Neonatal Stroke: A Case Series

Background: Neonatal stroke is an important complication to consider when assessing a newborn as it has the potential for chronic sequelae related to neurodevelopment. Injury to cerebral tissue occurs either by a disruption in arterial blood flow to the brain from a thrombus or embolism, also known as perinatal arterial ischemic stroke (PAIS), or from an interruption by a thrombus in a major cerebral vein, otherwise known as cerebral sinus venous thrombosis (CSVT). The incidence of PAIS is reported as 1 in 2,300 to 4,000 deliveries, while the much rarer neonatal CSVT is between 1 and 2.69 per 100,000 newborns. The risk factors for both involve a combination of maternal, fetal, and neonatal issues. Precipitating mechanisms for neonatal strokes include infection, trauma, arteriopathy/venopathy or primary thrombophilia. Nonspecific presentation such as hypotonia, lethargy, and apnea and the lack of clinical signs often results in a delayed diagnosis. Routine cranial ultrasonography may be normal, especially if a Doppler study is not included; MRI with MRA/MVR must be obtained to confirm the presence of an ischemic or hemorrhagic injury.

Objective: The objective of this study was to investigate the causes and clinical presentation of neonatal stroke, making distinctions between perinatal arterial ischemic stroke (PAIS) and venous stroke or cerebral sinus venous thrombosis (CSVT). The immediate neonatal outcomes are also explored in an effort to establish associations between the variety of ways these patients presented and the necessary follow-up after the diagnosis is assigned.

Methods: This was a retrospective chart review over three neonates that presented with signs neonatal stroke over 1 year (2014–2015). We first explore the case of a term male infant born to a 37-year-old with no known risk factors at 39.5 weeks' gestation (GA) via uncomplicated vaginal delivery and weighed 2610 g. Following admission to the nursery, the infant showed signs of feeding intolerance with episodes of emesis and shallow breathing. He had multiple hypoglycemic episodes. Thrombocytopenia was also evident. Tonic clonic movements lasting ~10 minutes were noted, leading to the administration of Phenobarbital and Ativan intravenously. Blood cultures and herpes PCR were obtained to assess for sepsis before starting ampicillin, gentamicin, and acyclovir. No clinical seizures were noted following the medications. An MRI showed evidence of an acute multifocal stroke involving the left posterior medial parietal and left occipital lobe. CT of the brain was negative for bleeding, midline shift, or fracture. The next patient is a 37.4 GA male infant weighing 3,480 g at birth. He was born via spontaneous vaginal delivery to a 24-year-old mother with no significant past medical history, limited prenatal care, and an unknown GBS status. Immediately following delivery, the infant had central apnea, resulting in positive pressure ventilation and chest compressions. He required prolonged mechanical ventilation. He developed hypotension, hyperthermia, tachycardia, and tachypnea. He was treated with broad spectrum antibiotics. EKG showed ST elevations and q waves consistent with inferior wall ischemia. Echocardiogram was essentially normal. Early after birth, noted to have posturing and was started on phenobarbital for presumed seizure activity but an EEG was negative. A head ultrasound was normal. However, an MRI confirmed the diagnosis of a perinatal stroke by showing abnormal signal intensity in the lower superior sagittal and bilateral transverse sinuses, indicating dural venous sinus thrombosis. A CT scan further illustrated the presence of a hyperdense region in the right occipital horn consistent with an intraventricular hemorrhage. Our final case is a preterm female infant born at 33 GA, weighing 1,720 g. The patient’s mother was a 23-year-old G1P0 admitted with severe preeclampsia and chest pain, for which she received one dose of magnesium sulfate. The patient proceeded to have prolonged decelerations, leading to an emergent C-section, under general anesthesia. At birth, the patient was hypotonic and cord blood gas showed severe metabolic acidosis. An echocardiogram demonstrated no evidence of an intracardiac thrombus. Head ultrasound was normal. An MRI done 12 days after delivery demonstrated a thrombus in the right transverse and straight sinuses, solidifying the diagnosis of CSVT. Four days later, repeat MRI/MRV revealed that the areas with previous thrombosis had started to re-canalize and showed no subsequent progression of the clot.

Results: All three of the patients presented here survived. They were discharged home on full enteral feeds and no oxygen requirements. Both of the term infants were transferred to a tertiary center for direct evaluation by a neurologist and hematologist and were eventually followed conservatively. Regarding the preterm baby with extensive CSVT, the possibility of a coagulation disorder was discussed with the hematologist. As a result, she was discharged home on low molecular weight heparin.

Conclusion/Significance: This is a descriptive study on the etiology, clinical presentation, management as well as necessary work up of neonatal stroke in a regional neonatal population. Trends and detailed incidence and prevalence are not available for this region, to our knowledge. Careful monitoring needs to be implemented for this rare outcome in the future. As the presentation of neonatal stroke is not the same in every infant, importance must be placed on identifying the potential risk factors and birth history of patients that have an increased likelihood of having this diagnosis. Our case series illustrated three different presentations of strokes in the perinatal period, further highlighting the need to keep CSVT and/or arterial bleeds high on the differential in an infant with similar signs and symptoms, regardless of gestational age and apparent lack of known risk factors. While the management of perinatal strokes is supportive, early recognition allows for the focus to be placed on treatment of underlying conditions and preventing further injury.

Fig. 1 Cavernous sinus venous thrombosis.