Objective: As the most common feature of Beckwith-Wiedemann syndrome (BWS), macroglossia may influence the quality of life, maxillofacial growth, and speech development of children. The retrospective study aimed to investigate the therapeutic effect of partial glossectomy combined with radiofrequency ablation (RFA) for macroglossia patients in BWS. Methods: A retrospective study was conducted in BWS-derived macroglossia patients who underwent partial glossectomy combined with RFA from May 2019 to January 2021. In total, 35 patients consisting of 17 males and 18 females met the inclusion criteria and underwent surgery by the same plastic surgeon. Demographic characteristics, BWS features, operation details, preoperative and postoperative outcomes, satisfaction evaluations, and subgroup analysis were collected and assessed. Results: Of the 35 patients involved, the average age at the time of surgery was 14.05±8.08 months, and the average surgery duration was 48.17±6.72 minutes. Only 1 patient suffered ventral tongue wound dehiscence, and the rest of the patients did not develop any other complications. The severity and frequency of tongue protrusion, drooling, snoring, and feeding difficulty were significantly ameliorated. The patient’s parents showed satisfaction towards the overall surgery, tongue’s appearance, and tongue’s motor function. Tongue’s height decreased from 32.09±1.16 mm before the operation to 29.29±1.33 mm after the operation. Conclusion: The partial glossectomy combined RFA exerts a safe, effective and viable technique to treat BWS-derived macroglossia.
Aim To investigate the expression profile of long non-coding RNAs (lncRNAs) in closed and patent sutures from single suture craniosynostosis patients and explore their underlying functions. Methods Using a lncRNA microarray assay to determine the differential expression profile of lncRNA in sutures from three single suture craniosynostosis patients (two sagittal, one uni-coronal). We performed the functional analysis and constructed the lncRNA-mRNA co-expression network to explore the biological functions. Results There were 98 up-regulated, 125 down-regulated lncRNAs and 88 up-regulated, 122 down-regulated mRNAs identified in the closed sutures compared to the patent sutures (fold change > 1.5, P < 0.05). GO enrichment and KEGG pathway analyses indicated that altered genes enriched in “extracellular matrix,” “skeletal system development,” and “osteoclast differentiation.” The lncRNAs-mRNAs co-expression network manifested the implication of lncRNA expression profiles in single suture craniosynostosis via interactions with mRNAs. Conclusion This is a novel study to present an expression profile of lncRNAs from craniosynostosis patients. lncRNAs may play a functional role in cranial suture development and premature fusion. The results provide a new perspective on the etiology of single suture craniosynostosis.
Objective: To establish a database and study the growth and development of Chinese children’s nasolabial units by 3-dimensional (3D) white-light scanning technology. Materials and Methods: From January 2018 to January 2019, 528 patients were selected preoperatively in our hospital, without cranial or maxillofacial deformities. The patients were placed in supine positions in a state of sedation, and the authors used a 3D white-light scanner to scan the children’s faces to measure the 3D morphological structure of their nasolabial areas. The results were presented with Graphpad Prism 8.0.2 to explore correlations and analyze trends. Results: The selected Chinese children consisted of 356 males (67.4%) and 172 females (32.6%). All nasolabial subunits grew fastest at 0 to 2 years old and at a slower rate from 2 to 15 years old, whereas the relevant growth of different sex was similar among contemporaries. Conclusions: Three-dimensional white-light scanning can quickly and accurately gain 3D imaging of nasolabial soft tissue to build a normal morphology database. Furthermore, every nasolabial subunit grows fastest at the age of 0 to 2 years.
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