Nonsurgical Consecutive Exotropia Following Childhood Esotropia: A Multicentered Study.

PURPOSE: Nonsurgical consecutive exotropia (NCX) occurs when an esotropia (ET) spontaneously converts to exotropia (XT) without surgical intervention. Although NCX is considered to occur in early-onset accommodative ET with high hyperopia, consensus on causation is lacking. We report the clinical characteristics of NCX and assess the response to conservative management. DESIGN: Retrospective, multicenter, observational case series. METHODS: Patients aged 6 months and older with an initial diagnosis of ET who converted to XT without surgical intervention. Sensory strabismus was excluded. Age, visual acuity, cycloplegic refraction, glasses prescriptions, deviation, and binocular vision were collected. RESULTS: Forty-nine children were included with a mean age of 3.5 ± 1.6 years and 8.4 ± 3.6 years at the time of ET and NCX, respectively. Mean refractive error was +4.40 ± 2.13 diopters (D) and +4.05 ± 2.74 D at the time of ET and NCX, respectively. Accommodative ET occurred in 60% of cases, and only 35.7% were high hyperopes. All but 1 patient presented with XT at distance. In response to the XT, a mean decrease in hyperopic prescription of 1.55 ± 0.48 D was given (N = 17); only 1 case reverted to ET. Eventually, 43% underwent XT surgery, with similar rates between those who had refractive management and those who did not. CONCLUSIONS: NCX occurs in both accommodative and nonaccommodative ET; high hyperopia is present in only one-third of cases. On average, drift to XT occurs within 5 years. Refractive management has a modest result. No predictive risk factors were identified. Our findings challenge hyperopia-linked theories of causation. Nonrefractive explanations, such as the role of the vergence system, deserve further study.

[Changes in accommodation in children with hyperopic anisometropia and unilateral amblyopia after laser refractive surgery and pleoptic treatment]. / Izmenenie akkomodatsii u detei s gipermetropicheskoi anizometropiei i odnostoronnei ambliopiei posle refraktsionno-lazernoi operatsii i pleopticheskogo lecheniya.

PURPOSE: This study comparatively analyzes the state of accommodation in children with hyperopic anisometropia and amblyopia after femtosecond laser-assisted in situ keratomileusis (Femto-LASIK) combined with pleoptic treatment, and after conventional pleoptic treatment. MATERIAL AND METHODS: The first group consisted of 30 children with medium and high hyperopia, high and medium amblyopia, and anisometropia greater than 3.0 diopters, who underwent Femto-LASIK in the amblyopic eye. The second group consisted of 28 children with similar local status, who were prescribed traditional correction and received conservative treatment. The follow-up period was 1.5 years. RESULTS: After 1.5 years, higher visual acuity (p<0.05) was achieved in the first group. A significant increase in the coefficient of accommodative response (CAR) was observed in the operated amblyopic eyes in group 1 - by 0.1±0.02 c.u. compared to the control group (p<0.05). In both groups there was an upwards trend for the coefficient of microfluctuations (CMF) in the amblyopic eye, but in the first group CMF increased more significantly (p<0.05). The objective accommodative response (OAR) and positive relative accommodation (PRA) of the amblyopic eye showed a double increase - by 1.0±0.23 and 0.9±0.38 diopters, respectively, at the end of treatment in the first group. The increase in similar indicators in the second group was insignificant (p<0.05). In children of the first group the difference in ciliary muscle thickness (CMT) of the amblyopic eye with disabled and enabled accommodation increased by 0.04±0.01 mm (p<0.05) in the anterior part of the ciliary muscle at the levels of CMTmax and CMT1. CONCLUSION: The data obtained in this study indicate the strong effect of refractive laser surgery in combination with pleoptic treatment on improving the visual acuity and the state of accommodation of the amblyopic and paired dominant eyes in children with hyperopic anisometropia, in contrast to conventional methods of treatment.

[Anatomical and morphometric features of anterior eye segment structures in hyperopia and the risk of developing primary angle-closure glaucoma]. / Anatomo-morfometricheskie osobennosti struktur perednego segmenta glaza pri gipermetropii i risk razvitiya pervichnoi zakrytougol'noi glaukomy.

PURPOSE: To study features of anatomical and morphometric parameters of the structures of anterior eye segment in young patients with moderate and high hyperopia in order to identify the signs of an increased risk of developing primary angle-closure glaucoma (PACG) and its acute attack. MATERIAL AND METHODS: The study included 160 eyes (80 patients) with axial length (AL) of less than 23 mm. Patients with moderate or high hyperopia were divided into two groups according to their age ranges (the 1st - 27 patients (54 eyes) under 40 years old; the 2nd - 27 patients (54 eyes) of 41-50 years old, the comparison group - 26 patients (52 eyes) of 42-50 years old with the initial stage of PACG. AL of the eyes, anterior chamber (AC) depth in the central zone, lens thickness (LT) in the optical zone were measured using IOL Master 700 («Carl Zeiss Meditec AG¼, Germany). AC volume and peripheral AC depth were measured using rotating Scheimpflug camera Pentacam («Oculus¼, Germany). RESULTS: While the average values of AL in patients of the 1st and 2nd groups were comparable, a statistically significant decrease in AC depth and a significant increase in LT were revealed in the 2nd group. There was a statistically significant increase in LT, a decrease in peripheral AC depth and AC volume in the comparison group relative to the 2nd group. In the 1st group: in 2 eyes of one 38-year-old patient the maximum proximity of all 3 indices to the median values of the group of patients with PACG was found; in 4 eyes of two other patients (35 and 38 years old), a combination of small AC volume with increased LT or small AC volume with small AC on the periphery was noted. CONCLUSION: Significant differences in terms of LT, peripheral AC depth and AC volume were found between age-comparable (41-50 years old) healthy individuals with short eyes and patients with initial PACG. In 11% of the eyes of healthy patients with hyperopia aged 21 to 40 years, there was a combination of two or three of the studied morphometric signs, which may indicate the risk of developing PACG.

Profile of infantile strabismus at a tertiary eye care center in India.

Purpose: To study the profile, risk factors, and management outcomes of infantile strabismus at a tertiary eye care center. Methods: We prospectively analyzed the data of infants (children less than 1 year of age) who presented at our institute from August 2018 to December 2019. We excluded infants who did not complete a minimum follow-up of 6 months. Detailed meticulous history based on a set of standardized questionnaires was obtained and a comprehensive ophthalmological examination of the child was performed. Data were collected regarding refractive error (astigmatism; myopia; hyperopia; anisometropia [<1.0 DS or >1.0 DS]; astigmatism [<1.0 DS or >1.0 DS]) and the type of strabismus. Results: During this period, we saw 4,773 infants, out of which 123 infants were diagnosed to have infantile-onset strabismus (hospital prevalence of 2.6%). Boys and girls were equally affected. Sixty-two patients had esotropia, 37 had exotropia, 2 had hypotropia, and 22 had pseudo strabismus. Prematurity, hypermetropia, and anisometropia had increased odds of developing esotropia, whereas delivery by cesarean section, delayed cry at birth, infantile seizures, parental consanguinity, delayed development of milestones, and myopia had increased odds of developing exotropia. Twenty-nine patients underwent a surgical correction. The mean deviation at the first visit was 42.59 ± 15.40 PD and 8.25 ± 12.70 PD at the last visit. For all patients who underwent a squint surgery, the change in ocular deviation was clinically and statistically significant (P-value <0.0001, paired t-test). Conclusion: The hospital prevalence of infantile strabismus in our cohort was found to be 2.6%. Our study suggests that esotropia is two-fold more common in our cohort as compared to exotropia. Further, our study highlights risk factors for the development of strabismus in infancy, which must be kept in mind and awareness must be created among pediatricians. Surgical correction should be considered early during the infantile period, because it may lead to promote the development of good binocular vision.

Angle κ Measurement and Its Correlation with Other Ocular Parameters in Normal Population by a New Imaging Modality.

SIGNIFICANCE: This study obtained normative database for angle κ using Orbscan 3. The average angle κ values were 2.3 ± 1.34° in the whole sample, 2.23 ± 1.36° in myopic eyes, and 3.3 ±1.5° in hyperopic eyes. This is very important to optimize refractive surgery outcomes. PURPOSE: The aim of this cross-sectional study was to obtain the value of mean angle κ in normal eyes of patients seeking laser vision correction, by the Orbscan 3. METHODS: The study was conducted on healthy eyes of people seeking laser refractive surgery. A total of 1815 eyes of 908 candidates were included. These were examined as a part of pre-operative assessment. Orbscan 3 was used to measure angle κ and its XY intercepts, keratometry readings, central corneal thickness, thinnest location, white-to-white diameter, and corneal asphericity of front surface (Qf) and back surface (Qb). RESULTS: The mean ± standard deviation angle κ in all eyes was 2.3 ± 1.34°. The values were 2.23 ± 1.36° in myopic eyes, 3.3 ±1.5° in hyperopic eyes, and 2.9 ±1.6° in emmetropic eyes. Positive correlation was found between angle κ and age, spherical equivalent, subjective sphere, and white-to-white diameter. On the other hand, axial length, average keratometry, central corneal thickness, pupil diameter, and asphericity Q front were negatively correlated with angle κ. CONCLUSIONS: The mean ± standard deviation angle κ value measured by the Orbscan 3 was 2.3 ± 1.34°. This could be attributed to different software used, in addition to the relatively high axial length in the studied eyes.

Acute comitant esotropia in a very young child due to combined mechanism: A case report.

We report a case of esotropia with high hyperopia in a 3-year-old female child. She was initially treated with hyperopic correction and noted to have residual esotropia, which was diagnosed as partial accommodative esotropia. Later when she presented with headache, she was diagnosed to have an intracranial tumour. To our surprise, after neurosurgical excision of tumour, her non-accommodative component of the esotropia resolved over 1 year implying that the intracranial lesion was an additional causative factor for this acute onset Accommodative esotropia. The child attained Orthophoria with the same hyperopic correction.

[Elastotonometry for evaluation of biomechanical characteristics of the eye after surgical hyperopia correction]. / Otsenka biomekhanicheskikh kharakteristik na osnove elastotonometrii posle operatsii po korrektsii gipermetropii.

Modern tonometers (Ocular Response Analyzer) can only show the presence of biomechanical disorders of the fibrous membrane, while elastotonometry reacts not only to their presence, but also to their type. PURPOSE: To build a mathematical model that would use elastotonometry to assess biomechanical properties of the eye in treatment of hyperopia with LASIK and FemtoLASIK surgeries. MATERIAL AND METHODS: The study included 64 operations: 34 FemtoLASIK surgeries, and 31 LASIK surgeries. All patients before and after surgery underwent standard examination necessary for keratorefractive surgery, including elastotonometry. The next step was the analysis of elastotonometry curves and eye finite element model in the software package Ansys (Ansys, Inc.; U.S.A.). During the analysis, the fibrous membrane was modeled by two spherical segments, which before elastotonometry were influenced only by true intraocular pressure (IOP). There was a problem of determining tonometric IOP when the cornea is pressured with flat bottomed loads during elastotonometry. It was taken into account that at the first stage of loading, the IOP was applied, and then the force corresponding to the weight of the tonometer. After tonometry, IOP increased so that the volume inside the composite membrane corresponded to the value before loading. RESULTS: Mathematical modeling has shown that after surgical treatment of hyperopia, flexural stiffness of the cornea decreases, elevation of the elastotonometry curve increases, and the higher the true IOP, the greater the elevation. The greatest increase in lift due to increased IOP is observed after LASIK operation, while after FemtoLASIK this change is not significant, in which case the dependence of tonometric IOP on the weight of the load is almost linear. CONCLUSION: The obtained results allow for elastotonometry to be recommend for further medical research as a promising method for assessing biomechanical characteristics of the fibrous membrane of the eye.

A pilot study: lenticule quality of hyperopic small incision lenticule extraction (SMILE) in rabbits.

BACKGROUND: To evaluate lenticule surface characteristics of small incision lenticule extraction (SMILE) for hyperopia correction in rabbits. METHODS: The left and right eyes of 8 rabbits were divided into two groups. The right eyes were assigned to a myopia group, and the left eyes to a hyperopia group. The rabbits received SMILE procedures with + 3.00 D and - 3.00 D correction for the hyperopia and myopia groups, respectively. Extracted lenticules were examined via scanning electron microscopy. Lenticules from odd-numbered rabbits were accessed with the anterior surface, and lenticules from even-numbered rabbits were observed with the posterior surface. A previously established scoring system was used to evaluate lenticule surface characteristics. Statistical analysis was conducted to compare the scores between the two groups. RESULTS: All procedures were performed successfully, and the lenticules were extracted smoothly. One myopia lenticule that was facing downward was handled failed in preparation for imaging, thus 15 lenticules were ultimately graded. Twelve lenticules exhibited smooth surfaces, and regularly arranged tissue bridges were observed in almost all regions. Three lenticules exhibited a partially rough surface and irregularities affecting more than 10% of the lenticules (2 in the hyperopia group and 1 in the myopia group). Rough lenticules occurred in twice as many lenticules in the hyperopia group compared to the myopia group. CONCLUSIONS: Scan quality of lenticules after SMILE for hyperopia correction is comparable to that of myopia lenticules. The shape of hyperopic lenticule may increase the difficulty of surgical manipulation and result in surface roughness.

Consultation Section: Refractive. Consecutive hyperopia after myopic laser in situ keratomileusis.

A 31-year-old woman was seen at our clinic with dissatisfying hyperopic error after a myopic laser treatment of her left eye in April 2018. The surgery took place 1 year previously. There were no abnormalities in her medical history.The preoperative corrected distance visual acuity (CDVA) in the left eye was 20/16 with -7.50 -0.50 × 75. The refraction in the right eye was -3.50 -1.25 × 90. The cycloplegic correction of the left eye preoperatively was -6.50 -0.50 × 68. A femtosecond-assisted laser in situ keratomileusis procedure was performed with an optical zone of 6.3 mm, an ablation zone of 7.97 mm, and a maximum ablation depth of 121 µm. The laser was set at a correction of -7.50 -0.5 × 75. The temperature and humidity during the laser treatment were 20 degrees and 47%, respectively. shows the excimer laser treatment data (AMARIS 750, SCHWIND eye-tech-solutions).At 1 week postoperatively the uncorrected distance visual acuity (UDVA) was 20/25, and at 1 month postoperatively the CDVA was 20/16 with +2.50. Slitlamp examination showed a clear cornea. No corneal topography was made at that timepoint. At referral 7 months later, the patient's UDVA was 20/100 and CDVA was 20/16 with +2.50 -1.25 × 140 in the left eye. The patient reports decreased vision since the treatment in 2018 and wants to see better without spectacles. shows the postoperative Scheimpflug topography of the left eye, flat keratometry (K1) 36.1 @ 178.4 and steep keratometry (K2) 36.6 @ 88.4. What do you believe could be the cause of this overcorrection? What is your advice in terms of correction of the refractive error for this patient?

Stability of corneal topography and aberrometry after hyperopic laser in situ keratomileusis with a 500-Hz excimer laser platform: A 3-year follow-up study.

PURPOSE: The aim of this study is to analyze the long-term stability of the corneal topography, the functional optical zone, and the refractive stability throughout 3 years following laser in situ keratomileusis surgery for hyperopia using a 500-Hz excimer laser system. METHODS: This retrospective consecutive observational case series study comprised 66 eyes that underwent laser in situ keratomileusis to correct hyperopia with a postoperative follow-up of 3 years. Laser in situ keratomileusis procedures were performed using the SCHWIND Amaris 500-Hz excimer laser. Main outcomes measured were stability of the functional optical zone at corneal topography and corneal aberrometry. RESULTS: Statistically significant differences were found in simulated keratometry (K2 (steep meridian) and Km (mean keratometry)) between 3 and 36 months postoperatively (p ⩽ 0.01); these differences disappeared at 12 and 36 months (p ⩾ 0.18). No statistically significant changes were observed in the horizontal and vertical diameter of the functional optical zone throughout the whole follow-up (p ⩾ 0.07). A statistically significant difference was found in the spherical aberration between 3 and 36 months (p = 0.02); this difference disappeared when compared between 12 and 36 months (p = 0.72). Statistically significant correlations were detected between the vertical functional optical zone and coma root mean square (r = -0.510, p < 0.01) and between the vertical functional optical zone and spherical aberration (r = 0.441, p = 0.02) 36 months after surgery. CONCLUSION: Following 3 years of hyperopic laser in situ keratomileusis with a 500-Hz Amaris excimer laser, keratometry, functional optical zone, and corneal aberrations remain stable from 1 year after surgery. Topographical regression is not observed in hyperopic laser in situ keratomileusis with this excimer laser technology from 1 year after surgery.

Permavision intracorneal inlay after sixteen years. Regression of initial refractive hyperopia.

According to the available scientific literature, 77 patients underwent Permavision inlay worldwide, between 2004 and 2007. This study reported about the use of Permavision intracorneal inlay to increase the central corneal curvature and to correct hyperopia. A 32-year-old male patient went to the Tecnolaser Clinic Vision ® facilities for a refractive study. Preoperative refraction without cycloplegia was +6.00 D in the right eye (RE) and +4.00 in the left eye (LE). The surgery was performed for both eyes on December 2, 2003. The Carriazo-Barraquer mechanical microkeratome (Moria) was used to create a 180 µm-thick corneal flap with a diameter of 8.5 mm. After lifting the flap, the corneal inlay was placed centrally above the pupil and the flap was re-positioned. In this case report, the patient reverted to the initial refractive situation. The first refractive regression appeared at twelve-month follow-up. After sixteen years, it was found a decrease in maximum corneal curvature, an increase in mean corneal densitometry percentage, and no important changes in the central corneal thickness. In the reported case, the cornea reverted to its original shape. In the scientific literature, this is the first case report of a non-explanted Permavision inlay after sixteen years.

Ocular characteristics in a variant microcephalic primordial dwarfism type II.

BACKGROUND: Microcephalic osteodysplastic primordial dwarfism, type II (MOPD II) is a rare disease that is assumed to be caused by a pericentrin (PCNT) gene mutation. Clinical manifestations have been reported in pediatrics and neurology; however, only a few ocular findings have been documented. CASE PRESENTATION: We present three unrelated cases of MOPD II with similar facial features and short stature. Unlike the cases described in the literature, all subjects had normal birth weight and height but their growth was retarded thereafter. In addition to delayed milestones, they have a broad forehead, maxillary protrusion, long peaked nose, high nasal bridge, low-set large ears, extreme reromicrogenia, and normal-sized teeth. These three patients had similar ocular manifestations with the short axial length associated with high hyperopia more than + 9 diopters (D) and macular scarring. The oldest subject was a 20 year-old male without neurological symptoms. One female subject had developed alopecia during the previous 2 years. The other female subject had moyamoya disease, but a genetic study revealed a normal PCNT gene. CONCLUSION: This is the first report of MOPD II focusing on ocular findings, suggesting that macular dystrophy and high hyperopia are the common ocular characteristics of MOPD II. Prompt referral to an ophthalmologist is essential. Although refractive amblyopia can be treated with optical correction, visual prognosis may be poor due to maculopathy.

Eye Conditions in Infants and Children: Myopia and Hyperopia.

Patients with poor vision screening results should be referred to an ophthalmology subspecialist for further testing to define the refractive error. Refractive errors are influenced by the optical power of the cornea and lens, along with the length of the eye (ie, total of the lens thickness, anterior, and vitreous chamber depth). Refractive errors include myopia, in which the visual image focuses in front of the retina, and hyperopia, in which the visual image focuses behind the retina. Patients with myopia, or nearsightedness, are able to see near objects better than those at a distance. Being outdoors or participation in physical activity outdoors may prevent myopia. Eyeglasses, surgery, and pharmacotherapy also have been studied to correct and prevent progression of myopia. Patients with hyperopia, or farsightedness, have good distance vision but may have more difficulty with reading. Another concern for children with hyperopia is development of strabismus because of refractive error. Eyeglasses and surgery are the management options for hyperopia.

Progressive High Hypermetropic Shift as a Refractive Surprise Following Glaucoma Filtration Surgery in a Phakic Child With Early-Onset Childhood Glaucoma Associated With Axenfeld-Rieger Anomaly.

PURPOSE: We report a case of progressive high hypermetropia following glaucoma filtration surgery in a child with Axenfeld-Rieger syndrome and congenital glaucoma. METHODS/RESULTS: We report a case of a 4-day-old female child presented as a case of Axenfeld-Rieger anomaly with secondary glaucoma in both eyes (OU), who underwent combined trabeculotomy with trabeculectomy in OU at the age of 3 weeks. On postoperative third month, cornea cleared and posterior embryotoxon was noted in OU with Habb's striae in OS. Intraocular pressure (IOP) was controlled and fundus was normal in OU. Refraction was +2.00 D sph. in OD and 3.00D sph. in OS and was observed. On postoperative eighth month, IOP was controlled in OU, whereas retinoscopy showed refraction of +4.00D sph./-2.00D cyl at 30 degrees in OD and +10.00 D sph/-3.00 cyl at 120 degrees in OS, glasses were prescribed and was asked to review. After 4 years, the patient presented with blurring of vision, the best-corrected visual acuity noted was 20/100 in OD and 20/320 in OS. IOP was 28 and 14 mm Hg in OD and OS, respectively, but with healthy optic disc in OU. Refractive error had increased and was +5.50D sph in OD and +14.00D sph/-5.00 D cyl at 90 degrees in OS. Corneal topography showed cornea plana in OU, which was more in OS (K1: 32.6D and K2: 38.9D) compared with OD (K1: 38.7D and K2: 40.1D). The patient was started on glaucoma medications in OD. Four months later, IOP was controlled and refraction was stable in OU. CONCLUSIONS: Present case is the first to describe the unusual presentation of progressive high hypermetropia in a child with Axenfeld- Rieger anomaly with congenital glaucoma after surgical intervention for glaucoma. Childhood glaucoma is classically associated with myopic shift in refraction and refraction is one of the most important clinical parameter measured at every follow-up visit. Although progressive hypermetropic shift is a rare occurrence, clinicians should be aware of this possibility. Keratomerty should be performed when such refractive surprises arise, which may help detect the clinical condition and the etiology for such presentation.

Avaliação e triagem da acuidade visual em escolares da primeira infância / Evaluation and screening of visual acuity in early childhood schoolchildren

Resumo Objetivo: Identificar a prevalência de alterações visuais em crianças de cinco anos em escolas públicas de Curitiba-PR. Métodos: As escolas foram selecionadas aleatoriamente dentro do município de Curitiba. As crianças com cinco anos completos em 2017 foram avaliadas com a tabela de Snellen, através de distância mínima correta para nitidez de imagem e teste de Hirschberg. Os pais responderam um questionário sobre uso de telas, sintomas oculares e histórico familiar da criança. Os resultados das avaliações foram analisados estatisticamente considerando nível de significância p≤0,05. Resultados: Em uma população de 459 crianças triadas, 219 (47,7%) pertenciam ao sexo feminino e 240 (52,3%) masculino, sendo que do total, 100 foram encaminhadas para avaliação oftalmológica especializada. A partir da triagem observou-se a prevalência de miopia de 10,7%, hipermetropia de 17,6% e estrabismo de 0,9%. Houve relação entre genitores com miopia e filhos míopes (p<0,05). Dentre as queixas oftalmológicas predominaram cefaleia (30,4%) e franzir de testa (10%). Conclusão: A prevalência de alterações visuais encontrada foi de 21,8%. A relação entre distúrbios visuais e o histórico familiar se mostrou estatisticamente significativa. Entretanto, apenas o tempo médio em frente à televisão apresentou influência, dentre os hábitos de vida, sobre as alterações da AV (p=0,028). Queixas oftalmológicas apesar de frequentes, não apresentaram correlação expressiva com a diminuição da acuidade visual.

Abstract Objective: To identify the incidence of visual impairment in 5-year-old children in public schools from Curitiba-PR. Methods: A selection of schools has been choosen randomly from Curitiba. The children, with completed 5 years at end of 2017 have been evaluated using Snellen table, trough minimum distance for image sharpness and Hirschberg test. Parents answered a questionnaire about the use of screens, ocular symptoms and family history of the child. Significance levels were defines as begin p≤0,05. Results: The results have shown that four hundred fifty-nine children were screened. Two hundred nineteen are female (47,7%) and two hundred fourty (52,3%), male. From all screened patients, one hundred were refered to specialized oftalmic evaluation. After trial completing, has been attained a prevalence value of 10,7% for myopia, 17,6% of hyperopia and 0,9% of strabismus. Strong correlation between parents and children has been undiscovered (p<0,05). From listed oftalmic complaints, headache (30,4%) and frown (10%) where most prevalent. Conclusion: It has been attained that the prevalence of visual acuity is 21,8%. The relation between visual acuity alteration and familiar history has been shown to be significant related. On the other side, the average time in front of television has been shown the only habit that has correlation with visual acuity reduction (p=0,028). Vision complaints, although very frequent, doesn't translate into increased probability of visual acuity alteration.

Síndrome de Moebius incompleto. Presentación de un caso clínico / Incomplete Moebius syndrome. Clinical case presentation

RESUMEN El síndrome de Moebius es un trastorno polimalformativo no progresivo que se caracteriza por parálisis facial congénita. Se define como una "parálisis congénita de los núcleos de los pares craneales VI y VII, cuyo espectro clínico es variable y se asocia a múltiples malformaciones óseas y musculares. Es poco frecuente y de etiología vascular, genética o multifactorial. El trabajo, basándose en los fundamentos teóricos más actualizados, pretendió describir las manifestaciones clínicas del síndrome de Moebius y su posible etiología, a propósito de un caso. Se trató de un paciente de 11 años de edad, que al nacimiento presentó asimetría facial, desviación de la comisura labial hacia la izquierda, boca semiabierta, lagrimeo constante y pabellón auricular derecho malformado. Por ser una entidad clínica poco conocida, se expuso el presente caso, portador de un síndrome de Moebius incompleto de causa vascular y multifactorial (AU).

ABSTRACT Moebius syndrome is a non-progressive poli-formative disorder characterized by facial congenital paralysis. It is defined as a congenital paralysis of the VI and VII cranial nerves nuclei, the clinical spectrum of which is variable and associated to several bone and muscular malformations. It is few frequent and has vascular, genetic or multifactorial etiology. This work, based on more updated theoretical fundaments, pretended to describe the clinical manifestations of the Moebius syndrome and its possible etiology on the purpose of a case. It is the case of a patient, aged 11 years, who presented facial asymmetry, lips commissure deviation to the left, semi-opened mouth, constant lagrimeo and deformed right auricular pavilion (pabellon auricular). Because it is a little known clinical entity, this case of a patient having an incomplete Moebius syndrome of vascular and multifactorial cause was presented (AU).

Clinical and imaging characteristics of posterior column ataxia with retinitis pigmentosa with a specific FLVCR1 mutation.

BACKGROUND: Posterior column ataxia retinitis pigmentosa (PCARP) with feline leukemia virus subgroup C cellular receptor 1 (FLVCR1) gene mutation is a rare disorder with significant ophthalmic features. MATERIALS AND METHODS: We conducted a retrospective case series study of patients diagnosed with PCARP and genetic testing positive for FLVCR1 mutation between 1 January 2015 and 1 October 2017 at the Children's Hospital of Pittsburgh. Clinical charts, visual fields, fundus autofluorescence, and spectral-domain optical coherence tomography (SD-OCT) were reviewed. RESULTS: Seven patients from three families were identified to have PCARP and FLVCR1 mutation. The median age at presentation was 13 years (range, 7-28 years). Common clinical exam findings were astigmatism, cataracts, and vitreous syneresis. Funduscopy on all patients revealed bull's eye maculopathy, retinal vessels attenuation, and bone spicule changes in the peripheral retina. Fundus autofluorescence showed bilateral hyperautofluorescent rings. SD-OCT demonstrated morphological changes, which differed based on age. The youngest sibling family exhibited peripheral loss, but subfoveal preservation of the outer retinal layers. These layers were lost in the oldest sibling family. Visual fields loss paralleled SD-OCT findings. CONCLUSION: There is limited published ophthalmic data on FLVCR1-related PCARP. We describe clinical and retinal imaging features in the one of the largest cohorts of affected patients in the literature. Given the availability of genetic testing for this phenotype, testing for FLVCR1 mutations should be considered in pediatric and adult patients with sensory ataxia and retinitis pigmentosa.

Ophthalmic manifestations in patients with Leigh syndrome, French Canadian type.

BACKGROUND: Leigh syndrome, French Canadian type is a rare neurodegenerative disease. To our knowledge, there have been no studies based on ocular findings published for this disease. The purpose of this study is to describe ophthalmic findings in these patients. PATIENTS: Six patients genetically identified as having the syndrome were included in this study. METHODS: Four patients had an ophthalmic examination with an ophthalmologist including evaluation of visual acuity, extraocular motility and lid position, orthoptic workup, evaluation of stereopsis, refraction, evaluation of pupils, color vision, slit-lamp biomicroscopy, measurement of intraocular pressure, and fundoscopy. Two patients had a chart review. RESULTS: Visual acuity ranged from 0.00 logmar to 1.55 logmar. Extraocular motility abnormalities and ptosis were noted in half of the patients. Strabismus was present in the entire cohort, and stereopsis was absent in half of these patients. Amblyopia was noted in 83% of individuals and suppression in 33%. Only one patient had nystagmus. Refraction varied throughout patients. It included severe hyperopia, myopia, astigmatism, and significant anisometropia. Pupils, anterior segment, fundus, and color vision were normal in all patients. Intraocular pressure was slightly elevated in one patient. CONCLUSION: Patients with Leigh syndrome, French Canadian type display a variety of ophthalmic findings, and screening at a young age is recommended.

Tomographic changes in eyes with hyperopic shift after triple Descemet membrane endothelial keratoplasty.

PURPOSE: To describe the tomographic changes in eyes with a hyperopic shift after triple Descemet membrane endothelial keratoplasty (DMEK). SETTING: Private cornea practice, Blue Ash, Ohio, USA. DESIGN: Retrospective case series. METHODS: Patients who had previous triple DMEK with preoperative and postoperative Scheimpflug corneal tomography (Pentacam) measurements and corresponding manifest refraction measurements were reviewed. Eyes with and without a hyperopic postoperative shift were compared at either the 1-month or 3- to 6-month follow-ups. Assessed parameters included demographics, cataract testing, visual acuity, manifest refraction, and Scheimpflug tomography data. RESULTS: Sixty-two eyes of 47 patients (21 men, 26 women) with Fuchs endothelial corneal dystrophy were included. Preoperative posterior flat keratometry (K), posterior steep K, posterior average K, central pachymetry, and asphericity (Q value) of the posterior cornea were significantly different when comparing the hyperopic and non-hyperopic group. At the postoperative 1-month and 3- to 6-month follow-ups, there was a significant between-group difference in change in postoperative anterior K (average), posterior K (average), central pachymetry, and posterior Q values compared with preoperative values. The combined preoperative criteria of posterior flat K (≥-5.25), central pachymetry (≥670 µm), and posterior Q value (≥0.5) had a sensitivity of 58% (11/19 correctly identified hyperopic shifts) and specificity of 81% (8/43 incorrectly identified). CONCLUSIONS: Eyes with a hyperopic surprise after triple DMEK demonstrated a significant change in posterior and anterior corneal curvature compared with eyes without a hyperopic surprise. This might be secondary to corneal edema because these eyes also demonstrated greater preoperative corneal pachymetry.