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Background: Post-hemorrhagic hydrocephalus (PHH), a common complication of severe intraventricular hemorrhage (IVH) in very low birth weight (BW) infants, is associated with significant morbidity and poor neurological outcomes. The objective of this study was to assess the current status of PHH and analyze the risk factors associated with the necessity of treatment for PHH in infants born between 22 and 28 weeks of gestation, specifically those with severe IVH (grade 3 or 4)., Methods: The analysis was conducted on 1,097 infants who were born between 22-28 gestational weeks and diagnosed with severe IVH, using data from the Korean Neonatal Network. We observed that the prevalence of PHH requiring treatment was 46.3% in infants with severe IVH., Results: Higher rates of mortality, transfer during admission, cerebral palsy, and ventriculoperitoneal shunt after discharge were higher in infants with PHH than in those without PHH. PHH in severe IVH was associated with a higher rate of pulmonary hemorrhage, seizures, and IVH grade 4 in the entire cohort. In addition, it was associated with a lower rate of small for gestational age and chorioamnionitis. In the subgroup analysis, high BW, outborn status, pulmonary hemorrhage, seizure, sepsis, and IVH grade 4 were associated with a higher incidence of PHH between 22 and 25 gestational weeks (GW). In infants born between 26 and 28 GW, a higher incidence of PHH was associated with seizures and IVH grade 4., Conclusion: It is necessary to maintain meticulous monitoring and neurological intervention for infants with PHH not only during admission but also after discharge. In addition, identifying the clinical factors that increase the likelihood of developing PHH from severe IVH is crucial., Competing Interests: The authors have no potential conflicts of interest to disclose., (© 2024 The Korean Academy of Medical Sciences.)

Objective: Recent evidence suggests that the survival of peri-viable infants with birth weight (BW) ≤500 g could be improved with better care practices in the neonatal intensive care unit (NICU). This study aimed to investigate the care quality level of NICU-dependent variations in the survival rate of infants with BW ≤500 g., Methods: To determine the quality of NICU care-dependent variations in the survival rate, 226 eligible infants of BW ≤500 g and ≥22 weeks gestation registered in the Korean Neonatal Network between 2013 and 2017 were grouped according to the survival rates of infants at 23-24 weeks gestation, reflecting the care quality level of each NICU as group I (≥50%, n = 107) and group II (<50%, n = 119)., Results: The survival rate of group I infants (40.2%, 43/107) was significantly higher than that of group II infants (14.3%, 17/119). Significantly reduced deaths from birth to the age of 7 days due to cardiorespiratory causes were the primary contributors to improved survival. In multivariable Cox hazard model analyses, besides the gestational age and BW, antenatal steroid use, cesarean section, pH, and base excess at admission were associated with improved infant survival., Conclusions: The survival rate of pre-viable infants with BW ≤500 g could be improved by providing better NICU quality care practices, including better cardiorespiratory management starting from delivery room resuscitation., (© 2022 The Author(s). Published by S. Karger AG, Basel.)

Objective: To determine the outcomes of very low birth weight infants (VLBWIs) following maternal mid-trimester prolonged preterm premature rupture of membranes (PPROM) and subsequent early pulmonary hypertension (PH)., Design: Prospective cohort study., Setting: A nationwide web-based registry of VLBWIs from 67 neonatal intensive care units., Patients: VLBWIs registered on the Korean Neonatal Network and born between 23 and 34 gestational weeks., Methods: VLBWIs exposed to maternal PPROM prior to 25 gestational weeks and lasting ≥7 days (PPROM25, n  = 402) were matched 1:1 with infants not exposed or exposed within 24 h to PPROM (CON, n  = 402), using propensity score matching. The PPROM25 group was subdivided into PPROM25 groups with or without early PH, defined as exposure to inhaled nitric oxide or other pulmonary vasodilators to treat PH within 3 days of life. Clinical variables and major outcomes were compared, and risk factors for mortality and morbidities were analyzed., Results: Of 1790 infants with maternal PPROM, the PPROM25 group comprised 402 (22.5%) infants. Survival rates were similar between the CON and PPROM25 groups (71.6% vs 74.4%); however, the incidence of bronchopulmonary dysplasia (BPD) differed (47.8% and 60.2%, p  < .05). Infants in the PPROM25 group with early PH had higher mortality (55.6%) and more severe intraventricular hemorrhage (IVH) (31.7%) than infants in the PPROM25 group without early PH (21.9% and 14.3%, respectively; p  < .05). In multivariate analysis, lower 5 min Apgar score and the presence of oligohydramnios increased the risk of development of early PH. The presence of PPROM25 was founded to be a significant risk factor for BPD and early PH in relation to mortality and severe IVH, respectively., Conclusions: In VLBWIs, prolonged exposure to maternal mid-trimester PPROM increased the risk of BPD. Subsequent early PH immediately after birth increased mortality and severe IVH, thus, requires special attention.

Background: Although the overall quality of high-risk neonatal care has improved recently, there is still concern about a difference in the quality of care when comparing off-hour births and regular-hour births. Moreover, there are no data in Korea regarding the impact of time of birth on mortality and morbidities in preterm infants., Methods: A total of 3,220 infants weighing < 1,000 g and born at 23-34 weeks in 2013-2017 were analyzed based on the Korean Neonatal Network data. Mortality and major morbidities were analyzed using logistic regression according to time of birth during off-hours (nighttime, weekend, and holiday) and regular hours. The institutes were sub-grouped into hospital group I and hospital group II based on the neonatal intensive care unit (NICU) care level defined by the mortality rates of < 50% and ≥ 50%, respectively, in infants born at 23-24 weeks' gestation., Results: The number of births during regular hours and off-hours was similar. In the total population and hospital group I, off-hour births were not associated with increased neonatal mortality and morbidities. However, in hospital group II, increased early mortality was found in the off-hour births when compared to regular-hour births., Conclusion: Efforts to improve the overall quality of NICU are required to lower the early mortality rate in off-hour births. Also, other sensitive indexes for the evaluation of quality of NICU care should be further studied., Competing Interests: The authors have no potential conflicts of interest to disclose., (© 2021 The Korean Academy of Medical Sciences.)

Background: To investigate the incidence of surgical intervention in very low birth weight (VLBW) infants and the impact of surgery on neurodevelopmental outcomes at corrected ages (CAs) of 18-24 months, using data from the Korean Neonatal Network (KNN)., Methods: Data from 7,885 VLBW infants who were born and registered with the KNN between 2013 to 2016 were analyzed in this study. The incidences of various surgical interventions and related morbidities were analyzed. Long-term neurodevelopmental outcomes at CAs of 18-24 months were compared between infants (born during 2013 to 2015, n = 3,777) with and without surgery., Results: A total of 1,509 out of 7,885 (19.1%) infants received surgical interventions during neonatal intensive care unit (NICU) hospitalization. Surgical ligation of patent ductus arteriosus (n = 840) was most frequently performed, followed by laser therapy for retinopathy of prematurity and laparotomy due to intestinal perforation. Infants who underwent surgery had higher mortality rates and greater neurodevelopmental impairment than infants who did not undergo surgery ( P value < 0.01, both). On multivariate analysis, single or multiple surgeries increased the risk of neurodevelopmental impairment compared to no surgery with adjusted odds ratios (ORs) of 1.6 with 95% confidence interval (CI) of 1.1-2.6 and 2.3 with 95% CI of 1.1-4.9., Conclusion: Approximately one fifth of VLBW infants underwent one or more surgical interventions during NICU hospitalization. The impact of surgical intervention on long-term neurodevelopmental outcomes was sustained over a follow-up of CA 18-24 months. Infants with multiple surgeries had an increased risk of neurodevelopmental impairment compared to infants with single surgeries or no surgeries after adjustment for possible confounders., Competing Interests: The authors have no potential conflicts of interest to disclose., (© 2019 The Korean Academy of Medical Sciences.)

Objective: To determine the updated outcomes of preterm infants with acute hypoxic respiratory failure attributable to presumed pulmonary hypoplasia (PH) following maternal midtrimester prolonged preterm premature rupture of membranes (PPROM)., Study Design: Among preterm infants with birthweight <1500 g and 23-34 weeks gestational age in a single center, infants exposed to maternal prolonged (≥7 days) PPROM before 25 gestational weeks (PPPROM25, n = 76) were retrospectively reviewed. They were 1:1 matched with infants of matched control group (n = 76) who were unexposed to or exposed to maternal PPROM within 24 hours of delivery by year, gestational age, and weight at birth, sex, and antenatal steroid exposure. The PPPROM25 group was subdivided into infants with and without acute hypoxic respiratory failure attributable to PH (with PH, n = 20, without PH, n = 56, respectively). Clinical characteristics and major outcomes were compared. Risk factors for mortality and morbidity were analyzed using a multivariate logistic regression in the PPPROM25 group., Results: The PH incidence rates were 1.3 and 26.3% and in the matched control and PPPROM25 group, respectively (p < .05). The survival rates were 92.1 and 81.6% in the matched control and PPPROM25 group (p > .05); 87.5 in the PPPROM25 group without PH and 65.0% in group with PH, respectively (p < .05). While there were no significant differences between matched control and PPROM25 group, the PPROM25 with PH group had a significantly higher rate of periventricular leukomalacia (PVL) and composite morbidity, including mortality, bronchopulmonary dysplasia, high-grade intraventricular hemorrhage, and PVL than PPPROM25 without PH group. PH was a significant risk factor for mortality and composite morbidity in the PPPROM25 group., Conclusions: Despite the improved outcomes in the infants with maternal prolonged PPROM before 25 gestational weeks, presumed PH is still a significant risk factor for their mortality and morbidity.

Infantile cortical hyperostosis, or Caffey's disease, usually presents with typical radiological features of soft tissue swelling and cortical thickening of the underlying bone. The disease can be fatal when it presents antenatally, especially before a gestational age of 35 weeks. This fatal, premature form of the disease is known to occur in various ethnic groups around the globe, and approximately 30 cases have been reported in English literature. This paper is unique in that it is the first paper to report a lethal form of prenatal-type infantile cortical hyperostosis diagnosed in South Korea. Born at gestational age of 27 weeks and 4 days, the patient had typical features of polyhydramnios, anasarca, hyperostosis of multiple bones, micrognathia, pulmonary hypoplasia, and hepatomegaly. The patient was hypotonic, and due to pulmonary hypoplasia and persistent pulmonary hypertension, had to be supported with high frequency ventilation throughout the entire hospital course. Due to the disease entity itself, as well as prolonged parenteral nutrition, liver failure progressed, and the patient expired on day 38 when uncontrolled septic shock was superimposed. The chromosome karyotype of the patient was normal, 46, XX, and COL1A1 gene mutation was not detected., Competing Interests: The authors have no potential conflicts of interest to disclose., (© Copyright: Yonsei University College of Medicine 2019.)