Αρχειοθήκη ιστολογίου

Αλέξανδρος Γ. Σφακιανάκης
ΩτοΡινοΛαρυγγολόγος
Αναπαύσεως 5
Άγιος Νικόλαος Κρήτη 72100
2841026182
6032607174

Πέμπτη 3 Φεβρουαρίου 2022

Metastatic Meningioma of the Neck: A Case Report and Systematic Review

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Background: Although meningiomas are the most common central nervous system neoplasms, extracranial metastases are exceedingly rare. There are even fewer reports of metastatic meningiomas to the neck. Methods: We described a patient with multiply recurrent orbital meningioma with metastasis to the neck found incidentally during neck exploration for composite resection and free tissue reconstruction. We performed a systematic review for all records pertaining to metastatic meningiomas to the cervical regions. Results: We found 9 previous reports of cervical metastatic meningiomas. Almost all cases underwent extensive local resection. There was no evidence of an association between the histological grade of the tumor and risk of metastasis to the neck. Cervical lymph node dissemination is more common in patients presenting after previous primary tumor resection. Conclusions: In the context of a neck mass, our findings suggest that metastatic meningioma should be included in the differential diagnosis, especially in patients with previous resections.
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Oculo-auriculo-vertebral spectrum: new genes and literature review on a complex disease

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J Med Genet. 2022 Feb 2:jmedgenet-2021-108219. doi: 10.1136/jmedgenet-2021-108219. Online ahead of print.

ABSTRACT

Oculo-auriculo-vertebral spectrum (OAVS) or Goldenhar syndrome is due to an abnormal development of first and second branchial arches derivatives during embryogenesis and is characterised by hemifacial microsomia associated with auricular, ocular and vertebral malformations. The clinical and genetic heterogeneity of this spectrum with incomplete penetrance and variable expressivity, render its molecular diagnosis difficult. Only a few recurrent CNVs and genes have been identified as causatives in this complex disorder so far. Prenatal environmental causal factors have also been hypothesised. However, most of the patients remain without aetiology. In this review, we aim at updating clinical diagnostic criteria and describing genetic and non-genetic aetiologies, animal models as well as novel diagnostic tools and surgical managemen t, in order to help and improve clinical care and genetic counselling of these patients and their families.

PMID:35110414 | DOI:10.1136/jmedgenet-2021-108219

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Prognostic scores in laryngeal cancer

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Eur Arch Otorhinolaryngol. 2022 Feb 2. doi: 10.1007/s00405-021-07233-2. Online ahead of print.

ABSTRACT

PURPOSE: To evaluate the utility of various pre-treatment prognostic scoring systems for overall survival (OS) in laryngeal cancer, including neutrophil-to-lymphocyte ratio (NLR), platelet-to-lymphocyte ratio (PLR), lymphocyte-to-monocyte ratio (LMR), modified Glasgow Prognostic Score (mGPS) and systemic immune-inflammatory index (SIII).

METHODS: We undertook a retrospective 5-year study of 220 patients with laryngeal squamous cell carcinoma undergoing active treatment.

RESULTS: On multivariate analysis, low NLR (≤ 2.415, p = 0.001, OR 3.851), low PLR (≤ 269.855, p = 0.002, OR 5.520), high LMR (> 2.225, p < 0.001, OR 0.458) and low SIII (≤ 1144.465, p = 0.003, OR 3.673) were significantly associated with improved OS, accounting for confounding factors of tumour subsite, T-stage and performance status. C-reacti ve protein (CRP) alone (p = 0.264) and mGPS (p = 0.350) were not significantly associated with OS.

CONCLUSIONS: NLR, PLR, LMR and SIII represent inexpensive, easily obtainable adjuvant decision-making tools which could help tailor individualised treatment regimes. Further investigation into the utility of combination scores and the role of different laryngeal subsites may be of interest.

PMID:35112153 | DOI:10.1007/s00405-021-07233-2

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Evaluating Risk of Noise-Induced Hearing Loss in Otologic Surgery

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Ann Otol Rhinol Laryngol. 2022 Feb 3:34894221075422. doi: 10.1177/00034894221075422. Online ahead of print.

ABSTRACT

OBJECTIVES: To evaluate risk for noise-induced hearing damage from otologic surgery-related noise exposure, given recent research indicating that noise levels previously believed to be safe and without long-term consequence may result in cochlear synaptopathy with subsequent degeneration of spiral ganglion neurons, degradation of neural transmission in respons e to suprathreshold acoustic stimuli, and difficulty understanding in background noise.

METHODS: A prospective observational study of surgeon noise exposure during otologic and neurotologic procedures was performed in a tertiary care center. Surgeon noise exposure was recorded in A- and C-weighted decibel scales (dBA, dBC), including average equivalent (LAeq) and peak (LApeak, LCpeak) levels and noise dose.

RESULTS: Sound measurements taken at the ear with continuous recording equipment during cadaveric otologic surgery demonstrated LAeq 80-83 dBA, LApeaks of 105 dBA, LCpeaks of 127 dBC, with noise doses of 0.9% to 6.7%. Sound level measurements during live surgery translabyrinthine approaches yielded lower LAeq of 72 to 74 dBA and lower noise doses compared with temporal bone lab measurements. Raw sound recordings during live surgery demonstrated narrow band, high frequency, high amplitude spikes between 4 and 12 kHz.

CONCLUSION: Noise exposure to surgeons, staff, and patients in the operating room is acceptable per NIOSH recommendations. Temporal bone lab noise exposures are greater, possibly due to poorly maintained drill systems and lack of noise shielding from microscope bulk, yet are also within NIOSH recommended levels.

PMID:35109716 | DOI:10.1177/00034894221075422

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Replacement and Immunomodulatory Activities of 20% Subcutaneous Immunoglobulin Treatment: A Single-Center Retrospective Study in Autoimmune Myositis and CVID Patients

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Front Immunol. 2022 Jan 17;12:805705. doi: 10.3389/fimmu.2021.805705. eCollection 2021.

ABSTRACT

BACKGROUND: Immunoglobulin (Ig) replacement therapy represents a life-saving treatment in primary antibody deficiencies. The introduction of subcutaneous Ig (SCIg) administration brings a major improvement in quality of life for patients, compared to the traditional intravenous administration. In recent years, an additional role has been proposed for Ig therapy for various inflammatory and immune-mediated diseases. Consequently, the use of SCIg has expanded from immunodeficiencies to immune-mediated diseases, such as polymyositis (PM) and dermatomyositis (DM). Given the rarity of these conditions, it is still difficult to evaluate the real impact of SCIg treatment on PM and DM, and additional data are constantly required on this topic, particularly for long-term treatments in real-life settings.

AIM: This study aimed to increase the knowledg e about the anti-inflammatory and immunomodulatory effects of SCIg treatment for myositis. To this aim, a long-term evaluation of the effectiveness of 20% human SCIg treatment (20% SCIg, Hizentra®, CSL Behring) was carried out in patients with PM/DM in care at our Center. In addition, an evaluation of the 20% SCIg therapy in CVID patients was provided. This analysis, beside adding knowledge about the use of SCIg therapy in this real-life setting, was intended as a term of comparison, regarding the safety profile.

RESULTS: Results support the beneficial effect and tolerability of long-term 20% SCIg therapy in PM/DM patients, reporting a significant improvement in creatine kinase levels, muscle strength, skin conditions, dysphagia, disease activity (MITAX score) and disability (HAQ-DI score). None of the patients reported systemic reactions. The duration of the reported local reactions was a few hours in 80% of the patients, and all resolved spontaneously. CVID patie nts reported an improvement in all the considered effectiveness parameters at the end of 20% SCIg therapy. The frequency of the adverse events reported by PM/DM patients was not different from what reported in CVID patients, where the use of SCIg therapy is more consolidated.

CONCLUSIONS: This study suggests that 20% SCIg treatment represents a viable and safe treatment for PM/DM patients and a valid therapeutic alternative to IVIg, with important advantages for patients' quality of life.

PMID:35111165 | PMC:PMC8801806 | DOI:10.3389/fimmu.2021.805705

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Oculo-auriculo-vertebral spectrum: new genes and literature review on a complex disease

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J Med Genet. 2022 Feb 2:jmedgenet-2021-108219. doi: 10.1136/jmedgenet-2021-108219. Online ahead of print.

ABSTRACT

Oculo-auriculo-vertebral spectrum (OAVS) or Goldenhar syndrome is due to an abnormal development of first and second branchial arches derivatives during embryogenesis and is characterised by hemifacial microsomia associated with auricular, ocular and vertebral malformations. The clinical and genetic heterogeneity of this spectrum with incomplete penetrance and variable expressivity, render its molecular diagnosis difficult. Only a few recurrent CNVs and genes have been identified as causatives in this complex disorder so far. Prenatal environmental causal factors have also been hypothesised. However, most of the patients remain without aetiology. In this review, we aim at updating clinical diagnostic criteria and describing genetic and non-genetic aetiologies, animal models as well as novel diagnostic tools and surgical managemen t, in order to help and improve clinical care and genetic counselling of these patients and their families.

PMID:35110414 | DOI:10.1136/jmedgenet-2021-108219

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Surgical strategy for squamous cell carcinoma of the external auditory canal: management of locally advanced cases with skull base involvement

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J Neurol Surg B Skull Base
DOI: 10.1055/a-1733-2585

Objective Surgical indications for advanced-stage squamous cell carcinoma (SCC) of the external auditory canal (EAC) are highly dependent on the skull base surgery team. The aim of this study was to evaluate the surgical outcomes in patients with SCC of the EAC and to clarify the surgical indication of far advanced cases using the T4-subclassification. Methods Patients with SCC of the EAC who underwent curative treatment from 2002-2021 were retrospectively reviewed at our hospital. Clinical and surgical results, including operative data, overall survival (OS), and disease-specific survival (DSS) were analyzed. To clarify the surgical indication for advanced stage, we proposed the T4-subclassification. Results In 46 patients, the tumors were in T1 stage in 8 patients, T2 in 10, T3 in 5, and T4 in 23. The 5-year DSS with T1, T2, T3, and T4 tumors were 100%, 85.7%, 100%, and 61.7%, respectively. No prognostic impacts for margin status were found between the 5-year OS and DSS (p = 0.23 and p = 0.13). Patients with far advanced stage (T4b) tumors were significantly associated with shorter DSS than those with early stage (T1/T2) and advanced stage (T3/T4a) tumors (p = 0.007 and p = 0.03). Conclusion The present study focused on patients with SCC of the EAC at a University hospital over a period of 20-years, especially with skull base involvement, and a T4-subclassification was proposed. Complete tumor resection in an en bloc fashion could help to achieve a good survival rate even in patients with locally advanced tumors.
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