Internal carotid artery false aneurysm as a fatal complication of transsphenoidal surgery.

False aneurysm of internal carotid artery (ICA) is a rare but serious vascular complication observed after transsphenoidal pituitary surgery. Here, we present a 41-year-old woman with a pseudoaneurysm in the ophthalmic1 segment of the left ICA after exoscopic transsphenoidal pituitary surgery. The initially uneventful procedure was complicated by a subarachnoidal hemorrhage 10 days after the surgery, which was confirmed by cranial computed tomography scan. The emergency angiography revealed a pseudoaneurysm of the ophthalmic1 part of the left ICA. Despite repeated endovascular treatments with a flow diverter and coiling, the patient experienced a re-bleeding with consecutive vasospasms, occlusion hydrocephalus and finally bifrontal intracerebral hemorrhage with fatal outcome. As a conclusion in irregular post-operative courses with for example headache, a post-operative magnetic resonance imaging with vessel presentation using TOF sequence and contrast-enhanced MRA might be recommended in order to detect a possible pseudoaneurysm in an early stage.

Major central nervous system complications after allogeneic stem cell transplantation: A large retrospective study on 888 consecutive adult patients.

OBJECTIVES: Major complications affecting the central nervous system (CNS) present a challenge after allogeneic stem cell transplantation (allo-SCT). METHODS: Incidence, risk factors, and outcome were retrospectively analyzed in 888 patients in a monocentric study. RESULTS: Cumulative incidence (CI) of major CNS complications at 1 year was 14.8% (95%CI 12.3%-17.2%). Median follow-up is 11 months. CNS complications were documented in 132 patients: in 36 cases, classified metabolic; 26, drug-related neurotoxicity (14 attributed to cyclosporine A, 4 to antilymphocyte globulin); 11, cerebrovascular (ischemic n = 8, bleeding n = 3); 9, infections; 9, psychiatric; and 9, malignant. The cause of CNS symptoms remained unclear for 37 patients (28%). Multivariate analysis demonstrated an association of CNS complication with patient age (P < .001). The estimated OS of patients with any CNS complication was significantly lower than in patients without neurological complications (P < .001), and the CI of non-relapse mortality (NRM) was higher for patients with CNS complication (P < .001). A significant negative impact on survival can only be demonstrated for metabolic CNS complications and CNS infections (NRM, P < .0001 and P = .0003, respectively), and relapse (P < .0001). CONCLUSION: CNS complications after allo-SCT are frequent events with a major contribution to morbidity and mortality. In particular, the situations of unclear neurological complications need to be clarified by intensive research.

Symptomatic Intraosseous Vascular Malformation of Infraorbital Rim: A Case Report With Literature Survey.

BACKGROUND/AIM: Intraosseous orbital hemangiomas or vascular malformations (VM) are rare. This report is intended to complement the experience of diagnosing and treating a rare vascular lesion at this site. Special attention is paid to three-dimensional imaging and the morphological distinction between the two entities in this location. CASE REPORT: A 54-year-old female was examined and surgically treated for an exophytic firm mass of the infraorbital, which had become palpable as a hard mass due to growth in size. At first, a bone tumor, for example, an osteoma, was suspected. Intraoperatively, an osseous expansion with distinct fenestrations of the newly grown bone's surface, was detected. The lesion was firmly attaching to the orbital rim. The densely vascularized tumor was well defined to the soft tissues but had grown in continuity from the orbital floor and rim. Vascularized cavities caused the tumor to have a slightly reddish color. The histological examination confirmed the suspicion of the lesion's vascular origin. The lesion's immunohistochemical expression profile approved the final diagnosis of intraosseous VM. CONCLUSION: The symptoms of intraosseous vascular lesions of the orbit are determined by location and size. Modern imaging techniques facilitate the estimation of tumor-like expansion of lesions. However, the imaging characteristics of intraosseous vascular lesions are very variable. The symptoms of the patient presented herein show that growth phases of a vascular orbital malformation can occur in later stages of life and are initially indistinguishable from a neoplasm. In individual cases, patient care necessitates advanced diagnostic measures to establish the diagnosis and determine surgical therapy.

Vestiges of Ossified Spheno-occipital Suture in an Elderly Patient With Down Syndrome and Lateral Skull Base Fracture.

BACKGROUND/AIM: Down syndrome (DS) patients often show characteristic changes in the skull, e.g. short cranial base. The synchondroses of the skull base have a significant influence on the shape of the skull. The sphenooccipital synchondrosis (SOS) is the last of the basal synchondroses to ossify. This report is about residual ossification of SOS in an elderly patient with DS. CASE REPORT: The 65-year-old DS patient was polytraumatized by a fall. In the course of treatment, a purulent otitis externa on the right side was diagnosed, which had developed as a result of the fracture of the fossa glenoidalis. Computed tomograms of the skull base showed the fracture of the mandibular condyle, glenoid fossa and vestiges of SOS. CONCLUSION: The coincidental finding of vestiges of SOS in an elderly patient with DS raises the question of whether cross-sectional skull base images can show differences in the ossification of SOS between DS patients and a normal population.

Symptomatic Vasospasms as a Life-Threatening Complication After Transsphenoidal Surgery.

OBJECTIVE: To identify symptomatic vasospasms as a rare complication after transsphenoidal surgery, with emphasis on management and outcomes. METHODS: In this retrospective study, the medical records of 1997 patients who underwent microscopic transsphenoidal surgery at our hospital between 2008 and 2016 were analyzed regarding postoperative vasospasm events, clinical management, and neurologic outcomes. RESULTS: Four patients (0.2%) were identified who developed neurologic deficits in the postoperative phase caused by proven vasospasms due to subarachnoid hemorrhage (SAH). All 4 patients were treated according to current state-of-the-art recommendations for SAH-triggered vasospasms and, as ultima ratio, intra-arterial spasmolysis. Nonetheless, all patients developed multilocular ischemic infarctions. Three patients recovered with no or only slight neurologic deficits (2 with a Glasgow Outcome Score [GOS] of 5; 1 with a GOS of 4), and 1 patient died, at 24 days after surgery. CONCLUSIONS: Although a rare complication, vasospasms after transsphenoidal surgery can lead to severe and multilocular ischemic infarctions with a wide variety of neurologic impairments. This rare complication should be considered in patients with unexpected postoperative neurologic deficits. Computed tomography (CT)/magnetic resonance imaging and (contrast-enhanced) CT/magnetic resonance angiography are appropriate diagnostic tools. Treatment of vasospasms, including the option of intra-arterial spasmolysis, should not be delayed.

Radiological Patterns of Brain Metastases in Breast Cancer Patients: A Subproject of the German Brain Metastases in Breast Cancer (BMBC) Registry.

Evidence about distribution patterns of brain metastases with regard to breast cancer subtypes and its influence on the prognosis of patients is insufficient. Clinical data, cranial computed tomography (CT) and magnetic resonance imaging (MRI) scans of 300 breast cancer patients with brain metastases (BMs) were collected retrospectively in four centers participating in the Brain Metastases in Breast Cancer Registry (BMBC) in Germany. Patients with positive estrogen (ER), progesterone (PR), or human epidermal growth factor receptor 2 (HER2) statuses, had a significantly lower number of BMs at diagnosis. Concerning the treatment mode, HER2-positive patients treated with trastuzumab before the diagnosis of BMs showed a lower number of intracranial metastases (p < 0.001). Patients with a HER2-positive tumor-subtype developed cerebellar metastases more often compared with HER2-negative patients (59.8% vs. 44.5%, p = 0.021), whereas patients with triple-negative primary tumors had leptomeningeal disease more often (31.4% vs. 18.3%, p = 0.038). The localization of Brain metastases (BMs) was associated with prognosis: patients with leptomeningeal disease had shorter survival compared with patients without signs of leptomeningeal disease (median survival 3 vs. 5 months, p = 0.025). A shorter survival could also be observed in the patients with metastases in the occipital lobe (median survival 3 vs. 5 months, p = 0.012). Our findings suggest a different tumor cell homing to different brain regions depending on subtype and treatment.

Pneumatization of the zygomatic process of temporal bone on computed tomograms.

PURPOSE: Zygomatic air cells (ZAC) are a variant of temporal bone pneumatization that needs no treatment. However, ZAC can have an impact on surgical procedures in the temporo-mandibular joint region. Recent reports suggest that computed tomography will disclose more ZAC than can be diagnosed on panoramic radiography. The aim of this study was to analyze ZAC prevalence on CT in a population that was not pre-selected by admission to a dental clinic. Furthermore, an extensive literature review was performed to assess the prevalence of ZAC and to address the impact of imaging technique on the definition of the item. MATERIAL AND METHODS: Digitalized cranial CTs of 2007 patients were retrospectively analyzed. The Frankfort horizontal was used to define a ZAC on sagittal CTs. RESULTS: In this study group, 806 were female (40.16%) and 1,201 were male (59.84%). Mean age was 49.96 years in the whole group (female: 55.83 years, male: 46.01 years). A ZAC was diagnosed in 152 patients (female: 66, male: 86). Unilateral ZAC surpasses bilateral findings (115 vs. 37 patients). ZAC were diagnosed in children 5 years of age and older. Sectional imaging techniques show a better visualization of the region of interest. However, presently an increase of ZAC prevalence attributable to imaging technique cannot conclusively be derived from the current literature. The normal finding of a ZAC on radiograms is a sharply defined homogenous transparent lesion restricted to the zygomatic process of the temporal bone that has no volume effect on the shape of the process. CONCLUSION: ZAC is an anatomical variant of the temporal bone that has come into focus of maxillofacial radiology due to its noticeable aspect on panoramic radiograms. The harmless variant can be expected in about one in thirteen individuals undergoing facial radiology. Panoramic radiograms appear to be sufficient to present ZAC of relevant size. However, in preparation for surgical procedures affecting the articular eminence the application of sectional images is recommended.

Angioarchitectural Risk Factors for Hemorrhage and Clinical Long-Term Outcome in Pediatric Patients with Cerebral Arteriovenous Malformations.

BACKGROUND: Intracranial arteriovenous malformation (AVM) in children is a rare diagnosis. Little is known about factors determining AVM rupture and appropriate treatment strategies to prevent hemorrhage and associated disability. Available data suggest that children are subject to an increased risk for AVM rupture compared with adults. METHODS: In 46 pediatric patients with AVM, demographic factors, clinical presentation, angioarchitectural features, and treatment regimens as well as clinical and radiologic outcomes were retrospectively analyzed. First-line treatment option was microsurgical resection of the disease, with or without preoperative embolization. RESULTS: Twenty-four boys (52.2%) and 22 girls (47.8%) with a mean age on admission of 12.4 years (4-18 years) were included. Mean follow-up was 4 years (median, 1.5; range, 0.1-16.4). Thirty-one children presented with intracerebral hemorrhage (67.4%). Small AVMs (<3 cm) ruptured in 83.3% (n = 25) and were shown to be more prone to hemorrhage than larger ones (P < 0.01). Small AVM size (P < 0.01; odds ratio [OR], 0.12; 95% confidence interval [CI] 0.02-0.59) and exclusive deep venous drainage (P < 0.01; OR, 29.74; 95% CI, 2.45-4445.34) were independent risk factors for hemorrhage in the presented cohort. Good long-term outcome was associated with a high score on the Glasgow Coma Scale on admittance (P < 0.05; OR, 0.148; 95% CI, 0.03-0.73). CONCLUSIONS: Two-thirds of children with AVM are admitted with intracerebral hemorrhage. Microsurgical resection was successful as confirmed by radiologic studies in 95%, and 79.5% of patients presented in a good clinical condition on follow-up (modified Rankin Scale 0 or 1). Microsurgical treatment is recommended if the lesion is accessible and angioarchitectural risk factors favor definitive treatment.

Cavernous sinus sampling in patients with Cushing's disease.

OBJECT Correct diagnosis and precise localization of adenomas in patients with Cushing's disease are essential for avoiding unsuccessful transsphenoidal pituitary exploration. In addition to the well-established inferior petrosal sinus sampling, preoperative cavernous sinus sampling (CSS) was introduced as a potentially improved way to predict adenoma lateralization. The authors present their results with CSS in a consecutive series of patients with Cushing's disease. METHODS During 1999-2014, transsphenoidal surgeries were consecutively performed in 510 patients with Cushing's disease. For most patients, suppression of cortisol in high-dose dexamethasone tests and stimulation of adrenocorticotropic hormone and cortisol after administration of corticotropin-releasing hormone were sufficient to prove the diagnosis of adrenocorticotropic hormone-dependent hypercortisolism. Of the 510 patients, 67 (13%) were referred to the department of neuroradiology for CSS according to the technique of Teramoto. The indications for CSS were unclear endocrine test results or negative MRI results. Data for all patients were retrospectively analyzed. RESULTS A central/peripheral gradient was found in 59 patients; lateralization to the left or right side was found in 51. For 8 patients with a central/peripheral gradient, no left/right gradient could be determined. For another 8 patients with equivocal test results, no central/peripheral gradient was found. No severe CSS-associated complications were encountered. Of the 51 patients who underwent transsphenoidal surgery, the predicted lateralization was proven correct for 42 (82%). CONCLUSIONS As MRI techniques have improved, the number of potential candidates for this invasive method has decreased in the past decade. However, because detecting minute adenomas remains problematic, CSS remains a useful diagnostic tool for patients with Cushing's disease.

[Fatal crossbow injury in an adolescent]. / Tödliche Armbrustverletzung im Jugendalter.

The authors present the case of a 14-year-old boy who died while handling a crossbow, which was a gift from a friend's mother. The bolt passed through the right nostril, penetrated the sinus sphenoidalis, the brain stem, the left occipital lobe and the occipital calvaria. Immediately after the accident, the victim was taken to a maximum care hospital. In spite of neurosurgical treatment and intensive care the victim died 4 days later. The case presented demonstrates that crossbows are not suitable as toys for underage persons, as they are deadly weapons which can cause serious penetrating injuries. In Germany, no license is required to buy and/or possess crossbows. In the authors' opinion, legal restrictions on the sale of crossbows and a special training of the users would be reasonable measures to reduce such accidents.

Clinical relevance of associated aneurysms with arteriovenous malformations of the posterior fossa.

The aim of this study was to determine the frequency of aneurysms associated with arteriovenous malformations (AVMs) of the posterior fossa and their relation to hemorrhagic presentation in comparison to supratentorial AVMs. We performed a retrospective analysis of 474 patients with intracranial AVMs treated in our center from 1990 to 2010. Patients were analyzed for AVM size, drainage type and their clinical course with focus on vessel anomalies including AVM-associated aneurysms. Seventeen (30%) of 57 posterior fossa AVMs versus 46 (11%) of 417 supratentorial AVMs were associated with aneurysms. In six (10.5%) versus seven (1.7%) patients, respectively, flow-associated aneurysms were the source of hemorrhage. Infratentorial location of an AVM was a significant risk factor for the incidence (p < 0.001) and rupture (p < 0.001) of AVM-associated aneurysms. Feeding artery aneurysms in particular represented a risk factor for hemorrhage in the overall group of AVM patients, independently of the location (p < 0.001). The majority of patients with a posterior fossa AVM were treated by combined embolization and surgical removal within one procedure (n = 33, 58%). Feeding artery aneurysms were excluded by endovascular coiling or surgical clipping whenever feasible. Overall treatment-associated permanent morbidity in the subgroup of posterior fossa AVMs was 11% (n = 6) and mortality 4% (n = 2). Posterior fossa AVMs display a significantly higher frequency of associated aneurysms of the adjacent vessels that are correlated to the high bleeding rate compared to AVMs of the supratentorial compartment. We therefore recommend aggressive AVM treatment including the exclusion of associated aneurysms as a minimal therapeutic goal whenever possible.

Blister-like aneurysms--a diagnostic and therapeutic challenge.

Blister-like internal carotid artery (ICA) aneurysms are known for their fragile and thin-walled morphology associated with a high risk of intraprocedural rupture. Neurosurgical and endovascular options are illustrated on three exemplary cases reviewing the diagnostic and therapeutic implications of these special aneurysms. A 49-year-old woman was admitted with subarachnoid hemorrhage (SAH) in which angiography showed a broad-based, small bulging ectasy of the terminal ICA segment. On the attempt of surgical clipping, the aneurysm ruptured leaving a tear in the ICA. After temporary clipping, the rims of the tear were approximated by sutures. Sufficient closure of the remaining leakage was achieved by circumferential wrapping which was secured by two clips. Postoperative angiography confirmed stenosis of the tightened ICA and patient recovered without neurological deficit. Surgical attempt on a second case with bulging of the C4-segment topped by a small aneurysm was fatal due to extensive laceration of the basal ICA intraoperatively. Endovascular stenting was the choice of treatment in a third SAH patient in which angiography was suspicious of a blister-like ICA aneurysm. Six-month follow-up was uneventful; the patient recovered well and further growth of bulging was not seen. Reviewing the literature, blister-like aneurysms tend to arise at uncommon sites not located at the arterial branches. Small and broad-based bulges with or without true saccular aneurysms have to be assessed as characteristic features of blister-like aneurysms. Rupture of the aneurysm involving the carrying artery has to be considered during therapeutic attempts, in which urgent strategies have to be kept in reserve preventing fatal outcome. Blister-like aneurysms is a hazardous affair for neurosurgeons and neuroradiologists as their fragile structure most likely will lead to intraoperative rupture. If endovascular treatment is not promising, wrapping and revascularization techniques come true to still be an important part of the neurosurgeons toolbox for reconstructing a vessel lumen and preserving a sufficient cerebral blood flow.

Desmoid-type infantile fibromatosis of the mandible: case report with long-term follow-up.

A 6-year-old girl was referred to Eppendorf University Hospital because of a suspected malignant mandibular tumour. An osteolytic lesion was depicted on panoramic radiograph in the premolar region. The tumour was resected with covering skin and the defect was closed by primary intention. Histological investigation revealed fibromatosis. No local recurrence of infantile fibromatosis occurred during seven years of follow-up. The differential diagnosis of fibro-osseous lesion of the facial region is challenging. Especially in children, caution should be exercised in the treatment of benign lesions that resemble malignant conditions derived from connective tissue, with special reference to long-term follow-up.

Pathophysiology and treatment of brain AVMs.

Cerebral arteriovenous malformations (AVMs) are a major source of intracerebral hemorrhage in younger adults. First, some basic ideas about AVM anatomy, the influences of pressure, macrovascular flow, perfusion and the "steal effect", and some recent observations in the field of inflammatory markers and genetics are briefly discussed. Then, some clinical aspects in the presentation and the natural course of AVMs are highlighted, with special emphasis on the prediction of hemorrhage. Finally, some problems of the current treatment options are mentioned, and future directions in diagnostics and therapy considered.

Abciximab is a safe rescue therapy in thromboembolic events complicating cerebral aneurysm coil embolization: single center experience in 42 cases and review of the literature.

BACKGROUND AND PURPOSE: The purpose of this study was to estimate the safety and efficacy of abciximab treatment in combination with prophylactic heparin, acetylsalicylic acid (ASA), and clopidogrel application in cases of thrombus formation complicating endovascular coil embolization in cerebral aneurysms. METHODS: Thromboembolic incidents during endovascular management of 515 consecutive cerebral aneurysms were observed in 48 cases (9.3%). Eight incidents were observed during embolization of incidental aneurysms (8/174; 4.6%, 95% CI: 2.0 to 8.9%). All patients underwent anticoagulation with heparin and platelet-inhibition with ASA during treatment procedure. In addition, clopidogrel orally was applied 3 days preoperatively in patients with incidental aneurysms. In case of thrombus formation, glycoprotein IIb-IIIa inhibitor abciximab was given in 42 cases. No coagulation-effective rescue treatment was conducted in 5 cases. One patient was treated with r-tPA. End points were infarction on follow-up cranial CT and the rate of intracranial hemorrhages. RESULTS: No infarcts on follow-up CT were observed after treatment with abciximab in 29/42 patients (69.0%, 95% CI: 52.9 to 82.4%). No coagulant rescue therapy was applied in 5 patients because of a small nonocclusive thrombus or good collateral blood supply, showing consecutive infarction on follow-up CT in 3 cases as did the 1 patient treated with r-tPA. No periprocedural bleedings or rebleedings were observed in any case. CONCLUSIONS: Abciximab was safe as rescue treatment in cases of thrombus formation during endovascular aneurysm coiling. In our study the use of Abciximab, in combination with prophylactic anticoagulation and antiaggregation, does not lead to additional intracranial hemorrhages or any extracranial bleeding complications.

Pupillary responses to single and sinusoidal light stimuli in diabetic patients.

We examined effects of diabetes mellitus (DM) on the pupillary light reflex (PLR). Phasic pupillary response to a single light stimulus (200 ms) (pPLR) and to continuous sinusoidal stimuli with four different frequencies (0.1, 0.3, 0.7, 1.3Hz) (cPLR) were examined in 52 DM patients and 21 control subjects. We asked: does recording and frequency analysis of cPLR together with short time fourier [STFT] analysis of pPLR differentiate better between DM patients and normal subjects than pPLR only?Initial pupil diameter was significantly decreased in the DM group. For pPLR. maximal contraction velocity (Vmax), Vmax of redilation 1, reflex-amplitude and pPLR latency were significantly reduced in those patients who also showed signs of diabetic autonomic neuropathy (DNP). Tests of dynamic pupillary light reflex (cPLR) revealed that all DM patients had a significantly reduced gain at lower frequencies. Pupil phase lag was greater at 0.1 and 0.3Hz and smaller at 0.7 and 1.3 Hz in the DNP group (p<0.001). Comparison of single pPLR recordings of 5 DNP patients with 5 subjects using short time fast fourier (STFT) analysis revealed a characteristic change from low frequency content in healthy subjects to high frequency content in DNP patients.Significant changes in the PLR in DM can be found only when symptoms of autonomic neuropathy have been shown. Both sympathetic and the parasympathetic nervous systems are affected by diabetic autonomic neuropathy. Only recording of cPLR, together with STFT of pPLR can identify significant pathological deficits of pupillary control in single cases.

MRI abnormalities in normal-appearing brain tissue of treated adult PKU patients.

PURPOSE: To detect possible subclinical pathological brain changes a study on adult phenylketonuria (PKU) patients by using quantitative MRI methods was performed, since neuropsychological and cognitive deficits in treated patients with PKU have not yet been shown to correlate clearly with the brain lesions identified by conventional MRI. MATERIALS AND METHODS: Eight subjects, four PKU patients with well-documented dietary treatment and four age- and sex-matched adult controls, underwent MRI, including a triple echo sequence and a diffusion tensor imaging sequence. Brain maps of T2 relaxation time (T2), relative proton density (PD), and fractional anisotropy (FA) as well as apparent diffusion coefficient (ADC) were derived for each subject. T2, PD, FA, and ADC were measured in 22 predefined regions of gray matter (GM) and white matter (WM) on the corresponding maps, and compared with those of four age-matched healthy adult controls. RESULTS: In addition to a prolonged T2 value measured in affected WM, as expected, we observed a significant shortening of the T2 relaxation time and reduction of ADC in normal-appearing brain tissue and an increased proton density in both GM and WM of the patients. No differences were observed in FA values between controls and patients. CONCLUSION: Repeatedly reduced T2 relaxation time, ADC, and increased proton density without changes in FA indicate a higher cell-packing density in normal-appearing brain without changes in the directedness of fibers. These structural changes may be related to neuropsychological and cognitive deficits in treated PKU patients.

Management of cerebral arteriovenous malformations associated with symptomatic congestive intracranial hypertension.

We report on management strategies and clinical outcomes in 4 cases of acute symptomatic congestive intracranial hypertension associated with cerebral arteriovenous malformations (AVMs). Congestion resulted from high-volume shunts exhausting the drainage capacity of the cerebral venous system in 3 patients, and from sudden venous outflow obstruction in 1 patient. Two AVMs were suggested to be surgically accessible, whereas two AVMs were deemed to be inoperable. Surgically accessible AVMs were treated with embolization followed by complete surgical resection. Inoperable AVMs were treated with partial embolization. Both AVM embolization followed by surgical resection and partial AVM embolization effectively reduced intracranial pressure and achieved sustained patient recovery. Hence, an endovascular approach may be considered to manage AVM-related congestive intracranial hypertension either in combination with surgical AVM removal, or as a palliative approach in inoperable AVMs.