Management of pancreatic arteriovenous malformation

Pancreatic arteriovenous malformations (AVM), while extremely rare, are frequently complicated by gastrointestinal bleeding. The elimination of pancreatic AVM is difficult once portal hypertension has developed. We describe herein a patient with congenital AVM of the pancreatic head presenting with recurrent episodes of melena, in whom pylorus-preserving pancreatoduodenectomy provided a means of definitive management. We also review the literature and focus on the diagnostic and therapeutic approaches. Angiography is always necessary to facilitate tactics of treatment, even if diagnosis has been established by non-invasive imaging modalities. To obtain complete regression, total extirpation of the affected organ, or at least the involved portion, should be performed before this disease leads to the lethal complications of gastrointestinal bleeding and portal hypertension. Transcatheter arterial embolization is the only alternative treatment for the control of hemorrhage. Received: January 27, 2000 / Accepted: April 6, 2000     

脑动静脉畸形实验动物模型的建立

脑动静脉畸形(AVM)实验性动物模型建立是研究AVM必不可少的。建立脑AVM实验性动物模型最常用的动物是猪，目前主要有如下几种猪的脑AVM模型建立的方式：利用猪的自然奇网模式，颈动脉一颈内静脉瘘模式，奇网一海绵窦交通模式，利用导管形成AVM模型以及颈总动脉与颈外静脉端一端吻合模式。随着此项工作的不断完善，对脑AVM的病理生理、血流动力学的认识定会进一步提高，这将会更有力地有助于AVM的治疗。     

Polypoid arteriovenous malformation in the jejunum of a child that mimics intussusception

Polypoid arteriovenous malformations, which are localized in the colon, are extremely rare in adults, with only 7 cases published to date. Here we present the case of a 6-year-old girl with a jejunal polypoid tumor that a sonogram and a computed tomographic scan have shown to resemble intussusception. Histologically, numerous large ectatic veins intermixed with small-caliber arteries, venules, arterioles, and capillaries were observed in the intestinal wall. A retrospective computed tomographic scan 3-dimensional angiographic reconstruction demonstrated that this may be a diagnostic characteristic. Clinical and morphologic comparisons with previously reported cases were discussed.     

Endovascular treatment of arteriovenous malformation

Vascular anomalies are common congenital or neonatal abnormalities. According to the approved classification of vascular lesions by Glowacki and Mulliken, hemangiomas and vascular malformations are distinguishable. Hemangiomas usually appear during the first days or weeks after birth and grow faster than the whole body of the infant. They are proliferating benign tumors that often involute. The opposite of hemangiomas, vascular malformations are present at birth, grow commensurately with the patient, demonstrate normal endothelial turnover, and never involute. The case of a young woman with an arteriovenous malformation (AVM) located on the left side of her face beneath the lower lip is described. The patient did not have any specific complaints except the cosmetic effect, which was a reddish and bluish discoloration of the skin over the lesion. The AVM was embolized with polyvinyl alcohol, and no subsequent surgery was performed. Follow-up ultrasound examination after a 12-month period showed no flow within the lesion area.     

Galenic arteriovenous malformation with precocious puberty

Pineal lesions may appear with precocious puberty. In this report, a patient with precocious puberty and macrogenitosomia caused by an arteriovenous malformation in the pineal region is presented. This vascular malformation was not visualized during investigations 3 years before the present series. It appears that the vascular malformation increased considerably in size within a 3-year period. This case suggests that some arteriovenous malformations may take a malignant course, increasing rapidly in size and behaving like tumors by causing destruction and compression of surrounding structures. This case seems to be unique because, to the best of our knowledge, an arteriovenous malformation associated with precocious puberty has never been described previously.     

Association of arteriovenous malformation and intracranial aneurysm in the posterior fossa

Two cases of a saccular aneurysm of the posterior inferior cerebellar artery associated with an anatomically related arteriovenous malformation are presented. The aneurysms were clipped and the arteriovenous malformations were totally extirpated in one-stage operations in both cases. The association of arteriovenous malformation and arterial aneurysm in the posterior fossa was reviewed in the literature.     

The management of giant arteriovenous malformation of the face

Summary A vascular malformation with arteriovenous shunts of the auricular area was treated with embolization followed by surgical excision in a young female. The value of combining embolization and surgery, and the timing of the latter, are discussed. The surgical treatment of AV malformations of the face is sometimes limited by the necessity to preserve important structures which are closely related to these lesions. A combined treatment including embolization and surgery offers an attractive solution in the management of such vascular malformations.     

Treatment of cerebral arteriovenous malformation

The management of cerebral arteriovenous malformation (AVM) is discussed. A series of 231 cases of AVM was treated from 1961 to March 1984 in our department. The treatments of these cases are classified as total removal 169, partial removal 8, feeder clipping 14, embolization 4 and conservative 36. Results at discharge and at follow-up are analyzed and the treatments evaluated. At follow-up, no rebleeding is reported in the patients who had undergone extirpation. Cases treated with other methods showed rebleedings and either their condition worsened due to hemorrhages or they died. On the basis of these results, it is suggested that AVM should be extirpated totally. Our surgical procedure consisted of temporary occlusion of feeders using cerebral protective substances, so called Sendai Cocktail (mannitol, Vitamin E, steroid). This procedure makes complete surgery safe. Surgical results of the cases which were totally extirpated were better than those reported in previous reports: the mortality rate was 3,6%, and the morbidity rate was 5,4%. Because of this, surgical treatment was preferred, that is when the location, size of AVM, age, patient's circumstances and the surgeon's experience allow. However, there are still cases which must be treated with other therapy. These patients are withheld surgery because they have inaccessible AVM or large AVM etc., whose operation would cause neurological deficits. For the treatment of these cases we have recently attempt the chemical embolization method with conjugated estrogen. This produced good outcomes. These non-surgical treatments such as embolization and irradiation have still some unsolved problems and progress in the future is anticipated.     

脑动静脉畸形出血的危险因素

目的探讨脑动静脉畸形(BAVM)出血的危险因素。方法收集DSA确诊的BAVM患者105例,分析BAVM出血的危险因素。结果单因素分析表明,BAVM破裂出血的危险因素有病灶位置、大小,供血动脉类型、数目,引流静脉类型、数目、形态及是否合并动静脉瘘;多因素Logistic分析发现,病灶位置、大小,供血动脉类型、数目,引流静脉数目及是否合并动静脉瘘是出血的危险因素,而引流静脉扩张可能是保护因素。结论病灶位于幕下或大脑深部、畸形团3cm、单支静脉引流、单纯穿支动脉供血及多支供血动脉、合并动静脉瘘是BAVM出血的危险因素。     

Aplasia cutis congenita associated with an immature arteriovenous malformation

Summary The first case of congenital defect of the skull and scalp in Japan, probably due to an immature arteriovenous malformation, is presented. In this case, an island of healthy scalp remained intact in the central area of the scalp defect, unlike previously reported cases. The dilated, tortuous scalp vessels apparently disappeared spontaneously only after multiple ligation. Because of slow wound healing, three successive operations were subsequently needed to cover the scalp defect. The cause of the aplasia cutis congenita and the delayed wound healing mechanism was investigated histologically.     

Total knee arthroplasty in vascular malformation

In Klippel–Trenaunay syndrome, vascular malformations are not only in skin and superficial soft tissues but also in deep tissues like muscles bones and joints. It is well documemted that these recurrent intraarticular bleeds can cause early arthritis and joint pain. Performing arthroplasty in such patients is difficult and fraught with complications. We describe such a case where navigated total knee arthroplasty was performed with success to avoid the problems of intra medullary alignment used in the presence of intra medullary vascular malformations. We also suggest certain measures when knee arthroplasty is considered in such patients.     

Choroid plexus arteriovenous malformation (AVM) in a newborn. Case report

We report here on an unusual case of AVM with intraventricular hemorrhage in a full-term newborn infant. Clinical and radiological findings are discussed in the light of the recent literature.     

Perirectal arteriovenous malformation treated by angioembolization and low anterior resection

We present a case of a child with ongoing lower gastrointestinal bleeding caused by a rectosigmoid arteriovenous malformation. To reduce perioperative bleeding, we performed preoperative angioembolization followed by subsequent resection by low anterior resection. Here we present the case and review the literature regarding neoadjuvent embolization for gastrointestinal and pelvic arteriovenous malformations.     

An arteriovenous malformation of the external ear in the pediatric population: A case report and review of the literature

The literature regarding arteriovenous malformations of the external ear is sparse. A case of a patient clinically diagnosed with an arteriovenous malformation of the external ear that was managed empirically with surgical excision, without recurrence, is presented. The pathogenesis, clinical presentation, radiological work up and management options regarding arteriovenous malformations are reviewed.     

Dural arteriovenous malformation presenting as cardiac failure in a neonate

Summary Dural arteriovenous malformation (AVM) is a rare clinical entity, presenting most commonly in adult patient with pulsatile tinnitus or haemorrhage. We report a case of a neonate with a massive dural arteriovenous malformation (AVM) with signs of cardiac failure soon after birth. The diagnosis was first suggested during angiocardiography when a hypertrophied left common carotid artery was detected. The AVM was supplied by branches from the left external carotid artery and the left veretebral artery and drained into the left transverse sinus near the torcula. This was excised in two stages. Post-operative angiography showed no residual malformation. The patient was well and developing normally 4 years after surgery. Review of the literature showed only 2 similar cases of dural AVM presenting in the neonatal period^4,5, both patients died, reflecting the high risks involved and the difficulties encountered in the management. Ligation of arterial supplies can be a useful temporary procedure in these high risk babies. Definitive excision should be performed later when the baby grows stronger.     

Appearance of an independent second arteriovenous malformation 3 years after subtotal resection of a first malformation

Cerebral arteriovenous malformations are thought to be congenital lesions consisting of artery-to-vein communications without an intervening capillary bed. They have been shown both to increase and to decrease in size and even to disappear spontaneously. We present a case of angiographic appearance of an independent right Sylvian fissure arteriovenous malformation 3 years after an incomplete resection of a separate right parietal arterio-venous malformation. Both arteriovenous malformations were subsequently totally resected. We propose that alterations in blood flow after manipulation of the first arteriovenous malformation produced conditions favoring enlargement of a previously unseen cryptic arteriovenous malformation. The appearance of a second arteriovenous malformation in the same patient is unique. Theories of origin and the natural history of arteriovenous malformations are discussed.     

Surgical resection of a cerebral arteriovenous malformation for treatment of superficial siderosis: case report

BACKGROUND

To our knowledge, there are only two reported cases of cerebral arteriovenous malformation associated with superficial siderosis. In both cases, the patients were asymptomatic and were discovered on retrospective review of magnetic resonance imaging.

CASE DESCRIPTION

We describe a case of superficial siderosis in a 47-year-old male with a remote history of closed head injury that presented with progressive hearing loss, cerebellar ataxia, and urinary incontinence. Lumbar puncture was indicative of active subarachnoid bleeding. Cerebral angiography revealed a small vascular malformation that was resected.

RESULTS

Pathological examination confirmed the diagnosis of the arteriovenous malformation. Six months after surgery the patient is neurologically stable with no further progression of clinical signs or symptoms.

CONCLUSIONS

We report the first case of surgical resection of an intracranial arteriovenous malformation for the treatment of superficial siderosis. We emphasize the necessity of a detailed evaluation when superficial siderosis is suspected to localize and resect potential bleeding sources, because the disease is progressive and often irreversible.     

Neuromagnetic assessment of epileptogenicity in cerebral arteriovenous malformation

Magnetoencephalographic (MEG) activities were recorded in five patients with cerebral arteriovenous malformation (AVM) who presented with epilepsy and no clinical history of intracranial hemorrhage, using a 37-channel DC superconducting quantum interference device (SQUID) system. While scalp-recorded electroencephalograms (EEG) failed to reveal paroxysmal discharge, MEGs demonstrated localized high frequency magnetic activity (HFMA). Magnetic source imaging (MSI) depicted the accumulation of equivalent current dipole (ECD) originating from HFMA around the nidus, and the ECD localization agreed well with spike localization on intraoperative electrocorticography (ECoG). These areas corresponded with the areas of hypoperfusion on single photon emission tomography and the intraoperative laser Doppler flow meter. We discussed the application of MEG in estimating interictal paroxysmal activity sources in patients with AVM and addressed the questions of its reliability and validity in source localization. Received: 16 August 1999 / Accepted: 14 March 2000     

脊髓血管畸形的诊断和治疗

报告脊髓血管畸形（ＡＶＭ）３３例。位于颈段３例，胸段１９例，胸腰段１１例；ＡＶＭ位于髓外硬脊膜下３０例，位于髓内及硬脊膜下３例。临床上多以双下肢麻木、无力为首发症状。手术切除２３例，血管内栓塞治疗８例，手术后再栓塞２例。痊愈１８例，明显好转５例，好转６例，无变化２例，加重２例。结合文献对其诊断和治疗等有关问题进行讨论。