Hydrocephalus in myelomeningocele.

PURPOSE: To investigate certain aspects of hydrocephalus in patients with myelomeningocele. METHODS: We retrospectively analyzed data of 1050 patients with myelomeningocele who underwent surgical treatment between June 1991 and June 2021. These patients were divided into three groups: group 1 consisted of patients who underwent surgery after the first 6 h of life, group 2 consisted of patients who underwent surgery within the first 6 h, and group 3 consisted of patients who underwent surgery during the fetal period and before 26 6/7 weeks of gestation. RESULTS: There were 125, 590, and 335 patients in groups 1, 2, and 3, respectively. In groups 1 and 2, 593 (83%) patients developed hydrocephalus after birth and required ventriculoperitoneal shunt placement in the maternity ward, mainly within the first 4 days of life. In contrast, in group 3, 24 (7.2%) patients required surgery to treat hydrocephalus after birth. Hydrocephalus was the primary cause of mortality in groups 1 and 2, with mortality rates of 35% and 10%, respectively. In group 3, the mortality rate was 0.8% and was not related to hydrocephalus. CONCLUSION: The onset of hydrocephalus is directly related to myelomeningocele closure in neurosurgery.

Luckenschadel Associated with Chiari Type II Malformation: An Autopsy Case Report.

Background Luckenschadel is commonly associated with Chiari II malformation and myelomeningocele. The aim of this case report was to revisit this entity. Case report: Antenatal imaging performed at 32 weeks gestation showed severe hydrocephalus with brain parenchymal thinning, cerebellar hypoplasia and lumbar myelomeningocele, suggestive of Chiari type II malformation. Stillbirth occurred before the pregnancy termination. X-rays showed a characteristic honeycomb appearance of the skull. The female 34-week-old stillborn was severely macerated and presented with thin and fenestrated skull, ruptured lumber myelomeningocele and diastematomyelia. Hydrocephalus could not be confirmed because of cerebral maceration. Histological examination did not reveal any significant visceral alteration. Conclusion: This case report highlights two key points. Luckenschadel should be kept in mind when Chiari II malformation is diagnosed. Autopsy with radiographic assessment is very useful in revealing this congenital defect which may escape prenatal detection.

Surgical management of symptomatic Chiari II malformation in infants and children.

PURPOSE: Variation exists in the surgical methods employed for decompression of Chiari II malformation (CIIM), yet an evaluation of these techniques has not been performed. The purpose of this study was to assess the efficacy of bony decompression (cervical laminectomy alone versus suboccipital craniectomy with laminectomy) with or without dural augmentation for the treatment of symptomatic CIIM. METHODS: Clinical records of children 0-18 years of age who underwent surgical repair of myelomeningocele or CIIM decompression at St. Louis Children's Hospital (SLCH) from 1990-2011 were reviewed. Signs/symptoms prompting decompression, surgical technique, operative parameters, and clinical outcomes were recorded for analysis. RESULTS: Thirty-three subjects were treated at SLCH for CIIM decompression. Twenty-six subjects underwent bony decompression only (21 cervical laminectomy alone, 5 suboccipital craniectomy + cervical laminectomy) while seven underwent bony decompression with upfront dural augmentation (three cervical laminectomy alone, four suboccipital craniectomy + cervical laminectomy). Median follow up was 5.0 years (range, 3 months-19 years). Symptomatic improvement was noted in 20/33 subjects (60.6%). Sixty-two (61.5%) percent of children who underwent bony decompression had symptomatic improvement, compared with 57.1% of those with upfront dural augmentation (p = 0.37). Estimated blood loss, operative time, and length of perioperative hospital stay appeared lower in the bony decompression group but were not statistically different in this limited cohort. CONCLUSIONS: The results from this series suggest that bony CIIM decompression via tailored cervical laminectomies alone, without suboccipital craniectomy or upfront dural augmentation, is a reasonable initial management approach for decompression of symptomatic CIIM.

Non-Targeted Metabolic Profiling of Cerebellum in Spina Bifida Fetal Rats.

Spina bifida, known more commonly as myelomeningocele, is a neural tube defect that results in herniation of the cerebellum through the foramen magnum into the central canal as part of the Chiari II malformation. Effects stemming from the herniated cerebellum and its metabolic profile have not been extensively studied. The objective of this study is to examine the metabolic effects of this disease on the cerebellum in utero through the utilization of a retinoid acid-induced Spina bifida rat model. Analysis of this model at mid-late (day 15) and term (day 20) of gestation in comparison to both non-exposed and retinoic acid-exposed non-myelomeningocele controls, the observed metabolic changes suggest that mechanisms of oxidative stress and energy depletion are at play in this neuro tissue. These notable mechanisms are likely to result in further damage to neural tissue as the fetus grows and the compressed cerebellum develops and herniates more due to myelomeningocele.

Frontal and occipital horn ratio is associated with multifocal intraparenchymal hemorrhages in neonatal shunted hydrocephalus.

OBJECTIVE Placement of a cerebrospinal fluid diversion device (i.e., shunt) is a routine pediatric neurosurgical procedure, often performed in the first weeks of life for treatment of congenital hydrocephalus. In the postoperative period, shunt placement may be complicated by subdural, catheter tract, parenchymal, and intraventricular hemorrhages. The authors observed a subset of infants and neonates who developed multifocal intraparenchymal hemorrhages (MIPH) following shunt placement and sought to determine any predisposing perioperative variables. METHODS A retrospective review of the electronic medical record at a tertiary-care children's hospital was performed for the period 1998-2015. Inclusion criteria consisted of shunt placement, age < 30 days, and available pre- and postoperative brain imaging. The following data were collected and analyzed for each case: ventricular size ratios, laboratory values, clinical presentation, shunt and valve type, and operative timing and approach. RESULTS A total of 121 neonates met the inclusion criteria for the study, and 11 patients (9.1%) had MIPH following shunt placement. The preoperative frontal and occipital horn ratio (FOR) was significantly higher in the patients with MIPH than in those without (0.65 vs 0.57, p < 0.001). The change in FOR (∆FOR) after shunt placement was significantly greater in the MIPH group (0.14 vs 0.08, p = 0.04). Among neonates who developed MIPH, aqueductal stenosis was the most common etiology (45%). The type of shunt valve was associated with incidence of MIPH (p < 0.001). Preoperative clinical parameters, including head circumference, bulging fontanelle, and coagulopathy, were not significantly associated with development of MIPH. CONCLUSIONS MIPH represents an underrecognized complication of neonatal shunted hydrocephalus. Markers of severity of ventriculomegaly (FOR) and ventricular response to CSF diversion (∆FOR) were significantly associated with occurrence of MIPH. Choice of shunt and etiology of hydrocephalus were also significantly associated with MIPH. After adjusting for corrected age, etiology of hydrocephalus, and shunt setting, the authors found that ∆FOR after shunting was still associated with MIPH. A prospective study of MIPH prevention strategies and assessment of possible implications for patient outcomes is needed.