Alveolar Cleft Size on Prenatal Two-Dimensional Ultrasonography Predicts Cleft of the Secondary Palate in Unilateral Cleft Lip.

SUMMARY: Prenatal diagnosis of cleft palate (CP) is challenging. The current study's objective was to investigate whether prenatal alveolar cleft width is associated with the likelihood of a cleft of the secondary palate in unilateral cleft lip (CL). The authors reviewed two-dimensional ultrasound (US) images in fetuses with unilateral CL from January of 2012 to February of 2016. Images of the fetal face were obtained with a linear and/or curved probe in the axial and coronal planes. Measurements of the alveolar ridge gap were taken by the senior radiologist. Postnatal phenotype findings were compared with prenatal findings. Thirty patients with unilateral CL met inclusion criteria; average gestational age was 26.67 ± 5.11 weeks (range, 20.71 to 36.57 weeks). Ten fetuses were found to have an intact alveolar ridge by prenatal US; postnatal examination confirmed intact secondary palate in all. Small alveolar defects (<4 mm) were noted in three fetuses; postnatal examination documented CP in a single patient. CP was confirmed in 15 of the remaining 17 fetuses who had alveolar cleft width greater than 4 mm. An alveolar defect of greater than or equal to 4 mm on prenatal US was associated with greater likelihood of a cleft of the secondary palate [c 2 (2, n = 30) = 20.23; P < 0.001]. In the setting of unilateral CL, prenatal US documentation of alveolar defects greater than or equal to 4 mm are highly predictive of the presence of a cleft of the secondary palate. Conversely, an intact alveolar ridge is associated with an intact secondary palate. CLINICAL QUESTION/LEVEL OF EVIDENCE: Diagnostic, II.

Mini thoracic CT adequately determines Haller index and decreases radiation exposure in children with pectus excavatum.

INTRODUCTION: The preoperative assessment of Pectus Excavatum (PE) is resource intensive. CT chest for the purpose of calculating a Haller index (HI) remains a central component and is necessary for third-party reimbursment for surgical correction. With the goal of minimizing radiation exposure, a strategy was introduced to perform a mini-Thoracic CT (mini-CT) for the calculation of HI. OPERATIVE TECHNIQUE: The mini-CT was performed as follows: a radio-opaque marker (ROM) was placed at the clinical deepest point of the deformity. The CT was then columnated to scan 3 cm above and 3 cm below the ROM. HI was calculated according to previously described technique. Seven children with PE who underwent mini-CT were age and weight matched to 7 children with PE who underwent standard low dose CT chest during the same time period. Radiation doses were evaluated using dose length product (DLP) and effective dose (mSv) between the two groups. Significance of differences was determined using the students t-test. The DLP of mini-CT compared to chest-CT was 17.9 vs 48.9,mGycm respectively. (p< 0.001) The mSv of the mini-CT compared to chest-CT was 0.32 vs 0.88, sMV respectively. (p<0.001) Both DLP and mSv were reduced by 63% in children who received a mini-CT. All children obtained insurance authorization and underwent uncomplicated Nuss repair. CONCLUSION: For children with pectus excavatum deformities the mini-Thoracic CT is an effective method to calculate the HI. Compard to the conventional low dose chest CT, the mini-CT strategy significantly reduces radiation exposure to the child by 63% with no impact on third-party authorizations or Nuss repair.

Complexities of Zika Diagnosis and Evaluation in a U.S. Congenital Zika Program.

The objective of the study was to describe the complexity of diagnosis and evaluation of Zika-exposed pregnant women/fetuses and infants in a U.S. Congenital Zika Program. Pregnant women/fetuses and/or infants referred for clinical evaluation to the Congenital Zika Program at Children's National (Washington, DC) from January 2016 to June 2018 were included. We recorded the timing of maternal Zika-virus (ZIKV) exposure and ZIKV laboratory testing results. Based on laboratory testing, cases were either confirmed, possible, or unlikely ZIKV infection. Prenatal and postnatal imaging by ultrasound and/or magnetic resonance imaging (MRI) were categorized as normal, nonspecific, or as findings of congenital Zika syndrome (CZS). Of 81 women-fetus/infant pairs evaluated, 72 (89%) had confirmed ZIKV exposure; 18% of women were symptomatic; only a minority presented for evaluation within the time frame for laboratory detection. Zika virus could only be confirmed in 29 (40%) cases, was possible in 26 (36%) cases, and was excluded in 17 (24%) cases. Five cases (7%) had prenatal ultrasound and MRI findings of CZS, but in only three was ZIKV confirmed by laboratory testing. Because of timing of exposure to presentation, ZIKV infection could not be excluded in many cases. Neuroimaging found CZS in 7% of cases, and in many patients, there were nonspecific imaging findings that warrant long-term follow-up. Overall, adherence to postnatal recommended follow-up evaluations was modest, representing a barrier to care. These challenges may be instructive to future pediatric multidisciplinary clinics for congenital infectious/noninfectious threats to pregnant women and their infants.

Prenatal diagnosis of intestinal nonrotation using magnetic resonance imaging: Is it possible?

BACKGROUND: Malrotation of the bowel refers to any variation in the rotation and fixation of the gastrointestinal tract during the first trimester and is most commonly detected postnatally. Nonrotation of the bowel and incomplete rotation of the bowel are subtypes of malrotation. OBJECTIVE: To determine if the nonrotation subtype of malrotation of the bowel can be detected on prenatal magnetic resonance imaging (MRI). MATERIALS AND METHODS: Cases from 2012 to 2018 with nonrotation of the bowel without obstruction confirmed by imaging, surgery and/or autopsy were compared to prenatal imaging. Prenatal imaging was retrospectively reviewed to determine if prenatal diagnosis of malrotation could be made. Exclusion criteria included diaphragmatic hernia, omphalocele and gastroschisis. RESULTS: Ten cases of nonrotation diagnosed postnatally by upper gastrointestinal series (upper GI)/small bowel follow-through (SBFT) or autopsy had prenatal MRI. Prenatal MR studies were performed for assessment of heterotaxy syndrome with congenital heart disease (6/10), congenital heart disease with additional anomalies (suspected VACTERL [vertebral, anorectal, cardiac, tracheoesophageal, renal, limb] and suspected lung agenesis, ventriculomegaly) (3/10) and skeletal dysplasia (1/10). Eight upper GI/SBFT cases demonstrated nonrotation of the bowel without obstruction with the small bowel completely on one side of the abdomen contralateral to the stomach and the colon ipsilateral to the stomach; four cases were confirmed by surgery. The small bowel in one upper GI/SBFT case was unilateral contralateral to the stomach with a meandering colon. One case had nonrotation diagnosed at autopsy. There were no cases of postnatal midgut volvulus. Retrospective review of the 10 cases had prenatal MRI performed between 23 and 37 weeks of gestation. The coronal plane was the most optimal plane to assess the position of the stomach, small bowel and colon in relationship to each other. The small bowel was best assessed on T2-weighted images while the colon was best assessed on T1-weighted images. A nonrotated position of the small bowel was present in all 10 fetal MRI cases mirroring postnatal findings, with the small bowel contralateral to the stomach in 9/10 cases and ipsilateral to the stomach (in the right abdomen) in 1/10 cases. The colon was visualized by prenatal MRI in 9/10 cases, with 1 case limited due to a lack of T1-weighted imaging. A nonrotated position of the colon contralateral to the small bowel was present in 7/9 cases. In 2/9 cases, the colon was wandering, positioned on both sides of the midline. Colonic position in all nine cases matched postnatal findings. No cases presented with prenatal bowel obstruction. CONCLUSION: Detection of nonrotation of the bowel is possible on prenatal MRI.

Fourteen-year follow-up of a child with acroscyphodysplasia with emphasis on the need for multidisciplinary management: a case report.

BACKGROUND: Acroscyphodysplasia has been described as a phenotypic variant of acrodysostosis type 2 and pseudohypoparathyroidism. In acrodysostosis, skeletal features can include brachydactyly, facial hypoplasia, cone-shaped epiphyses, short stature, and advanced bone age. To date, reports on this disorder have focused on phenotypic findings, endocrine changes, and genetic variation. We present a 14-year overview of a patient, from birth to skeletal maturity, with acroscyphodysplasia, noting the significant orthopaedic challenges and the need for a multidisciplinary team, including specialists in genetics, orthopaedics, endocrinology, and otolaryngology, to optimize long-term outcomes. CASE PRESENTATION: The patient presented as a newborn with dysmorphic facial features, including severe midface hypoplasia, malar flattening, nasal stenosis, and feeding difficulties. Radiologic findings were initially subtle, and a skeletal survey performed at age 7 months was initially considered normal. Genetic evaluation revealed a variant in PDE4D and subsequent pseudohypoparathyroidism. The patient presented to the department of orthopaedics, at age 2 years 9 months with a leg length discrepancy, right knee contracture, and severely crouched gait. Radiographs demonstrated cone-shaped epiphyses of the right distal femur and proximal tibia, but no evidence of growth plate changes in the left leg. The child developed early posterior epiphyseal arrest on the right side and required multiple surgical interventions to achieve neutral extension. Her left distal femur developed late posterior physeal arrest and secondary contracture without evidence of schypho deformity, which improved with anterior screw epiphysiodesis. The child required numerous orthopaedic surgical interventions to achieve full knee extension bilaterally. At age 13 years 11 months, she was an independent ambulator with erect posture. The child underwent numerous otolaryngology procedures and will require significant ongoing care. She has moderate intellectual disability. DISCUSSION AND CONCLUSIONS: Key challenges in the management of this case included the subtle changes on initial skeletal survey and the marked asymmetry of her deformity. While cone-shaped epiphyses are a hallmark of acrodysostosis, posterior tethering/growth arrest of the posterior distal femur has not been previously reported. Correction of the secondary knee contracture was essential to improve ambulation. Children with acroscyphodysplasia require a multidisciplinary approach, including radiology, genetics, orthopaedics, otolaryngology, and endocrinology specialties.

Normal size of the fetal adrenal gland on prenatal magnetic resonance imaging.

BACKGROUND: The adrenal gland plays a vital role in fetal growth. Many disease states such as congenital adrenal hyperplasia, hemorrhage and tumors can lead to morphological changes in the gland. Ultrasound measurements of normal adrenal sizes in the fetus reported in the literature have shown a trend of increasing size with gestational age. There is no literature available on standard fetal adrenal sizes or detailed appearance by fetal MRI. OBJECTIVE: The purpose of this study was to provide MR data on the size and signal characteristics of the fetal adrenal gland throughout the second and third trimesters. MATERIALS AND METHODS: In this retrospective review, we selected 185 prenatal MRIs obtained from Jan. 1, 2014, to May 31, 2017, with normal abdominal findings for inclusion. The adrenal glands were identified in coronal, sagittal or axial T2-W planes and coronal T1-W plane when available. We measured the length and thickness of the medial and lateral limbs of the right and left adrenal glands and recorded signal intensity on T1-W and T2-W sequences, gender and gestational age in each case. RESULTS: The gestational age (GA) ranged 18-37 weeks. Visibility of the adrenal glands on T2-W images was high (90.3-97.2%) up to 30 weeks of GA but declined afterward (47.5-62.2% at 31-37 weeks). Visibility on T1-W images increased with GA, ranging from 21.4% visibility at 18-22 weeks and increasing to 40% at 35-37 weeks. Mean lengths of the adrenal gland limbs steadily increased from 8.2 mm at 18-22 weeks to 11.0 mm at 35-37 weeks. In the second trimester, adrenal glands were low in signal intensity on T2-W images and were surrounded by hyperintense perirenal fatty tissue. In the third trimester, the glands became less distinct, with increasing signal and obliteration of perirenal tissue. The glands were moderately hyperintense on T1-W images throughout pregnancy, with increasing visibility as pregnancy progressed. CONCLUSION: Normal sizes and signal intensities for adrenal glands are reported. Visibility of adrenal glands on T2-W images was 90.3-97.2% up to 30 weeks but declined thereafter. Visibility on T1-W images increased in the third trimester. Adrenal gland sizes increased with gestational age.

Coordination of the Fetal Medicine Institute and the Cleft and Craniofacial Center: Application to Early Management of Infants With Cleft Lip and Palate.

BACKGROUND/PURPOSE: The primary objective of this study is to describe the authors' experience at the Children's National Health System with the coordination of the Fetal Medicine Institute and the Cleft and Craniofacial Center. This collaboration highlights the accuracy and completeness of prenatal diagnosis of cleft abnormalities with expedient postnatal management. METHODS: With Institutional Review Board approval, the authors retrospectively reviewed 74 patients referred for potential orofacial cleft and 44 met the inclusion criteria. Follow-up fetal ultrasonography is typically performed and three-dimensional imaging was performed when feasible. If questionable anomalies or facial findings are present on these studies, the authors proceed with fetal magnetic resonance imaging. A thorough consultation is held with the cleft team, resulting in a comprehensive plan of care. Postnatal examination confirmed the correct prenatal diagnosis in nearly all patients. RESULTS: Sensitivity and specificity for isolated unilateral cleft lip were 89% and 100%, respectively; for unilateral cleft lip and palate, sensitivity and specificity were 82% and 90%, respectively; for bilateral cleft lip and palate, sensitivity and specificity were 97% and 90%, respectively. Initial postnatal evaluation by the cleft surgeon occurred at an average age of 21 days after birth. All patients who were candidates for presurgical orthodontia were treated at an appropriate young age (mean: 66.5 days). CONCLUSIONS: Coordinated prenatal evaluation of patients with cleft lip/palate by multidisciplinary centers plays an important role in the care of these complex patients. The results of the authors' study demonstrated high sensitivity and specificity for the prenatal diagnosis of cleft lip/palate, leading to timely postnatal evaluation and treatment.

Sequential Neuroimaging of the Fetus and Newborn With In Utero Zika Virus Exposure.

Importance: The evolution of fetal brain injury by Zika virus (ZIKV) infection is not well described. Objectives: To perform longitudinal neuroimaging of fetuses and infants exposed to in utero maternal ZIKV infection using concomitant magnetic resonance imaging (MRI) and ultrasonography (US), as well as to determine the duration of viremia in pregnant women with ZIKV infection and whether the duration of viremia correlated with fetal and/or infant brain abnormalities. Design, Setting, and Participants: A cohort of 82 pregnant women with clinical criteria for probable ZIKV infection in Barranquilla, Colombia, and Washington, DC, were enrolled from June 15, 2016, through June 27, 2017, with Colombian women identified by community recruitment and physician referral and travel-related cases of American women recruited from a Congenital Zika Program. Interventions and Exposures: Women received 1 or more MRI and US examinations during the second and/or third trimesters. Postnatally, infants underwent brain MRI and cranial US. Blood samples were tested for ZIKV. Main Outcomes and Measures: The neuroimaging studies were evaluated for brain injury and cerebral biometry. Results: Of the 82 women, 80 were from Colombia and 2 were from the United States. In 3 of 82 cases (4%), fetal MRI demonstrated abnormalities consistent with congenital ZIKV infection. Two cases had heterotopias and malformations in cortical development and 1 case had a parietal encephalocele, Chiari II malformation, and microcephaly. In 1 case, US results remained normal despite fetal abnormalities detected on MRI. Prolonged maternal polymerase chain reaction positivity was present in 1 case. Of the remaining 79 cases with normal results of prenatal imaging, postnatal brain MRI was acquired in 53 infants and demonstrated mild abnormalities in 7 (13%). Fifty-seven infants underwent postnatal cranial US, which detected changes of lenticulostriate vasculopathy, choroid plexus cysts, germinolytic/subependymal cysts, and/or calcification in 21 infants (37%). Conclusions and Relevance: In a cohort of pregnant women with ZIKV infection, prenatal US examination appeared to detect all but 1 abnormal fetal case. Postnatal neuroimaging in infants who had normal prenatal imaging revealed new mild abnormalities. For most patients, prenatal and postnatal US may identify ZIKV-related brain injury.

Skeletal Consequences of Nephropathic Cystinosis.

Nephropathic cystinosis is a rare lysosomal storage disorder. Patients present in the first year of life with renal Fanconi syndrome that evolves to progressive chronic kidney disease (CKD). Despite the multiple risk factors for bone disease, the frequency and severity of skeletal disorders in nephropathic cystinosis have not been described. We performed systematic bone and mineral evaluations of subjects with cystinosis seen at the NIH (n = 30), including history and physical examination, serum and urine biochemistries, DXA, vertebral fracture assessment, skeletal radiographs, and renal ultrasound. Additionally, histomorphometric analyses are reported on six subjects seen at the UCLA Bone and Mineral Metabolism Clinic. In NIH subjects, mean age was 20 years (range, 5 to 44 years), 60% were CKD stages G1 to G4, and 40% had a renal transplant. Mean bone mineral density (BMD) Z-scores were decreased in the femoral neck, total hip, and 1/3 radius (p < 0.05). Low bone mass at one or more sites was present in 46% of subjects. Twenty-seven percent of subjects reported one or more long bone fractures. Thirty-two percent of subjects had incidental vertebral fractures, which were unrelated to transplant status. Long-bone deformity/bowing was present in 64%; 50% had scoliosis. Diffuse osteosclerosis was present in 21% of evaluated subjects. Risk factors included CKD, phosphate wasting, hypercalciuria, secondary hyperparathyroidism, hypovitaminosis D, male hypogonadism, metabolic acidosis, and glucocorticoid/immunosuppressive therapy. Sixty-one percent of the non-transplanted subjects had ultrasonographic evidence of nephrocalcinosis or nephrolithiasis. Histomorphometric analyses showed impaired mineralization in four of six studied subjects. We conclude that skeletal deformities, decreased bone mass, and vertebral fractures are common and relevant complications of nephropathic cystinosis, even before renal transplantation. Efforts to minimize risk factors for skeletal disease include optimizing mineral metabolism and hormonal status, combined with monitoring for nephrocalcinosis/nephrolithiasis. © 2018 This article is a U.S. Government work and is in the public domain in the USA.

Severe nasomaxillary hypoplasia (Binder phenotype) on prenatal US/MRI: an important marker for the prenatal diagnosis of chondrodysplasia punctata.

BACKGROUND: Chondrodysplasia punctata is a skeletal dysplasia caused by a diverse spectrum of etiologies, with outcomes ranging from antenatal demise to a normal life span. Prenatal detection can be challenging. OBJECTIVE: To review a series of cases of chondrodysplasia punctata associated with nasomaxillary hypoplasia, known as the Binder phenotype, and to highlight prenatal ultrasound and MRI findings, as well as postnatal MRI and radiographic findings. MATERIALS AND METHODS: We retrospectively reviewed ultrasound, MRI and radiographic imaging findings in postnatally confirmed cases of chondrodysplasia punctata from 2001 to 2017. We analyzed prenatal findings and correlated them with maternal history, postnatal imaging, phenotype, genetics and outcome. RESULTS: We identified eight cases, all with prenatal US and six of eight with prenatal MRI between 18 weeks and 32 weeks of gestational age. Reasons for referral included midface hypoplasia in four cases; family history in one case; intrauterine growth restriction in one case; short long-bones, intrauterine growth restriction and multicystic kidney in one case; and multiple anomalies in one case. In six cases, postnatal radiographs were performed. In four cases, postnatal spine MRI imaging was performed. The diagnosis of chondrodysplasia punctata was suggested in prenatal reports in six of eight fetuses. Seven of eight fetuses had Binder phenotype with severe nasomaxillary hypoplasia. Limb length was mildly symmetrically short in four of eight cases and normal in four of eight fetuses. Two of eight fetuses had epiphyseal stippling identified prenatally by US; this was present postnatally in six neonates on radiographs. Hand and foot abnormalities of brachytelephalangy were not detected on the prenatal US or MRI but were present in six of eigth fetuses on postnatal radiographs or physical exam. Four of eight fetuses had prenatal spine irregularity on US from subtle stippling. Six of eight had spine stippling on postnatal radiographs. One fetus had cervicothoracic kyphosis on prenatal US and MRI, and this was postnatally present in one additional neonate. One case had prenatally suspected C1 spinal stenosis with possible cord compression, and this was confirmed postnatally by MRI. There was a maternal history of systemic lupus erythematosus in two and hyperemesis gravidarum in one. Outcomes included one termination and seven survivors. CONCLUSION: Chondrodysplasia punctata can be identified prenatally but findings are often subtle. The diagnosis should be considered when a fetus presents with a hypoplastic midface known as the Binder phenotype. Maternal history of lupus, or other autoimmune diseases or hyperemesis gravidarum can help support the diagnosis.

X-linked hypomyelination with spondylometaphyseal dysplasia (H-SMD) associated with mutations in AIFM1.

An X-linked condition characterized by the combination of hypomyelinating leukodystrophy and spondylometaphyseal dysplasia (H-SMD) has been observed in only four families, with linkage to Xq25-27, and recent genetic characterization in two families with a common AIFM1 mutation. In our study, 12 patients (6 families) with H-SMD were identified and underwent comprehensive assessment accompanied by whole-exome sequencing (WES). Pedigree analysis in all families was consistent with X-linked recessive inheritance. Presentation typically occurred between 12 and 36 months. In addition to the two disease-defining features of spondylometaphyseal dysplasia and hypomyelination on MRI, common clinical signs and symptoms included motor deterioration, spasticity, tremor, ataxia, dysarthria, cognitive defects, pulmonary hypertension, nystagmus, and vision loss due to retinopathy. The course of the disease was slowly progressive. All patients had maternally inherited or de novo mutations in or near exon 7 of AIFM1, within a region of 70 bp, including synonymous and intronic changes. AIFM1 mutations have previously been associated with neurologic presentations as varied as intellectual disability, hearing loss, neuropathy, and striatal necrosis, while AIFM1 mutations in this small region present with a distinct phenotype implicating bone. Analysis of cell lines derived from four patients identified significant reductions in AIFM1 mRNA and protein levels in osteoblasts. We hypothesize that AIFM1 functions in bone metabolism and myelination and is responsible for the unique phenotype in this condition.

Achondroplasia in the Premature Infant: An Elusive Diagnosis in the Neonatal Intensive Care Unit.

Achondroplasia is a difficult prenatal diagnosis to make before the late second and third trimester. We describe two cases where an infant was born prematurely with no overt signs of achondroplasia. Despite multiple chest and abdominal radiographs during the neonatal course, the diagnosis was not made until term equivalent age was reached. We retrospectively reviewed these two cases to highlight the elusive findings of achondroplasia in the premature infant.

Prenatal magnetic resonance and ultrasonographic findings in small-bowel obstruction: imaging clues and postnatal outcomes.

BACKGROUND: Prenatal small-bowel obstruction can result from single or multiple atresias, and it can be an isolated abnormality or part of a syndrome. It is sometimes the first manifestation of cystic fibrosis. Accurate prediction of the level of obstruction and length of bowel affected can be difficult, presenting a challenge for counseling families and planning perinatal management. OBJECTIVE: To review the prenatal US and MRI findings of small-bowel obstruction and to assess whether fetal MRI adds information that could improve prenatal counseling and perinatal management. MATERIALS AND METHODS: We retrospectively reviewed 12 prenatally diagnosed cases of small-bowel obstruction evaluated by both US and MRI from 2005 to 2015. We analyzed gestational age at evaluation, US and MRI findings, gestational age at delivery and postnatal outcomes. RESULTS: The final diagnoses were jejunal atresia (7), ileal atresia (1), cystic fibrosis (3) and combined jejunal and anal atresia (1). Four of the eight with jejunal atresia were found to have multiple small-bowel atresias. Prenatal perforation was noted in three. We identified a trend of increasing complexity of bowel contents corresponding to progressively distal level of obstruction, as indicated by increasing US echogenicity and high T1 signal on MRI. Seven cases of jejunal atresia and one case of ileal atresia demonstrated small ascending, transverse and descending colon (microcolon) with filling of a normal-diameter rectum. In contrast, all three fetuses with cystic fibrosis and the fetus with jejunal-anal atresia demonstrated microcolon as well as abnormal paucity or absence of rectal meconium. Polyhydramnios was present in nine. Eight were delivered prematurely, of whom seven had polyhydramnios. The fetus with jejunal and anal atresia died in utero. Postnatally, three had short gut syndrome, all resulting from multiple jejunal atresias; these three were among a subset of four fetuses whose bowel diameter measured more than 3 cm. Eight infants had no further gastrointestinal complications. The presence of multiple atresias was not predicted by prenatal US or MRI. CONCLUSION: MR provides useful additional information regarding meconium distribution in the small bowel, which helps to clarify the level of obstruction. MR was additionally useful in the assessment of colon and rectal contents, serving as a fetal enema. Abnormally diminished meconium in the rectum suggests cystic fibrosis or combined small-bowel and colonic obstruction, information that is useful in counseling and preparing for postnatal care.

Magnetic Resonance Imaging Evaluation of Fetal Neural Tube Defects.

Spinal dysraphism occurs early in gestation because of an abnormality in the closure of the neural tube. Defects can be classified as open or closed lesions based on clinical and imaging features. Biochemical evaluation and ultrasound studies are used as screening tools for neural tube defects. Ultrasound alone can accurately diagnose most neural tube lesions. Magnetic resonance imaging has increasingly been used as an adjuvant study and is useful in the assessment of the degree of hindbrain herniation and evaluation of the fetal brain and spinal cord anatomy when ultrasound is limited. This additional information can be useful in counseling, helping to determine if fetal surgery is an option as well as helping to plan delivery and postnatal management.

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.

Antenatal Magnesium and Cerebral Palsy in Preterm Infants.

OBJECTIVE: To evaluate the relationship of maternal antenatal magnesium sulfate (MgSO4) with neonatal cranial ultrasound abnormalities and cerebral palsy (CP). STUDY DESIGN: In a randomized trial of MgSO4 or placebo in women at high risk of preterm delivery, up to 3 cranial ultrasounds were obtained in the neonatal period. Images were reviewed by at least 2 pediatric radiologists masked to treatment and other clinical conditions. Diagnoses were predefined for intraventricular hemorrhage, periventricular leukomalacia, intracerebral echolucency or echodensity, and ventriculomegaly. CP was diagnosed at 2 years of age by standardized neurologic examination. RESULTS: Intraventricular hemorrhage, periventricular leukomalacia, intracerebral echolucency or echodensity, and ventriculomegaly were all strongly associated with an increased risk of CP. MgSO4 administration did not affect the risk of cranial ultrasound abnormality observed at 35 weeks postmenstrual age or later. However, for the 82% of infants born at <32 weeks gestation, MgSO4 was associated with a reduction in risk of echolucency or echodensity. The reduction in risk for echolucency explained 21% of the effect of MgSO4 on CP (P = .04), and for echodensity explained 20% of the effect (P = .02). CONCLUSIONS: MgSO4 given prior to preterm delivery was associated with decreased risk of developing echodensities and echolucencies at <32 weeks gestation. However, this effect can only partially explain the effect of MgSO4 on CP at 2 years of age. TRIAL REGISTRATION: ClinicalTrials.gov: NCT00014989.

Fallopian tube torsion in the pediatric age group: radiologic evaluation.

Fallopian tube torsion is a rare but important cause of acute pelvic pain in young adolescent girls. It is a surgical emergency treated with either detorsion or salpingectomy. The imaging findings can be nonspecific and challenging. However, an accurate early diagnosis is essential for prompt surgical treatment. Our objective was to review whether imaging findings can be specific enough to suggest the diagnosis of tubal torsion prospectively in the appropriate clinical setting. An Institutional Review Board-approved retrospective review of our imaging database from 2005 to 2012 revealed 10 surgically proven cases of fallopian tube torsion. All cases had sonography performed; 5 cases had additional multidetector computed tomography. All 10 patients (9-17 years) presented with acute pelvic pain. Sonographic findings included dilated tubular structures in 6 of 10 cases: adjacent to a normal ipsilateral ovary in 5 of 6 and adjacent to a benign ovarian teratoma in 1. In 4 cases, no dilated tube was identified; 3 of 4 had a cystic mass separate from the ovaries, and 1 had the imaging appearance of a multicystic ovary. Computed tomographic findings in the 5 cases that underwent multidetector computed tomography included a dilated tubular structure in 3 of 5; 2 of 5 had a cystic adnexal mass identified. Although rare, tubal torsion should be considered in female adolescents with acute pelvic pain. Sonography should be the first imaging choice. When a tubular structure or a midline cystic mass associated with a normal ipsilateral ovary is noted, tubal torsion should be considered in the differential diagnosis.

Image Gently: progress and challenges in CT education and advocacy.

Significant progress has been made in radiation protection for children during the last 10 years. This includes increased awareness of the need for radiation protection for pediatric patients with international partnerships through the Alliance for Radiation Safety in Pediatric Imaging. This paper identifies five areas of significant progress in radiation safety for children: the growth of the Alliance; the development of an adult radiation protection campaign Image Wisely™; increased collaboration with government agencies, societies and the vendor community; the development of national guidelines in pediatric nuclear medicine, and the development of a size-based patient dose correction factor by the American Association of Physicists in Medicine, Task Group 204. However, many challenges remain. These include the need for continued education and change of practice at adult-focused hospitals where many pediatric CT exams are performed; the need for increased emphasis on appropriateness of pediatric imaging and outcomes research to validate the performance of CT studies, and the advancement of the work of the first pediatric national dose registry to determine the "state of the practice" with the final goal of establishing ranges of optimal CT technique for specific scan indications when imaging children with CT.