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Purpose Smooth muscle dysfunction syndrome (SMDS) due to heterozygous ACTA2 arginine 179 alterations is characterized by patent ductus arteriosus, vasculopathy (aneurysm and occlusive lesions), pulmonary arterial hypertension, and other complications in smooth muscle–dependent organs. We sought to define the clinical history of SMDS to develop recommendations for evaluation and management. Methods Medical records of 33 patients with SMDS (median age 12 years) were abstracted and analyzed. Results All patients had congenital mydriasis and related pupillary abnormalities at birth and presented in infancy with a patent ductus arteriosus or aortopulmonary window. Patients had cerebrovascular disease characterized by small vessel disease (hyperintense periventricular white matter lesions; 95%), intracranial artery stenosis (77%), ischemic strokes (27%), and seizures (18%). Twelve (36%) patients had thoracic aortic aneurysm repair or dissection at median age of 14 years and aortic disease was fully penetrant by the age of 25 years. Three (9%) patients had axillary artery aneurysms complicated by thromboembolic episodes. Nine patients died between the ages of 0.5 and 32 years due to aortic, pulmonary, or stroke complications, or unknown causes. Conclusion Based on these data, recommendations are provided for the surveillance and management of SMDS to help prevent early-onset life-threatening complications.

Several studies have demonstrated that chewing can be regarded as a preventive measure for cognitive impairment, whereas masticatory deficiency, associated with soft-diet feeding, is a risk factor for the development of dementia. At present the link between orofacial sensorimotor activity and cognitive functions is unknown. In subjects with unilateral molar loss we have shown asymmetries in both pupil size and masticatory muscles electromyographic (EMG) activity during clenching: the molar less side was characterized by a lower EMG activity and a smaller pupil. Since implant-prostheses, greatly reduced both the asymmetry in EMG activity and in pupil's size, trigeminal unbalance, leading to unbalance in the activity of the Locus Coeruleus (LC), may be responsible for the pupil's asymmetry. According to the findings obtained in animal models, we propose that the different activity of the right and left LC may induce an asymmetry in brain activity, thus leading to cognitive impairment. According to this hypothesis, prostheses improved the performance in a complex sensorimotor task and increased the mydriasis associated with haptic tasks. In conclusion, the present study indicates that the implant-prosthesis therapy, which reduces the unbalance of trigeminal proprioceptive afferents and the asymmetry in pupil's size, may improve arousal, boosting performance in a complex sensorimotor task.

Ocular complications of adenotonsillectomy are rare. The authors describe a 6-year-old boy who developed mydrasis and limitations of supraduction and infraduction after adenotonsillectomy. This was attributed to the hemorrhagic compression of the nerve in the cavernous sinus. This is the first report of pupil-involving oculomotor nerve palsy following adenotonsillectomy. [J Pediatr Ophthalmol Strabismus. 2019;56:e76–e78.]

Rationale Pupillometry can be used to characterize autonomic drug effects. Objective This study was conducted to determine the autonomic effects of 3,4-methylenedioxymethamphetamine (MDMA, ecstasy), administered alone and after pretreatment with reboxetine, duloxetine, clonidine, carvedilol, and doxazosin, on pupillary function. Methods Infrared pupillometry was performed in five placebo-controlled randomized studies. Each study included 16 healthy subjects (eight men, eight women) who received placebo–MDMA (125 mg), placebo–placebo, pretreatment–placebo, or pretreatment–MDMA using a crossover design. Results MDMA produced mydriasis, prolonged the latency, reduced the response to light, and shortened the recovery time. The impaired reflex response was associated with subjective, cardiostimulant, and hyperthermic drug effects and returned to normal within 6 h after MDMA administration when plasma MDMA levels were still high. Mydriasis was associated with changes in plasma MDMA concentration over time and longer-lasting. Both reboxetine and duloxetine interacted with the effects of MDMA on pupillary function. Clonidine did not significantly reduce the mydriatic effects of MDMA, although it produced miosis when administered alone. Carvedilol and doxazosin did not alter the effects of MDMA on pupillary function. Conclusions The MDMA-induced prolongation of the latency to and reduction of light-induced miosis indicate indirect central parasympathetic inhibition, and the faster recovery time reflects an increased sympathomimetic action. Both norepinephrine and serotonin mediate the effects of MDMA on pupillary function. Although mydriasis is lasting and mirrors the plasma concentration–time curve of MDMA, the impairment in the reaction to light is associated with the subjective and other autonomic effects of MDMA and exhibits acute tolerance.

Anisocoria is a significant finding in several ocular and potentially life-threatening neurological disorders. The angel's trumpet (Datura suaveolens), widely used as a garden plant, is a natural alkaloid with anticholinergic effects containing high levels of scopolamine. The authors present a pediatric case of acute anisocoria secondary to contact with the angel's trumpet plant. This case report emphasizes the importance of considering herbal mydriatics in patients with acute, isolated, unilateral mydriasis. It is also important to raise public awareness about the potential risks of the angel's trumpet plant, particularly in areas close to schools and playgrounds. [J Pediatr Ophthalmol Strabismus. 2018;55:e33–e35.]

Background The subconjunctival anesthesia with local anesthetics is considered as a low-risk procedure allowing ocular surgery without serious complications typical for retro- or parabulbar anesthesia, especially in patients with preexisting Optic Nerve damage. We report development of ipsilateral transient amaurosis accompanied with mydriasis and both, direct and consensual light response absence. Case presentation Three patients with advanced refractory glaucoma undergoing laser cyclophotocoagulation (CPC) for intraocular pressure lowering experienced these adverse effects just few minutes after subconjunctival injection of mepivacaine 2% solution (Scandicaine® 2%, without vasoconstrictor supplementation). The vision was completely recovered to usual values in up to 20 h after mepivacaine application. Extensive ophthalmological examination, including cranial magnetic resonance imaging (MRI), revealed no further ocular abnormalities, especially no vascular constriction or thrombotic signs as well as no retinal detachment. The oculomotor function remained intact. The blockade of ipsilateral ciliary ganglion parasympathetic fibers by mepivacaine may be the responsible mechanism. Systemic pathways as drug-drug interactions seem to be unlikely involved. Importantly, all three patients tolerated the same procedure previously or at a later date without any complication. Overall, our thoroughly elaborated risk management could not determine the causative factor explaining the observed ocular complications just in the current occasion and not at other time points. Conclusions Doctors should be aware and patients should be informed about such rare complications after subconjunctival local anesthetics administration. Adequate risk management should insure patients’ safety.

PURPOSE: To evaluate the effectiveness of the pupil center shift with changes in the state of pupil size and with other ocular variables. METHODS: Dynamic pupillometry with the Topolyzer Vario (Alcon Laboratories, Inc., Fort Worth, TX) was performed in 248 eyes of 124 patients scheduled for corneal laser refractive surgery. High-resolution images were obtained using the infrared-sensitive camera (incorporated in the videokeratoscope) under mesopic and photopic conditions. Measurements of pupil diameters, distance between the pupil center and keratoscopic axis, and spatial shift of the pupil center were obtained after analysis. RESULTS: The mean distance between the pupil center and the corneal vertex in mesopic and photopic conditions of illumination in myopic eyes was 0.27 ± 0.14 (range: 0.02 to 0.70 mm) and 0.24 ± 0.12 mm (range: 0.06 to 0.65 mm), respectively, whereas it was 0.36 ± 0.15 (range: 0.03 to 0.70 mm) and 0.31 ± 0.16 mm (range: 0.03 to 0.77 mm) in hyperopic eyes, respectively. The mean spatial pupil center shift was significant: 0.11 ± 0.07 mm (range: 0.02 to 0.57 mm) in myopic eyes and 0.12 ± 0.09 mm (range: 0.02 to 0.47 mm) in hyperopic eyes. The pupil center shifted consistently temporally as the pupil dilated. The pupil center shift was not significantly related to sex, age, eye (right or left), or refractive error. CONCLUSIONS: The mean distance between the pupil center and the corneal vertex is greater in hyperopic eyes than in myopic eyes, whereas the spatial shift of this pupil center has a temporal direction as the pupil dilates and is constantly small in all groups. However, pupil center shift can be important in a few patients. [[ J Refract Surg. 2014;30(10):694–700.]

The dilated pupil can present a significant challenge to the clinician. Although in most cases a complete history and physical examination is sufficient to make an accurate diagnosis, selected patients will require further investigation, including pharmacologic testing and neuroimaging. This review outlines the physiology, clinical features, and diagnostic approach to the most important causes of the dilated pupil. Particular attention is given to recent publications on this topic.

We report a patient who has a tremor and unilateral ptosis and mydriasis without extraocular muscle paresis from an intra-axial lesion demonstrated on neuroimaging. Previously extraocular muscles sparing partial third nerve palsy has been thought to be due to extra-axial lesion such as vascular compression. Compared to proposed models for arrangement of oculomotor fascicle, this case demonstrates that it is possible to damage the fibers destined for levator and pupillomotor function without affecting the extra-ocular muscles.