Venous ulceration in a young adolescent in relation to a congenital vascular tumor

ARTICLE

Auteur(s) : Lenny AG Jacobs-Sibelt¹, Paul NMA Rieu², Carine JM Van der Vleuten¹

¹Departments of Dermatology, Radboud University Nijmegen Medical Centre, PO Box 9101, 6500 HB Nijmegen, The Netherlands
²Pediatric Surgery, Radboud University Nijmegen Medical Centre, PO Box 9101, 6500 HB Nijmegen, The Netherlands

A 13-year-old adolescent boy was presented to our department with ulceration on the anterior lateral part of his lower left leg since he was 12 years old. We saw a moderately demarcated erythematosquamous hyperpigmented atrophic area of about 12 × 8 cm with central crusts and erosions (figure 1A). Proximally, an insufficient great saphenous vein and a perforating vein from the tibal anterial vein at the lateral-anterior part were seen on ultrasound investigations. Both were passing their incompetence to tributaries in the regressed tumor (figure 1B).

The patient told us that this ulcer was localized exactly in the region of an earlier regressed “hemangioma”. The vascular tumor was congenital and involuted spontaneously during the first years of his life. Photographs taken in the first week after birth showed a large indurated tumor with central erythema on the lower part of the left leg (figure 1C).

Ligation of the sapheno-femoral junction, short strip of the great saphenous vein and perforantectomy of the perforating vein were performed and the ulceration healed shortly after. Two years later our patient is still without any complaints.

Since the vascular anomaly in our patient spontaneously regressed within 2 years, the lesion is a vascular tumor and not a vascular malformation, according to the Mulliken classification system [1-3].

Different types of vascular tumor exist. Our patient had a congenital vascular tumor with spontaneous involution, most likely a so-called “Rapid Involuting Congenital Hemangioma” (RICH) [1, 2] and not a classical hemangioma of infancy.

Ulceration is relatively common in vascular tumors, especially classical infantile hemangiomas [4], but uncommon in adolescents with already-regressed congenital vascular tumors like RICH. We hypothesize that the varicose veins may have evolved in relation to the tumor, resulting in venous insufficiency causing subsequent ulceration. A large vascular tumor needs a higher arterial input and venous outflow. This may have resulted in a venous wall dilatation leading to valve dysfunction in our patient. Recent literature shows that dilatation of the vein can result in valve incompetence [5]. Adequate treatment of the varicose veins resulted in healing of the ulceration, implying an important cause of the ulceration in this patient.

In conclusion, we describe the first case of a triad of a regressed congenital vascular tumor, most likely RICH, varicose veins and ulcerations in a young adolescent. The increased blood supply in infancy probably resulted in venous reflux in adolescence, giving rise to venous ulceration at a very young age. Adequate treatment of the varicose veins caused complete healing of the ulceration, without relapse to date.

Acknowledgements

References

2 Enjolras O, Wassef M, Chapot R. Color atlas of vascular tumors and vascular malformations. New York: Cambridge University Press, 2007.

3 Mulliken JB, Glowacki J. Hemangiomas and vascular malformations in infants and children: a classification based on endothelial characteristics. Plast Reconstr Surg 1982; 69: 412-22.

4 Hermans DJ, Boezeman JB, Van de Kerkhof PC, et al. Differences between ulcerated and non12 ulcerated hemangiomas, a retrospective study of 465 cases. Eur J Dermatol 2009; 19: 152-6.

5 Raffetto JD, Khalil RA. Mechanisms of varicose vein formation: valve dysfunction and wall dilation. Phlebology 2008; 23: 85-98.