Lemierre syndrome is an extremely rare complication of mild-to-moderate pharyngeal infections. The authors present an unusual case of Lemierre syndrome in a 16-year-old boy with cavernous sinus thrombosis and right internal carotid artery narrowing without neurological sequelae, right subdural empyema, and cerebritis in the right temporal and occipital lobes. Neuroimaging also demonstrated right jugular vein thrombosis. Cultures of samples from the blood proved positive for the presence of Fusobacterium necrophorum. The patient underwent unilateral tonsillectomy, drainage of the peritonsillar abscess, and a myringotomy on the right side. Postoperatively the patient was treated conservatively with antibiotic therapy resulting in an excellent outcome.

Patients with congenital central hypoventilation syndrome (CCHS) (Ondine's curse syndrome) have impaired autonomic control of ventilation with intact voluntary control of respiration. Autonomic dysfunction and cardiac abnormalities are common in CCHS. Bradyarrhythmias are life-threatening and often require pacemaker insertion. We presented a case of a patient with CCHS suffering from long sinus pauses requiring cardiac pacemaker insertion. Patients with CCHS are at risk for pulmonary hypertension and cor pulmonale secondary to chronic hypoxia. Diaphragmatic pacing has been beneficial in some patients with CCHS. In this article, we review concomitant cardiac abnormalities and the occurrence of bradyarrhythmias in patients with CCHS.

To investigate the value of Doppler ultrasonography of the carotid arteries as a diagnostic test for the determination of brain death in children, we enrolled 17 patients in a blinded fashion in the pediatric intensive care unit of Memorial Miller Children's Hospital of Long Beach between the period of December 1990 and October 1992. After institutional review board approval and parental consent, children who sustained severe brain injury underwent Doppler ultrasonography study of their carotid arteries. Seven of 17 patients were diagnosed as having brain death by clinical criteria (complete loss of cerebral and brainstem functions) and electroencephalogram (EEG). Five of seven (71%) patients with the diagnosis of brain death had bilateral reverse flow (characteristic of increased cerebrovascular resistance and absent cerebral circulation) on their Doppler ultrasonography, yielding a specificity of 100% and sensitivity of 71.4% (P = 0.01). All surviving patients (five) and the five who did not fulfill the brain-death criteria at the time of Doppler ultrasonography and were later taken off life supportive measures had normal Doppler findings. These data indicate that Doppler ultrasonography of the carotid arteries is a very specific test and can be used as an adjunctive modality for determination of brain death in children.

We report herein a case of Factor XIII deficiency that remained undiagnosed until 2 years of age. Part of the delay in diagnosis was a consequence of testing that was performed on a blood sample obtained after plasma transfusion therapy for a life-threatening bleeding episode. Due to insufficient family follow-up after discharge from the hospital, the diagnosis was delayed 1 year until the child was rehospitalized and a pre-transfusion plasma sample was tested. The commonly accepted approach of using only a qualitative test for the diagnosis of factor XIII deficiency is challenged by this case report. Am. J. Hematol. 71:328-330, 2002.

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