Early recurrence of macular schisis in X-linked retinoschisis treated with vitrectomy for rhegmatogenous retinal detachment under silicone oil: case report and brief literature review.

X-linked retinoschisis (XLRS) is an inherited retinal degeneration affecting males, characterized by splitting of the retinal layers. We herein present the outcomes of surgical treatment in a case of XLRS complicated by rhegmatogenous retinal detachment (RRD). A 22-year-old male presented to the emergency department due to decreased visual acuity and visual field defect in his left eye Oculus Sinister (OS) of 1 week duration. The patient reported an early onset retinal degeneration and decreased visual acuity in both eyes since childhood in his past ocular history. Upon presentation, best corrected visual acuity (BCVA) was 6/30 on the right eye Oculus Dexter (OD) and 6/120 OS. Fundus examination revealed areas of peripheral retinal schisis, and the characteristic spoke wheel pattern on the macula of both eyes. In OS, a temporal RRD involving the macula was identified. The patient underwent surgical treatment with pars plana vitrectomy with internal limiting membrane (ILM) peeling, endolaser, and silicone oil (SO) tamponade. BCVA in OS improved to 6/60 and schistic cavities resolution was observed in the immediate postoperative period. The patient's BCVA further improved to 6/19 at 1 month, as foveal anatomy showed relative improvement. However, there was a rapid reappearance of schisis spaces in the macular area at this point, which was also followed by progressive deterioration of foveal schisis by 3 months post-operatively. The resorption and recurrence of lamellar macular schisis changes after ILM peel and presence of SO, highlights that although XLRS findings can temporarily improve upon surgical intervention, the pathogenetic mechanisms contributing to disease phenotype remain to be elucidated.

Impact of Atmospheric Conditions on Epistaxis Incidence.

PURPOSE: A possible correlation between epistaxis occurrence and atmospheric parameters' variation has long been hypothesized. This study aims to determine whether cumulative epistaxis incidence is related to seasonal variation and its relationship to monthly, weekly, and daily recordings of atmospheric measurements in the city of Patras, Greece. METHODS: In this retrospective study, data concerning the patients who presented with active epistaxis at the ED of a tertiary university hospital in Western Greece between January 2020 and December 2021 were collected. Only cases of spontaneous epistaxis were included in the study; patients bleeding secondarily due to a known mechanical cause, i.e., a tumor, trauma, or surgery, were excluded. The measurements of atmospheric parameters were supplied by the Department of Physics, University of Patras. RESULTS: In total, 230 cases of spontaneous, active epistaxis were evaluated in the ED over the course of the study. The median frequency of epistaxis presentations was two cases per week. Most of the patients were male, comprising 62.6% of the cohort, and the median age stood at 70 years, with an interquartile range (IQR) of 54 to 81 years. A minor yet statistically significant negative correlation between the incidence of epistaxis and mean relative humidity was observed both on a daily and weekly basis. Mean relative humidity emerged as a significant predictor for the incidence of epistaxis, both daily and weekly. Significantly lower mean relative humidity values were recorded during weeks with a high incidence of epistaxis cases (57.72% vs. 63.39%, p = 0.02). No discernible seasonality was observed in the frequency of epistaxis presentations to the ED. CONCLUSION: A modest yet statistically significant trend toward fewer epistaxis cases was observed in conditions of higher ambient humidity during the study period in the region of Western Greece.

Expression profiles of MMP-9 and EMMPRIN in chronic rhinosinusitis with nasal polyps.

Objective: Metalloproteinases (MMPs) are implicated in tissue remodeling in chronic rhinosinusitis with nasal polyps (CRSwNP). This study aimed to evaluate the expression profiles of MMP-9 and the extracellular matrix metalloproteinase inducer (EMMPRIN) in nasal polyps compared to healthy mucosa. Methods: Tissue samples from 37 CRSwNP patients undergoing functional endoscopic sinus surgery and mucosal specimens from 12 healthy controls were obtained intra-operatively. MMP-9 and EMMPRIN mRNA levels were assessed by reverse transcription-polymerase chain reaction and their protein expression by Western blot analysis. Results: MMP-9 mRNA expression levels were significantly elevated in CRSwNP compared to controls (p < 0.05). MMP-9 protein levels presented an increasing trend but with no statistical significance (p > 0.05). No statistically significant difference in EMMPRIN mRNA and protein levels was identified. Conclusions: Upregulation of MMP-9 in nasal polyps is evident and highlights its role in the pathogenesis of CRSwNP. The lack of concordance between MMP-9 mRNA and protein levels may be attributed to post-translational gene expression regulation. Although EMMPRIN expression was not significantly different between the two groups, its role remains to be elucidated. MMP-9 may be a valuable biomarker and treatment target in CRSwNP.

Periostin as a biomarker in chronic rhinosinusitis: A contemporary systematic review.

BACKGROUND: The role of periostin, a matricellular protein encoded by the POSTN gene, in chronic rhinosinusitis with nasal polyposis (CRSwNP) is reviewed. Periostin is considered a potential biomarker of endotype and may be useful for evaluating response to treatment. METHODS: Search terms in PubMed and Web of Science (1990-March 2022) included: ((periostin) OR (POSTN)) AND ((sinusitis) OR (nasal polyp) OR (CRSwNP) OR (CRS). The primary outcomes were differences in tissue, serum, and nasal lavage between CRSwNP and CRS without NP (CRSsNP) or controls. Associated factors reported to affect periostin expression, data regarding participants' clinical characteristics, disease endotypes, laboratory methods, and samples' origin were also pooled. Studies on <10 patients were excluded. RESULTS: Out of 101 records harvested through database searching, 29 prospective cross-sectional or case-control studies were eligible for review and qualitative analysis. Tissue sample origin, concurrent infection, current and past medication, primary or recurrent disease, allergic rhinitis, and smoking status should be considered as confounding factors for periostin levels. Periostin and POSTN messenger RNA (mRNA) levels were consistently and significantly higher in CRSwNP than CRSsNP and controls. Despite the distinctly different inflammation patterns among CRSwNP endotypes, periostin-related remodeling patterns seemed to be similar. CONCLUSION: Tissue and serum periostin levels, and POSTN expression appear elevated in CRSwNP, especially in eosinophilic inflammation, compared to CRSsNP and controls. Disease severity and comorbidities are also reflected in periostin and POSTN values. Carefully designed prospective studies may establish the role of periostin as a biomarker in CRSwNP and allow its incorporation in clinical practice.

Tongue graft-versus-host disease: remission with ruxolitinib.

Graft-versus-host disease (GvHD) is a potentially life-threatening and commonly encountered event of allogeneic haematopoietic stem cell transplantation. Here, we present a young adult male with primary refractory Hodgkin's lymphoma who received a transplant and developed cutaneous GvHD after donor lymphocyte infusion, which was managed with cyclosporine and steroids. However, while the patient was under immunosuppressive treatment, diffuse confluent whitish patches on the patient's tongue were observed. A biopsy of the tongue lesions revealed lichenoid, hyperkeratotic tissue changes and intraepithelial T-cell infiltration consistent with chronic GvHD. He was treated with mycophenolate mofetil for 6 months with minimal improvement. Janus-associated kinase inhibitor (ruxolitinib) treatment was commenced, with complete resolution of the tongue lesions and treatment discontinuation 5 months later. Currently, 5 years after allogeneic transplantation, he is in remission and does not need immunosuppressive therapy.

Mixed hemangioma of the external auditory canal and the tympanic membrane in a young woman: A case report.

Hemangiomas are benign vascular tumors frequently located in the head and neck area, but rarely encountered in the ear, often originally misdiagnosed due to their rarity and absence of specific clinical manifestations. We report the second case of a mixed hemangioma involving the external auditory canal in literature to date.

Pleomorphic adenoma of the maxillary sinus with orbital extension presenting with exophthalmos: a case report.

We report a case of pleomorphic adenoma (PA) of the maxillary sinus, an unusual and rare location of this frequent benign neoplasm originating from salivary glands and accounting for 70% of all salivary gland tumors, presenting with compressive phenomena from the orbit and the challenges in its differential diagnosis and management. A 66-year-old male presented with progressively worsening exophthalmos over the past 4 months and symptoms of nasal obstruction on the left side for the past year. Clinical evaluation and imaging studies revealed complete occupation of the left maxillary sinus by a mass with soft tissue characteristics, extending to the homonymous nasal cavity and orbit. Functional endoscopic sinus surgery (FESS) was performed. The surgical plan included septoplasty and medial maxillectomy and complete resection of the tumor with clear margins. The histopathology report was consistent with a PA of the maxillary sinus. PA of the maxillary sinus is an extremely rare disease entity. Orbital involvement due to extension of an undiagnosed PA as described in this case is very uncommon. Thorough investigation is required in cases of unilateral nasal obstruction. Complete surgical excision and close long-term follow-up are required due to the probability of tumor recurrence and potential malignant transformation.

Multiple symmetric lipomatosis (Madelung's disease) with symptoms and signs of hypertension, lipodystrophy, and dyspnea: A case report and literature review.

Madelung's disease generally refers to a benign symmetrical lipomatosis of the neck, but its presentation can vary. It is treated surgically and different approaches can be implemented. In cases of a threatened airway, a tracheostomy can be performed.

An Unusual Polypoid Septal Mucosal Melanoma: Overview and Diagnostic Pitfalls.

Sinonasal mucosa is an area of high melanocyte density compared to other mucosa-lined sites. Sinonasal mucosal melanomas (SNMM) most commonly arise from the nasal cavity and the paranasal sinuses. Due to their obscure anatomic location and lack of early symptomatology, SNMM are often diagnosed in an advanced stage. The majority of patients who present with symptoms complain of unilateral nasal dysfunction, such as obstruction and epistaxis. We hereby report a case of an 86-year-old female, who presented with a three-year history of progressive right-sided nasal obstruction and recurrent epistaxis. Posterior rhinoscopy and endoscopy revealed a polypoid, fleshy lesion whose coloration varied from mildly pigmented to amelanotic. Inverted sinonasal papilloma was included in the differential diagnosis due to MRI findings. Post-resection histopathology indicated a mucosal melanoma. Typically, amelanotic lesions are rare, more difficult to diagnose and associated with worse prognosis due to both their aggressiveness and delayed diagnosis.

Choroidal Thickness Evaluation in a Transfusion-Dependent Beta-Thalassemia Greek Population.

PURPOSE: To evaluate choroidal thickness in a group of beta-thalassemia patients as assessed by enhanced depth imaging optical coherence tomography. PATIENTS AND METHODS: This single-center, observational study involved transfusion-dependent beta-thalassemia (TD-ß-thal) patients and healthy controls. One eye of each participant was included in the study. Submacular and peripapillary choroidal thickness, as well as central macular thickness and retinal nerve fiber layer thickness, were evaluated. RESULTS: Thirty-eight TD-ß-thal patients (mean age 42 ± 10.7 years) and 22 healthy controls (mean age 40.3 ± 10.2 years) were included in the study. Subfoveal choroidal thickness was 297.4 ± 74.5 µm in the patient group and 358.4 ± 71.4 µm in the control group (p=0.003). Overall, in the submacular area, the choroid was found to be significantly thinner in the beta-thalassemia population compared to controls in all evaluated points, except for the spot located 1500 µm nasally to the fovea (p=0.093). In the peripapillary area, choroidal thickness was also significantly lower in the thalassemic population compared to the controls (nasal p=0.033, temporal p=0.01, superior p=0.01), except for the inferior quadrant (p= 0.191). We did not observe statistically significant differences in the retinal nerve fiber layer thickness and the central macular thickness between the two groups (p=0.658 and p=0.276, respectively). No correlations with hemoglobin, serum ferritin or iron levels emerged. Patients with the intermediate subtype appeared to have significantly thinner choroids than the ones with thalassemia major. CONCLUSION: Our findings suggest that choroidal thickness in the submacular and peripapillary area is significantly reduced in thalassemic patients, compared to healthy individuals. Choroidal thinning in beta-thalassemia possibly reflects the effect of chronic anemia and underlying hemodynamic changes on choroidal tissue.

Neck angioedema after anterior cervical discectomy and fusion with coexistent epiglottic cyst.

BACKGROUND: We present a case and reviewed the literature regarding airway obstruction and angioedema after an anterior cervical discectomy and fusion (ACDF). CASE DESCRIPTION: A 60-year-old female with degenerative cervical myelopathy and a previously undiagnosed epiglottic cyst underwent a C5-C6 ACDF; notably, the anesthesiologist found an epiglottic cyst when the patient was first intubated. Two hours postoperatively, the patient acutely developed severe neck swelling with airway obstruction due to angioedema. She was immediately treated with hydrocortisone and required a tracheostomy. The edema decreased markedly in the next 12 h and by the 3rd postoperative day it resolved. Three months later, she had no residual medical sequelae. CONCLUSION: Patients with epiglottic cysts who need cervical spine surgery should either first have the cyst treated or should be closely monitored postoperatively.

Impact of different therapeutic regimens on survival of patients with nasopharyngeal carcinoma.

PURPOSE: Nasopharyngeal carcinoma (NPC) demonstrates specific histo-genetic features that affect its biological behaviour. Our aim was to investigate the correlation between different therapeutic approaches and survival of patients with NPC in southwestern Greece based on specific clinicopathological features. METHODS: Seventy-two NPC patients (n=72) were treated between 1990 and 2014 at the University Hospital of Patras. Patient demographics, tumor histology, use of tobacco and alcohol, exposure to mutagenic agents, chosen treatment and survival were recorded. All patients were treated with radiotherapy (RT), chemotherapy, surgery or their combinations. RESULTS: In the patients who used immobilization mask during RT, the 5-year survival rate and overall survival was higher than the rest of patients (57% and 6 years vs. 13.6% and 3.36 years, p=0.0001, respectively)*. RT with mask combined with chemotherapy increased survival rates compared to RT without chemotherapy conventional regimen (p=0.0001). Additionally, patients who received chemotherapy demonstrated a 5-year survival of 51.6% compared to those without chemotherapy (11% p=0.0014). (*The 5-year survival rate group of patients refers to the percentage of people who will be alive 5 years after diagnosis. It does not include those who die from other diseases. Sometimes, this includes all people with a specific cancer type. Researchers call this an overall rate. In contrast, overall survival provides information for the length of time from either the date of diagnosis or the start of treatment for a disease, such as cancer, that patients diagnosed with the disease are still alive. In a clinical trial, measuring the overall survival is one way to see how well a new treatment works.) Conclusions: In the majority of examined NPC cases treated with the use of immobilization RT mask along with chemotherapy, a significantly better prognosis compared to conventional RT-chemotherapy treatment was observed. Thus, chemotherapy offers an advantage to patient survival as an adjuvant treatment regimen in conjunction with RT.