Postoperative changes in the superficial temporal artery and the external carotid artery duplex sonography after extra-intracranial bypass surgery in European Moyamoya disease.

INTRODUCTION: Despite Duplex ultrasonography being a noninvasive, easily repeatable, readily available and economical tool, this examination and its normal ranges are rarely described in Moyamoya disease (MMD). METHODS: Duplex ultrasonography examinations of the superficial temporal artery (STA) and external carotid artery (ECA) were performed preoperatively, postoperatively (within 30 days) and as follow-up exams (6 months postoperatively) after 32 cerebral revascularization procedures in 20 European patients with MMD. RESULTS: A significant higher mean diastolic flow of STA compared to preoperative values was found in postoperative (p<.000) and follow-up exams (p<.001) in Duplex ultrasonography. Postoperative and follow-up Duplex sonography of STA also showed a significantly higher mean systolic flow compared to preoperative values (p<.05 and p<.05). Also ECA showed significantly changes after bypass surgery (p<.05). CONCLUSION: Duplex ultrasonography of STA is a reliable and non-invasive tool to investigate hemodynamic changes after bypass surgery and to detect bypass patency in European patients with MMD.

Current status of intestinal transplantation in Japan.

The development of total parenteral nutrition (TPN) has dramatically improved the prognosis of patients afflicted with intestinal failure. However, TPN-related complications, which remain a problem for patients with intestinal failure can be addressed by intestinal transplantation, which can significantly improve their prognosis and quality of life. The first intestinal transplantation in Japan occurred in 1996. Of the 17 intestinal transplantations performed to date, six were obtained from deceased donors and 11 from living donors. The primary indications were: short gut syndrome (n = 8), intestinal function disorder (n = 7), and retransplantation (n = 2). In our experience, the 1-year and 5-year patient survival rates are 87% and 69%. All nine patients receiving transplants in the last 7 years have survived, which seem to be acceptable results for the treatment of intestinal failure. Relatively few intestinal transplantations have been performed to date, mainly due to the lack of national health insurance coverage for the procedure and the ban of the use of donors below 15 years of age. Liver failure patients are also ineligible because liver-intestine or multiorgan transplants are not allowed by current guidelines. Case numbers may increase in the future as the result of allowing for pediatric donors in the new Act on Organ Transplantation, which went into effect in July 2010. We continue to work on reforming national insurance coverage to cover multiorgan transplantations.

Graft loss and poor outcomes after living-donor liver transplantation owing to arterioportal shunts caused by liver needle biopsies.

BACKGROUND: Arterioportal shunts (APS) are well-known critical complications after liver transplantation (OLT). The aims of this study were to assess the frequency and causes of APS after OLT and to analyze APS patients with poor outcomes. PATIENTS: We evaluated 1415 OLT recipients retrospectively investigating APS cases. RESULTS: APS were detected in at least 9 patients (0.6%). All patients with APS had a history of posttransplant invasive procedures; percutaneous transhepatic cholangio drainage (n = 6) or needle biopsy (LNB; n = 3). Two patients with poor outcomes showed proximal APS caused by LNBs. The other 7 patients with distal APSs, showed stable conditions. Imaging findings in the 2 proximal APS patients revealed drastic changes in graft hemodynamics. Although they finally underwent re-OLT, their outcomes were poor, owing to fatal complications associated with advanced collaterals. CONCLUSION: We concluded that even careful LNBs can cause APS at unexpected points. Earlier, more aggressive treatments are required, especially for proximal APS patients.

Herpes simplex virus hepatitis after pediatric liver transplantation.

Herpes simplex virus (HSV) hepatitis has a fatal impact on the outcome of organ transplanted recipients. Here, we present a thought-provoking case of HSV hepatitis in a high-risk recipient after living-related liver transplantation (LRLT). A 1-month-old female newborn infant was affected by HSV encephalitis. Fulminant hepatic failure (FHF) of unknown etiology occurred suddenly at 4.4 years of age. Viral infections were ruled out as the cause of FHF. Intensive care including plasma exchange (PE) was started, and the preoperative treatments for ABO incompatibility were performed. Thereafter, LRLT was performed emergently. Although strong immunosuppression for ABO incompatibility was continued after LRLT, antibody-mediated rejection (AMR) occurred on postoperative day (POD) 4. PE was repeated and improvements were obtained. However, liver dysfunction appeared on POD 8. Histopathological findings of liver needle biopsy clearly revealed HSV hepatitis, although the results of HSV DNA and antibody titer in blood sample did not clearly indicate HSV infection. On POD 21, thrombotic microangiopathy (TMA) occurred and the plasma and immunoglobulin were replenished. Our pediatric recipient recovered successfully from AMR, HSV hepatitis, TMA, and repeated sepsis. We conclude that well considered therapy based on the real-time detection of HSV hepatitis is indispensable for the further improvements of outcome in HSV hepatitis after LRLT.

Human islet isolation for autologous transplantation: comparison of yield and function using SERVA/Nordmark versus Roche enzymes.

Islet autotransplantation (IAT) is used to preserve as much insulin-secretory capacity as possible in patients undergoing total pancreatectomy for painful chronic pancreatitis. The enzyme used to dissociate the pancreas is a critical determinant of islet yield, which is correlated with posttransplant function. Here, we present our experience with IAT procedures to compare islet product data using the new enzyme SERVA/Nordmark (SN group; n = 46) with the standard enzyme Liberase-HI (LH group; n = 40). Total islet yields (mean +/- standard deviation; 216,417 +/- 79,278 islet equivalent [IEQ] in the LH group; 227,958 +/- 58,544 IEQ in the SN group; p = 0.67) were similar. However, the percentage of embedded islets is higher in the SN group compared to the LH group. Significant differences were found in pancreas digestion time, dilution time, and digested pancreas weight between the two groups. Multivariate linear regression analysis showed the two groups differed in portal venous pressure changes. The incidence of graft function and insulin independence was not different between the two groups. The SN and LH enzymes are associated with similar outcomes for IAT. Further optimization of the collagenase/neutral protease ratio is necessary to reduce the number of embedded islets obtained when using the SN enzyme.

Intracranial dermoid cysts: variations of radiological and clinical features.

BACKGROUND: Intracranial dermoid cysts are uncommon, and their clinical features as well as surgical management differ from patient to patient. Dermoids are generally benign lesions, but may cause spontaneous complications such as meningitis and/or hydrocephalus due to rupture and epileptic seizures depending on their location. Little has been reported about characteristic imaging findings with resulting therapeutic considerations, and only a few reports exist about associated hydrocephalus. Imaging modalities have changed and can facilitate differential diagnosis and follow-up if applied correctly. In this paper, we attempt to contribute our clinical experience with the management of dermoid cysts. PATIENTS AND METHODS: The charts of five men and two women with intracranial dermoid cysts were retrospectively reviewed. The patients were treated between September 1993 and September 2006. Selected patients are presented in detail. RESULTS: Tumour location, size and radiographic characteristics varied in each patient. Clinical presentations comprised focal neurological deficits as well as epileptic seizures, persistent headache, mental changes and psycho-organic syndromes. One patient underwent delayed ventriculo-peritoneal shunting after ruptured fatty particles caused obstructive hydrocephalus. Despite dermoid rupture into the subarachnoid space, three patients never developed hydrocephalus. Diffuse vascular supra-tentorial lesions were seen in one patient as a result of aseptic meningitis. Diffusion-weighted imaging (DWI) hyperintensity in dermoids is related to decrease of water proton diffusion and should be used for both the diagnosis and follow-up of this lesion. CONCLUSION: Although dermoid cysts are known to be benign entities per se, their rupture can cause a wide range of symptoms including aseptic meningitis and/or hydrocephalus. This may be due to intraventricular obstruction and/or paraventricular compression. While rupture does not necessarily bring about hydrocephalus, radical removal of the tumour and close monitoring of ventricular size is required. Although not widely recognised as such, DWI is considered to be a useful imaging modality in the diagnosis and follow-up of dermoids.

Patient and aneurysm characteristics in multiple intracranial aneurysms.

INTRODUCTION: Multiple aneurysms occur in up to one-third of people with intracranial aneurysms. Of such patients, epidemiological data, clinical information, and aneurysm characteristics (of both unruptured and ruptured aneurysms in the same patients) were gathered in this retrospective review. PATIENTS AND METHODS: Ninety-nine patients operated on for multiple intracranial aneurysms at the Department of Neurosurgery, University Hospital Zurich, Switzerland, between 1994 and 2003 were assessed, 90% with subarachnoid hemorrhage (SAH), 10% with incidental aneurysms. RESULTS: The female to male ratio was 3:1, median age was 53 years. SAH symptoms included acute headache (74%), decrease of consciousness (54%), nausea and vomiting (40%), epileptic seizure (11%). Neurological signs were meningism (40%), cranial nerve paresis (12%), none (28%). Chronic headache was the major complaint (40%) in patients with incidental aneurysms, 20% had paresis of extraocular muscles. History of smoking and hypertension was present in 47% and 35%. There were 265 aneurysms (median number per patient, 2; range, 2-8), 95% were small (< or = 10 mm), 4.5% large, 0.5% giant (> 25 mm); 34% were ruptured, 66% unruptured (median size, 7 mm vs. 4 mm; p < 0.0001). Most aneurysms (27%) were on the middle cerebral artery bifurcation. Most ruptured aneurysms (18%) were on the anterior communicating artery and were 10 mm or smaller. Eighty-one percent of patients had (non-surgery related) SAH complications: cerebral vasospasm (44%), post-SAH hydrocephalus (36%), cerebral infarction (36%), intracerebral (25%) and intraventricular (21%) bleeding. Glasgow Outcome Scale score at 3 months was 4 or 5 in 73%. DISCUSSION: Ruptured aneurysms were significantly larger than unruptured ones. Although discussed controversially, most of our population's ruptured aneurysms were 10mm or smaller in size. Considering this, our study may contribute to improve the management of patients with intracranial aneurysms.

Thalamic cavernous angioma: paraculminar supracerebellar infratentorial transtentorial approach for the safe and complete surgical removal.

BACKGROUND: The thalamic cavernous angioma (CA) represents a neurosurgical challenge because of the critical neurologic functions of the thalamus and its surrounding structures and of their deep location inside the brain. Although the natural history of the thalamic CA remains undefined, several studies suggest the poor outcome of those patients especially if the symptomatic thalamic CA is treated conservatively. We describe the advantage of the paraculminar supracerebellar approach to the lesions in the brainstem. OBJECTIVE: We studied the usefulness and the safety of the paraculminar supracerebellar infratentorial transtentorial approach for the patients with thalamic CA. METHODS: One hundred and ninety two consecutive patients with CA were treated at the Department of Neurosurgery in the Zurich University Hospital between 1993 and 2003. Among these patients, we analyzed six patients (four female, mean age 43) with thalamic CA who underwent surgical removal with the paraculminar supracerebellar transtentorial approach. We retrospectively reviewed their medical charts, the neuroradiological images, and the operative notes/video records. RESULTS: Four patients of the six presented with thalamic hemorrhage. CA existed in the left thalamus in four patients and in the right in two. Preoperative symptoms included sensorimotor disturbance (three cases), double vision (three cases), Parinaud syndrome (one case), and thalamic pain (one case). All patients had the thalamic CA completely removed without any postoperative deterioration. CONCLUSIONS: This study suggests that for the removal of thalamic cavernous angioma the paraculminar supracerebellar infratentorial transtentorial approach provides the spacious surgical field with reduced risks of damaging and sacrificing surrounding vascular and neuronal system. This approach could proffer one of the best and safest surgical routes for the radical removal of thalamic cavernous angioma.

Peripheral large or giant fusiform middle cerebral artery aneurysms: report of our experience and review of literature.

Peripheral large and giant middle cerebral artery (MCA) aneurysms are rare and difficult to treat. We report our and others' experience with different possible modalities used to treat such lesions. Three patients were treated differently at our institution. One harboured a giant fusiform aneurysm on a peripheral branch of the superior trunk of the left MCA, and was treated by extracranial-intracranial (EC-IC) bypass and trapping of the aneurysm. The second patient was harbouring a large fusiform aneurysm on a peripheral branch of the inferior trunk of the right MCA, which was treated by trapping and excision without the need of an EC-IC bypass as assessed pre- and intraoperatively, while the last case was harbouring a giant fusiform aneurysm at the junction of M2-M3 and was treated by EC-IC bypass and end-to-end anastomosis after resection of the aneurysm. The aneurysms proved to be neither mycotic nor dissecting. The patients were clinically intact during their perioperative course without any postoperative complications and required no further treatment. Follow-up angiography demonstrated a functioning EC-IC bypass. Based on the surgical experience in these 3 cases and a review of the reported literature, the authors propose that when considering surgical treatment for such rarely encountered aneurysms, careful pre- and intraoperative evaluation including aneurysm trapping with or without EC-IC bypass when possible should be performed to obtain a satisfactory result without complication.

Hemorrhagic cerebral dissecting aneurysms: surgical treatments and results.

INTRODUCTION: Cerebral dissecting aneurysms are an increasingly recognized etiology of subarachnoid hemorrhage SAH and cerebral stroke. Hemorrhagic dissecting aneurysms of the anterior circulation have been considered to be somewhat different to those of the posterior circulation not only in terms of their pathophysiology, but also in terms of their management. Herewith our series of hemorrhagic dissecting aneurysms of the internal carotid artery ICA, vertebral artery VA, basilar artery BA and some of those of distal cerebral arteries is presented and compared to the series reported in the literature. Therapeutic consideration in the light of our experiences emphasizing the significance of aneurysm entrapment in combination with bypass surgery is presented. MATERIAL AND METHODS: During the last 13 years over 1000 patients with cerebral aneurysms were treated surgically in our department. Hemorrhagic dissecting aneurysms were diagnosed in 26 patients. Diagnosis was based on neuroradiological findings as well as intraoperative findings. All patients underwent surgical intervention. Clinical findings of these patients were analysed retrospectively. Follow-up outcomes were evaluated according to the Glasgow Outcome Scale GOS at 3 months after treatments. RESULTS: Location of 26 dissecting aneurysms was: ICA 11 cases (42%), VA 9 cases (35%), BA 3 cases, MCA 2 cases and PCA (P1 segment) one case. Primary surgical treatments were performed on day 3.7 of SAH on average. Clinical manifestation of dissecting aneurysms of the ICA and their outcome was more severe compared with those of the VA (p < 0.01): WNFS grade 3.1 vs 2.4 and GOS score 3.4 vs 4.3. As a conventional neck clipping procedure was problematic or impossible (aneurysm recurrence after clipping, premature rupture at the time of exposure or clipping), entrapment (or proximal ligation) plus EC-IC bypass procedure was the most frequent final definitive method of surgical treatment (9/26 35%: ICA 6/11, VA 1/9 and MCA 2/2) followed by proximal ligation or trapping only 7/26, neck clipping 7/26 and coating 4/26. CONCLUSIONS: Hemorrhagic dissecting aneurysms still remain problematic in their diagnosis and treatment. One has to be aware of the diagnostic possibility of dissecting aneurysms as an etiology of SAH. Neurosurgeons have to be prepared to be able to manage complex surgical situations also by the use of EC-IC bypass, as its combination with entrapment procedure can be the final treatment of choice. Less invasive endovascular technique is in evolution but its availability and superiority are still to be settled.

Emergency extra-intracranial bypass surgery in the treatment of cerebral aneurysms.

The need of an emergency bypass in hazardous situations during treatment of intracranial aneurysms has rarely been addressed in the literature. We report our 10 year experience with emergency bypass for aneurysm treatment. We retrospectively analyzed the data of patients who underwent emergency bypass surgery for the treatment of an intracranial aneurysm and compared the results with patients treated with bypass as a planned procedure during the same time period. Three groups were formed: group I, emergency bypass during clipping procedure; group II, emergency bypass for therapy refractory vasospasm; group III, planned bypass surgery. Sixteen patients (35%) out of 46 were treated with emergency bypass. In group I (11 patients) mortality was 37% and a good outcome (GOS 4 & 5) was achieved in 36%. In group II (5 patients) mortality was 20% and good outcome was reached in 60%. In group III (30 patients) mortality was 10% and good outcome was achieved in 86.6%. Outcome was worse in patients with additional SAH. An emergency bypass procedure as part of the aneurysm treatment should be considered in risky situations. Accurate timely decision-making is crucial combined with a fast and secure bypass technique. Treatment of refractory vasospasm with emergency bypasses may help to improve outcome in selected patients.

Bonnet bypass in multiple cerebrovascular occlusive disease.

UNLABELLED: The rationale and technique of the bonnet bypass procedure is discussed in two cases of multiple cerebrovascular occlusive disease. METHOD: Cerebral revascularization was achieved using respectively a radial artery interposition graft and a brachiocephalic vein interposition graft to connect the contralateral STA with a cortical branch of the ipsilateral MCA. FINDINGS: This alternate bypass technique proved to be an effective means of cerebral revascularization in selected cases where ipsilateral extracranial donor vessels were unavailable for classic STA-MCA bypass surgery. CONCLUSION: Clinical and hemodynamic improvement can be achieved by a bonnet bypass in selected cases of multiple cerebrovascular occlusive disease. In addition to its previously described role in skull base tumor surgery, the procedure should therefore earn consideration in the treatment of cerebral ischemia and stroke prevention.

Moyamoya angiopathy in Europe: the beginnings in Zurich, practical lessons learned, increasing awareness and future perspectives.

The number of patients, especially children, diagnosed with Moyamoya angiopathy and being referred to us for treatment from all across Europe, has increased over the last few years. An increase in awareness of the occurrence of stroke in children in the general and medical population might be the main cause of this phenomenon. Increasing awareness does not happen "spontaneously" nor does it manifest overnight! It requires regular platforms of communication between the general population and amongst the different medical specialists mainly neurologists, paediatric neurologists, neuropsychologists, neuroradiologists, neurorehabilitation specialists, nursing staff and neurosurgeons. Presently we were lucky to conduct the first Moyamoya Symposium ever to be conducted at a European-Japanese level with participation of specialists of this particular field from across Europe and Japan. Ever since the first child with Moyamoya was managed at the University hospital in Zurich some 7 years ago the number of patients referred to us from all across Europe increased rapidly. The importance of interdisciplinary communication, trust and support amongst specialists and increasing the awareness of the disease among the patients, medical personnel was and remains to be just as important as making the correct diagnosis and treatment of choice in these patients. We present the lessons we learned during these previous years and look into the future perspectives that require our further and urgent attention.

Continuous, but not occasional, oral ethanol intake reduces the success of intraportal transplanted islets of Langerhans: an experimental study.

BACKGROUND: Islet transplantation is gradually gaining acceptance for the treatment of type 1 diabetes mellitus. One of the unknown questions is alcohol intake; we have prohibited alcohol intake after islet transplantation although there is no solid evidence to support this. MATERIALS AND METHODS: In this study, we employed a mouse model to determine the effect of oral ethanol intake on transplanted islets. Either 500 or 150 islets were infused selectively into the right liver lobe of chemically induced diabetic mice. After transplantation, mice were orally administered either water (as a control) or various concentrations of ethanol for 14 consecutive days occasionally (once per day) or continuously (all intake was alcohol). Blood glucose levels were monitored and oral glucose tolerance tests (OGTT) performed. RESULTS: After 500 islets had been transplanted, all mice were cured from diabetes, but the continuous alcohol intake group showed significantly prolonged time to diabetes reversal and significantly lower glucose clearance rates by OGTT compared with the control group. After 150 islet transplantations, the diabetes cure rate in the continuous alcohol intake group was significantly lower than the control group (continuous alcohol vs control: 3/8 vs 11/12, P < .05). However, the occasional alcohol intake group showed no difference from the control group, even with as few as 150 islets transplanted per mouse. CONCLUSION: The present results demonstrated that continuous but not occasional alcohol intake reduced the success of intraportal islet transplantation.

An unusual case of a highly progressive supratentorial capillary haemangioblastoma - histopathological considerations.

We describe a case of a rapidly progressive, sporadic, non-von Hippel-Lindau (VHL) disease associated supratentorial capillary haemangioblastoma. Despite the classical histopathological presentation consisting of stromal cells and abundant capillaries, the tumour showed an unfavourable clinical course.

Intrasellar malignant peripheral nerve sheath tumor (MPNST).

Intracranial malignant peripheral nerve sheath tumors (MPNST) and intrasellar schwannomas are rare tumors. We describe a case of an intrasellar schwannoma with progression to a MPNST, a finding that, although very rare, extends the differential diagnosis of intrasellar lesions.

Decompressive hemicraniectomy in patients with subarachnoid hemorrhage and intractable intracranial hypertension.

BACKGROUND AND PURPOSE: To evaluate the outcome of patients with aneurysmal subarachnoid hemorrhage (aSAH) developing intractable intracranial hypertension and treated by decompressive hemicraniectomy (DHC). METHODS: Of 193 patients with aSAH 38 patients were treated with DHC after early aneurysm clipping. Indications for DHC were 1. Signs of brain swelling during aneurysm surgery (group 1: primary DHC). 2. Intracranial pressure- (ICP)-elevation and epidural, subdural or intracerebral hematoma after aneurysm surgery (group 2: secondary DHC due to hematoma) 3. Brain edema and elevated ICP without radiological signs of infarction (group 3: secondary DHC without infarction). 4. Brain edema and elevated ICP with radiological signs of infarction (group 4: secondary DHC with infarction). RESULTS: Thirty-one patients (81.6%) suffered from high grade aSAH Hunt & Hess 4-5. 21 belonged to group 1, five to group 2, six to group 3 and six to group 4. Of a total of 38 patients a good functional outcome according to Glasgow Outcome Score (GOS 4 & 5) could be reached in 52.6% of the cases. 26.3% survived severely disabled (GOS 3), no case suffered from a vegetative state (GOS 2) but 21.1% died (GOS 1). After 12 months good functional outcome could be achieved in 52.4% of the cases in group 1, in 60% in group 2, in 83.3% in group 3 and in 16.7% in group 4. CONCLUSIONS: In more than half of the patients with intractable intracranial hypertension after aSAH a good functional outcome could be achieved after DHC. Patients with progressive brain edema without radiological signs of infarction and those with hematoma may benefit most. The indication for DHC should be set restrictively if secondary infarcts are manifest.

Evaluation of islet transplantation from non-heart beating donors.

We evaluated islet transplantation from non-heart beating donors (NHBDs) with our Kyoto Islet Isolation Method. All patients had positive C-peptide after transplantation. The average HbA(1C) levels of the five recipients significantly improved from 7.8 +/- 0.4% at transplant to 5.2 +/- 0.2% currently (p < 0.01). Three patients with no or a single autoantibody became insulin independent while the other two patients with double autoantibodies reduced their insulin requirement but did not become insulin independent. C-peptide in patients who became insulin-independent gradually increased after each transplantation whereas C-peptide in patients who did not become insulin-independent from 3 months after the first transplantation to the next transplantation dramatically decreased. The beta-score of the three patients who became insulin independent was the best of eight. In conclusion, our method makes it feasible to use NHBDs for islet transplant into type 1 diabetic patients efficiently.

The role of intraoperative monitoring of oculomotor and trochlear nuclei -safe entry zone to tegmental lesions.

OBJECTIVE: A safe entry zone to tegmental lesions was identified based on intraoperative electrophysiological findings, the compound muscle action potentials (CMAP) from the extraocular muscles, and anatomic considerations. This entry zone is bordered caudally by the intramesencephalic path of the trochlear, laterally by the spinothalamic tract, and rostrally by the caudal margin of the brachium of the superior colliculus. METHODS: Four intrinsic midbrain lesions were operated upon via the safe entry zone using the infratentorial paramedian supracerebellar approach. All lesions involved the tegmentum and included an anaplastic astrocytoma, a metastatic brain tumor, a radiation necrosis, and a cavernous angioma. CMAP were bilaterally monitored from the inferior recti (for oculomotor function) and superior oblique (for trochlear nerve function) muscles. RESULTS: In three of four cases, CMAP related to the oculomotor nerve were obtained upon stimulation at the cavity wall after removal of the tumor. Stimulation at the surface of the quadrigeminal plate, however, did not cause any CMAP response. Using this monitoring as an indicator, the lesions were totally removed. CONCLUSIONS: In the surgery of tegmental lesions, CMAP monitoring from extraocular muscles is particularly helpful to prevent damage to crucial neural structures during removal of intrinsic lesions, but less so to select the site of the medullary incision. The approach via the lateral part of the colliculi is considered to be a safe route to approach the tegmental lesions.