Erick Ikoona
1, Israel Kalyesubula
2, Medi Kawuma
1
1 Department of Ophthalmology, Makerere University P.O. Box 7072, Kampala, Uganda
2. Department of Paediatrics, Makerere University, P.O. Box 7072, Kampala, Uganda.
Code Number: hs03015
ABSTRACT
Background: In Uganda the prevalence of HIV averages 12% as was reported to the STD/AIDS control surveillance unit. In Uganda there are approximately 30,000 HIV infected infants per year. The burden of HIV disease is high in Uganda and patients present with ocular complications. However, there is paucity of information and knowledge concerning ocular manifestations in the paediatric HIV/AIDS population and how they may differ from those of adults.
Objectives : To describe the ocular manifestations of HIV/AIDS infection in an African paediatric population. Generally the study will record the external ocular manifestations seen but specifically to document the intra-ocular lesions, in particular the retinal changes associated with paediatric HIV/AIDS.
Design: A cross-sectional hospital based study.
Setting: The study was conducted at the Paediatric Infectious Disease Clinic at Upper Mulago Hospital, in Kampala, Uganda.
Patients: Patients are those with positive HIV sero status, with or without symptoms and signs of AIDS. Parents/caretakers of the children were interviewed to obtain the socio-demographic data of the patients and a general physical as well as an ophthalmic examination were conducted to document any ocular problems.
Results: A total of 158 HIV -infected children were examined. The overall rate of ophthalmic involvement was 35%. The most common finding was a non-purulent conjuctivitis, observed in 12% of the patients, followed by perivasculitis of the peripheral retinal vessels, in 12 % of patients and molluscum contagiosum.
INTRODUCTION
Since it was first described in mid 1981 Acquired
Immunodeficiency Syndrome (AIDS) has become
a major concern to all doctors, irrespective of
their area of study or specialisation.
Ophthalmologists have not been spared. They are
often called upon to make the initial diagnosis of
AIDS; most often, however, they are requested to
help treat the ocular manifestations of related
opportunistic infections. These can have disastrous
consequences for sight, especially for patients who
are first seen when already markedly debilitated.
Practising ophthalmologists, especially
those in the developing world, are faced with the
following challenges. One, they must be able to
recognise and treat potentially sight-threatening
conditions. Two, they must be able to identify
unusual presentations of known organisms, so that
they are not regarded as part of the AIDS ocular syndrome. Three, ophthalmologists must be able to
identify and document manifestations that have not been
described before, or have been seen before but were
thought not to occur in AIDS patients.
The ocular complications in adult HIV/AIDS
patients have been well documented, especially in the
developed world1. Data available from developed countries
indicate that the pattern and prevalence of HIV-related
ocular disease in the paediatric population is different
from adult patients. The prevalence of cytomegalovirus
retinitis is especially low in children2, estimated at 5%, yet it
continues to be the commonest sight-threatening
complication in HIV/AIDS adult patients 3,4.
Other paediatric ocular manifestations as seen in
developed countries include neuro-developmental delay,
a condition often associated with neuro-ophthalmic
disorders
2 , a fatal AIDS-related embryopathy with
downward obliquity of the eyes, prominent palpebral
fissures, hypertelorism, and blue sclerae
5 . In Africa, the
scanty literature available appears to indicate that macular
oedema, retinal haemorrhages, conjuctival lesions and
perivasculitis of the retinal peripheral vessels are the
common manifestations
6 .
In order to assess the type and prevalence of
ocular involvement in paediatric HIV/AIDS patients in
Mulago Hospital, a study of such patients seen at the
Infectious Paediatric Disease Clinic was carried out.
MATERIALS AND METHODS
Patients/study population.
The study population included patients aged
less than one year old to sixteen years old attending
the Paediatric Infectious Disease Clinic at Upper
Mulago between May and October, 2001
Study design:
This was a cross-sectional descriptive study in
which HIV/AIDS paediatric patients were
assessed for ocular disease complications. One
hundred fifty eight children aged six months to 16
years were consecutively recruited and examined.
Inclusion criteria
- Children with HIV/AIDS aged 0 to less
than sixteen years attending the Paediatric
Infectious Disease Clinic.
- Children whose parents /caretakers gave
informed consent.
Exclusion criteria
- Children who were too sick to undergo a
detailed physical and ocular examination.
- Children whose parents/ caretakers declined
to give consent.
Ethical issues
The study was approved by the ethics committees
of Mulago hospital, the Makerere University
Faculty of Medicine, and the Department of
Ophthalmology. Written consent was obtained
from parents/ caretakers of the children who
participated in the study.
Sampling and sample size estimation
All patients attending the paediatric infections
disease clinic during the study period and who
fulfilled the inclusion criteria were enrolled
consecutively. On subsequent clinic days only new
patients were enrolled.
The sample size of 120 was calculated
using the formula for prevalence studies. We
assumed a 38% incidence of eye disorders among
180 paediatric HIV/AIDs patients over a one year
period, a precision of 5% and 95% confidence.
Examination
The parents /caretakers were briefly interviewed
to obtain socio-demographic data of each child.
This was followed by a full general examination
by the paediatrician and then an ophthalmic examination. Each child had cyclopentolate 2% eye drops
instilled in the eyes three times at an interval of five
minutes. After half an hour, an ocular examination was
carried out. First, an external ocular examination was done
using a light ( and a binocular coupe where necessary).
This was followed by a detailed funduscopy, using both
the direct ophthalmoscope, and the indirect , monocular
ophthalmoscope. Reference was made to each patient’s
case notes to ascertain any systemic conditions a patient
might have had in the past, for example pneumocystitis
carinii Pneumonia, tuberculosis or Kaposi’s sarcoma.
Statistical analysis
The quantitative data was entered into the EPI INFO
version 6 package of the computer. The entry was done
by the data entry clerk. The data was then cleaned and
analysed with the help of a statistician.
RESULTS
General: A total of 158 HIV- infected children, 87 girls
and 71 boys were identified. The average age at initial
examination was 3½ years (range ½ to 15¼ years).
Ophthalmological results: A total of 130 children had
a single examination, 20 children were seen twice, and 8
children were seen three or more times. The average rate
of ophthalmic involvement was 35% ( 55/158 patients).
Retinal findings: By far, the most common finding was
inflammation of the peripheral retinal vessels, which was
observed in 49(31%) of the patients. The lesions were
mainly located in the periphery and the equatorial regions.
Veins were involved more often than arteries. Discrete
lesions around the arteriolar walls, in the vicinity of the
diseased venules were seen. The perivasculitis were seen
as patches of fluffy white haziness around vessel walls.
The lesions were labelled perivasculitis if they presented
as irregular white patches around the vessel wall or as
sheathing if they produced a more regular, yellowish, linear
thickening of the involved vessels. Eighteen children had
involvement in one eye and 31 had bilateral lesions, giving
a total number of 80 affected eyes.
CMV retinitis was observed in six children (4%).
Fundus examination of these children revealed areas of
retinal necrosis, haemorrhages, and cotton wool spots
along the major vessels. We did not observe the isolated
cotton-wool spots so often seen in the adult CMV retinitis.
External findings: Three children had ophthalmic herpes
zoster.
Fourteen patients had conjunctival xerosis, which
responded favourably to oral vitamin treatment. All the
fourteen had clinical signs of kwashiorkor.
Sixteen children had molluscum contagiosum; 11 of them had extensive bilateral lesions involving both the upper and lower eyelids. Six of these children had extensive body involvement, including the upper limbs.
Eleven patients presented with central corneal ulcers, and one of them had active herpes zoster at the time.
Five children presented with ocular nerve palsy; two had bilateral involvement. Three had isolated sixth nerve involvement and two had a third nerve involvement. The two children who had bilateral involvement had sixth nerve palsy.
Sixteen children out of 20 were reported by parents /caretakers not to be producing tears (when crying), and had these abnormal shirmer tests.
DISCUSSION
Among adults with HIV/AIDS, the incidence of
8 ocular manifestations is high , varying between 50% and 90% (8). However, the incidence in2 paediatric patients is lower. Dennehy reported
7 an incidence of 20%, whereas Kestelyn et al studying children with HIV in Rwanda found an incidence of 33%. This figure of Kestelyn is similar to our finding of 35%.
The most significant finding is the high incidence (31%) of perivasculitis and/or sheathing of the peripheral retinal vessels. This particular finding is in agreement with that of Kestelyn etal7 . Padhani found an incidence of only 3% in children with AIDS in Muhimbili Medical Centre 9.This is in total contrasts to incidences of available studies both in Africa and in the Western World, where the incidence reported is 20% and above.
CMV retinitis, the second-most common
ocular manifestations in adult patients with AIDS10 was present in only 4% of our patients.
Smaller incidences of 1.6% and 1.8% have been reported by De Swet and Nussenblatt in a
cohort of 120 HIV infected children 11 . CMV and other infections ( tuberculosis, toxoplasmosis, cryptococcosis are less likely to develop in children because they have not yet been infected with these organisms, unlike adults in whom reactivation of potent infection occurs when the immune system deteriorates
12.
None of our patients presented with the typical isolated cotton-wool spots, which constitute the most frequent ocular manifestation of HIV infection in adults13 . Cotton-wool spots are thought to be the end result of a chain of events including elevated levels of circulating immune complexes, deposition of immune complexes and resultant microvascular lesions, ischaemia and finally stasis of axoplasmic flow. It is unclear why cotton-wool spots are not seen in children with AIDS.
External pathology of the eye and its adnexae was common in our patients, unlike say, in the study of
Kestelyn et al7 . We found fourteen children with manifest vitamin A deficiency. This is not surprising. All of the fourteen were preschool children, the vulnerable age group for vitamin A deficiency in developing counties.
Other studies have also reported also ocular manifestations such as corneal involvement and molluscum contagiosum9 . Ocular nerve palsies, however, are not commonly noted in HIV/AIDS in children. In our study, five children presented with ocular nerve palsies that could not be attributed to any cause other than AIDS.