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J Pediatr Orthop. 2020 Mar;40(3):e227-e236. doi: 10.1097/BPO.0000000000001406.

Surgical Management of Fibroadipose Vascular Anomaly of the Lower Extremities.

Author information

1
Departments of Orthopedic Surgery.
2
Department of Orthopedic Surgery, The Royal Children's Hospital, Melbourne, Vic., Australia.
3
Division of Hematology/Oncology.
4
Vascular Anomalies Center, Boston Children's Hospital, Boston, MA.
5
Division of Vascular and Interventional Radiology, Boston Children's Hospital and Harvard Medical School.
6
Pathology.
7
Surgery.
8
Plastics and Oral Surgery.

Abstract

BACKGROUND:

Fibroadipose vascular anomaly (FAVA) is a recently-defined vascular malformation often involving the extremities and presenting in childhood. Patients may present to orthopaedic surgeons with pain, swelling, joint contractures, and leg length discrepancy. There is no established therapy or treatment paradigm. We report on outcomes following surgical excision for patients with this condition.

METHODS:

Between 2007 and 2016, all 35 patients that underwent excision of lower-extremity FAVA were retrospectively reviewed using a combination of medical records, radiologic findings, and telemedicine reviews.

RESULTS:

Mean age at initial presentation was 12.3±6.8 years. Mean follow-up from time of definitive diagnosis at our institution was 66 months (range: 12 to 161 mo). Mean follow-up after surgery was 35 months (range: 6 to 138 mo). Females were affected more than males (71% vs. 29%). The most common location of FAVA was in the calf (49%), followed by the thigh (40%). The most commonly involved muscle was gastrocnemius (29%), followed by the quadriceps (26%). At latest follow-up after surgery, there was an improvement in the proportion of patients with pain at rest (63% vs. 29%), pain with activity (100% vs. 60%), as well as analgesia use (94% vs. 37%). Fourteen patients (40%) had symptomatic residual disease or recurrence of FAVA requiring further treatment. Six patients (17%) required further surgery and 6 (17%) required further interventional radiologic procedures. Three patients (9%) required eventual amputation for intractable pain and loss of function. Lesions with direct nerve involvement were associated with persistent neuropathic symptoms at latest follow-up (P=0.002) as well as symptomatic residual disease and/or recurrence requiring further treatment (P=0.01). Seventeen patients (49%) had 19 preoperative joint contractures. Eighteen of the 19 contractures (95%) had sustained improvement at latest follow-up.

CONCLUSIONS:

In carefully selected patients, surgical excision of FAVA results in improvement of symptoms. However, symptomatic residual disease and/or recurrence are not uncommon. Direct nerve involvement is associated with a worse outcome.

LEVEL OF EVIDENCE:

Level IV-case series.

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