先天性双眼特大角膜皮样瘤合并腭裂一例

患者,男,10个月.父母以双眼球向外突出、视力障碍为主诉就诊于我院眼科.检查:视力:双眼均为光感/30cm,上下睑无异常.右眼角膜呈圆球形(又似蘑菇状),向前外突出约12mm,表面布满新生血管,呈粉红色,突出于睑裂外,瘤体占据全部角膜,根部直径约11mm,连于角膜缘一周,角膜缘可见一灰黑色环状带.     

Surgical treatment of large corneal dermoid

Corneal and limbal dermoids are uncommon choristomatous corneal tumors. They clinically present as round or oval, whitish or yellowish cones protruding on the anterior surface of the eyeball. They are composed of ectodermal (keratinized epithelium, hairs, sebaceous and sudoriferous glands, nerves, smooth muscles and, less frequently, teeth) and mesodermal elements (fibrous tissue, fat, blood vessels and cartilage) combined in different proportion. If fat dominates in histology of the tumor, it is called a lipodermoid. A case of a two-year old boy with a large corneal dermoid on the right eye is presented. Dermoid covered almost the whole cornea and was associated with adherence of the atrophic iris to the posterior corneal surface and partial congenital cataract. Surgical therapy included excision of dermoid together with the superficial lamellae of the corneal stroma and penetrating keratoplasty. Satisfactory tectonic and esthetic outcome was achieved and has been maintained for four postoperative years now.     

Familial congenital corneal anaesthesia

Congenital corneal anaesthesia is a cause of severe corneal ulceration and scarring in childhood. Although uncommon, it may be underdiagnosed when present as an isolated entity. Measures such as the use of elbow splints and tarsorrhaphy may be necessary to prevent visual loss.
In rare instances, the condition may be inherited. A family is presented with autosomal dominant isolated congenital corneal anaesthesia, and the systemic associations and treatment of the condition are reviewed.     

角膜皮样瘤的诊治方法及术后复发原因分析

目的 对角膜皮样瘤诊治方法及术后复发原因的初步探讨.方法 回顾性分析43例经病理证实的角膜皮样瘤的临床、病理资料.19例行皮样瘤切除术,2例行皮样瘤切除+结膜遮盖术,1例行皮样瘤切除+羊膜移植术,21例行皮样瘤切除+角膜板层移植术.结果 随访半年～6年,39例治愈,4例单纯行皮样瘤切除术复发,二次手术行板层角膜移植术,随访5年无复发.结论 治疗角膜皮样瘤应根据角膜受累程度采用不同的手术方式,相应部位的角膜皮样瘤切除联合板层角膜移植术可以减少术后复发的几率.     

Perinatally ruptured dermoid cyst presenting as congenital oculomotor palsy

An eight-month-old girl presented with congenital exotropia and latent nystagmus. Further evaluation revealed congenital ptosis of the left eye and restriction of the elevation, depression and adduction of the left eye. A diagnosis of congenital oculomotor palsy was made. At the age of three months she had been examined by the neurologist because of retarded psychomotor development. All laboratory investigations were normal. At the age of eight months, a CT scan of the brain and orbit was found to be normal. The patient was treated for amblyopia. At the age of five, strabismus surgery was performed, and a large fibrous tumor encapsulating the superior and lateral rectus muscle was found. A biopsy was taken and pathology showed fibrous tissue containing a hair. Based on the clinical history, the diagnosis of a perinatally ruptured orbital dermoid cyst was made. Review of the previous CT and an additional CT showed enlargement of the left lateral orbital wall with a notch in the lateral wall, indicative of a dermoid cyst.     

Perinatally ruptured dermoid cyst presenting as congenital oculomotor palsy

An eight-month-old girl presented with congenital exotropia and latent nystagmus. Further evaluation revealed congenital ptosis of the left eye and restriction of the elevation, depression and adduction of the left eye. A diagnosis of congenital oculomotor palsy was made. At the age of three months she had been examined by the neurologist because of retarded psychomotor development. All laboratory investigations were normal. At the age of eight months, a CT scan of the brain and orbit was found to be normal. The patient was treated for amblyopia. At the age of five, strabismus surgery was performed, and a large fibrous tumor encapsulating the superior and lateral rectus muscle was found. A biopsy was taken and pathology showed fibrous tissue containing a hair. Based on the clinical history, the diagnosis of a perinatally ruptured orbital dermoid cyst was made. Review of the previous CT and an additional CT showed enlargement of the left lateral orbital wall with a notch in the lateral wall, indicative of a dermoid cyst.     

Familial congenital corneal anaesthesia.

Congenital corneal anaesthesia is a cause of severe corneal ulceration and scarring in childhood. Although uncommon, it may be underdiagnosed when present as an isolated entity. Measures such as the use of elbow splints and tarsorrhaphy may be necessary to prevent visual loss. In rare instances, the condition may be inherited. A family is presented with autosomal dominant isolated congenital corneal anaesthesia, and the systemic associations and treatment of the condition are reviewed.     

Self-inflicted corneal injuries in children with congenital corneal anaesthesia.

Severe corneal ulceration related to self-inflicted injury in the presence of congenital corneal anaesthesia is described in four boys under 2 1/2 years of age. The ulcers had failed to heal until it was recognised that the children were scratching their own eyes. The application of arm splints allowed rapid healing. Although corneal ulceration is a recognised complication of congenital corneal anaesthesia, this preventable cause of the ulceration has not previously been recognised. In two cases there were isolated recurrences which healed quickly with the reapplication of splints. All four children had good vision initially, and, although there were no overt gross development abnormalities, two had neurological signs on detailed investigation suggesting cerebellar or brain stem malformation and one had unilateral anophthalmos, talipes equinovarus, and patent ductus arteriosus. All the children showed normal intellectual development. Whether the eye scratching behaviour was the primary cause of the ulceration or merely an aggravating factor, the identification of this abnormal behaviour is important in any child with idiopathic corneal ulceration, as even in the presence of congenital corneal anaesthesia the eyes heal quickly with effective splinting of the elbows. It is therefore important to test sensation of the cornea and face and to consider the possibility of self-inflicted injury in children with refractory corneal ulceration, as in our cases there were no other consistent diagnostic features.     

Central corneal dermoid: a clinicopathologic correlation and review of the literature

The authors present a clinicopathologic correlation of a central corneal dermoid removed from a one-year-old child. Contrary to the initial clinical impression, the tumour involved the full thickness of the corneal stroma, necessitating a penetrating keratoplasty. After the graft became opaque, a second penetrating keratoplasty was required. In spite of the fact that the eye has remained amblyopic, its cosmetic appearance is excellent. Problems related to the management of central corneal dermoids are discussed.     

显微镜下瘤体切除联合新鲜羊膜覆盖治疗幼儿角膜皮样瘤

目的观察显微镜下瘤体切除联合新鲜羊膜覆盖术治疗幼儿角膜皮样瘤的手术疗效,探索角膜皮样瘤的临床治疗方法。方法对16例（16只眼）角膜皮样瘤患儿行显微镜下病灶切除联合新鲜羊膜覆盖术,术后随访6～12个月,观察角膜病灶愈合情况。结果随访期内全部患者未见皮样瘤复发,病灶处残留轻度角膜云翳,部分患儿术后视力不同程度提高。结论显微镜下瘤体切除联合新鲜羊膜覆盖术治疗儿童角膜皮样瘤,手术操作简单,不依赖于供体角膜组织,术后疗效较好,适用于儿童角膜皮样瘤的及早治疗。     

Spontaneous regression of congenital corneal opacity

Graefe's Archive for Clinical and Experimental Ophthalmology - To determine the incidence of spontaneous regression of congenital corneal opacity (CCO) and identify clinical factors associated...     

Penetrating keratoplasty in congenital corneal opacities

The clinical course and histopathological findings in 17 cases of congenital corneal opacity treated by penetrating keratoplasty are described. On the basis of the histopathological findings 9 of the cases of corneal opacity were diagnosed as Peter's anomaly, 5 cases as sclerocornea or mesenchymal dysgenesis, 2 as choristoma, and 1 case as leukoma. Only 2 of the corneal grafts performed in these infants remained clear. The underlying disease in the 2 cases where surgery was successful was Peter's anomaly, with a clear corneal periphery. From the analysis of the corneal buttons the authors deduce that graft failure in most such cases is due to severe structural changes of the host cornea. Three findings represented consistently unfavorable signs: (1) absence of Bowman's layer; (2) absence of Descemet's membrane and (3) corneal vascularization at the cut edge of the button. Immunoreaction or infection of the graft are rare causes of graft failure. Despite the extremely poor prognosis of bilateral congenital corneal opacities, penetrating keratoplasty should be attempted. The histopathological findings may be used as prognostic criteria for graft survival or the extent of anterior segment dysgenesis.     

A case of congenital corneal keloid

PURPOSE: To describe a case of unilateral comeal keloid and present the clinical and histopathological findings and the management. METHODS: A 23-year-old Asian male patient was examined for a white spot on the left cornea that had been present since birth. On biomicroscopic examination, a well-demarcated vascularized comeal mass was found located nasal to the center. The pupil was displaced superiorly, and gonioscopic examination showed peripheral iridocomeal adhesion at 12 o'clock. The patient underwent penetrating keratoplasty. RESULTS: Histopathologic study showed a variously thickened epithelial layer, an absence of Bowman's layer, subepithelial fibrovascular hyperplasia, and an absence of dermal elements. These histopathologic findings suggested a congenital comeal keloid. The central graft comea remained clear at 18 months after surgery and the patient was satisfied with the result. CONCLUSIONS: Penetrating keratoplasty may be an effective surgical option for congenital keloids in young adult patients.     

Central corneal thickness in congenital glaucoma

PURPOSE: The aim of this study was to compare central corneal thickness between eyes with congenital glaucoma and normal fellow eyes in unilateral glaucoma or less affected fellow eyes in bilateral glaucoma. METHODS: Eyes of consecutive phakic children with congenital glaucoma and previous glaucoma surgery were examined under chloral hydrate. Complete ophthalmologic examination, central corneal thickness (CCT), axial length, and corneal diameter measurements were performed. Patients were included in the study if presented with intraocular pressure (IOP) less than 21 mm Hg and no biomicroscopic signs of corneal edema. RESULTS: Nine patients were included in the study. The mean CCT in the more affected eye/glaucomatous eye was 522.3 +/- 65.2 microm and in the less affected eye/healthy eye was 579.7 +/- 44.5 microm. This difference was statistically significant (P = 0.0013). CONCLUSION: CCT was significantly thinner in glaucomatous eyes than in normal fellow eyes in phakic children with congenital glaucoma. This finding may be another confounding factor when measuring IOP in these patients.     

Unilateral congenital corneal staphyloma with retinal neovascularization

I present the light microscopic and ultrastructural findings in an unusual case of unilateral congenital corneal staphyloma. The anterior segment exhibited features of extensive Peters' anomaly whereas the posterior segment showed marked preretinal neovascularization and lack of axons as well as outer segments. These retinal changes might be secondary due to raised intraocular pressure and traction phenomena, but the complexity and extent of the described malformation argue against this.Presented at the 29th Annual Meeting of the European Ophthalmic Pathology Society, Coimbra, Portugal, June 1990