Abstract 1122‐000055: Parenchymal and Subarachnoid Angiogenesis in Patients with Vein of Galen Aneurysmal Malformation

2021 ◽  
Vol 1 (S1) ◽  
Author(s):  
Maximilian J Bazil ◽  
Maximilian J Bazil ◽  
Johanna T Fifi ◽  
Alejandro Berenstein ◽  
Tomoyoshi Shigematsu
Keyword(s):  
Blood Flow ◽  
De Novo ◽  
Vascular Malformations ◽  
Case Reports ◽  
Repeated Treatment ◽  
Vein Of Galen ◽  
Hypoxic Environment ◽  
Liquid Embolic ◽  
Embolization Treatment ◽  
Bilateral Thalamus

Introduction : Vein of Galen Aneurysmal Malformation (VGAM) is an arteriovenous malformation that accounts for 30% of all pediatric vascular malformations. VGAMs undergo significant remodeling of hemodynamic and structural anatomy due to angiogenesis. These changes not only affect the malformation on a molecular and morphological basis, but may also lead to alterations in planned surgical procedures. It is imperative to better understand the dynamic, angiogenic environment of the cerebrovasculature in order to more effectively treat this disease. Methods : We present 36 cases of secondary angiogenesis in VGAM. We also present three case reports of angiogenesis secondary to VGAM. Results : Pre‐interventional angiogenesis was identified in 16 patients (44.4%) and post‐interventional angiogenesis in 20 patients (55.6%) following a stage of embolization therapy. The cohort of patients with pre‐interventional secondary angiogenesis was significantly older than patients with post‐interventional angiogenesis at initial angiogram (12 months ± 40.1 months vs. 4.0 months ± 5.4 months; p<0.05). Choroidal VGAMs presented with angiogenesis more frequently than Mural VGAMs (4/14 Mural vs 32/42 Choroidal; p<0.01). Angiogenesis was localized to either the left, right, or bilateral thalamus in 2 cases, to the cisternal space surrounding the VOG in 16 cases, and both in 18 cases. Conclusions : Upon identification of secondary angiogenesis, our team’s strategy is to embolize the venous component of the fistula. The ideal strategy in our practice is cannulation of the primary feeder of the malformation, as close to the fistula as possible, and injection of highly concentrated n‐BCA glue (70%‐90%) in a transarterial approach. After multiple rounds of embolization, feeders become less dilated and may be difficult to distinguish from angiogenesis. In this pattern, we use low‐concentrate nBCA (40%‐50%) from an identifiable, proximal feeder and occlude the venous component of the fistula. We identified two patterns of secondary angiogenesis: 1) pre‐interventional angiogenesis identified at the initial diagnostic angiogram, 2) de‐novo, post‐interventional angiogenesis during the staged‐embolization treatment‐course. Occasionally, we noted random bursts of angiogenesis. A combination of the hypoxic environment, inflammation, and hemodynamic alterations to the VGAM caused by liquid embolic/coiling may lead to a burst of angiogenesis that subsides after repeated treatment. We hypothesize that the immature sinuses typically associated with VGAM patients, which experience a decrease in blood flow and subsequently narrow after embolization, may contribute to turbulent blood flow. Development of parenchymal and subarachnoid angiogenesis is common during the multi‐session treatment of VGAM. It represents the response to the angiogenic stimuli released from the draining vein. This angiogenesis can be observed to regress spontaneously or mature as we continue to treat the VGAM. It is unnecessary to embolize secondary angiogenesis outright and it is our recommendation to chiefly target primary feeders of the VGAM as close to the venous component as possible.

scholarly journals Brainstem cavernous malformations: a review with two case reports

2009 ◽  
Vol 67 (3b) ◽  
pp. 917-921 ◽  
Author(s):  
Adolfo Ramírez-Zamora ◽  
José Biller
Keyword(s):  
Radiation Therapy ◽  
De Novo ◽  
Vascular Malformations ◽  
Case Reports ◽  
Clinical Manifestations ◽  
Pregnant Patient ◽  
Complex Nature ◽  
Cavernous Malformations ◽  
Symptomatic Intracranial Hemorrhage ◽  
Brainstem Cavernous Malformation

Central nervous system (CNS) cavernous malformations (CMs) are developmental malformations of the vascular bed with a highly variable clinical course due to their dynamic nature. We present one case of "de novo" brainstem cavernous malformation after radiation therapy adding to the increasing number of reported cases in the medical literature, and the case of a pregnant patient with symptomatic intracranial hemorrhage related to brainstem CMs to illustrate the complex nature in management of these patients, followed by a review of clinical and radiographic characteristics. CMs account for 8-15% of all intracranial and intraspinal vascular malformations. Although traditionally thought to be congenital in origin, CMs may present as acquired lesions particularly after intracranial radiation therapy. Clinical manifestations are protean and surgical treatment should be considered for patients with progressive neurologic deficits.

scholarly journals Diagnosis of Fibrotic Distal Ileum Stenosis after Ischemic Enteritis Using Transabdominal Ultrasonography

2021 ◽  
pp. 568-577
Author(s):  
Ryo Katsumata ◽  
Noriaki Manabe ◽  
Masaki Matsubara ◽  
Jun Nakamura ◽  
Kazuma Kawahito ◽  
...  
Keyword(s):  
Blood Flow ◽  
Case Reports ◽  
Blood Perfusion ◽  
Distal Ileum ◽  
Wall Thickening ◽  
Current Case ◽  
Abdominal Ultrasonography ◽  
Transabdominal Ultrasonography ◽  
Stenotic Lesion ◽  
Ischemic Enteritis

Ischemic enteritis (IE) is a rare disorder which is caused by inadequate blood flow to small intestine. The diagnostic procedure of this disease has not sufficiently established because of its rarity. Here, we report a case of IE in a hemodialysis-dependent 70-year-old man and summarize the diagnostic options for IE. The patient was admitted to our hospital because of acute abdominal distention and vomiting. He presented with mild tenderness in the lower abdomen and slightly elevated C-reactive protein level as revealed by blood tests. Radiographic imaging showed small bowel obstruction due to a stricture in the distal ileum. Contrast-enhanced abdominal ultrasonography revealed a 7-cm stenotic site with increased intestinal wall thickening, which preserved mucosal blood perfusion. Elastography revealed a highly elastic alteration of the stenotic lesion, indicating benign fibrotic changes resulting from chronic insufficient blood flow. Based on a clinical diagnosis of IE with fibrous stenosis, a partial ileostomy was performed. After surgical treatment, oral intake was initiated without recurrence of intestinal obstruction. Pathological findings revealed deep ulceration with inflammatory cell infiltration at the stenotic site. Occlusion and hyalinization of the venules in the submucosal layer indicated IE. In addition to current case, we reviewed past case reports of IE. Through this case presentation and literature review, we summarize the usefulness and safety of transabdominal ultrasonography for diagnosing IE.

Identification of two novel de novo TUBB variants in cases with brain malformations: case reports and literature review

2021 ◽  
Author(s):  
Kazuki Watanabe ◽  
Mitsuko Nakashima ◽  
Satoko Kumada ◽  
Hideaki Mashimo ◽  
Mikako Enokizono ◽  
...  
Keyword(s):  
Literature Review ◽  
De Novo ◽  
Case Reports ◽  
Brain Malformations

scholarly journals Identification of a De Novoc.1000delA ANK1 mutation associated to hereditary spherocytosis in a neonate with Coombs-negative hemolytic jaundice-case reports and review of the literature 

2021 ◽  
Vol 14 (1) ◽  
Author(s):  
Lichun Xie ◽  
Zhihao Xing ◽  
Changgang Li ◽  
Si-xi Liu ◽  
Fei-qiu Wen
Keyword(s):  
De Novo ◽  
Hereditary Spherocytosis ◽  
Case Reports ◽  
De Novo Mutation ◽  
Case Presentation ◽  
Important Means ◽  
Genetic Detection ◽  
Membrane Defects ◽  
Severe Jaundice ◽  
Neonates And Infants

Abstract Background To strengthen the understanding of Hereditary Spherocytosis (HS) and determine the disease-causing mutation present with neonatal jaundice. HS is a hemolytic condition resulting from various erythrocyte membrane defects. Many different mutations result in HS, including mutations in ANK1. Case presentation A term neonate presented at ten hours with severe jaundice requiring exchange transfusion. At two months he was hospitalized due to repeated pallor and anemia requiring blood transfusions. Using next-generation sequencing, we discovered the responsible mutation in the proband but not in his parents; a heterozygous nucleotide variation of c.1000delA (p.1334Sfs*6) in ANK1. Thus hereditary spherocytosis was diagnosed. Conclusions Genetic detection is an important means of discovering the cause of hemolytic anemia in neonates and infants where routine diagnostic tests are unrevealing. We found a novel de novo mutation, c.1000delA (p.1334Sfs*6) in ANK1 that might account for other cases of HS in the Chinese population.

De novo convulsive status epilepticus in patients with multiple sclerosis treated with dalfampridine

2018 ◽  
Vol 25 (4) ◽  
pp. 618-621 ◽  
Author(s):  
Emilie Panicucci ◽  
Mikael Cohen ◽  
Veronique Bourg ◽  
Fanny Rocher ◽  
Pierre Thomas ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Status Epilepticus ◽  
De Novo ◽  
Case Reports ◽  
Brain Magnetic Resonance Imaging ◽  
Convulsive Status Epilepticus ◽  
Biological Tests ◽  
History Of ◽  
Magnetic Resonance Imaging Mri ◽  
History Of Epilepsy

Background: Dalfampridine extended release (DAL) is a broad-spectrum voltage-gated potassium channel blocker that is indicated in multiple sclerosis to improve the nerve conduction of demyelinated axons. Seizures are a known side effect of DAL, which is contraindicated in patients with a history of epilepsy. Objective: Three cases of multiple sclerosis (MS) with de novo convulsive status epilepticus (CSE) probably related to dalfampridine administration are described. Methods: No patients had a history of seizures or renal impairment. Biological tests were normal. A brain magnetic resonance imaging (MRI) showed diffuse cortical and subcortical atrophy without active inflammatory lesions. Results: All three patients presented with CSE that was attributed to DAL and so was discontinued. Conclusion: These case reports illustrate that, aside from seizures, de novo CSE is a potential complication of MS patients treated with DAL.

scholarly journals Case reports on erosive pustular dermatosis of scalp: a cross sectional study at a tertiary care centre

2020 ◽  
Vol 6 (2) ◽  
pp. 260
Author(s):  
Avanitaben D. Solanki ◽  
Neha S. Nagrani ◽  
Dhara V. Patel ◽  
Neela M. Patel ◽  
Jigna P. Barot ◽  
...  
Keyword(s):  
Case Reports ◽  
Tertiary Care ◽  
Cross Sectional Study ◽  
Neutrophilic Dermatosis ◽  
Repeated Treatment ◽  
Care Centre ◽  
Tertiary Care Centre ◽  
Cross Sectional ◽  
Proper Diagnosis ◽  
Folliculitis Decalvans

<p class="abstract">Erosive pustular dermatosis of the scalp (EPDS) has been reported as a rare chronic and progressive non-infectious inflammatory condition of the actinically damaged scalp characterized by crusts, erosions and pustules. It tends to occur on the scalp of older people who have usually been treated for diffuse actinic damage and actinic keratoses. The list of possible differential diagnosis is long and includes tinea capitis, folliculitis decalvans, neutrophilic dermatosis, autoimmune vesiculobullous disorders, malignancy etc. Notoriously difficult to treat, these cases tend to be chronic and progressive. These patients often present after repeated treatment to various scalp dermatosis that doesn’t result in clearing instead persistence and perpetuation of the process. A cross sectional prospective study was conducted in department of dermatology in a tertiary care centre in which six cases of EPDS attending outpatient department from 2017 to 2019 were included. EPDS is more common but under recognized condition, hence less cases are reported till date. Increased awareness of and proper diagnosis of this condition changes the approach towards these patients, protects the scalp from involuntary insults and other precipitating factors that hinders with a better outcome.</p>

The role of NOS2 and NOS3 in renal protein and arginine metabolism during early endotoxemia in mice

2005 ◽  
Vol 288 (4) ◽  
pp. F816-F822 ◽  
Author(s):  
Yvette C. Luiking ◽  
Marcella M. Hallemeesch ◽  
Wouter H. Lamers ◽  
Nicolaas E. P. Deutz
Keyword(s):  
Blood Flow ◽  
Renal Blood Flow ◽  
Protein Turnover ◽  
De Novo ◽  
Whole Body ◽  
Wild Type ◽  
Protein Amino Acid ◽  
Renal Metabolism ◽  
Lps Challenge ◽  
Baseline Conditions

Previously, we observed an enhanced renal protein synthesis and increased de novo arginine production in the early response to endotoxemia in wild-type Swiss mice (Hallemeesch MM, Soeters PB, and Deutz NE. Am J Physiol Renal Physiol 282: F316–F323, 2002). To establish whether these changes are regulated by nitric oxide (NO) synthesized by NO synthase isoforms NOS2 and NOS3, we studied C57BL6/J wild-type (WT), NOS2-deficient (NOS2−/−), and NOS3-deficient (NOS3−/−) mice under baseline (unstimulated) and LPS-treated conditions. The metabolism of renal protein, amino acid, and arginine was studied at the whole body level and across the kidney by infusing the stable isotopes l-[phenyl-2H5]phenylalanine, l-[phenyl-2H2]tyrosine, l- guanidino-[15N2]arginine, and l-[ ureido-13C,2H2]citrulline. Renal blood flow was measured using radioactive PAH extraction. Under baseline conditions, renal blood flow was significantly reduced in NOS2−/− mice (0.29 ± 0.01 vs. 0.48 ± 0.07 ml·10 g body wt−1·min−1 in WT) ( P < 0.05), and de novo arginine production was lower in NOS2−/− mice. After LPS challenge, renal protein turnover and arginine production increased in all three groups ( P < 0.05), even though renal de novo arginine synthesis did not increase. The expected increase in renal citrulline production and disposal after LPS was not observed in NOS2−/− mice ( P = 0.06). Collectively, these data show that NOS2 is constitutively expressed in the kidney and remarkably functional as it affects renal blood flow and de novo arginine production under baseline conditions and is important for the increase in renal citrulline turnover during endotoxemia. NOS3, in contrast, appears less important for renal metabolism. The increase in renal protein turnover during endotoxemia does not depend on NOS2 or NOS3 activity.

scholarly journals Malformations of the Fetal Dural Sinuses

2009 ◽  
Vol 36 (1) ◽  
pp. 72-77 ◽  
Author(s):  
Matthew McInnes ◽  
Katherine Fong ◽  
Andrea Grin ◽  
Karel ter Brugge ◽  
Susan Blaser ◽  
...  
Keyword(s):  
Arteriovenous Fistula ◽  
Gestational Age ◽  
Vascular Malformations ◽  
In Utero ◽  
Developmental Trajectory ◽  
Dural Sinus ◽  
Vein Of Galen ◽  
Dural Sinuses ◽  
Arteriovenous Shunts ◽  
Dural Sinus Malformation

Background:Dural sinus malformation (DSM) is a term used to describe congenital vascular malformations characterized by massive dilation of one or more dural sinuses: these dilatations are typically associated with arteriovenous shunts. Such malformations can present antenatally but their early natural history and anatomy is poorly defined.Methods:We reviewed five years of autopsy experience and retrieved three primary vascular malformations of the fetal dural sinuses with ultrasound, magnetic resonance imaging (MRI) and post-mortem correlation.Results:Fetal ultrasound and MRI obtained between 19 and 23 weeks gestational age demonstrated in all cases dilation of the dural sinuses. In two cases vascular thromboses were present in either the dilated dural sinus (one of three) or the associated arteriovenous fistula (one of three). All cases were autopsied at 22-23 weeks gestational age. In one there was imaging and autopsy evidence of remodeling of the dural sinuses associated with a pial arteriovenous fistula. In two cases, no arteriovenous malformation was identified on initial imaging, but only became evident at autopsy. One case showed morphological overlap with vein of Galen aneurysmal malformation, with a midline arteriovenous shunt and vein of Galen ectasia. The other demonstrated a perisylvian dural arteriovenous fistula.Conclusion:In utero thrombosis of feeding vascular malformations or of the dural sinus malformation may be prominent. The early in utero developmental trajectory of dural sinus malformation (DSM) is poorly defined and deserves further study.

Republished: Multiplug flow control technique as a novel transarterial curative approach for the endovascular treatment of cerebrovascular malformations

2021 ◽  
pp. neurintsurg-2021-017418.rep
Author(s):  
H Saruhan Cekirge ◽  
Isil Saatci
Keyword(s):  
Flow Control ◽  
Dural Arteriovenous Fistula ◽  
Vascular Malformations ◽  
The Other ◽  
Control Technique ◽  
Injection Time ◽  
Complete Occlusion ◽  
Transarterial Embolisation ◽  
Embolic Agents ◽  
Liquid Embolic

Herein, we describe the use of a novel multiplug flow control technique for the curative transarterial embolisation of cerebrovascular malformations using liquid embolic agents (LEAs). The idea behind the use of this technique is to substantially control or arrest flow during LEA injection, with multiple plugs simultaneously formed from microcatheters that are placed within all or multiple feeders, so that the penetration of LEAs is facilitated, with flow control decreasing the washout of a malformation. This technique enables the complete occlusion of a vascular malformation in a shorter injection time than that in other methods because penetration is achieved faster. Details of this technique have been described in the treatment of two cases: one case of unruptured temporal arteriovenous malformation and in the other with a falcotentorial dural arteriovenous fistula, in which the vascular malformations were successfully occluded with transarterial embolisation.

Bortezomib+Chemotherapy Based Salvage Combinations in Refractory AML, Preliminary Results

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 4000-4000
Author(s):  
Miklos Udvardy ◽  
Attila Kiss ◽  
Bela Telek ◽  
Robert Szasz ◽  
Peter Batar ◽  
...  
Keyword(s):  
Cell Lines ◽  
De Novo ◽  
Case Reports ◽  
Fatal Outcome ◽  
Normal Karyotype ◽  
Secondary Aml ◽  
Poor Risk ◽  
Group 2 ◽  
De Novo Aml ◽  
Group 1

Abstract Bortezomib (Velcade) proved to be the standard element of refractory myeloma 2nd and 3rd line treatment, while many studies are suggesting excellent results in 1st line. Proteasome inhibition, the block of angiogenesis, modification of the NF-kappa-B system seems to be a challenging target in other malignant diseases, including refractory acute myeloid leukemia (AML), as well. In vitro data clearly support, that bortezomib exerts antiproliferative and pro-apoptotic effects in different AML cell-lines, along with human AML cell cultures, and moreover bortezomib was able to restore, or at least improve anthracyclin and possibly ARA-C sensitivity in different cell-lines (including AML). More recently, a Phase I trial showed bortezomib monotherapy efficient (only in few percents) in childhood refractory acute leukemia. Some case reports were shown at ASH 2007. We have tried bortezomib containing first or second line combinations in 27 (14 female, 13 male, mean age 57.6 years) patients with refractory or poor risk AML, in a small retrospective survey. The combinations were as follows: HAM or Flag-Ida, combined with bortezomib 1,3 mg pro sqm, day O and seven). The following groups were considered as refractory or poor risk AML: De novo AML, 2nd line: No response/remission to first line standard treatment (“3+7”), n=2 (Velcade- Flag-Ida treatment) De novo AML 1st line: bilineal or biphenotypic (flow-cytometry) n=2 (Velcade-Flag- Ida treatment) De novo AML with complex (numerical or more than 3 abnormalities) karyotype or normal karyotype with flt-3 TKD mutation, n=9, 1st line (Velcade-Flag-Ida n=6, Velcade- HAM protocol, n=3) Secondary AML or AML with evidence of previous more than 6 mo duration high grade MDS, n=14, 1st line: (Velcade-Flag-Ida n=9, Velcade-HAM n=5) RESULTS: Complete remission (CR) 12/27, partial remission (PR) 9/27, no remission 5/27, progression during treatment: 1/27.Best responses were seen in de novo cases. CR had been achieved in all patients of group 1 (two standard risk patients not responding to 3+7 protocol), and group 2 (biphenotypic, bilineal). The CR rate was quite appreciable in group 3, i.e. 6/9 (complex karyotype or normal karyotype with FLt-3 mutation – the response rate was excellent with flt-3 mutated cases). In group 4. (MDS, secondary AML) the results were less impressive. There were no major differences according to protocol (Flag-Ida or HAM) Allogeneous stem cell transplantation could have been performed in 1st CR in two patients (one from group 1. and another from group 2.). One of them died due to relapse, the other one is in CR since then. The combinations seem to be relatively safe. Induction related death rate was low (1 elderly patient acute thrombocytopenic bleeding with refractory MDS-AML). 5 other patients had severe neutropenic sepsis (2 with fatal outcome). Pulmonary syndrome, which may follow Velcade+ARA-C had not been documented. Other adverse events did not differ from the pattern observed with standard induction therapies.

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