[Rare presentation of a scleral nevus with progression in size]. / Seltene Präsentation eines skleralen Nävus mit Größenprogredienz.

A 52-year-old male caucasian patient presented with a limbal subconjunctival pigmentation of unknown origin with progressive enlargement over the past years. Differential diagnoses included a malignant melanocytic lesion; therefore, an excisional biopsy was performed. Prior investigations showed no ciliary body or anterior chamber angle involvement. Histological and immunohistochemical analysis revealed the rare diagnosis of a scleral nevus. The clinical, histological and immunohistochemical findings as well as the relevant differential diagnoses are discussed.

[Blepharitis - When to Perform Surgery?]. / Blepharitis - wann greifen wir zum Skalpell?

AIM: Meibomian gland dysfunction/blepharitis is usually a harmless inflammatory process of the lid margin. However, it can be the first sign of a malignant lesion. Patients/Results: Clinical and histological findings are illustrated for 8 patients with changes in the lid margin and with the differential diagnosis of blepharitis. CONCLUSION: Tumours of the lid margin can mimic blepharitis or induce meibomian gland dysfunction. Conversely, blepharitis can present in a nodular form, which is highly indicative of a neoplasm. In these cases, surgical excision with ophthalmopathological evaluation of the tissue is mandatory, in order to reach the correct diagnosis and to treat the patient appropriately.

[Nodular eyelid margin and lip tumors without MEN2b syndrome in a 29 year old man]. / Lidkanten- und Lippentumoren ohne MEN2b-Syndrom bei einem 29-jährigen Mann.

Neuromas of the eyelid margin and lower lip were diagnosed in a 29-year-old man. As the combination of these lesions is indicative of multiple endocrine neoplasia type 2b (MEN2b) syndrome, the presence of a medullary thyroid carcinoma or a pheochromocytoma were excluded by a systematic work-up. A mutation in the RET proto-oncogene was not found by genetic testing. In summary, the patient presented with neuromas on the eyelid margin and lower lip without an association to a syndrome; however, patients with such neuromas should be screened for MEN2b syndrome due to the high mortality.

[Intraoperative OCT in ophthalmic microsurgery]. / Intraoperative OCT in der ophthalmologischen Mikrochirurgie.

Recent improvements in technology have enabled integration of SD-OCT into the optical path of an operating microscope. Here we present an overview of possible applications of iOCT (intraoperative optical coherence tomography) for retinal, glaucoma and corneal surgery. iOCT-based imaging can not only improve safety and quality of the surgery but also the learning curve of the surgeon. The ability to visualize barely visible or transparent structures with iOCT has achieved a significant added value intraoperatively, particularly in macular surgery, trabeculectomy and lamellar keratoplasty. Further, systematic assessment is warranted to define the usefulness of the iOCT more precisely.

[Mucinous sweat gland carcinoma of the eyelid]. / Muzinöses Schweißdrüsenkarzinom des Augenlids.

A 52-year-old patient presented with a painless nodular tumor of the upper left eyelid, which was first noticed 6 months prior to the initial presentation. The histopathological and immunohistochemical examination of the excised tumor revealed a mucinous sweat gland carcinoma. This very rare neoplasm (1/150,000 skin lesions) is located within the ocular adnexa in 40 % of cases. If completely excised the prognosis is usually good; however, due to the histological similarity to metastases of an adenocarcinoma, a hitherto unknown primary tumor at another site should be excluded.

[Salmon-pink colored conjunctival tumor with amyloid deposits]. / Lachsfarbener Bindehauttumor mit Amyloidablagerung.

An 82-year-old male patient presented with a salmon-pink colored conjunctival tumor of the left eye. A circumscribed, dense and whitish portion was detected by clinical examination. The histophological and immunhistochemical examination of the biopsy tissue revealed a CD20+ marginal zone lymphoma of the conjunctiva with amyloid deposits. Extranodal marginal zone lymphoma at this site is the most common lymphoma of the ocular adnexa and accounts for 5-10% of malignant diseases. An association with amyloid production is very rare and according to the current state of knowledge has no known impact on the outcome.

[Orbital metastasis of a previously unknown lung carcinoma mimicking posterior ischemic optic neuropathy]. / Orbitale Metastase eines bis dato unbekannten Lungenkarzinoms mit klinischem Aspekt einer posterioren ischämischen Optikusneuropathie.

A 65-year-old patient presented with increasing loss of vision in the right eye. A relative afferent pupillary defect as well as visual field perimetry deficits in an otherwise unremarkable eye led to the presumed diagnosis of ischemia of the optic nerve; however, further imaging revealed an extensive necrotic bronchial carcinoma in the left upper lobe metastasizing to the orbit with compression of the optic nerve. The clinical and histological features are discussed with respect to possible primary origins of orbital metastases.

[Routine investigation of foetal eyes--in what way and what for?]. / Routineuntersuchung fetaler Augen--wie und warum?

The investigation of foetal eyes not only allows for the observation of ocular development. It is supportive and sometimes even mandatory for the diagnosis of systemic and ocular syndromes. This review gives an overview about the investigation of foetal eyes, their assignment to developmental stages, challenges related to the investigation of foetal eyes, clinically relevant syndromes, and academic questions. The morphological development of the eye has been investigated since the 19th century and will not be covered in this article. The investigation of foetal eyes that have been collected during the routine paediatric autopsy, is complicated by artifacts. Artifacts are the result of autolysis, fixation, and mechanical manipulation. They have to be distinguished from genuine findings. Besides the search for findings such as coloboma or cataract, the morphological classification of the foetal eye is of importance. The anterior-posterior diameter allows for the diagnosis of microphthalmia. The case reports comprise Goldenhar's syndrome, MIDAS syndrome and others. In conclusion, the investigation of foetal eyes is often helpful and critical for paediatric diagnostics and should be performed with great care.

[Peripheral ulcerative keratitis in a 24-year-old patient]. / Hornhautrandulkus bei einer 24-jährigen Patientin.

A 24-year-old female patient was admitted to hospital with a deep stromal corneal defect on the right eye and a melting marginal keratitis on the left eye with a differential diagnosis of Mooren's ulcer. Despite intensive topical and systemic therapy, the ulcer perforated 3 days later and perforating keratoplasty à chaud was performed. The histological examination of the cornea showed a mainly intact corneal structure with a sharp demarcation line between the melting process close to the limbus and the unaffected tissue. The limbal area was interspersed with inflammatory cells. During the subsequent clinical course, despite intensive immunosuppressive therapy with Cellcept, systemic and local cyclosporin and methotrexate the left eye perforated and was subsequently treated by perforating keratoplasty. Under immunosuppression with methotrexate and local steroids no recurrence or progression has occurred so far.

[Orbital invasion of a maxillary ameloblastoma]. / Orbitale Infiltration durch ein Ameloblastom der Maxilla.

A 66-year-old man with a history of repeated surgery, external radiation and brachytherapy for ameloblastoma presented with a recurrence of the tumor with sinus, intraorbital and skull base infiltration. Histopathologic examination of the resected orbital and sinus tissue confirmed the diagnosis of ameloblastoma. Immunohistochemical staining for CD56 was strongly positive in the tumor cells. Although ameloblastoma is usually a low-grade malignant tumor, it can be locally aggressive with invasion of the surrounding tissue. Maxillary ameloblastomas are more likely to infiltrate the orbit.

[Clinico-pathological correlations: posterior compartment of the eye and orbit]. / Klinisch-pathologische Korrelationen: Befunde des hinteren Augenabschnitts und der Orbita.

Diseases of the posterior compartment and the orbit are characterised by histological findings, most of which can be reproduced clinically. Examples are the examination of calcifications in retinoblastoma by ultrasonography. In the present review, histological findings of tumour and other diseases of the posterior ocular compartment and the orbit are presented and correlated with the clinical pictures and imaging techniques: uveal melanoma, choroidal nevus, choroidal metastases, choroidal hemangioma, retinoblastoma, Coat's disease, sympathetic ophthalmia, pleomorphic adenoma (benign mixed tumour) of the lacrimal gland, dacryoadenitis, lymphoma, rhabdomyosarcoma, Langerhans cell histiocytosis, orbital metastases, and phthisical eyes. Histopathology is usually the gold standard for a definitive diagnosis. It is very important for residents and those in training to become familiar with clinico-pathological correlations as these provide insight in pathophysiological processes. Regarding ophthalmic surgery, ophthalmic pathology offers the possibility to study wound healing and complications. A close collaboration between clinicians and ocular pathologists allows for an optimised processing of the submitted tissue and diagnosis. Thus, pre- and postoperative care can also be improved. This outstanding knowledge that ophthalmologists have gained over the last decades and beyond, should be preserved and passed on to the next generations in order to maintain a high standard in ophthalmological care.

[Diffuse anterior retinoblastoma]. / Diffuses anteriores Retinoblastom.

Retinoblastoma is the most common primary intraocular tumor in childhood. Diffuse anterior retinoblastoma is an uncommon variant and usually occurs in comparatively older children. Typically, there is an extensive infiltration of the anterior segment by tumor cells clinically mimicking anterior uveitis with pseudohypopyon. The actual retinal focus is often very small and may not be detected despite a thorough histological examination. In this case report the clinical and histological findings of a diffuse anterior retinoblastoma are described.

[Analysis of an ophthalmic pathology cohort of human fetal eyes with regard to interesting findings]. / Analyse eines ophthalmopathologischen Kollektivs humaner fetaler Augen unter besonderer Berücksichtigung außergewöhnlicher Befunde.

OBJECTIVE: Information on the evaluation of prenatal ocular findings is sparse. This article provides an overview of the morphology in a cohort of human fetal eyes, with particular emphasis on interesting findings. METHODS: The study investigated 216 eyes from 115 human fetuses. RESULTS: The majority of fetal eyes presented with a regular phenotype. Rarely, unexpected findings were discovered in fetuses with or without systemic malformations. CONCLUSION: Routine evaluation of fetal eyes reveals-albeit rarely-new aspects providing further knowledge and occasionally enabling the exact classification of syndromes.

[Subcutaneous tumour in an adolescent]. / Subkutaner Tumor bei einem Jugendlichen.

A 13-year-old adolescent presented with an enlarging subcutaneous mass beneath the right eyebrow. Clinically suspicious for dermoid cyst or pilomatrixoma, it was decided to remove the tumour completely. Based on histopathological examination the diagnosis of intravascular papillary endothelial hyperplasia (IPEH) was made. Histopathological features are discussed with regard to the possible pathogenesis.

[Conjunctival myxoma: two cases. Histopathological examination and clinical course]. / Konjunktivales Myxom - zwei Kasuistiken. Histopathologische Aufarbeitung und klinische Korrelation.

Conjunctival myxoma is a rare and benign tumour without tendency for recurrence. Here, we present the histological features and the clinical course of two cases. Removal of each tumour was followed by histopathological examination including immunohistochemistry as well as electron microscopy. Besides the expected findings characteristic of conjunctival myxoma some features reflecting the possible aetiology of this entity are shown.

[Pilomatrixoma of the ocular adnexae: clinical and histologic analysis (13 cases)]. / Pilomatrixom der okulären Adnexe: Klinische und histologische Aufarbeitung von 13 Fällen.

BACKGROUND: Pilomatrixoma (calcifying epithelioma of Malherbe) is a rare benign tumour originating from the matrix of the hair root. In this study, we evaluated the clinical and histopathological characteristics of all the respective tumour specimens submitted to our laboratory since 1997. PATIENTS/MATERIALS AND METHODS: We reviewed the clinical and histologic findings of 13 pilomatrixomas localised to the ocular adnexae. Paraffin sections were stained with H&E and PAS and, in addition, immunohistochemistry was performed using markers for macrophages (CD68), for proliferative activity (Ki67), and for proliferation/apoptosis (CAS/CSE1L). RESULTS: All 13 pilomatrixomas were of reddish-livid colour and localised on the upper eyelid and/or near the eyebrow. The patients ages ranged from 4 to 84 years without preference for either gender. In 3 cases the correct diagnosis was made intraoperatively, while no pilomatrixoma was diagnosed prior to surgery. The most frequent false diagnoses were retention cyst (n = 3), atheroma (n = 2), and abscess (n = 2). There was no tumour recurrence in any of the cases within the time period presented. All 13 pilomatrixomas demonstrated the characteristic histology with areas of basophilic islands, shadow cells, calcification and a chronic granulomatous inflammation with foreign body giant cells in varying amounts. Immunhistochemical staining with anti-Ki67 and anti-CAS/CSE1L indicated a regular cellular maturation of the basophilic cells. No correlation was found between any of the histological or immunohistochemical markers and clinical parameters such as size, age and gender of the patient and duration of tumour presence prior to surgery. CONCLUSIONS: This case series represents one of the largest published so far, and underscores that pilomatrixoma can appear at any age. While it is usually not recognised clinically, it should be considered in the differential diagnosis especially for tumours of the upper eyelid. Our immunohistochemical findings support the basically benign nature of a pilomatrixoma but also point to the chronic inflammatory stimulus that can lead to complications after incomplete removal.