INTRODUCTION

Neurocutaneous syndromes comprise a number of con-
ditions characterised by specific skin manifestations with occasional ocular manifestations [1]. Sturge-Weber syn-
drome (SWS), a prominent mucocutaneous syndrome,
is characterised by facial cutaneous haemangioma, leptomeningeal angioma, and choroidal haemangioma [1]. Glaucoma occurs in 30–70% of cases of SWS [1], and reported mechanisms include goniodysgenesis [2] (childhood onset glaucoma) and elevated episcleral venous pressure (EVP) [2] (late onset glaucoma). Intraocular pressure (IOP)-lowering medications include systemic carbonic anhydrase inhibitors (CAIs) [2], topical β-blockers [2], and, more recently, topical Rho kinase inhibitors [3]. Reported surgical treatment options include angle surgery [4], filtering surgery [4], and combined angle and filtering surgery [5], all of which carry the risk of complications, most notably intraoperative or early postoperative suprachoroidal haemorrhage and/or effusion, from an existing choroidal haemangioma [1, 5]. However, late suprachoroidal effusion is an uncommon occurrence, to the best of the authors’ knowledge. This is a report of a case of delayed suprachoroidal effusion with shallow anterior chamber (AC) in an operated eye with SWS-associated glaucoma in
an 11-year-old child.

Publication of the case report was approved by the Ethics Committee of Alexandria University Faculty of Medicine
(IRB serial no. 0307713, date: 04/01/2026). Consent for publication of the case was verbally obtained from the care provider
of the child after thorough explanation of the condition.
The data are anonymised, and no individual can be identified from the submitted photos.

CASE PRESENTATION

An 11-year-old girl presented to the ophthalmology service of Alexandria Main University Hospital with SWS.
The child complained of frequent headache, and systemic evaluation revealed a facial port-wine stain involving the ophthalmic division of the right trigeminal nerve (for which she had received surface laser treatment 4 months earlier) with ipsilateral slight facial hypertrophy. Neuroimaging was free. Ophthalmic (office) examination of the right eye revealed an IOP of 25 mmHg, an axial length (AL) of 23.87 mm, a cup/disc (C/D) ratio of 0.8 with a diffuse choroidal haemangioma (the thickness of which, unfortunately, was not measured by B-scan ultrasound; US) manifesting as the tomato katsup fundus appearance, a best corrected visual acuity of 0.3 (decimal) (with −1.50 cylindrical dioptres × 100°), and ophthalmic US revealed a choroidal haemangioma. Topical combination β-blocker–CAI twice daily was prescribed, and follow-up revealed an IOP of 26 mmHg, AL of 22.63 mm, and C/D ratio
of 0.8 (on topical combination therapy). The left eye demonstrated an IOP of 16 mmHg, an AL of 21.37 mm, and C/D ratio of 0.5. The decision of surgery was taken, and the right eye was subjected to uneventful combined trabeculotomy-
trabeculectomy with mitomycin C (which is the routine primary surgical procedure for cases of childhood glaucoma in the authors’ institution). Serial follow-ups reveal an IOP
of 4 mmHg, AL of 21.87 mm, a C/D ratio of 0.6, and a pale
localised bleb at postoperative month 7. At postoperative
month 8, the child presented with an extremely shallow AC (central and peripheral, peripheral iridocorneal touch with no central lenticulocorneal touch) (Figure 1A, 1B), soft (unmeasurable) IOP, an intact Seidel test, a pale, diffuse, low-lying bleb (Figure 1C), and a fundus with no ophthalmoscopically visible abnormality. B-scan US revealed the underlying choroidal haemangioma with measured thickness ranging between
1.94 mm and 2.96 mm in different scan areas (Figure 1D, 1E), with a thin rim of suprachoroidal effusion measuring 0.53 mm
(Figure 1F). The child was prescribed topical atropine eye drops 3 times daily and systemic steroids. Three days later the AC remained shallow and the IOP remained soft while ophthalmic B-scan US revealed the same minimal rim of suprachoroidal effusion (0.44 mm). The child was maintained on the same treatment regimen until 8 days after the first presentation with shallow AC, at which time the child presented with a formed AC with almost normal depth (Figure 1G), then full normal depth
3 days later (Figure 1H), and the B-scan US revealed resolution of the suprachoroidal effusion (Figure 1I). The dose of atropine was reduced to once daily. The condition remained stable for 8 days, at which time the child presented again with shallow AC and soft IOP, upon which the dose of atropine was restored to the previous level of 3 times daily. The AC depth normalised again after
3 days and remained stable at a dose of atropine 3 times daily.
Subsequent follow-up revealed an IOP of 5 mmHg, a C/D ratio of 0.5, a pale, diffuse, low-lying bleb, and a formed AC up to
2 months after the initial event of shallow AC. Minute filiform peripheral anterior synechia (PAS) formed on the temporal aspect of the AC with a slightly distorted pupil drawn temporally (Figure 1J). Atropine eye drops were subsequently withdrawn, and the patient’s condition remained stable with formed AC. The child remains under follow-up as part of routine care in the study setting, which has, at the time of the last revision of this manuscript, completed almost 20 months after the reported incident of shallow AC and 28 months from the glaucoma surgery.

DISCUSSION

This case presents SWS-associated glaucoma in an 11-year-old child, suggesting an elevated EVP as the mechanism of elevated IOP and glaucoma, as reported in that age group [2]. The poor response to topical IOP-lowering medications parallels other reports [2], and so does the surgical option of combined trabeculotomy-trabeculectomy with mitomycin C with a reported success rate of over 90% [5]. The finding of a soft IOP on presentation with a shallow AC excluded suprachoroidal haemorrhage or aqueous misdirection as the pathologies underlying the shallow AC, both characteristically presenting with an elevated IOP. The fact that the bleb was low with a negative Seidel test excluded over-filtration as the underlying culprit, and indirect ophthalmoscopy confirmed the absence of a choroidal detachment. However, the B-scan US confirmed the presence of a diffuse, relatively thin suprachoroidal effusion. The time lapse of 8 months between the surgery and the presentation with a shallow AC explained the possible closure of the prophylactic inferior sclerotomy performed intraoperatively, a manoeuvre previously reported by the author
to guard against early postoperative suprachoroidal effusions in surgery for SWS-associated glaucoma [5]. The potential benefit of atropine in the deepening of shallow ACs through posterior pulling of the ciliary ring has already been reported [6]. However, the chronic use of atropine with subsequent persistence of the dilated pupil position and iris crowding in the angle
of an AC that was apparently somewhat shallow after all and not of normal depth eventually, obviously contributed to the formation of PAS in this case. This assumption remains hypothetical because the depth of the AC was not actually quantified by any imaging device such as ultrasound biomicroscopy or anterior segment optical coherence tomography, and hence the clinical judgement may have left some room for miniscule shallowness that passed unnoticed. Use of topical steroids was avoided for fear of inducing any bleb thinning or erosion; the role of steroids in bleb modelling is documented in published reports [7]. What remains debatable is the cause of resolution of the condition, apparently under the influence
of the systemic steroids, in accordance with other studies [8] citing the therapeutic effect of systemic steroids on suprachoroidal effusions. The need for topical atropine maintenance therapy for some time highlights the potent effect of atropine on the ciliary ring and its crucial role in eyes with postoperative shallow AC.

In conclusion, delayed suprachoroidal effusion developed 8 months after combined angle and filtering surgery for SWS-associated glaucoma in an 11-year-old girl, and it was resolved by systemic steroids and topical atropine.

DISCLOSURES

The authors declare no conflict of interest.

This work received no external funding.

Publication of the case report was approved by the Ethics Committee of Alexandria University Faculty of Medicine
(IRB serial no. 0307713, date: 04/01/2026). Consent for publication of the case was verbally obtained from the care provider
of the child after thorough explanation of the condition.
The data are anonymised and no individual can be identified from the submitted photos.

References