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Epidemiology, management and outcomes of vascular malformations of the upper extremity: a 12-year retrospective cohort study
Sophia Hu, BS
1; Yoshiko Toyoda, MD
1; Mimi Kim, BS
1; Tessa Muss, BA
1; Ashley E Chang, BA
1; Manisha Banala, BBA
2; Emily M Graham, MD
3; Deepthi Gunturi, MS
4; Ines C. Lin, MD
11University of Pennsylvania, Philadelphia, PA; 2Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA; 3The Children's Hospital of Philadelphia, Philadelphia, PA; 4Children's Hospital of Pennsylvania, Philadelphia, PA
Background: Vascular malformations (VMs) involving the upper extremity (UE) are a heterogeneous group of lesions that can pose unique functional challenges. Multiple management options for UE VMs exist and include conservative management, medications, procedures, and surgical debulking or excision, but updated and standardized guidelines for the diagnosis and management of UE VMs are limited. The objective of this longitudinal retrospective study was to review the epidemiology, management, and outcomes of pediatric patients with UE VMs at a large academic center.
Methods: A retrospective review of patients with UE VMs evaluated at the Children's Hospital of Philadelphia between 2010-2021 was performed. Demographics, lesion characteristics, treatment type and duration, complications, and final outcomes were recorded. ANOVA, chi-square, and Fisher's exact tests were used for data analysis.
Results: A total of 276 patients (average age 5.3 years old, range 0-25.4 years old) and 284 UE VMs were included in the study. Venous malformations were the most common VM (n=99, 34.9%), followed by capillary malformations (n=61, 21.5%), lymphatic malformations (n=31, 10.9%) and arteriovenous malformations (n=18, 6.3%). The hand was the most common site of VMs (n=91, 32.0%), followed by the forearm (n=82, 28.9%), upper arm (n=78, 27.5%), and shoulder (n=61, 21.5%). Over half of all patients underwent treatment (n=145, 52.5%), and the most common form of treatment was procedures other than surgery (88/276, 31.9%), followed by surgery (67/276, 24.3%) and medications (20/276, 7.2%). The overall rate of complications excluding recurrences was 6.9% (n=19; medications (n=6, 28.5%), surgery (n=14, 20.9%), procedures (n=10, 11.1%)). Patients who had underwent surgery at any time had the highest rate of complete resolution (32/67, 47.8%), followed by those treated with medications (5/21, 23.8%) and procedures (17/90, 18.8%).
Conclusion: Pediatric UE VMs in this study received the full spectrum of treatment modalities over the last decade. Many patients received multiple treatment modalities, and treatment of UE VMs should be tailored to specific lesion characteristics. Future studies should include a prospective database with collection of validated PROs to further characterize the decision-making process for UE VMs.
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