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Purpose of Review: The goal of this article is to review the anatomy and physiology of pupillary function and then employ that information to develop a comprehensive framework for understanding and diagnosing pupillary disorders., Recent Findings: The contribution of rods and cones to the pupillary light reflex has long been known. A third photosensitive cell type, the intrinsically photosensitive retinal ganglion cell, has recently been discovered. This cell type employs melanopsin to mediate a portion of the pupillary light reflex independent of rods and cones (the postillumination pupillary response) and photic regulation of circadian rhythm., Summary: The autonomic nervous system regulates pupil size in response to stimuli. The parasympathetic nervous system causes miosis in response to light and near visual stimuli. These stimuli activate supranuclear pathways that project to the Edinger-Westphal nuclei. The sympathetic nervous system causes mydriasis in response to a variety of arousing factors, both physiologic (wakefulness) and pathologic (pain). Abnormalities of physiologic function cause disturbances of pupil size, shape, and response to stimuli. The clinical approach to pupillary abnormalities should focus on the clinical and pharmacologic assessment of the pupil's expected response to diverse stimuli.

Competing Interests: There are no conflicts of interest.

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 α 1A -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.

A 2-month-old boy presented with an irregular left pupil. Over the course of 3 months, progressive pupil ovalization, corectopia, and ballooning of the thinned superior iris tissue caused obstruction of the visual axis. Because of concern for deprivational amblyopia, a sectoral pupilloplasty and sphincterotomies were performed, restoring pupil shape and clearing the visual axis. On postoperative examinations, the pupil remained round, the visual axis clear, and visual acuity of the left eye improved. Findings and clinical course suggested the diagnosis of idiopathic tractional corectopia, a rare congenital disorder of unknown cause that may lead to isolated unilateral progressive corectopia and visual axis obstruction. Patients should receive regular follow-up examinations., (Copyright © 2016 American Association for Pediatric Ophthalmology and Strabismus. Published by Elsevier Inc. All rights reserved.)

Frequently, in literature and curent practice, accessory iris membrane (AIM) and persistant pupillary membrane (PPM) are confused. Both AIM and PPM are congenital iris anomalies in which fine or thick iris strands arrise form the collarette and obscure the pupil. AIM, which is also called iris duplication, closely resembles the normal iris tissue in color and thickness and presents a virtual second pseudopupil aperture in the centre while PPM even in its extreme forms presents as a translucent or opaque membranous structure that extends across the pupil and has no pseudopupil. Mydriatiscs, laser treatment or surgery is used to clear the visual axis and optimize visual development. Surgical intervention is reserved for large, dense AIMs and PPMs. Our patient, a 29 year old male, has come with bilateral dense AIM, bilateral compound hyperopic astigmatism, BCVA OD = 0.6, BCVA OS = 0.4, IOP OU = 17 mmHg. To improve the visual acuity of the patient we decided to do a bilateral membranectomy, restoring in this way transparency of the visual axis. After surgery, the visual acuity improved to BCVA OD= 0.8, BCVA OS=0.8.

Purpose: A test of validation study was conducted to investigate the use of a novel computerized portable pupillometer to detect and quantify relative afferent pupillary defects (RAPDs)., Materials and Methods: Binocular pupillary response curves were recorded in patients with RAPDs clinically graded by an examiner (n = 32) and in normal subjects (n = 31) with RAPDs simulated using quantifiable dimmed light intensities. In 14 normal subjects, testing was repeated within two months. Pupillary constriction amplitude (CA), velocity (CV) and onset latency (COL) were used to calculate RAPDs., Results: RAPDs in normal subjects were 0.16 ± 0.12 log units (LU) (range = 0-0.38). In retested normals, inter-visit variability was 0.21 ± 0.12 LU (range = 0-0.42). Significant correlation was found between RAPD values and dimmed light intensity in normal subjects (Pearson's r = 0.87, p < 0.0001) and between clinician and pupillometer grading of RAPDs in patients (r = 0.81, p < 0.0001). Using the upper limit of the one-sided 95% confidence interval (CI) of the ratio of percentage change in CA (LU) as determined from normals, 21/23 (91%) patients with RAPDs ≥ 0.5 LU were distinguished from normals. The area under the receiver operating characteristic curve for distinguishing RAPDs ≥ 0.5 LU was 0.98 (95% CI = 0.95-1.00). RAPDs calculated using CA and CV correlated more strongly with the clinician's grading compared to COL (Steiger's test p < 0.0001)., Conclusions: This novel pupillometer correlated strongly with an expert examiner's clinical grading of RAPDs and detected clinically significant RAPDs with high sensitivity and specificity, suggesting it may have a prominent role as an objective clinical tool in the screening of patients with vision loss.

Background: Infants with hyperplastic persistent pupillary membranes (PPM) may be at risk for deprivation amblyopia due to obstructions of the visual axis. We describe the long-term visual and anatomic outcomes of a surgical technique for their removal., Methods: The medical records of consecutive patients <3 years of age who underwent surgical removal of PPMs between December 1998 and May 2012 were retrospectively reviewed. Each PPM was judged to be visually significant based on poor visual acuity, poor retinoscopic reflex, or inability to visualize the fundus. The surgical technique included injection of a viscoelastic agent beneath the pupillary strands to bow them anteriorly, careful peeling of residual adherent strands from the anterior lens capsule, and lysis of the strands at the pupillary margin with intraocular scissors. Pre- and postoperative visual and anatomic results were recorded., Results: This case series included 10 eyes of 6 patients: PPMs were bilateral in 4 patients and unilateral in 2. The patient age at time of surgery ranged from 2.5 months to 2.5 years (mean, 14 months). Mean postoperative follow-up was 5.3 years (range, 2.5-8 years). All patients had successful clearance of the visual axis and good visual acuity. No intraoperative complications occurred., Conclusions: All patients in this series had excellent visual and structural outcomes, with no significant complications. The technique described here may be considered for patients with visually significant PPMs to improve visual function and pupil appearance., (Copyright © 2014 American Association for Pediatric Ophthalmology and Strabismus. Published by Elsevier Inc. All rights reserved.)

Purpose: To report a case series of pupil abnormalities consistent with features of Urrets-Zavalia syndrome (UZS) after Descemet stripping automated endothelial keratoplasty (DSAEK) for corneal edema secondary to corneal endothelial cell dysfunction., Methods: Retrospective chart analysis of subjects who developed UZS after DSAEK at the University of Texas Southwestern Medical Center., Results: We present a series of 7 eyes with features consistent with UZS, after undergoing DSAEK. Elevated intraocular pressures (IOP) were noted in the early postoperative period in all cases. Five of 7 had graft dislocation in the postoperative period and required rebubbling or repeat DSAEK to obtain a well-apposed graft. Patients were followed for 3 to 14 months and showed improvement in visual acuity and IOP, but fixed dilated pupils persisted., Conclusion: A fixed irregular or dilated pupil is a rare complication that can be associated with DSAEK surgery. Patients with an elevated IOP and complicated postoperative course seem to be at greater risk for developing iris ischemia and pupil abnormalities consistent with the diagnosis of UZS.

Background and Objective: To investigate near infrared iris transillumination (NIRit) imaging as a new method to quantify pupil shape, size, and position because the imaging modality can uniquely provide simultaneous information regarding iris structural details that influence pupil characteristics and because exploration of related techniques could promote discovery helpful to clinical research and care., Patients and Methods: Digital NIRit images of normal and diseased eyes were used along with computer-assisted techniques to quantify four primary pupil parameters, including pupil roundness (PR), pupil ovalness (PO), pupil size (PS), and pupil eccentricity (PE). A combined measure of PR and PO was also developed (the pupil circularity index [PCI]). Repeatability of the measures was studied and example analyses were performed., Results: Pupil measures could be calculated for right eyes of 307 subjects (164 normal, 143 other), with fewer than 0.5% exclusions due to image quality. Repeatability study did not show significant bias (P < .05) for any of the four primary measures. Example analyses could show age-associated differences in pupil shape (≥ 50 year olds had less regular pupils than < 50 year olds: median PCI = 0.009 vs 0.006; P < .01) and that a group of pigment dispersion syndrome subjects (n = 27) had less regular pupils than a group of matched controls (PO = 0.9966 vs 0.9990; P < .05)., Conclusion: Digital NIRit imaging can provide novel, reliable, and informative methods to quantify pupil characteristics while providing simultaneous information about iris structure that may influence these parameters., (Copyright 2012, SLACK Incorporated.)

Purpose: To examine the effect of the posterior location of the dilator on iris anterior curvature during dilation., Methods: An in vivo human study, an ex vivo porcine experiment, and an in silico computational model were performed in parallel. Iris anterior curvature was measured in vivo before and after dilation by time-domain slit lamp optical coherence tomography (SL-OCT). All patients (n = 7) had undergone laser peripheral iridotomy to eliminate any pupillary block due to primary angle-closure glaucoma. In the ex vivo experiments, isolated porcine irides (n = 30) were secured at the periphery and immersed in an oxygenated Krebs-Ringer buffer. Dilation was induced pharmaceutically by the addition of 2.5% phenylephrine and 1% tropicamide. An in-house optical coherence tomography (OCT) system was used to obtain iris images before and after dilation. A finite element model was also developed based on typical geometry of the iris from the initial OCT image. The iris was modeled as a neo-Hookean solid, and the active muscle component was applied only to the region specified as the dilator., Results: An increase in curvature and a decrease in chord length after dilation were observed in both experiments. In both the in vivo and ex vivo experiments, the curvature-to-chord length ratio increased significantly during dilation. Computer simulations agreed well with the experimental results only when the proper anatomic position of dilator was used., Conclusions: The posterior location of the dilator contributes to the anterior iris bowing via a nonpupillary block dependent mechanism.

Assessing pupil reactivity using the swinging flashlight technique in patients with miotic pupils or heavily pigmented irides can be difficult due to the reflection of the examination light off the cornea. We describe a modification of the standard method of detecting pupil reactivity in these difficult-to-see pupils. With the patient facing forward and eyes looking in maximal upgaze, fixed at distance, the examiner shines the light at the limbus in the straight ahead direction. Improved view of the pupil in upgaze results from reduction of the blink reflex and elimination of the Purkinje-1 image with minimal refractive distortion by the cornea. Optical calculations and multimedia demonstrations are provided to document these findings.

Examination of the pupil offers an objective evaluation of visual function as well as the vegetative pathways to the eye. Essential information is gathered within a short time. This makes pupillary inspection a valuable part of the routine ophthalmological, neurological and general medical examinations. Due to the proximity of pupillary pathways to various anatomic structures, pupillary dysfunction can be caused by a variety of disorders, some of which may be life threatening. The ophthalmologist plays a key role in detecting pupillary disorders and in directing further investigations. Therefore, one should have a good knowledge of the diagnostic significance of pupillary function and dysfunction., (© Georg Thieme Verlag KG Stuttgart · New York.)

Pupillometry is a non-invasive technique, based on well-established neurophysiologic principles, that can be utilized to objectively characterize pathophysiologic demyelinating and neurodegenerative changes involving the pupillary reflex pathway. In animal models of human disorders, pupillometry derived reflex metrics could potentially be used to longitudinally monitor disease activity and responses to pharmacotherapies. These investigations would have important implications for translational initiatives focused on the identification and application of novel neuroprotective and restorative treatments for human diseases such as multiple sclerosis. Here, we have established normal reference values for various pupillary reflex metrics across different mouse strains. Ultimately, we anticipate that this new data will help to catalyze unique lines of inquiry using pupillometry methods.

Congenital fibrovascular pupillary membrane, rare in newborns, poses challenges for the ophthalmic surgeon. Predicting its clinical course is difficult, as is removing the membrane surgically without inducing a cataract. We describe a 22-day-old girl who presented with a pupillary membrane causing progressive decrease in the size of the dilated pupil, necessitating removal at 4 months of age. Histopathology demonstrated tissue of embryonic muscle origin, suggesting that the membrane was more likely a remnant of fetal iris than of fetal vasculature. The smooth muscle or fibroblastic elements in the tissue explains progressive contracture of the membrane.

Purpose: To study the ocular phenotype of Pierson syndrome and to increase awareness among ophthalmologists of the diagnostic features of this condition., Design: Retrospective, observational case series., Methods: A multicenter study of 17 patients with molecularly confirmed Pierson syndrome. The eye findings were reviewed and compared to pertinent findings from the literature., Results: The most characteristic ocular anomaly was microcoria. A wide range of additional abnormalities were found, including posterior embryotoxon, megalocornea, iris hypoplasia, cataract, abnormal lens shape, posterior lenticonus, persistent fetal vasculature, retinal detachment, variable axial lengths, and glaucoma. There was high interocular and intrafamilial variability., Conclusions: Loss-of-function mutations in laminin beta2 (LAMB2) cause a broad range of ocular pathology, emphasizing the importance of laminin beta2 in eye development. Patients with Pierson syndrome can initially present with ocular signs alone. In newborns with marked bilateral microcoria, Pierson syndrome should be considered and renal function investigated.

We report 3 siblings from a nonconsanguineous white family with bilateral persistent pupillary membranes with dissimilar forms of severity. Two of the patients required no treatment; 1 was treated surgically for occlusion of the pupillary axis and moderate visual acuity decrease. Anterior segment examination was otherwise normal in the siblings. Although persistent pupillary membranes are usually sporadic, cases in which they are associated with other anterior chamber abnormalities might be inherited autosomal dominant. To our knowledge, this is the first report of a familial isolated persistent papillary membrane case with no other anterior chamber abnormality.

We report a case of a 14-year-old girl who presented with impaired vision in the left eye since birth. Examination showed extensive bilateral persistent pupillary membranes associated with microcornea, cornea plana, and central localized posterior keratoconus in both eyes. The uncorrected visual acuity was 0.2 (logMAR) (20/32) in the right eye and 1.9 (logMAR) (20/1300) in the left eye, with no improvement with pinhole. Surgical excision of the membranes was done in both eyes using vitreous microscissors. At 6 months, the best corrected visual acuity was 0.1 (logMAR) (20/25) in the right eye and 1.3 (logMAR) (20/400) in the left eye. No significant intraoperative or postoperative complications were noted.

Although diabetic autonomic neuropathy involves most organs, diagnosis is largely based on cardiovascular tests. Light reflex pupillography (LRP) non-invasively evaluates pupillary autonomic function. We tested whether LRP demonstrates autonomic pupillary dysfunction in diabetics independently from cardiac autonomic neuropathy (CAN) or peripheral neuropathy (PN). In 36 type-II diabetics (39-84 years) and 36 controls (35-78 years), we performed LRP. We determined diameter (PD), early and late re-dilation velocities (DV) as sympathetic parameters and reflex amplitude (RA) and constriction velocity (CV) as parasympathetic pupillary indices. We assessed the frequency of CAN using heart rate variability tests and evaluated the frequency of PN using neurological examination, nerve conduction studies, thermal and vibratory threshold determination. Twenty-eight (77.8%) patients had abnormal pupillography results, but only 20 patients (56%) had signs of PN or CAN. In nine patients with PN, only pupillography identified autonomic neuropathy. Four patients had pupillary dysfunction but no CAN or PN. In comparison to controls, patients had reduced PD, late DV, RA and CV indicating sympathetic and parasympathetic dysfunction. The incidence and severity of pupillary abnormalities did not differ between patients with and without CAN or PN. LRP demonstrates sympathetic and parasympathetic pupillary dysfunction independently from PN or CAN and thus refines the diagnosis of autonomic neuropathy in type-II diabetics.

Seventy-seven diabetics with a duration of the disease ranging from 2 to 55 years (average 18.5 years) were studied with infrared videopupillometry. The prevalence of diabetic autonomic neuropathy at the pupillary control system (pANP) was studied comparatively using several pupillary tests. The average prevalence using age-dependent parameters was 30.2% [maximal pupillary area: 22.1%, maximal contraction velocity: 24.7%, contraction velocity at 1 s (CV1): 28.6%, and dilation velocity at 6 s (DV6): 45.5%]. Comparing these percentages to prevalences of other diabetic late complications, e.g. retinopathy (49.4%), DV6 seems to be good for the diagnosis of pANP. If CV1 and DV6 are expressed in percent of the maximal pupillary area (CV1% and DV6%), they become age-independent. The average CV1% and DV6% of diabetics differ highly significantly from those of normals (CV1%: 58.6 +/- 14.5 vs. 64.1 +/- 6.4%, 2 p less than 0.005, and DV6%: 6.0 +/- 2.9 vs. 7.3 +/- 1.1%, 2 p less than 0.001). The average prevalence of pANP using these age-independent parameters was 25.4%. These data suggest that the prevalence of pANP, especially disorders of pupillary dilation (DV6), is high in long-standing diabetes. Furthermore, CV1% and DV6% have proved to be valid parameters in finding differences in the light reflex in non-age-matched study groups.

Two cases are reported of optic tract lesions with complete homonymous hemianopia, characteristic optic atrophy and retinal nerve fiber bundle defect, and relative afferent pupillary defect (RAPD). RAPD was 0.6 log units in both cases. Infrared pupillography revealed pupillary light response of the ipsilateral side to be 1.2 or 1.3 times higher than that of the contralateral side. We confirmed that the detection of RAPD is helpful in differentiating optic tract lesions from suprageniculate lesions in cases of complete homonymous hemianopia.

The purpose of the study was to examine vegetative innervation of the pupil in different diseases associated and not associated with vegetative disorders. In addition to the ++clinico-neurological method, the main method of examination was to study the pupillary cycle (determination of the time of the pupillary cycle (TPC) with the aid of a slit lamp). Fifty healthy test subjects and 262 patients suffering from different somatic and neurologic diseases were examined. The patients were divided in 9 clinical groups: spinal amyotrophy, neural Charcot-Marie amyotrophy, Guillain-Barre syndrome, diabetes mellitus, thyrotoxicosis, hemicrania, Raynaud's disease, idiopathic hyperhidrosis, vegetative crises. The data obtained demonstrate varying grades of the rise of the TPC in the indicated diseases, which reflects varying degrees of vegetative innervation of the pupil insufficiency. However, no well-defined correlation was obtained between the intensity of the psychovegetative syndrome and the TPC. Therefore, it has been shown that the method of determining the TPC is unsophisticated and noninvasive and can be successfully used in different forms of pathology to identify vegetative insufficiency at different stages of the disease.