How much have the perinatal outcomes of triplet pregnancies improved over the last two decades?

OBJECTIVE: This study was conducted to demonstrate the temporal trends in perinatal outcomes of triplet pregnancies over the last two decades. METHODS: The medical records of patients with triplet pregnancies at two Korean tertiary-care hospitals from 1992 to 2012 were retrospectively reviewed in regard to maternal and neonatal outcomes. The study was divided into two periods for analysis: period I (1992–2001) and period II (2003–2012). RESULTS: Over a 21-year period, 65 women with triplet pregnancies and 185 neonates were analyzed. Period II, when compared with period I, was associated with improved maternal outcomes, characterized by a decreased incidence of preeclampsia (31.8% vs. 2.3%, P=0.002) and anemia (68.2% vs. 30.2%, P=0.003) during pregnancy. Regarding neonatal aspects, the composite morbidity of period II was significantly decreased compared with that of period I, as assessed with a generalized estimating equation for logistic regression (26.2% vs. 8.1%, P=0.03). Multivariable analysis revealed that the gestational age at delivery and the period were significantly associated with the composite neonatal morbidity (P<0.001 and 0.007, respectively). CONCLUSION: Improved neonatal morbidity was associated with a higher gestational age at delivery and with the more recent decade.

An extremely rare case of prenatally diagnosed absent both aortic and pulmonary valves

We describe a case of absent aortic and pulmonary valves, diagnosed at 16.4 weeks of gestation. Fetal echocardiography showed cardiomegaly with dilated both ventricles. No valve leaflets were observed in the aorta and pulmonary artery, and a typical to-and-fro flow pattern was noted in both great arteries on color Doppler imaging. Fetal hydrops was also detected. Follow-up ultrasonographic evaluation at 19 weeks demonstrated intrauterine fetal death. Postmortem autopsy revealed the absence of both aortic and pulmonary valve leaflets. To the best of our knowledge, this is the earliest diagnosed case of absent both aortic and pulmonary valves and only the second case to be diagnosed prenatally.

An extremely rare case of prenatally diagnosed absent both aortic and pulmonary valves

We describe a case of absent aortic and pulmonary valves, diagnosed at 16.4 weeks of gestation. Fetal echocardiography showed cardiomegaly with dilated both ventricles. No valve leaflets were observed in the aorta and pulmonary artery, and a typical to-and-fro flow pattern was noted in both great arteries on color Doppler imaging. Fetal hydrops was also detected. Follow-up ultrasonographic evaluation at 19 weeks demonstrated intrauterine fetal death. Postmortem autopsy revealed the absence of both aortic and pulmonary valve leaflets. To the best of our knowledge, this is the earliest diagnosed case of absent both aortic and pulmonary valves and only the second case to be diagnosed prenatally.

Impaction of an intrathoracic kidney acted as a shield against herniation of the abdominal viscera in a case of right congenital diaphragmatic hernia

We describe a case of an intrathoracic kidney combined with right congenital diaphragmatic hernia (CDH) that was diagnosed at 32 weeks of gestation. Although it has been well established that a right CDH shows a poorer outcome than a left CDH, our present case showed a good outcome because there was no herniation of other abdominal viscera, except for the right kidney. Our findings in this case indicate that impaction of the intrathoracic kidney may act as a 'shield' against further herniation of other abdominal viscera into the thoracic cavity.

Outcome of Inhaler Withdrawal in Patients Receiving Triple Therapy for COPD / 결핵및호흡기질환

BACKGROUND: The purpose of this study was to document outcomes following withdrawal of a single inhaler (step-down) in chronic obstructive pulmonary disease (COPD) patients on triple therapy (long-acting muscarinic antagonist and a combination of long-acting beta2-agonists and inhaled corticosteroid), which a common treatment strategy in clinical practice. METHODS: Through a retrospective observational study, COPD patients receiving triple therapy over 2 years (triple group; n=109) were compared with those who had undergone triple therapy for at least 1 year and subsequently, over 9 months, initiated inhaler withdrawal (step-down group, n=39). The index time was defined as the time of withdrawal in the stepdown group and as 1 year after the start of triple therapy in the triple group. RESULTS: Lung function at the index time was superior and the previous exacerbation frequency was lower in the stepdown group than in the triple group. Step-down resulted in aggravating disease symptoms, a reduced overall quality of life, decreasing exercise performance, and accelerated forced expiratory volume in 1 second (FEV1) decline (54.7+/-15.7 mL/yr vs. 10.7+/-7.1 mL/yr, p=0.007), but there was no observed increase in the frequency of exacerbations. CONCLUSION: Withdrawal of a single inhaler during triple therapy in COPD patients should be conducted with caution as it may impair the exercise capacity and quality of life while accelerating FEV1 decline.

Safe childbirth in Korea: the endless challenge

Precise prenatal care and safe childbirth under the guidance of a qualified obstetrician are essential reproductive healthcare services that enhance the survival and wellbeing of mothers and newborns. However, during recent decades many maternity hospitals and clinics in Korea have closed, particularly those in rural areas. In addition, the number of obstetricians providing care for pregnant women is declining. This reduction in obstetric services is a growing political issue in Korea. Several policies and programs have tried to accelerate and expand the provision of essential maternity services, especially in rural areas, but there have been numerous barriers to overcome, including malpractice litigations. Now is the time to discuss the role of current and future government programs in the improvement of obstetric care and, moreover, to develop outlines for progress in underserved areas.

Endobronchial ALK-Positive Anaplastic Large Cell Lymphoma Presenting Massive Hemoptysis / 결핵및호흡기질환

Primary anaplastic large cell lymphoma (ALCL) of the lung is highly aggressive and quite rare. We report here a case of anaplastic lymphoma kinase-positive endobronchial ALCL, that was initially thought to be primary lung cancer. A 68-year-old woman presented with hemoptysis, dyspnea, and upper respiratory symptoms persisting since 1 month. The hemoptysis and and bronchial obstruction lead to respiratory failure, prompting emergency radiotherapy and steroid treatment based on the probable diagnosis of lung cancer, although a biopsy did not confirm malignancy. Following treatment, her symptoms resolved completely. Chest computed tomography scan performed 8 months later showed increased and enlarged intra-abdominal lymph nodes, suggesting lymphoma. At that time, a lymph node biopsy was recommended, but the patient refused and was lost to follow up. Sixteen months later, the patient revisited the emergency department, complaining of persistent abdominal pain since several months. A laparoscopic intra-abdominal lymph node biopsy confirmed a diagnosis of ALCL.

Intrauterine therapy for macrocystic congenital cystic adenomatoid malformation of the lung

OBJECTIVE: To report on our experiences with thoracoamniotic shunting and/or the injection of a sclerosing agent (OK-432) to treat fetuses diagnosed with macrocystic congenital cystic adenomatoid malformation (CCAM) of the lung. METHODS: A retrospective study was undertaken in six fetuses with macrocystic CCAM at our institute that had been confirmed by postnatal surgery between August 1999 and January 2012. RESULTS: Six fetuses that had been diagnosed with macrocystic CCAM were analyzed. The median gestational age at diagnosis was 23.5 weeks (range, 19.5-31.0 weeks), and at the time of primary treatment was 24.0 weeks (range, 20.5-31.0 weeks). The mean size of the largest cyst at the initial assessment was 42.5+/-15 mm. Four fetuses were associated with mediastinal shifting, and one also showed fetal hydrops. All fetuses underwent a shunting procedure within the cysts, one case among them was also treated with OK-432. After the completion of all procedures, the mean size of the largest cyst was all decreased (14.2+/-12 mm). The median gestational age at delivery was 38.0 weeks (range, 32.4-40.3 weeks). All of the newborns underwent the surgical resection at a median age of 6 days (range, 1-136 days) and are currently doing well without any complications. CONCLUSION: We suggest that intrauterine decompression therapy to manage fetal macrocystic CCAM is recommendable treatment for good perinatal outcome.

Thanatophoric dysplasia in a dichorionic twin confirmed by genetic analysis at the early second trimester: A case report and literature review

Thanatophoric dysplasia (TD) is caused by mutation of the gene that encodes fibroblast growth factor 3 (FGFR3). Owing to the poor prognosis for TD, prenatal diagnosis is critical to optimal perinatal management. We report here a case of TD in twin pregnancy, which was prenatally diagnosed by DNA analysis following amniocentesis at 15 weeks, and was managed by selective fetal termination. Prenatal ultrasonography and molecular analysis to detect TD-specific mutations enable accurate diagnosis of FGFR3-related TD in utero and appropriate obstetrical management at early gestation during twin pregnancy.

Changes in the Cesarean Section Rate in Korea (1982-2012) and a Review of the Associated Factors

Although Cesarean section (CS) itself has contributed to the reduction in maternal and perinatal mortality, an undue rise in the CS rate (CSR) has been issued in Korea as well as globally. The CSR in Korea increased over the past two decades, but has remained at approximately 36% since 2006. Contributing factors associated with the CSR in Korea were an improvement in socio-economic status, a higher maternal age, a rise in multiple pregnancies, and maternal obesity. We found that countries with a no-fault compensation system maintained a lower CSR compared to that in countries with civil action, indicating the close relationship between the CSR and the medico-legal system within a country. The Korean government has implemented strategies including an incentive system relating to the CSR or encouraging vaginal birth after Cesarean to decrease CSR, but such strategies have proved ineffective. To optimize the CSR in Korea, efforts on lowering the maternal childbearing age or reducing maternal obesity are needed at individual level. And from a national view point, reforming health care system, which could encourage the experienced obstetricians to be trained properly and be relieved from legal pressure with deliveries is necessary.

Variety of prenatally diagnosed congenital heart disease in 22q11.2 deletion syndrome

OBJECTIVE: To analyze the spectrum of prenatally diagnosed congenital heart disease in a Korean population with 22q11.2 deletion syndrome, and to provide guidelines for screening 22q11.2 deletion prenatally. METHODS: This retrospective study evaluated 1,137 consecutive fetuses that had prenatal genetic testing for 22q11.2 deletion because of suspected congenital heart disease between September 2002 and December 2012, at Asan Medical Center, Seoul, Korea. RESULTS: Main cardiovascular diseases in the 53 fetuses with confirmed 22q11.2 deletions were tetralogy of Fallot (n = 24, 45%), interrupted aortic arch (n = 10, 19%), ventricular septal defect (n = 5, 9%), double outlet right ventricle (n = 4, 8%), and coarctation of the aorta (n = 4, 8%). Other cardiac defects were rarely associated with 22q11.2 deletion. One fetus had persistent truncus arteriosus, one had aortic stenosis, and one had hypoplastic right heart syndrome. Two fetuses had normal intracardiac anatomy with an isolated right aortic arch, and one had an isolated bilateral superior vena cava. CONCLUSION: A variety of congenital heart diseases were seen during the prenatal period. Conotruncal cardiac defects except transposition of great arteries were strongly associated with 22q11.2 deletion. When such anomalies are diagnosed by fetal echocardiography, genetic testing for 22q11.2 deletion should be offered. Even if less frequent deletion-related cardiac defects are detected, other related anomalies, such as thymic hypoplasia or aplasia, should be evaluated to rule out a 22q11.2 deletion.

Prenatal diagnosis of atrial isomerism in the Korean population

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.

A case report of prenatally diagnosed tetrasomy 18p

Tetrasomy 18p, one of the most commonly observed isochromosomes, consists of two copies of the p arms on chromosome 18[i(18p)]. It is known as a de novo occurrence of non-disjunction or centromeric mis-division during meiosis II in the vast majority of cases. It has a prevalence of 1/140,000-180,000 live births and affects both genders equally. A 28-year-old woman was referred at 33+2 weeks gestation to rule out fetal congenital heart disease. Her prenatal ultrasonography showed intrauterine growth retardation, cardiomegaly, and imperforate anus. Doppler ultrasonographic finding showed fetal anemia. Tetrasomy 18p was confirmed by conventional karyotyping and fluorescence in situ hybridization. Because of its very low prevalence rate, only several cases of tetrasomy 18p has been reported worldwide and it has not yet been reported in Korea before. Therefore, we report a case of prenatally diagnosed tetrasomy 18p.

Conservative multidisciplinary management of placenta percreta following in vitro fertilization

Placenta percreta is an extremely rare and the most severe form of placental invasion, that is associated with severe maternal morbidity and mortality. We report a case of nulliparous woman who underwent 10 cycles of in vitro fertilization (IVF) without any known risk factors. We conserved her uterus by spontaneous vaginal delivery, leaving the placenta in situ, pelvic arterial embolization, and primary resection of the remaining placental tissues. This case demonstrates that repetitive IVF is a possible risk factor for placental invasion, and that conservation of the uterus can be achieved in such cases using a multidisciplinary approach.

The Issues in Obstetric No Fault Compensation Act and Suggestions for the Improvement / 대한주산의학회잡지

As part of the medical dispute mediation act, no fault compensation legislation was enacted in 2011 and took effect from April 2013 in Korea. According to this law, obstetricians should share thirty percet of the compensation expenses with the government. Although the government argues that this law can contribute to the safe obstetric practice environment, most of obstetricians are concerning about the negative effects that can be exerted by the act, including decline in new OB/GYN residents' applications, dropping number of delivering clinics and hospitals, and ultimately, devastating the obstetric practice in this country. In this review, we summarize the process and issues in no fault compensation. We also introduce no fault compensation system in Japan and compare it with ours. Finally, we will give suggestions for the improvement of the law.

Neonatal Characteristic of Congenital Cystic Adenomatoid Malformation of the Lung Requiring Early Operation and Preoperative Intervention

PURPOSE: To identify neonatal characteristics associated with congenital cystic adenomatoid malformation (CCAM) who required early operations and to introduce preoperative interventions to delay definitive operations until stabilized. METHODS: A retrospective review of dataset was performed from January 2000 to December 2011 for neonates admitted to NICU at Asan Medical Center with prenatally diagnosed CCAM. Variable prenatal and postnatal factors were compared for those who required early operations to those asymptomatic neonates who required elective operations at later age. RESULTS: A total of 60 patients were enrolled and patients were divided into 2 groups according to time of operation. Median time of surgery for group 1 (n=12, 20%) and group 2 (n=48, 80%) was 5.5 days and 504 days, respectively. Maternal characteristics including age, parity, preterm labor, oligohydramnios were similar in between two groups. Factors associated with early operation included prenatal history of polyhydramnios (OR 23, P=0.001), who had undergone fetal interventions (OR 47, P=0.001), low 1 and 5 minute Apgar scores and increasing fetal fluid-filled cystic sizes (OR 26, P=0.013). Of those 3 neonates who required preoperative interventions to decrease air-filled cysts to relieve initial respiratory symptoms were successful and all underwent for definitive operations during NICU hospitalizations. All survived. CONCLUSION: Most neonates with CCAM undergo elective operations during infancy. However, about 20% neonates with polyhydramnios, fetal intervention, increasing fetal cystic mass and born with low Apgar scores required early operations for whom prenatal and postnatal interventions prior to definitive surgery can improve survival rate.

Identification of Characteristics and Risk Factors Associated with Mortality in Hydrops Fetalis

PURPOSE: The objectives were to identify the characteristics of neonates with hydrops fetalis, and to identify the risk factors associated with mortality. METHODS: A retrospective review of AMC (Asan Medical Center) dataset was performed from January 1990 to June 2009. The characteristics of 71 patients with hydrops fetalis were investigated and they were divided into two groups: the survived group and the expired group. Various perinatal and neonatal factors in two groups were compared to find out risk factors associated with mortality based on univariate analysis, followed by multiple regression analyses (SPSS version 18.0). RESULTS: Of those 71 neonates (average gestational age: 33 weeks, birth weight: 2.6 kg), 38 survived, 33 died, resulting in overall mortality rate of 46.5%. The most common etiology was idiopathic followed by chylothorax, cardiac anomalies, twin-to-twin transfusion, meconium peritonitis, cardiac arrythmias, and congenital infections. Factors that were associated independently with mortality in logistic regression analyses were low 5-minutes Apgar score, hyaline membrane disease and delayed in achieving 50th percentile ideal body weight for appropriate gestational age by 10 days. CONCLUSION: In this study, 5-minutes Apgar score, hyaline membrane disease and delayed in achieving 50th percentile ideal body weight for appropriate gestational age by 10 days were significant risk factors associated with mortality in hydrops fetalis. Therefore, the risk of death among neonates with hydrops fetalis depends on the illness immediately after birth and severity of hydrops fetalis. Informations from this study may prove useful in prediction of prognosis to neonates with hydrops fetalis.

A case of pseudo-Meigs' syndrome caused by metastatic ovarian tumor from gastric cancer during pregnancy / 대한산부인과학회지

Pseudo-Meigs' syndrome is a syndrome that includes hydrothorax and ascites secondary to ovarian tumors other than solid benign fibroma. In this report, we present the case of a 29-years-old female who complained of abdominal distension with peripheral edema during her third trimester. Two solid masses of 15 cm dimension in the left abdomen and 7 cm dimension in the right abdomen were detected by ultrasound and chest X-ray revealed right pleural effusion. Magnetic resonance imaging (MRI) confirmed the ovarian masses and ascites. As the patient had regular uterine contractions, we decided to perform emergency cesarean section because of previous cesarean section history. A laparotomy was performed and pathologists confirmed the presence of a metastatic adenocarcinoma. Four days following the surgery, gastroduodenoscopy revealed a huge ulcerofungating mass was visible in greater curvature of stomach. The final diagnosis was metastatic adenocarcinoma from gastric cancer. Although pseudo-Meigs' syndrome is very rare, it should be carefully considered when evaluating female complaining with ascites in ovarian tumor.

The usefulness of Doppler ultrasonography and the perinatal outcome of fetal anemia treated with intraumbilical venous transfusion / 대한산부인과학회지

OBJECTIVE: We undertook this study to determine the clinical characteristics and the prognostic factors of neonatal survival in patients with fetal anemia who were treated by intraumbilical venous transfusion (IUT). METHODS: From July 2000 to March 2009, 16 cases of fetal anemia were diagnosed at Asan Medical Center in Seoul, Korea. These patients underwent intraumbilical venous transfusions and were thus included in our study. Doppler measurement of the middle cerebral artery peak systolic velocity was performed before and after cordocentesis in all fetuses. RESULTS: The gestational age at the time of the diagnosis of anemia ranged from 21.3 to 33.6 weeks. There was a linear correlation between pre- and post-procedure fetal hemoglobin (Hb,MoM, (x)) and the MCA-PSV (MoM, (y)), i.e., y=0.810-0.229x, r2=0.542, CI 0.316-0.141, p<0.005; and y=1.374-0.391x, r2=0.499, CI 0.584-0.197, p<0.005. The survival was better in patients with severe anemia than those with mild to moderate anemia (p<0.05), and survival was better in patients with anemia of a known cause than those with anemia of an unknown cause (p<0.001). CONCLUSION: In fetuses with anemia, the severity of the anemia before IUT and the change of hemoglobin concentration after IUT, can be estimated noninvasively using Doppler ultrasonography, on the basis of an increase in the peak velocity of systolic blood flow in the middle cerebral artery. Both severity and etiology were meaningful factors for the survival of neonates with fetal anemia who were treated by intraumbilical venous transfusion. Although fetuses have severe anemia, they expected improved survival through IUT. These data are valuable information for use when counseling the parents of an affected fetus.

Placental Mesenchymal Dysplasia Associated with a Fetal Unilateral Multicystic Dysplastic Kidney: A Case Report / 대한주산의학회잡지

Placental mesenchymal dysplasia (PMD) is a rare placental disorder that may coexist with a normal fetus but is frequently associated with fetal growth restriction, fetal demise, prematurity, and Beckwith-Wiedemann syndrome, as well as with various fetal anatomical and vascular anomalies. In this case, ultrasonography performed at 14 weeks of gestation revealed multiple, variable sized cysts in the placenta along with a morphologically normal fetus. Serial sonographic examinations found that the fetus became small for gestational age and had a dysplastic right kidney. After delivery at 33 weeks of gestation, histopathologic examination of the placenta showed enlarged, hydropic stem villi with cavity formation and absence of trophoblastic proliferation and pseudoinclusions, all of which were consistent with PMD. Neonatal abdominal ultrasonography showed multiple cysts in the right kidney consistent with a multicystic dysplastic condition. This appears to be the first case of PMD associated with fetal multicystic dysplastic kidney.