1/4. Anaesthesia for sturge-weber syndrome.A 6-month-old boy with sturge-weber syndrome was scheduled for congenital glaucoma and left buphthalmus surgery. physical examination revealed haemangioma throughout the right trigeminal nerve, congenital glaucoma, left megalocornea and bilateral buphthalmus. Examination of the eye was performed under general anaesthesia, was followed 2 days later by trabeculotomy. No premedication was given to the patient. After induction of anaesthesia with halothane, O2 and N2O muscle relaxation was achieved with atracurium and he was intubated gently. No difference was observed in vital signs during surgery. At the end of the operation he was given oxygen 100% and extubated, muscle relaxant reversal was with atropine and neostigmine. No complication was observed in the post-operative period.- - - - - - - - - - ranking = 1keywords = anaesthesia (Clic here for more details about this article) |
2/4. Megalocornea and persistent hyperplastic primary vitreous masquerading as congenital glaucoma.A 7-month-old infant referred with suspected bilateral congenital glaucoma was noted under general anaesthesia to have simple megalocornea in association with contralateral glaucoma in an eye suspected of harbouring an intraocular tumour. Following enucleation, histopathology demonstrated persistent hyperplastic primary vitreous. The presence of simple megalocornea of autosomal recessive inheritance in association with contralateral persistent hyperplastic primary vitreous in a buphthalmic eye is of ophthalmic interest, and appears to be unique.- - - - - - - - - - ranking = 0.5keywords = anaesthesia (Clic here for more details about this article) |
3/4. Anterior segment dysgenesis in mosaic turner syndrome.AIMS/BACKGROUND: Females with turner syndrome commonly exhibit ophthalmological abnormalities, although there is little information in the literature documenting findings specific to turner syndrome mosaics. Ophthalmic findings are described in four patients with mosaic turner syndrome. All had anterior chamber abnormalities and all four had karyotypic abnormalities with a 45, X cell line. The possible relation between the karyotypic and the phenotypic findings in these patients is discussed. methods: Four girls with mosaic turner syndrome underwent a full ophthalmological assessment, including examination under anaesthesia where indicated. RESULTS: Three of the four patients presented with congenital glaucoma. Two had the karyotype 45, X/46, X, idic(Y) and one a 45, X/47, XXX karyotype. The remaining child had a Rieger malformation of the iris and the karyotype 45, X/46, X, r(X). CONCLUSIONS: These findings suggest that turner syndrome mosaicism (where there are two abnormal cell lines) is associated with anterior segment dysgenesis. The findings in these four patients are compared with those seen in other mosaic phenotypes and it is postulated that the presence of two or more genetically different cell lines may have an adverse effect on anterior segment development.- - - - - - - - - - ranking = 0.5keywords = anaesthesia (Clic here for more details about this article) |
4/4. Suture supported P C IOL in a homocystinuric child.A homocystinuric child presented with a secondary pupillary block glaucoma due to anteriorly subluxated lens. After removal of the subluxated lens, a suture supported posterior chamber IOL was implanted. Postoperative complication of cerebral venous thrombosis following general anaesthesia was managed with high doses of pyridoxine special diet and drugs.- - - - - - - - - - ranking = 0.5keywords = anaesthesia (Clic here for more details about this article) |