Intracranial volumetric evaluation in postnatally repaired myelomeningocele infants.

INTRODUCTION: Most myelomeningocele (MMC) cases present with ventriculomegaly or hydrocephalus, yet a comprehensive volumetric assessment of MMC intracranial structures is lacking. This study aimed to provide baseline data on intracranial structural volumes immediately after birth in MMC infants who underwent repair surgeries after birth (postnatal repair). METHODS: In this retrospective single-center study, we analyzed 52 MMC infants undergoing postnatal repair, utilizing head computed tomography scans at birth for volumetric assessment. Intracranial volume (ICV), lateral ventricles volume (LVV), choroid plexus volume (CPV), and posterior cranial fossa volume (PCFV) were measured. Hydrocephalus was classified into no hydrocephalus, progressive hydrocephalus, and hydrocephalus at birth. Comparative analysis employed the Wilcoxon rank-sum test. Receiver operating characteristic (ROC) analysis discriminated cases with and without ventriculoperitoneal shunt (VPS). RESULTS: The median values were 407.50 mL for ICV, 33.18 mL for LVV, 0.67 mL for CPV, and 21.35 mL for PCFV. Thirty-seven cases (71.15%) underwent VPS. ROC analysis revealed an LVV cut-off value of 6.74 mL for discriminating cases with and without VPS. Progressive hydrocephalus showed no significant difference in ICV but significantly larger LVV compared to no hydrocephalus. Hydrocephalus at birth demonstrated statistically larger ICV and LVV compared to the other two types. CONCLUSION: Baseline volumetric data were provided, and volumetric analysis exhibited statistical differences among three hydrocephalus types. These findings enhance our understanding of intracranial volumetric changes in MMC, facilitating more objective assessments of MMC cases.

Growth patterns and ratios of posterior cranial fossa structures in the Japanese pediatric population: a study utilizing CT scans.

PURPOSE: The changes in the proportion of posterior cranial fossa structures during pediatric development remain unclear. This retrospective study aimed to investigate the growth patterns and ratios of these structures using CT scans. METHODS: Head CT scans of pediatric patients with minor head trauma from Osaka Women's and Children's Hospital between March 2006 and May 2023 were analyzed. The study segmented the intracranial volume (ICV), posterior cranial fossa volume (PCFV), cerebellum volume (CBMV), and brainstem volume (BSV). Correlation coefficients were calculated among the parameters. Patients aged 0 to 10 years were divided into 15 age-related clusters, and mean and standard deviation values were measured. Growth curves were created by plotting mean values sequentially. Ratios such as PCFV/ICV and (CBMV + BSV)/PCFV were examined. Statistical analyses, including unpaired t tests and logarithmic curve fitting, were performed. RESULTS: A total of 234 CT scans (97 from females, 115 from infants under 1 year of age) were analyzed. Positive correlations were observed among the parameters, with the strongest between PCFV and CBMV. The growth curves for ICV, PCFV, CBMV, and BSV exhibited a two-phase process, with rapid growth until approximately 4 years of age, followed by stabilization. The ratios PCFV/ICV and (CBMV + BSV)/PCFV showed increasing trends from birth onwards, stabilizing by 4 and 1 years of age, respectively. CONCLUSION: This study provides insights into the growth patterns and ratios of posterior cranial fossa structures in the pediatric population. The findings demonstrate a two-phase growth process and increasing trends in the examined ratios.

Normal growth curve of choroid plexus in children: implications for assessing hydrocephalus due to choroid plexus hyperplasia.

OBJECTIVE: Pediatric hydrocephalus requires evaluation while accounting for growth of the intracranial structures, but information on choroid plexus growth in children is lacking. This study aimed to create normal growth curves for intracranial volume, choroid plexus volume, and lateral ventricles volume. Additionally, the authors aimed to objectively assess the degree of hydrocephalus caused by choroid plexus hyperplasia (CPH) and to examine the impact of surgical procedures. METHODS: This retrospective study analyzed the head CT scans of pediatric patients with minor head trauma treated at Osaka Women's and Children's Hospital between March 2006 and May 2023. The study segmented and calculated intracranial, choroid plexus, and lateral ventricles volumes. The study also calculated the correlation coefficients among these 3 parameters. Patients aged 0 to 10 years were divided into 15 age-related clusters, and mean ± SD values were calculated for each cluster. Growth curves were created by plotting mean values sequentially. Volume obtained from patients with CPH were z-normalized using mean and SD values and compared. RESULTS: A total of 229 CT scans (94 from females) were analyzed, and positive correlations were observed among intracranial volume, choroid plexus volume, and lateral ventricles volume, with the strongest correlation between the choroid plexus and lateral ventricles volumes. The growth rate of intracranial volume was rapid until approximately 20 months of age, while those of choroid plexus volume and lateral ventricles volume increased rapidly until approximately 1 year of age. Subsequently, choroid plexus volume and lateral ventricles volume plateaued at 1.5 ml and 10 ml, respectively. Three patients with CPH were enrolled and quantitatively evaluated on the basis of the z-normalized volume. Notable abnormal volumes of the choroid plexus (range z-normalized values 24.11-51.17) and lateral ventricles (46.78-122.36) were observed. In 2 patients, improvements in the z-normalized values of intracranial volume and lateral ventricles volume were observed after surgical interventions. Additionally, in 1 patient, choroid plexus volume was reduced by approximately 24% (range z-normalized values 51.17-38.93) after bilateral endoscopic plexus coagulation. CONCLUSIONS: This study provides normal growth curves for intracranial volume, choroid plexus volume, and lateral ventricles volume. Knowledge of these normal values holds the potential for objective assessment of abnormal values associated with hydrocephalus and choroid plexus diseases such as CPH.

Eight patients with Williams syndrome and craniosynostosis.

Williams syndrome (WS) is a well-known genetic syndrome caused by a microdeletion on chromosome 7q11.23 encompassing the elastin gene. It is characterized by distinctive facies, congenital cardiovascular malformations, intellectual disabilities, and various other manifestations. Some patients were reported with craniosynostosis. Here, we report 8 WS cases diagnosed with craniosynostosis using three-dimensional cranial computed tomography. These findings suggest that craniosynostosis may occur more frequently in WS patients than expected.

Guideline for management and treatment of fetal and congenital hydrocephalus: Center Of Excellence-Fetal and Congenital Hydrocephalus Top 10 Japan Guideline 2011.

INTRODUCTION: Hydrocephalus does not indicate a single clinical entity, but includes a variety of clinicopathological conditions caused by excessive cerebrospinal fluid (CSF) based on the disturbed circulation. Recent progress in prenatal neuroimagings such as MRI and ultrasound echoencephalography on fetus enables to understand clinicopathological conditions of CSF circulation disorder in conjunction with morphological changes in the central nervous system properly. It has been revealed that the CSF dynamics develop in the theory of evolution from the immature brain, as in the animals with the minor CSF pathway predominance, towards matured adult human brain together with the completion of the major CSF pathway: the "Evolution Theory in CSF Dynamics". Now, we can analyze CSF circulation dynamically and also analyze the flow velocity and direction of CSF movement. CENTER OF EXCELLENCE-FETAL HYDROCEPHALUS TOP 10 JAPAN: Along with this technical improvement, the standards of clinicopathological evaluation of hydrocephalus as well as the classification and concept of hydrocephalus shall undergo a major upgrade. Based on such remarkable improvement in the recent practical diagnostic evaluation of fetal hydrocephalus, it is now required to update the guideline for management and treatment of fetal and congenital hydrocephalus, and a nationwide study group; Center of Excellence-Fetal Hydrocephalus Top 10 Japan, was organized in 2008 in Japan. The retrospective analysis of 333 cases of congenital hydrocephalus indicated a fact that 43% of these cases were diagnosed prenatally, and the majority of cases were treated in these top 10 institutes in Japan. Now, congenital hydrocephalus diagnosed immediately after birth is regarded as to be based on embryonic stage; brain disorder in patients with congenital hydrocephalus should be considered in conjunction with neuronal mature process of embryonic stage. The fact is supported by the current trends in hydrocephalus research represented by "Perspective Classification of Congenital Hydrocephalus" and "Multi-categorical Hydrocephalus Classification". The ultimate goal of hydrocephalus treatment remains achieving arrested hydrocephalus by shunt surgeries. In the future, to achieve arrested hydrocephalus, minimum quantity of CSF to be drained should be elucidated. Consideration for accurate operative indication of ETV along with new neuroendoscopic device development and analysis of CSF circulation is expected in the future. The data in this prospective multicenter analysis in this guideline are credited in Oxford Evidence level 2b (Grade II).

Spinal lipomas in children--surgical management and long-term follow-up.

Since its introduction, MR imaging has been easy to perform on all children with lumbosacral cutaneous stigmata, and has enabled the phenomenal refinement of spinal pathology. We investigated the overall outcomes of children with spinal lipomas at the Osaka Medical Center and Research Institute for Maternal and Child Health in Osaka, Japan. Between 1991 and 2003, 76 children with a tethered cord underwent a total of 90 surgical procedures at our institutes. Of this cohort, 67 cases had spinal lipomas. The mean age of patients at first operation for asymptomatic lipoma was 22.4 months (range: 1 month to 16 years, trim mean: 10.1 months, mode: 5 months), except 4 cases. The mean total follow-up for the cohort since the first surgical procedure was 7.2 years (trim mean: 7.9 years). Since the introduction of MR imaging, the reoperation rate for symptoms or signs in our series was 16.4% (11 cases). Regarding the 13 subsequent reoperations (2 patients had 2 operations), 3 patients were reoperated on due to multiplication of the lipomas after untethering, 3 due to urologic symptoms and 5 due to orthopedic signs. There were 2 cases who also had to undergo reoperation early due to CSF leakage. Filum and conus lipomas have similar tethering pathologies, but differ in the outcome following surgery. Filum lipomas are benign, and therefore surgery is safe and effective. Conus lipomas are more difficult to manage.

Quantitative follow-up analysis by computed tomographic imaging in neonatal hydrocephalus.

We sought a simple and accurate method to monitor neonatal hydrocephalic infants using standard computed tomographic scans. Volume measurements were made by means of pixel counting using a personal computer and a drawing device, as a graphic tablet system, over computed tomographic scans of six infants with neonatal hydrocephalus and four age-matched control infants. The mean value (763.9 +/- 83.3 cm(3)) of the volume of the cranium in the hydrocephalic group was two times higher than that in the age-matched control infants (360.4 +/- 41.4 cm(3)), P < 0.00001. Sequential changes of the ventricular/intracranial volume ratio steadily decreased after cerebrospinal fluid diversion by means of a "two-step procedure" as early in postnatal life as feasible. The mean value (0.67 +/- 0.12) of the lateral ventricle/intracranial volume ratio at birth improved to 12 months of age (0.26 +/- 0.14), P < 0.05. This study has documented, by means of quantitative analysis of serial scans, a statistically significant increase in the neonatal hydrocephalic brain volume after cerebrospinal fluid shunting.

Multiplication of infantile spinal lipomas after surgical untethering. Report of two cases.

The authors reviewed 63 cases of patients with spinal lipomas who had undergone surgical untethering in childhood and found two infants in whom multiplication of the lipomas occurred within 1 year postoperatively. More importantly, the lipomas dramatically increased in size during infancy. In each case, the pathological findings on reoperation were identical to those in the first surgery. Subsequent imaging revealed no change in size of the lesions by 10 and 7 years after reoperation, respectively. This potential for growth of the lipomas should be kept in mind in infants with spinal lipomas who should undergo follow-up neuroimaging.

Tethered cord associated with anorectal malformation.

Ten children with a tethered cord and also an anorectal malformation are reported in this document. The anorectal malformations comprised 5 vesicointestinal fissures, 2 cloacal exstrophies, 2 rectovesical fistulas and 1 rectobulbar fistula. All of the patients underwent colostomy in advance of surgery for untethering of the spinal cord. Although their neurologic deficits had previously been considered static, they were subjected to radiographic examination of the caudal spine and found to have a tethered cord. These 10 children were among 55 children with a tethered cord surgically treated at the Division of Neurosurgery of the Osaka Medical Center and Research Institute for Maternal and Child Health during the last 11 years. Data were obtained for these 10 children (6 boys and 4 girls, mean age 1.7 years) who underwent surgical untethering. Several hypotheses are offered to explain this association. Anorectal malformations are related to underlying spinal cord anomalies, which may be amenable to neurosurgical correction. Eight of our patients had no skin stigma of the lumbosacral region, in contrast to an ordinary tethered cord. Spinal cord imaging is necessary to closely scrutinize these children.