[Painful anisocoria in a five-year-old child: A rare diagnosis which must not be missed]. / Anisocorie douloureuse chez un enfant de cinq ans : un diagnostic rare à ne pas méconnaître.

Congenital ectropion uveae (CEU) is a rare anomaly of the embryonic development of the anterior segment of the eye. We report the case of a 5-year-old child with an undiagnosed CEU who was treated urgently for an acute angle closure attack. CASE DESCRIPTION: A 5-year-old child was referred urgently for evaluation of anisocoria with mydriasis of the right eye and severe headache. Brain imaging with contrast injection was initially performed in the pediatric emergency department and ruled out central nervous system pathology. The initial examination of the right eye revealed an intraocular pressure (IOP) of 37mmHg, corneal edema, congenital ectropion uveae, mydriasis with pupillary block, a closed angle on gonioscopy, and a clear lens. The examination of the left eye was unremarkable, with no visible CEU. The initial management consisted of medical treatment with topical glaucoma drops and miotics and acetazolamide at 10mg/kg/d. Re-evaluation under general anesthesia showed persistent mydriasis and no resolution of the pupillary block. Filtering surgery was performed in the absence of a complete response to medical treatment, allowing control of IOP without drops and complete regression of the corneal edema. DISCUSSION: CEU is a rare malformation, and pressure complications represent an insignificant proportion of pediatric glaucoma cases. The acute presentation of acute angle closure in this potentially blinding short-term setting, however, makes detection and management difficult in very young children in a great deal of pain. Only one similar case has been reported in the pediatric literature. CONCLUSION: Acute angle closure complicating CEU is exceptional and difficult to diagnose in a pediatric context. Parents of children with this predisposing condition should be informed of the need to consult urgently when clinical signs of elevated intraocular pressure appear.

Fijación de lentes intraoculares a esclera sin suturas / Sutureless scleral intraocular lens fixation

La corrección quirúrgica de la afaquia se realiza mediante varias técnicas que permiten fijar los lentes intraoculares en la cámara posterior, suturados al sulcus ciliar o por fijación transescleral de las hápticas sin suturas. El cirujano determina cuándo, dónde y cómo, además del tipo de lente a implantar. Se presenta un paciente con una afaquia traumática del ojo izquierdo, con agudeza visual sin corrección de cuenta dedos a un metro y refracción dinámica de +8,00 dioptrías con agudeza visual mejor corregida de 0,8 por cartilla de Snellen. Tensión ocular de 16 mmHg. En el examen biomicroscópico con lámpara de hendidura del ojo izquierdo se observó midriasis traumática. Se le realizó examen con biomicroscopia indirecta y resultó sin alteraciones. Se implantó el lente intraocular de tres piezas (Tecnis ZA9003) de la cámara posterior y se fijaron las hápticas a la esclera sin utilizar suturas. Al mes de la cirugía la agudeza visual mejor corregida fue la unidad de visión(AU)

Surgical aphakia correction is based on several techniques allowing to fix intraocular lenses in the posterior chamber, sutured to the ciliary sulcus or by sutureless transcleral fixation of the haptics. The surgeon will decide when, where and how, as well as the lens type to implant. A case is presented of a male patient with traumatic aphakia of his left eye, finger counting uncorrected visual acuity at one meter and dynamic refraction of +8.00 diopters with best corrected visual acuity of 0.8 by the Snellen chart. Ocular tension was 16 mmHg. Biomicroscopic slit lamp examination of the left eye found traumatic mydriasis. Indirect biomicroscopy did not find any alteration. A three-piece intraocular lens (Tecnis ZA9003) was implanted in the posterior chamber, fixing the haptics to the sclera without the use of sutures. One month after surgery, best corrected visual acuity was the vision unit(AU)

Pharmacological pupil dilatation after sinus surgery.

Orbital complications after endoscopic sinus surgery are serious problems. Inadvertent contamination of the eye by pharmacological solution can lead to early postoperative anxiety to patients and high concern to surgeons. This is a rare case report of retrograde epinephrine flow through lacrimal duct in sinus surgery with learning tips during postoperative assessment for reassurance in temporary pharmacological effect rather than serious complication.

Pupil-Involving Oculomotor Nerve Palsy Following Tonsillectomy and Adenoidectomy.

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.].

Pourfour du Petit Syndrome: A Rare Association With Cluster Headache.

Pourfour du Petit syndrome is an uncommon cause of eyelid retraction, associated with unilateral mydriasis and hemifacial hyperhidrosis. This syndrome is caused by hyperactivity of the ipsilateral oculosympathetic pathway and needs to be recognized because it has an opposite clinical presentation but the same topographic and diagnostic value as Horner syndrome. The authors report a rare case of Pourfour du Petit syndrome associated with cluster headache and discuss pathophysiological hypotheses, clinical presentation, complementary exams, pharmacologic testing, treatment options, and prognosis. Early detection of these symptoms may lead to swift diagnosis and treatment.

Iris anomalies and the incidence of ACTA2 mutation.

BACKGROUND: Central cysts of the iris pigment epithelium, or iris flocculi, are frequently reported in the literature in association with thoracic aortic aneurysm and dissection due to smooth muscle alpha-actin 2 (ACTA2) mutations. Children with ACTA2 mutations may also present with congenital mydriasis. We report our experience regarding the frequency of ACTA2 mutation in children with the above iris anomalies. METHODS: This is a retrospective, consecutive case series of all children presenting for iris flocculi or congenital mydriasis at a single tertiary centre from October 2012 to December 2016. RESULTS: 13 children with iris flocculi and 3 with congenital mydriasis presented during the study period. 10 children with iris flocculi completed genetic testing, and none were positive for ACTA2 mutation. All children with congenital mydriasis presented with a multisystem smooth muscle dysfunction syndrome; two of these three children tested positive for missense R179 ACTA2 mutations. CONCLUSIONS: In this series, ACTA2 mutation or copy number variation was not detected in children presenting for iris flocculi, whereas congenital mydriasis was associated with R179 mutation in both cases that tested positive for ACTA2 mutation. The case of congenital mydriasis without typical cardiac features of the R179 ACTA2 phenotype or intracranial vasculopathy was negative for ACTA2 mutation. While all children presenting with these iris anomalies should be offered a genetic evaluation, incidence data should inform genetic counselling, particularly in the absence of a family history of aneurysm or sudden death, or systemic signs of smooth muscle dysfunction.

Benign Episodic Unilateral Mydriasis in a Flight Nurse.

BACKGROUND: Benign episodic unilateral mydriasis is one cause of anisocoria. This phenomenon is thought to be related to an imbalance between the sympathetic and parasympathetic nervous systems. There is a documented association with migraines, but asymptomatic cases have also been reported. A challenge with all cases is the level of investigation required to exclude more sinister causes of nervous system dysfunction. In a dynamic flight environment, additional considerations need to be made, such as varying light levels and use of night vision devices. CASE REPORT: A 27-yr-old woman on deployment to Afghanistan as a flight nurse presented to the role one clinic with right-sided mydriasis. The patient denied headache or any history of migraines. A dilated right pupil that was reactive to light was found on exam. Symptoms and exam findings resolved shortly after initial presentation. We consulted an ophthalmologist who requested patient transfer for review. He made a diagnosis of benign episodic unilateral mydriasis. DISCUSSION: There are a variety of causes for anisocoria. A thorough history and examination are required to avoid unnecessary investigations that may not be locally available in the more austere deployed military settings. From an operational perspective, the decision needs to be made regarding the maintenance of flight status. Consideration needs to be given to patient care capability when treating a flight nurse. In cases of rapid resolution such as this, removal from operational status is not reasonable should a clinician be confident of the diagnosis.Schiemer A. Benign episodic unilateral mydriasis in a flight nurse. Aerosp Med Hum Perform. 2017; 88(5):500-502.

Extracorporeal Life Support in Multisystem Smooth Muscle Dysfunction Syndrome.

We describe an infant with congenital mydriasis, patent ductus arteriosus (PDA), pulmonary hypertension, and cystic lung disease. She had all the major components of multisystemic smooth muscle dysfunction syndrome. Due to progressive respiratory deterioration, she required surgical PDA interruption, extracorporeal life support, and subsequent prolonged respiratory support. Genetic testing revealed ACTA2 R179H mutation and cystic lung disease on biopsy.

Mydriasis revealing vascular and osteodural compression of the oculomotor nerve: An observational study on five cases.

PURPOSE: To describe a form of neurovascular compression of the third cranial nerve (CNIII) in idiopathic mydriasis, in which a neurovascular "conflict" exists between the oculomotor nerve, the posterior communicating artery and the clinoid process, using high-resolution magnetic resonance imaging (MRI) with fast imaging employing steady acquisition (FIESTA) sequences. METHODS: An 18-month prospective, observational and monocentric case series report was performed. MRI was performed on 5 consecutive patients with idiopathic, unilateral, persistent and nonreactive mydriasis (pure intrinsic palsy of the CNIII). Patients with diplopia, ptosis or ophthalmoplegia were excluded. Cerebral MRI focused on the CNIII pathway from the mesencephalon to the cavernous sinus entry, particularly on the cisternal segment: image acquisition was performed on a 3 Tesla MRI; the protocol included fast imaging employing steady acquisition (FIESTA) and three-dimension time of flight (3D TOF) sequences. RESULTS: All patients presented a neurovascular compression point, involving the CNIII clamped between a tortuous posterior communicating artery (PCoA) and the posterior clinoid process at the entrance of the cavernous sinus. No cases occurred in the root entry zone. There was no compression on the contralateral side. No tumors or aneurysms were found. Thus, the mydriasis was caused by CNIII compression. CONCLUSION: MRI, including FIESTA sequences, revealed a new type of neurovascular conflict between the CNIII, PCoA and posterior clinoid process in patients with incomplete oculomotor palsy. Non-aneurysmal CNIII compression should be considered as a differential diagnosis in the work-up of idiopathic mydriasis. The role of MRI in the work-up of anisocoria should be considered.

Bilateral Dilated Nonreactive Pupils in a Neonate After Surgery.

Fixed and dilated pupils are disturbing when encountered during a physical examination in the pediatric intensive care unit, particularly when sedation or neuromuscular blockade confounds the neurologic examination. Rocuronium, a nondepolarizing neuromuscular drug, does not cross the blood-brain barrier and is not considered a causative agent for fixed mydriasis. We report a case of bilateral fixed and dilated pupils in a 1-week-old low-birth-weight neonate, which we contend was secondary to centrally mediated neuromuscular blockade.

Urrets-Zavalia syndrome after deep anterior lamellar keratoplasty

Introduction: Urrets-Zavalia syndrome is an uncommon complication of the deep anterior lamellar keratoplasty in keratoconus. The manifestations of this syndrome are an irreversible mydriasis, iris atrophy and secondary glaucoma. Case report: Deep anterior lamellar keratoplasty was done for keratoconus with a presumably healed corneal hydrops in a 21-year-old Caucasian man. The graft remained clear, but the surgery was complicated by a fixed, dilated pupil, patches of iris atrophy, ectropium of the iris pigment layer and glaukomflecken in the lens. Conclusion: Although safer than penetrating keratoplasty, the deep anterior lamellar by not trying to secure an unhealed Descemet's membrane with air. Instead, a new Descemet's membrane transplanted within a penetrating graft is a safer choice.

Síndrome de Chandler / Chandler's syndrome

El síndrome de Chandler es una de las variaciones clínicas del síndrome iridocorneoendotelial, donde el denominador común es una alteración del endotelio corneal cuyo diagnóstico es, en ocasiones, muy difícil de realizar por la complejidad y la variabilidad de sus signos y síntomas. Se presenta una paciente de 67 años con disminución importante de la visión del ojo izquierdo. A la exploración existía un edema corneal severo, una midriasis pupilar, una corectopia leve hacia el sector temporal con tracción iridiana hacia el ángulo y sinequias anteriores angulares en el lado de la corectopia. La realización de un recuento endotelial demostró alteraciones cuali y cuantitativas del endotelio en el ojo izquierdo. Se concluyó que la microscopia especular es fundamental para el diagnóstico y la evaluación en casos sospechosos de síndrome de Chandler(AU)

Chandler's syndrome is a clinical variant of the iridocorneal endothelial syndrome in which the common aspect is the altered corneal endothelium that is sometimes very difficult to be diagnosed on account of the complexity and variability of signs and symptoms. This article was aimed at showing the effectiveness of specular microscopy for diagnosis. Here is a 67 years-old female patient who had significant reduction of vision in her left eye. On examination, there were observed severe corneal edema, pupillary mydriasis, mild corectopia towards the temporal sector with angled iridal traction and anterior angle synechias on the corectopia side. The endothelial counting revealed qualitative and quantitative alterations in the left eye. It was concluded that the specular microscopy is the key to diagnosis and assessment of suspected Chandler's syndrome cases(AU)

Ocular abnormalities in patients treated with a novel anti-GD2 monoclonal antibody, hu14.18K322A.

PURPOSE: To determine the incidence of and factors associated with the development of mydriasis and impaired accommodation in patients with refractory or recurrent neuroblastoma receiving the anti-GD2 antibody hu14.18K322A. METHODS: The medical records of eligible patients with refractory or recurrent neuroblastoma who received escalating doses of hu14.18K322A, ranging from 2 to 70 mg/m(2)/dose for 4 consecutive days every 28 days, were retrospectively reviewed to identify ocular abnormalities arising during the treatment period. RESULTS: A total of 38 patients (median age, 7 years; 23 males) were included. All patients underwent comprehensive eye examinations prior to each course of therapy. Mydriasis was seen in 13 patients (34%), and impaired accommodation was seen in 9 (24%), indicating a dose-related effect between hu14.18K322A and both mydriasis (P = 0.021) and impaired accommodation (P = 0.029). Age and sex were not associated with ocular abnormalities. Ocular symptoms resolved in the majority of patients after the drug was discontinued. CONCLUSIONS: Side effects of mydriasis and impaired accommodation have a dose-dependent relationship with hu14.18K322A. These side effects do not warrant discontinuation of treatment, as they usually resolve after completion of therapy. Management of ocular side effects should focus on treating symptoms with manifest refraction, bifocals, or tinted spectacles.

Urrets-Zavalia syndrome after Descemet membrane endothelial keratoplasty.

PURPOSE: To report a case of Urrets-Zavalia syndrome (UZS) after Descemet membrane endothelial keratoplasty (DMEK). METHODS: A 74-year-old woman with Fuchs endothelial dystrophy and inconspicuous ocular history developed UZS after DMEK surgery. The intraoperative and postoperative course is presented. RESULTS: After uneventful DMEK surgery, intraocular pressure was elevated up to 40 mm Hg on the first postoperative day. A small bleed from the peripheral wide-open iridectomy in the 12 o'clock position in the otherwise deep anterior chamber was observed. On the sixth postoperative day, a 4-mm-wide pupil, nonreactive to light, was noted. One year after surgery, the fixed medium mydriasis (4 mm) persisted and best-corrected vision was 0.1 logMAR. No pupillary reaction was noted after application of 0.2% or 2% pilocarpine. CONCLUSIONS: Filling the anterior chamber with air to secure fixation of a grafted Descemet membrane carries the risk of early acute postoperative ocular hypertension. This can lead to iris sphincter defects resulting in a fixed dilated pupil after DMEK surgery. Large patent iridectomy in the 12 o'clock position is insufficient to prevent this. Patients undergoing DMEK surgery should be informed about this potential complication.

Unilateral mydriasis after maxillary osteotomy: a case series and review of the literature.

PURPOSE: To present 4 cases of unilateral mydriasis associated with orthognathic surgery and to review the differential diagnosis and management related to this condition. MATERIALS AND METHODS: Four cases of unilateral mydriasis associated with orthognathic surgery were identified from the authors' institutional experience. All maxillary osteotomies performed by the authors' department from 2001 to 2013 were identified based on Current Procedural Terminology codes; 4 cases of unilateral mydriasis were found. Cases are presented and the literature is reviewed. RESULTS: Two male and 2 female patients with an age range of 16 to 34 years developed unilateral mydriasis after maxillary osteotomy; the estimated prevalence is 0.004%. Although the precise cause can be difficult to determine, in this series 1 case was attributable to swelling affecting contents of the superior orbital fissure, 1 was related to edema or medications, and 2 were pharmacologically induced. CONCLUSION: Although rare, a review of the differential diagnosis for and management of unilateral mydriasis associated with orthognathic surgery is pertinent to those who perform corrective jaw surgery.