bluuebunny/crossref_metadata_2025_split huggingface dataset:
Ophthalmologic Findings in the Cornelia de Lange Syndrome
Authors: Levin Alex V , Seidman David J , Nelson Leonard B , Jackson Laird G 
Published: April 2021

ABSTRACT
Cornelia de Lange Syndrome (CDLS) is characterized by mental retardation, growth retardation, skeletal anomalies, and a characteristic facies. We examined 22 children with CDLS to ascertain the relative frequencies of oculofacial and ophthalmologic abnormalities. We confirm prior reports of the frequent findings of brow hypertrichosis, synophrys, and long arcuate eyelashes. In addition we documented the association with ptosis, nystagmus, and high myopia. Contrary to previous reports, we did not find blue sclera or corneal, pupillary, or retinal disorders to be part of this syndrome. Hypertelorism and telecanthus were found, but they were dependent on the method of calculation. Two children had optic nerve pallor. We also report the new findings of poor macular reflex, chin lifts induced by ptosis, hypertropia, and nasolacrimal duct fistula.
Congenital Corneal Anesthesia: Problems in Diagnosis
Authors: Shorey Pavan , Lobo Grace 
Published: April 2021

ABSTRACT
Congenital corneal anesthesia (CCA) is a rare clinical entity that presents a whole gamut of problems before lending itself to a diagnosis by the clinician. Not recognizing the history of self-inflicted corneal injuries, not evaluating the corneal sensations in a child with recurrent corneal ulceration, misdiagnosis of such cases as viral keratitis, and not ruling out all the secondary causes of corneal anesthesia, as well as the other causes of self-inflicted injuries, are some of the pitfalls in the diagnosis of congenital corneal anesthesia.
Four cases representative of different aspects of CCA are presented. The problems in the diagnosis of these cases are discussed. A clinical classification of congenital corneal anesthesia is suggested and the systemic anomalies associated with this disorder are enumerated.
The Acuity Card Procedure: Longitudinal Assessments
Authors: Hartmann E Eugenie , Ellis George S , Morgan Keith S , Love Angela , May James G 
Published: April 2021

ABSTRACT
Traditional methods of visual assessment in preverbal pediatric patients rely on refined but subjective measurement techniques. A standard ophthalmologic examination includes evaluation of a child's fixation patterns, with performance ranked on the basis of ability to fix and follow an object (F & F) or maintain central, steady fixation (CSM). In the hands of a skilled clinician, these evaluations are important for diagnosis and treatment. Documentation of quantitative changes in visual abilities of preverbal patients, however, has only recently become feasible.
We began using the acuity card procedure in our pediatric clinical practice more than 3 years ago. This assessment, a modified version of the standard Forced-Choice Preferential Looking paradigm (FPL), provides quantitative evaluation of visual functioning in preverbal patients. The total number of patients assessed on one or more occasions exceeds 900. Of this group, we followed 83 patients with at least four acuity card evaluations on separate visits. Thirty of these patients, all with different diseases, have been evaluated with acuity cards on six or more visits.
We found the information provided by the acuity card assessments extremely helpful in quantifying the developmental and therapeutic changes in vision, previously monitored only qualitatively.
Head Nodding Associated With Intermittent Esotropia
Authors: Rubin Steven E , Slavin Michael L 
Published: April 2021

ABSTRACT
Head nodding (to-and-fro turning about the vertical cervical axis) associated with abnormal eye movements may be seen in spasmus nutans and congenital nystagmus. In the absence of abnormal eye movements, it may be indicative of neurological disease (eg, cerebellar disease, basal ganglia dysfunction). We report a neurologically normal infant without nystagmus but with intermittent head nodding and intermittent esotropia, whose head movements manifested only when his eyes were straight. The head movements ceased with the occlusion of either eye or spontaneous onset of esotropia. When his head was forcibly stabilized, he immediately developed esotropia. The head movement presumably facilitated fusion, although the mechanism of action is unknown.
Stickler's Syndrome in the Cleft Palate Clinic
Authors: Kronwith Stephen D , Quinn Graham , McDonald Donna M , Cardonick Elyce , Onyx Pam , LaRossa Donato , Borns Patricia , Stambolian Dwight E , Zackai Elaine H 
Published: April 2021

ABSTRACT
Stickler's syndrome is a much underdiagnosed entity in the ophthalmic population. It is a dominantly inherited disease of connective tissue whose ocular findings include moderate to severe myopia, vitreoretinal degeneration, retinal detachments, cataracts, and glaucoma. Non-ophthalmologic findings include cleft palate, midfacial hypoplasia (Figs 1-2), radiographic changes of spondyloepiphyseal dysplasia, narrow pelvis, and broad femoral neck.
Twenty percent of patients with Stickler's syndrome will have a cleft palate. We undertook a study to determine the incidence of Stickler's syndrome in patients with an isolated cleft palate, and to see if this screening process would be useful in making an early diagnosis of the syndrome and in genetic counseling.
It is important to distinguish this syndrome from that of isolated cleft palate in order to: 1) insure early detection of myopia and monitor for signs of retinal detachment, cataract, and glaucoma; and 2) provide definitive recurrence counseling for families (50% vs 2.3%).
The Ocular Pathology of Trisomy 22: Report of Two Cases and Review
Authors: Antle C Mark , Pantzar J Tapio , White Valerie A 
Published: April 2021

ABSTRACT
Two cases of full trisomy 22 with the associated gross and microscopic pathology are reported. These cases demonstrate the typical craniofacial and organ system anomalies previously reported in trisomy 22 but also exhibit uveal colobomas that, within the spectrum of chromosome 22 anomalies, are usually restricted to the so-called "cat eye" syndrome. The attendant microscopic ocular findings represent, to the best of our knowledge, the first such report in the literature.
Mesectodermal Leiomyoma of the Ciliary Body: New Evidence for Neural Crest Origin
Authors: Yu Dao-Yi , Cohen S B , Peyman G , Tso M O M 
Published: April 2021

ABSTRACT
An 8-year-old white boy had a mass of the ciliary body in his left eye. Both the translucent characteristics and the magnetic resonance imaging findings suggested that this lesion was cystic. Fluid aspiration biopsy and iridocyclectomy were performed. By light microscopy the neoplasm showed the characteristic appearance of a neuroid tumor; however, on electron microscopic examination, the tumor exhibited characteristic features of a smooth muscle neoplasm and was diagnosed as a mesectodermal leiomyoma. Observation of melanin granules in scattered tumor cells further confirmed that the tumor had the same origin as uveal melanocytes, which also derive from the neural crest.
Recovery of Phorias Following Monocular Occlusion
Authors: Holmes Jonathan M , Kaz Kian M 
Published: April 2021

ABSTRACT
Fourteen nonstrabismic volunteers were monocularly patched for 2 and 24 hours in separate experiments. Horizontal and vertical phorias were measured at 6 m and 30 cm, at 30-second intervals, for at least 30 minutes, following removal of the patch. After 24 hours of monocular occlusion, the initial change from baseline at 6 m ranged from 9.5 prism diopters exo to 7 δ eso and 6.5 δ hyper to 3 δ hypo. At 30 cm, the initial change ranged from 7.5 δ exo to 4 δ eso and 1 δ hyper to 1 δ hypo. In all but three subjects, phorias returned to within 2 δ of baseline by 3 minutes, and in all subjects by 25 minutes. After 2 hours of monocular occlusion, the range of initial change from baseline was similar to 24 hours of occlusion, but all phorias returned to within 2 δ of baseline by 2.5 minutes.
Therefore, we suggest that ocular alignment should not be routinely measured within 3 minutes of removing a patch. If patched for 24 hours, a few individuals will require up to 25 minutes for stabilization of their deviation. Further studies might address these effects in patients with subnormal fusion and stereopsis.
Regression Pattern in Retinopathy of Prematurity
Authors: Preslan Mark W , Butler John 
Published: April 2021

ABSTRACT
We characterized the regression pattern of retinopathy of prematurity (ROP) for 266 infants examined over a 22-month period. Infants were included in the evaluation with a birth weight of less than or equal to 1500 g. Regression of retinopathy was observed in all but 11 infants, who were treated for threshold ROP. ROP limited to the peripheral retina resolved around term (40 weeks postconceptional age). Posterior ROP and/or stage 3 ROP underwent a protracted course of resolution, often not reaching zone 3 until 42 to 45 weeks after conception. Permanent retinal/vascular sequelae of ROP were observed in 10% of infants with ROP. The most common abnormality was failure to completely vascularize the temporal retinal periphery. Overall, retinal morbidity from ROP was an infrequent occurrence (18 of 266 premature infants - 6.8%).
Randomized Clinical Trial of Surfactant Prophylaxis in Retinopathy of Prematurity
Authors: Holmes Jonathan M , Cronin Cathleen M , Squires Peggy , Myers Thomas F 
Published: April 2021

ABSTRACT
Prophylactic lung surfactant Is commonly used to reduce the severity of neonatal respiratory distress syndrome in premature infants. There is disagreement in the literature regarding the effect of prophylactic lung surfactant on the incidence of retinopathy of prematurity (ROP). Sixty-four Infants, gestatlonal age 23 to 32 weeks, birth weight 610 to 1250 g, were randomized to receive either intratracheal bovine surfactant prophylaxis or air control, at our institution, as part of a national doublemasked multicenter trial. Forty-eight of these infants survived and underwent complete ophthalmologic examinations by a single masked examiner. ROP data were gathered retrospectively. ROP developed in 1 9 of the 23 (83%) who received surfactant and 15 of the 25 (60%) controls (P = .1). Analysis of the worst stage of ROP for each infant also revealed no difference between the surfactant and control groups (P = .4). Our retrospective analysis of ROP data in a prospective double-masked randomized study revealed no significant effect of surfactant on the incidence or severity of ROP.
Susceptibility to Retinopathy of Prematurity in Alaskan Natives
Authors: Arnold Robert W , Kesler Kenneth , Avila Emilio 
Published: April 2021

ABSTRACT
Threshold retinopathy of prematurity occurred in 11 of 34 Alaskan natives compared with 10 of 93 non-natives. Natives constitute 16% of the state population. This significant Alaskan native preponderance was not explained by differences in prenatal or intensive care unit morbidity except that the intervals from birth to extubation and birth to cryotherapy were shorter for natives.
A Gyroscope Deviometer for Measurement of Strabismus
Authors: Miller Kevin M , Cooper Kim L , Guyton David L 
Published: April 2021

ABSTRACT
We describe a hand-held gyroscope deviometer that an examiner can use to measure strabismic deviations without prisms. The deviometer is ideally suited for use with small children who will not allow prisms to be held near their eyes, and adults with poor fixation in one eye. The device consists of a fixation light attached to a gimbal-mounted single-axis gyroscope. The gyroscope provides the necessary reference for horizontal deviations. Gravity provides the reference for vertical deviations. As the patient observes his or her reflection with the fixing eye in a plane mirror at the end of the room, the examiner positions the corneal light reflex in the pupil of the deviating eye and presses a rocker switch to the "reset" position, zeroing the deviometer. The examiner then aims the deviometer fixation light at a reflection of itself in a plane mirror located on the wall behind the subject and presses the rocker switch to the "read" position. Horizontal and vertical deviations are displayed to the nearest degree. The accuracy of the device is dependent upon the examiner's ability to position the corneal light reflex correctly in the pupil of the deviating eye.
Brain Electrical Activity Mapping in the Study of Visual Development and Amblyopia in Young Children
Authors: Thouvenin Dominique , Tiberge Michel , Arne Jean-Louis , Arbus Louis 
Published: April 2021

ABSTRACT
Brain electrical activity mapping (BEAM) allows the study of electrical visual reactivity on a computerized electroencephalogram (EEG). We carried out 150 BEAM studies on 120 infants to evaluate the usefulness and reliability of this noninvasive technique In the assessment of vision in very young children, compared with other methods (clinical testing, preferential looking, and visual evoked potentials). BEAM demonstrated amblyopia at a cortical level and showed specific electrical signs of amblyopia. The visual reactivity was variably affected depending on the type of amblyopia present. In addition, different results of BEAM corresponded to different kinds of visual maturation delay and strabismus in the absence of amblyopia. BEAM appears to be useful in the initial screening and during treatment of deprivation and strabismic amblyopia, especially when other methods have failed to elicit the level of vision.
Can Adjustable Suture Surgery Be Performed With Conscious Sedation?
Authors: Carruthers Jean D A , Mills Keith , Bagaric Diana 
Published: April 2021

ABSTRACT
We retrospectively reviewed the charts of 50 consecutively referred adult patients with horizontal ocular misalignment only. We wanted to evaluate anesthesia by conscious sedation as an alternative to general anesthesia for the primary surgical stage of adjustable suture repair. Parameters regarding patient acceptance, strabismus correction, and health-care facility utilization were studied. Conscious sedation is a safe and effective alternative to general anesthesia for adjustable suture repair of horizontal misalignment in adult patients.
Anterior Transposition of the Inferior Oblique in the Treatment of Unilateral Superior Oblique Palsy
Authors: González Caleb , Cinciripini Grace 
Published: April 2021

ABSTRACT
Anterior transposition of the inferior oblique (ATIO), is an accepted surgical procedure for the treatment of primary inferior oblique overaction and dissociated vertical deviation. Our study was undertaken to see if ATIO could be useful in the treatment of preselected unilateral superior oblique palsy (SOP) patients. Three consecutive patients with unilateral SOP with preoperative primary-position hypertropia averaging 27 Δ, Knapp class V, underwent ATlO. The results were excellent and none of these patients developed primary-position hypotropia. Complications of ATIO in our patients consisted of some elevation deficiency, elevation of the lower Hd in upgaze, and reduced inferior "scierai show" in the surgically treated eye. We are proposing that ATIO be considered as a beneficial operation in unilateral SOP patients with at least 25 Δ of preoperative primaryposition hypertropia.
Pseudohypopyon in Acute Myelogeneous Leukemia
Authors: Ellis Anna , Clarke William N , Noel Léon-Paul 
Published: April 2021

ABSTRACT
An 11-month-old boy presented with uniocular hypopyon, elevated intraocular pressure, and iris nodules. A differential diagnosis of physical abuse, infection, retinoblastoma, juvenile xanthogranuloma, and histiocytosis X were considered but initial physical and laboratory investigations all had normal results. Three weeks following initial presentation, the child developed fulminant acute myelogenous leukemia. The clinical course, investigations, and outcome are reviewed.
Vertical Shift of the Medial Rectus Muscles in the Treatment of A-Pattern Esotropia: Analysis of Outcome
Authors: deB Ribeiro Geraldo , Brooks Steven E , Archer Steven M , Del Monte Monte A 
Published: April 2021

ABSTRACT
A retrospective study was performed on 18 consecutive patients with A-pattern esotropia and no apparent oblique muscle dysfunction, mechanical restriction, paresis, or previous muscle surgery. All patients underwent graded bilateral medial rectus recession for their esotropia with simultaneous vertical upshift to treat the A-pattern. The quantitative relationship between amount of upshift, amount of A-pattern correction, preoperative A-pattern, and preoperative esotropia was examined. We found that the amount of A-pattern correction was closely correlated with the size of the A-pattern preoperatively (r=0.83), independent of amount of upshift. While the change in A-pattern did correlate with the amount of the upshift (r=0.60), it was not a significant independent predictor of the surgical response. The amount of recession had little influence on the effectiveness of the procedure in correcting the vertical incomitance, and the transposition did not seem to affect the correction of the basic esotropia, adversely. We conclude that medial rectus recession with vertical upshift of the muscle insertions is an effective procedure for correcting the vertical incomitance in A-pattern esotropia, and that the amount of A-pattern correction achieved is determined primarily by the size of the preoperative A-pattern and not the amount of upshift.
Botulinum A Toxin Treatment of a Deviated Orbital Implant
Authors: Wortham Edwin , Choi Rosan Y , Young Steven R 
Published: April 2021

ABSTRACT
We report a case of progressive deviation of an implant that resulted in conjunctival thinning and an inadequately fit prosthesis. Such a case may have been treated with conjunctival grafting or replacement of the implant with an hydroxyapatite sphere. In this case, injection of botulinum A toxin (Botox) into the contracted medial rectus muscle contributed to the successful refitting of a new and stable prosthesis and improvement of the conjunctival integrity. Possible explanations of the progressive shift in the position of the implant and its postinjection stability are discussed.
Bilateral Monocanalicular Silicone Loop: Predictable Home Removal of Nasolacrimal Stents
Authors: Arnold Robert W 
Published: April 2021

ABSTRACT
Premature dislocation, a potential disadvantage of conventional, unilateral silicone intubation for lacrimal disease, can be alleviated with a firmly-tied, bilateral, superior canaliculus intubation. Since the looped silicone stent can be removed easily at home by parents, this technique is particularly beneficial for remote patients for whom postoperative, clinic removal is a financial burden.
Bilateral Infantile Cataractogenesis in a Patient With Deficiency of Complex I, A Mitochondrial Electron Transport Chain Enzyme
Authors: Ciulla Thomas A , North Kathryn , McCabe O'Ine , Anthony Douglas C , Korson Mark S , Petersen Robert A 
Published: April 2021

ABSTRACT
Progressive bilateral cataracts developed in infancy in a 5-month-old girl with deficiency of complex I, a mitochondrial electron transport chain enzyme. In the newborn period, she had severe lactic acidosis and the diagnosis of complex I deficiency was confirmed by mitochondrial respiratory chain assay on muscle biopsy. By 5 months, she had completely opaque nuclear sclerotic cataracts, with loss of fixation and the red reflex. She underwent bilateral, sequential cataract extraction. The lens aspirate was submitted for cytologie analysis and electron microscopy, which revealed increased intracellular glycogen and swollen mitochondria. To our knowledge the association of complex I deficiency with cataracts in infancy has not been reported previously. The diagnosis of a respiratory chain enzyme defect in infancy is an indication for early ophthalmic evaluation to identify cataracts that may result in visual loss. Conversely, the recognition of cataracts in infants with unexplained neurologic disease or metabolic acidosis may necessitate further evaluation for metabolic etiologies, including mitochondrial disorders.
Infant Random Dot Stereoacuity Cards
Authors: Birch Eileen E , Salomão Solange 
Published: April 2021

ABSTRACT
Purpose: The overall goal was to develop a simple test of random dot Stereoacuity that can be used during the first 24 months of life to measure sensory outcomes following treatment of ophthal moped iatric disorders, both in the context of clinical trials and in the context of clinical management.
Methods: A series of random dot Stereoacuity test cards were constructed using vectographic vertical bar stimuli with crossed disparities ranging from 1735 to 45 sec. A two-alternative, forced-choice, preferential-looking test protocol was used to measure Stereoacuity in 95 healthy term infants (173 tests) over the 1.5- to-24-month age range. Success rate, validity, and tolerance limits for normal performance as a function of age were evaluated.
Results: Jhe success rate was high (158 of 173 tests; 91.3%). The maturation of random dot Stereoacuity was similar to that measured In earlier lab-based preferentiallooking and visual evoked potential protocols; few Infants younger than 2 months of age demonstrated stereopsls although by 12 months, mean Stereoacuity was 2.1 log sec (120 sec). A further improvement to 1.7 log sec (56 sec) was seen during months 18 to 24, consistent with data obtained in an earlier opérant random dot protocol. Tolerance limits for normal ranged from approximately 1000 sec at 6 months to 100 sec after 12 months.
Conclusions :ihe Infant random dot Stereoacuity cards provide a simple, quick, and portable preferential-looking test of random dot Stereoacuity for infants that has a high success rate and excellent concordance with Stereoacuity outcomes from laboratory-based protocols.
Evaluation of a Hand-held Autorefractor in Children Younger Than 6
Authors: El-Defrawy Sherif , Clarke William N , Belec France , Pham Ba 
Published: April 2021

ABSTRACT
Purpose; To evaluate a hand-held portable a uto refractor in a pediatrie population and compare results with those found on retinoscopy by experienced pediatrie retinoscopists.
Methods: One-hundred and two children aged 5 to 72 months were examined with and without cycloplegia using an autorefractor, and the results compared with those found on standard retinoscopy by two ophthalmologists masked as to the autorefractor findings. Results were converted from conventional notation using plus cylinder to the h-space notation and compared in threedimensional h-space.
Results: There was remarkable agreement between the results found by autorefraction and manual retinoscopy using loose lenses or a phoropter.The findings were similar both for sphere and cylinder across the age ranges studied.
Conclusions: The Nikon Retinomax is an accurate instrument to estimate refractive error in children younger than 6 and could prove useful in the office, the operating room, or as a screening device.