Congenital airway abnormalities in neonates

Airway malformations such as laryngeal atresia, tracheal agenesis and subglottic stenosis are rare and present at birth with significant respiratory distress with or without stridor. There may be an initial improvement on bag and mask ventilation. Repeated attempts at intubation are met with failure. The related embryology and clinical aspect of airway malformations have been discussed. The prognosis in tracheal agenesis is universally fatal but cases with laryngeal atresia and subglottic stenosis may be saved with prompt tracheostomy and later surgical reconstruction.     

Congenital airway abnormalities in neonates

Airway malformations such as laryngeal atresia, tracheal agenesis and subglottic stenosis are rare and present at birth with significant respiratory distress with or without stridor. There may be an initial improvement on bag and mask ventilation. Repeated attempts at intubation are met with failure. The related embryology and clinical aspect of airway malformations have been discussed. The prognosis in tracheal agenesis is universally fatal but cases with laryngeal atresia and subglottic stenosis may be saved with prompt tracheostomy and later surgical reconstruction.     

电子支气管镜在儿童上气道梗阻性疾病中的诊断价值

目的 评价电子支气管镜在儿童上气道梗阻性疾病中的诊断价值及安全性.方法 对91例上气道梗阻患儿行电子支气管镜检查,总结分析病因.结果 (1) 儿童上气道梗阻病因依次为先天性喉气管软化合并感染(45例)、声门下异物(13例)、喉气管占位性病变(9例)、声门区水肿(6例)、气管狭窄(5例)、喉咽腔病变(咽后脓肿3例、肿物2例)、声门区增生(3例)、声门下狭窄(2例)、声带麻痹(2例)、喉蹼(1例);(2) 不同年龄段病因不同:新生儿期依次为声带麻痹、喉气管软化;~6个月依次为先天性喉气管软化、会厌囊肿,其他少见病因如气管狭窄、咽后脓肿、喉蹼等疾病;~1岁依次为喉气管软化、气管狭窄、声门下异物等;~3岁依次为声门下异物、声门区水肿、喉气管软化等;>3岁儿童1例为喉乳头状瘤.(3) 疾病好发的年龄段不同:先天性喉气管软化好发于6个月以下婴儿,气管异物好发于1~3岁幼儿,先天性会厌囊肿多见于3个月以下婴儿.(4) 并发症:16例有一过性血氧饱和度下降,11例出现气管支气管痉挛,术后8例有一过性发热,6例有短期喉鸣加重.结论 电子支气管镜在儿童上气道梗阻性疾病的诊断中起重要作用,可迅速、直观地判断病变部位和性质,避免误诊,有助于指导治疗.

Abstract：

Objective To investigate the diagnostic value and safety of bronchovideoscope in the pediatric upper airway obstruction.Methods Bronchovideoscope was performed in 91 pediatric patients with upper airway obstruction.The etiology was analyzed and summarized.Results (1) Our study showed that the etiology of pediatric upper airway obstruction were as follow in turn:congenital laryngo-trachemalacia (45 cases),subglottic foreign body (13 cases),laryngotracheal occupying lesion (9 cases),vocal area edema (6 cases),tracheal stenosis (5 cases),laryngopharyngeal lesion (3 cases of retropharyngeal abscess,2 cases of tumor),vocal area hyperplasia (3 cases),subglottic stenosis (2 cases),glottic paralysis (2 cases),laryngeal web (1 case).(2) The common etiology of pediatric upper airway obstruction was different with age.Neonatal period:glottic paralysis,laryngomalacia in turn;1~6 month:laryngo-trachemalacia,tracheal stenosis,subglottic foreign body in turn;1~3 year:subglottic foreign body,vocal area edema,laryngo-trachemalacia in turn,>3 year:laryngeal papilloma.(3) Specific disease had predominant age:laryngo-trachemalacia predominated in infants less than 6 month;tracheal foreign body was most common in child aged 1~3 years old;epiglottic cyst was most common in infant less than 3 month.(4) Complication:during procedure,16 patients had transient decrease of saturation of blood oxygen and 11 patients had tracheobronchial spasm.After procedure,8 patients had transient fever and 6 patients had transient aggravation of laryngeal stridor.Conclusion Bronchovideoscope plays an important role in the diagnosis of pediatric upper airway obstruction.It can directly identify position and nature of disease,and then guide treatment.     

Pediatric airway surgery

Pediatric airway surgery is a challenging field in pediatric surgery. Laryngotracheal stenosis has a variety of congenital and acquired conditions that require precise assessment and tailored treatment for each individual patient. About 90% of acquired conditions are represented by subglottic stenosis (SGS) resulting as a complication of tracheal intubation. Congenital tracheal stenosis (CTS) is a rare and life-threatening malformation, usually associated with complete tracheal rings along a variable length of the trachea. Tracheomalacia (TM) is a process characterized by flaccidity of the supporting tracheal cartilage, widening of the posterior membranous wall, and reduced anterior-posterior airway caliber. The clinical presentation can vary from almost asymptomatic patients to near fatal airway obstruction. There is considerable variation in both the morphologic subtypes and the prognosis of pediatric airway. The patients are divided into three clinical groups (mild, moderate, and severe). A further division was proposed according to the presence or absence of associated anomalies. The definitive diagnosis of pediatric airway was made by means of rigid bronchoscope and computed tomography scan with three-dimensional reconstruction (3D-CT). Rigid bronchoscopy and 3D-CT confirmed the diagnosis in all the cases. Other associated anomalies include congenital heart disease, vascular anomalies, and BPFM (maldevelopment of aerodigestive tract). After definitive diagnosis of pediatric airway lesions, surgical intervention should be considered. Surgical strategy was presented on each lesion.     

Subglottic Ductal Cysts Associated with Complete Tracheal Ring Deformity: Coexistence of Two Rare Airway Abnormalities with Fatal Outcome

Subglottic cysts are rare and may cause airway obstruction. Most cases are acquired secondary to endotracheal intubation, even short-term, particularly in the premature neonate. Complete tracheal rings are rare anomalies associated with tracheal stenosis. To our knowledge, the two have not been reported coexisting. A 16-month-old ex-premature boy was found unresponsive, with his tracheostomy tube dislodged. Tracheomalacia, subglottic stenosis, and a laryngeal cyst had been diagnosed shortly after birth, and the cyst was surgically treated at that time. At autopsy, a complete tracheal ring was noted in the subglottic region, above the tracheostomy site, and the tracheal diameter was markedly decreased in this area. In addition, multiloculated cysts were present at that level, arising from both anterolateral tracheal walls. These completely occluded the airway. To our knowledge, this is the first case of a combination of tracheal ring anomaly and subglottic cysts. Pediatric pathologists must be aware of iatrogenic lesions associated with care of the premature neonate, particularly as the age of viability continues to decrease.     

The upper airway: congenital malformations

The upper airway extends from the nasal aperture to the subglottis and can be the site of multiple types of congenital malformations leading to anatomical or functional obstruction. This can cause severe respiratory distress. Newborns are obligate nasal breathers; therefore nasal obstruction can lead to airway compromise and respiratory distress. The etiologies are varied and include, choanal atresia, pyriform aperture stenosis, and rarely tumors such as glioma, encephalocele, teratoma, or dermoid. More common upper airway congenital anomalies include laryngomalacia, vocal cord paralysis, and subglottic stenosis. Laryngolmalacia is the most common congenital laryngeal anomaly. Inspiratory stridor often does not present until two weeks after birth and resolves by 18 months of age. Most cases are managed with watchful waiting. Severe cases require a surgical intervention. Bilateral vocal cord paralysis is usually idiopathic. In certain cases, paralysis may occur secondary to central nervous system abnormality including Arnold-Chiari malformation, cerebral palsy, hydrocephalus, myelomeningocele, spina bifida, or hypoxia. Severe cases may necessitate endotracheal intubation and tracheostomy. Congenital subglottic stenosis is the third most common laryngeal anomaly. It is defined as a diameter of less than 4mm of the cricoid region in a full-term infant, and less than 3mm in a premature infant. This condition is the most common laryngeal anomaly that requires tracheotomy in newborns. Laryngotracheoplasty may be required to achieve decanulation. Knowledge of the upper airway embryological development and congenital anomalies is off prime importance in assessing the newborn with respiratory distress. In most cases flexible endoscopy establishes the diagnosis. Management is tailored to each condition and its degree of severity.     

Congenital tracheal malformations

Congenital malformations of the trachea include a variety of conditions that cause respiratory distress in neonates and infants. A number of anomalies are self-limiting while others are life-threatening and require immediate therapy. The prevalence of congenital airway malformations has been estimated to range between 0.2 and 1 in 10,000 live births. The most frequent congenital tracheal malformations are: tracheomalacia, congenital tracheal stenosis, laryngotracheal cleft and tracheal agenesis. The management of congenital tracheal malformations is complex and requires an individualized approach delivered by a multidiscipilinary team within centralized units with the necessary expertise.     

先天性心脏病围手术期气管狭窄的综合治疗

目的 通过分析先天性心脏病围手术期气管狭窄患儿的治疗及转归,探讨不同原因导致气管狭窄的治疗方法.方法 回顾性分析2012年1月至2015年10月收治的60例气管狭窄合并先天性心脏病患儿的临床资料.其中,男42例,女18例;年龄1～144个月,平均4个月;体重(6.65±3.40) kg.先天性心脏病合并先天性气管狭窄39例,继发性气管狭窄21例.结果 先天性气管狭窄合并先天性心脏病39例,实施先天性心脏病矫治+气管狭窄旷置28例,先天性心脏病矫治+气管成形术5例,先天性心脏病矫治+气管支架置入1例;术后远期死亡共计4例,放弃手术治疗死亡4例,放弃治疗门诊随诊1例.继发性气管狭窄21例,实施心脏畸形矫治后气管软化狭窄减轻,不需手术治疗.结论 先天性心脏病合并先天性气管狭窄的主要致病原因在于其完全性软骨环导致气管内径增速缓慢,气道梗阻严重,即使实施心脏手术也不能完全改善其气管狭窄症状,必要时需同时实施气道成形术.继发性气管狭窄的主要致病原因在于气管临近大血管的压迫,缓解血管环畸形后气管狭窄即刻缓解,疗效满意,无需实施气管成形术.     

Severe upper airway stenosis in a boy with partial monosomy 16p13.3pter and partial trisomy 16q22qter

We report the case of a boy with a de novo partial monosomy 16p13-pter and partial trisomy 16q22-qter detected by fluorescence in situ hybridization using subtelomeric probes for 16p and 16q. The boy had facial characteristics, skeletal features, congenital heart defects, an imperforate anus, urogenital malformations, pre/postnatal growth retardation, and psychomotor retardation, most of which have been reported both in partial monosomy 16p and partial trisomy 16q. In addition, he suffered from upper airway stenosis due to possible laryngeal stenosis with subglottic webs. The upper airway stenosis could be a rare complication of partial monosomy 16p or partial trisomy 16q, or a nonspecific malformation resulting from chromosomal abnormalities.     

Spiral computed tomography with 3-dimensional reconstruction for the diagnosis of tracheobronchial stenosis

We report on the usefulness of spiral computed tomography (CT) with 3-dimensional (3D) reconstruction in the diagnosis of tracheobronchial abnormalities based on three cases of children with congenital tracheobronchial strictures. Images were reconstructed using a curved planar reformat and shaded surface display. The images obtained from our three cases of tracheal stenosis, subglottic stenosis and tracheobronchial strictures were extremely clear. Spiral CT with 3D reconstruction provides excellent anatomic delineation of the tracheobronchial airway, and is safe and less invasive than tracheobronchography.     

Anomalous Left Pulmonary Artery Without Sling Formation

A case of aberrant left pulmonary artery originating from the right pulmonary artery resembling pulmonary artery sling malformation, but without true sling formation, is presented. Anomalous left pulmonary artery was accompanied with long-segment proximal tracheal stenosis and other congenital malformations typically associated with PAS complex. The anomalous pulmonary artery passed anterior to the trachea; therefore, no true sling was formed. Because no airway compression by the aberrant left pulmonary artery was detected, the proximal tracheal stenosis was thought to be primary.     

纤维支气管镜在大血管疾病合并气道狭窄中的诊断价值

目的 评价纤维支气管镜(简称纤支镜)在先天性大血管病变合并气道狭窄中的诊断价值及安全性.方法回顾性分析2005年10月至2009年6月NICU收治的经纤支镜检查确诊气道狭窄的先天性大血管病变患儿34例,其中血管环10例,主动脉梗阻性病变24例,年龄6 d～11个月,体重2.2～8.7 kg[(4.6±1.4)kg];记录纤支镜发现的气道狭窄情况、CT及手术所见确诊的心血管畸形及气道狭窄情况,总结分析纤支镜检查发现的气道狭窄与心血管畸形的关系、气道狭窄的处理及转归.结果 所有病例均在NICU或手术过程中顺利完成纤支镜检查.(1)34例气道狭窄的首发症状为气促、喘鸣、反复肺部感染和呼吸机依赖.(2)10例血管环患儿均先经纤支镜检查,发现气道外压性狭窄,提示血管环,气道狭窄以气管下段为主;9例经CT、1例经手术证实为血管环,其中7例伴先天性气管狭窄.(3)24例主动脉病变中5例为术前CT提示气管狭窄,其中1例纤支镜提示气道狭窄,余19例在主动脉梗阻性畸形矫治术中、术后经纤支镜发现气道压迫狭窄.24例主动脉病变中21例有左主支气管狭窄,2例有先天性气管狭窄.纤支镜检查与CT检查对气管狭窄的诊断基本吻合.(4)34例纤支镜检查过程中2例出现一过性血氧饱和度下降,5例出现一过性心动过速,其余患儿无不良反应.结论 纤支镜检查在大血管畸形伴气道狭窄的诊断中起重要作用,具有准确、安全、方便、快捷的优点,但对气管狭窄的情况、气管周围结构和血管畸形的了解需结合CT检查.     

Safety and efficacy of flexible endoscopy in children with bronchopulmonary dysplasia

Because concern has been raised about the efficacy and safety of flexible fiberoptic bronchoscopy (FFB) in pediatric patients with chronic cardiopulmonary disorders, we reviewed the results of 129 flexible endoscopies performed on 47 children with a history of bronchopulmonary dysplasia (BPD) at our institution over a 44-month period. Indications for FFB; weight and age of the patient; and procedure format, including medication usage, findings, specimen results, and complications, were analyzed. Evaluation of previously diagnosed subglottic stenosis and airway abnormalities were the two most common indications (33% and 32%, respectively). Persistent or recurrent infiltrates or atelectasis, need for cultures, stridor, failure to extubate, hoarseness, and persistent wheeze were also cited. Endoscopic diagnoses included adenoidal hypertrophy, laryngomalacia, vocal cord abnormalities, interarytenoid membrane, subglottic stenosis, granulomas, tracheobronchomalacia, stenosis, obstruction, generalized inflammation/edema, polyps, tracheal bronchi, and anomalous bronchial anatomy. Cytomegalovirus, pneumococcus, nontypeable Haemophilus influenzae, Pseudomonas, or mixed gram-negative flora were isolated from some patients without tracheostomy. Minor complications (transient bradycardia, mild nasopharyngeal bleeding, and mild worsening of upper airway obstruction) occurred in 3.1% of procedures, but no severe complications occurred. Management was directly affected by procedure results in 41% of procedures. We concluded that the FFB can be a safe, useful procedure in the management of children with BPD.     

Subglottic Stenosis

Subglottic stenosis refers to narrowing of the airway diameter below the vocal folds and may be congenital or acquired. Typical signs and symptoms range from recurrent croup and exertional stridor to complete airflow obstruction requiring tracheotomy. Management of moderate and severe subglottic stenosis often requires intricate surgical techniques. To optimize the success of these surgeries, a thorough assessment of the child’s airway, lungs, reflux, and swallow needs to be evaluated. In order to provide concerted and coordinated care between typically otolaryngology (ENT), pulmonary, gastroenterology (GI), speech, swallow and language pathologists (SLP), “aerodigestive” teams have been developed and increasing in prevalence at children’s medical hospitals. This article sets out to provide a brief overview of an aerodigestive program and evaluation, review a few of the more common laryngotracheal conditions, and the surgical techniques involved to augment the airway.     

MRI in tracheal stenosis by innominate artery in children

Pediatric airway obstruction due to anomalies of the course of the innominate artery may produce respiratory distress. MR imaging of the trachea was performed after bronchoscopy on forty-one children with congenital tracheal stenosis. Bronchoscopy only allows the evaluation of the lumen of the trachea, and the degree and location of collapse, and it may be difficult to determine the etiology of the tracheal narrowing. In eighteen out of the forty-one patients MR imaging showed a compression of the trachea by the innominate artery. The MR imaging diagnoses were subsequently compared for accuracy with the diagnoses determined by direct surgical observations. MR imaging of the trachea, the surrounding tissue and vessels allows the evaluation of the cause of tracheal compression and the degree and location of collapse. For evaluation of the cause of airway obstruction, MRI is an ideal method depicting detailed anatomic structure without employing ionizing radiation or intravenous contrast medium.     

Review of intubation in severe laryngotracheobronchitis

Of 208 children who required relief of severe airway obstruction due to laryngotracheobronchitis by an artificial airway (nasotracheal intubation or tracheostomy) during a 10-year-period, 181 (87%) were intubated and later extubated. Twenty-seven children (13%) had tracheostomies performed. The tracheostomies were for severe subglottic narrowing precluding the passage of an adequate size endotracheal tube in 10 children, and for severe endotracheal tube trauma in 17 children. Five children developed acquired subglottic stenosis (2.4% of 208) and 1 of these has a retained tracheostomy. One child died of cardiac disease. The remaining 202 children had no long-term complications of laryngotracheobronchitis, intubation, or tracheostomy. It is concluded that nasotracheal intubation is a satisfactory artificial airway for laryngotracheobronchitis. Endoscopic evaluation in a selected group of these children will identify those with significant intubation trauma or severe subglottic narrowing in whom continued intubation may cause permanent subglottic damage. The low incidence of acquired subglottic stenosis in this series supports the practice of selective endoscopy and tracheostomy.     

Respiratory distress in neonates

Respiratory distress due to either medical or surgical causes occurs commonly in neonates. It is the most common cause of admission to a neonatal surgical intensive care facility in a tertiary care hospital. The distress can be caused by a variety of clinical conditions; common conditions treated in medical intensive care units are transient tachypnea of the new born, respiratory distress syndrome, pulmonary air leak and pneumothorax. In surgical causes of respiratory distress in neonates the underlying mechanisms include airway obstruction, pulmonary collapse or displacement and parenchymal disease or insufficiency; the common causes are congenital diaphragmatic hernia, congenital cystic adenomatoid malformation, congenital lobar emphysema and esophageal atresia with or without tracheo-esophageal fistula. Obstructive lesions of the new born airway include choanal atresia, macroglossis, Pierre-Robin syndrome, lymphangioma, teratoma or other mediastinal masses, cysts, subglottic stenosis and laryngo tracheomalacia. Imaging plays a very major role in the pre-operative diagnosis of these conditions and proper pre-operative resuscitation helps in improving the results of surgery dramatically.     

婴幼儿气道狭窄的诊疗现状与研究进展

婴幼儿气道狭窄是由先天性或后天性因素所造成的呼吸道管腔缩小,导致呼吸和发音功能障碍.近些年,随着纤维支气管镜在NICU与PICU应用的发展,先天性婴幼儿气道狭窄的诊断率明显提高;随着极早产、极低出生体重儿存活率的增高,气管插管相关性婴幼儿气道狭窄的发病率也在逐步升高;随着外科手术方式的进步以及纤维支气管镜诊疗应用的普及,过去诊疗困难、病死率极高的先天性气道畸形狭窄,在诊治与预后方面也有了巨大的提升.该文综述近年来婴幼儿气道狭窄的诊疗现状与新进展.     

The ex utero intrapartum treatment (EXIT) procedure: Application of a new therapeutic paradigm

The ex utero intrapartum treatment (EXIT) procedure is a term given to a technique that can transform a potentially fatal neonatal emergency to a controlled intervention with an improved outcome. It has revolutionised the care of prenatally diagnosed congenital malformations in which severe upper airway obstruction is anticipated. An extended period of utero‐placental circulation can be utilised to avoid profound cardiopulmonary compromise. Its therapeutic applications have been broadened to include fetuses with congenital diaphragmatic hernia after tracheal plugging, high‐risk intrathoracic masses, severe cardiac malformations and conjoined twins. It requires the co‐ordination of a highly skilled and experienced multidisciplinary team. The recent enthusiasm for the EXIT procedure needs to be balanced against maternal morbidity. Specific indications and guidelines are likely to be refined as a consequence of ongoing advances in fetal intervention and antenatal imaging.     

Inspiratory stridor in a 12-month-old girl

Congenital tracheal stenosis is a rare disorder, characterized by the presence of focal or diffuse complete tracheal cartilage rings, resulting in a fixed tracheal narrowing. The entity may be seen in isolation or in conjunction with other malformations that include an anomalous right upper lobe bronchus. We here report the case of a 12-month-old girl who had severe bi-segmental congenital tracheal stenosis with the interposition of a tracheal bronchus between the two stenotic segments.