Rapidly progressive cerebral atrophy following a posterior cranial fossa stroke: Assessment with semiautomatic CT volumetry.

BACKGROUND: The effect of posterior cranial fossa stroke on changes in cerebral volume is not known. We assessed cerebral volume changes in patients with acute posterior fossa stroke using CT scans, and looked for risk factors for cerebral atrophy. METHODS: Patients with cerebellar or brainstem hemorrhage/infarction admitted to the ICU, and who underwent at least two subsequent inpatient head CT scans during hospitalization were included (n = 60). The cerebral volume was estimated using an automatic segmentation method. Patients with cerebral volume reduction > 0% from the first to the last scan were defined as the "cerebral atrophy group (n = 47)," and those with ≤ 0% were defined as the "no cerebral atrophy group (n = 13)." RESULTS: The cerebral atrophy group showed a significant decrease in cerebral volume (first CT scan: 0.974 ± 0.109 L vs. last CT scan: 0.927 ± 0.104 L, P < 0.001). The mean percentage change in cerebral volume between CT scans in the cerebral atrophy group was -4.7%, equivalent to a cerebral volume of 46.8 cm3, over a median of 17 days. The proportions of cases with a history of hypertension, diabetes mellitus, and median time on mechanical ventilation were significantly higher in the cerebral atrophy group than in the no cerebral atrophy group. CONCLUSIONS: Many ICU patients with posterior cranial fossa stroke showed signs of cerebral atrophy. Those with rapidly progressive cerebral atrophy were more likely to have a history of hypertension or diabetes mellitus and required prolonged ventilation.

Application of the Scale for the Assessment and Rating of Ataxia (SARA) in pediatric oncology patients: A multicenter study.

Posterior cranial fossa (PCF) tumors in childhood are often associated with ataxia as well as other motor, neurobehavioral and linguistic impairment. The use of a reliable outcome measure is mandatory to evaluate the severity of impairment and monitor rehabilitation effectiveness. The aim of this work is to explore the validity of the Scale for the Assessment and Rating of Ataxia (SARA) in pediatric subjects with ataxia secondary to PCF tumor resection and evaluate the influence of age and comorbidities. Seventy eight patients (3-18 years) were recruited in 5 centers from 2016 to 2018. The age effect on SARA was analyzed by correlating total SARA scores and item scores with age and gradually excluding youngest subjects. The comorbidity effect was evaluated by comparing the ataxia-only group vs a group of subjects with ataxia + dysfunction of cranial nerves or cerebellar mutism (CM) and a group of patients with ataxia + hemiparesis. Several negative correlations between SARA scores and age were found under age 9. Differences between ataxia-only group and the other two groups were closely associated with specific comorbidities (e.g. speech disturbance in cranial nerves or CM group (p value < 0.001) and gait, stance, sitting and finger chase in the hemiparetic group (mean p value 0.022)).

A prospective study on fetal posterior cranial fossa assessment for early detection of open spina bifida at 11-13 weeks.

The objective of this study was to test three measurements: brain stem (BS), intracranial translucency (IT) and brain stem to occipital bone distance (BSOB), as well as one landmark: cisterna magna (CM) visibility, for early diagnosis of open spina bifida (OSB) in a low risk population. A prospective observational study was undertaken in a university hospital. A sample of 1479 women consented to participate between 20 September 2013 and 30 June 2015. Measurements were performed from the mid-sagittal view, as is routinely used for nuchal thickness assessment. CM visibility was assessed qualitatively as the third anechoic band in the posterior cranial fossa (PCF). All pregnancies were screened with a combination of maternal serum alpha-fetoprotein and second trimester anomaly scan and followed until delivery. Predictive values were calculated for each marker. We were able to diagnose two OSB cases and highly suspect one Dandy-Walker malformation case at the first trimester scan by the observation of PCF. PCF characteristics of OSB cases were increased BS diameter, increased BS-BSOB ratio and non-visualization of the CM. All the markers demonstrated high sensitivity and specificity but CM visibility reached the highest positive predictive value. Due to relatively high false positive rates, PCF measurements could not reach a satisfactory performance to validate their clinical use as a single marker. CM visibility has the advantage of being a qualitative marker and reduces the need for sophisticated and time-consuming measurements. Intracranial translucency and BS-BSOB ratio measurements should be used when the CM visibility is absent or in doubt.

National trends, complications, and hospital charges in pediatric patients with Chiari malformation type I treated with posterior fossa decompression with and without duraplasty.

BACKGROUND: The treatment of type 1 Chiari malformation (CM-1) with posterior fossa decompression without (PFD) or with duraplasty (PFDD) is controversial. The authors analyze both options in a national sample of pediatric patients. METHODS: Utilizing the Kids' Inpatient Database, CM-1 patients undergoing PFD or PFDD from 2000 through 2009 were analyzed. RESULTS: 1,593 patients with PFD and 1,056 with PFDD were evaluated. The average age was 10.3 years, slightly younger in PFD (9.8 vs. 10.9 years, p = 0.001). PFDD patients were more likely White (81.2 vs 75.6%, p = 0.04) and less likely admitted emergently (8.4 vs. 13.8%, p = 0.007). They also underwent more reoperations (2.1 vs. 0.7%, p = 0.01), had more procedure-related complications (2.3 vs. 0.8%, p = 0.003), a longer length of stay (4.4 vs. 3.8 days, p = 0.001) and higher charges (USD 35,321 vs. 31,483, p = 0.01). CONCLUSIONS: This large national study indicates that PFDD is performed more often in Caucasians, less so emergently, and associated with significantly more complications and immediate reoperations, while PFD is more frequent in those with syringomyelia and more economical, requiring fewer hospital resources. Overall, PFD is more favorable for CM-1, though it would be prudent to conduct a prospective trial, as this analysis is limited by data on preoperative presentations and long-term outcomes.

Influence of head positioning on the assessment of Chiari-like malformation in Cavalier King Charles spaniels.

Chiari-like malformation (CM) is almost omnipresent in the Cavalier King Charles spaniels (CKCS), often leading to syringomyelia (SM). Morphometric studies have produced variable results concerning relationship between the brain parenchyma within the caudal cranial fossa (CCF) and SM. The present study assesses the effect of head position, one potential confounder. Magnetic resonance images of CKCS with CM were reviewed in extended and flexed head positions. Volumes were calculated from transverse T2-weighted brain images. Mid-sagittal images were used for measurement of cerebellar herniation and CSF space between cerebellum and brainstem. Fourteen CKCS were included into the study, seven dogs with CM and seven with CM/SM. There was no difference between the relative brain parenchyma within the CCF in extended position and flexed position, or the brain parenchyma within the rostral and middle cranial fossae proportion. Cerebellar herniation and CSF space between cerebellum and brainstem were significantly increased in the flexed position. Cerebellar herniation and CSF space differed significantly between CM and CM/SM in a flexed head position. Volumetric measurements did not vary with head position. Cerebellar herniation and CSF space between the cerebellum and the brainstem were larger in a flexed head position.

Posterior fossa volume increase after surgery for Chiari malformation Type I: a quantitative assessment using magnetic resonance imaging and correlations with the treatment response.

OBJECT: The aim of this paper was to measure the posterior fossa (PF) volume increase resulting from a given-sized occipital craniectomy in Chiari malformation Type I surgery and to analyze its correlations with the PF size and the treatment response, with the perspective of tailoring the amount of bone removal to the patient-specific PF dimensions. METHODS: Between January 2005 and June 2006, 11 adult patients with symptomatic Chiari malformation Type I underwent a standardized PF decompression. A prospective evaluation with clinical examination, functional grading, and MR imaging measurement protocols was performed pre- and postoperatively. A method is reported for the measurement of PF volume (PFV) after surgery. The degree of PFV increase was compared with the preoperative size of the PF and with the clinical outcome. RESULTS: All 11 patients improved postoperatively, with complete and partial recovery in 4 and 7 patients, respectively. No postoperative complication occurred after a mean follow-up period of 45 months. The mean relative increase in PFV accounted for 10% (range 1.5%-19.7%) of the initial PFV; the increase was greater in cases in which the PF was small (r = -0.52, p = 0.09) and the basiocciput was short (r = -0.37, p = 0.2). A statistically significant positive correlation was found between the degree of PFV increase and the treatment response (p = 0.014); complete recovery was observed with a PFV increase of 15% and partial recovery with an increase of 7%. CONCLUSIONS: The treatment response is significantly influenced by the degree of PFV increase, which is dependent on the size of the PF and the extent of the craniectomy, suggesting that the optimal patient-specific PFV increase could be predicted on the basis of preoperative MR imaging and enhancing the perspective that the craniectomy size could be tailored to the individual PFV.

Assessment of cognitive function before and after surgery for posterior cranial fossa lesions using computerized and conventional tests.

Cognitive function has not been well studied after neurosurgery for posterior fossa lesions despite its potential importance in determining surgical indications and approaches. The present study evaluated changes in cognitive functions after posterior fossa surgery to detect any differences between the middle cranial fossa and lateral suboccipital approaches in 50 patients with posterior fossa lesions such as tumors and vascular diseases. Twenty-five patients underwent surgery via the middle fossa and 25 via the lateral suboccipital approaches. Computerized test battery (CogState) and conventional neuropsychological tests (serial seven-word learning test and mini-mental state examination) were examined before, 1 month after, and 3 months after surgery. All scores of the neuropsychological tests remained within normal limits after surgery. However, the scores of one computerized test battery and serial seven-word learning tests decreased significantly 1 month after surgery and recovered within 3 months, indicating temporary deterioration of short-term memory in the middle fossa group. The computerized tests detected significantly larger numbers of patients with worsened results than the conventional tests. The middle fossa approach and operation time showed correlations with the postoperative neuropsychological declines. The computerized tests could be performed easily and were beneficial for detecting subtle changes of the cognitive function after surgery. Cognitive function, especially short-term memory, may decline temporarily with the middle fossa approach and long operation time.

Posterior fossa reconstruction: a surgical technique for the treatment of Chiari I malformation and Chiari I/syringomyelia complex--preliminary results and magnetic resonance imaging quantitative assessment of hindbrain migration.

Experimental models have shown that Chiari I malformation is a primary paraaxial mesodermal insufficiency occurring after the closure of the neural folds takes place. According to these hypotheses, a small posterior fossa caused by an underdeveloped occipital bone would be the primary factor in the formation of the hindbrain hernia. The main objective in the surgical treatment of Chiari I malformation and related syringomyelia is directed to restore normal cerebrospinal fluid dynamics at the craniovertebral junction. The most widely accepted surgical approach is to perform a craniovertebral decompression of the posterior fossa contents with or without a dural graft. It has been emphasized that suboccipital craniectomy should be small enough to avoid downward migration of the hindbrain into the craniectomy. This slump of the hindbrain has been verified by studies using postoperative assessment by magnetic resonance imaging. Our aim in this study is to present a modification of the conventional surgical technique, which we have called posterior fossa reconstruction (PFR). Ten patients were operated on using this technique and compared with a historical control group operated on with the classic approach of making a small suboccipital craniectomy, opening the arachnoid, and closing the dura with a graft. To evaluate the morphological results in both groups objectively, preoperative and postoperative measurements of the relative positions of the fastigium and upper pons above a basal line in the midsagittal T1-weighted magnetic resonance images were obtained. In those cases with syringomyelia, syringo-to-cord ratios were calculated. The mean age of the PFR group was 35 +/- 16 years (mean +/- SD); in the control group it was 35.2 +/- 12 years. In the PFR group, the formation of an artificial cisterna magna was observed in every case; it was observed in only one case in the control group. An upward migration of the cerebellum was seen in all cases in the PFR group, with a mean ascent of the fastigium of 6.2 mm. A significant downward migration of the cerebellum was observed in seven cases in the control group. No significant differences were found in both groups when comparing syringo-to-cord ratios. This leads us to conclude that PFR is more effective than conventional surgical approaches in restoring the normal morphology of the craniovertebral junction. This allows cranial ascent of the hindbrain verified by magnetic resonance imaging and good short-term clinical results. Because PFR is mainly an extraarachnoidal approach, complications related to surgery using this technique can be kept to a minimum.

Magnetic resonance imaging in the assessment of medullary compression in achondroplasia.

Children with achondroplasia may be at increased risk of developing apneic episodes and of dying unexpectedly. The risks seem to be related to neural axis compression by an abnormal cranial base and may be complicated by the development of hydrocephalus. We used magnetic resonance imaging to study five children with achondroplasia. All of them demonstrated a discrepancy between the size of the brain stem and the foramen magnum. Comprehensive prospective assessment of infants with achondroplasia, including the use of new imaging techniques, will provide important information concerning the natural history of the relationship of the neural axis to the bony posterior fossa and upper cervical spine in this condition. It may also help to identify those patients at risk before the development of life-threatening medullary compression.