Ex Utero Intrapartum Treatment procedure in two fetuses with airway obstruction

( Joohee Lee ),( Mi-young Lee ),( Yeni Kim ),( Jae-yoon Shim ),( Hye-sung Won ),( Pil-ryang Lee ),( Ahm Kim ) 대한산부인과학회 2016 대한산부인과학회 학술대회 Vol.102 No.-

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 adequate time to secure the airway while preventing fetal hypoxia. Here, we report two cases of fetal airway obstruction that were successfully managed using the EXIT procedure, with extensive preoperative planning and under a professional multidisciplinary team. Case 1: A 32-year-old woman was referred to our institution at 20 weeks of gestation with a diagnosis of a fetal neck mass. Ultrasonographic and magnetic resonance imaging findings revealed a 8.5cm-sized huge lymphangioma with suspected upper airway compression. Delivery was accomplished by a cesarean section with the EXIT procedure at 38.3 weeks of gestation. The fetus was endotracheally intubated while maintaining uteroplacental circulation. The baby is now 18 months old and does not show any signs of developmental or cognitive delay. Case 2: A 31-year-old woman was referred to our institution at 21 weeks of gestation with suspected congenital high airway obstruction syndrome (CHAOS). Ultrasonography revealed symmetrically enlarged, hyperechoic, and homogeneous lungs with an inverted diaphragm, anteriorly displaced heart, and polyhydramnios, all of which were suggestive of CHAOS. The baby was delivered via the EXIT procedure at 38 weeks of gestation, and a tracheostomy was performed. The baby is now five months old and is developing normally.

Postnatal outcomes of fetus with prenatally suspected morphological abnormality in the ear

( Jihoon Lee ),( So-yeon Kim ),( Mi-young Lee ),( Jin-hoon Chung ),( Hye-sung Won ),( Pil-ryang Lee ) 대한산부인과학회 2021 대한산부인과학회 학술대회 Vol.107 No.-

임신 중 심장질환

이필량 ( Pil Ryang Lee ) 대한주산의학회 2003 大韓周産醫學會雜誌 Vol.14 No.3

소염제가 배양된 양막세포에서 Lipopolysaccharide의 자극에 의한 Cyclooxygenase-2 단백질 발현에 미치는 영향

이필량 ( Pil Ryang Lee ),정복경 ( Bok Kyung Jung ),김소라 ( So Ra Kim ),강지안 ( Ji Ahn Kang ),신명신 ( Myung Shin Shin ),원혜성 ( Hye Sung Won ),이인식 ( In Sik Lee ),김암 ( Ahm Kim ) 대한주산의학회 2000 大韓周産醫學會雜誌 Vol.11 No.4

Perinatal Outcome Of Twin Reversed Arterial Perfusion Sequence: A Single Center`s Experience

( Mi Young Lee ),( Hye Sung Won ),( Kyung A Lee ),( Kyu Sang Kyeong ),( Eun Jin Jeon ),( Jae Yoon Shim ),( Pil Ryang Lee ),( Ahm Kim ) 대한산부인과학회 2012 Obstetrics & Gynecology Science Vol.55 No.8

Objective To report our experience of using radiofrequency ablation (RFA) to treat fetuses diagnosed with twin reversed arterial perfusion (TRAP) sequence and to evaluate the perinatal outcome of pump twins. Methods Twenty-six fetuses diagnosed with the TRAP sequence were retrospectively analyzed between July 1998 and September 2011 at Asan Medical Center. Four were lost to follow-up after diagnosis and, therefore, were excluded from further evaluation. The perinatal outcomes of pump twins were evaluated by reviewing the medical records. Results Twenty-two fetuses were diagnosed with the TRAP sequence during the study period, including 15 monochorionic-diamniotic pregnancies and four monochorionic-monoamniotic pregnancies. Three patients had triplet pregnancies. The median gestational age at diagnosis was 17.4 weeks (range, 11.0 to 27.0 weeks). Of these 22 cases, in utero RFA was performed in 11 (50%), alcohol ablation in one (4.5%) and the remaining 10 (45.5%) underwent conservative management. The median gestational age at in utero intervention was 21.0 weeks (range, 17.6 to 25.0 weeks). The overall neonatal survival rate was 77% (17 of 22). The median gestational age at delivery was 37.3 weeks (range, 30.2 to 40.1 weeks). All of the surviving infants are doing well without any complications. Conclusion For fetuses with the TRAP sequence, proper in utero treatment with RFA enables to continue the pregnancy with a good prognosis.

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.

OB-23 : Perinatal outcome of VACTERL association

( Mi Kyung Lee ),( Mi Young Lee ),( Jae Yoon Shim ),( Pil Ryang Lee ),( Ahm Kim ),( Hye Sung Won ) 대한산부인과학회 2012 대한산부인과학회 학술대회 Vol.99 No.-

To evaluate the perinatal outcome of the fetuses with prenatal diagnosed VACTERL association. We retrospectively reviewed the 13 fetuses who were diagnosed VACTERL association prenatally between 2008 and 2013. Prenatal records and postnatal outcomes were assessed. The median gestational age at diagnosis was 28.2 weeks (range, 21.2-38.0 weeks). The male to female ratio was 10:3. The most common observed VACTERL component was cardiac anomalies (n=13), followed by vertebral anomalies (n=8), anal atresia (n=8), tracheoesophageal fistula/ esophageal atresia (n=6), renal anomalies (n=6) and limb anomalies (n= 4). The most frequent combination was VACT (n=3), followed by VAC (n=2) and VACL (n=2). All fetuses were born alive. The median gestational age at delivery was 38.0 weeks (range, 29.3-39.4 weeks) and the median birth weight was 2361g (range, 1390-3100g). Only one neonate died after cardiac surgery and one was lost to follow up in the neonatal period. The overall survival rate was 92%. All survivors required repeated surgeries with medical management during the follow-up period. Although VACTERL association had a low mortality rate, there was a significant morbidity associated with congenital malformations. Therefore, parents of fetuses with VACTERL association should be strongly encouraged to undertake extensive counseling regarding the postnatal morbidity of VACTERL association.

최우수 논문상 : 최우수 논문상 (모체태아의학)

이미영 ( Mi Young Lee ),( Hye Sung Won ),( Kyung A Lee ),( Kyu Sang Kyeong ),( Eun Jin Jeon ),( Jae Yoon Shim ),( Pil Ryang Lee ),( Ahm Kim ) 대한산부인과학회 2012 대한산부인과학회 학술대회 Vol.99 No.-

Objective: To report our experience of using radiofrequency ablation (RFA) to treat fetuses diagnosed with twin reversed arterial perfusion (TRAP) sequence and to evaluate the perinatal outcome of pump twins. Methods: Twenty-six fetuses diagnosed with the TRAP sequence were retrospectively analyzed between July 1998 and September 2011 at Asan Medical Center. Four were lost to follow-up after diagnosis and, therefore, were excluded from further evaluation. The perinatal outcomes of pump twins were evaluated by reviewing the medical records. Results: Twenty-two fetuses were diagnosed with the TRAP sequence during the study period, including 15 monochorionic-diamniotic pregnancies and four monochorionic-monoamniotic pregnancies. Three patients had triplet pregnancies. The median gestational age at diagnosis was 17.4 weeks (range, 11.0 to 27.0 weeks). Of these 22 cases, in utero RFA was performed in 11 (50%), alcohol ablation in one (4.5%) and the remaining 10 (45.5%) underwent conservative management. The median gestational age at in utero intervention was 21.0 weeks (range, 17.6 to 25.0 weeks). The overall neonatal survival rate was 77% (17 of 22). The median gestational age at delivery was 37.3 weeks (range, 30.2 to 40.1 weeks). All of the surviving infants are doing well without any complications. Conclusion: For fetuses with the TRAP sequence, proper in utero treatment with RFA enables to continue the pregnancy with a good prognosis.

모체태아의학

이미영 ( Mi-young Lee ),( Hye-sung Won ),( Ji Eun Park ),( Jae-yoon Shim ),( Pil-ryang Lee ),( Ahm Kim ),( Jung Bok Lee ) 대한산부인과학회 2018 대한산부인과학회 학술대회 Vol.104 No.-

Objectives To establish normal reference ranges for the fetal left modified myocardial performance index (Mod-MPI) measured by the Auto Mod-MPI system and evaluate Mod-MPI changes in recipients of twin-to-twin transfusion syndrome (TTTS) before and after fetoscopic laser coagulation. Methods This was a prospective longitudinal study of normal singleton fetuses from 12.0 to 40.0 weeks of gestation. TTTS cases treated by laser coagulation were reviewed for Mod-MPI measurements of recipients. Results All measurements were performed using the Auto Mod-MPI system by a single experienced operator. Among a total 447 examinations from 222 fetuses, we were unable to measure the Mod-MPI in two cases, and therefore, 445 examinations were analyzed. The median Mod-MPI consistently increased from 0.44 to 0.56 throughout gestation. The median isovolumetric contraction time (ICT) and isovolumetric relaxation time (IRT) also increased with advancing gestational age. The ejection time (ET) increased until 27 weeks of gestation and decreased thereafter. In the 30 recipients, Mod-MPI, ICT, and IRT increased before laser coagulation and significantly decreased after laser coagulation. Conclusion Normal reference values for left Mod-MPI were established using the Auto Mod-MPI system, and these might be useful for assessing cardiac function in TTTS. ⓒ 2016 John Wiley & Sons, Ltd.

산전진단된 지속 좌측 상대정맥 (persistent Left superior vena cava)의 임상적 의의

이진경 ( Jin Kyong Lee ),원혜성 ( Hye Sung Won ),이상훈 ( Sang Hun Lee ),임소희 ( So Hee Lim ),김민균 ( Min Gyun Kim ),심재윤 ( Jae Yoon Shim ),이필량 ( Pil Ryang Lee ),김암 ( Ahm Kim ) 대한산부인과학회 2009 Obstetrics & Gynecology Science Vol.52 No.10

목적: 최근 산전심초음파검사 시 상부 종격에서 관찰되는 삼혈관 영상에 대한 중요성이 대두되면서 지속 좌측 상대정맥의 진단율이 증가되고 있다. 이에 본 연구에서는 선천성 심질환과의 연관성뿐만 아니라 구조적 심기형을 동반하지 않은 단독 지속 좌측 상대정맥을 가진 환아들의 예후를 조사하여 지속 좌측 상대정맥의 임상적 의의에 대해 밝혀보고자 하였다. 연구 방법: 2001년 5월부터 2008년 5월까지 서울아산병원에서 시행한 총 17,100명의 임신 중기 정밀초음파검사에서 지속 좌측상대정맥으로 진단된 85명을 대상으로 의무기록과 산전 초음파 영상들을 후향적으로 분석하였다. 결과: 총 85예의 지속 좌측 상대정맥이 산전 진단되었으며 단독 지속 좌측 상대정맥은 15예에서 진단되었다. 85예 중 동반된 심기형으로는 심실 중격 결손, 양대혈관 우심실 기시, 방실 중격 결손이 각각 54.1%, 17.6%, 17.6%로 높게 나타났고 15예의 단독 지속 좌측 상대정맥 중 산후에 전 폐정맥 환류이상으로 진단된 1예와 경과 소실된 1예를 제외한 13예는 특별한 합병증없이 양호한 상태로 확인되었다. 결론: 지속 좌측 상대정맥이 태아 선천 심기형을 예측하는 강력한 인자로 사용될 수 있으며, 심기형을 동반하지 않은 단독지속 좌측 상대정맥은 출생 후 특별한 문제가 없는 정맥계 기형 중 하나로 산전상담 시 참고할 수 있겠다. Objective: To determine the clinical significance of persistent left superior vena cava (PLSVC) in a fetus with and without cardiac and extra-cardiac anomalies. Methods: This was a retrospective review of all cases of PLSVC detected prenatally at our institution between May 2001 and May 2008. This retrospective study included 85 fetuses with PLSVC who were diagnosed based on the presence of an additional vessel identified to the left of the pulmonary artery in the three-vessel view of the heart. Patient charts and recorded images were reviewed in order to identify associated conditions and outcomes. Telephone interviews were conducted to check patients` conditions in cases of isolated PLSVC. Results: Eighty-five cases of PLSVC were detected prenatally during this study period. Of these 85 fetuses, 11 were aborted due to associated, prenatally proven, severe congenital heart anomalies or chromosomal anomalies, and 52 fetuses were delivered. The cases for other 22 fetuses were lost to follow-up. Postnatal echocardiography was performed in the 33, surviving patients, and PLSVC was confirmed in 32 of these patients. The most common associated congenital cardiac anomalies were seen included VSD, AVSD, and DORV (54.1%, 17.6% and 17.6%, respectively) (Table 3). PLSVC was also seen in seven cases (8.2%) of right isomerism and in four cases (4.7%) of left isomerism. In only two cases was the coexistence of PLSVC and extra-cardiac anomalies noted in this study. Fifteen cases were prenatally diagnosed as isolated PLSVC and all of them had live births. The follow-up period in our isolated PLSVC patients ranged from 0.5 to 84 months (Mean 24.5 months). Thirteen of these infants were doing well at the time of preparing this document and one case was diagnosed as TAPVR on postnatal echocardiography and one case was lost to follow-up. Conclusion: We strongly suggest that PLSVC is a benign vascular malformation and does not affect to the patient after birth. However, PLSVC is frequently associated with heterotaxy syndromes as well as other cardiac malformations and can be misdiagnosed as TAPVR. So if we find PLSVC in prenatal ultrasonography, meticulous inspection of the fetal anatomy must be performed.