[Clinical characteristics, diagnosis and treatment of nasal cartilage mesenchymal hamartoma in infants].

Objective: To explore the clinical characteristics, pathological features, and diagnosis and treatment strategies of nasal chondromesenchymal hamartoma (NCMH) in infants and young children. Methods: A retrospective analysis was conducted on seven cases of NCMH infants and young children admitted to Beijing Children's Hospital, Capital Medical University from April 2015 to January 2022. The cohort included 5 males and 2 females, aged from 6 days to 2 years and 3 months. General information, clinical symptoms, imaging findings, treatment plans, postoperative complications, recurrence and follow-up time were collected, summarized and analyzed. Additionally, immunohistochemical characteristics of the lesion were examined. Results: The clinical symptoms of 7 children included nasal congestion, runny nose, open mouth breathing, snoring during sleep, difficulty feeding, and strabismus. All patients underwent electronic nasopharyngoscopy examination, with 5 cases of tumors located in the right nasal cavity and 2 cases in the left nasal cavity. No case of bilateral nasal cavity disease was found. All 7 patients underwent complete imaging examinations, with 5 patients underwent MRI and CT examinations, 1 patient underwent CT examination only, and 1 patient underwent MRI examination only. The CT results showed that all tumors were broad-based, with uneven density, multiple calcifications and bone remodeling, and some exhibited multiple cystic components. The MRI results showed that the tumor showed low signal on T1 weighted imaging and high or slightly high signal on T2 weighted imaging. All patients were diagnosed through histopathological examination and immunohistochemistry, including 7 cases of Ki-67 and SMA (+), 5 cases of S-100 and Vimentin (+), and all EMA and GFAP were negative. All patients underwent endoscopic resection surgery through the nasal approach, with 3 cases using navigation technology. Five cases of tumors were completely removed, and two cases of tumors were mostly removed. No nasal packing was performed after surgery, and no postoperative nasal, ocular, or intracranial complication occurred in all patients. Follow up assessments conducted 6 to 84 months post-surgery revealed no instances of tumor recurrence in any of the patients. Conclusions: The clinical symptoms of children with NCHM mainly depend on the size and location of the tumor. Nasal endoscopic surgery is the main treatment method. In cases where critical structures like the skull base or orbit are implicated, staged surgical interventions may be warranted. Long-term follow-up is strongly advised to monitor for any potential recurrence or complications.

[Diagnosis and management of orbital and cranial complications of pediatric acute rhinosinusitis].

Objective: To review the clinical characteristics, to illustrate diagnosis and management experience of orbital and cranial complications of pediatric acute rhinosinusitis. Methods: The clinical data of 24 children with orbital and cranial complications of acute rhinosinusitis who received endoscopic sinus surgery combined with drug treatment in Beijing Children's Hospital from January 2017 to December 2021 were retrospectively reviewed. There were 19 boys and 5 girls. The age varied from 13 to 159 months, with a median 47.5 months. The following diagnoses were obtained: 12 isolated subperiosteal orbital abscess, 2 associated with preseptal abscess, 2 associated with intraorbital abscess, 7 associated with optic neuritis, and 1 associated with septic cavernous sinus thrombosis. Clinical characteristics, organism isolated and outcomes were analyzed through descriptive methods. Results: All 24 patients presented with fever; 9 presented with nasal congestion and purulent discharge. The clinical manifestations of orbital infection included orbital edema, pain, proptosis and displacement of globe in all patients, while visual impairment was recognized in 7 children. Purulent drainage was cultured in 17 patients, among which 12 were positive. All patients underwent nasal endoscopic surgical interventions uneventfully, excluding one patient who required a second surgical procedure. Follow-up period ranged from 5 to 64 months. All patients resolved fully, with the exception of 2 children who got permanent blindness with visual loss preoperative. There was no recurrence or death. Conclusions: Orbital and cranial complications of pediatric acute rhinosinusitis could be severe with an occult onset. For patients with vison impairment, any signs of intracranial complications and a lack of response to conservative management, an urgent endoscopic intervention is needed.

[Nasal endoscopic modified mucosal flap technique for repair of congenital choanal atresia in newborns and infants].

Objective: To describe and evaluate the surgical effect and prognosis of nasal endoscopic modified mucosal flap technique for repair of congenital choanal atresia in newborns and infants. Methods: The clinical data of 38 newborns and infants with congenital choanal atresia who underwent nasal endoscopic surgery in Beijing Children's Hospital between January 2016 and May 2018 were retrospectively analysed, including 13 males and 25 females. The age ranged from 5 days to 3 years old at the time of operation (15 cases were newborns). The clinical data, imaging data, treatment effect and prognosis were collected. According to the different surgical methods, the patients were divided into the conventional operation group and the modified mucosal flap technique group. The designs of the modified mucosal flap technique were designed according to the type of congenital choanal atresia. The cross-over L-shaped flaps were performed in patients with unilateral atresia, and the mirrored L-shaped flaps were performed in patients with bilateral atresia. All the patients were followed up for 2-3 years, and the follow-up parameters included the times of operations, length of hospital stay, restenosis rate and incidence of complications. Study data was analyzed using SAS version 9.4 statistical software. Results: Sixteen cases underwent conventional operation while 22 patients underwent modified mucosal flap technique under nasal endoscope. The lightest weight (2 200 g) and the youngest age (5 days) of the patients came from the modified mucosal flap technique group. Compared with the conventional operation group under nasal endoscope, the modified mucosal flap technique group had fewer times of operations (1.14±0.47 vs 2.69±1.20, t=5.552, P<0.001), shorter hospital stay ((7.70±3.22) d vs (14.37±19.16) d, t=2.960, P=0.005), lower rate of postoperative restenosis (9.1% vs 43.8%, χ²=6.156, P=0.013), and lower rate of the incidence of complications (13.6% vs 43.8%, χ²=5.955, P=0.015), the differences were statistically significant. Conclusion: The nasal endoscopic modified mucosal flap technique is feasible for repairing congenital choanal atresia in newborns and infants, which can significantly reduce the incidence of postoperative restenosis and complications.

[Clinical efficacy of the combined diagnosis and management for children with airway allergic diseases].

Objective: To evaluate the clinical efficacy of the combined diagnosis and management in children with airway allergic diseases(bronchial asthma, allergic rhinitis). Methods: This observational study belongs to cluster sampling cases, which included the clinical data from children with airway allergic diseases in Allergy Department and Otorhinolaryngology Department of Beijing Children's Hospital from April to December in 2015. They were followed up every three months during 12 months. All the subjects were required to continuously record daily symptom by diary card. ACT/c-ACT, VAS, treatment steps to control asthma, respiratory infections, wheeze, pulmonary function(FEV1%pred,FEV1/FVC,PEF%pred,FEF25%pred,FEF50%pred,FEF75%pred,MMEF%pred), FeNO were assessed in every visiting. The mean±standard deviation was used for the measurement data in accordance with normal distribution. Comparing the pulmonary function indexes at every point, the measurement data with normal distribution and uniform variance were analyzed by single factor analysis of variance, and the measurement data with uneven variance were tested by non-parametric rank sum test. Results: Among 147 recruited participants, 106 completed the combined diagnosis and management. The airway allergic diseases control rate was 87.7% at 12 months after the combined diagnosis and management. At every point, the average daily symptom score and VAS score which were significantly lower than at the baseline(H=35.854,P=0.000)[ 1.2(0.7,2.2),0.6(0.2,1.5),0.4(0.1,1.0),0.5(0.1,1.1) vs 2.0(1.0,3.5)],(H=39.559,P=0.000)[2.5(0.5,4.7),2.2(0.3,4.4),1.8(0.2,4.6),1.6(0.3,3.8) vs 6.9(4.1,9.8)]. ACT/c-ACT score at 3, 6, 9, 12 months were significantly higher than at the baseline (H=79.695,P=0.000) [25.0(22.5,27.0),26.0(24.0,27.0),25.0(23.0,27.0),25.0(24.0,27.0) vs 20.0(17.0,22.0)]. FEV1%pred and FEF25%pred at 3, 6 months were significantly higher than at the baseline (F=3.563,P=0.007)(104.7±12.6 vs 96.8±14.5,103.0±10.3 vs 96.8±14.5),(F=2.456,P=0.046)(96.6±22.0 vs 85.0±21.9,93.3±18.0 vs 85.0±21.9). PEF%pred at 3, 6, 9, 12 months after the combined diagnosis and management were significantly higher than at the baseline(F=5.497,P=0.000)(105.1±18.1,101.2±15.3,99.7±17.1,99.8±17.5 vs 90.3±17.8). FeNO at 3, 6, 9, 12 months respectively were no significantly differences at the baseline(F=0.751,P=0.558)(25.7±23.6 vs 30.7±25.6,25.9±16.5 vs 30.7±25.6,27.5±20.2 vs 30.7±25.6,30.6±19.6 vs 30.7±25.6).The respiratory infections rate were 69.8%(74/106),67.0%(71/106),60.4%(64/106),51.9%(55/106) at 3, 6, 9, 12 months respectively. The wheezing rate was 24.5%(26/106),14.2%(15/106),11.3%(12/106),7.5%(8/106) at 3, 6, 9, 12 months respectively. Conclusions: The combined diagnosis and management can significantly improve the control level of children's airway allergic diseases, which should be implemented in the management of children's airway allergic diseases.

[Pediatric nasal neuroglial heterotopia: report of 13 cases].

Objective: To summarize clinical features and our experience of the diagnosis and treatment of pediatric nasal neuroglial heterotopia (NGH). Methods: Clinical data of 13 nasal NGH patients in Beijing Children's Hospital from August 2014 to October 2019 were retrospectively reviewed, including 9 boys and 4 girls, aged from 1 to 38 months with median age of 5 months. Radiological workups and excision of nasal NGH under general anesthesia were performed for all patients. B ultra-sound and MRI were performed for all external and mixed lesions, while ultra-low-dose CT scan and MRI for all intranasal type. Surgical approaches were dependent on location and extent of the lesions according to radiographic workup, including extranasal or transnasal endoscopic approach. Patients were followed up regularly after operation to evaluate the effect. Initial presentation, locations, imaging findings, surgical approaches and follow-up results were analyzed through descriptive statistical method. Results: Eight nasal NGH patients presented with an internal nasal mass and nasal obstruction, which belonged to intranasal type. Three patients presented with an external nasal mass which belonged to extranasal type and 2 patients had mixed lesions. The sites included nasal dorsum (n=5), anterior to the middle turbinate (n=5) and olfactory cleft (n=3). Surgical resections were done through median rhinotomy approach (n=5) or transnasal endoscopic approach (n=8). All the operations were successful and no complication occurred. All cases were followed up from 3 to 65 months. No recurrence was encountered. Conclusions: Nasal NGH is a rare lesion with atypical clinical presentation. Preoperative imaging including CT scan and MRI is essential for evaluation of the location, extent of the disease and for making the surgical plan. Treatment requires complete surgical excision.

[Excision for congenital nasal dermoid and sinus cyst in children].

Objective: To explore the surgical effect and experience of endoscope-assisted excision for congenital nasal dermoid and sinus cyst (NDSC) in children. Methods: Fifty-three patients with congenital NDSC treated in Beijing Children's Hospital from January 2007 to December 2018 were retrospectively reviewed, including 30 boys and 23 girls, with the age ranging from 9 to 145 months (mean age 35.6 months). The ultra-low-dose CT scan and MRI of the paranasal sinuses were performed for all patients. Excisions of NDSC under general anesthesia were performed for all patients, and surgical approaches were dependent on location and extent of the lesions according to radiographic workups. All intra-osseous patients and complicated superficial cases underwent surgical excision of NDSC and nasal reconstruction with the assistance of endoscope. Initial presentation, medical history, imaging workups, surgical approaches, complications, rates of recurrence and cosmetic outcomes were evaluated. Descriptive statistics was used for the results analysis. Results: Among 53 cases, the most common presentation included a nasal-glabella mass (n=21, 39.6%), a dorsal punctum (n=13, 24.5%) and a dorsal mass (n=9, 17.0%). The sites of NDSC included nasal glabella (n=22, 41.5%), nasal bridge (n=27, 50.9%) and nasal tip (n=4, 7.5%). Of all patients, 24 cases (45.3%) had superficial lesions, 19 cases (35.8%) had intraosseous extension into the frontonasal bones, 10 cases (18.9%) extended intracranially but remained extradural. Surgical approaches included transverse incision (n=22, 41.5%), minimal midline vertical incision (n=27, 50.9%) and external rhinoplasty (n=4, 7.5%). All NDSC were successfully excised and no nasal reconstruction needed. All cases were followed up from 9 to 151 months with a mean of 67.3 months. Five patients (9.4%) with recurrence were observed and were managed successfully with reoperation. During the follow-up, no nasal deformity was noted, and cosmetic outcome was favorable for all patients. Conclusion: Endoscope-assisted excision has the advantage of clear vision, small trama and low recurrence rate for children with NDSC.

[Endothelial dysfunction in children with obstructive sleep apnea syndrome].

Objective: To evaluate the endothelial function in obstructive sleep apnea syndrome(OSAS) children and to identify related factors of endothelial dysfunction. Methods: This was a cross-sectional study. Children with habitual snoring (snoring ≥3 nights per week) admitted to the ward of otolaryngology, head and neck surgery, Beijing Children's Hospital were recruited to this study between 1(st) June 2015 and 1(st) March 2016. All children aged 3 to 11 years and of them 245 were boys and 110 were girls. All subjects underwent an overnight polysomnography (PSG), as well as endothelial function testing. All subjects were grouped into primary snoring (PS) and OSAS group according to the obstructive apnea hypopnea index (OAHI). T test or Wilcoxon test were used to compare the differences in PSG results between the two groups, and univariate and multivariate correlation analyses were used to explore the relevant factors affecting the endothelial function. Results: A total of 355 subjects were enrolled and 248 had OSAS, and 107 had PS. There were no significant differences in age, gender and body mass index (BMI) Z-score between the two groups (all P>0.05). OSAS group had higher OAHI, oxgen desaturation index and respiratory related arousal index (5.2 (2.2, 13.2) vs. 0.4 (0.1, 0.7), 4.1 (2.0, 13.1) vs. 0.5 (0.1, 1.0), 2.5 (1.0, 4.8) vs. 0.4 (0.1, 0.9), Z=-14.957, -11.790, -10.378, all P<0.01), and lower minimum oxygen saturation and reactive hyperemia index (RHI) than those of PS (0.89 (0.85, 0.92) vs. 0.94 (0.91, 0.95), 1.2±0.2 vs. 1.1±0.1, Z=-9.337, t=5.354, P<0.01). Univariate regression analysis showed that RHI was linearly correlated with age (parameter estimate=0.017, P<0.01), gender (parameter estimate=0.065, P<0.01), OAHI (parameter estimate=-0.023, P<0.01), oxygen desaturation index (parameter estimate=-0.019, P<0.01), respiratory related arousal index (parameter estimate=-0.031, P<0.01), and oxygen saturation nadir (parameter estimate=0.067, P=0.045). The relationship between BMI Z-score and RHI was quadratic. Multivariate regression analysis showed that age (parameter estimate=0.015, P<0.01), BMI Z-score (parameter estimate=0.040, P<0.01), BMI Z-score quadratic form (parameter estimate=-0.010, P<0.01), respiratory related arousal index (parameter estimate=-0.020, P<0.01) were independently correlated with RHI. Conclusions: Children with OSAS have significant endothelial dysfunction compared with PS. Frequent arousals due to obstructive respiratory events during sleep may be a candidate risk factor for endothelial dysfunction in children with OSAS.

[Clinical analysis of head and neck neurogenic tumor in childhood].

Objective:To investigate the clinical characteristics, imaging features and treatment of neurogenic tumor in chilehood and to improve the experience in diagnosis and treatment for the disease. Method:The twenty-nine inpatients of histopathologically proven neurogenic tumor from January 2015 to December 2018 were retrospectively analyzed. The pathological types, clinical characteristics, imaging findings, and management were analyzed. Result: There were five cases of schwannoma aged from 9 years to 14 years, five cases of neurofibroma aged from 9 months to 9 years, and nineteen cases of neuroblastic tumor aged from 3 months to 5 years in our series. The chief manifestations were soft tissue masses, snore, and Honer syndrome. As the tumors had different components pathologically, on scans they presented as masses with heterogeneous density. Schwannoma showed as oval masses with clear margins, with the characteristic of"tail sign". The imaging findings of neurofibromas showed unclear boundaries and plexiform neurofibromas showed multiple clumps with visible separation. The ultrasonography of neuroblastic tumor was characterized by hypoechoic, heterogeneous and spotty calcification. All the cases underwent surgical resections. In 5 cases of schwannoma, it was confirmed that the tumors originated from the vagus nerve during the operation, which could be completely removed without postoperative complications. Two cases of neurofibromas were completely resected, 2 cases were only partially resected, and 1 case which located at the entrance of the esophagus was resected under supportive laryngoscopic for three times. Thirteen of 19 children with neuroblastogenic tumors underwent resection in our department after chemotherapy, and 2 patients with postoperative recurrence underwent another operation. Conclusion:Neuroblastoma is the most common neurogenic tumor in the head and neck of children. Most neurogenic tumors have their corresponding characteristics on imaging and should be differentiated. Schwannoma has clear boundary and intact capsule, so it is easier to complete resection. The neurofibroma is commonly difficult to remove with safety margin because of its unclear boundaries. Neuroblastoma should be treated according to the lesion range and stage. Most of the lesions are difficult to remove completely due to the wide range, peripheral blood vessels and important nerves. So preoperative chemotherapy is generally required.

[Analysis of sleep quality and related factors among children in Beijing].

Objective: To investigate the sleep quality of children in Beijing and to analyze the related factors. Methods: The data were collected from the survey of 3-14 years old children in 7 urban districts of Beijing in 2015. By using multi-stage stratified cluster random sampling method, 26 kindergartens and primary and secondary schools in 7 districts and counties, including Xicheng, Chaoyang, Changping, Shunyi, Fangshan, Huairou and Mentougou, were randomly selected, with a total of 11 420 children. Children's sleep status was investigated with Children's Sleep Questionnaire (PSQ), and the proportion of children with sleep quality problems when the PSQ score was greater than 7. Various sleep related factors were investigated with self-made questionnaire. A multilevel model was used to analyze the relationship between PSQ score and related factors. Results: The average PSQ score of the children was 3.60±2.69. The proportion of children with sleep quality problems was 8.87%(816/9 198). Multilevel model analysis showed that the younger the children, the higher the PSQ score (<6 years old vs. 6~12 years old vs. >12 years old: 3.94±2.58 vs. 3.58±2.66 vs. 3.30±2.84, F=33.015, P<0.001); male PSQ score higher than female (3.89±2.75 vs. 3.30±2.60, t=10.560, P<0.001); and snoring, obesity, father/mother snoring, playing games before bed, surfing the internet, eating and other factors were statistically related to PSQ. Conclusions: Sleep quality of children in Beijing should not be neglected, especially preschool children with high PSQ scores. Parents should pay attention to children's snoring problems and try to reduce some pre-sleep behaviors that may affect sleep quality.

Efficacy and safety of sublingual immunotherapy with Dermatophagoides farinae drops in pre-school and school-age children with allergic rhinitis.

BACKGROUND: The safety and efficacy of sublingual immunotherapy (SLIT) have been confirmed by many studies. However, in China, the research on efficacy and safety in young and older children with allergic rhinitis (AR) is still rare. OBJECTIVE: The aim of this retrospective study is to evaluate the efficacy and safety of SLIT with Dermatophagoides farinae drops in pre-school and school-age children with AR. METHODS: A total of 282 subjects aged 2-13 years with AR received a two-year course of sublingual immunotherapy along with pharmacotherapy. According to the age, patients were defined as the pre-school group (2-6 years old, n=116) and school-age group (7-13 years old, n=166). Total nasal rhinitis symptom scores (TNSS), visual analogue score (VAS) and total medication scores (TMS) were evaluated at four time points: baseline, after SLIT for half a year, one year and two years. The adverse events (AEs) were evaluated at each visit. RESULTS: After two-year SLIT, the four rhinitis symptom scores, TNSS, VAS and TMS scores were significantly lower than baseline (all P<0.05). The comparison of efficacy between one and two-year duration showed no significant difference in global clinical outcomes (all P>0.05). In addition, there were no significant differences between the pre-school and school-age group in TNSS (all P>0.05), VAS (all P>0.05) and TMS scores (P>0.05) after SLIT for half a year, one year and two years. No severe systemic AEs were reported. CONCLUSION: SLIT with D. farinae drops is clinically effective and safe in pre-school and school-age patients with house dust mites (HDMs)-induced AR.