Nigerian Journal of Paediatrics

Aids related kaposis sarcoma in a four year old child the challenge of a missed opportunity

Niger J Paediatr 2017; 44 (4): 185 – 189

CASE REPORT

Aiyekomogbon JO

CC – BY

Aids- related kaposi’s 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 Kaposi’s 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

Kaposi’s 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;

Kaposi’s

sarcoma;

immunodeficiency

syndrome

Missed opportunity; Paediatric

(AIDS)-related

illness.

Her

AIDS; Skin rashes

Introduction

Kaposi, first described Kaposi’s sarcoma (KS) in 1872

as a rare multifocal angioproliferative tumour involving

blood and lymphatic vessels. In 1981, first case of

A Vienna-based Hungarian dermatologist, Moritz

186

AIDS- associated Kaposi’s sarcoma was reported by

onset of the body swellings. The body swellings were

Friedman-Kien et al This aggressive form of Kaposi’s

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

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).

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

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-

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

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.

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

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 parents’ 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

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

is most frequently affected, then the gums.

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.

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

Kaposi’s 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 child’s 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-

‘echo - free’ 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

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.

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

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.

There are very few reported cases of HIV-

homogenous opacities with

related KS among children in Nigeria , and the index

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

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

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 Kaposi’s 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 wouldn’t 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 patient’s 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

Conflict of Interest: None

AIDS- associated KS were parts of this patient’s presen-

Funding: None

tation, and both connote severe disease. This could

explain why the patient could not survive despite prompt

intervention at our level.

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