Multidisciplinary Management of Congenital Diaphragmatic Hernia: from Womb to Home

( Byong Sop Lee ) 대한주산의학회 2022 Perinatology Vol.33 No.2

Despite recent advances in prenatal diagnosis and newborn treatment strategies, the survival rate of congenital diaphragmatic hernia (CDH) has not changed much in the range of 60% to 80% over the past 20 years. Obstetricians, pediatricians, and pediatric surgeons play a pivotal role in prognosis prediction, perioperative stabilization, and diaphragmatic repair, respectively. The two cardinal underlying pathologies are lung hypoplasia and persistent pulmonary hypertension, which sometimes lead to early mortality and long-term cardiopulmonary sequelae even after timely treatment in the newborn period. The observed/expected lung-to-head ratio in antenatal ultrasound is a strong predictor of mortality, and provides guidance in family counseling on critical decisions such as in utero transfer and extracorporeal membrane oxygenation (ECMO). In the CDH, the efficacy of pulmonary vasodilators including inhaled nitric oxide is unclear, and hemodynamic support should be individually tailored based on invasive monitoring and echocardiography as well. ECMO plays a definite role as a last resort to rescue CDH. However, the implementation of ECMO can be a miraculous life-saving bridge to a surgical correction, but rather can be a meaningless life-sustaining machine. Controversy continues over the timing and technique of surgical repair to reduce mortality and postoperative recurrence. Although late death of CDH is rare after hospital discharge, a significant proportion of patients experience a wide range of long-term burden of associated morbidities including feeding difficulties, pulmonary dysfunction, reduced exercise capacity, and neurodevelopmental impairment. Recent clinical trials on fetal tracheal occlusion therapy are promising in that it turns out to improve the survival rate of severe CDH.

A Contrast Nephropathy in a Preterm Infant Following Preoperative Embolization of Giant Sacrococcygeal Teratoma

Lee, Byong Sop Korean Society of Pediatric Nephrology 2017 Childhood kidney diseases Vol.21 No.1

Newborn infants with huge and highly vascular sacrococcygeal teratoma (SCT) are frequently subjected to renal hypoperfusion secondary to high-output cardiac failure. Any underlying renal dysfunction is a significant risk factor for the development of contrast-induced nephropathy (CIN). However, reports on CIN in infants are rare. I report here a case of a premature infant born at 28 weeks and 3 days of gestation with a huge SCT who survived preoperative embolization and surgical resection but presented with persistent non-oliguric renal failure that was suggestive of CIN. During radiological intervention, a contrast medium had been administered at about 10 times the manufacturer-recommended dose for pediatric patients. Despite hemodynamic stabilization and normalization of urine output immediately following surgery, the patient's serum creatinine and cystatin-C levels did not return to baseline until 4 months after birth. No signs of reflux nephropathy were observed in follow-up imaging studies. Dosing guidelines for the use of a contrast medium in radiological interventions should be provided for infants or young patients.

Management of Persistent Pulmonary Hypertension in Preterm Infants

Lee, Byong Sop 대한신생아학회 2021 Neonatal medicine Vol.28 No.1

Persistent pulmonary hypertension of the newborn (PPHN) is a consequence of the failure of a decrease in the elevated pulmonary vascular resistance after birth. Pulmonary vasodilators, including inhaled nitric oxide (iNO), have been the mainstream of targeted therapy for PPHN, but no drugs have been proven to be effective in preterm infants with PPHN. The fetus remains hemodynamically stable despite lower arterial oxygen tension and pulmonary blood flow as compared to full-term newborns. This adaptation is due to the lower oxygen requirement and high oxygen-carrying capacity of fetal circulation. The immature lungs of preterm infants are more vulnerable to reactive oxygen species, and the response of pulmonary vascular dilatation to blood oxygen tension is blunted in preterm infants. Recently, iNO has been reported to be effective in a selected group of preterm infants, such as those with prolonged preterm rupture of membrane-oligohydramnios-pulmonary hypoplasia sequence. PPHN in preterm infants, along with maximum supportive treatment based on fetal physiology and meticulous assessment of cardiovascular function, is in dire need of new treatment guidelines, including optimal dosing strategies for pulmonary vasodilators.

Nutritional strategy of early amino acid administration in very low birth weight infants

Lee, Byong Sop The Korean Pediatric Society 2015 Clinical and Experimental Pediatrics (CEP) Vol.58 No.3

Relative to a fetus of the same gestational age, very low birth weight (VLBW) infants are more likely to be underfed and to undergo growth restriction during their early hospital stay. The current trend towards "early and aggressive" nutritional strategies in VLBW infants aims to overcome the early nutritional deficiency and thereby boost postnatal catch-up growth, simultaneously improving long-term neurodevelopmental outcomes. Although the minimum starting amino acid (AA) dose to prevent negative nitrogen balance is well established, the upper limit and the rate of increase of early AA doses are controversial. Most randomized controlled trials show that early and high-dose (target, 3.5 to 4.9 g/kg/day) AA regimens, with or without high nonprotein calories, do not improve long-term growth and neurodevelopment. High-dose AA supplementation may lead to early metabolic disturbances and excessive or disproportionate plasma AA levels, particularly in infants of very low gestational age. Further large studies are needed to clarify the optimal strategy for early administration of parenteral AA doses in VLBW infants.

Ex utero intrapartum treatment procedure in two fetuses with airway obstruction

( Joohee Lee ),( Mi-young Lee ),( Yeni Kim ),( Jae-yoon Shim ),( Hye-sung Won ),( Euiseok Jeong ),( Byong Sop Lee ),( Ki-soo Kim ),( Woo-jong Choi ),( Yoon Se Lee ) 대한산부인과학회 2018 Obstetrics & Gynecology Science Vol.61 No.3

The ex utero intrapartum treatment (EXIT) procedure was introduced to reduce fetal hypoxic damage while establishing an airway in fetuses with upper and lower airway obstruction. Delivery of the fetal head and shoulders while maintaining the uteroplacental circulation offers time to secure the fetal airway. Here, we report two cases of EXIT procedure for fetal airway obstruction, which were successfully managed with extensive preoperative planning by a professional multidisciplinary team.

주산기 가사의 저체온 치료

이병섭 ( Byong Sop Lee ) 대한주산의학회 2011 Perinatology Vol.22 No.1

Several well-designed multicenter clinical trials of therapeutic hypothermia, maintaining rectal temperature of 33-34℃ for 72 hours in neonates suffered from perinatal asphyxia, have demonstrated both safety and efficacy of therapeutic hypothermia in improving survival and neurodevelopmental outcomes. However, issues regarding the optimal cooling method, the target temperature and the duration of the hypothermia remain unsettled. To overcome limited efficacy of therapeutic hypothermia in the subgroup of infants with severe hypoxic ischemic encephalopathy, a few promising hypothermia-combined therapies, targeting the several steps in the pathogenesis of hypoxic ischemic encephalopathy, are now under investigation. Further data on the neurodevelopmental outcome of the study population of the finished or ongoing clinical trials, during the childhood period or thereafter, are required to settle therapeutic hypothermia as a standard of care against perinatal asphyxia. Nationwide establishment of efficient patient referral system and intimate communication of cooling protocol between obstetricians and neonatologists will make therapeutic hypothermia in neonates more available in Korea.

미숙아의 영양 평가

이병섭 ( Byong Sop Lee ) 대한주산의학회 2008 Perinatology Vol.19 No.1

Campomelic Dysplasia with Sex Reversal Harboring a Novel Frameshift Mutation

Ha Na Lee,Chae Young Kim,Euiseok Jung,Beom Hee Lee,Byong Sop Lee,Ellen Ai Rhan Kim,Ki-Soo Kim 대한신생아학회 2020 Neonatal medicine Vol.27 No.4

Campomelic dysplasia (CD) is a rare genetic disease characterized by skeletal dysplasia that also affects several other organ systems. CD is caused by a SOX9 mutation. We here report a case of CD with a 46, XY karyotype and female external genitalia. This child was born with a weight of 3.12 kg after 37 weeks of gestation. She exhibited a number of characteristic features including a small thoracic cage, bowing of both femurs, clubbed feet, hypoplastic scapula, 11 pairs of ribs, a bell-shaped narrow thorax, micrognathia, macroglossia, a cleft palate, a flattened nasal bridge, and low set ears. She experienced additional distress because of the presence of a tracheal ring and because she had tracheomalacia. CD was diagnosed through nucleotide sequence analysis. A frameshift mutation, c.235delC (p.Gln79Argfs*31), was identified in the SOX9 gene that has not previously been reported.

Prenatal diagnosis of atrial isomerism in the Korean population

( Mi Young Lee ),( Hye Sung Won ),( Jae Yoon Shim ),( Pil Ryang Lee ),( Byong Sop Lee ),( Ellen Ai Rhan Kim ),( Young Hwue Kim ),( Jeong Jun Park ),( Tae Jin Yun ),( Ahm Kim ) 대한산부인과학회 2014 Obstetrics & Gynecology Science Vol.57 No.3

Objective To report our experiences in the prenatal diagnosis of atrial isomerism and postnatal outcomes. Methods A total of 80 fetuses prenatally diagnosed with atrial isomerism were retrospectively analyzed between 1999 and 2011 at a single institution. Results Of 43 fetuses with prenatally diagnosed right atrial isomerism (RAI), 40 cases were analyzed. The diagnostic accuracy was 93%. The main intracardiac anomalies in RAI were atrioventricular septal defect (AVSD), abnormal pulmonary venous connection, bilateral superior vena cava (BSVC), and pulmonary atresia. Among 28 live births, three infants were lost to follow up, and the overall survival rate was 60%. Of 37 fetuses with prenatally diagnosed left atrial isomerism (LAI), 35 were evaluated. The diagnostic accuracy was 97%. The main intracardiac anomalies in LAI were ventricular septal defect, BSVC, AVSD, double outlet right ventricle, and bradyarrhythmia. Among seven patients with bradyarrhythmia, only one showed a complete atrioventricular block. All fetuses had an interrupted inferior vena cava with azygous continuation. The overall survival rate was 90%. Conclusion Our study confirms the previous findings of fetal atrial isomerism. We also demonstrates a much lower prevalence of AVSD and complete heart block in LAI and a better survival rate in RAI. Although the postnatal outcomes for RAI were worse than those for LAI, successful postnatal surgery with active management improved the survival rate.

Effects of Recombinant Human Erythropoietin Administration in Premature Infants with Severe Intraventricular Hemorrhage: A Single-Center Experience

Ha Na Lee,JeongMin Lee,Soo Hyun Kim,Sung Hyeon Park,Jiyoon Jeong,Euiseok Jung,Byong Sop Lee 대한신생아학회 2023 Neonatal medicine Vol.30 No.2

Purpose: We investigated the effects of early postnatal administration of erythropoietin (EPO) on neurodevelopmental outcomes and morbidities in preterm infants with severe grades of intraventricular hemorrhage (IVH). Methods: We retrospectively reviewed the medical records of preterm infants of gestational age 23+0 weeks to 31+6 weeks, who were diagnosed with severe grades of IVH and received EPO over at least 2 weeks. We compared clinical characteristics, major complications, and neurodevelopmental impairment between the two groups. The primary outcome was severe neurodevelopmental impairment at 18 to 26 months of corrected age. Severe neurodevelopmental impairment was defined as a mental developmental index or psychomotor developmental index of <70 on the Bayley Scales of Infant Development II or diagnosis of cerebral palsy. Results: The study included 33 preterm infants (mean gestational age 25.2±1.6 weeks and mean birth weight 775.1±224.5 g). EPO was administered at a dose of 400 or 1,000 IU/kg thrice weekly and was maintained over a mean period of 58.6± 25.9 days beginning from 10.7±6.9 days after birth. We observed no difference in perinatal characteristics between the EPO (n=14) and the control group (n=19). Similarly, severe neurodevelopmental impairment rates did not differ between the EPO (85.7%) and control groups (78.9%). The incidence of neonatal morbidities including bronchopulmonary dysplasia, necrotizing enterocolitis, and retinopathy of prematurity was also similar between the EPO and control groups. Conclusion: Early administration of EPO did not reduce the risk of severe neurodevelopmental impairment in preterm infants with severe IVH.