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 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 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.