Etiology of Acute Deterioration in a Very Preterm Twin Infant

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An 11-day-old second-born twin infant boy presented with a sudden onset of pallor, mottling, and lethargy. He was born to a 27-year-old primigravida with an unremarkable twin pregnancy. She presented at 28 + 2 weeks gestation with threatened preterm labor. Before delivery, she received antenatal steroids and magnesium sulfate. A male infant with a birth weight of 1220 g (68th centile) and head circumference of 27.8 cm (91st centile) was born. He received delayed cord clamping, and Apgar scores were 8 and 9 at 1 and 5 minutes, respectively. He developed respiratory distress syndrome (Figure 1A), requiring short-term intubation and surfactant, with subsequent extubation to continuous positive airway pressure. Cranial ultrasonography (CUS) on day 7 detected a right-sided subependymal hemorrhage (Figure 2A).On day 11, he had sudden onset pallor and mottling followed by desaturations and apnea. He was reintubated and initiated on high-frequency oscillatory ventilation. The radiograph had diffuse pulmonary haziness (Figure 1B), and there was frank blood in the endotracheal tube, indicative of acute pulmonary hemorrhage. He received fluid resuscitation, packed red cells, fresh frozen plasma, vitamin K, and a tranexamic acid infusion.Hemodynamically significant patent ductus arteriosus (PDA)CoagulopathySepsisDisseminated intravascular coagulationHe underwent extensive investigation for acute clinical deterioration with pulmonary hemorrhage (Table 1).Based on laboratory findings with cerebrospinal fluid (CSF) examination, enteroviral meningitis was diagnosed, complicated with a hemodynamically significant PDA and syndrome of inappropriate antidiuretic hormone.After 72 hours of acute pulmonary bleeding, he developed multiple episodes of supraventricular tachycardia requiring vagal maneuvers and adenosine and esmolol infusion followed by propranolol maintenance. CUS on day 19 (30-week 5-day postmenstrual age [PMA]) showed a left-sided grade 3 intraventricular hemorrhage (Figure 2B), with a rapid progression of left-sided ventriculomegaly within a week (Figure 3). Temporizing measures such as lumbar punctures were done twice followed by a ventricular access device (Ommaya reservoir) placement. Despite multiple taps, the serial CUS showed persistent left ventricular dilatation (Figure 2C). Magnetic resonance imaging brain at 35-week 4-day PMA revealed the left lateral ventricle communicating with the third ventricle through the widened left foramen of Monroe. There were no signs of encephalomalacia or infarcts. The right lateral ventricle was normal sized and drained via the Ommaya reservoir; however, it was not in communication with the right foramen of Monroe because of linear synechia (Figure 2D). Subsequently, via ventriculoscopy, scar tissue was identified and removed to reestablish communications between lateral ventricles. Ommaya reservoir was replaced by a ventriculoperitoneal shunt, which eventually stabilized the ventricular sizes. He was discharged at 40 weeks PMA with a weight of 2950 g (seventh percentile) and a head circumference of 34 cm (21st percentile). He was in room air, gaining weight steadily, and breastfeeding ad lib. There were no sick contacts, and the twin had no symptoms.Meningitis is an important morbidity in neonates, with an incidence of 2.7 per 1000 neonatal intensive care unit (NICU) admissions.1 Bacterial etiology is more common than viral, and the distinction between the two depends on specific viral testing.2,3 With molecular diagnostic tests, the detection of viral meningitis has become easier and faster.4Enterovirus was identified as the most common nonbacterial pathogen causing late-onset meningitis in a large NICU cohort.1 Enteroviruses are nonenveloped RNA viruses belonging to the family of Picornaviridae and can present with febrile illnesses, respiratory tract infections, conjunctivitis, skin lesions, and multisystemic involvement.5Neonatal transmission of Enterovirus occurs by transplacental/vertical or postnatal route by respiratory droplets. Maternal isolation of infection is uncommon.6 Nosocomial-acquired infections have less morbidity and mortality as compared to vertically acquired infections.7 In our case, we could not determine the route of transmission. As he presented at 11 days, it leans toward a postnatal diagnosis, as vertical usually presents in the first week. Fever, lethargy, rash, irritability, apnea, vomiting, seizures, and loose stools are key presenting symptoms.6,8–15 The acute presentation with life-threatening pulmonary hemorrhage is infrequent. We speculated that the underlying prematurity, respiratory distress, and hemodynamically significant PDA were contributors.CSF findings could include absent pleocytosis to CSF leucocyte count > 100/mm3.13,16 There is high variability in CSF protein and glucose. We provide a summary of the findings from reported cases in Table 2. Polymerase chain reaction (PCR) nucleic acid amplification tests establish the diagnosis.2Enterovirus PCR has been recommended in cases of high suspicion of meningitis, sepsis-like disease, or fever without a source in infants under 3 months of age.17 Nearly two-thirds of neonates with enteroviral meningitis have a positive rectal swab, and one-fifth have a positive throat swab.6Severe enteroviral infection, as in our case, complicated by hepatitis, coagulopathy, and arrhythmia is less frequently associated with meningitis and often presents with sepsis-like illness with multiorgan dysfunction.8,16 In a systematic review of 237 severe cases, only 10% reported meningoencephalitis in both preterm (56%) and term (44%) infants with no sex predilection, and two-thirds presented in the first week of life. Key multiorgan dysfunctions were hepatitis (16%), myocarditis (4%), and central diabetes insipidus (4%).8 Detection of myocarditis with Enterovirus is ominous and is associated with the highest mortality rates of nearly 38%.8Management is usually supportive. There is a theoretical benefit of intravenous immunoglobulin (IVIG) replacement therapy because the severity of illness may be due to a lack of maternal antibody transfer. Institutional practice varies, as controlled studies have not demonstrated benefits in clinical outcomes, and we did not use IVIG in the current case.18 However, more recent retrospective studies have reported benefits of early IVIG therapy within 72 hours of onset in reducing mortality from nearly 50% in late treatment to 7% in early treatment in severe infections.19 More controlled studies are needed to clarify the role of IVIG.20 Antivirals have also been investigated and did not show benefits of improved survival or faster viral clearance.4Long-term neurological complications are uncommon, and white matter abnormalities on magnetic resonance imaging brain and cerebral palsy have been reported.6,8,10,21 Hydrocephalus requiring neurosurgical interventions is even more rare.6 In our case, grade 3 intraventricular hemorrhage was detected a week before hydrocephalus, raising a possibility of inappropriately triggered inflammatory response following both posthemorrhagic and postinfectious processes, leading to altered CSF dynamics, arachnoid granulation dysfunction, or ependymal scarring.At the neonatal follow-up visit at 2 weeks corrected age (CA), he was noted to be making appropriate developmental progress. Prechtl’s General Movement Assessment at 3 months CA was normal, with “fidgety movements,” and his motor optimality score was 24/28. The Hammersmith Infant Neurological examination at 6.5 months CA demonstrated a score of 67, which was within the optimal range for infants born very preterm. At 13 months CA, he crawled with a 4-point reciprocal crawl, pulling to stand, cruising along furniture, and taking steps with his hands held. He was babbling and using several word approximations, demonstrating gestures and following 1-step instructions. He is now 17 months CA and continues to be followed for his developme

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