Cases reported "Scleral Diseases"

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1/160. Peripheral lamellar keratoplasty for corneoscleral cyst: three case reports.

    PURPOSE: To examine whether peripheral lamellar keratoplasty (LKP) using preserved cornea was effective for the treatment of corneoscleral cysts. methods: Three patients with corneoscleral cysts underwent peripheral lamellar keratoplasty. Two patients had no history of trauma or ocular surgery and were considered to have congenital cysts. The other patient had a history of strabismus surgery that had been performed 7 years previously. The anterior wall of the cysts was removed by trephination, and the epithelial membrane lining the posterior wall was peeled off. Lamellar corneal buttons obtained from preserved corneas then were put in place and secured with 8-10 interrupted sutures. In one case, because the cyst was large and extended to the pupillary axis, peripheral LKP was performed for removal of the scleral and peripheral corneal cyst, and the inner wall of the central corneal cyst was removed with vigorous irrigation and a spatula. RESULTS: Histologic examination showed that all of the cysts were lined with nonkeratinizing epithelial cells. In all three cases, cysts have not reformed after a 1-5-year follow-up. CONCLUSIONS: The cysts were lined in epithelial cells, and removal of these epithelial cells was considered to be important for the prevention of recurrence. Peripheral LKP is effective for the treatment of corneoscleral cysts, since this procedure removes displaced epithelial cells and reconstructs the thin part of the cornea and sclera. ( info)

2/160. Gingival fibromatosis combined with cherubism and psychomotor retardation: a rare syndrome.

    Gingival fibromatosis is frequently an isolated condition, but rarely associated with some uncommon syndromes. This paper describes an 11-year-old patient with pronounced gingival enlargement, cherubic facial appearance, and psychomotor retardation and discusses the major aspects of the case. The most striking finding orally was the presence of grossly hyperplastic gingiva, which completely covered all teeth except the occlusal surfaces of some teeth. The swelling in the lower part of the face and the appearance of sclera beneath the iris suggest cherubism. The diagnosis was confirmed by the detection of giant cell regenerative granuloma and perivascular eosinophilic particles and osteoclasts after biopsy of the mandible. In this case, surgery was the only effective way to treat the patient. A full-mouth gingivectomy procedure was performed under general anesthesia in 2 stages. The case was followed for 12 months and no recurrence was seen. An appropriate oral hygiene regimen was established. ( info)

3/160. Bilateral periopticointrascleral hemorrhages associated with traumatic child abuse.

    PURPOSE: To report a case of bilateral periopticoscleral hemorrhages associated with traumatic child abuse. methods: Postmortem gross examination and histopathologic studies of both eyes and the optic nerves of a 6-month-old infant who died from subdural hematoma. RESULTS: Gross examination and histopathologic step sections disclosed bilateral intrascleral hemorrhages around both optic nerves. In addition, bilateral diffuse multilayered retinal, vitreous, and sublaminar (beneath the internal limiting membrane) hemorrhages were present. CONCLUSION: Periopticointrascleral hemorrhages are characteristic of blunt head trauma and may constitute important forensic evidence in cases of suspected child abuse. ( info)

4/160. Scleromalacia following trabeculectomy with intraoperative mitomycin C.

    PURPOSE: To report the development of scleromalacia in pediatric age as a complication of trabeculectomy with the adjunctive use of intra-operative mitomycin C. patients: Reported are 2 patients who developed scleromalacia following trabeculectomy combined with intra-operative 0.4 mg/ml mitomycin C application for 5 minutes for refractory glaucoma in aphakic bullous keratopathy and penetrating keratoplasty. RESULTS: Scleromalacia localised in the surgical area developed in 2 young patients. Scleromalacia remained stable in both patients after 6 and 24 months follow-ups. CONCLUSIONS: It is suggested that this procedure be done more selectively, especially in young patients, with a shorter application time and using a lower concentration. ( info)

5/160. Cyanoacrylate tissue adhesive augmented tenoplasty: a new surgical procedure for bilateral severe chemical eye burns.

    PURPOSE: To report on cyanoacrylate tissue adhesive augmented tenoplasty, a new surgical procedure for bilateral severe chemical eye injuries. methods: A 26-year-old man presented with bilateral severe (grade IV) chemical burns involving the eye, periorbital tissues, face, and neck. Despite adequate medical therapy, corneal, limbal, and scleral ulceration progressed in both eyes. Secondary pseudomonas keratitis necessitated therapeutic penetrating keratoplasty in the right eye. Tenoplasty and glued-on rigid gas permeable contact lens were unsuccessful to arrest progression of corneolimboscleral ulceration in the left eye. We applied n-butyl cyanoacrylate tissue adhesive directly on the ulcerating corneal, limbal, and scleral surface to augment tenoplasty. RESULTS: The left ocular surface healed with resultant massive fibrous tissue proliferation and symblepharon on the nasal side. Ocular surface rehabilitation resulted in a vascularized leukomatous corneal opacity with upper temporal clear cornea. The patient achieved visual acuity of 6/36 in the left eye. CONCLUSION: We suggest that cyanoacrylate tissue adhesive-augmented tenoplasty can be undertaken to preserve ocular integrity and retain visual potential in a severe chemical eye injury. ( info)

6/160. Ocular ochronosis in alkaptonuria patients carrying mutations in the homogentisate 1,2-dioxygenase gene.

    AIMS: To assess the involvement of the recently identified human homogentisate 1,2-dioxygenase gene (HGO) in alkaptonuria (AKU) in two unrelated patients with ochronosis of the conjunctiva, sclera, and cornea. methods: A mutation screen of the entire coding region of the HGO gene was performed using single stranded conformational analysis after polymerase chain reaction with oligonucleotide primers flanking all 14 exons of the HGO gene. Fragments showing aberrant mobility were directly sequenced. RESULTS: Two homozygous missense mutations, L25P and M368V, were identified, each of which leads to the replacement of a highly conserved amino acid in the HGO protein. CONCLUSIONS: The authors describe a novel mutation, L25P, in the German population and bring to 18 the total number of known HGO mutations. ( info)

7/160. Repair of scleral perforation with preserved scleral and amniotic membrane in Marfan's syndrome.

    To describe the surgical technique used in the repair of a large scleral perforation in a patient with Marfan's syndrome and a past history of various surgical interventions in both eyes. Scleral homograft and amniotic membrane transplant were used to reconstruct the large scleral defect present in his left eye. One month after surgical intervention, the patient showed excellent restoration of the scleral perforation without signs of inflammation or infection. The combination of scleral homograft and amniotic membrane transplant constitute an effective alternative to autologous scleral and conjunctival grafts when these cannot be used. ( info)

8/160. Posterior scleral choristoma in the organoid nevus syndrome (linear nevus sebaceus of Jadassohn).

    PURPOSE: To highlight the association of posterior osseous and/or cartilaginous ocular choristomas with epibulbar choristomas and the nevus sebaceus of Jadassohn. DESIGN: Small case series. PARTICIPANTS: Four patients with the organoid nevus syndrome. methods: Clinical and histopathologic studies in four patients with epibulbar lesions and nevus sebaceus of Jadassohn. MAIN OUTCOME MEASURES: Ophthalmoscopic findings of peripapillary lesions. Computed tomographic and ultrasonographic characteristic of posterior scleral lesions. Ocular histopathologic findings in one globe from one of the study subjects. RESULTS: Three patients had the triad of posterior osseous/cartilaginous ocular choristomas, anterior epibulbar choristomas, and nevus sebaceus of Jadassohn and one patient had anterior epibulbar choristomas and posterior osseous/cartilaginous ocular choristomas. ultrasonography and computed tomography were valuable in detecting scleral ossification or epibulbar cartilage or both. The ophthalmoscopic findings were similar to those of a choroidal osteoma. CONCLUSIONS: The presence of posterior osseous/cartilaginous ocular choristomas in a patient with epilepsy or epibulbar lesions or both suggests the diagnosis of nevus sebaceus of Jadassohn. Osseous/cartilaginous ocular choristomas should be suspected in patients with nevus sebaceus of Jadassohn and peripapillary hypopigmented fundus lesions. ( info)

9/160. Sclerochoroidal calcification associated with gitelman syndrome.

    PURPOSE: To investigate sclerochoroidal calcification in a patient with Gitelman syndrome. METHOD: Case report. Bilateral fundus abnormalities observed in a 58-year-old woman were documented with fluorescein angiography and tomodensitometry. RESULTS: Symmetric yellow-white subretinal lesions were observed in the superotemporal midperiphery of the fundus of each eye. Tomodensitometry examination was consistent with calcium deposition. The medical history included gitelman syndrome. Sclerochoroidal calcification probably resulted from the severe hypomagnesemia. CONCLUSION: gitelman syndrome may be a cause of sclerochoroidal calcification. ( info)

10/160. Conjunctival rhinosporidiosis associated with scleral melting and staphyloma formation: diagnosis and management.

    PURPOSE: Although conjunctival rhinosporidiosis is endemic in india, associated scleral melting and staphyloma formation are quite rare. We report clinical features and management in three patients with this unusual presentation. methods: Retrospective review of case records of three patients with conjunctival rhinosporidiosis. RESULTS: infection occurred in young, healthy adults and was localized to the forniceal conjunctiva in all patients. The affected conjunctiva had numerous grey-white spherules, but a polyp-like lesion was not present in any patient. diagnosis was based on clinical features and examination of scrapings from the involved conjunctiva. Treatment was surgical, with excision of infected conjunctival tissues and staphyloma repair with homologous sclera or autogenous periosteum. Failure to recognize the conjunctival pathology in one patient resulted in recurrence of the staphyloma. CONCLUSION: Conjunctival rhinosporidiosis can be associated with scleral staphyloma in young, healthy, adults. Differentiating this entity from idiopathic scleral ectasia requires knowledge of the clinical features of conjunctival rhinosporidiosis and a high index of clinical suspicion. ( info)
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