Operative Procedure in a Suprasellar Paediatric Lesion of the Optic Chiasm with Hydrocephalus Caused by a Papillary Glioneuronal Tumour.

INTRODUCTION: Paediatric tumours in the sellar and parasellar regions present clinical and surgical challenges due to anatomical position and behaviour. We illustrate a rare case which caused obstructive hydrocephalus. CASE PRESENTATION: The study included a 14-year-old girl with a glioneuronal tumour (40 mm) originating from the optic chiasm, obliterating the aqueduct, with consequent triventricular hydrocephalus. The patient underwent extended endoscopic endonasal surgery and repair of the skull-base deficiency using a multi-layer technique with fascia lata. The 12-month follow-up showed no complications or recurrences, with recovery in visual acuity. CONCLUSION: The immediate placement of external ventricular drainage, in combination with an extended trans-sphenoidal approach, is a safe and feasible option to treat suprasellar paediatric lesions with hydrocephalus.

Diagnostic Workflow in Competitive Athletes with Ventricular Arrhythmias and Suspected Concealed Cardiomyopathies.

The diagnosis of structural heart disease in athletes with ventricular arrhythmias (VAs) and an apparently normal heart can be very challenging. Several pieces of evidence demonstrate the importance of an extensive diagnostic work-up in apparently healthy young patients for the characterization of concealed cardiomyopathies. This study shows the various diagnostic levels and tools to help identify which athletes need deeper investigation in order to unmask possible underlying heart disease.

Post-traumatic Delayed Facial Nerve Palsy: Report of 2 Cases and Systematic Review.

OBJECTIVE: Delayed facial nerve palsy (dFNP) secondary to head injury is definitely uncommon. Although the mechanism of immediate facial nerve paralysis is well-studied, its delayed presentation remains debated. Given the dearth of available information, we reported herein our experience with 2 cases of posttraumatic dFNP. This systematic review aimed to evaluate all available information on dFNP and to assess treatment outcome also comparing conservatively and surgically approaches. DATA SOURCES: Pubmed, Scopus, and Web of Science databases were systematically screened. REVIEW METHODS: The protocol of this investigation was registered on PROSPERO in April 2023 and the systematic review was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-analyses statement. RESULTS: Both patients in the case studies showed a complete recovery within 2 to 3 months after the head trauma. One of them still reported a subjective taste alteration at last control. After the application of the inclusion-exclusion criteria, 9 manuscripts with adequate relevance to this topic were included in the systematic review. The study population consisted of 1971 patients with a diagnosis of posttraumatic facial nerve palsy, of which 128 with a dFNP. CONCLUSIONS: dFNP due to head trauma is a rarely encountered clinical entity, and optimal treatment still remains to be elucidated. Based on the reported data, it seems rational to propose a conservative approach for dFNP with steroid administration as a first line in most cases, indicating surgery in severe and/or refractory cases.

Reversible Causes of Atrioventricular Block.

Atrioventricular blocks may be caused by a variety of potentially reversible conditions, such as ischemic heart disease, electrolyte imbalances, medications, and infectious diseases. Such causes must be always ruled out to avoid unnecessary pacemaker implantation. Patient management and reversibility rates depend on the underlying cause. Careful patient history taking, monitoring of vital signs, electrocardiogram, and arterial blood gas analysis are crucial elements of the diagnostic workflow during the acute phase. Atrioventricular block recurrence after the reversal of the underlying cause may pose an indication for pacemaker implantation, because reversible conditions may actually unmask a preexistent conduction disorder.

Ventricular tachycardia ablation through radiation therapy (VT-ART) consortium: Concept description of an observational multicentric trial via matched pair analysis.

Introduction: Monomorphic ventricular tachycardia (VT) is a life-threatening condition often observed in patients with structural heart disease. Ventricular tachycardia ablation through radiation therapy (VT-ART) for sustained monomorphic ventricular tachycardia seems promising, effective, and safe. VT-ART delivers focused, high-dose radiation, usually in a single fraction of 25 Gy, allowing ablation of VT by inducing myocardial scars. The procedure is fully non-invasive; therefore, it can be easily performed in patients with contraindications to invasive ablation procedures. Definitive data are lacking, and no direct comparison with standard procedures is available. Discussion: The aim of this multicenter observational study is to evaluate the efficacy and safety of VT-ART, comparing the clinical outcome of patients undergone to VT-ART to patients not having received such a procedure. The two groups will not be collected by direct, prospective accrual to avoid randomization among the innovative and traditional arm: A retrospective selection through matched pair analysis will collect patients presenting features similar to the ones undergone VT-ART within the consortium (in each center independently). Our trial will enroll patients with optimized medical therapy in whom endocardial and/or epicardial radiofrequency ablation (RFA), the gold standard for VT ablation, is either unfeasible or fails to control VT recurrence. Our primary outcome is investigating the difference in overall cardiovascular survival among the group undergoing VT-ART and the one not exposed to the innovative procedure. The secondary outcome is evaluating the difference in ventricular event-free survival after the last procedure (i.e., last RFA vs. VT-ART) between the two groups. An additional secondary aim is to evaluate the reduction in the number of VT episodes comparing the 3 months before the procedure to the ones recorded at 6 months (from the 4th to 6th month) following VT-ART and RFA, respectively. Other secondary objectives include identifying the benefits of VT-ART on cardiac function, as evaluated through an electrocardiogram, echocardiographic, biochemical variables, and on patient quality of life. We calculated the sample size (in a 2:1 ratio) upon enrolling 149 patients: 100 in the non-exposed control group and 49 in the VT-ART group. Progressively, on a multicentric basis supervised by the promoting center in the VT-ART consortium, for each VT-ART patient enrollment, a matched pair patient profile according to the predefined features will be shared with the consortium to enroll a patient that has not undergone VT-ART. Conclusion: Our trial will provide insight into the efficacy and safety of VT-ART through a matched pair analysis, via an observational, multicentric study of two groups of patients with or without VT-ART in the multicentric consortium (with subgroup stratification into dynamic cohorts).

Successful ventricular tachycardia radioablation in a patient with previous chemical pleurodesis: A case report.

Introduction: Stereotactic arrhythmia radioablation (STAR) is a novel technique for the ablation of ventricular tachycardia in patients with contraindications to standard procedures, i.e., radiofrequency ablation. Case presentation: We report the case of a 73-year-old man with non-ischemic dilated cardiomyopathy and recurrent VT episodes. Electroanatomic mapping showed VT prevalently of epicardial origin, but direct epicardial access through subxyphoid puncture could not be performed due to pleuropericardial adhesions from a past history of chemical pleurodesis. STAR was performed, with no VT recurrence at 6 months follow-up. Conclusions: Previous experiences with STAR have demonstrated its importance in the management of patients with refractory VT in whom other ablation strategies were not successful. Our case report highlights the use of STAR as a second choice in a patient with an unfavorable VT anatomical location and technical limitations to an optimal radiofrequency ablation. Moreover, it confirms STAR's effectiveness in the ablation of complex transmural lesions, which are more often associated with non-ischemic structural heart disease.

Reversible Causes of Atrioventricular Block.

Atrioventricular blocks may be caused by a variety of potentially reversible conditions, such as ischemic heart disease, electrolyte imbalances, medications, and infectious diseases. Such causes must be always ruled out to avoid unnecessary pacemaker implantation. Patient management and reversibility rates depend on the underlying cause. Careful patient history taking, monitoring of vital signs, electrocardiogram, and arterial blood gas analysis are crucial elements of the diagnostic workflow during the acute phase. Atrioventricular block recurrence after the reversal of the underlying cause may pose an indication for pacemaker implantation, because reversible conditions may actually unmask a preexistent conduction disorder.

Endoglin (CD105) expression in neurofibromatosis type 2 vestibular schwannoma.

BACKGROUND: Neurofibromatosis type 2 (NF2) is an autosomal dominant, multiple neoplasia syndrome characterized by bilateral vestibular schwannomas (VSs). Endoglin is a proliferation-associated protein expressed in angiogenic endothelial cells. The aim of this study was to investigate endoglin expression in a series of NF2-associated VSs, as compared with a group of sporadic VSs. METHODS: Using image analysis, vessel cross-sectional area (AA) and density (VD) were calculated from CD105 expression in 7 NF2-associated VSs and 14 size-matched sporadic VSs. RESULTS: There were no significant differences between NF2-associated VSs and sporadic cases in terms of AA (P = .28), or VD (P = .39). A positive correlation emerged between tumor growth rate (measured on contrast-enhanced MRI) and VD in the cohort of NF2-associated VSs (rho = 0.95, P = .05). CONCLUSIONS: Further investigations are needed to ascertain the feasibility of (a) measuring circulating endoglin levels to monitor tumor growth rate and (b) targeting tumor neoangiogenesis with anti-endoglin approaches in NF2-associated VS.

Hearing Preservation Surgery via Retrosigmoid Approach with Retrolabyrinthine Meatotomy in Small Vestibular Schwannoma.

Objectives Vestibular schwannomas (VS) extending into the internal auditory canal (IAC) are currently considered as an unfavorable condition for hearing preservation surgery (HPS) via retrosigmoid (RS) approach due to the poor direct visualization of the facial and cochlear nerves course through the IAC and the fundus during microsurgery. Design The operative steps are described in a surgical instructional video. Setting The surgery took place at a tertiary-care center. Participants Patient is a 45-year-old man who was incidentally diagnosed with an extrameatal VS extending for 9 mm into the left cerebellopontine angle (CPA). Hearing function at diagnoses was excellent, with pure tone average (PTA) = 15 dB and speech discrimination score (SDS) = 100% (class A according to the Tokyo classification) and minimal impairments on auditory brainstem response (ABR). Given these preoperative features in small VS, hearing was the main function to look into in the treatment planning. Initial observation or HPS were proposed. Results The patient underwent surgical excision for HPS via RS approach combined with retrolabyrinthine meatotomy (RLM). RLM enables the complete exposure of the IAC to the fundus, after drilling the bony surface of the posterior IAC wall, under guidance of the anatomical landmarks, namely, the endolymphatic duct, the blue lines of the posterior, and superior semicircular canals, and common crus. Nor intra-, neither postoperative complications occurred. Histologic examination confirmed the diagnosis of VS. A 3-month short-term follow-up revealed a class B hearing function with PTA = 30 dB, SDS = 100%, and normal facial nerve status. Conclusions RLM via RS approach proved to be effective for HPS, enabling the full course of the facial and cochlear nerves through the IAC to be directly exposed. The link to the video can be found at: https://youtu.be/KC1S4pxpLCk .

Endoglin-based assessment of neoangiogenesis in sporadic VIII cranial nerve schwannoma.

Although the diagnosis and treatment of sporadic vestibular schwannoma has improved in recent years, no factors capable of predicting its growth have been identified as yet. Endoglin (CD105) is a proliferation-associated protein expressed in angiogenic endothelial cells, and a potential prognostic indicator for several solid tumors. The aim of the present study was primarily to investigate the expression and role of CD105 in a series of sporadic vestibular nerve schwannomas. In 71 consecutive cases of vestibular schwannoma, vessel cross-sectional area and density were calculated from immunohistochemically assessed CD105 expression using image analysis to correlate them with: (i) tumor dimensions; and (ii) tumor growth rate measured on high-resolution contrast-enhanced MRI (ceMRI). Based on assessments of CD105 expression, a significant positive correlation was identified between vessel cross-sectional area and tumor size at the time of surgery (p = 0.0024), and between vessel density and tumor size (p = 0.013). Vessel cross-sectional area (p = 0.0006) and vessel density (p = 0.003) were significantly greater in tumors measuring ≥10 mm in size than in those <10 mm. Conversely, when tumor growth rate could be calculated from two or more ceMRI (38 cases), there was no significant correlation between tumor growth rate and cross-sectional vessel area or vessel density as assessed with CD105. Further investigations are needed to ascertain the feasibility of: (i) using circulating endoglin assay to monitor tumor growth; and (ii) targeting neoangiogenesis with anti-endoglin antibodies in sporadic vestibular schwannoma.