Ligneous conjunctivitis: Fresh-frozen plasma and heparin use intra-and postoperatively, a report of two cases.

Ligneous conjunctivitis is a rare chronic form of recurrent membranous inflammation and plasminogen deficiency. Ocular manifestations may be associated with sites other than mucous membranes, such as the oral cavity, internal ear, respiratory, genitals, and kidney. Treatment is extremely difficult because of the lack of topic plasminogen drops, and a high volume is required for systemic supplementation. This report aimed to present two patients with ligneous conjunctivitis treated with membrane excision, topical fresh-frozen plasma, and heparin intra-, and postoperatively. No recurrence was found in the ligneous membrane in the 12-month follow-up. The use of topical fresh-frozen plasma and heparin after membrane excision could be effective to avoid recurrence.

Acquired ankyloblepharon post purulent membranous conjunctivitis and its novel surgical management: A case report.

INTRODUCTION: Ankyloblepharon is a partial or complete fusion of eyelids by webs of skin. It may be congenital or acquired. Herein we report a rare case of acquired bilateral ankyloblepharon that developed after purulent membranous conjunctivitis. We will also discuss its unique surgical management which has not been reported previously in the literature. CASE: A 21 year old male presented with complaints of narrowing of eyes for the last six months following an attack of acute purulent membranous conjunctivitis. Surgical excision of the adhesions was done. We used conjunctival autograft to cover the raw area left after excision as a new modality of treatment. The graft was successfully taken up and no recurrence of adhesions was found at 1 year follow up. OBSERVATIONS: Various modalities of treatment for ankyloblepharon have been reported which includes excision of tissue and suturing and amniotic graft. Here we have used conjunctival autograft on the raw area after excision of adhesions and the result obtained was good. CONCLUSION: To the best of our knowledge this is the first and a rare case of acquired ankyloblepharon in a young male following a single episode of purulent membranous conjunctivitis. Conjunctival autograft can be successfully used to cover the raw areas left after excision of adhesions.

Intra-lenticular caterpillar seta in ophthalmia nodosa.

PURPOSE: To report the clinical course of ophthalmia nodosa with a retained lenticular seta misdiagnosed and treated as non-infectious posterior uveitis for 7 consecutive years. METHODS: Meticulous clinical examination led to discovery of the caterpillar seta embedded in the crystalline lens and the intravitreal setae. RESULTS: Lens-sparing pars plana vitrectomy and removal of free-floating vitreal seta resulted in complete resolution of vitritis and uveitis. CONCLUSION: Embedded seta within a clear lens may remain sequestered, and may be left untouched under close observation, precluding a clear lens extraction in such patients.

Ocular Sequelae of Stevens-Johnson Syndrome: A Comprehensive Approach.

The chronic sequelae of Stevens-Johnson syndrome cause severe ocular morbidity. Among the various manifestations, including dry eye, cicatrization causing forniceal shortening, adnexal disorders, lid margin keratinization, and limbal stem cell deficiency, keratinization of the lid margin leads to progressive ocular surface damage through constant blink-induced microtrauma. This damage induces corneal punctate keratopathy and neovascularization and can produce epithelial defects leading to corneal infection or perforation. Management strategies for chronic ocular sequelae are broadly categorized into ocular surface stabilization procedures, tectonic procedures, and visual rehabilitation procedures. Interventions in the acute stage of the disease include amniotic membrane grafting. Timely intervention helps to prevent deterioration of the ocular surface in these eyes.

A Case of Subconjunctival Dirofilariasis in Bulgaria.

We report a case of subconjunctival invasion of Dirofilaria repens in a woman from Southern Bulgaria. Dirofilariasis is an extremely rare parasitic disease for Bulgaria. It occurs as an endemic parasitosis in the Mediterranean countries. It is caused by helminths of the genus Dirofilaria, which are parasites in dogs, cats, foxes, wolves - the final hosts of the parasite. It may very rarely occur in humans, the infection triggered by a bite of bloodsucking mosquitoes, which are the intermediate hosts of Dirofilaria. The clinical forms of dirofilariasis in humans are subcutaneous, pulmonary and ocular, the brain, testes and mammary glands being rarely affected. A 51-year-old patient presented with migrating edema in the area of the left temple and upper eyelid, with redness and swelling of the conjunctiva, as well as a subjective sense of movement under the eyelid of the left eye, lasting for about ten days. The subconjunctival localization of a moving 9.5-cm nematode was established, it was surgically removed and histologically studied. This case of ocular subconjunctival dirofilariasis is of clinical interest due to the rarely observed pathology.

Amniotic membrane transplantation for ligneous conjunctivitis in a doberman.

An approximately 2-year-old female Doberman Pinscher was referred for the evaluation of bilateral, chronic proliferative conjunctivitis. Ophthalmic examination revealed bilateral thick, opaque pseudomembranes originating from the conjunctivae that prevented visualization of the cornea and interior structures of the eye. Histopathological findings of biopsies of the pseudomembranes were consistent with ligneous conjunctivitis. Serum plasminogen activity levels were within the normal range. Treatment with topical and systemic anti-inflammatory and immunosuppressive drugs did not improve the conjunctival lesions. The pseudomembranes were surgically excised, and the conjunctival surfaces were reconstructed with amniotic membrane. At final re-examination two years postsurgery, there was no evidence of recurrence of the pseudomembranes. To the authors' knowledge, this is the first reported case of the successful treatment of canine ligneous conjunctivitis with amniotic membrane transplantation.

[Tarsal Kink: Pathognomonic Presentation and Therapy of a Rare Congenital Upper Eyelid Malformation]. / Tarsusknickung ("Tarsal Kink"): Pathognomonische Präsentation und Therapie einer seltenen kongenitalen Oberlidfehlbildung.

BACKGROUND: Presentation of a congenital abnormality that is rare, but follows a distinct course and can be diagnosed and cured promptly if the pathognomonic presentation is recognized. A congenital tarsal kink leads to a malposition of the upper eyelid margin that must not be missed, as it will lead to ulcerative keratitis if it is not treated. CASE PRESENTATION: An otherwise healthy newborn was presented after delivery with forceps with marked unilateral purulent secretion and blepharospasm. DIFFERENTIAL DIAGNOSIS: Neonatal dacryocystitis, gonococcal infection, congenital entropion with ulcerative keratitis, tarsal kink. EXAMINATION: It was not possible to fully examine the lid and cornea with the baby awake. Due to total inversion of the lid margin, no lashes could be seen. Under general anesthesia, the tarsal kink, with complete inversion of the lid margin and a corneal ulcer, was confirmed. TREATMENT: The literature offers several methods to correct this rare malposition, all of which aim to strengthen the anterior lamella to correct the kink. After incision of the kink and repositioning of the tarsus and securing the position with fixation sutures, the ulcer healed quickly and completely; lid closure and lid contour were normal and symmetrical. SUMMARY: Complete inversion of the lid margin is the pathognomonic sign of tarsal kink, giving the impression of "missing" lashes, accompanied by blepharospasm, followed by purulent secretion and corneal ulceration. The condition must not be misdiagnosed as only immediate correction can prevent severe damage.

Topical Plasminogen as Adjunctive Treatment in Recurrent Ligneous Conjunctivitis.

Ligneous conjunctivitis is a rare, autosomal recessive, membranous conjunctivitis characterized by a deficiency in type 1 plasminogen. The absence of normal plasmin activity results in the formation of fibrin-rich, membranous material that typically manifests on the palpebral conjunctiva. Surgical treatment often causes irritation of the conjunctiva and accelerated recurrence of pseudomembranes. In this interventional case report, the authors document the results of treatment with topical plasminogen following conjunctival pseudomembrane excision in a 32-year-old woman. The patient underwent pseudomembrane excision in the OS followed immediately by hourly topical application of plasminogen eye drops. The plasminogen was prepared from pooled human plasma purchased under Food and Drug Administration approval from DiaPharma. Follow-up evaluation at 1 week, 1 month, and 5 months showed no evidence of recurrent pseudomembranous change. Adjunctive topical plasminogen application appears to be an effective and safe method of controlling pseudomembrane recurrence in patients with ligneous conjunctivitis.

Surgical Correction of Giant Fornix Syndrome.

PURPOSE: To describe a surgical procedure and its outcomes for the management of chronic pseudomembranous kerato-conjunctivitis secondary to giant fornix syndrome (GFS). METHODS: Retrospective case series of 6 patients undergoing fornix shortening surgery for giant fornix syndrome. RESULTS: Surgery produced complete resolution of symptoms in 5/6 (83%) patients and complete relief prior to partial relapse in 1. Mean follow up was 18 months (range: 3-41 months). In the 4 (of 6) patients who had measurements taken, the mean upper eyelid forniceal depth reduced from 21.25 mm (n = 4, SD: 2.87) preoperatively to 16.5 mm (n = 4, SD: 2.65) postoperatively. CONCLUSIONS: Fornix reconstruction may be an effective and well-tolerated treatment for refractory GFS. Resection of excess conjunctiva restores the normal anatomy within the conjunctival cul-de-sac thereby reducing the incidence of protein coagulum formation.

The Usage of a Conjunctival Flap to Improve Retention of Boston Type 1 Keratoprosthesis in Severe Ocular Surface Disease.

PURPOSE: The Boston keratoprostheses type 1 devices (KPro) are utilized in cases unfavorable to penetrating keratoplasty. The prognosis remains guarded in cases of ocular surface disease due to risk of tissue necrosis. We describe a novel surgical approach using a conjunctival flap with a delayed opening to improve retention. METHODS: In three patients with advanced cicatrizing conjunctivitis, a Type 1 keratoprosthesis was stabilized using a full tarsal conjunctival flap. Three months postoperatively, an opening was created in the flap overlying the optical portion of the device. RESULTS: All patients had no device related complications over a mean follow-up period of 17.7 months (range 15-21 months) and vision remained excellent at better than 20/200 for all patients. CONCLUSIONS: Utilization of a tarsal flap either primarily as part of a two stage modified technique or secondarily in cases of tissue necrosis and impending device extrusion might maximize retention of the type 1 KPro.

Adjuvant Role of Amniotic Membrane Transplantation in Acute Ocular Stevens-Johnson Syndrome: A Randomized Control Trial.

PURPOSE: To evaluate the adjuvant role of amniotic membrane transplantation (AMT) in cases of acute ocular Stevens-Johnson syndrome (SJS). DESIGN: Prospective randomized controlled clinical trial. PARTICIPANTS: Twenty-five patients (50 eyes) with acute ocular SJS who presented within 4 weeks of onset of symptoms were recruited. METHODS: The eyes were randomized into 2 groups that underwent either AMT with medical therapy (MT; n = 25) or standard MT alone (n = 25). The patients were evaluated at presentation and during follow-up at 1 week and 1, 3, and 6 months. The parameters evaluated were the best-corrected visual acuity (BCVA), Schirmer test, tear film breakup time (TBUT), conjunctival congestion, corneal haze, vascularization, conjunctivalization, and limbal stem cell involvement. Lid edema, symblepharon, ankyloblepharon, ectropion, entropion, trichiasis, and metaplastic lashes also were analyzed. MAIN OUTCOME MEASURES: Maintenance of BCVA and stable ocular surface. RESULTS: At the end of 6 months, the mean BCVA was significantly better in the AMT group (0.068±0.10 logMAR units) compared with the MT group (0.522±0.52 logMAR units; P = 0.042). The mean TBUT in the AMT and MT groups was 9.92±4.1 and 6.96±4.5 seconds, respectively (P = 0.015). The mean Schirmer test results in the AMT and MT groups were 15.4±6.3 and 8.64±5.4 mm, respectively (P < 0.001). Conjunctival congestion persisted in 44% (11/25) in the MT group compared with 4% (1/25) in the AMT group (P = 0.03) at the end of the 6-month follow-up. No case in the AMT group demonstrated corneal haze, limbal stem cell deficiency, symblepharon, ankyloblepharon, or lid-related complications. Among eyes in the MT group, corneal haze occurred in 44% (11/25; P = 0.001), corneal vascularization and conjunctivalization in 24% (6/25; P = 0.03), symblepharon in 16% (4/25; P = 0.12), ankyloblepharon in 4% (1/25; P = 1.00), ectropion and entropion in 8% (2/25; P = 0.47), and trichiasis and metaplastic lashes in 24% (6/25; P = 0.03) eyes. CONCLUSIONS: Amniotic membrane transplantation is a useful adjunct to conventional MT in maintaining BCVA and a stable ocular surface in cases of acute ocular SJS. Furthermore, the adjunctive use of AMT also helps to prevent intermediate-term ocular cicatricial sequelae.

Delayed Boston Keratoprosthesis Exchange due to a Preceding Vitreoretinal Surgery with Intraoperative Choroidal Detachment.

PURPOSE: The aim is to describe the main characteristics of an anterior/posterior segment surgery and how to resolve intraoperative complications. Setting/Venue: The anterior and posterior segment surgical video was created at the Department of Ophthalmology, Virgin Macarena University Hospital, Seville, Spain. METHODS: We present the case of a male with Stevens-Johnson syndrome and severe limbal deficiency who needed a Boston type 1 keratoprosthesis, reaching a visual acuity of 0.4 (0.05 before surgery). In the course of follow-up, he developed corneal melting with perforation, immune vitritis, and a large epimacular membrane. We decided to perform a 23-gauge vitrectomy associated with keratoprosthesis exchange. As a consequence of inappropriate anesthesia, the patient woke up during the surgery, provoking a retinal tear besides a choroidal detachment. These damages needed endolaser photocoagulation as well as silicone oil tamponade, forcing us to postpone the exchange. An intravitreal dexamethasone implant was also injected. Two months later, the silicone oil was removed, and the Boston keratoprosthesis was replaced by a new type 1 model with a titanium back plate, which likely improves biocompatibility and retention and may reduce complications such as retroprosthetic membranes and stromal corneal melts. RESULTS: Good anatomical results were achieved, and visual acuity slightly improved to 0.2. CONCLUSIONS: Combined anterior and posterior segment surgery represents a great challenge that can improve not only visual acuity but also quality of life in patients with severe diseases such as Stevens-Johnson syndrome.

Diagnostic Delay and Amniotic Membrane Transplantation in a Child With Atypical Stevens-Johnson Syndrome.

A 4-year-old boy presented with a 6-day history of severe non-limbic-sparing conjunctivitis. Atypical Stevens-Johnson syndrome with a possible cause of Mycoplasma pneumoniae was suspected as the precipitant of the clinical symptoms. The patient recovered with amniotic membrane transplantation and intravenous immunoglobulin therapy despite an initial delay in diagnosis.

Root dentin anomaly and a PLG mutation.

We report a Thai girl affected with plasminogen deficiency, Type I. Ligneous conjunctivitis was first observed when she was one-month-old. The newly recognized findings include tapered incisor roots as a result of thin root dentin, generalized short tooth roots, and mandibular prognathism. Mutation analysis of PLG demonstrated homozygous c.1193G>A missense mutation. The parents were heterozygous for c.1193G>A mutation. The c.1193G>A mutation is novel and predicted to cause amino acid substitution p.Cys398Tyr. Thin root dentin in the patient who was affected with PLG mutation and immunolocalization of Plg during early root development in mice imply the role of plasminogen in root dentin formation.

[Conjunctival rhinosporidiois diagnosed in a biopsy specimen]. / Rinosporidiosis conjuntival diagnosticada mediante estudio histopatológico.

11 years old girl, from south region of Chile, without history of travels outside Chile nor the province, complaints of red eye with blepharitis and blood-tingued epiphora. Eye exam revealed a pseudomembrane. Clinical diagnosis was folicular conjunctivitis. A surgical removal was performed and the lesion sent to biopsy analysis. On microscopic examination numerous 50-150 microm cysts with keratinous wall and numerous endospores were found. Rinosporidiosis is an infection caused by Rhinosporidium seeberi that frecuently affects nasal cavity but could infect eye, urogenital tract and airways. This infections is considered endemic in Asia and Africa, but it is very important to have the suspicious among polyps in these areas because travel to endemic areas is become more frecuently.