Training in cataract surgery in Spain: analysis of the results of a survey of the European Board of Ophthalmology in a Spanish cohort.

INTRODUCTION: A survey conducted by the European Board of Ophthalmology (EBO) revealed significant differences in the surgical training of the ophthalmology residents in Europe, including a disparity between the sexes and a variation in the experience on cataract surgery (CC) between them. This study is about the Spanish sub-cohort of the survey, and its objective is to present and analyse the peculiarities of ophthalmology training in Spain within the European context, as well as discussing ways to harmonise and improve that training throughout the EU. METHODS: We analyse data of the Spanish participants in the EBO exams, defining subgroups by the Autonomous Communities existing in Spain. RESULTS: 93 of 135 requested participants (68.9%) responded. A 60.2% passed the EBO exam between 2021 and 2022, being mostly women (65.59%) aged 31 years old on average. The 91.4% were right-handed, coming from 13 of the 17 Spanish autonomous communities, although mostly from the Community of Valencia, Madrid and Catalonia. Respectively, 16.1%, 3.2% and 8.7% of the respondents said they have completed 10 or more training sessions on animal eyes, synthetic eyes and through the virtual reality simulator. This training was correlated with greater self-confidence in the management of a posterior capsular tear during surgery (p .025). All respondents manifested to have already performed stages of the CC. The average number of operations reported was 181.6 with regional disparities. A significant difference is observed between the sexes against women (-28.3%, p 0.03). DISCUSSION: Ophthalmologists in Spain, much more than other European countries, have greater opportunities for surgical training, with surgical procedures during the residency, that nearly triples those made by the others. Spanish women refer, like their European colleagues, to be in disadvantage in learning opportunities about cataract surgery. The Simulation Based Medical Education (SBME) allows to respond to the training deficit and complements the training on the patient. Although we demonstrate a significant correlation between the number of procedures carried out and self-confidence to operate simple cases, the SBME would be a complementary tool in self-confidence in front of a complication like capsular rupture. CONCLUSION: Spain massively adopts the model named by us "surgery for all", despite the underrepresentation of women in this area, emphasising a need for cultural change that the SBME could facilitate.

Oral ulcers as a presentation of secondary syphilis.

The incidence of syphilis is increasing, and it typically presents in patients with known risk factors, often to genitourinary physicians. Patients presenting to a dermatologist or ophthalmologist will more likely have secondary syphilis, with the potential for having the associated complications. Early recognition is therefore vital to limit both the disease and risk of further contact spread. In this review, we include two case histories demonstrating the value of recognizing oral signs. Additionally, we review the currently accepted diagnostic and therapeutic recommendations.

Diagnosis of Alagille syndrome-25 years of experience at King's College Hospital.

OBJECTIVE: The aim of the study was to study the clinical and histological features of Alagille syndrome (AGS) at presentation comparing the value of the various modalities before the implementation of genetic diagnosis. PATIENTS AND METHODS: We performed a retrospective analysis of the records of 117 children diagnosed as having AGS after referral to King's College Hospital between 1980 and 2005. RESULTS: Cholestasis was seen in 104 of 117 (89%), characteristic facies in 91 of 117 (77%), posterior embryotoxon in 72 of 117 (61%), butterfly vertebrae in 44 of 117 (39%), heart disease (most often peripheral pulmonary stenosis) in 107 of 117 (91%), and renal disease in 27 of 117 (23%). Serum cholesterol levels of >5 mmol/L were seen in 52 of 86 (60.4%). Liver biopsy showed characteristic features of paucity of interlobular bile ducts in 59 of 77 (76.6%) children younger than 16 weeks of age, in 10 of 14 (71.4%) between 16 weeks and 1 year of age, and in 8 of 12 (66.66%) older than 1 year of age. Other biopsy findings were those of nonspecific hepatitis and biliary features. Iminodiacetic acid scans showed no excretion of isotope into the bowel after 24 hours in 21 of 35 (60%), and small/no gallbladder on ultrasound was seen in 29 of 104 (27.8%). Eleven of 117 (9.4%) had a diagnostic laparotomy and operative cholangiography, 2 proceeding to Kasai portoenterostomy before referral to our unit. CONCLUSIONS: Clinical features of AGS are not as consistently informative as suggested in the literature. Hypercholesterolaemia is nonspecific but may be a helpful pointer. Histology is not characteristic in 25%; hepatobiliary iminodiacetic acid scan and ultrasound may suggest a false diagnosis of biliary atresia in 60% and 28%, respectively, supporting the concept that infants with liver disease warrant early referral to a specialist centre. The advent of genetic diagnosis will redefine the syndrome with likely effects on the prognosis of the defined group.

Visual-evoked potential evidence of chiasmal hypoplasia.

PURPOSE: To show that chiasmal hypoplasia or aplasia need not be an isolated developmental anomaly and to examine the spectrum of associated clinical findings to explore the possibility that these patients may represent a phenotypic manifestation of a developmental gene anomaly. DESIGN: An observational case series. PARTICIPANTS: Five infants, between several weeks and 7 months of age, in whom the electrophysiologic characteristic of chiasmal hypoplasia had been noted were included. METHODS: Flash electroretinography and flash and pattern visual-evoked potentials (VEPs) were elicited from all patients. Clinical ophthalmologic examinations, including funduscopy, were performed, and all patients had magnetic resonance imaging (MRI) brain scans. MAIN OUTCOME MEASURES: The occipital distribution of monocular VEP response peaks was studied. The symmetry of lateral channel responses was compared for monocular stimulation. RESULTS: All five patients had a crossed asymmetry in the monocular VEP occipital distribution, which is consistent with a paucity of fibers crossing at the chiasm. The MRI findings supported this electrophysiologic observation, illustrating degrees of chiasmal hypoplasia and variable coincidence of other midline abnormalities of the brain. Optic disc appearances varied from normal to hypoplastic and colobomatous. CONCLUSIONS: The ophthalmologic and MRI findings of five patients who showed a crossed asymmetry in monocular flash VEPs are consistent with a paucity of axons crossing at the chiasm. The similarities between achiasmia in humans and mice due to a Pax2 gene anomaly are discussed.

Ocular abnormalities in Alagille syndrome.

OBJECTIVE: To assess the type and frequency of ocular abnormalities occurring in Alagille syndrome (AS) in a large group of affected patients and their parents and the potential pathogenetic role of fat-soluble vitamin deficiency. DESIGN: Observational case series. PARTICIPANTS: Twenty-two children with AS and 23 of their parents participated. MAIN OUTCOME MEASURES: Participants underwent full ophthalmic examination, including refraction, orthoptic examination, keratometry, slit-lamp examination, and funduscopy. Corneal diameter measurement was performed in a subset of nine and fluorescein angiography in a subset of six. Serum levels of vitamins A and E and cholesterol were measured. RESULTS: The most common ocular abnormalities in patients with AS were posterior embryotoxon (95%), iris abnormalities (45%), diffuse fundus hypopigmentation (57%, a previously unreported finding), speckling of the retinal pigment epithelium (33%), and optic disc anomalies (76%). Microcornea was not associated with large refractive errors, and visual acuity was not significantly affected by these ocular changes. Vitamin levels were normal. Ocular abnormalities including posterior embryotoxon, iris abnormalities, and optic disc or fundus pigmentary changes were detected in one parent in 36% of cases. CONCLUSIONS: Alagille syndrome is associated with a characteristic group of ocular findings without apparent serious functional significance and probably unrelated to fat-soluble vitamin deficiency. Simple ophthalmic examination of children with neonatal cholestatic jaundice and their parents should allow early diagnosis of AS, eliminating the need for extensive and invasive investigations.

Transscleral diode laser in the treatment of retinopathy of prematurity.

PURPOSE: To assess the outcome of contact transscleral diode laser (TSDL) in the treatment of threshold retinopathy of prematurity (ROP). METHOD: TSDL was performed in 14 eyes of 8 babies who presented to the paediatric ophthalmic service at King's College Hospital between 1996 and 1997 with threshold ROP (median post-conceptual age 26 + 1 weeks, median birthweight 835 g) by a single surgeon. Follow-up ranged from 9 to 41 weeks (median 21 weeks). RESULTS: In 11 eyes (79%) regression of ROP occurred after a single laser treatment with a good anatomical outcome. In 3 eyes (21%) there was an unfavourable response with the development of traction retinal detachment. These include both eyes of one baby who rapidly progressed to stage IV ROP. One other eye developed a fibrotic band a few months after treatment. No significant complications of laser treatment were observed. CONCLUSION: These initial results indicate that TSDL photocoagulation is an effective and technically straightforward alternative to cryotherapy in the treatment of ROP.

Prenatal diagnosis of orbital heterotopic brain tissue.

Biopsy of fetal tissues is a relatively new procedure for the diagnosis of congenital malformations. The authors report the first case in which this technique has been applied to an orbital mass, in which heterotopic brain tissue was diagnosed by prenatal biopsy and excised in infancy. The wider implications of such intrauterine procedures are discussed.

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.

Topical steroid use in the treatment of ocular alkali burns.

BACKGROUND: Ocular alkali burns can be associated with a poor visual outcome. The release of collagenases and proteases after the injury leads to corneoscleral melting. The role of topical steroids in such patients is controversial as they have been postulated to exacerbate corneoscleral melting. METHODS: 30 patients were reviewed retrospectively after admission to King's College Hospital with alkali burns between 1990 and 1993. All patients were treated with an intense and prolonged regimen of topical steroids and topical and systemic vitamin C. RESULTS: 22 patients had mild injuries and eight had severe injuries as estimated by the Roper-Hall grading system. 23 patients were treated with topical steroids for > 10 days and 22 patients were treated with topical vitamin C for more than 10 days. One patient with a severe injury developed corneoscleral melting. CONCLUSION: Prolonged treatment with topical steroids when used in conjunction with topical vitamin C is not associated with corneoscleral melting.

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.

Bilateral symmetrical branch retinal artery occlusions.

We present a report of a 52-year-old hypertensive patient with documented bilateral symmetrical branch retinal artery occlusions involving the maculae. The patient presented with no visual symptoms and maintained 6/5 unaided visual acuity in each eye. Although the incidence of retinal artery occlusion in hypertensive patients is well documented, symmetrical bilateral branch retinal artery occlusions suggest a possible anatomical vascular predisposition.

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.

Corneal perforation as a complication of epidermolysis bullosa acquisita.

Epidermolysis bullosa acquisita (EBA) is now recognised as a histopathologically distinct condition. Ocular complications of hereditary epidermolysis bullosa (EB) have been well documented, but little has been reported with respect to the ocular manifestations associated with the acquired form. A 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.