A 2-day-old girl develops seizures and a bulging anterior fontanelle. Temperature is 36.8 C (98.2 F), pulse is 180/min, and capillary refill is 4 seconds. Cardiac examination reveals tachycardia. Neurologic examination shows rhythmic contractions of the upper extremities, tongue thrusting, and lip smacking. Head ultrasound is shown below: Which of the following conditions most likely led to this patient's condition?

Question

A 2-day-old girl develops seizures and a bulging anterior fontanelle.  Temperature is 36.8 C (98.2 F), pulse is 180/min, and capillary refill is >4 seconds.  Cardiac examination reveals tachycardia.  Neurologic examination shows rhythmic contractions of the upper extremities, tongue thrusting, and lip smacking.  Head ultrasound is shown below:  Which of the following conditions most likely led to this patient's condition?

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11ec25c8_d6f4_8a3c_bc81_df997de8526d_MD0003_00 This newborn's presentation and ultrasound findings are consistent with an intraventricular hemorrhage (IVH) due to ruptured germinal matrix vessels. The germinal matrix is a fetal structure that eventually gives rise to neurons; it contains fragile vasculature that is prone to rupture with acute changes in cerebral perfusion (eg, hypotension, hypo- or hyperventilation, increased venous pressure). Involution of the germinal matrix begins at gestational age 32 weeks; therefore, prematurity is the primary risk factor for IVH. IVH typically occurs within the first few days of life and can present with anemia, tachycardia, and a bulging fontanelle due to hemorrhage. Nonspecific signs such as apnea, hypotonia, and decreased movements are also common, and seizures occur in a minority of cases. However, newborns with IVH are often asymptomatic. Cranial ultrasound is diagnostic; the hemorrhage can be limited to the germinal matrix or enter the lateral ventricles, as seen on this patient's ultrasound. Treatment is generally supportive (eg, seizure management, oxygenation/ventilation, nutrition). Long-term sequelae include neurodevelopmental conditions such as cerebral palsy and intellectual disability. ultrasound (Choice A) Brain arteriovenous malformations (AVM) cause shunting of oxygenated blood directly to draining veins, resulting in ischemic changes to the parenchyma bypassed by the malformation. Hemorrhage due to an AVM can occur with increasing age, but prematurity is the most likely cause of IVH in the neonatal period. Brain arteriovenous malformations (Choice B) A ruptured berry aneurysm is very rare in newborns and would result in subarachnoid, not intraventricular, bleeding. (Choice C) Congenital infections are often associated with CNS abnormalities, such as intracranial calcifications (eg, Toxoplasma gondii, cytomegalovirus) and ventriculomegaly (eg, Zika virus). Congenital infections are not associated with IVH. (Choice D) Placental abruption can be associated with hypoxic ischemic encephalopathy, which may show cerebral edema or white matter damage on ultrasound. Abruption is uncommonly associated with IVH. Educational objective: The primary risk factor for intraventricular hemorrhage is prematurity due to fragile germinal matrix vessels. Symptomatic newborns may have apnea, seizures, and a bulging fontanelle as well as signs of acute blood loss (eg, anemia, tachycardia). Cranial ultrasound is diagnostic. References: Intraventricular hemorrhage and neurodevelopmental outcomes in extreme preterm infants. (http://www.ncbi.nlm.nih.gov/pubmed/24379238) Pathogenesis and prevention of intraventricular hemorrhage. (http://www.ncbi.nlm.nih.gov/pubmed/24524446)

A 2-Day-Old Girl Develops Seizures and a Bulging Anterior Fontanelle