scholarly journals Gingival Bleeding of a High-Flow Mandibular Arteriovenous Malformation in a Child with 8-Year Follow-Up

2015 ◽  
Vol 2015 ◽  
pp. 1-5 ◽  
Author(s):  
Elvira Ferrés-Amat ◽  
Jordi Prats-Armengol ◽  
Isabel Maura-Solivellas ◽  
Eduard Ferrés-Amat ◽  
Javier Mareque-Bueno ◽  
...  
Keyword(s):  
Imaging Study ◽  
Neck Region ◽  
Arterial Embolization ◽  
High Flow ◽  
Gingival Bleeding ◽  
Clinical Presentations ◽  
Wide Range ◽  
Life Threatening ◽  
Flow Through

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.

Updates and Advances: Pediatric Musculoskeletal Infection Imaging Made Easier for Radiologists and Clinicians

2021 ◽  
Vol 25 (01) ◽  
pp. 167-175
Author(s):  
Michael S. Furman ◽  
Ricardo Restrepo ◽  
Supika Kritsaneepaiboon ◽  
Bernard F. Laya ◽  
Domen Plut ◽  
...  
Keyword(s):  
Clinical Practice ◽  
Imaging Techniques ◽  
Daily Clinical Practice ◽  
Infants And Children ◽  
Imaging Evaluation ◽  
Musculoskeletal Infection ◽  
Wide Range ◽  
Life Threatening ◽  
Optimal Patient Management

AbstractInfants and children often present with a wide range of musculoskeletal (MSK) infections in daily clinical practice. This can vary from relatively benign superficial infections such as cellulitis to destructive osseous and articular infections and life-threatening deep soft tissue processes such as necrotizing fasciitis. Imaging evaluation plays an essential role for initial detection and follow-up evaluation of pediatric MSK infections. Therefore, a clear and up-to-date knowledge of imaging manifestations in MSK infections in infants and children is imperative for timely and accurate diagnosis that, in turn, can result in optimal patient management. This article reviews an up-to-date practical imaging techniques, the differences between pediatric and adult MSK infections, the spectrum of pediatric MSK infections, and mimics of pediatric MSK infections encountered in daily clinical practice by radiologists and clinicians.

scholarly journals A 15-year pheochromocytoma and paraganglioma experience in a single centre: a Singapore perspective

2021 ◽  
Author(s):  
Y Lee ◽  
LYR Tan ◽  
SAKK Abdul Shakoor
Keyword(s):  
Positive Family History ◽  
Distant Metastases ◽  
Disease Recurrence ◽  
Haemodynamic Instability ◽  
Preoperative Preparation ◽  
Presenting Symptoms ◽  
Clinical Presentations ◽  
Wide Range

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.

Prognostic value of non-ischemic ring-like left ventricular scar pattern in patients with apparently idiopathic ventricular arrhythmias: a CMR imaging study

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
G Nucifora ◽  
D Muser ◽  
S Castro ◽  
R Casado Arroyo ◽  
D Benhayon ◽  
...  
Keyword(s):  
Ventricular Arrhythmias ◽  
Prognostic Significance ◽  
Imaging Study ◽  
Left Ventricular ◽  
High Rate ◽  
Life Threatening ◽  
History Of ◽  
Group A ◽  
Group B

Abstract Background The presence of left ventricular (LV) late gadolinium enhancement (LGE) at cardiac magnetic resonance (CMR) has been correlated to life-threatening arrhythmic events in patients with apparently idiopathic ventricular arrhythmias (VAs). Aim of the present study was to investigate the prognostic significance of a specific LV-LGE phenotype characterized by a subepicardial/midmyocardial “ring-like” pattern of fibrosis. Methods Out of a total of 518 consecutive patients with apparently idiopathic VAs who underwent CMR study, 79 (15%) had evidence of LV-LGE. Of these, 23 (4%) patients had LV LGE with ring-like pattern, defined as subepicardial or midmyocardial LGE involving at least 3 contiguous segments in the same slice (group A), while 56 (11%) patients had LV LGE with no ring-like pattern (group B). The remaining 439 patients had no LGE (group C). The end-point of the study was a composite SCD, resuscitated cardiac arrest and nonfatal episodes of ventricular fibrillation or documented sustained ventricular tachycardia. Results Group A patients were more frequently males compared to groups B and C (96% vs. 79% vs. 52%; p<0.01) and had more frequently a family history of SCD and/or cardiomyopathy (30% vs. 11% vs. 5%; p<0.01). All patients in Group A showed VAs with a predominant RBBB morphology vs. 38 (68%) patients in Group B and 65 (15%) in Group C (p<0.01). During a follow-up of 63±39 months, the composite outcome occurred in 13 patients (57%) in Group A vs. 11 (20%) in Group B and 2 (1%) in Group C (p<0.01). Conclusion In patients with apparently idiopathic VAs, a nonischemic LV-LGE with a ring-like pattern at CMR is associated with a high rate of malignant arrhythmic events during follow-up. Funding Acknowledgement Type of funding source: None

scholarly journals Disfiguring high-flow cervicofacial arteriovenous malformations

2020 ◽  
Vol 2020 (10) ◽  
Author(s):  
Alaa Nabawi ◽  
Nader Abraham ◽  
Ayman Nabawi
Keyword(s):  
Oral Cavity ◽  
Vascular Malformation ◽  
Arteriovenous Malformations ◽  
Case Series ◽  
High Flow ◽  
Vascular Anomalies ◽  
Maxillofacial Region ◽  
Clinical Onset ◽  
Life Threatening

Abstract Arteriovenous malformations (AVMs) are congenital vascular anomalies resulting from defects in angiogenesis. Approximately 40% of AVMs go undetected after birth and only experience the delayed clinical onset of symptoms in adulthood. AVMs are rare, representing only 1.5% of all vascular anomalies. The most common sites for the aberrant vascular nidus are the oral cavity and maxillofacial region, which represent 50% of the cases. AVMs are the most challenging and life-threatening form of vascular malformation. Exsanguination, thrombus detachment and embolization are the most hazardous operative risks. Small case series revealed a 75% recurrence rate during a 5-year follow-up, which adds another layer of complexity to their management. Large lesions in the head and neck cause deformation to the patient and present a challenge to the surgeon during their excision among vital structures and reconstruction of the 3D complex defects.

scholarly journals Right Atrial Diverticulum in an Adult Woman with Left Bundle Branch Block

2020 ◽  
Vol 20 (4) ◽  
pp. e394-396
Author(s):  
Behnam Shakerian ◽  
Mohammad H. Mandegar
Keyword(s):  
Left Bundle Branch Block ◽  
Imaging Study ◽  
Right Atrial ◽  
Magnetic Resonance Imaging Study ◽  
Resonance Imaging ◽  
Bundle Branch Block ◽  
Clinical Presentations ◽  
History Of ◽  
The Right

Right atrial diverticulum is a very rare anomaly. It is an outpouching arising from the right atrial free wall. Clinical presentations vary widely but some cases are associated with supraventricular tachycardia and atrial flutter/fibrillation. The incidence/prevalence of this anomaly is not available because only a few cases have been reported. We report a 38-year-old female patient who presented to the Heart Clinic, Tehran, Iran in 2019 with a history of dyspnea and chest pain. Electrocardiography revealed left bundle branch block. Following a magnetic resonance imaging study, the patient was diagnosed with a right atrial diverticulum. She underwent surgical resection of the diverticulum. The post-operative course was uneventful and no recurrence of the arrhythmia was detected during the six months of follow-up. To the best of the authors’ knowledge, this combination has not been described in the literature. Keywords: Right Atrium; Diverticulum; Left Bundle Branch Block; Case Report; Iran.

scholarly journals Mechanisms of Neuronal Damage in Acute Hepatic Porphyrias

Diagnostics ◽  
2021 ◽  
Vol 11 (12) ◽  
pp. 2205
Author(s):  
Andrea Ricci ◽  
Elena Di Pierro ◽  
Matteo Marcacci ◽  
Paolo Ventura
Keyword(s):  
Pyridoxal Phosphate ◽  
Aminolevulinic Acid ◽  
Neuronal Damage ◽  
Current Evidence ◽  
Metabolic Demand ◽  
Clinical Presentations ◽  
Wide Range ◽  
Different Types ◽  
Life Threatening ◽  
Functional Deficiency

Porphyrias are a group of congenital and acquired diseases caused by an enzymatic impairment in the biosynthesis of heme. Depending on the specific enzyme involved, different types of porphyrias (i.e., chronic vs. acute, cutaneous vs. neurovisceral, hepatic vs. erythropoietic) are described, with different clinical presentations. Acute hepatic porphyrias (AHPs) are characterized by life-threatening acute neuro-visceral crises (acute porphyric attacks, APAs), featuring a wide range of neuropathic (central, peripheral, autonomic) manifestations. APAs are usually unleashed by external “porphyrinogenic” triggers, which are thought to cause an increased metabolic demand for heme. During APAs, the heme precursors δ-aminolevulinic acid (ALA) and porphobilinogen (PBG) accumulate in the bloodstream and urine. Even though several hypotheses have been developed to explain the protean clinical picture of APAs, the exact mechanism of neuronal damage in AHPs is still a matter of debate. In recent decades, a role has been proposed for oxidative damage caused by ALA, mitochondrial and synaptic ALA toxicity, dysfunction induced by relative heme deficiency on cytochromes and other hemeproteins (i.e., nitric oxide synthases), pyridoxal phosphate functional deficiency, derangements in the metabolic pathways of tryptophan, and other factors. Since the pathway leading to the biosynthesis of heme is inscribed into a complex network of interactions, which also includes some fundamental processes of basal metabolism, a disruption in any of the steps of this pathway is likely to have multiple pathogenic effects. Here, we aim to provide a comprehensive review of the current evidence regarding the mechanisms of neuronal damage in AHPs.

Athletic participation in the young patient with an implantable cardioverter-defibrillator

2017 ◽  
Vol 27 (S1) ◽  
pp. S132-S137
Author(s):  
Rachel Lampert ◽  
Ian Law
Keyword(s):  
Implantable Cardioverter Defibrillator ◽  
Young Patient ◽  
Sports Participation ◽  
Implantable Cardioverter Defibrillators ◽  
Device Therapy ◽  
Cardioverter Defibrillator ◽  
Major Injury ◽  
Wide Range ◽  
Life Threatening

AbstractThe decision of whether to allow a young patient with an implantable cardioverter-defibrillator to continue to participate in sports is complex and multi-factorial. The positive physical and psychosocial impact of sports participation must be weighed against the potential adverse events associated with implantable cardioverter-defibrillators. Arrhythmias appear to be more prevalent in athletes and occur more frequently during physical activity or competition/practice, but there is growing evidence that device therapy is effective in athletes across a wide range of competitive sports. Failure of a device to convert a life-threatening arrhythmia, major injury from a shock, and increased lead failure have thus far not been reported in the prospective Implantable Cardioverter-Defibrillator Sports Registry, but follow-up remains relatively short. Thoughtful consideration of disease state, arrhythmia risk, and the potential dangers of device therapy during the desired sports is imperative before allowing participation. Frank discussion with children and families regarding the possibility of shocks during sports, as well as at other times, is imperative. Ongoing and future studies will help guide these decisions.

scholarly journals Evaluation of a Nep-Score Threshold and the Derived Nep-D Score in Predicting Survival of Patients With Typical and Atypical Bronchial Carcinoids

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A1017-A1018
Author(s):  
Irene Gagliardi ◽  
Mario Tarquini ◽  
Elisa Giannetta ◽  
Patricia Borges de Souza ◽  
Giovanni Lanza ◽  
...  
Keyword(s):  
Prognostic Factors ◽  
Primary Tumor ◽  
Prognostic Score ◽  
Neuroendocrine Neoplasms ◽  
Bronchial Carcinoids ◽  
Accessible Information ◽  
Score Threshold ◽  
Clinical Presentations ◽  
Wide Range

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 >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.

scholarly journals Hemorrhagic plaques in the oral cavity: A clue to diagnosing thrombocytopenia

2021 ◽  
Vol 12 (2) ◽  
pp. 166-166
Author(s):  
Aswathi Raj ◽  
Amina Asfiya ◽  
Malcolm Pinto ◽  
Manjunath Shenoy M ◽  
Spandana P Hegde ◽  
...  
Keyword(s):  
Oral Cavity ◽  
Idiopathic Thrombocytopenic Purpura ◽  
Oral Manifestations ◽  
Platelet Destruction ◽  
Thrombocytopenic Purpura ◽  
Platelet Production ◽  
Gingival Bleeding ◽  
Clinical Presentations ◽  
Wide Range ◽  
Hemorrhagic Bullae

Idiopathic thrombocytopenic purpura is a disorder with a myriad of possible clinical presentations. The mechanism of thrombocytopenia involves both increased platelet destruction and impaired platelet production. The patient can manifest a wide range of symptoms: from asymptomatic or minimal gingival bleeding to profuse bleeding from any site. The disease may first present itself to the dermatologist in cutaneous findings such as petechiae, purpura, and mucosal manifestations in the form of gingival bleeding and hemorrhagic bullae. The diagnosis of idiopathic thrombocytopenic purpura is mostly done by exclusion. In this report, we present two cases with characteristic oral manifestations, who were diagnosed, on investigation, with idiopathic thrombocytopenic purpura. The patients were successfully treated with immunosuppressive therapy. The report aims to raise awareness that would help in enabling prompt referral to the appropriate specialty, especially because of the rarity of this presentation.

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
Keyword(s):  
Vein Of Galen ◽  
Management Options ◽  
Good Outcome ◽  
Poor Outcome ◽  
Clinical Presentations ◽  
Wide Range ◽  
Management Perspective ◽  
Institutional Experience ◽  
Intracranial Vascular Malformation

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.

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