Objective The objective of the study was to examine the effect of selective fetoscopic laser photocoagulation (SFLP) vs serial amnioreduction (AR) on perinatal mortality in severe twin-twin ...transfusion syndrome (TTTS). Study Design This was a 5 year multicenter, prospective, randomized controlled trial. The primary outcome variable was 30 day postnatal survival of donors and recipients. Results There was no statistically significant difference in 30-day postnatal survival between SFLP or AR treatment for donors at 55% (11 of 20) vs 55% (11 of 20) ( P = 1.0, odds ratio OR 1, 95% confidence interval CI 0.242 to 4.14) or recipients at 30% (6 of 20) vs 45% (9 of 20) ( P = .51, OR 1.88, 95% CI 0.44 to 8.64). There was no difference in 30 day survival of 1 or both twins on a per-pregnancy basis between AR at 75% (15 of 20) and SFLP at 65% (13 of 20) ( P = .73, OR 1.62, 95% CI 0.34 to 8.09). Overall survival (newborns divided by the number of fetuses treated) was not statistically significant for AR at 60% (24 of 40) vs SFLP 45% (18 of 40) ( P = .18, OR 2.01, 95% CI 0.76 to 5.44). There was a statistically significant increase in fetal recipient mortality in the SFLP arm at 70% (14 of 20) vs the AR arm at 35% (7 of 20) ( P = .25, OR 5.31, 95% CI 1.19 to 27.6). This was offset by increased recipient neonatal mortality of 30% (6 of 20) in the AR arm. Echocardiographic abnormality in recipient twin Cardiovascular Profile Score is the most significant predictor of recipient mortality ( P = .055, OR 3.025/point) by logistic regression analysis. Conclusion The outcome of the trial did not conclusively determine whether AR or SFLP is a superior treatment modality. TTTS cardiomyopathy appears to be an important factor in recipient survival in TTTS.
Objective The purpose of this study was to determine the ability of liver position and lung-to-head ratio to predict outcome in isolated left congenital diaphragmatic hernia. Study Design We reviewed ...prenatal studies and postnatal outcomes of congenital diaphragmatic hernia between January 1996 and January 2006. Results Eighty-nine patients received prenatal and postnatal care at 1 institution. In fetuses with liver up, extracorporeal membrane oxygenation was required in 39 of 49 fetuses (80%), compared with 10 of 40 fetuses (25%) for those with liver down ( P < .0001). Overall survival rate was 45%, compared with 93% for those with liver down ( P < .00005). Low lung-to-head ratio (<1.0) predicted increased incidence of extracorporeal membrane oxygenation (75%; P = .036) and lower survival (35%; P = .0003). However, when measured at <24 weeks of gestation, lung-to-head ratio was not predictive of outcome (extracorporeal membrane oxygenation, P = .108; survival, P = .150); liver position remained highly predictive (extracorporeal membrane oxygenation, P = .006; survival, P = .001). Conclusion Liver position is the best prenatal predictor of outcome in isolated left congenital diaphragmatic hernia. Lung-to-head ratio alone should not be used to counsel families regarding mid gestational management choices.
Background Intrapericardial teratoma is a rare, lethal tumor often detected in fetal life. Tumor mass and pericardial effusion cause cardiac tamponade that, if relieved, could be life-saving. Optimal ...timing of intervention and methods for effective fetal treatment are unknown. Objective We describe our single-center experience with fetal intrapericardial teratoma including the first report of successful in utero surgical resection with survival to term. Study Design We reviewed our database for suspected fetal intrapericardial teratoma. On fetal ultrasound and echocardiography tumor size was estimated by calculation of an ellipse and analyzed in relation to Doppler-derived fetal cardiac output, venous flow patterns, hydrops, and outcome. Results Eight fetuses with suspected intrapericardial teratoma were seen from 2009 through 2015. Gestational age at initial presentation ranged from 21-34 (median 26) weeks. Two cases mimicked the appearance of intrapericardial teratoma, but had no serial change in cardiac output over time and were ultimately determined to be other types of tumor. In 6 cases of true intrapericardial teratoma, tumor growth was extremely rapid and associated with progressive decline in cardiac output (to <400 mL/kg/min) manifesting in hydrops and death if left untreated. One case was treated successfully at 31 weeks through ex utero intrapartum delivery with tumor resection while on placental support. Another case underwent open fetal surgery and resection at 24 weeks, with resumption of gestation until delivery at 37 weeks with excellent outcome. Conclusion Fetal intrapericardial teratoma can be successfully managed utilizing serial surveillance and by treatment in a timely manner prior to the predictable onset of hydrops, determined through increasing tumor size and a declining cardiac output. Surgical resection in utero is possible, with good results.
Abstract Congenital diaphragmatic hernia (CDH) is a congenital anomaly that presents with a broad spectrum of severity that is dependent upon components of pulmonary hypoplasia and pulmonary ...hypertension. While advances in neonatal care have improved the overall survival of CDH in experienced centers, mortality and morbidity remain high in a subset of CDH infants with severe CDH. Prenatal predictors have been refined for the past two decades and are the subject of another review in this issue. So far, all randomized trials comparing prenatal intervention to standard postnatal therapy have shown no benefit to prenatal intervention. Although recent non-randomized reports of success with fetoscopic endoluminal tracheal occlusion (FETO) and release are promising, prenatal therapy should not be widely adopted until a well-designed prospective randomized trial demonstrating efficacy is performed. The increased survival and subsequent morbidity of CDH survivors has resulted in the need to provide resources for the long-term follow up and support of the CDH population.
Objective The reproductive and gynecologic outcomes for women after the pregnancy complicated by open maternal-fetal surgery (OMFS) were evaluated. Study Design The retrospective review identified 93 ...women with OMFS from a single institution (1996–2007). Consent and questionnaires were sent to women. Institutional review board approval was obtained from the Committee for Protection of Human Subjects. Results The total return rate was 57.3%. Total pregnancies reported were 47, with 36 delivering after 20 weeks' gestation. The uterine dehiscence and rupture rates were 14% and 14%, respectively. Fetal anomalies occurred in 4 subsequent pregnancies. Normal conception occurred in 98% of subsequent pregnancies. Gynecologic issues were reported by 8 women, with infertility, abdominal pain, and ovarian and uterine factors. Conclusion The reproductive outcomes of uterine dehiscence (14%) and rupture (14%) in a subsequent pregnancy continue to be a major counseling issue for OMFS. Fertility and gynecologic factors do not appear to be increased for women undergoing OMFS.
A 7-year-old girl was referred for ophthalmological examination after the result of a routine school vision screening identified unilateral vision loss. Fundus examination showed bilateral but ...markedly asymmetric macular exudates and optic disk edema. After the results of two blood pressure measurements were within normal limits, a third markedly elevated measurement revealed malignant hypertension and led to a diagnosis of pheochromocytoma, a rare catecholamine-secreting tumor. The tumor was resected, and 6 months later the patient’s blood pressure had normalized; however, although visual acuity had improved to 20/20 in the right eye, it remained 20/200 in the left eye, with decreased disk edema but the persistence of the macular exudates. The identification of an abdominal malignancy through a school vision screening may have saved this child’s life. The need for repeated blood pressure measurement is also highlighted.
Objective A morphometric study was performed to examine the effects of prenatal glucocorticoids, which were administered 48 hours before birth, on muscularization of small pulmonary arterioles (<60 ...μm diameter) in lambs with diaphragmatic hernia (DH) after fetal tracheal occlusion (TO). Study Design DH was created in 23 fetal sheep at 65 days gestation. TO was performed in 16 of 24 fetuses between 110 and 140 days of gestation; 9 of the fetuses were exposed prenatally to betamethasone (0.5 mg/kg body weight) 48 hours before delivery. Six sham-operated animals served as controls. Sections of paraffin that were embedded in lung tissues were stained with Elastin-Van Gieson, and the percentage of medial wall thickness (MWT) was determined. Results The percentage of MWT in DH lambs (29.6% ± 1.9%) was increased compared with sham animals (18.1% ± 1.3%) and was not different from that of DH/TO animals (30.3% ± 1.7%). In DH/TO + glucocorticoid lambs, the percentage of MWT (24.6% ± 1.2%) was significantly lower than in the DH/TO group but was higher than the sham group. Conclusion In fetuses who underwent prolonged TO therapy for severe DH, prenatal glucocorticoid treatment decreased medial hypertrophy of pulmonary arterioles by approximately 19%. We speculate that such structural changes may have contributed to improve gas exchange that was observed in this model.
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