Treating refractory corneal hydrops in a male patient with vernal keratoconjunctivitis and mental retardation: a case report

Abstract

Background: Keratoconus is the most common noninflammatory bilateral corneal ectasia. Vernal keratoconjunctivitis (VKC) and eye rubbing may be associated with keratoconus in children and young adults. Timely management of advanced keratoconus is important to improve visual quality. In addition, it is challenging to carry out VKC treatment with an intent to avoid the occurrence of punctate epithelial keratitis, ulceration, or corneal neovascularization on corneal grafts.

Case presentation: We report the case of an 18-year-old male patient with a long-term history of mental retardation due to megalencephaly presenting with acute onset of corneal hydrops with prominent bulging and refractory steroid-induced glaucoma of the right eye. The topography of the right eye was unavailable due to advanced ectasia, and that of the left eye revealed central steepening with inferior-superior dioptric asymmetry. According to the clinical findings, the patient was diagnosed with keratoconus. Because of progressive corneal opacity and neovascularization, the patient underwent penetrating keratoplasty (PK) with combination of interrupted and intrastromal running suturing after receiving a preoperative subconjunctival injection of bevacizumab in his right eye, followed by lower eyelid correction. After surgery, the patient was treated with 0.1% tacrolimus dermatological ointment, 0.1% cyclosporine eye drops, artificial tears, and 0.5% loteprednol for keratoplasty and VKC. Repeated education on avoiding eye rubbing was offered to the patient. Two years after PK treatment, his best-corrected visual acuity of the right eye successfully improved from hand motion at 10 cm preoperatively to 6/20 postoperatively.

Conclusions: Large-diameter PK with intrastromal suturing technique for advanced keratoconus could achieve better visual outcomes and avoid suture-related complications. In addition, tacrolimus dermatological ointment rather than tacrolimus topical eye drops or ointment showed satisfactory efficacy when combined with topical cyclosporine and steroid that no significant VKC reactivation were noted after PK.

Keywords: Case report; Corneal hydrops; Intrastromal suturing; Keratoconus; Penetrating keratoplasty; Tacrolimus; Vernal keratoconjunctivitis.

Fig. 1

Pre-PK slit-lamp photography and ASOCT of the right eye and topography of the left eye. A ASOCT image of the right eye showing corneal hydrops with severe corneal edema. B, C Topographic images of the left eye showing central steepening with inferior-superior dioptric asymmetry. D Slit-lamp photographic image demonstrating severe corneal edema, total opacity with neovascularization of the right eye from limbus to limbus, epithelial bullae, and bulging eye with extensive corneal neovascularization superiorly. E Image taken 2 weeks later shows 360-degree peripheral corneal neovascularization. PK, penetrating keratoplasty; ASOCT, anterior segment optical coherence tomography

Fig. 2

The surgical image and schematic diagram of the combined interrupted and continuous intrastromal suturing in PK. A Large-diameter PK with a combination of interrupted and intrastromal running suturing (red arrow). B The technique of combined interrupted and continuous intrastromal suturing was performed first with eight radial interrupted sutures (coarse solid lines), followed by intrastromal sutures (dotted line). The needle’s entry and exit points were the same to ensure that the intrastromal sutures passes over the intrastromal and deep layers rather than the epithelial layer alone. Finally, the graft-host junction was strengthened by additional eight sutures (fine solid lines). PK, penetrating keratoplasty

Fig. 3

Pathologic findings obtained using light microscopy and transmission electron microscopy. A H&E staining image at 40× magnification showing Bowman’s layer with focal thickening (red star) and detachment (red arrow). B CD31 staining image at 200× magnification showing focal vascularization of the stroma (red star). C Transmission electron microscopy image of the stroma showing focal vascularization (red star) (original magnifications: 1400×). D Transmission electron microscopy image showing some equivocal collagen fibril disarray in the Bowman’s layer (red box) (original magnifications: 1400×). H&E, Hematoxylin and eosin. *We used light microscopy Eclipse E600 (Nikon Instruments Inc., Tokyo, Japan) with Nikon DS-Ri2 camera, and software of NIS-Elements documentation. The transmission electron microscopy JEM-1400 (JEOL Inc., Tokyo, Japan) is equipped with a CMOS camera, Silicon drift detectors, filters of NCB11, ND8, ND32, and software of RUBY 2 CCD

Fig. 4

Postoperative external photography of the right eye after penetrating keratoplasty. A, B Images taken at 3 months after PK showing a persistent central corneal epithelial defect with vernal keratoconjunctivitis of the right eye. C, D Images taken 6 months after PK and 3 months after epiblepharon correction showing no corneal opacity or edema. The image also shows resolved trichiasis and epithelial defect with minimal superficial punctate keratitis. No suture-related complications, such as suture infiltration and loosening were noted over our intrastromal continuous running sutures (red star). PK, penetrating keratoplasty