Objective:To analyze the patterns of intercellular communication in facioscapulohumeral muscular dystrophy (FSHD) by single-cell nuclear transcriptome sequencing.Methods:Bilateral asymmetrical lesions mouth orbicular muscle of two patients with FSHD and mouth orbicular muscle of two healthy patients were selected. Six samples were obtained, and were divided into control group, mild group and severe group. The normal orbicularis muscle sample was collected from 2 healthy individuals (the control group). The muscle samples in the mild group were from two patients with relatively normal muscle sides, and the samples in the severe group were from two patients with more severe muscle damage sides. Single-cell nuclear transcriptome sequencing was performed on all cells of the three groups. Reduced dimension clustering and cell definition were performed to identify differentially expressed genes and enrichment pathways. Intercellular communication patterns among major cell types and key signaling pathways were explored by cellular communication analysis.Results:Differential gene expression analysis of FSHD bilateral muscle samples identified 46 functionally differentially expressed genes associated with the disease in different cell types, related to apoptosis, oxidative stress, immune inflammation, and muscle function. Intercellular communication was generally increased in the severe group. Fibro-adipogenic progenitors (FAPs) and macrophages are important signaling sources in the abnormal muscle microenvironment of FSHD and are closely associated with disease progression. There are six unique signaling pathways in the mild group, including bone morphogenetic proteins (BMP), transforming growth factor-β (TGF-β), CXC motif chemokine ligand (CXCL), adhesion G protein-coupled receptor E5 (ADGRE5), interleukin-16 (IL-16), and wingless-type MMTV integration site family (WNT) signaling pathways. These signaling pathways are mainly involved in the interaction between macrophages, FAPs, and adipocytes and may be involved in the regulation of fat deposition and fibrosis changes in the diseased muscle.Conclusions:Single-cell nuclear transcriptome sequencing provides a relatively comprehensive pattern of intercellular communication between key cell types in FSHD, providing an appropriate reference for understanding the intercellular regulatory mechanisms of the FSHD muscle microenvironment.

Objective:To investigate the effects of asymmetric trans-sutural distraction osteogenesis for severe midfacial hypoplasia in growing patients with unilateral cleft lip and palate.Methods:All the growing patients with unilateral cleft lip and palate combining severe midfacial hypoplasia treated with asymmetric trans-sutural distraction osteogenesis from January 2011 to December 2020 in the Plastic Surgery Hospital, Chinese Academy of Medical Sciences were analyzed retrospectively. The distraction system consisted of the bone-borne traction hooks, nickel-titanium shape memory alloy spring and rigid external distractor. Asymmtric distraction could be achieved by exerting different traction force to bilateral maxilla. The technique for relapse prevention included moderate overcorrection, prompt orthodontic treatment and face mask therapy. The changes of midfacial symmetry between the cleft and non-cleft side were analyzed by comparing the distance of subspinale A-point(A), intersection of lateral border of piriform aperture and plane parallel to midsagittal reference plane(LPA), most inferior point on nasomaxillary suture(INM) and most inferior point on profile of pterygomaxillary suture(PTM) to coronal reference plane between preoperative and postoperative, and the asymmetric ratio of LPA, INM and PTM were calculated.Results:A total of 38 patients, (34 males and 4 females, aged from 8-15 years) were included. All of the patients completed the distraction and achieved a satisfied improvement in the midface convexity and overjet. The postoperative 1-3 years follow-up showed the midface protrusion and occlusal overcoverage after distraction caused by overcorrection tended to become harmonious in the following 6 to 12 months, and 3 patients with no face mask therapy done had relapse on maxilla in various degrees after 3 years follow-up. The measurement findings of 25 patients showed the average advancement of A point was (12.67±4.62) mm(range, 6.21-23.28 mm). The distances of INM, LPA and PTM to CR plane on the cleft side were significantly less than that on the non-cleft side before the distraction( P<0.05). However, after the distraction, these distance differences of INM and PTM between the cleft and non-cleft side were eliminated ( P>0.05). The distance difference of bilateral LPA was reduced, but still significant less on the cleft side( P<0.05). Compared before and after distraction, the asymmetric rate of INM, LPA and PTM was significantly decreased( P<0.05). Conclusions:Asymmetric trans-sutural distraction osteogenesis offers an effective method for the early treatment of severe midfacial hypoplasia in growing patients with unilateral cleft lip and palate, and restores the midfacial symmetry.

Objective:To investigate the effects of asymmetric trans-sutural distraction osteogenesis for severe midfacial hypoplasia in growing patients with unilateral cleft lip and palate.Methods:All the growing patients with unilateral cleft lip and palate combining severe midfacial hypoplasia treated with asymmetric trans-sutural distraction osteogenesis from January 2011 to December 2020 in the Plastic Surgery Hospital, Chinese Academy of Medical Sciences were analyzed retrospectively. The distraction system consisted of the bone-borne traction hooks, nickel-titanium shape memory alloy spring and rigid external distractor. Asymmtric distraction could be achieved by exerting different traction force to bilateral maxilla. The technique for relapse prevention included moderate overcorrection, prompt orthodontic treatment and face mask therapy. The changes of midfacial symmetry between the cleft and non-cleft side were analyzed by comparing the distance of subspinale A-point(A), intersection of lateral border of piriform aperture and plane parallel to midsagittal reference plane(LPA), most inferior point on nasomaxillary suture(INM) and most inferior point on profile of pterygomaxillary suture(PTM) to coronal reference plane between preoperative and postoperative, and the asymmetric ratio of LPA, INM and PTM were calculated.Results:A total of 38 patients, (34 males and 4 females, aged from 8-15 years) were included. All of the patients completed the distraction and achieved a satisfied improvement in the midface convexity and overjet. The postoperative 1-3 years follow-up showed the midface protrusion and occlusal overcoverage after distraction caused by overcorrection tended to become harmonious in the following 6 to 12 months, and 3 patients with no face mask therapy done had relapse on maxilla in various degrees after 3 years follow-up. The measurement findings of 25 patients showed the average advancement of A point was (12.67±4.62) mm(range, 6.21-23.28 mm). The distances of INM, LPA and PTM to CR plane on the cleft side were significantly less than that on the non-cleft side before the distraction( P<0.05). However, after the distraction, these distance differences of INM and PTM between the cleft and non-cleft side were eliminated ( P>0.05). The distance difference of bilateral LPA was reduced, but still significant less on the cleft side( P<0.05). Compared before and after distraction, the asymmetric rate of INM, LPA and PTM was significantly decreased( P<0.05). Conclusions:Asymmetric trans-sutural distraction osteogenesis offers an effective method for the early treatment of severe midfacial hypoplasia in growing patients with unilateral cleft lip and palate, and restores the midfacial symmetry.

Objective:To evaluate the aesthetic outcome of the pretarsal orbicularis-tarsus fixation technique in incisional double eyelid blepharoplasty.Methods:Postoperative results were evaluated from 798 patients who underwent the orbicularis-tarsus fixation blepharoplasty from January 2015 to December 2018. Based on an incisional maneuver, soft tissue was carefully removed to expose the superior edge of tarsus, then the pretarsal orbicularis oculi muscle was anchored on the tarsus. For skin closure, both skin margins, a small bite of subcutaneous tissue and the pretarsal fascia were interruptedly sutured to enhance cicatricial adhesion.Results:All patients successfully underwent double eyelidplasty using this modified technique. The follow-up period ranged from two to forty-six months, with a mean period of twenty months. Ninety-six percent of the patients were satisfied with postoperative outcomes. Twenty-two cases of palpebral fold asymmetry and ten cases of unsatisfactory fold formation near the inner canthus were encountered. However, all the defects had been well improved by minor revisions. No supratarsal crease drooping or disappearing, suture spitting out, scar hypertrophy or crease depression were observed postoperatively.Conclusions:The orbicularis-tarsus fixation method is a reliable technique for double eyelid plasty. It enables high feasibility and long-lasting result, with lower risk of postoperative complications.

Following repairing cleft lip by labial-nasal muscle tension bands reconstruction, our team analyzed six groups of velopharyngeal muscles by Micro-CT scanning and three-dimensional reconstruction, including levator veli palatini muscle, palatopharyngeus muscle, tensor veli palatini, palatoglossus, uvularis and superior constrictor of pharynx. It was found that palatopharyngeus and levator veli palatini cooperate with each other to regulate the movement of the soft palate. The structure around the pterygoid hamulus was a fine pulley-like structure that should not be destroyed easily. The velopharyngeal muscle tension bands were proposed by analyzing the biomechanics and spatial structure of six groups of muscles. The main muscle tension band was composed of palatine aponeurosis and uvularis. The first auxiliary tension band was composed of levator veli palatini and palatopharyngeus. The second auxiliary tension band was composed of the palatopharyngeus and superior constrictor of the pharynx. A new cleft palate repair technique based on reconstruction of the velopharyngeal muscle tension bands was proposed. This new method reconstructed the biomechanical relationship of the three groups of muscle tension bands and protected the structure around the pterygoid hamulus to reduce the damage. Biomechanical simulation of velopharyngeal muscle was realized primarily.

Objective:To present the clinical results and potential complications of modified maxillary distraction osteogenesis in the treatment of moderate to severe maxillary hypoplasia for patients with cleft lip and palate.Methods:All the cases were treated with a modified distraction osteogenesis in the Plastic Surgery Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College from July 2017 to December 2019. A rigid external distraction(RED) with intranasal bone-borne traction hooks was performed after the Le Fort Ⅰ osteotomy and a maxillary internal rigid fixation was done immediately after 3-4 weeks of RED device distraction. Paired-samples t-test was used to analyze the three-dimensional reconstruction and measurements of the patient’s preoperative (T0) and immediate completion of traction (T1) cranial CT maxillofacial bony structures. Results:Fifteen patients (12 males and 3 females, ranging from 14 to 25 years in age) with moderate to severe maxillary hypoplasia were analyzed retrospectively. There were significant differences ( P<0.05) in all measures of maxillary and mandibular morphology at the completion of distraction compared to preoperatively. The mean advancement of A point (subspinale) was (10.69±9.01) mm, and the mean increase in ANB (subspinale-nasion-supramental) was (13.53±7.14)°. The average increase of alveolar plane and mandibular plane was (3.97±5.87)° and (4.65±3.67)° separately. The average growth of anterior facial height was (5.63±4.41) mm. The maxilla moved forward and downward, increasing the midface prominence and improving the facial contour. The traction process had an effect on the position of the mandible, with 5 in 15 patients experiencing moderate to severe decreased mouth opening, and the mouth opening limitation was relieved by mouth opening training after fixation at the end of distraction. One patient experienced temporomandibular joint (TMJ) dislocation, which was relieved by manual repositioning and mouth opening training after maxillary fixation and sagittal split ramus osteotomy. Conclusions:Modified maxillary distraction osteogenesis can effectively advance the maxilla, allowing clockwise rotation of mandible, improving facial contour, and significantly shortening the traction time. Limited mouth opening and TMJ dislocation may occur during traction.

Following repairing cleft lip by labial-nasal muscle tension bands reconstruction, our team analyzed six groups of velopharyngeal muscles by Micro-CT scanning and three-dimensional reconstruction, including levator veli palatini muscle, palatopharyngeus muscle, tensor veli palatini, palatoglossus, uvularis and superior constrictor of pharynx. It was found that palatopharyngeus and levator veli palatini cooperate with each other to regulate the movement of the soft palate. The structure around the pterygoid hamulus was a fine pulley-like structure that should not be destroyed easily. The velopharyngeal muscle tension bands were proposed by analyzing the biomechanics and spatial structure of six groups of muscles. The main muscle tension band was composed of palatine aponeurosis and uvularis. The first auxiliary tension band was composed of levator veli palatini and palatopharyngeus. The second auxiliary tension band was composed of the palatopharyngeus and superior constrictor of the pharynx. A new cleft palate repair technique based on reconstruction of the velopharyngeal muscle tension bands was proposed. This new method reconstructed the biomechanical relationship of the three groups of muscle tension bands and protected the structure around the pterygoid hamulus to reduce the damage. Biomechanical simulation of velopharyngeal muscle was realized primarily.

Objective:To present the clinical results and potential complications of modified maxillary distraction osteogenesis in the treatment of moderate to severe maxillary hypoplasia for patients with cleft lip and palate.Methods:All the cases were treated with a modified distraction osteogenesis in the Plastic Surgery Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College from July 2017 to December 2019. A rigid external distraction(RED) with intranasal bone-borne traction hooks was performed after the Le Fort Ⅰ osteotomy and a maxillary internal rigid fixation was done immediately after 3-4 weeks of RED device distraction. Paired-samples t-test was used to analyze the three-dimensional reconstruction and measurements of the patient’s preoperative (T0) and immediate completion of traction (T1) cranial CT maxillofacial bony structures. Results:Fifteen patients (12 males and 3 females, ranging from 14 to 25 years in age) with moderate to severe maxillary hypoplasia were analyzed retrospectively. There were significant differences ( P<0.05) in all measures of maxillary and mandibular morphology at the completion of distraction compared to preoperatively. The mean advancement of A point (subspinale) was (10.69±9.01) mm, and the mean increase in ANB (subspinale-nasion-supramental) was (13.53±7.14)°. The average increase of alveolar plane and mandibular plane was (3.97±5.87)° and (4.65±3.67)° separately. The average growth of anterior facial height was (5.63±4.41) mm. The maxilla moved forward and downward, increasing the midface prominence and improving the facial contour. The traction process had an effect on the position of the mandible, with 5 in 15 patients experiencing moderate to severe decreased mouth opening, and the mouth opening limitation was relieved by mouth opening training after fixation at the end of distraction. One patient experienced temporomandibular joint (TMJ) dislocation, which was relieved by manual repositioning and mouth opening training after maxillary fixation and sagittal split ramus osteotomy. Conclusions:Modified maxillary distraction osteogenesis can effectively advance the maxilla, allowing clockwise rotation of mandible, improving facial contour, and significantly shortening the traction time. Limited mouth opening and TMJ dislocation may occur during traction.

Birth defects is one of the most important problems of human society. External birth defect is one of the major research fields of plastic surgery. This kind of disorders damages the body image of patients in mild or severe way, affects severely the social confidence of the patients themselves and their families. we summarized the research progress of the common external birth defects (the incidence top 10) from the aspects of pathogenesis, prevention, treatments and so on, in order to provide reference materials to the prevention and treatment of them.

Due to insufficient understanding of the mechanism of the lesser-form cleft lip in clinical practice, the " muscle anatomical reduction" method, which is the general treatment option for the typical cleft lip, is also preferred for the treatment of lesser-form cleft lip, and the repair effect is often far from the patient′s expectation. Based on the anatomical study and clinical observation of the lesser-form cleft lip in more than ten years, the author systematically describes the characteristics regarding the disarrangement of muscle and explains the confusing clinical features of the lesser-form cleft lip. In addition, the pathological mechanism of the lesser-form cleft lip is further classified, and the muscle biomechanical biomimetic repair technique of this defect-" Yin′s nasolabial muscle frame" is proposed to guide clinical operations. This article preliminarily summarizes the author′s new understanding of the lesser-form cleft lip in recent years. The nasolabial Muscle Tension Lines Group(MTLG) theory is applied to analyze and solve puzzles in the lesser-form cleft lip. This process itself also serves as a clinical practice test of the MTLG theory.