Pediatric Penetrating Keratoplasty: Indications and Outcomes R. Limaiem, A. Chebil, A. Baba, N. Ben Youssef, F. Mghaieth, and L.El Matri ABSTRACT Purpose. This report evaluates patient characteristics, indications, and outcomes of pediatric keratoplasty, and identifies variables that help to predict poor surgical outcomes. Methods. We undertook a retrospective review of all cases in our department of primary penetrating keratoplasty performed in children 14 years of age or younger between January 2003 and December 2009. Results. Sixteen primary penetrating keratoplasties were performed during the study interval. Mean age was 11.2 years (3 to 14 years) and the gender ratio was 2. The mean duration of follow-up was 16 months (2 to 36 months).The surgical indications were acquired traumatic opacities in 6 cases, keratoconus in 5 cases, corneal perforation secondary to infectious keratitis in 3 cases, hereditary corneal dystrophy in 1 case, and acquired non-traumatic opacities secondary to congenital glaucoma in one case. The initial visual acuity was less than 1/20 in 68% of cases and the mean final visual acuity after 1 year was 2/10. The graft was clear in 52% of cases after 1 year of follow-up. Postoperative complications were graft failure (24%), ocular inflammation (5%), and ocular trauma (19%). Conclusion. Penetrating keratoplasty in children has been documented to have a higher rate of graft failure and a worse visual prognosis than adult keratoplasty. Poor prognosis outcomes were especially caused by noncooperation of parents and postoperative ocular trauma. ENETRATING keratoplasty in children is characterized by the frequency of graft rejection and poor visual prognosis. It poses several problems: assessment of injury, scleral rigidity, and vitreous surge. The results of the transplant also depend on postoperative monitoring (dropping points, infection, hypertension, and amblyopia). The aim of our study is to evaluate patient characteristics, indications, and outcomes of pediatric keratoplasty, and to identify variables that may help predict poor surgical outcomes.
P
Table 1. Indications for Pediatric Penetrating Keratoplasty Indications
%
Traumatic opacity Keratoconus Abces drilled Herpetic keratitis Hereditary corneal dystrophy Corneal opacity secondary to congenital glaucoma
38 31 13 6 6 6
RESULTS METHODS In this retrospective study, the records of 16 cases of pediatric penetrating keratoplasty (15 patients) were reviewed between January 2003 and December 2008. The patients underwent surgery in Hedi Rais Institute of Ophthalmology (Department B). By reviewing the patients’ medical records, history, clinical examination results, clinical and pathological diagnoses, and outcomes of keratoplasty of each patient was gathered. Follow-up data were collected until 3 years postoperatively (mean follow-up time was 16 months; range, 2 to 36 months). The SPSS13.0 software package was used to analyze the data. The level of statistical significance was P ⬍ 0.05. © 2011 by Elsevier Inc. All rights reserved. 360 Park Avenue South, New York, NY 10010-1710 Transplantation Proceedings, 43, 649 – 651 (2011)
A total of 16 eyes from 15 pediatric patients up to 14 years of age underwent penetrating keratoplasty and were enrolled in this 5-year study. The median age at surgery was 11.2 years. Patients’ age ranged from 3 to 14 years. Both eyes were operated in one case. The gender distribution involved 67% (n ⫽ 10) boys and 33% (n ⫽ 5) girls. From the Department B of Ophthalmology, Hedi Rais Institute of Ophthalmology, Tunis, Tunisia. Address reprint requests to Ahmed Chebil, MD, Boulevard 9 avril 1006 Bab saadoun Tunis, Tunisia. E-mail: chebilahmed@ yahoo.fr 0041-1345/–see front matter doi:10.1016/j.transproceed.2011.01.055 649
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LIMAIEM, CHEBIL, BABA ET AL Table 2. Vision Outcomes of Pediatric Penetrating Keratoplasty
Patient No.
1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16
Preoperative Acuity
Postoperative Acuity
3 meters 1/10 1.5 meter 1.5 meter LP LP LP 50 cm 1/20 LP LP LP 1/20 1/20 LP 1 meter
1/20 5/10 3/10 1 meter 1/10 3/10 1 meter 5/10 1/10 LP 2 meters LP 1/10 1 meter LP 1/10
Abbreviation: LP, light perception.
The classification by Stulting et al1 was used to group the participants into nontraumatic, acquired traumatic, and congenital indications. The acquired nontraumatic group included 50% (n ⫽ 8) of patients while 37.5% (n ⫽ 6) of patients contributed to the acquired traumatic group, and the congenital group included 12.5% (n ⫽ 2) of participants. These groups were further subdivided into specific indication groups as shown in Table 1. Keratoconus (31% of the total number of cases, n ⫽ 5) was the most common indication in the acquired nontraumatic group (Table 1). In children, vision assessment can be difficult. Documentation of the changes in best-corrected visual acuity (BCVA) on an E-chart was the preferred method of assessment if the child was cooperative. If vision assessment was not possible because the child could not resist closing his/her better eye, subjective methods were used. The functional outcomes for postoperative visual acuity (VA) in this series of pediatric penetrating keratoplasties are shown in Table 2 and Fig 1. The initial VA was less than 1/20 in 68% of cases and the mean final VA after 1 year was 2/10. Vision outcome was significantly better for keratoplasty 1 year postoperatively compared to preoperative visual acuity
Fig 1. Comparison between initial and final visual acuity.
Fig 2.
A clear graft.
(P ⬍ 0.001). Of all patients, 25% (n ⫽ 4) achieved a BCVA of 3/10 or better. The graft was clear in 52% of cases (Fig 2). Graft rejection (Fig 3) was found in 31% of cases (n ⫽ 5) within an average of 12 months (1 case of congenital glaucoma, 1 case of traumatic corneal opacity, 1 case of perforated abscess, and 1 case of keratoconus). Four traumas on grafts were noted after early delays (8 days, 15 days, 2 months, and 7 months). One case presented a graft rejection. One patient had intraocular inflammation (Table 3). DISCUSSION
The cornea in children is more flexible and less rigid than in adults. Examinations before and after operation, medical treatments, and nursing procedures are all more difficult in children than in adults. Because of the anatomical and physical characteristics of a child’s sclera, the crimpling of the eyeball during operation, forward displacement of the lens and iris diaphragm, as well as synechia, often occur in pediatric keratoplasty. The majority of infants and young children are hyperopic, have a shallow anterior chamber, and a narrow and undeveloped iridocorneal angle. Furthermore, congenital corneal opacities are occasionally con-
Fig 3.
Graft rejection.
PEDIATRIC PENETRATING KERATOPLASTY
651
Table 3. Anatomic Results of Penetrating Keratoplasty Anatomic Results
%
Clear graft Rejection Trauma on graft Inflammation
52 24 19 5
comitant with a smaller eyeball and cornea.2 Therefore, iris anterior synechia and intraocular pressure elevation, both being high-risk factors of graft rejection, occur more frequently after pediatric keratoplasty. Congenital corneal opacity and corneal dystrophies are the major indications for pediatric keratoplasty in developed countries.3,4 However, opacity in keratoconus and perforation caused by trauma were the leading reasons for performing penetrating keratoplasty in children in our study. Various indications for pediatric keratoplasty have been reported.5,6,7 Table 4 provides a comparison between the present study and other several important reports published in the 21st century. All studies presented used an age criterion of 14 years or younger. The proportion of keratoplasties which was performed for congenital indications was 16% to 34%, 46% to 74% for acquired non-traumatic, and 10% to 31% for acquired traumatic indications. Here, in particular, the proportion of keratoplasties performed for acquired nontraumatic indications (50%) was lower than in other reports. However, acquired traumatic indications was much higher than that in most previously published reports. Reported clear graft rates after pediatric keratoplasty range between 50% and 60%. Stulting et al1 reported a 60% probability of obtaining a clear graft at 1 year in congenital opacities and 70% in acquired corneal opacities. In our series, 50% of eyes had clear grafts at the last follow-up visit. In the current study, there was no significant difference in graft survival rate among the groups. There is no general agreement on the issue of influence of preoperative corneal
condition on graft outcome. Our series showed that prolonged corneal graft survival can be achieved in childhood. More importantly, vision outcome after corneal transplantation was significantly better. The median visual acuity achieved in pediatric keratoplasty may range from light perception to 20/200. Nine (56%) had greater than 1/20 vision in our study, 29 of which had greater than 2/10. Poor visual outcome after pediatric keratoplasty can be attributed to amblyopia, frequent graft failures, optical distortion induced by the graft, and the associated ocular pathology. Dana et al2 reported amblyopia treatment as the only independently significant prognosticator for visual improvement after surgery. CONCLUSION
Our series shows that prolonged corneal graft survival can be achieved in childhood among patients grafted for either congenital or acquired causes of corneal opacity. Amblyopia and postoperative astigmatism were thought to be responsible for poor vision in the majority of clear grafts. Intensive amblyopic therapy may promote visual recovery. REFERENCES 1. Stulting RD, Sumers KD, Cavanagh HD, et al: Penetrating keratoplasty in children. Ophthalmology 91:1222, 1984 2. Zaidman GW, Flanagan JK, Furey CC: Long-term vision prognosis in children after corneal transplant surgery for Peters anomaly type I. Am J Ophthalmol 144:104, 2007 3. McClellan K, Lai T, Grigg J, et al: Penetrating keratoplasty in children. Br J Ophthalmol 87:1212, 2003 4. Dana MR, Moyes AL, Gomes JA, et al: The indications for and outcome in pediatric keratoplasty: a multicenter study. Ophthalmology 102:1129, 1995 5. Patel HY, Ormonde S, Brookes NH, et al: The indications and outcome of pediatric corneal transplantation in New Zealand: 1991–2003. Br J Ophthalmol 89:404, 2005 6. Aasuri MK, Garg P, Gokhle N, et al: Penetrating keratoplasty in children. Cornea 19:140, 2000 7. Sharma N, Prakash G, Titiyal JS, et al: Pediatric keratoplasty in India: indications and outcomes. Cornea 26:810, 2007
Table 4. Indications of Pediatric Penetrating Keratoplasty Reported in Literature Study 6
Auasuri et al Patel et al5 Zaidman et al2 Current study
Number of Patients
Acquired non-traumatic n (%)
Acquired Traumatic n (%)
Congenital n (%)
154 58 168 16
85 (55%) 43 (74%) 87 (52%) 8 (50%)
22 (14%) 6 (10%) 24 (14%) 6 (37.5%)
47 (31%) 9 (16%) 57 (34%) 2 (12.5%)