Rika Sugibayashi

Twin Anemia-Polycythemia Sequence after Laser Surgery for Twin-Twin Transfusion Syndrome and Maternal Morbidity

Twin anemia-polycythemia sequence (TAPS) is characterized by large inter-twin hemoglobin value differences without inter-twin amniotic fluid discordance. The management of post-laser TAPS remains controversial. Hence, more studies on TAPS, together with the associated maternal complications and outcome of the fetuses and infants are needed. Between 2003 and 2012, we performed 287 cases of fetoscopic laser photocoagulation for twin-twin transfusion syndrome. Among the 114 who were placed under our care until delivery, three cases of TAPS occurred. In one case, we conducted intrauterine intravenous transfusion, while in the other two cases, we adopted expectant management. We performed an emergency caesarean section at 27-30 weeks of gestation in all cases due to a severe condition of anemia in the TAPS donor. Two cases with antenatal TAPS stage 4 had severe maternal complications; one had minute pulmonary embolism, while the other had Mirror syndrome. All three pairs of infants survived. One TAPS donor and one TAPS recipient had neurodevelopmental impairment; bilateral deafness at 9.5 years old and spastic paralysis at 2 years old, respectively. In conclusion, post-laser TAPS in a higher stage can cause severe maternal complications. Close observations for both fetuses and mothers are required for such cases.

Forty cases of twin reversed arterial perfusion sequence treated with radio frequency ablation using the multistep coagulation method: a single-center experience.

To report the pregnancy outcomes of patients with twin reversed arterial perfusion (TRAP) sequence treated by radiofrequency ablation (RFA).

Stomach herniation predicts fetal death or non-reassuring fetal status in gastroschisis at late pregnancy

few have assessed the findings associated with IUFD or NRFS.Theobjectiveofthisstudywastoelucidatesuchantenatal findings.We identified 24 cases of fetal gastroschisis, which werediagnosed antenatally, all delivered at our hospital betweenJuly 2003 and July 2012. We excluded four cases of inducedand one case of spontaneous abortion all before 22weeks ofgestation. In the 19 cases, which were expectantly managedduring pregnancy, check up with ultrasonography wasperformed every 2weeks until 34weeks of gestation afterdiagnosis. After 34weeks, ultrasonography was performedonce a week, and a nonstress test was performed twice a weekuntil delivery. In all cases, elective cesarean section wasscheduled near term. The timing of the cesarean delivery wasdetermined on the basis of obstetric indications. Thisretrospective study was conducted with the approval of theinstitutional review board of the National Center for ChildHealth and Development in Japan.We classified the 19 cases into two groups, that is, eithercomplicatedbyIUFDorNRFS( n=7)orcaseswithreassuringfetalstatus(RFS) (n=12). In the IUFD/NRFS group, there was one fetaldeath at 34weeks of gestation and six cases undergoingemergency cesarean section due to NRFS. The RFS groupconsisted of 12 cases all delivered by scheduled cesarean section.We compared prenatal (e.g., stomach herniation,

Fetoscopic laser photocoagulation for amniotic fluid discordance bordering on twin–twin transfusion syndrome: Feasibility, perinatal and long-term outcomes

This study investigated the feasibility and safety of fetoscopic laser photocoagulation (FLP) for amniotic fluid discordance (AFD) bordering on twin–twin transfusion syndrome (TTTS) with an absent or reverse end‐diastolic velocity (AREDV) in the umbilical artery (UA), and evaluated the perinatal and long‐term outcomes.

Fetoscopic laser photocoagulation for twin–twin transfusion syndrome

The aim of this study was to review fetoscopic laser photocoagulation (FLP), which ablates placental vascular anastomoses to treat twin–twin transfusion syndrome (TTTS). A review of studies reporting on the procedures, outcomes, complications and nonconventional applications of FLP for TTTS was conducted. FLP has been established as the primary treatment for monochorionic twin pregnancy associated with TTTS at 16–26 weeks. FLP is the only therapy that directly addresses the underlying pathophysiology. The recent technique modification of FLP, referred to as the ‘Solomon technique’, induces selective coagulation to connect the anastomoses ablation sites and has been introduced to reduce residual anastomoses. The perinatal survival following FLP improved significantly with advances in the technique after its introduction. The recent survival rates of both twins and at least one twin are 70% and more than 90%, respectively. However, there is still an 11–14% risk of long‐term neurodevelopment impairment. The premature rupture of membranes that leads to preterm labor is a common complication after FLP. FLP is a valuable treatment option for feto‐fetal transfusion syndrome in triplets and for TTTS after 26 weeks. FLP for selective intrauterine growth restriction may be potentially beneficial when accompanied by abnormal Doppler findings and oligohydramnios. FLP is the optimal treatment option for TTTS at 16–26 weeks of gestation. FLP appears to be applicable in triplets, TTTS after 26 weeks and cases of selective intrauterine growth restriction with abnormal Doppler findings and oligohydramnios. FLP is the most common and successful fetal intervention. Improvement in the neurodevelopmental outcomes after FLP is a future focus.

Fetal lung size after thoracoamniotic shunting reflects survival in primary fetal hydrothorax with hydrops

To assess the role of lung size and abnormal Doppler findings in the umbilical artery (UA) in determining the outcomes of fetuses with primary fetal hydrothorax (FHT) associated with hydrops who underwent thoracoamniotic shunting (TAS).

Stomach herniation prior to nonreassuring fetal status in a case of fetal gastroschisis

The neonatal prognosis in gastroschisis is generally good; however, intrauterine fetal death (IUFD) and nonreassuring fetal status (NRFS) sometimes occur in fetal cases of gastroschisis. Previously, we reported stomach herniation as a possible risk factor for IUFD or NRFS in fetuses with gastroschisis. We report a case of fetal gastroschisis showing stomach herniation and increased velocity of the umbilical venous (UV) flow the day before NRFS by electronic fetal monitoring (EFM). A 27‐year‐old pregnant woman was followed up from 16 weeks’ gestation due to fetal gastroschisis. At 33 + 1 weeks’ gestation, EFM displayed a reassuring fetal state, while the ultrasound examination revealed fetal stomach herniation and an increased flow velocity of the UV in the abdomen. One day later, EFM displayed NRFS, and the patient underwent emergency caesarean section. This case showed stomach herniation preceding the occurrence of NRFS. Stomach herniation may be a predictor of NRFS in fetuses with gastroschisis.

Chronic histiocytic intervillositis in three consecutive pregnancies in a single patient: Differing clinical results and pathology according to treatment used

Chronic histiocytic intervillositis (CHI) is an extremely rare pathological condition but is strongly associated with severe obstetric complications and has a high recurrence rate. The management of this condition has not yet been established. We describe herein the occurrence of CHI in the late second–third trimester in each of three consecutive pregnancies in a single patient with four previous consecutive early miscarriages. In this patient, each of the three complicated pregnancies was managed with one of the following, respectively: low‐dose aspirin; heparin plus low‐dose aspirin; and prednisolone plus low‐dose aspirin. CHI was histologically confirmed in all three pregnancies, but the clinical results and pathology (e.g. extent of histiocytic infiltration) in each pregnancy clearly differed with treatment. Both combination treatments eventuated in a live birth. Immunosuppressive therapy seemed to produce better clinical results by restricting the extent of the affected areas. The elevated alkaline phosphatase associated with the CHI was assumed to have no clinical prognostic value.

Prenatally diagnosed, right-sided congenital diaphragmatic hernia complicated by hepatic pulmonary fusion and intrathoracic kidney

Right-sided, congenital diaphragmatic hernia is not common but accounts for about 20% of all congenital diaphragmatic hernia. It sometimes has serious complications, such as intrathoracic kidney and hepatic pulmonary fusion. Here we report a rare case of right-sided diaphragmatic hernia, complicated by intrathoracic kidney and hepatic pulmonary fusion. Repair of the diaphragm via the abdominal approach was difficult and the disease recurred. The second operation was through a right thoracotomy and was successful. The biopsied specimen of the diaphragm contained hepatocytes and bile duct cells which strongly suggested the close relationship between liver and the diaphragm in this case. Right-sided, congenital diaphragmatic hernia (R-CDH) is rare compared to left-sided CDH and accounts for about 20% of the disease. R-CDH is sometimes difficult to diagnose prenatally and Duess et al. reported that prenatal diagnosis and patch closure may be risk factors for increased mortality. Furthermore, R-CDH may be complicated by some specific pathophysiological states, such as intrathoracic kidney and hepatic pulmonary fusion, which is rare but causes complicated and difficult problems for the surgical repair of the diaphragm. In this report, we describe a rare case with the combination of intrathoracic kidney and hepatic pulmonary fusion in R-CDH and discuss the etiology of the disease and surgical treatment. Correspondence to: Yutaka Kanamori, Division of Surgery, Department of Surgical Specialties, National Center for Child Health and Development, Tokyo, Jap2-10-1 Okura, Setagaya, Tokyo 157-8535, Japan, Tel; +81-3-3416-0181, Fax; +81-3-3416-2222; E-mail: kanamori-y@ncchd.go.jp.

Fetal Thoracoamniotic Shunting in a Case of Congenital Pulmonary Airway Malformations with Hydrops Fetalis.

Aim  We report a case of congenital pulmonary airway malformation (CPAM) with hydrops in which the fetus underwent thoracoamniotic shunting. Case Report  A 40-year-old (G1P1) woman was diagnosed with a macrocystic CPAM. Thoracoamniotic shunting was performed at 19 weeks of gestation but not well drained and was successfully performed again at 23 weeks. However, the CPAM volume ratio, abdominal circumference, and amniotic fluid index started increasing from 28 weeks and hydrops worsened. The insufficient shunting and the fetal cardiac failure had to be considered. At 32 weeks, a male infant with general edema and massive ascites was born weighing 3,362 g (+4.79 SD) with Apgar scores of 2 and 4. The infant was intubated and high-frequency oscillation and nitric oxide therapies were instituted. The resection of CPAM was performed on day 2. Nasal continuous positive airway pressure was instituted on day 16. The infant was discharged and prescribed with home oxygen therapy (HOT) on day 65. The infant was able to leave the HOT at 30 months and is currently 34 months of age in good condition. Conclusion  Fetal thoracoamniotic shunting may be life-saving in CPAM complicated by hydrops and that this treatment might be sufficient to cure the child.