Abigail K Zamora1, Wesley E Barry2, Danuta Nowicki3, Shadassa Ourshalimian3, Fariba Navid4, Joseph M Miller5, Chadi Zeinati5, Dean M Anselmo6. 1. 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. Electronic address: abigail.zamora@med.usc.edu. 2. 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. 3. Division of Pediatric Surgery, Children's Hospital Los Angeles, Los Angeles, CA. 4. 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. 5. 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. 6. 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. Electronic address: DAnselmo@chla.usc.edu.
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.
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.