Percutaneous versus Open Fetal Surgical Intervention for Sacrococcygeal Teratomas: Is Less Really More?

INTRODUCTION: Sacrococcygeal teratomas (SCTs) may require in utero intervention for survival. Open surgical intervention (OSI) was first described, but increasing reports of percutaneous intervention (PI) with variable indications and outcomes exist. We reviewed the literature for all published cases of fetal SCT intervention and compared OSI to PI cohorts. METHODS: A keyword search of PubMed was conducted. Inclusion criteria were as follows: data available per individual fetus including gestational age at intervention, type of intervention, primary indication, survival, gestational age at birth, and complications. Complications were grouped into categories: placenta/membrane, procedural, or hemorrhagic. Failure was defined as little/no improvement or recurrence of the primary indication. χ2 analysis was performed for solid tumor PI versus OSI to assess significant trends in these intervention groups. A meta-analysis was not feasible due to small numbers and heterogeneity. RESULTS: Twenty-seven articles met inclusion criteria. In the PI group, 38 fetuses underwent intervention for solid tumors, 21 for cystic tumors, and 3 for solid and cystic tumor components. Among fetuses with solid tumors, OSI was associated with lower need for multiple interventions (0% vs. 31.6%, p = 0.01) and higher survival to discharge (50% vs. 39.5%, p = 0.02). A fetal intervention was performed in the absence of hydrops/early hydrops in 45% of fetuses receiving PI, compared to 21% receiving OSI. Failure to resolve the primary indication was higher in the PI group (55.9% vs. 11.1% OSI, p = 0.02). The overall complication rates were high in both groups (90% OSI, 87% PI), though bleeding was unique to the PI group (26.5%). Preemptive cyst drainage, for purely cystic tumors, was universally successful and associated with a low complication risk (18.2%). CONCLUSIONS: For solid tumors, OSI appears to be superior with regard to survival to discharge, fewer interventions, and lower failure rates. PIs to drain a cyst may facilitate delivery or preempt future complications, though consideration should be given to long-term oncologic outcomes.

Cervical lymphatic malformations: Prenatal characteristics and ex utero intrapartum treatment.

BACKGROUND: The ex utero intrapartum treatment (EXIT) is utilized to transition fetuses with prenatally diagnosed airway obstruction to postnatal life. We describe the unique clinical course, diagnosis, treatment, and outcomes of patients with cervical lymphatic malformation (CLM) managed with EXIT. METHODS: Review of fetuses with diagnosed CLM was delivered by EXIT (2001-2018) in a tertiary referral fetal center. Outcomes included survival, tracheostomy at discharge, neonatal course after delivery, and pulmonary hypoplasia. Data are reported as median [range] and rate (%). RESULTS: Out of 45 patients delivered by EXIT, 10 were delivered for CLM: seven had polyhydramnios, one had nonimmune hydrops, five delivered preterm, and three were emergency EXITs. The EXIT time and estimated blood loss were 125 minutes (95, 158) and 900 mL (500, 1500), respectively. Airway was secured in all. There was one neonatal death (day 8) with prematurity, sepsis, and pulmonary hypoplasia. Three out of nine were discharged with a tracheostomy. CONCLUSION: In CLM, close monitoring for structural neck involvement and development of polyhydramnios are important and may be an indication for EXIT as the optimal delivery mode. An experienced multidisciplinary team is a key factor for an effective approach to the obstructed airway in CLM.

Use of venovenous ECMO for neonatal and pediatric ECMO: a decade of experience at a tertiary children's hospital.

BACKGROUND: Advances in extracorporeal membrane oxygenation (ECMO) have led to increased use of venovenous (VV) ECMO in the pediatric population. We present the evolution and experience of pediatric VV ECMO at a tertiary care institution. METHODS: A retrospective cohort study from 01/2005 to 07/2016 was performed, comparing by cannulation mode. Survival to discharge, complications, and decannulation analyses were performed. RESULTS: In total, 160 patients (105 NICU, 55 PICU) required 13 ± 11 days of ECMO. VV cannulation was used primarily in 83 patients with 64% survival, while venoarterial (VA) ECMO was used in 77 patients with 54% survival. Overall, 74% of patients (n = 118) were successfully decannulated; 57% survived to discharge. VA ECMO had a higher rate of intra-cranial hemorrhage than VV (22 vs 9%, p = 0.003). Sixteen VA patients (21%) had radiographic evidence of a cerebral ischemic insult. No cardiac complications occurred with the use of dual-lumen VV cannulas. There were no differences in complications (p = 0.40) or re-operations (p = 0.85) between the VV and VA groups. CONCLUSION: Dual-lumen VV ECMO can be safely performed with appropriate image guidance, is associated with a lower rate of intra-cranial hemorrhage, and may be the preferred first-line mode of ECMO support in appropriately selected NICU and PICU patients. LEVEL OF EVIDENCE: II.

Outcome and Treatment of Antenatally Diagnosed Nonimmune Hydrops Fetalis.

INTRODUCTION: The objectives of this study were to evaluate the outcome of nonimmune hydrops fetalis in an attempt to identify independent predictors of perinatal mortality. MATERIAL AND METHODS: A retrospective cohort study was conducted including all cases of nonimmune hydrops from two tertiary care centers. Perinatal outcome was evaluated after classifying nonimmune hydrops into ten etiological groups. We examined the effect of etiology, site of fluid accumulation, and gestational age at delivery on postnatal survival. Neonatal mortality and hospital discharge survival were compared between the expectant management and fetal intervention groups among those with idiopathic etiology. RESULTS: A total of 142 subjects were available for analysis. Generally, nonimmune hydrops carried 37% risk of neonatal mortality and 50% chance of survival to discharge, which varies markedly based on the underlying etiology. Ascites was an independent predictor of perinatal mortality (p value = 0.003). There was nonsignificant difference in neonatal mortality and hospital discharge survival among idiopathic cases that were managed expectantly versus those in whom fetal intervention was carried out. DISCUSSION: The outcome of nonimmune hydrops varies largely according to the underlying etiology and the presence of ascites is an independent risk factor for perinatal mortality. In our series, fetal intervention did not offer survival advantage among fetuses with idiopathic nonimmune hydrops.

Fetal left-sided cardiac structural dimensions in left-sided congenital diaphragmatic hernia - association with severity and impact on postnatal outcomes.

OBJECTIVES: Fetuses with congenital diaphragmatic hernia (CDH) demonstrate varying degrees of left heart hypoplasia. Our study assesses the relationship between fetal left-sided cardiac structural dimensions, lung size, percentage liver herniation, lung-to-head ratio, postnatal left-sided cardiac structural dimensions, and postnatal outcomes. METHODS: We performed a retrospective cohort study of fetuses with left-sided CDH who had prenatal echocardiographic, ultrasound, and magnetic resonance imaging examinations at our institution between January 2007 and March 2015. Postnatal outcomes assessed include use of inhaled nitric oxide (iNO), use of extracorporeal membrane oxygenation, and death. RESULTS: Fifty-two fetuses with isolated left-sided CDH were included. Multivariate logistic regression models indicated that smaller fetal aortic valve z-score was associated with postnatal use of iNO (p = 0.03). Fetal mitral valve z-score correlated with lung-to-head ratio (p = 0.04), postnatal mitral valve z-score correlated with percent liver herniation (p = 0.03), and postnatal left ventricular end-diastolic dimension z-score correlated with liver herniation <20% (p = 0.04). CONCLUSION: We identified associations between smaller fetal left-sided cardiac structural dimensions and classic CDH indices. Smaller aortic valve z-score was associated with iNO use; however, left heart dimensions showed no association with extracorporeal membrane oxygenation or mortality. Further study into the impact of left-sided hypoplasia on outcomes in CDH is worthy of evaluation in a larger, prospective study. © 2017 John Wiley & Sons, Ltd.

Fetal Treatment 2017: The Evolution of Fetal Therapy Centers - A Joint Opinion from the International Fetal Medicine and Surgical Society (IFMSS) and the North American Fetal Therapy Network (NAFTNet).

More than 3 decades ago, a small group of physicians and other practitioners active in what they called "fetal treatment" authored an opinion piece outlining the current status and future challenges anticipated in the field. Many advances in maternal, neonatal, and perinatal care and diagnostic and therapeutic modalities have been made in the intervening years, yet a thoughtful reassessment of the basic tenets put forth in 1982 has not been published. The present effort will aim to provide a framework for contemporary redefinition of the field of fetal treatment, with a brief discussion of the necessary minimum expertise and systems base for the provision of different types of interventions for both the mother and fetus. Our goal will be to present an opinion that encourages the advancement of thoughtful practice, ensuring that current and future patients have realistic access to centers with a range of fetal therapies with appropriate expertise, experience, and subspecialty and institutional support while remaining focused on excellence in care, collaborative scientific discovery, and maternal autonomy and safety.

Defining and predicting 'intrauterine fetal renal failure' in congenital lower urinary tract obstruction.

BACKGROUND: The aim of this study was to identify predictors of 'intrauterine fetal renal failure' in fetuses with severe congenital lower urinary tract obstruction (LUTO). METHODS: We undertook a retrospective study of 31 consecutive fetuses with a diagnosis of LUTO in a tertiary Fetal Center between April 2013 and April 2015. Predictors of 'intrauterine fetal renal failure' were evaluated in those infants with severe LUTO who had either a primary composite outcome measure of neonatal death in the first 24 h of life due to severe pulmonary hypoplasia or a need for renal replacement therapy within 7 days of life. The following variables were analyzed: fetal bladder re-expansion 48 h after vesicocentesis, fetal renal ultrasound characteristics, fetal urinary indices, and amniotic fluid volume. RESULTS: Of the 31 fetuses included in the study, eight met the criteria for 'intrauterine fetal renal failure'. All of the latter had composite poor postnatal outcomes based on death within 24 h of life (n = 6) or need for dialysis within 1 week of life (n = 2). The percentage of fetal bladder refilling after vesicocentesis at time of initial evaluation was the only predictor of 'intrauterine fetal renal failure' (cut-off <27 %, area under the time-concentration curve 0.86, 95 % confidence interval 0.68-0.99; p = 0.009). CONCLUSION: We propose the concept of 'intrauterine fetal renal failure' in fetuses with the most severe forms of LUTO. Fetal bladder refilling can be used to reliably predict 'intrauterine fetal renal failure', which is associated with severe pulmonary hypoplasia or the need for dialysis within a few days of life.

Factors associated with fetal shunt dislodgement in lower urinary tract obstruction.

OBJECTIVE: To identify factors associated with fetal shunt dislodgement in lower urinary tract obstruction (LUTO). METHODS: We conducted a retrospective study of 42 consecutive fetuses with a diagnosis of LUTO in a tertiary fetal center between April 2013 and November 2015. Possible factors associated with prenatal shunt dislodgment were evaluated in fetuses who underwent shunt placement, including gestational age at diagnosis, gestational age at procedure, presence of 'keyhole sign', initial fetal bladder volume and wall thickness, prenatal ultrasonographic renal characteristics, amniotic fluid volume, presence of ascites prior to shunting, and type of fetal shunt. RESULTS: Nineteen (46.3%) fetuses underwent shunt placement at a median gestational age of 19 (range: 16.3-31.1) weeks. Shunt dislodgement occurred in 10 (52.6%) patients. A total of 35 procedures were performed; among which 16 (45.7%) were repeat procedures. The only prenatal factor associated with shunt dislodgement was the type of the shunt; Kaplan-Meier analysis indicated that the Rocket was associated with increased likelihood of remaining orthotopic (p = 0.04). CONCLUSION: Fetal shunt dislodgement occurs in approximately half of the patients and appears to be associated with the type of the shunt. Future research is necessary to develop better shunt systems and to investigate different fetal therapeutic approaches. © 2016 John Wiley & Sons, Ltd.

Improving the Prediction of Neonatal Outcomes in Isolated Left-Sided Congenital Diaphragmatic Hernia by Direct and Indirect Sonographic Assessment of Liver Herniation.

OBJECTIVES: Liver herniation can be assessed sonographically by either a direct (liver-to-thoracic area ratio) or an indirect (stomach position) method. Our objective was to evaluate the utility of those methods to assess liver herniation for the prediction of neonatal outcomes in patients with isolated left-sided congenital diaphragmatic hernia (CDH). METHODS: We conducted a retrospective cohort study of all patients with CDH who had prenatal assessment and were delivered at Texas Children's Hospital between January 2004 and April 2014. The predictive value of sonographic parameters for mortality and the need for extracorporeal membrane oxygenation was evaluated by univariate, multivariate, and factor analysis and by receiver operating characteristics curves. RESULTS: A total of 77 fetuses with isolated left-sided CDH were analyzed. The lung-to-head ratio, liver-to-thorax ratio, and stomach position (according to the classifications of Kitano et al [Ultrasound Obstet Gynecol 2011; 37:277-282] and Cordier et al [J Matern Fetal Neonatal Med 2015; 28:190-195]) were significantly associated with both neonatal outcomes (P < .03). Significant correlations were observed between all of these sonographic parameters. A combination of the liver-to-thorax ratio and stomach position (Kitano) or stomach position (Cordier) with the lung-to-head ratio increased the area under the receiver operating characteristic curve of the lung-to-head ratio for mortality prediction (0.86 [95% confidence interval, 0.74-0.98], 0.83 [0.72-0.95], and 0.83 [0.74-0.92], respectively). CONCLUSIONS: Sonographic measurements of liver herniation (liver-to-thorax ratio and stomach position) are predictive of neonatal outcomes in isolated left-sided congenital diaphragmatic hernia. Our study shows that the combination of those sonographic measurements of liver herniation and lung size improves the accuracy of predicting mortality in those fetuses.

Communication about maternal-fetal surgery for myelomeningocele and congenital diaphragmatic hernia: preliminary findings with implications for informed consent and shared decision-making.

OBJECTIVE: To examine the style and content of consultations for maternal-fetal surgery and draw conclusions about best practices for informed consent and shared decision-making. STUDY DESIGN: Qualitative study of 15 h-long consultations with women diagnosed with fetal myelomeningocele (MMC, n=11) or congenital diaphragmatic hernia (CDH, n=4) who were potential candidates for maternal-fetal surgery at a large children's hospital in the Southwestern US. RESULTS: Major findings were that physicians tended to discuss the risks of fetal prognosis qualitatively more often than quantitatively (70% compared to 30%) and when mortality was a risk the "positive" (percentage survival) frame was always given rather than the morality frame. On average, families only talked 15% of the time and 45% of all their questions were about diagnostic or surgical procedure clarification. CONCLUSION: Efforts should be made to minimize qualitative presentation of risk, which can be vague and confusing to patients. Both survival and mortality frames should be used to avoid biased decision-making. Communication and decision support tools that facilitate more shared decision-making between families and physicians are needed.

Management of appendiceal carcinoid tumors in children.

BACKGROUND: Appendiceal carcinoid tumors, also know as well-differentiated neuroendocrine neoplasms, are rare lesions detected incidentally after appendectomy in children. There are limited data about the natural history of these tumors, and guidelines regarding family counseling and need for additional surgery or follow-up imaging are not established in the pediatric age group. The purpose of this study was to review our institutional experience with appendiceal carcinoid tumors to provide data that might improve management. METHODS: After institutional review board approval, the charts of all patients treated at our institution for an appendiceal carcinoid tumor between 2002 and 2014 were reviewed. Data collected included patient demographics, pathologic details, postoperative management, and follow-up information. Descriptive analyses were performed. RESULTS: Twenty-eight patients were identified, which represents an incidence of 0.2% of children undergoing appendectomy during that time interval. The mean age at surgery was 13.8 ± 2.1 y; 54% were females. Two patients had symptoms suspicious for carcinoid syndrome at presentation, though none had evidence of metastatic disease. The mean tumor size was 0.73 ± 0.4 cm. Five patients (18%) underwent subsequent ileocecectomy or right hemicolectomy because of pathologic findings of invasion of the mesoappendix (n = 4) or lymphovascular invasion and subserosal extension (n = 1), two of whom had residual disease in the resected specimen (one in a lymph node). No recurrences have been detected at mean follow-up of 1.8 y. CONCLUSIONS: Appendiceal carcinoid tumors are discovered incidentally in about 0.2% of children undergoing appendectomy. Based on findings from a large contemporary series, we can conclude that these tumors are generally small and demonstrate lymphovascular invasion or mesenteric extension in fewer than 20% of cases. Prospective, multicenter studies are necessary to better define the indication for ileocecectomy and follow-up imaging protocols.

Giant omphaloceles: surgical management and perinatal outcomes.

BACKGROUND: The purpose of this study was to describe the current management and outcomes of infants with omphalocele. METHODS: The medical records of all patients treated for omphalocele at a large children's hospital from January, 2003-February, 2014 were reviewed. Patients were classified as having an isolated omphalocele or omphalocele with minor or major associated anomalies. Prenatal data collected included fetal magnetic resonance imaging-based observed-to-expected total fetal lung volumes. Giant omphalocele (GO) was defined as >50% of liver in the omphalocele sac. RESULTS: Of 95 patients, 59 presented prenatally and had comprehensive fetal center evaluation. Of 82 live-born infants, 21 had chromosomal and 25 had major associated anomalies. No live-born baby with an isolated defect (n = 19) died, whereas mortality was 41% and 17% for those with major and minor anomalies, respectively (P = 0.006). Infants with major anomalies had significantly longer median length of intubation (36 versus 0 versus 0 d; P = 0.04) and hospital stay (157 versus 28.5 versus 18 d; P < 0.001) compared with those with minor or no anomalies. Of 40 infants with GO, the majority (85%) were managed surgically by delayed closure with a median age at repair of 10 mo (range, 3.4-23.6 mo). Six-month survival was 80%. None of the delayed repair patients required a later operative revision, whereas 2 of 5 with early repair did. CONCLUSIONS: The presence of associated anomalies is the strongest predictor of morbidity and mortality in fetuses or neonates with omphalocele. In patients with GO, delayed closure is associated with good outcomes, but larger, prospective studies comparing delayed to early closure are needed to determine the optimal timing of repair.

5, 4, 3, 2, 1: embryologic variants of pentalogy of Cantrell.

BACKGROUND: The purpose of this study was to evaluate our experience with pentalogy of Cantrell and the various embryologic variants. MATERIALS AND METHODS: Patient charts and diagnostic imaging studies of all fetuses evaluated at Texas Children's Fetal Center for pentalogy of Cantrell between April 2004 and June 2014 were reviewed retrospectively. Data collected from patient charts included demographic information, clinical presentation, fetal and postnatal imaging findings, operative treatment, pathologic evaluation, and outcomes. RESULTS: There were 10 patients who presented with embryologic variants of pentalogy of Cantrell over a 6-y period. Two cases displayed the full range of embryologic defects observed, and eight cases exhibited variants of the classic pentalogy. Sternal and pericardial defects were each present in 40% of patients. Additional anomalies present included pulmonary hypoplasia, pulmonary artery stenosis, and chromosomal abnormalities. Four patients presented with diaphragmatic defects but no defect in the pericardium, and one patient presented with a defective pericardium but no associated diaphragmatic defect, suggesting highly specific losses of somatic mesoderm during embryologic development. One patient was lost to follow-up, and a second patient underwent termination of pregnancy. Five of the remaining eight patients survived, one of which had the full range of embryologic defects and now attends preschool but requires speech and occupational therapy. The remaining surviving patients have developed without serious sequelae. CONCLUSIONS: This report highlights the spectrum of anomalies observed in the pentalogy of Cantrell and demonstrates that these fetuses can survive but with substantial morbidity.

Revisiting outcomes of right congenital diaphragmatic hernia.

BACKGROUND: Studies comparing outcomes of right- and left-sided congenital diaphragmatic hernia (R-CDH and L-CDH) have yielded conflicting results. We hypothesized that R-CDH is associated with higher short-term pulmonary morbidity than L-CDH. METHODS: We reviewed all CDH patients at a tertiary children's hospital over 10 y. In prenatally diagnosed CDH, the observed-to-expected total fetal lung volume and percentage liver herniation (%LH) were calculated using fetal magnetic resonance imaging-based measurements. Outcomes were compared in patients with isolated CDH. Patients were subsequently matched by %LH to compare outcomes. RESULTS: Of 189 CDH patients, 37 (20.1 %) were R-CDH and 147 (79.9%) were L-CDH. Those with R-CDH were prenatally diagnosed at a significantly lower rate (40.5% versus 73.5%; P < 0.001) and later gestational age (26.5 ± 7.7 versus 22.6 ± 5.65 wk; P = 0.062). There was no difference in observed-to-expected total fetal lung volume between those with R-CDH and L-CDH (30.2 ± 11.1% versus 33.1 ± 14.2%; P = 0.471). Fetuses with R-CDH had a higher %LH than those with L-CDH (37.5 ± 14.1% versus 18.6 ± 12.2%; P < 0.001). Patients with isolated R-CDH had a higher need for extracorporeal membrane oxygenation than L-CDH (48% versus 27%; P = 0.055). There was no difference in duration of tracheal intubation, hospital stay, need for supplemental oxygen at 30-d of life or 6-mo mortality between groups. There was no difference in mortality and pulmonary morbidity when patients were matched by %LH. CONCLUSIONS: Compared to those with L-CDH, fetuses with R-CDH are less likely to be diagnosed prenatally and have a higher need for extracorporeal membrane oxygenation. The sidedness of the hernia defect was not associated with differences in short-term pulmonary morbidity in this large, contemporary single-institution experience of neonates with CDH.

Predictive Value of MRI Findings for the Identification of a Hernia Sac in Fetuses With Congenital Diaphragmatic Hernia.

OBJECTIVE: The purpose of this study was to identify MRI features of diaphragmatic hernia sac, as well as to assess the accuracy of diagnosing a sac prenatally. MATERIALS AND METHODS: All fetal MRI examinations performed for intrapleural congenital diaphragmatic hernia (CDH) from 2004 to 2013 were retrospectively reviewed by two pediatric radiologists blinded to the hernia sac status (defined intraoperatively or at autopsy). Reviewers noted whether a sac was present on the basis of identification of the following four MRI findings: 1, meniscus of lung posterior or apical to the hernia contents; 2, encapsulated appearance of hernia contents, exerting less than expected mass effect on the heart and mediastinum; 3, presence of pleural fluid outlining a sac from above; and 4, presence of ascites outlining a sac from below. Sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) were calculated for each finding and for various combinations. Contingency tables, chi-square testing, and logistic regression were applied to calculate the probability of a sac. RESULTS: Ninety patients were included: 21 with and 69 without a sac. The first three MRI findings correlated with the presence of a sac. Logistic regression yielded high predicted probability of a sac when one finding was identified (finding 1, 94.4%; finding 2, 96.2%). Adding a second and a third finding improved the probability to 99.7% and 99.9%, respectively. Sensitivity and specificity for the presence of a sac were 0.43 and 0.97, respectively. PPV and NPV were 83.8% and 80%, respectively. CONCLUSION: On fetal MRI, presence of a hernia sac in CDH can be diagnosed with high specificity when indicative findings are present.

Comparison between laparoscopically assisted and standard fetoscopic laser ablation in patients with anterior and posterior placentation in twin-twin transfusion syndrome: a single center study.

BACKGROUND: The objective of our study was to compare outcomes following laparoscopically assisted procedure (LAP group) with those seen following a standard approach used in patients with either an anterior placenta (SAP group) or posterior placenta (SPP group). METHOD: This was a retrospective review of all the cases of twin-twin transfusion syndrome treated in our fetal center from October 2011 to July 2013. Technical characteristics of the procedure, perinatal survival outcome, and maternal morbidity were compared. RESULTS: The laser procedure time was significantly longer in the SAP group (44 ± 10 min) in contrast with SPP (19.3 ± 13.9 min, p < 0.001) and LAP group (32 ± 11 min, p: 0.012). Preterm premature rupture of membranes (PPROM) before 32 and 34 weeks of pregnancy was significantly more common with LAP versus SAP and SPP (90 vs 33.3 and 70.8% for 32 weeks respectively, p: 0.015; 100 vs 50 and 79.1% for 34 weeks respectively, p: 0.021). In terms of maternal morbidity and neonatal outcome, there were no significant differences between the three groups. CONCLUSION: LAP may be useful in cases where SAP is not feasible. Despite the increased risk of PPROM with LAP, perinatal survival and maternal outcomes are similar to that seen in SAP and SPP patients.

Standardization of Sonographic Lung-to-Head Ratio Measurements in Isolated Congenital Diaphragmatic Hernia: Impact on the Reproducibility and Efficacy to Predict Outcomes.

OBJECTIVES: The purpose of this study was to evaluate the impact of standardization of the lung-to-head ratio measurements in isolated congenital diaphragmatic hernia on prediction of neonatal outcomes and reproducibility. METHODS: We conducted a retrospective cohort study of 77 cases of isolated congenital diaphragmatic hernia managed in a single center between 2004 and 2012. We compared lung-to-head ratio measurements that were performed prospectively in our institution without standardization to standardized measurements performed according to a defined protocol. RESULTS: The standardized lung-to-head ratio measurements were statistically more accurate than the nonstandardized measurements for predicting neonatal mortality (area under the receiver operating characteristic curve, 0.85 versus 0.732; P = .003). After standardization, there were no statistical differences in accuracy between measurements regardless of whether we considered observed-to-expected values (P > .05). Standardization of the lung-to-head ratio did not improve prediction of the need for extracorporeal membrane oxygenation (P> .05). Both intraoperator and interoperator reproducibility were good for the standardized lung-to-head ratio (intraclass correlation coefficient, 0.98 [95% confidence interval, 0.97-0.99]; bias, 0.02 [limits of agreement, -0.11 to +0.15], respectively). CONCLUSIONS: Standardization of lung-to-head ratio measurements improves prediction of neonatal outcomes. Further studies are needed to confirm these results and to assess the utility of standardization of other prognostic parameters.

Pulmonary capillary hemangiomatosis in a neonate with congenital diaphragmatic hernia.

Pulmonary capillary hemangiomatosis (PCH) is a rare cause of pulmonary hypertension (PHTN). We present a neonate with congenital diaphragmatic hernia (CDH) and concurrent PCH. Severe PHTN was unrelenting and death occurred at 4 months. Diagnosis of PCH is challenging in the setting of CDH and portends a poor prognosis.

Comparing characteristics and outcomes in infants with prenatal and postnatal diagnosis of esophageal atresia.

BACKGROUND: Previous studies of infants with esophageal atresia (EA) suggest those diagnosed prenatally have worse outcomes because of a higher incidence of associated anomalies. The purpose of this study was to compare characteristics and outcomes of infants with EA diagnosed after fetal center evaluation to those diagnosed postnatally. METHODS: The records of all neonates treated for EA at our institution from 2002-2012 were reviewed. Infants with a prenatal diagnosis of EA were compared with those postnatally diagnosed using chi-square and Student t-test as appropriate. RESULTS: Of 91 patients treated with EA during the study period, 15 (16%) were diagnosed prenatally at our fetal center. Although those prenatally diagnosed had a higher incidence of pure EA and polyhydramnios, the gestational age and birth weight in that group were similar to those diagnosed postnatally. There were no differences in outcomes between groups with regard to the incidence of major cardiac anomalies, surgical complications, hospital length of stay, and survival. CONCLUSIONS: Treatment at a tertiary care center provides excellent outcomes for all infants with EA, despite an 80% frequency of concurrent anomalies. Prenatal diagnosis of EA and attentive obstetric management of polyhydramnios decrease the risk for prematurity and prematurity-associated morbidity.