Postnatal outcomes and risk factor analysis for patients with prenatally diagnosed oropharyngeal masses.

OBJECTIVES: To describe our experience treating prenatally diagnosed oropharyngeal masses in a novel, multidisciplinary collaboration. To identifying outcomes and risk factors associated with adverse postnatal outcomes. METHODS: This is a sixty-two patient case series at an academic referral center. Patients with prenatally diagnosed oropharyngeal masses were identified through a programmatic database and confirmed in the electronic health record. RESULTS: Sixty-two patient with prenatally diagnosed oropharyngeal mass were identified, with prenatal imaging at our institution confirming this diagnosis in fifty-seven patients, short term outcomes analysis conducted on forty-four patients, and long-term outcomes analysis conducted on seventeen patients. The most common pathology was lymphatic malformations (n = 27, 47.4%), followed by teratomas (n = 22, 38.6%). The median mass volume from all available patient imaging (n = 57) was 60.54 cm3 (range 1.73-742.5 cm3). Thirteen pregnancies were interrupted, six infants expired, and thirteen cases had an unknown fetal outcome. Confirmed mortality was 6/57 patients with imaging-confirmed oropharyngeal masses (10.5%). Fourteen (56%) of the surviving patients (n = 25) were delivered by Ex Utero Intrapartum Treatment (EXIT) procedure and the median NICU stay was thirty-six days (range: 3-215 days). There was no association between airway compression/deviation/displacement, stomach size, polyhydramnios, or mass size and mortality. Seventeen patients had more than one year of follow-up (mean 5.3 ± 2.4 years). These seventeen patients underwent general anesthesia a total of ninety-two times (mean 5.4 ± 4.3) and had a total of twenty-three mass-related surgeries. The great majority of patients required an artificial airway at birth, feeding support, and speech/swallow therapy. CONCLUSIONS: Oropharyngeal mass involvement of key anatomic structures-the neck, upper thorax, orbit, and ear, has a greater association with mortality than mass size. Regardless of the size and involved structures, oropharyngeal masses are associated with a high burden of intensive medical care and surgical care beginning at or before birth.

Risk factor analysis and outcomes of airway management in antenatally diagnosed cervical masses.

PURPOSE: To investigate antenatally-determined imaging characteristics associated with invasive airway management at birth in patients with cervical masses, as well as to describe postnatal management and outcomes. STUDY DESIGN: A retrospective analysis of 52 patients with antenatally diagnosed neck masses was performed using single-center data from January 2008 to January 2019. Antenatal imaging, method of delivery, management, and outcomes data were abstracted from the medical record and analyzed. RESULTS: Antenatal diagnosis of neck masses in this cohort consisted of 41 lymphatic malformations (78.8%), 6 teratomas (11.5%), 3 hemangiomas (5.8%), 1 hemangioendothelioma (1.9%), and 1 giant foregut duplication cyst (1.9%). Mean gestational age at time of diagnostic imaging was 29 weeks 3 days (range: 19w4d - 37w). Overall, 22 patients (42.3%) required invasive airway management at birth, specifically 18 patients (34.6%) required endotracheal intubation and 4 (7.7%) required tracheostomy. 15 patients (28.8%) underwent ex-utero intrapartum treatment (EXIT) for the purposes of securing an airway. Polyhydramnios, tracheal deviation and compression, and anterior mass location on antenatal imaging were significantly associated with incidence of invasive airway intervention at birth, EXIT procedure, and tracheostomy during the neonatal hospitalization (p < 0.025; Fisher's exact test). Logistic regression analysis demonstrated statistically significant association between increasing antenatally-estimated mass volume and incidence of invasive airway management at birth (p = 0.02). Post-natal cervical mass management involved surgical excision (32.7%), sclerotherapy (50%), and adjuvant therapy with rapamycin (17.3%). Demise in the neonatal period occurred in 4 (7.7%) patients. CONCLUSION: This series documents the largest single-center experience of airway management in antenatally diagnosed cervical masses. Fetal imaging characteristics may help inform the appropriate method of delivery, airway management strategy at birth, and prenatal counseling.

Maternal Depressive Risk in Prenatal versus Postnatal Surgical Closure of Myelomeningocele: Associations with Parenting Stress and Child Outcomes.

INTRODUCTION: Depressive risk is higher for mothers of infants with chronic medical conditions. The present study examined maternal depressive risk and associations with parent and child outcomes among mothers of young children who were randomized to either prenatal or postnatal surgical closure for myelomeningocele. METHODS: Using the Management of Myelomeningocele Study database, maternal depressive risk was examined at 3 time points as follows: prior to birth, 12 months, and 30 months post birth. Separate multivariate analyses examined associations among change in depressive risk (between baseline and 30 months), parenting stress, and child outcomes at 30 months. RESULTS: Mean scores were in the minimal depressive risk range at all the time points. Post birth depressive risk did not differ by prenatal versus postnatal surgery. Mean change scores reflected a decrease in depressive risk during the first 30 months. Only 1.1-4.5% of mothers reported depressive risk in the moderate to severe range across time points. Increased depressive risk during the first 30 months was associated with increased parenting stress scores and slightly lower child cognitive scores at 30 months. CONCLUSION: Most mothers reported minimal depressive risk that decreased over time, regardless of whether their infant underwent prenatal or postnatal surgery. Only a small percentage of mothers endorsed moderate to severe depressive risk, but an increase in depressive risk over time was associated with higher parental stress and slightly lower child cognitive development.

The Management of Myelomeningocele Study: Short-Term Neonatal Outcomes.

INTRODUCTION: The Management of Myelomeningocele Study was a multicenter randomized trial to compare prenatal and standard postnatal repair of myelomeningocele (MMC). Neonatal outcome data for 158 of the 183 randomized women were published in The New England Journal of Medicine in 2011. OBJECTIVE: Neonatal outcomes for the complete trial cohort (N = 183) are presented outlining the similarities with the original report and describing the impact of gestational age as a mediator. METHODS: Gestational age, neonatal characteristics at delivery, and outcomes including common complications of prematurity were assessed. RESULTS: Analysis of the complete cohort confirmed the initial findings that prenatal surgery was associated with an increased risk for earlier gestational age at birth. Delivery occurred before 30 weeks of gestation in 11% of neonates that had fetal MMC repair. Adverse pulmonary sequelae were rare in the prenatal surgery group despite an increased rate of oligohydramnios. There was no significant difference in other complications of prematurity including patent ductus arteriosus, sepsis, necrotizing enterocolitis, periventricular leukomalacia, and intraventricular hemorrhage. CONCLUSION: The benefits of prenatal surgery outweigh the complications of prematurity.

Two's Company: Multiple Thoracic Lesions on Prenatal US and MRI.

BACKGROUND: Congenital pulmonary airway malformations (CPAM), bronchopulmonary sequestrations (BPS), and CPAM-BPS hybrid lesions are most commonly solitary; however, >1 lung congenital lung lesion may occur. OBJECTIVES: To assess the frequency of multiple congenital thoracic anomalies at a high-volume referral center; determine prenatal ultrasound (US) and magnetic resonance imaging (MRI) features of these multifocal congenital lung lesions that may allow prenatal detection; and determine the most common distribution or site of origin. METHODS: Database searches were performed from August 2008 to May 2019 for prenatally evaluated cases that had a final postnatal surgical diagnosis of >1 congenital lung lesion or a lung lesion associated with foregut duplication cyst (FDC). Lesion location, size, echotexture, and signal characteristics were assessed on prenatal imaging and correlated with postnatal computed tomographic angiography and surgical pathology. -Results: Of 539 neonates that underwent surgery for a thoracic lesion, 35 (6.5%) had >1 thoracic abnormality. Multiple discrete lung lesions were present in 19 cases, and a lung lesion associated with an FDC was present in 16. Multifocal lung lesions were bilateral in 3 cases; unilateral, multilobar in 12; and, unilobar multisegmental in 4. Median total CPAM volume/head circumference ratio for multifocal lung lesions on US was 0.66 (range, 0.16-1.80). Prenatal recognition of multifocal lung lesions occurred in 7/19 cases (36.8%). Lesion combinations were CPAM-CPAM in 10 cases, CPAM-BPS in 5, CPAM-hybrid in 2, hybrid-hybrid in 1, and hybrid-BPS in 1. Of 5 unilateral, multifocal lung lesions, multifocality was prenatally established through identification of a band of normal intervening lung or intrinsic differences in lesion imaging features. CONCLUSIONS: Although less common, multiple thoracic abnormalities can be detected prenatally. Of multifocal lung lesions, the most common combination was CPAM-CPAM, with a unilateral, multilobar distribution. Prenatal recognition is important for pregnancy counseling and postnatal surgical management.

A Model of Group Prenatal Care for Patients with Prenatally Diagnosed Fetal Anomalies.

The model of group prenatal care was initially developed to include peer support and to improve education and health-promoting behaviors during pregnancy. This model has since been adapted for populations with unique educational needs. Mama Care is an adaptation of the CenteringPregnancy Model of prenatal care. Mama Care is situated within a national and international referral center for families with prenatally diagnosed fetal anomalies. In December 2013, the Center for Fetal Diagnosis and Treatment at Children's Hospital of Philadelphia began offering a model of group prenatal care to women whose pregnancies are affected by a prenatal diagnosis of a fetal anomaly. The model incorporates significant adaptations of CenteringPregnancy in order to accommodate these women, who typically transition their care from community-based settings to the Center for Fetal Diagnosis and Treatment in the late second or early third trimester. Unique challenges associated with caring for families within a referral center include a condensed visit schedule, complex social needs such as housing and psychosocial support, as well as an increased need for antenatal surveillance and frequent preterm birth. Outcomes of the program are favorable and suggest group prenatal care models can be developed to support the needs of patients with prenatally diagnosed fetal anomalies.

Prenatal Repair of Myelomeningocele and School-age Functional Outcomes.

BACKGROUND AND OBJECTIVES: The Management of Myelomeningocele Study (MOMS), a randomized trial of prenatal versus postnatal repair for myelomeningocele, found that prenatal surgery resulted in reduced hindbrain herniation and need for shunt diversion at 12 months of age and better motor function at 30 months. In this study, we compared adaptive behavior and other outcomes at school age (5.9-10.3 years) between prenatal versus postnatal surgery groups. METHODS: Follow-up cohort study of 161 children enrolled in MOMS. Assessments included neuropsychological and physical evaluations. Children were evaluated at a MOMS center or at a home visit by trained blinded examiners. RESULTS: The Vineland composite score was not different between surgery groups (89.0 ± 9.6 in the prenatal group versus 87.5 ± 12.0 in the postnatal group; P = .35). Children in the prenatal group walked without orthotics or assistive devices more often (29% vs 11%; P = .06), had higher mean percentage scores on the Functional Rehabilitation Evaluation of Sensori-Neurologic Outcomes (92 ± 9 vs 85 ± 18; P < .001), lower rates of hindbrain herniation (60% vs 87%; P < .001), had fewer shunts placed for hydrocephalus (49% vs 85%; P < .001) and, among those with shunts, fewer shunt revisions (47% vs 70%; P = .02) than those in the postnatal group. Parents of children repaired prenatally reported higher mean quality of life z scores (0.15 ± 0.67 vs 0.11 ± 0.73; P = .008) and lower mean family impact scores (32.5 ± 7.8 vs 37.0 ± 8.9; P = .002). CONCLUSIONS: There was no significant difference between surgery groups in overall adaptive behavior. Long-term benefits of prenatal surgery included improved mobility and independent functioning and fewer surgeries for shunt placement and revision, with no strong evidence of improved cognitive functioning.

The experience of parents with children with myelomeningocele who underwent prenatal surgery.

BACKGROUND: Prenatal surgery for myelomeningocele (MMC) has been demonstrated to have benefits over postnatal surgery. Nevertheless, prenatal surgery requires a significant emotional, physical, and financial commitment from the entire family. METHODS: Mixed methods study of parents' perceptions regarding provider communication, treatment choices, and the family impact of having a child with MMC. RESULTS: Parents of children with MMC (n= 109) completed questionnaires. Parents were well informed and reported gathering information about prenatal surgery from a wide range of sources. After a fetal diagnosis of MMC, most learned about their options from their obstetrician, although one-third were not told about the option of prenatal surgery. About one-fourth of these parents felt pressure to undergo one particular option. Half of parents said that having a child with MMC has had a positive impact on them and their family, while the other half indicated that having a child with MMC has had both positive and negative impacts. The most commonly noted positive impacts were changes in parental attitudes, as well as having new opportunities and relationships. The most frequently reported negative impacts concerned relational and financial strain. The vast majority of parents indicated that they would still undergo prenatal surgery if they could travel back in time with their present knowledge. CONCLUSIONS: A better understanding of the parental experiences and perspectives following prenatal surgery will play an important role in providing overall support for parents and family members.

The Management of Myelomeningocele Study: full cohort 30-month pediatric outcomes.

BACKGROUND: Previous reports from the Management of Myelomeningocele Study demonstrated that prenatal repair of myelomeningocele reduces hindbrain herniation and the need for cerebrospinal fluid shunting, and improves motor function in children with myelomeningocele. The trial was stopped for efficacy after 183 patients were randomized, but 30-month outcomes were only available at the time of initial publication in 134 mother-child dyads. Data from the complete cohort for the 30-month outcomes are presented here. Maternal and 12-month neurodevelopmental outcomes for the full cohort were reported previously. OBJECTIVE: The purpose of this study is to report the 30-month outcomes for the full cohort of patients randomized to either prenatal or postnatal repair of myelomeningocele in the original Management of Myelomeningocele Study. STUDY DESIGN: Eligible women were randomly assigned to undergo standard postnatal repair or prenatal repair <26 weeks gestation. We evaluated a composite of mental development and motor function outcome at 30 months for all enrolled patients as well as independent ambulation and the Bayley Scales of Infant Development, Second Edition. We assessed whether there was a differential effect of prenatal surgery in subgroups defined by: fetal leg movements, ventricle size, presence of hindbrain herniation, gender, and location of the myelomeningocele lesion. Within the prenatal surgery group only, we evaluated these and other baseline parameters as predictors of 30-month motor and cognitive outcomes. We evaluated whether presence or absence of a shunt at 1 year was associated with 30-month motor outcomes. RESULTS: The data for the full cohort of 183 patients corroborate the original findings of Management of Myelomeningocele Study, confirming that prenatal repair improves the primary outcome composite score of mental development and motor function (199.4 ± 80.5 vs 166.7 ± 76.7, P = .004). Prenatal surgery also resulted in improvement in the secondary outcomes of independent ambulation (44.8% vs 23.9%, P = .004), WeeFIM self-care score (20.8 vs 19.0, P = .006), functional level at least 2 better than anatomic level (26.4% vs 11.4%, P = .02), and mean Bayley Scales of Infant Development, Second Edition, psychomotor development index (17.3% vs 15.1%, P = .03), but does not affect cognitive development at 30 months. On subgroup analysis, there was a nominally significant interaction between gender and surgery, with boys demonstrating better improvement in functional level and psychomotor development index. For patients receiving prenatal surgery, the presence of in utero ankle, knee, and hip movement, absence of a sac over the lesion and a myelomeningocele lesion of ≤L3 were significantly associated with independent ambulation. Postnatal motor function showed no correlation with either prenatal ventricular size or postnatal shunt placement. CONCLUSION: The full cohort data of 30-month cognitive development and motor function outcomes validate in utero surgical repair as an effective treatment for fetuses with myelomeningocele. Current data suggest that outcomes related to the need for shunting should be counseled separately from the outcomes related to distal neurologic functioning.

Fetal Lymphatic Malformations: More Variable Than We Think?

Lymphatic malformations are benign lesions that result from abnormal development of the lymphatic and venous systems. These lesions may be detected during routine prenatal ultrasound screening, and typically demonstrate imaging findings of a multiseptate cystic lesion lacking solid components, vascularity, and calcifications. We report 73 cases of prenatally detected lymphatic malformations and describe greater variability in their prenatal sonographic appearance than previously reported, including purely cystic lesions and mixed cystic and solid lesions with calcifications. Appreciation of this increased variability is important in providing accurate prenatal diagnosis, counseling, and management.

The Management of Myelomeningocele Study: obstetrical outcomes and risk factors for obstetrical complications following prenatal surgery.

BACKGROUND: The Management of Myelomeningocele Study was a multicenter randomized trial to compare prenatal and standard postnatal closure of myelomeningocele. The trial was stopped early at recommendation of the data and safety monitoring committee and outcome data for 158 of the 183 randomized women published. OBJECTIVE: In this report, pregnancy outcomes for the complete trial cohort are presented. We also sought to analyze risk factors for adverse pregnancy outcome among those women who underwent prenatal myelomeningocele repair. STUDY DESIGN: Pregnancy outcomes were compared between the 2 surgery groups. For women who underwent prenatal surgery, antecedent demographic, surgical, and pregnancy complication risk factors were evaluated for the following outcomes: premature spontaneous membrane rupture ≤34 weeks 0 days (preterm premature rupture of membranes), spontaneous membrane rupture at any gestational age, preterm delivery at ≤34 weeks 0 days, nonintact hysterotomy (minimal uterine wall tissue between fetal membranes and uterine serosa, or partial or complete dehiscence at delivery), and chorioamniotic membrane separation. Risk factors were evaluated using χ2 and Wilcoxon tests and multivariable logistic regression. RESULTS: A total of 183 women were randomized: 91 to prenatal and 92 to postnatal surgery groups. Analysis of the complete cohort confirmed initial findings: that prenatal surgery was associated with an increased risk for membrane separation, oligohydramnios, spontaneous membrane rupture, spontaneous onset of labor, and earlier gestational age at birth. In multivariable logistic regression of the prenatal surgery group adjusting for clinical center, earlier gestational age at surgery and chorioamniotic membrane separation were associated with increased risk of spontaneous membrane rupture (odds ratio, 1.49; 95% confidence interval, 1.01-2.22; and odds ratio, 2.96, 95% confidence interval, 1.05-8.35, respectively). Oligohydramnios was associated with an increased risk of subsequent preterm delivery (odds ratio, 9.21; 95% confidence interval, 2.19-38.78). Nulliparity was a risk factor for nonintact hysterotomy (odds ratio, 3.68; 95% confidence interval, 1.35-10.05). CONCLUSION: Despite the confirmed benefits of prenatal surgery, considerable maternal and fetal risk exists compared with postnatal repair. Early gestational age at surgery and development of chorioamniotic membrane separation are risk factors for ruptured membranes. Oligohydramnios is a risk factor for preterm delivery and nulliparity is a risk factor for nonintact hysterotomy at delivery.

Impact on family and parental stress of prenatal vs postnatal repair of myelomeningocele.

BACKGROUND: The Management of Myelomeningocele Study was a multicenter, randomized controlled trial that compared prenatal repair with standard postnatal repair for fetal myelomeningocele. OBJECTIVE: We sought to describe the long-term impact on the families of the women who participated and to evaluate how the timing of repair influenced the impact on families and parental stress. STUDY DESIGN: Randomized women completed the 24-item Impact on Family Scale and the 36-item Parenting Stress Index Short Form at 12 and 30 months after delivery. A revised 15-item Impact on Family Scale describing overall impact was also computed. Higher scores reflected more negative impacts or greater stress. In addition, we examined Family Support Scale and Family Resource Scale scores along with various neonatal outcomes. Repeated measures analysis was conducted for each scale and subscale. RESULTS: Of 183 women randomized, 171 women completed the Impact on Family Scale and 172 completed the Parenting Stress Index at both 12 and 30 months. The prenatal surgery group had significantly lower revised 15-item Impact on Family Scale scores as well as familial-social impact subscale scores compared to the postnatal surgery group (P = .02 and .004, respectively). There was no difference in total parental stress between the 2 groups (P = .89) or in any of the Parenting Stress Index Short Form subscales. In addition, walking independently at 30 months and family resources at 12 months were associated with both family impact and parental stress. CONCLUSION: The overall negative family impact of caring for a child with spina bifida, up to 30 months of age, was significantly lower in the prenatal surgery group compared to the postnatal surgery group. Ambulation status and family resources were predictive of impact on family and parental stress.

Identifying expectant parents at risk for psychological distress in response to a confirmed fetal abnormality.

The aim of the study was to determine the incidence of psychological distress among expectant women carrying fetuses with prenatal diagnosed abnormalities and their partners. A 2-year retrospective medical chart review was completed of 1032 expectant mothers carrying fetuses with a confirmed anomaly, and 788 expectant fathers, who completed the CFDT Mental Health Screening Tool. Furthermore, 19.3 % of women and 13.1 % of men reported significant post-traumatic stress symptoms, and 14 % of men and 23 % of women scored positive for a major depressive disorder. Higher risk was noted among expectant parents of younger age and minority racial/ethnic status, and women with post-college level education and current or prior use of antidepressant medications. Heightened distress was noted within fetal diagnostic subgroups including neck masses, sacrococcygeal teratomas, neurological defects, and miscellaneous diagnoses. Incorporating screening tools into prenatal practice can help clinicians better identify the potential risk for psychological distress among expectant parents within high-risk fetal settings.

Bladder Function After Fetal Surgery for Myelomeningocele.

BACKGROUND: A substudy of the Management of Myelomeningocele Study evaluating urological outcomes was conducted. METHODS: Pregnant women diagnosed with fetal myelomeningocele were randomly assigned to either prenatal or standard postnatal surgical repair. The substudy included patients randomly assigned after April 18, 2005. The primary outcome was defined in their children as death or the need for clean intermittent catheterization (CIC) by 30 months of age characterized by prespecified criteria. Secondary outcomes included bladder and kidney abnormalities observed by urodynamics and renal/bladder ultrasound at 12 and 30 months, which were analyzed as repeated measures. RESULTS: Of the 115 women enrolled in the substudy, the primary outcome occurred in 52% of children in the prenatal surgery group and 66% in the postnatal surgery group (relative risk [RR]: 0.78; 95% confidence interval [CI]: 0.57-1.07). Actual rates of CIC use were 38% and 51% in the prenatal and postnatal surgery groups, respectively (RR: 0.74; 95% CI: 0.48-1.12). Prenatal surgery resulted in less trabeculation (RR: 0.39; 95% CI: 0.19-0.79) and fewer cases of open bladder neck on urodynamics (RR: 0.61; 95% CI: 0.40-0.92) after adjustment by child's gender and lesion level. The difference in trabeculation was confirmed by ultrasound. CONCLUSIONS: Prenatal surgery did not significantly reduce the need for CIC by 30 months of age but was associated with less bladder trabeculation and open bladder neck. The implications of these findings are unclear now, but support the need for long-term urologic follow-up of patients with myelomeningocele regardless of type of surgical repair.

Thoracoamniotic shunts for the management of fetal lung lesions and pleural effusions: a single-institution review and predictors of survival in 75 cases.

PURPOSE: Hydrops and pulmonary hypoplasia are associated with significant morbidity and mortality in the setting of a congenital lung lesion or pleural effusion (PE). We reviewed our experience using in utero thoracoamniotic shunts (TA) to manage fetuses with these diagnoses. METHODS: A retrospective review of fetuses diagnosed with a congenital lung lesion or pleural effusion who underwent TA shunt placement from 1998-2013 was performed. RESULTS: Ninety-seven shunts were placed in 75 fetuses. Average gestational age (±SD) at shunt placement and birth was 25±3 and 34±5 weeks. Shunt placement resulted in a 55±21% decrease in macrocystic lung lesion volume and complete or partial drainage of the PE in 29% and 71% of fetuses. 69% of fetuses presented with hydrops, which resolved following shunt placement in 83%. Survival was 68%, which correlated with GA at birth, % reduction in lesion size, unilateral pleural effusions, and hydrops resolution. Surviving infants had prolonged NICU courses and often required either surgical resection or tube thoracostomy in the perinatal period. CONCLUSION: TA shunts provide a therapeutic option for select fetuses with large macrocystic lung lesions or PEs at risk for hydrops and/or pulmonary hypoplasia. Survival following shunting depends on GA at birth, reduction in mass size, and hydrops resolution.

Fetal myelomeningocele repair: the post-MOMS experience at the Children's Hospital of Philadelphia.

BACKGROUND: Fetal myelomeningocele (fMMC) repair has become accepted as a standard of care option in selected circumstances. We reviewed our outcomes for fMMC repair from referral and evaluation through surgery, delivery and neonatal discharge. MATERIAL AND METHODS: All patients referred for potential fMMC repair were reviewed from January 1, 2011 through March 7, 2014. Maternal and neonatal data were collected on the 100 patients who underwent surgery. RESULTS: 29% of those evaluated met the criteria and underwent fMMC repair (100 cases). The average gestational age was 21.9 weeks at evaluation and 23.4 weeks at fMMC repair. Complications included membrane separation (22.9%), preterm premature rupture of membranes (32.3%) and preterm labor (37.5%). Average gestational age at delivery was 34.3 weeks and 54.2% delivered at ≥35 weeks. The perinatal loss rate was 6.1% (2 intrauterine fetal demises and 4 neonatal demises); 90.8% of women delivered at the Children's Hospital of Philadelphia and 3.4% received transfusions. With regard to the neonates, 2 received ventriculoperitoneal shunts prior to discharge; 71.1% of neonates had no evidence of hindbrain herniation on MRI. Of the 80 neonates evaluated, 55% were assigned a functional level of one or more better than the prenatal anatomic level. CONCLUSION: In an experienced program, maternal and neonatal outcomes for patients undergoing fMMC repair are comparable to results of the MOMS trial.

Open fetal surgery for central bronchial atresia.

Mainstem or lobar bronchial atresia is associated with massive pulmonary hyperplasia, contralateral pulmonary hypoplasia, non-immune hydrops and a fatal fetal prognosis. Open fetal surgery currently provides a potential therapeutic option for management of a fetus with this diagnosis. We present 2 cases of open fetal surgery for bronchial atresia at the level of the mainstem and lobar bronchus. The first case involved a left pneumonectomy performed at 26 weeks' gestation. After successful intraoperative resuscitation for fetal cardiac arrest, the fetus survived until 32 weeks' gestation when preterm premature rupture of membranes prompted delivery. The neonate expired on day of life (DOL)1. The second case involved a left upper lobectomy performed at 24(3)/7 weeks' gestation. The fetus was delivered at 34(3)/7 weeks' gestation and, after a prolonged NICU course including a completion pneumonectomy and tracheostomy, was discharged home at 8 months of life. This second case is the first report of survival following open fetal surgery for central bronchial atresia. This report highlights the potential benefit of fetal lung resection for central bronchial atresia while describing the many challenges to achieve the successful management of a fetus with this condition.

Perinatal management of congenital oropharyngeal tumors: the ex utero intrapartum treatment (EXIT) approach.

PURPOSE: To present our experience in the perinatal management of fetuses with large oropharyngeal tumors by ex utero intrapartum treatment (EXIT). METHODS: We performed a retrospective chart review of all patients with congenital oropharyngeal tumor who underwent an EXIT procedure between May 2006 and June 2012. RESULTS: Four patients were included in the series, three females and one male. The diagnoses were epignathus (n=2) and congenital epulis (n=2). Three EXITs were done at term and one at late preterm due to premature rupture of membranes. Median maternal time under anesthesia was 185 min (range: 166-281) and median maternal operative time was 99 min (range: 85-153). Median maternal blood loss was 550 ml (range: 350-2000); one mother required a blood transfusion. Mean maternal hospital stay was 4 days. Median hysterotomy-to-cord clamp time was 24 min (range: 18-66). Mean fetal birth weight was 2.7 kg (range: 2.4-3). The airway was successfully accessed and secured under placental circulation in all cases. In the two patients with congenital epulis the tumors were resected at the base of their pedicles and the airway accessed via direct laryngoscopy before the umbilical cord was clamped. One patient with epignathus underwent a retrograde tracheal intubation under placental circulation and had the tumor resected thereafter. The second patient with epignathus had a tracheostomy done under placental circulation and then had tumor debulking immediately after the EXIT. The maternal morbidity was minimal and there were no mortalities. CONCLUSIONS: We conclude that the EXIT procedure is the ideal delivery strategy for fetuses with prenatally diagnosed oropharyngeal tumors and potential airway obstruction at birth. Patients with prenatally diagnosed oropharyngeal tumors should be promptly referred to a fetal treatment center with a dedicated multidisciplinary team and EXIT capabilities.

Sonographic features of rare posterior fetal neck masses of vascular origin.

The purpose of this series is to describe the grayscale and color Doppler sonographic characteristics as well as the histopathologic features of rare solid posterior neck masses identified on prenatal sonography in pregnant patients. We conducted a retrospective review of detailed fetal sonographic examinations of second- and third-trimester pregnancies referred to the Center for Fetal Diagnosis and Treatment at the Children's Hospital of Philadelphia for suspected fetal neck masses from June 1998 to December 2011. Eight predominately solid posterior neck masses were identified on 139 studies performed during the study period. Of the 7 cases in which follow-up was available, 6 were confirmed as hemangiomas, and 1 was confirmed as a kaposiform hemangioendothelioma with Kasabach-Merritt syndrome. The most common sonographic features were hypervascularity (7) and calcifications (5). Posterior solid fetal neck masses are rare anomalies. Hemangioma is the most common etiology and should be suggested as the likely diagnosis rather than teratoma, even in the presence of calcifications.

The Garbose Family Special Delivery Unit: a new paradigm for maternal-fetal and neonatal care.

Birth defects remain the leading cause of infant mortality in the United States according to the Centers for Disease Control. For many anomalies, etiology remains unknown, management and outcome vary widely, and treatment is costly. Great strides have been made in prenatal diagnosis, yet standardized prenatal care and delivery protocols are needed for women carrying fetuses with specific birth defects such as diaphragmatic hernia, lung lesions, abdominal wall defects, congenital heart disease and so forth. In an effort to standardize and improve the overall care for mother and baby with a known birth defect, the Garbose Family Special Delivery Unit was opened at the Children's Hospital of Philadelphia in June 2008. This paper describes an innovative healthcare delivery model focusing on the rationale for developing a specialized delivery unit, the facility design and development process, care model, team and services, and the types of fetal diagnoses and maternal admission criteria for the SDU.