[Orbital emphysema with exophthalmos following transconjunctival pars plana vitrectomy]. / Orbitales Emphysem mit Exophthalmus nach transkonjunktivaler Pars-plana-Vitrektomie.

The case example presented shows that an orbital emphysema with exophthalmos can occur as a rare complication of a transconjunctival pars plana vitrectomy. Close monitoring of the patient symptoms, ocular motility, intraocular pressure and the fundus showed no evidence of compressive optic neuropathy or perfusion abnormalities through orbital vessels. The exophthalmos resolved spontaneously within a few days without any consequences.

[Good outcome in Acanthamoeba keratitis]. / Akanthamöbenkeratitis mit gutem Outcome.

A 31-year-old woman presented with mild keratitis of her left eye. The use of daily disposable contact lenses, regularly rinsed with tap water, and the development of perineural corneal infiltrates resulted in the clinical suspicion of infection with Acanthamoeba. Corneal epithelial scraping probe for polymerase chain reaction on Acanthamoeba confirmed the clinical diagnosis. Although the treatment was limited to low-dose propamidine monotherapy because of reduced tolerability, the keratitis healed with almost no scarring after 13 months of consequent medication.

[Memantine for optic nerve atrophy in Friedreich's Ataxia]. / Memantin bei Optikusatrophie in Friedreich-Ataxie.

A 24-year-old patient with Friedreich's ataxia presented with advanced visual loss due to optic nerve atrophy. After interdisciplinary consultation and after obtaining informed consent, an off-label therapy with the N-methyl-D-aspartate (NMDA) antagonist memantine was initiated. In a 1-year follow-up no further loss of the nerve fiber layer could be detected by optical coherence tomography (OCT) and visual acuity remained stable. Despite the limitations of this single and time limited case observational study, memantine should be discussed as an option for treatment of acute optic nerve atrophy in Friedreich's ataxia.

Clinical presentation, treatment, and outcomes in presumed intraocular tuberculosis: experience from Newcastle upon Tyne, UK.

PURPOSE: To report the clinical manifestations and treatment outcomes of patients with presumed intraocular tuberculosis (TB) seen at the Newcastle Uveitis Service, Royal Victoria Infirmary, Newcastle upon Tyne, UK over a 10-year period. METHODS: Retrospective review of case notes. RESULTS: A total of 21 patients were identified. Occlusive retinal vasculitis was the commonest ophthalmological presentation (12 patients). Eight patients (38%) were found to have underlying active systemic TB (four with mediastinal lymphadenopathy, three with pulmonary TB, one with cutaneous TB). Constitutional or respiratory symptoms, elevated inflammatory markers, and an abnormal chest radiograph were poor indicators of active TB. Two patients had inactive intrathoracic TB. Eleven patients had latent TB. Eighteen patients received anti-tuberculous treatment. Final visual acuity was better than or equal to initial visual acuity in 14 out of 16 patients who completed at least 6 months of standard anti-tuberculous treatment. CONCLUSIONS: Most patients with presumed intraocular TB have latent TB, but a significant minority has hitherto undetected active TB. Our series suggests that either proven or presumed intraocular TB occurs frequently in the absence of constitutional or respiratory symptoms, elevated inflammatory markers, or an abnormal chest radiograph. A minimum of 6 months standard anti-tuberculous treatment provides good visual outcomes in the majority of patients.

[Acute macular neuroretinopathy]. / Akute makuläre Neuroretinopathie.

A 24-year-old female patient presented with sudden onset of paracentral visual field defects of the right eye, which were noticed 2 months before the clinical examination. The slit lamp examination of the anterior and posterior segments showed unremarkable findings but three oval, well demarcated, hyporeflective areas were noticed in the right posterior pole in infrared fundus photographs. A similar small area was found in the left posterior pole. Optical coherence tomography (Cirrus OCT) showed a structural defect of the hyperreflective bands of the outer retina. The diagnosis of acute macular neuroretinopathy was made.

[Recurrent malignant solitary fibrous tumor of the orbit]. / Rezidivierender maligner solitärer fibröser Tumor der Orbita.

A 53-year-old female patient presented with a palpable mass in the right medial orbit which had been present for 18 months. The left eye had been enucleated in childhood due to a perforating injury. An orbital biopsy performed ex domo showed unspecific inflammatory tissue but no definite diagnosis was made and systemic therapy with corticosteroids was unsuccessful. A second orbital biopsy was performed in our clinic and revealed a solitary fibrous tumor (SFT) of the orbit. Medial orbitotomy with tumor excision followed and the histological examination of the excised tumor established the diagnosis of SFT of the orbit with focal sarcomatous transformation. The patient presented 3 years later with a new orbital mass in a control MRI examination. Relapse was suspected and orbitotomy with excision of the mass was performed. The histological examination revealed a relapse of the SFT with sarcomatous transformation in the whole extent of the tumor. Radiotherapy with a dosis of 59.4 Gy was subsequently performed. The patient remained tumor-free in the most recent control examination 3 years later.

[Vascular malformations of the orbit: haemodynamic classification and therapy concepts--a review]. / Vaskuläre Malformationen der Orbita: Hämodynamische Klassifikation und Therapiekonzepte--ein Review.

Vascular malformations of the orbit present special diagnostic and therapeutic challenges for the ophthalmologist. The haemodynamic characteristics and localisation of these lesions play a key role for therapy planning. Thorough clinical and radiological examinations are indispensable for patient counselling. Any decision for surgical intervention should be made on an individual basis after careful consideration of the intra- and postoperative risks. An interdisciplinary approach by an experienced team of radiologists, interventional radiologists, maxillofacial surgeons and ophthalmologists is in many cases necessary in order to achieve an optimal therapeutic result.

[Adenoid cystic carcinoma of the lacrimal gland: report of two cases and literature review]. / Adenoidzystisches Karzinom der Tränendrüse: Bericht von zwei Fällen und Literaturüberblick.

The adenoid cystic carcinoma of the lacrimal gland is a rare malignant orbital tumour, which has a generally poor prognosis. The therapy for adenoid cystic carcinoma of the lacrimal gland remains controversial. We present two cases of patients with adenoid cystic carcinoma of the lacrimal gland, who were treated with radical surgery in combination with radiotherapy. The therapeutic results are discussed with reference to relevant literature reports.

[Radiotherapy as therapeutic option for primary localised orbital lymphomas of low malignancy]. / Die Strahlentherapie als Therapieoption bei primären lokalisierten niedrig malignen orbitalen Lymphomen.

PURPOSE: The aim of this study was to assess the efficacy of radiotherapy as first line therapy for the treatment of primary localised orbital lymphomas of low malignancy (St IE) based on our cases. METHODS: 7 patients with histologically proven primary localised orbital lymphomas of low malignancy treated with radiotherapy were retrospectively reviewed. The dose was 40 Gy for unilateral and 36 Gy for bilateral lymphoma. The follow-up period was 12 - 60 months after the end of the therapy (mean follow-up period: 36 months). RESULTS: Complete regression was achieved in 6 patients 12 - 24 months after the end of radiotherapy. In one patient an increase of tumour mass was observed 3 months after radiotherapy. Chemotherapy was subsequently performed and the patient was free of disease 2 years later. In 2 cases cataract surgery had to be performed for post-radiotherapy cataract formation. CONCLUSION: The majority of orbital lymphomas are localised St. IE tumours of low malignancy. Radiotherapy is the first-line therapy in these cases. Success rates of > 90 % are reported in the literature.

[Idiopathic non-specific orbital inflammation: anatomic classification and treatment options]. / Idiopathische nicht spezifische orbitale Entzündung: anatomische Klassifikation und Therapieoptionen.

BACKGROUND: The aim of this study was to identify the clinical presentation of idiopathic non-specific orbital inflammations (NSI) in relation to the anatomic pattern of involvement in the orbit and to evaluate the treatment options. PATIENTS AND METHODS: 35 cases of patients with NSI were retrospectively reviewed. In all cases the diagnosis was confirmed by biopsy of the lesion. 10 patients exhibited a severe amount of fibrosis in biopsy specimens in terms of an idiopathic sclerosing non-specific orbital inflammation (SNSI). The patients were treated with corticosteroids, radiotherapy or surgery (en-block excision or orbital decompression/debulking). Follow-up data were available for 27 / 35 patients for a period of 2 to 24 months after initial diagnosis. RESULTS: The anatomic pattern of involvement was anterior in 7 / 35, intraconal in 5 / 35, diffuse in 1 / 35, apical in 2 / 35, and lacrimal in 20 / 35 patients. 16 patients with NSI were initially treated with systemic corticosteroids. 12 / 16 patients had a complete regression of symptoms, 1 / 16 patients had partial regression and 3 / 16 patients had no improvement. 4 patients with SNSI were initially treated with systemic corticosteroids. 3 / 4 patients with SNSI had no improvement under steroid therapy. 1 patient with SNSI received no treatment and no progression was observed on follow-up. 7 patients (3 with NSI and 4 with SNSI) were treated surgically. In 5 / 7 (2 with NSI and 3 with SNSI) surgical therapy was the initial treatment due to well-circumscribed lesions in orbital imaging, whereas in 2 / 7 orbital decompression with debulking was performed after failure of systemic corticosteroids. 5 / 7 patients had a complete resolution of symptoms, whereas 2 / 7 had a partial resolution with persistent postoperative diplopia. 3 patients were treated with radiotherapy. In 2 / 3 previous steroid therapy was unsuccessful in controlling the disease. 2 / 3 of these patients had complete regression and 1 / 3 partial regression of symptoms. CONCLUSIONS: Classification of NSI according to their anatomic pattern of involvement provides a useful framework for the clinical evaluation of these patients. Systemic steroids should be the first-line therapy in cases with NSI. However, if severe fibrosis in terms of SNSI is present, corticosteroid therapy is very likely to fail, so that surgical therapy should be considered early, especially in cases of relatively well-circumscribed lesions.

[Erdheim-Chester disease of the orbit with compressive optic neuropathy]. / Erdheim-Chester-Erkrankung der Orbita mit Kompressionsneuropathie.

A 60-year-old man presented with left exophthalmos and deterioration in visual acuity of slow evolution. Bilateral orbital Erdheim-Chester disease was diagnosed. Systemic evaluation revealed a retroperitoneal fibrosis. Treatment with interferon-alpha followed, but bilateral compressive optic neuropathy with visual acuity deterioration and visual field defects evolved. Bilateral orbital decompression was performed.