Marie Restori

Posterior scleritis: Clinical features, systemic associations, and outcome in a large series of patients

OBJECTIVE To document the clinical features, systemic associations, and visual outcome in a large number of patients with posterior scleritis. DESIGN Retrospective, noncomparative case series. PARTICIPANTS There were 137 patient records showing patients with a diagnosis of posterior scleritis who were attending or had attended the scleritis clinic at Moorfields Eye Hospital between 1974 and 1996. Ninety-nine records were suitable for detailed analysis. METHODS The medical records and B-mode ultrasound examinations were reviewed. MAIN OUTCOME MEASURES The clinical features, systemic associations, treatment, and outcome of each patient were determined. RESULTS Posterior scleritis occurred at all ages. The mean age at onset was 49.3 years. Posterior scleritis began before age 40 in 30% of patients and was twice as common in women as in men. The B-mode ultrasound examination showed diffuse and nodular changes in the posterior sclera. Necrotizing posterior scleritis was not identified. Twenty-nine percent of patients had an associated systemic disease that included systemic vasculidites, autoimmune diseases, and lymphoma. Such patients more commonly had nodular changes on B-mode ultrasound examination. Early treatment controlled posterior scleral inflammation and limited visual loss. Thirty-one percent of patients lost two or more lines of vision. Statistical analysis revealed that patients older than age 50 had an increased risk of having an associated systemic disease and were more likely to experience visual loss. Patients with associated systemic disease required more aggressive immunosuppressive therapy and more frequently had accompanying anterior scleritis. There was no association between unilateral, bilateral, or recurrent disease and the presence of systemic disease or visual loss from posterior scleritis. CONCLUSIONS The B-mode ultrasound examination reveals that posterior scleritis occurs far more often than previously thought and can lead to rapid and permanent visual loss. All patients with posterior scleritis must be assumed to be at risk of visual loss. Forty percent of patients had no anterior scleral inflammation, and 9% had no detectable physical signs. All patients need to be investigated for an associated systemic disease and all require early treatment to minimize loss of vision.

Ultrasonic assessment of rhinostomy size following external dacryocystorhinostomy

AIM To assess the dimensions and patency of the surgical epithelial fistula after external dacryocystorhinostomy, using B mode ultrasonography to define the postoperative soft tissue anastomosis. METHODS 12 patients undergoing 16 external dacryocystorhinostomies, with the creation of large osteotomies, were included in a prospective study. The horizontal and vertical dimensions of the bone ostium was recorded during surgery and compared with the ultrasonographic dimensions of the soft tissue anastomosis at 1 day, 2 weeks, and 6 months after surgery. Functional patency was confirmed with dye testing and irrigation. RESULTS Compared with an osteotomy of between 100 and 380 mm2 (mean 235 mm2), the soft tissue anastomosis on the day after surgery was, in all cases, markedly smaller (72–252 mm2; mean 144 mm2, or 61% of the bone window). The soft tissue anastomosis decreased to between 8 and 208 mm2 (mean 98 mm2; 68% of immediate postoperative value) at 2 weeks and 3–208 mm2 (mean 71 mm2; 49% of immediate postoperative value) at 6 months. 14 of the 16 (88%) dacryocystorhinostomies were functional at the end of the study, the two failures being associated with marked contracture of the soft tissue anastomosis; the outcome of surgery correlated significantly with the area of the anastomosis at 2 weeks (χ2 = 16.3; p<0.01) and at 6 months (χ2= 16.0, p= 0.01). CONCLUSIONS B mode ultrasonography provides a simple and effective method for assessing the size of the soft tissue anastomosis after external dacryocystorhinostomy and there is a significant reduction in size after surgery, to which the functional outcome of surgery appears related. As the initial soft tissue anastomosis cannot be larger than (and is, on average, about 60% of) the area of the osteotomy, this emphasises the paramount importance of a large rhinostomy to the success of lacrimal surgery.

Ocular enlargement following infantile corneal opacification

Congenital hereditary endothelial dystrophy (CHED) is not generally thought to be associated with other ocular abnormalities. Ultrasonography in a series of twenty eyes (ten patients) with CHED shows ocular enlargement similar to that occurring in uncomplicated axial myopia. There was an inverse relationship between the degree of enlargement and the visual acuity or visual result following penetrating keratoplasty suggesting that infantile corneal oedema sufficient to cause stimulus deprivation may result in abnormal enlargement of the globe.

Rapid B-scanning of the vitreous.

Ultrasonic examination is an essential investigation in most patients awaiting vitrectomy. Rapid B-scanning of the vitreous is described utilising a new system capable of high resolution and good tonal quality. Several patterns of haemorrhagic invasion of the vitreous cavity are illustrated, together with detachment, collapse, and retraction of the vitreous gel.

Natural history of periocular capillary haemangiomas: changes in internal blood velocity and lesion volume

AimAlthough the clinical characteristics of childhood periocular capillary haemangiomas are well known, serial measurements of blood velocity and lesion size are unknown. This investigation was designed to measure the changes in maximum blood velocity and estimated size of lesion in children with capillary haemangioma not requiring active intervention.Study designRetrospective case-note review for a cohort of children with capillary haemangioma involving the eyelid and orbit.Patients and methodsChildren with periocular capillary haemangioma, under the care of the Orbital unit at Moorfields Eye Hospital between 1996 and 2005, were monitored clinically and with repeated ultrasonographic examination. Volume estimates were calculated as an ovoid based on the three maximum orthogonal measurements for the haemangioma, and blood velocity was assessed by Colour Flow Mapping, Colour Doppler Energy Imaging, and Spectral Doppler techniques using a Sequoia 512 Acuson scanner.ResultsTwenty-four children (12 boys) had initial assessment by 18 months of age, and the haemangioma increased in size in 14/24 (58%), the increase being between 4 and 931% of initial volume estimate. The largest measured size for an individual haemangioma appears inversely related to the childs age at measurement, this mirroring a similar trend in measurements for the maximum blood velocity. Blood velocity measurements also tend to decrease with time, the peak velocity being before 1 year of age in the majority (15/24; 62%). In many children, both volume estimates and blood velocities show a cyclic variation—this occurring with increasing intervals between the maxima, before a final decay in both parameters.Although, for the whole group, there was no correlation (correlation coefficient=0.29) between estimated size and measured blood velocity, some individual children showed a significant correlation between the two parameters. The age at maximum blood velocity appeared to precede the age at maximum volume in most children, and in many there was an orbital anomaly detectable on ultrasonographic examination, even with complete clinical resolution of the haemangioma.ConclusionsUltrasonographic examination of periocular capillary haemangiomas show that these lesions have a very high blood velocity in feeding vessels—about 2–3 orders of magnitude greater than normal capillary beds—and that the velocity and volume of such lesions undergo a cyclic variation during their natural history. Evidence suggests that both velocity and volume decrease with time, although often not returning to zero on ultrasonography (unlike the clinical resolution of the lesions). In most children, blood velocity peaks before volume estimates and this might suggest that decreasing perfusion leads to later tissue atrophy and involution of the haemangioma.

Evaluation of iris and iridociliary body lesions with anterior segment optical coherence tomography versus ultrasound B-scan

Aims To compare anterior segment optical coherence tomography (AS-OCT) with ultrasound B-scan (USB) in evaluating iris and iridociliary body lesions. Methods Image features and resolution comparison between AS-OCT and USB in 126 patients (126 eyes) presenting with iris or iridociliary body lesion. Bland–Altman plots were generated to assess the level of agreement between the two techniques. Results The three most common diagnoses were iris naevi (62 (49.2%)), iris pigment epithelial cysts (23 (18.3%)) and iris melanoma (11 (8.7%)). Image feature comparison for USB was better than AS-OCT in visualising all tumour margins (81 (64.3%) vs 59 (46.8%)), posterior tumour margin (54 (42.9%) vs 16 (12.7%)) and producing less posterior shadowing (121 (96%) vs 43 (34.1%)). Image resolution comparison revealed USB to be slightly better for resolving the overall tumour (45 (35.7%) vs 43 (34.1%)) and posterior tumour surface (70 (55.6%) vs 32 (25.4%)) but AS-OCT was better for resolving the anterior (62 (49.2%) vs 4 (3.2%)) and lateral tumour surface (62 (49.2%) vs 31 (24.6%)). Comparing the three most common diagnoses, USB was better for visualising iris pigment epithelial cysts (12 (52.2%) vs 2 (8.7%)) and iris melanoma (7 (63.6%) vs 1 (9.1%)) but AS-OCT was better (28 (45.2%) vs 15 (24.2%)) for visualising iris naevi. Bland–Altman plots showed good agreement between the two techniques for lesions smaller than 3 mm in base and 2 mm in elevation. Conclusions AS-OCT is superior to USB for imaging small lesions pertaining to the anterior iris but USB is better for imaging larger iris lesions with posterior or ciliary body extension.

The effect of intralesional steroid injections on the volume and blood flow in periocular capillary haemangiomas.

Aim: To examine the effect of steroid therapy on the volume estimates and blood flow characteristics of childhood periorbital capillary haemangiomas. Patients and Methods: Children at risk of amblyopia due to periorbital haemangiomas were treated with intralesional steroid injections (between 1 and 4 courses) and serial assessment of the volume and blood—flow characteristics of the lesions measured using colour Doppler ultrasonography. The characteristics of the haemangiomas in these children were compared with a cohort of untreated cases. Results: Eight of nine treated children were female, this proportion being significantly different from the equal sex distribution of an untreated cohort (p < 0.05). All children in the steroid-treated group presented within 1 month of birth, compared to the untreated children, who presented at an average of 2.1 months of age (range 0—14, median 2.9 months) (p = 0.04) and they required significantly longer follow-up in the Orbital service (mean 65 months, range 26—105), compared with an average of 35 months (range 4—92, median 23) in the untreated group (p = 0.002). The maximum estimated volume of the lesions were significantly larger in the treated group (treated group mean 8.9 ml, untreated group mean 4.1 ml; p = 0.016), with a trend towards higher maximum measured blood velocities in the treated group (treated mean 64 cm compared with untreated mean 52 cm; p = 0.1). Steroid injections appear to reduce the volume and blood flow of haemangiomas, this suppression persisting for several months (between 5 and 20) before the lesion later displays the cyclic fluctuations in volume and flow seen with untreated lesions. All treated haemangiomas had some residual vascular anomaly, detectable on ultrasonography, at last follow-up—this being despite absence of clinical signs in most cases. Conclusion: Periorbital capillary haemangiomas requiring steroid therapy for risk of amblyopia were significantly commoner in females, were larger lesions and presented at an earlier age. Intralesional steroids appear to cause a reduction of blood flow, with a transient reduction in volume and a suppression of the natural cyclic variation seen without treatment. The changes after a course of steroid therapy appear to last for between 5 and 20 months, this period of suppression of the lesion probably being particularly useful during infancy and early childhood when the child is at greatest risk of amblyopia.

Ultrasound Imaging of No-Needle 1-Quadrant Sub-Tenon Local Anaesthesia for Cataract Surgery

Objective: To show that direct inferior-nasal 1-quadrant infiltration of local anaesthetic directly to the sub-Tenon space provides anaesthesia for cataract extraction and to assess the distribution of solution. Results: Ultrasound imaging of sub-Tenon delivery to the inferiornasal quadrant reveals rapid movement of solution from this quadrant to the temporal side and posteriorly out of Tenons space to the anterior intra-conal region. Conclusions: This distribution of solution posteriorly out of Tenons space is evidence that initial sub-Tenon irrigation can become anterior intra-conal anaesthesia.

Errors leading to unexpected pseudophakic ametropia.

Purpose Determination of the reasons for clinically significant unplanned ametropia following cataract surgery and the results of management of the ametropia.Methods Retrospective review of 11 consecutive cases of tertiary referral for management of pseudophakic ametropia to the authors. Corrective surgery involved either lens implant exchange or LASIK refractive surgery. Final outcome was assessed by uncorrected and best spectacle corrected visual acuity and manifest refractive outcome.Results Five cases (45%) were due to significant error in axial length determination at pre-surgery biometry. Six cases (55%) were due to surgeon or surgical team error, where the surgeon implanted a lens of power at variance with that specified pre-operatively. Nine patients elected to undergo refractive surgery to correct the ametropia and 2 elected to wear a spectacle lens. Seven underwent lens implant exchange and 2 patients underwent LASIK keratorefractive surgery. Eight of nine patients were within 1 dioptre of intended spherical equivalent after refractive surgery and 1 patients was —1.5 dioptre myopic.Conclusions Most cases of serious unintended ametropia after cataract surgery are avoidable. Care should be taken with the biometry and procedural checks to minimise error. When lens implant exchange or LASIK was performed the final refractive results were satisfactory.

Association of CHRDL1 Mutations and Variants with X-linked Megalocornea, Neuhäuser Syndrome and Central Corneal Thickness

We describe novel CHRDL1 mutations in ten families with X-linked megalocornea (MGC1). Our mutation-positive cohort enabled us to establish ultrasonography as a reliable clinical diagnostic tool to distinguish between MGC1 and primary congenital glaucoma (PCG). Megalocornea is also a feature of Neuhäuser or megalocornea-mental retardation (MMR) syndrome, a rare condition of unknown etiology. In a male patient diagnosed with MMR, we performed targeted and whole exome sequencing (WES) and identified a novel missense mutation in CHRDL1 that accounts for his MGC1 phenotype but not his non-ocular features. This finding suggests that MMR syndrome, in some cases, may be di- or multigenic. MGC1 patients have reduced central corneal thickness (CCT); however no X-linked loci have been associated with CCT, possibly because the majority of genome-wide association studies (GWAS) overlook the X-chromosome. We therefore explored whether variants on the X-chromosome are associated with CCT. We found rs149956316, in intron 6 of CHRDL1, to be the most significantly associated single nucleotide polymorphism (SNP) (p = 6.81×10−6) on the X-chromosome. However, this association was not replicated in a smaller subset of whole genome sequenced samples. This study highlights the importance of including X-chromosome SNP data in GWAS to identify potential loci associated with quantitative traits or disease risk.