Verrucous venous malformations in children: A case for surgical excision

• Large, paediatric surgical series involving VVM management demonstrating durable outcomes and disease control with excision • Advanced reconstructive strategies enabled closure in complex, extensive lesions • Somatic MAP3K3 variants and GLUT1 positivity support molecular–histopathologic spectrum of VVMs Verrucous venous malformations (VVMs) are rare congenital vascular anomalies characterized by ectatic venous channels in the dermis and subcutis, with overlying verrucous epidermal changes. Typically presenting in early childhood, they may cause pain, bleeding, infection, and psychosocial distress. Diagnosis and management are often difficult due to clinical and histopathologic overlap with other vascular lesions. Non-surgical therapies have been trialled with limited success, and surgical excision remains the primary treatment. However, the efficacy of surgery, particularly when combined with reconstructive techniques, has not been systematically compared to alternative modalities. Twenty-three paediatric patients (aged one month to 17 years) with congenital or early-onset vascular lesions suspected to be VVMs were evaluated at a tertiary vascular anomalies clinic between 2019 and 2025. Clinical presentation, imaging, histopathology, IHC, and NGS results of a 32-gene vascular anomaly panel were reviewed. All patients underwent biopsy or excision. Data were collected retrospectively from operative reports, pathology results, and follow-up documentation. Twenty-three patients (12 male, 11 female; mean age 6.6 years) were included in this series. Lesions were present at birth in all 23 children. Pain and ulceration were the most common symptoms reported, and most lesions demonstrated progressive enlargement. All were solitary and located on the limbs (n=19) or trunk (n=4), with a predilection for the lower limb. Imaging suggested low-flow vascular malformation in 14 patients. Twenty children underwent surgical excision. Excision techniques included elliptical excision, rhomboid flap reconstruction, and, in extensive lesions, staged excision with dermal regeneration templates, negative pressure wound therapy (NPWT) and split-thickness skin grafts (STSG). Adjunctive therapies were trialled in seven patients, including laser treatments such as Pulsed Dye Laser (PDL), Fractional Carbon Dioxide Laser (CO₂) and Neodymium-Doped Yttrium Aluminum Garnet Laser (Nd:YAG), microneedling, sclerotherapy, and topical sirolimus. Histopathology confirmed VVM in 19/21 children; immunohistochemistry showed GLUT1 positivity in 18/19 and D2-40 in 8/19. MAP3K3 mutations were identified in 8/20 patients and a KRIT1 variant in one. Postoperative recovery was uncomplicated in most lesions. No major complications or functional impairments were observed, and most patients achieved satisfactory aesthetic and functional outcomes. Quality-of-life assessment using the BBSIP demonstrated consistent functional and psychosocial benefit. VVMs present diagnostic and therapeutic challenges due to their overlap with other vascular anomalies and variable histopathologic and molecular features. Surgical excision remains the cornerstone of treatment, with reconstructive strategies such as biodegradable temporizing matrixes (BTM) and STSG aiding closure in extensive or anatomically complex lesions. Adjunctive therapies, including laser and microneedling, may further enhance postoperative cosmetic outcomes. Importantly, BBSIP assessment confirmed that surgery offers substantial functional and psychosocial improvements. A multidisciplinary, individualized approach is essential to achieving durable symptom relief, minimizing recurrence, and optimising care for these rare and complex lesions.

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Journal
Journal of Pediatric Surgery Open
Published
2026-01-15
DOI
https://doi.org/10.1016/j.yjpso.2026.100265
Primary Topic
Vascular Malformations and Hemangiomas
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article
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article

Verrucous venous malformations in children: A case for surgical excision

Halah Hassan, Hannah Triscott, Roy M. Kimble
Journal of Pediatric Surgery Open
Vascular Malformations and Hemangiomas
article

Verrucous venous malformations in children: A case for surgical excision

Halah Hassan, Hannah Triscott, Roy M. Kimble
article en

Abstract

• Large, paediatric surgical series involving VVM management demonstrating durable outcomes and disease control with excision • Advanced reconstructive strategies enabled closure in complex, extensive lesions • Somatic MAP3K3 variants and GLUT1 positivity support molecular–histopathologic spectrum of VVMs Verrucous venous malformations (VVMs) are rare congenital vascular anomalies characterized by ectatic venous channels in the dermis and subcutis, with overlying verrucous epidermal changes. Typically presenting in early childhood, they may cause pain, bleeding, infection, and psychosocial distress. Diagnosis and management are often difficult due to clinical and histopathologic overlap with other vascular lesions. Non-surgical therapies have been trialled with limited success, and surgical excision remains the primary treatment. However, the efficacy of surgery, particularly when combined with reconstructive techniques, has not been systematically compared to alternative modalities. Twenty-three paediatric patients (aged one month to 17 years) with congenital or early-onset vascular lesions suspected to be VVMs were evaluated at a tertiary vascular anomalies clinic between 2019 and 2025. Clinical presentation, imaging, histopathology, IHC, and NGS results of a 32-gene vascular anomaly panel were reviewed. All patients underwent biopsy or excision. Data were collected retrospectively from operative reports, pathology results, and follow-up documentation. Twenty-three patients (12 male, 11 female; mean age 6.6 years) were included in this series. Lesions were present at birth in all 23 children. Pain and ulceration were the most common symptoms reported, and most lesions demonstrated progressive enlargement. All were solitary and located on the limbs (n=19) or trunk (n=4), with a predilection for the lower limb. Imaging suggested low-flow vascular malformation in 14 patients. Twenty children underwent surgical excision. Excision techniques included elliptical excision, rhomboid flap reconstruction, and, in extensive lesions, staged excision with dermal regeneration templates, negative pressure wound therapy (NPWT) and split-thickness skin grafts (STSG). Adjunctive therapies were trialled in seven patients, including laser treatments such as Pulsed Dye Laser (PDL), Fractional Carbon Dioxide Laser (CO₂) and Neodymium-Doped Yttrium Aluminum Garnet Laser (Nd:YAG), microneedling, sclerotherapy, and topical sirolimus. Histopathology confirmed VVM in 19/21 children; immunohistochemistry showed GLUT1 positivity in 18/19 and D2-40 in 8/19. MAP3K3 mutations were identified in 8/20 patients and a KRIT1 variant in one. Postoperative recovery was uncomplicated in most lesions. No major complications or functional impairments were observed, and most patients achieved satisfactory aesthetic and functional outcomes. Quality-of-life assessment using the BBSIP demonstrated consistent functional and psychosocial benefit. VVMs present diagnostic and therapeutic challenges due to their overlap with other vascular anomalies and variable histopathologic and molecular features. Surgical excision remains the cornerstone of treatment, with reconstructive strategies such as biodegradable temporizing matrixes (BTM) and STSG aiding closure in extensive or anatomically complex lesions. Adjunctive therapies, including laser and microneedling, may further enhance postoperative cosmetic outcomes. Importantly, BBSIP assessment confirmed that surgery offers substantial functional and psychosocial improvements. A multidisciplinary, individualized approach is essential to achieving durable symptom relief, minimizing recurrence, and optimising care for these rare and complex lesions.

Journal of Pediatric Surgery OpenVol. 14
The University of Queensland (AU), Children's Health Queensland Hospital and Health Service (AU)
Openalex Percentile: Top 87%
Vascular Malformations and Hemangiomas
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