Abstract
Purpose
To report on the etiologies and prognosis of macular hole (MH) in children and to explore the indicators of spontaneous hole closure and poor final visual outcome (vision worse than 20/200).
Design
Consecutive, retrospective case series.
Methods
A consecutive series of patients aged less than 16 years with a full-thickness macular hole treated from 2013 to 2019 in a singer tertiary center was retrospectively reviewed. Data collected from charts included age, sex, best-corrected visual acuity (BCVA), etiology of MH, size of MH, clinical findings, operations, and anatomic and functional outcomes. Logistic regression models were built to establish the predisposing factors.
Results
Forty eyes of 40 patients were included. Patients were predominantly male with a mean age of 8.3 years. Among the etiologies, trauma prevailed in 29 (72.5%) eyes. Twenty-nine patients underwent surgery, and 18 (62.1%) had traumatic MH. All had achieved hole closure. BCVA improved at the final visit. Spontaneous closure was found in 10 (25%) eyes after an average 2 months after trauma. Regression analysis showed that a relatively smaller macular hole ( P = .006) was likely to experience spontaneous closure. Presence of macular lesions ( P = .001) was identified as risk factor for poor final vision.
Conclusions
Most pediatric MH was caused by blunt trauma. BCVA improved after MH closed, regardless of surgery or spontaneous closure. Smaller MH secondary to trauma was more likely to experience spontaneous closure with an average time of 2 months. Presence of macular lesions was a risk factor for final poor vision.
Highlights
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Trauma is the most prevalent cause of macular holes in children.
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Poor macular lesions is a risk factor for final poor vision.
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Smaller macular hole caused by trauma is more likely to have spontaneous closure.
Macular hole (MH) is commonly idiopathic and age-related. The occurrence of MH in pediatric patients is quite rare. According to previous reports, most pediatric MH cases result from blunt trauma, and cases due to other reasons are rarely reported and are mostly case reports. Currently, the best practice for MH in children has not been ascertained. Spontaneous closure with visual improvement had been reported. Vitrectomy had also been found to effectively close traumatic MH in children and improve vision. , In addition, the prognosis of these defects in children is worse because of delayed diagnosis and complicating conditions.
Despite the surgical intervention being similar to that in adult patients, pediatric MH have their own less well known particularities in terms of etiology, clinical features, and prognosis. In this study, the authors retrospectively analyzed data from a cohort of pediatric patients with MH and described the etiologic profiles, clinical characteristics, surgical management, and anatomic and visual outcomes. Further, logistic regression analysis was conducted to determine the factors influencing the anatomic and functional outcomes.
Methods
Patients and Clinical Examinations
This retrospective study was approved by the Ethics Committee of Xin Hua Hospital affiliated with Shanghai Jiao Tong University School of Medicine and was conducted in accordance with the Declaration of Helsinki. Informed consent was obtained from the parents or legal guardians of all patients.
The data of all patients under 16 years of age diagnosed with full-thickness macular hole (FTMH), of any nature, in the Shanghai Jiao Tong University School of Medicine affiliated with Xin Hua Hospital between January 2013 and April 2019 were retrospectively reviewed.
All patients were evaluated by a panel of retinal specialists and underwent comprehensive age-appropriate ophthalmic examinations. This included best-corrected visual acuity (BCVA), which was measured using a standard Snellen chart and converted to a logarithm of the minimum angle of resolution (logMAR) for the statistical analysis. For visual acuity of counting fingers or worse, the following conversion was used: counting fingers, 20/1,600, 1.9 logMAR; hand motion, 20/4,000, 2.3 logMAR; light perception, 20/8,000, 2.6 logMAR; no light perception, 20/16,000, 2.9 logMAR; intraocular pressure (IOP), via an iCare tonometer (Icare Finland Oy, Vantaa, Finland); slit-lamp examination; dilated fundus examination; wide-field fundus photography using either a RetCam (Clarity Medical Systems, Pleasanton, California, USA) or an Optos 200Tx (Optos, Inc, Marlborough, Massachusetts, USA); and optical coherence tomography (RTVue XR100–2, Optovue Inc, Fremont, California, USA). The OCT scan modes included radial lines (12×9 mm) and horizontal lines (12×9 mm). The diameter of the MH was measured as the narrowest diameter of the 12-radial-line scan pattern centered on the MH.
The inclusion criteria in this study were patients ≤16 years of age with FTMH.
The exclusion criteria included a history of intraocular surgery and follow-up period less than 3 months.
The following data were collected: age, sex, laterality of MH, etiology of MH, size of MH, concomitant findings, time interval between trauma and presentation, time interval between trauma and surgery, time from trauma to spontaneous hole closure, duration of follow-up (minimum 3 months), type and number of operations, initial and final BCVA, anatomic outcome, and complications.
Surgical Procedures
Patients were offered surgery if no morphologic improvement was found (decreased macular hole diameter or resolution of retinal detachment) after 1 month of watchful waiting. All participants (parents or legal guardians) provided written informed consent after being educated about the possible benefits and risks of the procedures. One patient refused surgery.
All surgeries were carried out by a single experienced surgeon (P.Z.).
The common surgical procedure consisted of a standard, 23-gauge 3-port vitrectomy with inner limiting membrane (ILM) peeling under general anesthesia. Induced posterior vitreous detachment was performed, and the peripheral vitreous was then removed as extensively as possible. The peripheral retina was checked circumferentially, and laser photocoagulation was applied if any retinal tears or a lattice were detected. The ILM was removed with the end-gripping forceps under the assistance of brilliant blue G or 0.125% indocyanine green. Additional procedures (described below) were performed based on the surgeons’ preference, guided mainly by the size of the MH and stiffness of the hole margin.
Retinal reattachment was completed using perfluorocarbon liquid in eyes with proliferative vitreoretinopathy or giant tear retinal detachment.
At the end of the operation, a fluid–air exchange was performed, and the primary tamponade (silicone oil or 15% perfluoropropane [C3F8]) was based on the intraoperative findings.
All patients were asked to maintain a face-down position for the subsequent 2 weeks (postoperative position for younger patients is showed in Supplemental Pictures ).
Fresh blood coating
In some cases, fresh blood was obtained from the patient’s antecubital vein and gently injected to cover the macular area. After approximately 30 seconds, extra blood was removed with a soft-tipped flute needle (see Supplemental Video ).
Insertion of the ILM flap into the macular hole
A small piece of the ILM was peeled off to create a free flap. The ILM flap was then trimmed using the vitrectomy probe to the appropriate size (almost the same or slightly larger in diameter as the macular hole to be repaired) and inserted into the MH using microforceps. In some cases, fresh blood was used to cover the free flap of the ILM and macular area (see Supplemental Video ). Then, the remaining ILM in the macular area was removed as far as the vascular arcade and the optic nerve head.
Statistical Analysis
SPSS software, version 22.0 (IBM Corp, Armonk, New York, USA), was used for all statistical analyses. Initial and final BCVA were divided into 2 groups: group 1, BCVA less than 20/200; group 2, BCVA better than 20/200. Categorical data were compared using χ 2 and Fisher exact tests, and continuous variables, using Student t test. Forward stepwise logistic regression analysis was used to analyze the influencing factors of spontaneous closure and the final functional outcome.
To evaluate the indicators of spontaneous closure, traumatic MH was divided into a subgroup and the categorical dependent variable was the presence or absence of spontaneous hole closure. Age, initial BCVA, size of MH, concomitant ocular findings such as presence or absence of retinal detachment or macular lesions, and OCT features such as presence or absence of epiretinal membrane or macular cysts were considered to be the possible predictors of the spontaneous hole closure.
To analyze the prognostic factors of final visual outcome, the nonmissing final visual acuity outcomes (n=37) were divided into 2 categories: poor (BCVA less than 20/200) and good (BCVA better than 20/200) outcomes. Age, etiology of MH, initial BCVA, concomitant ocular findings such as presence or absence of retinal detachment or macular lesions, and OCT features such as presence or absence of epiretinal membrane or macular cysts were considered to be possible predictors of the final functional outcome.
A P value of ≤.05 was considered to indicate statistical significance.
Results
Baseline Characteristics and Associated Ocular Findings
A total of 40 eyes in 40 patients were included in the study. Patients were predominantly male (75%; 30/40), with a mean age of 8.3±4.0 years (range, 5 months-16 years). The mean follow-up period was 9.4±10.0 months (range, 3-48 months). The clinical and demographic characteristics of pediatric MH at presentation are detailed in Table 1 . The etiology of the FTMH was traumatic in 29 (72.5%) patients. Among these, 28 were closed-globe injuries and 1 was an open-globe injury. The traumatic causes of pediatric MH at presentation are detailed in Table 2 . In 5 cases, a definite cause of trauma could not be elicited. The other etiologies ( Figure 1 ) included the following: X-linked retinoschisis (XLRS) in 2 (5%) eyes, high myopia (n=2; 5%), incontinentia pigmenti (n=2; 5%), optic pit (n=1; 2.5%), ocular toxocariasis (n=1; 2.5%), and retinopathy of prematurity (n=1; 2.5%). The other 2 cases (5%) were idiopathic. Representative fundus pictures of MH secondary to different etiologies in pediatric patients are shown in Figure 2 . Based on OCT imaging, the mean diameter of the FTMH was 575.8 μm (range, 109-1,290 μm).
| Factor | Mean ± SD or n (%) |
|---|---|
| Age, y | 8.3±4.0 |
| Gender | |
| Male | 30 (75) |
| Female | 10 (25) |
| Laterality of MH, n (%) | |
| Right | 24 (60) |
| Left | 16 (40) |
| Size of MH, μm | 575.8 ± 296.5 |
| Initial BCVA, logMAR | 1.13 ± 0.43 |
| Final BCVA | 0.83 ± 0.61 |
| Follow-up duration, mo | 9.4 ± 10.0 |
| Cause | No. of Cases |
|---|---|
| Explosion | |
| Firecrackers | 2 |
| Assault | |
| Stick | 1 |
| Plastic | 1 |
| Fist | 1 |
| Whip | 1 |
| Book | 1 |
| Rock | 2 |
| Sports accident | |
| Football | 4 |
| Sandbag | 1 |
| Racket | 1 |
| Tumble/fall | 6 |
| Others | |
| Donkey-kick | 1 |
| Laser pen | 1 |
| BB bullet | 1 |
| Cause not known | 5 |
Associated ocular findings in the traumatic group (n=29) included retinal detachment in 10 eyes (34.5%), including 7 macula-off retinal detachment and 3 macula-on detachment. Among eyes with macula-off retinal detachment, all were associated with MH. Presence of macular lesions (commotio macula) was found in 4 eyes (13.8%), peripheral retinal lattice or tears in 6 eyes (20.7%), dialysis in 2 eyes (6.9%), cataract in 2 eyes (6.9%), vitreous hemorrhage in 2 eyes (6.9%), submacular hemorrhage in 2 eyes (6.9%), and endophthalmitis in 1 eye (3.4%). On OCT scans, intraretinal cysts were noted in 21 eyes (72.4%) and atypical epiretinal membrane was noted in 1 eye (3.4%). In the nontraumatic group (n=11), retinal detachment was noted in 7 eyes (63.6%), including 5 macula-off retinal detachment and 2 macula-on detachment. Among eyes with macula-off retinal detachment, 3 were associated with MH and 2 were tractional retinal detachment. Peripheral retinal break was noted in 1 eye (9.1%), presence of macular lesions (chorioretinal atrophy) in 3 eyes (27.3%). Vitreous veil and granuloma were found in 1 eye (9.1%). On OCT scans, intraretinal cysts was noted in 8 eyes (72.7%).
Three patients did not cooperate with the OCT scan and BCVA measurement because of their young age.
Surgical Intervention and Anatomic Outcome
Twenty-nine eyes received vitreoretinal surgery, and among these, 18 were caused by trauma. The clinical characteristics of pediatric MH that underwent surgical intervention are showed in Table 3 . The mean time from trauma to surgery was 12.5 months (range: 1-36 months, median: 6 months). Silicone oil tamponade was performed in 4 eyes because of complicated retinal detachment. Single-operation closure was accomplished in 22 (75.9%) cases ( Figure 3 ) . Seven (24.1%) patients underwent repeat surgery to achieve hole closure ( Figure 4 ) . Of the 29 eyes that underwent surgery, 23 (79.3%) received single ILM peeling as the initial operation; among these, single-operation closure was achieved in 17 eyes (73.9%). Three eyes (10.3%) received ILM peeling and blood coating as the initial operation; among these, single-operation closure was achieved in 2 eyes. Another 3 eyes (10.3%) received ILM peeling, ILM flap insertion, and blood coating as the initial operation; among these, single-operation closure was achieved in all eyes. Seven patients underwent secondary surgery. Among these, 5 patients (71.4%) received enlarged ILM peeling with blood coating, and 2 patients (28.6%) received enlarged ILM peeling, ILM flap insertion, and blood coating. No intraoperative complications were found. Removal of silicone oil was performed in all 4 cases after the retina was attached and the macular hole sealed. Anatomic closure was achieved in all eyes that underwent surgery (100%) at the last follow-up.
| Patient ID/Age (y)/Gender | Cause | Interval Between Injury and Surgery (d) | Size of MH (μm) | Associated Ocular Findings | Preoperative BCVA (Snellen; logMAR) | Postoperative BCVA (Snellen; logMAR) | Number of Surgeries | Operation |
|---|---|---|---|---|---|---|---|---|
| 1/10/M | Trauma | 3 | 576 | Peripheral retinal lattice | 20/125; 0.8 | 20/100; 0.7 | 1 | ILMP + blood + C3F8 |
| 2/16/M | Trauma | 1 | 690 | RD, dialysis | 20/400; 1.3 | 20/200; 1.0 | 1 | ILMP + C3F8 |
| 5/9/F | Trauma | 1 | 636 | RD | 20/100; 1.0 | 20/63; 0.5 | 1 | ILMP + C3F8 |
| 6/12/M | Trauma | 2 | 453 | Peripheral retinal tears | 20/200; 1.0 | 20/50; 0.4 | 1 | ILMP + C3F8 |
| 9/6/F | Trauma | 36 | 741 | — | 20/125; 0.8 | 20/40; 0.5 | 1 | ILMP + Insertion + blood + C3F8 |
| 11/6/F | Trauma | 5 | 594 | MD | 20/100; 0.7 | 20/63; 0.5 | 2 | 1. ILMP + C3F8 2. EILMP + blood + C3F8 |
| 12/4/M | Trauma | 6 | 612 | — | 20/200; 1.0 | 20/100; 0.7 | 1 | ILMP + C3F8 |
| 13/12/M | Trauma | 36 | 306 | Peripheral retinal lattice | 20/160; 0.9 | 20/165; 0.8 | 1 | ILMP + C3F8 |
| 14/14/M | Trauma | 6 | 271 | Peripheral retinal lattice | 20/100; 0.7 | 20/40; 0.3 | 1 | ILMP + C3F8 |
| 16/10/M | Trauma | 6 | 906 | — | 20/1600; 1.9 | 20/125; 0.8 | 1 | ILMP + C3F8 |
| 18/3/F | Trauma | 24 | 601 | Cataract, endophthalmitis | 20/1000; 1.7 | 20/1600; 1.9 | 1 | ILMP + C3F8 |
| 19/8/M | Trauma | 3 | 910 | Commotio retinae | 20/1600; 1.9 | 20/1600; 1.9 | 1 | ILMP + insertion + blood + C3F8 |
| 20/7/M | Trauma | 12 | 660 | Cataract, RD retinal tears | 20/200; 1.0 | 20/100; 0.7 | 2 | 1. ILMP + SO 2. Lensectomy + SO removal + EILMP + Insertion + blood + C3F8 |
| 23/5/M | Trauma | 6 | 386 | MD, commotio retinae | 20/160; 0.9 | 20/16000; 2.9 | 1 | ILMP + C3F8 |
| 24/5/M | X-linked retinoschisis | — | 989 | RD, peripheral retinal hole | 20/200; 1 | 20/160; 0.9 | 2 | 1. ILMP + blood + C3F8 2. EILMP + blood + C3F8 |
| 25/0.4/F | Retinopathy of prematurity | — | FTMH | RD | N/A | N/A | 1 | ILMP + C3F8 |
| 26/10/F | Idiopathic | — | 553 | — | 20/160; 0.9 | 20/32; 0.2 | 1 | ILMP + C3F8 |
| 27/7/M | Trauma | 36 | 268 | MD | 20/500; 1.4 | 20/200; 1.0 | 2 | 1. ILMP + SO 2. SO removal + EILMP + blood + C3F8 |
| 29/10/M | High myopia | — | 1290 | RD, macular atrophy | 20/4000; 2.3 | 20/1600; 1.9 | 2 | 1. ILMP + SO 2. SO removal + lensectomy + retinotomy + EILMP + blood + SO |
| 30/9/M | Idiopathic | — | 579 | — | 20/200; 1.0 | 20/63; 0.5 | 1 | ILMP + C3F8 |
| 31/0.7/F | Incontinentia pigmenti | — | N/A | RD | N/A | N/A | 1 | ILMP + C3F8 |
| 33/3/F | Incontinentia pigmenti | — | N/A | RD | N/A | N/A | 1 | ILMP + C3F8 |
| 34/4/M | X-linked retinoschisis | — | 459 | — | 20/200; 1 | 20/200; 1 | ILMP + C3F8 | |
| 35/5/F | Ocular toxocariasis | — | 758 | Vitreous veil, granuloma | 20/100; 0.7 | 20/50; 0.4 | 2 | 1. ILMP + C3F8 2. EILMP + Insertion + blood + C3F8 |
| 36/8/M | Optic pit | — | 966 | MD, macular atrophy | 20/160; 0.9 | 20/125; 0.8 | 1 | ILMP + blood + C3F8 |
| 37/13/M | High myopia | — | 919 | MD, macular atrophy | 20/400; 1.3 | 20/1000; 1.7 | 1 | ILMP + SO |
| 38/6/M | Trauma | 12 | 590 | RD, retinal tears, dialysis | 20/200; 1 | 20/63; 0.5 | 2 | 1. ILMP + C3F8 2. EILMP + blood + C3F8 |
| 39/9/M | Trauma | 24 | 650 | — | 20/200; 1.0 | 20/50; 0.4 | 1 | ILMP + C3F8 |
| 40/6/M | Trauma | 6 | 801 | — | 20/200; 1.0 | 20/63; 0.5 | 1 | ILMP + insertion + blood + C3F8 |
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