Albinism is the term applied to a heterogeneous group of genetically determined disorders characterized by hypopigmentation and affecting the eyes. After describing the clinical features of albinism in general, the authors discuss the various forms of oculocutaneous albinism, ocular albinism, and albinoidism that are of interest to the ophthalmologist. Emphasis is placed on the ocular features of each form. The visual pathway abnormalities and the clinical management of albinism are discussed.
Anterior capsular contraction is commonly observed following capsulorhexis in phacoemulsification surgery. This study demonstrates that the maximum rate of contraction occurs in the first 6 weeks following surgery and is more pronounced with silicone lens implants.
Postgrad Med J 2003;79:531-532 Retrobulbar haemorrhage is a rare and potentially sight threatening complication after peribulbar local anaesthetic injection for cataract surgery. A case is reported of a woman who developed this complication and was subsequently found to have been taking Gingko biloba extract tablets which may have predisposed her to developing the haemorrhage.A 65 year old woman was admitted for routine phacoemulsification and intraocular lens implantation to her left eye. She had no past medical history and denied taking any medications. Her preoperative blood pressure was 135/70 mm Hg and preoperative visual acuity in her left eye was 6/18 unaided. For local anaesthesia, a 25 gauge needle of 25 mm length containing 5 ml 2% lignocaine and 5 ml 0.75% bupivicaine was inserted in the inferotemporal region of her left orbit. Immediately after injection of 5 ml of the local anaesthetic mixture, there was sudden proptosis and bruising of her lower lid which extended medially and superiorly. The patient complained of pain in her left eye and reduction of vision. Her eyelids were tense and her ocular movements restricted. A diagnosis of acute retrobulbar haemorrhage was made and urgent lateral canthotomy and inferior cantholysis was performed to decompress the orbit.She was re-examined two hours later and no longer had any proptosis or restriction of ocular movement. The visual acuity in her left eye was unchanged at 6/18 unaided, the intraocular pressure in the left eye was 16 mm Hg, and her left optic disc and fundus appeared healthy with no evidence of any vascular occlusion. Further detailed questioning revealed that she had been taking Gingko biloba extract tablets 40 mg three times a day for the past two years. Haematological investigations showed a normal blood count, prothrombin and partial thromboplastin times. Serum urea, creatinine, and liver function tests were normal.
Four cases of albinism with haemorrhagic diathesis (Hermansky-Pudlak syndrome) are presented. The cases displayed wide phenotypic variation. Electroretinography was performed on all four patients and was found to be normal. One patient developed a cutaneous malignant melanoma. 104 copyright.
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