Mitchell B. Strominger

Wall-eyed bilateral internuclear ophthalmoplegia in central nervous system cryptococcosis.

Only one case of wall-eyed bilateral internuclear ophthalmoplegia (WEBINO) has been described in central nervous system cryptococcosis. The disorder was initially unilateral, then became bilateral with skew deviation and vertical upgaze deficit. We report a case of WEBINO in central nervous system cryptococcosis in a patient with acquired immune deficiency syndrome. Magnetic resonance imaging revealed high signal on T2 images in the right midbrain, left frontal vertex, left splenium, and cerebellum. With treatment, the internuclear ophthalmoplegia improved; however, the convergence insufficiency remained. Disruption of input from cortical supranuclear locations or the region of the rostral interstitial nucleus of the medial longitudinal fasciculus has been proposed as a mechanism in the absence of convergence. This correlates in our patient with the lesions seen on magnetic resonance images.

Acute macular neuroretinopathy associated with the use of norepinephrine reuptake inhibitors: a case series and OCT findings.

PURPOSE The purpose of this study was to report a case series of patients with acute macular neuroretinopathy in which patients concomitantly used oral norepinephrine reuptake inhibitors. METHODS Retrospective case review. PATIENTS Three patients with retinal findings consistent with acute macular neuroretinopathy are presented. CONCLUSION The use of oral neuromodulators should be considered in cases of acute macular neuroretinopathy.

Comparison of primary position measurements and abduction deficit between type 1 Duane syndrome and sixth cranial nerve palsy.

Background: Unilateral Duane retraction syndrome type 1 (DRS-I) and unilateral sixth nerve palsy (6NP) present with limitation of abduction, incomitant esotropia, and frequently, a compensatory head turn. The purpose of this study was to compare the mean primary position measurement and to correlate this with the abduction deficit to determine if these measurements may be used to differentiate between the 2 conditions when other clinical signs of DRS-I (globe retraction, changes in lid fissure height, and upshoots/downshoots) are subtle. Methods: A database search of patients examined over a 5-year period revealed 69 cases of DRS-I and 62 cases of unilateral 6NP. Primary position measurements both at distance and near and limitation of abduction on version testing were recorded and compared. Results: Mean abduction deficit was −3.5 ± 0.1 for DRS-I and −2.6 ± 0.2 for 6NP (P = 0.0004). Mean esotropia at near was 8.4 ± 1.1 prism diopters (PD) for DRS-I and 27.2 ± 2.4 PD for 6NP (P < 0.0001). Mean esotropia at distance was 10.3 ± 1.3 PD for DRS-I and 36.4 ± 2.4 PD for 6NP (P < 0.0001). The mean distance-near disparity for DRS-I was 1.94 ± 0.62 PD and 9.19 ± 1.28 PD for 6NP (P < 0.0001). The age-group of ≤2 years consisted of 23 DRS-I and only 2 6NP cases. The age-group between >2 years and <18 years had 41 DRS-I and 16 6NP cases, respectively. Finally, the age-group of ≥18 years had only 5 DRS-I and 44 6NP cases (P < 0.0001). Conclusion: Patients with DRS-I showed greater abduction deficit yet significantly less esotropia in primary position than those with 6NP. Patients with 6NP were more likely to have a significant distance-near disparity. In addition, patients with DRS-I tended to be younger than those with 6NP. This report documents that DRS-I and 6NP can be differentiated based on magnitude of primary position esotropia, comparison of primary position esotropia with severity of abduction deficit, distance-near disparity, and patient age.

Ocular Infection in Children

Ocular infections in children are related to environmental factors and exposures that are age and developmentally dependent. Thus the site of infection may vary and be tissue dependent, whether it involves the orbit, conjunctiva, cornea, or retina, all giving clues to the etiology and time of primary inoculation. Age of presentation along with the site of infection helps to determine the most likely cause and location. Primarily, infectious ocular diseases in children can be divided in four broad categories. These include intrauterine and perinatal infections, ophthalmia neonatorum, conjunctivitis, and orbital and adnexal infections. Intrauterine and perinatal infections covered in this chapter include the TORCHES (toxoplasmosis; rubella; cytomegalovirus (CMV); herpes viruses, including Epstein-Barr; syphilis) and lymphocytic choriomeningitis virus. The section on ophthalmia neonatorum will primarily cover Neisseria gonorrhoeae and Chlamydia trachomatis. Viral and bacterial conjunctivitis will be discussed. Finally how to differentiate between preseptal and orbital cellulitis including treatments will be emphasized.

Supraventricular tachycardia in a child undergoing strabismus surgery

To the Editors: Supraventricular tachycardia (SVT) encompasses all types of tachycardia that start from above the bifurcation of the bundle of His with a heart rate ranging from 150 to 250 beats/min in children.1,2 Paroxysmal SVT occurs suddenly and can end rapidly.1 We describe a healthy 3-year-old boy who developed primary SVT during intraoperative extraocular muscle manipulation. Our patient underwent surgery to correct inferior oblique overaction. Under laryngeal mask airway anesthesia (propofol, fentanyl, ketorolac, isoflurane, and sevoflurane), the inferior oblique muscle was isolated using muscle hooks through a fornix incision. A 6-0 polyglactin suture was then placed through the muscle near its insertion. The patient then went into SVT. Contact with the muscle was stopped, but SVT continued at 227 beats/min. Carotid massage, ice placed on the forehead, and adenosine 1.5 mg 3 2 doses had no effect. A 12-lead electrocardiogram demonstrated features of reentry SVT (Figure). An additional 3 mg of adenosine restored sinus rhythm at 142 beats/min. One more incidence of SVT occurred, which was treated with 3 mg of adenosine. The inferior oblique muscle was re-isolated, and there was an episode of bradycardia treated with glycopyrrolate. The cardiac rhythm stabilized and surgery continued without complication. Subsequent transthoracic echocardiography was normal and the patient started propranolol 10 mg orally three times daily. In retrospect, a family history was positive for Wolff–Parkinson–White (WPW) syndrome in a first cousin. SVT is the most common form of arrhythmia in children and 90% is mediated through a reentry mechanism. This is facilitated by the presence of an accessory pathway in addition to the normal atrioventricular node and Purkinje system.3 Jacobson et al. detailed paroxysmal SVT during strabismus surgery in a 17-year-old with WPW syndrome.4 Paroxysmal reentrant SVT occurs in WPW syndrome using an accessory retrograde pathway that is manifest on electrocardiogram with a short PR interval and Delta waves.5 When these features are absent in reentrant SVT, presence of a concealed pathway is presumed.3 Our patient’s electrocardiogram did not have the characteristics of WPW and thus the reentrant SVT was mediated by a concealed pathway. It is speculated that in our case and in the patient with WPW, hypotension induced by anesthesia may have increased sympathetic tone, favoring conditions for a reentry circuit via the preexisting accessory pathway. The SVT may have been initiated by an ectopic escape beat in the setting of bradycardia from vagal stimulation induced by manipulation of the muscle. Acute treatment of SVT includes vagal maneuvers such as Valsalva maneuver and cold water immersion of the face. If these treatments fail, intravenous adenosine should be used; it works by inducing transient AV block that terminates SVT by interrupting the reentry pathway and allowing restoration of sinus rhythm.1 Final treatment options include elective cardioverison or procainamide. Chronic SVT treatment can involve the use of propranolol or other antiarrhythmic agents and catheter ablation.1

Morphological Findings of Extraocular Myopathy with Chronic Progressive External Ophthalmoplegia

Mitochondrial diseases are a large group of disorders resulting from mutations of nuclear DNA (nDNA) and mitochondrial DNA (mtDNA). Patients present clinically with multiple manifestations, including myopathies and multiple system disorders. Establishing a specific diagnosis often requires extensive clinical and laboratory evaluation. In this study of 2 adult patients with presumptive mitochondrial disease, the authors have identified distinctive morphological changes in medial rectus muscle biopsies that confirm the diagnosis of chronic progressive external ophthalmoplegia (CPEO). These findings demonstrate the usefulness of electron microscopy using medial rectus muscle in the diagnosis of adult patients with a slowly progressive course of mild skeletal weakness and CPEO.