P.E. Adams

Early presentation of endomyocardial fibrosis in a 22-month-old child: a case report.

We present an unusual, biopsy-proven case of endomyocardial fibrosis in a 22-month-old male child, which progressed rapidly resulting in death. The patient was born to a father originating from Mozambique, where the disease is endemic but who had not himself travelled there, suggesting a genetic link. Other remarkable features were the presence of a right ventricular diverticulum, and a positive Mycoplasma pneumoniae immunoglobulin M enzyme-linked immunosorbent assay test.

Diagnosis and Treatment of Listeria monocytogenes Endophthalmitis: A Systematic Review

ABSTRACT Purpose: Describe patient characteristics, treatment, and vision outcomes of Listeria monocytogenes endophthalmitis, an exceedingly rare form of listeriosis. Methods: L. monocytogenes endophthalmitis cases in human adults, located through Medline (32) and from disease surveillance centers (11). L. monocytogenes conjunctivitis and keratitis were excluded. Results: Most cases occurred in 2000–2015 (22/43), and almost all in Europe or North America (40/43). Patients were a median 61 years, 57% male (24/42) and half were immunosuppressed. Median days from entering care to diagnosis was 8 (IQR = 5–17). Only four were exogenous infections. L. monocytogenes was identified in 31/35 of anterior eye fluid samples (89%). Antibiotic regimens varied markedly (mostly ≥3 drugs). At diagnosis, most were blind in the affected eye (85%, 28/33), only a third regained normal vision (12/36). Older patients had poorer outcomes. Conclusions: Cases increased over time. Diagnostic delays were common and visual impairment often refractory to treatment, especially in older adults. The condition’s rarity and variation in treatment makes it difficult to identify optimum therapy.

Tetralogy of Fallot with absent pulmonary valve syndrome: A 34-year African single centre experience

Background: Absent pulmonary valve syndrome (APVS) is most commonly associated with Tetralogy of Fallot (TOF). TOF with APVS is characterised by features of TOF with either rudimentary ridges or complete absence of pulmonary valve tissue. This is often associated with varying degrees of pulmonary stenosis and severe pulmonary regurgitation with massive dilatation of the proximal branch pulmonary arteries, causing compression of the tracheobronchial tree. Hence, respiratory symptoms are a common presenting feature. Methods: Cases of TOF with APVS were extracted from the paediatric cardiology database at Chris Hani Baragwanath Academic Hospital (CHBAH) for January 1981 - April 2016. Results: A total of 15 patients with TOF and APVS were seen at CHBAH over the 34-year study period. TOF with APVS comprised 3% of all TOF patients. Ten (67%) patients presented before 1 year of age. The majority of patients (67%) had respiratory symptoms as their cause of presentation, of which 6 (40%) were infants. Fourteen (93%) patients were described to have the characteristic to-and-fro murmur at presentation. Five patients (33%) were suspected of having 22q11.2 deletion syndrome, but only 4 were tested and 2 were found to be positive. A third of patients underwent surgical intervention and 4 of the 15 patients (27%) were known to be alive at 10 years of age. Conclusions: TOF with APVS compromised 3% of all TOF patients, correlating with the literature. It is associated with the 22q11.2 deletion syndrome and was suspected in 33% of patients. TOF with APVS should be strongly considered in a child less than 1 year presenting with respiratory symptoms and a characteristic to-andfro murmur.

Coronary artery fi stulas in children: Experience from a Southern African tertiary care centre complex

Coronary artery fi stulas (CAF) are unusual coronary artery connections with low pressure cardiac chambers or vessels. The majority are congenital, but can also be acquired. Complications include heart failure, myocardial infarction and arrhythmias. Symptomatic and large CAF require treatment and options include surgical ligation or percutaneous device embolisation of the fi stula which has emerged as a less invasive and equally effi cacious management modality. Careful interrogation of the CAF is required prior to occlusion in order not to compromise normal coronary artery vasculature that may arise from the fi stula which can lead to myocardial ischaemia and infarction. Several reported cases highlight thrombus formation within large CAF after surgical ligation with propagation of the thrombus into coronary vessels arising proximally, resulting in myocardial compromise. We present a series of 6 children with CAF, 2 were treated by percutaneous embolisation (one developed a myocardial infarction post procedure) and 3 were treated surgically.