Objective:Explore the clinical diagnosis and treatment strategies of recurrent multiple chondritis in children with airway involvement as the main cause. Methods:From June 2021 to May 2023, five children with relapsing polychondritis were admitted to our department, all of whom met the Damiani criteria. Tracheotomy was performed in all five patients. Endoscopy indicated subglottic stenosis, with one case classified as grade Ⅲ and four cases as grade Ⅳ according to the Myer-Cotton classification. Enhanced CT scans of the head and neck showed fixed lumen narrowing and stenosis, including thickening of the soft tissue in the laryngeal cavity and partial absorption of the laryngotracheal cartilage anterior wall. In one patient, tracheal wall thickening and deformation with stenosis and calcification involved the posterior tracheal wall. One patient underwent "laryngeal tracheal reconstruction with hyoid graft, T-tube implantation, and bioabsorbable corticosteroid-eluting stent implantation", while three patients underwent "balloon dilatation, T-tube implantation, and bioabsorbable corticosteroid-eluting stent implantation" . Follow-up was performed every two months after the initial surgery, and three bioabsorbable corticosteroid-eluting stents were placed on the upper left, upper right, and lower T-tube, respectively, during this time. The entire follow-up period was six months. Results:All the 5 cases were in the outpatient department, of which 1 case had been extubation, 3 cases were still in the outpatient follow-up, and the remaining 1 case was still being treated in the rheumatology and immunology department due to poor control of the primary disease. Conclusion:In relapsing polychondritis children with airway involvement, tracheotomy could be used to quickly improve the symptoms of dyspnea in the disease progression. In the stable stage of the disease, the surgical method of 'Balloon dilatation + T-tube implantation + Bioabsorbable corticosteroid-eluting stents implantation' was adopted to reduce the secondary injury caused by surgical trauma as much as possible and improve the survival and quality of life of the children.
Humans ; Polychondritis, Relapsing/surgery* ; Child ; Male ; Female ; Tracheotomy ; Laryngostenosis ; Child, Preschool ; Trachea/surgery* ; Adolescent ; Stents

Objective:To explore whether a portable monitor device（PMD） can be used to evaluate the efficacy of supraglottoplasty（SGP） in neonates with severe laryngomalacia（LM）. Methods:A retrospective analysis of 11 neonates diagnosed as severe LM treated in our hospital from January 2020 to November 2023 was performed. All neonates underwent SGP under general anesthesia and overnight PMD monitoring before and after surgery.The changes of sleep monitoring parameters before and after surgery were compared, SPSS 18.0 statistical software was applied and Wilcoxon symbolic-rank l test was used. P<0.05 showed statistical difference. Results:One week after surgery, the obstructive apnea-hypopnea index（OAHI）, obstructive apnea index（OAI）, oxygen desaturationindex（ODI） of 11 children were lower than those before operation, and the minimum pulse oximetry saturation（SpO2） was higher than that before operation, P<0.05, the difference was statistically significant, and there was no significant difference in sleep apnea hypopnea index（AHI） compared with that before operation. One month after surgery, AHI, OAHI, OAI and ODI were lower than those before operation, and the minimum SpO2 and mean SpO2 were higher than those before operation, P<0.05, the difference was statistically significant. Conclusion:Some parameters of PMD examination can be used to evaluate the efficacy of SGP in neonatal severe LM, which is an important auxiliary means for the evaluation of neonatal LM.
Humans ; Retrospective Studies ; Laryngomalacia/surgery* ; Infant, Newborn ; Male ; Female ; Postoperative Period ; Sleep Apnea, Obstructive/surgery* ; Oximetry ; Polysomnography ; Glottis/surgery* ; Infant ; Treatment Outcome

Objective: To analyze the clinical characteristics of patients with Mucopolysaccharidosis ⅣA (MPS ⅣA). Methods: A retrospective study was conducted on 111 patients with MPS ⅣA in Xinhua Hospital of Shanghai Jiao Tong University School of Medcine from December 2008 to August 2020, confirmed by enzyme activity and genetic testing. General situation, clinical manifestations and enzyme activity test results were analyzed. According to the clinical manifestations, it can be divided into severe, intermediate and mild group. The independent sample t test was used to compare the birth body length and weight of children with that of normal boys and girls, and group comparisons of enzyme activities were evaluated by median test. Results: One hundred and eleven unrelated patients, 69 males and 42 females, were classified into 3 subtypes: severe (n=85), intermediate (n=14), and mild (n=12). The age at symptom onset were 1.6 (1.0, 3.0) years, and at diagnosis were 4.3 (2.8, 7.8) years. Skeletal manifestations were observed in all patients and consisted mainly of pectus carinatum (96/111, 86.5%), motor dysfunction (78/111, 70.3%), spinal deformity (71/111, 64.0%), growth retardation (64/111, 57.7%), joint laxity (63/111, 56.8%) and genu valgum (62/111, 55.9%). Eighty-eight patients (88/111, 79.3%) with MPS ⅣA were also along with non-skeletal manifestations, mainly including snoring (38/111, 34.2%), coarse faces (34/111, 30.6%), and visual impairment (26/111, 23.4%). The most common skeletal manifestation was pectus carinatum (79 cases), and non-skeletal manifestation was snoring (30 cases) and coarse faces (30 cases) in severe patients, pectus carinatum (13 cases) and snoring (5 cases) in intermediate type, motor dysfunction (11 cases) and snoring (3 cases) and visual impairment (3 cases) in mild patients. The height and weight of severe patients began to fall below -2 s at 2-<5 years and 5-<7 years, respectively. At the age of 10-<15 years, the standard deviation score of the height of severe patients reached (-6.2±1.6) s in males and (-6.4±1.2) s in females, and the score of weight got (-3.0±1.1) s in males and (-3.5±0.5) s in females. The height of intermediate patients began to fall below -2 s at the age of 7-<10 years, and the standard deviation score of height were -4.6 s and -3.6 s in 2 males, and -4.6 s and -3.8 s in 2 females at the age of 10-<15 years. The weight remained within -2 s in 72.0% (18/25) of intermediate patients compared to age-matched healthy children. In the mild patients with MPS ⅣA, the mean standard deviation score of height and weight was within -2 s. The enzyme activities of mild patients (2.02 (1.05, 8.20) nmol/(17 h·mg)) were both significantly higher than that of intermediate (0.57 (0.47, 0.94) nmol/(17 h·mg)) and severe (0.22 (0, 0.59) nmol/(17 h·mg)) patients (Z=9.91, 13.98, P=0.005, 0.001), and the enzyme activity of intermediate patients was significantly higher than that of severe patients (Z=8.56, P=0.010). Conclusions: The clinical manifestations of MPS ⅣA are charactered by pectus carinatum, motor function impairment, spinal deformity and growth retardation. The clinical characteristics, growth rate and enzyme activity differ among the 3 subtypes of MPS ⅣA.
Male ; Child ; Female ; Humans ; Adolescent ; Mucopolysaccharidosis IV ; Pectus Carinatum ; Retrospective Studies ; Snoring ; China ; Mucopolysaccharidoses ; Growth Disorders ; Vision Disorders

Objective: To investigate outcomes of laryngotracheal reconstruction (LTR)with anterior and posterior costal cartilage grafts in severe pediatric subglottic stenosis (SGS) or laryngeal web (LW). Methods: A review of patients with severe subglottic stenosis or laryngeal web between January 2020 and January 2022 was performed. Demographic features including gender, age at diagnosis, age at surgery, etiology, airway support, and other comorbidities were collected preoperatively. Patients were evaluated in surgical site, breathing, swallowing, phonation and complications postoperatively.Descriptive analysis was used in this research. Results: Eight patients were included: six with grade Ⅲ SGS following Cotton-Myer grading scale, and two with type Ⅲ LW following Cohen's classification. All patients underwent LTR with anterior and posterior costal cartilage grafts. Five patients underwent single-stage LTR (ssLTR), and three patients underwent double-stage LTR (dsLTR). Seven out of eight patients were able to successfully extubate or decannulate with normal swallowing function; four patients had mild hoarseness, and three had moderate hoarseness. One patient failed in extubation, and underwent tracheotomy. Conclusions: LTR with anterior and posterior costal cartilage grafts is an effective and safe treatment for severe SGS or LW. Careful preoperative assessment of disease severity and overall medical status will help selection between ssLTR and dsLTR, thereby maximizing patient outcomes for both modalities.
Child ; Humans ; Constriction, Pathologic/complications* ; Costal Cartilage ; Hoarseness ; Laryngeal Diseases/complications* ; Laryngostenosis/etiology* ; Retrospective Studies ; Treatment Outcome

Objective:To explore the perioperative airway management and treatment of newborns with micrognathia and laryngomalacia. Methods:From January to December 2022, a total of 6 newborns with micrognathia and laryngomalacia were included. Preoperative laryngoscopy revealed concomitant laryngomalacia. These micrognathia were diagnosed as Pierre Robin sequences. All patients had grade Ⅱ or higher symptoms of laryngeal obstruction and required oxygen therapy or non-invasive ventilatory support. All patients underwent simultaneous laryngomalacia surgery and mandibular distraction osteogenesis. The shortened aryepiglottic folds were ablated using a low-temperature plasma radiofrequency during the operation. Tracheal intubation was maintained for 3-5 days postoperatively. Polysomnography（PSG） and airway CT examination were performed before and 3 months after the surgery. Results:Among the 6 patients, 4 required oxygen therapy preoperatively and 2 required non-invasiveventilatory support. The mean age of patients was 40 days at surgery. The inferior alveolar nerve bundle was not damaged during the operation, and there were no signs of mandibular branch injury such as facial asymmetry after the surgery. Laryngomalacia presented as mixed type: type Ⅱ+ type Ⅲ. The maximum mandibular distraction distance was 20 mm, the minimum was 12 mm, and the mean was 16 mm. The posterior airway space increased from a preoperative average of 3.5 mm to a postoperative average of 9.5 mm. The AHI decreased from a mean of 5.65 to 0.85, and the lowest oxygen saturation increased from a mean of 78% to 95%. All patients were successfully extubated after the surgery, and symptoms of laryngeal obstruction such as hypoxia and feeding difficulties disappeared. Conclusion:Newborns with micrognathia and laryngomalacia have multi-planar airway obstruction. Simultaneous laryngomalacia surgery and mandibular distraction osteogenesis are safe and feasible, and can effectively alleviate symptoms of laryngeal obstruction such as hypoxia and feeding difficulties, while significantly improving the appearance of micrognathia.
Humans ; Infant, Newborn ; Infant ; Micrognathism/surgery* ; Laryngomalacia/surgery* ; Treatment Outcome ; Mandible/surgery* ; Airway Obstruction/surgery* ; Intubation, Intratracheal ; Laryngeal Diseases ; Osteogenesis, Distraction ; Oxygen ; Retrospective Studies

Congenital laryngomalacia is the most common disease causing laryngeal stridor in infants. The pathogenesis has not yet been clearly concluded. It may be related to abnormal development of laryngeal cartilage anatomical structure, neuromuscular dysfunction, gastroesophageal and laryngeal reflux disease, etc. The typical manifestations of the disease are inspiratory laryngeal stridor and feeding difficulties, which can be divided into mild, moderate and severe according to the severity of symptoms. The diagnosis is mainly based on clinical symptoms, signs and endoscopy, among which endoscopy is an important diagnostic basis. The treatment of laryngomalacia depends on the severity of symptoms. Mild and some moderate congenital laryngomalacia children can be relieved by conservative treatment, and severe and some moderate congenital laryngomalacia children should be treated by surgery. Supraglottic plasty is the main surgical method, which can effectively improve the symptoms of laryngeal stridor, dyspnea, feeding difficulties and growth retardation in most children, and the surgical effect is good.
Infant ; Child ; Humans ; Laryngomalacia/therapy* ; Respiratory Sounds/etiology* ; Larynx/surgery* ; Laryngeal Diseases/surgery* ; Endoscopy/adverse effects* ; Laryngismus

OBJECTIVE: To investigate the correlation between the medial meniscal indentation index (MDI) and medial tibiofemoral articular cartilage damage more than 3 degrees in patients aged 40 to 60 years old with suspected or complicated knee osteoarthritis at non-weight-bearing position, and to determine the predictive threshold. METHODS: From June 2016 to June 2020, a total of 308 patients who underwent initial knee arthroscopic exploration for chronic knee pain were collected. The age ranged from 36 to 71 years old with an average of(56.40±1.82) years old, including 105 males and 203 females. And patients with extra-articular malformations (abnormal force lines), a history of trauma, inflammatory arthritis and other specific arthritis were excluded. Finally, 89 eligible cases were obtained, aged from 42 to 60 years old with an average of (59.50±0.71) years old, including 45 males and 44 females. The degree of cartilage damage in the medial compartment of the knee joint was recorded, which was divided into two groups(≥degree 3 andcartilage injury of more than grade 3 were determined by further binary Logistic regression analysis. If MDI was taken as an independent risk factor, receiver operating characteristic (ROC) analysis was performed to confirm whether it had diagnostic value for cartilage damage of above degree 3 and calculate the critical value of MDI. RESULTS: A total of 89 eligible patients were obtained. Univariate analysis showed age, BMI, MDI and meniscus injury may be the independent risk factors for cartilage damage of more than 3 degrees, further binary Logistic regression analysis confirmed that MDI[OR=1.66, 95%CI(1.64, 1.69), P=0.01]and BMI [OR=1.58, 95%CI(1.17, 2.15), P=0.03] were independent risk factors for cartilage injury of more than degree 3 in enrolled patients. ROC analysis showed that MDI had more diagnostic value than BMI, and the critical value was 0.355 with a sensitivity of 89.1% and a specificity of 88.2%. CONCLUSION In doubt or accompanied by 40 to 60 years old patients with knee osteoarthritis, the MDI measured by non-weight-bearing knee MRI has predictive value for cartilage injury of more than degree 3 in medial tibiofemoral joint, and the critical value for diagnosis of cartilage injury of more than degree 3 is 0.355.
Male ; Female ; Humans ; Adult ; Middle Aged ; Aged ; Osteoarthritis, Knee/surgery* ; Cartilage, Articular/surgery* ; Knee Joint/surgery* ; Meniscus ; Menisci, Tibial/surgery* ; Cartilage Diseases ; Magnetic Resonance Imaging/adverse effects*

Relapsing polychondritis (RP) is a disease that confounds clinicians worldwide due to the difficulty in confirming its diagnosis and determining the best treatment plan. We report a case of a 34-year-old lady who presented with bilateral inflamed auricular swelling and red eyes, which turned out to be relapsing polychondritis. The patient was successfully treated with long-term oral steroids and is on regular follow-up. This report highlights how the subtle signs of her condition were picked up, leading to her diagnosis of RP and the treatment regime that successfully reversed her symptoms. The challenge of picking up subtle signs of the disease is best met by working as a team in a multidisciplinary setting. A literature study shows that RP is a rare disease that can present with many symptoms.
Polychondritis, Relapsing

Objective: To investigate the reduction effect of hoding cricoarytenoid joint reduction with visual laryngoscope under intravenous anesthesia. Methods: The therapeutic effects of 40 patients with arytenoid dislocation(AD)treated by closed reduction in the single center from January 2020 to September 2021 were retrospectively analyzed, including 21 males and 19 females, median age 48 years. The etiology, symptoms, preoperative evaluation methods, reduction mode, reduction times, and the recovery of arytenoid cartilage movement and sound after reduction were evaluated and analyzed. Results: All patients had obvious hoarseness and breath sound before treatment. Under stroboscopic laryngoscope or electronic nasopharyngoscope, different degrees of vocal cord movement disorder and poor glottic closure can be seen. There were 28 cases of left dislocation, 9 cases of right dislocation and 3 cases of bilateral dislocation. The etiology of dislocation of cricoarytenoid joint: 25 cases (62.5%) of tracheal intubation under general anesthesia were the most common causes, was as follows by laryngeal trauma, gastroscopy, cough, vomiting and so on. Among them, 28 cases of reduction were initially diagnosed in our department, and 12 cases were diagnosed later after failure of reduction treatment. Of the 40 patients, 6 underwent reduction 24 hours after dislocation; 18 cases from 3 days to 1 month; 7 cases from 1 to 3 months; 6 cases were reset in 3~6 months; Over 6 months in 3 cases. After one reduction, 10 cases (10/40, 25%) recovered normal pronunciation, 14 cases (14/40, 35%) recovered normal pronunciation after two reduction, 10 cases (10/40, 25%) recovered normal pronunciation after three times, 2 cases (2/40, 5%) recovered normal pronunciation after four times, and 1 case (2.5%) recovered normal pronunciation after five times. Thin slice CT scan of larynx and cricoarytenoid joint reconstruction showed the types of AD: subluxation in 37 cases (92.5%) and total dislocation in 3 cases; 28 cases of left dislocation, 9 cases of right dislocation and 3 cases of bilateral dislocation; 29 cases (72.5%) had posterior dislocation and 11 cases (27.5%) had anterior dislocation. All patients were treated by intravenous anesthesia with arytenoid cartilage clamped by cricoarytenoid joint reduction forceps under visual laryngoscope. The curative effect was evaluated by stroboscopic laryngoscope and/or voice analysis at 1-2 weeks after operation. The vocal cord movement returned to normal and the pronunciation was good in 37 cases (92.5%). Conclusions: Hoding cricoarytenoid joint reduction with the vision laryngoscope under intravenous anesthesia is easy to operate and the reduction effect is more stable. It is a effective method for AD.
Anesthesia, Intravenous/adverse effects* ; Arytenoid Cartilage/injuries* ; Female ; Humans ; Intubation, Intratracheal/adverse effects* ; Joint Dislocations/therapy* ; Laryngeal Diseases/etiology* ; Laryngoscopes/adverse effects* ; Male ; Middle Aged ; Retrospective Studies

OBJECTIVE: To analyze radiological characteristics of Muller-Weiss disease, evaluate the clinical value of the imaging examination in diagnosis of Muller-Weiss disease. METHODS: The imaging data of 26 patients with Muller-Weiss disease were collected from September 2015 to August 2020, including 7 males and 19 females, aged 43 to 68 years old with an average of (52.7±4.6) years old. In the X-ray examination observed the shape and position of the navicular bone. The talar-first metatarsal angle(TFM) was measured on the weight-bearing anteroposterior radiograph. The arch angle and angle between mid-axis of talus and mid-axis of the first metatarsal(Meary angle) were measured on the weight-bearing lateral radiographs. The morphology, density, adjacent joint space and position of the navicular bone were evaluated by computed tomography(CT), and magnetic resonance imaging(MRI) was used to observe the shape, signal, cartilage and surrounding soft tissue changes of the navicular bone. RESULTS: Among 26 patients, 21 cases were unilateral and 5 cases were bilateral;X-ray examination showed that the lateral part of navicular bone of foot was compressed and flattened, showing"comma like"or"drop like", navicular moved to the medial side, partial fragmentation of bone, peripheral articular hyperplasia, uneven density and narrowing of relationship gap. According to Meary angle and deformity degree of the affected foot on the lateral X-ray of the load-bearing foot, Maceira staging was performed. There were 0 cases in stageⅠ, 2 cases in stage Ⅱ, 11 cases in stage Ⅲ, 9 cases in stage Ⅳand 4 cases in stage Ⅴ. CT examination showed bone fragmentation, medial displacement of navicular bone and formation of the talocalcaneal joint. MRI examination showed the irregular shape and uneven signal of navicular bone, narrowing of joint space, talocalcaneal joint surface hyperplasia and cartilage destruction, tarsal joint effusion and swelling of surrounding soft tissue. CONCLUSION Muller-Weiss disease has specific imaging manifestation, and an accurate diagnosis can be made based on the patient's age, gender, and clinincal history. Preoperative imaging examination can stage the disease, help clinicians to formulate better surgical plans, and postoperative imaging examination can better evaluate the surgical effect.
Adult ; Aged ; Bone Diseases/diagnostic imaging* ; Cartilage Diseases ; Female ; Foot Diseases/diagnostic imaging* ; Humans ; Hyperplasia/pathology* ; Male ; Middle Aged ; Talus/pathology* ; Tarsal Bones/surgery* ; Tarsal Joints