Hematology/Oncology and Neurosurgery services were consulted immediately. A central line was placed without complications. The patient was treated with factor IX 80 units/kg once, then received 40 units/kg every 12 hours. He was also transfused with 1 unit of packed red blood cells. His PT and PTT were monitored every 12 hours.
Neurosurgical recommendations included correcting the patient's factor level prior to any surgical intervention and treatment with decadron, to decrease cerebral edema. Intracranial pressure was managed by keeping the head of the patient's bed elevated at 30°, with the head midline. He was kept mildly hypothermic; ventilation and oxygenation were maintained through mechanical ventilation. Sedation and analgesia were achieved with continuous infusions of midazolam and fentanyl.
A repeat CT scan was done the following day; no significant change from the previous scan was seen. A transcranial Doppler scan was done to rule out vasculopathy related to Hb SS. The Doppler evaluation was within normal limits. This confirmed that the spontaneous hemorrhage was secondary to hemophilia, rather than to Hb SS.
On hospital day 2, the patient was taken to the operating room where a craniotomy was performed, with evacuation of the intracranial hemorrhage in the left frontal temporal area. Approximately 40 cc of blood was evacuated.
On postoperative day 1, the patient developed bilateral pleural effusions, with bilateral lower lobe atelectasis/pneumonia. In light of his Hb SS disease, imaging was consistent with acute chest syndrome. The patient was treated with an exchange transfusion. On postoperative day 2, he was extubated without difficulty.
The patient was subsequently transferred to the pediatric ward in stable condition. He was treated for pneumonia with clindamycin and vancomycin for 10 days. While in the hospital, he received physical therapy. Prior to discharge, the patient had resumed normal activity and speech. As an outpatient, he continued to receive prophylaxis with factor IX twice a week.
Medscape Pediatrics. 2006;8(2) © 2006
Cite this: A 2-Year-Old With Intracranial Hemorrhage - Medscape - Aug 07, 2006.