MRI features of craniofacial fibrous dysplasia.

A 13-year-old female with a 4-year history of monostotic fibrous dysplasia had noticed a progressive proptosis of the right eye and diplopia on upward gaze for 4 weeks. A few years previously an incisional biopsy of the skull had verified the presumed diagnosis of fibrous dysplasia with recurrent bleeding into pathologic cystic bony structures of the skull. The patient was known to have craniofacial fibrous dysplasia with involvement of the frontal and intermediate cranial base, the posterior ethmoidal labyrinth, and the sphenoidal and maxillary sinuses.

Current concepts in the classification, diagnosis and treatment of hemangiomas and vascular malformations of the head and neck.

There are many different classifications of vascular anomalies. As the correct classification of the vascular lesion has a direct influence on therapy it is difficult to decide which treatment should be considered as the treatment of choice. Based on an extensive review of the literature and personal experience of the treatment of more than 200 patients with hemangiomas or vascular malformations of the head and neck, a clinical classification is described that allows vascular lesions to be categorized in order to plan purposeful treatment.

[The efferent lacrimal ducts of the human. Morphological and biochemical studies].

Purpose: Infections occur frequently in the region of the efferent tear ducts. Exact knowledge of the anatomical structure and of cellular defense mechanisms is necessary to understand the pathological processes. This study analyzed the efferent tear ducts with regard to physiological function and possible defense mechanism against infections.

[Differential diagnosis of sinugenic enophthalmos].

Background: Spontaneous enophthalmos without recent trauma is a rare condition. Its origin is difficult to evaluate.

Method: Specific properties of this symptom complex are presented based on a literature review and on case reports.

[Mutations in the keratin gene as a cause of Meesman-Wilke corneal dystrophy and autosomal dominant skin cornification disorders].

Background: Meesmann's corneal dystrophy (OMIM 122,100) is a rare autosomal dominant disorder of the corneal epithelium. It manifests in early childhood and affects both eyes. The disease is characterized by variable patterned dot-like corneal opacities and intraepithelial vesicles, which can be seen by slit-lamp examination and retro-illumination.

A novel keratin 12 mutation in a German kindred with Meesmann's corneal dystrophy.

Aim: To study a kindred with Meesmann's corneal dystrophy (MCD) to determine if a mutation within the cornea specific K3 or K12 genes is responsible for the disease phenotype.

Methods: Slit lamp examination of the cornea in four members of the kindred was carried out to confirm the diagnosis of MCD. The region encoding the helix initiation motif (HIM) of the K12 polypeptide was polymerase chain reaction (PCR) amplified from genomic DNA derived from affected individuals in the kindred.

Molecular genetics of Meesmann's corneal dystrophy: ancestral and novel mutations in keratin 12 (K12) and complete sequence of the human KRT12 gene.

Recently, we identified the first mutations in corneal keratins K3 and K12 in families with Meesmann's corneal dystrophy (MCD). Here, we sequenced all regions of the human K12 gene, to enable mutation detection for all exons using genomic DNA as a template. The human K12 genomic sequence spans 5919 bp and consists of eight exons.

Primary orbital leiomyoma and leiomyosarcoma.

A case of an extremely rare primary orbital leiomyoma in a 25-year-old male patient is presented who had a lifelong history of deviation of the left eye globe with slight enophthalmos and reduced motility. Because of pain and increasing deviation of the eye the tumor was totally resected. On histologic examination the tumor showed ossification which is extremely rare so that a calcifying fibroma had to be ruled out.

Functional anatomy of the human efferent tear ducts: a new theory of tear outflow mechanism.

Background: The mechanism of lacrimal drainage under physiological conditions is controversial. The aim of this study was to analyze the three-dimensional architecture of human efferent tear ducts from functional and clinical points of view. A new theory of tear outflow is discussed.

Functional anatomy of human lacrimal duct epithelium.

Resorption of tear fluid in the lacrimal ducts has hitherto been controversial; one reason for this has been insufficient knowledge of the anatomical structure and function of the lacrimal duct epithelium. The present study analyzes the structure of lacrimal duct epithelium by means of histological, histochemical, immunohistochemical and electronmicroscopical methods and draws a conclusion about its physiological function regarding its role in immunodeficiency. Investigations were performed on 31 lacrimal systems of 17 male and 14 female individuals (aged 54-88 years).

[ORBIT-NET. Discussion forum on orbitology on the internet].

Introduction: A basic service in the public network "Internet" is electronic mail (e-mail). E-mail makes the participation in discussion groups possible by mailing contributions to the discussion to all members electronically. To complement the existing list of ophthalmologic discussion groups ORBIT-NET was introduced.

Bilateral cataract after phakic posterior chamber top hat-style silicone intraocular lens.

Background: New strategies have been developed for surgical treatment of high myopia. Recently, implantation of minus power posterior chamber intraocular lenses (IOL) into phakic eyes has been introduced.

Methods: We report a 37 year-old female patient who developed bilateral anterior subcapsular cataract 7 years after minus-power, top hat-style silicone posterior chamber IOL implantation in Russia.

Primary antiphospholipid antibody syndrome and retinal occlusive vasculopathy.

Purpose: To report a 31-year-old healthy patient with retinal venous occlusion in his left eye attributable to primary antiphospholipid antibody syndrome.

Methods: The patient was examined clinically. Multiple serologic and clinical investigations were performed to determine the causative disease.

Mutations in cornea-specific keratin K3 or K12 genes cause Meesmann's corneal dystrophy.

The intermediate filament cytoskeleton of corneal epithelial cells is composed of cornea-specific keratins K3 and K12 (refs 1,2). Meesmann's corneal dystrophy (MCD) is an autosomal dominant disorder causing fragility of the anterior corneal epithelium, where K3 and K12 are specifically expressed. We postulated that dominant-negative mutations in these keratins might be the cause of MCD.

[Changes in extracellular matrix in the lamina cribosa of patients with secondary glaucoma].

The aim of this study was to analyze the localization and distribution of extracellular matrix in normal and glaucomatous damaged optic discs using immunohistochemical methods. Five eyes donated for corneal allografting without any history of glaucoma and three other eyes with secondary glaucoma were studied. Immunohistochemical reactions were performed with antibodies against collagen types I, III, IV and VI and against laminin, proliferating antigen KI67 and GFAP.

Circular surgical cyclopexy after extensive traumatic cyclodialysis.

The authors operated on a patient who had bilateral ocular hypotony after severe blunt ocular trauma with subsequent circular cyclodialysis and luxation of the lenses. Because of the specific situation, none of the previously reported procedures could be applied. A new surgical technique was used to circularly refixate the ciliary body ab interno.

[Orbital hematomas].

Background: Orbital hematomas may occur spontaneously, as a result of vascular anomalities, or they may be induced by trauma or occur following paranasal sinus surgery. The retrobulbar hematoma requires special attention because of its potential compression of the optic nerve may compromise vision or cause blindness.

Patients And Methods: We report on four cases: two subperiostal orbital hematomas, a spontaneous retrobulbar hematoma, and one orbital hematoma due to trauma.

SEM studies of the collagen architecture of the human lamina cribrosa: normal and pathological findings.

The arrangement of the collagen fibrils of the lamina cribrosa was analyzed by using scanning electron microscopy with clinical regard to morphological alterations in cases of glaucoma at different stages. Adult eyes with no apparent pathological changes and specimens from patients with absolute glaucoma were studied. In the lamina cribrosa of nonpathological eyes the collagen fibrils are arranged circularly around the points of passage of axons and vessels.

Scanning electron-microscopic studies of the collagen architecture of the human sclera--normal and pathological findings.

The arrangement of the collagen fibrils of the human sclera was analyzed in the region of the limbus cornea, the corneoscleral trabeculum, at the zone of muscle insertion and at defined areas of the internal and external surface of the sclera. Adult eyes with no apparent pathological alterations and the sclera of a patient with staphyloma were examined by scanning electron microscopy. The investigations were performed to describe regularities in the collagen architecture in normal and pathologically altered eyes to understand pathomorphologic and pathophysiologic changes in scleral diseases.

[Sandwich intraocular lens implant: a concept for aphakia correction in children].

Background: In the management of congenital cataracts the correction of aphakia is still an unsatisfactorily solved problem. As far as surgical techniques and materials are concerned, the implantation of an IOL seems to be justified even in younger children; but choosing the refractive power of the lens is somewhat difficult regarding the expected growth of the eye.

Materials And Methods: A new type of IOL is presented as a solution for this problem.

[Emergency therapy of traumatic orbital hematoma with acute visual impairment].

Midfacial injuries, surgery of the orbit or the paranasal sinuses as well as retrobulbar anesthesia can be the cause of a traumatic laceration of the ophthalmic artery and/or its branches prompting an extensive orbital hemorrhage with consecutive blindness. Since the neurosensory retina does not tolerate an ischemia of more than 1-3 hours, therapy has to be emergent: an extensive lateral horizontal canthotomy and vertical cantholysis, and if the hypertony of the globe persists, splitting of the periorbita in the temporal lower lid region are mandatory. The surgical details are presented.

[Coronal incision as the surgical approach to the orbits].

The coronal incision of the skin extends from the ipsilateral preauricular region to the other side parallel to the coronal suture. The skin flap can then be mobilized down to nearly the floor of the orbits. We routinely use this incision in the following diseases and surgical procedures of the orbit: (1) osteo- and orbitotomies in premature synostosis of skull bones and craniofacial dysplasias; (2) frontobasal midface traumatology involving the orbit; (3) removal of fronto-ethmoidal mucoceles and tumors with orbital involvement; (4) bilateral medial three-wall and lateral one-wall decompression in Graves' disease; (5) removal of tumors from the upper and medial level of the orbit via a transperiostal incision or an extended supraorbital resection.