Objective:To prospectively evaluate the development of palate and hyoid bone in infants with Pierre Robin sequence patients.Methods:A retrospective study was performed on 168 patients younger than 3 months ago who underwent bilateral mandibular distraction osteogenesis in the Department of Burn and Plastic Surgery, Children’s Hospital of Nanjing Medical University from January 2017 to December 2018. Using three dimensional-computed tomography (3D-CT) scan to reconstruct and survey the pattern of palate and hyoid bones. According to the palate morphology, the patients were divided into cleft palate group (CP group) and high palate arch group (HAP group). And according to the severity, they were divided into normal group, mild group and severe group based on the morphology of hyoid bone. Data were analyzed including the preoperative age, gender, body weight, respiratory (endotracheal intubation) and feeding support (nasal feeding) before and after distractors placement, and nutrition (serum albumin level) before and 3 months after the operation (before the distractors removal). Normal distribution datais represented by Mean±SD, and non-normal distribution data is represented to M ( P25, P75). Independent sample t-test was used to compare the age, body weight and serum albumin level. Kruskal-Wallis rank sum test was used to compare the postoperative intubation and nasal feeding time in each group. Paired sample t-test was used to compare the serum albumin before and after the operation. The preoperative gender, tracheal intubation, and nasal feeding of the children between each group were analyzed by Fisher’s precise test. P<0.05 considered the difference to be statistically significant. Results:168 infants (79 boys and 89 girls) with Pierre Robin sequence were included. The age was (36±22) d, the body weight at admission was (3.6±0.5) kg, and the preoperative intubation rate was 18.5% (31/168), the preoperative nasal feeding rate was 33.3% (56/168). 122 (72.6%) have cleft palate, and 46 (27.4%) have highly arched palate. There was no significant difference in admission age, gender, preoperative respiratory support or serum albumin between the two groups( P>0.05). In terms of feeding support, the HAP group (45.7%, 21/46) had a higher preoperative nasal feeding rate than the CP group (28.7%, 35/122) ( P=0.044), and had significantly longer postoperative nasal feeding time than CP group ( H=11.565, P=0.001), the differences were statistically significant. However, there was no significant difference in the time of endotracheal intubation between the two groups ( P>0.05). The breathing and feeding difficulties of the two groups of children were significantly improved three months after operation, and there was no need for endotracheal intubation and nasal feeding after the operation, and the serum albumin was significantly increased compared with those before the operation ( P<0.001). Further observation of hyoid bone morphology showed that normal hyoid bone morphological development in 76 cases (45.2%, normal group), complete hyoid body or hyoid ramus structure with smaller shape than normal in 57 cases (33.9%, mild group), and partial or complete hyoid bone structure absence in 35 cases (20.8%, severe group). There was no significant difference in the age of admission and preoperative serum albumin between the normal and mild group ( P>0.05); the age of the severe group was significantly younger than the normal and mild group, and the preoperative serum albumin level was also lower than the other two groups, and the difference was statistically significant ( P<0.05). In terms of preoperative respiratory support, the endotracheal intubation rate was higher in the severe group (34.3%) compared with the normal group (14.5%) or the mild group (14.0%), the difference was statistically significant ( P<0.05). There was no statistically significant difference between the normal group and the mild group ( P>0.05). In terms of preoperative nasal feeding, the difference in nasal feeding rate between the mild group (40.4%), the severe group (45.7%) and the normal group (22.4%) was statistically significant ( P<0.05), while there was no statistically significant difference between the mild and severe group ( P>0.05). Compared with the normal and mild group, the severe group had significantly longer postoperative endotracheal intubation time and nasal feeding time, and the difference was statistically significant ( P<0.05), while the mild group and the normal group had no significant difference Significance ( P>0.05). Serum albumin level of the three groups increased significantly at 3 months after operation, and the differences were statistically significant compared with those before operation ( P<0.001). Conclusions:Infants with Pierre Robin sequence exist different degrees of abnormal morphology of the upper palate and hyoid bone. Patients with HAP have more severe swallowing and feeding problems, and babies with severe hyoid bone deformities are more serious, with severe respiratory and feeding problem. Therefore, early evaluation of the morphology of the palate and hyoid bone may conduce to assess postoperative prognosis, respiratory and feeding monitoring, and related prevention and treatment as soon as possible.

Objective:To prospectively evaluate the development of palate and hyoid bone in infants with Pierre Robin sequence patients.Methods:A retrospective study was performed on 168 patients younger than 3 months ago who underwent bilateral mandibular distraction osteogenesis in the Department of Burn and Plastic Surgery, Children’s Hospital of Nanjing Medical University from January 2017 to December 2018. Using three dimensional-computed tomography (3D-CT) scan to reconstruct and survey the pattern of palate and hyoid bones. According to the palate morphology, the patients were divided into cleft palate group (CP group) and high palate arch group (HAP group). And according to the severity, they were divided into normal group, mild group and severe group based on the morphology of hyoid bone. Data were analyzed including the preoperative age, gender, body weight, respiratory (endotracheal intubation) and feeding support (nasal feeding) before and after distractors placement, and nutrition (serum albumin level) before and 3 months after the operation (before the distractors removal). Normal distribution datais represented by Mean±SD, and non-normal distribution data is represented to M ( P25, P75). Independent sample t-test was used to compare the age, body weight and serum albumin level. Kruskal-Wallis rank sum test was used to compare the postoperative intubation and nasal feeding time in each group. Paired sample t-test was used to compare the serum albumin before and after the operation. The preoperative gender, tracheal intubation, and nasal feeding of the children between each group were analyzed by Fisher’s precise test. P<0.05 considered the difference to be statistically significant. Results:168 infants (79 boys and 89 girls) with Pierre Robin sequence were included. The age was (36±22) d, the body weight at admission was (3.6±0.5) kg, and the preoperative intubation rate was 18.5% (31/168), the preoperative nasal feeding rate was 33.3% (56/168). 122 (72.6%) have cleft palate, and 46 (27.4%) have highly arched palate. There was no significant difference in admission age, gender, preoperative respiratory support or serum albumin between the two groups( P>0.05). In terms of feeding support, the HAP group (45.7%, 21/46) had a higher preoperative nasal feeding rate than the CP group (28.7%, 35/122) ( P=0.044), and had significantly longer postoperative nasal feeding time than CP group ( H=11.565, P=0.001), the differences were statistically significant. However, there was no significant difference in the time of endotracheal intubation between the two groups ( P>0.05). The breathing and feeding difficulties of the two groups of children were significantly improved three months after operation, and there was no need for endotracheal intubation and nasal feeding after the operation, and the serum albumin was significantly increased compared with those before the operation ( P<0.001). Further observation of hyoid bone morphology showed that normal hyoid bone morphological development in 76 cases (45.2%, normal group), complete hyoid body or hyoid ramus structure with smaller shape than normal in 57 cases (33.9%, mild group), and partial or complete hyoid bone structure absence in 35 cases (20.8%, severe group). There was no significant difference in the age of admission and preoperative serum albumin between the normal and mild group ( P>0.05); the age of the severe group was significantly younger than the normal and mild group, and the preoperative serum albumin level was also lower than the other two groups, and the difference was statistically significant ( P<0.05). In terms of preoperative respiratory support, the endotracheal intubation rate was higher in the severe group (34.3%) compared with the normal group (14.5%) or the mild group (14.0%), the difference was statistically significant ( P<0.05). There was no statistically significant difference between the normal group and the mild group ( P>0.05). In terms of preoperative nasal feeding, the difference in nasal feeding rate between the mild group (40.4%), the severe group (45.7%) and the normal group (22.4%) was statistically significant ( P<0.05), while there was no statistically significant difference between the mild and severe group ( P>0.05). Compared with the normal and mild group, the severe group had significantly longer postoperative endotracheal intubation time and nasal feeding time, and the difference was statistically significant ( P<0.05), while the mild group and the normal group had no significant difference Significance ( P>0.05). Serum albumin level of the three groups increased significantly at 3 months after operation, and the differences were statistically significant compared with those before operation ( P<0.001). Conclusions:Infants with Pierre Robin sequence exist different degrees of abnormal morphology of the upper palate and hyoid bone. Patients with HAP have more severe swallowing and feeding problems, and babies with severe hyoid bone deformities are more serious, with severe respiratory and feeding problem. Therefore, early evaluation of the morphology of the palate and hyoid bone may conduce to assess postoperative prognosis, respiratory and feeding monitoring, and related prevention and treatment as soon as possible.

An 8-year-old female child was admitted to Children’s Hospital of Nanjing Medical University due to paroxysmal dyspnea on July 19, 2019. After thorough examination, she was considered to suffer from pulmonary lymphangioma and underwent an exploratory thoracotomy. The diffuse distribution of lymphangioma was seen during surgery, and part of the lymphangioma tissues was excised for histopathology. The patient was diagnosed as diffuse pulmonary lymphangiomatosis according to the history, CT imaging and postoperative pathology. She was treated with sirolimus at a starting dose of 0.9 mg every 12 h. Through monitoring the blood drug concentration regularly and adjusting the drug dose, the blood drug concentration was maintained at 10-15 ng/ml. Two weeks later, the child’s dyspnea improved but she developed refractory chylothorax, which was considered related to surgical wound. Considering that the child had thoracic drainage tubes for a long time, 10 mg of betamethasone, which was dissolved in 20 ml of physiological saline, was injected into each drainage tube to reduce the effusion of pleural effusion, and the drainage tubes were successfully removed after 3 injections. Unfortunately, the child’s symptoms recurred two months later, and she eventually died of respiratory failure despite active treatment, with a duration of nearly one year. The earlier the onset of diffuse pulmonary lymphangiomatosis occurs, the worse the prognosis is. Surgical resection may not be appropriate for such diffuse lesions, and there is a risk of refractory chylothorax, further research should explore the pathogenesis of the disease and targeted drug therapy.

An 8-year-old female child was admitted to Children’s Hospital of Nanjing Medical University due to paroxysmal dyspnea on July 19, 2019. After thorough examination, she was considered to suffer from pulmonary lymphangioma and underwent an exploratory thoracotomy. The diffuse distribution of lymphangioma was seen during surgery, and part of the lymphangioma tissues was excised for histopathology. The patient was diagnosed as diffuse pulmonary lymphangiomatosis according to the history, CT imaging and postoperative pathology. She was treated with sirolimus at a starting dose of 0.9 mg every 12 h. Through monitoring the blood drug concentration regularly and adjusting the drug dose, the blood drug concentration was maintained at 10-15 ng/ml. Two weeks later, the child’s dyspnea improved but she developed refractory chylothorax, which was considered related to surgical wound. Considering that the child had thoracic drainage tubes for a long time, 10 mg of betamethasone, which was dissolved in 20 ml of physiological saline, was injected into each drainage tube to reduce the effusion of pleural effusion, and the drainage tubes were successfully removed after 3 injections. Unfortunately, the child’s symptoms recurred two months later, and she eventually died of respiratory failure despite active treatment, with a duration of nearly one year. The earlier the onset of diffuse pulmonary lymphangiomatosis occurs, the worse the prognosis is. Surgical resection may not be appropriate for such diffuse lesions, and there is a risk of refractory chylothorax, further research should explore the pathogenesis of the disease and targeted drug therapy.