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Purpose: To investigate the clinical and histopathological features of congenital fibrovascular pupillary membrane (CFPM) in Chinese patients. Methods: This retrospective study reviewed CFPM cases treated at Beijing Children's Hospital. The clinical manifestations, approaches of treatment, outcomes, and histopathological findings were collected and analyzed. Results: A total of 33 patients with CFPM were reviewed. All patients had unilateral eye involvement. A total of 21 eyes (63.64%) had a white membrane that partially covered the pupil and 12 eyes (36.36%) had a membrane that completely covered the pupil. Of the 12 eyes with a complete pupillary membrane, 6 (50%) had glaucoma. For eyes with a partial pupillary membrane, 11 eyes (52.38%) were followed up at the outpatient clinic without surgery and 10 eyes (47.62%) underwent membranectomy and pupilloplasty due to visual axis blockage. For the 12 eyes with a complete pupillary membrane, 6 eyes (50%) with normal intraocular pressure (IOP) received membranectomy and pupilloplasty combined with iridectomy, and 1 (16.67%) of these 6 eyes underwent a reoperation after 5 months due to a recurrent membrane. Furthermore, 6 eyes (50%) with glaucoma had membranectomy, pupilloplasty, iridectomy, and goniosynechialysis. Among these 6 eyes, 2 eyes (33.33%) underwent a reoperation due to the recurrence of a membrane and 4 eyes (66.67%) had a pale optic disc. The histopathological findings revealed that these membranes were mainly composed of fibrous tissue, lymphocytes, pigment epithelial cells, and vascular tissues. Conclusions: CFPM has diverse manifestations, including a partial pupillary membrane, a complete pupillary membrane with normal IOP, and a complete pupillary membrane with glaucoma. Timely diagnosis and treatment are critical when the visual axis is blocked and/or the IOP is high. [ J Pediatr Ophthalmol Strabismus . 2021;58(2):105–111.]

PURPOSE: To present a case where following Implantable Collamer Lens (ICL) implantation the patient developed toxic anterior segment syndrome (TASS) with a subsequent pupillary block as a consequence of the occlusion of the AquaPORT (STAAR Surgical) flow hole. METHODS: Case report and literature review. RESULTS: A V4c toric ICL (STAAR Surgical) was implanted in the left eye of a 32-year-old woman. After 1 week, the lens was 15° off axis and uneventful lens alignment correction was performed. At postoperative day 1, an intraocular pressure (IOP) of 11 mm Hg and mild corneal edema were observed. At postoperative day 7, there was an uncorrected distance visual acuity of 20/800, an IOP of 44 mm Hg, diffuse corneal edema, and fibrin strands in the anterior chamber. TASS was diagnosed and topical steroids, cycloplegia, antiglaucoma drops, and oral acetazolamide were prescribed. At postoperative day 9, dispersed endothelial pigment with a fixed mid-dilated pupil were identified. Anterior segment optical coherence tomography showed strands of fibrin blocking the central ICL hole and angle closure. Pupillary block related to the fibrin occluding the AquaPORT hole consecutive to TASS was diagnosed. The patient underwent ICL removal, but had a persistent atonic, hyporeflexive pupil as a complication. CONCLUSIONS: This case demonstrates that an AquaPORT hole may not be enough to prevent pupillary block in cases with postoperative intraocular inflammation, causing severe postoperative complications such as Urrets-Zavalia syndrome. Clinicians should consider this diagnosis in cases with corneal edema in the early postoperative period following AquaPORT ICL insertion. [ J Refract Surg . 2020;36(12):856–859.]

The authors describe a challenging case of unilateral retinoblastoma in a patient referred for xanthocoria. A 3-year-old boy was referred for unilateral xanthocoria and disordered retinal vasculature, suggestive of Coats disease. Further investigation revealed diffuse subretinal tumor seeding and areas of calcification, consistent with retinoblastoma. Enucleation was performed and histopathology confirmed exophytic retinoblastoma. This case highlights that xanthocoria, although often encountered in patients with Coats disease, can sometimes be associated with retinoblastoma. As such, retinoblastoma should be considered in the differential diagnosis for children with both leukocoria and xanthocoria. [ J Pediatr Ophthalmol Strabismus. 2022;59(X):e32–e34.]

BACKGROUND AND OBJECTIVE: Pierson syndrome is a rare genetic disease defined by congenital nephrotic syndrome in association with microcoria. The authors aim to describe the posterior segment and retinal features in Pierson syndrome. PATIENTS AND METHODS: A retrospective chart review of nine patients diagnosed with Pierson syndrome was ascertained. Details of ophthalmic history, ocular examination, retinal imaging, and surgical interventions were obtained during a median duration of 17 months of follow-up (range: 6 to 60 months). Retinal interventions included scatter laser photocoagulation and surgical retinal repair. RESULTS: Sixteen eyes of nine patients were included. The axial length of five eyes with flat retina was 26.59 mm ± 0.99 mm. Highly myopic features including tessellated fundus with accompanying optic disc pallor, unidentifiable cup, and abnormal retinal vascular emanation from the disc were observed in all eyes (100%), whereas 12 eyes (75%) had parapapillary chorioretinal atrophy. Features of abnormal retinal vascularization included avascular peripheral retina on fluorescein angiography, aberrant course of the temporal arcades in 13 eyes (81.3%), and straightened nasal retinal blood vessels in 12 eyes (75%). Tortuous retinal blood vessels were observed in three eyes (18.75%). Surgical repair was performed in five out of seven eyes with rhegmatogenous retinal detachment (RRD). Recurrence was observed in all eyes, which required two to three procedures to achieve final reattachment. CONCLUSIONS: Combined features of high axial myopia with incomplete peripheral vascular maturation characterize the posterior segment in Pierson syndrome. Careful posterior segment examination is essential to detect RRD or retinal neovascularization. [ Ophthalmic Surg Lasers Imaging Retina . 2020;51:618–627.]

Myelinated retinal nerve fiber layer is a benign retinal condition that is usually an incidental finding in a healthy patient. Visual acuity is typically not compromised, but some cases have other associated ocular conditions that can affect vision. Diagnosis in challenging atypical cases requires imaging studies to rule out other pathologies. The authors describe the case of a 2-year-old girl who presented with atypical optic nerve fiber myelination. [ J Pediatr Ophthalmol Strabismus . 2020;57:e56–e58.]

A 39-year-old man with Marfan syndrome underwent scleral fixation using a three-piece foldable intraocular lens and experienced repeated pupillary intraocular lens captures secondary to a pliable iris. As a result, the patient needed an additional pupilloplasty. In the opposite eye, a silicone plate haptic intraocular lens was used for scleral fixation because it was thought that the plate design could prevent pupillary intraocular lens capture, even in cases involving a pliable iris. At the 27-month follow-up examination, the patient showed good centration of the intraocular lens in the eye that used the silicone plate haptic intraocular lens without pupillary intraocular lens capture.

BACKGROUND AND OBJECTIVE To investigate the incidence and outcome of optic neuropathy associated with thyroid-related orbitopathy. PATIENTS AND METHODS All patients diagnosed as having optic neuropathy associated with thyroid-related orbitopathy who underwent treatment for optic neuropathy between January 1, 1999, and March 1, 2003, were reviewed. Demographic and clinical data were extracted from the oculoplastic registry (electronic medical record). RESULTS Optic neuropathy occurred in 20 of 595 patients with thyroid-related orbitopathy (3.4%). All patients received systemic steroids, 7 patients received orbital steroid injections, and 2 patients were treated with orbital radiotherapy. Orbital decompression was performed in all 26 orbits. Visual acuity improved from a mean of 20/150 to 20/30 (P < .001). Color vision improved from a mean of 5.2/14 to 11/14 (P = .001). The afferent pupillary defect disappeared shortly after treatment for all but 1 case, and improved in all cases. Exophthalmos decreased from 26.4 ± 2.5 to 21.5 ± 2.1 mm (P < .001; 95% confidence interval, 3.8 to 5.7). CONCLUSIONS Optic neuropathy manifests rarely in patients with thyroid-related orbitopathy. Monitoring visual acuity or afferent pupillary response may be a reliable way of assessing and monitoring optic neuropathy. In this study, patients had improved visual acuity and optic nerve function after a combination of medical and surgical treatment. [Ophthalmic Surg Lasers Imaging 2006;37:284-290.] AUTHORS From the Jules Stein Eye Institute and the Department of Ophthalmology, David Geffen School of Medicine at UCLA, Los Angeles, California. Accepted for publication March 10, 2006. Address reprint requests to Guy J. Ben Simon, MD, Goldschleger Institute, Sheba Medical Center, Tel Hashomer, Israel 52621.

Leukocoria, a presenting sign in several significant pediatric ocular conditions, can be artefactually produced by off-axis flash photography in healthy eyes. The authors demonstrate the conditions needed to produce this phenomenon, which is more commonly seen in children due to their larger-sized pupils, photogenicity, and frequent off-axis shots.

This case report describes the unusual finding of bilateral atonic pupils after cataract extraction by phacoemulsification in an 84-year-old woman with multiple cardiopulmonary risk factors.

The authors present the youngest reported child with proliferative sickle retinopathy. A 3-year, 6-month-old boy presented with leukocoria in the left eye, left esotropia, and dense free-floating white vitreous cells obscuring the fundus, suspicious for endophytic retinoblastoma. Ultrasonography depicted dense debris in the vitreous with no distinct calcific echo or retinal mass. Transcorneal, transzonular fine-needle aspiration biopsy of the vitreous confirmed the presence of dehemoglobinized vitreous red blood cells without tumor. The opposite eye showed peripheral retinal pigment epithelial proliferation and fibrosis with angiographic peripheral ischemia. Hemoglobin electrophoresis disclosed sickle trait (AS). The findings were classified as proliferative sickle cell retinopathy with vitreous hemorrhage in the left eye and non-proliferative sickle cell retinopathy in the right eye. Leukocoria generally raises warnings for retinoblastoma, but can also reflect chronic vitreous hemorrhage.

Reverse pupillary block is introduced as a new cataract-implant complication and as the principle mechanism in pigmentary dispersion syndrome (PDS) and pigmentary glaucoma (PG), causing posterior iris bowing and zonular rubbing. This mechanism was investigated by performing laser iridotomy on six patients. Following surgery, the iris moved forward completely and permanently to the planar position. A new technique, locating the iridotomy by transillumination, reduced the laser energy needed for iridotomy. Laser iridotomy located with transillumination is recommended as the treatment for iris-zonular rubbing in PDS and PG caused by reverse pupillary block.

The authors report a case of reversible tadpole pupil, which occurred during a routine primary uncomplicated bimedial recession in a 2½-year-old child. This case highlights a phenomenon underreported by the ophthalmic community significant in the assessment of depth of anesthesia.

We present a rare case of anterior capsule adherence to the iris following extracapsular cataract extraction with posterior chamber intraocular lens implantation and leading to Pseudophakie pupillary block. There were no synechiae at the pupillary margins associated with the capsule/iris adherence, but aqueous was entrapped behind the iris and intraocular pressure rose. Laser iridotomy was temporarily beneficial, but it had to be repeated several times.