| PURPOSEEpicanthus and congenital ptosis are two of the most commonly seen ocular diseases in childhood.Children with oversized epicanthus are often mistaken as esotropia(pseudoesotropia)and brought to clinics by their parents for consulting.Congenital ptosis is a well-known risk factor for amblyopia in children.However,in both the public health care system which is responsible for screening,and the pediatric ophthalmology clinic where the treatment is delivered,subjective evaluations are still the main methodology applied.There is a lacking of standard objective method to quantitatively evaluate the visual developmental status in children.Therefore,it is critical to establish a system which emphasizes on the restoring the normal functional development with intervention strategies derived from precise measurement of children's development of craniofacial characteristics and visual function.1.we implemented a series of population-based screenings for congenital eyelid diseases in children located in the city of Tianjin.Based on the screening results,we mapped out the characteristic parameters of children's eyelid and craniofacial development,established the normal developmental trend of children's inner canthus across age,and provided a precise description of the dynamical development inner canthus.We also built a full-scope evaluation system ranging from screening,referral,diagnosis,to intervention pseudoesotropia caused by epicanthus,and established a standard for future clinical works.2.We quantified the prevalence of congenital ptosis in children of Han ethnics in city of Tianjin.3.We investigated the influence of congenital ptosis on visual development by observing its direct or indirect deprivation effect on the visual system.Moreover,we pointed out clearly the best time window for surgical intervention.This could be used as clinical reference for the prevention and management of amblyopia related to ptosis.4.We studied the pathological changes of the eyelid in children with congenital ptosis and explored the histological causes of congenital ptosis.METHODS1.In 2016,we screened 17588 children aged 3 to 17 years of age in Tianjin with the SPOT image camera system.We analyzed the developmental trend of epicanthus in children,including the normal values of interpupillary distance(IPD),inter epicanthal folds distance(IEFD)and Inter epicanthal folds distance/Interpupillary distance ratio(EFDDP).Based on these data,we mapped out the normal developmental chart of the eyelids.Refractive development data collection was perform in 35 patients with congenital epicanthus.2.In 2017,based on the epidemiological survey method and the whole population screening model,28190 children aged 6-13 years old in 43 primary schools in Beichen District of Tianjin were subjected to group screening for developmental eye diseases including ptosis.The suspected cases were referred to the pediatric Ophthalmology Department of Tianjin Medical University Eye Hospital,where comprehensive examinations were performed and the clinical data to establish a database were collected.3.We collected cases with monocular congenital ptosis from pediatric ophthalmology department of Tianjin Medical University Eye Hospital.After dividing the cases into groups with different severity,we analyzed the relationship between severity of ptosis and the development of refractive errors,such as the ratio of Axial globe length and Corneal curvature radius(AL/CR).4.We collected of orbital tissues,including orbital septum,sputum fat,orbicularis oculi muscle,levator palpebrae and frontal muscle tissue,during the surgery,for pathological analysis.The subjects'samples were divided into different groups according to severity of ptosis.The potential association between pathological changes and severity of ptosis were investigated.RESULTS1.The developmental trends of IEFD and IPD are roughly parallel to each other between 3 to 6 years of age and between 13 to 17 years of age.However,there are divergence between 7 to 12 years of age.EFDPD,the ratio of IEFD over IPD,changes mainly in three development stages:before the age of 6,IEFD and IPD develop at a similar and fast rate,resulting in a stable EFDPD ratio;from 7 to 12 years old,although both IEFD and IPD begin to slow down,the EFDDP ratio decreases as IEFD slows down greater;after reaching 13 years old,IEFD and IPD again develop at a similar rate and the EFDPD ratio is flattened again.Preschool children(<6 years old)have a higher prevalence rate of epicanthus than other age groups,and the rate declines trend with age.Children with an EFDPD of more than 0.65 are more easily considered by parents as having strabismus.2.A total of 28190 children(14986 males and 13204 females)from 43 primary schools in the city of Tianjin are screened.51 subjects were diagnosed with ptosis(0.18%),including 32 males(0.21%)and 19 females(0.14%).There was no statistically significant difference between the sexes(?~2=1.626,P=0.129).Among the confirmed children,there are 40 monocular cases(21 right eyes and 19 left eyes;24males and 16 females)and 11 binocular cases(8 males,3 females).There was no statistically significant difference between the eyes(?~2=0.177,P=0.674),and no significant difference in the prevalence among the ages(?~2=10.335,P=0.170)3.For subjects with congenital monocular ptosis,there is no significant difference in axial length between the affected eye and the fellow eye,even in subjects with severe ptosis.Further,there is no statistically significant difference in AL/CR between the affected eye and the fellow eye.However,after binning the subjects according the severity of ptosis,there are statistically significant differences in the incidence of hyperopia and the equivalent refractive error among the groups.In the group with sever ptosis,there is a significant shift toward hyperopia in refractive errors and the AL/CR was significantly smaller than that of the mild group and the normal group.There was a significant correlation between the AL/CR and the severity of the ptosis with the more severe ptosis associated with slower ocular elongation.24.4%of the children develop into anisometropia,which is significantly higher than the rate reported in normal children(1.6%-4.3%).43.9%of children with monocular congenital ptosis developed amblyopia,which is significantly higher than that of normal children(3.0%-3.2%).4.In the groups with congenital ptosis,all aponeurosis specimen showed abnormalities.Out of all cases,12(70.6%)orbicularis muscles showed varying degrees of muscle fiber dysplasia(abnormal fiber appearance,abnormal distribution of intimal distribution,and red staining between fibers).Between group A and B,there was no significant difference in the observed number of the orbicular dysplasia.Masson staining showed red or purple muscle fibers with clear striates,and some increased connective tissue located in between.The red stained material under HE staining showed a light green center and a red periphery under Masson staining.Although fibers with uneven thickness were observed with Desmin staining,those fibers were HE staining negative for Desmin expression.It suggests that these substances may not be striated muscle fibers.Moreover,those substance are revealed as yellow-green understand the sulfur T staining,which indicates more likely to be amyloid substances.CONCLUSIONS1.Preschool children(<6 years old)have a significantly higher rate of epicanthus.Between the ages of 7 and 12,with the development of the face,the condition improves.At 13 years old,EFDPD ratio tends to be stable(EFDPD=0.58)and children with an EFDPD ratio of>0.65 are often considered by the parents to be"cross-eyed"and be brought to the clinic.Congenital epicanthus has no effect on children's refractive development.2.This study reports a prevalence of congenital ptosis is 0.18%,which is not associated with age,gender,or eye disease factors.The incidence of astigmatism in children with ptosis was 2.87 times that of the normal population;The incidence of anisometropia was 5.24 times that of the normal population;The incidence of myopia and hyperopia was not different from that of the normal population.3.In congenital ptosis,due to the occlusion of the visual axis and compression of the cornea,there are further changes in corneal morphology,curvature,and refractive power.The consequent blurred vision leads to lesser use of the affected eye,retards the normal development,and increases the chance of anisometropia and amblyopia.4.This study finds that in children with congenital ptosis,besides dysplasia of the levator,there is also dysplastic histopathology in orbicularis oculi and amyloid in the muscle fiber interstitial space. |
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