Suprachoroidal hemorrhage (SCH), resulting from the rupture of the long or short ciliary arteries with blood accumulation in the suprachoroidal space, is a rare but vision-threatening complication of intraocular surgery [
1,
2]. The incidence of SCH ranges from 0.5% to 1.08% in patients undergoing penetrating keratoplasty (PK) [
3-
5] and from 0.7% to 8.3% following glaucoma drainage device implantation [
6-
8].
Most SCH cases associated with PK occur during surgery [
3-
5], whereas SCH related to glaucoma surgery develop postoperatively in most cases [
8]. These findings suggest that acute hypotony or large fluctuations in intraocular pressure (IOP) contribute to SCH development. Studies in adults have identified advancing age and arteriosclerotic disease as major risk factors for SCH associated with PK. However, these factors are not relevant in children. Moreover, reports of pediatric SCH, particularly after PK, are scarce.
This study aims to describe the ocular findings, clinical presentation, management, and outcomes of SCH in pediatric patients who underwent PK or Ahmed glaucoma valve (AGV) implantation.
Materials and Methods
Ethics statement
According to our institutional guidelines, the institutional review board approval was not required for retrospective case reports involving fewer than five cases. To comply with the journal’s requirements, the informed consent was obtained from the parent of each pediatric patient for the use of their clinical data and images for research and educational purposes. The study was conducted in accordance with the principles of the Declaration of Helsinki.
Study design
The medical records of consecutive patients who underwent PK or AGV implantation at our hospital from 2012 to 2023 were reviewed. In total, 58 pediatric patients (aged 3 months to 13 years) underwent PK and 89 (aged 8 months to 15 years) underwent AGV implantation. Among these, three perioperative SCH cases were identified: two associated with PK and one with AGV implantation. The clinical characteristics, anterior segment photographs, ultrasonographic images, treatments, and the anatomical and visual outcomes of these three patients were analyzed.
Results
The incidence of SCH associated with pediatric PK at our institution was 3.45 % (2 of 58 cases), and that related to AGV implantation was 1.12% (1 of 89 cases).
Case 1
A 6-year-old girl with Peters anomaly, CG, and partial aniridia in both eyes (
Fig. 1A, 1B) was scheduled for PK in her left eye. She had previously undergone two PK procedures in the left eye, 65 and 36 months earlier, and one in the right eye, 60 months earlier, both of which resulted in graft rejection (
Fig. 1C). During the initial PK procedures, the lenses in both eyes spontaneously extruded, leading to aphakia. For IOP control, she was maintained on topical brinzolamide/timolol (Elazop, Novartis), latanoprost (Monoprost, Samil), and apraclonidine (Iopidine, Novartis). Since her visual acuity (VA) was better in the left eye before graft rejection, a third PK was planned for that eye.
Preoperatively, VA was hand motion in both eyes. IOP measured under general anesthesia with a rebound tonometer was 31.1 mmHg in the right eye and 31.5 mmHg in the left, despite maximal IOP-lowering medications including intravenous mannitol and topical antiglaucoma medications.
During surgery, the host cornea was trephined, and the donor cornea secured with four cardinal 10-0 nylon sutures. Abruptly, the retinal reflex turned bright red, and the globe became firm, indicating an increase in IOP. Vitreous hemorrhage (VH) was observed through the donor cornea, but the anterior chamber remained deep with no prolapse of vitreous or hemorrhage. After placing 28 additional sutures to complete the procedure, intraoperative B-scan ultrasonography was performed, revealing a rapid progression of SCH and VH originating from the nasal side in real-time (
Fig. 1D).
Postoperatively, the patient was closely monitored for SCH and IOP with conservative management including topical and systemic steroids, topical atropine, and dorzolamide/timolol (Cosopt, Santen). One week after surgery, SCH and VH started to gradually resolve, leading to near-complete resolution by 7 months (
Fig. 1E, 1F). The corneal graft remained clear for 1 month but became fully vascularized and opacified by 7 months (
Fig. 1G, 1H). Three years later, ultrasonography showed normal architecture of the posterior segment; however, the patient lost light perception.
Case 2
A 1-year-old girl with Peters anomaly, CG, and total aniridia in both eyes (
Fig. 2A, 2B) was scheduled to undergo PK in her left eye (
Fig. 2C). She had undergone PK in her right eye 6 months earlier and was maintained on topical dorzolamide/timolol (Cosopt) for IOP control.
Preoperative examination showed that she could fix-and-follow a moving object at a distance of 50 cm with her right eye, while the left eye had only light perception. Preoperative intravenous mannitol was administered, along with topical antiglaucoma medications IOP measured at the operative bed under general anesthesia was 26.8 to 27.5 mmHg in the right eye and 24.4 to 24.8 mmHg in the left eye.
Immediately after trephination of the host cornea, the lens was spontaneously expelled, resulting in aphakia. The PK was completed successfully, including water-tight suturing of the donor cornea with 32 stitches made with 10-0 nylon, without further complications.
On the first postoperative day, the graft was clear, the anterior chamber was deep and quiet, and IOP was 16.5 mmHg. Ultrasonography revealed a dome-shaped choroidal detachment with underlying hemorrhage (
Fig. 2D). Two days after surgery, the SCH expanded into dense hemorrhage within the vitreous cavity and anterior chamber (
Fig. 2E, 2F). The IOP remained between 10 and 11 mmHg, and the patient did not report any pain. Starting from 2 weeks after surgery, both SCH and VH gradually subsided; however, retrocorneal fibrovascular proliferation rapidly developed (
Fig. 2G, 2H), leading to phthisis by 2 months. Nine years later, her right eye maintained fix-and-follow vision with a prosthetic in the left eye.
Case 3
A 9-year-old boy with Peters anomaly, CG, and partial aniridia in both eyes (
Fig. 3A-3D) underwent an AGV implantation in his right eye. He had previously undergone PK in the right and left eyes at 4 and 3 months of age, respectively, and cataract surgery in the right and left eyes at 13 and 14 months, respectively. Seven years after surgery, graft rejection and retinal detachment occurred in the left eye, while the right graft remained clear. Despite receiving a Xen implant (Allergan Inc) at age 7 years, his IOP in the right eye remained uncontrolled, reaching up to 40 mmHg.
Preoperative examination prior to AGV implantation showed that his VA was 0.02 in the right eye and light perception in the left eye, with IOP measurements of 32.4 to 36.0 mmHg in the right eye and 12.7 to 24.0 mmHg in the left. The surgery proceeded without specific incidents.
Two days after AGV implantation, the patient presented to the emergency room with severe ocular pain and sudden vision loss in the right eye. VA decreased to light perception, and IOP ranged from 9.3 to 10.7 mmHg. The corneal graft was clear, and the anterior chamber was deep and clear (
Fig. 3E). Ultrasonography revealed “kissing choroidals,” indicative of SCH with retinal apposition (
Fig. 3F). Starting 2 weeks after surgery, the SCH and choroidal detachment resolved, with complete regression observed by 3 months (
Fig. 3G, 3H). His VA in the right eye improved to 0.04 with a −8.00 diopters sphere correction. Three years later, best-corrected VA was 0.06 in the right eye, and IOP was maintained between 23.2 and 27.8 mmHg on topical medications including dorzolamide/timolol (Cosopt), latanoprost (Monoprost), and apraclonidine (Iopidine).
Discussion
In this study, we report three SCH cases in pediatric patients who underwent PK or AGV implantation: one intraoperative nonexpulsive SCH during PK, one delayed postoperative SCH following PK, and one postoperative SCH after AGV implantation. Between 2012 and 2023, at our institution, 58 pediatric patients (aged 3 months to 13 years) underwent PK, and 89 patients (aged 8 months to 15 years) underwent AGV implantation. Thus, the incidence of SCH was 3.45% (2 of 58 cases) in pediatric PK patients and 1.12% (1 of 89 cases) following AGV implantation. By comparison, the reported incidence of SCH in adults undergoing PK ranges from 0.5% to 1.08% [
3,
4], suggesting that SCH associated with PK may be more common in children. Conversely, previous reports indicate that the incidence of SCH associated with AGV implantation in the pediatric population ranges from 3.2% to 4.75% [
9-
11], which is higher than the incidence observed in our cohort.
Previous studies have identified glaucoma (or elevated preoperative IOP), myopia, aphakia, increased axial length, previous intraocular surgery such as cataract surgery and vitrectomy, and early postoperative hypotony as ocular risk factors for perioperative SCH [
3-
5,
7-
9,
12]. In our case series, all three patients had bilateral Peters anomaly, CG, and aniridia (two with partial aniridia and one with total aniridia). Two eyes were aphakic (with an intact posterior capsule) and one was pseudophakic at the time of SCH. Despite preoperative use of maximal IOP-lowering medications, IOP measurements remained high at the time of surgery. Therefore, large fluctuations in IOP in hypertensive eyes, combined with poor scleral rigidity and weak zonular support, may have contributed to ciliochoroidal vascular fragility and precipitated SCH in our patients.
Therapies for SCH include conservative management with systemic and topical steroids, topical atropine, and IOP control, as well as surgical drainage via sclerotomy. The primary goals of surgical drainage are retinal reattachment and IOP control. The timing of drainage remains controversial. Clot lysis occurs within 1 to 2 weeks and facilitates surgical drainage. However, because of the risk of rebleeding, some studies support delaying drainage beyond 1 week [
7,
9]. Conversely, other reports advocate earlier surgical intervention, especially in cases of delayed SCH [
13-
15]. In our series, all three cases showed resolution of SCH, starting between 1 and 2 weeks, with conservative management. Despite anatomical recovery, visual outcomes were poor in the PK-related cases, resulting in loss of light perception, whereas the AGV-related case fully regained vision. These findings suggest that SCH following glaucoma surgery may have a more favorable visual outcome compared to SCH associated with PK.
Our study has limitations. The small number of cases prevented a comprehensive risk-factor analysis across patients with diverse ocular and systemic characteristics. Therefore, our findings regarding incidence, ocular characteristics, and outcomes of SCH should be interpreted with caution. Future research involving larger cohorts is necessary to identify risk factors for SCH in pediatric patients and to establish optimal management strategies for this challenging complication in children with congenital ocular anomalies.
In summary, we report three pediatric cases of SCH that occurred during or shortly after PK or AGV implantation. All three patients had Peters anomaly, CG, aniridia, and aphakia (or pseudophakia). SCH resolved with conservative management in all cases; however, the visual outcomes were poor, especially in the PK-related cases. Although the number of cases is small, our findings demonstrate the importance of recognizing the risks of both intraoperative and postoperative SCH and exercising caution in pediatric patients with complex anterior segment dysgenesis undergoing PK or glaucoma drainage device implantation.