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Nigerian J Paediatrics 2017 vol 44 issue 1

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Aids related kaposis sarcoma in a four year old child the challenge of a missed opportunity
Niger J Paediatr 2017; 44 (4): 185 Ė 189
CASE REPORT
Aiyekomogbon JO
CC Ė BY
Aids- related kaposiís sarcoma in
Ifeorah IK
a four year old child: the
challenge of a missed opportunity
DOI:http://dx.doi.org/10.4314/njp.v44i4.3
Accepted: 1st April 2017
Abstract : Background: AIDS-
mother also tested positive to Hu-
related Kaposiís sarcoma (KS) is
man
Immunodeficiency
virus
Aiyekomogbon, JO (
)
an AIDS-defining illness and is
(HIV) and has been on highly ac-
Department of Radiology, College
now increasingly recognized in
tive
anti-retroviral
therapy
of Health Sciences, University of
children infected with HIV. Many
(HAART) for a year. Physical ex-
Abuja, Abuja Nigeria
of these cases are missed due to
amination revealed moderate pal-
Email:
low index of suspicion. Vertical
lor, bilateral pitting pedal oedema,
femimogbon2002@yahoo.com
transmission of HIV is the com-
and matted non-tender peripheral
monest route of transmission in
lymphadenopathy. There were
Ifeorah IK
children and this is preventable by
papular and nodular skin lesions
Department of paediatrics,
early maternal antenatal
with a reddish solid lesion on the
108 Nigerian Air Force Hospital,
diagnosis, early commencement
hard palate. She tested positive to
Abuja Nigeria
of HAART by the mother and
HIV. Abdominal ultrasound scan
Email: fikky17@yahoo.com
adoption of the safest possible
revealed moderate hepatomegaly
mode of delivery. The index case
with ascites, while chest x-ray
did not benefit from these
showed bilateral interstitial pneu-
services, making her acquire HIV
monitis, right hilar lymphadenopa-
and then develop AIDS-defining
thy, right pulmonary nodule and
illness (KS) at a tender age of 4
ipsilateral pleural effusion. Exci-
years.
sional biopsy of one of the skin
Aim and objective: The study is
nodules confirmed the diagnosis of
aimed at emphasizing the need for
Kaposiís sarcoma. She was com-
all pregnant women to have ante-
menced on HAART and antibiot-
natal screening as soon as preg-
ics, with a unit of blood transfused.
nancy is confirmed so as to pre-
Her condition deteriorated, which
vent vertical transmission of HIV
necessitated referral to university
to the unborn child. It is also
of Abuja Teaching Hospital where
aimed at creating awareness in
she eventually died after a day of
clinical practice so as to increase
admission.
index of suspicion among
Conclusion:
Paediatric AIDS-
clinicians when evaluating chroni-
related KS is becoming
cally ill children.
increasingly common in Nigeria
Case Description: GG was a
and as such, high index of suspi-
4year old girl who presented at
cion is required while evaluating
108 Nigerian Air force Hospital,
pediatric patients. This, coupled
Abuja with non-itchy skin rashes
with early commencement of
of two weeks duration, general-
HAART is required to avert the
ized body swelling, cough and
pitfalls observed in this case; bear-
haematochezia of one week
ing in mind that childhood AIDS-
duration, and fever of four days
related KS is lethal.
duration. She was a paternal or-
phan having lost her father a year
Keywords : Generalized lympha-
prior to presentation to Acquired
denopathy;
Kaposiís
sarcoma;
immunodeficiency
syndrome
Missed opportunity; Paediatric
(AIDS)-related
illness.
Her
AIDS; Skin rashes
Introduction
Kaposi, first described Kaposiís sarcoma (KS) in 1872
as a rare multifocal angioproliferative tumour involving
blood and lymphatic vessels. In 1981, first case of
1
A Vienna-based Hungarian dermatologist, Moritz
186
AIDS- associated Kaposiís sarcoma was reported by
onset of the body swellings. The body swellings were
Friedman-Kien et al This aggressive form of Kaposiís
2
first noted in the lower limbs and thereafter on the face.
sarcoma is the most frequent cancers in patients with
There was no diminution in urine volume and the body
HIV infection. HIV-related KS was 20,000 times more
3
swelling did not regress with ambulation. The body
prevalent when compared with uninfected population
rashes were discoid, hyperpigmented skin lesions found
before the advent of highly active anti-retroviral therapy
all over the body but more on the extremities. There was
(HAART).
4
no associated itching and no antecedent history of drug
It is a well known fact that Human immunodeficiency
use, insect bite or allergy prior to onset of the rashes.
virus (HIV) infection predisposes to several opportunis-
There was however an associated one week history of
tic infections and neoplasms, such as KS and non-
cough and passage of bloody stools. There was no
Hodgkins Lymphoma (NHL). While KS is the most
5
history of bleeding from any other site and no history of
common neoplasm seen in HIV-infected adults, it is rare
abdominal pains or swellings. At the time of presenta-
in childhood AIDS in western countries. It is an AIDS-
6
tion, there was a four day history of fever for which
defining disease in 0.5% of children in Europe, com-
patient was commenced on paracetamol.
pared with 0.9% in the United States. This observation
7
is however different in Africa as KS is increasingly
No medical or unorthodox intervention had been sought
being recognized among infected children in many Afri-
at the time of presentation. The mother had routine ante-
can countries.
8
natal care at a local primary health facility and had
spontaneous vaginal delivery. There had been no history
Different types of KS have been documented. These are
of hospital admissions, blood transfusions or scarifica-
epidemic (AIDS-related) KS, classic (Mediterranean)
tions in the past. Haemoglobin genotype was unknown
KS, endemic (African) KS and iatrogenic (transplanted-
but there was no history suggestive of sickle cell dis-
related) KS. The AIDS-related KS is the commonest. It
ease. Her immunization status was up to date according
is note-worthy that all varieties of KS are associated
to the National Programme on Immunization (NPI)
with Herpes virus, and irrespective of the types, KS pre-
Schedule. She was exclusively breastfed up to six
sents similarly but have differences in clinical aggres-
months with complementary feeds introduced thereafter
sions, prognosis and treatment. AIDS-associated KS
9
and breastfed for up to a year. Her milestones were
typically presents with cutaneous lesions that begins as
within normal limits when compared with her sibling
one or several red to purple-red macules, rapidly pro-
and peers. She was the second of her parentsí two chil-
gressing to papules, and plaques, with a predilection for
dren in a monogamous family setting of low social class,
head, back, neck, limbs, trunk and mucous membranes.
and a paternal orphan having lost her father a year prior
Lesions could also be found in the stomach and
to presentation to AIDS related illness. The mother was
intestines, lymphnodes and the lungs. Mouth is
9
screened for HIV at the same time and found to be reac-
involved in about 30% of cases and is the initial site in
tive and subsequently commenced on HAARTS.
15% of AIDS-related KS. In the mouth, the hard palate
10
is most frequently affected, then the gums.
10
On examination, she was a pre-school age child not in
any painful distress, afebrile, acyanosed, anicteric, but
KS is not curable, but often treatable. In KS-associated
moderately pale and she hadbilateral pitting pedal
with immune deficiency, treating the cause of the im-
oedema up to the middle of the thigh. There was signifi-
mune system dysfunction can impair or abolish the
cant matted non-tender peripherallymphadenopathy
progression of KS. Radiation therapy or cryosurgery,
(supraclavicular, axillary and inguinal groups). There
and chemotherapy combined with HAARTS are found
were also multiple, brownish-black lesions noted at the
useful in its treatment.
11,12
Despite strict adherence to the
extremities and all over the body with those on the limbs
outlined treatment protocol, the fatality associated with
being nodular (figure 1). She was 16kg which was 89%
AIDS-related KS in children is high. Progression of dis-
of expected. Mid-upper Arm Circumference was 13.5cm
ease is rapid, with high mortality in the initial hospitali-
and Occipito-frontal Circumference was 48cm.She was
zation.
13
not dyspnoeic and had good air entry bilaterally. Also,
This case is presented to create awareness among clini-
she had normal volume regular pulse with a blood pres-
cians and other health workers, on AIDS-related
sure of 90/50mmHg and an apex beat located at the 5th
Kaposiís sarcoma in paediatric age group so as to avert
left intercostal space mid clavicular line. Examination of
the observed missed opportunity in the nearest future. It
the digestive system however revealed a reddish lesion
emphasizes the need for early antenatal diagnosis and
involving the hard palate. The abdomen was protuberant
commencement of active management of an infected
with a reducible umbilical hernia, the liver was 6cm
mother so as to prevent mother to child transmission of
enlarged below the right costal margin, smooth surfaced
HIV.
and non tender. There was no other palpable intra-
abdominal organ and no demonstrable ascites clinically.
Case presentation
The childís retroviral screening using rapid antibody
testing was positive. Full blood Count showed a Packed
GG was a four year old girl who presented to our health
Cell Volume of 20%,with normal white cell and platelet
facility (108 Nigerian Air force Hospital, Abuja) with
counts but with the white cells having a relative neutro-
generalized body swelling of one week duration with
philia for age. The liver function test showed severe
associated body rashes noted at about two weeks prior to
hypoproteinemia and hypoalbuminemia. Urinalysis,
187
urine microscopy and renal function tests were within
normal limits. Patient had an excision biopsy of a skin
Fig 4: Abdominal ultrasono-
nodule with histology result showing a tumour contain-
gram at the level of the
ing proliferating spindle cells arranged in bundles, sepa-
psoas muscles showing
rated by sinusoidal spaces. Focal areas of old haemor-
Ďecho - freeí extra -luminal
fluid collection (ascites) at
rhage with haemosiderin pigments were also present.
the right iliac fossa.
Chest X-Ray done at presentation showed non-
homogeneous opacities with background nodularity and
streakiness at the upper and mid zones bilaterally, more
marked on the left. The right hilum was full and lobu-
lated with convex outer margin, connoting hilar lympha-
Discussion
denopathy (figure 2). The recesses, heart and bony tho-
rax were initially unremarkable. At the interval of two
Human immunodeficiency virus (HIV) infection results
weeks however, she had a repeat chest x-ray done in
ultimately into Acquired Immunodeficiency syndrome
view of repeated non-abating cough and severe respira-
(AIDS) if not managed early and appropriately. The
tory distress. Findings at this time showed right pleural
main modes of transmission is via sexual intercourse,
effusion and a rounded nodular opacity of soft tissue
vertical transmission from mother to child, blood and
density at the right mid zone, in addition to the earlier
blood products transfusion, and sharing of needles or
findings (figure 3). Simultaneously, an abdominal ultra-
sharp objects, particularly among intravenous drug
sound scan was done as a result of gradual abdominal
abusers (IDA). Vertical transmission was the most
14
distension despite good bowel habit. This revealed mod-
likely mode of transmission in the index case.
erate hepatomegally with a scites (figure 4). The remain-
Patients could be asymptomatic at the beginning (group
ing abdominal viscera were preserved, and no abdomi-
A), but later develop symptoms but do not have AIDS-
nal lymphadenopathy was observed sonographically.
defining condition. This group is termed group B (AIDS
-related complex-ARC) based on Centre for Disease
In view of the aforementioned clinical, radiological and
control (CDC) classification. Group C in this category
laboratory findings, a diagnosis of AIDS-related Kaposi
includes patients who have AIDS-defining illness,
Sarcoma was made which was confirmed histologically.
which is the group the index patient belonged to. At this
She was commenced on Highly Active Anti Retroviral
point, opportunistic infections and secondary neoplasm
Therapy (HAART), antibiotics and transfused once on
such as KS, NHL and other neoplasm set in.
14,15
account of severe anaemia. She was then referred to
KS is the most common non-infectious AIDS-defining
University of Abuja Teaching hospital when her condi-
diagnosis in HIV disease.
15
It is relatively rare among
tion deteriorated, and she subsequently died after a day
children compared to adults. In the developing countries
of admission.
however, frequent lesions of KS have been reported
among children in central and southern Africa, and this
had been attributed to the endemic presence of Human
Fig 1: Clinical photograph of the
Herpes virus type 8 (HHV8). There is evidence from
16
child showing generalized
oedema with hyperpigmented
epidemiologic, serologic, and molecular studies that KS
nodular skin lesions on the limbs,
is associated with this virus. The disease starts as a reac-
chest, abdomen and ear lobes.
tive polyclonal angioproliferative response to the virus,
in which polyclonal cells change to oligoclonal cell
populations that expand and undergo malignant transfor-
Fig 2: Chest X-Ray showing non-
mation.
17
There are very few reported cases of HIV-
homogenous opacities with
related KS among children in Nigeria , and the index
18
background nodularity and
case happened to be the first in our institution which has
streakiness in the upper and
a dedicated HIV treatment centre.
middle zones of both lungs,
more marked on the left. The
right hilum is full and lobulated
This histological proven case of KS in a 4 year old child
with convex outer margin,
in Abuja, Nigeria further adds to the existing literature
connoting lymphadenopathy.
showing the increasing trend of AIDS-associated KS in
developing countries. This is still relatively rare hence;
high index of suspicion is required when evaluating a
chronically sick child, so as to avert further diagnostic
Fig 3: Chest X-Ray of the
pitfalls observed in the index case.
same patient at two weeks
interval showing right pleural
The mother had antenatal care at a primary health centre
effusion with a rounded nodu-
in Abuja, but she was not screened of HIV as the facility
lar opacity of soft tissue den-
for the test was not available at the health centre. She
sity in the right middle zone, in
was therefore not diagnosed antenatally and not com-
addition to fig.2 findings.
menced on HAARTS until when the husband was diag-
nosed a year prior to the presentation of the child to our
hospital. This further confirms the poor accessibility of
188
Nigerians to quality health care delivery. This needs to
As earlier noted, KS is not curable but treatable. AIDS-
be worked upon so as to avert this scenario in the near-
associated KS usually shrinks upon commencement of
est future.
HAART. Patients with a few local lesions may be
treated with radiotherapy and cryosurgery, but chemo-
Between 1981 and 1990 in Zaria, Nigeria, four children
therapy with HAART is more effective in those with
with KS were histologically diagnosed, and none of
widespread disease. Also, affectation of internal organs
them was HIV positive. In another report from the
18
as in the index case could be treated with Interferon
Alpha, Liposomal anthracyclines or paclitaxel.
10,11,12
same institution between 1991 and 1995, two of the
8
four cases of childhood KS were HIV positive, and two
These drugs are not readily available in Nigeria and
were also noted in Maiduguri within the same period.
[19]
when available, they are not affordable. It is therefore
One would expect this to decrease significantly with the
recommended that PEPFAR and other donor agencies
advent of widely available HAART in Nigeria, particu-
include them as part of the drugs for paediatric AIDS
larly in Abuja, the Federal Capital Territory. Diagnostic
patients, especially those that have already developed
flaws were however noted in the index case. Her parents
AIDS- related Kaposiís sarcoma. Our patient could not
were not diagnosed in good time so, the child did not
survive after commencing HAART due to overwhelm-
benefit from the widely available prevention of mother
ing pulmonary and other systemic affectations. She
to child transmission (PMTCT) of HIV. Her father died
therefore did not benefit from other outlined regimen.
a year prior to her presentation as a result of AIDS-
Early presentation could have averted this. This was first
defining illness. It was at that point that the mother was
hampered by inability to diagnose the mother antena-
screened and diagnosed of HIV infection. The index
tally due to poor facilities at the primary health centre
case was three years old then, and the attending physi-
she attended. Antenatal HIV screening should be en-
cian did not deem it necessary to screen her, more so
forced and facilities for such made available at all levels
that she was the last child. If peradventure she was diag-
of antenatal care, primary, secondary or tertiary health
nosed earlier, she wouldnít have developed AIDS -
centre. Local, State and Federal Governments should
defining KS as she would have commenced HAART. It
increase allocation for Primary Health Care services as it
was only at the time of recent and only presentation at
is the most accessible health facility for over 70% of
our health facility that her status was known. We con-
Nigerians. The staffers of such institutions should be
sidered this a missed opportunity.
better trained and retrained on all aspects of care of HIV
patients. A vibrant system for home visits and home-
KS lesions are nodules that may be red, brown, purple or
based care is also advocated as that will go a long way
even black depending on the patientís skin colour. The
to avert the observed missed opportunity.
lesions are usually non-itchy and papular, and the com-
monly affected areas are the lower limbs, face, mouth,
and genitalia. The index case had her lesions at the
aforementioned sites, which arouse the suspicion of the
Conclusion
attending paediatrician, making him to screen her the
first day she presented to him. She also had a reddish
Paediatric AIDS-related KS is becoming increasingly
nodular mass on the hard palate, which is the common-
common in Nigeria and as such, physicians are admon-
est site of oral KS lesions.
11,19, 20
In addition, the index
ished to have high index of suspicion while evaluating
patient presented with heamatochezia, hepatomegaly
pediatric patients. This coupled with early commence-
and ascites. The heamatochezia could be due to deposi-
ment of HAART is required to avert the pitfalls ob-
tion of KS lesions on the stomach and/or intestines. This
served in this case; bearing in mind that childhood AIDS
gastrointestinal tract lesion and hepatic enlargement are
-related KS is lethal.
indicative of visceral involvement which connotes
severity of the disease with attendant high fatality
rate. Also, interstitial pneumonitis and pulmonary
20
Conflict of Interest: None
AIDS- associated KS were parts of this patientís presen-
Funding: None
tation, and both connote severe disease. This could
20
explain why the patient could not survive despite prompt
intervention at our level.
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