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Case reportBMJ case reports · 2026

Significant clinical, functional and radiological response with sirolimus in refractory complex orbital venolymphatic malformation.

Ankita Aishwarya, Rachna Agarwal, Vaibhav Jain

OphthalmologyCase report

Sirolimus treatment was followed by marked visual improvement in a child with refractory orbital malformation.

Significant clinical, functional and radiological response with sirolimus in refractory complex orbital venolymphatic malformation.

Ankita Aishwarya … Vaibhav Jain

BMJ case reports · 2026

Background

Orbital venolymphatic malformations (orbital venolymphatic malformations) are rare congenital vascular anomalies that may cause progressive proptosis, visual loss, facial disfigurement and marked psychosocial burden.

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Results

6/9-2

vision sharper still: reading almost normal-size letters, up from finger-counting

More results

Management is challenging especially when the disease is multifocal, diffuse and with limited response to conventional therapies.

After multidisciplinary evaluation, oral sirolimus was initiated with written informed consent from the parents in a refractory setting.

The child resumed normal activities.

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Conclusion

This case highlights the potential role of systemic mechanistic target of rapamycin (mTOR) inhibition in complex, refractory orbital venolymphatic malformations and highlights careful patient selection and long-term follow-up.

Post

Significant clinical, functional and radiological response with sirolimus in refractory complex orbital venolymphatic malformation.

Sirolimus treatment was followed by marked visual improvement in a child with refractory orbital malformation.

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