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OBJECTIVE: Patients with severe laryngomalacia (LM) and high-risk comorbidities require surgical interventions such as supraglottoplasty. However, evidence supporting epiglottopexy for these patients is scarce. This study aims to report the surgical outcomes of external double needle puncture using a single thread for epiglottopexy in severe LM patients with high-risk comorbidities.
METHODS: This retrospective study was conducted at a single tertiary hospital. We enrolled 32 patients under 12 months with severe LM who underwent external double needle puncture epiglottopexy. We compared clinical factors between the successful and failed groups and identified risk factors for the failure of epiglottopexy.
RESULTS: Of the airway surgical outcomes, 22 (68.7 %) patients were in the successful group. In the failed group (n = 10, 32.3 %), patients received tracheostomies due to uncontrolled saliva (n = 3), the need for mechanical ventilation (n = 3), and uncontrolled LM (n = 4). There was a statistically significant difference in body weight at the time of surgery and the proportion of associated comorbidities between the two groups. Gestational age was the only factor significantly associated with successful surgical outcomes in both univariate and multivariate logistic regression analyses (odds ratio = 2.263; 95 % confidence interval, 1.042-4.918; P = 0.039).
CONCLUSIONS: External double needle puncture epiglottopexy is an effective surgical method for patients with LM who present with a retroflexed floppy epiglottis and high-risk comorbidities. Low gestational age is a major risk factor for surgical failure. Consideration of factors such as the need for mechanical ventilation and uncontrolled saliva should be prioritized before and after surgery to enhance surgical success.

Tracheal stenosis and paradoxical vocal fold motion are both common laryngological diagnoses that can present with similar symptoms of dyspnea. Co-morbid psychiatric issues can complicate diagnostic accuracy and lead to logical fallacies in the attribution of symptom etiology. We present a case of a 38-year-old female who presented repeatedly to the emergency department with respiratory distress, inspiratory stridor, wheezing, and anxiety. On examination, she had stridor that appeared to correlate with episodes of elevated anxiety and bedside laryngoscopy which showed intermittent paradoxical vocal fold motion. A computed tomography scan showed 40% narrowing of the distal tracheal lumen, but symptoms were felt to be inconsistent and out of proportion to stenosis. She was seen several more times in the ED and eventually followed up in the laryngology clinic, where she had a tracheoscopy showing Cotton Meyer grade III stenosis. This unique case highlights the logical fallacies that may lead to misdiagnosis when evaluating stridorous patients with comorbid personality and anxiety disorders.

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: Moderate laryngomalacia lies in the grey zone where the parents and treating team might hesitate to decide the treatment plan for the child. Neurodevelopmental assessment of the child was neither assessed nor incorporated in surgical decision-making in the past.
OBJECTIVE: To evaluate the neurodevelopmental outcome of moderate laryngomalacia treatment strategies (surgery versus conservative).
METHODS: We conducted an observational multicentric cohort study. We compared two groups of patients according to their moderate laryngomalacia treatment strategy: surgical treatment and no treatment. The neurodevelopmental outcome was assessed by Griffiths-III developmental scales.
RESULTS: A total of 150 children were diagnosed with moderate laryngomalacia of which 56 were successfully treated with surgery and 94 improved without intervention. The median Griffiths-III developmental quotients (DQs) of subscales A, B, C, E, and general development were significantly higher in the surgically treated group compared to conservatively treated ones.
CONCLUSIONS: Untreated moderate laryngomalacia cases have worse neurodevelopmental outcomes than surgically treated cases.

OBJECTIVE: Laryngomalacia is the most common pediatric laryngeal anomaly. The pathophysiology of laryngomalacia is not well defined; the leading hypothesis suggests weak laryngeal tone and neuromuscular discoordination. Only a few studies explored the histopathology of the laryngeal submucosal nerves, with reported nerve hypertrophy. Our study aims to describe the histopathology of submucosal nerves in specimens obtained from children with severe laryngomalacia compared to pediatric cadaveric controls.
METHODS: Prospective study.
METHODS: Tertiary care children\'s hospital.
METHODS: Histologic and immunohistochemical sections of supraglottic tissue from 26 children with severe laryngomalacia and six pediatric autopsies were digitally scanned and assessed with image analysis software (QuPath), resulting in the identification and measurement of 4561 peripheral nerves and over 100,000 foci of neurofilaments.
RESULTS: Chronic inflammation was noted in all patients. Eosinophils were rare. The mean nerve area and perimeter were significantly smaller for patients with laryngomalacia compared to the control group (1594.0 ± 593.2 μm^2 vs. 2612.1 ± 2824.0 μm^2, p < 0.0001, and 158.8 ± 30.3 μm vs. 217.6 ± 165.0 μm, p < 0.0001). Nerve-per-area unit was significantly greater for patients with laryngomalacia compared to controls (1.39E-05 vs. 6.19 E-06, p = 0.009). The mean area and the number of neurofilaments per total nerve area were similar. Immunohistochemistry for calretinin, a marker for intestinal ganglion cells in Hirschsprung disease, was absent from all specimens.
CONCLUSIONS: This series includes a comparison of all identifiable nerve fibers obtained from children with severe laryngomalacia and shows that the mucosal nerves are smaller on average than controls. These findings fail to provide support for significant morphologic peripheral nerve pathology in laryngomalacia.

Objective: To analyze the clinical characteristics and the risk factors associated with severe laryngomalacia in children. Methods: In this study, the clinical data of children (0-18 years), including gender, age at presentation, preterm delivery, low birth weight, delivery mode, feeding mode, fetal delivery, medical comorbidities, maternal gestational age at presentation, and calcium supplementation during pregnancy, diagnosed with laryngomalacia between January 2013 and January 2023 were retrospectively analyzed. The children were divided into mild-moderate and severe groups. Several risk factors were compared and analyzed between the 2 groups. The statistically significant risk factors were included in the logistic regression analysis. Results: A total of 224 children with severe laryngomalacia were enrolled in this study. The ratio of male to female patients was 1.55:1. All patients had severe laryngomalacia manifested by inspiratory laryngeal stridor. The average age of patients at symptom presentation was 2.7 (1.5-5.2) months. There were significant differences between the 2 groups in the age at presentation, premature delivery, low birth weight, medical comorbidities, and calcium supplementation during pregnancy (P < .05). Multivariate logistic regression analysis showed that premature delivery [odds ratio (OR) = 3.177, 95% confidence interval (CI): 2.329-4.334], low birth weight (OR = 3.188, 95% CI: 2.325-4.370), and medical comorbidities (OR = 1.434, 95% CI: 1.076-1.909) were independent risk factors for severe laryngomalacia (P < .05). Conclusion: Children with severe laryngomalacia exhibited persistent stridor at an earlier age at presentation. Premature delivery, low birth weight, and medical comorbidities were potential risk factors for severe laryngomalacia in children.

UNASSIGNED: The pathology of laryngomalacia is still not clear. The aim of this study was to investigate the relationship between vitamin D levels and laryngomalacia, and to evaluate vitamin D levels according to the classification of laryngomalacia.
UNASSIGNED: This retrospective study was conducted in the Kahramanmaraş Sütçü İmam University Medicine Faculty\'s Otorhinolaryngology Clinic between June 2014 and January 2021. Laryngomalacia was classified. Laboratory tests for all patients included calcium (Ca), phosphorus (P), parathormone (PTH), blood urea nitrogen (BUN), creatinine (Cre), alanine transaminase (ALT), and 25-hydroxy vitamin D (25-OH-D).
UNASSIGNED: Evaluations were performed for 64 infants with laryngomalacia, including 41 male and 23 female infants with a mean age of 4.6 ± 3.0 months, and a control group of 64 healthy infants with a mean age of 4.5 ± 2.8 months. A statistically significant difference was determined between the laryngomalacia group and the control group with respect to 25-OH-D and PTH levels (p < 0.001). When data were examined according to laryngomalacia types, a statistically significant difference was determined between the groups for 25-OH-D, Ca, P, PTH, and ALT values. The 25-OH-D level was statistically significantly lower in the severe laryngomalacia group than in the mild and control groups (p < 0.001). A statistically significant difference was determined between the moderate and severe laryngomalacia groups and the control group regarding PTH levels (p < 0.001).
UNASSIGNED: Vitamin D deficiency may have a role in the etiology of laryngomalacia, and this view is supported by the finding that there was a decrease in vitamin D levels associated with laryngomalacia classification. In addition, the reduction in PTH levels in infants with laryngomalacia may be explained by the change in Ca metabolism. It would be appropriate for further studies to investigate the response to vitamin D replacement therapy in patients with moderate and severe laryngomalacia.

Laryngomalacia (LM) and laryngeal cleft (LC) can independently cause dysphagia but rarely can occur concomitantly. We discuss the presentation, decision-making, and swallow outcomes following surgical correction of combined LM and LC. We present four patients with combined LM and an LC who underwent both primary supraglottoplasty (SGP) and laryngeal cleft repair (LCR). Each patient presented with recurrent choking or coughing with feeds. Stridor was only present in two patients. Patients with SGP saw the resolution of stridor when present, but dysphagia persisted in all four cases. LCR clinically and objectively resolved all symptoms of dysphagia. We found that flexible fiberoptic laryngoscopy is not always reliable at detecting combined pathology. Patients presenting with persistent dysphagia following SGP should be suspected of having interarytenoid pathology. We recommend a staged surgical approach with SGP before LCR.

BACKGROUND: The Koolen-de Vries syndrome (KdVS) is a relatively new rare disease caused by micro-deletion of 17q21.31 which was first reported by Koolen in 2006. Typical phenotypes for KdVS include hypotonia, developmental delay, moderate intellectual disability, and characteristic facial dysmorphism. Up to now, there was only one case report about anesthesia management of patient diagnosed KdVS. It was a 2-year-old girl who experienced an MRI exam under anesthesia.
METHODS: We described a 21-month-old boy who planned to undergo an orchidopexy under general anesthesia diagnosed with KdVS. He had an intellectual disability, characteristic facial dysmorphism, tracheo/laryngomalacia, patent foramen ovale, and cryptorchidism related to KdVS. Due to the complex condition especially the presence of tracheo/laryngomalacia, we took some special measures, including reducing the amount of long-acting opioid, keeping the spontaneous breath, performing a caudal block, and applying the laryngeal mask. But the laryngeal mask was changed to an endotracheal tube because it failed to provide adequate ventilation. The boy experienced mild laryngeal spasm and hypoxia after extubation, but lateral position and etomidate eased his breathing problem and re-intubation was avoided. It is indicated that anesthesia management for patients with orphan disease is a real challenge for all anesthesia providers.
CONCLUSIONS: The Koolen-de Vries syndrome is a relatively new orphan disease involving multiple systems. Keeping spontaneous breath, evaluating airway potency to anesthetics, applying endotracheal tube, and post-extubation lateral or prone position may be helpful for airway management for patient with hypotonia and tracheo/laryngomalacia. KdVS patient needs prolonged post-anesthesia monitoring and/or medication for airway complications.