#Madelungdeformity: Insights into a Rare Congenital Difference Utilizing Social Media

Abstract

PURPOSE: Madelung deformity is extremely rare and surgeons see very few, if any, cases during their surgical career.1 The literature is scarce, and the published small-powered studies inadequately describe functional status, let alone consider the patients’ perspective.2 This is remarkable, since it has been shown that congenital hand differences have a profound and lifelong impact on the physical, mental, and social aspects of patients’ lives.3 The purpose of this study is to provide clinicians with a proof of concept of harnessing social media to assess patient outcomes in rare populations, shed light on the burden that Madelung deformity patients carry, and allow for an in-depth overview of demographic characteristics to comprehend the clinical spectrum of patients. METHODS: Using an universal patient-reported outcome tool entitled Patient-Reported Outcomes Measure Information System (PROMIS), we collaborated with several social media communities to conduct a survey. PROMIS Short Form scores were calculated and compared between unoperated and operated patients. Correlations between the scores were calculated using the Spearman’s Rank correlation coefficient; correlation strength was interpreted as low (<0.3), moderate (0.3–0.5), or high (>0.5). RESULTS: Of the 207 persons that opened the survey, a total of 133 participants (64%) completed the survey. A total of 55 participants (49%) had undergone previous surgical correction of the wrist with a mean age of 20.5 ± 9.5 at first surgery, and 2.4 ± 2.7 surgeries in total. Calculated PROMIS scores for adults were as follows: pain intensity 4.9 ± 2.8, pain interference 57.6 ± 10.0, upper extremity 35.2 ± 8.1, depression 53.8 ± 11.1, anxiety 55.4 ± 11.4, and social participation 42.5 ± 7.7 with no significant differences between operated and unoperated patients. Scores for children included pain intensity 5.0 ± 2.8, pain interference 55.7 ± 11.3, upper extremity function 24.6 ± 10.4, depressive symptoms 57.7 ± 11.3, anxiety 57.3 ± 11.9, and peer relationships 42.2 ± 10.3 with significant more pain interference in operated as opposed to unoperated patients (62.3 ± 5.2 versus 51.0 ± 12.0; P = 0.045). CONCLUSIONS: This first, and largest to date, study of patient-reported outcome measures in Madelung deformity indicates that patients have a poorer quality of life as compared to the general population with impacts on physical, mental, and social health, especially children show severely decreased upper extremity functioning. Upper extremity function shows an inversely high correlation with pain intensity, pain interference, and depression. Utilizing social media, we were able to compensate for the rarity of Madelung deformity by engaging an international audience. REFERENCES: 1. Flatt AE. The Care of Congenital Hand Anomalies. Quality Medical Publishing; 1994. 2. Peymani A, Johnson AR, Dowlatshahi AS, et al. Surgical management of Madelung deformity: a systematic review. Hand (NY). 2019;14:725–734. 3. Franzblau LE, Chung KC, Carlozzi N, et al. Coping with congenital hand differences. Plast Reconstr Surg. 2015;135:1067–1075.