Subdural Hemorrhage as an Early Presentation in a Case of Sotos Syndrome.

Subdural hemorrhages (SDHs) in the pediatric population are associated with a high mortality and morbidity and may present in the context of abusive head trauma. Diagnostic investigations for such cases often include evaluation for rare genetic and metabolic disorders that can have associated SDH. Sotos syndrome is an overgrowth syndrome associated with macrocephaly and increased subarachnoid spaces and rarely with neurovascular complications. Here, we report two cases of Sotos syndrome, one with SDH during infancy who underwent repeated evaluation for suspected child abuse prior to the Sotos syndrome diagnosis and the other with enlarged extra-axial cerebrospinal fluid spaces, demonstrating a possible mechanism for SDH development in this setting. These cases suggest that some individuals with Sotos syndrome may be at elevated risk of developing SDH in infancy and that Sotos syndrome should be on the differential diagnosis during a medical genetics evaluation in cases of unexplained SDH, especially in the setting of macrocephaly.

Magnetic resonance neuroimaging after laser for twin-twin transfusion syndrome with single fetal demise.

BACKGROUND: Neurologic injury in the surviving twin is a risk after single fetal demise in a monochorionic pregnancy. OBJECTIVE: This study aimed to describe fetal magnetic resonance neuroimaging findings in pregnancies complicated by single fetal demise after laser photocoagulation for twin-twin transfusion syndrome. STUDY DESIGN: This was a single-center retrospective analysis of a cohort of prospectively collected patients in a monochorionic twin registry who had fetoscopic laser photocoagulation for twin-twin transfusion syndrome with single fetal demise at follow-up. Magnetic resonance neuroimaging was offered 3 to 4 weeks after the demise to assess for potential neurologic sequelae. Magnetic resonance images were interpreted by 2 board-certified neuroradiologists and classified as normal, mildly abnormal, or severely abnormal. The groups were compared on the basis of recipient vs donor demise using the Fisher exact test and Mann-Whitney U test. Multivariate logistic regression was performed to determine risk factors for abnormal magnetic resonance neuroimaging. RESULTS: In 378 laser photocoagulation procedures, 64 cases (16.9%) of single demise were identified (36 in the donor group and 28 in the recipient group). Of note, 6 patients had rupture of membranes with nonviable delivery (3 from each group). Moreover, 40 patients (69%) underwent magnetic resonance imaging. Of those patients, 12 (30%) had abnormal findings: 10 (83%) were associated with mild changes, and 2 (17%) were associated with severe findings. Abnormal magnetic resonance neuroimaging was seen in 3 of 22 patients (14%) after donor demise and 9 of 18 patients (50%) after recipient demise (P=.02). Logistic regression revealed that recipient vs donor demise was an independent risk factor for abnormal magnetic resonance imaging. In addition, 2 pregnancies with severe magnetic resonance imaging findings had complicated courses. CONCLUSION: Mildly abnormal magnetic resonance neuroimaging findings were common after laser photocoagulation for twin-twin transfusion syndrome complicated by single fetal demise and were more common in cases of recipient demise than donor demise. Severe magnetic resonance neuroimaging findings in this series were limited to patients with complicated peri- or postoperative courses.

Improved Postoperative Metrics with Modified Myofascial Closure in Fetal Myelomeningocele Repair.

BACKGROUND: The effect of modifications in fetal myelomeningocele (fMMC) closure techniques has not been extensively studied. OBJECTIVE: To study the effect of a modified closure technique on fMMC postnatal patient outcomes: hydrocephalus, hindbrain herniation, and cyst development. METHODS: We performed single-center retrospective study of a subset of post-MOMS (Management of Myelomeningocele Study) trial patients who underwent fMMC closure. After January 2015, the fetal myofascial closure technique was modified. Needlepoint monopolar cautery was used to raise dural lined myofascial flaps to create a more robust closure. Outcomes between the pre- and postmodification groups were compared with regard to hindbrain herniation, hydrocephalus, and cyst development. Families who transitioned care to local institutions were contacted via telephone for outcome information. RESULTS: From January 2011 to May 2016, data were reviewed from 119 fMMC closure patients. Patients without full follow-up data were excluded from the final analysis. Cerebrospinal fluid diversion was seen in 32 of 74 patients with the standard technique compared to 14 of 45 with the modified closure and was significantly decreased in postmodification when compared to that of the MOMS trial (P = .01). Hindbrain herniation resolution was significantly decreased in both the pre- and postmodification groups compared to that of the MOMS trial (P < .01). Prior to January 2015 with standard closure, 23 cysts required resection whereas no cysts required resection in the modified repair group (P < .01). CONCLUSION: Modified myofascial closure for fMMC closure is safe and feasible. The new approach reflects a decreased rate of cyst development requiring surgical resection, and a trend for improved rates of hindbrain herniation and hydrocephalus.

Detailed Analysis of Hydrocephalus and Hindbrain Herniation After Prenatal and Postnatal Myelomeningocele Closure: Report From a Single Institution.

BACKGROUND: The Management of Myelomeningocele Study (MOMS) demonstrated that fetal myelomeningocele (fMMC) closure results in improved hydrocephalus and hindbrain herniation when compared to postnatal closure. OBJECTIVE: To report on the outcomes of a single institution's experience in the post-MOMS era, with regard to hydrocephalus absence and hindbrain herniation resolution. METHODS: A single-center retrospective study of a subset of post-MOMS patients who underwent fetal/postnatal myelomeningocele closure was performed. Primary outcomes included cerebrospinal fluid (CSF) diversion status and hindbrain herniation resolution. Families were contacted via telephone for outcome information if care was transitioned to outside institutions. Univariate/multivariable analyses were performed using several prenatal and postnatal variables. RESULTS: From January 2011 to May 2016, data were reviewed from families of 62 postnatal and 119 fMMC closure patients. In the postnatal group, 80.6% required CSF diversion compared to 38.7% fetal cases (P < .01). Hindbrain herniation resolution occurred in 81.5% fetal repairs compared to 32.6% postnatal (P < .01). In the fetal group, fetal/premature neonatal demise occurred in 6/119 (5.0%) patients. There was a 42.0% decrease (95% CI -55.2 to -28.8) and 48.9% increase (95% CI 33.7 to 64.1) in risk difference for CSF diversion and hindbrain herniation resolution, respectively, in the fetal group. On univariate analysis for both groups, prenatal atrial diameter, frontal-occipital horn ratio, and hindbrain herniation resolution were significantly associated with the absence of clinical hydrocephalus. The treatment of hydrocephalus was significantly delayed in the fetal group compared to the postnatal group (10 mo vs 13.8 d). CONCLUSION: This study demonstrates the benefits of fMMC closure with regard to CSF dynamics.

Use of Fetal Magnetic Resonance Image Analysis and Machine Learning to Predict the Need for Postnatal Cerebrospinal Fluid Diversion in Fetal Ventriculomegaly.

Importance: Which children with fetal ventriculomegaly, or enlargement of the cerebral ventricles in utero, will develop hydrocephalus requiring treatment after birth is unclear. Objective: To determine whether extraction of multiple imaging features from fetal magnetic resonance imaging (MRI) and integration using machine learning techniques can predict which patients require postnatal cerebrospinal fluid (CSF) diversion after birth. Design, Setting, and Patients: This retrospective case-control study used an institutional database of 253 patients with fetal ventriculomegaly from January 1, 2008, through December 31, 2014, to generate a predictive model. Data were analyzed from January 1, 2008, through December 31, 2015. All 25 patients who required postnatal CSF diversion were selected and matched by gestational age with 25 patients with fetal ventriculomegaly who did not require CSF diversion (discovery cohort). The model was applied to a sample of 24 consecutive patients with fetal ventriculomegaly who underwent evaluation at a separate institution (replication cohort) from January 1, 1998, through December 31, 2007. Data were analyzed from January 1, 1998, through December 31, 2009. Exposures: To generate the model, linear measurements, area, volume, and morphologic features were extracted from the fetal MRI, and a machine learning algorithm analyzed multiple features simultaneously to find the combination that was most predictive of the need for postnatal CSF diversion. Main Outcomes and Measures: Accuracy, sensitivity, and specificity of the model in correctly classifying patients requiring postnatal CSF diversion. Results: A total of 74 patients (41 girls [55%] and 33 boys [45%]; mean [SD] gestational age, 27.0 [5.6] months) were included from both cohorts. In the discovery cohort, median time to CSF diversion was 6 days (interquartile range [IQR], 2-51 days), and patients with fetal ventriculomegaly who did not develop symptoms were followed up for a median of 29 months (IQR, 9-46 months). The model correctly classified patients who required CSF diversion with 82% accuracy, 80% sensitivity, and 84% specificity. In the replication cohort, the model achieved 91% accuracy, 75% sensitivity, and 95% specificity. Conclusion and Relevance: Image analysis and machine learning can be applied to fetal MRI findings to predict the need for postnatal CSF diversion. The model provides prognostic information that may guide clinical management and select candidates for potential fetal surgical intervention.

Fetal ventriculomegaly: Diagnosis, treatment, and future directions.

Fetal ventriculomegaly (VM) refers to the enlargement of the cerebral ventricles in utero. It is associated with the postnatal diagnosis of hydrocephalus. VM is clinically diagnosed on ultrasound and is defined as an atrial diameter greater than 10 mm. Because of the anatomic detailed seen with advanced imaging, VM is often further characterized by fetal magnetic resonance imaging (MRI). Fetal VM is a heterogeneous condition with various etiologies and a wide range of neurodevelopmental outcomes. These outcomes are heavily dependent on the presence or absence of associated anomalies and the direct cause of the ventriculomegaly rather than on the absolute degree of VM. In this review article, we discuss diagnosis, work-up, counseling, and management strategies as they relate to fetal VM. We then describe imaging-based research efforts aimed at using prenatal data to predict postnatal outcome. Finally, we review the early experience with fetal therapy such as in utero shunting, as well as the advances in prenatal diagnosis and fetal surgery that may begin to address the limitations of previous therapeutic efforts.

Correlations of atrial diameter and frontooccipital horn ratio with ventricle size in fetal ventriculomegaly.

OBJECTIVE Fetal ventriculomegaly (FV), or enlarged cerebral ventricles in utero, is defined in fetal studies as an atrial diameter (AD) greater than 10 mm. In postnatal studies, the frontooccipital horn ratio (FOHR) is commonly used as a proxy for ventricle size (VS); however, its role in FV has not been assessed. Using image analysis techniques to quantify VS on fetal MR images, authors of the present study examined correlations between linear measures (AD and FOHR) and VS in patients with FV. METHODS The authors performed a cross-sectional study using fetal MR images to measure AD in the axial plane at the level of the atria of the lateral ventricles and to calculate FOHR as the average of the frontal and occipital horn diameters divided by the biparietal distance. Computer software was used to separately segment and measure the area of the ventricle and the ventricle plus the subarachnoid space in 2 dimensions. Segmentation was performed on axial slices 3 above and 3 below the slice used to measure AD, and measurements for each slice were combined to yield a volume, or 3D VS. The VS was expressed as the absolute number of voxels (non-normalized) and as the number of voxels divided by intracranial size (normalized). A Pearson correlation coefficient was used to measure the strength of the relationships between the linear measures and the size of segmented regions in 2 and 3 dimensions and over various gestational ages (GAs). Differences between correlations were compared using Steiger's z-test. RESULTS Fifty FV patients who had undergone fetal MRI between 2008 and 2014 were included in the study. The mean GA was 26.3 ± 5.4 weeks. The mean AD was 18.1 ± 8.3 mm, and the mean FOHR was 0.49 ± 0.11. When using absolute VS, the correlation between AD and 3D VS (r = 0.844, p < 0.0001) was significantly higher than that between FOHR and 3D VS (r = 0.668, p < 0.0001; p = 0.0004, Steiger's z-test). However, when VS was normalized, correlations were not significantly different between AD and 3D VS (r = 0.830, p < 0.0001) or FOHR and 3D VS (r = 0.842, p < 0.0001; p = 0.8, Steiger's z-test). For GAs of 24 weeks or earlier, AD correlated more strongly with normalized 3D VS (r = 0.902, p < 0.0001) than with FOHR (r = 0.674, p < 0.0001; p < 0.0001, Steiger's z-test). After 24 weeks, there was no difference in correlations between linear measures (AD or FOHR) and 3D VS (r > 0.9). Correlations of linear measures with VS in 2 and 3 dimensions were similar, and inclusion of the subarachnoid space did not significantly alter results. CONCLUSIONS Findings in the study support the use of AD as a measure of VS in fetal studies as it correlates highly with both absolute and relative VS, especially at early GAs, and captures the preferential dilation of the occipital horns in patients with FV. Compared with AD, FOHR similarly correlates with normalized VS and, after a GA of 24 weeks, can be reported in fetal studies to provide continuity with postnatal monitoring.

Comparison Between 1.5-T and 3-T MRI for Fetal Imaging: Is There an Advantage to Imaging With a Higher Field Strength?

OBJECTIVE: Fetal MRI at 3 T is emerging as a promising modality for evaluating fetal anatomy. The objective of this study was to compare the quality of images obtained with commonly used fetal imaging sequences at 1.5 T and 3 T. We hypothesized that the visualization and anatomic detail of fetal structures would be better at 3 T than at 1.5 T. MATERIALS AND METHODS: A retrospective search of the radiology department database at our institution identified 58 fetal MRI examinations performed at 3 T to evaluate body abnormalities during the period from July 2012 to February 2014. A blind comparison was conducted between these examinations and 58 1.5-T MRI examinations of age-matched fetuses undergoing evaluation for similar abnormalities during the same period. The anatomic structures analyzed included the bowel, liver, kidney, airway, cartilage, and spine. Scores for the depiction of anatomic structures ranged from 0 to 4, with 4 denoting the best depiction. RESULTS: Fetal imaging at 3 T was associated with higher imaging scores in the evaluation of the cartilage and spine when single-shot turbo spin-echo (SSTSE) and steady-state free precession (SSFP) sequences were used and in the assessment of most structures (e.g., bowel, liver, kidney, cartilage, and spine) when SSFP sequences were used. The mean scores for all structures evaluated with the use of SSTSE sequences were higher when MRI was performed at 3 T than at 1.5 T; similar findings were noted when SSFP sequences were used. Evaluation of imaging scores with regard to gestational age showed that scores improved with increasing gestational age on 1.5-T MRI but not on 3-T MRI. Overall, more imaging artifacts were found when imaging was performed at 3 T than at 1.5 T. CONCLUSION: An overall advantage to performing fetal imaging at 3 T was made evident by the higher imaging scores obtained with 3-T MRI versus 1.5-T MRI when different fetal anatomic structures were evaluated. These higher scores were predominantly associated with use of SSFP sequences. The findings of this study and future advancements in MRI software and 3-T protocols may allow optimal visualization and examination of fetal pathologic abnormalities, thus better identifying fetal and maternal needs both prenatally and postnatally.

Diagnostic features of myelomeningocele: the role of ultrafast fetal MRI.

Fetal magnetic resonance imaging (MRI) is a useful adjunct to ultrasound in the evaluation of neural tube defects. The development of ultrafast imaging techniques has contributed to the increasing clinical use of fetal MRI. This review summarizes the utility, safety and technical aspects of fetal MRI and the role it plays in the evaluation of myelomeningocele. Emphasis is placed on the benefit of fetal MRI as it pertains to presurgical planning and perinatal management.

Fetal magnetic resonance imaging: jumping from 1.5 to 3 tesla (preliminary experience).

Several attempts have been made at imaging the fetus at 3 T as part of the continuous search for increased image signal and better anatomical delineation of the developing fetus. Until very recently, imaging of the fetus at 3 T has been disappointing, with numerous artifacts impeding image analysis. Better magnets and coils and improved technology now allow imaging of the fetus at greater magnetic strength, some hurdles in the shape of imaging artifacts notwithstanding. In this paper we present the preliminary experience of evaluating the developing fetus at 3 T and discuss several artifacts encountered and techniques to decrease them, as well as safety concerns associated with scanning the fetus at higher magnetic strength.

Intraoperative neurophysiological monitoring in patients undergoing tethered cord surgery after fetal myelomeningocele repair.

OBJECT: Fetal myelomeningocele closure has been shown to be advantageous in a number of areas. In this study, the authors report on neural function in patients who had previously undergone fetal myelomeningocele repair and returned to the authors' institution for further surgery that included intraoperative neurophysiological monitoring. METHODS: The authors retrospectively reviewed data obtained in 6 cases involving patients who underwent fetal myelomeningocele repair and later returned to their institution for spinal cord untethering. (In 4 of the 6 cases, the patients also underwent removal of a dermoid cyst [3 cases] or removal of an epidermoid cyst [1 case] during the untethering procedure.) Records and imaging studies were reviewed to identify the anatomical level of the myelomeningocele as well as the functional status of each patient. Stimulated electromyography (EMG) and transcranial motor evoked potential (tcMEP) recordings obtained during surgery were reviewed to assess the functional integrity of the nerve roots and spinal cord. RESULTS: During reexploration, all patients had reproducible signals at or below their anatomical level on stimulated EMG and tcMEP recordings. Corresponding to these findings, prior to tethering, all patients had antigravity muscle function below their anatomical level. CONCLUSIONS: All 6 patients had lower-extremity function and neurophysiological monitoring recording signals at or below their anatomical level. These cases provide direct evidence of spinal cord and nerve root conductivity and functionality below the anatomical level of the myelomeningocele, further supporting that neurological status improves with fetal repair.

Prenatal diagnosis of hemimegalencephaly.

BACKGROUND: In recent literature, there have been case reports of prenatal diagnosis of hemimegalencephaly, an extremely rare entity characterized by enlargement of all or portions of 1 cerebral hemisphere and intractable seizures. A unique case is presented of hemimegalencephaly of a fetus diagnosed in utero. METHODS: A 27-year-old woman presented at 32 weeks' gestation for fetal magnetic resonance imaging after an abnormal fetal ultrasound. Fetal magnetic resonance imaging showed hemimegalencephaly of the left cerebral hemisphere with abnormal gyration. RESULTS: The patient was born via cesarean section at 39 weeks' gestation. He had continuous infantile spasms and partial-onset seizures starting on day 1 of life, and electroencephalography showed burst suppression. The patient's seizures were initially managed with antiepileptics, prednisolone, and a ketogenic diet; however, he was hospitalized multiple times because of status epilepticus. At 6 months of age, he underwent a successful anatomic left hemispherectomy. CONCLUSIONS: In utero diagnosis of complex developmental brain anomalies allows a multidisciplinary approach to provide optimal prenatal patient treatment and parental counseling.

Maturational differences in thalamocortical white matter microstructure and auditory evoked response latencies in autism spectrum disorders.

White matter diffusion anisotropy in the acoustic radiations was characterized as a function of development in autistic and typically developing children. Auditory-evoked neuromagnetic fields were also recorded from the same individuals and the latency of the left and right middle latency superior temporal gyrus auditory ~50ms response (M50)(1) was measured. Group differences in structural and functional auditory measures were examined, as were group differences in associations between white matter pathways, M50 latency, and age. Acoustic radiation white matter fractional anisotropy did not differ between groups. Individuals with autism displayed a significant M50 latency delay. Only in typically developing controls, white matter fractional anisotropy increased with age and increased white matter anisotropy was associated with earlier M50 responses. M50 latency, however, decreased with age in both groups. Present findings thus indicate that although there is loss of a relationship between white matter structure and auditory cortex function in autism spectrum disorders, and although there are delayed auditory responses in individuals with autism than compared with age-matched controls, M50 latency nevertheless decreases as a function of age in autism, parallel to the observation in typically developing controls (although with an overall latency delay). To understand auditory latency delays in autism and changes in auditory responses as a function of age in controls and autism, studies examining white matter as well as other factors that influence auditory latency, such as synaptic transmission, are of interest.

Abnormal brain development and maturation on magnetic resonance imaging in survivors of severe congenital diaphragmatic hernia.

PURPOSE: The aim of the study was to evaluate the incidence of abnormal brain maturation in survivors of severe congenital diaphragmatic hernia (CDH). MATERIAL AND METHODS: Between July 2004 and December 2009, 50 CDH survivors underwent detailed brain magnetic resonance (MR) imaging before discharge. Magnetic resonance images were analyzed to evaluate the presence of structural brain abnormalities and to calculate overall brain maturation using the total maturation score (TMS). RESULTS: Thirty-two children (64%) underwent MR imaging between 39 and 43 weeks of gestation, allowing for evaluation of the TMS. Eighteen (36%) underwent MR imaging between 44 and 69 weeks of gestation, allowing for structural analysis of brain maturity only. The mean TMS was 14.1 ± 1.2 and significantly lower than reported age-matched normative data in infants without CDH (15.3 ± 1.0, P = .02). The TMS in 4 patients (12.5%) corresponded to a delay of 1 month in structural brain development. Eight infants (25%) demonstrated a 2-week delay. Periventricular leukomalacia was detected in 9 (18%), incomplete development of the opercula in 7 (14%), various degrees of intracranial hemorrhage in 24 (48%), and prominent extraaxial fluid spaces in 20 (40%) cases. CONCLUSIONS: Brain maturation in infants with severe CDH appears to be delayed. Long-term neurodevelopmental follow-up is needed to determine the significance of a lower-than-expected TMS and the presence of structural brain abnormalities on functional outcomes in this population.

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.

MEG detection of delayed auditory evoked responses in autism spectrum disorders: towards an imaging biomarker for autism.

Motivated by auditory and speech deficits in autism spectrum disorders (ASD), the frequency dependence of superior temporal gyrus (STG) 50 msec (M50) and 100 msec (M100) neuromagnetic auditory evoked field responses in children with ASD and typically developing controls were evaluated. Whole-cortex magnetoencephalography (MEG) was obtained from 17 typically developing children and 25 children with ASD. Subjects were presented tones with frequencies of 200, 300, 500, and 1,000 Hz, and left and right STG M50 and M100 STG activity was examined. No M50 latency or amplitude Group differences were observed. In the right hemisphere, a Group x Frequency ANOVA on M100 latency produced a main effect for Group (P=0.01), with an average M100 latency delay of 11 msec in children with ASD. In addition, only in the control group was the expected association of earlier M100 latencies in older than younger children observed. Group latency differences remained significant when hierarchical regression analyses partialed out M100 variance associated with age, IQ, and language ability (all P-values <0.05). Examining the right-hemisphere 500 Hz condition (where the largest latency differences were observed), a sensitivity of 75%, a specificity of 81%, and a positive predictive value (PPV) of 86% was obtained at a threshold of 116 msec. The M100 latency delay indicates disruption of encoding simple sensory information. Given similar findings in language impaired and non-language impaired ASD subjects, a right-hemisphere M100 latency delay appears to be an electrophysiological endophenotype for autism.

Developmental correlation of diffusion anisotropy with auditory-evoked response.

White matter diffusion anisotropy in the acoustic radiations of the auditory pathway was characterized as a function of development in children and adolescents. Auditory-evoked neuromagnetic fields were also recorded from the same individuals, and the latency of the left and right superior temporal gyrus auditory response of approximately 100 ms was also obtained. White matter diffusion anisotropy increased with age. There was a commensurate shortening of the auditory-evoked response latency with increased age as well as with increased white matter diffusion anisotropy. The significant negative correlation between structural integrity of white matter pathways and electrophysiological function (response timing) of distal cortex supports a biophysical model of developmental changes in white matter myelination, conduction velocity, and cortical response timing.

Intradural inclusion cysts following in utero closure of myelomeningocele: clinical implications and follow-up findings.

OBJECT: The goal in this study was to evaluate the incidence and clinical implications of the development of cutaneously derived intradural inclusion cysts (ICs) following fetal myelomeningocele (fMMC) closure. METHODS: Retrospective databases and responses to a parental questionnaire were reviewed to determine the incidence, clinical presentation, and outcomes of fMMCs in children in whom ICs developed at follow-up. RESULTS: Prior to the National Institutes of Health (NIH)-sponsored Management of Myelomeningocele Study (MOMS), 54 patients underwent fMMC closure at the authors' institution. Sixteen (30%) presented with symptomatic tethered cord syndrome (TCS) at a median age of 27 months (range 4-93 months). Ten (63%) of the 16 (19% of the total) developed TCS in association with an intradural IC. In 9 (90%) of 10 patients, the IC was seen on preoperative MR imaging, and in 1 it was found during surgery. Four additional children (7% of the total) with evidence of an IC on surveillance MR imaging are currently asymptomatic at 94, 84, 60, and 60 months of age, respectively. All but 1 (an L-3 level lesion) IC developed in infants with L-4 and L-5 defects. After cyst removal, 6 children are asymptomatic at a median follow-up of 36 months (range 12-63 months). Following IC removal, 4 children lost normal bladder function and now require clean intermittent catheterization, and 1 lost normal leg function and now requires a walking aid for ambulation. Histologically, 8 lesions were dermoid, 1 was an epidermoid, and 1 was a mixed dermoid-epidermoid IC. Three patients developed another IC and required its removal at 24, 39, and 51 months, respectively. One required another tethered cord release within 57 months after IC removal. CONCLUSIONS: Cutaneously derived intradural ICs can develop following fMMC surgery. Deterioration of bladder function, risk of recurrence, and loss of lower-extremity function appear to be the most important long-term complications of IC in children with fMMCs. The ongoing NIH-sponsored MOMS may help determine whether children with fMMC are at increased risk of IC development compared with children treated with postnatal MMC closure. Parents seeking fMMC closure should be informed about the possibility of IC formation and the potential clinical consequences.

High prevalence of temporomandibular joint arthritis at disease onset in children with juvenile idiopathic arthritis, as detected by magnetic resonance imaging but not by ultrasound.

OBJECTIVE: To determine the prevalence of temporomandibular joint (TMJ) disease in a cohort of children with new-onset juvenile idiopathic arthritis (JIA), and to compare magnetic resonance imaging (MRI) with ultrasound (US) for the detection of acute and chronic changes of TMJ arthritis. METHODS: Between January 2005 and April 2007, children with newly diagnosed JIA were prospectively evaluated for TMJ arthritis. Prior to imaging, jaw pain and disability were assessed with questionnaires and physical examination. The TMJs of all patients were imaged with MRI and US within 8 weeks of diagnosis. RESULTS: Of the 32 patients enrolled, 78% were female, and the median age was 8.6 years (range 1.5-17.2 years). Acute TMJ arthritis was diagnosed in 75% of the children by MRI and in none by US; chronic arthritis was diagnosed in 69% by MRI and in 28% by US. Findings of both acute and chronic TMJ disease were detected by MRI in 53% of the patients. Of those with acute TMJ arthritis, 71% were asymptomatic, and 63% had normal findings on jaw examination. Fifty-six percent of patients with acute disease had an improved maximal incisal opening after corticosteroid injection. Among these responders, 56% had been asymptomatic and had normal jaw examination findings. CONCLUSION: TMJ arthritis was present in the majority of patients with new-onset JIA. Findings on MRI along with responses to treatment among asymptomatic patients with normal jaw examination findings suggest that a history review and physical examination are not sufficient to screen for TMJ disease. Our results also suggest that MRI and US findings are not well correlated, and that MRI is preferable for the detection of TMJ disease in new-onset JIA.