Indications for Pediatric Ocular Prosthesis Fitting at a Referral Center in the Middle East

Abstract

Trauma is the major reason for globe loss in adults; however, there are less data regarding the causes for globe loss in children. We reviewed the underlying diagnoses of children who underwent ocular prosthesis fitting over a 1-year period at a referral eye hospital in the Middle East and found retinoblastoma, trauma, and congenital microphthalmia or anophthalmia to be the most common diagnoses, respectively. Enucleation and evisceration were the most common procedures and were exclusively performed for retinoblastoma and trauma, respectively. Ocular morbidity from the most common diagnoses related to pediatric globe loss in the region could be decreased by improved family education, safety precautions, and genetic counseling.

Introduction

The aim of an ocular prosthesis (artificial eye) is to reconstruct a near-normal appearance for a patient with a disfigured globe or anophthalmic socket. A disfigured globe can be a congenital malformation or the result of trauma, infection, or other severe ocular diseases. An anophthalmic socket can be congenital or the result of surgery for globe disfigurement or surgery for ocular cancer. Trauma is the major reason for globe loss in adults; however, there are less data regarding causes for globe loss in children. To investigate this, we reviewed the underlying diagnoses of children who underwent ocular prosthesis fitting over a 1-year period at a referral eye hospital in the Middle East.

Methods

Institutional Board Approval was granted for this retrospective study. The medical records of all children (12 years old or younger) treated at the ocular anaplastology clinic of King Khaled Eye Specialist Hospital in Riyadh, Saudi Arabia during the year 2014 were reviewed.

Results

A total of 199 patients (104 males, 95 females; 215 eyes) were identified. All were Saudi Arabian. The age range of patients was 3 days–9.33 years (mean 33.31 months; median 15.03 months; and standard deviation 23.99). Custom-made ocular prosthesis was fitted for 199 eyes: in the right eye only in 77 (38.69%) cases, in the left eye only in 106 (53.27%), and bilaterally in 16 (8.04%).

The indications for ocular prosthesis fitting (referring to number of eyes) were as follows: 88/199 (44.22%) retinoblastoma (five of which were bilateral), 38/199 (19.10%) trauma, 38/199 (19.10%) microphthalmia, 11/199 (5.53%) anophthalmia, four endophthalmitis, three congenital glaucoma, one Coats' disease, and several other indications [Table 1]. In the trauma group, the main types of injury were sharp object perforating injury, burst injury, traffic accidents, gunshots, and falls.

Table 1.

Breakdown of “Others” category

Diagnosis	n
Persistent fetal vasculature	6
Peters anomaly	4
Retinopathy of prematurity	4
Medulloepithelioma	2
Optic nerve coloboma	1
Sturge Weber syndrome and secondary glaucoma	1
Anterior segment dysgenesis	1
Total	19

The eye had been removed surgically by enucleation or evisceration for 117 patients (124 eyes). All enucleations (104 patients) were for retinoblastoma, and all eviscerations (13) were following trauma. There was a male predominance in cases due to trauma with 68.42% males and 31.58% females, and in cases due to retinoblastoma there were 58.89% males and 41.11% females.

Discussion

Most major published studies that address epidemiology for globe loss report results for almost exclusively adult populations[1,2,3,4] with the top three indications being trauma, tumors (nonretinoblastoma), and infections, respectively. In this study, we reviewed indications for ocular prosthesis fitting in children at a referral center in the Middle East to gain a better understanding of reasons for globe loss in children in the region. Retinoblastoma, trauma, and congenital microphthalmia or anophthalmia were the most common diagnoses. Enucleation and evisceration were the most common procedures and were exclusively performed for retinoblastoma and trauma, respectively.

There are two studies of globe loss from the LV Prasad Institute in India with results that included a substantial number of children[5,6] and one study from the Shandong Eye Institute in China that focused on reasons for pediatric enucleations.[7] In a retrospective 3-year study (1995–1998) of enucleations at the LV Prasad Institute, Vemuganti et al.[5] reported that 85% of their 150 cases (151 eyes) that underwent enucleation were children under 15 years old. Major indications in the cohort were tumor (mostly retinoblastoma) in 74 (49%), staphyloma in 38 (25%), and acute injury in 20 (13%). In a retrospective 3-year study (2002–2005) of children 16 years or younger who underwent ocular prosthetic fitting at the same center, Raizada et al.[6] reported the patient diagnoses of retinoblastoma (136/222), anterior staphyloma (24/222), and ocular inflammation (19/222) to be the major reasons, respectively, for enucleation or evisceration. For disfigured globe, the top three causes were posttrauma (53/71), ocular inflammation (10/71), and postsurgery (vitreoretinal or cataract) (7/71). In a retrospective review (2001–2015) of reasons for enucleation in children under 14 years old at a referral center in Northern China, Zhang et al.[7] noted trauma (37/71), retinoblastoma (16/71), and congenital abnormality (12/71) to be the major diagnoses. The authors also noted that over the period studied the rate of pediatric enucleations had decreased.

In the current study of Saudi Arabian patients, retinoblastoma was the number one reason for pediatric globe loss. This reflects the later presentation and management of retinoblastoma that occurs in less-developed countries, often related to lack of awareness,[8] and is in agreement with results from the two studies from the LV Prasad Institute.[5,6] A campaign to improve family education is a cost-effective strategy that could decrease the enucleation burden from retinoblastoma.[9] This has been successful in other areas of the world that formerly had a high incidence of extraocular presentations.[8]

Trauma was the second-most common cause for pediatric globe loss, and it is consistently a major cause for globe loss in adults or children in most series from worldwide.[1,2,3,4,5,6,7] Public health policies are effective in decreasing pediatric globe loss from trauma,[10] and thus such polices are important to implement. Examples include restrictions on the sale of certain consumer products, mandatory vehicle seat belts, laminated windscreens in vehicles, and public education.[10]

The third-most common diagnosis associated with pediatric globe loss in our population was microphthalmia/anophthalmia, and this is the only of our top three diagnoses not in the top three diagnoses of either of the two studies from the LV Prasad Institute.[5,6] Congenital abnormality was considered the third-most common diagnosis in the study from China,[7] but the absolute number of cases over the study was low (12 cases in that 15-year study in contrast to over 50 cases in the current 1-year study). The relatively large number of microphthalmia/anophthalmia cases in this region is likely related to the common practice of endogamy, consanguinity, and large family size that enhances the expression of otherwise rare recessive diseases.[11] Genetic counseling would be expected to decrease this burden.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.