A Case to Consider…
A 2 ½ year-old male with a history of intrauterine growth restriction and small for gestational age is experiencing a several days history of increased thirst and urination at night. Except for occasional bouts of nausea and vomiting sometimes related to traveling in a car, he is otherwise healthy. While traveling with his parents on a long car trip (6-7 hours), he complained of abdominal pain and developed nausea and vomiting. He was given Dramamine without relief. He continued to have nausea, vomiting and dry heaving. After arriving home, he immediately vomited, slipped and fell in the bathroom, hitting the back of his head on the tile floor. No loss of consciousness was reported. He continued to complain of abdominal pain until he had a large bowel movement. Feeling somewhat better, he went to bed.
At approximately 01:30 a.m., he again complained of abdominal pain and while sitting on the toilet he developed a blank stare with right sided spasms of his hands. An ambulance was called, and he was transported to a community hospital in the next town. Upon arrival, he was found to have continued seizure like activity of the right side and was given a weight-appropriate dose of Ativan. He was then intubated for airway protection. Further assessment found him profoundly hypertensive in the 250/130 range. He was given Labetalol and Hydralazine with little benefit. A head CT scan was negative for an acute source of seizure activity. The critical care transport team was called for transfer to a pediatric hospital with neurology services 15 minutes away by air.
Transport Team Arrival
Upon arrival of the transport team, the patient was found intubated and having seizure like activity with accompanied severe hypertension. He was given Midazolam and a Diprivan infusion was initiated with resolution of seizure activity. Due to a very short transport time, he was given bolus doses of Esmolol with little improvement in hypertension. Upon arrival to the Pediatric Emergency Department (ED), he was started on Nicardipine and eventually admitted to the Pediatric Intensive Care Unit (PICU). An MRI of the head was consistent with Posterior Reversible Encephalopathy Syndrome (PRES).
What is Posterior Reversible Encephalopathy Syndrome (PRES)?
Posterior reversible encephalopathy syndrome (PRES) is an encephalopathy which is radiologically characterized by consistent MRI signals in the bilateral posterior regions of the cerebral hemispheres in the brain. Common clinical symptoms are headache, seizures, visual disturbance, and altered mental status. These are often found associated with severe hypertension which causes encephalopathy by one of two pathways. The first is increased cerebral vessel pressure secondary to the failure of auto regulation causing brain hyperperfusion. This leads to endothelial injury and vasogenic edema. The second is increased cerebral vessel vasoconstriction by auto regulation causing brain hypoperfusion leading to cerebral ischemia and vasogenic edema. Click on the following video / podcast to learn more about what we know about this disorder to date:
Interventions to control seizures and blood pressure to minimize the impact of these pathways should be implemented as soon as possible to avoid long term sequela. Once achieved, the return to baseline neurologic function should return in 24-48 hours.
Patient Disposition
Further patient evaluation found mid-aortic syndrome and renal artery stenosis as the cause of hypertension. The patient was transitioned to oral medications for blood pressure control and referred to vascular surgery for outpatient follow-up. His PRES began to resolve as his renal artery stenosis was definitively managed.
Take Home Points
- This should be at the top of your differentials list in any child with mental status changes and malignant hypertension
- Mitigating seizure activity and preserving brain function is our primary focus of acute management, followed by the management of blood pressure.
- Definitive management rests upon treating and reversing the cause (in this case, what was causing the malignant hypertension).
References
Gavrilovici, C., Miron, I., Voroneanu, L., Bădărau, S., & Stârcea, M. (2017). Posterior reversible encephalopathy syndrome in children with kidney disease. International urology and nephrology, 49(10), 1793-1800.
Ghali, M. G., Davanzo, J., Leo, M., & Rizk, E. (2019). Posterior reversible encephalopathy syndrome in pediatric patients: pathophysiology, diagnosis, and management. Leukemia & lymphoma, 60(10), 2365-2372.
Heo S, Cho HJ, Jeon IS. A case of posterior reversible encephalopathy syndrome in a child with myelodysplastic syndrome following allogenic bone marrow transplantation. Pediatr Hematol Oncol. 2010;27(1):59-64. doi:10.3109/08880010903420661 https://pubmed.ncbi.nlm.nih.gov/30281063/
Kamide, T., Tsutsui, T., Misaki, K., Sano, H., Mohri, M., Uchiyama, N., & Nakada, M. (2017). A pediatric case of reversible cerebral vasoconstriction syndrome with similar radiographic findings to posterior reversible encephalopathy syndrome. Pediatric Neurology, 71, 73-76.