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Reported is an atypical, severe case of Hallermann-Streiff syndrome combined with progeria, bilateral microphthalmus, cataracts, and normal chromosome count. A plea is made for study into the teratology of abnormal chemical, metabolic, and other forces that attack the early stages of the development of the human fetus and produce multiple anomalies.
The epicanthal fold is a semilunar flap of eyelid skin on the medial aspect of the eye. Many surgical techniques have been described to eliminate the epicanthal fold, which is a feature uniquely predominant among the Asian population. However, many surgeons avoid epicanthoplasty because of the fear of visible scar formation. From January of 2002 to April of 2004, 118 patients with medial epicanthal folds underwent epicanthoplasty using a periciliary Y-V advancement procedure for the prevention of visible scar formation. The mean age of the patients was 25.2 years (range, 16 years to 56 years). The median follow-up period was 6 months (range, 2 weeks to 2 years). We performed periciliary Y-V epicanthoplasty only when a patient had wide interepicanthal distance (over 32 mm) or a completely eclipsed lacrimal caruncle. Most of the patients attained satisfactory results, and there were few complications in our cases. Scarring of the medial canthal area has not been a problem with this technique because we designed incisions along the eyelashes and skin-mucosal junctions. We have found that our periciliary Y-V epicanthoplasty is a very reliable and effective method for eliminating the epicanthal fold with minimal scar formation.
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Oculoplastic procedures well within the capabilities of most ophthalmic surgeons are described. Only those conditions most commonly encountered are discussed and the corrective surgery is illustrated. If required, all procedures described may be carried out under local anaesthesia in a minor operations room. The operative techniques described are those used by the author.
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A young girl with ocular albinism and the Hermansky-Pudlak syndrome is described. Ocular albinism generally occurs in males. In this condition, the pigmentation of the skin and hair is nearly normal, and the melanin pigment abnormality is limited to the eyeballs. The chief complaints are visual disturbance, nystagmus, and photophobia. A 3-year-old girl was recently brought to our hospital with nystagmus, which she had exhibited since the age of 1 year. Funduscopy resulted in a diagnosis of ocular albinism. Further investigations, specifically, microscopy of her platelets, led us to conclude that she had Hermansky-Pudlak syndrome.
AIMS: To determine the characteristics of trichiasis patients presenting for surgery in Wolayta Zone of Ethiopia. METHODS: Patients referred for trichiasis surgery by community health agents were evaluated by trained integrated eye care workers (IECWs) for the presence of trichiasis, locations of inturned lashes, severity of trichiasis, corneal opacity, and visual acuity. RESULTS: 1635 individuals with trichiasis presented for surgery. 82% had bilateral trichiasis; 91% of patients reported trichiasis duration of >2 years. Epilation was practised by over three fourths of the study subjects. A high proportion of patients tested positive for ocular Chlamydia trachomatis at presentation. 17% had monocular blindness and 8% were binocularly blind. Corneal opacity was highly associated with the trichiasis duration and severity and visual loss was associated with corneal opacity. CONCLUSION: Severe trichiasis reflects the magnitude of the trachoma problem in Ethiopia. Visual impairment due to trichiasis is highly associated with disease severity and duration. Early intervention to correct trichiasis before it become severe is recommended to prevent visual impairment.
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AIMS/BACKGROUND: This paper reports on the findings of a cost and effectiveness study of the trachoma control programme (TCP) in Burma. The TCP began in 1964 employing non-surgical interventions (community education and mass treatment with topical antibiotics) and surgical correction of trichiasis. METHODS: Fixed and variable costs of the TCP are assessed over 30 years (1964-93) and apportioned to either surgical or non-surgical interventions. The change in the prevalence of trachoma blindness during this period is used to calculate cases of visual impairment prevented by the TCP. The years of life saved because of premature mortality averted and from living in a handicapped state are added to yield a single measure of utility called handicap adjusted life years (HALYs). RESULTS: The cost effectiveness of the TCP is $54 per case of visual impairment prevented: $193 and $47 for surgical and non-surgical interventions respectively. The cost utility of the TCP is $4 per HALY averted: $10 and $3 for surgical and non-surgical interventions respectively. Results are highly sensitive to the 1965 prevalence of blindness, the choice of discount rate, and the effectiveness of both interventions. CONCLUSIONS: Thirty years of trachoma control in Burma are associated with a remarkable decline in trachomatous blindness. Both surgical and non-surgical interventions are cost effective means of preventing trachomatous visual impairment. Discussion focuses on methodological limitations and implications for research and policy.
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