Πέμπτη 3 Φεβρουαρίου 2022

Changes in the Sphenoid Bone Encountered During the Endoscopic Endonasal Transsphenoidal Approach

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Objectives

We estimated volume changes in the posterior bony wall of the sphenoid sinus, as well as alterations in nasal function (including olfactory function and subjective symptoms), after sphenoid mucosal repositioning using the endoscopic endonasal transsphenoidal approach (EETSA).

Methods

During 2010 and 2021, 13 patients underwent sphenoid mucosal repositioning during EETSA, while 24 patients (the control group) did not. Pre- and postoperative paranasal sinus computed tomography and the Mimics program were used to evaluate three-dimensional changes in the posterior wall of the sphenoid sinus. All patients underwent the Connecticut Chemosensory Clinical Research Center (CCCRC) test, the Cross-Cultural Smell Identification Test (CCSIT), Nasal Obstruction Symptoms Evaluation (NOSE), the Sino-Nasal Outcome Test-20 (SNOT-20), and visual analog scale (VAS) evaluation.

Results

The increase in the volume of the posterior wall of the sphenoid sinus after surgery was objectively smaller in the sphenoid mucosal repositioning group than in the control group (P = .046). However, this did not affect olfactory function (as revealed by the CCCRC test or the CCSIT) or subjective symptoms (as revealed by the NOSE, SNOT-20, and VAS scores) (all P > .05).

Conclusion

Surgical closure via sphenoid mucosal repositioning during EETSA reduces the volume of the posterior wall of the sphenoid sinus and facilitates re-operation. We suggest that sphenoid mucosal repositioning is appropriate during EETSA.

Level of Evidence

4 Laryngoscope, 2022

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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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Association Between Clinical Symptoms, Electrodiagnostic Findings, Clinical Outcome, and Prodromal Symptoms in Patients With Bell's Palsy

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Ann Otol Rhinol Laryngol. 2022 Feb 3:34894221075107. doi: 10.1177/00034894221075107. Online ahead of print.

ABSTRACT

OBJECTIVE: This study aimed to determine which prodromal symptoms frequently occur in patients with Bell's palsy and evaluate the association between these symptoms and clinical severity of paresis or the severity of facial nerve injury.

MATERIALS AND METHODS: The study included 86 patients with Bell's palsy between August 2018 and April 2020. Severity l evels of Bell's palsy and facial nerve damage were evaluated using the House-Brackmann (H-B) grading scale and electrodiagnostic study, respectively. Subsequently, a self-reported questionnaire on prodromal symptoms was administered. To assess the degree of recovery, the H-B grade was reported at 9 weeks and 6 months after the onset of paralysis.

RESULTS: The most common prodromal symptoms were postauricular pain, sensory decline in the tongue, headache on the affected side, myalgia, facial sensory decline on the affected side, taste impairment, and dry eye. Taste impairment was significantly correlated with severe facial paralysis reported at 9 weeks after onset (P < .05) and was not related to the severity of paresis assessed at initial examination or 6 months after onset or on electrodiagnostic findings.

CONCLUSIONS: The prodromal symptoms of Bell's palsy were not associated with the severity of facial nerve injury in an electrodiagnostic study. Taste impair ment was related to clinical severity of paralysis at subacute stage, 9 weeks after onset, but it was not associated with long-term prognosis.

PMID:35109694 | DOI:10.1177/00034894221075107

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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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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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Georg Thieme Verlag KG Rüdigerstraße 14, 70469 Stuttgart, Germany

Article in Thieme eJournals:
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