Abstract
Brain abscess is a known complication of cyanotic congenital heart diseases. We report the case of a four-year-old girl whose first presentation in the hospital was because of symptoms referable to the neurological system. These symptoms are similar to those seen in cerebral malaria as well as other infections of the central nervous system, e.g. meningitis and encephalitis. Detailed history, painstaking examination, and investigations will however help in differentiating these conditions. In the index case however, the illness was fatal due to the late presentation, which is not uncommon in this environment. In a resource-poor environment such as Nigeria, this is a reminder that proper history taking, clinical examination, and subsequent investigations will aid in early diagnosis and subsequent management of such cases to reduce childhood mortality. It also underscores the need for increased awareness among primary health care providers.
<Learning objective: In developing countries where definitive treatments for most congenital heart diseases are not available, complications such as brain abscesses are not uncommon. Primary health care givers should be aware of these complications in these patients. Symptoms such as headaches, fever, and convulsions are common in children living in malaria endemic regions and may mimic the symptoms seen in brain abscess. A high index of suspicion is required to make prompt diagnosis and give appropriate treatment while treating children with congenital heart disease.>
Keywords: Cyanotic, Congenital, Tetralogy, Brain, Abscess
Introduction
Tetralogy of Fallot, the commonest cyanotic congenital heart disease, may be fraught with various life-threatening complications, ranging from hypercyanotic spells to cerebrovascular events. Infective neurological complications are well known, but their prevalence has significantly reduced in developed countries, consequent on early diagnosis and prompt surgical correction of the congenital heart defect. Cyanotic heart disease accounts for 12.8–69.4% of all cases of brain abscesses with identified risk factors in several series, with the incidence being higher in children [1], [2]. Tetralogy of Fallot is the most common cardiac anomaly associated with brain abscess. Brain abscess is a known complication of cyanotic congenital heart diseases. We report the case of a four-year-old girl whose first presentation in the hospital was because of symptoms referable to the neurological system. The illness was however fatal due to the late presentation, which is not uncommon in this environment. In a resource-poor environment such as in Nigeria, this is a reminder that proper history taking, clinical examination and subsequent investigations will aid in early diagnosis and subsequent management of such cases to reduce childhood mortality.
Case report
ME, a four-year-old girl, was referred from a comprehensive health center with complaints of recurrent frontal headaches of 2 weeks duration, fever, and 3 episodes of generalized tonic-clonic convulsions with subsequent loss of consciousness 1 day before presentation. There was a history of non-projectile vomiting prior to presentation. There was no discharge from craniofacial orifices. She had been managed on an outpatient basis with some intramuscular injections without relief of symptoms.
She was the last of six children in a monogamous family setting. Both parents were subsistence farmers without any formal education.
Examination revealed a cyanosed, plethoric child with conjunctival injection, Grade 4 digital clubbing, and an axillary temperature of 38.6 °C. She had scarification marks on her forehead and was unconscious (modified Glasgow Coma Score was 4/15), with global hypotonia. Respiratory rate was 65 breaths/min and breath sounds were vesicular. Heart rate was 176 beats/min; heart sounds were normal and there was a grade 3/6, systolic murmur heard loudest at the left sternal border.
Packed cell volume was 67%, blood film for malaria parasites was positive and showed neutrophilia. Cerebrospinal fluid was turbid with a protein of 913 mg/dl and glucose of 3.5 mmol/l; random blood glucose was 8.2 mmol/l. Microscopy showed numerous white blood cells with Gram-negative cocco-bacilli.
Despite instituting immediate treatment with high-dose ampicillin for meningitis and intravenous artesunate for cerebral malaria, the patient remained critically ill and succumbed 1 h after arrival in the hospital.
Postmortem examination of the heart revealed infundibular pulmonary stenosis, right ventricular hypertrophy, and a large ventricular septal defect with an overriding aorta, confirming a diagnosis of tetralogy of Fallot (Fig. 1). There was a purulent exudate at the base of the right frontal lobe of the brain (Fig. 2) as well as an abscess in the frontal lobe on the same side (Fig. 3).
Fig. 1.

Postmortem demonstration of the ventricular septal defect (VSD) and aorta overriding with probe passing through the VSD. LV, left ventricle.
Fig. 2.

Postmortem of the brain showing congested blood vessels and purulent discharge at the base of the brain.
Fig. 3.

Postmortem of the brain showing large frontal lobe abscess.
Discussion
Cerebral abscess is a well-known complication of cyanotic congenital heart diseases, commonest of which is tetralogy of Fallot. Meningitis is a less common complication. The index case highlights some of the limitations existing in the developing countries in the management of children with congenital heart disease.
This child's presentation, with two concomitant life-threatening complications, was the first time a diagnosis of tetralogy of Fallot was made. Similar delays have previously been noted in developing countries [3]. In more advanced countries, the diagnosis would likely have been made in-utero, or at worst, by neonatal screening for congenital heart disease, facilities for both of which are not widely available in Nigeria at the present time. Closely related to this fact is the low index of suspicion by the various health providers involved in the management of children at the onset of the illness.
In a 55-year review of historical trends in the Children's Hospital, Boston in Massachusetts, USA, congenital heart diseases were the topmost predisposing factors for intracerebral abscess. The symptoms found to be of note in their patients were new onset headache, associated with vomiting or photophobia and fever [4], [5].
In this environment, the holoendemicity of malaria often causes a delay in the diagnosis of meningitis/cerebral abscess, especially in children. The symptomatology of fever, headache, and vomiting are common to all three conditions [6]. The diagnosis in the index case was further confounded by the absence of localizing signs and the positive blood film for malaria parasites. In resource-poor settings however, the presence of neutrophilia on the peripheral film should heighten the suspicion of a possible bacterial co-infection in the patient. The availability of cranial computed tomography or magnetic resonance imaging would have further aided the diagnosis and management of the child.
Although the culture of the cerebrospinal fluid was not carried out, microscopy revealed Gram-negative cocco-bacilli. Earlier documentations had reported Gram-positive cocci in patients with intracerebral abscesses [2], [7].
The late presentation of this patient with no previous diagnosis of cyanotic congenital heart disease coupled with the brain abscess and meningitis contributed significantly to the fatality of the child.
While a decline is being observed in such occurrences in developed countries of the world, this serves as a reminder that there is a need for more attention to be paid to the developing world in the areas of training, assistance with echocardiography machines, and surgical correction of these defects thereby ensuring global wellbeing and reduction in childhood mortality.
Conflict of interest
We declare that we have no conflicting interests whether financial or otherwise in relation to this work.
Acknowledgments
We acknowledge the fact that the case report was a poster presentation at the World Congress Paediatric Cardiology and Cardiac Surgery 2013 in Capetown South Africa. This report was not funded by any organization.
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