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.
METHODS: 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.

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.
METHODS: 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.
CONCLUSIONS: 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.

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.

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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.

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.

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.

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.

BACKGROUND: 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.
CONCLUSIONS: 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.
METHODS: Level 4.

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.