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Paeds Vivashaematology-oncology-and-transfusion

Paeds Vivas · haematology-oncology-and-transfusion

Brain and spinal tumours: Viva

Branching clinical structured oral on brain and spinal tumours in children, covering the recognition of raised intracranial pressure, the posterior fossa predominance with medulloblastoma, cerebellar pilocytic astrocytoma, ependymoma and brainstem glioma, the triad of the diffuse intrinsic pontine glioma, the molecular subgroups of medulloblastoma, the urgent magnetic resonance imaging pathway, the perioperative dexamethasone and the management of hydrocephalus, the risk-adapted craniospinal irradiation and chemotherapy, and the classic diagnostic pitfalls around the progressive headache and the new squint.

branching clinical structured oral4 min readVerification in progress

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Prompt
A seven-year-old girl is brought in with four weeks of progressive early morning headache that wakes her from sleep, vomiting that relieves the headache, and a new squint. The examination shows papilloedema, a right abducens palsy and a wide-based ataxic gait. The examiner asks how you frame the problem, what you do in the first hours, how you confirm and stage the diagnosis, and how you would counsel the family across the different tumour possibilities.

This is a branching oral built to probe the reasoning that holds the location and the molecular classification at the centre, and to expose the candidate who has memorised the headline without the corners. The questions escalate from the framing to the stabilisation, the imaging, and the definitive management, with deliberate probes into the pitfalls. [3]

Opening question: framing the problem

The examiner opens with the history and the examination and asks: how do you frame this problem in a single sentence, and what is your first priority? [2]

A strong answer names the syndrome of raised intracranial pressure with a posterior fossa sign, the ataxia, and states that the progressive early morning headache and vomiting with the papilloedema is a tumour until imaging proves otherwise. The first priority is the relief of the raised pressure, because the obstructive hydrocephalus from a posterior fossa mass can progress to the herniation within hours. [11]

Model answer. This child has raised intracranial pressure from a posterior fossa tumour with obstructive hydrocephalus, almost certainly a medulloblastoma until imaging proves otherwise. My first priority is to relieve the pressure with the dexamethasone and the neurosurgical management of the hydrocephalus, and to reach the urgent magnetic resonance imaging of the brain and the whole neuraxis. [3]

Probe one: the resuscitation

The examiner presses: tell me exactly what you do in the first four hours, and why you choose the dexamethasone. [11]

A strong answer reproduces the dexamethasone at zero point one to zero point two milligrams per kilogram per dose every six hours for the vasogenic oedema, tapered after the surgery, with the head elevation and the isotonic fluids. The hydrocephalus is managed with the external ventricular drain at the surgery or before it, and the endoscopic third ventriculostomy is the alternative. The falling consciousness or the Cushing response triggers the emergency measures. [11]

Paediatrics Fellowship Pro

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You have read the opening of this viva. The complete unit — every section and its primary-source references — is part of the Paediatrics Fellowship fellowship library.

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References6ShowHide
  1. [2]Pollack IF, Agnihotri S, Broniscer A Childhood brain tumors: current management, biological insights, and future directions J Neurosurg Pediatr, 2019.PMID 30835699
  2. [3]Northcott PA, Robinson GW, Kratz CP Medulloblastoma Nat Rev Dis Primers, 2019.PMID 30765705
  3. [7]van den Bent M, Saratsis AM, Geurts M H3 K27M-altered glioma and diffuse intrinsic pontine glioma: Semi-systematic review of treatment landscape and future directions Neuro Oncol, 2024.PMID 38102230
  4. [8]Bornhorst M, Frappaz D, Packer RJ Pilocytic astrocytomas Handb Clin Neurol, 2016.PMID 26948364
  5. [11]Malbari F, Staggers KA, Minard CG Provider views on perioperative steroid use for patients with newly diagnosed pediatric brain tumors J Neurooncol, 2020.PMID 32026434
  6. [12]Rey-Casserly C, Diver T Late effects of pediatric brain tumors Curr Opin Pediatr, 2019.PMID 31693589
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