Isolated neonatal bilateral vocal cord paralysis revealing a unilateral medullary defect: a case report.

BACKGROUND: Congenital bilateral vocal cord paralysis is a rare occurrence. Approximately half the cases are associated with a major comorbidity, usually neurological, neuromuscular or malformative. CASE PRESENTATION: In a male newborn, respiratory distress syndrome and stridor were observed immediately following birth. The cause was bilateral vocal cord paralysis in the adducted position. Neuroradiological investigation revealed a unilateral discontinuity between the upper pons and the right medulla oblongata. Hypoplasia of the right posterior hemiarches of C1-C2 and the right exo-occipital bone was observed, as was a small clivus. MR angiography showed the absence of the distal right vertebral artery, with hypoplasia and parietal irregularities of the proximal segments. Respiratory autonomy was not obtained despite endoscopic laser cordotomy, corticosteroid therapy and nasal continuous positive airway pressure. The infant died at the age of 4 weeks after treatment was limited to comfort care. CONCLUSIONS: A medullary lesion is an exceptional cause of congenital bilateral vocal cord paralysis. The strictly unilateral neurological and vascular defect and the absence of associated intracranial or extracranial malformation make this clinical case unique and suggest a disruptive mechanism. This case also highlights the help provided by advanced neuroimaging techniques, i.e. fibre tracking using diffusion tensor imaging, in the decision-making process.

[Phoniatric outcomes of endoscopic arytenoid abduction lateropexy in patients with transient bilateral vocal cord paralysis]. / Endoszkópos arytaenoid abdukciós lateropexia foniátriai eredményei átmeneti kétoldali gégebénulás esetén.

INTRODUCTION: Dyspnea caused by bilateral vocal cord paralysis often requires surgical intervention to prevent acute asphyxiation. The regeneration of the laryngeal nerves may last weeks or months and it is difficult to predict the outcome. In the past decades, several open and endoscopic surgical techniques have been introduced for treatment to avoid tracheostomy, however, these procedures with resection of the glottis resulted in irreversible changes in the laryngeal structure, thus the voice quality decreased over a long-term period. AIM: Endoscopic arytenoid abduction lateropexy is an accepted reversible, minimally invasive technique that provides an immediate patent airway by the lateralisation of the arytenoid cartilage with a suture. The aim of our study was to analyze the phonatory and respiratory outcomes of this treatment concept. METHOD: Two patients suffering from bilateral vocal cord palsy were treated with endoscopic arytenoid abduction lateropexy. After recovery of the vocal cord movements, the sutures were removed. Spirometric and phoniatric results of the two patients were analysed after suture removal. RESULTS: Good spirometric parameters and normal voice quality were detected in both cases. CONCLUSIONS: These results prove the high reversibility of the minimally invasive endoscopic arytenoid abduction lateropexy. Lateralization suture can be removed in the case of vocal cord movement recovery, and phonation may be physiological. Orv Hetil. 2018; 159(29): 1188-1192.

Polyhydramnios associated with congenital bilateral vocal cord paralysis: A case report.

RATIONALE: Polyhydramnios may develop when the fetus cannot swallow amniotic fluid or the amount of fetal urine increases. Occasionally, unpredictable fetal abnormalities can be diagnosed postnatally. Bilateral vocal cord paralysis in the fetus may cause polyhydramnios, which could be related to impaired prenatal swallowing. PATIENT CONCERN: A 36-year-old multipara underwent an emergent cesarean section because of polyhydramnios and active labor at 35 + 5 weeks of gestation and gave birth to a girl. DIAGNOSIS: The neonate cried feebly and exhibited cyanosis as well as very weak response to stimuli. Chest retraction and stridor were observed. Laryngoscopic examination revealed no movement in both the vocal cords, and bilateral vocal cord paralysis was diagnosed. INTERVENTIONS: When the baby was 40 days old, she underwent tracheostomy to alleviate the persistent stridor and oral feeding difficulties. OUTCOMES: She was discharged at the age of 60 days while in the tracheostomy state. LESSONS: Securing the airway of neonates with bilateral vocal cord paralysis, tracheoesophageal fistula, or muscular dystrophy, which can be detected after delivery in pregnant women with idiopathic polyhydramnios, is important. Therefore, pregnant women with idiopathic polyhydramnios must be attended to by experts, such as neonatologists, anesthesiologists, or otolaryngologists, who can secure the airway.

Prognosis of congenital idiopathic abductor laryngeal paralysis with laryngeal electromyography.

OBJECTIVE: To assess if laryngeal electromyography could provide additional information in the prognosis of congenital bilateral idiopathic abductor vocal cord paralysis (abCBILP). METHODS: We performed a retrospective review over a 23-year period (1995-2018) of all children younger than 13 years old in the Medical University of Innsbruck. RESULTS: We identified five infants with abCBILP. Fiberoptic laryngeal endoscopy revealed bilateral vocal cord immobility in adduction without accompanying laryngeal findings. Tracheostomy rate was 100%. Tracheostomy was performed after minimum 3 weeks of non-improvement with continuous positive airway pressure treatment or intubation. In all patients, repeated laryngeal electromyography revealed volitional activity. All patients showed excellent full late recovery in the second quinquennium of life. CONCLUSION: Presence of volitional activity in laryngeal electromyography could identify patients with excellent prognosis. Prospective laryngeal electromyography studies are required in more patients with abCBILP in order to document laryngeal electromyography findings also by patients with poor late recovery. Patients with poor prognosis could be selected for early laryngeal intervention to avoid tracheostomy-dependent issues. LEVEL OF EVIDENCE: 4 Laryngoscope, 130:E252-E257, 2020.

Kommerell's diverticulum: rare cause of unilateral vocal cord palsy.

Kommerell's diverticulum is a relatively rare congenital aortic arch anomaly. A 56-year-old man presented with complaint of gradually progressive hoarseness of voice since 6 months. Clinical examination revealed no evidence of palpable neck mass or cervical lymphadenopathy. Indirect laryngoscopy showed right vocal cord in median position and was immobile during phonation with normal left vocal cord. This was followed by contrast-enhanced CT (CECT) of the neck and upper chest for further evaluation. CT scan showed features of right vocal cord palsy. CECT also showed right-sided aortic arch with aberrant origin of the left subclavian artery. Aneurysmal dilatation of the aortic arch was noted at the take off of aberrant left subclavian artery suggesting Kommerell's diverticulum. Therefore, diagnosis of right vocal cord palsy due to compression of the right recurrent laryngeal nerve (RLN) by Kommerell's diverticulum was kept. Left RLN palsy did not occur in this case because the left RLN likely courses around the normal segment of the aortic arch just proximal to the origin of the left subclavian artery. As the patient was mildly symptomatic, he was kept on conservative treatment with close follow-up. The present case report highlights importance of the inclusionn of the superior mediastinum on CT scan in case of suspected vocal cord palsy to exclude vascular causes of RLN paralysis.

Congenital laryngeal paralysis in Alaskan Huskies: 25 cases (2009-2014).

OBJECTIVE To characterize congenital laryngeal paralysis (CLP) in Alaskan Huskies. DESIGN Prospective case series. ANIMALS 25 Alaskan Huskies with CLP. PROCEDURES Data were collected for each dog regarding signalment; history; results of physical, orthopedic, neurologic, and laryngeal examinations; esophagraphic findings; treatments; histologic findings; and outcomes. RESULTS Severely affected dogs were profoundly dyspneic at birth or collapsed after brief exercise; less affected dogs reportedly tired easily or overheated with minimal exercise. Mean age at initial onset of clinical signs was 6.4 months. Blue eyes, white facial markings, and oral mucosal tags or tissue bands were noted in 23 (92%), 19 (76%), and 13 (52%) dogs. Neurologic examination revealed signs of mononeuropathy of the recurrent laryngeal nerve but not of polyneuropathy. Histologic examination revealed neurogenic atrophy of the cricoarytenoideus dorsalis muscle but no polyneuropathy. Eight (32%) dogs underwent unilateral cricoarytenoid lateralization, resulting in substantial clinical improvement, including ability to compete in sled dog races. Without surgery, 4 (16%) dogs died of asphyxiation, 10 (40%) had spontaneous improvement of clinical signs (but insufficient improvement to race), and 3 (12%) remained affected. Results of pedigree analysis suggested an autosomal recessive mode of CLP inheritance, with variable penetrance. CONCLUSIONS AND CLINICAL RELEVANCE CLP in the evaluated Alaskan Huskies involved mononeuropathy of the recurrent laryngeal nerves, without polyneuropathy. Most affected dogs had blue eyes, white facial markings, and oral mucosal tags or tissue bands. Given the apparent genetic component to CLP in this breed, we recommend that dogs with these features be prevented from breeding.

Natural history of tracheostomy-dependent idiopathic congenital bilateral vocal fold paralysis.

OBJECTIVE: To identify the age at which spontaneous improvement in vocal fold function occurs that will allow decannulation to be performed in tracheostomy-dependent children with isolated idiopathic congenital bilateral vocal fold paralysis (BVFP). STUDY DESIGN AND SETTING: Retrospective chart review in tertiary pediatric center. RESULTS: Three children were identified who underwent tracheostomy between 13 and 45 days old. Two patients have been decannulated at age 5 years 11 months and 7 years 1 month, but both have persistent symptoms of upper airway obstruction. One patient remains tracheostomy-dependent at 4 years of age with only minimal vocal cord abduction. CONCLUSIONS: Spontaneous improvement in vocal fold function sufficient to allow decannulation appears to occur during the second quinquennium of life. SIGNIFICANCE: Conservative treatment could be considered as an alternative to surgery in severe idiopathic congenital BVFP.

Congenital anomalies of the larynx.

Congenital laryngeal anomalies are relatively rare. However, they may present with life-threatening respiratory problems in the newborn period. Associated problems with phonation and swallowing may prevent a baby from thriving. Stridor is the most common presenting symptom of congenital laryngeal abnormalities. Often, it is associated with dysphagia, aspiration, and failure to thrive. Endoscopy is essential for evaluation and diagnosis in most cases. The differential diagnosis includes laryngeal cysts, atresia and stenosis, vocal fold immobility, and subglottic hemangiomas. In this article, the authors discuss in detail the evaluation and treatment for each condition.

A new solution for neonatal bilateral vocal cord paralysis: Endoscopic arytenoid abduction lateropexy.

OBJECTIVES/HYPOTHESIS: Bilateral vocal cord paralysis in early childhood is a life-threatening condition, which often requires immediate intervention. One of the treatment options is a quick, reversible simple suture vocal cord lateralizing technique, whereby the arytenoid cartilage is directly lateralized to the normal abducted position. Considering pediatric laryngeal anatomy, a small endolaryngeal thread guide instrument was designed for precise suture insertion. STUDY DESIGN: New instrument validation. METHODS: Four newborns had inspiratory stridor immediately after birth; two had to be intubated. Laryngotracheoscopy revealed bilateral vocal cord paralysis. Unilateral, left-sided endoscopic arytenoid abduction lateropexy was performed with supraglottic jet ventilation on the 4th, 5th, 5th, and 27th day of life for the four patients, respectively. RESULTS: All babies remained intubated for 3 to 7 days with an uncuffed tracheal tube. After extubation, no dyspnea or swallowing disorder occurred. A subjective quality of life questionnaire, laryngotracheoscopy, clinical growth charts, and voice analysis showed satisfactory functional results. CONCLUSIONS: Minimally invasive, quick, reversible endoscopic arytenoid abduction lateropexy might be a more favorable solution for neonatal bilateral vocal cord paralysis than earlier treatment strategies. In one step, the airway can be maintained without the risk of any permanent damage to voice production. Good swallowing function is also preserved. The specially modified endolaryngeal thread guide instrument gives a fast and effective option for creating the lateralized arytenoid position even in the technically challenging surgical context of a neonate larynx. LEVEL OF EVIDENCE: 4. Laryngoscope, 127:1608-1614, 2017.

Congenital bilateral vocal cord paralysis.

Congenital upper airway obstruction is a relatively rare but important cause of major respiratory problems in the neonatal period. Vocal cord paralysis is the second most common cause of congenital airway obstruction presenting with neonatal stridor. It is often the reason for the failure of neonates to wean from the respiratory support. A retrospective analysis of medical record review was conducted. There were seven paediatric patients diagnosed with bilateral vocal fold paralysis in the past three years, of which five were recently diagnosed. All patients underwent flexible with/without rigid bronchoscopes to confirm the diagnosis. This case series highlight our experience in managing the problem of bilateral vocal cord paralysis in the paediatric population, with particular emphasis on their clinical presentations, associated complications and both upper and lower airway abnormalities. The management options and outcome of these patients will also be discussed.

Congenital bilateral vocal cord paralysis and the role of glycine.

OBJECTIVES: We sought to modify normal laryngeal constrictor (LC) motoneuron activity to induce a pattern of aberrant LC muscle function that may serve as a model of congenital bilateral vocal cord paralysis. METHODS: Single unit extracellular recordings of functionally identified LC motoneurons were made in anesthetized Sprague-Dawley rats, and the response to both intravenous and iontophoretic application of the glycine antagonist strychnine was studied. RESULTS: The postinspiratory firing pattern of LC motoneurons became inspiratory after intravenous injection of strychnine (4 of 5 rats), but no change was recorded in response to strychnine iontophoresis (7 of 8 rats). CONCLUSIONS: Blockade of glycinergic inhibitory neurotransmission by strychnine, acting above the level of the LC motoneuron, causes LC motoneurons to fire during inspiration rather than after inspiration. This observation suggests that impaired glycine neurotransmission may be an underlying mechanism that explains the clinical manifestations of congenital bilateral vocal cord paralysis.

Familial bilateral abductor vocal cord paralysis.

Familial bilateral abductor vocal cord paralysis is a rare entity with few prior descriptions in the literature. Modes of inheritance include X-linked, autosomal recessive, and autosomal dominant. A case of this condition in a father and son is presented. Signs and symptoms at presentation, diagnosis, therapeutic considerations, and modes of inheritance are discussed.

Spontaneous recovery of bilateral congenital idiopathic laryngeal paralysis: systematic non-meta-analytical review.

OBJECTIVES: To systematically review the frequency and time to spontaneous recovery in pediatric patients with bilateral congenital idiopathic laryngeal paralysis (BCILP). METHODS: The databases of Medline, EMBASE, Scopus, CINAHL, Cochrane Library and Proquest Dissertations were searched for English language articles reporting on laryngeal paralysis in pediatric patients. A bibliography search of the selected studies was done to identify additional articles. We included prospective or retrospective case-series studies of children and neonates diagnosed with BCILP at age <60 days and confirmed by direct laryngoscopy, with sufficient follow up and objective assessment for recovery. Two authors independently extracted the data and assessed the quality of each study. Discrepancies were resolved by consensus and adjudication by a third author. RESULTS: Of the 4229 articles identified by the search, only one study met our inclusion criteria. The study was a retrospective case series, and was of low quality. The mean age at diagnosis was fourteen days. Sixty-five percent of the patients recovered spontaneously, and the mean time to recovery was twenty-five months. Tracheostomy was performed in 71% of the patients. CONCLUSIONS: The available literature is of low quality and provides weak evidence on the natural history of BCILP in pediatric population.

Idiopathic bilateral vocal cord paralysis in infants: Case series and literature review.

OBJECTIVES/HYPOTHESIS: Vocal fold paralysis is the second most common congenital laryngeal anomaly in newborns. Bilateral paralysis is a severe condition and often remains of unknown etiology. We report our experience of congenital idiopathic bilateral vocal fold paralysis in newborns and infants, and discuss the therapeutic options. STUDY DESIGN: Retrospective review. METHODS: A retrospective review was carried out at a single tertiary referral center over a 15-year period of children presenting with congenital idiopathic bilateral laryngeal paralysis (CIBP). RESULTS: Twenty-six patients were identified and managed over period of the study (mean follow-up, 6.7 years). A tracheostomy was eventually performed in 14 patients, 12 during the neonatal period. Endoscopic treatment was performed in 16 patients and an external approach in five patients after endoscopic failure. Three patients were managed by watchful waiting. Spontaneous recovery occurred in 16 patients (median age, 14.5 months), including nine with a tracheostomy. Decannulation was achieved in 12 patients (median age, 42 months), and noninvasive positive pressure ventilation (NPPV) was applied in six patients. CONCLUSIONS: Only inhomogeneous series of pediatric bilateral laryngeal palsies have been published so far, making it difficult to identify prognostic and predictive markers, as well as therapeutic guidelines. Focusing on CIBP, our data suggest that the management strategy must take into account the possibility of a delayed spontaneous recovery. NPPV is an interesting therapeutic tool in this situation. A systematic somatic assessment is mandatory to look for associated conditions.

Congenital vocal cord paralysis with possible autosomal recessive inheritance: case report and review of the literature.

We describe an infant with congenital vocal cord paralysis born to consanguineous parents. While autosomal dominant and X-linked inheritance have been previously reported in this condition, we conclude that the degree of parental consanguinity in this case strongly suggests autosomal recessive inheritance. Although we cannot exclude X-linked inheritance, evidence from animal studies demonstrates autosomal recessive inheritance and provides a possible molecular basis for congenital vocal cord paralysis.

Familial laryngeal paralysis.

Vocal fold paralysis (VFP) is the second most frequent cause of congenital stridor. Although often due to birth trauma, infection, and brainstem abnormalities, most cases are idiopathic. Infrequently, a family history of VFP is elicited, identifying a role for genetic factors in laryngeal function. This study describes a family in which an autosomal dominant form of familial laryngeal abductor paralysis segregates. The typical physical findings, diagnostic and therapeutic considerations, and possible molecular mechanisms of this disorder are discussed in detail.

Respiratory distress in a pregnant woman with congenital vocal cord paralysis.

BACKGROUND: Vocal cord paralysis is one of the most common congenital laryngeal lesions. Hoarseness and dysphonia are common in unilateral cases. Stridor and respiratory distress are seen in bilateral cases. CASE: A 33-year-old primigravida with bilateral congenital vocal cord paralysis presented in the third trimester with a 2-week history of bronchitis and progressive shortness of breath. She developed stridor, her respiratory status deteriorated, and she was intubated with difficulty. She went into cardiac arrest, was resuscitated and placed on a ventilator, and a tracheotomy was performed. Her condition improved, she remained stable until 38 weeks, and was delivered by cesarean. CONCLUSION: Congenital vocal cord paralysis may result in life-threatening respiratory distress during pregnancy. Tracheotomy placement may be useful in the management of these patients.

Familial vocal cord dysfunction associated with digital anomalies.

Familial vocal cord dysfunction is a rare condition that has been reported in only a few instances. This is a report of identical male twins, both of whom had congenital bilateral abductor vocal cord paresis associated with finger deformities. The vocal cord paresis progressed to paralysis that required tracheotomy, then returned to a slowly resolving paresis during which the vocal cords had uncoordinated motion generally known as synkinesis. Another male sibling and the mother had a history of stridor during infancy and finger deformities. Several other relatives had digital abnormalities, and an infant first cousin with finger abnormalities required a tracheotomy for vocal cord paralysis.

Congenital bilateral recurrent nerve paralysis and ptosis: a new syndrome?

Bilateral vocal cord paralysis is a rare, but not unheard of, occurrence in the perinatal period. It is often incompatible with life unless the problem is recognized promptly, the child intubated, and--following this--a tracheotomy performed. Bilateral congenital ptosis is a very rare occurrence and is almost always associated with either significant central nervous system deficit or with other oculomotor problems. The combination of congenital bilateral recurrent nerve paralysis and congenital ptosis has not been previously reported in the English language literature. The author has had the opportunity to manage two patients, a father and his daughter, both of whom manifested this unusual combination of symptoms. Detailed case histories are presented. Both of the patients were successfully managed by temporary tracheotomy followed by reinnervation of the larynx, with eventual extubation and satisfactory day-to-day airway. Although only two cases represent an inadequate number to determine a new syndrome, these striking findings in a father and his only offspring suggest the possibility that this may, indeed, represent a new symptom complex of congenital origin.