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.     

Vocal cord paralysis secondary to impacted esophageal foreign bodies in young children

Impacted foreign bodies in the esophagus can result in respiratory symptoms including stridor and aphonia. Several mechanisms have been proposed to explain these symptoms, but the possibility of vocal cord paralysis and its cause has not been adequately emphasized. Two cases of young children with esophageal foreign body are described; both presented with respiratory symptoms, 1 with aphonia and the other with stridor. In both cases, the symptoms were secondary to vocal cord paralysis. A possible mechanism of recurrent nerve injury is proposed.     

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 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.     

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.     

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.     

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.     

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.     

吸气性喉喘鸣婴儿112例电子喉镜检查结果分析

目的 探讨0～1岁婴儿吸气性喉喘鸣的病因及电子喉镜在婴儿喉部疾病诊断中的应用和价值.方法 回顾性分析了2006年6月-2007年7月112例0-1岁吸气性喉喘鸣婴儿电子喉镜检查的结果,其中男76例,女36例.按初诊年龄分为3组:新生儿组(A组)24例,-6月龄组(B组)63例,-12月龄组(C组)25例.结果 ①112例中喉软骨软化症82例(占73.21%);喉部炎症、声带麻痹、声门下占位各3例(各占2.68%);喉乳头状瘤、声门上软组织增生各2例(分别占1.79%);会厌旁占位、咽侧壁占位、舌根部占位、咽后壁脓肿各1例(分别占0.89%);13例(11.61%)息儿喉部结构正常.②三组中,喉软骨软化症所占比例分别为75.00%、85.71%、40.00%,其他喉部病变所占比例分别为20.83%、9.52%、24.00%.③喉软骨软化症在A组和B组中所占比例差异无统计学意义(P>0.05),而在C组中所占比例与前两组相比差异均有统计学意义(P<0.05).结论 喉软骨软化症仍是0-1岁婴儿吸气性喉喘鸣的主要病因,随着月龄增长有自愈的倾向;其他喉部病变也是吸气性喉喘鸣的重要病因,需尽早诊断治疗.电子喉镜检查安全、快速、无创、清晰,对0-1岁婴儿喉部疾病的诊断具有不可替代的价值.     

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.     

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.     

Vocal cord paralysis in neuraxial malformation

Vocal cord paralysis can occur in children bom with spina bifida cystica who may have Arnold-Chiari malformation and hydrocephalus. Two cases are reported to illustrate this. In one, the vocal cord paralysis was of the bilateral abductor type and was due, most probably, to primary brainstem disease. In the other, vagal involvement and brainstem dysgenesis seem to operate in combination to cause a unilateral paramedian cord paralysis. Relevant literature is briefly reviewed.     

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.     

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.     

ECHOCARDIOGRAPHIC DIAGNOSIS OF AN ANEURYSM OF THE MEMBRANOUS VENTRICULAR SEPTUM

Abstract. A patient with a small ventricular septal defect complicated by an aneurysm of the membranous ventricular septum is reported. The aneurysm was diagnosed by left ventricular angiocardiography and, non-invasively, by echocardiography. The clinical significance of the anomaly is discussed in relation to the pertinent literature.     

Stridor and airway compromise

A graduated approach to the diagnosis of airway lesion will rarely fail. The availability of state-of-the-art optical equipment, excellent anesthesia, and high-quality medical care have remarkably reduced the morbidity and mortality of airway diseases. Electrodiagnosis of vocal cord paralysis will differentiate fixed arytenoids from neurogenic paralysis in candidates for laryngeal repair and will help define the prognosis of neurogenic paralysis. The excellent results from epiglottoplasty in treating laryngomalacia and laryngotracheal reconstruction for stenosis make obsolete any management with tracheotomy and watchful waiting. Close cooperation and good communication between the pediatrician and the otolaryngologist remain the key points for successful management.     

The importance of recognizing paradoxical vocal fold dysfunction: A case report of a 13-year-old girl presenting with stridor

The present report details the case of a 13-year-old girl who presented to the emergency department with stridor. Treatment for presumed reactive airway disease was attempted with antibiotics, nebulized adrenaline masks and high-dose corticosteroids. Over the next month, she presented repeatedly in a similar fashion and was admitted to hospital on three separate occasions. Ultimately, she was referred to the Centre for Paediatric Voice and Laryngeal Function at The Hospital for Sick Children (Toronto, Ontario) for a speech-language pathology evaluation and direct laryngoscopy. The patient was diagnosed with paradoxical vocal fold dysfunction. After a brief treatment session with a speech-language pathologist, her stridor completely resolved and paradoxical inspiratory vocal fold adduction was no longer visualized on direct laryngoscopy. The present case highlights the fact that paradoxical vocal fold dysfunction can mimic other entities that present with stridor, and misdiagnosis can result in significant morbidity. Investigation into a patient’s social history and stressors can facilitate the diagnosis, and can avoid unnecessary and potentially harmful medical and surgical interventions.