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. 2021 May 19:29:71-81.
doi: 10.1016/j.jpra.2021.05.002. eCollection 2021 Sep.

Management of Fibro-adipose Vascular Anomalies (FAVA) in Paediatric Practice

C Lipede  1 , D Nikkhah  1 , R Ashton  1 , G Murphy  1 , A M Barnacle  2 , P A Patel  2 , G D Smith  1 , D M Eastwood  3   4 , B Sivakumar  2
Affiliations

Management of Fibro-adipose Vascular Anomalies (FAVA) in Paediatric Practice

C Lipede et al. JPRAS Open. .

Abstract

Fibro-adipose vascular anomaly (FAVA) is a discrete type of vascular anomaly. We describe our experience managing FAVA at a tertiary level paediatric hospital and offer a treatment algorithm.

Methods: A retrospective review of 27 patients with proven FAVA was undertaken. All patients had undergone MRI and USS evaluation. Patient demographics, presenting concerns, treatment methods, and outcomes were recorded and evaluation with the paediatric outcomes data collection instrument (PODCI) completed a minimum of 12 months after definitive treatment.

Results: Mean age at presentation was 8.9 years (range: 9 m-17.4 y) and mean post-treatment follow-up was 7.4 y (range: 2 y-11.6 y). Twenty of 27 lesions affected the lower limb. Severe neurogenic-type pain was present in 23 cases and contractures across joints in 11 cases. Sclerotherapy with sodium tetradecyl sulphate was used in 11 cases, with no improvement in symptoms. Cryoablation provided pain relief in 3/4 cases, but contracture subsequently increased in one patient and pain recurred in another.Fourteen cases underwent surgery (four surgical excisions alone, 10 in combination with other procedures). Three patients required four further surgical procedures that include one amputation for intractable pain and poor function.PODCI evaluations suggest overall good function, with surgical management and interventional radiology that provide comparable results. Surgery did correct deformity.

Conclusion: If conservative measures or cryoablation fail to achieve symptomatic control, surgical excision should be considered, combined with adjunctive procedures, to correct contractures and balance muscle forces.Relief of pain may compensate for the loss of muscle mass and overall improves function. Multidisciplinary team working is essential.

Keywords: FAVA; fibroadipose vascular anomaly; outcomes; paediatric; treatment.

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Figures

Figure 1
Figure 1
An H&E-stained histological section of a FAVA excised from a gastrocnemius muscle showing abundant mixed fibro-adipose tissue, features specific to this diagnosis. In some fibro-fatty tissue predominates, whereas in others, there are dense bands of fibro-collagenous tissue with diffuse fibrotic infiltration of muscle and extension along fascial planes. The abnormal veins are often thin walled and dysplastic, and the fibrotic tissue may encase the neurovascular structures.
Figure 2
Figure 2
a: Axial T1-weighted MR image through the thigh of a 5-year-old male demonstrating heterogeneous high-signal abnormality within vastus intermedius, in keeping with FAVA. The rounded low-signal areas within the lesion correspond to large dysplastic veins within the lesion, typical of this disease. Figure 2b: Sagittal fat-saturated gadolinium-enhanced MR image of the lower limb of a 15-year-old male, demonstrating patchy enhancement of a FAVA lesion involving the gastrocnemius.
Figure 3
Figure 3
Intraoperative image after the exploration of the flexor aspect of the thigh (posterior approach) demonstrating the involvement of the epineurium of the sciatic nerve.
Figure 4
Figure 4
Intraoperative image after the extensive decompression of median and ulnar nerves in a case involving the majority of the flexor compartment of the forearm and demonstrating typical macroscopic appearance of the involved musculature.
Figure 5
Figure 5
Intraoperative image of a forearm following debulking of involved palmaris longus and part of flexor digitorum superficialis.
Figure 6
Figure 6
PODCI (paediatric outcomes data collection instrument) scores for operative and nonoperatively treated FAVA cases.
Figure 7
Figure 7
Suggested algorithm for the management of FAVA lesions in an MDT (multidisciplinary team) setting.
See this image and copyright information in PMC

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