An Overview of Rare and Unusual Clinical Features of Bietti’s Crystalline Dystrophy

Bietti’s crystalline dystrophy (BCD) is a rare disease presenting with the appearance of intraretinal crystalline deposits and varying degrees of chorioretinal atrophy commencing at the posterior pole. Within time, intraretinal crystals gradually disappear and chorioretinal atrophy extends beyond the macula even resulting in complete chorioretinal atrophy. Concomitant corneal crystals can be noted in 1/2 - 1/3 of the patients, and the presence of corneal crystals is not a must for establishing the diagnosis. For the past decade, genetic evaluations and newer imaging modalities expand our knowledge about the disease. CYP4V2 gene is found to be the gene responsible for the disease process and new mutations are still being described. Modern imaging modalities, such as a spectral domain optical coherence tomography (SD-OCT) shed light on the anatomic features of the disease. By this, we reiterate the rare and unusual clinical features of BCD

An Overview of Rare and Unusual Clinical Features of Bietti’s Crystalline Dystrophy

Bietti’s crystalline dystrophy (BCD) is a rare disease presenting with the appearance of intraretinal crystalline deposits and varying degrees of chorioretinal atrophy commencing at the posterior pole. Within time, intraretinal crystals gradually disappear and chorioretinal atrophy extends beyond the macula even resulting in complete chorioretinal atrophy. Concomitant corneal crystals can be noted in 1/2 - 1/3 of the patients, and the presence of corneal crystals is not a must for establishing the diagnosis. For the past decade, genetic evaluations and newer imaging modalities expand our knowledge about the disease. CYP4V2 gene is found to be the gene responsible for the disease process and new mutations are still being described. Modern imaging modalities, such as a spectral domain optical coherence tomography (SD-OCT) shed light on the anatomic features of the disease. By this, we reiterate the rare and unusual clinical features of BCD

Lectrocardiographic Precordial St Segment Depression and Itslocalization in Inferior Myocardial Infarction: Correlation Withleft Anterior Descending Artery Occlusion and Multivessel Disease

DergiPark: 379041tmsjAims: The etiology and pathophysiology of precordial ST segment depression seen in inferior myocardial infarction have been investigated in numerous studies. Still there is much debate going on about its clinical implications. This study will explore the correlation between precordial ST segment depression in acute inferior myocardial infarction and concomitant coronary artery pathology. We focus on this subject for the reason that it may help clinicians in treatment planning and stratification of patients into relative low risk and high risk groups.Methods: Medical records of 77 patients who have been diagnosed with acute inferior myocardial infarction were retrospectively examined. Coronary angiography had been performed on all of them for the indication of inferior myocardial infarction. Patients’ first 24 hour ECGs were scanned and classified into 3 groups. Group 1 consists of patients without any precordial ST segment depression? Group 2 consists of patients with maximum precordial ST segment depression in leads V1 to V3? Group 3 consists of patients with maximum precordial ST segment depression in leads V4 to V6. The presence of precordial ST segment depression and its correlation with the anatomy of leftanterior descending artery and multi vessel disease were examined to determine a statistical significance.Results: When the data for 3 groups was examined in regards to occlusion of left anterior descending artery ( gt;50%), no statistical significance was found (p=0.439). Likewise, the presence of precordial ST segment depression was not significant for multi vessel disease in any of the groups (p=0.849).Conclusion: Taking the amount of inconsistent results that are being gathered on this subject into account, our interpretation in respect of these results is that it would not be appropriate to make an inference about coronary occlusion based on the presence and distribution of precordial ST segment depression before coronary angiographic procedures. Yet, in the light of previous researches and the interpretation that precordial ST segment depression is correlated to cardiac reserve and mortality, the subject of precordial ST segment depression in inferior myocardial infarction still carries great importanc

Intraosseous Mucoepidermoid Carcinoma of the Jaw: Report of Three Cases

Mucoepidermoid carcinoma (MEC) is the most common malignant tumor of the salivary glands and is seen most commonly in the parotid gland. Intraosseous MECs can be rarely seen and is mostly detected in the posterior part of the mandible. This condition can be acknowledged as an entity different from salivary gland MECs. In this case series, we delineated three patients of intraosseous MEC, two mandible tumors and one maxillary tumor and discussed the diagnostic characteristics and treatment methods of this rare entity

Bilateral Intravitreal Ranibizumab Injection and Panretinal Photocoagulation in a 16-Year-Old Girl with Severe Vaso-Occlusive Lupus Retinopathy

A 16-year-old girl with fever of unknown origin and bilateral vaso-occlusive retinopathy with retinal neovascularization, preretinal hemorrhage, and serous macular detachment was treated with single bilateral 0.5 mg intravitreal ranibizumab injection prior to aggressive PRP with success. No systemic steroids or immunosuppressive therapy was employed at that time. She received the diagnosis of “systemic lupus erythematosus” five years after this episode with further systemic symptoms. In certain cases with vaso-occlusive type of lupus retinopathy, anti-VEGF agents may be administered in addition to panretinal photocoagulation to achieve better visual and anatomic outcome

Translation and Validation of Chronic Otitis Media Benefit Inventory (COMBI) in Turkish Language

Objective: The aim of this study was to translate the Chronic Otitis Media Benefit Inventory (COMBI) into Turkish and evaluate the questionnaire’s reliability in assessing benefit outcomes in patients after ear surgery.Methods: A total of 30 patients with chronic otitis media who underwent ear surgery were asked to fill out the translated Turkish version of the COMBI questionnaire six months after their surgery twice, 10 days apart. The patients were divided into two groups in two different ways based on surgical and audiological outcomes. These were surgical success (SS), surgical failure (SF), audiological success (AS), audiological failure (AF) groups. Student's -t test was performed to show the differences in the COMBI scores of the groups. Cronbach’s alpha value was calculated to test internal consistency, Pearson’s correlation coefficient was measured to demonstrate test-retest reliability and factor analysis was run.Results: The average score of the SS group was 51.08 and significantly higher than the score of the SF group (42.40) (p=0.012). There was, however, no statistically significant difference between the average scores of the AS and the AF groups (p=0.330). A Cronbach’s alpha value of 0.862 was found. Pearson’s r value was calculated 0.810. Factor analysis showed that a three-factor solution explained the 70.2% of the variance.Conclusion: The Turkish version of the COMBI is a valid test with high test-retest reliability. It should be used with objective assessment tools for better understanding how patients benefit from the surgery

Atypical Metastasis to the Head and Neck Region: An Analysis of 11 Patients

Objective:We present 11 patients with distant metastases to the head and neck from an infraclavicularly located primary tumor and discuss the management strategies including the clinical presentation, treatment modalities, and prognosis.Methods:The retrospective data of the pathology reports and operation notes of 1239 patients who had undergone any kind of oncological surgical intervention between 2005 and 2017 were analyzed. All of the 11 patients included in the study were evaluated in our department’s tumor board, and all patients with an operable lesion had undergone surgery. Inoperable patients were treated with chemotherapy and/or radiotherapy.Results:The average age of the patients was 64.3 (48– 88) years. Primary tumors were located in the lung (2), breast (2), ovary (2), prostate (2), kidney (1), and colon (1) and the primary lesion could not be determined in one patient. The most common symptom was newly occurred painless swelling (9/11, 81.8%) at the metastatic site. Four patients without any other distant metastases were operated. Of these four patients, two died during follow-up due to systemic disease, and the other two are alive and disease-free. Three of the seven inoperable patients were treated with chemotherapy and the other four with radiotherapy. The prognosis of this group was worse.Conclusion:Although metastasis to the head and neck is not common, it is vital to keep in mind while approaching a patient with a lesion at the head and neck region especially if there is a history of lung, breast, and genitourinary cancers. Despite the poor prognosis, diminishing the tumor burden would increase the treatment success

Remodeling in Patients with in Situ Fixation for a Slipped Capital Femoral Epiphysis

Objective: This study has investigated the amount of bone remodeling in patients with a slipped capital femoral epiphysis (SCFE) treated with in situ fixation until closure of the epiphysis and the factors affecting remodeling. Method: Patients who underwent surgery for SCFE between January 2010 and January 2015 were retrospectively screened: Twenty-four male and 7 female patients (mean age 12.6 +- 1.9 years) were included in the study. Gender, age, history, and laterality of trauma, duration of hip pain (acute, chronic, acute on chronic background), and hip radiographs were evaluated. The Southwick and alpha angles were measured, and the factors affecting remodeling were assessed. The statistical analyses were conducted using SPSS 25.0 (IBM Corp., Armonk, NY); 95% confidence levels were calculated and p < 0.05 was considered to indicate statistical significance. Results: The preoperative displacement angles measured on the anteroposterior and lateral radiographs were 15.03° +- 9.1° and 25.93° +- 14.1° and at the last follow-up they were 11.63° +- 8.7° and 21.6° +- 12.1°, respectively. The alpha angles measured on the lateral radiographs preoperatively and at the end of follow-up were 52.33° +- 11.6° and 47.87° +- 11.8°, respectively. Significant remodeling was reflected in the angles measured on the anteroposterior and lateral X-ray images. Greater preoperative displacement angle was associated with less remodeling. Conclusion: Preoperative displacement affects the degree of postoperative remodeling. In patients with severe epiphyseal displacement, open reduction is an option but in situ pinning should be considered in that it is less invasive and more physiological