Melanie Kazlas

Diplopia after surgical repair of orbital floor fractures.

Summary: Blowout fractures of the orbit are common sequelae to blunt facial trauma. Many aspects of this injury have been studied, in particular, the timing of and indications for surgical intervention. Although diplopia is often an indication for surgery and is presented to patients as a potential postoperative complication, the incidence of diplopia after surgical repair of orbital blowout fractures has not been well studied. We retrospectively studied 54 patients who underwent repair of an orbital blowout fracture. A minimum of 6 months follow‐up was available for all patients included in the study. A total of 47 of 54 (86%) patients had clinically significant diplopia preoperatively, and 20 of 54 (37%) remained diplopic. A total of 17 of 54 (31%) fractures involved the medial wall and orbital floor, and 13 of these 17 patients (86%) had postoperative diplopia. Patients with combined orbital floor and medial wall fractures appear to be at higher risk for clinically significant diplopia postoperatively than those with fractures of the orbital floor only. The explanation for this observation may be related to a greater difficulty in restoring the preoperative contour of orbits with combined fractures.

Toxic epidermal necrolysis in children: medical, surgical, and ophthalmologic considerations.

Toxic epidermal necrolysis is a rare acute inflammatory multisystem life-threatening condition characterized by widespread epidermal necrosis and profound toxic systemic reaction. Implicated etiologic agents in children include drugs, infections, and autoimmune diseases. The pathophysiology includes separation of the epidermis at the dermal–epidermal junction of both skin and extracutaneous epithelium and mucous membranes. The general consensus is that expeditious transfer to a burn center, maintenance of fluid and electrolyte balance, temperature maintenance, control of evaporative losses, avoidance of use of complicating drugs as corticosteroids and topical sulfa compounds, aggressive septic surveillance, vigorous nutritional support via nasoenteric tube, early ophthalmologic consultation, and appropriate wound care with a regimen of therapy relying on basic principles of treatment of partial-thickness epidermal wounds predict better outcome in the treatment of this disease process. The course of toxic epidermal necrolysis in children, even though dramatic at onset, leads to low mortality when managed appropriately. The current limited published evidence does not clearly delineate differences in epidemiology, pathogenesis, and prognosis of severe skin reactions in children as compared with adults. In this article, we review the available literature on the pathogenesis, clinical features, pathophysiology, treatment, and complications of this rare disease in children.

Assessing Binocular Interaction in Amblyopia and Its Clinical Feasibility

Purpose To measure binocular interaction in amblyopes using a rapid and patient-friendly computer-based method, and to test the feasibility of the assessment in the clinic. Methods Binocular interaction was assessed in subjects with strabismic amblyopia (n = 7), anisometropic amblyopia (n = 6), strabismus without amblyopia (n = 15) and normal vision (n = 40). Binocular interaction was measured with a dichoptic phase matching task in which subjects matched the position of a binocular probe to the cyclopean perceived phase of a dichoptic pair of gratings whose contrast ratios were systematically varied. The resulting effective contrast ratio of the weak eye was taken as an indicator of interocular imbalance. Testing was performed in an ophthalmology clinic under 8 mins. We examined the relationships between our binocular interaction measure and standard clinical measures indicating abnormal binocularity such as interocular acuity difference and stereoacuity. The test-retest reliability of the testing method was also evaluated. Results Compared to normally-sighted controls, amblyopes exhibited significantly reduced effective contrast (∼20%) of the weak eye, suggesting a higher contrast requirement for the amblyopic eye compared to the fellow eye. We found that the effective contrast ratio of the weak eye covaried with standard clincal measures of binocular vision. Our results showed that there was a high correlation between the 1st and 2nd measurements (r = 0.94, p<0.001) but without any significant bias between the two. Conclusions Our findings demonstrate that abnormal binocular interaction can be reliably captured by measuring the effective contrast ratio of the weak eye and quantitative assessment of binocular interaction is a quick and simple test that can be performed in the clinic. We believe that reliable and timely assessment of deficits in a binocular interaction may improve detection and treatment of amblyopia.

Ocular injuries in shaken baby syndrome

Child abuse is defined as endangerment of a child at the hands of a parent or a guardian. The incidence of child abuse in the United States is reported as 1% or more than 1 million children annually. Many cases are thought to go unreported. More than 10% of traumatic injuries in children are nonaccidental. Of children having pediatric nonaccidental trauma, up to 20% sustain fatal injuries. Of those who survive child abuse, 15% will have permanent physical abnormalities. Countless more will experience incalculable emotional damage. Eye findings occur in more than 40% of all child abuse cases and in up to 95% of shaken baby syndrome (SBS) cases. SBS is a severe form of childhood physical abuse with few outward signs of physical injury. The incidence of this syndrome is estimated to be up to 4,000 cases per year. The child sustains a whiplash type of injury resulting from profound forcible shaking while being grabbed by the shoulders, torso, or hips. The high-velocity acceleration-deceleration forces exerted on the unsupported head and neck result in severe intracranial and intraocular damage seen all too frequently by the medical professional.

Pediatric intraocular lens implantation: historic perspective and current practices.

Pediatric cataract extraction poses many challenges for the ophthalmologist different than those in the adult population, involving surgical timing, surgical technique, and aphakic visual rehabilitation. Early surgery is necessary in children to avoid dense sensory deprivation amblyopia; and aggressive management of aphakia after cataract extraction is important to avoid refractive or anisometropic amblyopia. Pediatric patients who remain aphakic after cataract extraction require either spectacle or contact lens (CL) correction. Unilateral aphakia poses a particular challenge owing to anisometropia and aniseikonia, making spectacle correction unsuitable. In addition, aphakic eyeglasses are associated with optical distortion and are often poorly tolerated by children for social reasons. The use of CLs can be difficult in the pediatric population at best and is often intolerable to either parents or patients. CL use is associated with problems such as keratitis, poor compliance owing to difficulty with lens insertion and removal, and frequent lens loss, all of which could be associated with compromised amblyopia treatment and poor visual outcomes. Owing to these limitations of nonsurgical treatment of aphakia in the pediatric population, primary intraocular lens (IOL) implantation has become standard in older children undergoing cataract extraction. As primary IOL implantation has become more broadly used, the age in which the procedure is acceptable and appropriate has steadily

A Retrospective Review of Orbital Decompression for Thyroid Orbitopathy with Endoscopic Preservation of the Inferomedial Orbital Bone Strut

PURPOSE To determine incidence of new-onset diplopia, resolution of preexisting diplopia, and impact on proptosis resulting from endoscopic orbital decompression with and without preservation of the inferomedial orbital strut for thyroid orbitopathy. METHODS Retrospective review of all patients undergoing endoscopic 2- or 3-wall decompression with or without preservation of the strut for thyroid orbitopathy from January 2012 to June 2015. RESULTS Twenty-six patients (45 orbits) were included and divided into 4 primary categories: 2-wall decompression with strut preservation (4 orbits, 8%), 2-wall decompression with strut removal (7 orbits, 16%), 3-wall decompression with strut preservation (27 orbits, 60%), and 3-wall decompression with strut removal (7 orbits, 16%). The incidence of new-onset diplopia was 20% (2/10 patients without preoperative diplopia) overall and 16% in the strut preservation group (1/6 patients without preoperative diplopia). Resolution of diplopia occurred in 4 of 16 patients (25%) with preoperative diplopia, and all 4 had been treated with a 3-wall decompression with strut preservation. Resolution of diplopia in the group treated with strut preservation was 36% (4/11 patients with preoperative diplopia), and 0% of the 5 diplopic patients treated without strut preservation. Reduction in proptosis was statistically greater in those treated with strut removal (p = 0.003). CONCLUSIONS This study demonstrates that endoscopic orbital decompression with preservation of the inferomedial bone strut results in a comparable to lower rate of new-onset diplopia compared with other reported techniques. When combined with 3-wall balanced decompression, this technique demonstrates a high rate of resolution of preexisting diplopia.

Multicentric myofibromatosis presenting as a large congenital eyelid myofibroma

Infantile myofibromatosis is a rare mesenchymal neoplasm that commonly involves the head and neck but rarely the eyelid. We report the case of a newborn boy referred for evaluation of a left eyelid lesion that occluded the visual axis. Urgent biopsy was performed to evaluate for malignancy. Histopathologic analysis demonstrated myofibroma. Although these lesions have been reported to regress spontaneously, debulking surgery was performed to prevent sensory or anisometropic amblyopia. Follow-up systemic evaluation revealed numerous subcutaneous and deep soft tissue lesions. There was no visceral involvement.

Imaging Appearance of the Lateral Rectus–Superior Rectus Band in 100 Consecutive Patients without Strabismus

BACKGROUND AND PURPOSE: The lateral rectus–superior rectus band is an orbital connective tissue structure that has been implicated in a form of strabismus termed sagging eye syndrome. Our purpose was to define the normal MR imaging and CT appearance of this band in patients without strabismus. MATERIALS AND METHODS: Orbital MR imaging and CT examinations in 100 consecutive patients without strabismus were evaluated. Readers graded the visibility of the lateral rectus–superior rectus band on coronal T1WI, coronal STIR, and coronal CT images. Readers determined whether the band demonstrated superotemporal bowing or any discontinuities and whether a distinct lateral levator aponeurosis was seen. Reader agreement was assessed by κ coefficients. Association between imaging metrics and patient age/sex was calculated by using the Fisher exact test. RESULTS: The lateral rectus–superior rectus band was visible in 95% of coronal T1WI, 68% of coronal STIR sequences, and 70% of coronal CT scans. Ninety-five percent of these bands were seen as a continuous, arc-like structure extending from the superior rectus/levator palpebrae muscle complex to the lateral rectus muscle; 24% demonstrated superotemporal bowing; and in 82% of orbits, a distinct lateral levator aponeurosis was visible. Increasing patient age was negatively associated with lateral rectus–superior rectus band visibility (P = .03), positively associated with lateral rectus–superior rectus band superotemporal bowing (P = .03), and positively associated with lateral levator aponeurosis visibility (P = .01). CONCLUSIONS: The lateral rectus–superior rectus band is visible in most patients without strabismus on coronal T1WI. The age effect with respect to its visibility and superotemporal bowing could represent age-related connective tissue degeneration.

AML presenting with a preleukemic episode and acquired heterochromia in a child with macrosomia

To the Editor: We describe a unique case of childhood acute myeloid leukemia (AML) presenting with the rare constellation of pseudohypopyon, a preceding transient episode of pancytopenia, and a presumed underlying overgrowth syndrome. A 2-year-old female child presented with 1 month of bilateral conjunctival injection. On examination, she had acquired heterochromia, with her left eye having changed to a different color (Figure 1, written informed consent for patient photography obtained). Formal ophthalmology evaluation with slit lamp revealed ecchymosis of both eyelids, bilateral subconjunctival hemorrhage, and a blue right iris with a golden-brown left iris. The left eye also had increased intraocular pressure and a grayish-white vascular mass in the inferior aspect of the anterior chamberwith diffuse gelatinousmaterial covering the iris surface. The optic nerves and retinae appeared normal. There were cells in the anterior chamber at such high density that they layered out by gravity, defined as a pseudohypopyon, and the collection of cellswas so large that it appeared to be an iris mass, causing acquired heterochromia. Flow cytometry and bone marrow biopsy revealed a diagnosis of AML with mixed-lineage leukemia gene rearrangement detected by florescence in situ hybridization. There was no leukemia in the cerebrospinal fluid. Magnetic resonance imaging of the head showed medullary and extramedullary involvement of the orbits without optic nerve or leptomeningeal enhancement. Induction chemotherapy was initiated and within days, the iris mass began to resolve and the iris color began to revert to its normal blue hue. Six months earlier, the patient had been hospitalized with pancytopenia; bone marrow biopsy showed hypocellularity with no blasts and normal cytogenetics. No specific etiology was identified and blood counts normalized over the following 6 weeks. Overall, she had been healthy apart from an undefined overgrowth syndrome. At birth, she was at the 85th percentile for length and weight, and reached above the 95th percentile in both by 4 months of age, indicating a general macrosomia. At the time of her leukemia diagnosis, she was far above the 95th percentiles for height, weight, and head circumference. Her development was normal except for mild gross motor delay attributed to hermacrosomia. She had been evaluated by pediatric endocrinology and genetics and evaluation revealed no unifying diagnosis. Reports of ocular manifestations are more common in the pediatric acute lymphoid leukemia (ALL) literature, though this may largely be due to the greater incidence of ALL in children.1,2 Bitirgen et al. performed a retrospective study of 185 patients who underwent full ophthalmologic examination at the time of diagnosis with leukemia, noting ocular involvement to be more common in myeloid than lym-

Corneal Diseases in Children: Amblyopia Management

Amblyopia is an additional challenge to the visual rehabilitation of a child with a corneal opacity. Although amblyopia is defined as impaired vision, usually in one eye, that is structurally normal, amblyopia often coexists in a child with a corneal disorder. Key concepts include critical period of visual development, assessment of vision in children, identification of amblyopia risk factors, definition of amblyopia, and treatment options for amblyopia. The critical period of visual development spans birth to about age 10, a time in which proper visual input from each eye is paramount in order to “teach” the visual cortex the characteristics of excellent vision. There are a variety of methods to assess visual acuity; often a decision about visual impairment needs to be made over several office visits to obtain reproducible and accurate information. Amblyogenic risk factors include asymmetric refractive error (anisometropia), high refractive error in both eyes (ametropia), misaligned visual axes (strabismus), and media opacity. In the preverbal child, a decision to treat amblyopia is made on the basis of an identified amblyogenic risk factor or demonstration of asymmetric visual acuity. There may be a difference between the eyes on preferential looking testing or consistent objection to patching one eye versus the other. In the verbal child, amblyopia is confirmed when a two-line or greater difference in vision is identified, despite correction of any refractive error. Familiarity with treatment options, such as glasses, patching, pharmacologic, and optical penalization is crucial to improve vision.