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 Table of Contents  
Year : 2022  |  Volume : 2  |  Issue : 1  |  Page : 72-73

Non-Descemet stripping endothelial keratoplasty in an adolescent with aphakia and glaucoma drainage device

1 Pediatric Ophthalmology and Ocular Genetics, Wills Eye Hospital, Philadelphia, PA, USA; Department of Ophthalmology, Ajou University School of Medicine, Suwon, South Korea
2 Cornea Service, Wills Eye Hospital; Department of Ophthalmology, Sidney Kimmel Medical College, Thomas Jefferson University, Philadelphia, PA, USA
3 Pediatric Ophthalmology and Ocular Genetics, Wills Eye Hospital; Department of Ophthalmology, Sidney Kimmel Medical College, Thomas Jefferson University, Philadelphia, PA, USA
4 Department of Ophthalmology, Flaum Eye Institute, University of Rochester, Rochester, NY, USA

Date of Submission29-Jun-2021
Date of Acceptance08-Sep-2021
Date of Web Publication07-Jan-2022

Correspondence Address:
Dr. Alex V Levin
Pediatric Ophthalmology and Ocular Genetics, Flaum Eye Institute, 601 Elmwood Avenue, Box 659, Rochester - 14642, NY
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_1725_21

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We describe the case of a 14-year-old patient with secondary endothelial failure that was treated with non-Descemet stripping endothelial keratoplasty (nDSEK). To our knowledge, this is the first report on nDSEK in the setting of aphakia and a glaucoma drainage device in a child.

Keywords: Adolescent, aphakia, Descemet stripping endothelial keratoplasty, glaucoma drainage device

How to cite this article:
Chung SA, Meghpara B, Price JM, Levin AV. Non-Descemet stripping endothelial keratoplasty in an adolescent with aphakia and glaucoma drainage device. Indian J Ophthalmol Case Rep 2022;2:72-3

How to cite this URL:
Chung SA, Meghpara B, Price JM, Levin AV. Non-Descemet stripping endothelial keratoplasty in an adolescent with aphakia and glaucoma drainage device. Indian J Ophthalmol Case Rep [serial online] 2022 [cited 2022 Jan 28];2:72-3. Available from: https://www.ijoreports.in/text.asp?2022/2/1/72/334904

Pediatric Descemet stripping endothelial keratoplasty (DSEK) can be surgically challenging as children are less likely to maintain postoperative supine positioning, have smaller anterior segments, have a Descemet membrane (DM) that is difficult to identify and strip, and can present with ocular comorbidities such as the presence of aphakia or a glaucoma drainage device.[1],[2],[3],[4],[5],[6] We herein report on a 14-year-old patient with aphakia and a glaucoma drainage device undergoing non-DM stripping endothelial keratoplasty (nDSEK).

  Case Report Top

A 14-year-old boy presented with blurred vision and photophobia in the left eye. His history is remarkable for bilateral juvenile open-angle glaucoma (JOAG), diagnosed at age 6. His JOAG, initially controlled with medical treatment, required surgical management starting at age 8: Bilateral nasal goniotomy followed by bilateral tube implantation and temporal goniotomy in the left eye. The patient developed idiopathic anterior uveitis in the left eye at age 9. Systemic workup was negative and onset was prior to tube placement in this eye. The uveitis, ultimately uncontrolled with topical corticosteroid, eventually required methotrexate and adalimumab. He underwent cataract extraction and was left aphakic due to the risk of uveitis at age 11, YAG capsulotomy at age 12, and anterior vitrectomy with removal of vitreous from the tube shortly thereafter. The uveitis and glaucoma had since been controlled.

At age 14, the patient presented with corneal edema in the left eye and a decline in best-corrected visual acuity from 20/25 to 20/300 over the course of four months. Intraocular pressure (IOP) was 18 mm Hg in the left eye on dorzolamide-timolol drops. Corneal thickness was 942 μm (previously 663 μm). The anterior chamber was quiet with diffuse peripheral anterior synechiae (PAS). The proposed etiology of corneal edema was a combination of multiple surgical procedures and repetitive trauma from the tube secondary to frequent eye rubbing. The edema did not improve with hypertonic saline ointment. A clinical diagnosis of endothelial failure was made and nDSEK was performed. A temporal clear corneal incision was made, and limited dissection of PAS was performed to create enough space to insert and position the corneal graft. DM stripping was attempted; however, it was adherent to the overlying stroma and could not be performed. After careful measurement, it was determined there was enough room to implant an 8-mm donor lenticule to both positions between the PAS and maximize the number of endothelial cells implanted. The graft was loaded on a Busin glide and inserted into the anterior chamber. The graft was carefully centered and an air bubble was injected into the anterior chamber. A 100% anterior chamber air fill was desired, anticipating that some air may escape via the tube, but it could not be maintained. Gas tamponade using 10% sulfur hexafluoride (SF6) was then attempted and a full anterior chamber gas fill at physiologic pressure was achieved. The patient remained supine positioning for one hour, at which point he was examined and found to have an attached graft with 70% gas fill of the anterior chamber. He was discharged with instructions to maintain supine positioning, and postoperative steroid and antibiotic drops were started.

The graft remained attached and the edema cleared over the following 6 weeks at which point the vision improved to 20/25, and he was cleared to resume aphakic contact lens use [Figure 1] and [Figure 2]. Nine months postoperatively, the patient developed unexplained bilateral IOP elevation with resultant corneal epithelial edema in the left eye. IOP could not be controlled medically and the patient underwent successful micropulse cyclophotocoagulation. At last follow-up, 21 months post nDSEK, the cornea remained clear and IOP was controlled on topical medication.
Figure 1: Slit-lamp image 7 months after nDSAEK showing a clear central cornea. The patient has a YAG posterior capsulotomy through an otherwise fibrotic posterior capsule. The pupil is pharmacologically dilated. The glaucoma drainage tube can be seen superotemporally

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Figure 2: Anterior segment optical coherence tomography 7 months after nDSAEK showing a graft uniformly adhered to the posterior cornea. There is contact between the edge of the graft and a retrocorneal fibrotic membrane indicated by arrows

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  Discussion Top

Adequate air tamponade is difficult in the setting of aphakia and in presence of a tube shunt as the air can either migrate posteriorly or out the tube. In addition, the tube makes positioning of the graft challenging as contact with the donor endothelium increases the risk of graft failure. Of the 62 pediatric endothelial keratoplasty cases reported to date, only four patients had aphakia, three had a history of previous trabeculectomy, and none had a glaucoma drainage tube in place.[1],[2],[4],[5]

This case with diffuse PAS, aphakia, and a glaucoma drainage device demonstrates several modifications to our standard DSEK technique to overcome these surgical challenges. To create sufficient room in the anterior chamber for donor insertion, limited dissection of PAS was performed. Given its propensity of recurrence in children, complete dissection of all PAS was not attempted in order to limit resultant inflammation. Concern for graft dislocation was high in this case given the presence of a tube shunt in a unicameral eye. Both factors make it difficult to fill the anterior chamber with sufficient air needed to tamponade the graft against the host stroma. A high-pressure air tamponade is a step critical for graft attachment in DSEK. In this case, complete air fill was unsuccessful, however when 10% SF6 gas was used a full anterior chamber fill was achieved, albeit only at a physiologic pressure. Compared to air, SF6 gas has a higher surface tension, longer half-live, and better buoyancy,[7] which may help sequester the gas in the anterior chamber maintaining tamponade of the donor cornea, rather than escaping posterior into the vitreous cavity or out the tube shunt. SF6 gas at an isoexpansile 20% concentration could also be used; however, to limit the chance of gas expansion and resultant IOP elevation, a lower concentration was used. DM could not be stripped in our case. This is not uncommon in children as DM is strongly adherent to the stroma in this population. Previous reports confirm leaving DM neither hinders graft attachment nor influences visual outcome.[3],[8],[9] In addition to meticulous surgical techniques, intensive postoperative glaucoma management is essential. Worsening of preexisting glaucoma after DSEK is a well-recognized risk factor for graft failure; thus, prompt intervention for glaucoma facilitates DSEK survival.[10]

  Conclusion Top

With technique modification, DSEK can be successfully performed in children with uveitis, aphakia, and glaucoma drainage tube. We believe this procedure warrants consideration for corneal endothelial cell failure in this patient population.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Anwar HM, El-Danasoury A. Endothelial keratoplasty in children. Curr Opin Ophthalmol 2014;25:340-6.  Back to cited text no. 1
Medsinge A, Nischal KK. Paediatric keratoplasty: Choices and conundrums. Br J Ophthalmol 2013;97:1225-7.  Back to cited text no. 2
Busin M, Beltz J, Scorcia V. Descemet-stripping automated endothelial keratoplasty for congenital hereditary endothelial dystrophy. Arch Ophthalmol 2011;129:1140-6.  Back to cited text no. 3
Yang F, Hong J, Xiao G, Feng Y, Peng R, Wang M, et al. Descemet stripping endothelial keratoplasty in pediatric patients with congenital hereditary endothelial dystrophy. Am J Ophthalmol 2020;209:132-40.  Back to cited text no. 4
Goshe JM, Li JY, Terry MA. Successful Descemet's stripping automated endothelial keratoplasty for congenital hereditary endothelial dystrophy in a pediatric patient. Int Ophthalmol 2012;32:61-6.  Back to cited text no. 5
Ramappa M, Ashar J, Vaddavalli PK, Chaurasia S, Murthy SI. Endothelial keratoplasty in children: Surgical challenges and early outcomes. Br J Ophthalmol 2012;96:1149-51.  Back to cited text no. 6
Cekic O, Ohji M. Intraocular gas tamponades. Semin Ophthalmol 2000;15:3-14.  Back to cited text no. 7
Masaki T, Kobayashi A, Yokogawa H, Saito Y, Sugiyama K. Clinical evaluation of non-Descemet stripping automated endothelial keratoplasty (nDSAEK). Jpn J Ophthalmol 2012;56:203-7.  Back to cited text no. 8
Caldwell MC, Afshari NA, Decroos FC, Proia AD. The histology of graft adhesion in descemet stripping with endothelial keratoplasty. Am J Ophthalmol 2009;148:277-81.  Back to cited text no. 9
Quek DT, Wong T, Tan D, Mehta JS. Corneal graft survival and intraocular pressure control after descemet stripping automated endothelial keratoplasty in eyes with pre-existing glaucoma. Am J Ophthalmol 2011;152:48-54.  Back to cited text no. 10


  [Figure 1], [Figure 2]


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