Glycogen storage disease type I
ICD-10 E74.0 · ICD-11 5C51.3.6

What to do when watchful waiting fails to achieve adenoma regression in Glycogen storage disease type I

Clinical scenario

In glycogen storage disease type I (GSD I), hepatocellular adenomas are a recognised complication. The initial approach is conservative management — watchful waiting combined with optimisation of metabolic control — under which regression of adenomas may occur.

When the first line has not succeeded

If conservative management with watchful waiting and optimisation of metabolic control has not achieved regression of adenomas — or if there is an increase in size, number, or bleeding — escalation beyond conservative care is indicated.

Next-step approach (partial overview)

The protocol for this situation involves local treatment directed at the liver adenomas. Several interventional options are included in the structured regimen, particularly when an increase in size, number, or bleeding is noted. The full criteria, sequence, and complete details remain in the protocol.

Instant Access to Structured Evidence-Based Regimens
References

DOI: 10.1038/gim.2014.128

Initially, the management of liver adenomas in the GSD I population should be conservative (Box 3).

There are reports of the use of percutaneous ethanol injection as the initial treatment of enlarging liver adenomas.

Percutaneous ethanol injections, radiofrequency ablation, and partial liver resection are treatment options for liver adenomas (especially if an increase in size, number, or bleeding is noted).

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