Descemet Stripping Endothelial Keratoplasty in Pediatric Patients with Congenital Hereditary Endothelial Dystrophy





Purpose


To report the long-term outcomes of Descemet stripping endothelial keratoplasty (DSEK) with suture-assisted donor lenticule insertion performed in different age groups for pediatric patients with congenital hereditary endothelial dystrophy (CHED).


Design


Retrospective case series.


Methods


Pediatric patients with CHED who underwent DSEK from January 2010 to January 2016 were enrolled. Patients were divided into 2 groups according to their ages: the infant group and the child group. Long-term clinical outcomes and complications were compared.


Results


Thirty eyes of 16 patients were included: 19 eyes (10 patients) in the child group and 11 eyes (6 patients) in the infant group. The average duration of follow-up was 4.08 ± 1.90 years (range 2.5–8.5 years). Corneal transparency scores of the 2 groups on postoperative day 7 were not statistically different. The average postoperative best-corrected visual acuity (BCVA) in the infant group (logMAR 0.32 ± 0.11) was better than that in the child group (logMAR 0.54 ± 0.20; ( P = .01). Thirty-three percent of cases in the child group and 86% of cases in the infant group had postoperative BCVA achieved or better than logMAR 0.4. Average endothelial cell loss in the child group was 31.21% ± 9.17%. Lenticule detachment occurred in 3 cases in the child group.


Conclusions


Improved visual outcomes could be achieved in infant patients with CHED after DSEK without significant complications. Suture-assisted donor lenticule insertion techniques, Descemet membrane stripping, and postoperative sedation are advocated technical points.


Highlights





  • DSEK for children with congenital hereditary endothelial dystrophy.



  • Different age groups in pediatric patients.



  • Long-term outcome.



Congenital hereditary endothelial dysfunction (CHED), which is characterized by bilateral diffuse corneal edema, typically presents at birth or in early infancy as a common cause of childhood corneal opacification and often results in amblyopia. Histopathologically, corneal edema is attributable to abnormalities in the Descemet–endothelium complex: diffuse thickening and lamination of Descemet’s membrane (DM) and sparse and atrophic endothelial cells. Although CHED is primarily a disease of the corneal endothelium and DM, penetrating keratoplasty (PK) has been the traditional mainstay of treatment for these patients. However, PK is a challenging task in pediatric patients compared with adults. Low scleral rigidity and positive vitreous pressure offer a high risk of vision-threatening complications during surgery, such as suprachoroidal hemorrhage. Compared with adults, pediatric patients after PK face an increased risk of infection and rejection because of frequent suture loosening, which requires early exchange or removal and subsequent general anesthesia support. Even when clear grafts are obtained, visual rehabilitation may be complicated by intractable amblyopia because of high and unpredictable astigmatism. In addition, late traumatic dehiscence in PK wounds is more common in children than in adults, given the unpredictable nature of their daily activities. For these reasons, surgery has often been avoided or delayed for as long as possible in children with CHED, which often compromises the visual development of these patients.


Currently, targeted corneal endothelium transplantation techniques, such as Descemet stripping endothelial keratoplasty (DSEK), have been preferred over PK for endothelial dysfunctions in children because of the advantages of rapid visual recovery, stabilized refractive error, decreased likelihood of amblyopia, and decreased risk of traumatic globe rupture. Despite these advantages, there are few series describing DSEK in pediatric patients with CHED and even fewer involving infants.


This series reported the outcomes of DSEK performed in 30 phakic eyes of 16 pediatric patients with CHED in different age groups.


Methods


This retrospective cohort study was approved by the Institutional Review Board of Peking University Third Hospital. The informed consents of embracing images and data in this study were received from the patients’ parents.


The medical records and eye bank records of all the pediatric patients with CHED who underwent DSEK performed by Dr Hong at the Ophthalmology Department of Peking University Third Hospital from January 2010 to January 2016 were analyzed. Patients were divided into 2 groups according to their ages at the time of surgery: the infant group included patients <1 year of age and the child group included patients ≥1 year of age. Retrospective data collection included demographics, family histories, and medical and ocular surgical histories. Other preoperatively recorded data included the best-corrected visual acuity (BCVA; logMAR visual chart), intraocular pressure (IOP), slit-lamp biomicroscopy findings, and endothelial density of the donor tissue.


Surgical Technique


The main surgical steps are illustrated in Figure 1 .




Figure 1


Descemet stripping endothelial keratoplasty with suture-assisted donor lenticule insertion technique. Scoring of the Descemet membrane using a reverse Terry–Sinskey hook according to the circular mark (A); scraping of the recipient bed with a Terry scraper (B); pulling the folded donor lenticule into a Busin’s glide with forceps (C); fixing a 10-0 prolene suture as an anchoring stitch on the donor lenticule at the 6 o’clock position (D); the anchoring stitch was placed in the anterior chamber and pulled through the incision at 6 o’clock direction with a hook (E); donor lenticule delivery with the assistance of Busin’s glide and the anchoring stitch (F); donor lenticule unfolding in the anterior chamber (G); air injection to maintain 75% air volume in the anterior chamber (H).


Preparation of the Donor Tissue


The donor tissues stored in McCarey–Kaufman medium were obtained from the Eye Bank of Peking University Third Hospital and were evaluated for suitability for DSEK. The donor graft dissection was performed with a Moria CB microkeratome (head 350-400 μm blade depth; Moria Surgical, Doylestown, Pennsylvania, USA) in 26 eyes and manually using a Moria ALTK artificial anterior chamber (Moria Surgical) in 4 eyes because of the flexible characteristics of the tissues of pediatric donors <2 years of age. The donor tissues were prepared by the surgeon immediately before surgery, and the dissected buttons were stored in McCarey–Kaufman medium during the surgical interim.


Preparation of the Recipient Bed


All surgeries were performed under general anesthesia. A circular mark with a diameter of 7.5 or 8.0 mm was made on the anterior corneal surface to delineate where to strip the DM. A main scleral tunnel was made at the 12 o’clock position. Using Healon GV (Abbott Medical Optics, Abbott Park, Illinois, USA) to maintain the anterior chamber, scoring of DM with a reverse Terry–Sinskey hook (Bausch and Lomb Surgical, St. Louis, Missouri, USA) and scraping of the recipient bed with a Terry Scraper (Bausch and Lomb Surgical) were performed. All DM specimens were sent for pathological analysis.


Donor Lenticule Insertion


Because of the phakic status of all patients, pilocarpine was applied preoperatively to protect the transparent crystalline lenses, and the donor lenticule was inserted into the anterior chamber using a suture-assisted donor lenticule insertion technique to minimize manipulations in the anterior chamber. The donor lenticule overlying a bed of cohesive viscoelastic was carefully folded and pulled into Busin’s glide (Moria Surgical) with forceps, and a 10-0 prolene suture was fixed at the 6 o’clock position as an anchoring stitch. The anchoring stitch was tied to create a loop, after which it was placed in the anterior chamber and pulled through the incision in the 6 o’clock direction with a hook. After evacuation of all the viscoelastic material, an anterior chamber maintainer was inserted through the temporal incision to provide irrigation. The donor lenticule was pulled into the anterior chamber through the main scleral tunnel at the 12 o’clock position with the assistant of Busin’s glide and the anchoring stitch. Once inserted, the donor lenticule was unfolded in balanced salt solution (BSS, Alcon, Fort Worth, Texas, USA).


To prevent postoperative pupillary block by air bubbles, peripheral iridectomy was performed. The scleral incision was then interrupted sutured with 10-0 prolene sutures. The anchoring stitch was cut after the donor lenticule was positioned and centered. A complete air fill was performed and maintained for 10 minutes, followed by gentle partial release of air to maintain 75% air volume in the anterior chamber. The patient was maintained in the supine position for 4 hours. For children who could not cooperate, postoperative sedation was provided by an anesthesiologist.


Postoperative Management


Postoperatively, topical drops including 1% prednisolone acetate, tobramycin (Tobrex, Alcon Laboratories, Inc., Fort Worth, Texas, USA), cyclosporin (1%; North China Pharmaceutical Company, Ltd., Shijiazhuang, Hebei Province, China), and artificial tears 4 times daily were prescribed for the first week. The dosages were reduced gradually as clinically indicated and stopped 12 months postsurgery.


Patients were reviewed at 1, 3, 7, and 30 days and at 3, 6, and 12 months postoperatively and once per year thereafter. BCVA was tested at each visit using a logMAR visual chart. For patients who were too young to cooperate for the vison test, whether they could fix and follow (FF) light was checked using a torch. IOP (noncontact tonometer), lenticule status, corneal clarity, and complications were recorded at each visit. Anterior segment optical coherence tomography (Carl Zeiss Meditec, Dublin, California, USA) and endothelial evaluation with confocal microscopy (Heidelberg Engineering, GmBH, Dossenheim, Germany) were performed when possible and appropriate. Sutures were removed within the first month postoperatively. All patients were referred to a pediatric ophthalmologist for amblyopia therapy 1 month after DSEK.


Statistics


Postoperative corneal transparency was scored according to slit-lamp photographs taken on postoperative day 7 that were compared with preoperative photographs. The criterion of corneal transparency scores were as follows: −1, worse than preoperative transparency; 0, the same as preoperative transparency; +1, better than preoperative transparency but not completely transparent 1; and +2, transparent. Differences in postoperative corneal transparency between the 2 groups were compared with the Mann–Whitney rank sum test. The differences in BCVA between the 2 groups were compared with independent sample t tests. Patients in the child group whose preoperative and postoperative BCVA scores were both available were compared with paired sample t tests.


Histopathology of Removed DM Specimens


To assess the pathologic features of DM and the endothelium in CHED, histopathologic procedures were performed in all the removed DM specimens, including hematoxylin–eosin and periodic acid–Schiff staining. The samples were observed by light microscopy (original magnification ×100; Cytation5, BioTek, Winooski, Vermont, USA).


One of the removed DM specimens was further inspected using scanning electron microscopy. The sample was fixed with 2% glutaraldehyde in an 80 mM sodium cacodylate buffer (pH 7.2–7.4, 320–340 mOsm/kg), processed with gold-coated magnetic particles for solid-phase immunoassays and, after the critical point of drying, examined with a scanning electron microscope (JEOL JSM-7000F; JEOL, Peabody, Massachusetts, USA). The sample was screened at low magnification (original magnification ×30–×200) and pictured at higher magnifications (original magnification ×60.000).




Results


Preoperative and postoperative clinical data of the patients with CHED in this study are listed in Table 1 .



Table 1

Preoperative and Postoperative Clinical Data of the Patients With Congenital Hereditary Endothelial Dystrophy in 2 Groups
























































































































































































































































































































































































Age at OP (Year/Month) Group (Child/Infant) Patient No. Gender Eye BCVA Preoperatively (log MAR) BCVA Postoperatively (Last Follow-Up, logMAR) ECL (Last Follow-Up, %) CTS (Postoperative Day 7) Follow-Up (Year) Complication
6 M I 6 M OD No FF 0.18 NA NA 4 No
6 M I 6 M OS No FF 0.18 NA NA 4 No
8 M I 12 M OD No FF 0.4 NA 2 2.7 No
9 M I 14 F OD No FF 0.4 NA 1 2.6 No
9 M I 14 F OS No FF 0.48 NA 2 2.6 No
10 M I 9 F OD No FF 0.3 NA 2 3.2 No
10 M I 9 F OS No FF 0.3 NA 2 3.2 No
11 M I 16 M OD No FF FC but UCVC NA 2 2.5 No
11 M I 16 M OS No FF FC but UCVC NA 2 2.5 No
12 M I 15 F OD No FF FC but UCVC NA 1 2.5 No
12 M I 15 F OS No FF FC but UCVC NA 1 2.5 No
2 Y C 1 F OD FF but UCVC 0.88 NA −1 8.5 Dislocation and converted to PK
2 Y C 1 F OS FF but UCVC 0.4 45 −1 8.3 Dislocation and Reposition
3 Y C 3 M OD HM but UCVC 0.3 53 −1 6.8 Dislocation and Reposition
3 Y C 3 M OS HM but UCVC 0.3 22 1 6.8 No
4 Y C 5 F OD FC 0.48 30 2 4.1 No
4 Y C 5 F OS FC 0.48 21 2 4.1 No
4 Y C 7 M OD HM 0.7 NA 1 3.3 No
4 Y C 7 M OS HM 0.48 NA 1 3.3 No
6 Y C 11 F OS 1 0.48 25 1 2.7 No
6 Y C 13 F OD 0.88 0.48 NA 2 2.6 No
6 Y C 13 F OS 0.78 0.4 NA 2 2.6 No
7 Y C 2 M OD 0.88 0.4 34 0 7.7 No
7 Y C 2 M OS 0.78 0.4 25 1 7.5 No
8 Y C 4 M OD 1 0.48 40 2 5 No
8 Y C 4 M OS 1 0.78 28 2 5 No
12 Y C 10 M OD 1 0.7 32 NA 2.9 No
12 Y C 10 M OS 1 0.6 24 NA 2.8 No
13 Y C 8 M OD 1.5 1 28 2 3.2 No
13 Y C 8 M OS 1.5 0.88 30 2 2.8 No

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Mar 14, 2020 | Posted by in OPHTHALMOLOGY | Comments Off on Descemet Stripping Endothelial Keratoplasty in Pediatric Patients with Congenital Hereditary Endothelial Dystrophy

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