Acute Corneal Hydrops in Children with Primary Infantile Glaucoma: A Report of 31 Cases over 23 Years at the LVPEI

June Acute Corneal Hydrops in Children with Primary Infantile Glaucoma: A Report of 31 Cases over 23 Years at the LVPEI Anil K. Mandal 0 1 0 Editor: James Fielding Hejtmancik, National Eye Institute , UNITED STATES 1 Jasti V Ramanamma Children's Eye Care Centre, L V Prasad Eye Institute , Hyderabad , India - OPEN ACCESS Data Availability Statement: Institutional Review Board (IRB) restrictions make data unsuitable for public deposition. An anonymized data set will however be made available for researchers who meet the criteria for access to confidential data upon request to the corresponding author or IRB. Funding: This study was supported by Hyderabad Eye Research Foundation. The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript. Competing Interests: The authors have declared that no competing interests exist. Purpose Methods Relatively little data exist regarding the outcomes of children with primary infantile glaucoma presenting with acute corneal hydrops. The aim of our study was to determine the surgical outcome of children of infantile glaucoma who presented with acute corneal hydrops. In total, 38 eyes of 31 consecutive children of infantile glaucoma presented with acute corneal hydrops who underwent primary combined trabeculotomy-trabeculectomy (CTT) by a single surgeon from January 1990 to December 2012 at the LV Prasad Eye Institute (LVPEI), a tertiary eye care centre in Southern India were enrolled in this retrospective study. Primary outcome measures were intraocular pressure (IOP) control (IOP 16 mmHg under anaesthesia or IOP 21 mmHg without anaesthesia) and clearance of corneal edema. Secondary outcome measures were visual acuity (VA), corneal diameter, bleb appearance, intraoperative and postoperative complications. Results Mean age at presentation was 6.4 months (range, 2–11 months) and seven eyes (23%) had bilateral affliction. At presentation, all eyes (100%) had moderate to severe degree of corneal edema with a mean preoperative IOP of 25.6 ±5.1 mmHg. Postoperatively, the IOP reduced to 12.0 ± 3.8 mmHg (difference = -13.6, 95% CI = -15.7 to -11.5, t = -13.18, p<0.0001), and the percentage reduction in IOP was 53.05%. Preoperatively 83% of the eyes were on antiglaucoma medication, and postoperatively 2 eyes (5.3%) required 1 antiglaucoma medication for control of IOP. Preoperatively, corneal edema was present in all eyes and postoperatively it cleared in all of them. Significant myopic astigmatism was present in 28 eyes (74%), the commonest being compound myopic astigmatism (75%) followed by simple myopic astigmatism (21%). Normal VA (best-corrected VA; BCVA 20/60) was achieved in 44.4% of the eyes and 22.2% eyes had low vision (BCVA, <20/60 to 20/400). Complete success (IOP control and clearance of corneal oedema) was obtained in 94.7% eyes. There were no significant intraoperative or postoperative complications. Two thirds of the patients showed low, elevated functional filtering bleb. No patient had any bleb leak, blebitis or bleb related endophthalmitis. The median follow-up was 36 months (range 2–228 months). Conclusions Primary CTT is safe and effective in controlling IOP, resulting in complete clearance of corneal edema with modest visual improvement in children of infantile glaucoma presenting with acute corneal hydrops. The outcome of the study will have a positive impact on counseling the parents preoperatively. Introduction Infantile glaucoma is a form of developmental glaucoma that manifest after one month of birth until 3 years of age.[ 1 ] It manifests with megalocornea associated with increase in intraocular pressure (IOP) but it may remain undetected as the parents believe that the megalocornea may be a manifestation of “beautiful eye”. Consequently, parents of such children may not seek needful attention for medical advice. However some of the children with infantile glaucoma may go onto develop sudden corneal clouding because of acute corneal hydrops.[ 2–4 ] This is a medical emergency that prompts the parents to seek consultation with an ophthalmologist or a pediatrician. A detailed ophthalmic evaluation is mandatory to rule out the other causes of acute corneal hydrops. Once the diagnosis is confirmed, early surgical intervention is required to control the IOP. Although there are various surgical options available in the armamentarium of glaucoma surgeons, goniotomy is technically difficult because of corneal edema.[ 5–7 ] External trabeculotomy is a suitable alternative as it is possible to perform this procedure even with severe degrees of corneal edema [ 8–11 ]; however, the long-term results of trabeculotomy are not encouraging in Indian patient population.[ 12, 13 ] Another surgical option in the form of primary combined trabeculotomy–trabeculectomy (CTT) is the best alternative as has been reported in various ethnic populations from different parts of the world.[ (...truncated)