Supratentorial abnormalities in the Chiari II malformation, II: tectal morphologic changes.
ABSTRACT The sonographic diagnosis of fetal myelomeningocele has improved mainly because the diagnostic focus has shifted from observation of spinal manifestations to observation of cranial abnormalities. Intracranial diagnostic criteria rely on abnormalities in the posterior fossa. We describe abnormalities in tectal morphologic characteristics that, although well described on magnetic resonance imaging, have received little attention in the sonographic literature. This study analyzed the frequency of this observation and technical aspects that improve its visualization.
From a database of obstetric sonograms, we identified all cases of sonographically detected myelomeningocele. The search covered the years 1999 to 2007. We retrospectively reviewed the fetal intracranial findings with special attention to elongation or "beaking" of the tectum.
A total of 89 fetuses were identified. The mean and median gestational ages were 22 weeks 4 days and 22 weeks 2 days, respectively. Of the 89 cases, 59 (66%) had an abnormal tectal shape. The abnormality was seen in 77% of cases judged to be suboptimally visualized and 62% of cases with good visualization. Tectal abnormalities were seen equally well in fetuses before and after 24 weeks. Finally, tectal abnormalities were seen more frequently as the severity of posterior fossa findings increased.
Tectal morphologic alteration is a common supratentorial feature of the Chiari II malformation on prenatal sonography both before and after 24 weeks' gestation. Its frequency increases with the severity of posterior fossa abnormalities. Therefore, it may be useful as a supratentorial indicator of both the presence and, potentially, the severity of the Chiari II malformation.
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ABSTRACT: Ultrasound finding of the 'tulip sign' has been described as "unique sonographic picture of hypospadias". In some special conditions, if a fetus with spina bifida or a complex malformation which includes spina bifida, sits in breech presentation, this finding could indicate a neurogenic incontinence, like in our case. The autors assumed that in this case, the ventral flexion of the penis was the result of the neurogenically induced atony of the penis and the gravitational force.Gynaecologia et Perinatologia 06/2010; 19(2):97-100.
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ABSTRACT: An up-to-date, evidence-based review of the neurosurgical literature reveals that clinical outcomes following the operative management of Chiari I and II malformations have improved dramatically since these congenital disorders were first recognized as surgical diseases. A detailed assessment of major measurable postoperative parameters, including improvement in clinical signs and symptoms, resolution of syringomyelia, and progression of scoliosis, proves these procedures to be safe and effective when performed in a timely manner by an experienced neurosurgeon. Patients with CM-I routinely report a significant reduction in headache, neck pain, apnea, and syrinx-related symptoms and encounter low rates of complication or reoperation after posterior fossa decompression using a bone-only or intradural approach. Neonates and infants with CM-II have higher rates of symptomatic improvement and reversal of impairment when an operative intervention is made at the first sign of brainstem dysfunction. The current trend of less invasive bone-only surgical approaches, if shown in larger prospective trials to be superior to traditional decompressions with dural opening, will only add to the modern-day neurosurgeon’s ability to achieve excellent clinical outcomes with minimal risk in the treatment of patients with Chiari I and II malformations.The Chiari Malformations, 01/2013: pages 333-344; , ISBN: 978-1-4614-6368-9
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ABSTRACT: 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.Child s Nervous System 02/2013; 29(7). DOI:10.1007/s00381-013-2040-9 · 1.16 Impact Factor