Prognostic factors in non-malignant and non-cirrhotic patients with portal cavernoma: An 8-year retrospective single-center study

AIM:To evaluate the outcome of non-malignant and non-cirrhotic patients with portal cavernoma and to determine the predictors for survival.METHODS:Between July 2002 and June 2010,we retrospectively enrolled all consecutive patients admitted to our department with a diagnosis of portal cavernoma without abdominal malignancy or liver cirrhosis.The primary endpoint of this observational study was death and cause of death.Independent predictors of survival were identified using the Cox regression model.RESULTS:A total of 64 patients were enrolled in the study.During a mean follow-up period of 18±2.41mo,7 patients died.Causes of death were pulmonary embolism(n=1),acute leukemia(n=1),massive esophageal variceal hemorrhage(n=1),progressive liver failure(n=2),severe systemic infection secondary to multiple liver abscesses(n=1)and accident(n=1).The cumulative 6-,12-and 36-mo survival rates were 94.9%,86%and 86%,respectively.Multivariate Cox regression analysis demonstrated that the presence of ascites(HR=10.729,95%CI:1.209-95.183,P=0.033)and elevated white blood cell count(HR=1.072,95%CI:1.014-1.133,P=0.015)were independent prognostic factors of non-malignant and non-cirrhotic patients with portal cavernoma.The cumulative 6-,12-and 36-mo survival rates were significantly different between patients with and without ascites(90%,61.5%and 61.5%vs 97.3%,97.3%and 97.3%,respectively,P=0.0008).CONCLUSION:The presence of ascites and elevated white blood cell count were significantly associated with poor prognosis in non-malignant and non-cirrhotic patients with portal cavernoma.

Navigation-assisted transcortical transventricular approach for paraventricular cavernoma:A technical note and review of the literature

The surgery of paraventricular cavernoma remains a challenge for the neurosurgeon.Few approaches have been specially described for paraventricular cavernoma in literature.We present a patient with a symptomatic paraventricular cavernoma in the dorsal portion of the right lateral ventricles roof causing ventricular hemorrhage,and review his surgical approaches.This patient underwent a navigationassisted transcortical transventricular approach resulting in a complete resection without any neurologic deficits.The approach allows a safe and effective resection of paraventricular cavernomas.

Case Report of a Septum Pellucidum Cavernoma Surgically Resected via Inferior Parietal Approach and Short Literature Review

Background and Importance: Cavernomas of the septum pellucidum represent an extremely rare subtype of CNS cavernous angiomas which are a rare subtype of CNS tumors. Intraventricular cavernomas of the body of the lateral ventricle are usually treated by the transcallosal approach. Clinical Presentation: We present a case of a fifty-eight year old man with a septum pellucidum cavernoma successfully treated operatively by means of the inferior parietal transcortical transventricular approach. This unusual and scarcely documented tumor was situated at the posterior third of the septum pellucidum. The patient had a good recovery with no focal deficit. Conclusion: Septum pellucidum cavernomas are extremely rare lesions. Inferior parietal transcortical approach is a safe alternative to the commonly used transcallosal approach for lesions of the posterior half of the septum pellucidum.

A latent form of essential thrombocythemia presenting as portal cavernoma

Essential thrombocythemia is frequently associated with abdominal thrombotic complications including portal cavernoma as a consequence of chronic portal vein thrombosis.Essential thrombocythemia in a latent form is difficult to identify at onset due to the absence of an overt disease phenotype.In the presented case report,the diagnosis of essential thrombocythemia was initially missed because the typical disease phenotype was masked by bleeding and hypersplenism.The correct diagnosis was only reached when the patient experienced persistent thrombocytosis and pseudohyperkalemia after a shunt operation.

Resection of Intracranial Giant Cavernous Malformation: Case Report and Literature Review

Cerebral cavernous malformations are a rare and congenital vascular malformation that can present as a challenge in neurosurgical management. The term “giant cerebral cavernous malformations” still does not have a clear definition in the literature, with a wide variety of results. It is known, however, that there is an association between the size of the cavernoma and postoperative sequelae, especially in those with a size greater than 3 cm in its largest diameter. We present a case report of resection of a giant brain cavernoma measuring approximately 8 cm in its largest diameter, emphasizing on clinical presentation, diagnoses and postoperative evolution. Additionally, we performed a comprehensive review of the existing literature on the subject, addressing the epidemiology, pathophysiology, diagnostic methods, treatment options, and prognosis associated with this condition.

Parasellar extra-axial cavernoma mimicking meningioma: a case report

Parasellar extra-axial cavemomas are rare lesions.The authors report a case of extra-axial cavemoma in a 50-year-old male patient,who presented with occipital headache and double vision.The magnetic resonance imaging showed an enhancing extra-axial dural-based mass in the left parasellar region invading cavernous sinus,hyper-intense on T2-weighted images,iso-intense on Tl-weighted images and high relative cerebral blood velocity on magnetic resonance perfusion.The patient underwent a left pterional craniotomy and parasellar space occupying lesion was excised.Histopathology was suggestive of cavernous hemangioma.

Portal Cavernoma Cholangiopathy in Children and the Management Dilemmas

Portal cavernoma cholangiopathy(PCC)is one of the most harrowing complications of extrahepatic portal venous obstruc-tion,as it determines the long-term hepatobiliary outcome.Although symptomatic PCC is rare in children,asymptomatic PCC is as common as that in adults.However,there are major gaps in the literature with regard to the best imaging strategy and management modality in children.Moreover,natural history of PCC and effect of portosystemic shunt surgeries in children are unclear.Neglected PCC would lead to difficult or recalcitrant biliary strictures that will require endoscopic ther-apy or bilioenteric anastomosis,both of which are challenging in the presence of extensive collaterals.There are limited studies on the effect of portosystemic shunt surgeries on the outcome of PCC in children compared to adults.In this review,we aimed to collate all existing literature on PCC in childhood and also compare with adult studies.We highlight the difficul-ties of this disease to provide a comprehensive platform to foster further research on PCC exclusively in children.

Holmes' tremor caused by midbrain cavernoma

Holmes' tremor has been postulated as a syndromeattributed to those lesions that interrupt the dentate-thalamic and the nigrostriatal tracts thus causing both anaction and a rest tremor.It may arise from variousunderlying structural disorders including multiplesclerosis,stroke,or tumors.So far,to our knowledge,fewstudies on Holmes' tremor secondary to cavernoma havebeen reported.Here we report a case of disabling tremor,who harbored a cavemoma in the midbrain.

门静脉海绵状瘤胆管病诊治进展

门静脉海绵状瘤胆管病(portal cavernoma cholangiopathy,PCC)为门静脉海绵状瘤继发的胆管形态结构异常,多见于肝外门静脉阻塞(extrahepatic portal venous obstruction,EHPVO)患者,其发病机制尚不完全明确,可能与胆道受压、缺血、炎症等有关。磁共振胰胆管造影是其首选检查方法,有症状者可给予内镜治疗、外科手术或介入治疗,但无统一的治疗标准。本文旨在对PCC的发病机制、临床表现、诊断、治疗方式等研究进展作一综述,以期提高临床医师对该病的认识。

脊髓海绵状血管瘤的诊治特点

目的:探讨脊髓海绵状血管瘤的诊治方法和特点.方法:1991～2001年治疗脊髓海绵状血管瘤22例,均进行MRI检查,16例行全脊髓血管造影.全组均经后背正中直切口显微手术治疗.结果:22例均为单发,1例术前未能明确诊断,术后证实为海绵状血管瘤.颈段8例,颈胸段6例,胸段4例,腰段2例,圆锥马尾部位2例.MRI显示团块状短T1长T1信号,中间由点条状长T1短T2信号分隔,形成桑葚状混杂信号团,周边绕以低信号团,尤以T2WI显著.随访3个月～2年,全组均未见复发,12例术后功能明显恢复.结论:MRI是脊髓海绵状血管瘤术前最可靠的诊断方法.全脊髓血管造影可以排除其它脊髓血管畸形.显微手术切除是脊髓海绵状血管瘤的优选治疗方法,术中精细的显微操作是手术成功的关键.

脑干内血管畸形的诊断及治疗——附100例病例报告

目的 总结脑干内血管畸形的诊断、治疗经验教训,为提高临床诊疗水平提供借鉴。方法 回顾性地总结本所1987～1997年间经显微手术治疗的脑干血管畸形100例(105个病灶)病历资料,对脑干内血管畸形的发生、发展、病理学分类、临床及影像学表现、手术时机及手术方法等进行分析、总结。结果 脑干内的血管畸形多为海绵状血管瘤,有的同时合并毛细血管扩张症或静脉畸形。本组67%的病例经历1次以上的出血发作,女性患者的平均出血次数有高于男性的趋势。本组100例无手术死亡,多数手术效果良好。92%的病例得到6个月～11年的随访,出血复发率为4%。结论 对于症状性出血的脑干内血管畸形,应积极、稳妥地采取显微外科手术治疗。反复出血会加重症状,使病灶变硬,增大手术难度。对无症状者或影像学上无占位效应的病灶,暂时无需手术,但应保持临床及影像学的随诊。

颅骨骨膜窦(附1例报告及文献复习)

目的总结颅骨骨膜窦的诊治。方法报道右额顶部颅骨骨膜窦1例,并结合国内外相关文献,讨论其发病机制、临床表现及诊治原则。结果颅骨骨膜窦的发生原因可分为先天性和获得性两种,后者主要为外伤性,偶有自发性的报道。本例病人为外伤后形成,呈哑铃状生长,病变通过板障静脉与上矢状窦沟通。结论颅骨骨膜窦是一种罕见的血管畸形病变,熟悉其病因、临床表现等有助于其诊治。

中颅凹底海绵窦旁硬膜型巨大海绵状血管瘤的放射治疗

目的探讨放射治疗对巨大硬膜型海绵状血管瘤的治疗效果。方法回顾性分析8例中颅凹底海绵窦旁硬膜型海绵状血管瘤的放射治疗效果。结果全部病例肿瘤体积均有不同程度缩小。放疗后1年、2年肿瘤体积缩小比率均数分别为28.8%与54.5%。结论对于肿瘤巨大手术难以切除的硬膜型海绵状血管瘤,可以先行放疗,待肿瘤体积明显缩小后,选择合适的手术时机。

Therapeutic approaches for portal biliopathy: A systematic review

Portal biliopathy(PB) is defined as the presence of biliary abnormalities in patients with non-cirrhotic/nonneoplastic extrahepatic portal vein obstruction(EHPVO) and portal cavernoma(PC). The pathogenesis of PB is due to ab extrinseco compression of bile ducts by PC and/or to ischemic damage secondary to an altered biliary vascularization in EHPVO and PC. Although asymptomatic biliary abnormalities can be frequently seen by magnetic resonance cholangiopancreatography in patients with PC(77%-100%), only a part of these(5%-38%) are symptomatic. Clinical presentation includes jaundice, cholangitis, cholecystitis, abdominal pain, and cholelithiasis. In this subset of patients is required a specific treatment. Different therapeutic approaches aimed to diminish portal hypertension and treat biliary strictures are available. In order to decompress PC, surgical porto-systemic shunt or transjugular intrahepatic porto-systemic shunt can be performed, and treatment on the biliary stenosis includes endoscopic(Endoscopic retrograde cholangiopancreatography with endoscopic sphincterotomy, balloon dilation, stone extraction, stent placement) and surgical(bilioenteric anastomosis, cholecystectomy) approaches. Definitive treatment of PB often requires multiple and combined interventions both on vascular and biliary system. Liver transplantation can be considered in patients with secondary biliary cirrhosis, recurrent cholangitis or unsuccessful control of portal hypertension.

门静脉血栓形成机制及诊疗新进展

门静脉血栓形成机制复杂多样,深入理解门静脉血栓形成机制有助于正确治疗和积极预防其并发症。我们分别介绍了门静脉血栓的定义、分级、分型、发病机制和急、慢性门静脉血栓的临床表现,着重阐述了门静脉血栓的抗凝治疗、溶栓治疗、肠梗死的治疗和急、慢性PVT治疗的进展情况。

Imaging and radiological interventions in extra-hepatic portal vein obstruction

Extrahepatic portal vein obstruction(EHPVO) is a primary vascular condition characterized by chronic long standing blockage and cavernous transformation of portal vein with or without additional involvement of intrahepatic branches, splenic or superior mesenteric vein. Patients generally present in childhood with multiple episodes of variceal bleed and EHPVO is the predominant cause of paediatric portal hypertension(PHT) in developing countries. It is a pre-hepatic type of PHT in which liver functions and morphology are preserved till late. Characteristic imaging findings include multiple parabiliary venous collaterals which form to bypass the obstructed portal vein with resultant changes in biliary tree termed portal biliopathy or portal cavernoma cholangiopathy. Ultrasound with Doppler, computed tomography, magnetic resonance cholangiography and magnetic resonance portovenography are non-invasive techniques which can provide a comprehensive analysis of degree and extent of EHPVO, collaterals and bile duct abnormalities. These can also be used to assess in surgical planning as well screening for shunt patency in post-operative patients. The multitude of changes and complications seen in EHPVO can be addressed by various radiological interventional procedures. The myriad of symptoms arising secondary to vascular, biliary, visceral and neurocognitive changes in EHPVO can be managed by various radiological interventions like transjugular intra-hepatic portosystemic shunt, percutaneous transhepatic biliary drainage, partial splenic embolization, balloon occluded retrograde obliteration of portosystemic shunt(PSS) and revision of PSS.

儿童门脉海绵样变的诊断、治疗和预后追踪(附9例临床分析)

目的 总结儿童门脉海绵样变的诊治经验，了解不同治疗方法的效果及预后。方法 对在我院确诊的９ 例１ ～１１ 岁门脉海绵样变患儿的诊断和治疗效果进行分析，并追踪预后。结果 儿童门脉海绵样变以上消化道出血和脾大为首诊原因，超声波检查阳性诊断率为８８．９ ％ ，假阳性率为２．３ ％ ，脾切除＋ 门体分流术后上消化道出血次数减少，每年≤１ 次，比单纯脾切除或脾切除＋ 断流术效果好。结论 有门脉高压表现的儿童应注意门脉海绵样变的可能，超声波可作为常规的临床检查手段，条件许可时脾切除＋ 分流术应作为首选的治疗措施。

Portal biliopathy

Portal biliopathy refers to cholangiographic abnormalities which occur in patients with portal cavernoma. These changes occur as a result of pressure on bile ducts from bridging tortuous paracholedochal, epicholedochal and cholecystic veins. Bile duct ischemia may occur due prolonged venous pressure effect or result from insufficient blood supply. In addition, encasement of ducts may occur due fibrotic cavernoma. Majority of patients are asymptomatic. Portal biliopathy is a progressive disease and patients who have long standing disease and more severe bile duct abnormalities present with recurrent episodes of biliary pain, cholangitis and cholestasis. Serum chemistry, ultrasound with color Doppler imaging, magnetic resonance imaging with magnetic resonance cholangiopancreatography and magnetic resonance portovenography are modalities of choice for evaluation of portal biliopathy. Endoscopic retrograde cholangiography being an invasive procedure is indicated for endotherapy only. Management of portal biliopathy is done in a stepwise manner. First, endotherapy is done for dilation of biliary strictures, placement of biliary stents to facilitate drainage and removal of bile duct calculi. Next portal venous pressure is reduced by formation of surgical porto-systemic shunt or transjugular intrahepatic portosystemic shunt. This causes significant resolution of biliary changes. Patients who persist with biliary symptoms and bile duct changes may benefit from surgical biliary drainage procedures(hepaticojejunostomy or choledechoduodenostomy).

脑干海绵状血管瘤的显微手术治疗

目的探讨脑干海绵状血管瘤的诊断和治疗经验。方法本组18例脑干海绵状血管瘤中,病灶位于一侧大脑脚3例,桥脑及桥脑臂9例,桥脑-延髓4例,延髓2例。分别采用经幕下小脑上入路、一侧颞枕小脑幕入路、桥小脑角入路和枕后正中入路显微手术治疗。结果血管瘤镜下全切17例,次全切除1例。眩晕病人术后症状明显减轻,除1例术后新出现复视外,其余颅神经损害患者症状减轻,或无变化。结论显微手术治疗脑干海绵状血管瘤是可行的,早期手术可能取得较好的效果。