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Washington University Experience | VASCULAR | Herniation, uncal | 10A0 Case 10 History
Case 10 History ---- The patient was a 22 year old woman without significant past medical history. On 08/22, she delivered her second child at an outside institution, with complications of chorioamnionitis, endometritis, and associated fever (temperature of 101.7o F). On 08/30, she presented to an outside hospital with headache and generalized seizures. CT, EEG and LP were all within normal limits. She was started on Dilantin and discharged on 09/02. On 09/22, she visited her primary care physician with complaints of sinus pressure, skin rash, and conjunctival erythema with tearing. It is unclear if the patient received doxycycline for suspected Rocky Mountain Spotted Fever. On 10/02 the patient was admitted to an outside hospital with fever, worsening rash, and progressively increasing liver function tests. Dilantin was discontinued, and the patient was started on Tegretol. At that time, a head CT which was read as normal with suspected mastoiditis on the left. Abdominal ultrasound revealed a thickened gallbladder wall. On 10/07 the patient was admitted to BJH with a clinical diagnosis of submassive fulminant hepatic failure. Upon admission, the patient was jaundiced, had diffuse fine petechiae, a blanching rash, and trace vaginal blood. She was noted to be agitated with mental status changes, and was alternatively described as comatose. It was felt that the acute hepatic failure was due to a hypersensitivity reaction, possibly due to Dilantin or Tegretol, less likely due to an infection. During the hospital course, head CT showed progressive cerebral edema, for which she was treated with steroids and mannitol. Electroencephalography identified multifocal seizures, which were treated with Propofol. By 10/08 the patient's INR had peaked at 14.22. She underwent an orthotopic liver transplant on 10/10. On 10/13 she was noted to be hypernatremic, with a serum sodium of 172, which was subsequently normalized over several days. On 10/14 MRI showed subacute bilateral medial occipital lobe and medial right temporal lobe infarcts, as well as diffuse hypoxic injury, although an unusual presentation of reversible posterior leukoencephalopathy could not be excluded. The patient’s hospital course was also complicated by a urinary tract infection with growth of Pseudomonas. Although she showed some clinical improvement for several days following her liver transplant, cerebral edema worsened near the end of her hospital course. The patient expired on 10/17. ---- Her autopsy findings included global hypoxic/ischemic damage, marked cerebral edema with uncal herniation, bilateral PCA infarcts, cerebellar tonsillar herniation, and secondary brainstem (Duret) hemorrhage with numerous Alzheimer type 2 astrocytes in cortex and basal ganglia as expected in hepatic encephalopathy.
