaly: major midline encephaloceles (type I anomaly; 4 cases), large superomedial defects of the orbital wall (type II; 3 cases), or defects of the greater wing of the sphenoid (type III; 3 cases). CONCLUSIONS: Clinicians should be aware of the possibility of significant cranio-orbital or cranionasal anomalies in patients with orbital venous anomalies; these anomalies can vary from minor defects in the cranial base to large encephalomeningoceles. This rare association should not be overlooked when orbital varices become markedly inflamed, because intranasal encephaloceles are a known predisposition to recurrent meningitis.- - - - - - - - - - ranking = 0.055401710842243keywords = malformation (Clic here for more details about this article) |
9/34. Pulmonary varix: blood flow is essential in the diagnosis.A 15-year-old girl presented with left-sided chest pain. A chest radiograph demonstrated a tubular structure in the left hemithorax. A helical CT angiogram suggested the diagnosis of an arteriovenous malformation, but selective pulmonary arteriography diagnosed the presence of a varix and excluded a pulmonary arteriovenous malformation.- - - - - - - - - - ranking = 2keywords = arteriovenous malformation, malformation (Clic here for more details about this article) |
10/34. Cervical varix accompanied by placenta previa in twin pregnancy.BACKGROUND: Uterine cervical varix is a rare complication in pregnant women and can be the cause of obstetric hemorrhage in the vagina resulting in adverse events for both the mother and fetus. CASE: A 34-year-old Japanese woman was hospitalized at 18 weeks gestation because of cervical varix and placenta previa. Prophylactic tocolysis successfully controlled the obstetric hemorrhage. At 27 weeks gestation, emergent cesarean section was performed because of intractable hemorrhage from the marginal placenta previa. Intraabdominal findings revealed no vascular malformation of the uterus, and the operation was performed uneventfully. A speculum examination of the vagina and cervix at 1 month postpartum were unremarkable. CONCLUSION: It is important to recognize the clinical features and available treatments for cervical varix.- - - - - - - - - - ranking = 0.055401710842243keywords = malformation (Clic here for more details about this article) |
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