External validity of the chiari severity index and outcomes among pediatric chiari I patients treated with intra- or extra-Dural decompression.

INTRODUCTION: Chiari malformation type-1 (CM-1) may be treated by intradural (ID) or extradural (ED) posterior fossa decompression, although the optimal approach is debated. The Chiari Severity Index (CSI) is a pre-operative metric to predict patient-defined improvement after CM-1 surgery. In this study, we evaluate the results of ID versus ED decompression and assess the external validity of the CSI. METHODS: We performed a retrospective cohort study of pediatric CM-1 patients undergoing decompression at a single academic children's hospital. Characteristics of headache, syrinx, and myelopathy were collected to derive CSI grade. The primary outcome measure was pre-operative symptom resolution. The proportion of patients with favorable outcome was tabulated for each of the three CSI grades and compared to previously published results. RESULTS: From 2004 to 2014, 189 patients underwent ID (48%) or ED (52%) decompression at the Children's Hospital of Philadelphia (CHOP). Follow-up ranged from 1 to 75 months. Rates of symptom resolution (58-64%) and reoperation (8%) were similar regardless of surgical approach. Although proportions of favorable outcomes differed between the CHOP and Washington University (WU) cohorts, the difference was not related to CSI grade (p = 0.63). Furthermore, there was no difference in the proportion of favorable outcomes between the two cohorts regardless of ID (p = 0.26) or ED approach (p = 0.11). CONCLUSIONS: Equivalent rates of symptom resolution and reoperation following ID and ED decompression support the ED approach as a first-line surgical option for pediatric CM-1 patients. In addition, our findings provide preliminary evidence supporting the generalizability of the CSI and its use in future comparative trials.

Prenatal surgery for myelomeningocele and the need for cerebrospinal fluid shunt placement.

OBJECT: The Management of Myelomeningocele Study (MOMS) was a multicenter randomized trial comparing the safety and efficacy of prenatal and postnatal closure of myelomeningocele. The trial was stopped early because of the demonstrated efficacy of prenatal surgery, and outcomes on 158 of 183 pregnancies were reported. Here, the authors update the 1-year outcomes for the complete trial, analyze the primary and related outcomes, and evaluate whether specific prerandomization risk factors are associated with prenatal surgery benefit. METHODS: The primary outcome was a composite of fetal loss or any of the following: infant death, CSF shunt placement, or meeting the prespecified criteria for shunt placement. Primary outcome, actual shunt placement, and shunt revision rates for prenatal versus postnatal repair were compared. The shunt criteria were reassessed to determine which were most concordant with practice, and a new composite outcome was created from the primary outcome by replacing the original criteria for CSF shunt placement with the revised criteria. The authors used logistic regression to estimate whether there were interactions between the type of surgery and known prenatal risk factors (lesion level, gestational age, degree of hindbrain herniation, and ventricle size) for shunt placement, and to determine which factors were associated with shunting among those infants who underwent prenatal surgery. RESULTS: Ninety-one women were randomized to prenatal surgery and 92 to postnatal repair. The primary outcome occurred in 73% of infants in the prenatal surgery group and in 98% in the postnatal group (p < 0.0001). Actual rates of shunt placement were only 44% and 84% in the 2 groups, respectively (p < 0.0001). The authors revised the most commonly met criterion to require overt clinical signs of increased intracranial pressure, defined as split sutures, bulging fontanelle, or sunsetting eyes, in addition to increasing head circumference or hydrocephalus. Using these modified criteria, only 3 patients in each group met criteria but did not receive a shunt. For the revised composite outcome, there was a difference between the prenatal and postnatal surgery groups: 49.5% versus 87.0% (p < 0.0001). There was also a significant reduction in the number of children who had a shunt placed and then required a revision by 1 year of age in the prenatal group (15.4% vs 40.2%, relative risk 0.38 [95% CI 0.22-0.66]). In the prenatal surgery group, 20% of those with ventricle size < 10 mm at initial screening, 45.2% with ventricle size of 10 up to 15 mm, and 79.0% with ventricle size ≥ 15 mm received a shunt, whereas in the postnatal group, 79.4%, 86.0%, and 87.5%, respectively, received a shunt (p = 0.02). Lesion level and degree of hindbrain herniation appeared to have no effect on the eventual need for shunting (p = 0.19 and p = 0.13, respectively). Similar results were obtained for the revised outcome. CONCLUSIONS: Larger ventricles at initial screening are associated with an increased need for shunting among those undergoing fetal surgery for myelomeningocele. During prenatal counseling, care should be exercised in recommending prenatal surgery when the ventricles are 15 mm or larger because prenatal surgery does not appear to improve outcome in this group. The revised criteria may be useful as guidelines for treating hydrocephalus in this group.

Myofascial Closure of Intradural Inclusion Cysts following in utero Myelomeningocele Repair.

Myelomeningocele is one of the most common congenital malformations. A randomized controlled trial, known as the Management of Myelomeningocele Study (MOMS), demonstrated that closure during the fetal period can be performed relatively safely and be of significant benefit to patients. However, postnatally, patients can develop resultant symptoms from a tethered cord and inclusion cysts; this often requires surgical treatment. Repeat surgery in this population can be challenging due to the age of the patients, the extent of surgical exposure needed and the need for resection of dermal and epidermal tissues in the midline. We describe our approach for closure of these complex defects using lateral fasciocutaneous flaps with relaxing incisions made in the posterior axillary line, in order to minimize tension and maximize soft tissue coverage of the midline.

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

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

Fetal myelomeningocele closure: technical considerations.

Myelomeningocele (MMC) is one of the most common serious congenital malformations. Typically this condition has been treated with closure of the MMC defect shortly after birth. In general, surgery for MMC aims to provide a multilayered closure to provide protection to the neural elements, prevent leakage of spinal fluid and reduce infection risks. A randomized controlled trial, the Management of Myelomeningocele Study (MOMS), has shown that closure during the fetal period can be performed relatively safely and can result in significant benefit to the child. Whereas the surgical technique of prenatal closure of an MMC defect is similar to a postnatal closure, there are some important technical differences. The goal of this paper is to describe the technique of fetal closure of MMC defects, highlight the unique steps that are needed for this surgery and delineate some potential pitfalls.

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.

Conservative management of presumed low-grade gliomas in the asymptomatic pediatric population.

OBJECTIVE: The optimal management of asymptomatic children with small, nonenhancing intracranial lesions presumed to be low-grade gliomas (LGGs) is not entirely clear in the literature. However, surgical intervention via resection or biopsy is not without risk and is of questionable long-term benefit in children with stable lesions. We present a series of 12 patients with incidentally detected, small, nonenhancing, intracranial lesions that were managed with watchful waiting and serial magnetic resonance imaging (MRI) scans. METHODS: We retrospectively reviewed a series of 12 children (eight boys, four girls) with T1 hypointense and T2 hyperintense intracranial lesions <2 cm without enhancement or surrounding edema. RESULTS: Most patients (n = 5, 41.7%) received MRI studies after suffering a traumatic injury with evidence of an abnormality seen on computed tomography scan. Others received MRI scan as part of headache work-up (n = 4, 33.3%). The majority of lesions were located infratentorially (n = 8, 66.7%), whereas other locations included the frontal lobe and thalamus. The median age of the patients upon identification of the intracranial abnormality was 10 years (range, 1-19 years of age). Patients were followed for a median of 16.7 months (range, 2.7-59.5 months). The most common diagnosis based on clinical and radiographic features of these lesions consisted of LGG. No patient underwent surgery, radiation therapy, or chemotherapy except one patient, in whom the lesion grew in size. Surgical pathologic diagnosis in this case confirmed World Health Organization grade II astrocytoma. CONCLUSIONS: Our case series suggests that conservative management and close follow-up of incidental radiographic lesions consistent with LGGs is a safe and effective initial strategy in the pediatric population. In cases in which lesion size or quality changes, surgical resection may be necessary to confirm diagnosis. Further studies that include a larger number of patients and longer follow-up period are required to compare outcomes between this approach and initial surgical, radiation, or chemotherapy management strategies.

Direct cardiac ventriculoatrial shunt: technical note.

There are a number of choices for placement of the distal catheter during ventricular shunting for hydrocephalic patients. In very rare instances, patients with multiple revisions can no longer have their shunt placed in the routine locations. We describe the placement of the distal catheter into the atrium through direct cardiac access, a technique described decades ago but rarely needed in clinical practice. This can be a useful location in the limited number of patients who have exhausted other more routine locations.

Surgical treatment of brain tumors in infants younger than six months of age and review of the literature.

OBJECTIVE: Brain tumors are rare in infants who are younger than six months of age. These tumors can be challenging to treat surgically. We analyzed a modern series of patients treated by a multidisciplinary team at a tertiary care center and performed a literature review of this unique population. METHODS: Retrospective clinical data were collected for patients surgically treated for intracranial mass lesions at The Children's Hospital of Philadelphia from 1998 to 2007. Dermoid cysts and other skull-based lesions were excluded from the analysis. RESULTS: Sixteen patients younger than six months of age underwent surgery for primary intracranial mass lesions. The median age of the patients at surgery was 5.2 months (range, 1.4-6 months of age). Children most often presented with a bulging fontanelle, hydrocephalus, or macrocephaly (seven patients). Vomiting was seen in five patients, cranial nerve palsies in one patient, and seizures in three patients. All patients had tumor resections and postoperatively were monitored in the intensive care unit. The final pathology consisted of atypical teratoid/rhabdoid tumor (three patients), primitive neuroectodermal tumor/medulloblastoma (three patients), choroid plexus papilloma (two patients), astrocytoma (two patients), ganglioglioma (two patients), desmoplastic infantile ganglioglioma (two patients), glioblastoma multiforme (one patient), and choroid plexus carcinoma (one patient). Two intraoperative deaths occurred. Of the surviving 14, a gross total resection was achieved in four. Adjuvant therapy was determined by a multidisciplinary team composed of neuro-oncology, neurosurgery, and radiation oncology. Seven patients were treated with chemotherapy, and one patient had proton beam therapy. Five-year overall survival was 45%. The eight surviving patients had neurological sequelae, and developmental outcome was variable. CONCLUSIONS: Brain tumors are uncommon in children younger than six months of age. Patients present with a variety of tumor pathologies. Children who survive have neurological sequelae. More studies are necessary to understand the impact that different treatment options, tumor pathology, and tumor location have on neurological outcome.

The role of concurrent fusion to prevent spinal deformity after intramedullary spinal cord tumor excision in children.

OBJECTIVES: Spinal deformity is a common development after laminectomy and resection of pediatric intramedullary spinal cord tumors. Our objective is to compare the occurrence of postlaminectomy spinal deformity in children with intramedullary spinal cord tumors that underwent decompression with fusion at the time of surgery to those that did not undergo fusion. METHODS: A retrospective chart review of 255 children with spinal cord tumors treated at 2 tertiary pediatric cancer centers between was performed. Of these, 52 patients with a biopsy-proven intramedullary spinal cord tumor had complete clinical records and radiographic data. Preoperative spinal alignment, surgical treatment, postoperative deformity, and risk factors for deformity were evaluated. All patients had at least 2-year follow-up. RESULTS: There were 18 females and 34 males with an average age of 8.1 ± 4.1 years. The average time to latest follow-up was 7.6 ± 5.3 years. Moderate or severe postresection spinal deformity (scoliosis > 25 degrees and/or sagittal plane abnormality > 20 degrees requiring bracing or surgery) developed in 57% (21/37) of resections without fusion (laminectomy or laminoplasty alone), and in 27% (4/15) of those with fusion (P = 0.05). Among skeletally mature children, 18 of 28 (64%) developed deformity after laminectomies and laminoplasties, compared with 22% (2/9) of the patients in the fusion group (P = 0.03). Removal of >3 lamina (P = 0.04) was associated with development of postoperative deformity. CONCLUSIONS: In the surgical treatment of patients with intramedullary spinal cord tumors, those that undergo instrumentation or in situ fusion at the time of spinal cord tumor excision are significantly less likely to develop postresection spinal deformity. LEVEL OF EVIDENCE: 3, Retrospective comparative study.

Symptomatic increased intracranial pressure due to arachnoid cysts.

Intracranial arachnoid cysts are typically benign lesions, but rarely, they may cause signs and symptoms relating to increased intracranial pressure. We report 4 pediatric patients with arachnoid cysts who received successful surgical treatment after failing to respond to conservative medical therapies. After undergoing a shunting procedure, each patient experienced improvement in symptoms, resolution of optic disc swelling, and reduction or elimination of medications necessary to manage the condition. These cases illustrate the potential for arachnoid cysts to outstrip compensatory mechanisms and cause signs and symptoms requiring definitive surgical intervention.

Morphometric analysis of posterior fossa after in utero myelomeningocele repair.

OBJECT: Myelomeningocele (MMC) is characterized by a defect in caudal neurulation and appears at birth with a constellation of neuroanatomical abnormalities, including Chiari malformation Type II. The authors investigated the effects of antenatal versus postnatal repair of MMC through a quantitative analysis of morphometric changes in the posterior fossa (PF). METHODS: The authors retrospectively reviewed the records of 29 patients who underwent in utero MMC repair, 24 patients who underwent postnatal repair, and 114 fetal and pediatric controls. Tonsillar displacement, cerebellum length, pons length, clivus-supraocciput (CSO) angle, and PF area were compared in antenatal and postnatal MMC repair groups as well as in controls without neural tube defects by using t-tests and correlation coefficients. RESULTS: Initially, the in utero CSO angle was significantly more acute in all patients with MMC--prenatally and postnatally repaired--as compared with controls (57.8° vs 75.4°, p < 0.001); however, the angle rapidly changed and became similar to that in controls between 30 and 31 weeks' gestation to approximately 80°, with antenatal repair having little effect. Postnatally, the CSO angle decreased in controls (R = -0.58) and in the antenatal repair group (R = -0.17). The cerebellum and pons length demonstrated no significant differences in any group. Overall, tonsil descent was corrected in the antenatal repair group as compared with postnatal repair (p < 0.001), and the PF area increased in all 3 groups in utero. Growth was less rapid in patients with MMC compared with controls, but this was corrected by antenatal repair (p = 0.015). CONCLUSIONS: Myelomeningocele was associated with tonsillar herniation and a smaller PF than in control fetuses. Antenatal surgical repair corrected both abnormalities. The CSO angle began significantly more acutely in patients with MMC, but normalized with development regardless of when surgery was performed. Determining the clinical effects of antenatal repair requires further follow-up.

A randomized trial of prenatal versus postnatal repair of myelomeningocele.

BACKGROUND: Prenatal repair of myelomeningocele, the most common form of spina bifida, may result in better neurologic function than repair deferred until after delivery. We compared outcomes of in utero repair with standard postnatal repair. METHODS: We randomly assigned eligible women to undergo either prenatal surgery before 26 weeks of gestation or standard postnatal repair. One primary outcome was a composite of fetal or neonatal death or the need for placement of a cerebrospinal fluid shunt by the age of 12 months. Another primary outcome at 30 months was a composite of mental development and motor function. RESULTS: The trial was stopped for efficacy of prenatal surgery after the recruitment of 183 of a planned 200 patients. This report is based on results in 158 patients whose children were evaluated at 12 months. The first primary outcome occurred in 68% of the infants in the prenatal-surgery group and in 98% of those in the postnatal-surgery group (relative risk, 0.70; 97.7% confidence interval [CI], 0.58 to 0.84; P<0.001). Actual rates of shunt placement were 40% in the prenatal-surgery group and 82% in the postnatal-surgery group (relative risk, 0.48; 97.7% CI, 0.36 to 0.64; P<0.001). Prenatal surgery also resulted in improvement in the composite score for mental development and motor function at 30 months (P=0.007) and in improvement in several secondary outcomes, including hindbrain herniation by 12 months and ambulation by 30 months. However, prenatal surgery was associated with an increased risk of preterm delivery and uterine dehiscence at delivery. CONCLUSIONS: Prenatal surgery for myelomeningocele reduced the need for shunting and improved motor outcomes at 30 months but was associated with maternal and fetal risks. (Funded by the National Institutes of Health; ClinicalTrials.gov number, NCT00060606.).

Energy expenditure in obesity associated with craniopharyngioma.

BACKGROUND AND PURPOSE: Obesity is a common yet incompletely understood complication of childhood craniopharyngioma. We hypothesized that craniopharyngioma is associated with specific defects in energy balance compared to obese control children. METHODS: Eleven craniopharyngioma patients were recruited for a study on body composition and energy balance. Eight subjects were obese. The obese craniopharyngioma patients had a mean age (+/-SD) of 11.2 +/- 1.7 years. The average body mass index z score was 2.33 (+/-0.32). A previously studied group of obese children (BMI z score 2.46 +/- 0.46) served as controls. Resting energy expenditure (REE) was determined by indirect calorimetry and body composition by dual energy X-ray absorptiometry in all children. RESULTS: Obese craniopharyngioma patient subjects had increased mean (+/-standard error) fat-free mass compared to obese controls (57% +/- 0.88 % vs 50.0% +/- 0.87%, p = 0.02). The obese craniopharyngioma patients had a 17% lower REE compared to values expected from the World Health Organization equation (1,541 +/- 112.6 vs 1,809 +/- 151.8 kcal; p = 0.01). In contrast, the obese control children had measured REE within 1% of predicted (1,647 +/- 33.2 vs. 1,652 +/- 40.2; p = 0.8). In a linear regression model, REE remained significantly lower than predicted after controlling for FFM. CONCLUSIONS: Lower REE may be a factor contributing to obesity in children with craniopharyngioma. Further study is needed into the mechanisms for reduced energy expenditure in patients with craniopharyngioma.

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.

Duplication of 7q34 in pediatric low-grade astrocytomas detected by high-density single-nucleotide polymorphism-based genotype arrays results in a novel BRAF fusion gene.

In the present study, DNA from 28 pediatric low-grade astrocytomas was analyzed using Illumina HumanHap550K single-nucleotide polymorphism oligonucleotide arrays. A novel duplication in chromosome band 7q34 was identified in 17 of 22 juvenile pilocytic astrocytomas and three of six fibrillary astrocytomas. The 7q34 duplication spans 2.6 Mb of genomic sequence and contains approximately 20 genes, including two candidate tumor genes, HIPK2 and BRAF. There were no abnormalities in HIPK2, and analysis of two mutation hot-spots in BRAF revealed a V600E mutation in only one tumor without the duplication. Fluorescence in situ hybridization confirmed the 7q34 copy number change and was suggestive of a tandem duplication. Reverse transcription polymerase chain reaction-based sequencing revealed a fusion product between KIAA1549 and BRAF. The predicted fusion product includes the BRAF kinase domain and lacks the auto-inhibitory N-terminus. Western blot analysis revealed phosphorylated mitogen-activated protein kinase (MAPK) protein in tumors with the duplication, consistent with BRAF-induced activation of the pathway. Further studies are required to determine the role of this fusion gene in downstream MAPK signaling and its role in development of pediatric low-grade astrocytomas.

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.

Endovascular and surgical treatment of ruptured cerebral aneurysms in pediatric patients.

OBJECTIVE: Pediatric cerebral aneurysms are rare. There are very few recent studies that focus on the multidisciplinary treatment of ruptured aneurysms. We reviewed our pediatric endovascular and surgical experience with ruptured cerebral aneurysms. METHODS: Pediatric patients aged 16 years and younger who were admitted with a diagnosis of aneurysmal subarachnoid hemorrhage and treated at the Children's Hospital of Philadelphia were included in this analysis. RESULTS: Twelve patients with 13 aneurysms (4 male patients and 8 female patients; age range, 4 months-16 years; mean age, 5.1 years), were admitted with subarachnoid hemorrhage during the past 12 years. The majority of patients were admitted in good clinical condition; 31% were in Hunt and Hess Grade II, and 31% were in Hunt and Hess Grade III. The remaining patients were in poor clinical condition and were in Hunt and Hess Grade IV (23%) or Grade V (15%). Computed tomography revealed that 15% of the patients were in Fisher Grade 2, 23% were in Fisher Grade 3, and 62% were in Fisher Grade 4. Endovascular techniques were used in the treatment of 5 aneurysms, and microsurgery was used in the treatment of 8 aneurysms. In the endovascular group, aneurysm sizes ranged from 2 to 35 mm (mean, 12.6 mm); 3 aneurysms were in the anterior circulation, and 2 were in the posterior circulation. In the microsurgery group, 6 aneurysms were in the anterior circulation, and 2 were in the posterior circulation; sizes ranged from 3 to 15 mm (mean, 6.8 mm). Sixty-nine percent of the patients were independent at follow-up. CONCLUSION: Contemporary endovascular and microsurgical techniques can be used effectively to treat ruptured cerebral aneurysms in pediatric patients. In the time period studied, the techniques were equally effective when used in the appropriate patients.

The changing epidemiologic spectrum of single-suture synostoses.

PURPOSE: Classic literature indicates an infrequency of metopic craniosynostosis (3 to 10 percent) compared to other single-suture craniosynostosis. Recent observation challenges these conceptions, warranting long-term demographic analysis. METHODS: Syndromic craniofacial dysostoses and multiple suture involvement were exclusion criteria, leaving only single suture synostoses treated between 1975 and 2004. A chart review was performed and patient information recorded. Chi square analysis and Fisher's exact were used to determine differences in patient characteristics. A Moran's I statistic was used to determine differences in spatial means and whether changes in incidence of single-suture synostoses over time are a function of regional phenomena. RESULTS: Over 800 patients presented to The Children's Hospital of Philadelphia with a diagnosis of single-suture synostosis. There was an outpacing of sagittal suture involvement over other sutures. Data demonstrate a decrease in unicoronal synostosis and an increase in metopic synostosis (p = 0.011). Geostatistical analysis reveals increasing separation between populations over the study period: 9.8 miles in the first 5 years to 20.8 miles in the last 5 years. Metopic maternal age increased between 1975-1989 and 1990-2004 (p = 0.002, 0.0002), while unicoronal maternal age did not. The proportion of male patients increased considerably in the metopic group compared to unicoronals from 1990-2004 (p = 0.0001), as did the proportion of syndromic metopic patients (p = 0.02). Plausible etiologies for these epidemiological shifts are discussed. CONCLUSIONS: Metopic synostosis is on the rise. Changing demographic bases and increasing proportions of syndromic patients may be clues to the etiology of this epidemiologic event.