Objective:This study aims to summarize the surgical efficacy of macroglossia reduction combined with radiofrequency ablation of Beckwith-Wiedemann Syndrome (BWS).Methods:A retrospective analysis was conducted on the clinical data of patients with macroglossia who received treatment at the Department of Otolaryngology-Head and Neck Surgery, Xiamen Children’s Hospital between May 2019 and July 2022. All patients with macroglossia underwent either one-stage or staged anterior tongue V-shaped incision massive resection, followed by needle tip low-temperature plasma radiofrequency ablation on the lateral and base of the tongue. The postoperative complications in pediatric patients were monitored, including the incision shape of the tongue body, restoration of tongue body structure and function. MRI scans of the tongue were performed at 3 and 12 months post-operation to evaluate tissue healing, changes in tongue volume, and whether there was successful retraction of the enlarged tongue into the oral cavity for evaluating treatment efficacy (cured or not cured).Results:The study included 70 cases, comprising 31 male and 39 female patients, with an average age of 15.4 months (ranging from 5 months to 4 years old). Among them, 68 cases underwent macroglossia reduction combined with radiofrequency ablation at the same time, and 2 cases underwent macroglossia reduction surgery first in other hospitals, and tongue body radiofrequency ablation was performed one year after the surgery. All children were followed up foran average follow-up time of 18 months (6 to 36 months). There were no serious complications such as massive hemorrhage and tongue hematoma. After the operation, 14 cases of superficial rupture of tongue tip wound were gradually improved after 5-7 days of no oral eating, and recovered after 4-12 weeks of liquid diet. Thirteen cases had suture reactions in the tongue back, which were self-resolving within 2 to 3 weeks with no special treatment. All patients showed significant reduction in tongue size after the surgery, which could be completely inserted into the oral cavity, and the treatment effect was satisfactory.Conclusion:Macroglossia reduction combined with radiofrequency ablation is a feasible and effective method for the treatment of BWS with macroglossia and is recommended for application.

Objective:This study aims to summarize the surgical efficacy of macroglossia reduction combined with radiofrequency ablation of Beckwith-Wiedemann Syndrome (BWS).Methods:A retrospective analysis was conducted on the clinical data of patients with macroglossia who received treatment at the Department of Otolaryngology-Head and Neck Surgery, Xiamen Children’s Hospital between May 2019 and July 2022. All patients with macroglossia underwent either one-stage or staged anterior tongue V-shaped incision massive resection, followed by needle tip low-temperature plasma radiofrequency ablation on the lateral and base of the tongue. The postoperative complications in pediatric patients were monitored, including the incision shape of the tongue body, restoration of tongue body structure and function. MRI scans of the tongue were performed at 3 and 12 months post-operation to evaluate tissue healing, changes in tongue volume, and whether there was successful retraction of the enlarged tongue into the oral cavity for evaluating treatment efficacy (cured or not cured).Results:The study included 70 cases, comprising 31 male and 39 female patients, with an average age of 15.4 months (ranging from 5 months to 4 years old). Among them, 68 cases underwent macroglossia reduction combined with radiofrequency ablation at the same time, and 2 cases underwent macroglossia reduction surgery first in other hospitals, and tongue body radiofrequency ablation was performed one year after the surgery. All children were followed up foran average follow-up time of 18 months (6 to 36 months). There were no serious complications such as massive hemorrhage and tongue hematoma. After the operation, 14 cases of superficial rupture of tongue tip wound were gradually improved after 5-7 days of no oral eating, and recovered after 4-12 weeks of liquid diet. Thirteen cases had suture reactions in the tongue back, which were self-resolving within 2 to 3 weeks with no special treatment. All patients showed significant reduction in tongue size after the surgery, which could be completely inserted into the oral cavity, and the treatment effect was satisfactory.Conclusion:Macroglossia reduction combined with radiofrequency ablation is a feasible and effective method for the treatment of BWS with macroglossia and is recommended for application.

Objective:To investigate the maxillofacial morphological development of children with different types of cleft palate after cleft palate repair.Methods:The clinical data of children who underwent cleft palate repair in the Department of Plastic Surgery, Children’s Hospital of Fudan University from January 2015 to December 2020 and normal children during the same period were retrospectively analyzed. All the included children were treated by the same physician at 8 to 18 months of age with loose incision and Langenbeck repair for cleft palate. X-ray cephalometric data and facial three-dimensional scanning data were obtained from the maxillofacial database of the Plastic Surgery Department of the hospital. According to the type and age of cleft palate, the children with cleft palate were divided into 6 groups: Group 1, 8 months to 1 years old children with grade Ⅰ cleft palate before operation; Group 2, 8 months to 1 year old children with grade Ⅱ-Ⅲ cleft palate before operation; Group 3, 2-3 years old children with grade Ⅰ cleft palate after operation; Group 4, 2-3 years old children with grade Ⅱ-Ⅲ cleft palate after operation; Group 5, 4-6 years old children with grade Ⅰ cleft palate after surgery; Group 6, 4-6 years old children with grade Ⅱ-Ⅲ cleft palate after operation. According to age, normal children were divided into three groups: Group 7, 8 months to 1 year old normal children; Group 8, 2-3 years old normal children; Group 9, 4-6 years old normal children. Analysis of variance was used to compare the measurement data of normal distribution between groups. Dunnett- t test was used to compare the maxillofacial features of children with grade Ⅰ cleft palate and grade Ⅱ-Ⅲ cleft palate with normal children of the same age. Results:A total of 183 children with cleft palate were included, including 84 females and 99 males, aged (3.6±2.1) years. There were 302 normal children, including 114 females and 188 males, aged (4.1±1.9) years. Groups 1 to 9 included 23, 46, 19, 37, 23, 35, 83, 105 and 114 children, respectively. The analysis result showed that the horizontal length, vertical height and angle of maxillary and mandibular were not significantly different from that of normal children of the same age in the preoperative cephalometric and three-dimensional scanning result of the children with grade Ⅰ and Ⅱ-Ⅲ cleft palate. However, the horizontal length, vertical height and angle of maxillary and mandibular were significantly different from that of normal children of the same age at 2-3 years and 4-6 years after surgery. Moreover, the difference between maxillofacial characteristics of children with grade Ⅱ-Ⅲ cleft palate and normal children of the same age is more significant than that of children with grade Ⅰ cleft palate.Conclusion:Maxillofacial morphological development is inhibited at different ages in children with different types of cleft palate after repair, which is manifested as the backward development of maxillofacial horizontal length, vertical height and angle of maxillary and mandible. Moreover, the maxillofacial morphological development inhibition was more serious in children with grade Ⅱ-Ⅲ cleft palate than in children with grade Ⅰ cleft palate.

Objective:To investigate the maxillofacial morphological development of children with different types of cleft palate after cleft palate repair.Methods:The clinical data of children who underwent cleft palate repair in the Department of Plastic Surgery, Children’s Hospital of Fudan University from January 2015 to December 2020 and normal children during the same period were retrospectively analyzed. All the included children were treated by the same physician at 8 to 18 months of age with loose incision and Langenbeck repair for cleft palate. X-ray cephalometric data and facial three-dimensional scanning data were obtained from the maxillofacial database of the Plastic Surgery Department of the hospital. According to the type and age of cleft palate, the children with cleft palate were divided into 6 groups: Group 1, 8 months to 1 years old children with grade Ⅰ cleft palate before operation; Group 2, 8 months to 1 year old children with grade Ⅱ-Ⅲ cleft palate before operation; Group 3, 2-3 years old children with grade Ⅰ cleft palate after operation; Group 4, 2-3 years old children with grade Ⅱ-Ⅲ cleft palate after operation; Group 5, 4-6 years old children with grade Ⅰ cleft palate after surgery; Group 6, 4-6 years old children with grade Ⅱ-Ⅲ cleft palate after operation. According to age, normal children were divided into three groups: Group 7, 8 months to 1 year old normal children; Group 8, 2-3 years old normal children; Group 9, 4-6 years old normal children. Analysis of variance was used to compare the measurement data of normal distribution between groups. Dunnett- t test was used to compare the maxillofacial features of children with grade Ⅰ cleft palate and grade Ⅱ-Ⅲ cleft palate with normal children of the same age. Results:A total of 183 children with cleft palate were included, including 84 females and 99 males, aged (3.6±2.1) years. There were 302 normal children, including 114 females and 188 males, aged (4.1±1.9) years. Groups 1 to 9 included 23, 46, 19, 37, 23, 35, 83, 105 and 114 children, respectively. The analysis result showed that the horizontal length, vertical height and angle of maxillary and mandibular were not significantly different from that of normal children of the same age in the preoperative cephalometric and three-dimensional scanning result of the children with grade Ⅰ and Ⅱ-Ⅲ cleft palate. However, the horizontal length, vertical height and angle of maxillary and mandibular were significantly different from that of normal children of the same age at 2-3 years and 4-6 years after surgery. Moreover, the difference between maxillofacial characteristics of children with grade Ⅱ-Ⅲ cleft palate and normal children of the same age is more significant than that of children with grade Ⅰ cleft palate.Conclusion:Maxillofacial morphological development is inhibited at different ages in children with different types of cleft palate after repair, which is manifested as the backward development of maxillofacial horizontal length, vertical height and angle of maxillary and mandible. Moreover, the maxillofacial morphological development inhibition was more serious in children with grade Ⅱ-Ⅲ cleft palate than in children with grade Ⅰ cleft palate.

Objective:To investigate the effect of tranexamic acid on perioperative hemorrhage of cranioplasty for craniosynostosis.Methods:A retrospective analysis was performed on the clinical data of children who received cranial remodeling from the Children’s Hospital of Fudan University from February 2015 to October 2018. The patients were divided into two groups: tranexamic acid treatment group (TXA group) and no tranexamic acid treatment group (control group). The average intraoperative blood loss, the average amount of blood transfusion, postoperative average amount of blood transfusion, average drainage volume, and changes of postoperative hemoglobin and blood coagulation function were observed. The measurement data were described by M ( Q1, Q3) and analyzed with Mann-Whitney U test. Results:A total of 41 patients were included in this study. There were 26 patients in TXA group, 18 males and 8 females, aged 9.5 (7.0, 38.8) months; in the control group, there were 15 patients, 10 males and 5 females, aged 9.2 (6.6, 18.3) months. The results showed that there was no significant difference in preoperative hemoglobin level between the two groups ( P>0.05). The hemoglobin level of TXA group was higher than that of control group immediately after operation and 24 h and 72 h after operation ( P<0.05). Total intraoperative blood loss and blood loss per kilogram of body weight in TXA group were lower than those in control group [150.0 (57.5, 225.0) ml vs. 200.0(80.0, 300.0) ml, P<0.05; 13.6(6.8, 18.5) ml/kg vs. 15.8(7.0, 27.0) ml/kg, P<0.01]. The amount of intraoperative transfusion volume per kilogram of body weight in TXA group was less than that in control group [15.0(8.8, 17.7) ml/kg vs.17.1 (13.0, 23.3) ml/kg, P<0.05]. There were no significant differences between the two groups in postoperative total transfusion volume, transfusion volume per kilogram of body weight, total drainage volume and drainage volume per kilogram of body weight ( P>0.05). There was no significant difference in prothrombin time (PT), activated partial thromboplastin time (APTT) and fibrinogen between the two groups before and after surgery ( P<0.05). Conclusion:TXA can reduce the amount of blood loss and blood transfusion in craniosynostosis in children.

Objective:To investigate the effect of tranexamic acid on perioperative hemorrhage of cranioplasty for craniosynostosis.Methods:A retrospective analysis was performed on the clinical data of children who received cranial remodeling from the Children’s Hospital of Fudan University from February 2015 to October 2018. The patients were divided into two groups: tranexamic acid treatment group (TXA group) and no tranexamic acid treatment group (control group). The average intraoperative blood loss, the average amount of blood transfusion, postoperative average amount of blood transfusion, average drainage volume, and changes of postoperative hemoglobin and blood coagulation function were observed. The measurement data were described by M ( Q1, Q3) and analyzed with Mann-Whitney U test. Results:A total of 41 patients were included in this study. There were 26 patients in TXA group, 18 males and 8 females, aged 9.5 (7.0, 38.8) months; in the control group, there were 15 patients, 10 males and 5 females, aged 9.2 (6.6, 18.3) months. The results showed that there was no significant difference in preoperative hemoglobin level between the two groups ( P>0.05). The hemoglobin level of TXA group was higher than that of control group immediately after operation and 24 h and 72 h after operation ( P<0.05). Total intraoperative blood loss and blood loss per kilogram of body weight in TXA group were lower than those in control group [150.0 (57.5, 225.0) ml vs. 200.0(80.0, 300.0) ml, P<0.05; 13.6(6.8, 18.5) ml/kg vs. 15.8(7.0, 27.0) ml/kg, P<0.01]. The amount of intraoperative transfusion volume per kilogram of body weight in TXA group was less than that in control group [15.0(8.8, 17.7) ml/kg vs.17.1 (13.0, 23.3) ml/kg, P<0.05]. There were no significant differences between the two groups in postoperative total transfusion volume, transfusion volume per kilogram of body weight, total drainage volume and drainage volume per kilogram of body weight ( P>0.05). There was no significant difference in prothrombin time (PT), activated partial thromboplastin time (APTT) and fibrinogen between the two groups before and after surgery ( P<0.05). Conclusion:TXA can reduce the amount of blood loss and blood transfusion in craniosynostosis in children.

Objective:To summarize the surgery effect of tongue volume reduction for macroglossia patients with Beckwith-Wiedemann syndrome (BWS).Methods:The clinical data of children diagnosed as BWS based on clinic symptoms and gene tests, who received operation in Department of Plastic Surgery, Children’s Hospital of Fudan University from July 2012 to December 2019 were analyzed retrospectively. All the patients underwent dorsal tongue bell-shaped resection and ventral V-shaped resection. By pulling tongue tip, the tongue body was cut and dissected along the marking line followed by suturing the muscle layer of the central part of the tongue tissue. Intermittent mattress suture of the dorsal and ventral mucous wounds of the tongue. After operation, all patients kept the trachea intubation and were transferred to PICU for further treatment and received early nasal feeding. The short-term postoperative complications, and long-term follow-up of the tongue body shape and clinical symptoms were recorded.The clinical effect was evaluated by onesurgeon and the parents.The effect was graded into four levels: dissatisifaction, average, satisifaction and great satisifaction.Results:A total of 61 children were included in this study, including 30 males and 31 females, with an average age of 8.5 months. The length of the tongue exposed outside at the resting state was 1.34-2.62 cm, with a median of 1.87 cm. Symmetric hypertrophy was found in 52 cases (85.2%), lateral hypertrophy in 9 cases (14.8%). There were 3 cases with respiratory obstruction (4.9%), 35 cases with salivation (57.4%), 4 cases suffering difficulty of feeding and dysphagia (6.6%). All children underwent the operation successfully, and the average operation time was 35.8 min. Among the short-term complications, there were 3 ventral wounds dehiscence, none of which needed sutured again. By prolonging nasal feeding time, the wounds were all healed in the secondary stage. Respiratory obstruction occurred in 1 patient after operation, and the symptoms were relieved after tongue retraction. The mean follow-up time was 3.3 years (2 months to 7 years), and 1 patient was inadequately corrected and good condition was got after second aryoperation. The remaining children obtained satisfactory resting tongue position and appearance, and the postoperative clinical symptoms were improved. In the long-term follow-up, the comprehensive evaluation results of the resting tongue position and clinical symptoms of the children by surgeon and parents were satisfied in 10 cases (16.4%), very satisfied in 50 cases (82.0%), and average in 1 case (insufficient correction after the first operation).Conclusions:Central resection and volume reduction of tongue body is a simple, effective and safe surgical technique in children with giant tongue diagnosed as BWS.

Objective:To explore the complications of case with craniosynostosis after cranial vault remolding.Methods:The clinical data with craniosynostosis who underwent cranial vault remolding in our hospital from January 2012 to January 2018 were selected and retrospectively analyzed.Results:There were 61 children, including 42 males and 19 females. Among them 17 with scaphocephaly, mean age at surgery was 8.2 months (range, 6-48 months); 13 with trigonocephaly, mean age at surgery 13 months (range, 6-29 months) ; 17 with plagiocephaly, mean age at surgery 15.2 months (range, 7-48 months); 14 with syndromic craniosynostosis, mean age 18.7 months (range, 6-60 months). Complications occurred in 15 cases of 13 children, including 1 death case (1.6%), who died of severe pneumonia from adenovirus infection in pediatric intensive care unit(PICU). Two cases suffered with cerebrospinal fluid leakage (3.3%) , which was self-healed after 2 weeks; three with dural defects(4.9%) , which were treated with dural repair and manual patch covering; 5 with conjunctivitis(8.2%), which was cured with eyedrops; 1 with occipital pressure ulcers(1.6%), which was cured with debridement and dressing; 3 with occipital bald(4.9%), one of which was caused by the above-mentioned occipital pressure ulcers. These three cases are followed up for further observations.Conclusions:Complications related to cranial vault remolding have various manifestations, and severe cases can be life-threatening. It is very important to actively prevent and promptly treat relevant issues after surgery.

Objective:To summarize the surgery effect of tongue volume reduction for macroglossia patients with Beckwith-Wiedemann syndrome (BWS).Methods:The clinical data of children diagnosed as BWS based on clinic symptoms and gene tests, who received operation in Department of Plastic Surgery, Children’s Hospital of Fudan University from July 2012 to December 2019 were analyzed retrospectively. All the patients underwent dorsal tongue bell-shaped resection and ventral V-shaped resection. By pulling tongue tip, the tongue body was cut and dissected along the marking line followed by suturing the muscle layer of the central part of the tongue tissue. Intermittent mattress suture of the dorsal and ventral mucous wounds of the tongue. After operation, all patients kept the trachea intubation and were transferred to PICU for further treatment and received early nasal feeding. The short-term postoperative complications, and long-term follow-up of the tongue body shape and clinical symptoms were recorded.The clinical effect was evaluated by onesurgeon and the parents.The effect was graded into four levels: dissatisifaction, average, satisifaction and great satisifaction.Results:A total of 61 children were included in this study, including 30 males and 31 females, with an average age of 8.5 months. The length of the tongue exposed outside at the resting state was 1.34-2.62 cm, with a median of 1.87 cm. Symmetric hypertrophy was found in 52 cases (85.2%), lateral hypertrophy in 9 cases (14.8%). There were 3 cases with respiratory obstruction (4.9%), 35 cases with salivation (57.4%), 4 cases suffering difficulty of feeding and dysphagia (6.6%). All children underwent the operation successfully, and the average operation time was 35.8 min. Among the short-term complications, there were 3 ventral wounds dehiscence, none of which needed sutured again. By prolonging nasal feeding time, the wounds were all healed in the secondary stage. Respiratory obstruction occurred in 1 patient after operation, and the symptoms were relieved after tongue retraction. The mean follow-up time was 3.3 years (2 months to 7 years), and 1 patient was inadequately corrected and good condition was got after second aryoperation. The remaining children obtained satisfactory resting tongue position and appearance, and the postoperative clinical symptoms were improved. In the long-term follow-up, the comprehensive evaluation results of the resting tongue position and clinical symptoms of the children by surgeon and parents were satisfied in 10 cases (16.4%), very satisfied in 50 cases (82.0%), and average in 1 case (insufficient correction after the first operation).Conclusions:Central resection and volume reduction of tongue body is a simple, effective and safe surgical technique in children with giant tongue diagnosed as BWS.

Objective:To explore the complications of case with craniosynostosis after cranial vault remolding.Methods:The clinical data with craniosynostosis who underwent cranial vault remolding in our hospital from January 2012 to January 2018 were selected and retrospectively analyzed.Results:There were 61 children, including 42 males and 19 females. Among them 17 with scaphocephaly, mean age at surgery was 8.2 months (range, 6-48 months); 13 with trigonocephaly, mean age at surgery 13 months (range, 6-29 months) ; 17 with plagiocephaly, mean age at surgery 15.2 months (range, 7-48 months); 14 with syndromic craniosynostosis, mean age 18.7 months (range, 6-60 months). Complications occurred in 15 cases of 13 children, including 1 death case (1.6%), who died of severe pneumonia from adenovirus infection in pediatric intensive care unit(PICU). Two cases suffered with cerebrospinal fluid leakage (3.3%) , which was self-healed after 2 weeks; three with dural defects(4.9%) , which were treated with dural repair and manual patch covering; 5 with conjunctivitis(8.2%), which was cured with eyedrops; 1 with occipital pressure ulcers(1.6%), which was cured with debridement and dressing; 3 with occipital bald(4.9%), one of which was caused by the above-mentioned occipital pressure ulcers. These three cases are followed up for further observations.Conclusions:Complications related to cranial vault remolding have various manifestations, and severe cases can be life-threatening. It is very important to actively prevent and promptly treat relevant issues after surgery.