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.

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.

BACKGROUND: Nontoxic nodular goiter is one of the most prevalent thyroid conditions worldwide. Thyroidectomy for large goiters has a relatively high risk of postoperative airway obstruction, with tracheomalacia being one of the potential complications.
METHODS: A 61-year-old woman complained of a lump in her neck for 45 years. The node is progressively enlarged, but she did not experience any breathing difficulty, hoarseness, or pain while swallowing. A total thyroidectomy was then performed. The histopathologic examination revealed colloid goiter. During the procedure, evaluation of the trachea revealed a tracheomalacia, so a tracheotomy was then performed on the patient. After a follow-up period of three months, the patient was no longer experiencing tracheomalacia, and the tracheostomy was successfully closed.
CONCLUSIONS: Surgery has been considered an acceptable approach for managing non-toxic goiter. The most common indications are compressive symptoms, substernal extension, inability to control hyperthyroidism through medication, and a suspicion of malignancy. However, thyroidectomy for large goiter carries a relatively high risk of postoperative respiratory obstruction. Diagnosing tracheomalacia can be challenging and often relies on bronchoscopy to assess the airway and observe the collapse of cartilage and membranes. Acquired tracheomalacia can be managed through internal or external stenting or tracheostomy.
CONCLUSIONS: Total thyroidectomy has been recommended as a suitable procedure for non-toxic and toxic multinodular goiter. Tracheomalacia may occur following thyroidectomy in patients with thyroid enlargement. Tracheostomy effectively manages tracheomalacia by creating a channel across the malacia\'s focal segment, restoring the airway\'s patency.

Tracheal agenesis (TA) is a rare congenital anomaly with an incidence of 1 per 50,000 newborns. It appears at birth with severe respiratory distress, cyanosis, and inaudible crying. Prompt esophageal intubation and long-term management of the esophageal airway are essential to overcome this catastrophic condition. In the long-term management, external stenting of the esophageal airway has been reported as promising to support the fragile esophageal wall; this technique was taken from the surgery for tracheomalacia. We experienced a case of an infant with tracheal agenesis whose respiratory status was stabilized after external esophageal stenting. The stenting was performed based on a lesson learned in the extensive experience in the surgical treatment for tracheomalacia, and the surgical techniques for successful stenting are herein described.

Operating on a huge and long-standing goiter is challenging to the surgeon and anesthetist because of the possibility of tracheomalacia and collapse of the tracheal rings after extubation. We report our innovation of tenting the trachea to the skin using sutures (passed through the strap and sternocleidomastoid muscles) to prevent post-thyroidectomy tracheomalacia.

We report a rare case of airway obstruction caused by megaesophagus associated with achalasia. A 78-year-old man was admitted with post meal dyspnea, decreased consciousness, expiratory and inspiratory wheezing, and respiratory distress. Arterial blood gas analysis showed findings of marked acute respiratory acidosis (pH 7.18, PaCO2 75 mmHg, PaO2 225 mm Hg, HCO3- 22 mmol/L). An emergency laryngoscopy was performed because of a suspected airway obstruction, but no abnormalities were observed from the airway to the glottis. Noninvasive positive pressure ventilation (NPPV) was immediately introduced, and the respiratory rate and breathing pattern was normalized. A chest X-ray showed an enlarged upper mediastinal outline and an ill-defined border of the trachea. A computed tomography (CT) scan showed an enlarged esophagus with a maximum diameter of 9.90 cm, compressing the trachea to the back of the sternal notch. Following removal of the esophageal contents using a nasogastric tube, NPPV was discontinued with no respiratory episodes. After he was stabilized, he was transferred to another hospital for endoscopic myotomy. In a review of the literature, we identified 66 cases of airway obstruction due to achalasia, mainly in older women. None of the patients received NPPV. As a differential diagnosis for acute airway obstruction, achalasia-related airway obstruction should be considered, particularly in older women. Furthermore, since this condition is suspected to involve tracheomalacia, NPPV may be a useful respiratory support therapy.

Prolonged intubation is associated with several complications leading to upper airway obstruction, including tracheal stenosis and tracheomalacia. Tracheostomy may potentially decrease the risk of tracheal injury in patients with upper airway obstruction. The ideal timing to perform tracheostomy remains controversial. Prolonged intubations were particularly common during the initial phase of the coronavirus disease 2019 (COVID-19) pandemic. This study aimed to present a series of five cases of upper airway complications in patients who underwent mechanical ventilation in the setting of COVID-19 and discuss their clinical aspects, risk factors, and therapeutic strategies.

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Tracheomalacia refers to diffuse or segmental tracheal weakness. Most commonly, tracheomalacia develops after prolonged endotracheal intubation or tracheostomy. Surgical management is warranted in symptomatic patients with severe tracheomalacia. Relief of airway obstruction via stenting often provides immediate improvement in both airflow and symptoms. However, stent placement is associated with significant complications. Here, we present the case of a 71-year-old man who was brought to the emergency department with acute respiratory distress. The patient was known to have tracheomalacia with tracheoesophageal fistula. He had multiple medical comorbidities, including longstanding hypertension, diabetes mellitus, and asthma. The patient had a progressive decline in his level of consciousness and was admitted to the intensive care unit for further management. Despite the maximum ventilatory support, the patient did not achieve an adequate oxygenation level. The patient underwent tracheal stent placement by the interventional radiology team. The insertion was unsuccessful despite three attempts. The tracheal stent had migrated into the upper esophagus on the first and second insertion attempts. Because the patient was unstable to tolerate further attempts, the multidisciplinary team recommended the insertion of an esophageal stent to cover the tracheoesophageal fistula. Despite this, the patient continued to have air leakage with progressive worsening of his respiratory condition as he developed multiorgan failure and died. The management of tracheomalacia in the setting of the tracheoesophageal fistula may pose several challenges. The present case highlights an essential complication of stent placement with the stent migrating into the tracheoesophageal fistula, which is an unusual site of migration. A multidisciplinary approach is crucial in the management of difficult cases of tracheomalacia.

Tracheal stenosis and tracheomalacia in patients with congenital scoliosis are serious and rare conditions caused by congenital dysplasia, postintubation injury, trauma, and tracheal tumor. Anesthesia of a child with tracheal stenosis is challenging for anesthesiologists. We describe an 8-year-old female patient developed severe tracheal stenosis and tracheomalacia after growing rod implantation for congenital scoliosis. Comprehensive assessment of preoperative pulmonary function and airway morphology, which can be neglected clinically, should be performed in congenital scoliosis patients.