A 12-year-old girl who presented with a 4-week history of high grade, continuous fever, only temporarily relieved by use of oral analgesic (paracetamol), a 3-week history of migratory joint pains and swelling involving the elbow, knee and ankle joints with generalized skin eruptions of 2 weeks duration prior to presentation involving the trunk and extremities but sparing the face, non-itchy, accentuated after taking a warm bath and a 1-week history of difficulty with breathing, easy fatigueability, orthopnea and bilateral leg swelling which necessitated referral from the referring hospital.
There was no preceding history of a sore throat or features of an upper respiratory tract infection few weeks to onset of symptoms. Neither was there a prior history of similar complaints in the past nor a history of abdominal or facial swelling and oliguria. She was in junior secondary school and resided in the dormitory (boarding school). She received treatment at two different private hospitals where she was commenced on analgesics and antibiotics. She was transfused with blood at the second private hospital on account of packed cell volume of 25% and referred to our hospital for further management.
Ante-natal, birth and post-natal periods were uneventful. She achieved motor (gross and fine) and speech milestones at the appropriate age. Her past results in school showed good academic performance. She had received all vaccines according to the National Program on Immunization schedule. She was the third of four children in a monogamous family.
On examination at presentation, she was in respiratory distress (flaring alae nasi, intercostal and subcostal recessions), febrile with a temperature of 38.5 °C, with generalized circular and coalescing skin eruptions on the trunk, back and extremities sparing the face with pale centres and serpiginous borders (Figs. 1, 2, and 3), mildly pale, acyanosed in room air with pedal oedema up to the mid-shin. She was tachypnoeic with a respiratory rate of 68 cycles/min, with basal coarse crepitation bilaterally on auscultation of the lungs. She had tachycardia with a pulse rate of 135 beats/min which was regular, normal volume and synchronous with other peripheral pulses. She had normal blood pressure for age. The precordium was hyperactive with displaced and diffuse apex beat located at the 6th left intercostal space anterior axillary line and a first, second and third heart sounds with a grade 3 pan-systolic murmur loudest at the apex and radiating to the left axilla. Abdominal examination revealed a soft, smooth and tender hepatomegaly with a palpable edge 6cm below the right costal margin. Musculoskeletal system examination revealed swollen left elbow, left knee and right ankle joints which were markedly tender.
An initial diagnosis of congestive cardiac failure secondary to carditis in a child with acute rheumatic fever was made. Chest radiograph showed cardiomegaly with CTR of 62%. Electrocardiography showed sinus tachycardia, right atrial enlargement and right axis deviation. Echocardiography (Fig. 4) revealed dilated right atrium, dilated LV and slightly thickened mitral valve with no movement restriction. There was prolapse of the anterior leaflet of the mitral valve and grade 3 mitral regurgitation with ejection fraction of 70%. Right sided function was normal but there was mild tricuspid regurgitation with a pressure gradient of 25 mmHg in keeping with a mild pulmonary artery hypertension. The full blood count study showed features of iron deficiency anaemia with packed cell volume of 28%, thrombocytosis with platelet count of 669,000/mm3, reduced mean corpuscular volume (MCV) of 69fL and mean corpuscular haemoglobin (MCH) of 22.5pg with increased red cell distribution width (RDW) of 19.2%. The full blood count also revealed neutrophilia with left shift and presence of toxic granulations. Erythrocyte sedimentation was elevated with a value of 83mm/h. Anti-streptolysin O titre was also elevated at 2020 IU/ml; throat swab microscopy, culture and sensitivity were negative for streptococcus while serum electrolytes, urea and creatinine were within normal limits.
A diagnosis of acute rheumatic fever was made using the Jones criteria. She had three major criteria (erythema marginatum, polyarthritis, carditis) and two minor criteria (fever, elevated erythrocyte sedimentation rate) with evidence of recent streptococcal infection. She was admitted, commenced on intranasal oxygen and nursed in cardiac position with anti-failure (intravenous frusemide, oral enalapril, anti-inflammatory drugs (Aspirin) and antibiotics and low salt diet (second-generation cephalosporin). Secondary prophylaxis was commenced with intramuscular benzathine penicillin 600,000 IU every 3 weeks after a test dose. Remarkable clinical improvement was made; joint pains and swelling and symptoms of congestive cardiac failure resolved within 1 week while the fever and skin eruptions resolved within the second and third week of admission respectively. She was discharged after 4 weeks on admission. She is being followed up regularly in the cardiology clinic and to continue with three weekly benzathine penicillin prophylaxis.
