Paediatric cerebellar glioblastoma - long-term survival following surgery and adjuvant chemoradiotherapy: A case report and literature review.

Introduction: Paediatric cerebellar glioblastoma is an exceptionally rare clinical entity, with very few cases described in the literature. In the majority of reported cases, prognosis is extremely poor, despite surgical and oncological management. The paucity of data results in lack of consensus as to the optimal management of these patients, with the objective of prolonging survival. Research question: Do patient or tumour characteristics suggest more favourable rates of progression-free survival in paediatric cerebellar glioblastoma? Material and methods: Tumour histopathology plus retrospective molecular analysis of archived samples, as well treatment strategy and patient characteristics of a six-year-old child with cerebellar glioblastoma and prolonged progression-free survival were assessed. Characteristics in the published literature that inferred prolonged survival were identified and compared. Results: Paediatric cerebellar glioblastoma is extremely rare, with only a handful of cases reported over several decades, during which time diagnostic and therapeutic techniques have evolved markedly. Consequently, the scarcity of data with sufficient granularity means that limited conclusions can be drawn. Specific clinical and histopathological factors (i.e. female sex, young age, EGFR negativity and surgical resection plus adjuvant chemoradiotherapy) may indicate a more favourable progression-free survival. Discussion and conclusion: Rates of progression-free survival in this rare condition are generally poor, however, several patient and tumour characteristics may infer more favourable prognosis. As increasingly refined means of diagnosis and characterisation are developed, particularly as a result of advances in molecular analyses, more adjuvant treatment options are likely to come on stream in future.

Efficacy and safety of the Miethke programmable differential pressure valve (proGAV®2.0): a single-centre retrospective analysis.

PURPOSE: Achieving decompression without CSF over-drainage remains a challenge in hydrocephalus. Differential pressure valves are a popular treatment modality, with evidence suggesting that incorporation of gravitational units helps minimise over-drainage. This study seeks to describe the utility of the proGAV®2.0 programmable valve in a paediatric population. METHODS: Clinical records and imaging of all patients fitted with proGAV®2.0 valves and Miethke fixed-pressure valves between 2014 and 2019 at our tertiary centre were analysed. Patient demographics, indication for shunt and valve insertion/revision and time to shunt/valve revision were collected. Ventricular linear metrics (fronto-occipital horn ratio (FOHR) and fronto-occipital horn width ratio (FOHWR)) were collected pre- and post-valve insertion. Microsoft Excel and SPSS v24 were used for data collection and statistical analysis. RESULTS: Eighty-eight proGAV®2.0 valves were inserted in a population of 77 patients (n = 45 males (58%), mean age 5.1 years (IQR: 0.4-11.0 years)). A total of 102 Miethke fixed-pressure valves were inserted over the same time period. Median follow-up was 17.5 months (1.0-47.3). One (1.1%) proGAV®2.0 was revised due to over-drainage, compared to 2 (1.9%) fixed-pressure valves (p > 0.05). ProGAV®2.0 insertion resulted in a significant decrease in the mean number of revisions per patient per year (1.77 vs 0.25; p = 0.01). Overall shunt system survival with the proGAV®2.0 was 80.4% at 12 months, and mean time to revision was 37.1 months, compared to 31.0 months (95%CI: 25.7-36.3) and 58.3% in fixed-pressure valves (p < 0.01). Significant decreases were seen following proGAV®2.0 insertion in both FOHR and FOHWR, by 0.014 (95%CI: 0.006-0.023, p = 0.002) and 0.037 (95%CI: 0.005-0.069, p = 0.024) respectively. CONCLUSION: The proGAV®2.0 provides effective decompression of hydrocephalic patients, significantly reduces the number of valve revisions per patient and had a significantly greater mean time to revision than fixed-pressure valves.

Intracranial Epidermoid Lesions: our Experience of 38 Cases.

BACKGROUND: The clinical outcome following surgical resection of intracranial epidermoid lesions is not well documented. We describe a case series evaluating the clinical presentation and outcome following surgery in patients diagnosed with these lesions. MATERIALS AND METHODS: A retrospective study conducted over a 4-year period. RESULTS: A total 38 patients who underwent surgery for intracranial epidermoid lesions were identified. Of these, 20 were supertentorial and 18 were infratentorial lesions. 33 patients presented with headache. 10 patients with supratentorial lesions presented with seizures. Patients with suprasellar epidermoid lesions presented with headache and visual problems. 14 of the 18 patients with cerebellopontine angle lesions presented with symptoms of trigeminal neuralgia. Post-operatively, 3 patients with cerebellopontine angle tumors developed facial weakness which was transient in 2 patients but permanent in 1 patient. Visual impairment in patients with suprasellar epidermoid lesions improved post-operatively. Hormonal replacement therapy was required in 2 patients. Recovery of 4 patients was complicated by Cerebrospinal fluid (CSF) leak while meningitis developed in 2 patients. One patient operated for cerebellopontine angle epidermoid died post operatively. CONCLUSIONS: Epidermoid lesions can develop anywhere in the cranial cavity. Like other space occupying lesions they often present with symptoms of raised ICP and with specific neurological deficits depending on location of the tumour. Surgical outcome is excellent with low complication rates.

Cyclical vomiting syndrome secondary to a Chiari I malformation-a case report.

BACKGROUND: Cyclical vomiting syndrome is a disorder characterised by recurrent episodes of profuse vomiting. There are no cases in the literature on the management of children with presenting with cyclical vomiting syndrome and a Chiari malformation type I. DISCUSSION: We report the case of a 12-year-old child diagnosed with cyclical vomiting syndrome and a Chiari malformation type I. The patient received symptomatic relief following a craniocervical decompression.

Intracerebral Masson's Tumor-Slow-Filling Vascular Lesion Demonstrated by Indocyanine Green Video Angiography.

BACKGROUND: Intravascular papillary endothelial hyperplasia, or Masson's tumors, are benign vascular lesions that are rarely seen intracranially. The vascular characteristics of these lesions are also unknown. CASE DESCRIPTION: We report the case of a 24-year-old male patient with a 3-year history of headache and dizziness. Neuroradiologic imaging showed a slow-growing lesion consistent with a low-grade glioma. Intraoperative appearance was of a vascular lesion that was slow filling as demonstrated with indocyanine green video angiography. Histologic analysis following resection revealed intravascular papillary endothelial hyperplasia (Masson's tumor). CONCLUSION: Masson's tumors are slow-filling vascular lesions. The preoperative diagnosis of this lesion is difficult as it can mimic a neoplastic lesion. Conservative and surgical treatment options should therefore be carefully considered. Patients with subtotal resection must undergo long-term follow-up surveillance imaging as recurrence is a possibility.

Xanthogranulomatous hypophysitis: a rare and often mistaken pituitary lesion.

UNLABELLED: Xanthogranulomatous hypophysitis (XGH) is a very rare form of pituitary hypophysitis that may present both clinically and radiologically as a neoplastic lesion. It may either be primary with an autoimmune aetiology and can occur in isolation or as a part of autoimmune systemic disease or secondary as a reactive degenerative response to an epithelial lesion (e.g. craniopharyngioma (CP), Rathke's cleft cyst, germinoma and pituitary adenomas) or as a part of a multiorgan systemic involvement such as tuberculosis, sarcoidosis or granulomatosis. It may also present with a variation of symptoms in children and adults. Our case series compares the paediatric and adult presentations of XGH and the differential diagnoses considered in one child and two adult patients, highlighting the wide spectrum of this condition. Endocrine investigations suggested panhypopituitarism in all three patients and imaging revealed a suprasellar mass compressing the optic chiasm suggestive of CP or Rathke's cleft cyst in one patient and non-functioning pituitary macroadenoma in two patients. Magnetic resonance imaging (MRI) demonstrated mixed signal intensities on T1- and T2-weighted sequences. Following endoscopic transsphenoidal surgery, histological analysis revealed necrotic material with a xanthogranulomatous reaction confirming XGH in two patients and a necrobiotic granulomatous chronic inflammatory infiltrate with neutrophils in one patient, which is not typical of current descriptions of this disorder. This case series describes the wide spectrum of XGH disease that is yet to be defined. Mixed signal intensities on T1- and T2-weighted MRI sequences may indicate XGH and diagnosis is confirmed by histology. Histological variation may indicate an underlying systemic process. LEARNING POINTS: XGH is a rare form of pituitary hypophysitis with a wide clinical and histological spectrum and can mimic a neoplastic lesion.XGH primarily presents with growth arrest in children and pubertal arrest in adolescents. In adults, the presentation may vary.A combination of hypopituitarism and mixed signal intensity lesion on MRI is suggestive of XGH and should be considered in the differential diagnosis of sellar lesions.Radical surgery is the treatment of choice and carries an excellent prognosis with no recurrence.

Volkman's Contracture, Persistent Limb Ischaemia, and Amputation: A Complication of Brachial Artery Catheterisation for Haemodynamic Monitoring Using PiCCO.

We report a case of a 64-year-old woman who was admitted to intensive care unit with multiorgan failure secondary to Plasmodium falciparum malaria. Haemodynamic monitoring using the transpulmonary thermodilution with pulse contour analysis system (PiCCO) was achieved via the left brachial artery. Two days later, a flexion deformity of the left hand was noted, and examination revealed left lower arm ischaemia. Removal of the catheter resulted in an immediate improvement of the contracture. However, distal pulses were still absent, and the arm remained ischaemic resulting in a below elbow amputation. This is the first documented case of a persistent limb ischaemia following the insertion of an arterial catheter for haemodynamic monitoring with PiCCO. We therefore highlight the need for regular assessment of limb perfusion after arterial catheterisation for haemodynamic monitoring with PiCCO. In addition, the brachial artery should be avoided, and femoral artery catheterisation is recommended.