Craniopharyngioma
ICD-10 D44.4 ICD-11 2F7A.Y&XA9787

Treatment of Craniopharyngioma in Children (Age Under 18 Years)

Craniopharyngioma follows a bimodal age distribution, with one of its two incidence peaks occurring in the paediatric population. Managing this tumour in children under 18 requires a surgical strategy that differs meaningfully from adult practice.

Clinical scenario: This protocol applies to children under 18 years of age with a diagnosis of craniopharyngioma. The paediatric peak of incidence falls between ages 5 and 10, making age-specific guidance essential for this patient group.

Approach overview

Management centres on surgical strategies tailored to tumour location — with emphasis on preserving critical structures rather than pursuing aggressive resection. The specific approach depends on whether there is compression of visual pathways, raised intracranial pressure, or hydrocephalus.

Full regimen, procedural decision algorithm, and evidence grading available below →
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References

DOI: 10.1016/j.ando.2024.07.002

The age distribution of CP is bimodal, with a first peak in incidence observed in children (between the ages of 5 and 10), and a second peak in adults (between the ages of 55 and 69).

In children, suprasellar craniopharyngiomas require limited surgery to simply decompress the visual pathways and marsupialize the cysts, without attempting to pull towards the cisterns or cleave the ventricular wall, using an endoscopic transventricular or trans-sylvian approach (weak).

In the case of intracranial hypertension due to obstructive hydrocephalus associated with a craniopharyngioma, ventriculoperitoneal shunting or cyst treatment (endoscopic emptying or placement of an Omaya drain) is recommended (strong).

In the case of symptomatic optic tract compression, priority should be given to treatment of hydrocephalus and/or surgical decompression of the chiasma via a pterional trans-sylvian approach or endoscopic cystostomy (strong).

Intrasellar craniopharyngioma that does not extend beyond the diaphragm should be treated endonasally by endoscopy or microscopy.

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