Subconjunctival epidermoid cysts in Gorlin-Goltz syndrome.

Epidermoid cysts are common benign cysts which occur particularly on the skin of the face, neck and upper trunk. Subconjunctival location of these cysts is very rare and, until today, only seen in patients with Gorlin-Goltz syndrome. Histopathological examination of these cysts show similarities with odontogenic keratocysts, a typical clinical manifestation of Gorlin-Goltz syndrome.

The use of propranolol in the treatment of periocular infantile haemangiomas: a review.

Infantile haemangiomas (IH) are benign vascular tumours characterised by their very rapid growth. Although usually innocuous, periocular IH can cause serious visual loss through induction of strabismic, deprivational or anisometropic astigmatism. Common treatment modalities for these IH include intralesional and systemic oral steroids; however, both treatments are associated with potentially severe side effects. A report was published recently demonstrating the impressive effect of propranolol in the treatment of IH. This exciting finding has provoked a paradigm shift in the management of this condition. So far little has been reported in the specific ophthalmologic literature, although case reports are emerging. This review gives an overview of the recent findings and includes the authors' experience with 10 patients treated with propranol.

Diagnosis and treatment of a superficial upper eyelid arteriovenous malformation.

UNLABELLED: Combined embolization and surgical excision as a safe treatment for a high flow superficial upper eyelid anteriovenous malformation. INTRODUCTION: Orbital arteriovenous malformations (AVM) may cause significant morbidity including chronic pain and cosmetic disfigurement. These rare lesions require a multidisciplinary approach. A case of an orbital high-flow AVM treated subsequently by an mterventional radiologist and oculoplastic surgeon is presented. MATERIALS AND METHODS: Clinical assessment, computed tomography and digital substruction angiography revealed a high-flow AVM. Transvenous and percutaneous embolization was followed by surgical excision. RESULTS: A 1.5 cm, slowly growing, compressible pulsating lesion in the left upper eyelid of a 23 year old man was found. Eye motility and ophthalmologic examination were normal. Valsava manoeuvre didn't increase the size. Imaging revealed a high-flow AVM feeded by the arteria temporalis superficialis (itself feeded by the a. supraorbitalis and supratrochlearis). Draining vessels were the superior ophthalmic vein and a superficial lateral orbital vein. This malformation was first embolized. To prevent iatrogenic embolization of the sinus cavernous, the vein draining into the superior ophthalmic was occluded by a transvenous approach, followed by a percutaneous injection with diluted glue (equal amounts of glubran-2 and lipiodol). The remaining low-flow lesion was then safely excised. CONCLUSION: High-flow orbital AVM represent a considerable treatment challenge. Good cosmetical and functional outcome was obtained safely by subsequent embolization and surgical excision. When treating these rare malformations it is of the highest importance to coordinate the interdisciplinary efforts.

[Results of penetrating keratoplasty in syphilitic interstitial keratitis]. / Résultats obtenus par kératoplastie transfixiante dans le cas d'une kératite syphilitique interstitielle.

PURPOSE: To compare the results in our patient series after penetrating keratoplasty (PKP) for syphilitic interstitial keratitis (IK) with those described in the literature. METHODS: Retrospective case series in which visual acuity (VA), graft clarity, rejection episodes, intraocular pressure and endothelial cell density (ECD) were examined postoperatively. RESULTS: Postoperative VA improved in all cases. There was no evidence of wound dehiscense or occurrence of retrocorneal membrane formation in any case. Postoperative inflammation was not more severe in our patients with syphilitic IK compared to patients undergoing PKP for other reasons. A normal decline in ECD proved that there was no subclinical inflammation as well. CONCLUSION: PKP for syphilitic IK has a good prognosis in our case series as far as graft survival is concerned. Improvement in VA was present in all cases, though sometimes limited. In our case series, we experienced less postoperative complications than described in the older literature, which is probably due to better microsurgical techniques used nowadays.

Delay in diagnosis and outcome of Acanthamoeba keratitis.

PURPOSE: To evaluate the outcome of Acanthamoeba keratitis with respect to the delay in diagnosis. METHODS: A retrospective review of the records of 14 patients treated for Acanthamoeba keratitis was carried out. Delay in diagnosis was correlated with risk factors, clinical presentation, method of diagnosis, final visual acuity and need for penetrating keratoplasty. RESULTS: Based on the time interval between the first symptoms and the diagnosis of Acanthamoeba keratitis, it appeared that patients could be divided into two groups: an early treatment group (group I), consisting of six patients treated within 18 days of onset of symptoms, and a late treatment group (group II), composed of eight patients treated beyond that time. There were no statistically significant differences between the two groups as far as risk factors, clinical presentation, accuracy of diagnosis and method of diagnosis were concerned, although more extensive and deeper corneal involvement was noted in group II. Improvement in visual acuity following medical therapy was seen in all six patients in the early group and in three (37%) of the eight patients in the late group. One patient in group I needed urgent penetrating keratoplasty for corneal necrosis. In group II, two patients underwent penetrating keratoplasty à chaud to prevent corneal perforation and three patients needed penetrating keratoplasty to restore functional visual acuity. CONCLUSION: A diagnostic delay of less than 18 days between onset of symptoms and start of anti-amoebic treatment results in a better final VA after medical treatment and obviates the need for urgent and elective penetrating keratoplasty.

Results of penetrating keratoplasty in syphilitic interstitial keratitis.

PURPOSE: To compare the results in our patient series after penetrating keratoplasty (PKP) for syphilitic interstitial keratitis (IK) with those described in the literature. METHODS: Retrospective case series in which visual acuity (VA), graft clarity, rejection episodes, intraocular pressure and endothelial cell density (ECD) were examined postoperatively. RESULTS: Postoperative VA improved in all cases. There was no evidence of wound dehiscense or occurrence of retrocorneal membrane formation in any case. Postoperative inflammation was not more severe in our patients with syphilitic IK compared to patients undergoing PKP for other reasons. A normal decline in ECD proved that there was no subclinical inflammation as well. CONCLUSION: PKP for syphilitic IK has a good prognosis in our case series as far as graft survival is concerned. Improvement in VA was present in all cases, though sometimes limited. In our case series, we experienced less postoperative complications than described in the older literature, which is probably due to better microsurgical techniques used nowadays.

Corneal decompensation in a boy with Kearns-Sayre syndrome.

This paper describes the clinical history of a young boy with Kearns-Sayre syndrome. The first presenting symptom of Kearns-Sayre syndrome in this boy was corneal edema with photophobia and tearing.

The effect of duration and timing of systemic cyclosporine therapy on corneal allograft survival in a rat model.

BACKGROUND: Systemic cyclosporine A (CsA) remains a valuable treatment option in the prevention of corneal graft rejection, but the question of timing and duration of this systemic therapy remains unresolved. The effect of a pre- and postoperative dosing schedule, related to the expected moment of rejection, was examined in a rat model. METHODS: All AO (strain) recipients of PVG grafts were assigned to the following treatment groups: Group 1 (controls), groups 2-5 (a postoperative treatment regimen of CsA for 5, 10, 15 and 30 days respectively) and groups 6 and 7 (CsA preoperatively for 5 days and postoperatively for another 5 or 10 days respectively). Corneal allografts were clinically evaluated and blood CsA levels were measured at various time points. RESULTS: Untreated controls rejected their allografts after 13 days. Regression analysis showed a strongly significant positive correlation between graft survival time and duration of cyclosporine therapy. There was no difference in graft survival between groups 3 (CsA 10 days) and 4 (CsA 15 days). A pre-operative dosing schedule of CsA followed by postoperative treatment had no advantage over a solely postoperative treatment regimen. The moment of rejection was characterized by a low to undetectable CsA concentration. CONCLUSION: The present study demonstrates a significant influence of the duration of systemic CsA administration on allograft survival time. However, preoperative administration of CsA does not seem to have an additional influence on graft survival, which is in line with the biological evidence of the mechanism of action of CsA on the efferent arm of graft rejection.

Bilateral corneal melting in a patient with paraneoplastic pemphigus.

An 80-year-old man, with a solid abdominal tumor and multiple skin lesions, was admitted to the hospital because of a perforated right cornea and an impending perforation of the left. The clinical, histological, immunohistological and immunoprecipitation findings of the skin lesions were consistent with Anhalt's criteria for paraneoplastic pemphigus (PNP). The underlying malignancy proved to be an incurable peripheral neuronal shaft tumor. Both conjunctivae appeared normal. The right eye revealed a flat anterior chamber, due to a spontaneous, central corneal perforation. The central part of the left cornea had severely thinned, resulting in a descemetocele, which eventually perforated. Multiple surgical interventions were needed to restore the anterior chamber in both eyes. Although a causative association between PNP and corneal perforation could not be demonstrated, we think that corneal melting should be added to the list of ocular complications in patients with PNP.

Acanthamoeba keratitis: a review.

Acanthamoeba keratitis is caused by protozoa and characterised by a protracted course. All patients presenting with a therapy-resistant keratitis, even non-contact lens wearers, should be examined for the presence of Acanthamoeba by means of specific cultures, histopathological stainings and--if necessary--a corneal biopsy. The combination of clinical signs, such as excessive pain, a radial keratoneuritis and in a later phase a stromal ring infiltrate, together with a suggestive history (contact lenses, polluted water) is an important factor for the early diagnosis. Because of improved clinical detection and earlier diagnosis, the infection can often be controlled with a combination therapy of polyhexamethylene biguanide or chlorhexidine with propamidine and neomycine. This results in a better visual prognosis and a decreased need for therapeutic keratoplasty.