Wagih Aclimandos

Ocular ultrasound in Alagille syndrome a new sign

BACKGROUND Alagille syndrome (AS) is one of six forms of familial intrahepatic cholestasis, all of which present with neonatal jaundice and paucity of intrahepatic bile ducts. Differentiation of these individual syndromes is crucial as their treatments and prognoses vary. It is the ophthalmic features, posterior embryotoxon on particular, that distinguish AS. METHODS The authors performed full ocular examination, including A- and B-scan ultrasound, refraction, and, where possible, fluorescein angiography in 20 unrelated children with AS and 8 with non-AS-related cholestasis. RESULTS There was ultrasound evidence of optic disc drusen in at least one eye in 95% and bilateral disc drusen in 80% of patients with AS but in none of the patients who were non-AS at the time of examination. Independent review of hard-copy scans suggested drusen in at least one eye in 90% of the cases and bilateral drusen in 50%, although this latter figure rose to 65% on review of the angiograms. This is markedly higher than the incidence in the normal population (0.3%-2%). Axial lengths were shorter than expected for the older age group (older than 10 years of age), but this was not associated with gross ametropia. CONCLUSION This strong association of AS and optic disc drusen has not been reported previously and represents not only the first significant association between a systemic condition and disc drusen but also a possibly useful tool in the diagnosis of AS, especially in young children.

Sickle retinopathy in patients with sickle trait

Sickle trait is traditionally considered a benign condition by ophthalmologists. Three cases of sickle retinopathy in subjects with sickle trait are reported. In all cases the onset of retinopathy was related to other contributing factors: in one case a traumatic hyphaema and raised intraocular pressure, in two others diabetes mellitus. Patients with sickle trait are at risk of retinopathy if coincident ocular or systemic disease is present.

Screening for ophthalmic manifestations of sickle cell disease in the United Kingdom

There are marked variations in the manifestations of sickle disease in different populations. The ocular complications of this condition amongst the Afro-Caribbeans living in the United Kingdom have not previously been reported. We present the preliminary results of an ophthalmic screening programme at Kings College Hospital, London. One hundred eyes of 50 patients with sickle cell disease were assessed. Full ocular examination was performed including fundus fluorescein angiography. We have looked at the haematological and clinical profile of the patients involved as well as the number of days spent in hospital during the year preceding the eye examination. The incidence of grade II retinopathy was found to be significantly higher than grade I in SC disease. This concurs with the results of the Jamaican screening and confirms that these patients are at higher risk of visual impairment than those with SS disease. Our results also agree with the Jamaican experience which suggest that visual morbidity is mostly due to complications of proliferative sickle retinopathy (PSR). However, the findings in patients without proliferative changes are different; in particular, angioid streaks leading to disciforms are an important cause of visual loss in Jamaica, but were not seen in any of the 98 eyes examined in this study. No correlation was found between the grade of retinopathy and age, sex, systemic complications and various haematological parameters except for the percentage of haemoglobin F, which was significantly higher in patients with grade I (7.6) compared with grade II (4.2) retinopathy (p = 0.0127).

Five-Decade Profile of Women in Leadership Positions at Ophthalmic Publications

OBJECTIVE To evaluate the balance between the sexes of published ophthalmic material at the editorial, reviewer, and author levels. DESIGN Cross-sectional study of 3 journals, American Journal of Ophthalmology, Archives of Ophthalmology, and Ophthalmology, for 1969, 1979, 1989, 1999, and 2009. The data were compared with ophthalmologist-in-training and physician profile in major contributing states from North America and Europe during the same period. RESULTS Of the 3 major ophthalmology journals, none had a female editor-in-chief. For all journals, the proportion of editorial board members who were women increased from 3.3% in 1969 to 18.8% in 2009. For all journals and all years, women composed a higher proportion of first authors (29.2% in 2009) compared with senior authors (22.9% in 2009), reviewers (18.9% in 2009), or assistant editors (12.5% in 2009). There was an abrupt shift toward women after 1989 in first authorship in Ophthalmology (1969, 4.6%; 1979, 5.4%; 1989, 12.3%; and 1999, 20.2%), Archives of Ophthalmology (1969, 6.6%; 1979, 5.1%; 1989, 15.6%; and 1999, 28.6%), and American Journal of Ophthalmology (1969, 5.6%; 1979, 4.2%; 1989, 9.2%; and 1999, 23.9%). There was also an abrupt increase in female senior authorship for American Journal of Ophthalmology after 1989 (1979, 8.5%; 1989, 8.1%; and 1999, 18.3%). The increase in female first authorship during the 5 decades was parallel with the increase in US female physicians. CONCLUSIONS Women ophthalmologists are authoring publications in increasing numbers that match their prevalence in the academic and overall workforce. However, all editors are men. This discrepancy relates to the relatively younger generation of female ophthalmologists or selection bias, a subject that requires further investigation.

Retinopathy in haemoglobin C trait

Retinopathy associated with sickle-C and sickle cell disease is well described. Sickle trait and haemoglobin C trait are generally considered benign conditions, with infrequent systemic manifestations. Rare cases of retinopathy in sickle trait, in the presence of contributory factors, exist and we recently reported three such patients. The occurrence of retinopathy in haemoglobin C trait is even less well documented. Haemoglobin C does not cause red blood cell sickling but is known to decrease erythrocyte plasticity and increase blood viscosity. We report three cases in which haemoglobin C trait was associated with significant peripheral vascular occlusion and seafan formation (confirmed by fluorescein angiography) similar to that seen in sickle retinopathy. Two patients had coexistent systemic disease (hypertension and diabetes mellitus). Vitreous haemorrhage was the presenting feature in two patients. It is evident that haemoglobin C trait may be associated with sight-threatening complications.

History and future of the European Board of Ophthalmology Diploma examination

Purpose:  The European Board of Ophthalmology Diploma (EBOD) examination has evolved over the last few years, especially with the introduction of negative marking (−0.5 points) for incorrect or blank answers (0 points for don’t know option), which aimed to improve the quality and reliability of the examination.

The effect of surgery on the AC/A ratio

The accommodative convergence/accommodation (AC/A) ratio is used clinically as a diagnostic tool in the assessment of convergence excess and near esotropia and the differentiation of true and simulated divergence excess. An abnormally high AC/A ratio has been implicated in the aetiology of some forms of strabismus but the evidence for alteration in the ratio following surgery is inconclusive. In a prospective study of 38 patients the effect of routine squint surgery was investigated in concomitant strabismus. The AC/A ratio was found to decrease significantly after surgery in both esotropes and exotropes. Furthermore a trend was demonstrated which suggested an increased risk of overcorrection in esotropes with a high (≥7:1) pre-operative AC/A ratio.

Corneal perforation as a complication of epidermolysis bullosa acquisita

Epidermolysis bullosa acquisita (EBA) is now recognised as a histopathologically distinct condition.1-3 Ocular complications of hereditary epidermolysis bullosa (EB) have been well documented,4 but little has been reported with respect to the ocular manifestations associated with the acquired form.5-8A patient with EBA and sarcoidosis - an association that does not appear to have been previously reported - developed spontaneous peripheral corneal melting and perforation. The defect healed with the use of a bandage contact lens, antibiotics, mydriatics and pulsed intravenous steroids, and resulted in a satisfactory visual outcome.

Evaluation of adding item-response theory analysis for evaluation of the European Board of Ophthalmology Diploma examination.

Purpose:  To investigate whether introduction of item‐response theory (IRT) analysis, in parallel to the ‘traditional’ statistical analysis methods available for performance evaluation of multiple T/F items as used in the European Board of Ophthalmology Diploma (EBOD) examination, has proved beneficial, and secondly, to study whether the overall assessment performance of the current written part of EBOD is sufficiently high (KR‐20 ≥ 0.90) to be kept as examination format in future EBOD editions.

Procedural aspects of the organization of the comprehensive European Board of Ophthalmology Diploma examination.

The comprehensive European Board of Ophthalmology Diploma (EBOD) examination is one of 38 European medical specialty examinations. This review aims at disclosing the specific procedures and content of the EBOD examination. It is a descriptive study summarizing the present organization of the EBOD examination. It is the 3rd largest European postgraduate medical assessment after anaesthesiology and cardiology. The master language is English for the Part 1 written test (knowledge test with 52 modified type X multiple-choice questions) (in the past the written test was also available in French and German). Ophthalmology training of minimum 4 years in a full or associated European Union of Medical Specialists (UEMS) member state is a prerequisite. Problem-solving skills are tested in the Part 2 oral assessment, which is a viva of 4 subjects conducted in English with support for native language whenever feasible. The comprehensive EBOD examination is one of the leading examinations organized by UEMS European Boards or Specialist Sections from the point of number of examinees, item banking, and item contents.