Pediatric Lemierre’s Syndrome: A Comprehensive Literature Review

Author:

Lavalle Salvatore1,Masiello Edoardo2,Cocuzza Salvatore3ORCID,Pavone Piero4ORCID,Di Nora Alessandra4ORCID,Calvo-Henriquez Christian56ORCID,Lechien Jerome Rene67ORCID,Yanez Miguel Mayo68ORCID,Praticò Andrea1,Ceccarelli Manuela1ORCID,Iannella Giannicola9ORCID,Pace Annalisa9,Parisi Federica Maria3,Magliulo Giuseppe9ORCID,Maniaci Antonino16ORCID

Affiliation:

1. Faculty of Medicine and Surgery, University of Enna Kore, 94100 Enna, Italy

2. Radiology Unit, Department Clinical and Experimental, Experimental Imaging Center, Vita-Salute San Raffaele University, 20132 Milan, Italy

3. Department of Medical, Surgical Sciences and Advanced Technologies “GF Ingrassia” ENT Section, University of Catania, 95123 Catania, Italy

4. Department of Child and Experimental Medicine, Section of Pediatrics and Child Neuropsychiatry, University of Catania, 95100 Catania, Italy

5. Service of Otolaryngology, Hospital Complex of Santiago de Compostela, 15701 Santiago de Compostela, Spain

6. Head and Neck Study Group of Young-Otolaryngologists of the International Federations of Oto-Rhino-Laryngological Societies (YO-IFOS), 75000 Paris, France

7. Department of Anatomy and Experimental Oncology, Mons School of Medicine, University of MONS, 7022 Mons, Belgium

8. Department of Otorhinolaryngology-Head and Neck Surgery, Complexo Hospitalario Universitario A Coruña (CHUAC), 15006 A Coruña, Spain

9. Department of ‘Organi di Senso’, University “Sapienza”, Viale dell’Università, 33, 00185 Rome, Italy

Abstract

Background: Lemierre syndrome is a rare, potentially fatal complication of oropharyngeal infections characterized by septic thrombophlebitis of the internal jugular vein. It primarily affects healthy adolescents and young adults. Its incidence declined after the antibiotic era, but it may have resurged in recent decades, likely due to judicious antibiotic use and increasing bacterial resistance. Prompt diagnosis and treatment are imperative to prevent significant morbidity and mortality. Methods: Lemierre syndrome has been called “the forgotten disease,” with a reported incidence of around 3.6 cases per million. The mean age at presentation is around 20 years old, though it can occur at any age. Lemierre Syndrome follows an oropharyngeal infection, most commonly pharyngitis, leading to septic thrombophlebitis of the internal jugular vein. F. necrophorum is the classic pathogen, though other organisms are being increasingly isolated. Metastatic infections, especially pulmonary, are common complications. Contrast-enhanced CT of the neck confirming internal jugular vein thrombosis is the gold standard for diagnosis. Long-course broad-spectrum IV antibiotics covering anaerobes are the mainstays of the disease’s treatment. Anticoagulation may also be considered. Mortality rates are high without treatment, but most patients recover fully with appropriate therapy. Conclusions: Lemierre syndrome should be suspected in patients with prolonged pharyngitis followed by unilateral neck swelling and fevers. Early diagnosis and prompt antibiotic therapy are key, given the potential for disastrous outcomes if untreated. An increased awareness of Lemierre syndrome facilitates its timely management.

Publisher

MDPI AG

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