BACKGROUND AND OBJECTIVES: Adequate evaluation of the upper airway is critical in the management of pediatric patients with stridor or extubation failure. For this purpose, we designed an evaluation protocol of the upper airway for these patients, in collaboration with Dept. of Pediatrics, Intensive care team and Anesthesiology. Here we present the clinical results of our evaluation protocol and provide information about the etiology and management of the upper airway problems. SUBJECTS AND METHOD: Clinical data of 380 pediatric patients (M:F=231:149) having airway evaluation for their problems (stridor or extubation failure) were retrospectively analyzed. Among them, patients of age less than 3 months ranked first (30.0%). Comorbidities of pulmonary diseases (30.8%) and cardiovascular diseases (29.5%) were found. The pre and post-evaluation diagnosis, management and prognosis were evaluated and the usefulness of an airway evaluation protocol was discussed. RESULTS: Frequent pre-evaluation diagnoses were subglottic stenosis (55.2%), laryngomalacia (12.6%) and tracheal stenosis (9.2%) and these were changed to subglottic stenosis (44.5%), laryngomalacia (9.7%), tracheal stenosis (6.6%) and no abnormality (14.5%). Particularly, 50% of pre-diagnosis laryngomalacia, 25% of subglottic stenosis and 37% of tracheal stenosis were corrected to other causes by airway evaluation. The procedures were exam only (41.6%), endoscopic dilatation (20.8%) and tracheostomy (17.9%). In 190 out of 380 (50.0%), extubation was successful, but 151 patients (39.7%) had tracheostomy tube. CONCLUSION: Adequate evaluation of the upper airway in pediatric patients with stridor or extubation failure can facilitate the diagnosis and management of their problems.
Anesthesiology ; Cardiovascular Diseases ; Comorbidity ; Constriction, Pathologic ; Cooperative Behavior ; Diagnosis ; Dilatation ; Humans ; Critical Care ; Laryngomalacia ; Lung Diseases ; Pediatrics ; Prognosis ; Respiratory Sounds* ; Retrospective Studies ; Tracheal Stenosis ; Tracheostomy

We report a case of de novo 7q interstitial deletion detected by conventional karyotyping and by microarray of amniotic fluid sampled during the prenatal period. A 32-year-old pregnant woman was evaluated at our hospital following detection of increased nuchal translucency at 12 weeks and 5 days of gestation. Conventional karyotyping revealed 46,XX,del(7)(q21q22) in 20 interphase mitotic cells, and high-resolution microarray revealed 12.8 Mb (90,625,014-103,430,901) deletion in the region 7q21.13q22.1. Both parents had normal karyotypes. After birth, the neonate displayed several anomalies, including palatine cleft, upslanted and wide palpebral fissure, low-set ears, micrognathia, microcephaly, ventriculomegaly, subglottic tracheal stenosis, hearing loss, and hand/foot deformities, including brachydactyly, polydactyly, and cutaneous syndactyly. This case study helps explain the phenotype-genotype relationship in patients with 7q21.13q22.1 deletion.
Adult ; Amniotic Fluid ; Brachydactyly ; Congenital Abnormalities ; Ear ; Female ; Hearing Loss ; Humans ; Infant, Newborn ; Interphase ; Karyotype ; Karyotyping ; Microcephaly ; Nuchal Translucency Measurement ; Parents ; Parturition ; Polydactyly ; Pregnancy ; Pregnant Women ; Prenatal Diagnosis* ; Syndactyly ; Tracheal Stenosis

Child ; Diagnostic Errors ; Foreign Bodies ; diagnosis ; Humans ; Trachea ; Tracheal Stenosis ; diagnosis

OBJECTIVETo explore the diagnostic and therapeutic methods for perioperative children with congenital heart disease (CHD) with airway stenosis in pediatric intensive care unit (PICU).

METHODFiberoptic bronchoscopy was used for the diagnosis of 100 CHD cases in PICU who were clinically considered to have possible airway malformation because of complicated difficult-to-control lung infection, atelectasis and failure with the ventilator after surgery from January 2010 to October 2011. Cases who were confirmed to have severe airway stenosis by bronchoscopy and weaning from the ventilator after surgery were treated with balloon expandable stents into the desired position in the bronchoscopy.

RESULTThere were 73 cases (73%) of CHD patients with airway abnormalities, including 31 cases of severe stenosis (31%), moderate stenosis in 29 cases (29%), mild stenosis in 13 cases (13%). Nine of the 10 children in whom the mechanical ventilation was hard to be stopped after surgery because of severe airway stenosis were weaned from mechanical ventilation successfully by fiberoptic bronchoscopy, while one case died from primary disease with severe sepsis after the placement of bronchial stents.

CONCLUSIONCHD children with difficult-to-control lung infection, atelectasis and failure with ventilator after surgery are often complicated with airway abnormalities. The therapeutic bronchoscopy with airway stent can be used for cases with weaning from the ventilator because of severe airway stenosis.

Airway Obstruction ; diagnosis ; etiology ; therapy ; Bronchoscopy ; methods ; Constriction, Pathologic ; Female ; Follow-Up Studies ; Heart Defects, Congenital ; complications ; diagnosis ; surgery ; Humans ; Infant ; Infant, Newborn ; Intensive Care Units, Pediatric ; Lung Diseases ; diagnosis ; etiology ; therapy ; Male ; Perioperative Period ; Respiration, Artificial ; Stents ; Trachea ; abnormalities ; Tracheal Stenosis ; diagnosis ; etiology ; therapy ; Treatment Outcome

OBJECTIVETo investigate the clinical characteristics, diagnosis and therapy of Keutel syndrome, and thereby to minimize the misdiagnosis.

METHODData of a case of Keutel syndrome diagnosed at the Provincial Hospital Affiliated to Shandong University were analyzed and related literature were reviewed.

RESULTAn 8-month-26-day-old boy was presented with inspiratory and expiratory stridor and wheezing after movement on lung auscultation. His craniofacial appearance was characterized by midfacial hypoplasia with a broad depressed nasal bridge. The nose was small and flat. A grade 2-3/6 systolic murmur was heard between the second and third ribs at left edge of the sternum. The end phalanges of his fingers were thickened. Chest radiograph showed tracheobronchial cartilage calcification and tracheobronchial stenosis. Echocardiographic examination revealed the right pulmonary stenosis. With endoscopic surgery, antiobstructive and antibiotic therapy clinical symptoms were improved. Three weeks later he died of lung reinfection after he was discharged from our hospital. English literature search with "Keutel syndrome" as the key word at "PubMed" showed 22 articles covering 26 patients, and the clinical symptoms were hearing loss (91%), persistent respiratory symptoms (68%), recurrent otitis media/sinusitis (67%), growth development delay (52%) in turn, and signs were brachytelephalangism (100%), low nasal bridge (95%), midfacial hypoplasia (93%), cardiac murmur (69%), and auxiliary examinations showed abnormal cartilage calcification (100%), pulmonary arterial stenosis (72%), tracheobronchial stenosis (50%).

CONCLUSIONThe diagnosis of Keutel syndrome should be considered in patients with brachytelephalangism, abnormal cartilage calcification, peripheral pulmonary stenosis, and midfacial hypoplasia. Tracheal stenosis was main clinical manifestation in part of patients.

Abnormalities, Multiple ; diagnosis ; diagnostic imaging ; therapy ; Bone and Bones ; diagnostic imaging ; Calcinosis ; diagnosis ; diagnostic imaging ; therapy ; Cartilage ; diagnostic imaging ; Cartilage Diseases ; diagnosis ; diagnostic imaging ; therapy ; Diagnosis, Differential ; Hand Deformities, Congenital ; diagnosis ; diagnostic imaging ; therapy ; Humans ; Infant ; Male ; Pulmonary Valve Stenosis ; diagnosis ; diagnostic imaging ; therapy ; Radiography, Thoracic ; Retrospective Studies ; Tomography, X-Ray Computed ; Tracheal Stenosis ; diagnosis ; diagnostic imaging

BACKGROUND AND OBJECTIVES: Vascular rings refer to anomalies of the great arteries that cause respiratory or feeding problems. The purpose of this study was to analyze a series of patients with vascular rings and evaluate associated risk factors for mortality. SUBJECTS AND METHODS: A retrospective review of all patients identified with vascular rings between 1997 and 2010 in the Seoul National University Children's Hospital. RESULTS: Thirty-five patients were diagnosed with vascular rings (median age at diagnosis, 7 months). The vascular rings of 32 patients were confirmed by cardiac computed tomography or magnetic resonance imaging. The types of vascular rings were: a double aortic arch in ten patients, a right aortic arch with persistent left ligamentum arteriosum in seven, an aberrant subclavian artery in seven, a pulmonary sling in eight, and others types in three patients. Eleven patients were asymptomatic. Gastrointestinal and respiratory symptoms were seen in ten and sixteen patients, respectively. Associated cardiovascular anomalies were present in fifteen patients. Twenty patients with definite symptoms underwent surgical treatment. The median interval between diagnosis and operation was 6 days. Four patients eventually died; three deaths were associated with complex heart diseases, and one had pulmonary artery sling with severe tracheal stenosis. Only the presence of a complex heart disease significantly influenced mortality (p=0.002). CONCLUSION: Vascular rings include several types of anomalies, each with a different prognosis and symptoms. The presence of a complex heart disease was significantly associated with mortality. Early diagnosis and timely surgery in symptomatic patients are essential.
Aneurysm ; Aorta, Thoracic ; Arteries ; Cardiovascular Abnormalities ; Deglutition Disorders ; Early Diagnosis ; Heart Diseases ; Humans ; Magnetic Resonance Imaging ; Prognosis ; Pulmonary Artery ; Retrospective Studies ; Risk Factors ; Subclavian Artery ; Tracheal Stenosis

BACKGROUNDVascular rings are uncommon anomalies in which preferred strategies for diagnosis and management may vary among institutions. In this study, we reported our approach and a review of our 5-year experience.

METHODSFrom May 2006 to April 2011, 45 children (31 boys) with vascular rings underwent surgical repair at Beijing Children's Hospital. Nineteen patients (26%) had associated heart anomalies.

RESULTSThere were two hospital deaths. At follow-up, 11 patients still had intermittent respiratory symptoms, but these symptoms had no effect on growth or physical activities. No patients required reoperation.

CONCLUSIONSThe rates of misdiagnosis and missed diagnosis of vascular rings are higher than those of other congenital heart diseases. A high index of clinical suspicion coupled with the use of computed tomography enables early diagnosis. Surgical repair can be performed successfully, although a number of patients will have persistent symptoms.

Child, Preschool ; Female ; Humans ; Infant ; Male ; Tracheal Stenosis ; mortality ; surgery ; Vascular Malformations ; diagnosis ; mortality ; surgery

Child ; Humans ; Laryngostenosis ; diagnosis ; therapy ; Tracheal Stenosis ; diagnosis ; therapy

Child ; Child, Preschool ; Female ; Heart Defects, Congenital ; complications ; Humans ; Infant ; Infant, Newborn ; Male ; Tracheal Stenosis ; diagnosis ; therapy

OBJECTIVE: To study the clinical manifestations and treatments of closed injuries of the cervical trachea. METHOD: We carried out a retrospective study of the clinical manifestations, diagnosis and treatment of patients with closed injuries of the cervical trachea that have been treated in our hospital over the last ten years. We analyzed commonly occurring clinical problems, key points of surgical treatment, and postoperative recovery. Fourty-one patients with acute closed injuries of the cervical trachea underwent emergency tracheotomy or anesthesia orotracheal intubation and surgical repair of the trachea within 24 to 48 hours. Twenty six patients had their tracheotomy tubes extubated after surgery and had their anesthesia orotracheal tubes extubated within 48 hours postoperatively; tracheotomy tubes remained in the airway in 15 patients postoperatively, and of them, 12 had extubation successfully in 2 weeks postoperatively, while the remaining 3 were left intubated due to laryngotracheal stenosis. Among the 3, 2 underwent further surgical repair and recovery, and 1 had a stent inserted. RESULT: Twenty-eight patients fully recovered postoperatively. Ten patients mostly recovered postoperatively exception for hoarseness, and their fundamental phonation function recovered within 2 to 3 months but with poor movement of the vocal cords. The breathing and swallowing function of the other 3 patients recovered after the surgery, but they suffer from hoarseness. CONCLUSION In the treatment of closed disruption of the cervical trachea, prompt diagnosis and timely surgical repair of the structure and function of the trachea are key to saving the patients' lives and avoiding tracheal stenoses.
Adolescent ; Adult ; Female ; Humans ; Male ; Middle Aged ; Neck Injuries ; diagnosis ; surgery ; Retrospective Studies ; Rupture ; Trachea ; injuries ; Tracheal Stenosis ; Tracheotomy ; Young Adult