Karin S. Bierbrauer

Reflections on the Natural History of Lipomyelomeningocele

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.

Anisotropic Diffusion Properties in Infants with Hydrocephalus: A Diffusion Tensor Imaging Study

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.

Outcome, reoperation, and complications in 99 consecutive children operated for tight or fatty filum.

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 Childrens 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.

Chordoma: A case report

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.

Chiari type I malformation in children and adolescents with cystic fibrosis.

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.

Head injuries in children from plastic hairbeads

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.

Comparison of standard, prone and cine MRI in the evaluation of tethered cord

BackgroundTethered 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.ObjectiveWe 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.Materials and methodsChildren 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.ResultsThirteen 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.ConclusionConus 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.

Hindbrain Herniation in Chiari II Malformation on Fetal and Postnatal MRI

The authors examined the neuroimaging findings with a focus on hindbrain herniation and ventricular size in fetuses with open spinal dysraphism and compared them with postnatal imaging features in groups undergoing prenatal-versus-postnatal repair. Thirty-two of 102 (31.3%) fetuses underwent in utero repair of open spinal dysraphism; 68.6% (70/102) underwent postnatal repair. Of those who underwent prenatal repair 81.3% (26/32) had resolved cerebellar ectopia postnatally. Of those who had severe cerebellar ectopia (grade 3) that underwent postnatal repair, 65.5% (36/55) remained grade 3, while 34.5% (19/55) improved to grade 2. They conclude that most fetuses who undergo in utero repair have resolved cerebellar ectopia postnatally. BACKGROUND AND PURPOSE: As the practice of in utero repair of myelomeningoceles becomes more prevalent, knowledge of the expected MR imaging findings has become increasingly important. Our aim was to examine neuroimaging findings with a focus on hindbrain herniation and ventricular size in fetuses with open spinal dysraphism and to compare them with postnatal imaging features in groups undergoing prenatal-versus-postnatal repair. MATERIALS AND METHODS: Single-center retrospective analysis was performed on MRIs of fetuses with open spinal dysraphism from January 2004 through July 2015 with available postnatal imaging. One hundred two fetuses were included. Reports from available fetal ultrasound were also examined. Images were reviewed by 2 board-certified fellowship-trained pediatric neuroradiologists. Descriptive analyses were performed to demonstrate the distribution of the imaging findings. RESULTS: Thirty-two of 102 (31.3%) fetuses underwent in utero repair of open spinal dysraphism; 68.6% (70/102) underwent postnatal repair. Ninety-four of 102 (92.2%) fetuses had cerebellar ectopia. Of those who underwent prenatal repair (26 grade 3, 6 grade 2), 81.3% (26/32) had resolved cerebellar ectopia postnatally. Of those who had severe cerebellar ectopia (grade 3) that underwent postnatal repair, 65.5% (36/55) remained grade 3, while the remaining 34.5% (19/55) improved to grade 2. The degree of postnatal lateral ventriculomegaly in those that underwent prenatal repair (20.3 ± 5.6 mm) was not significantly different from that in those that underwent postnatal repair (21.5 ± 10.2 mm, P = .53). Increased Chiari grade was significantly correlated with decreased head size for gestational age on fetal sonography (P = .0054). CONCLUSIONS: In fetuses with open spinal dysraphism and severe Chiari II malformation that do not undergo prenatal repair, most have no change in the severity of cerebellar ectopia/Chiari grade. However, in fetuses that undergo in utero repair, most have resolved cerebellar ectopia postnatally.

Abnormal fractional anisotropy in hydrocephalic children: a DTI study

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

Identification and management of neonatal skull fractures

Objective: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.Study Design: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.Results: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.Conclusion:Skull fractures are more common than previously reported in neonates after accidental falls, but few infants with skull fractures require neurosurgical intervention.