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OBJECTIVE The goal of this study was to assess the social determinants that influence access and outcomes for pediatric neurosurgical care for patients with Chiari malformation type I (CM-I) and syringomyelia (SM). METHODS The authors used retro- and prospective components of the Park-Reeves Syringomyelia Research Consortium database to identify pediatric patients with CM-I and SM who received surgical treatment and had at least 1 year of follow-up data. Race, ethnicity, and insurance status were used as comparators for preoperative, treatment, and postoperative characteristics and outcomes. RESULTS A total of 637 patients met inclusion criteria, and race or ethnicity data were available for 603 (94.7%) patients. A total of 463 (76.8%) were non-Hispanic White (NHW) and 140 (23.2%) were non-White. The non-White patients were older at diagnosis (p = 0.002) and were more likely to have an individualized education plan (p < 0.01). More non-White than NHW patients presented with cerebellar and cranial nerve deficits (i.e., gait ataxia [p = 0.028], nystagmus [p = 0.002], dysconjugate gaze [p = 0.03], hearing loss [p = 0.003], gait instability [p = 0.003], tremor [p = 0.021], or dysmetria [p < 0.001]). Non-White patients had higher rates of skull malformation (p = 0.004), platybasia (p = 0.002), and basilar invagination (p = 0.036). Non-White patients were more likely to be treated at low-volume centers than at high-volume centers (38.7% vs 15.2%; p < 0.01). Non-White patients were older at the time of surgery (p = 0.001) and had longer operative times (p < 0.001), higher estimated blood loss (p < 0.001), and a longer hospital stay (p = 0.04). There were no major group differences in terms of treatments performed or complications. The majority of subjects used private insurance (440, 71.5%), whereas 175 (28.5%) were using Medicaid or self-pay. Private insurance was used in 42.2% of non-White patients compared to 79.8% of NHW patients (p < 0.01). There were no major differences in presentation, treatment, or outcome between insurance groups. In multivariate modeling, non-White patients were more likely to present at an older age after controlling for sex and insurance status (p < 0.01). Non-White and male patients had a longer duration of symptoms before reaching diagnosis (p = 0.033 and 0.004, respectively). CONCLUSIONS Socioeconomic and demographic factors appear to influence the presentation and management of patients with CM-I and SM. Race is associated with age and timing of diagnosis as well as operating room time, estimated blood loss, and length of hospital stay. This exploration of socioeconomic and demographic barriers to care will be useful in understanding how to improve access to pediatric neurosurgical care for patients with CM-I and SM.

Fetal MRI and neonatal MRI of the central nervous system (CNS) are complementary tools that can help to accurately counsel and direct the management of children with anomalies of the central nervous system. Postnatal MRI can add to fetal MRI by allowing for monitoring of changes in the severity of disease, better delineation of a suspected prenatal anomaly, evaluation for secondary pathologies related to the primary diagnosis, and surgical management direction. In this review we discuss the roles of fetal and neonatal MRI in the diagnosis and treatment of congenital anomalies of the CNS through a series of case examples and how both are important in patient management.

BACKGROUND: Occipital-cervical fusion (OCF) and ventral decompression (VD) may be used in the treatment of pediatric Chiari-1 malformation (CM-1) with syringomyelia (SM) as adjuncts to posterior fossa decompression (PFD) for complex craniovertebral junction pathology. OBJECTIVE: To examine factors influencing the use of OCF and OCF/VD in a multicenter cohort of pediatric CM-1 and SM subjects treated with PFD. METHODS: The Park-Reeves Syringomyelia Research Consortium registry was used to examine 637 subjects with cerebellar tonsillar ectopia ≥ 5 mm, syrinx diameter ≥ 3 mm, and at least 1 yr of follow-up after their index PFD. Comparisons were made between subjects who received PFD alone and those with PFD + OCF or PFD + OCF/VD. RESULTS: All 637 patients underwent PFD, 505 (79.2%) with and 132 (20.8%) without duraplasty. A total of 12 subjects went on to have OCF at some point in their management (PFD + OCF), whereas 4 had OCF and VD (PFD + OCF/VD). Of those with complete data, a history of platybasia (3/10, P = .011), Klippel-Feil (2/10, P = .015), and basilar invagination (3/12, P

Proteus syndrome is a mosaic genetic overgrowth disorder caused by a postzygotic, mosaic activating mutation in AKT1. Rare prenatal presentations include segmental tissue overgrowth, and skeletal and CNS anomalies. We present the first report of prenatally diagnosed and molecularly confirmed Proteus syndrome. Prenatal imaging identified megalencephaly, brain and eye malformations, focal soft tissue enlargement, and ambiguous genitalia. Exome sequencing performed on cultured amniocytes demonstrated an AKT1 pathogenic variant consistent with Proteus syndrome, and postnatal examination confirmed the diagnosis. Postnatal Sanger sequencing could not identify the AKT1 pathogenic variant. This case underscores the importance of prenatal exome sequencing on cultured amniocytes for mosaic overgrowth disorders, as well as provides additional information on the prenatal phenotype of Proteus syndrome, and highlights the impact of prenatal diagnosis on postnatal management.

Background Ehlers-Danlos syndrome (EDS) is a group of rare congenital disorders of connective tissue that result in tissue fragility and joint hyperextensibility. Owing to its rarity, outcomes of pediatric spine surgery in patients with EDS are poorly characterized. Although it has been suggested that complication rates are high, few studies have characterized these complications. Methods Pediatric National Surgery Quality Improvement Program data from 2012–2016 were analyzed. Patients with EDS undergoing spine surgery were identified along with patients without EDS undergoing the same surgeries using International Classification of Diseases, Ninth Revision and Current Procedural Terminology codes. Results Of 369,176 total patients, 279 were determined to have EDS. Of these, 56 patients underwent spine surgery; 46% were male and 54% were female (P = 0.108). Mean age at surgery was 11.59 years (P = 0.888) with a range of 1.77–17.33 years. The most common procedure was arthrodesis (n = 37). There were no differences in unplanned reoperations (n = 4, P = 0.119), wound infections or disruptions (n = 2, P = 0.670), or overall complications (n = 25, P = 0.751). Blood transfusions were required in 41% of patients with EDS, but this was not significant compared with patients without EDS undergoing the same procedures (n = 23, P = 0.580). The total amount of blood transfused (P = 0.508), length of hospital stay (P = 0.396), and total operative time (P = 0.357) were not different from control subjects. Conclusions Pediatric patients with EDS do not appear to be at a higher risk of bleeding or other complications during spine surgery as reported in past case series. This is the largest retrospective review of its kind that has been performed in this patient population.

Purpose The purpose of the study is to examine MRI findings of the brain and spine on prenatal and postnatal MRI following intrauterine repair of open spinal dysraphism (OSD) by open hysterotomy and fetoscopic approaches. Materials and methods This study is a single-center HIPAA-compliant and IRB-approved retrospective analysis of fetal MRIs with open spinal dysraphism from January 2011 through December 2018 that underwent subsequent prenatal repair of OSD. Results Sixty-two patients met inclusion criteria: 47 underwent open repair, and 15 underwent fetoscopic repair, with an average gestational age of 22.6 ± 1.4 weeks at initial MRI. On postnatal MRI, spinal cord syrinx was seen in 34% (16/47) of patients undergoing open versus 33.3% (5/15) undergoing fetoscopic repair (P = 0.96). Postnatally, there was no significant difference in hindbrain herniation between the open versus fetoscopic repair groups (P = 0.28). Lateral ventricular size was significantly larger in the open (20.9 ± 6.7 mm) versus the fetoscopic repair (16.1 ± 4.9 mm) group (P = 0.01). Conclusion Though lateral ventricular size in the open repair group was larger than the fetoscopic repair group, this can likely be explained by initial selection criteria used for fetoscopic repair. Other postoperative imaging parameters on postnatal MRI were not significantly different between the two groups.

OBJECTIVE Scoliosis is common in patients with Chiari malformation type I (CM-I)–associated syringomyelia. While it is known that treatment with posterior fossa decompression (PFD) may reduce the progression of scoliosis, it is unknown if decompression with duraplasty is superior to extradural decompression. METHODS A large multicenter retrospective and prospective registry of 1257 pediatric patients with CM-I (tonsils ≥ 5 mm below the foramen magnum) and syrinx (≥ 3 mm in axial width) was reviewed for patients with scoliosis who underwent PFD with or without duraplasty. RESULTS In total, 422 patients who underwent PFD had a clinical diagnosis of scoliosis. Of these patients, 346 underwent duraplasty, 51 received extradural decompression alone, and 25 were excluded because no data were available on the type of PFD. The mean clinical follow-up was 2.6 years. Overall, there was no difference in subsequent occurrence of fusion or proportion of patients with curve progression between those with and those without a duraplasty. However, after controlling for age, sex, preoperative curve magnitude, syrinx length, syrinx width, and holocord syrinx, extradural decompression was associated with curve progression > 10°, but not increased occurrence of fusion. Older age at PFD and larger preoperative curve magnitude were independently associated with subsequent occurrence of fusion. Greater syrinx reduction after PFD of either type was associated with decreased occurrence of fusion. CONCLUSIONS In patients with CM-I, syrinx, and scoliosis undergoing PFD, there was no difference in subsequent occurrence of surgical correction of scoliosis between those receiving a duraplasty and those with an extradural decompression. However, after controlling for preoperative factors including age, syrinx characteristics, and curve magnitude, patients treated with duraplasty were less likely to have curve progression than patients treated with extradural decompression. Further study is needed to evaluate the role of duraplasty in curve stabilization after PFD.

Mild traumatic brain injury (mTBI) comprises the majority of pediatric traumatic brain injury. Children with mTBI even with traumatic intracranial hemorrhage (tICH) rarely experience a clinically significant neurologic decline (CSND). The utility of routine surveillance imaging in the pediatric population also remains controversial, especially owing to concerns about the risks of radiation exposure at a young age. This study aims to identify demographic or injury-related characteristics that may facilitate recognition of children at risk of progression with mTBI.We performed a retrospective review of patients16 years old with mTBI (GCS 13-15) and tICH admitted to a Level I pediatric trauma center between 2009 and 2014. Management of these patients was directed by the Cincinnati Children's Hospital Medical Center Minor Head Injury Algorithm. We reviewed each chart with emphasis on patient demographics, injury specific data, and radiographic or clinical progression.154 patients met inclusion criteria with mean age of 4 [0-16]; 116 sustained an tICH and 38 patients had isolated skull fractures. Repeat neuroimaging was obtained in 68 patients (59%). Only 9 patients (13%) with tICH had radiographic progression, none of which resulted in CSND. In addition, 9 patients experienced CSND, leading to neurosurgical intervention in 6 patients. Notably, none of these patients had repeat imaging prior to their neurologic changes. Both CSND and need for intervention were significantly higher in patients with epidural hematomas than other types of tICH (19.2% vs. 1.1%, p = 0.002). Of 154 patients, 19 did not have documented follow-up, 135 were seen as outpatients and 65 (48%) had follow up neuroimaging. All patients who had surveillance imaging in the outpatient setting had stable or resolved tICH.Few children with mTBI and tICH experience clinical decline. Importantly, all patients that required neurosurgical intervention were identified by clinical changes rather than via repeat imaging. Our study suggests that in the vast majority of cases, clinical monitoring alone is safe and sufficient in patients in order to avoid exposure to repeat radiographic imaging.Level III, prognostic and epidemiological.

OBJECTIVES: Increased intracranial pressure (ICP) has been suggested in legal settings as an alternative cause of retinal hemorrhages (RHs) in young children who may have sustained abusive head trauma. We assessed the prevalence and characteristics of RHs in children with increased ICP. METHODS: We conducted a prospective, multicenter study of children RESULTS: Fifty-six children (27 boys) were studied (mean age 15.4 months; range 1–43 months). All of the children had elevated ICP that required intervention. One child had papilledema. No child (0%; 95% confidence interval: 0%–6.4%) or eye (0%; 95% confidence interval: 0%–3.3%) was found to have an RH. Causes of increased ICP included hydrocephalus, intraventricular hemorrhage, congenital malformations, malfunctioning shunts, and the presence of intracranial space-occupying lesions. CONCLUSIONS: Although acute increased ICP can present in children with a pattern of peripapillary superficial RHs in the presence of papilledema, our study supports the conclusion that RHs rarely occur in the absence of optic disc swelling and do not present beyond the peripapillary area in the entities we have studied.

The purpose of this study was to examine differences between patients with myelomeningocele and those with myelocele with respect to brain imaging findings at fetal MRI.A single-center retrospective analysis was performed of fetal MRI examinations revealing open spinal dysraphism from 2004 through 2016 with available diagnostic postnatal spinal MR images in conjunction with neurosurgical follow-up findings. Images were reviewed by two board-certified fellowship-trained pediatric neuroradiologists. Relevant clinical data were recorded.The study included 119 fetal MRI examinations of patients with open spinal dysraphism. Myeloceles were found in 29.4% (35/119) of these examinations and myelomeningoceles in the others. All (35/35) myeloceles showed grade 3 (severe) Chiari II malformations. Only 73.8% (62/84) of myelomeningoceles showed grade 3 Chiari II malformation. Clinically significant spinal kyphosis was found in 5.0% (6/119) of fetuses, and all of these fetuses had grade 3 Chiari II malformations. The size of the spinal dysraphic defect had significant positive correlation with lateral (p0.0001) and third (p = 0.006) ventricular size. Mean volume of the myelomeningocele sac was significantly different among Chiari II grades and inversely proportional to Chiari II grade (p = 0.0009).Larger spinal dysraphic defects correlated with increased ventricular size at fetal MRI. All of the fetuses with myelocele or kyphosis had severe Chiari II malformations. Larger myelomeningocele sac size was associated with lower grade of Chiari II malformation, suggesting that myelomeningocele sac formation may be protective against hindbrain herniation.

BACKGROUND AND PURPOSE: Fetal MRI has become a valuable tool in the evaluation of open spinal dysraphisms making studies comparing prenatal and postnatal MRI findings increasingly important. Our aim was to determine the accuracy of predicting the level of the spinal dysraphic defect of open spinal dysraphisms on fetal MR imaging and to report additional findings observed when comparing fetal and postnatal MR imaging of the spine in this population. MATERIALS AND METHODS: A single-center retrospective analysis was performed of fetal MRIs with open spinal dysraphisms from 2004 through 2016 with available diagnostic postnatal spine MR imaging. Images were reviewed by 2 board-certified fellowship-trained pediatric neuroradiologists. Corresponding clinical/operative reports were reviewed. RESULTS: One hundred nineteen fetal MRIs of open spinal dysraphisms were included. The level of the osseous defect between fetal and postnatal MR imaging was concordant in 42.9% (51/119) of cases and was 1 level different in 39% (47/119) of cases. On postnatal MR imaging, type II split cord malformation was seen in 8.4% (10/119) of cases, with only 50% (5/10) of these cases identified prospectively on fetal MR imaging. Syrinx was noted in 3% (4/119) of prenatal studies, all cervical, all confirmed on postnatal MR imaging. CONCLUSIONS: Fetal MR imaging is accurate in detecting the level of the spinal dysraphic defect, which has an impact on prenatal counseling, neurologic outcomes, and eligibility for fetal surgery. In addition, fetal MR imaging is limited in its ability to detect split cord malformations in patients with open spinal dysraphisms. Although rare, fetal MR imaging has a high specificity for detection of cervical spinal cord syrinx.

BACKGROUND AND PURPOSE: Subependymal grey matter heterotopias are seen in a high proportion of children with Chiari II malformation and are potentially clinically relevant. However, despite its growing use, there is little in the literature describing its detection on fetal MRI. Our aim was to evaluate the accuracy in diagnosing subependymal gray matter heterotopias in fetuses with spinal dysraphism on fetal MR imaging. MATERIALS AND METHODS: This study is a retrospective analysis of 203 fetal MRIs performed at a single institution for spinal dysraphism during a 10-year period. Corresponding obstetric sonography, postnatal imaging, and clinical/operative reports were reviewed. RESULTS: Of the fetal MRIs reviewed, 95 fetuses were included in our analysis; 23.2% (22/95) were suspected of having subependymal gray matter heterotopias on fetal MR imaging prospectively. However, only 50% (11/22) of these cases were confirmed on postnatal brain MR imaging. On postnatal brain MR imaging, 28.4% (27/95) demonstrated imaging findings consistent with subependymal gray matter heterotopia. Only 40.7% (11/27) of these cases were prospectively diagnosed on fetal MR imaging. CONCLUSIONS: Fetal MR imaging is limited in its ability to identify subependymal gray matter heterotopias in fetuses with spinal dysraphism. It is believed that this limitation relates to a combination of factors, including artifacts from fetal motion, the very small size of fetal neuroanatomy, differences in imaging techniques, and, possibly, irregularity related to denudation of the ependyma/subependyma in the presence of spinal dysraphism and/or stretching of the germinal matrix in ventriculomegaly.

OBJECTIVEMethylprednisolone sodium succinate (MPSS) has been studied as a pharmacological adjunct that may be given to patients with acute spinal cord injury (ASCI) to improve neurological recovery. MPSS treatment became the standard of care in adults despite a lack of evidence supporting clinical benefit. More recently, new guidelines from neurological surgeon groups recommended no longer using MPSS for ASCI, due to questionable clinical benefit and known complications. However, little information exists in the pediatric population regarding MPSS use in the setting of ASCI. The aim of this paper was to describe steroid use and side effects in patients with ASCI at the authors’ Level 1 pediatric trauma center in order to inform other hospitals that may still use this therapy.METHODSA retrospective chart review was conducted to determine adherence in ordering and delivery according to the guideline of the authors’ institution and to determine types and frequency of complications. Inclusion criteria included age < 17 years, blunt trauma, physician concern for ASCI, and admission for ≥ 24 hours or treatment with high-dose intravenous MPSS. Exclusion criteria included penetrating trauma, no documentation of ASCI, and incomplete medical records. Charts were reviewed for a predetermined list of complications.RESULTSA total of 602 patient charts were reviewed; 354 patients were included in the study. MPSS was administered in 59 cases. In 34 (57.5%) the order was placed correctly. In 13 (38.2%) of these 34 cases, MPSS was administered according to the recommended timeline protocol. Overall, only 13 (22%) of 59 patients received the therapy according to protocol with regard to accurate ordering and administration.Among the patients with ASCI, 20 (55.6%) of the 36 who received steroids had complications, which was a significantly higher rate than in those who did not receive steroids (8 [24.2%] of 33, p = 0.008). Among the patients without ASCI, 10 (43.5%) of the 23 who received steroids also experienced significantly more complications than patients who did not receive steroids (50 [19.1%] of 262, p = 0.006).CONCLUSIONSHigh-dose MPSS for ASCI was not delivered to pediatric patients according to protocol with a high degree of reliability. Patients receiving steroids for pediatric ASCI were significantly more likely to experience complications than patients not receiving steroids. The findings presented, including complications of steroid use, support removal of high-dose MPSS as a treatment option for pediatric ASCI.

Congenital anomalies of the spinal cord can pose a diagnostic dilemma to the radiologist. Several classification systems of these anomalies exist. Antenatal ultrasound and fetal magnetic resonance imaging is playing an increasingly important role in the early diagnosis and management of patients. Understanding the underlying anatomy as well as embryology of these disorders can be valuable in correctly identifying the type of spinal cord dysraphic defect. Hereditary spinal cord diseases are rare but can be devastating. When the onset is in adulthood, delay in diagnosis is common. Although the spine findings are nonspecific, some imaging features combined with brain imaging findings can be distinctive. Sometimes, the radiologist may be the first to raise the possibility of these disorders.

Object Syringomyelia can be diagnosed in isolation but is more commonly found in the presence of craniocervical junction anomalies or spinal dysraphism. The origin of syringomyelia has been hypothesized to be either congenital or acquired. The purpose of this study was to determine the incidence of syringomyelia within the fetal and postnatal population with neural tube defects (NTDs). Methods A review was performed of the authors' fetal MRI database of pregnancies with imaging between March 2004 and November 2011 for evaluation of an intrauterine anomaly detected via prenatal ultrasonography. Those cases with an NTD were then selected and a chart review was performed of all prenatal and postnatal imaging as well as available clinical history. Results A total of 2362 fetal MRI examinations were performed, and 109 of these were patients with an NTD. Of the 2362 studies reviewed, 2 cases of fetal syringomyelia were identified. Both fetal syrinxes were identified in fetuses with CSF flow disturbances (1 case each of encephalocele and myelomeningocele). Both fetal MRI examinations were performed late in gestation, at 31 and 38 weeks, respectively. The patient with an encephalocele was excluded from the spinal NTD population; therefore a syrinx was identified in 0.08% (2/2362) of the entire population of fetuses who underwent MRI, or 0.9% (1/109) of fetuses with a spinal NTD. Sixty-three of the 109 patients with an NTD had postnatal clinical data available for review. Twenty-nine (46%) of 63 had a syrinx identified during the follow-up period. Of this group, 50 patients had an open NTD and 27 (54%) of 50 developed a syrinx. Among the patients with an open NTD who developed a syrinx, only 7% did not have or develop hydrocephalus, compared with 35% of the patients who did not develop a syrinx (p < 0.05). There were nonsignificantly more frequent shunt revisions among those patients who developed a syrinx, and a syrinx developed in all patients who required surgical Chiari malformation decompression or tethered cord release. The initial identification of a spinal cord syrinx varied greatly between patients, ranging from 38 weeks gestation to greater than 4 years of age. Conclusions These data suggest that syringomyelia is not a congenital embryonic condition. A syrinx was not identified in fetuses who underwent imaging for other intrauterine anomalies. In the population of patients with NTDs who are known to be at high risk for developing syringomyelia, the pathology was only identified in 2 third-trimester fetuses or postnatally, typically in the presence of hydrocephalus, shunt placement, Chiari malformation decompression, or tethered cord release. The study supports the authors' hypothesis that a syrinx is an acquired lesion, most likely due to the effects of abnormal CSF flow.

Spina bifida is the most common nonchromosomal birth defect, resulting in permanent disability of multiple organ systems, yet compatible with long-term survival. Important advances across various disciplines have now improved survival among the spina bifida population. Although the majority of individuals living with spina bifida are now adults, there are few publications in the neurosurgical literature regarding the care of adults with spina bifida, associated medical conditions, surgical interventions, and long-term complications. The major goals for transitioning adult patients with spina bifida are preservation of function and promotion of independence as well as general overall health. Nevertheless, many gaps exist in our knowledge and understanding of the complex needs of this aging patient population. The goal of this paper was to provide a comprehensive updated review of the literature regarding the challenges and considerations involved in the transitional care to adulthood for patients with spina bifida. Unique to this review, the authors provide a first-hand personal communication and interview with an adult patient with spina bifida that discusses many of these challenges with transition.

The purpose of this study is to identify differences in findings between open and closed spinal dysraphisms seen on fetal MR images.A single-institution retrospective analysis of fetal MR images for spinal dysraphism was performed. Postnatal images and clinical and operative reports were reviewed.Sixteen fetuses with postnatally confirmed closed spinal dysraphisms were included. Of these, 25% (4/16) had posterior fossa anomalies, 12.5% (2/16) had ventriculomegaly, and 37.5% (6/16) had OEIS (omphalocele, exstrophy, imperforate anus, and spinal defects) complex. Of 90 fetuses with postnatally confirmed open spinal dysraphism, 95.6% (86/90) had posterior fossa anomalies, 85.6% (77/90) had ventriculomegaly, and none had OEIS complex. Twenty fetuses with open spinal dysraphism were randomly selected to compare with fetuses with closed spinal dysraphisms. Continuity of the epidermal and subcutaneous tissues with the sac wall on fetal MR images was seen in 93.8% (15/16) of patients with closed spinal dysraphisms, as opposed to 5% (1/20) of patients with open spinal dysraphisms. The mean (± SD) sac wall thickness was less in open (0.7 ± 0.6 mm) than closed (2.9 ± 1.3 mm; p0.001) spinal dysraphism. None of the fetuses had T1-hyperintense fat within the defect.On fetal MR images, closed spinal dysraphisms tend to have a sac wall in continuity with the epidermal and subcutaneous tissues, a thicker sac wall, fewer posterior fossa anomalies, and high association with OEIS complex.

Objective Recent studies have suggested that retethering in patients operated for a tight or fatty filum is higher than previously predicted. In this retrospective review, outcome, complications, and risk of reoperation for recurrent tethered cord syndrome (TCS) at our own institution were investigated. Methods The medical records of 100 consecutive children who underwent initial division of the filum terminale at Cincinnati Children's Hospital Medical Center (November 1995–May 2006) for a tight or fatty filum were reviewed. One patient was excluded due to previous spinal surgery at an outside institution. Presenting symptoms/signs, magnetic resonance imaging findings, complications, postoperative symptoms/signs, and need for reoperation were recorded. Mean follow-up for 97 of the 99 patients was 33 months; 80 were followed for 6 months or more and 68 were followed for 12 months or more. Results The most common presenting symptoms were bladder and/or bowel dysfunction, followed by gait abnormality, back pain, and spasticity. At last follow-up, 85 patients were improved or stable, whereas 12 patients had at least one symptom or sign that had worsened. Five children required a second operation for recurrent TCS. Mean time to reoperation was 58 months (range 22–73 months). Arachnoid adhesions accounted for the retethering in four of five patients. There were a total of 12 complications in 9 patients including 5 wound infections, 4 cerebrospinal fluid leaks, 1 pseudomeningocele, 1 stitch abscess, and 1 transient headache. Conclusions Division of a tight or fatty filum, in this consecutive series of pediatric patients, resulted in improved or stable neurological symptoms in 88% of patients. However, the complication and reoperation rate for recurrent TCS were not insignificant. Future studies aimed at reducing complications and retethering in this population may be warranted.

Object The goal in this study was to compare the integrity of white matter before and after ventriculoperitoneal (VP) shunt insertion by evaluating the anisotropic diffusion properties with the aid of diffusion tensor (DT) imaging in young children with hydrocephalus. Methods The authors retrospectively identified 10 children with hydrocephalus who underwent both pre- and postoperative DT imaging studies. The DT imaging parameters (fractional anisotropy [FA], mean diffusivity, axial diffusivity, and radial diffusivity) were computed and compared longitudinally in the splenium and genu of the corpus callosum (gCC) and in the anterior and posterior limbs of the internal capsule (PLIC). The patients' values on DT imaging at the pre- and postshunt stages were compared with the corresponding age-matched controls as well as with a large cohort of healthy children in the database. Results In the gCC, 7 of 10 children had abnormally low preoperative FA values, 6 of which normalized postoperatively. All 3 of the 10 children who had normal preoperative FA values had normal FA values postoperatively as well. In the PLIC, 7 of 10 children had abnormally high FA values, 6 of which normalized postoperatively, whereas the other one had abnormally low postoperative FA. Of the remaining 3 children, 2 had abnormally low preoperative FA values in the PLIC; this normalized in 1 patient after surgery. The other child had a normal preoperative FA value that became abnormally low postoperatively. When comparing the presurgery frequency of abnormally low, normal, and abnormally high FA values to those postsurgery, there was a statistically significant longitudinal difference in both gCC (p = 0.02) and PLIC (p = 0.002). Conclusions In this first longitudinal DT imaging study of young children with hydrocephalus, DT imaging anisotropy yielded abnormal results in several white matter regions of the brain, and trended toward normalization following VP shunt placement.

BACKGROUND AND PURPOSE: Diffusion tensor imaging (DTI) can noninvasively detect in vivo white matter (WM) abnormalities on the basis of anisotropic diffusion properties. We analyzed DTI data retrospectively to quantify the abnormalities in different WM regions in children with hydrocephalus during early infancy. MATERIALS AND METHODS: Seventeen infants diagnosed with hydrocephalus (age range, 0.13–16.14 months) were evaluated with DTI and compared with 17 closely age-matched healthy children (age range, 0.20–16.11 months). Fractional anisotropy (FA), mean diffusivity (MD), axial diffusivity, and radial diffusivity values in 5 regions of interest (ROIs) in the corpus callosum and internal capsule were measured and compared. The correlation between FA and age was also studied and compared by ROI between the 2 study groups. RESULTS: Infants with hydrocephalus had significantly lower FA, higher MD, and higher radial diffusivity values for all 3 ROIs in the corpus callosum, but not for the 2 ROIs in the internal capsule. In infants with hydrocephalus, the increase of FA with age during normal development was absent in the corpus callosum but was still preserved in the internal capsule. There was also a significant difference in the frequency of occurrence of abnormal FA values in the corpus callosum and internal capsule. CONCLUSIONS: This retrospective DTI study demonstrated significant WM abnormalities in infants with hydrocephalus in both the corpus callosum and internal capsule. The results also showed evidence that the impact of hydrocephalus on WM was different in the corpus callosum and internal capsule.

Suspected skull fractures in the neonate are uncommon and present a management dilemma. We hypothesized that skull fractures are more common than reported in the literature and that few infants require any intervention. We retrospectively reviewed the charts of 21 infants referred to our level IV Neonatal Intensive Care Unit for possible skull fracture over a 3-year period after birth trauma, suspicious findings on clinical exam, or accidental falls in the birth hospital. Skull films at the birth hospital were unreliable for fracture in 23% of cases. Seven of nine infants with accidental falls had fracture on computed tomography scan. Only three infants required neurosurgical intervention, all after severe birth trauma associated with instrumentation. Skull fractures are more common than previously reported in neonates after accidental falls, but few infants with skull fractures require neurosurgical intervention.

Depressed skull fractures overlying the major venous sinus are often managed nonoperatively because of the high associated risks of surgery in these locations. In the presence of clinical and radiographic evidence of sinus occlusion, however, surgical therapy may be necessary. The authors present the case of a 9-year-old boy with a depressed skull fracture overlying the posterior third of the superior sagittal sinus. After initial conservative treatment, delayed signs of intracranial hypertension and a symptomatic tonsillar herniation with tonsillar necrosis developed. Possible causes as well as diagnostic and treatment options are reviewed.

Introduction: Laparoscopy is being used for assistance in ventriculoperitoneal (VP) shunt placement. We have found it especially useful to guide distal catheter placement in children who have had multiple VP shunt revisions and previous abdominal surgery. We therefore reviewed our experience utilizing laparoscopy for VP shunt placement over the past year. Methods: We conducted a retrospective review of all children undergoing laparoscopy for VP shunt who have had previous abdominal surgery. Results: Nine children underwent 11 laparoscopic procedures for VP shunt placement. All children had undergone multiple abdominal procedures, and all had previously undergone open VP shunt placement. The mean age of all children undergoing laparoscopy was 11.4 years. Shunt infection (in 6 children) and shunt malfunction (in 5 children) were the reasons for VP shunt revision. A single 3-mm port was used in 9 of 11 procedures. All children had adhesions visualized, 2 required adhesiolysis, and in one VP shunt was not pos...

A retrospective analysis of patients with lipomyelomeningocele cared for at two referral centers was completed to derive relationships between neurological function and patient age. Thirty-seven percent of 177 patients had intact neurological function on initial examination. Neurological deficits were progressive and linked with a logarithmic relationship to increasing patient age. Each child with intact examination retained normal bladder function following lipomyelomeningocele repair and release of cord tethering; complications of surgery were limited. Our analysis suggests that surgery on patients with intact function offers greater long-term protection of critical function than is offered by conservative management and expectant care. We recommend repair of lipomyelomeningocele at the time of diagnosis regardless of patient age or neurological function.

Tethered cord syndrome (TCS) is defined by abnormal traction on the spinal cord that confines its movement. Surgical cord release usually stops neurological deterioration; therefore, early and accurate neuroradiological diagnosis is important. Supine MRI is the imaging modality of choice, but prone MRI and cine MRI can demonstrate cord movement.We compared the diagnostic accuracies of standard MRI, prone MRI and cine MRI in patients with clinical suspicion of TCS and evaluated inter-reader reliability for MR imaging.Children who underwent MRI for suspicion of TCS were retrospectively identified. Supine, prone and cine MRI studies were re-read by two pediatric neuroradiologists. Conus level, filum appearance and cord movement were documented.Thirteen of 49 children had tethered cord documented at surgery. Conus level had the highest diagnostic accuracy (sensitivity 69-77%, specificity 94%, positive predictive value 82-83%, negative predictive value 89-92%, correct diagnosis 88-90%) and highest between-reader concordance (98%). Prone and cine MRI did not add to the accuracy of the supine imaging.Conus level provides the highest diagnostic accuracy and inter-reader reliability in TCS. Until a larger series is evaluated, it remains questionable whether prone or cine MRI provides enough additional diagnostic information to warrant routine use.

Chiari type I malformation is characterized by herniation of the cerebellar tonsils through the foramen magnum. An association between Chiari type I malformation and cystic fibrosis (CF) has not previously been established. We report on five children and adolescents with CF in whom Chiari type I malformations were diagnosed. Three patients were 17-18 years old at time of diagnosis, one was 3 years old, and one was 10 months of age. All patients were followed at the Cystic Fibrosis Center at St. Christopher's Hospital for Children and were diagnosed with the malformations between June 1988 and June 1997. Over this same period, 400 CF patients 18 years or younger were followed routinely. All patients had the diagnosis of Chiari type I confirmed by brain-stem MRI. Neurologic findings included swallowing dysfunction, syncopal episodes, numbness of extremities, recurrent vomiting, and headaches. No two patients had the same presenting neurologic findings. Our data suggest that Chiari type I malformation is more common in CF than in the general population. The possibility of Chiari type I malformation should be included in the differential diagnosis of unexplained neurologic complaints in patients with CF.

Address: 1Paediatric Neuroimaging Research Consortium, Dept. of Radiology, Cincinnati Children's Hospital Medical Centre, 3333 Burnet Ave., Cincinnati, Ohio, 45229, USA, 2Division of Pediatric Neurosurgery, Cincinnati Children's Hospital Medical Centre, 3333 Burnet Ave., Cincinnati, Ohio, 45229, USA and 3Dept. of Radiology, Cincinnati Children's Hospital Medical Centre, 3333 Burnet Ave., Cincinnati, Ohio, 45229, USA * Corresponding author

Much controversy still exists about the relative advantages and disadvantages of anteriorly vs. posteriorly placed shunts in terms of infection rate and duration of function. During a 27-month period, all 121 patients seen requiring new shunt insertions were prospectively randomized to anterior or posterior placement. The mean duration of follow-up was 15 months. 70% of the shunts in the posteriorly placed group vs. 59% of the shunts in the anteriorly placed group did not require further surgery during the study period. In a life-table analysis of shunt survival as a function of placement, the shunts in the posteriorly placed group 'survived' slightly longer without malfunctioning or becoming infected than the anteriorly placed shunts. Therefore, the authors conclude that anteriorly placed shunts offer no advantage over posteriorly placed shunts in terms of shunt malfunction or infection.

BACKGROUND Chordomas are tumors of notochordal origin that account for approximately 1%-4% of all primary malignant bone tumors. The majority of patients with chordomas have a poor surgical prognosis due to extent of disease at diagnosis. These lesions have been previously classified based solely on their location. METHODS We describe here a case report of a posterior epidural C5-T1 chordoma that was discovered in a young patient who presented with weakness and paresthesia in all four extremities. This lesion was notable for its extraosseous and extradural characteristics. RESULTS C5-T1 laminectomy with gross total resection of the mass led to complete resolution of all symptoms. There has been no evidence of tumor recurrence to date. CONCLUSIONS We propose here a new classification system for chordomas that emphasizes the difference in resectability of these lesions depending on the space they occupy and the presence or absence of an osseous connection.

Background. Plastic hairbeads are often worn as decorative hair fasteners by children. Serious, penetrating head injuries may result from their use and have been observed in some children following a fall. Objective. The objective of this report is to describe the imaging findings in children who have sustained head injury while wearing plastic hairbeads. Materials and methods. Three children with significant head injuries resulting from embedded hairbeads are described. Three additional cases of minor head injury reported to the Consumer Product Safety Commission are summariz- ed. Results. One child sustained a minimally depressed skull fracture without brain injury. The second child required surgical repair of a depressed skull fracture complicated by a parenchymal hemorrhage and dural tear. A third child required surgical evacuation of an organized, liquefied epidural hematoma 2 weeks after an initial evaluation at an outside emergency room. Conclusion. Children wearing plastic hairbeads are at risk for severe head injury following a fall. Caution must accompany their use.

Cyst formation in brain tumors is not uncommon. Associated pathological features in brain tumors are readily disclosed by advanced neuroimaging studies such as computed tomography (CT) and magnetic resonance imaging (MRI). Direct visualizations of these pathological conditions were not possible by angiography or pneumoencephalography, which provided only indirect images shown by displaced cerebral blood vessels or cerebrospinal fluid (CSF) spaces. Predicting the histological nature of the intracranial mass may be possible when it is accompanied by a cyst. Intravenous contrast agents for CT or MRI provide further information as to whether or not the cyst wall consists of neoplastic cells. Although the term cystic tumor is commonly used in clinical practice, it would be more appropriate to classify these lesions into two groups: one, mural tumor with cyst, and another, cyst within tumor. The former does not contain neoplastic cells in the cyst wall, whereas the cyst wall of the latter is composed of neoplastic cells. On neuroimaging, contrast-enhancing cyst walls have neoplastic cells; therefore, this observation is important in planning resection of these tumors (Figures 15.1 and 15.2).