Τρίτη 15 Ιουνίου 2021

Neurofibromatosis 2: Primary Modality of Hearing Rehabilitation with Cochlear Implant

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Abstract

Auditory Brainstem Implants have been recommended as the gold standard in hearing rehabilitation of Neurofibromatosis Type 2 patients who lose hearing completely in both the ears and the cochlear nerves are not stimulable. Some patients have undergone cochlear implantation, in whom the cochlear nerve was spared during surgery or have undergone stereotactic radiotherapy preserving the function of the cochlear nerve. Here we report a case in whom we chose cochlear implantation prior to any definitive treatment for the tumour itself during the 'wait and watch' period. The reasons in favour of this approach have been discussed in this article. Post switch-on the implant is benefitting the patient satisfactorily and she is on regular follow up for monitoring the bilateral tumours.

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Management of Cervical Tracheo-Esophageal Fistula by Lateral Cervical Approach: Our Experience’

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Abstract

Acquired Tracheo-esophageal fistula (TEF) is a challenging and complicated condition. The laryngeal protection is lost in acquired TEF cases due to the established connection between the esophagus and the airways leading to aspiration, pneumonia, and acute respiratory distress syndrome. Malignancy contributes to about 80% of acquired TEF. Nonmalignant causes for TEF include prolonged ventilation, trauma (iatrogenic, penetrating, or blunt injury), foreign bodies, corrosive burns, and granulomatous infections. With the advancements in critical care, the incidence of TEF post-ventilation is on the rise in recent decades. We would like to share our experience managing ten cases of nonmalignant acquired cervical TEF by the lateral cervical approach at our institute. Apart from the isolated TEF cases, one patient with concomitant tracheal stenosis was repaired simultaneously with good postoperative results. TEF was identified in two cases following removal of T-tube an d solid stent respectively and was repaired successfully with lateral cervical approach with strap muscle flap interposition.

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Human Neutrophil Elastase Mediates MUC5AC Hypersecretion via the Tumour Necrosis Factor-α Converting Enzyme-Epidermal Growth Factor Receptor Signalling Pathway in vivo

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Objectives: The objective of this study is to examine the role of the tumour necrosis factor-α converting enzyme-epidermal growth factor receptor (TACE-EGFR) pathway in human neutrophil elastase (HNE)-induced MUC5AC mucin expression in mice. Method: Four groups of mice, treated with HNE alone (HNE group), HNE plus TACE inhibitor (HNE + TAPI-2 group), HNE plus EGFR inhibitor (HNE + AG1478 group), and untreated (control group), were used in the experiment. Histopath ological changes were monitored by haematoxylin-eosin (HE) and periodic acid-Schiff (PAS) staining. TACE, EGFR, and MUC5AC expression in the nasal mucosa were determined using immunohistochemistry. The expression of p-EGFR, EGFR, and TACE protein was analysed on Western blots, and MUC5AC protein levels were assessed via ELISA. TACE, EGFR, and MUC5AC expression in the nasal mucosa were determined using real-time quantitative PCR. Results: Compared to the control group, HE-stained tissues from the HNE group showed an irregular epithelium as well as goblet cell and submucosal glandular hyperplasia. In the nasal mucosa, strongly positive fuchsia granules were seen in PAS staining and significant increases in TACE, EGFR, MUC5AC mRNA, and protein expression were detected (p #x3c; 0.01). The HNE + TAPI-2 and HNE + AG1478 groups had significantly less goblet cell and submucosal gland hyperplasia as well as weaker PAS staining. Compared to mice treated with HNE alone, in HNE + TAPI-2-treated mice, the levels of TACE, EGFR, and MUC5AC mRNA and protein as well as p-EGFR protein were significantly reduced (p #x3c; 0.01). In HNE + AG1478-treated mice, EGFR and MUC5AC mRNA and protein levels and p-EGFR protein expression were reduced significantly (p #x3c; 0.01), but the difference in TACE mRNA and protein expression between the HNE + AG1478 and HNE groups was not significant (p #x3e; 0.05). Conclusion: Using a newly developed, stable experimental model of nasal hypersecretion in mice, we showed that TAPI-2 or AG1478 inhibited HNE-induced MUC5AC production. This suggests that MUC5AC mucin expression in vivo is mediated by a cascade involving the HNE-TACE-EGFR signalling pathway.
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Intracranial venous sinus stenting for the treatment of lateral sinus stenoses: An analysis of 200 patients

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Diagn Interv Imaging. 2021 Jun 11:S2211-5684(21)00138-8. doi: 10.1016/j.diii.2021.05.008. Online ahead of print.

ABSTRACT

PURPOSE: The purpose of this study was to analyze the long-term efficacy and safety of intracranial venous sinus stenting in a large cohort of patients with any type of presentation of primary lateral venous sinus stenosis (VSS).

MATERIALS AND METHODS: A retrospective cohort study was performed including patients treated by venous sinus stenting for symptomatic VSS from 2012 to 2019. Successful primary resolution of symptoms without adjunctive treatment or recurrence, and complications after stenting were analyzed at the last follow-up time point.

RESULTS: Two-hundred patients were included. There were 14 men and 186 women with a mean age of 39±14 (SD) years (age range: 13-75 years). Presenting symptoms included venous pulsatile tinnitus in 168 patients (168/200; 84%), idiopathic intracranial hypertension in 1 00 patients (100/200; 50%) and/or spontaneous cerebrospinal fluid leak in 35 patients (35/200; 17%). The overall rate of successful primary resolution of any typical presenting symptoms was 79% (95% CI: 73-85%). This rate ranged from 74% to 93% depending on the symptom with no significant difference between patients with and those without idiopathic intracranial hypertension (P=0.08). Recurrence rate was 10% (95% CI: 6-14%). No death or permanent morbidity were observed during a median follow-up of 2.2 years (Q1, Q3: 1.4, 3.3; range: 1-7.7 years).

CONCLUSION: Our study shows that venous sinus stenting has a low morbidity and high success rate at long-term follow-up for the treatment of idiopathic intracranial hypertension, venous pulsatile tinnitus or spontaneous cerebrospinal fluid leak associated with VSS. The excellent safety suggests considering this treatment as first-line treatment when medical management is ineffective or poorly tolerated.

PMID:34127434 | DOI:10.1016/j.diii.2021.05.008

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A Study of JCIH (Joint Commission on Infant Hearing) Risk Factors for Hearing Loss in Babies of NICU and Well Baby Nursery at a Tertiary Care Center

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Abstract

Babies in Neonatal Intensive Care Units (NICU) have an additional risk for hearing loss due to various risk factors like, prematurity, low birth weight, mechanical ventilation, hyperbillirubinemia, ototoxic drugs, low APGAR score etc. as compared to the babies from well baby nursery (WBN) who, poses risk factors mostly family history, syndromic deafness. So the present study was aimed know the risk factors responsible for hearing loss in NICU and WBN babies and to assess the incidence of deafness. A total of 800 babies from NICU (n = 402) and WBN (n = 398) underwent hearing screening from a tertiary care center. Hearing screening was done using two staged screening protocol as per JCIH guidelines with Distortion product Evoked Otoacoustic Emissions (DPOAE) and Automated Auditory Brainstem Responses (A-ABR). According to DPOAE test, 311 from NICU and 383 from WBN passed the test and during second screening, 80 out of 91 from NICU and 11 o ut of 13 from WBN passed the DPOAE test. Further BERA was done at the 3rd month of corrected age where 6 out of 11 showed positive responses from NICU and 3 babies from WBN had profound hearing loss. Data analysis revealed that family history of deafness, anemia and hypertension in ANC, TORCH in mother, low Apgar score and hyperbillirubinemia in newborns were a major risk factor for hearing impairment. We conclude that the diagnoses of auditory disorders at early stage due to various risk factors are important since appropriate therapeutic intervention and rehabilitation would help in better development of children.

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Neurosurgical anatomy of the floor of the third ventricle and related vascular structures

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Surg Radiol Anat. 2021 Jun 14. doi: 10.1007/s00276-021-02785-8. Online ahead of print.

ABSTRACT

PURPOSE: Anatomical knowledge of the floor of the third ventricle (FTV) is essential in avoiding surgical complications during endoscopic third ventriculostomy. The purpose of this study was to characterize the morphometry of FTV and related arteries, particularly the basilar artery (BA), as well as the factors that influence it.

METHODS: Twenty-six formalin-fixed adult brai ns and two hundred adult brain MRIs were studied focusing on FTV and related arteries. Dimensions of interest were measured using image analysis software. Morphometric data obtained were statistically analysed.

RESULTS: Distances between FTV, intermammillary sulcus (IMS), infundibulum, BA bifurcation, and posterior communicating arteries (PCoAs) were described on the cadavers and the MRIs. Distance between right and left PCoAs was greater at their anterior extremity (p < 0.001). Right PCoA was longer (p = 0.016). BA was lateralized in 58.4% of cases and its calibre was larger in males (p < 0.001). The distance from BA apex to FTV was inversely correlated with BA diameter (p < 0.001) and age (p = 0.004). Distance from IMS to infundibulum and the distance between both PCoAs were greater in MRI series when compared to cadaver series (p < 0.001).

CONCLUSIONS: A quantitative description of the morphometry of the region of the FTV and related vessels was obtained, helping neurosurgeons in planning their surgical approach. The distance from BA apex to FTV was shorter in individuals with larger BA calibre and in older subjects. MRI studies were qualitatively superior to cadaveric studies in evaluating the anatomy of this region.

PMID:34128100 | DOI:10.1007/s00276-021-02785-8

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Malignant struma ovarii with late recurrence harbouring high microsatellite instability

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BMJ Case Rep. 2021 Jun 14;14(6):e242081. doi: 10.1136/bcr-2021-242081.

ABSTRACT

Struma ovarii (SO) is a rare ovarian teratoma containing abundant mature thyroid tissue. Malignant transformation is even less common and distant metastasis is documented in about 5%-10%. The time from diagnosis of primary SO to metastatic disease varies. As malignant SO is rare, there are no uniform diagnostic criteria or treatment guidelines. Management is usually extrapolated from that of thyroid mal ignancy. We report a patient who relapsed 12 years from the initial diagnosis and metastasised to the lungs 5 years after the first recurrence. Our patient was treated with total thyroidectomy followed by radioactive iodine, and retreated on progression in the lungs. The tumour harboured high microsatellite instability and treatment with programmed cell death protein 1 inhibitor was initiated. This case shows the long latency of SO with the rare phenomenon of metastasis. It also highlights the importance of molecular testing for rare cancers such as this.

PMID:34127502 | DOI:10.1136/bcr-2021- 242081

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