Spontaneous Resolution of Chiari 1-Associated Syringomyelia: A Report of Two Cases.

BACKGROUND: Traditional teaching is that Chiari 1 malformations with syrinx should be operated as soon as possible. We present 2 cases of a radiologically proven Chiari 1 malformation with syrinx which were treated nonoperatively and improved radiologically. METHODS: Two children with an MRI-proven Chiari 1 malformations were followed up nonoperatively for 7 years (2010-2017). One was a boy aged 8 years and the other a girl aged 9 years at first presentation. Their parents were not interested in a surgical option and so it was decided to adopt a wait-and-watch policy. Both patients were followed up with a regular half-yearly clinical examination and yearly MRI studies (2010-2017). RESULTS: It was found that, in both of them, the initial cough during swallowing and the headache improved. Serial MRI showed that the hind-brain protrusion was reduced and syrinx diminished or resolved well. CONCLUSIONS: Some children with Chiari I and syringomyelia may show spontaneous resolution of symptoms and syringomyelia. Large registry studies will be needed to determine how likely it is that Chiari-associated syringes will resolve, and whether there are particular anatomic or neurologic differences between those who require surgery and those who don't.

Non-infantile desmoplastic cerebellar ganglioglioma in a patient with multiple congenital anomalies: a rare association.

Numerous syndromes with craniofacial, cardiac, cutaneous and vascular anomalies have been described in the literature. Here we report on a 9-year-old girl who was diagnosed as having a clavicular arteriovenous malformation with limb enlargement, an axillary port wine stain and macrocephaly with frontal bossing. She was investigated for enlarged head size; although she did not have hydrocephalus as initially suspected, she was found to have a right cerebellar lesion. This was excised and the pathology report was diagnostic of desmoplastic ganglioglioma. The posterior fossa is a very rare location for gangliogliomas. This was a desmoplastic variant that is only encountered in infants. Thus, the location and the pathology made the tumour rare. In addition, the cranio-vasculo-cutaneous lesions made this previously unreported association noteworthy.

An Analysis of Eosinophil- and Basophil-Based Indices in Patients with Glioblastoma and their Correlation with Survival.

BACKGROUND: Glioblastoma (GBM) is the most frequently diagnosed malignant brain tumor in adults. GBM is usually lethal within 24 months of diagnosis, despite aggressive multimodality treatment. Although it has been established that cancer-related inflammation is associated with worse outcomes, the role of eosinophils, basophils, atopy, and allergy in glioma biology is only gradually being delineated. In this study, we aimed to examine if eosinophil-based and basophil-based indices were altered in patients with GBM compared with healthy controls. We also aimed to study if there was any correlation between these indices and patient-related and tumor-related factors and survival. METHODS: This study was a retrospective analysis of prospectively maintained databases. Data pertaining to patient-related and tumor-related factors, hemograms, and survival data were obtained from the electronic medical records of selected patients. Correlations between eosinophil-based and basophil-based indices and these factors were studied, as was the association with overall survival. RESULTS: All the indices were altered in patients with GBM compared with normal healthy controls. The absolute eosinophil count was higher and the neutrophils/eosinophils ratio was lower in the better prognosis groups: those with better performance status; those without features of increased intracranial pressure or altered sensorium at presentation; those with ATRX-retained tumors that did not overexpress p53; and in the long-term survivors. The total lymphocyte count/basophils ratio and the absolute eosinophil count both independently predicted survival in a multivariate analysis. CONCLUSIONS: The absolute eosinophil count was consistently higher in the better prognosis groups and is likely to be incorporated into prognostic models for GBM.

Management of Large Dural Defect with CSF Leak in Hypertelorism Correction.

THE RATIONALE: Dural tear is a serious complication during hypertelorism corrective surgeries. Identifying the tear and managing requires considerable expertise. Managing large dural tears correctly is necessary to prevent cerebrospinal fluid (CSF)-related complications in craniofacial surgery. PATIENT CONCERNS: The patient presented with hypertelorism as a part of the Tessier Cleft 0 and sought to correct the widely placed eyes. DIAGNOSIS: Large critical-sized dural tear during modified box osteotomy surgery. TREATMENT: Besides successful modified box osteotomy surgery, the critical-sized dural tear was managed with fascia lata and fibrin glue. OUTCOMES: There was no CSF leak or related complication postsurgically indicating successful sealing and healing of the dural tear. TAKE-AWAY LESSONS: The synergistic mechanism by which fascia lata graft and fibrin glue help to hermetically seal the critical-sized defect, especially when there are variable amounts of hydrostatic-hydrodynamic forces of CSF exerting pressure on the patched area, is discussed.