Pediatric Interventions
Tushar Garg, MD (he/him/his)
Postdoctoral Research Fellow
Johns Hopkins University School of Medicine
Disclosure information not submitted.
Natalie Y. Ring, MD
Resident
Johns Hopkins University
Ryan W. England, MD
Resident
Johns Hopkins University
Clifford Weiss, MD, FSIR, FCIRSE
Professor of Radiology and Biomedical Engineering
Johns Hopkins Univeristy School of Medicine
To assess and compare the factors affecting pain and bothersome symptoms in patients with low-flow vascular malformations (VMs) involving different parts of the body using a validated Patients-Reported Outcome Measure for Vascular Malformations Questionnaire (PROVAM).
Materials and Methods:
From 7/2019 to 2/2022, 327 consecutive patients completed 439 PROVAM at our vascular anomalies clinic. The 30-item instrument includes free text questions, which assess the most bothersome symptoms and factors improving or worsening vascular malformation pain. All responses to these questions were evaluated using a directed content analysis approach. The most bothersome symptom was categorized into four conceptual domains of PROVAM: physical (PD), emotional/social well-being (ESD), and functional impact [head and neck (H&N); upper extremity, lower extremity, and trunk (ULT)] (FD). The rates across different groups (H&N, Upper extremity: UE, Lower extremity: LE, Trunk: Tr) were compared using a chi-squared test.
Results:
323 of 439 (73.6%) questionnaires included information about the most bothersome symptom; most of the responses affected the PD (261/323; 80.8%), followed by ESD (52/323; 16.1%) and FDs (H&N, 15/323; 4.6% vs. ULT, 46/323; 14.2%). The PD was more commonly affected in those with LE VMs (LE, 121/136; 89% vs. H&N, 48/73; 65.8% vs. UE, 63/77; 81.8% vs. Tr, 41/53; 77.4%, p< .001) and ESD was more commonly affected in those with H&N VMs (H&N, 22/73; 30.1% vs. UE, 12/77; 16% vs. LE, 8/136; 5.9% vs. Tr, 15/53; 28.3%, p< .001). 319 of 439 (72.7%) questionnaires included information about factors affecting pain change, in which use of pain medications (LE, 53/145; 36.6% vs. H&N, 15/65; 23.1% vs. UE, 16/75, 21.3% vs. Tr, 9/48; 18.8%, p=.02), and positional elevation (LE, 46/145; 31.7% vs. H&N, 13/65; 20% vs. UE, 18/75; 23.9% vs. Tr, 5/48, 10.3%, p=.02) significantly improved pain in patients with LE VMs compared to other locations. Similarly, increased activity made LE (LE, 80/145; 55.2% vs. H&N, 23/65; 35.4% vs. UE, 39/75; 52% vs. Tr, 21/48; 43.8%, p=.049), decreased activity made Tr (Tr, 8/48; 16.7% vs. H&N, 0/0; 0% vs. UE, 1/75; 1.3% vs. LE, 14/145; 9.7%, p=.0006), and stress (H&N, 8/65; 12.4% vs. UE, 2/75; 2.6% vs. LE, 0/0; 0% vs. Tr, 2/48; 4.2%, p=.0001) made H&N pain worse compared to others.
Conclusion:
The content analysis of these responses provides a rare insight into the various factors that affect quality of life of patients with VMs based on their locations. This information may aid physicians in counselling patients with VMs on symptom management.