Background: Currently, large-sample epidemiological studies on congenital upper limb differences (CULD) in China are relatively rare. This report presents our centre's experience on the spectrum of diseases and related factors. Methods: Information was collected from patients with CULD who underwent surgical treatment at our centre from September 2018 to October 2023. Data collection included patient name, gender, age, clinical features and diagnosis, family history, parents' age, parents' medical history, pregnancy history and family income. Bivariate relationships between these variables were examined. Results: The average age of the patients was 4 years; 1,398 patients (99%) were aged 9 months to 18 years, and 17 patients (1%) were adults. There were 848 males (60%) and 567 females (40%), with a statistically significant gender distribution (p < 0.05). The most common differences were polydactyly and syndactyly, accounting for 47% and 14%, respectively. Syndromic patients accounted for 14%, and cardiac conditions were the most prevalent non-musculoskeletal issue (55%). First-born patients accounted for 54% of patients, and the average age of parents for first-born patients was 30 ± 5 years for fathers and 29 ± 2 years for mothers. For non-first-born patients, the average age of parents was 33 ± 6 years for fathers and 32 ± 5 years for mothers, with a statistically significant difference between the two groups (p < 0.001). Conclusions: In China, congenital limb differences are still dominated by polydactyly and syndactyly. Heart conditions occur most frequently in patients with syndromes. Level of Evidence: Level IV (Epidemiological).
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