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Washington University Experience | VASCULAR | Herniation, tonsillar | 4A0 Case 4 History
Case 4 History ---- The patient was a 7 year old female who was in her usual state of health until approximately 2 1/2 weeks prior to admission, at which time she developed fever as well as anorexia. She was treated as an outpatient with a seven day course of Bactrim for presumed otitis media. She continued to have symptoms and the fever recurred following completion of the course of antibiotics. She developed diarrhea, dehydration, and was seen at a local hospital on 7/1. At that time, she had pancytopenia and was transferred to SLCH. She also had a history of tick exposure, several of which had been removed from her body over the past month. At the time of transfer to SLCH, she was irritable without neck rigidity. Mild periorbital edema was noted at that time. Approximately five hours after admission, she had a seizure. Head CT was performed and was negative. She continued to have a progressive coagulopathy and developed DIC. Over the course of 7/2 her neurologic status deteriorated to coma with extensor posturing of the left leg. A repeat head CT demonstrated diffuse cerebral edema. A general work-up demonstrated PCR positive for Ehrlichia. In addition, a bone marrow biopsy was performed and showed large histiocytes consistent with a hemophagocytic syndrome secondary to infection. Her hemoglobin decreased from 9.3 to 1.1 over the course of 7-2 and she had a decrease in respiratory status as well as an increase in generalized edema. Her pupils became unresponsive. Early in the morning on 7/3 her left pupil became dilated to approximately 6 mm, while her right remained pinpoint. At that time she had no evidence of brainstem function. An emergent head CT revealed a diffuse bilateral cortical signal changes with almost complete obliteration of the basilar cisterns. She also had evidence of autonomic instability with a temperature at 92°F. She developed a Pseudomonas superinfection. Progressive, global CNS damage developed which was irreversible in nature and, following discussion with the family, aggressive treatment was discontinued and the patient was pronounced dead on 7/3. ---- At autopsy the brain weighed 1430 grams with findings which support CNS Ehrlichiosis (PCR positive for Ehrlichia chaffeensis) whose organisms were seen by Giemsa stain in CNS macrophages. This patient's extensive cerebral edema is consistent with her clinical course and likely secondary not only to her Ehrlichia infection but also the superimposed Pseudomonas infection.
