To the Editors:

Septic embolism is a pathologic process most frequently associated with infective endocarditis (IE),1 endogenous bacterial endophthalmitis is the result of septic embolization to the eye, manifesting as either conjunctivitis, uveitis, iritis or hypopyon, and tends to occur in older patients with comorbid conditions,2,3 being extremely rare in the pediatric age.4,5 Nonetheless, while IE is also very rare in the pediatric age (0.43 per 100,000),6 higher rates are seen in children with congenital heart diseases (cumulative rate under 18 years of 0.6%),7 particularly in children/young adults with Marfan syndrome, in which mitral valve IE is found in 2.5% of patients, according to Rybczynski et al.8

A 14-year-old girl with Marfan syndrome and mitral valve prolapse medicated with losartan, presented to the pediatrics emergency department with a 5-day history of vomits, fever and vision changes. On initial physical examination the girl was drowsy, hypotensive, and had a palmar-plantar purpuric petechial exanthema. Blood tests revealed normal hemoglobin (13.0 g/dL) and leucocytes (7.10 × 10 E3/μL, with 86% neutrophils), thrombocytopenia (51,000 × 10 E3/μL), elevated C-reactive protein (452.7 mg/L), elevated d-dimers (20,797 ng/mL) and acute kidney injury (urea 136 mg/dL, creatinine 1.29 mg/dL and GFR 50 mL/min/1.73 m2). She was immediately admitted to the pediatric intensive care unit where she was stabilized (3 × 10 mL/Kg of NaCl 0.9% and noradrenaline 0.1 mcg/Kg/min perfusion), and treated with broad-spectrum antibiotics. Initial transthoracic echocardiography (TTE) investigation was inconclusive. One day after admission, a milky white fluid level in the inferior part of the anterior chamber (Fig. 1) was noticed. Considering the ocular changes, a hypopyon in the context of septic embolism from IE was assumed. At this stage, Angio-CT was performed, revealing several spleen and liver emboli and right pleural effusion, confirming a septic embolization process.

FIGURE 1.:

Milky white fluid level in the inferior part of the anterior chamber.

Although the preferred method for IE investigation is TTE, it can miss valvular vegetation at an early stage. Therefore, repeated TTE, or more invasive transesophageal echocardiography,9 associated with a high index of suspicion concerning septic embolization, is essential for the diagnosis. In this case, multiple TTEs were performed, and on day 2 a vegetation on the mitral valve was finally observed, confirming IE. On day 3, a methicillin-sensitive Staphylococcus aureus was isolated on blood cultures (consistent with previous report showing that Staphylococcus species were most commonly identified)6 and antibiotics were adjusted. The girl was subsequently transferred to a Pediatric Cardiac Care Center where she was submitted to cardiac surgery for removal of vegetations and mitral valve prothesis implantation. One week after surgery, an asymmetric tetraparesis (worse on the right side) was detected and a cerebral angiography revealed a mycotic aneurism that was subsequently treated by endovascular embolization. She is currently undergoing a rehabilitation program with partial recovery of neurological deficits.

In conclusion, although highly rare, the hypopyon leads us to consider IE and septic embolization to the eye and other organs. This allowed us to develop a more directed treatment for this patient. We highlight the necessity for a high index of suspicion of IE in patients with cardiac comorbid conditions.

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