Isolated ectopia lentis with partial anterior dislocation and pupillary block: a case report.

BACKGROUND: Ectopia lentis is the dislocation of the natural crystalline lens and usually presents in the setting of trauma or other systemic diseases. Herein, we describe a case of an otherwise healthy four-year-old boy with isolated ectopia lentis whose partial lens dislocation was captured on a smartphone by the patient's father several days prior. CASE PRESENTATION: A four-year-old boy with no past medical, developmental, or trauma history presented with bilateral partial anterior lens dislocation with pupillary block. Initial ophthalmic evaluation two months prior was notable for uncorrected visual acuity at 20/100 OD, 20/250 OS, bilateral iridodenesis, and partially dislocated lenses inferonasally OD and inferiorly OS on slit lamp. Genetic testing found no abnormalities. Ten months later, the patient developed sudden onset of left eye pain. A dislocated lens and temporarily dilated left pupil were captured on a smartphone by the patient's father. He was evaluated 3 days later after a second episode and found to have hand motion vision OS, a fixed 8 mm left pupil with the crystalline lens subluxed into the pupil space and accompanying intraocular pressure OS of 40 mmHg. The lens was surgically removed with a limited anterior vitrectomy. Four and a half years after surgery, visual acuity was 20/125 OS with aphakic correction. The right eye eventually underwent prophylactic lensectomy and was 20/30 in aphakic correction. CONCLUSIONS: This report presents a unique presentation of isolated ectopia lentis with anterior lens dislocation and pupillary block and illustrates the role of smartphone photography in assisting in the triage of eye emergencies.

Reverse pupillary block, in contemporary scleral intraocular lens procedures.

BACKGROUND: To evaluate the frequency and anterior segment optical coherence tomography parameters of patients with scleral fixated intraocular lenses (IOL) and reverse pupillary block (RPB). METHODS: Retrospective analysis at a tertiary care centre (Department for Ophthalmology and Optometry, Medical University of Vienna, Austria). We researched our records for patients who underwent scleral fixated IOL implantation from January 2018 till February 2023. Patients were included only if there was at least one adequate post-operative scan of anterior segment optical coherence tomography (AS-OCT) available. Initially, AS-OCT scans were assessed for IOL tilt and decentration employing a 3D scan and then later for anterior chamber angle (ACA), aqueous anterior chamber depth (AQD), pupil diameter and iris-IOL distance using the 2D scan at a 0° angle. Both an iris-IOL distance of 0 or less and an ACA of more than 70° were required to define an RPB. RESULTS: A total of 110 patients met the inclusion criteria, 41 were treated using the Carlevale, 33 the four flanged, 24 the Yamane and 12 the Scharioth technique, respectively. RPB was found in 32 patients (29%). Twenty patients with RPB were treated using YAG peripheral iridotomy, mean ACA decreased from 91.91° ± 13.77 to 61.02° ± 8.52, (p < 0.001), mean AQD decreased from 4.67 mm ± 0.47 to 4.31 ± 0.36 mm (p < 0.001) and mean iris-IOL distance increased from -0.09 ± 0.04 to 0.33 ± 0.30 (p < 0.001). CONCLUSIONS: RPB is found in a third of eyes who have undergone scleral fixated IOL implantation without iridectomy. YAG peripheral iridotomy is a potent option to treat RPB, and subsequently reduce the risk of iris chafing and secondary inflammation or glaucoma.

A rare case of congenital pupillary abnormality: a case report.

BACKGROUND: Congenital anomalies of the pupil are quite varied, including abnormal size, shape, color, response to stimulus, and function. We are here reporting an unusual case presented with the absence of pupillary opening with folds of iris tissue at the center. Only an extremely small pupil (diameter < 0.5 mm) could be observed during the operation. CASE PRESENTATION: A 15-year-old male patient visited our outpatient clinic due to vision difficulty in his right eye for more than ten years. The best-corrected visual acuity was 2.0 logMAR and 0 logMAR for the right and left eye, respectively. There were amblyopia, astigmatism and constant exotropia in his right eye. Ophthalmic examination of the right eye showed flat iris root, minimal iris pigmentation, and the pupil area was entirely covered by iris tissue. Lens status and fundus evaluation could not be commented. The left eye was found to be within normal limit. Based on ophthalmic examination, the admission diagnosis was given as acorea. Pupilloplasty was performed on the right eye due to the situation that the iris tissue blocked the visual axis, which led to visual impairment and stimulus deprivation amblyopia. However, an extremely small pupil at the center of his pupillary area was observed during the operation. The postoperative course was favorable, and a normal pupil was secured. Hospital discharge diagnosis was given as microcoria, and amblyopia treatment was followed. CONCLUSIONS: We report a rare case of congenital pupillary abnormality. The further diagnosis was given as microcoria, which should be differentiated from acorea. For this kind of pupil disorder which blocks the visual axis, early diagnosis and treatment can help prevent the development of stimulus deprivation amblyopia.

Late Diagnosis of Congenital Toxoplasmosis with Macrocephaly in Dizygotic Twins after Incidental Detection of Leukocoria: A Case Report.

Untreated congenital toxoplasmosis remains an important cause of neurologic and ocular disease worldwide. However, congenitally infected infants may not have signs and symptoms their physicians recognize, leading to delayed diagnosis and missed opportunities for treatment. We describe a pair of twins diagnosed with congenital toxoplasmosis at 11 months of age following incidental detection of leukocoria in one twin.

Case Report: Combined Ischemic Optic Neuropathy and Central Retinal Artery Occlusion after Starting Hemodialysis.

SIGNIFICANCE: This case highlights ocular ischemia after hemodialysis resulting in permanent vision loss. Fifteen percent of the U.S. population suffers from chronic kidney disease. Eye care providers should recommend risk factor modifications to their patients with end-stage renal disease before hemodialysis is started to prevent loss of vision. PURPOSE: The purpose of this study was to demonstrate a case of concurrent nonarteritic anterior ischemic optic neuropathy and central retinal artery occlusion in the setting of hemodialysis initiation. CASE REPORT: A 68-year-old Irish man with end-stage renal disease undergoing dialysis presented, complaining of 3 weeks of progressive vision loss in his left eye. His medical history is complex and includes extensive cardiac disease, bilateral carotid stenosis, and peripheral vascular disease. His surgical history includes a right carotid endarterectomy, bilateral lower extremity amputations, and an aortic valve replacement. Clinical examination revealed light perception vision with an afferent pupillary defect in the left eye and count finger peripheral vision only in the superior temporal quadrant of his vision. The dilated fundus examination showed significant pallid disc edema and focal areas of retina whitening with attenuated peripapillary vasculature in the left eye. This edema was confirmed by optical coherence tomography and supported optic nerve and retinal infarction. A temporal artery biopsy confirmed no evidence of arteritis. CONCLUSIONS: Hemodynamic disruption during dialysis in patients with end-stage renal disease and overlying anemia can result in optic nerve and retinal infarction. Patients who are predisposed to nonarteritic anterior ischemic optic neuropathy or central retinal artery occlusion should be educated on this before starting dialysis to ensure careful blood pressure monitoring.

Autonomic Nervous System Indices in Patients with Systemic Sclerosis without Overt Cardiac Disease.

BACKGROUND: Systemic sclerosis (SSc) is a connective tissue disease that may affect the heart and the autonomic nervous system (ANS). There is little knowledge regarding the degree of ANS involvement in SSc patients with unknown cardiac disease. OBJECTIVES: To evaluate cardiac and pupillary autonomic functions in patients before cardiac involvement has emerged. METHODS: The study comprised 19 patients with SSc and 29 healthy controls. Heart rate variability (HRV) analysis for time and frequency domains, as well as deep breathing test and Ewing maneuvers, were performed in all patients. Automated pupillometry for the evaluation of pupillary diameter and pupillary light reflex was completed in 8 SSc patients and 21 controls. RESULTS: Both groups had similar characteristics, except for medications that were more commonly or solely prescribed for SSc patients. Compared with control subjects, the SSc patients had significantly lower HRV parameters of NN50 (15.8 ± 24.4 vs. 33.9 ± 33.1, P = 0.03), pNN50 (4.9 ± 7.4% vs.10.8 ± 10.8%, P = 0.03), and triangular index (11.7 ± 3.4 vs. 15.7 ± 5.8, P = 0.02). Abnormal adaptive responses in heart rate changes were recorded during deep breathing tests and Ewing maneuvers. There was no significant difference in any of the pupillometric indices or other HRV parameters within groups. CONCLUSIONS: SSc patients may manifest cardiac autonomic dysfunction, while their autonomic pupillary function is seemingly spared. The role of certain medications, the significance of differential organ involvement, as well as the prognostic value of our findings should be evaluated in future studies.

Significant correlations between photopic negative response, afferent pupillary defect, and mean defects of visual fields in asymmetric optic nerve disorders.

PURPOSE: The photopic negative response (PhNR) is a negative wave following the b-wave of the photopic electroretinogram (ERG). The PhNR originates from the retinal ganglion cells (RGCs), and it can be used to assess the function of RGCs noninvasively and objectively. The purpose of this study was to determine whether the relative amplitudes (affected/normal eye) of the PhNR are significantly correlated with the degree of the relative afferent pupillary defect (RAPD) in eyes with unilateral or asymmetrical damage of the optic nerve. METHODS: The PhNRs of the full-field photopic ERGs were measured. In addition, videopupillography and automated perimetry were performed on 27 cases with asymmetrical optic nerve disorders including glaucoma. The differences of these assessments were expressed by the relative amplitudes of the PhNRs of the two eyes, the neutral density (ND) filter required to equate the amplitudes of the pupillary light reflexes between the two eyes, and differences of the mean defects (ΔMDs) of the sensitivities of the Humphrey visual fields. The correlations between these values were determined by linear regression analyses. RESULTS: The relative PhNR amplitudes were significantly and negatively correlated with the ΔMDs (R2 = 0.58, P = 0.0001). In addition, the relative PhNR amplitudes were moderately but significantly and positively correlated with the RAPDs (R2 = 0.36, P = 0.002). CONCLUSION: The relative amplitudes of the PhNR of the affected eyes to the contralateral eyes indicate an asymmetric alteration of the RGCs, and they can be used to monitor the physiology of the RGCs objectively.

Arteritic Orbital Ischemia Producing Afferent and Efferent Pupillary Defects.

A 67-year-old woman presented with acute loss of vision to no light perception (NLP), a right afferent pupillary defect, and anisocoria with a nonreactive and dilated pupil in the right eye. Fundus examination showed pallid optic disc edema and a central retinal artery occlusion (CRAO) in the right eye. A temporal artery biopsy showed giant cell arteritis (GCA). Orbital involvement in GCA has been reported previously. However the combination of an afferent and efferent pupillary defect, NLP vision, pallid disc edema, and a CRAO in an elderly patient is likely a unique clinical combination that should strongly suggest GCA. Clinicians should be aware of the myriad presentations of GCA, including orbital ischemia.

Delayed Orbital Apex Syndrome in the Post-Reconstructed Orbit.

Delayed orbital apex syndrome (OAS) is rare during orbital blowout fracture reconstruction. A 30-year-old woman fractured the right orbital floor in a fall and undergone repairing operation 2 weeks later. After severe sneezing on postoperative day 10, she gradually arose vision loss, ophthalmoplegia, ptosis with a dilated and fixed pupil within few hours, then consulted our department and was diagnosed as OAS. Computed tomography scan showed displaced implant and retrobulbar emphysema resulting in a constellation of compression to orbital apex. Therefore, an exploratory operation was engaged to reposition the implant and reduce the emphysema concurrent with mega-dose steroids. The patient regained vision immediately and resolved all symptoms at the 6 months follow-up.

Radiation-Induced Optic Neuropathy: Clinical and Imaging Profile of Twelve Patients.

BACKGROUND: Radiation-induced optic neuropathy (RON) is a form of delayed radionecrosis of the anterior visual pathways, which develops within months to years after external cranial irradiation and causes severe and irreversible vision loss. Small series reports have adequately documented its clinical features, but imaging characteristics have been less completely described. METHODS: We accrued cases from the University of Michigan Neuro-Ophthalmology Clinic files and from cases coded as "radiation optic neuropathy" at the University of Michigan Medical Center between 1994 and 2017. All patients had undergone 3D-conformal linear accelerator (photon) external beam radiation. We collected clinical details of vision loss, including the temporal relationship to radiation. A single neuroradiologist (E.A.L.) evaluated all available magnetic resonance imaging (MRI) studies, noting the presence of enhancement, expansion, or volume loss of the optic nerves or chiasm, corresponding T2 signal abnormalities, and the absence of demyelination or confounding compressive lesions. RESULTS: Twelve patients (15 eyes) met inclusion criteria. Vision loss was usually monocular at outset, but both optic nerves were eventually involved in 3 (25%) patients. Although usually sudden in onset, vision loss often declined slowly over many months, frequently to finger counting, or worse without recovery. An afferent pupillary defect was always present at the time of presentation. Most affected optic discs were pale at the time of first visual symptoms, indicating that subclinical optic nerve damage had been present for several weeks. The latency from completion of radiation to onset of vision loss ranged from 7 to 48 months (average: 18 months). In 2 patients, radiation was delivered to the whole brain, rather than being limited to the anterior visual pathway. MRI typically displayed a discrete region of enhancement of the affected prechiasmatic optic nerve, often with expansion and high T2 signal in the enhancing segment. In 3 affected eyes, enhancement was apparent on imaging completed 3-6 weeks before the onset of vision loss. In one patient, segmental prechiasmatic enhancement became evident only on repeat MRI completed 7 months after vision loss. The duration of enhancement among 9 eyes with follow-up MRIs was at least 2 months, but in one case, enhancement was still present on a study performed 17 months after treatment. CONCLUSIONS: This study further delineates the profile of RON. Visual loss is often acute, profound, and monocular but may decline slowly after acute onset and later affect both optic nerves. High-resolution MRI of the optic nerves usually will display enhancement of a discrete segment of the intracranial prechiasmatic optic nerve, often with accompanying expansion and T2 hyperintensity. In some cases, these imaging features may precede vision loss. They may be subtle or appear after vision loss. Enhancement lingers for a wide interval, ranging in this study from 2 to at least 17 months. Recognition of these imaging characteristics assists in confirmation of the diagnosis of RON.

Clinical Determination of Brain Death in Children Supported by Extracorporeal Membrane Oxygenation.

BACKGROUND/OBJECTIVE: Children supported by extracorporeal membrane oxygenation (ECMO) are at risk of catastrophic neurologic injury and brain death. Timely determination of brain death is important for minimizing psychological distress for families, resource allocation, and organ donation. Reports of successful determination of brain death in pediatric patients supported by ECMO are limited. The determination of brain death by clinical criteria requires apnea testing, which has historically been viewed as challenging in patients supported by ECMO. We report eight pediatric patients who underwent a total of 14 brain death examinations, including apnea testing, while supported by veno-arterial ECMO (VA-ECMO), resulting in six cases of clinical determination of brain death. METHODS: We performed a retrospective review of the medical records of pediatric patients who underwent brain death examination while supported by VA-ECMO between 2010 and 2018 at a single tertiary care children's hospital. RESULTS: Eight patients underwent brain death examination, including apnea testing, while supported by VA-ECMO. Six patients met criteria for brain death, while two had withdrawal of technical support after the first examination. During the majority of apnea tests (n = 13/14), the ECMO circuit was modified to achieve hypercarbia while maintaining oxygenation and hemodynamic stability. The sweep flow was decreased prior to apnea testing in ten brain death examinations, carbon dioxide was added to the circuit during three examinations, and ECMO pump flows were increased in response to hypotension during two examinations. CONCLUSIONS: Clinical determination of brain death, including apnea testing, can be performed in pediatric patients supported by ECMO. The ECMO circuit can be effectively modified during apnea testing to achieve a timely rise in carbon dioxide while maintaining oxygenation and hemodynamic stability.

Reverse Pupillary Block After Retinal Detachment Surgery in an Eye with Toric Implantable Collamer Lens.

PURPOSE: To report an interesting phenomenon, after vitreoretinal surgery, in an eye with toric implantable collamer lens (ICL). METHODS: A 20-year-old male presented with raised intraocular pressure (IOP) after the retinal detachment surgery in the left eye. He had toric ICL in both the eyes, and left eye showed shallow peripheral anterior chamber depth (ACD), narrow iridocorneal angle (ICA), increased ICL vault and pigments debris blocking central hole. After dilation, peripheral ACD deepened, ICL vault decreased and ICA increased, with posterior bowing of iris. RESULTS: Patient was managed with topical steroids and antiglaucoma medications. A month later, after inflammation subsided, central hole of ICL became patent, peripheral ACD deepened, ICA increased and IOP was normal without medications, however, after dilation, posterior bowing of iris persisted. He was speculated to have unusual presentation of reverse pupillary block in the left eye after mydriasis. This phenomenon was not observed in the right eye. CONCLUSION: Reverse pupillary block, after dilation is an unusual phenomenon, which was seen in a silicone-filled eye with toric ICL implant. In cases of inflammatory debris blocking Aquaport opening of ICL, conservative management with topical steroids and antiglaucoma medications can help reduce inflammation and IOP, alleviating need for any intervention, such as laser iridotomy.

Advanced glaucoma following Nd:YAG capsulotomy: a case report.

BACKGROUND: We present a case of aphakic pupil block caused by vitreous prolapse into the anterior chamber following Nd:YAG capsulotomy. CASE PRESENTATION: This resulted in advanced glaucoma in a young patient, which presented a significant clinical management challenge. CONCLUSIONS: Ultimately, at the time of writing, her intraocular pressure and uveitis were well controlled, however the long-term outcome remains uncertain, given the uncompromising natural history of her complicated ocular condition.

Primary Assessment of the Patient With Orbital Fractures Should Include Pupillary Response and Visual Acuity Changes to Detect Occult Major Ocular Injuries.

PURPOSE: Sight-threatening injuries associated with orbital fractures are of major concern to maxillofacial surgeons whom are often the first asked to assess these patients. Eliciting signs and symptoms that are predictive of these injuries would allow expedited ophthalmic consultation and appropriate management. We hypothesized that abnormal pupillary response is predictive of major ocular injuries. PATIENTS AND METHODS: A retrospective cohort study of patients with facial fractures was instituted with review of all associated ophthalmic injuries. The primary predictor variables were the presence or absence of post-traumatic ocular symptoms and signs (visual acuity change, diplopia, flashes and floaters, pain on globe movement, abnormal pupillary response, restriction of eye movement, and visual field defects). Secondary predictors were pattern of fracture and mechanism of fracture. The primary outcome variable was the presence or absence of major ocular injury assessed during formal ophthalmology consultation. Descriptive statistics were calculated as categorical values. Correlation between the presence or absence of predictors and outcome (major ocular injury) was calculated using χ2 analysis, with the significance value set at P ≤ .01. RESULTS: The study included 75 patients (25% of whom were female patients) with a mean age of 41 ± 22 years. We recorded 165 minor ocular injuries and 43 major ocular injuries. The mechanisms of injury included assault (48%, n = 36), motor vehicle accident (21%, n = 16), fall (17%, n = 13), sport (11%, n = 8), and occupational (3%, n = 2). The fracture pattern included zygomaticomaxillary (36%, n = 27), isolated orbital floor (25%, n = 19), complex (20%, n = 15), and isolated orbital nonfloor (19%, n = 14). Of the primary outcome predictors, only abnormal pupillary response (odds ratio, 36; P < .001) and subjective visual acuity changes (odds ratio, 10; P < .001) were predictive of major ocular injury. The mechanism of injury and pattern of fracture were not predictive of major ocular injury. CONCLUSIONS: During primary assessment of the patient with orbital fractures, abnormal pupillary response and subjective visual acuity changes are key predictors of occult major ocular injury.

Is Tadpole Pupil in an Adolescent Girl Caused by Denervation Hypersensitivity?

Tadpole pupil is a rarely encountered phenomenon caused by episodic, segmental iris dilator muscle spasm of short duration (2-15 minutes), occurring in clusters without a known precipitating factor. It has most commonly been described in women aged 28 to 48 years. A few hypotheses on pathogenesis have been discussed but none has been proved. Here, we present an adolescent girl with bilateral tadpole pupil that appeared during physical exercise. This is the first pediatric case of tadpole pupil, not caused by preceding surgery, to be published. Based on (1) this case in which tadpole pupil developed when the norepinephrine level rose during exercise, (2) the high ratio of patients with tadpole pupil who concurrently have or later develop Horner syndrome, in which denervation hypersensitivity is well described, (3) a previous report of a patient with both tadpole pupil and Horner syndrome who had denervation hypersensitivity on pharmacological testing, (4) a 29-year-old man with unilateral tadpole pupil induced by exercise, and (5) a 19-year-old man with bilateral tadpole pupil and possible autonomic neuropathy, we suggest denervation hypersensitivity as a probable pathogenic mechanism causing tadpole pupil. In addition, a suggestion for investigations to be performed in future pediatric cases is provided.

Reverse pupillary block after implantation of a scleral-sutured posterior chamber intraocular lens: a retrospective, open study.

BACKGROUND: To report the clinical features of patients with reverse pupillary block (RPB) after scleral-sutured posterior chamber intraocular lens (PC IOL) implantation and biometric changes after laser peripheral iridotomy (LPI). METHODS: Eight patients attending our hospital's ophthalmology outpatient clinic, who developed RPB after implantation of a scleral-sutured PC IOL due to subluxation of the crystalline lens or IOL, were investigated in this retrospective, observational study. RESULTS: Preoperative evaluations showed angle pigmentation in all cases and iridodonesis in 2 cases. Two subjects had used an α1A-adrenoceptor antagonist for benign prostatic hyperplasia. Pars plana or anterior partial vitrectomy was performed in all cases. All eyes showed an extremely deep anterior chamber, a concave iris configuration, and contact between the IOL optic and the iris at the pupillary margin. Pupil capture was detected in 2 cases. The mean (± SD) anterior chamber angle (ACA) was 89.91 ± 10.06°, and the anterior chamber depth (ACD) was 4.42 ± 0.16 mm before LPI. After LPI, the iris immediately became flat with a decreased ACA (51.70 ± 2.59°; P = 0.018) and ACD (4.14 ± 0.15 mm; P = 0.012). After LPI, the intraocular pressure decreased from 19.75 ± 3.77 mmHg to 15.63 ± 4.30 mmHg (P = 0.011), and the spherical equivalent decreased from -0.643 ± 0.385 D to - 0.875 ± 0.505 D (P = 0.016). CONCLUSION: Concomitant vitrectomy, angle pigmentation, and iridodonesis may be risk factors for RPB after scleral-sutured PC IOL implantation. LPI is effective for relieving the RPB.

Identification of Clinically Relevant Groups of Patients Through the Application of Cluster Analysis to a Complex Traumatic Brain Injury Data Set.

In neurological intensive care units (NICUs) we are collecting an ever increasing quantity of data. These range from patient demographics and physiological monitoring to treatment strategies and outcomes. The BrainIT database is an example of this type of rich data source. It contains validated data on 264 patients who suffered traumatic brain injury (TBI) admitted to 22 NICUs in 11 European countries between March 2003 and July 2005 [1, 6].