Familial vocal cord dysfunction

Vocal cord paralysis is a common cause of neonatal stridor. Familial vocal cord dysfunction, however, is unusual. All three siblings in one family had neonatal stridor. Vocal cord dysfunction was confirmed after endoscopic examination in two of the children; a temporary tracheotomy was required by one child. Results of evaluation, including pulmonary function tests, suggest discrete dysfunction localized to the neuromuscular pathway responsible for vocal cord abduction. Endoscopy is of prime importance in the diagnosis of vocal cord dysfunction. In considering therapy, the physician must weigh both the potentially life-threatening nature of vocal cord paralysis, as well as the likelihood of eventual spontaneous resolution of many familial and idiopathic cases.     

Ductus aneurysm as a rare cause of inspiratory stridor in the newborn infant

A paralysis of the left vocal cord was seen by laryngoscopy in a 3-days-old boy with inspiratory stridor. A ductus aneurysm was established by angiocardiography, which was believed to be the cause of the vocal cord paralysis. Within 8 days after the angiocardiography the aneurysm became smaller and the stridor disappeared. Twelfth months later the control-angiocardiography showed the total obliteration of the ductal aneurysm.     

Stridor: intracranial pathology causing postextubation vocal cord paralysis

During an 18-month period in a pediatric intensive care unit, nine patients with vocal cord paralysis were identified using flexible bronchoscopy. When tracheally extubated, each child was found to have stridor. The children ranged in age from 17 days to 5 1/2 years. Two patients had unilateral paralysis, but neither required tracheostomy. Seven patients displayed bilateral abductor vocal cord paralysis. Of these, six patients required tracheostomy. Surgical injury to the recurrent laryngeal nerve was the probable cause in two patients. The other seven patients had neurologic disorders with documented or suspected increases of intracranial pressure. Four of the seven patients with bilateral abductor vocal cord paralysis regained cord mobility within 4 months. Both children with unilateral cord paralysis have no stridor and vocalize well 1 year later. Cord paralysis in the setting of intracranial hypertension probably results from compression or ischemia of the vagus nerve before it exits the skull. Early visualization of the larynx should be done in patients who become stridulous when extubated, especially those with prior thoracic procedures or with neurologic disorders associated with intracranial hypertension.     

Neonatal vocal cord paralysis following extracorporeal membrane oxygenation

Five cases of unilateral vocal cord paralysis/paresis were diagnosed following extracorporeal membrane oxygenation for newborn respiratory failure. All were right sided and transient in nature. None of the five patients had other findings commonly associated with vocal cord palsy. The extracorporeal membrane oxygenation procedure requires surgical dissection in the carotid sheath on the right side of the neck, an area immediately adjacent to both the vagus and recurrent laryngeal nerve. It is speculated that vocal cord paralysis in these infants was acquired as a result of the extracorporeal membrane oxygenation cannulation. Although the vocal cord paralysis resolved in all cases, two patients had difficult courses after extracorporeal membrane oxygenation. Therefore, laryngoscopic examination should be considered for patients after extracorporeal membrane oxygenation.     

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.     

Atypical presentations in Chiari II malformation

Myelomeningocele with Chiari II malformation and hydrocephalus is a common association seen in infants with a congenital failure of neurulation. Here we report two cases of such an association presenting with different sets of problems. The first patient presented with severe inspiratory stridor due to bilateral abductor vocal cord paralysis, which was relieved completely within 24 h of definitive surgery. The second patient experienced intraoperative cardiac arrest. Definitive surgery was followed after successful cardiopulmonary resuscitation. The cause of the perioperative sequence of events in both cases is attributed to the associated pathologies of Chiari II malformation.     

Neonatal vocal cord palsy. Clinical and therapeutical approaches

Between 1998 and 2000, four newborns have been observed for laryngeal stridor occurred some hours after birth. Otorhinolaryngologic examinations, as well as cardiac, neuroradiologic and serologic investigations have been performed to formulate the diagnosis and verify the etiology. The fiberoptic laryngoscopy showed a bilateral paralysis of the vocal cord in two newborns, a monolateral paralysis of the left vocal cord in another and in the last one, instead, a bilateral cordal hypomobility. The follow-up performed till the age of one year showed a complete remission of the symptomatology in two newborns, respectively in the one with monolateral paralysis and in the other affected by hypomobility of the vocal cords; of the two newborns with bilateral paralysis, instead, one is dead because of Haemophilus Influenzae epiglottitis, three weeks after discharge without physicians' consensus, while in the other patient, affected by lobar holoprosencephaly, it was necessary to perform a tracheotomy because of a severe obstructive apnea. The lobar holoprosencephaly, is a cerebral malformation characterized by the partial separation of the cerebral hemispheres, and it is described for the first time associated with bilateral vocal cords paralysis.     

超细电子鼻咽喉镜在诊断婴幼儿咽喉疾病中的应用

目的 探讨电子鼻咽喉镜在婴幼儿咽喉疾病诊断中的应用价值.方法 采用Pentax-1130型超细电子鼻咽喉镜对121例喉喘鸣伴有不同程度吸气性呼吸困难或声音嘶哑的婴幼儿(包括新生儿)进行了检查.结果 121例患儿中喉软化症66例(54.5%),咽喉部先天性囊肿29例(24.0%),(2.5%),咽后肿物、喉纤维瘤、声门型喉蹼、下咽异物和气管狭窄各1例(0.8%).结论 121例伴有不同程度吸气性呼吸困难的喉喘鸣患儿中,除喉软化症以外,有近一半(45.5%)的患儿经过电子鼻咽喉镜检查在咽喉部发现了器质性病变.超细电子鼻咽喉镜在诊断婴幼儿及新生儿咽喉疾病中具有很大优势,随诊随查,不必预约,不需要镇静,可在自然呼吸状态下检查,并发症少,图像清晰,结果可靠,在临床上可推广使用.     

Bilateral idiopathic vocal cord palsy

We present 3 cases of bilateral vocal cord palsy who presented with acute respiratory distress with features of upper airway obstruction requiring tracheostomy. No cause could be found despite clinical evaluation and laboratory investigations. This diagnosis should be considered when child presents with upper airway obstruction emergency after ruling out other important causes of stridor and laryngoscopic examination is warranted in such cases for diagnosis.     

Neonatal vocal cord paralysis-an early presentation of hereditary neuralgic amyotrophy due to a mutation in the SEPT9 gene

Hereditary neuralgic amyotrophy is a rare autosomal dominant disorder involving recurrent episodes of painful brachial plexus neuropathies. Involvement of other nerves has been described in some families. The age of onset is from infancy to adulthood. Mutations in the SEPT9 gene were identified in approximately half of the hereditary neuralgic amyotrophy families.We evaluated a family with six affected members from three generations with a point mutation in the SEPT9 gene. One of the patients presented in the neonatal period with vocal cord paralysis necessitating intubation and prolonged ventilation.The neonatal presentation of vocal cord paralysis broadens the phenotypic spectrum of hereditary neuralgic amyotrophy. The identification of a SEPT9 mutation in a neonate with respiratory distress due to vocal cord paralysis expands the differential diagnosis in these patients.     

672例儿童声嘶的病因分析及对策

目的探讨儿童声嘶的常见病因及预防措施。方法回顾性分析672例声嘶患儿临床资料，对声嘶原因及诊治结果进行分析。结果本组672例中，慢性喉炎303例，占45．08％；声带小结188例，占27．97％；喉软骨软化症89例，占13．24％；声带瘫痪11例，占1．63％；喉乳头状瘤38例，占5．6％；喉异物12例，占1．78％，先天性喉室带发音困难6例，占0．89％；先天性喉蹼8例，占1．19％；会厌囊肿4例，占0．59％；声带肿块3例，占0．44％；声带息肉6例，占0．89％；喉真菌感染1例，占0．14％；喉结核3例，占0．45％。全组依据不同病因采取相应治疗，疗效满意，好转及治愈率为92．26％。结论儿童声嘶病因复杂，应采取针对性治疗措施，慢性喉炎、声带小结与喉软骨软化症是引起儿童声嘶的主要病因。     

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.     

Respiratory obstruction and apnea in infants with bilateral abductor vocal cord paralysis, meningomyelocele, hydrocephalus, and Arnold-Chiari malformation

This report describes 21 infants and children with bilateral abductor vocal cord paralysis and associated meningomyelocele, Arnold-Chiari malformation, and hydrocephalus. Two life-threatening forms of respiratory distress are distinguished: (1) upper airway obstruction due to bilateral abductor cord paralysis and (2) apnea. Clinically significant episodes of apnea were documented in 13 infants. Ten infants had evidence of aspiration and dysphagia. Vocal cord paralysis, apnea, aspiration, and dysphagia were frequently temporally related to increased intracranial pressure.     

Congenital stridor due to bilateral vocal cord palsy

Congenital stridor is one of the rare presentations of respiratory distress at birth. The commonest cause of congenital stridor is laryngomalacia, which accounts for 60% of the causes. The other common causes are congenital subglottic stenosis and vocal cord palsy (VCP). VCP is usually unilateral and most often linked with birth trauma, and is temporary. Bilateral palsy can be associated with other congenital anomalies. The current report describes a case of congenital bilateral VCP, not related to birth trauma and severe enough to require tracheostomy.     

Congenital airway anomalies

Congenital airway anomalies (CAA) include a variety of conditions that cause respiratory distress in neonates and infants. These malformations occur at various anatomic levels and manifest in a wide spectrum of airway symptoms, with presentation significantly influenced by the level at which obstruction occurs as well as by the severity of obstruction. The prevalence of congenital airway malformations has been estimated to range between 0.2 and 1 in 10,000 live births. The most frequent CAA are laryngomalacia, bilateral vocal cord paralysis, subglottic stenosis, laryngeal webs, subglottic hemangioma, tracheomalacia, congenital tracheal stenosis, laryngotracheal cleft, and tracheal agenesis.     

Ground zero: not asthma at all

Upper airway obstruction is commonly misdiagnosed as asthma. We report on four children with recurrent respiratory symptoms who had been erroneously diagnosed as having asthma and who received anti‐asthma medication for several years. The evaluation of spirometry tracing was neglected in all cases. Subglottic stenosis, tracheomalacia secondary to tracheo‐esophageal fistula, double aortic arch, and vocal cord dysfunction were suspected by direct inspection of the flow–volume curves and eventually diagnosed. The value of clinical history and careful evaluation of spirometry tracing in children with persistent respiratory symptoms is critically discussed.     

Stridor caused by vocal cord malfunction associated with emotional factors

We describe two adolescent patients in whom a disorder of the vocal cords associated with emotional factors resulted in acute episodes of stridor. Adduction of the vocal cords on inspiration and abduction on expiration was found on indirect laryngoscopy. The problem responded to either placebo treatment or psychotherapy. The similarity between vocal cord dysfunction presenting as stridor and that presenting as asthma is discussed. The importance of diagnosing these functional problems in children is emphasized in order to avoid unnecessary diagnostic procedures and hazardous treatment.     

Inspiratory stridor as the only symptom of esophageal foreign body

An eleven month old infant girl presented with a two-month history of inspiratory stridor. Analysis of her symptoms indicated airway obstruction located in the middle of the trachea; a barium esophagogram revealed an esophageal foreign body with tracheal compression. By endoscopy, a chestnut shell was extracted from the esophagus. In infancy, esophageal foreign bodies may cause mainly respiratory symptoms.     

A pediatric case of life-threatening airway obstruction caused by a cervicomediastinal thymic cyst

Most patients with thymic cysts complain of a slowly enlarging, asymptomatic cervical mass. Only 6–10% suffer dysphagia, dyspnoea, stridor, cervical pain or vocal paralysis. In some rare cases sudden onset of severe dyspnoea or asphyxia is the first symptom, especially in neonates and small infants. We report a unique case of a 20-month-old child, who required emergency tracheal intubation due to asphyxia. Cervicomediastinal thymic cyst might need to be included in causes of life-threatening airway obstruction in young children.