1/3. 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 = 1keywords = anaesthesia (Clic here for more details about this article) |
2/3. Unilateral hypoplasia of the trigeminal ganglion.CASE REPORT: We report a case of unilateral anaesthesia of the V1 (ophthalmic) division of the trigeminal cranial nerve presenting with persistent corneal erosions and ulceration secondary to trigeminal ganglion hypoplasia. The patient had a lifelong history of unexplained left-sided ophthalmic symptoms for which numerous diagnoses were provided. Cranial nerve testing demonstrated partial trigeminal dysfunction on the left side. Further investigation eliminated viral etiologies, and subsequent magnetic resonance imaging determined that the patient had a hypo-plastic left trigeminal ganglion. COMMENTS: We present the case to alert clinicians to the possibility of this rare condition.- - - - - - - - - - ranking = 0.16666666666667keywords = anaesthesia (Clic here for more details about this article) |
3/3. 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 = 1.1666666666667keywords = anaesthesia (Clic here for more details about this article) |