Preeti J. Thyparampil

Interexpert agreement in the identification of macular location in infants at risk for retinopathy of prematurity

OBJECTIVE To characterize variability in the identification of the macular center among retinopathy of prematurity (ROP) experts. METHODS A printed set of 25 wide-angle retinal images was compiled from infants at risk for ROP using a commercially available camera. Ten recognized ROP experts were asked to mark the macular center on each image. For each image, we measured the distance from the optic disc center to the marked macular center. Distances were standardized by normalizing the horizontal optic disc diameter in each image to 0.93 mm. In images with visible peripheral disease, interexpert agreement on the presence of zone I disease was also determined. RESULTS For the image with the least variability among experts, mean (SD) distance from the optic disc to the macular center was 3.69 (0.21) mm (range, 3.13-3.81 mm). For the image with the greatest variability among experts, distance from the optic disc to the macular center was 4.32 (1.19) mm (range, 3.21-7.19 mm). In 7 of 21 images (33%) with visible peripheral disease, there would have been disagreement among experts in the diagnosis of zone I disease based on identification of the macular center. Among the 10 experts, in 17 of 25 images (68%), 1 expert identified the distance between the optic disc and macular center to be greater than 1 SD from the mean. CONCLUSION Significant variability exists among experts in identification of the macular center from wide-angle images, which raises concerns about the reliability of zone I ROP diagnosis.

Plus disease in retinopathy of prematurity: Quantitative analysis of vascular change

PURPOSE To examine the relationship between rate of vascular change and plus disease diagnosis. DESIGN Retrospective observational case-control study. METHODS Wide-angle images were taken bilaterally from 37 infants at 31 to 33 weeks and 35 to 37 weeks postmenstrual age (PMA). The semi-automated Retinal Image multiScale Analysis system was used to measure parameters for all arteries and veins: integrated curvature, diameter, and tortuosity index. A reference standard diagnosis (plus vs not plus) was defined for each eye by consensus of 5 experts at 35 to 37 weeks PMA. Weekly rate of change in parameters was compared in eyes with plus vs not plus disease. Receiver operating characteristic area under the curve (AUC) was calculated for plus disease detection based on 1) weekly rates of parameter change between 31 to 33 weeks and 35 to 37 weeks PMA and 2) parameter values at 35 to 37 weeks only. RESULTS Weekly rates of change in all venous parameters were significantly different in eyes with plus vs not plus disease, particularly for tortuosity index (P < .0004) and diameter (P = .018). Using weekly rate of change, AUC for plus disease detection was highest for venous tortuosity index (0.819) and venous diameter (0.712). Using the 35 to 37-week PMA image only, AUC was highest for venous integrated curvature (0.952) and diameter (0.789). CONCLUSION Rate of change in venous, but not arterial, parameters is correlated with plus disease development in this data set. This did not appear to contribute information beyond analysis of an image at 35 to 37 weeks PMA only.

Cyclic exotropia in a child

Cyclic strabismus is an uncommon disorder in which strabismus alternates with orthotropia in alternating 24-hour periods, although cycles of other lengths have also been described. Alternate-day exotropia is a rare form of cyclic strabismus which, to date, has been reported in few patients. We describe a case of alternate-day exotropia in a child who had previously undergone surgical correction of constant esotropia.

Eyelid lipomas: A case report and review of the literature

Simple lipomas of the eyelid are rare. We present a case of a 61-year-old man, who presented with 6 months of a slowly worsening blepharoptosis. On examination, that patient was noted to have a palpable, soft mass in the medial left upper eyelid. Histopathological examination of the mass revealed mature adipose tissue most consistent with lipoma. Simple lipomas of the eyelid are very unusual but should be considered in the differential diagnosis of patients presenting with mechanical ptosis.

Localized Immunoglobulin Light Chain Amyloid of the Conjunctiva Confirmed by Mass Spectrometry Without Evidence of Systemic Disease

Current guidelines recommend a thorough systemic workup in patients with newly diagnosed immunoglobulin amyloid light chain (AL) amyloidosis because of a close association with an underlying plasma cell proliferative disorder. Rarely, however, AL amyloidosis may present as a localized, benign disease without systemic involvement. Few previous reports have documented localized AL amyloid of the conjunctiva, and all these used immunohistochemistry for amyloid typing, which can be inaccurate. The authors identified 2 cases of AL amyloidosis confirmed by mass spectrometry localized to the conjunctiva and without evidence of systemic disease as determined by thorough systemic workup. Despite the very sensitive diagnostic technique of mass spectrometry, AL amyloidosis of the conjunctiva may not have an association with systemic plasma cell proliferative disorder. It is important to recognize conjunctiva as a site where localized amyloidosis can occur, even with the AL subtypes, as this entity usually exhibits a benign course and may be managed conservatively.

Late-Onset Development of Eyelid Ptosis in Chronic Progressive External Ophthalmoplegia: A 30-Year Follow-up

ABSTRACT Chronic progressive external ophthalmoplegia (CPEO) is a mitochondrial myopathy with slowly progressive, often symmetric blepharoptosis and limitation of ocular motility in all directions of gaze. The authors present an unusual case of CPEO that initially presented with the unique features of markedly asymmetric facial weakness and the lack of blepharoptosis. However, over the subsequent three decades, the patient developed progressive facial and eyelid dysfunction more consistent with a classical description of CPEO. The authors present the 30-year progression of this patient to emphasise the chronic and progressive nature of the condition.

Compressive Optic Neuropathy in Thyroid Eye Disease.

Thyroid eye disease (TED), or Graves ophthalmopathy, is an autoimmune inflammatory condition of the orbit. TED most commonly occurs in the setting of hyperthyroidism (85%) but can also be seen with hypothyroid (10%)or euthyroid states. The pathophysiology is not completely understood but is thought be due to the binding of autoantibodies to orbital fibroblast cells which express thyroid-stimulating hormone receptor (TSHR) or insulin-like growth factor-1 receptor (IGF1R). Activated fibroblasts produce inflammatory molecules and extracellular matrix components, leading to the classic clinical findings of eyelid retraction, proptosis, lagophthalmos, extraocular motility restriction, and optic neuropathy. The severity of TED is often independent of the level of thyroid gland activity. Although the physical effects of TED can lead to varying degrees of visual and ophthalmic morbidity, the most urgent and severe effect is compressive optic neuropathy (CON). CON can result in loss of visual acuity, decreased color vision, and constriction of visual field. If not promptly and appropriately treated, this loss of visual function can become permanent despite subsequent aggressive therapy. The successful management of patients with TED and CON requires careful evaluation for clinical activity, close monitoring for disease progress, and aggressive treatment when loss of visual function is detected.

Immunosuppressant-Related Lower Eyelid Edema in Transplant Patients

Solid organ transplantation is the preferred method of treatment for a number of advanced medical conditions, but it requires systemic immunosuppression to prevent transplant rejection. The authors report 2 unique cases of persistent eyelid edema following solid organ transplantation believed to be related to their systemic immunosuppression. The eyelid findings developed after initiation of the immunosuppressant sirolimus. In 1 patient, the eyelid edema has persisted despite discontinuation of the medication. In the second patient, the immunosuppression could not be altered; therefore, he underwent surgical excision of the edematous lower eyelid. Sirolimus associated eyelid edema is an important medication side effect for ophthalmic and eyelid specialists to consider when a patient with a history of organ transplantation presents with localized noninflamed eyelid edema. This edema can persist despite discontinuation of the medication. Surgical excision of the edematous eyelid can achieve good results.

Primary Neuroendocrine Tumor of the Orbit Presenting With Acute Proptosis

Primary neuroendocrine tumors of the orbit are exceedingly rare and typically present with gradual, progressive exophthalmos. In this report, an otherwise healthy 64-year-old woman undergoes resection of a well-differentiated neuroendocrine tumor after presenting with acute proptosis. An extensive clinical and radiographic evaluation reveals no other evidence of disease, establishing the diagnosis of a primary neuroendocrine tumor. The case presentation is followed by a brief review of the classification, presentation, and evaluation of orbital neuroendocrine tumors.

Changes in Intraocular Pressure During Orbital Floor Fracture Repair

Purpose:To evaluate changes in intraocular pressure (IOP) during orbital floor fracture repair. Methods:Retrospective chart review of patients undergoing isolated orbital floor fracture repair with implant placement at a level 1 trauma center and tertiary care center. Patients with combined zygomaticomaxillary or medial wall fracture repair, or other significant ocular injuries were excluded. IOP measurements using a handheld applanation tonometer were recorded immediately after induction of anesthesia, immediately after orbital floor implant placement, and on postoperative day 1. Results:Nine patients who underwent isolated orbital floor fracture repair were examined. There was a statistically significant mean decrease of 4.66 mmHg (p < 0.05) in IOP from prior to surgical to immediately after implant placement. There was also a significant mean increase in IOP of 7.44 mmHg (p < 0.05) at postoperative day 1 compared with immediately after implant placement. There was no statistically significant difference in IOP between prior to surgical incision and postoperative day 1. Conclusions:All patients who underwent orbital floor fracture repair in this study had a significant decrease in IOP during orbital floor fracture repair that returned to immediately preoperative levels by postoperative day 1. This initial decrease in IOP may be because of intraoperative manipulation of the globe.