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Lymphatic Malformation Architecture: Implications for Treatment With OK-432
Claudia C Malic1, Regan Guilfoyle, Rebecca J M Courtemanche
1*Children's Hospital of Eastern Ontario, Ottawa †University of Alberta Hospital, Edmonton ‡University of British Columbia §BC Children's Hospital, Vancouver, Canada.
The Journal of Craniofacial Surgery
|August 24, 2017
Summary
This study identifies three new lymphatic malformation (LM) architectures: open-cell microcystic, closed-cell microcystic, and lymphatic channels. Open-cell microcystic LMs show better response to OK-432 treatment than closed-cell types.
Area of Science:
- Vascular Anomalies
- Pediatric Surgery
- Sclerotherapy
Background:
- Lymphatic malformations (LM) are congenital vascular anomalies with diverse clinical presentations.
- Current classification systems for LM may not fully capture architectural nuances relevant to treatment response.
Purpose of the Study:
- To describe novel architectural types of lymphatic malformations (LM).
- To elucidate the relationship between these new LM architectures and treatment outcomes with OK-432.
- To expand the current classification of LM based on observed architectural subtypes.
Main Methods:
- Retrospective review of patients with LM treated with OK-432 at BC Children's Hospital (December 2002 - January 2012).
- Analysis of lesion architecture under fluoroscopic guidance to identify novel subtypes.
- Correlation of LM architecture with response to OK-432 sclerotherapy.
Main Results:
- Three novel LM architectures were identified: open-cell microcystic, closed-cell microcystic, and lymphatic channel.
- Open-cell microcystic LMs demonstrated complete or good response to OK-432 (3/3), attributed to free cyst communication.
- Closed-cell microcystic LMs showed partial responses (2/2) due to localized cysts, while lymphatic channels had partial responses.
- Macrocystic lesions had a high rate of complete or good response (14/19).
Conclusions:
- The identification of open-cell microcystic, closed-cell microcystic, and lymphatic channel architectures refines LM classification.
- Treatment response to OK-432 varies significantly with LM architecture.
- Open-cell microcystic LMs respond more favorably to OK-432 than closed-cell microcystic LMs, with lymphatic channels also showing potential response.
- These findings have direct clinical implications for tailoring OK-432 sclerotherapy based on LM architecture.

