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Erschienen in: Pediatric Radiology 3/2020

01.03.2020 | Original Article

Clinical and imaging features in fibro-adipose vascular anomaly (FAVA)

verfasst von: Mohammad Amarneh, Raja Shaikh

Erschienen in: Pediatric Radiology | Ausgabe 3/2020

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Abstract

Background

Fibro-adipose vascular anomaly (FAVA) is a rare and recently described complex vascular malformation. The clinical and imaging features and morphology can be confusing and often overlap with features of other vascular malformations and tumors.

Objective

To present the imaging characterization of FAVA in association with clinical features.

Materials and methods

We retrospectively evaluated clinical and imaging (MRI, sonography and venography) findings in FAVA. We documented the clinical presentation, lesion morphology, imaging characteristics, tissue distribution, pattern of contrast enhancement and vascular characteristics.

Results

Thirty-eight people (31 female, 7 male) ages 1–30 years (mean 12 years) were diagnosed with FAVA based on clinical findings combined with imaging or histopathological findings (n=17). Most lesions were in the lower extremity (n=36). Three patterns were noticed: focal, focal infiltrative and diffuse.

Conclusion

Fibro-adipose vascular anomaly, a rare and complex vascular malformation, has distinct clinical and imaging features that a radiologist should be familiar with to avoid delay in diagnosis and to direct appropriate management.
Literatur
1.
Zurück zum Zitat Alomari AI, Spencer SA, Arnold RW et al (2014) Fibro-adipose vascular anomaly: clinical-radiologic-pathologic features of a newly delineated disorder of the extremity. J Pediatr Orthop 34:109–117CrossRef Alomari AI, Spencer SA, Arnold RW et al (2014) Fibro-adipose vascular anomaly: clinical-radiologic-pathologic features of a newly delineated disorder of the extremity. J Pediatr Orthop 34:109–117CrossRef
2.
Zurück zum Zitat Luks VL, Kamitaki N, Vivero MP et al (2015) Lymphatic and other vascular malformative/overgrowth disorders are caused by somatic mutations in PIK3CA. J Pediatr 166:1048–1054CrossRef Luks VL, Kamitaki N, Vivero MP et al (2015) Lymphatic and other vascular malformative/overgrowth disorders are caused by somatic mutations in PIK3CA. J Pediatr 166:1048–1054CrossRef
3.
Zurück zum Zitat Shaikh R, Alomari AI, Kerr CL et al (2016) Cryoablation in fibro-adipose vascular anomaly (FAVA): a minimally invasive treatment option. Pediatr Radiol 46:1179–1186CrossRef Shaikh R, Alomari AI, Kerr CL et al (2016) Cryoablation in fibro-adipose vascular anomaly (FAVA): a minimally invasive treatment option. Pediatr Radiol 46:1179–1186CrossRef
4.
Zurück zum Zitat Uller W, Fishman SJ, Alomari AI (2014) Overgrowth syndromes with complex vascular anomalies. Semin Pediatr Surg 23:208–215CrossRef Uller W, Fishman SJ, Alomari AI (2014) Overgrowth syndromes with complex vascular anomalies. Semin Pediatr Surg 23:208–215CrossRef
5.
Zurück zum Zitat Hein KD, Mulliken JB, Kozakewich HP et al (2002) Venous malformations of skeletal muscle. Plast Reconstr Surg 110:1625–1635CrossRef Hein KD, Mulliken JB, Kozakewich HP et al (2002) Venous malformations of skeletal muscle. Plast Reconstr Surg 110:1625–1635CrossRef
6.
Zurück zum Zitat Adams DM, Trenor CC 3rd, Hammill AM et al (2016) Efficacy and safety of sirolimus in the treatment of complicated vascular anomalies. Pediatrics 137:e20153257CrossRef Adams DM, Trenor CC 3rd, Hammill AM et al (2016) Efficacy and safety of sirolimus in the treatment of complicated vascular anomalies. Pediatrics 137:e20153257CrossRef
Metadaten
Titel
Clinical and imaging features in fibro-adipose vascular anomaly (FAVA)
verfasst von
Mohammad Amarneh
Raja Shaikh
Publikationsdatum
01.03.2020
Verlag
Springer Berlin Heidelberg
Erschienen in
Pediatric Radiology / Ausgabe 3/2020
Print ISSN: 0301-0449
Elektronische ISSN: 1432-1998
DOI
https://doi.org/10.1007/s00247-019-04571-6

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