Orbito-oculoplastic diseases in lagos: a 4-year prospective study.

AIMS: To determine the prevalence of orbito-oculoplastic diseases and thereby contributing a data base to the emerging orbito-oculoplastics subspecialty in the sub-Saharan region. SETTINGS AND
DESIGN: A tertiary eye care centre.
MATERIALS AND METHODS: A descriptive prospective study was done in a tertiary eye care center. Demographic and clinico-pathological diagnoses of 269 patients presenting to the oculoplastics clinic over a 4-year period (January 2008 -December 2011) were collected at entry and during follow-up visits. These were patients referred from the general ophthalmology clinics of the study center, secondary eye care centers within and from neighboring states. Patients were categorized based on the primary diagnosis after examination by the oculoplastics surgeon. Ethical issues were considered though data collection did not involve direct patient participation. STATISTICAL ANALYSIS USED: Microsoft Excel 2007 software.
RESULTS: Two hundred and sixty-nine patients were seen with 141 (52.4%) females. Leading etiological factors were trauma; 81 (30.0%), congenital anomalies 55 (21.0%) and tumors 44 (16.0%). Eyelid diseases were the most frequent 115 (42.8%), with ectropion 36 (31.0%) and ptosis 33 (29.0%) being the most common. Orbital and peri-orbital lesions 44 (16.4%) were mainly dermoids 12 (27.3%) and capillary heamangioma 4 (9.1%) in the pediatric age while thyroid orbitopathy 11 (25.0%) was predominant in adults. Ocular and degenerative diseases were retinoblastoma in 4 (16.7%) and phthisis bulbi in 10 (33.3%). Contracted socket was 22 (84.0%) of socket pathologies. Primary conjunctival diseases occurred less often, rather Stevens-Johnson syndrome in 9 (30.0%) and its ocular complications were more frequent.
CONCLUSIONS: It is expected that this survey will provide a database for oculoplastics surgeons and ophthalmologists in an emerging subspecialty and thus enhance training focus and equipment acquisition.

INTRODUCTION

Orbito-oculoplastic diseases present as orbital, ocular and adnexal lesions. They may arise from congenital anomalies, involutional changes, trauma, metabolic diseases and tumors. The resulting visual dysfunction, anatomical disfigurement and cosmetically unacceptable appearance predispose to psychosocio-economic isolation and educational deprivation. Common lesions such as ectropion and entropion have been mainly a disease of the elderly.12 Large population-based studies reported (2.9-3.9%) of ectropion12 and (2.1%) entropion.1 The global prevalence and incidence of ptosis is not clearly defined due to insufficient data.3 Of all orbito-ocular tumors, retinoblastoma have been found to have a prevalence rate ranging between 38.1% and 57.3%.456 Askira7 et al. recorded 1.4% incidence. A majority of studies have focused on individual disease entity. To our knowledge there have been no studies on the prevalence of orbito-oculoplastic diseases in our environment. The purpose of this study therefore is to determine the prevalence of orbito-oculoplastic diseases in a young oculoplastics unit, over its 4-year period of existence and to provide a database in this region where oculoplastics as a sub-specialization is in its formative years.

MATERIALS AND METHODS

All patients referred from the general ophthalmology clinics of the study tertiary eye center and secondary eye care centers within and from neighboring states were included in the study. Demographic data and clinico-pathological diagnoses were collated at entry and during follow-up (prospective studies). The primary diagnosis of each patient was selected. Disease conditions were analyzed along an etiological and anatomical distribution pattern. Ethical issues were considered, though data collection did not involve patients' direct participation. Statistical analysis was done using the Microsoft Excel 2007 software.

RESULTS

Two hundred and sixty-nine (269) patients presented during the study period. They constituted 1.4% of all new patients presenting for eye care over the 4-year period. There were 141 (52.4%) females and 128 (47.6%) males at a ratio 1.1:1. The age distribution pattern [Figure 1] shows that children aged 0-9 years constituted 61 (22.7%), followed by those aged 20-29 years constituting 52 (19.3%). Least presenting were patients aged 80 years and above. The etiological distribution of diseases is as shown in Figure 2. Trauma 81 (30.0%) ranked highest; followed by congenital lesions 55 (21.0%) and tumors 44 (16.0%).

Figure 1

Age distribution

Figure 2

Aetiological pattern

Anatomical distribution of diseases

The general anatomical pattern is as shown in Table 1 with eyelid diseases 115 (42.8%) most prevalent while orbital and periorbital 44 (16.4%) lesions ranked next.

Table 1

General anatomic distribution pattern

Diseases of the eyelid

Eyelid disorders [Figure 3] constitute 115 (42.8%) of all diseases; with ectropion as the most common clinical disorder constituting 36 (31.0%) of all eyelid lesions. Ptosis was the second most common 33 (29.0%) with a large proportion being congenital in onset. The third common disorder was lid tumors 20 (17.0%).

Figure 3

Diseases of the eyelid

Lacrimal system disorders

The most frequent occurrences were epiphora in 6 (30.0%) and congenital nasolacrimal duct obstruction in 4 (20.0%). Punctal stenosis and dacryocystitis affected 3 (15.0%) each.

Diseases of the conjunctiva

Conjunctival diseases comprised 11.2%. Stevens-Johnson syndrome was most prevalent affecting 9 (30.0%). There were 4 (13.3%) cases of squamous cell carcinoma.

Ocular and degenerative diseases

Few cases of primary ocular lesions attended clinic during the study period, mainly retinoblastoma 4 (13.3%), while phthisis bulbi 10 (33.3%) was the most common degenerative ocular disease.

Orbital and peri-orbital diseases

Affected 44 (16.4%) as shown in Table 1. The majority of orbital lesions [Table 2] were dermoid cyst (orbital and peri-orbital) in 12 (27.3%) and the second most common was thyroid orbitopathy 11 (25.0%).

Table 2

Orbital and periorbital diseases

Socket disorders

Contracted socket, 22 cases (84.6%), was the most common cause of acquired socket lesion while microphthalmia and anophthalmia 2 cases (7.7%), were the congenital causes.

DISCUSSION

A variety of orbito-oculoplastics diseases presented during the study period. These diseases and deformities tend to impact negatively on the psycho-social, economic as well as the educational achievement of affected persons. Quality of life also tends to decline. The threat to vision is also enormous. It is important to note that there were slightly more females (52.4%) than males and this is not unexpected in view of the imposed cosmetic disfigurement to which females are more sensitive. Two peak age groups were noted: at 0-9 years when parents are critical of congenital anomalies and 20-29 years when the young adult is easily embarrassed by deformities that make socio-economic integration difficult.

Etiological factors

Trauma 81 (30.0%) was the leading etiological factor, usually following severe facial injury and this is responsible for the high prevalence 36 (31.0%) of ectropion in this study. This differs from the findings of earlier authors who reported that ectropion occurs more commonly due to involutional changes.12 Congenital anomalies 55 (21.0%) were the second leading reason for seeking oculoplastics care and this is of higher prevalence compared to the findings of (14.0%) and (13.3%), respectively, from previous studies.89 Tumors, the third most common etiological factor, made up 44 (16.0%) [Figure 2] while 20 (17.0%) of all eyelid diseases [Figure 3] were tumors mainly neurofibromatosis in 11 (9.6%). Dermoids 12 (27.3%) [orbital and peri-orbital] and capillary hemangioma (9.1%) were the predominant benign early childhood tumors [Table 2]. This is again much higher than 5.2% dermoids recorded in a survey of childhood diseases carried out in a similar tertiary center serving a much smaller population.9 Diseases of the eyelid constituted 115 (42.8%) of all lesions [Table 1]. This is significantly much higher than (7.9%) occurrence8 in a similar geographic environment and could be due to the fact that this study was carried out on patients presenting specifically to the oculoplastics subspecialty clinic in a more densely populated state. The most frequent eyelid disease was ectropion 36 (31.0%). Ectropion may allow for ocular exposure with the risk of potentially vision-threatening sequelae. Al-Yousuf10 noted that 5% of his study population had abnormal lid position as risk factor for microbial keratitis. Ptosis 33 (29.0%) is the second leading cause of eyelid diseases, with a risk of amblyopia in the pediatric age. It contributed 38.2% of all congenital lesions. Epiphora, 6 (30.0%), and nasolacrimal duct obstruction, 4 (20.0%), among the adult and pediatric age groups, respectively, were the main lacrimal system diseases. Lacrimal system diseases comprised (7.0%) of all pathologies in contrast with the findings of Suppapong et al.,11 who recorded (45%) in all patients presenting with acquired lacrimal duct obstruction. This could be ascribed to the difference in the anatomical structures of the nasolacrimal passages, the black race having wider and shorter nasolacrimal duct which facilitates tear drainage.121314]

Conjunctival diseases

Occurred less commonly, with Stevens-Johnson syndrome as the most prevalent 9 (30.0%). Stevens-Johnson syndrome carries high risk of permanent visual loss from chronic and progressive dry eye and corneal vascularization. It has been documented to account for blindness in 35% of affected patients.15

Ocular and degenerative eye diseases

Phthisis bulbi (33.3%), painful and unsightly blind eye, 11 (23.3%), and anterior staphyloma, 5 (16.7%), were the major degenerative ocular disorders. This is similar to the findings of previous authors who have reported these as major indications for evisceration1617 in patients who sought relief and improved cosmesis.

Orbital and periorbital diseases

These were the second most common diseased conditions seen during the study period constituting 44 (16.4%). Major childhood neoplasia was dermoid cyst and capillary hemangioma whereas thyroid orbitopathy18 and subconjunctival orbital fat prolapse were the prevailing orbital diseases of adulthood. The prevalence of thyroid orbitopathy (25%) is noted in this study and this could be explained by the fact that thyroid eye disease is one of the major leading causes of orbital disease. Orbital tumors when malignant could be life-threatening while proptosis resulting from orbital tumors and thyroid orbitopathy are possible contributory factors to visual loss from optic nerve compression and associated exposure keratopathy. Capillary hemangioma, though present in only 4 (9.1%), is a significant congenital anomaly. This is because the presence of massive capillary hemangioma of the eyelid obstructing the visual axis predisposes an infant to amblyopia and astigmatism, thereby impairing visual development at the early years, if left unattended. Severe ocular injuries and ocular tumors, most commonly retinoblastoma, were the principal causes of eye removal in the study by Eze.19 Enock20 reported (80.95%) of his study population and (19.05%) respectively had evisceration and enucleation. In Madagascar2149.0% underwent similar procedures. The fall-out of these procedures is contracted anophthalmic socket particularly in cases without orbital implant. Presently only few centers have recently begun providing orbital implants in our study environment. This may explain the high prevalence of contracted anophthalmic socket (84.6%) in this study. Neurogenic diseases had remarkably low prevalence and occurred in association with other medical conditions for which patients were referred for medical therapy. This article has attempted to evaluate orbito-oculoplastics diseases presenting to the oculoplastics unit. The most prevailing etiological factors were trauma, congenital anomalies and tumors. Frequently occurring disorders were ectropion, ptosis, contracted socket, phthisis bulbi, dermoid cyst and thyroid orbitopathy.

CONCLUSION

It is expected that this study will provide the ophthalmic and oculoplastic surgeons a broad knowledge of the distribution pattern of prevalent orbito-oculoplastic diseases and thus help in directing focus on patients care, skills and infrastructure acquisition in the formative years of oculoplastics as a subspecialty in the sub-Saharan region.