CLINICAL SCIENCES Outcomes of Pediatric Surgery at a Tertiary Care Center in Rural Southern Ethiopia

Oren Tomkins, MD, PhD; Itay Ben-Zion, MD; Daniel B. Moore, MD; Eugene E. Helveston, MD

Objective: To evaluate the etiologies, management, and (n=33), congenital -related (n=3), partially ab- outcomes of pediatric in a rural sub-Saharan Afri- sorbed cataracts (n=3), and congenital rubella infec- can setting. tions (n=2). At presentation, visual acuity ranged from 6/60 to light perception, with 13 eyes (14%) having am- Methods: A retrospective, consecutive case series of pa- bulatory vision (better than hand motion). The mean post- tients presenting to a tertiary referral center in southern operative visual acuity was significantly improved, rang- Ethiopia during a 13-month period. All patients under- ing from light perception to 6/9. Seventy-five eyes (82%) went clinical examination, were diagnosed as having cata- achieved ambulatory vision. Of the 61 eyes with an im- ract on the basis of standard clinical assessment, and im- planted intraocular , 56 (92%) reached ambulatory mediately underwent surgical management. Visual acuity visual acuity following surgery. This was significantly results were grossly divided into ambulatory and non- greater than preoperative visual acuity results (PϽ.001). ambulatory vision according to patient age and coopera- tion. Conclusions: The underlying cause and management of pediatric cataracts in the developing world can differ sig- Results: Ninety-one eyes of 73 consecutive patients (57 nificantly from that commonly reported in the litera- boys and 16 girls) were included in the study. The mean ture. The effects of appropriate intervention on both vi- (SEM) age at diagnosis was 7.1(0.5) years (range, 0.5-15 sual outcome and associated survival statistics may be years). Fifty-five patients had unilateral cataract and 18 profound. had bilateral cataract. Cataracts were categorized accord- ing to the etiologic cause: congenital (n=50), traumatic Arch Ophthalmol. 2011;129(10):1293-1297

HERE ARE AN ESTIMATED 1.4 ficiency and measles should decrease, and million blind children cataract will likely become even more worldwide, with roughly prominent as a cause of blindness. 320 000 of affected individu- Cataract extraction has become the most als residing in sub-Saharan frequent pediatric intraocular surgery per- Africa.T1 There is wide regional and socio- formed in the United States. Advances in economic variation in the etiology of child- pediatric cataract surgery instrumenta- hood blindness, but it is estimated that tion and technique have led to a signifi- worldwide 200 000 children are blind from cant decrease in complication rates.11,12 cataract, with an additional 20 000 to 40 000 Conversely, in developing countries, the born each year with .2 A delayed time to treatment as well as lim- VISION 2020 report3 estimated that 133 000 ited resources result in a poor outcome in cataract-blind children live in developing many cases.13 A significant number of chil- countries. The prevalence of cataract as a dren remain blind because of deprivation Author Affiliations: cause for severe and , surgical complications, and Department of , blindness may reach more than 30% of cases limited rehabilitation.4,14 Bnai Zion Medical Center, in the developing world,3-9 and it is con- With a limited number of reported hos- Haifa, Israel (Drs Tomkins and sidered one of the most common causes of pital studies demonstrating surgical out- Ben-Zion); ORBIS International, avoidable and treatable blindness, follow- comes in pediatric cataract patients in New York, New York ing vitamin A deficiency, measles, and cor- Africa,15,16 we present a retrospective study (Drs Ben-Zion and Helveston); 1,10 and Department of neal scarring. With implemention of ba- of the outcome of consecutive pediatric Ophthalmology, University of sic preventative health care measures, such cataract operations, performed by a single Washington School of as immunization and nutritional supple- surgeon (I.B.-Z.), during a 13-month pe- Medicine, Seattle (Dr Moore). mentation, the prevalence of vitamin A de- riod at a tertiary care center in Ethiopia.

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©2011 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/25/2021 METHODS A B

A retrospective study of all consecutive pediatric patients, younger than 15 years, who were diagnosed as having unilat- eral or bilateral cataract from July 31, 2007, through August 1, 2008, in rural southern Ethiopia was conducted at a single cen- ter at Hawassa University School of Medicine. The hospital is a referral center for an estimated population of 15 million people, with nearly half being children younger than 15 years. This work was part of the ORBIS International and Cyber-Sight project implementing pediatric ophthalmology services in Ethio- pia. The work was approved by the Hawassa University School C of Medicine ethics review board. All patients were diagnosed as having cataract on the basis of standard clinical assessment and criteria. Before surgery, written informed consent was given for each patient by an accompanying guardian. All patients un- derwent a full clinical eye examination of the anterior and pos- terior segments. When clinically possible, intraocular lens (IOL) power was estimated according to biometric measurements (axial D length and keratometry) based on the SRKII formula. In cases when it was not possible to obtain such measurements, fellow eye refraction was used to help determine the required IOL. Younger children underwent evaluation under anesthesia fol- lowed immediately by surgical treatment, whereas older chil- dren (Ͼ7 years) underwent a local anesthesia (LA) trial, and Figure 1. Late presentation of pediatric cataract. A, Traumatic cataract. when successful, surgery was performed under LA. Visual acu- B, Anterior dislocation of lens. Bilateral congenital cataract before (C) and after (D) surgery. ity (VA) was measured or estimated, depending on the pa- tient’s age and level of cooperation, with the aid of a translator for the tribal language. To facilitate comparing VA results among capsular cataract extraction or cataract aspiration was per- children of different ages, verbal skills, and cultural back- formed, and in 6 eyes, an intracapsular cataract extrac- ground, the VA results were recorded into 2 main categories: tion was performed. An IOL was implanted in 61 eyes (60 (1) ambulatory—VA at this level results in better than hand posterior chamber IOLs and 1 anterior chamber IOL; mean motion vision (including the ability to follow an object, fixate [range] power, 23 [17-27] diopters). Anterior vitrectomy and follow an object, and finger counting or better), and (2) was performed in 44 cases, 5 eyes underwent synechioly- nonambulatory VA—this was limited to hand motion or light sis, and in 4 cases a corneal or scleral tear was repaired. perception. Statistical analysis was performed using SPSS, version 13 (SPSS Following surgery, several complications were noted: Inc, Chicago, Illinois). All results are presented as mean SEM. 1 eye with a traumatic cataract developed IOL dislocation that required IOL repositioning, 2 eyes had secondary vi- sual axis opacities and underwent anterior vitrectomy, 1 RESULTS eye developed a significant anterior chamber reaction with a fibrinous membrane covering the IOL requiring ante- Ninety-one eyes of 73 consecutive patients (57 boys and rior vitrectomy and membrane peel, 3 eyes had a tran- 16 girls) were included in the study. The mean age at di- sient raise in intraocular pressure requiring topical medi- agnosis was 7.1(0.5) years (range, 0.5-15 years). Fifty- cation, and 2 eyes had choroidal effusions that were five patients had unilateral cataract and 18 had bilateral managed conservatively (Table). There was no statisti- cataract. cally significant difference in the complications rate be- Cataracts were categorized according to the etiologic tween patients who underwent surgery under general an- cause: congenital (n=50), traumatic (n=33), congenital esthesia and those who did so under LA. No cases of retinal glaucoma-related (n=3), partially absorbed cataracts (n=3), detachment or were noted during the fol- and congenital rubella infections (n=2) (Figure 1). Pa- low-up period. tients presenting subsequent to trauma all had unilateral Mean follow-up time was 7.8(0.4) months (range, 2-13 cataract and were generally older than those presenting months), and all patients attended at least 3 follow-up ex- with idiopathic cataract (8.4[0.7] vs 6.1[0.7] years; P=.04, aminations (1 day, 1 week, and 1 month after the opera- t test). tion). The mean postsurgical uncorrected VA was signifi- At presentation, uncorrected VA ranged from 6/60 to cantly improved, ranging from light perception to 6/9 light perception, with 13 eyes (14%) having ambulatory (Figure 2B). Seventy-five eyes (82%) achieved ambula- vision (better than hand motion) (Figure 2A). Of the tory vision (Figure 2C). Of the 61 eyes with an implanted 18 patients with bilateral cataract, 12 had on IOL, 56 (92%) reached ambulatory VA following surgery presentation. (Figure 2C, inset). This was significantly greater than pre- All patients underwent cataract extraction surgery. Fifty- surgical VA results (PϽ.001, ␹2 test). This improvement five operations were performed under general anesthesia was maintained also among the bilateral cataract subpopu- and 36 with LA. Patients who had operations under LA lation (PϽ.001, ␹2 test). There was no significant differ- were older than those who had general anesthesia (10.1[0.6] ence in VA results between unilateral congenital and trau- vs 4.9[0.5] years; PϽ.001, t test). In 85 eyes, an extra- matic cases. Among verbal bilateral cataract patients

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©2011 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/25/2021 A 65 60 55 50 45 40 35 30

No. of Eyes 25 20 15 10 5 0 6/9 6/12 6/15 6/18 6/24 6/30 6/60 6/120 F+F 6M 4M 3M 2M 1M FC HM LP LPI NLP Visual Acuity

B 14 13 12 11 10 9 8 7 6 No. of Eyes 5 4 3 2 1 0 6/9 6/12 6/15 6/18 6/24 6/30 6/60 6/120 F+F 6M 4M 3M 2M 1M FC HM LP LPI NLP Visual Acuity

C 150 ∗ Ambulatory 60 Nonambulatory

100 30 No. of Eyes

No. of Eyes 0 50 IOL No IOL

0 Before Surgery After Surgery

Figure 2. Visual acuity results for patients before (A) and after (B) surgery. The dashed line divides the results into ambulatory and nonambulatory categories. C, Following surgery, significantly more children had ambulatory vision than before surgery. This was most pronounced among patients with an implanted intraocular lens (IOL) (inset). FϩF indicates fixates and follows; FC, finger count; HM, hand motions; LP, light perception; M, meter; NLP, no light perception.

(15 patients), those diagnosed preoperatively with nys- cataract surgery, with 3 findings: (1) cataract etiology was tagmus (n=12) had reduced VA results compared with the primarily congenital, followed by traumatic; (2) following VA results of patients without nystagmus (PϽ.001, Mann- cataract surgery, ambulatory vision improved signifi- Whitney rank sum test). No relationship was found be- cantly, especially if an IOL was implanted; and (3) among tween final VA and patient sex, age at presentation, or type bilateral cataract patients, VA results were better for chil- of anesthesia. dren who presented preoperatively with no nystagmus. In the developed world, the prevalence of pediatric cata- COMMENT ract ranges from 1 to 15 cases per 10 000 children.2 Most cases are idiopathic, followed by hereditary and intrauter- In this study, we identified and managed operated cases of ine causes,17 with trauma responsible for 10% to 29% of pediatric cataract recruited from patients presenting to a cases.18,19 As such, in the developed world, most child- rural tertiary center in south Ethiopia during a 13-month hood cataracts are diagnosed immediately following birth period. We followed up 91 eyes of 73 patients following or even prenatally. In the developing world and espe-

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©2011 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/25/2021 When treating pediatric cataract, the need for early Table. Complications Among 91 Patients Following Surgery surgery is influenced by the desire to minimize the oc- currence of amblyopia and severe visual impairment. In No. of congenital cases, it has been suggested that operating by Complication, by Type of Anesthesia Patients 6 weeks of age is needed to ensure minimal residual am- General blyopia.23 Because time to medical diagnosis and treat- Intraocular lens dislocation 1 ment in developing countries may be significantly de- Visual axis opacity 1 Elevated intraocular pressure 2 layed, cataract remains a major debilitating cause resulting 24 Choroidal effusion 1 in lasting amblyopia and severe visual impairment. Con- Local sequently, an accurate evaluation of VA is of great im- Anterior reaction 1 portance, yet testing such a diverse patient population, Visual axis opacity 1 including many who are nonverbal because of age or cul- Elevated intraocular pressure 1 tural or linguistic difficulties, remains a major technical Choroidal effusion 1 obstacle. Therefore, although every effort was made to achieve the best VA, the reality is that even these rela- tively low levels of visual improvement may be suffi- cially in rural areas where the availability of medical care cient, because the basic day-to-day needs of people in such is scant, identifying and treating perinatal conditions may regions can be reduced to their ability to be indepen- be extremely delayed. In these regions, blind children are dent or their need to rely on constant assistance. Fur- less likely to survive, and many cases may never reach medi- thermore, this may be directly related to their survival, cal attention. In our study, 36% of cataracts were caused because approximately 30% of blind children in Africa by trauma (8 girls and 25 boys). This high percentage re- are not expected to reach the age of 10.25 This led us to flects the large share of avoidable causes of cataract in de- explore alternative methods for determining effective VA veloping countries, such as trauma and intraocular infec- results. All VA results were divided into 2 main catego- tion, as well as the relatively late age at presentation (Ͼ7 ries, as previously described26: ambulatory and nonam- years). A substantial percentage of our patients also came bulatory. Whereas ambulatory VA patients may be re- to us with evidence of local traditional therapy ap- garded as grossly independent, nonambulatory patients proaches manifesting in temporal scarring (data not require constant assistance from a second person in their shown), which emphasizes that the only treatment avail- everyday activities. Following cataract surgery, espe- able had been sought, although producing poor results. cially if an IOL is implanted, VA may increase signifi- Such findings stress that pediatric cataract cases seen in cantly, reducing the socioeconomic burden of such chil- developing countries may not reflect the same causes as dren and increasing their own chance of survival. Among those found in the developed world. Addressing prevent- our patients, we found results affirming this view, with able causes and increasing the availability of medical care the percentage of patients with ambulatory VA increas- for pediatric cases may change the distribution of cata- ing from 14% to 82% (92% among IOL-implanted eyes). ract causes in such countries. This division, though crude, may create a clearer repre- Operating on patients in rural settings with limited medi- sentation with regard to patients’ daily activity and sur- cal facilities and support leads to differences in how these vival. Although most cases showed improvement of VA, cataracts are managed compared with accepted clinical ap- traumatic cataracts presented the smallest change and the proaches in the developed world. Although common prac- greatest improvement, with some cases advancing from tice in surgically managed pediatric cataract may be per- light perception to as good as 6/9 (data not shown). Be- forming operations under general anesthesia, such cause trauma is generally related to older children, it bears conditions are not always available in remote rural set- a smaller effect on amblyopia than do congenital cases tings. Although LA can be considered for any child under and may result in a better outcome. On the other hand, such conditions, it is best restricted to older patients. In the damage caused by trauma may be uncorrectable, lead- our cohort, 36 eyes were operated on under LA (53% trau- ing to little or no improvement. The large percentage of matic and 47% congenital). The mean age for patients un- traumatic cataracts among our patients stresses their part dergoing LA was 10 years, with the lowest age being 6 years. in , specifically in regions where chil- Visual acuity results and complication rates of these pa- dren are more exposed to injury.27 tients were comparable with those of patients operated on Among our cohort of patients, 18 had bilateral cata- under general anesthesia. Local anesthesia combined with ract and 12 of them had nystagmus. Preoperative nys- sedation is a common practice in certain regions20 and tagmus may also be considered an indicator of poor VA should be regarded as a valid option in appropriate cir- results in children with bilateral cataract.28-30 The VA re- cumstances. One must also consider the increased risk and sults of these children were significantly worse than those complexity related to general anesthesia21 and the pos- of children with no nystagmus, emphasizing the need for sible lack of adequate resources to support and to treat a expedited surgery in bilateral cases. substantial number of patients in some settings. This is es- A key drawback to our study is its retrospective na- pecially relevant to rural medical centers where anesthe- ture, as well as the limited time for follow-up: the mean sia is often delivered not by specialist physicians but by was 7.4 months and the longest was 12 months. Al- anesthesia technicians.22 Our results support the view that though VA results may be determined within several weeks in areas of limited medical resources, cataract operations of the operation, long-term results and specifically the effect under LA may be considered for older children. of surgery on amblyopia remain to be explored.

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©2011 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/25/2021 In this study, we explored the causes of cataract in a 10. Kello AB, Gilbert C. Causes of severe visual impairment and blindness in children rural region of a developing country and described sur- in schools for the blind in Ethiopia. Br J Ophthalmol. 2003;87(5):526-530. 11. Francis PJ, Berry V, Bhattacharya SS, Moore AT. The genetics of childhood cataract. gical approaches and results. Although late presenta- J Med Genet. 2000;37(7):481-488. tion to medical assistance affects surgical success, treat- 12. Moore DB, Ben Zion I, Neely DE, et al. Accuracy of biometry in pediatric cataract ing such patients may still achieve a change in their ability extraction with primary intraocular lens implantation. J Cataract Refract Surg. to perform activities of daily living and may lighten the 2008;34(11):1940-1947. burden on their local community. The limited re- 13. Mwende J, Bronsard A, Mosha M, Bowman R, Geneau R, Courtright P. Delay in presentation to hospital for surgery for congenital and developmental cataract sources available require physicians to regard treatment in Tanzania. Br J Ophthalmol. 2005;89(11):1478-1482. options appropriate to local life and needs. As in previ- 14. Kishiki E, Shirima S, Lewallen S, Courtright P. Improving postoperative fol- ous works, our study supports the need for patient edu- low-up of children receiving surgery for congenital or developmental cataracts cation and increasing local medical resources to change in Africa. J AAPOS. 2009;13(3):280-282. the course of avoidable pediatric blindness. 15. Yorston D, Wood M, Foster A. Results of cataract surgery in young children in east Africa. Br J Ophthalmol. 2001;85(3):267-271. 16. Bowman RJ, Kabiru J, Negretti G, Wood ML. Outcomes of bilateral cataract sur- Submitted for Publication: June 24, 2010; final revi- gery in Tanzanian children. 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