Association between malformation type, location and functional deficits in lymphatic malformations of the head and neck in children

Abstract

Purpose: Lymphatic malformations (LM) are congenital malformations of the lymphatic system, mainly located in the head and neck area. They can be staged based on location according to de Serres and based on different morbidity items using the Cologne Disease Score (CDS), a clinical staging system. In many cases, functional impairment greatly affects the life of patients suffering from lymphatic malformations. The present study aims to analyze a cohort of pediatric patients with LM.

Methods: A retrospective analysis of 144 pediatric patients with head and neck LM was performed. Location, type of malformation (microcystic, macrocystic, mixed), scoring according to two different scoring systems and therapy were analyzed. Kruskal-Wallis test was used to analyze the difference in CDS between the patient groups and Dunn's test was used for post-hoc pairwise comparison.

Results: The average age at presentation was 6.1 years. The most common sites were neck (47%), cheek/parotid gland (26%), tongue (17%) and orbit (8%). Macrocystic malformations dominated the lateral neck, while microcystic malformations were predominantly localized in the tongue and floor of mouth. Macrocystic malformations (mean CDS 9.44) were associated with significantly better CDS than microcystic (mean CDS 7.11) and mixed (mean CDS 5.71) malformations (p < 0.001). LM in stage V according to de Serres had the lowest values (mean CDS: 4.26). The most common therapeutic procedures were conventional surgical (partial) resection, laser therapy and sclerotherapy with OK-432.

Conclusions: There is an association between malformation type, location according to de Serres and CDS in children with LM of the head and neck. Patients with microcystic and mixed malformations in stage V had lowest CDS levels.

Keywords: Cologne disease score; Lymphangioma; Lymphatic malformation; Vascular malformation.

Fig. 1

Age at presentation

Fig. 2

Distribution of macrocystic, microcystic and mixed lymphatic malformations according to the staging system of de Serres et al. [5]

Fig. 3

Distribution of the CDS of macrocystic, microcystic and mixed lymphatic malformations

Fig. 4

Distribution of the CDS according to the staging system of de Serres et al. [5]

Fig. 5

Box plot demonstrating CDS scores for the different stages according to de Serres [5]. Dunn’s test with a Bonferroni corrected alpha of 0.005 indicated significant differences between some of the groups (*p = 0.003, **p < 0.0001)