1/4. Congenital sensory neuropathy. Ophthalmological implications.The authors examined a patient presenting with congenital sensory neuropathy with selective loss of small myelinated nerve fibres. The appearance of (bilaterial) keratitis or corneal ulceration in early childhood is strongly suggestive of congenital corneal anaesthesia. Concomitant symptoms such as anisocoria, abnormal pupillary reaction, diminished tear production and disturbed sensibility to pain and temperature point to a generalized disease: one of the hereditary sensory and autonomic neuropathies. In order to establish a definite diagnosis, elaborate neurological examination, including ultrastructural study of a muscle-nerve biopsy, is required. Tarsorrhaphy, therapeutic flushfitting PMMA scleral lenses and hydrophilic HEMA contact lenses are advocated, in order to protect the cornea. The results with high-water-content hydrophilic contact lenses are promising, those of keratoplasty limited.- - - - - - - - - - ranking = 1keywords = anaesthesia (Clic here for more details about this article) |
2/4. 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.- - - - - - - - - - ranking = 6keywords = anaesthesia (Clic here for more details about this article) |
3/4. 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.- - - - - - - - - - ranking = 7keywords = anaesthesia (Clic here for more details about this article) |
4/4. Presumed ophthalmic herpes zoster after contralateral cataract extraction.herpes zoster ophthalmicus (HZO) may occur spontaneously, but can be precipitated by stress, trauma, debility or systemic illness. A case is here reported of Herpes zoster involving the contralateral eye, associated with a midline skin rash, following cataract extraction under local anaesthesia.- - - - - - - - - - ranking = 1keywords = anaesthesia (Clic here for more details about this article) |