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Society for Pediatric Radiology – Poster Archive

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Ibe Donald,  Navallas Irujo Maria,  Aquino Michael

Final Pr. ID: Poster #: CR-001

Lemierre’s syndrome is an extremely rare condition characterized by initial oropharyngeal infection with development of septic thrombophlebitis and subsequently disseminated septic microemboli. The syndrome remains a disease of considerable morbidity and mortality. The incidence is approximately 3.6 cases per 1 million per year. It is commonly caused by gram-negative Fusobacterium necrophorum. However, less than a third of cases is brought on by other anaerobic bacteria. Here we present a rare case of a Lemierre’s syndrome in a child caused by methicillin-resistant Staphylococcus aureus (MRSA).

A 4-year old male presented to emergency department with unremitting fever, progressive painful submandibular swelling concerning for Ludwig’s angina, vesicular lesions on the skin, and decreased level of consciousness. The patient was reported to have fallen onto his chin with resultant lip laceration and tooth avulsion. Fever and neck swelling developed two days later. Computed tomography (CT) of the neck revealed findings in keeping with clinically suspected Ludwig’s angina including: soft tissue gas, and diffuse fat stranding involving the sublingual, perioral, and right submandibular spaces with extension to right sternoclavicular muscle, carotid and jugular vessels. No abscess was identified but a focal non-occlusive thrombus was seen in the right internal jugular vein. Additionally, the lung apices demonstrated multiple patchy densities raising concern for Lemierre’s syndrome and prompting further evaluation with a contrast-enhanced chest CT. Chest CT confirmed the diagnosis demonstrating multiple, variable-sized, randomly distributed lung nodules with cavitation, and multifocal consolidation consistent with septic emboli. Blood culture and skin swab of vesicular lesions were positive for MRSA. On further discussion, it was revealed that the patient’s father was recently treated for MRSA abscess. The patient was placed on intravenous antibiotics (vancomycin, rifampin, meropenem) and anticoagulants (tinazaparin) with improvements in symptoms and imaging findings within six weeks post admission.

The learning points include:
1) the need to critically evaluate lung apices and vasculature on neck CT in patients with evidence of soft tissue neck/oropharyngeal infection
2) despite the rarity of Lemierre’s syndrome, multiple cases caused by MRSA have been described.
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Authors:  Ibe Donald , Navallas Irujo Maria , Aquino Michael

Keywords:  SEPTIC EMBOLI, THROMBOPHLEBITIS, ludwig's angina

Gagnon Marie-helene,  Richer Edward,  Alazraki Adina

Final Pr. ID: Poster #: CR-030

A 16 year old previously healthymale patient who initially presented with daily recurrent fevers and chills lasting 5-10 minutes. His parents brought him to a tertiary care hospital after developing vomiting and weight loss in the setting of progressively worsening fevers. Initial work up was remarkable for elevated CRP and a negative infectious workup. An MR enterography ordered to evaluate for a cause of weight loss demonstrated incidental lung base lesions. Follow up CTA revealed a lesion within the right ventricle and multiple lung nodules, concerning for endocarditis and septic emboli. He underwent two surgical resections over two months for the ventricular mass. Pathology revealed findings consistent with necrotizing endocarditis. During his hospital course, he was found to have a left cephalic venous thrombosis and was placed on Xarelto, later developing a right subclavian vein thrombus while on anticoagulation. He was readmitted 1 month later after presenting for right chest and right upper quadrant pain. Initial chest X-ray revealed right lower lobe mass and follow up CTA revealed two large right pulmonary artery pseudo-aneurysms. He underwent cardiac catheterization for pre-operative coiling of the pseudo-aneurysms and subsequent right lower lobectomy. Pathology revealed necrotizing arteritis and pulmonary artery pseudo-aneurysms. Based on clinical presentation and further work up, he was diagnosed with Hughes Stovin Syndrome and started on Cytoxan and Apixaban. While not yet considered in remission, his symptoms have improved since initiating treatment.

Hughes Stovin Syndrome (HSS) is a rare disorder characterized by thrombophlebitis as well as multiple pulmonary and/or bronchial aneurysms. There is a male predilection, usually presenting between 12-48 years. There have been less than 40 cases published in the English literature. HSS is thought to be a variant of Bechet’s but does not present with oral or genital ulcers. The lack oral and genital ulcers in this patient excluded a diagnosis of Bechet’s. The underlying cause of HSS is unclear but is believed to be due to angiodysplasia or infection. Since there is no formally described diagnostic criteria, the clinical presentation of thrombophlebitis with pulmonary artery aneurysms characterize the disease.

We present this case to illustrate the clinical presentation of a rare and possibly under recognized syndrome.
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Authors:  Gagnon Marie-helene , Richer Edward , Alazraki Adina

Keywords:  Vasculitis, Aneurysm, Thrombophlebitis