An Intraventricular Schwannoma with Associated Hydrocephalus and Ventricular Entrapment: A Case Report.
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ABSTRACT:
SUBMITTER: Curran-Melendez SM
PROVIDER: S-EPMC4520980 | biostudies-other | 2015 Jul
REPOSITORIES: biostudies-other
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Project description:Schwannoma is a benign peripheral nerve sheath tumor originating from Schwann cells. Most intracranial schwannomas arise from vestibular nerve and schwannoma in the suprasellar region is extremely rare. A 64-year-old man presented with walking disturbance and blurred vision for three months. Lateral hemianopsia in the left eye and brachybasia were observed. Magnetic resonance imaging revealed a suprasellar tumor with strong contrast enhancement associated with communicating hydrocephalus. The cerebrospinal fluid tap test improved gait disturbance. Hypothalamic stimulation test revealed hypo-reaction of GH, FSH and LH. After ventriculo-peritoneal shunting, the tumor was totally removed via a bilateral front-basal approach with a clinical diagnosis of craniopharyngioma. No adhesion was observed between the tumor and surrounding structures such as meninges and brain. The histopathological diagnosis was schwannoma. Here we report a case of suprasellar schwannoma associated with communicating hydrocephalus that has not ever been previously reported, with special reference to its pathogenesis.
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Project description:BackgroundLeft ventricular noncompaction cardiomyopathy (LVNC) is characterized by prominent ventricular trabeculations on cardiovascular imaging. Acquired reversible LVNC has not been reported in pediatrics without a genetic background.Case presentationA 9-year-old girl with a ventriculoperitoneal (VP) shunt for neonatal posthemorrhagic hydrocephalus was referred due to exacerbation of hydrocephalus caused by VP shunt dysfunction. Transthoracic echocardiography (TTE) revealed depressed left ventricular (LV) systolic function and thick prominent trabeculae in the LV, predominantly in the apex, suggesting LVNC. Following treatment with extraventricular drainage for hydrocephalus, prominent trabeculation of the LV was diminished on TTE within 3 months. Genetic testing using next-generation sequencing was performed, and no significant variants were identified.ConclusionsWe revealed for the first time a pediatric case of reversible LVNC without genetic predisposition. This case report provides valuable information on the pathogenesis of acquired LVNC and suggests that detailed evaluation is required to elucidate the diagnosis of this wide spectrum of etiologic-pathogenetic disorders.
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Project description:BackgroundIntraventricular haemorrhage (IVH) patients with acute obstructive hydrocephalus (AOH) who require external ventricular drainage (EVD) are at high risk for poor outcomes. Intraventricular fibrinolysis (IVF) with low-dose recombinant tissue plasminogen activator (rtPA) can be used to improve patient outcomes. Here, we evaluated the impact of IVF on the risk of death and the functional outcomes in IVH patients with AOH.MethodsThis prospective cohort study included IVH patients with hypertensive intracranial haemorrhage complicated by AOH who required EVD. We evaluated the risk of death and the functional outcomes at 1 and 3 months, with a specific focus on the impact of combined EVD with IVF by low-dose rtPA.ResultsBetween November 30, 2011 and December 30, 2014, 80 patients were included. Forty-five patients were treated with EVD alone (EVD group) and 35 received IVF (EVD+IVF group). The 30- and 90-day mortality rates were lower in the EVD+IVF group than in the EVD group (42.2 vs. 11.4%, p = 0.003, and 62.2 vs. 20%, p < 0.001, respectively). The Graeb scores were significantly lower in the EVD+IVF group than in the EVD group (p ≤ 0.001) during the first 3 days and on day 7 after assignment. The 30-day good functional outcome (modified Rankin Scale [mRS] score 0-3) was also higher in the EVD+IVF group than in the EVD group (6.7 vs. 28.6%, p = 0.008). However, the 90-day good functional outcome (mRS score 0-3) did not significantly increase in the EVD+IVF group (30.8% in the EVD group vs. 51.6% in the EVD+IVF group, p = 0.112).ConclusionsIn our prospective observational study, EVD+IVF was associated with a lower risk of death in IVH patients. EVD+IVF improved the chance of having a good functional outcome at 1 month; however, this result was no longer observed at 3 months.
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Project description:BackgroundUhl's anomaly is a rare congenital heart disease characterized by 'Paper-like thinning of the right ventricular (RV) wall'. Since most patients with Uhl's anomaly die in infancy or childhood, the adult cases of this disorder have been limitedly reported and there were no past report describing this anomaly with ventricular tachycardia (VT), in which catheter ablation was successfully performed.Case summaryWe report the case of a 43-year-old man with a suggested Uhl's anomaly and VT. He underwent a catheter ablation of the recurrent VT. An electrophysiological study showed the VT (cycle length = 460 ms) with a right bundle branch block pattern and inferior axis represented QRS alternans. Concealed entrainment by pacing from the anterior right ventricle (RV) suggested the mechanism of this VT was re-entry and the QRS alternans was simultaneously reproduced during the pacing. Furthermore, the intracardiac electrograms exhibiting local conduction blocks were documented on the RV free wall near the apex. The electrograms seen in a 2:1 ratio were coincident with slurs in the initial QRS on the 12-lead electrocardiogram. Since the anterior RV wall was considered to be a part of the VT circuit, a linear ablation in that area was performed resulting in VT termination.DiscussionThe local conduction blocks near the RV apex were related to the mechanism of the QRS alternans during the VT in this case. That might be based on the variations in the myocardium lying in the RV, which is the specific feature of Uhl's anomaly.
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Project description:BackgroundThe majority of ventricular tachycardias (VTs) occurs in patients with structural heart disease and is associated with an increased risk of sudden cardiac death. These VT are scar-related and may develop in patients with ischaemic or non-ischaemic cardiomyopathies.Case summaryWe describe a 44-year-old patient without any pre-existing cardiovascular disease, presenting with the first documentation of a haemodynamically unstable sustained fast VT with a cycle length of 250 ms. He reported a suicidal attempt with a self-made handgun aged 16 when he had shot himself in the thorax and had injured the myocardium. After presenting with the VT coronary artery disease was excluded through cardiac catheterization. A cardiovascular magnetic resonance study showed a localized myocardial scar in the left ventricular free wall starting from the subepicardium and correlating to the scar described 28 years ago by the thoracic surgeons. In an electrophysiological study, non-sustained VT were easily inducible. Presuming a causal relationship between the fast VT and the epicardial scar, a single-chamber implantable cardioverter-defibrillator was implanted and beta-blocker therapy was initiated.DiscussionScar-related VT often occur many years after an acute event, e.g. an acute myocardial infarction. This case highlights, that any cardiac trauma, even a superficial epicardial projectile-related damage with subsequent scarring, may cause a VT after many years and to our knowledge for the first time describes the occurrence of a VT due to mechanical damage to the myocardium by a gunshot.
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Project description:Introduction and importanceSchwannomas are benign tumors commonly found in the cranial vault at the cerebellopontine angle. Schwannomas could arise from any nerve that has Schwann sheath. Their most common extra-cranial localization is the parapharyngeal space. Schwannomas do not usually metastasize, and recurrence is uncommon.Case presentationWe present a case of a sympathetic trunk schwannoma in a patient that presented with dysphagia and a painful neck mass. Oro-pharyngeal examination revealed the presence of a mass filling-up the left tonsillar fossa, and pushing the uvula to the right side. The larynx was also deviated to the right due to the mass effect. The mass was resected en-bloc with the involved part of the sympathetic trunk. The patient developed Horner's syndrome postoperatively.Clinical discussionThe occurrence of Sympathetic trunk Schwannomas is very rare. The majority of patients presented with a cervical mass and non-specific symptoms. The most effective treatment is surgical resection. Our experience suggests sympathetic trunk Schwannomas as a differential diagnosis of slow-growing neck masses and asserts that the surgical resection is the main treatment.ConclusionThe occurrence of Schwannomas in the sympathetic trunk is rare. High clinical suspicion is required to achieve the preoperative diagnosis. The optimal management is the total surgical resection. Recurrence is uncommon when the mass is totally resected.
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Project description:BackgroundIntracranial intraparenchymal schwannomas (IS) are rare tumors that have mainly been described in case reports. Here, we report on a case of a brainstem IS and included a comprehensive literature review.Case descriptionA 74-year-old man presented with progressive gait disturbances. CT- and MRI-imaging revealed a contrast-enhancing mass accompanied by a cyst in the dorsolateral pons. Hemangioblastoma was suspected and surgery was advised. During surgery, gross total resection of a non-invasive tumor was performed. Postoperative recovery was uneventful. Based on histopathological examination, the intraparenchymal brainstem tumor was diagnosed as schwannoma.ConclusionOur extensive review illustrates that ISs are benign tumors that most often present in relatively young patients. Malignant cases have been described but form an extremely rare entity. Preoperative diagnosis based on radiological features is difficult but should be considered when peritumoral edema, calcifications, and cysts are noted. In benign cases, gross total resection of the lesion is curative. To adequately select this treatment and adjust the surgical strategy accordingly, it is important to include IS in the preoperative differential diagnosis when the abovementioned radiological features are present.
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Project description:Hypoglossal Schwannomas are extremely rare benign slow-growing neoplasms, which originate from the 12th cranial nerve. To date, and to the best of our knowledge, only 40 cases of dumbbell-shaped Hypoglossal Swchannomas have been published in the world literature. We report our experience with a 66 years old male patient, who was diagnosed with a solido-cystic lesion at the right cerebello-pontine angle arising from XIIth cranial nerve. He was treated with surgery via midline suboccipital approach which led to sub-total removal of the tumor and improvement of the symptoms within 3 months. This case highlights the importance of an accurate suspicion diagnosis of hypoglossal schwannoma as well as the treatment options including surgery and radiosurgery.
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