Lymphatic malformations of the head and neck: introduction of a disease score for children, Cologne Disease Score (CDS).
OBJECTIVE
A clinical staging system for children with lymphatic malformations that would allow for a standardized comparison of disease and treatment outcomes.


METHODS
We developed an examination sheet for the determination of a disease score ranging from 0 (worst) points to 10 (best) points, Cologne Disease Score (CDS). Disfigurement, dysphagia, dysphonia, dyspnea and an observer statement towards progression were contributing to CDS. Each parameter yielded two, one or zero points. Two points were given when no limitation was seen in the patient concerning the respective item. One point was given at mild limitation and zero points were given when considerable limitation in the respective item could be observed. We evaluated 26 patients with lymphatic malformations of the head and neck retrospectively by completing one examination sheet for each patient-visit.


RESULTS
Four patients had an initial CDS value of three or less points (severe disease-group). After therapy the mean value of their score increased only slightly to 3.9+/-2.6 points. Six patients had an initial CDS value of four, five or six points (advanced disease-group). After treatment their mean score value increased dramatically to 9.0+/-1.5 points (P<0.001). Sixteen patients initially had seven points or higher (moderate disease-group), they had 9.8+/-0.4 points after treatment.


CONCLUSIONS
The evaluation of the CDS was easy and expeditious. The score itself was a good predictor in view of the outcome. This new disease score for paediatric patients might be appropriate to evaluate therapeutic trials in paediatric patients.
Wittekindt
Claus
C
Clinic of Otorhinolaryngology, Head and Neck Surgery, University of Cologne, Cologne, Germany. claus.wittekindt@uni-koeln.de
Michel
Olaf
O
Streppel
Michael
M
Roth
Bernhard
B
Quante
Gero
G
Beutner
Dirk
D
Guntinas-Lichius
Orlando
O
D000293:Adolescent; D000328:Adult; D000970:Antineoplastic Agents / Q000008:administration & dosage; D002648:Child; D002675:Child, Preschool; D003680:Deglutition Disorders / Q000473:pathology; D004417:Dyspnea / Q000473:pathology; D005260:Female; D006257:Head; D006258:Head and Neck Neoplasms / Q000188:drug therapy / Q000473:pathology* / Q000601:surgery; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008202:Lymphangioma / Q000188:drug therapy / Q000473:pathology* / Q000601:surgery; D044148:Lymphatic Abnormalities / Q000473:pathology* / Q000601:surgery; D008297:Male; D008875:Middle Aged; D009333:Neck; D010844:Picibanil / Q000008:administration & dosage; D011237:Predictive Value of Tests; D012720:Severity of Illness Index; D014832:Voice Disorders / Q000473:pathology
D000970:Antineoplastic Agents; D010844:Picibanil