Early ophthalmic growth deficits in preterm-born children are not compensated for during later development.

The renewed focus on eye growth in preterm-born children was primarily triggered by Danish cohort studies, including the Copenhagen Project, which focused on children born from 1959-1961. The retinotoxic effects of excessive oxygen on premature neonates had long been clarified and therapeutically adjusted for. Later, ultrasound oculometry and keratometry established that ocular size deficits, linked to development, also occurred in normally developing children, not just frail outliers. This indicated that general catch-up had not been achieved. This paper discusses whether one early segment of eye development does not occur in preterm, and here even in more robust neonates, without later compensation.

Scleroderma en Coup de Sabre, Parry-Romberg Hemifacial Atrophy and Associated Manifestations of the Eye, the Oral Cavity and the Teeth: A Danish Follow-Up Study of 35 Patients Diagnosed between 1975 and 2015.

BACKGROUND: Scleroderma en coup de sabre (ECDS) and Parry-Romberg idiopathic hemifacial atrophy (IHA) may affect the eyes, oral cavity, teeth and possibly the brain. OBJECTIVE: Systematic follow-up study of ECDS/IHA-associated manifestations including ophthalmic and dental status. METHODS: Medical records of ECDS and IHA patients diagnosed in a 40-year period (1975-2015) were reviewed, and patients were re-examined. RESULTS: Thirty-five patients were included. Twenty-two patients (63%) had ECDS and 4 patients (11%) IHA. In 9 cases (26%), ECDS and IHA were found in the same patient. The ipsilateral eye was affected in 9 patients (26%). Ipsilateral abnormalities of the teeth and the tongue were found in 13 (46%) out of 28 examined. Eleven (31%) had extrafacial scleroderma on the trunk or the extremities. Neurological findings were not verified as ECDS/IHA related. CONCLUSION: ECDS and IHA are related and often overlap with concomitant affections of the connective tissues of the face on the ipsilateral side. Ocular and dental abnormalities are common and follow the distribution of the primary affection, for example, a paramedian line in the front and segmental affection of the maxilla and the mandible. The affections point to predisposing dysmorphogenetic events in embryonal life affecting the face, with abnormality of crest cells at the stage when they migrate from behind over the scalp or laterally to the face to mix up with mesenchymal tissues of the frontonasal, maxillary and mandibular processes. The study emphasizes that routine evaluation of ECDS and IHA should include ophthalmological and dental specialist examinations.

Autosomal-recessive posterior microphthalmos is caused by mutations in PRSS56, a gene encoding a trypsin-like serine protease.

Posterior microphthalmos (MCOP) is a rare isolated developmental anomaly of the eye characterized by extreme hyperopia due to short axial length. The population of the Faroe Islands shows a high prevalence of an autosomal-recessive form (arMCOP) of the disease. Based on published linkage data, we refined the position of the disease locus (MCOP6) in an interval of 250 kb in chromosome 2q37.1 in two large Faroese families. We detected three different mutations in PRSS56. Patients of the Faroese families were either homozygous for c.926G>C (p.Trp309Ser) or compound heterozygous for c.926G>C and c.526C>G (p.Arg176Gly), whereas a homozygous 1 bp duplication (c.1066dupC) was identified in five patients with arMCOP from a consanguineous Tunisian family. In one patient with MCOP from the Faroe Islands and in another one from Turkey, no PRSS56 mutation was detected, suggesting nonallelic heterogeneity of the trait. Using RT-PCR, PRSS56 transcripts were detected in samples derived from the human adult retina, cornea, sclera, and optic nerve. The expression of the mouse ortholog could be first detected in the eye at E17 and was maintained into adulthood. The predicted PRSS56 protein is a 603 amino acid long secreted trypsin-like serine peptidase. The c.1066dupC is likely to result in a functional null allele, whereas the two point mutations predict the replacement of evolutionary conserved and functionally important residues. Molecular modeling of the p.Trp309Ser mutant suggests that both the affinity and reactivity of the enzyme toward in vivo protein substrates are likely to be substantially reduced.

The Longitudinal Danish High Myopia Study, Cohort 1948: at age 66 years visual ability is only occasionally affected by visual field defects.

PURPOSE: A descriptive study on visual fields, as part of a 50-year follow-up of high myopia in an unselected cohort-based Danish sample, now aged 66 years. METHODS: In a Copenhagen 1948 birth cohort (n = 9243), 39 individuals aged 14 years were identified with myopia of at least -6 D, and with regular clinical follow-ups since then. In 2002 (n = 34, age 54 years) and 2008 (n = 32, age 60), the individual ambulatory visual field was outlined by kinetic Goldmann large object perimetry (IV or V,4e). At age 66 years, 28 attended for the 2014-2015 follow-up, at which smaller Goldmann objects (II and I,4e) were added, further to identify relative defects. RESULTS: Repeated large object perimetry disclosed statistically significant general peripheral narrowing over the 12-13-year test period, though slight and without practical implications. Two new cases showing absolute defects were however added to the three already known. The addition of small Goldmann objects disclosed relative defects in another eight participants, in some to suggest a refraction-related pattern (fundus ectasia; uncorrected high myopia). However, comparing eyes with and without defects, statistical importance could not be attached to the degree of myopia, fundus ectasia or optic disc morphology (χ2 , n.s.). CONCLUSION: (i) Serial large object Goldmann isopters over the 'senior' decade up to age 66 demonstrated a slight general peripheral narrowing by age of visual fields in high myopia. (ii) Overall 42% of the participants had absolute or relative defects (in 5 and 8, respectively), however, without socio-visual consequences when binocular. (iii) Visual field loss by age still appears a minor issue in clinically unselected high myopia.

Choroidal thickness at age 66 years in the Danish high myopia study cohort 1948 compared with follow-up data on visual acuity over 40 years: a clinical update adding spectral domain optical coherence tomography.

BACKGROUND AND PURPOSE: A population-based Copenhagen birth year 1948 cohort with high myopia recorded since age 14 years (spherical equivalent less than or equivalent to -6 D) has been followed over 50 years. Despite complications, current follow-ups have outlined a better visual prognosis than usually drawn from selected clinical series in the literature. For the present status at age 66 years, focus was on visual ability and choroidal thickness. METHODS: Twenty-eight of the original 39 participants were available in 2014. Medical history was updated. Best-corrected visual acuity (BCVA) data were compared with subfoveal choroidal thickness (SFCT), now measured by enhanced depth optical coherence tomography. RESULTS: Due to at least better eye visual acuity (VA), all patients had maintained their everyday visual capacity. Only one participant was marginal regarding visual status for a driver's licence; low vision was not on record. Based on all eyes, choroidal thickness correlated negatively with axial length (AL), which also held for the fraction with high myopia (AL >26.5 mm). In high myopia, the mean choroidal subfoveal thickness was 114 ± 75 µm versus 182 ± 94 µm in lower myopia (p = 0.01). CONCLUSION: Despite the generally maintained individual visual capacity in the series, significant correlation could be demonstrated between SFCT and (i) axial elongation and (ii) recorded VA, with a negative and a positive sign, respectively. Overall, the visual prognosis was relatively benign, in particular when compared with the selected high myopia hospital series that predominate in the ophthalmic literature.

Optic disc drusen: longitudinal aspects, with emphasis on visual field constriction and enlarged blind spot: a retrospective hospital-based clinical series.

PURPOSE: To examine long-term data on optic disc drusen (ODD) from an outpatient hospital series that indicated more cases with advanced visual field constriction than is apparent from other clinical reports. The underlying pathophysiology is discussed, also with regard to enlarged blind spot, which, in view of the small disc at risk, may seem a paradox. METHODS: This is an observational retrospective study on an eye clinic series (n = 49), focusing on visual acuity, kinetic/static perimetry, and longitudinal trends, to include the question of eventual visual incapacity. RESULTS: Forty-nine patients (32 female and 17 male; bilateral ODD in 45) aged 5-76 years (median age 29 years for both sexes) were included in the study. Observation time was 1-54 years, with serial data recorded over at least 3 years in 25 patients. Visual field defects were found in 32 patients, with ODD considered responsible for the visual field defects demonstrated. Advanced unilateral concentric constriction (for the largest Goldmann object) was recorded in 10 patients, and bilaterally in 2. With regard to nonexplanatory side diagnoses, 2 patients had pituitary adenoma, 1 had a cavernous sinus meningioma, and 1 had neurosurgery for an arachnoid cyst. CONCLUSIONS: We found more cases of marked visual field constriction than reported in other clinical series. A few such cases appeared acute and vascular, but the main trend was clinically quiet over time. All 49 patients could manage visually in daily life.

Hereditary high hypermetropia in the Faroe Islands.

PURPOSE: To characterize the phenotype of two families with high hypermetropia from the Faroe Islands. METHODS: Ophthalmologic evaluation including ultrasound oculometry and anthropometric measurements. RESULTS: Of the 40 examined family members, 15 individuals (8 males, 7 females; ages: 6-77 years; mean: 36.5 years) had small deep-set eyes with high hypermetropia (median: + 16.5 D; range: + 7.75 to + 22), short axial eye length (< 21 mm), and a thickened eye wall. The median corrected visual acuity was 0.4 (0.2-0.9). Ocular complications included angle-closure glaucoma in six eyes, uveal effusion in three eyes, cataract in two eyes, and esotropia with amblyopia in three eyes. An emergency case of uveal effusion and retinal detachment after Yag iridotomy eventually responded to systemic corticosteroids and scleral resection surgery with a slow visual recovery. No associated ocular or systemic malformations were found in the series. In addition to the two examined families, six smaller Faroese families with high hypermetropia are briefly reported. CONCLUSIONS: The study highlights the signs and symptoms of a rare hereditary phenotype characterized by a short axial length mainly confined to the posterior segment of the eye, a shallow anterior chamber, and a thickened eye wall. The morphological characteristics predispose for sight-threatening complications such as angle-closure glaucoma, chorioretinal pathology including uveal effusion, and amblyopia. Regular ophthalmic follow-up is therefore of obvious importance in families known to have small eyes/high hypermetropia. An endemic high prevalence in the Faroe Islands suggests the presence of a founder effect, and further genetic research would probably indicate pseudodominant rather than dominant transmission

Refraction and visual acuity in a national Danish cohort of 4-year-old children of extremely preterm delivery.

PURPOSE: A recent threefold increase in laser treatment for advanced retinopathy of prematurity (ROP) triggered a nationwide preschool ophthalmic and developmental status among extremely preterm survivors. Here, we discuss refraction and visual acuity. METHODS: Survivors (n = 178) from a national birth cohort (February 2004 to March 2006) of gestational age <28 weeks (PT) and 56 full-term (FT) controls attended for evaluation at age 4 years. Cycloplegic refraction and keratometry were achieved by Retinomax autokeratorefractor and visual acuities by symbol recognition (HOTV, logMAR). RESULTS: The refractive distribution presented a myopic tail (4.5%) and a hyperopic tail (11.9% ≥+2.5 D) as special preterm features, and corneas were more curved. Astigmatism and anisometropia were only marginally increased, and visual acuities were generally good. Best-corrected binocular median logMAR visual acuity was 0.1 in FT and 0.2 in PT, in Snellen equivalents 0.8 and 0.63. Snellen acuity ≤0.5 occurred across the ROP subgroups, but mainly in those with at least ROP stage 3. Two children had low vision. CONCLUSIONS: The overall fair outcome for refraction and function is in accordance with other recent northern Europe experience. The results differ in particular from the poorer ophthalmic outcomes reported in the pioneer US treatment studies (cryotherapy for ROP and ETROP). The diode laser ablations (n = 32) appeared effective in our series; except one child, all treated subjects had good or fair social vision at the age of 4 years.

Ophthalmic experience over 10 years in an observational nationwide Danish cohort of Fabry patients with access to enzyme replacement.

PURPOSE: Enzyme replacement therapy (ERT) was offered from year 2001 to patients with Fabry disease. The ophthalmic experience was analysed, as part of a general 10-year status. METHODS: A retrospective observational series comprising 39 patients (25 females, 14 males) closely followed by the endocrinologists, and with regular ophthalmic control. Time of inclusion was when the option of ERT was started, at age 11-60 years. Eye data (standard eye examination, including retinal imaging) were incomplete in five, due to death or non-attendance, and five patients had refused treatment. RESULTS: Vision was normal throughout, except in two young males with total unilateral central retinal artery occlusion, prior to and during enzyme replacement, respectively. Cornea verticillata and conjunctival vessel ectasies were common. Tortuosity of retinal arterioles and venules was recorded in eight and 18 patients, respectively, and phlebopathy in 22, although generally without evidence of loss of vessel wall integrity. Systemic vascular lesions with or without loss of function were recorded for kidney (n = 23), heart (n = 17) and brain (n = 7), and an association was suggested between nephropathy and abnormal morphology of retinal vessels. CONCLUSIONS: Thirteen of 32 patients on ERT showed a reduction of corneal deposits over the study period. Abnormal ocular vessel morphology was a frequent finding. In contrast to the function loss related to systemic ischaemic lesions, we found no indication of impairment of visual parameters in 37. Compared to other Fabry series, two of 39 patients with serious unilateral occlusive retinal disease may appear a high number. The presence of retinal tortuosity is discussed, possibly reflecting haemodynamic events related to vessel wall deposits, but could also be 'constitutional', as part of the Fabry inheritance.

A nationwide Danish study of 1027 cases of congenital/infantile cataracts: etiological and clinical classifications.

OBJECTIVE: To study the distribution of congenital/infantile cataract in the entire population of Denmark according to etiological and clinical classifications. DESIGN: Population-based cohort study with retrospective chart review. PARTICIPANTS: All children (0 to 17 years old) who were born between 1959 and 2001 and registered with congenital/infantile cataract in Denmark during the period 1977 to 2001. Cases were ascertained from the mandatory Danish National Register of Patients, and all medical records were reviewed. METHODS: Etiological and clinical classifications of the cataract cases were based on information from the medical records. MAIN OUTCOME MEASURES: Classification of congenital/infantile cataract according to presumed etiology; gender; clinical appearance, including laterality and morphology; and the time trends according to etiology and laterality. RESULTS: A total of 1027 children with congenital/infantile cataract, 529 boys and 498 girls, were included, of whom 64% were bilateral. Males predominated with bilateral cataract, whereas females predominated with unilateral cases. Isolated cataract was the most frequent clinical presentation (71% of all cases), followed by an even proportion of cataract associated with additional ocular dysmorphology and cataract associated with systemic anomalies. Almost two thirds of all cases had an unknown etiology (idiopathic). Idiopathic cases showed a higher proportion of unilateral cataract and of additional ocular dysmorphology compared with cases of known etiology. The etiology was unknown in 87% of unilateral cases and in 50% of bilateral cases. The distribution by presumed etiology was stable during the study period, except for cataract caused by maternal infections, which decreased mainly due to the elimination of congenital rubella. CONCLUSIONS: With the exception of the decline of congenital rubella, the proportion of congenital/infantile cataract cases of unknown, genetic, and infectious origins has been stable since the late 1970s. The causes of 87% of unilateral cataracts and 50% of bilateral congenital/infantile cataracts remain unknown, making the prevention of the disease a continuing challenge.

Infantile nystagmus and visual deprivation: foveal instability and refractive development in a low vision register series.

PURPOSE: To evaluate whether effects of early foveal motor instability due to infantile nystagmus might compare to those of experimental visual deprivation on refraction in a childhood series. METHODS: This was a retrospective analysis of data from the Danish Register for Blind and Weaksighted Children with infantile nystagmus recorded as prime diagnosis. We perused 90 records of children now aged 10-17 years, some of whom eventually exceeded the register borderline of 0.3 as best-corrected visual acuity. Spherical equivalent refraction was the primary outcome parameter, but visual acuity, astigmatism, and age were further considered. The series comprised 48 children with nystagmus as single diagnosis, whereas 42 had clinical colabels (Down syndrome [13], dysmaturity [9], and mental retardation, encephalopathy [20]). RESULTS: Median binocular visual acuity was 0.3 in the full series, and median refraction was emmetropia in all subgroups. Compared with Danish control data, myopia was over-represented, and generally of juvenile onset. The Down syndrome subgroup was separated from the remainder by an even higher myopia prevalence. Astigmatism above 1 D cylinder value was recorded in 52% of all cases. CONCLUSIONS: The prevalence of myopia and astigmatism was higher among children with nystagmus than in controls. Myopia was mainly juvenile, and not related to the period of infancy when the motor foveal smear is considered most disturbing and possibly influencing visual development.

Juvenile eye growth, when completed? An evaluation based on IOL-Master axial length data, cross-sectional and longitudinal.

PURPOSE: To test Sorsby's classical statement of axial eye growth as completed at the age of 13 years, with a view also to differentiating between basic eye growth and juvenile elongation associated with eventual refractive change towards myopia. METHODS: (i) A total of 160 healthy eyes close to emmetropia were included in a cross-sectional set-up (age 4-20 years, 91 males, 69 females), and (ii) 78 longitudinal data sets (67 male and 11 female annual repeat exams over 2-7 years, n=30; age span 4-19 years) were available for evaluating individual axial elongation. The IOL-Master equipment was preferred for conventional ultrasound oculometry due to its extreme repeatability of measuring values, thus making it well fitted for evaluating very small differences. In particular, this had bearing for the decelerating end phase of growth in the longitudinal investigation. RESULTS: Sorby's statement about age 13 as general limit found support from the cross-sectional data, which suggested stable emmetropic eye size from about 11-12 years, with an average apparently outgrown male emmetropic value of 23.5 mm versus females' 22.9 mm. The longitudinal data, however, showed emmetropic growth also beyond this age, with individual data to establish continued axial elongation also at age 13-18 years. The final basic teenage growth is however minute and without practical implications. CONCLUSIONS: Individual ocular growth curves have indicated axial elongation to occur also after the age of 13 years. With regard to the - mainly academic - discrepancy between cross-sectional and longitudinal results, bigger samples are needed, and the juvenile myopic trend has to be acknowledged.

Human parallels to experimental myopia? A literature review on visual deprivation.

Raviola and Wiesel's monkey eyelid suture studies of the 1970s laid the cornerstone for the experimental myopia science undertaken since then. The aim has been to clarify the basic humoral and neuronal mechanisms behind induced myopization, its eye tissue transmitters in particular. Besides acquiring new and basic knowledge, the practical object of the research is to reduce the burden of human myopia around the world. Acquisition and cost of optical correction is one issue, but associated morbidity counts more, with its global load of myopia-associated visual loss and blindness. The object of the present PubMed literature-based review is to evaluate apparent similarities between experience from disturbed imaging in experimental laboratory science and varieties within the spectrum of childhood human myopia. So far, the main impression is that macroscopical optical deprivation appears absent in the prevalent types of human myopia, nor is myopia a regular sequel where early eye pathology has led to poor imaging and optical deprivation. Optical aberrations of a higher order are a relatively new issue in myopia research, and microstructural deprivation is only marginally dealt within the survey. Links between experimental and human myopia appear mainly occasional, and with only few examples in humans where factual parallels appear credible. Clinical and epidemiological data on refraction remain important, in particular with a view to life style and environmental factors. Such knowledge may further serve as inspiration to the laboratory research, which aims at solving the basic enigmas on a tissue level.

The highly myopic eye--oculometric considerations.

After a brief historical oculometry review, the purpose is to bring a longitudinal update on an unselected Danish high myopia series, which was first presented at 1978 SIDUO. The sample was recruited from a Copenhagen school cohort (N=9,423, born 1948) as 14-year-olds with myopia of at least -6 D (n=39) in 1962. Clinical follow-up studies were conducted in 1976, 1986, 1995 and 2002, with A-scan ultrasound oculometry (Kretztechnik 7000, immersion method; and Sonometrics 400, contact method) as a regular part of the schedule. For comparison, IOL Master evaluations were added in 2002. The following results and conclusions were made: (a) up to age 54, most subjects had maintained good or fair vision; (b) as in other myopia series, axial and vitreous length were found to be the most important optical parameters, with corneal curvature radius ranking next; (c) however, as previously described by Francois and Goes, not all long eyes have flat corneas. In our high myopia series, a significant subgroup could thus be discerned who had peaked corneas, which led to mathematically slight reduction of the usual positive correlation between axial length and curvature radius in the ametropia population; and (d) we found positive correlation between myopia increase in adult age and axial elongation, stressing that, in contrast to other outgrown body structures, the adult eye globes may still expand in size. This feature seems to be found not only in the eyes with posterior staphyloma, but also in myopic eyes of regular round shape.

Bilateral symmetric autosomal dominant sector chorioretinopathy with late maculopathy: a review based on a case with 48 years follow-up.

PURPOSE: To describe the long-term course of bilateral symmetric autosomal dominant sector chorioretinopathy in a 79-year-old man who was diagnosed at age 31. METHODS: Ophthalmic examinations including fundus photography, fluorescein and indocyanine angiography, adaptometry, and ocular electrophysiology were performed at intervals from 1962 to 2010. RESULTS AND CONCLUSIONS: The patient experienced no visual symptoms during his entire working life, but acquired an exudative maculopathy in his left eye at age 67. Two years later, a central subretinal neovascular membrane affected the right eye. The latter responded only temporarily to photodynamic therapy (Visudyne), and from age 71 he had best-corrected visual acuities <0.1 in both eyes, with eccentric fixation. We are not aware of any report on late macular involvement in this disorder. Whether the maculopathy should be considered a late manifestation of the sector chorioretinopathy or a coincidental occurrence of age-related macular degeneration remains unsettled.

Eye features in three Danish patients with multisystemic smooth muscle dysfunction syndrome.

BACKGROUND: A de novo mutation of the ACTA2 gene encoding the smooth muscle cell α-actin has been established in patients with multisystemic smooth muscle dysfunction syndrome associated with patent ductus arteriosus and mydriasis present at birth. OBJECTIVE: To describe the structural ocular findings in three Danish children with this new syndrome and evaluate the possible functional consequences for visual development of the poorer imaging condition. RESULTS: Unresponsive mydriatic pupils with scalloping wisps of persistent pupillary membrane from the iris collarette were an early indicator of this rare genetic disorder in all three cases. Tortuousity of retinal arterioles was the main posterior pole finding, apparent during the first year of life and with a tendency to increase with age. In one case, it progressed to an aneurysmal-like state with breakdown of the blood-retinal barrier. CONCLUSIONS: Congenital mydriasis is an extremely rare pupil anomaly and is the feature for the early diagnosis of this new syndrome. The ophthalmologist should act in close collaboration with other specialists owing to the risk of aortic and cerebrovascular diseases and other complications associated with this disorder.

Late subsequent ocular morbidity in retinopathy of prematurity patients, with emphasis on visual loss caused by insidious 'involutive' pathology: an observational series.

PURPOSE: This paper discusses late complications in eyes of surviving premature infants typically occurring years into the steady state attained after the initial ophthalmic events associated with preterm birth. The study focuses on insidious visual loss, as well as eventual vitreoretinal and anterior segment disorders. METHODS: We carried out a retrospective uncontrolled study of a case series (n = 31) of late complications to early ocular changes associated with very preterm delivery. The birth years of our subjects extended back to 1941, but only 15 more recent cases fulfilled contemporary criteria for a diagnosis of retinopathy of prematurity (ROP). RESULTS: Fourteen cases involved vitreoretinal pathology (median observation time 14 years, range 6-46 years) with late detachment and/or vitreous haemorrhage. Twelve cases primarily involved anterior segment pathology (median observation time 37 years, range 7-58 years). Eyes in the latter category often showed features of early arrested growth and anterior traction, which, in four eyes, eventually led to enucleation or exenteration as a result of a blind and painful terminal state. The last five cases (observation periods 22-38 years) were labelled 'miscellaneous'. Among the 31 patients, we identified a subgroup in which insidious visual loss was a common feature (n = 20). Initially, some of these cases showed evidence of cicatricial ROP and low vision from early in life. Others retained useful vision and did not present obvious ocular incidents related to the actual visual acuity decline. CONCLUSIONS: Based on a variety of clinical ophthalmic preterm appearances, we propose the hypothesis that, in addition to increased structural vulnerability, a reduced functional reserve may pave the way for further abiotrophy or involution over time. This may manifest as reduced resistance to later ophthalmic events, such as disease or trauma, or simply the impact of age.

Eye shape and peripheral visual field recording in high myopia at approximately 54 years of age, as based on ultrasonography and Goldmann kinetic perimetry.

PURPOSE: This study describes the posterior pole contour and visual field (VF) findings in an unselected series of adults (aged approximately 54 years) with high myopia. METHODS: In 1962, 39 14-year-old teenagers with myopia of >or=6 D, in one or both eyes, were identified in a 1948 Copenhagen birth cohort (n=9243). At a follow-up in 2002, 61 eyes (52 with high myopia and nine fellow eyes; n=31 subjects) were examined by ultrasound B-scan, to allow: (a) measurement of the axial vitreous length supplementary to the customary axial A-scan, and (b) assessment of the posterior pole contour of the eye. Further, the large-object (V, 4e) kinetic Goldmann perimetry isoptre was recorded to outline the ambulatory VF. RESULTS: An irregular posterior pole contour was observed in 15 eyes, unilaterally in 11 eyes and bilaterally in four (two subjects). Nasal fundus ectasia was suggested in six and other deviations from the sphere in seven subjects. Kinetic Goldmann perimetry showed marginal restriction in 14 eyes (12 subjects). Larger defects were found in four eyes (three subjects); the corresponding axial lengths exceeded 32 mm in the two subjects with unilateral defects, and symmetrical inferior binasal defects, probably unrelated to the myopia, were found in the bilateral subject. CONCLUSIONS: Unselected adult-age high myopia mainly presented with a regular eye shape that was close to spherical. An irregular shape suggesting posterior staphyloma was seen in 13 of the 32 subjects under study, mainly unilaterally. No subjects were restricted in everyday activities by VF defects. Marginal Goldmann perimetry restrictions were described in 12 and significant defects in three subjects. Generally, longer eyes more often tended to show irregular posterior eye contours and/or VF defects.

Oculometry findings in high myopia at adult age: considerations based on oculometric follow-up data over 28 years in a cohort-based Danish high-myopia series.

PURPOSE: To present and discuss oculometry data in a series of adults with high myopia followed between the ages of 26 and 54 years. Emphasis is on axial length (AL) findings and corneal curvature radius (Crad). METHODS: Thirty-four out of the 39 individuals recruited as teenagers from a Copenhagen 1948 birth cohort with myopia of at least 6 D have had current follow-up exams, to include AL measurements (by ultrasound, 1974-2002; the latter year also with the Zeiss IOLMaster) and keratometry. The cross-sectional and longitudinal analyses are based primarily on the eyes with high myopia; however, the fellow eye is also assessed in unilateral cases. RESULTS: At age 54 years, the maximum myopia in the series was -26 D; the highest AL value was 35.4 mm. The myopia had increased in most, with an increase from the 26-year oculometry baseline averaging 1.0 D [standard deviation (SD) 1.84]. Ultrasound measurements over the 28 years gave a significant correlation between axial eye elongation and myopia progression of adult age (r = 0.65). The regression line was y = 0.43 + 0.36x, with myopia increase on the x-axis. Throughout sessions, the association between AL and refraction was given by correlation coefficients numerically above 0.8, whereas AL and Crad had r-values of 0.3-0.5. However, a mean Crad in the sample of 7.66 (SD 0.28) mm meant that the more general expectancy of rather flat corneas in high myopia was not fulfilled. Our data further suggested a reduction in lens power over the study period. CONCLUSION: In relation to refraction, AL and Crad remain the two main oculometry parameters. Apparently the correlation patterns regarding the cornea that are broadly valid for axial ametropia in the population cannot be extended to the marginal high myopia tail of the distribution. A significant proportion of eyes with high myopia thus had steeper corneas than expected, as a so-called index contribution (albeit a small one) to the marginal refractive error.