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.

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.

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.

[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.

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.

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 laryngeal anomalies.

INTRODUCTION: It is essential for clinicians to understand issues relevant to the airway management of infants and to be cognizant of the fact that infants with congenital laryngeal anomalies are at particular risk for an unstable airway. OBJECTIVES: To familiarize clinicians with issues relevant to the airway management of infants and to present a succinct description of the diagnosis and management of an array of congenital laryngeal anomalies. METHODS: Revision article, in which the main aspects concerning airway management of infants will be analyzed. CONCLUSIONS: It is critical for clinicians to understand issues relevant to the airway management of infants.

Congenital laryngeal anomalies / Anomalias congênitas da laringe

Introduction: It is essential for clinicians to understand issues relevant to the airway management of infants and to be cognizant of the fact that infants with congenital laryngeal anomalies are at particular risk for an unstable airway. Objectives: To familiarize clinicians with issues relevant to the airway management of infants and to present a succinct description of the diagnosis and management of an array of congenital laryngeal anomalies. Methods: Revision article, in which the main aspects concerning airway management of infants will be analyzed. Conclusions: It is critical for clinicians to understand issues relevant to the airway management of infants. .

Introdução: As anomalias congênitas da laringe estão em risco de uma via aérea instável, sendo essencial que o clínico tenha uma boa compreensão dos problemas relevantes para o manuseio das vias aéreas de bebês. Objetivos: Familiarizar os clínicos com os problemas relevantes para o manuseio das vias aéreas de bebês e apresentar uma descrição sucinta do diagnóstico e tratamento de uma série de anomalias congênitas da laringe. Método: Artigo de revisão, no qual serão tratados os principais aspectos relacionados ao manuseio das vias aéreas de bebês. Conclusões: É essencial que o clínico tenha um bom conhecimento dos problemas relevantes ao manuseio das vias aéreas de bebês. .

Applications for transoral robotic surgery in the pediatric airway.

OBJECTIVES/HYPOTHESIS: To report preliminary experience in the utilization of transoral robotic surgical (TORS) techniques in pediatric airway surgery. STUDY DESIGN: Retrospective case series. METHODS: A retrospective chart review was performed on all pediatric patients undergoing robotic airway surgery at a university-based children's hospital between August 2010 and December 2012. RESULTS: Three pediatric patients underwent robotic repair of various airway anomalies between August 2010 and December 2012. Case 1 is a 15-year-old boy with posterior glottic stenosis for which a robotic-assisted posterior cricoid split with cartilage graft placement was attempted but ultimately required conversion to an open technique. Case 2 is a 6-year-old male with Trisomy 21 who had robotic-assisted endoscopic repair of a type II laryngeal cleft, with subsequent resolution of his chronic aspiration and successful initiation of oral feeding. Case 3 is a 3-year-old female who underwent robotic-assisted left posterior cordectomy and subtotal arytenoidectomy for idiopathic bilateral vocal cord paralysis, resulting in improved Passy-Muir valve tolerance. CONCLUSION: TORS is being increasingly utilized in the field of head and neck surgery as it is frequently less invasive and often demonstrates improved outcomes compared with traditional approaches. This study details our experience utilizing TORS in the management of pediatric airway anomalies and represents one of the earliest such series in the literature. Although TORS has the potential to improve the management of pediatric airway disorders, larger, prospective studies are needed to better elucidate its feasibility and efficacy. LEVEL OF EVIDENCE: 4.

[Congenital laryngeal stridor - an interdisciplinary problem]. / Wrodzony stridor krtaniowy­ problem interdyscyplinarny.

UNLABELLED: The most common causes of laryngeal stridor are laryngomalacia (60%), vocal cord paralysis (VCP) (10 %) and subglottic laryngeal stenosis. Majority of cases of VCP are idiopathic, less frequently it is the effect of abnormalities in central nervous system (Arnold - Chiari syndrome, hydrocephalus, neonatal hypoxia). Differential diagnosis should also include anomalies of aortic arch and its branches (vascular rings). The authors present two cases of neonatal congenital laryngeal stridor. In the first case the girl presented with VCP of unknown etiology. The perinatal period was normal, ultrasound of central nervous system and neurologic examination revealed no abnormalities. Due to sustained VCP in control laryngeal ultrasound examinations, tracheostomy was performed in the third month of life. In the second case, stridor was caused by laryngomalacia and subglottic laryngeal stenosis of first grade according to Meyer-Cotton scale (larynx lumen diameter <4 mm). The diagnosis was established by laryngotracheobronchoscopy. In both children angiotomography was performed and vascular ring was diagnosed (aberrant right subclavian artery). Vascular anomaly was suspected in barium X-ray. In both cases echocardiographic examination did not visualize the fourth vessel of the aortic arch. Chest X- rays were normal. Both children had no symptoms caused by vascular ring. CONCLUSION: Diagnosis of congenital laryngeal stridor is an indication for complete evaluation to establish the cause of the airway obstruction. The differential diagnosis should include laryngomalacia, vocal cord paralysis, subglottic laryngeal stenosis, congenital anomalies of large vessels and abnormalities of the central nervous system.

Paralyzed neonatal larynx in adduction. Case series, systematic review and analysis.

OBJECTIVE: Bilateral vocal cord abductor paralysis (BVCAbP) is considered a rare cause of stridor in the newborn. The goal of this work is to present a case series and to review systematically the literature on bilateral vocal cord abductor paralysis in the newborn to better characterize the current knowledge on this entity. METHODS: We performed a systematic literature review with Medline (1950-2011). The authors screened all cases of BVCAbP reported and selected those affecting newborns. RESULTS: Out of the 129 articles screened, 16 were included. A total of 69 cases could be retrieved and analyzed. Associated co-morbidities were found in 54% of the patients, most notably malformative conditions (intracranial or other), or a positive perinatal history (trauma/asphyxia, prematurity). Tracheostomy placement was required in 59% of children, and of these 44% were successfully decannulated. In terms of functional outcome full recovery or improvement were seen in 61% of patients. Major underlying co-morbidities affected negatively the functional outcome (p=.004), but not the need for tracheostomy (p=.604) or the decannulation success rate (p=.063). CONCLUSION: BVCAbP in the newborn is a serious cause of airway obstruction. It can be seen either in a context of multisystem anomalies or as an isolated finding. Newborns with major co-morbidities affecting their normal development are more likely to have poor functional outcomes and to remain tracheostomy-dependant.

Vocal cordotomy.

Congenital bilateral vocal fold paralysis (BVFP) is the second most common cause of stridor in neonates. Etiologies of BVFP include neurologic, cardiopulmonary malformations, iatrogenic, traumatic, and idiopathic. One half of children with BVFP will require a tracheostomy for upper airway obstruction. Because more than 50% of BVFP will resolve spontaneously, many advocate surgical intervention to achieve decannulation after the age of one. The goal of surgery is to provide an adequate airway to allow decannulation with minimal impact on speech and swallowing. There is no one procedure accepted as the gold standard or first-line treatment to achieve decannulation in children with BVFP. The author's preference is to perform a vocal cordotomy as a first line for an endoscopic approach.

A novel endoscopically placed stent to relieve glottic obstruction from bilateral vocal fold paralysis.

OBJECTIVE: Congenital bilateral vocal fold paralysis (BVP) is a rare but serious condition often requiring a tracheostomy to temporize the airway. In cases of idiopathic BVP, studies suggest waiting twelve months prior to laryngeal surgery because of a high rate of spontaneous recovery. Therefore a less invasive and reversible intervention would be optimal. A prospective study in a piglet model was undertaken to evaluate the efficacy of a novel spring-loaded stenting device designed to maintain laryngeal patency in an in vivo animal model of BVP. METHODS: Eight Yorkshire piglets had BVP induced by surgical division of the recurrent laryngeal nerves. Stents were endoscopically deployed between the arytenoid vocal processes. Animals were recovered and monitored for stridor, dietary intake, and weight gain. Animals were sacrificed after five days. Airway resistance using a calibrated manometer was measured at four time-points: baseline, BVP induction, stent insertion, and pre-sacrifice. RESULTS: Six of eight animals survived greater than five days with an average weight gain of 1.9kg (p=0.003). Relative inspiratory resistance increased from baseline after inducing BVP (1.00 vs. 1.468, p=0.0315) and decreased to baseline levels with stent insertion (1.468 vs. 1.092, p=0.0238). Expiratory resistance was not significantly influenced by stage of measurement (p=0.236). Of the two animals not surviving the protocol, one had an unrelated anesthesia complication and the other a malpositioned stent. CONCLUSION: The novel stent was successful in relieving the inspiratory resistance associated with BVP, without compromising swallowing and daily function. This may hold promise in temporarily securing the pediatric airway in the setting of BVP.

Poland-Mobius syndrome in an infant girl.

Mobius syndrome is a rare condition of unclear origin, characterized by a unilateral or bilateral congenital facial weakness with impairment of ocular abduction, which is frequently associated with limb anomalies . Poland described a condition in which there was unilateral absence of pectoralis major muscle and ipsilateral syndactyly. The combination of Poland-Mobius syndrome is rare, with an estimated prevalence 1:500 000. We describe a case of Poland-Mobius syndrome in association with congenital bilateral vocal fold immobility. To our knowldge, this is the first report of such an association between Poland-Mobius syndrome and congenital bilateral vocal fold immobility.

[Congenital bilateral vocal cord paralysis]. / Medfødt bilateral stemmebåndsparese.

Congenital bilateral vocal cord paralysis (CBVCP) is a rare but potentially life-threatening condition and awareness of the condition is necessary to ensure early diagnosis and treatment. This case describes a 25-month-old boy suffering from CBVCP. The main symptoms at birth were inspiratory stridor combined with a normal voice and feeding problems. The difficulties in achieving the right diagnosis are demonstrated, and the treatment so far, including tracheotomy and a feeding tube, is outlined. The importance of fibre optic laryngoscopy in both diagnosis and control is stressed.

Paradoxical vocal cord movement in newborn and congenital idiopathic vocal cord paralysis: two of a kind?

The second most common cause of stridor reported in the newborn is bilateral vocal cord paralysis (BVCP) and one-third of the cases have been categorized as idiopathic. During the last year four children with stridor since birth were referred to our department for examination. Videotaped flexible laryngoscopy, carried out with the patient awake or under general anaesthesia with a spontanous respiration, revealed instead of abduction of the vocal cords during inspiration, rather an active adductory movement. Consequently instead of BVCP, we made the diagnosis paradoxical vocal cord movement (PVCM). One of the twins required a tracheostomy, the three other patients have been observed without the need of further treatment. No previous publications have described PVCM in newborn. However, our observations and video recordings clearly show that the stridor in our four patients is due to PVCM. This is possibly the same condition as earlier reported as congenital, idiopathic BVCP where incoordinated vocal cord movement or dyskinesia has been a part of the laryngoscopic findings. The mechanism behind PVCM in this age group or site of lesion is unclear.