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.

Use of Image Guidance During Pediatric Venovenous Extracorporeal Membrane Oxygenation Cannulation: A Survey of the American Pediatric Surgical Association.

This study evaluated practices for image guidance during placement of bicaval dual-lumen (BCDL) venovenous extracorporeal membrane oxygenation (VV-ECMO) cannulas in pediatric and adolescent patients and elucidates reasoning behind surgeon practices. A survey covering VV-ECMO practice and opinions was distributed by the American Pediatric Surgical Association (APSA) to all attending members. A total of 110 pediatric surgeons responded (11.3%). During initial BCDL cannula placement, 67.7% of surgeons reported using bimodal imaging with either fluoroscopy (38.4%) or x-ray (29.3%) plus echocardiography. Although 37.4% of surgeons used serial x-rays during cannula placement, only 5.9% believed it was best practice to do so ( P < 0.0001). Rather, 60.4% believed that fluoroscopy was the standard. Among surgeons not using fluoroscopy, 27.6% (13.3% of respondents) reported fluoroscopy added unnecessary complexity or that they preferred another modality. More frequently, reasons for not using fluoroscopy are related to resource limitations. Echocardiography use to confirm cannula position was considered best practice by 92.1% of surgeons, with 86.9% utilization. Therefore, most pediatric surgeons use multimodal imaging during cannulation and consider it best practice. Fluoroscopy is preferred, but its use is frequently limited by hospital resources. Echocardiography is widely available and used. These data represent increased consensus among surgeons and present opportunities for modernization of hospital resources and standards.

Discontinuation of the OriGen® Dual-Lumen Right Atrial Cannula Decreased Venovenous ECMO Usage in Neonates and Older Children: A Survey of the American Pediatric Surgical Association.

INTRODUCTION: Dual-lumen cannulas for venovenous (VV)-ECMO are widely used in pediatric patients. The popular OriGen® dual-lumen right atrial cannula was discontinued in 2019 without a comparable replacement. METHODS: A survey covering VV-ECMO practice and opinions was distributed to attending members of the American Pediatric Surgical Association. RESULTS: 137 pediatric surgeons responded (14%). Prior to discontinuation of the OriGen®, 82.5% offered VV-ECMO to neonates, and 79.6% cannulated with the OriGen®. Following its discontinuation, those that offered only venoarterial (VA)-ECMO to neonates increased to 37.6% from 17.5% (p = 0.0002). An additional 33.8% changed their practice to sometimes use VA-ECMO when VV-ECMO was indicated. Reasons for not incorporating dual-lumen bi-caval cannulation into practice included risk of cardiac injury (51.7%), inexperience with bi-caval cannulation in neonates (36.8%), difficulty with placement (31.0%), and recirculation and/or positioning problems (27.6%). For the pediatric/adolescent population, 95.5% of surgeons offered VV-ECMO prior to OriGen® discontinuation. Few switched to exclusive VA-ECMO (1.9%) when the OriGen® was discontinued, but 17.8% of surgeons began to incorporate selective use of VA-ECMO. CONCLUSION: Discontinuation of the OriGen® cannula drove pediatric surgeons to alter their cannulation practices, dramatically increasing VA-ECMO use for neonatal and pediatric respiratory failure. These data may suggest a need for targeted education accompanying major technological shifts. LEVEL OF EVIDENCE: Level IV.

Fetal abdominal tumors and cysts.

This article reviews the contemporary diagnosis and management of the most common abdominal neoplasms and cystic lesions diagnosed in the fetus. Fetal tumors discussed include teratomas (sacrococcygeal, cervical or mediastinal), mesoblastic nephroma, nephroblastoma (Wilms' tumor), neuroblastoma, and hepatoblastoma. Fetal abdominal cystic lesions discussed include ovarian cyst, choledochal cyst, intestinal duplication cyst, mesenteric cyst, simple hepatic cyst, and meconium pseudocyst. We discuss the rare indications for fetal intervention or fetal surgery and other perinatal management, including prenatal interventions and fetal surgery for sacrococcygeal teratoma. The lesions reviewed are detected by widespread use of screening ultrasonography during pregnancy. Work-up for these abnormalities may include fetal MRI which enhances the diagnostic accuracy of abdominal tumors and cystic lesions and can aid in characterization of the lesion in relationship to surrounding anatomic structures. Accurate prenatal diagnosis of such lesions permits recommendations for optimal location and timing of delivery, and inclusion of appropriate caregivers and expertise to facilitate postnatal management. Perinatal management of the fetus with a neoplasm requires consideration of the optimal timing and mode of delivery, and pediatric oncology and surgical specialty care. The majority of tumors diagnosed antenatally have good prognosis with current multimodality treatment.

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.

Early vs late resection of asymptomatic congenital lung malformations.

PURPOSE: To examine postsurgical outcomes of a consecutive series of children treated with elective operations for congenital lung malformations (CLM). METHODS: A retrospective review was performed on a prospectively collected dataset of all fetuses evaluated for a CLM between July 2001 and June 2016. Prenatal findings, operative treatment and postnatal outcomes were collected. Children having elective operations were divided in two groups based on age at time of surgery. RESULTS: Of 220 fetuses, 143 had operations and follow-up at our center. Six had open fetal lobectomy, 17 had EXIT-to-resection, 16 infants had urgent resection for symptoms and 110 with asymptomatic lesions had elective resection. Of these 110, the median fetal maximum CVR was 0.8 [range 0.1-2.2], and median age at operation was 4 (1.5-60) months (58% had resection at ≤4 months). Overall complication rate, including air-leak and pleural effusion, was 15%. When comparing those having resection at ≤4 months to those >4 months, there were no significant differences in complication rates or length of stay. Operative time was shorter for patients with early resection (154 ±â€¯59 vs 181 ±â€¯89, p = 0.05). No infant having resection at ≤4 months was readmitted. Overall, children survived with good recovery. CONCLUSION: Early elective resection of congenital lung malformations prior to 4 months of age is feasible and not associated with increased operative risk. TYPE OF STUDY: Restropective study. LEVEL OF EVIDENCE: Level III.

Evaluating quality of life of extracorporeal membrane oxygenation survivors using the pediatric quality of life inventory survey.

PURPOSE: This study assesses the impact of extracorporeal membrane oxygenation (ECMO) associated morbidities on long-term quality of life (QOL) outcomes. METHODS: A single center, retrospective review of neonatal and pediatric non-cardiac ECMO survivors from 1/2005-7/2016 was performed. The 2012 Pediatric Quality of Life Inventory™ (PedsQL™) survey was administered. Clinical outcomes and QOL scores between groups were compared. RESULTS: Of 74 patients eligible, 64% (35 NICU, 12 PICU) completed the survey. Mean time since ECMO was 5.5±3years. ECMO duration for venoarterial (VA) and venovenous (VV) were similar (median 9 vs. 7.5days, p=0.09). VA ECMO had higher overall complication rate (64% vs. 36%, p=0.06) and higher neurologic complication rate (52% vs. 9%, p=0.002). ECMO mode and ICU type did not impact QOL. However, patients with neurologic complications (n=15) showed a trend towards lower overall QOL (63/100±20 vs. 74/100±18, p=0.06) compared to patients without neurologic complications. A subset analysis of patients with ischemic or hemorrhagic intracranial injuries (n=13) had significantly lower overall QOL (59/100±19 vs. 75/100±18, p=0.01) compared to patients without intracranial injuries. CONCLUSION: Neurologic complication following ECMO is common, associated with VA mode, and negatively impacts long-term QOL. Given these associations, when clinically feasible, VV ECMO may be considered as first line ECMO therapy. TYPE OF STUDY: Retrospective review. LEVEL OF EVIDENCE: II.

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.

A novel multimodal computational system using near-infrared spectroscopy predicts the need for ECMO initiation in neonates with congenital diaphragmatic hernia.

BACKGROUND/PURPOSE: The purpose of this study was to develop a computational algorithm that would predict the need for ECMO in neonates with congenital diaphragmatic hernia (CDH). METHODS: CDH patients from August 2010 to 2016 were enrolled in a study to continuously measure cerebral tissue oxygen saturation (cStO2) of left and right cerebral hemispheres. NIRS devices utilized were FORE-SIGHT, CASMED and INVOS 5100, Somanetics. Using MATLAB©, a data randomization function was used to deidentify and blindly group patient's data files as follows: 12 for the computational model development phase (6 ECMO and 6 non-ECMO) and the remaining patients for the validation phase. RESULTS: Of the 56 CDH patients enrolled, 22 (39%) required ECMO. During development of the algorithm, a difference between right and left hemispheric cerebral oxygenation via NIRS (ΔHCO) was noted in CDH patients that required ECMO. Using ROC analysis, a ΔHCO cutoff >10% was predictive of needing ECMO (AUC: 0.92; sensitivity: 85%; and specificity: 100%). The algorithm predicted need for ECMO within the first 12h of life and at least 6h prior to the clinical decision for ECMO with 88% sensitivity and 100% specificity. CONCLUSION: This computational algorithm of cerebral NIRS predicts the need for ECMO in neonates with CDH. LEVEL OF EVIDENCE: II.

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.

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.

Fetoscopic Open Neural Tube Defect Repair: Development and Refinement of a Two-Port, Carbon Dioxide Insufflation Technique.

OBJECTIVE: To describe development of a two-port fetoscopic technique for spina bifida repair in the exteriorized, carbon dioxide-filled uterus and report early results of two cohorts of patients: the first 15 treated with an iterative technique and the latter 13 with a standardized technique. METHODS: This was a retrospective cohort study (2014-2016). All patients met Management of Myelomeningocele Study selection criteria. The intraoperative approach was iterative in the first 15 patients and was then standardized. Obstetric, maternal, fetal, and early neonatal outcomes were compared. Standard parametric and nonparametric tests were used as appropriate. RESULTS: Data for 28 patients (22 endoscopic only, four hybrid, two abandoned) are reported, but only those with a complete fetoscopic repair were analyzed (iterative technique [n=10] compared with standardized technique [n=12]). Maternal demographics and gestational age (median [range]) at fetal surgery (25.4 [22.9-25.9] compared with 24.8 [24-25.6] weeks) were similar, but delivery occurred at 35.9 (26-39) weeks of gestation with the iterative technique compared with 39 (35.9-40) weeks of gestation with the standardized technique (P<.01). Duration of surgery (267 [107-434] compared with 246 [206-333] minutes), complication rates, preterm prelabor rupture of membranes rates (4/12 [33%] compared with 1/10 [10%]), and vaginal delivery rates (5/12 [42%] compared with 6/10 [60%]) were not statistically different in the iterative and standardized techniques, respectively. In 6 of 12 (50%) compared with 1 of 10 (10%), respectively (P=.07), there was leakage of cerebrospinal fluid from the repair site at birth. Management of Myelomeningocele Study criteria for hydrocephalus-death at discharge were met in 9 of 12 (75%) and 3 of 10 (30%), respectively, and 7 of 12 (58%) compared with 2 of 10 (20%) have been treated for hydrocephalus to date. These latter differences were not statistically significant. CONCLUSION: Fetoscopic open neural tube defect repair does not appear to increase maternal-fetal complications as compared with repair by hysterotomy, allows for vaginal delivery, and may reduce long-term maternal risks. CLINICAL TRIAL REGISTRATION: ClinicalTrials.gov, https://clinicaltrials.gov, NCT02230072.

Prenatal diagnosis and outcome of fetal gastrointestinal obstruction.

INTRODUCTION: The purpose of this study was to evaluate the accuracy of prenatal diagnosis for fetuses with gastrointestinal (GI) obstruction with correlation to postnatal outcomes. METHODS: Fetuses diagnosed with GI obstruction (excluding esophageal and duodenal) were reviewed for those evaluated between 2006 and 2016. Prenatal diagnosis and imaging studies were compared to postnatal findings. Outcomes evaluated included diagnostic accuracy, rate of other anomalies, neonatal length of stay, incidence of short bowel syndrome, and discharge with TPN or gastrostomy. RESULTS: Forty-eight patients were diagnosed prenatally with obstruction. Six patients were excluded owing to incomplete records and follow-up. Twelve fetuses were diagnosed with ultrasound alone, and thirty-four with ultrasound and MRI. A diagnosis of obstruction was accurate in 88.1% (n=37/42) with a positive predictive value of 91.3%, while US with MRI had an accuracy of 84.4%. Associated anomalies were highest among fetuses with anorectal obstruction (90.1%), compared to large (50%) or small bowel obstruction (28%). Survival rate was lowest for anorectal obstruction (54.5%), compared to large or small bowel obstruction (100% for both). CONCLUSION: Fetal MRI is an accurate modality in the diagnosis of fetal GI obstruction and can complement findings characterized by ultrasound. Fetuses with anorectal obstruction have a higher rate of associated anomalies and the lowest survival. LEVEL OF EVIDENCE: IIb.

Use of renal near-infrared spectroscopy measurements in congenital diaphragmatic hernia patients on ECMO.

INTRODUCTION: This study tests the hypothesis that renal tissue oxygen saturation as measured by Near Infrared Spectroscopy (NIRS) would correlate with urine output in neonates with congenital diaphragmatic hernia (CDH) on extracorporeal membrane oxygenation (ECMO). METHODS: Between 2012 and 2015, neonates with CDH were enrolled as part of a comprehensive study that provided renal/cerebral/abdominal NIRS monitoring for the duration of ECMO support. Continuous NIRS measurements, mean arterial pressure, and urine output were recorded. Periods of anuria (NU), adequate urine output >1ml/kg/h (AU), and low urine output <1ml/kg/h (LU) were noted and analyzed. RESULTS: Over 1500h of continuous renal NIRS were obtained from six neonates. NIRS values were significantly different during periods of AU, LU, and anuria (84±6%, 76±3%, and 67±6%, p<0.01). ROC curves identified NIRS >76% as highly predictive of adequate urine output (AUC=0.96). MAP was significantly lower only in anuric patients, 36.42±10.26, compared to patients with AU and LU - 42.99±5.25 and 42.85±7.4, respectively (p<0.001). CONCLUSION: Renal NIRS measurements correlate with urine production. Lower values are noted as urine output declines and precedes a decline in MAP. Renal NIRS may have promise as a non-invasive means of determining adequacy of renal perfusion and urine output in neonates with complex fluid shifts. LEVEL OF EVIDENCE: IIb.

Feasibility and Outcomes of Fetoscopic Tracheal Occlusion for Severe Left Diaphragmatic Hernia.

OBJECTIVE: To evaluate feasibility and initial outcomes of fetoscopic tracheal occlusion for severe diaphragmatic hernia compared with a historical cohort who had not received fetal tracheal occlusion. METHODS: Outcomes in a prospective observational cohort who underwent fetoscopic tracheal occlusion for severe fetal left diaphragmatic hernia without associated anomalies were compared with our historical nontreated cohort of matched fetuses of similar severity. Fetuses were classified using the same ultrasonography and magnetic resonance imaging methodology-prospectively in the fetoscopic tracheal occlusion group and retrospectively in the historical nontreated cohort. Obstetric and postnatal outcomes were evaluated and compared. RESULTS: Between January 2004 and June 2015, 218 fetuses with diaphragmatic hernia were evaluated. Twenty (9%) fetuses had severe left diaphragmatic hernia (lung-head ratio 1.0 or less and liver herniation), of which 9 of 20 were managed without tracheal occlusion. Eleven were offered the procedure and in 10, it was successful. Mean (±standard deviation) gestational age was 27.9±1.1 weeks at attempted balloon placement, 34.1±1.1 weeks at removal, and 35.3±2.2 weeks at delivery. One patient required an ex utero intrapartum treatment procedure at delivery to remove the balloon. There were no maternal complications or fetal deaths. All neonates underwent postnatal repair with a patch. The 6-month, 1-year, and 2-year survival rates were significantly higher in our treated cohort than in our nontreated historical cohort (80% compared with 11%, risk difference 69%, 95% confidence interval [CI] 38-100%, P=.01; 70% compared with 11%, risk difference 59%, 95% CI 24-94%, P=.02; and 67% compared with 11%, risk difference 56%, 95% CI 19-93%, P=.04, respectively) with reduced need for extracorporeal membrane oxygenation (30% compared with 70%, risk difference 40%, 95% CI 10-79%, P=.05). CONCLUSION: Fetoscopic tracheal occlusion is feasible and is associated with improved postnatal outcomes in severe left diaphragmatic hernia. CLINICAL TRIAL REGISTRATION: ClinicalTrials.gov, https://clinicaltrials.gov, NCT00881660.

Urologic Considerations in the Separation of Thoracoomphalopagus Conjoined Twins.

Conjoined twins are seen in approximately 1/500,000 live births, and therefore surgical management of urologic anomalies in conjoined twins has not been extensively reported. Various degrees of sharing of the urinary tracts and genitalia can be seen in different types of conjoined twins. Detailed preoperative imaging, including magnetic resonance imaging, computed tomography, and voiding cystourethrogram, is essential to define the anatomy and planning of a successful separation. We describe the urologic presentation, evaluation, and treatment of thoracoomphalopagus conjoined twins.

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.