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A multidisciplinary approach to management of abdominal lymphatic malformations

  • Abigail K Zamora
    Affiliations
    Division of Pediatric Surgery, Children's Hospital Los Angeles, Los Angeles, CA

    Department of Surgery, Keck School of Medicine, University of Southern California, Los Angeles, CA
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  • Wesley E Barry
    Affiliations
    Division of Pediatric Surgery, Children's Hospital Los Angeles, Los Angeles, CA

    Department of Surgery, Keck School of Medicine, University of Southern California, Los Angeles, CA
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  • Danuta Nowicki
    Affiliations
    Division of Pediatric Surgery, Children's Hospital Los Angeles, Los Angeles, CA
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  • Shadassa Ourshalimian
    Affiliations
    Division of Pediatric Surgery, Children's Hospital Los Angeles, Los Angeles, CA
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  • Fariba Navid
    Affiliations
    Division of Hematology and Oncology, Cancer and Blood Diseases Institute, Children's Hospital Los Angeles, Los Angeles, CA

    Department of Pediatrics, Keck School of Medicine, University of Southern California, Los Angeles, CA
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  • Joseph M Miller
    Affiliations
    Division of Interventional Radiology, Children's Hospital Los Angeles, Los Angeles, CA

    Department of Radiology, Keck School of Medicine, University of Southern California, Los Angeles, CA
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  • Chadi Zeinati
    Affiliations
    Division of Interventional Radiology, Children's Hospital Los Angeles, Los Angeles, CA

    Department of Radiology, Keck School of Medicine, University of Southern California, Los Angeles, CA
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  • Dean M Anselmo
    Correspondence
    Corresponding author at: Children's Hospital Los Angeles, 4650 Sunset Blvd, Department of Surgery, Mailstop #100, Los Angeles, CA 90027. Tel.: +1 323 361 5193; fax: +1 323 361 3534.
    Affiliations
    Division of Pediatric Surgery, Children's Hospital Los Angeles, Los Angeles, CA

    Department of Surgery, Keck School of Medicine, University of Southern California, Los Angeles, CA
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      Abstract

      Background/purpose

      Abdominal lymphatic malformations (LM) are a rare subset of vascular anomaly caused by abnormal development of the lymphatic system. They are classified as macrocystic, microcystic or combination macrocystic and microcystic. Surgical resection, percutaneous sclerotherapy, and medical therapy are all employed to treat these complex and often symptomatic lesions. No standardized treatment algorithm exists currently. The purpose of this study was to establish a multidisciplinary treatment approach to abdominal LMs.

      Methods

      A retrospective observational study was conducted from 2013 to 2019 on patients with abdominal LMs at a single tertiary children's hospital vascular anomalies center. Demographics, imaging, and treatment modality were recorded. Clinical and/or radiographic response to the primary treatment modality as well as complications was the outcomes measured.

      Results

      Nineteen patients (12 macrocystic, 5 microcystic and 2 combined) were identified, with a median age at diagnosis of 2.2 years (range 0.1–20.8 years). Sclerotherapy was the most common primary treatment, followed by surgical resection and sirolimus. No difference in clinical response (p = 0.58) or complications (p = 0.31) was observed based on primary treatment or subtypes.

      Conclusions

      Based on our institutional experience, we propose an LM subtype-based treatment algorithm for abdominal LMs. It employs a multidisciplinary approach, and results in satisfactory patient outcomes with minimal complications.

      Level of evidence

      Level III, retrospective comparative study.

      Abbreviations:

      LM (lymphatic malformation), ISSVA (International Society for the Study of Vascular Anomalies)

      Key words

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