Unilateral visual loss resulting from orbital encroachment of an ethmoidal juvenile trabecular ossifying fibroma

Ossifying fibromas are mainly found in the mandible and maxilla. Reports of them arising in the ethmoid sinuses and orbits are rare. We present a case of an otherwise healthy 20-year-old man with gradual onset of right visual disturbance signified by right relative afferent pupillary defect due to a large unilateral ossifying fibroma arising from the ethmoid sinus compressing the medial half of the right orbit. We emphasise the multidisciplinary combined endoscopic endonasal and external approach to ensure a successful debulking of the fibroma

Prevalence of Rare and Common Bleeding Disorders in Kurdistan Province of Iran

Background: Congenital bleeding disorders (CBD) are a group of coagulopathies with different clinical and laboratory features. The prevalence of these disorders in different parts of the world is variable. Iran as a country with a high rate of parental consanguinity has a high rate of CBDs. This study was to report the prevalence of these disorders in Kurdistan province, west of Iran.Methods and materials: This descriptive study was conducted on patients suspected of a congenital bleeding disorder referred to hemophilia center of this province for evaluation of underlying bleeding diathesis. Diagnosis and classification of disorders were made by routine and specific laboratory tests.Results: Out of 107 patients, 65.4% affected by common bleeding disorders (hemophilia A and B), 23.4% affected by rare bleeding disorders (RBDs) and 11.2% had inherited platelet disorders. Factor VII deficiency (64%) was the most common RBDs and 9 patients had von Willebrand disease. Out of three patients with inherited platelet disorders, two had Glanzmann thrombasthenia.Conclusion: CBD pattern though has similar patterns with total pattern of the country, some of the inherited platelet disorders are more common in Kurdish province. Determination of prevalence and distribution of these disorders can improve health system planning and resource allocation.Keywords: Congenital bleeding disorders, Rare bleeding disorders, Common bleeding disorders, inherited platelet disorder

Students’ Educational Problems, Presented in the University’s Educational Councils from 2002 to 2005, Shahrekord University of Medical Sciences

Background & Objective : An educational council, one of the most important parts of each university, plays the main role in solving students’ educational problems. This study was aimed to analyze the students’ educational problems in Shahrekord University of Medical Sciences, according to the councils’ minutes from 2002 to 2005. Methods : In this study, 333 cases of the students’ educational problems were extracted from the minutes of 66 educational councils’ meetings during a period of four years. They were analyzed according to the students’ study field, level, turn (morning or evening courses), sex, and the state of being native. Results : Among all, request for omitting a course, educational leave, and registering a course were the most frequent problems. Non native students’ educational problems were more than native ones (6.8% and 4.3% respectively) and males’ problems (9.8%) were more than females’ (3.8%) (p=0.0001). Educational problems among students of medicine (8.4%), radiology (7.6%), and field of fighting against diseases (5.9%) were more frequent than others. The difference between the frequency of problems in the students attending morning classes (5.3%) and those attending evening classes (5.2%) was not significant. Conclusion : This study revealed that the students’ educational problems were different in various study fields. There were significantly more problems in male and non native students (who are less supported by the families) compared to female and native ones. Family, financial and health problems, lack of enough knowledge among students and tutors about educational rules stood amongst the most important factors leading to the students’ educational problems. Keywords: Student’s educational problem, Educational council, University of Medical Sciences

Erdheim-Chester Disease:Two cases from an ophthalmic perspective

Purpose: We report two patients who presented initially to ophthalmology clinics with symptoms and signs of orbital inflammation that led to a diagnosis of Erdheim-Chester Disease (ECD). Observations: ECD is a rare form of non-Langerhans cell histiocytosis (LCH) which is characterised by multi-system organ involvement and poor prognosis with standard therapies. Both patients were positive for the BRAF V600E mutation on genetic testing and were treated with the BRAF inhibitors Vemurafenib and Dabrafenib respectively. These cases highlight the variable clinical presentation and course of ECD, the classical radiological and histopathological findings, and the high degree of clinical suspicion necessary to reach this diagnosis. Conclusions and importance: The combination of xanthelasma and bilateral, diffuse intraconal orbital masses must suggest to the clinician the possibility of ECD; and consideration to arrange further investigation with a full body CT or FDG PET/CT scan should be given, even in the absence of wider systemic symptoms or signs. With the advent of targeted therapies such as BRAF inhibitors, it is of even more importance that a diagnosis of ECD is established in a timely manner in order to give these patients the best chance of reduced morbidity and increased survival

Acquired external punctal stenosis: surgical management and long-term follow-up.

PURPOSE: To introduce and assess the results of a long-term follow-up of a one-snip punctoplasty with monocanalicular stent (Mini Monoka) for acquired external punctal stenosis (AEPS) with and without associated internal punctal and canalicular stenosis. DESIGN: Prospective non-comparative interventional case series. METHODS: Thirty-five eligible patients (53 eyes) with AEPS underwent a horizontal one-snip punctoplasty and Mini Monoka tube insertion by or under supervision of a consultant Oculoplastic surgeon from June 1999 to May 2002. Diagnostic probing and irrigation were performed before operation and after operation at the last follow-up. Patients with canalicular obstruction, nasolacrimal duct stenosis and obstruction, and those with less than 6 months' follow-up were excluded. The Chi-square (X(2)), Fisher's exact, Pearson correlation, and multiple logistic regression analysis tests, with 95% confidence interval when appropriate, were used for statistical analysis. RESULTS: The age range was 39 to 90 years (mean: 67.2, SD: 11.8, SE: 2). Twenty-seven patients (77.1%) were female. There was a normal canalicular system in 21 (39.6%), lower canalicular stenosis in 10 (18.8%), and internal punctal stenosis in 22 (41.5%) eyes. Postoperative follow-up was from 6 to 41 months (mean: 18.5, SD: 9.2, SE: 1.2). There was a 77.4% complete functional success, 7.5% partial functional success, and 96.2% anatomical success at the last follow-up. The success rate was not significantly different between the eyes with and without preoperative internal punctal and canalicular stenosis (p = 0.4). The lower success rate was significantly correlated with a final abnormal probing and irrigation (p < 0.01). CONCLUSION: The use of a monocanalicular Mini Monoka stent together with a one-snip punctoplasty is helpful to prevent the recurrence of punctal stenosis in the healing phase and addresses the associated internal punctal and canalicular stenosis

Lacrimal Sac Mucoepidermoid Carcinoma with Metastases to the Cavernous Sinus Following Dacryocystorhinostomy Treated with Stereotactic Radiotherapy

Purpose: We report a very good outcome in a 44-year-old woman in whom cancer was missed as the cause of nasolacrimal duct obstruction and dacryocystitis and which was deemed inoperable after spreading to the cavernous sinus. Case Report: The patient was referred to our unit 12 months following uneventful right dacryocystorhinostomy for nasolacrimal duct obstruction. This had been complicated by the formation of a significant canthal swelling 6 months later, which had been excised at that time. The symptom of nasolacrimal duct obstruction and scar recurrence prompted the referral to our unit. Examination and biopsy confirmed a malignancy. Despite extensive surgery, including concurrent radical neck dissection and parotidectomy, within 6 months, her mucoepidermoid carcinoma was found to have spread to the cavernous sinus, restricting blood flow from the carotid and causing an abducens nerve palsy. Though deemed inoperable at first, Gamma Knife stereotactic radiosurgery was sought as treatment for her disease, resulting in a good outcome 4 years after surgery. Conclusion: Experience from this case suggests the importance of considering malignancy as a cause in young patients when presenting with nasolacrimal duct obstruction. In such cases, and perhaps for all patients, biopsy specimens should be submitted as many tumours are found incidentally at the time of dacryocystorhinostomy. Whilst the external approach to dacryocystorhinostomy may identify abnormal anatomy intraoperatively, prompting biopsy, this is less likely with an endonasal approach where osteotomy precedes sac visualisation. The endonasal approach may therefore be less appropriate in such cases where malignancy is suspected as osteotomy may aid in the spread

The prevalence of lower eyelid fornix fat prolapse: A diagnostic measure of involutional entropion

Purpose: In our experience, lower lid involutional entropion is commonly associated with inferior fornix fat prolapse and non-eversion of the tarsal plate. We aimed to assess the prevalence of this sign as an early diagnostic indicator of lower lid involutional entropion in symptomatic and control groups. Methods: Prospective, comparative, observational case series. Fornix fat prolapse and failure of tarsal eversion in patients with involutional entropion were studied. Fornix fat prolapse was assessed by pulling the lower lid margin to the inferior orbital rim and comparing the meniscus of the protruding fat. The absence of tarsal eversion and lid laxity were also assessed. Patients were reassessed following correction using radiofrequency entropion surgery. Results: Sixty eyes of 50 Caucasian patients with involutional entropion underwent entropion correction. Fornix fat prolapse was found preoperatively in 83.3% (50 eyes), with complete reversal of fat prolapse in 76% (38 eyes) over a mean follow-up of 18.9 months, after successful surgery (P < 0.01). None of the 100 eyes (50 patients) in the control group showed fornix fat prolapse or absence of lower lid tarsal eversion (P < 0.01). Conclusion: There is high prevalence of fornix fat prolapse and failure of tarsal eversion (BB sign) in involutional entropion, with reversal after successful eyelid surgery. The absence of fornix fat prolapse in normal controls suggests that this is an important, underlying anatomical etiology, with diagnostic and prognostic value