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Year : 2016  |  Volume : 28  |  Issue : 2  |  Page : 142-144

Trabeculectomy with scleral patch graft for advanced glaucoma in ehler–danlos syndrome

Department of Glaucoma, Aravind Eye Hospital, Coimbatore, Tamil Nadu, India

Date of Web Publication20-Mar-2017

Correspondence Address:
Dhanya Cheriyath
Glaucoma Consultant, Al Salama Eye Hospital, Kannur, Kerala
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/kjo.kjo_32_16

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We report a case of secondary glaucoma in Ehler–Danlos syndrome (EDS) Type VI, which was uncontrolled with maximal medical therapy, and was subsequently managed surgically. EDS comprises a subgroup of heritable connective tissue disorders characterized by generalized joint hypermobility, skin hyperextensibility, and tissue fragility. The surgical challenges in these patients are presence of scleral thinning, vascular fragility, and poor wound healing. Surgical intervention such as trabeculectomy in EDS has not been reported in the literature. With this case report, we share our trials and tribulations in managing this patient with trabeculectomy without antimetabolites, with unexpected modification in the surgery and success.

Keywords: Ehler–Danlos syndrome (EDS), intractable glaucoma, scleral patch graft, trabeculectomy

How to cite this article:
Cheriyath D, Bharathi S, Raman GV. Trabeculectomy with scleral patch graft for advanced glaucoma in ehler–danlos syndrome. Kerala J Ophthalmol 2016;28:142-4

How to cite this URL:
Cheriyath D, Bharathi S, Raman GV. Trabeculectomy with scleral patch graft for advanced glaucoma in ehler–danlos syndrome. Kerala J Ophthalmol [serial online] 2016 [cited 2023 Feb 8];28:142-4. Available from: http://www.kjophthal.com/text.asp?2016/28/2/142/202477

  Introduction Top

Ehler–Danlos Syndrome (EDS) comprises a subgroup of heritable connective tissue disorders, and its Type VI is of interest to ophthalmologist because of its ocular involvement along with joint and skin manifestations. Ocular manifestations include epicanthal folds, blue sclera, keratoconus, keratoglobus, glaucoma, myopia, retinal detachment, and rarely angioid streaks.[1] Surgical intervention for medically uncontrolled glaucoma in EDS patients can be challenging because of extreme scleral thinning, tissue fragility, and poor wound healing. We report a case of presumed EDS type VI with secondary angle closure glaucoma that was managed successfully with trabeculectomy with scleral patch graft and without antimetabolites.

  Case Report Top

A 29-year-old female [Figure 1] presented with gradual painless progressive loss of vision in the right eye for 5 years. Past history was significant for cataract extraction with intraocular lens implantation and vitreoretinal surgery 3 years back in the left eye. She was diagnosed with glaucoma in the right eye and was on four antiglaucoma medications. Family history revealed consanguineous marriage in parents, and none of her family members were affected. Systemic examination revealed short stature, kyphosis arachnodactyly, keloids [Figure 2], and hyperextensibility of joints [Figure 3]. Her best corrected Snellen's visual acuity (BCVA) was 1/60 in the right eye, and there was perception of light with inaccurate projection of rays in the left eye. Anterior segment examination revealed 360° ciliary staphyloma and clear cornea in both eyes. Anterior chamber was shallow in the right eye with sluggishly reacting pupil and a clear lens. Left eye showed a mid-dilated pupil and a posterior chamber intraocular lens in situ. Intraocular pressure (IOP) by Goldmann applanation was 28 mmHg in the right eye and 12 mmHg in the left eye. Gonioscopy revealed occludable angles in the the right eye and open angles in the left eye. Fundus examination showed cup-disc ratio of 0.9:1 in the right eye and retinal detachment in silico n oil filled globe in the left eye. Pachymetry values were 392 microns and 379 microns in the right and left eye, respectively. Keratometry readings were K1 = 53.5@180, K2 = 54.0@ 90 in the right eye and K1 = 53.5@180, K2 = 54.5@90 in the left eye. Axial length by IOL master (Version Carl Zeiss Meditec) was 26.89 mm and 33.20 mm in the right and left eye, respectively.
Figure 1: Preoperative picture

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Figure 2: Arachnodactyly and keloid

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Figure 3: Hyperextensibility of joints

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We performed a Nd:YAG laser peripheral iridotomy (LPI) in the right eye for occludable angles. One week later, LPI was patent but her IOP was 32 mmHg with maximum medical therapy. She was advised trabeculectomy, and the inferotemporal site was chosen because of the relative absence of ciliary staphyloma and scleral thinning. Trabeculectomy was performed as per the standard procedure. A fornix-based conjunctival flap was raised, and after adequate cautery, an attempt was made to lift a partial thickness scleral flap. The resultant scleral flap was very thin and accidentally got amputated from the scleral bed. In the spur of the moment, we decided to close the scleral bed with a cadaveric donor scleral patch graft. The anterior chamber was entered and a block of sclera was punched using a Kelly–Descemets punch and a peripheral iridectomy was made. A cadaveric scleral patch graft of size 4 × 4 mm was fashioned and sutured to the limbus using two interrupted 10/0 nylon sutures anteriorly and a mattress suture posteriorly. The conjunctiva was brought over the scleral patch graft and wing sutures were applied using 8/0 polyglactin sutures. Immediate postoperative period was uneventful [Figure 4]. Consecutive follow-ups have shown a satisfactory intraocular pressure control in the low teens and retention of preoperative visual acuity. One year after surgery, the right eye showed a diffuse bleb with scleral patch graft in situ [Figure 5] with an intraocular pressure of 10 mmHg and maintained a visual acuity of 5/60.
Figure 4: POD 1 image showing bleb with scleral patch graft

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Figure 5: Post operative image at 1 year showing diffuse bleb with scleral patch graft in situ

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  Discussion Top

EDS type VI patients have either a defective or an absence of enzyme lysyl hydroxylase resulting in abnormal collagen crosslinking along with marked genetic heterogeneity within type VI.[1] The trabecular basement membranes contain collagen types I, III, IV, and V,[2] and defective collagen in these patients can result in abnormal aqueous outflow leading to glaucoma. However, Pollack et al. described glaucoma that occurred secondary to spontaneous caroticocavernous fistula in an EDS type IV patient.[3]

Globe rupture has been reported in these patients either spontaneously or following trivial trauma,[1],[4] and our apprehension of the same in the presence of uncontrolled IOP made our decision to surgically intervene. Surgical options such as tube shunts and laser cyclophotocoagulation are not viable options because of obvious reasons. Trabeculectomy becomes difficult due to the thin nature of the sclera; hence, as in our case, additional support in the form of scleral patch graft was required. Minimal manipulation during surgery is advised with special caution while suturing. Even retinectomy as a treatment modality for intractable glaucoma in EDS has been described in the literature.[5]

It is gratifying to see that our patient did not have any intraoperative or postoperative complication, achieved adequate intraocular pressure control, and retained her preoperative vision. Even though a single case with limited follow-up is not adequate in evaluating the efficacy of the procedure, it is important to keep in mind options of such a procedure in the best interest of maintaining the patient's quality of life.

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

Krachmer JH, Mannis MJ, Holland EJ. Cornea. 3rd edition. US Elsevier Health Book; 2011.  Back to cited text no. 1
Murphy CG, Yun AJ, Newsome DA, Alvarado JA. Localization of extracellular proteins of the human trabecular meshwork by indirect immunofluorescence. Am J Ophthalmol 1987;104:33-43.  Back to cited text no. 2
Pollack JS, Custer PL, Hart WM, Smith ME, Fitzpatrick MM. Ocular complications in Ehlers-Danlos syndrome type IV. Arch Ophthalmol 1997;115:416-9.  Back to cited text no. 3
Puri P, Gupta M, Chan J. Spontaneous perforation of the globe in Ehlers Danlos syndrome. Eye 2001;15:553-4.  Back to cited text no. 4
Joussen AM, Walter P, Jonescu-Cuypers CP, Koizumi K, Poulaki V, Bartz-Schmidt KU. Retinectomy for treatment of intractable glaucoma: Long term results. Br J Ophthalmol 2003;87:1094-102.  Back to cited text no. 5


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]

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