ABSTRACT. A 3.5-year-old healthy boy with 4 days of fever was referred to the emergency department for respiratory distress. The physical examination was remarkable for stupor, tachycardia, tachypnea, and dyspnea. Initial blood tests showed pancytopenia. He rapidly developed torticollis. I n 1936, Lemierre described a syndrome that consisted of septicemia with an anaerobic agent (later defined as Fusobacterium necrophorum), thrombophlebitis of cervical veins, and distal metastatic abscesses, developed several days after an infection of the pharynx and tonsils. 1 Since then, several dozen case reports have been published. [2][3][4][5][6][7][8] Most reported cases are in adolescents and young adults. Only a few patients under the age of 10 years have been reported. 2 We report a 3.5-year-old boy who presented with fever and torticollis and received a diagnosis of Lemierre's syndrome (LS). Screening for thrombophilia revealed two risk factors for hypercoagulability.
CASE PRESENTATIONA 3.5-year-old healthy boy with fever up to 39°C for 4 days presented to our emergency department (ED) with fever, respiratory distress, and mental status changes. He was examined twice by pediatricians in his local clinic and received a diagnosis of having a viral infection. On the evening before admission, he refused to move his neck. On the morning of admission, the child was in respiratory distress and was referred to our ED by his pediatrician.On arrival in the ED, he was stuporous, rectal temperature was 39.3°C, heart rate was 145 beats per minute (bpm), blood pressure was 88/39 mm Hg, respiratory rate was 60 bpm, and oxygen saturation was 100% on 10 L/minute of oxygen delivered via a mask with reservoir. On physical examination, he had intercostal retractions and was grunting. He had mild neck rigidity, pharynx and tonsils were without erythema or exudate, a 3/6 systolic ejection murmur was heard at the left sternal border, there was good air entry bilaterally, and he had strong peripheral pulses. His liver span was 14 cm, there was a palpable spleen tip, and he had a purulent discharge from his right ear.The child was volume resuscitated with 40 mL/kg of normal saline and treated with 100 mg/kg of Ceftriaxone after blood, urine, and cerebrospinal fluid (CSF) cultures were obtained. Blood tests revealed pancytopenia, white blood cell (WBC) count of 2300/ L, hemoglobin of 9.4 g/dL, and platelet count of 94 000/ L. Erythrocyte sedimentation rate was 110 mm in the first hour. CSF tests showed mild pleocytosis with 16 WBC/ L, no red blood cells, glucose of 86 mg%, and protein of Ͻ10 mg%. No bacteria were seen on Gram stain. Urine examination by dipstick was negative.Chest radiograph revealed mild cardiac enlargement. Echocardiography demonstrated normal cardiac anatomy with a shortening fraction of 36%. He rapidly developed torticollis, and computerized tomography (CT) to rule out a parapharyngeal abscess was performed. The CT demonstrated a 12-mm thrombus in the right internal jugular vein with an adjoining inflammatory mass (Fig 1)...