Volume 56 · Issue 03 · 2020

This issue focuses on the research progress of strabismus, amblyopia and pediatric ophthalmology. The responsible editorial committee responsible for the planning, composition and review of the key contents of this issue is Professor Zhang Wei.

On the occasion of the 70^th anniversary of the founding of Chinese Journal of Ophthalmology, we review the establishment and development of Chinese Association for Pediatric Ophthalmology and Strabismus (CAPOS) and the major advances in the diagnosis and treatment of pediatric eye diseases in China since 1949, so as to remember our predecessors and inspire young generations, and to provide important reference for the future clinical and scientific research.(Chin J Ophthalmol, 2020, 56:161-165)

There are many pathogenic factors of strabismus. In addition to the changes of alignment and ocular movement, there may be abnormal pathways and development of extraocular muscles, abnormalities of orbital and intraorbital connective tissue, paralysis of cranial nerves (oculomotor nerve, trochlear nerve, and abductor nerve), and nuclear and supranuclear lesions, accompanied by other cerebral dysplasia sometimes. In recent years, advances in imaging technology and its application in the professional field of strabismus have made it possible to clearly observe the eye, orbital, intracranial, and innervation changes, which is helpful to clarify the etiology of strabismus and extraocular muscle-related diseases, and to provide a basis for the diagnosis and treatment of strabismus. Strabismus specialists should learn and pay attention to the application of imaging examination, so as to achieve in-depth understanding and accurate treatment. (Chin J Ophthalmol, 2020, 56: 166-170)

Horizontal strabismus, the most common type of strabismus, can be complicated by the disorder of inferior oblique muscle or superior oblique muscle. It may cause problems such as compensatory head posture, vertical diplopia, torsional diplopia, and abnormal binocular fusion. In horizontal strabismus cases, the disorder of oblique muscles should be recognized, and appropriate surgical methods should be chosen, so that the best postoperative ortho and binocular vision could be obtained. (Chin J Ophthalmol, 2020, 56: 171-175)

The Preferred Practice Pattern (PPP) issued by the American Academy of Ophthalmology is currently internationally recognized as the authoritative diagnosis and treatment guidelines for ophthalmology. In September 2017, the 2017 edition of the PPP for esotropia and exotropia was officially released. This version of the PPP uses the latest quality of evidence and strength of recommendations grading, and the 2012 version of the PPP is updated based on the most reliable clinical trial results or the collective judgment and evaluation of the existing basis by the expert committee. This article interprets the 2017 version of PPP for esotropia and exotropia from the aspects of definition, epidemiological investigation, diagnosis and treatment of strabismus, and compares it with the 2012 version of PPP, hoping to help ophthalmologists deeply understand the updated key points of the 2017 version of PPP and further promote the standardization of strabismus diagnosis and treatment in China. (Chinese Journal of Ophthalmology, 2020, 56:176-182)

To evaluate the effect of extraocular muscle surgery in treating secondary esotropia with diplopia after orbital decompression for thyroid-associated ophthalmopathy (TAO).

Retrospective case series study. Eleven secondary esotropic patients with diplopia after orbital decompression for TAO who underwent extraocular muscle surgery during March 2016 and October 2018 in Tianjin Eye Hospital were included. All patients had new onset esotropia after decompression surgery and underwent strabismus surgery. Deviation angle, diplopia, and ocular movement were observed preoperatively and postoperatively. Surgeries were carried out under the monitored anesthesia care combined with local anesthesia, and the eye alignment was adjusted to orthotropia with no diplopia in the operation with the technique of intraoperative adjustable suture and forced duction test results. All the patients were followed up.

The ages of the patients were from 26 to 42 years (1 male and 10 females). The deviation angles were 10-98 prism diopter. Orbital CT scan showed that the horizontal rectus thickness was increased at different levels, and the thickness of the medial rectus was increased more than that of the lateral rectus. Two patients had monocular medial rectus recession only, 2 had bilateral medial rectus recession only, 2 had monocular medial rectus recession combined with lateral rectus resection, and the other 5 had bilateral medial rectus recession with monocular lateral rectus resection. The recession of medial rectus muscle was 3.5 to 7.5 mm in 11 patients and 2.0 to 6.0 mm in 7 patients with the resection of lateral rectus muscle. Diplopia of the 11 patients disappeared with orthotropia at primary gaze position after the extraocular muscle surgery. The limitation of abduction changed from preoperative (-1.91±1.04) to postoperative (-0.64±0.81). All the patients were satisfied with the surgical results. At the end of the follow-up (6 to 24 months), the results of the patients were stable, and no over-correction was found.

Extraocular muscle surgery with intraoperative adjustable suture technique can effectively treat TAO patients with secondary esotropia with diplopia after orbital decompression. (Chin J Ophthalmol, 2020, 56: 183-188)

To assess the accuracy of photoscreening for detecting refractive amblyopia risk factors (ARFs) in Chinese preschool children aged 4 to 5 years.

A cross-sectional study. Comprehensive ocular examinations were conducted for preschool children in Nanjing, China from September to December, 2016. Photoscreening (Plusoptix A12C) was applied for refractive screening without cycloplegia. Voluntary children and children suspected of eyes abnormalities received cycloplegic retinoscopy (CR). Results of photoscreening and CR were compared using Wilcoxon signed rank test, and Bland-Altman plot were used to assess the agreement between the photoscreener and CR. According to the updated preschool vision screening guidelines from American Association for Pediatric Ophthalmology and Strabismus (AAPOS) in 2013, CR was adopted for identifying children with ARFs, which was considered as a golden standard. Based on the golden standard, the accuracy of 5 sets of referral criteria (including sensitivity standard, Matta/Silbert standard, AAPOS2013 standard, Alaska Blind Child Discovery standard, specificity standard) for photoscreener were tested. Receiver operating characteristics curves were constructed applied to evaluate the quality of the photoscreener in refractive ARFs detection and to find probably the best cut-off points.

In total, 1 986 children [mean age, (4.57±0.29) years] received comprehensive examinations, including 1 084 boys and 902 girls. The test ability of photoscreening was 99.04% (1 967/1 986) in the preschool children, and 96.56%(1 827/1 892) of the children got a reliable result within three screening attempts. In 538 children who had data of CR, refractive error of one child exceeded the upper limit of the photoscreener value setting, which was directly categorized as hyperopia, so in the end, 537 children were included to analyze the comparison between the two tests. The measurement values of photoscreening were lower than those of CR in sphere, cylinder and spherical equivalent [(0.75 (0.50, 1.25) D vs. 1.25 (1.00, 1.75) D, Z=-10.36, P<0.01; -0.50 (-0.75, -0.25) D vs. -0.25 (-0.75, 0.00) D, Z=-11.10, P<0.01; 0.63 (0.38, 0.88) D vs. 1.00 (0.75, 1.50) D, Z=-13.33, P<0.01]. The 95% limit of agreement cover rates between the photoscreening and CR in sphere, cylinder and spherical equivalent was 96.28% (517/537), 95.34% (512/537) and 96.65% (519/537), respectively. Based on the golden standard, 47 (8.74%) children had refractive ARFs, and the range of sensitivity, specificity, Youden index, positive predictive values and negative predictive values for detecting refractive ARFs of the 5 common used referral criteria was from 63.83% to 97.87%, from 53.36% to 97.56%, from 0.51 to 0.80, from 16.73% to 74.51% and from 96.57% to 99.62%, respectively. Considering particular refractive ARFs on the basis of the receiver operating characteristic curves, the optimal cut-off point for astigmatism was set at 1.38 D.

Photoscreening could be an applicable tool to detect refractive ARFs in preschool children. (Chin J Ophthalmol, 2020, 56: 189-196)

To observe the number of blood vessels and the anatomical characteristics of the anterior ciliary vessels (ACVs) in the horizontal rectus muscles of patients with concomitant horizontal strabismus.

Cross-sectional study. From July 2016 to September 2019 patients with concomitant horizontal strabismus treated by realignment surgeries in Xiamen Eye Center of Xiamen University were included. Patients who had previous operations on eyes were excluded. The high resolution surgical imaging of ACVs in the horizontal rectus muscles was performed during operation. The photos of ACVs in muscles were tagged and observed to compare the distribution of the various numbers of blood vessels and morphological characteristics of the ACVs in the medial and lateral rectus muscles. In addition, muscle tissues about 4 mm with an intact sheath were gained from patients undergoing the muscle resection. The muscle sections were stained with hematoxylin-eosin staining, and the arteriovenous properties and their distribution in muscles and fascia tissues were observed. The chi-square test was used for statistical analysis.

A total of 387 patients with concomitant horizontal strabismus (176 with esotropia, 211 with exotropia) were enrolled in this study, among whom 198 were male and 189 were female. The age ranged from 1 to 68 years (median, 13 years). Photos of ACVs in 742 horizontal rectus muscles from 383 patients were observed. Of the 351 medial rectus muscles, 24 (6.8%) contained 1 major (or bundle of) ACV, 163 (46.4%) contained 2 major (or bundles of) ACVs, and 164 (46.7%) contained 3 or more major (or bundles of) ACVs. Of the 391 lateral rectus muscles, 161 (41.2%) contained 1 major (or bundle of) ACV, 156 (39.9%) contained 2 major (or bundles of) ACVs, and 74 (18.9%) contained 3 or more major (or bundles of) ACVs. The distribution of ACVs in the medial and lateral rectus muscles was statistically different (χ²=133.87, P<0.01). According to the number and morphological characteristics of vessels, the ACVs in the medial and lateral rectus muscles were divided into 4 and 3 categories, respectively, as well as various types and subtypes. The rate of patients whose both eyes had the same type of ACVs in the medial or lateral rectus muscles was 31.6% (25/79) and 26.1% (29/111), respectively, and there was no significant difference (χ²=0.69, P=0.41). However, the ACVs of the same type from both eyes of the same patient were not identical in morphology. The ACVs in the medial and lateral rectus muscles with collateral circulation accounted for 3.7% (13/351) and 24.3% (95/391), respectively, and the difference was statistically significant (χ²=63.07, P<0.01). The hematoxylin-eosin staining of the medial lateral rectus tissues from 4 patients showed that the ACVs contained arteries and veins. The vessels in the medial rectus muscles were distributed in muscle tissue and fascia tissue, while the vessels in the lateral rectus muscles were only distributed in fascia tissue.

Compared with the lateral rectus muscles, there are about 50% medial rectus muscles contain more than 3 major (or bundles of) vessels in patients with concomitant horizontal strabismus. The accompanying patterns of arteries and veins of ACVs are varied. ACVs are distributed in muscle tissue of the medial rectus muscles and in fascia tissue of the medial and lateral rectus muscles. (Chin J Ophthalmol, 2020, 56: 197-204)

To analyze the clinical histopathologic characteristics of lacrimal glands and possible mechanisms of lacrimal gland prolapse in blepharochalasis (BC).

A case-controlled study of 23 consecutive patients with prolapse of lacrimal glands in BC was performed. All samples were obtained during surgery from the Department of Ophthalmology, Beijing Tongren Hospital, Capital Medical University between January 2009 and December 2016. The lacrimal tissue included prolapsed lacrimal glands (30 samples) and controls from the donors in the eye bank of Beijing Tongren Hospital, Capital Medical University (8 samples). Hematoxylin-eosin staining, special staining, immunohistochemistry and colloidal gold-labeled pre-embedded indirect immunogold electron microscopy (Gold-IIEM) were performed to analyze the histopathologic characteristics of the samples. The nonparametric Wilcoxon signed-ranks test was carried out for statistical analysis.

Among the 23 patients with lacrimal gland prolapse in BC, there were 3 males and 20 females. The mean age of morbidity was 11 years old (7-16 years). In the 8 normal control cases, 3 males and 5 females were included. The mean age was 15 years (10-20 years). In the 30 prolapsed lacrimal gland samples, hematoxylin-eosin staining showed enlargement of glandular lumina accompanied by inflammatory infiltrates of interstitial tissue in 2 samples. Marked loosening of collagen fibers of the obtainable lacrimal fascia was observed. The results on immunohistochemical staining demonstrated an increased level of immunocytes in the 30 prolapsed lacrimal gland samples, including IgA (+++, ++, +, -; 12, 11, 4, 3 vs. 0, 0, 1, 7; Z=-3.892), CD3⁺T cells (+++, ++, +, -; 2, 19, 7, 2 vs. 0, 0, 1, 7; Z=-4.168), matrix metalloproteinase (MMP)-3 (+++, ++, +, -; 0, 0, 11, 19 vs. 0, 0, 0, 8; Z=-2.005) and MMP-9 (+++, ++, +, -; 14, 14, 0, 2 vs. 0, 0, 0, 8; Z=-4.552) (all P<0.05). IgG, IgM, CD20 and C1-inhibitor were either absent or expressed at background level in the 30 prolapsed lacrimal gland samples (all P>0.05). Gold-IIEM showed zymogon granules in lacrimal glands were out of shape. MMP-3 and MMP-9 colloidal gold particles existed on the zymogon granules, and MMP-3 colloidal gold particles also existed on the surface of lacrimal gland epithelial cells.

The histopathological changes in the lacrimal glands of lacrimal gland prolapse with BC include inflammatory infiltration, elastic fiber degeneration, marked loosening of the supporting fascia tissue, and an increased level of immunocytes, including IgA, CD3⁺T cells, MMP-3 and MMP-9. The results suggest that lacrimal gland prolapse with BC may result in the immuno-pathogenetic mechanisms with the involvement of cell-mediated immune responses. (Chin J Ophthalmol, 2020, 56: 205-210)

To investigate the autofluorescence findings of retinal astrocytic hamartoma (RAH) in patients with tuberous sclerosis complex (TSC).

It was a retrospective case series study. Twenty-three patients (35 eyes) who were referred to Department of Internal Medicine and Department of Ophthalmology, Peking Union Medical College Hospital between November 2012 and June 2018 with established TSC-associated RAH diagnosis were included. The findings of fundus autofluorescence, fundus photos and spectral-domain optical coherence tomography (SD-OCT) were retrospectively reviewed. RAH lesions were classified into three types based on the morphology shown in fundus photos. The fundus autofluorescence features of TSC-associated RAH were described. The Welch's test and Fisher's exact test were used for statistical analysis.

The patients were 8 males and 15 females aged (28±9) years old (range, 15-55 years). Seventy-two RAH lesions were examined, including 59 type 1 RAHs, 7 type 2 RAHs and 6 type 3 RAHs. According to fundus autofluorescence, type 1 RAHs could be further divided into reduced, speckled and background autofluorescence patterns, among which the hypoautofluorescence pattern accounted for the majority (69.5%, 41/59), while the speckled pattern was usually accompanied by outer retinal disorganization and discontinuation of photoreceptor outer segment as revealed by SD-OCT. No significant difference was revealed in tumor thickness for reduced, speckled and background autofluorescence patterns of type 1 RAHs [(490.2±97.9) vs. (589.2±221.6) vs. (463.0±76.2) μm respectively, F=1.426, P=0.283]. Among type 1 RAHs, the number of reduced autofluorescence pattern lesions found in perifoveal, peripapillary, inferonasal, inferotemporal, superonasal, superotemporal quadrants were 9, 4, 4, 7, 4, 13 respectively, while that of speckled autofluorescence pattern lesions were 3, 0, 3, 2, 3, 2 and background autoflurorescence pattern lesions 3, 0, 1, 1, 0, 0. No significant difference was revealed in location distribution (P=0.452) either. Type 2 RAHs featured numerous hyperautofluorescent spots or plaques, and calcification in type 2 RAHs varied in autofluorescence intensity. Type 3 RAHs, combining the features of type 1 and 2 RAHs, were characterized by central hyperautofluorescent spots and hypoautoflurescent rim, but the area of hyperautofluorescence was smaller than that of calcification as shown in fundus photos.

In TSC, the fundus autofluorescence of RAHs varies from hypoautofluorescence to hyperautofluorescence patterns according to RAH types. The retinal involvement and calcification degree of TSC-associated RAHs could be reflected on the autofluorescence, which was beneficial to the full assessment. (Chin J Ophthalmol, 2020, 56: 211-216)

To characterize the clinical features of patients with Behcet′s uveitis.

Retrospective case series study. Medical records of patients who were diagnosed as Behcet′s uveitis in Peking Union Medical College Hospital between January 1998 and November 2015 were retrospectively reviewed. Information about the clinical manifestation, complication, best corrected visual acuity (logarithm of the minimum angle of resolution) and medication was collected. The correlation of visual acuity and the disease course, as well as inflammation relapse times before and after biological agents applied was analyzed. Chi square test and Wilcoxon signed-rank test were applied for statistical analysis.

A total of 132 patients (233 eyes) with Behcet′s uveitis were included, among which 104 (78.8%) were male and 28 (21.2%) were female, with statistically significant difference (χ²=43.76, P<0.01). The average age at the onset of uveitis was (28.9±8.7) years, with most patients at 21-40 years (102 cases, 77.3%). Oral aphthae (132 cases, 100.0%) were the most frequent extraocular manifestations, followed by dermatologic lesions (103 cases, 78.0%), genital ulcers (90 cases, 68.2%), arthritis (44 cases, 33.3%) and positive pathergy test (39 cases, 29.5%). Ocular involvement was bilateral in 76.5% (101 cases) of the patients, and panuveitis was the most common type (110 cases, 83.3%). Complications were seen in 80.7% (188/233) of the eyes, with cataract (58.8%, 137/233) and macular edema (59.1%, 114/193) being the most common anterior and posterior segment complications, respectively. At the eighth year of uveitis, best corrected visual acuity decreased to less than 0.3 in 20.9% (9/43) of the eyes, and more than 1.0 in 39.5% (17/43) of the eyes. A combination of glucocorticoids and immune modulators remained the first line therapy (97.0%, 128/132) for Behcet′s uveitis. For 16 refractory cases, an addition of biological agents significantly reduced the inflammation relapse [0.5 (0.0, 1.5) times per year vs. 4.0 (1.0, 5.5) times per year, Z=-2.81, P=0.005].

Behcet′s uveitis mainly affects working-age males and manifests as bilateral panuveitis. Complications are commonly seen in uveitic eyes. Patients′ visual acuity is significantly impaired. A combination of glucocorticoids and immune modulators is effective for most patients, but for refractory cases, an addition of biological agents provides better effects. (Chin J Ophthalmol, 2020, 56: 217-223)

A patient with a complaint of progressive diplopia for 8 years and tilted head for half a year went to the ophthalmology department. Physical examination showed that the patient's right eye could not turn internally past the midline. High-resolution magnetic resonance imaging of the orbit showed local nodular thickening of the inferior trunk of the oculomotor nerve in the right eye, thickening of the medial rectus muscle branch, and thinning of the abdomen of the medial rectus muscle in the right eye. Combined with clinical and imaging features, the final diagnosis was oculomotor nerve branch mass in right orbit; Paralysis of branches of the oculomotor nerve in the right eye; Right medial rectus palsy; Paralytic exotropia of the right eye. Two months later, binocular strabismus correction surgery was performed. Intraoperative passive distraction test showed that the lateral rectus muscle of the right eye had no resistance. After operation, the patient's right eye position was slightly over-corrected, the right eye was basically in place, and there was no obvious diplopia in all directions. (Chinese Journal of Ophthalmology,2020，56：224-226）

One patient who complained about the discovery of a gray-brown mass in the medial canthus of the left eye for half a year went to the ophthalmology department. Histopathological examination after resection of the mass of the left eyelid showed that the wall of the mass was composed of keratinized lamellar squamous epithelium, and the inside was composed of hair epithelial cells with rich cytoplasm and light staining, and multiple angular cysts were formed inside. There was no recurrence after 2 years of follow-up. (Chinese Journal of Ophthalmology,2020，56：227-228）

Surgeon IntroductionZhang Wei, MD, chief physician, professor, doctoral supervisor, director of strabismus and pediatric ophthalmology department of Tianjin Eye Hospital. Head of Strabismus and Pediatric Ophthalmology Group of Ophthalmology Branch of Chinese Medical Association, chairman of Strabismus and Pediatric Ophthalmology Committee of Ophthalmologists Branch of Chinese Medical Doctors Association, editorial board member of Chinese Journal of Ophthalmology and other academic journals, and "131" innovative talents in Tianjin.

The patient was a 31-year-old female. Because of blurred vision in both eyes for 10 years, he went to the Department of Ophthalmology, Affiliated Hospital of Jining Medical College. No previous history of systemic disease and ocular disease. No abnormalities were found on the whole body examination. Eye examination: visual acuity was 0.5 for the right eye (corrected visual acuity was 1.0) and 0.3 for the left eye (corrected visual acuity was 0.6); IOP was 16 mmHg (1 mmHg =0.133 kPa) in the right eye and 15 mmHg in the left eye; The eyeball movement in all directions is normal, and the eye position is in the right position; There is no conjunctiva congestion and edema in both eyes, the cornea is transparent, the pupil diameter is about 2.5 mm, and it is sensitive to light; The right eye has clear iris texture, no anterior and posterior adhesion, normal pupil area, left eye pupil residual membrane, about 16 pigment cords connected to the iris curl wheel and the central area of the anterior lens capsule (Quintessence Picture 1), the lens is transparent; Fundus examination showed no abnormalities. Clinical diagnosis: refractive error in both eyes; Residual membrane of the pupil of the left eye.

Intermittent exotropia is a common form of childhood strabismus, which is mainly treated by surgery. However, there are still controversies about the natural course of the disease, the assessment of the disease severity, the choice of the surgery time, the determination of the target eye position and the choice of the treatment method. This article reviews the clinical research progress of intermittent exotropia, aiming to provide the basis for the early detection, scientific diagnosis and standard treatment of intermittent exotropia. (Chin J Ophthalmol, 2020, 56: 231-234)

The diagnosis and treatment of rotational strabismus is always one of the clinical problems that is complicated in strabismus specialty and difficult for ophthalmologists, even those who specialize in strabismus. The clinical manifestation of rotational strabismus is different from that of horizontal or vertical strabismus, and the examination method is relatively special, so it is easy to miss or misdiagnose in clinical work. This article starts with vigilance against rotary strabismus caused by central nervous system lesions, and emphasizes on the classification, etiology, clinical manifestations, diagnosis and differential diagnosis, and treatment methods of rotary strabismus, in order to provide reference for ophthalmologists to clinically understand and deal with rotary strabismus. (Chinese Journal of Ophthalmology,2020，56：235-240）