Vein of Galen aneurysmal malformation—clinical and angiographic spectrum with management perspective: an institutional experience

2016 ◽  
Vol 9 (2) ◽  
pp. 159-164 ◽  
Author(s):  
Himanshu Agarwal ◽  
Leve Joseph Devarajan Sebastian ◽  
Shailesh B Gaikwad ◽  
Ajay Garg ◽  
Nalini K Mishra

Background and purposeVein of Galen aneurysmal malformation (VGAM) is a rare developmental intracranial vascular malformation. We analyzed the clinical presentations, imaging findings, angioarchitecture, management options, and outcome in a demographically heterogeneous set of VGAM patients.MethodsWe retrospectively analyzed cases of VGAM from our departmental archive collected between 1988 and January 2015. Demographic, clinical, therapeutic, and follow-up details were obtained for each patient from the available records.ResultsWe identified 36 patients with VGAM including 6 neonates, 18 infants, 7 children aged 2–10 years, and 5 adults. Macrocrania was the commonest presenting feature. Type of fistulae was mural in 14 and choroidal in 18 patients while 4 had a thrombosed sac at presentation. In 3 cases the dilated venous sac had connection with the deep venous system. Bilateral jugular atresia and stenosis were seen in 9 and 6 patients, respectively. Giant venous sac (>4 cm) was significantly correlated with mural type (p=0.0001). Dural arterial recruitment was seen in 4 patients including 3 adults. Among the 23 patients treated by endovascular means, 14 had a good outcome, 5 had a poor outcome, and 4 died. A significant correlation was noted between jugular atresia and poor outcome (p=0.003).ConclusionsWe encountered a wide range of demographic, clinical, and angiographic features in VGAM. Mural type malformations were associated with giant venous sacs. Good outcome after embolization was seen in selected neonates and in most of the infants, children, and adults. Jugular atresia was significantly associated with poor outcome.

1990 ◽  
Vol 157 (3) ◽  
pp. 351-354 ◽  
Author(s):  
A. L. Mina Bergem ◽  
Alv A. Dahl ◽  
Cato Guldberg ◽  
Helge Hansen

As a result of follow-up studies published in 1937 and 1939, Langfeldt divided schizophrenia into two groups; ‘typical schizophrenia’ which had a poor outcome, and the ‘schizophreniform psychoses' which had a less typical clinical picture of schizophrenia and a good outcome. Langfeldt's cases of schizophreniform psychoses were reclassified according to the ICD–9 and DSM–III–R diagnostic systems. Most of the schizophreniform psychoses did not appear ‘schizophrenia-like’ at all, but turned out to be mainly affective disorders. Those included in Langfeldt's diagnosis of ‘schizophreniform psychoses' were found to be too heterogenous to validate the existence of this syndrome.


2017 ◽  
Vol 89 (1) ◽  
pp. 105-111 ◽  
Author(s):  
Diane F van Rappard ◽  
Antoine Klauser ◽  
Marjan E Steenweg ◽  
Jaap Jan Boelens ◽  
Marianna Bugiani ◽  
...  

ObjectiveTo determine whether proton magnetic resonance spectroscopic imaging is useful in predicting clinical course of patients with metachromatic leukodystrophy (MLD), an inherited white matter disorder treatable with haematopoietic cell transplantation (HCT).Methods21 patients with juvenile or adult MLD (12 HCT-treated) were compared with 16 controls in the same age range. Clinical outcome was determined as good, moderate or poor. Metabolites were quantified in white matter, and significance of metabolite concentrations at baseline for outcome prediction was assessed using logistic regression analysis. Evolution of metabolic changes was assessed for patients with follow-up examinations.ResultsIn this retrospective study, 16 patients with baseline scans were included, 5 with good, 3 with moderate and 8 with poor outcome, and 16 controls. We observed significant group differences for all metabolite concentrations in white matter (p<0.001). Compared with controls, patients had decreased N-acetylaspartate and glutamate, and increased myo-inositol and lactate, most pronounced in patients with poor outcome (post hoc, all p<0.05). Logistic regression showed complete separation of data. Creatine could distinguish poor from moderate and good outcome, the sum of glutamate and glutamine could distinguish good from moderate and poor outcome, and N-acetylaspartate could distinguish all outcome groups. For 13 patients (8 with baseline scans), one or more follow-up examinations were evaluated, revealing stabilisation or even partial normalisation of metabolites in patients with moderate and good outcome, clearly visible in the ratio of choline/N-acetylaspartate.ConclusionIn MLD, quantitative spectroscopic imaging at baseline is predictive for outcome and aids in determining eligibility for HCT.


Author(s):  
Ansari Sumayya Shamim ◽  
◽  
Reena Jatin Wani ◽  
Hema Prakash Relwani ◽  
Kruti Doshi ◽  
...  

We are presenting here a case of polycystic ovarian syndrome, an endocrine disorder, afflicting 5-13% females of reproductive age, causing 70-80% infertility associated with pituitary macroadenoma i.e. size >10mm& prolactin level >250 ng/mI. PCOS has a higher relationship with metabolic disturbance and hypothalamic-pituitary-ovarian axis function disorder. Prolactinoma is present in about 50% women with hyperprolactinemia and 33% present with galactorrhea. Our patient was a 22 yrs nulligravida married since 3 yrs first presenting 5 yrs back with complaints of secondary amenorrhea with galactorrhea and infertility. On evaluation she was diagnosed as PCOS and pituitary macroadenoma on MRI. We medically treated her with tab. cabergoline in consultation with endocrinologist and neurosurgeons opinion. Being a compliant patient with regular follow up she conceived twice with good outcome and breastfed her babies on medical management only. Now she has 2 children (male child of 3 yrs and 1 month). Dilemma in management options will be discussed. Summary: Inspite of having both Polycystic Ovarian Syndrome (PCOS) & Pituitary Macroadenoma, which warrant surgical management (major causes of Infertility) selected cases like ours can be managed medically successfully.


Author(s):  
Y Lee ◽  
LYR Tan ◽  
SAKK Abdul Shakoor

Introduction: Pheochromocytomas (PCC) and paragangliomas (PGL) are rare endocrine tumours. The objective of this study was to describe our experience with these two entities in a Singapore population. Methods: We identified patients with positive histopathological confirmations of PCC and PGL who were treated at a tertiary Singapore hospital between January 2000 and December 2015. The results were analysed for clinical presentations, treatment and long-term outcomes. Results: A total of 27 cases (20 PCC, 7 PGL) were identified over a 15-year period. One case of PGL developed bilateral disease on follow-up. There were 17 male and 10 female patients with a median age of 57 (range 24–77) years. A positive family history was uncommon and present in only 3.7% of patients. Uniquely, the top three presenting symptoms were abdominal discomfort, palpitations and diaphoresis. Despite adequate preoperative preparation, intraoperative haemodynamic instability occurred in 70.4% and early postoperative hypotension occurred in 11.1% of patients. After surgery, hypertension was resolved in 41.2% (7/17) and diabetes mellitus in 60% (3/5). Disease recurrence was reported in 22.2% and distant metastases in 14.8%. At the end of the follow-up period (median 35 [range 3–148] months), 70.4% were still alive. Conclusion: PCC and PGL can present with a wide range of symptoms. Intraoperative haemodynamic instability was frequent despite good preoperative preparation. Disease recurrences and metastasis occurred in up to one-fifth of the patients. Genetic screening should be offered to patients with PCC and PGL.


2015 ◽  
Vol 2015 ◽  
pp. 1-5 ◽  
Author(s):  
Elvira Ferrés-Amat ◽  
Jordi Prats-Armengol ◽  
Isabel Maura-Solivellas ◽  
Eduard Ferrés-Amat ◽  
Javier Mareque-Bueno ◽  
...  

Intraosseous arteriovenous malformations (AVMs) in the head and neck region are uncommon. There are several types and they can have a wide range of clinical presentations. Depending on the blood flow through the AVM, the treatment may be challenging for the attending team and may lead to life-threatening hemorrhages. A clinical case report is presented. A 9-year-old girl, seen for gingival bleeding during oral hygiene, was found to have a high-flow AVM located within and around the mandible. Two-stage treatment consisted of intra-arterial embolization followed by intraoral injection of a sclerosing agent 8 weeks later. At the 8-year follow-up, imaging study showed no evidence of recurrent lesion inside or outside the bone. The final outcome is a correct occlusion with a symmetric facial result. This case shows that conservative treatment may be the first treatment option mostly in children. Arteriography and transcortical injection were enough to control the AVM.


Stroke ◽  
2020 ◽  
Vol 51 (Suppl_1) ◽  
Author(s):  
Reda M Chalhoub ◽  
Ali M Alawieh ◽  
Mohammad Anadani ◽  
Maya Eid ◽  
Adam Arthur ◽  
...  

Introduction: Elderly patients, octogenarians and nonagenarians, were excluded or under-represented in the majority of stroke endovascular thrombectomy (ET) trials. There is conflicting data on the outcomes of ET in the elderly. We evaluated age-dependent outcomes of ET for stroke in a large dataset from the Stroke Thrombectomy and Aneurysm Registry (STAR). Methods: Patients undergoing ET for acute ischemic stroke at 12 comprehensive stroke centers in the US and Europe between 01/2013 and 12/2018 were reviewed. Data was collected retrospectively from patient charts, procedure notes, and patient follow-up in neurology clinics. The primary endpoint was the modified Rankin score (mRS) at 90-days which was dichotomized into good outcome (mRS 0-2) or poor outcome (mRS 3-6). Results: Of 3,850 patients reviewed, 2,827 had 90-day follow-up (mean age 69±14), and were divided into 6 age groups: 20-49 (G1, 10%), 50-59 (G2, 10%), 60-69 (G3, 23%), 70-79 (G4, 27%), 80-89 (G5, 21%), 90 or more (G6, 4%). When adjusted for confounding variables, age was an independent predictor of poor outcome (OR=1.4, p<0.001) and mortality (OR=1.5, p<0.0001). When used as categorical variable, adjusted OR (aOR) for good outcomes were significantly lower in groups G2-G6 compared to G1 (p<0.01, figure), and OR for mortality were significantly higher in G2-G6 compared to G1 (p<0.01, figure). An age increment of 10 years was associated with 23% higher odds of symptomatic hemorrhage, and 50% higher odds of mRS 5-6. The impact of procedure time on good outcome (mRS 0-2) was also age-dependent with aOR=0.84 (p<0.05) in G1,2 compared to aOR=0.65 (p<0.05) in G5,6. Conclusions: Age is a major predictor of functional recovery after ET, and this study demonstrates a clear age-dependent increase in rate of mortality and poor outcomes after ET with exponentially worse outcomes above 80 years of age. Complication rates were not age-dependent. Further studies are required to optimize patient selection for ET in the elderly.


Neurosurgery ◽  
2010 ◽  
Vol 66 (6) ◽  
pp. 1128-1133 ◽  
Author(s):  
Byung Moon Kim ◽  
Dong Joon Kim ◽  
Dong Ik Kim ◽  
Sung Il Park ◽  
Sang Hyun Suh ◽  
...  

Abstract OBJECTIVE To evaluate clinical presentation, safety, techniques, clinical and angiographic outcomes, and prognostic factors of coiling for remnant/recurred aneurysm after clipping. METHODS Twenty-four consecutive patients (11 men and 13 women; mean age, 52 years) with 24 recurred/remnant aneurysms after clipping underwent coil embolization between September 2000 and December 2008. Clinical presentations of remnant/recurred aneurysms, safety, techniques, clinical and angiographic outcomes, and prognostic factors of coil embolization were retrospectively evaluated. RESULTS Twenty-two aneurysms initially presented with subarachnoid hemorrhage and the other two, with mass effect. Eight aneurysms presented with rebleeding and 16 aneurysms were found on follow-up CT angiogram (n = 12) or catheter angiogram (n = 4). The interval between clipping and coiling ranged from 8 days to 114 months (mean, 31 months). Twelve were treated by using single-catheter, 6 by stent-assisted, 4 by multicatheter, 1 by both balloon- and catheter-assisted, and 1 by balloon-in-stent technique. Immediate postembolization angiogram revealed complete obliteration (n = 19) or residual neck (n = 5). Procedure-related permanent morbidity and mortality rates were 4.2% (1 of 24) and 0%, respectively. There was no rebleeding during clinical follow-up for 3 to 82 months (mean, 24 months). Presentation with rupture after clipping was the only significant predictor of poor outcome (P &lt; .05). CONCLUSION Coiling seems to be a safe and effective retreatment option for remnant/ recurred aneurysm after clipping. Presentation with rupture after clipping is the only predictor of poor outcome. For routine/regular follow-up after clipping, CT angiography may be the imaging modality advisable for detection of remnant/recurred aneurysm.


2019 ◽  
Vol 90 (3) ◽  
pp. e52.3-e52
Author(s):  
J Fahmy ◽  
T Boumrah ◽  
S Trippier ◽  
A Hainsworth ◽  
J Madigan ◽  
...  

ObjectivesTo study the factors associated with outcome in poor grade SAH in a busy tertiary centre.DesignRetrospective records review.SubjectsAll Patients with SAH WFNS grades IV and V admitted Jan 2016-Dec 2017.MethodsWe admitted 379 SAH patients, 84 (22%) were poor grade (n=84, 33M/51F mean age 60.7±1.4 y). Outcome was assessed by Modified Rankin Scale (mRS) scores before surgery and at latest follow up (3–6 months). mRS was dichotomised as good (0–3) and poor (4–6).Spearman’s rank-order test evaluated correlation between latest mRS and all other variables (WFNS grade, GCS, Motor score of GCS, age, sex, smoking, hypertension, intraventricular haemorrhage (IVH) and intracerebral haemorrhages (ICH)).Results63 patients (75%) had poor outcome, of which 46 (55%) died (44 with 30 days), versus 21 (25%) had good outcome. Spearman’s correlation analysis revealed that patients with smaller aneurysms (3.3±0.4 mm in good outcome patients vs 11.3±1.2 mm in poor outcome) (rs=0.37, p=0.009), who are younger (rs=0.24, p=0.03), have higher GCS (rs=−0.24, p=0.03), higher motor score (rs=0.25, p=0.02), lower WFNS grade (rs=0.3, p=0.007) and received coiling of aneurysms vs no treatment (rs=−0.39, p<0.0001) had better outcome. There was no significant correlation in outcome with ICH, IVH, external ventricular drain insertion, location of aneurysms, smoking, hypertension, other co-morbidities or sex.ConclusionsIn poor grade SAH, younger patients with smaller aneurysms, higher GCS and higher motor score who received endovascular coiling had better outcome.


2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A1017-A1018
Author(s):  
Irene Gagliardi ◽  
Mario Tarquini ◽  
Elisa Giannetta ◽  
Patricia Borges de Souza ◽  
Giovanni Lanza ◽  
...  

Abstract Background: Typical and atypical bronchial carcinoids (TBC and ABC) display a wide range of clinical presentations and may behave very differently. Survival prognostic markers are necessary to better define therapeutic strategies. AIM: verify that the NEP-Score, recently proposed as prognostic score, can be applied in a homogeneous TBC and ABC cohort and identify a derivate prognostic marker taking into account clinical and pathological characteristics at diagnosis. Methods: Age, site of primary tumor, primary tumor surgery, symptoms, Ki67, timing of metastases of 64 patients including TBC and ABC were evaluated to calculate the NEP-Score at the end of follow-up (NEP-T). We then assessed a derivative score considering the NEP-Score at diagnosis (NEP-D): this score does not consider the appearance of new metastases during follow-up. We then considered the patients that were alive or dead at the end of follow-up (EOF). A NEP-Score threshold to predict survival was investigated. Results: live patients at EOF displayed a mean NEP-T and mean NEP-D significantly lower as compared to those that were dead. A NEP-T threshold &gt;138 significantly predicts survival. ABC relapsed more frequently as compared to TBC. Male gender as well as previous malignancy were negative prognostic factors for survival. Conclusions: We found that NEP-Score is applicable to a series of bronchial neuroendocrine neoplasms. In addition, we propose NEP-D as a simple, quick and cheap prognostic score that can help clinicians in decision making. Moreover, the use of a NEP-D threshold can predict NEN aggressiveness and may be used to define the best personalized therapeutic strategy. Furthermore we found additional prognostic factors that together with the NEP-Score could improve prognosis evaluation at diagnosis by using easily accessible information.


1990 ◽  
Vol 36 (2) ◽  
pp. 346-348 ◽  
Author(s):  
P Rosenthal ◽  
M Haight

Abstract To assess the utility of the serum aspartate aminotransferase/alanine aminotransferase (AST/ALT) ratio in a group of infants with liver disorders, we retrospectively analyzed the charts of 73 infants with chronic liver disorders. Patients were considered as having either a good outcome (n = 40) or a poor outcome (n = 33), based upon the clinical course. AST and ALT in serum were measured simultaneously at the time of initial presentation and at various follow-up visits during the first 13 months after birth. At presentation (mean age 1.65 months), there was no difference in the AST/ALT ratios between the good (1.61 +/- 0.62; mean +/- SD) and poor (1.65 +/- 0.78) outcome groups (P = 0.81). However, over time, the AST/ALT ratio increased in patients in the poor-outcome group and decreased in patients in the good-outcome group. Calculating the AST/ALT ratio appears to be an easy, early, and reliable prognostic indicator for infants with hepatic disease, and may be a useful measure for evaluating liver-disease patients.


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