Fat embolism syndrome (FES) is a common clinical entity that can occasionally have significant neurological sequelae. The authors report a case of cerebral fat embolism and FES that required surgical management of intracra nial pressure (ICP). They also discuss the literature as well as the potential need for neurosurgical management of this disease entity in select patients. A 58yearold woman presented with a seizure episode and altered mental status after suffering a right femur fracture. Head CT studies demonstrated hypointense areas consistent with fat globules at the graywhite matter junction predominantly in the right hemisphere. This CT finding is unique in the literature, as other reports have not included imaging performed early enough to capture this finding. Brain MR images obtained 3 days later revealed T2hyperintense areas with restricted diffusion within the same hemisphere, along with midline shift and subfalcine herniation. These findings steered the patient to the operating room for decompressive hemicraniec tomy. A review of the literature from 1980 to 2012 disclosed 54 cases in 38 reports concerning cerebral fat embolism and FES. Analysis of all the cases revealed that 98% of the patients presented with mental status changes, whereas only 22% had focal signs and/or seizures. A good outcome was seen in 57.6% of patients with coma and/or abnormal posturing on presentation and in 90.5% of patients presenting with mild mental status changes, focal deficits, or sei zure. In the majority of cases ICP was managed conservatively with no surgical intervention. One case featured the use of an ICP monitor, while none featured the use of hemicraniectomy.
OBJECTIVEThe authors reviewed 20 years’ experience with the surgical management of open myelomeningocele in a well-defined retrospective cohort from a single large academic medical center. Their goal was to define the characteristics of a modern cohort of children with myelomeningocele to allow for evidence-based decision-making for the treatment of these patients.METHODSAfter IRB approval was obtained, the authors queried an operative database maintained by the Department of Neurological Surgery at Children’s Hospital of Pittsburgh for patients who underwent closure of a myelomeningocele between 1995 and 2015. They identified 153 infants, and a retrospective chart review was performed.RESULTSEighty-eight percent of the patients required placement of a ventriculoperitoneal shunt, and 15% of these patients acquired shunt-related infections. Eighteen percent of patients underwent Chiari malformation type II (CM-II) decompression. Sixteen percent of patients underwent a tethered cord release. Three percent of patients died within the 1st year of life. Predictors of an early demise included poor Apgar scores, large head circumference, and need for early CM-II decompression. Functional motor outcome was slightly better than predicted by anatomical level of defect.CONCLUSIONSMyelomeningoceles represent a severe birth defect with life-threatening complications. The authors provide long-term follow-up data and insight into factors that contribute to early death.
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