Perkins, Jonathan A.

ObjectiveThe Lymphatic Malformation Function (LMF) instrument is a preliminary parent‐report assessment designed to measure outcomes in children with cervicofacial lymphatic malformation (LM). This study aimed to assess the measurement properties of the LMF, refine it, test criterion validity, and evaluate the test‐retest reliability.Study DesignCross‐sectional.SettingTwo pediatric tertiary referral centers.SubjectsParents of 60 children from 6 months to 15 years old with cervicofacial LM.MethodsParents were recruited via mail and online. The LMF was administered on paper or online initially and again within 21 days. Response distributions and interitem correlations were examined for item reduction. Exploratory factor analysis was conducted on retained items. Cronbach’s α, Spearman correlation, and intraclass correlation (ICC) coefficients were calculated to test internal consistency, criterion validity (compared to stage), and test‐retest reliability, respectively.ResultsOne item was removed due to a floor effect. The response scale was collapsed from a 5‐point scale to a 3‐point scale due to skewness. Six items were discarded due to redundancy (interitem correlations >0.7); 2 items were discarded due to factor loadings <0.4. Exploratory factor analysis revealed a 2‐factor structure explaining 84% of variance, and the domains Signs and Impacts had good internal consistency (all Cronbach’s α >0.80 and <0.90), significant association with stage (P <. 05), and good overall test‐retest reliability (ICC, 0.82).ConclusionThe LMF has been refined into a 12‐item, 2‐domain instrument measuring LM‐specific signs and impacts with internal consistency, criterion validity, and test‐retest reliability.

ObjectiveTo develop general and site‐specific treatment effect and outcome measures to standardize the reporting of head and neck lymphatic malformation (HNLM) treatments.Study DesignConsensus statement/expert opinion.SettingMultiple tertiary academic institutions.Subjects and MethodsThe modified Delphi method is an iterative process of collecting expert opinions, refining opinions through discussion and feedback, statistically aggregating opinions, and using these aggregates to generate consensus opinion in the absence of other data. The modified Delphi method was used by a multi‐institutional group of otolaryngology and interventional radiology experts in the field of vascular anomalies to formulate a list of recommended reporting outcomes for the study and treatment of head and neck lymphatic malformations.ResultsThrough 3 rounds of iteration, 10 expert panelists refined 98 proposed outcome measures and 9 outcome categories to a final consensus set of 50 recommended outcome measures in 3 global categories (general, demographics, and treatment complications) and 5 site‐specific categories (orbit, oral cavity, pharynx, larynx, and neck).ConclusionsWe propose the first consensus set of standardized reporting measures for clinical and treatment outcomes in studies of HNLMs. Consistent outcome measures across future studies will facilitate comparison of treatment options and allow systematic review. We hope that these guidelines facilitate the design and reporting of subsequent HNLM studies.

ObjectiveTo propose an anatomic classification for fetal nuchal lymphatic anomalies that will be clinically useful and to evaluate the classification’s value in predicting chromosomal abnormalities, pregnancy outcomes, other associated fetal anomalies, and spontaneous resolution of these lesions.Study DesignRetrospective cohort study.SettingTertiary academic hospital and affiliated tertiary children’s hospital.Subjects and MethodsMother‐baby pairs diagnosed with fetal nuchal lymphatic anomalies in a prenatal ultrasound database. Anomalies were classified as nuchal thickening, dorsal lymphatic malformation, or ventral lymphatic malformation. Pregnancy outcomes, prevalence of chromosomal and anatomic abnormalities, and rates of spontaneous lesion resolution were determined for each group.ResultsThe study included 189 patients: 58 with nuchal thickening, 120 with dorsal lymphatic malformation, and 11 with ventral lymphatic malformation. In fetuses for whom chromosomal analysis was available, chromosomal abnormalities were strongly associated with dorsal lymphatic malformations (83%), less associated with nuchal thickening (29%), and not associated with ventral lymphatic malformations. Dorsal lymphatic malformation predicted high rates of elective (43%) and spontaneous (20%) termination of pregnancy and showed the strongest association with cardiac, renal, and skeletal anomalies. Nuchal thickening was more likely to resolve in utero than dorsal lymphatic malformations, while no ventral lymphatic malformation resolved spontaneously.ConclusionsFetal nuchal anomalies demonstrate significant and clinically important prognostic differences depending on their anatomic location. The simple classification system proposed here therefore provides useful information to clinicians involved in the pre‐ and postnatal management of children with these anomalies.