Primary Alveolar Rhabdomyosarcoma of the Thyroid: A Case Report.

Malignancies in paediatric age group are the second leading cause of death, next only to accidents. The variety of malignancies involving the head and neck region in paediatric age group is bewildering. Rhabdomyosarcoma of thyroid gland is one such entity very rarely seen. We present the case of an adolescent who presented with a neck mass and suffered rapid deterioration. Establishment of final diagnosis was possible only because of a skilfully performed pathological examination which revealed a thyroid gland rhabdomyosarcoma. An alveolar variety of rhabdomyosarcoma presenting in the thyroid gland is extremely rare. To the best of our knowledge, only two cases of the aforementioned are documented as yet. Through this report, we aim at highlighting the possibility of such an occurrence and vigilance on part of the treating surgeon so that timely intervention can be instituted.

The anesthetic management of a child with ohtahara syndrome and severe stridor: a case report.

BACKGROUND: Ohtahara syndrome is a progressive developmental and epileptic encephalopathy that manifests in the early infantile period. This rare condition is characterized by intractable seizures, psychomotor retardation, and poor prognosis. To date, there are a handful of case reports regarding the anesthetic management of children with Ohtahara syndrome. However, limited reports exist of patients with Ohtahara syndrome who present with difficult airways. This report describes our airway findings and general anesthetic management of a pediatric patient with Ohtahara syndrome undergoing diagnostic bronchoscopy for severe inspiratory stridor. CASE PRESENTATION: A 14-month-old, 9 kg, male patient with Ohtahara syndrome presented with a year-long history of severe inspiratory stridor and was scheduled for bronchoscopy with lavage. On exam, the patient had noisy breathing, was non-verbal with developmental delay, and had poor head control with significant central hypotonia. The patient was induced with ketamine and general anesthesia was maintained with propofol. Bronchoscopic evaluation was completed uneventfully and revealed a diagnosis of laryngotracheomalacia. The patient's breathing was maintained spontaneously throughout the procedure and no seizures were noted. In the post anesthesia care unit, the patient's respiratory and cardiovascular function were stable. CONCLUSIONS: This report documents the unusual finding of severe inspiratory stridor in a 14-month-old child diagnosed with Ohtahara syndrome and our anesthetic management during their diagnostic bronchoscopy. Currently, documentation of complex airway pathology present in patients with Ohtahara syndrome is limited and should be further evaluated. This will assist pediatric anesthesiologists as these patients may require careful preoperative assessment, thoughtful airway management, and surgical alternatives on standby.

Imaging studies in evaluating children with stridor. / Los estudios por imágenes en la evaluación del niño con estridor.

Stridor is a symptom of upper airway obstruction and may result from congenital or acquired causes. The diagnosis is usually clinical. If further investigation is necessary for differential diagnosis, endoscopy is the method of choice in most cases. Imaging studies are complementary to endoscopy. They allow evaluation of laryngeal and tracheobronchial pathology and extrinsic airway compressions due to tumors or vascular malformations and define a lesion's location, extent, and characteristics. They are helpful in cases of diagnostic doubt and when endoscopy is unavailable. It is essential to understand the anatomy and pathophysiology of the respiratory tract and to be aware of the indications and limitations of complementary examinations for proper diagnosis. The different imaging modalities available to evaluate stridor in pediatrics are described, and their advantages are discussed.

El estridor es un síntoma de obstrucción de la vía aérea superior y puede ser resultado de causas congénitas o adquiridas. El diagnóstico suele ser clínico. Si es necesaria una investigación adicional para el diagnóstico diferencial, la endoscopia es el método de elección en la mayoría de los casos. Los estudios por imágenes son complementarios a la endoscopia. Permiten evaluar la patología laríngea y traqueobronquial, las compresiones extrínsecas de la vía aérea por tumores o malformaciones vasculares y definir la localización, extensión y características de una lesión. Son útiles en casos de duda diagnóstica y cuando la endoscopia no está disponible. Es fundamental comprender la anatomía y fisiopatología del tracto respiratorio, y ser conscientes de las indicaciones y limitaciones de los exámenes complementarios para el diagnóstico adecuado. Se describen las diferentes modalidades de imágenes disponibles para evaluar el estridor en pediatría y se discuten sus ventajas.

Single stage cricoid split laryngoplasty with costochondral rib grafting is a novel approach to treat subglottic stenosis in a paediatric patient: A case report.

INTRODUCTION AND IMPORTANCE: Subglottic stenosis (SGS) appears to be a commonly encountered condition in the paediatric age group. Single stage cricoid split laryngoplasty with costochondral rib grafting in paediatric patients is a unique, innovative, and advanced operation in nature. Morbidity and mortality rates can be minimized with early diagnosis and prompt treatment. PRESENTATION OF CASE: Presenting the case of a 13-month-old child diagnosed with Grade II SGS who was managed for cricoid split laryngoplasty with a costochondral rib graft. It was a unique strategy for providing infants and neonates with symptomatic SGS with a safe and efficient substitute for long-term tracheostomy. When healing was completed, the patient regained the function of their airway. The approach was successful, and preventable to long-term tracheostomy. DISCUSSION: Performing this procedure early in children has shown higher rates of success and it is safe and effective. Further extensive research and studies need to be conducted in this domain, and every patient's status should be reviewed time and again to tend to their specific needs, and the choice of procedure should be made optimally based on clinical evaluations. CONCLUSION: Successful management of a 13-month-old child with Grade II subglottic stenosis through cricoid split laryngoplasty with costochondral rib grafting is a challenging and novel approach to treating single-stage SGS.

Laryngotracheitis and Coronavirus Disease 2019 (COVID-19) in a Three-Year-Old Child: A Case Report and Literature Review.

This case report details an atypical etiology of laryngotracheitis (croup) in a three-year-old child diagnosed with coronavirus disease 2019 (COVID-19). Unlike typical croup cases, the patient required hospitalization and multiple administrations of racemic epinephrine for respiratory distress. The author highlights the importance of considering COVID-19 (severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2)) as a potential etiology of croup in children. This distinction is crucial as such cases may necessitate more intensive medical intervention and prolonged monitoring compared to standard croup treatment protocols. The patient reported here did not require intensive care admission or respiratory support.

Prevalence and Clinical Characteristics of Pediatric Lower Airway Malacia: Case Series from a Tertiary Center in Turkey.

Introduction: Lower airway malacia (LAM) is characterized by a reduction in the cross-sectional luminal area during quiet respiration. There is no gold standard diagnostic test; however, flexible fiberoptic bronchoscopy (FFB) is most frequently utilized. The exact prevalence and incidence of LAM are unknown. This study aimed to determine the prevalence rates of pediatric patients diagnosed with LAM, offer a detailed understanding of their demographic and clinical characteristics, and investigate distinctions between two specific types of LAM, namely, tracheomalacia (TM) and bronchomalacia (BM). Materials and Methods: Patients younger than 18 years diagnosed with LAM using FFB were included in this retrospective case series. Demographic and clinical characteristics and comorbid disorders were compared between patients with isolated BM and those with isolated TM or tracheobronchomalacia (TM/TBM). Results: Among 390 patients who underwent FFB, 65 (16.6%) were diagnosed with LAM, 16 (24.6%) with TM, and 56 (86.2%) with BM. The median age at diagnosis was 15 months. Among them, 59 (90.8%) had other comorbidities; gastrointestinal (GI) disorders were the most common (38.5%). The most common indications for bronchoscopy were recurrent/prolonged lower respiratory tract infections (LRTI) or wheezing (43.1%), while the most frequently observed respiratory physical examination finding was stridor (35.4%). Patients with TM/TBM had significantly higher frequencies of premature births, stridor, retraction, and GI disorders. Conclusion: Patients with stridor without typical laryngomalacia features or recurrent or prolonged LRTI should undergo prompt evaluation for LAM. The potential coexistence of GI disorders such as gastroesophageal reflux disease and swallowing dysfunction should also be considered.

Gossypiboma larynx: a rare cause of post-tracheostomy stridor-case report and review of literature.

BACKGROUND: Gossypiboma, a retained surgical sponge with a foreign body reaction, is an unusual but serious complication seen in open abdominal surgeries. It is exceptionally rare following head and neck surgeries. Here, we present a case of Gossypiboma of the upper airway following tracheostomy. CASE PRESENTATION: A 32-year-old male presented with stridor and difficulty breathing one-month post-tracheostomy after a severe head injury following a road traffic accident. A neck radiograph was unremarkable, and a computed tomography (CT) scan of the neck showed a well-defined homogenous curvilinear membrane extending from the hypopharynx to the upper trachea. Bronchoscopic evaluation of the larynx and upper trachea revealed a retained surgical sponge, which was retrieved. The patient's breathing improved drastically post intervention. CONCLUSION: Gossypiboma may go undetected in radiographs and may also present atypically as a homogenous membrane on a CT scan of the neck. Though rare, retained surgical items can have profound medicolegal and professional consequences on physicians. Hence, a strong clinical suspicion and vigilance for gossypiboma is necessary for patients presenting with respiratory distress post-tracheostomy.

Bilateral Vocal Fold Abduction Dysfunction: A Report of Two Neonatal Cases.

Stridor is caused by oscillation of the narrowed upper airway. The most common cause of neonatal stridor is laryngomalacia, followed by vocal fold abduction dysfunction. Herein, we present two neonatal cases of idiopathic dysfunction of vocal fold abduction. A neonate was admitted to the neonatal intensive care unit (NICU) on day 4 of life for inspiratory stridor, intermittent subcostal retraction, and cyanosis. A second neonate was admitted to the NICU on day 7 of life for inspiratory stridor and cyanosis when crying. Neither patient had dysmorphic features or unusual cardiac ultrasonography findings. The diagnosis was confirmed by laryngo-bronchoscopy. Conservative treatment with biphasic positive airway pressure was effective in both cases and symptoms resolved within a few months. Resolution of vocal fold abduction dysfunction was confirmed by repeat endoscopy. Clinical manifestations of vocal fold abduction dysfunction vary widely. Although most cases resolve spontaneously, prolonged tube feeding, or even tracheostomy, is needed in some severe cases. Diagnosis of vocal fold abduction dysfunction requires a laryngo-bronchoscopy study; thus, there may be a large number of undiagnosed patients. Vocal fold abduction dysfunction should be considered in the differential diagnosis for neonatal inspiratory stridor.

Severity of sleep apnea as a prognostic factor for mortality in patients with multiple system atrophy.

BACKGROUND: We determined whether the severity of sleep apnea increases the risk of mortality in patients with multiple system atrophy (MSA) with and without stridor. MethodsThis retrospective study included patients who underwent polysomnography within one year after diagnosis of probable MSA. Stridor, sleep apnea, and arousal from sleep were determined using full-night polysomnography. Disease severity was measured using the Unified MSA Rating Scale (UMSARS). Survival data were collected and analyzed using Cox regression analysis. RESULTS: Sixty-four patients with MSA were included. During a median follow-up of 34.5 months, 49 (76.6 %) patients died. Stridor was present in 56.3 % of patients. Patients with stridor had more severe sleep apnea and shorter sleep time than those without, but the hazard ratio (HR) for death did not differ between patients with and without stridor. Among patients without stridor, apnea-hypopnea index ≥30/h (HR, 6.850; 95 % confidence interval [CI], 1.983-23.664; p = 0.002) and a score of UMSARS I + II (HR, 1.080; 95 % CI, 1.040-1.121; p < 0.001) were independently associated with death. In contrast, among patients with stridor, frequent arousals from sleep (HR, 0.254; 95 % CI, 0.089-0.729; p = 0.011) were a significant factor associated with longer survival, while MSA-cerebellar type tended to be associated with poor survival (HR, 2.195; 95 % CI, 0.941-5.120; p = 0.069). CONCLUSION: The severity of sleep apnea might be a significant predictor of shorter survival in MSA patients without stridor, whereas frequent arousals from sleep might be a significant predictor for longer survival in MSA patients with stridor.

Isolated IgG4 related disease of the trachea.

IgG4 related disease (IgG4-RD) is a multisystem inflammatory disease and can affect several organs including salivary glands, orbits, lungs, pancreas, kidneys and lymph nodes. Up to 40 % of patients have allergic manifestations including asthma, chronic rhinosinusitis, eczema and asthma. Commonly pulmonary manifestations include pulmonary nodules ranging from <1 to 5 cm in diameter, interstitial opacities and mediastinal lymphadenopathy. Rarely, IgG4-RD presents as isolated tracheal disease. Symptoms include dyspnea and stridor due to airway narrowing. Diagnosis of IgG4-RD including tracheal IgG4-RD requires a biopsy. The histologic specimen is characterized by lymphoplasmacytic infiltrate with high density of IgG4 positive plasma cells, and storiform fibrosis (a cartwheel appearance of fibroblasts and inflammatory cells). Up to 30 % of patients with IgG4-RD have normal serum IgG4 levels. The mainstay of therapy is glucocorticoids for those with systemic disease. Rituximab is an alternative for those who cannot tolerate glucocorticoids or those with disease recurrence. Patients with tracheal disease often require balloon dilation. Recurrence is common in patients and up to two thirds of patients have residual disease despite treatment. These patients often require surgical resection of affected area for symptomatic relief.

Vallecular Cysts in Newborns: A Case Series Demystifying the Obscured Anomaly.

A vallecular cyst is a rare diagnosis in newborns presented with stridor, which poses a significant threat to the well-being of infants. This potentially life-threatening condition is associated with a range of complications, including respiratory distress, feeding difficulties, and failure to thrive. Through this case series, we aim to shed light on the suspicion of vallecular cysts in newborns presenting with stridor and the complexities encountered during their management, highlighting the importance of early recognition and intervention. We presented a case series consisting of three newborns who presented with stridor and respiratory distress symptoms to our center. All three cases were diagnosed using a flexible laryngoscope, and surgical intervention was done. The vallecular cyst was removed, and subsequent follow-up showed no recurrence of the lesion. This case series highlights the importance of early suspicion and recognition of vallecular cysts in newborns, emphasizing the thorough examination during diagnostic evaluations. Proper surgical planning and appropriate ventilation strategies are essential for the successful management and resolution of symptoms.

Upper airway manifestations of anti-IgLON5 disease: Otorhinolaryngological point of view.

INTRODUCTION: Anti-IgLON5 disease is a recently described neurological disorder with multisystemic features. The disease is characterized by the presence of IgLON5 antibodies in serum and cerebrospinal fluid. Our objective is to describe in detail the otorhinolaryngological manifestations of this disease, which are frequent and may include dysphagia, dysarthria, vocal cord paralysis and laryngospasm. METHODS: In this study, we present a series of 9 patients with anti-IgLON5 disease and otolaryngological manifestations. Patients were evaluated between July 2012 and March 2022 by video-polysomnography, fiber-optic laryngoscopy, and functional endoscopic evaluation of swallowing. RESULTS: The median age was 71 years, and 5 (56%) were female. Video-polysomnography showed a NREM/REM parasomnia in 6 patients (67%), obstructive sleep apnea in 8 (88%), stridor during sleep in 7 (78%) and central apneas in 1 (11%). Six out of the 9 patients (67%) presented episodes of acute respiratory failure that required mechanical ventilation, 6 had vocal fold palsy with 4 of them requiring tracheostomy (3 had to be performed on an emergency basis). Dysphagia occurred in 8 patients (89%). Prominent upper airway secretion and sialorrhea was also present in 3 cases. CONCLUSION: The anti-IgLON5 disease exhibits extensive otolaryngological symptoms, mainly affecting the upper airway. These symptoms affect the quality of life and can be life-threatening. Prompt acute management is essential for stridor, dyspnea, and dysphagia. Given the potential severity of the symptoms and rarity of the disease, it is important for otolaryngologists to be familiar with anti-IgLON5 disease. LEVEL OF EVIDENCE: Level 4.

Recurrent Pediatric Respiratory Distress From a Challenging Vascular Anomaly: The Uncrossing Operation for Circumflex Aorta.

The authors report a case of circumflex aorta causing persistent respiratory distress in a 9-year-old boy who had previously undergone vascular ring division and multiple aortopexies. The uncrossing operation was performed, with symptomatic relief of both posterior and right-sided tracheal compression from the transverse aorta and right aortic arch, respectively.

Anesthetic Management of a Massive Cystic Hygroma of the Neck in a Neonate.

Cystic hygroma of the neck, a congenital benign tumor of the lymphatic system, is a potential cause of neonatal airway obstruction leading to stridor. Meticulous airway evaluation, case appropriate preparation, and use of advanced technology, including videolaryngoscope and ultrasonography, can facilitate the safe management of the difficult airway.

Risk of Post-Intubation Laryngotracheal Stenosis With Respect to COVID-19 Status in a Large Multicenter Cohort Cross-Sectional Study.

OBJECTIVES: Occurrence of post-intubation laryngotracheal stenosis (LTS) with respect to COVID-19 status. DESIGN: Retrospective cross-sectional inpatient database. SETTING: Eleven Midwest academic and community hospitals, United States. PATIENTS: Adults, mechanically ventilated, from January 2020 to August 2022, who were subsequently readmitted within 6 months with a new diagnosis of LTS. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Six thousand eight hundred fifty-one COVID-19 negative and 1316 COVID-19 positive patients were intubated and had similar distribution by age (median 63.77 vs. 63.16 yr old), sex (male, 60.8%; n = 4173 vs. 60%; n = 789), endotracheal tube size (≥ 7.5, 75.8%; n = 5192 vs. 75.5%; n = 994), and comorbidities. The ICU length of stay (median [interquartile range (IQR)], 7.23 d [2.13-16.67 d] vs. 3.95 d [1.91-8.88 d]) and mechanical ventilation days (median [IQR], 5.57 d [1.01-14.18 d] vs. 1.37 d [0.35-4.72 d]) were longer in the COVID-19 positive group. The occurrence of LTS was double in the COVID-19 positive group (12.7%, n = 168 vs. 6.4%, n = 440; p < 0.001) and was most commonly diagnosed within 60 days of intubation. In multivariate analysis, the risk of LTS increased by 2% with each additional ICU day (hazard ratio [HR], 1.02; 95% CI, 1.02-1.03; p < 0.001), by 3% with each additional day of ventilation (HR, 1.03; 95% CI, 1.02-1.04; p < 0.001), and by 52% for each additional reintubation (HR, 1.52; 95% CI, 1.36-1.71; p < 0.001). We observed no significant association COVID-19 status and risk of LTS. CONCLUSIONS: The occurrence of post-intubation LTS was double in a COVID-19 positive cohort, with higher risk with increasing number of days intubated, days in the ICU and especially with the number of reintubations. COVID-19 status was not an independent risk factor for LTS.

Successful Management of Retropharyngeal Lymphangioma with Stridor Using Sirolimus: A Case Report.

Lymphatic malformations (LMs) are one of the congenital malformations of the lymphatic system in the body. The patient usually presents with head and neck swelling, airway compression, and/or airway obstruction. The diagnosis of retropharyngeal LMs can be challenging due to their rare occurrence. We report a case of a five-month-old boy diagnosed with retropharyngeal LMs. He presented with a three-day history of fever, cough, and stridor and was initially treated for acute bronchiolitis. A lateral neck radiograph revealed prevertebral widening, suggesting retropharyngeal collection. The patient's condition worsened, requiring intubation in the operating room and proceeding with aspirations and drainage. However, the symptoms recurred after a few days, necessitating re-intubation, repeated aspirations and drainage procedures. The patient was intubated, and the neck's magnetic resonance imaging (MRI) confirmed retropharyngeal LMs. An elective tracheostomy was performed and was treated with sirolimus. The patient had a successful tracheostomy decannulation and showed no recurrence during follow-up.

Stridor caused by endobronchial lymphoma in a middle-aged woman.

Diffuse large B-cell lymphoma, primarily nodal in nature, can present with rare endobronchial involvement, underscoring the importance of considering it in the differential diagnoses of endobronchial lesions.

An Uncommon Epiglottic Cyst Presentation in an Adolescent: A Case Report and Literature Review.

Epiglottic cysts are benign lesions of the larynx that are relatively rare beyond infancy age. A 17-year-old adolescent male patient presented to the outpatient specialized oropharyngeal clinic with inspiratory stridor, chronic sore throat, and progressive dyspnea symptoms over the past eight months. Examination by a headlight and a tongue depressor showed a large cystic lesion arising from the hypopharynx. A neck computed tomography (CT) scan revealed a 4 cm oval cyst attached to the lingual epiglottic surface. The relatively large epiglottic cyst was drained directly in the clinic and was later removed by microlaryngosurgery with traditional microinstrumentation in a follow-up visit. Subsequent recovery was uneventful. Regardless of the rarity of epiglottic cysts in adolescents, doctors should keep in mind this etiology as early diagnosis and management could spare the patient from life-threatening complications or tracheostomy and unneeded medical costs.

Surgical management of Chiari II malformation: a systematic review of literature.

PURPOSE: Chiari II malformation (CM-II) is a congenital malformation of the posterior fossa associated with myelomeningocele. Of the symptomatic patients, 10-33% require surgical treatment. To this date, there is not a consensus about the best surgical technique, and whether to do duroplasty. METHODS: A literature search of the PubMed database and crossed references was performed, per PRISMA guidelines. Data regarding demographic features, extent of cervicomedullary deformity, clinical presentation, surgical techniques, and clinical outcomes were extracted. Pearson's chi-squared test was applied. The p-values under 0.05 were considered statistically significant. RESULTS: Twenty studies (N = 330) were analyzed. C3 and C4 levels represented 56.4% of the lowest tonsil displacement. The most reported symptom was dysphagia/swallowing dysfunction (53.8%). Suboccipital craniectomy (SOC) and cervical spine expansion (CSE) with duroplasty were the most reported technique. Dural augmentation was performed in 57.4% of the patients. After surgery, 59.6% observed an improvement in symptoms and quality of life, 12.5% were unchanged, and 27.8% had a worsened clinical status. The mortality rate was 2.5% during the first month after surgery, and 17.4% at the last follow-up evaluation. Patients who underwent CSE presented a better clinical outcome (p = 0.002). The SOC procedure could not be correlated with symptom improvement (p = 0.06). CONCLUSION: CM-II is associated with high morbidity and mortality. An early onset symptomatic CM-II demands intervention, which provided an improvement of outcome in most patients included in this review. The best surgical technique and the exact effect of the surgical management of CM-II on mortality are not yet clear.