Alejandro Fuerte, MD
Newborn neurovascular diseases are common and are responsible for permanent disability. Early diagnosis and correct management are important to decrease the morbidity and mortality of these diseases. Studies are currently underway to advance this field and achieve more effective therapies.
Based on highlighted articles, below I present a short review of the most common perinatal neurovascular diseases.
- Germinal matrix hemorrhage (GMH): The germinal matrix is vascular tissue. It rarely persists in newborns to term because it disappears by around 33 weeks. GMH is, therefore, more common in pre-term infants. Although it typically originates in the periventricular region, blood can invade the ventricular system. Risk factors include weight < 1.5 kg and gestational age < 34 weeks. The findings of transcranial Doppler ultrasound determine the severity of the hemorrhage. A study of bleeding diathesis should be included in the diagnosis. There is no treatment protocol, although some studies confirm that the administration of corticosteroids to the mother in situations of threatened pre-term birth reduces the incidence and mortality of GMH. Currently, the best strategy to reduce the incidence of GMH is the prevention of pre-term birth. As far as surgical treatment is concerned, ventricular lavage or ventriculostomy is usually performed. The prognosis is usually related to the severity of the hemorrhage and the appearance of complications such as periventricular leukomalacia (25-75% of cases) and hydrocephalus (11-30% of cases). For the treatment of hydrocephalus, CSF ventriculoperitoneal shunting is, in many cases, the best option, and it has been shown to improve long-term psychomotor development.
- Neonatal hypoxic ischemic encephalopathy (HIE): HIE occurs as a result of an abnormal exchange of gases during childbirth, resulting in a deficit of oxygen and glucose. It is suffered by 0.1-0.8% of newborns in developed countries. There are many risk factors involved, such as gestational age > 41 weeks, prolonged rupture of the membrane, abnormalities in the cardiotocography, meconium, shoulder dystocia, umbilical cord around the neck and ineffective use of the vacuum extractor. Diagnosis is established by detecting intrapartum metabolic acidosis in the presence of brain dysfunction. It is a serious pathology responsible for 20% of cases of cerebral palsy and whose mortality index ranges between 25-80% depending on the severity. The only treatment available is therapeutic hypothermia, which significantly reduces death and severe disability. Erythropoietin, melatonin, cannabinoid derivatives, and stem cells are currently being investigated. Magnesium sulphate is a promising therapy in developing countries because of its low cost.
- Perinatal stroke: Perinatal stroke refers to the presence of ischemic or hemorrhagic brain injury between 20 weeks of fetal life and postnatal day 28. In this type of stroke, it is essential to ask about family history, as well as to investigate risk factors during childbirth or diseases of the progenitor.
- Perinatal ischemic stroke: It can be arterial or venous (cerebral venous thrombosis). The incidence of perinatal arterial stroke is approximately 0.006 – 0.043% among live newborns. The incidence of perinatal venous thrombosis is 0.003%. The most frequent onset is seizure. There are numerous risk factors involved (systemic disease, congenital heart disease, placental pathology, infection). Diagnosis should include brain imaging, vessel studies, heart tests, and thrombophilia testing; cerebrospinal fluid analysis and genetic testing are less frequent diagnostic tests. The reported incidence of cerebral palsy in infants with arterial ischemic stroke is 26-68%. In terms of treatment, intravenous fibrinolysis and mechanical thrombectomy are not indicated due to the frailty of the blood vessels. Prevention with antiplatelet and anticoagulant agents is not routinely recommended due to the low risk of recurrence. Venous thrombosis is treated with anticoagulants.
- Perinatal hemorrhagic stroke (Intraparenchymal hemorrhage): The incidence of hemorrhagic stroke is approximately 0.0009 – 0.015% among live newborns. Although associated with several risk factors, like vascular malformations and bleeding diatheses like coagulopathy and thrombocytopenia, most perinatal hemorrhages are idiopathic. Encephalopathy is the most frequent clinical debut. The mortality rate is around 20%. The main medical treatment aims to correct thrombopenia and/or coagulation factor deficiencies and to administer vitamin K. However, surgery is still necessary in many of these cases.
I would like to thank Dr. Irene Alonso and Dr. Cristina Duque (pediatrician and neonatologist, respectively) for helping me on this blog post.