1/10. Two cases of orbital infarction syndrome.Orbital infarction syndrome is defined as ischemia of all intraorbital and intraocular structures. It is a rare disease caused by rich anastomotic vascularization of the orbit. It can occur secondary to different conditions, such as, acute perfusion failure, systemic vasculitis, orbital cellulitis and vasculitis. It results in orbital and ocular pain, total ophthalmoplegia, anterior and posterior segment ischemia, and acute blindness. We report here upon two cases of orbital infarction with similar presentations but with different causes, namely, mucormycosis and as a postoperative complication of intracranial aneurysm, discuss the possible mechanisms of orbital infarction, and present a review of the literature on the topic. The prompt recognition of clinical pictures and rapid diagnosis is essential for the early treatment of orbital infarction, since its progression is very rapid and it can be even fatal.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/10. aspergillosis of the maxillary sinus as a complication of overfilling root canal material into the sinus: report of two cases.aspergillosis of the maxillary sinus is a relatively rare disease in nonimmunocompromised patients. In recent years a number of cases of aspergillosis of the maxillary sinus have been reported in association with overextension of root canals fillings with certain root canal cements. It has been suggested that zinc oxide-based root canal cements might promote the infection with the Aspergillus species. In particular aspergillus fumigatus has been found to be associated with the maxillary sinus infection. Radiographically the unique appearance of a dense opacity foreign body reaction in the maxillary sinus was considered a characteristic finding in maxillary sinus aspergillosis. Because this association of overfilling of root canal cements and aspergillosis of the maxillary sinus is not too well known we report two cases of young healthy female patients with the characteristic findings, both radiographically and clinically. In both patients the first maxillary molar was involved. patients were symptomless and the diagnosis was made accidently. However at surgical inspection both patients revealed aspergillomas, including the overextended root canal cement. The surgical procedure is described as are the microscopic findings in both cases showing the characteristic branching hyphae and conidophores typical of Aspergillus. Overextension into the maxillary sinus with root canal cements has to be avoided; material has to be removed from the sinus because otherwise aspergillosis infection may ensue.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/10. Conservative treatment of vertical diplopia in a patient with silent sinus syndrome.Silent sinus syndrome is a rare disease of the maxillary sinus characterized by bony absorption processes leading to progressive sinus wall thinning with consecutive enophthalmos and hypoglobus. It represents a benign cause of acquired enophthalmos and is often accompanied by painless vertical diplopia, the latter treated surgically in all cases published to date. We report a 56-year-old patient with silent sinus syndrome in whom vertical diplopia was treated with prisms showing that conservative treatment alone may, in mild cases, be an effective alternative to reconstructive surgery.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/10. Successful treatment of rhinocerebral mucormycosis with liposomal amphotericin b and surgery in two diabetic patients with renal dysfunction.The zygomycetes are a class of fungi that can cause a variety of infections in humans. Rhinocerebral mucormycosis is a rare disease and usually affects diabetic or immunosuppressed patients. The disease progresses rapidly and is usually fatal despite aggressive surgical and medical therapy. We report the management of two cases of rhino-sinusal and orbital mucormycosis in diabetic patients on treatment with corticosteroids, and mild renal impairment, successfully treated with a combination of aggressive surgical debridement and liposomal amphotericin b.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/10. Invasive maxillary aspergillosis after dental extraction. Case report and review of the literature.Paranasal sinus aspergillosis has usually been considered a rare disease, but it is seen more frequently in both immunocompromised and immunocompetent persons. Invasion may reach the sinuses via the nose or from the mouth after dental procedures. Even though the infection is usually limited to one or more sinuses, it may in certain cases extend to vascular or intracranial structures with a fatal outcome. In these cases, aggressive treatment is justified. We report a case of maxillary sinus aspergillosis that developed after dental extraction. attention should be given to this possibility even after an apparently uncomplicated dental extraction.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
6/10. Fibrous dysplasia of the sphenoid sinus and skull base presents in an adult with localized temporal headache.Fibrous dysplasia (FD) of the sphenoidal sinus is a rare disease, especially during adulthood. We report a case of FD of the right sphenoidal sinus in an adult male patient who presented with nonspecific symptoms limited to headache localized to the right temporal area and to the inferior orbital rim of both sides. magnetic resonance imaging revealed a dense mass that occupied the entire right sphenoidal sinus and skull base with typical ground-glass opacification and bony sclerosis of the whole sphenoidal wall. The diagnosis of FD was confirmed on pathological examination of a biopsy taken through sphenotomy. The patient underwent a subcranial craniotomy for tumor resection. After more than 4 years of follow-up, the patient was disease-free. On the basis of these clinical features, it is important to consider sphenoidal FD in both young and adult patients complaining of an unexplained headache, because it may present unusually with headache localized to the temporal region or the inferior orbital rim.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
7/10. Management of spontaneous enophthalmos due to silent sinus syndrome: a case report.Silent sinus syndrome (SSS) is a rare disease exhibiting unilateral enophthalmos and hypoglobus. A 26-year-old white female presented with right side enophthalmos and hypoglobus. There was no history of previous trauma or maxillary sinus diseases. A CT scan showed an opacified right maxillary antrum with decreased volume and downward bowing of the right orbital floor. From clinical and radiological findings the diagnosis SSS was made. Biopsies were collected from the maxillary sinus for the exclusion of malignancy. Two months later orbital floor reconstruction was carried out. Before antrostomy of the affected maxillary sinus, a relative enophthalmos of 4mm was determined. Five days after antrostomy the value reduced to 2.3mm. During the following 2 months the enophthalmos remained constant. At the end of the operation for orbital floor reconstruction it was 0.1mm. Five days after surgery the relative enophthalmos increased to 0.8mm. The value remained constant during the following 3 months. Initial antrostomy of the affected maxillary sinus may lead to a relevant, spontaneous reduction of enophthalmos. After a minimum period of 2 months a re-evaluation should be made, if a reconstruction of the orbital floor is still necessary for the correction of the globe position.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
8/10. Primary localized amyloidosis of the nose and paranasal sinuses. A case report with immunohistochemical observations and a review of the literature.Primary localized amyloidosis of the nose and nasopharynx is a rare disease. We present a case and review seven additional cases from the English literature. The ages of the patients ranged from 8 to 86 years; there was no sex predominance. Symptoms were nasal obstruction, epistaxis, and impaired hearing. physical examination revealed a nasal mass or glue ears. The lesions were composed of amyloid and chronic inflammatory cells, mainly plasma cells. Ours is the first case of nasal amyloidosis in which the type of amyloid was determined immunohistochemically to be amyloid light chain (AL) lambda. The main treatment was surgical. Recurrences developed. Determination of the biochemical nature of this amyloid clarified its pathogenesis and may influence treatment. amyloidosis should be considered in the differential diagnosis of nasal obstruction, epistaxis, and glue ears, even in the pediatric age group.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
9/10. Tumefactive fibroinflammatory lesion of the head and neck: a management strategy.Tumefactive fibroinflammatory lesions are rare, benign, fibrosclerosing masses in the head and neck that appear clinically as malignancies. Twenty-one cases have been described since first reported in 1975. While all authors agree on the lesion's histopathologic characteristics, multiple variations in appearance, treatment, and results have been reported. We review a case and compile all previously reported cases. Common sites of involvement were sinonasal tract (41%), neck (37%), and parotid (23%). Symptoms frequently reported were pain (50%) and hard mass (55%). Five patients (23%) eventually developed extra-head and neck fibrosclerosing lesions. Treatments have included surgery, radiation, and corticosteroids, occasionally in combination. Results have varied depending on size and location of lesion. Based on this review, a discussion of this rare disease and recommendations regarding diagnosis and treatment are given.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
10/10. Parasellar Aspergillus granuloma extending from the sphenoid sinus: report of two cases.BACKGROUND: sphenoid sinus aspergillosis is a rare disease known to show an aggressive course with high mortality. early diagnosis, though difficult, is required to prevent lethal fungal meningoencephalitis. CASE REPORT: We describe two cases of parasellar Aspergillus granuloma extending from the sphenoid sinus clinically indistinguishable from intracranial neoplasms. In the first patient, the fungus colony was visualized by computed tomography (CT) and magnetic resonance imaging (MRI) as a calcified concretion and total removal was curative. In the second patient, partial removal and subsequent antifungal therapy had minimal effect. CONCLUSIONS: The prognosis of the patients with this disease depends on prompt surgical treatment before intradural invasion occurs, and CT and MRI are useful diagnostic maneuvers for detecting calcified Aspergillus colonies.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
| | Next -> |