Clinical Factors in Trunk Capillary Malformations in the Neonate: When to Suspect Other Associated Malformations? A Case–Control Study

Abstract

Introduction Capillary malformations (CMs) can be sporadic or syndromic, in association with other underlying venous malformation (VM) or lymphatic malformation (LM). The objective of this study is to describe the clinical patterns in the neonate that allow us to differentiate sporadic CMs from those associated with other vascular malformations.

Materials and Methods A case–control study was performed in neonates with CM located in the trunk, followed at our institution between 2008 and 2018. The patients were divided into two groups: group A (cases: CM associated with VM or LM) and group B (controls: sporadic CM without associated malformations). Demographic and clinical variables collected in the clinical history were evaluated (color, location, multifocality, bilaterality, position regarding the vascular axis, and involvement of the midline).

Results Thirty-eight patients were included (18 cases and 20 controls) without differences in gender and age. In group A, the totality of patients presented CM with uniform color and lateral location (p < 0.001). In this group, bilateral and multifocal involvements were lower than in group B, without significant differences between both groups. The distribution of CMs in group A was always parallel to the vascular axis and the midline was always respected, without observing these characteristics in the group B (p < 0.001).

Conclusion The presence of a CM in the trunk of a neonate with uniform color, lateral location, parallel position to the vascular axis, and absence of involvement of the midline should make us suspect other underlying vascular malformations, which should be studied with complementary tests.

Keywords

capillary malformations - vascular malformations - neonates - case–control study

• References

• 1 Jacobs AH, Walton RG. The incidence of birthmarks in the neonate. Pediatrics 1976; 58 (02) 218-222
• 2 Rozas-Muñoz E, Frieden IJ, Roé E, Puig L, Baselga E. Vascular stains: proposal for a clinical classification to improve diagnosis and management. Pediatr Dermatol 2016; 33 (06) 570-584
• 3 Boon LM, Ballieux F, Vikkula M. Pathogenesis of vascular anomalies. Clin Plast Surg 2011; 38 (01) 7-19
• 4 Uller W, Fishman SJ, Alomari AI. Overgrowth syndromes with complex vascular anomalies. Semin Pediatr Surg 2014; 23 (04) 208-215
• 5 López-Gutiérrez JC, Redondo P, Ivars M. Fingertip capillary malformation and associated disorders: report of 9 cases. Pediatrics 2017; 140 (01) e20162967
• 6 Dasgupta R, Fishman SJ. ISSVA classification. Semin Pediatr Surg 2014; 23 (04) 158-161
• 7 Maari C, Frieden IJ. Klippel-Trénaunay syndrome: the importance of “geographic stains” in identifying lymphatic disease and risk of complications. J Am Acad Dermatol 2004; 51 (03) 391-398
• 8 Waelchli R, Aylett SE, Robinson K, Chong WK, Martinez AE, Kinsler VA. New vascular classification of port-wine stains: improving prediction of Sturge-Weber risk. Br J Dermatol 2014; 171 (04) 861-867
• 9 Sillard L, Léauté-Labreze C, Mazereeuw-Hautier J. , et al. Medial fronto-facial capillary malformations. J Pediatr 2011; 158 (05) 836-841
• 10 Yang B, Li L, Zhang LX, Sun YJ, Ma L. Clinical characteristics and treatment options of infantile vascular anomalies. Medicine (Baltimore) 2015; 94 (40) e1717
• 11 Azizkhan RG. Complex vascular anomalies. Pediatr Surg Int 2013; 29 (10) 1023-1038
• 12 Behravesh S, Yakes W, Gupta N. , et al. Venous malformations: clinical diagnosis and treatment. Cardiovasc Diagn Ther 2016; 6 (06) 557-569
• 13 Hage AN, Chick JFB, Srinivasa RN. , et al. Treatment of venous malformations: the data, where we are, and how it is done. Tech Vasc Interv Radiol 2018; 21 (02) 45-54
• 14 Trenor III CC, Chaudry G. Complex lymphatic anomalies. Semin Pediatr Surg 2014; 23 (04) 186-190