Monochorionic Twins: TTTS, TAPS, and Selective Fetal Growth Restriction.

With an increasing incidence of twin gestations, understanding the inherent risks associated with these pregnancies is essential in modern obstetrics. The unique differences in placentation in monochorionic twins leads to unique complications, including twin-to-twin transfusion syndrome, the twin anemia-polycythemia sequence, and selective fetal growth restriction. Not only does the understanding of the monochorionic placenta lead to an understanding of the pathophysiology of the complications of monochorionic twins, but it also has led to the development of highly effective directed fetal therapy via fetoscopic laser coagulation used in twin-to-twin transfusion syndrome.

The Special Delivery Unit: A Novel Concept for Mother-Baby Continuity for Major Fetal Abnormalities.

OBJECTIVE: This article describes the experience in the planning and development of a special delivery unit (SDU) at our free-standing children's hospital in Austin, Texas. STUDY DESIGN: Description of various aspects of the development of the SDU. In addition, telephone surveys were obtained from five other institutions regarding the planning and current status of their SDUs. RESULTS: Since the advent of the SDU at Children's Hospital of Philadelphia in 2008, several free-standing children's hospitals have opened similar units at their institutions. Developing an obstetrical unit in a children's hospital can be a daunting task on many fronts. The costs of providing 24-hour obstetrical, nursing, and anesthesiology coverage must be considered. Although most SDUs are associated with a fetal center and fetal surgery/interventions, some units function exclusively for the delivery of pregnancies complicated by major fetal conditions where the neonate will require immediate surgical care or other interventions. CONCLUSION: Research on the cost-effectiveness and the effect of SDUs on clinical outcome, teaching, and patient satisfaction is warranted. KEY POINTS: · Specialized delivery units are becoming more common at free-standing children's hospitals.. · The primary aim of the SDU is to maintain mother-baby continuity in cases of congenital anomalies.. · Developing an obstetrical unit at a pediatric hospital is a daunting task..

Recipient umbilical artery elongation (redundancy) in twin-twin transfusion syndrome.

BACKGROUND: Chronic hypertension in adults causes arterial lengthening in major arteries, but the effects of early fetal hypertension on the twin-twin transfusion syndrome recipient's vascular architecture remains unknown. OBJECTIVE: We hypothesize that arterial cord redundancy is related to recipient hypertension and subsequent heart failure. Our objectives were to: (1) establish a 3-dimensional color Doppler ultrasound method of measuring umbilical arterial length relative to its corresponding venous segment in the umbilical cord using artery vein angle; (2) compare recipient artery vein angle to gestational age-matched controls; and (3) test the association of artery vein angle with recipient heart failure. STUDY DESIGN: We compared 3 groups prospectively: twin-twin transfusion syndrome pregnancies undergoing fetoscopic laser surgery (preoperatively) and 2 groups of gestational age-matched controls: uncomplicated monochorionic-diamniotic twin pregnancies and healthy singletons. Using a 3-dimensional color-Doppler volume image of 5 cm of cord near the placental insertion, we traced the umbilical artery and vein producing umbilical artery:vein length, (artery vein index) and measured the artery vein angle between umbilical artery and vein. Correlation of artery vein angle to twin-twin transfusion syndrome stage, maximum vertical pocket, umbilical arterial indices, ductus venosus Doppler, and brain natriuretic peptide were performed. We used pulsed-wave and tissue Doppler to measure tissue Doppler velocities and indexed cardiac output and correlated these with artery vein angle. Comparative statistics, including multivariable linear regression, examined the relationship between umbilical arterial Doppler indices and artery vein angle. RESULTS: Artery vein angle and artery vein index correlated significantly (R2, 0.86; P < .0001), hence, artery vein angle was used for analysis. Mean artery vein angle was 33.1 ± 31.5 degrees in recipients (n = 44), 9.5 ± 6 degrees in monochorionic-diamniotic (n = 11; 22 fetuses), and 8.9 ± 8.3 degrees in singleton controls (n = 16) (P < .001). An artery vein angle ≥26 degrees (>95th percentile for controls) was measured in 52% recipients. Artery vein angle was higher in twin-twin transfusion syndrome stage 3R vs 1 (P = .001). Artery vein angle increased with increasing umbilical arterial pulsatility index (P < .001), and decreased with increasing resistance index (P = .02) after adjusting for gestational age. Interrater agreements to categorize abnormal artery vein angle values was 95% (P < .001). Abnormal ductus venosus Doppler and elevated recipient amniotic fluid N-terminal pro-brain natriuretic peptide/protein levels correlated significantly with artery vein angle. Abnormal artery vein angles were associated with decreased indexed cardiac output, lower tissue Doppler velocities, higher right-sided Tei indices, and severe tricuspid regurgitation. CONCLUSION: Umbilical arterial lengthening occurs in 52% of recipients and is associated with abnormal Doppler flows, low systolic tissue Doppler velocities, reduced cardiac output, and elevated markers of cardiac failure. This may reflect chronicity and severity of hypertension in the recipient fetus. Further research is needed to explore the mechanisms of elongation and long-term implications.

Correlating Prenatal Imaging Findings of Fetal Ventriculomegaly with the Need for Surgical Intervention in the First 3 Months after Birth.

BACKGROUND/AIMS: This study evaluates the predictive value of prenatal imaging measurements regarding the need for cerebrospinal fluid (CSF) diversion for fetal hydrocephalus in the first 3 months after birth. METHODS: We retrospectively reviewed a consecutive case series of patients with fetal hydrocephalus from January 2011 to December 2014 (n = 45). Prenatal measurements included head circumference (HC), biparietal diameter (BPD), and lateral ventricle (LV) width. Patients requiring CSF diversion within 12 weeks of birth were compared to those who did not require CSF diversion using the Wilcoxon rank sum test, and receiver-operating characteristic analysis was used to evaluate threshold values. RESULTS: CSF diversion was required within 12 weeks of birth in 30 of 45 patients. Mean LV width (mm) during the entire pregnancy was greater for the surgery group than the nonsurgery group. Neither BPD nor HC showed differences between the groups. A mean LV size ≥15 mm predicted the need for CSF diversion with a sensitivity of 67% and specificity of 73%. CONCLUSION: LV width is the prenatal imaging measurement that best predicts the need for postnatal CSF diversion.

Survey of Parent Experiences in Prenatal Visits for Infants With Cleft Lip and Palate.

OBJECTIVE: The purpose of this study was to identify the factors that influence the parent's choice of cleft team/surgeon. DESIGN: A 10-question survey was used to elucidate factors that influenced parents in choosing their cleft surgeon. No identifiers of the origin of the study were used to improve parent objectivity. SETTING: The setting for this study was an online survey. PARTICIPANTS: Participants in this study were the parents of children who were born with cleft lip and/or palate. INTERVENTIONS: Parents were contacted anonymously via national, established social media websites that were independently run by the parents themselves. MAIN OUTCOME MEASURES: The main outcome measures were information regarding choice of cleft team/surgeon, source of patient referrals, and use of online media in decision making. RESULTS: A total of 112 responses were received. Of the parents, 77% sought prenatal evaluation with at least one cleft surgeon. Maternal-fetal medicine specialists were the most frequent (42%) referral source, followed by primary obstetricians (14%) and pediatricians (12%). The surgeon/cleft team's experience level and overall personality were ranked as the most important, whereas the least important was distance traveled. Of the parents, 95% used the Internet or social media for research prior to their prenatal visit; 96% of the parents found the prenatal visit helpful, and the most useful topics discussed were treatments (surgical, nonsurgical) and feeding techniques. CONCLUSION: This study identifies factors used to choose a cleft team/surgeon. Parents are more concerned with the experience level, reputation, and environment of the cleft team/surgeon than the distance traveled to get to the center.

Twin reversed arterial perfusion sequence: a new method of parabiotic twin mass estimation correlated with pump twin compromise.

OBJECTIVES: The purpose of this study was to test the hypothesis that using the formula of a prolate ellipsoid to estimate parabiotic twin mass correlates better with findings of pump twin compromise than using the sonographic method of Moore et al (Am J Obstet Gynecol 1990; 163:907-912). METHODS: A 10-year retrospective review was performed to identify all cases of the suspected twin reversed arterial perfusion (TRAP) sequence. Parabiotic twin mass was estimated by summing body and extremity volumes calculated using the prolate ellipsoid formula (width × height × length × 0.523). Parabiotic twin mass was also estimated using the sonographic Moore method [1.21 × length(2) - (1.66 × length)]. Parabiotic twin mass estimated by both methods was correlated with sonographic findings associated with increased risk of pump twin compromise. RESULTS: Fifty-nine pregnancies complicated by TRAP were identified. Using the prolate ellipsoid formula, the parabiotic twin mean sizes ± SD (as a percentage of pump twin weight) were 103.0% ± 52.0% and 56.9% ± 44.3% in cases with and without pump twin compromise (P = .0005), respectively. Using the sonographic Moore method, the mean parabiotic twin sizes were 122.9% ± 54.3% and 99.6% ± 62.8% in cases with and without pump twin compromise (P = .14). The median estimated masses of the parabiotic twin were 197 ± 219 g using the prolate ellipsoid formula and 310 ± 212 g using the sonographic Moore method (P = .0001). A parabiotic twin size greater than 70% of the pump twin correlated with findings associated with increased risk of pump twin compromise when using the prolate ellipsoid formula (P = .002) but not the sonographic Moore method (P = .09). CONCLUSIONS: Sonographic findings associated with increased risk of pump twin compromise correlate better with prolate ellipsoid estimates of parabiotic twin mass. The median estimated mass of the parabiotic twin was statistically larger when calculated by the sonographic Moore method than by the prolate ellipsoid formula.

Open and endoscopic fetal myelomeningocele surgeries display similar in-hospital safety profiles in a large, multi-institutional database.

BACKGROUND: Open intrauterine fetal myelomeningocele repair has demonstrated decreased ventriculoperitoneal shunting and improved motor outcomes despite maternal and fetal risks. Few data directly compare the safety of open vs endoscopic approaches. OBJECTIVE: This study aimed to analyze in-hospital maternal and fetal outcomes of pregnant patients undergoing open vs endoscopic fetal myelomeningocele repair using a large, multi-center database. STUDY DESIGN: This was a review of the Pediatric Health Information System database from October 1, 2015, to December 31, 2021. All patients who underwent open or endoscopic fetal myelomeningocele repair according to the International Classification of Diseases, Tenth Revision, were identified. Demographics, gestational age, and outcomes were analyzed. Descriptive and univariate statistics were used. RESULTS: A total of 378 pregnant patients underwent fetal myelomeningocele repair. The approach was endoscopic in 143 cases (37.8%) and open in 235 cases (62.2%). Overall postprocedural outcomes included no maternal in-hospital mortalities or intensive care unit admissions, a median length of stay of 4 days (interquartile range, 4-5), 14 cases (3.7%) of surgical and postoperative complications, 6 cases (1.6%) of intrauterine infections, 12 cases (3.2%) of obstetrical complications (including preterm premature rupture of membranes), 3 cases (0.8%) of intrauterine fetal demise, and 16 cases (4.2%) of preterm delivery. Compared with an open approach, the endoscopic approach occurred at a later gestational age (25 weeks [interquartile range, 24-25] vs 24 weeks [interquartile range, 24-25]; P<.001) and had an increased rate of intrauterine infection (6 [4.2%] cases vs 0 [0%] case; P=.002). There was no difference between approaches in the rates of surgical complications, obstetrical complications, intrauterine fetal demise, or preterm deliveries. CONCLUSION: Compared with an open approach, endoscopic fetal myelomeningocele repair displays a comparable rate of fetal complications, including intrauterine fetal demise and preterm delivery, and a similar in-hospital maternal safety profile despite an association with increased intrauterine infection.

Use of magnetic resonance imaging in prenatal prognosis of the fetus with isolated left congenital diaphragmatic hernia.

OBJECTIVES: To investigate the prognostic value of magnetic resonance-calculated fetal lung volumes (FLV) in fetuses with isolated left congenital diaphragmatic hernia (L-CDH) who receive standardized prenatal and postnatal care at a single institution. MATERIALS AND METHODS: A retrospective review was undertaken to identify fetuses with isolated L-CDH between 2001 and 2010. RESULTS: Eighty-five cases of isolated L-CDH were identified. The overall survival was 65% (55/85). Survival was 45% if there was 'liver up' (23/51) and 94% if there was 'liver down' (32/34). Univariate statistical analysis showed that 'liver up' (p=0.001), lung-to-head ratio (LHR) at diagnosis (p=0.009), observed/expected (O/E) LHR (p=0.01), total FLV (p=0.03), right LV (p=0.04), magnetic resonance imaging (MRI) observed versus expected (O/E) FLV (p=0.002), intrathoracic versus intraabdominal stomach (p=0.002), percentage of herniated liver (p=0.004), and postnatal extracorporeal membrane oxygenation use (p=0.001) are predictive of postnatal survival. Multivariate analysis of only prenatal factors showed that the most important determinants of postnatal outcome are percentage of herniated liver, presence of liver up, and MRI O/E FLV. CONCLUSION: Herniated intrathoracic liver expressed as 'liver up' or as percent herniated liver and MRI measurement of FLV expressed as a O/E ratio are strong prenatal indicators of postnatal survival.

Fetal MRI-calculated total lung volumes in the prediction of short-term outcome in giant omphalocele: preliminary findings.

OBJECTIVE: To determine the value of fetal MRI-calculated total lung volumes (TLV) in the prediction of short-term outcome in patients with giant omphalocele (GO). MATERIAL AND METHODS: We reviewed all cases of GO undergoing fetal MRI after 21 weeks' gestation and receiving postnatal care at our institution between 2003 and 2010. Observed/expected (O/E) TLV was calculated using age-matched TLV normograms [Radiology 2001;219:236-241]. Postnatal outcomes were stratified based on O/E TLV above or below 50% of expected. RESULTS: Seventeen GO cases fulfilled the entry criteria. The mean age at fetal MRI evaluation was 25.8 ± 4.8 weeks' gestation. The mean GO TLV (21.0 ± 13.2) was lower than age-matched population norms (p < 0.001), resulting in a mean O/E TLV of 52.3 ± 16.8%. The mean gestational age at delivery was 36.8 ± 1.6 weeks. Overall survival was 94%. Fourteen (88%) infants underwent staged reduction, and 2 underwent silver sulfadiazine treatment and delayed repair. Infants with ≤50% of predicted O/E TLV (n = 11, 65%) had lower Apgar scores at birth (p = 0.03), prolonged ventilatory support (p = 0.004), delayed oral intake (p = 0.03), and longer hospitalization (p = 0.03) compared to patients with ≥50% of expected O/E TLV. Two infants (both O/E TLV <50%) required tracheostomy placement. CONCLUSION: In the assessment of GO fetuses, MRI-based O/E TLV of <50% was predictive of increased postnatal morbidity.

Amniotic fluid levels of glial fibrillary acidic protein in fetal rats with retinoic acid induced myelomeningocele: a potential marker for spinal cord injury.

OBJECTIVE: The objective of this study was to determine whether amniotic fluid levels of glial acidic fibrillary protein (GFAP) would reflect myelomeningocele-related neurodegeneration in the rat model of retinoic acid-induced myelomeningocele, which is a model that is very similar to human myelomeningocele and develops the entire spectrum of disease severity including features of the Chiari II malformation. STUDY DESIGN: Time-dated (embryonic day 10) pregnant Sprague-Dawley rats were gavage fed 60 mg/kg/bodyweight retinoic acid that had been dissolved in olive oil or olive oil alone. Myelomeningocele, retinoic acid-exposed no myelomeningocele, and control fetuses were harvested at specific time points throughout gestation. A standard set of pinching tests was performed to interrogate the sensorimotor reflex arc of hindpaws and tails. Amniotic fluid-GFAP levels were analyzed by standard enzyme-linked immunosorbent assay techniques. RESULTS: Amniotic fluid-GFAP levels were similar between groups at embryonic days 14, 16, and 18, respectively. Compared with control fetuses, amniotic fluid GFAP levels were significantly increased in myelomeningocele fetuses at embryonic days 20 and 22 (P < .001). Defect size (P < .001), presence of clubfoot deformity (P = .0004), and absence of sensorimotor function (P < .01) at embryonic day 22 correlated with amniotic fluid-GFAP levels. CONCLUSION: Amniotic fluid-GFAP levels appear to correlate with spinal cord injury as gestation proceeds in fetal rats with myelomeningocele.

Open fetal surgery for myelomeningocele.

Despite efforts at prevention through the use of preconception folic acid, spina bifida remains one of the most common congenital anomalies of the central nervous system that is compatible with life. It is, however, associated with a significant degree of lifelong morbidity. The development of open fetal surgery for myelomeningocele (MMC) has been a long process but one that serves as a model for how new procedures and technologies need to be properly evaluated before being brought into mainstream medical practice. Even so, risks and benefits need to be evaluated for each patient. The currently available studies have been carried out on a highly selected patient population where the fetal findings provided the maximum opportunity for benefit from prenatal closure of the MMC defect. There is the potential that as the surgery becomes more widely available, pressure will be brought to bear to perform surgery in cases where the likelihood for benefit is decreased and yet the risks are not. The only way to duplicate the results of the current studies is to follow the methodology and criteria that were used in the studies. This will mean that not every fetus with an MMC will be a candidate for in utero surgery. The balance of risk to benefit will continue to evolve as further technological advances are evaluated and more follow-up information is obtained.

Fetal myelomeningocele surgery: preschool functional status using the Functional Independence Measure for children (WeeFIM).

OBJECTIVE: To study preschool functional status in children following fetal myelomeningocele (fMMC) surgery. MATERIAL AND METHODS: Prior to the NICHD-MOMS trial, 30 fMMC underwent standardized neurodevelopmental examination at 5 years of age. Functional status was determined with the Functional Independence Measure (WeeFIM), which assesses self-care, mobility, and cognitive independence. RESULTS: Evaluations were completed in 26 (87%). Mean cognitive (93.0 ± 21.9), self-care (66.5 ± 23.9), mobility (82.3 ± 19.5), and total (77.9 ± 20.3) functional quotient of fMMC children were significantly lower than age-matched population norms (P < 0.01). Complete caregiver independence was achieved by 22 (84%), 10 (38%), 16 (62%), and 15 (58%) fMMC children for cognition, self-care, mobility, and total functional outcome, respectively. Cognitive, mobility, and total independence were higher in non-shunted than shunted fMMC children (P = 0.02, P = 0.02, and P < 0.01, respectively) and in fMMC children with average neurodevelopmental scores (P < 0.001, P = 0.01, and P < 0.01, respectively). Self-care independence tended to be higher in the non-shunted group and in fMMC children with normal neurodevelopmental outcome (P = 0.07 and P = 0.09, respectively). CONCLUSION: The majority of fMMC children achieved cognitive and mobility independence, but continue to require significant assistance in self-care. Non-shunted and fMMC children with normal neurodevelopmental outcome were more likely to be independent in daily living activities. Better understanding of the extent of functional limitations following fMMC surgery will allow for more effective early interventions geared toward maximizing independence in everyday tasks in all environments.

Preschool neurobehavioral outcome following fetal myelomeningocele surgery.

OBJECTIVE: It was the aim of this study to assess the prevalence of preschool neurobehavioral problems in children following fetal myelomeningocele (fMMC) surgery. METHODS: Prior to the Management of Myelomeningocele Study, 30 fMMC patients underwent a standardized neurodevelopmental examination at 5 years of age. The prevalence of behavioral problems was assessed by the Child Behavior Check List (CBCL), which includes a total problem score and 2 broad-band indices (internalizing and externalizing problems). fMMC children were classified as having normal, at-risk or clinically significant scores based on calculated T scores. RESULTS: Twenty-two (73%) fMMC families completed the evaluation. Mean age at delivery following fMMC surgery was 35.5 ± 1.6 weeks. The prevalence of 'at-risk' or 'clinically significant' scores for internalizing, externalizing and total behavioral problems was similar to general population norms. No difference in overall internalizing (p = 0.19), externalizing (p = 0.54) and total behavioral (p = 0.18) scores was found between non-shunted and shunted fMMC children. However, shunted fMMC children were more likely to experience anxiety and depressive (p = 0.02), pervasive developmental (p = 0.03) and withdrawn (p = 0.06) behavior. Oppositional defiant, attention deficit and hyperactivity problems were not found. No correlation was found between overall neurodevelopmental outcome and abnormal CBCL scores. CONCLUSIONS: fMMC surgery and subsequent preterm delivery is not associated with increased behavioral problems, impaired social interactions and restricted behavior patterns. Shunted fMMC children were more likely to have scores in the 'at-risk' or 'clinically significant' range for multiple CBCL subindices. Early detection of behavioral problems following fMMC surgery provides an opportunity for targeted and timely intervention enabling affected fMMC children to be socially successful.

Preschool neurodevelopmental outcome of children following fetal myelomeningocele closure.

OBJECTIVE: We sought to investigate the preschool neurodevelopmental outcomes of children following fetal myelomeningocele (fMMC) surgery. STUDY DESIGN: Prior to the Management of Myelomeningocele Study trial, 54 children underwent fMMC closure at our institution. Thirty (56%) returned at 5 years of age for standardized neurocognitive examination. Scores were grouped as high-average, average, mildly delayed, and severely delayed by SD intervals. RESULTS: Mean verbal intelligence quotient (VIQ), performance intelligence quotient (PIQ), and full intelligence quotient (FIQ) scores were within normal population range. High-average or average scores for VIQ, PIQ, FIQ, and processing speed were found in 93%, 90%, 90%, and 60%, respectively. Mean FIQ and processing speed of nonshunted children were significantly higher than for those who required shunt placement (P=.02 and P=.01, respectively). Mean VIQ and PIQ tended to be higher in nonshunted fMMC children (P=.05). CONCLUSION: The majority of fMMC children in this highly selective population had average preschool neurodevelopmental scores. fMMC children who did not require shunt placement were more likely to have better scores.

Placental Anatomy and Function in Twin Gestations.

With an increasing incidence of twin gestations, understanding the inherent risks associated with these pregnancies is essential in modern obstetrics. The unique differences in placentation in twins contribute to the increased risks. Monochorionic twins are susceptible to complications because of their unique placental architecture, including twin-to-twin transfusion syndrome, the twin anemia-polycythemia sequence, selective intrauterine growth restriction, and the twin reversed arterial perfusion sequence. Knowing the clinical correlations of placental anatomy in these gestations helps perinatal pathologists perform a more informed placental evaluation, allowing for better care for the mother and her children.

When is fetoscopic release of amniotic bands indicated? Review of outcome of cases treated in utero and selection criteria for fetal surgery.

OBJECTIVES: The objectives are to analyze the outcomes of fetal interventions for fetal limb abnormalities associated with amniotic band syndrome (ABS), to compare the outcome with the known natural history, and to establish selection criteria for fetal intervention. MATERIAL AND METHODS: In a Medline search, six cases of prenatal fetoscopic interventions for ABS were found. An unpublished case was added. RESULTS: Review of the seven cases of treated ABS in utero suggests that abnormal, but present blood flow at Doppler distal to the area constricted by the band may optimally identify cases suitable for fetal surgery. We propose a prenatal classification in stages of cases of ABS based on ultrasound and Doppler findings. Premature rupture of membranes (PROM) occurred in five patients (71%). The median gestational age (GA) at delivery was 34.8 weeks (range 32 to 39). The median time between procedure and PROM was 6 weeks (range 4 days to 14.3 weeks). The median time between procedure and delivery was 11.8 weeks (range 5-17). CONCLUSION: The use of a uniform prenatal classification of cases of ABS may allow a more precise correlation of prenatal findings and postnatal outcome.

Sacrococcygeal teratomas: prenatal surveillance, growth and pregnancy outcome.

OBJECTIVE: Prenatal surveillance and growth characteristics are evaluated in a cohort of fetuses with sacrococcygeal teratomas (SCT) as part of risk assessment. METHODS: Retrospective review of 23 fetuses with SCT: prenatal diagnosis, surveillance, delivery, and early postnatal outcome are reported. RESULTS: Cardiac output failure physiology requires serial evaluation. The size of the SCT determines obstetrical risks and mode of delivery. An SCT growth rate approaching >150 cm(3) per week may be associated with increased perinatal mortality risks. Maternal morbidity is related mainly to polyhydramnios and preterm labor. CONCLUSIONS: Perinatal mortality is approximately 43%. Maternal-fetal surgery for fetal physiologic deterioration is not frequent, but serial surveillance is required to minimize fetal morbidity/mortality and maternal morbidity. Rapid SCT growth rates may be associated with increased risk of perinatal mortality.

Lower extremity neuromotor function and short-term ambulatory potential following in utero myelomeningocele surgery.

OBJECTIVE: To evaluate lower extremity neuromotor function (LENF) and short-term ambulatory potential following fetal myelomeningocele (fMMC) closure. METHODS: Retrospective chart review of 54 children that underwent fMMC closure at our institution prior to the NIHCD-MOMS trial. Neonatal LENF was compared to predicted function based on spinal lesion level assigned by prenatal ultrasound. Ambulatory status was classified as independent walkers (walks without assistive appliances), assisted walker (requires walking aid), and non-ambulatory (wheelchair bound). RESULTS: Thoracic, lumbar, and sacral level lesions were present in 4, 44 and 6 fMMC infants, respectively. 31/54 of fMMC children (57.4%; median: 2 levels, range: 1-5) had better than predicted, 13/54 (24.1%) same as predicted and 10/54 (18.5%; median: 1 level, range: 1-2) worse than predicted LENF at birth. At a median follow-up age of 66 months (36-113), 37/54 (69%) walk independently, 13/54 (24%) are assisted walkers, and 4/54 (7%) are wheelchair dependent. The strongest factors predicting a lower likelihood to walk independently were higher-level lesion (>L4, p = 0.001) and the development of clubfoot deformity after fetal intervention (p = 0.026). Despite the observed improved ambulatory status, structured evaluation of coordinative skills revealed that the majority of independent ambulators and all children that require assistive devices to walk experience significant deficits in lower extremity coordination. CONCLUSIONS: We observed that fMMC surgery in this highly selective population results in better than predicted LENF at birth and short-term ambulatory status. However, fMMC toddlers continue to demonstrate deficits in movement coordination that are characteristic for children with spina bifida.

Prenatal course and postnatal management of peripheral bronchial atresia: association with congenital cystic adenomatoid malformation of the lung.

OBJECTIVE: Peripheral bronchial atresia (PBA), a newly identified fetal lung lesion, is often asymptomatic and managed nonoperatively. However, recent studies suggest that bronchial atresia plays a role in the etiology of microcystic maldevelopment present in congenital cystic adenomatoid malformations (CCAM) which require resection to decrease the risk of infection, pneumothorax and malignant degeneration. The purpose of this study was to evaluate the prenatal radiographic and postnatal computed tomography (CT) scan/pathology findings with attention to the pathologic diagnosis of microcystic maldevelopment in infants with the presumptive diagnosis of PBA. METHODS: A retrospective review of prenatal and postnatal records of patients diagnosed with fetal lung lesions was performed. Two groups of patients were identified: (1) patients diagnosed with PBA on postnatal CT scan (n = 16), and (2) patients with the pathologically confirmed diagnosis of PBA independent of postnatal CT findings (n = 23). RESULTS: Prenatal ultrasound diagnosis of these lesions included CCAMs, hybrid lesions, bronchopulmonary sequestrations and bronchial atresia. Eleven of the 16 patients in group 1 with the postnatal radiologic diagnosis of PBA underwent surgical resection, 6 of which were found to have microcystic changes consistent with CCAM. Evaluation of the 23 patients in group 2 with pathologically confirmed PBA identified 65% that had evidence of microcystic maldevelopment consistent with the small cyst type of CCAM. CONCLUSION: Radiographically diagnosed PBA as well as pathologically confirmed PBA is frequently associated with microcystic changes consistent with the small cyst type of CCAM. Thus, operative management should be considered for PBA to decrease CCAM-associated risks.