Intraoperative high-field magnetic resonance imaging, multimodal neuronavigation, and intraoperative electrophysiological monitoring-guided surgery for treating supratentorial cavernomas

Objective: To determine the beneficial effects of intraoperative high-field magnetic resonance imaging (MRI), multimodal neuronavigation, and intraoperative electrophysiological monitoring-guided surgery for treating supratentorial cavernomas. Methods: Twelve patients with 13 supratentorial cavernomas were prospectively enrolled and operated while using a 1.5 T intraoperative MRI, multimodal neuronavigation, and intraoperative electrophysiological monitoring. All cavernomas were deeply located in subcortical areas or involved critical areas. Intraoperative high-field MRIs were obtained for the intraoperative“visu-alization”of surrounding eloquent structures,“brain shift”corrections, and navigational plan updates. Results: All cavernomas were successfully resected with guidance from intraoperative MRI, multimodal neuronavigation, and intraoperative electrophysiological monitoring. In 5 cases with supratentorial cavernomas, intraoperative “brain shift” severely deterred locating of the lesions;however, intraoperative MRI facilitated precise locating of these lesions. During long-term (>3 months) follow-up, some or all presenting signs and symptoms improved or resolved in 4 cases, but were unchanged in 7 patients. Conclusions: Intraoperative high-field MRI, multimodal neuronavigation, and intraoperative electrophysiological monitoring are helpful in surgeries for the treatment of small deeply seated subcortical cavernomas.

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.

Vascular complications of liver abscess: A literature review

Extensive vascular network and proximity to the gastrointestinal tract make the liver susceptible to abscess formation.While pyogenic liver abscesses account for the majority of liver abscesses in the Western world,amebic liver abscesses are more prevalent in tropical and developing nations.Most liver abscesses heal without complications.However,various vascular complications can occur in these patients,including compression of the inferior vena cava,thrombosis of the portal vein and/or hepatic veins,hepatic artery pseudoaneurysm,direct rupture into major vessels or the pericardium,and biliovascular fistula.These compli-cations can present significant clinical challenges due to the potential for hae-morrhage,ischemia,and systemic embolism,thereby increasing the risk of morbidity and mortality.Mechanical compression,flow stasis,inflammation,endothelial injury,and direct invasion are some of the proposed mechanisms that can cause vascular complications in the setting of a liver abscess.For the diag-nosis,thorough assessment,and therapeutic planning of vascular complications,more sophisticated imaging techniques such as multidetector computed tomo-graphy angiography or magnetic resonance angiography may be necessary.Although most vascular complications resolve with abscess treatment alone,additional interventions may be required based on the nature,severity,and course of the complications.This article aims to provide a systematic update on the spectrum of vascular complications of liver abscesses,offering insights into their pathogenesis,diagnosis,and management strategies.

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.

Portal biliopathy

Biliary ductal changes are a common radiological finding in patients with portal hypertension, however only a small percentage of patients (5%-30%) develop symptomatic bile duct obstruction. The exact pathogenesis is not clear, but an involvement of factors such as bile duct compression by venous collaterals, ischemia, and infection is accepted by most authors. Although endoscopic retrograde cholangiopancrea- tography was used to define and diagnose this condition, magnetic resonance cholangiopancreatography is currently the investigation of choice for diagnosing this condition. Treatment is indicated only for symptomatic cases. Portosystemic shunts are the treatment of choice for symptomatic portal biliopathy. In the majority of patients, the changes caused by biliopathy resolve after shunt surgery, however, 15%-20% patients require a subsequent bilio-enteric bypass or endoscopic management for persistent biliopathy. There is a role for endoscopic therapy in patients with bile duct stones, cholangitis or when portosystemic shunt surgery is not feasible.

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.

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).

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.

One step minilaparotomy-assisted transmesenteric portal vein recanalization combined with transjugular intrahepatic portosystemic shunt placement: A novel surgical proposal in pediatrics

Transjugular intrahepatic portosystemic shunt(TIPS) placement is a standard procedure for the treatment of portal hypertension complications. When this conventional approach is not feasible, alternative procedures for systemic diversion of portal blood have been proposed. A one-step interventional approach, combining minilaparotomy-assisted transmesenteric(MAT) antegrade portal recanalization and TIPS, is described in an adolescent with recurrent esophageal varice bleeding and portal cavernoma(PC). A 16-yearold girl was admitted to our Unit because of repeated bleeding episodes over a short period of time due to esophageal varices in the context of a PC. A portal vein recanalization through an ileocolic vein isolation with the MAT approach followed by TIPS during the same session was performed. In the case of failed portal recanalization, this approach, would also be useful for varice endovascular embolization. Postoperative recovery was uneventful. Treatment consisting of propanolol, enoxaparin and a proton pump inhibitor was prescribed after the procedure. One month post-op, contrast enhanced computed tomography confirmed the patency of the portal and intrahepatic stent grafts. No residual peritoneal fluid was detected nor opacification of the large varices. Endoscopy showed good improvement of the varices. Doppler ultrasound confirmed the accelerated flow in the portal stent and hepatopetal flow inside the intrahepatic portal branches. Three months post-op, TIPS maintained its hourglass shape despite a slight expansion. Portal hypertension and life threatening conditions related to PC would benefit from one-step portal recanalization. MAT-TIPS is feasible and safe for the treatment of PC even in children. This minimally invasive procedure avoids or delays surgical treatment or re-transplantation when necessary in pediatric patients.

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.

A latent form of essential thrombocythemia presenting as portal cavernoma

Essential thrombocythemia with abdominal thrombotic is frequently associated 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.

<i>De Novo</i>Cavernous Malformation after Radiosurgery

Background: The general use of radiation therapy and radiosurgery as first-line or adjuvant treatment of central nervous system (CNS) lesions has been associated with several harmful effects. Case Description: We report the case of a 16-year-old male patient, submitted at the age of seven to surgical removal of a world heath organization (WHO) grade II diffuse astrocytoma and referred for adjuvant radiosurgery after three further surgeries due to local recurrences. Seven years after radiosurgical treatment, he presented with symptoms of intracranial hypertension. Magnetic resonance imaging (MRI) at the time showed a cavernous malformation in the tumor bed, histologically confirmed, without tumoral recurrence. Conclusion: This case report emphasizes the relation between radiosurgery and the appearance of the novo cavernous malformations, only described previously in one adult patient.

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.

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 syndrome .attributed to those lesions that interrupt the dentatethalamic and the nigrostriatal tracts thus causing both an action and a rest tremor. It may arise from various underlying structural disorders including multiple sclerosis, stroke, or tumors. So far, to our knowledge, few studies on Holmes＇ tremor secondary to cavemoma have been reported. Here we report a case of disabling tremor, who harbored a cavemoma in the midbrain.

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.

Surgical treatment of brainstem cavernous malformations with three basic skull base approaches and minimally invasive techniques: Observations in 20 patients

Objective: Brainstem cavernous malformation(BSCM) is extremely challenging for neurosurgeons in terms of surgical approach choices. In this article, we summarized our experience in skull base approaches of BSCM, and elucidated the advance of surgical treatments of brain stem cavernous malformation through reviewing recent relevant articles. Methods: We retrospectively reviewed 20 consecutive patients who underwent resection between May 1, 2014 and April 30, 2016. Only midline suboccipital, subtemporal approach and retrosigmoid approach were used in this series. The diagnoses of all patients were confirmed by radiological and histological examination. Results: All 20 patients were completely extirpated without surgical-related mortality.The mean follow-up period was 9.5 months(range, 2-20 months). Of the 20 patients, 80%symptomatic patients underwent surgery after first bleeding episode within 3 months,20% after two or more bleeding episodes by magnetic resonance imaging. After resection and during follow-up, 75% of patients had an improvement in their modified Rankin scale(m RS) scores, whereas 10% were worse compared with their preoperative presentation; 15% were unchanged. Conclusion: Appropriate basic surgical approach and minimally invasive techniques are necessary in preventing impairment of neurologic function. The three common basic skull base approaches, combined with minimally invasive techniques can handle most of BSCMs with good surgical results.