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Systematic Approach for Children With Orbital Malformation With Sirolimus Use
Sponsor: Ain Shams University
Summary
The goal of this interventional study is to Set a systematic management approach for orbital lymphatic malformation and to study the safety and efficacy of sirolimus in children and adolescents with orbital lymphatic malformation\]. The main questions it aims to answer are: \[what is the safety of using sirolimus in patients with orbital lymphatic malformation\]? \[what is the efficacy of sirolimus in patients with orbital lymphatic malformation\]? Participants will be divided into three groups based on their symptoms, extent and type of orbital lymphatic malformation Group 1 ( will receive injection sclerotherapy): All Patients with symptomatic lymphatic malformation except those with complex malformation , Group 2 (will receive sirolimus on 0.8 mg per m2 twice daily for 6 months ):A) Patients with symptomatic complex lymphatic malformation. B) Patients in group 1 if there is progression in lesion size or ineffective local therapy Group 3 ( will receive both injection sclerotherapy and sirolimus):A) Patients in group 1 and patients with complex lymphatic malformation if there is concern for complications like hemorrhage, amblyopia and vision loss since diagnosis.
Official title: Systematic Management Approach for Children and Adolescents With Orbital Lymphatic Malformation
Key Details
Gender
All
Age Range
1 Year - 18 Years
Study Type
INTERVENTIONAL
Enrollment
30
Start Date
2025-11-01
Completion Date
2027-11-01
Last Updated
2026-01-30
Healthy Volunteers
No
Conditions
Interventions
Injection Sclerotherapy
For group 1: They will be assigned to sclerotherpay .Local intralesional trancutaneous injection dose will be 0.6 mg / kg of bleomycin dissolved in the total volume of the lesion calculated from dimensions of orbital lesion in MRI orbit with contrast. Intralesional injection frequency will be guided by clinical response of the patients and imaging studies.If there is response clinically, injection will be continued every one month until there is no further response or if the lesion is reaching 75% of lesion size Or if there is no response from first injection.For lid and conjunctival components local injection will be for small , moderate and large lesions 2 IU, 4 IU, 6 IU respectively and further injection every month guided by lesion response.Less than 25% reduction will not receive further injection, if there is 25%- 50 % reduction injection will continue till reaching 75% or more clinical reduction in size
Sirolimus (Rapamune®)
Dose will be 0.8 mg/m2 twice daily orally for 6 months ,published literature for sirolimus' use in the pediatric population recommends a starting dose of sirolimus 1.6 mg/m2/day divided twice daily (10) with target trough level of 8-15 ng/ml will be done after 2 weeks from starting therapy
both injection sclerotherapy and sirolimus
They will be assigned to sclerotherpay .Local intralesional trancutaneous injection dose will be 0.6 mg / kg of bleomycin dissolved in the total volume of the lesion calculated from dimensions of orbital lesion in MRI orbit with contrast. Intralesional injection frequency will be guided by clinical response of the patients and imaging studies.If there is response clinically, injection will be continued every one month until there is no further response or if the lesion is reaching 75% of lesion size Or if there is no response from first injection.For lid and conjunctival components local injection will be for small , moderate and large lesions 2 IU, 4 IU, 6 IU respectively and further injection every month guided by lesion response.Less than 25% reduction will not receive further injection, if there is 25%- 50 % reduction injection will continue till reaching 75% or more clinical reduction in size sirolimus will be given in a dose of 0.8 mg per m2 twice daily .
Locations (1)
Ain Shams University
Cairo, Cairo Governorate, Egypt