Comparative analysis of the results of surgical treatment for Peters anomaly and sclerocornea in young children

Purpose: to study and compare the results of primary penetrating keratoplasty (PKP) in young children with Peters’ anomaly and sclerocornea. Material and methods. The survival rates of the graft after primary PKP were studied in 58 children (67 eyes) under 5 years of age with congenital corneal opacity in Peters’ anomaly (43 patients) and sclerocornea (15 patients). The biological result of the surgery was assessed by the Kaplan — Meier method, which was used to determine the “survival” of the graft. The functional result (taking into account the child’s age) was estimated approximately: by the child's tracking of toys from a certain distance and by the preferred gaze method. The corneal discs removed during the surgery were subjected to histological examination. Results. The overall survival rate of the grafts was 68%. Ultimately, 2 years after surgery, graft survival in children with Peters anomaly was 68%, while in children with sclerocornea it was only 20%; after 5 years, it was 56% and 7%, respectively. The mean survival time and survival rate differed significantly in patients with Peters anomaly and in patients with sclerocornea: survival time 125.3 ± 13.9 versus 36.4 ± 15.1 months (p = 0.014), survival — 82.5% versus 20.0% (p = 0.02). The presence of vascularization in the limbus and on the periphery of the cornea, as well as the diameter of the recipient’s cornea, significantly correlated with the development of graft rejection. Conclusion. After PKP, high graft survival was observed in patients aged 5 years and younger with Peters anomaly, whereas graft survival was low in patients with sclerocornea.

1. Kotran PR, Pozhar AM. Congenital corneal opacities. International Ophthalmology Clinic. 1992; 32 (1): 93–105. doi: 10.1097/00004397-199203210-00010

2. Katsman LR, Reiser BJ. Pediatric Corneal Opacities. 2016. Available from: https://www.aao.org/disease-review/pediatric-corneal-opacities.

3. Sonksen PM, Dale N. Visual impairment in infancy: influence on the development of the nervous system and neurobiological processes. Dev Med Child Neurol. 2002 Nov; 44 (11): 782–91. doi: 10.1017/s0012162201002936

4. Arffa RC. Congenital anomalies. Grayson's corneal diseases. In: Arffa RC, ed. St. Louis: Mosby; 1997.

5. Rapuano SJ, Laibson PR, Cohen EJ. Congenital corneal opacities in the practice of corneal direction. Сornea. 2004, August; 23 (6): 565–70. doi: 10.1097/01.ico.0000126317.90271.d8

6. Bermejo E, Martinez-Frias ML. Congenital malformations of the eyes: clinical and epidemiological analysis of 1,124,654 consecutive births in Spain. Am J Med Genet. 1998 Feb 17; 75 (5): 497–504. PMID: 9489793.

7. Shi W, Jin H, Li S, Liu M, Xie L. Indications for pediatric keratoplasty in northern China. Clin Exp Ophthalmol. 2007 November; 35 (8): 724–7. doi: 10.1111/j.1442-9071.2007.01618

8. Zhang Y, Liu Y, Liang Q, et al. Indications and results of end-to-end keratoplasty in newborns and children, Beijing, China. Сornea. 2018 October; 37 (10): 1243–8. doi: 10.1097/ICO.0000000000001695

9. Dale NJ, Tadic V, Sonksen P. Social communicative variations in 1-3-year-old children with severe visual impairments. Child Care Health Dev. 2014 March; 40 (2): 158–64. doi: 10.1111/cch.12065

10. Matafzi A, Islam L, Kelberman D, Snowden JK, Nischal KK. Chromosomal abnormalities and genetics of congenital corneal opacities. Mol Vis. 2011; 17: 1624–40. PMID: 21738392.

11. Maillette de Buy Wenniger-Prick LJ, Hennekam RC. Plus Peters syndrome: an overview. Ann Jeune. 2002, April-June; 45 (2): 97–103. doi: 10.1016/s0003-3995(02)01120-6

12. Zaidman GW, Flanagan JK, Furey CC. Long-term vision prognosis in children after corneal transplant surgery for Peters type I anomaly. Am J Ophthalmol. 2007 July; 144 (1): 104–8. doi: 10.1016/j.ajo.2007.03.058

13. Nischal KK, Set J, Jay V, Makin LD, Rutman DS. Clinical and pathological correlation of congenital corneal opacity using ultrasound biomicroscopy. Br J Ophthalmol. 2002, January; 86 (1): 62–9. doi: 10.1136/bjo.86.1.62

14. Shigeyasu C, Yamada M, Mizuno Y, et al. Clinical signs of anterior segment dysgenesis associated with congenital corneal opacities. Сornea. 2012 March; 31 (3): 293–8. doi: 10.1097/ICO.0b013e31820cd2ab

15. Ozerki H, Shirai S, Nozaki M, et al. Ocular and systemic signs of Peters' anomaly. Graefes Arch Clin Exp Ophthalmol. 2000, October; 238 (10): 833–9. doi: 10.1007/s004170000177

16. Townsend VM. Congenital anomalies of the cornea. In: Kaufman HE, Baron BA, MacDonald MB, eds. Сornea. London: Butterworth-Heinemann; 1999.

17. Nishal KK. Genetics of congenital corneal opacification--impact on diagnosis and treatment. Cornea. 2015 Oct;34 Suppl 10: S24-34. doi: 10.1097/ICO.0000000000000552

18. Dana MR, Shaumberg DA, Moshes AL, Gomez JA. Corneal transplantation in children with Peters anomaly and mesenchymal dysgenesis. Multicenter study of pediatric keratoplasty. Ophthalmology. 1997, October; 104 (10): 1580–6. doi: 10.1016/s0161-6420(97)30093-1