Importance: Presence of laryngotracheal abnormalities is associated with increased morbidity and higher mortality rate in esophageal atresia patients.Objective: Determine the prevalence of laryngotracheal abnormalities (LTA) in a prospectively collected cohort of patients treated for esophageal atresia and/or tracheoesophageal fistula (EA/TEF). Analysis of the impact of those airway anomalies in early post-operative outcomes was performed.Patients and Methods: This was a review of a prospectively collected database, including patients from January 2008 to December 2017. Patients enrolled in the present study were treated in a high-volume referral center. Present study included all newborn-infants consecutively treated for EA/TEF. All patients were evaluated by flexible laryngotracheoscopy performed under local anesthesia in spontaneous breathing. In case of airway malformation suspected during flexible endoscopy, a rigid endoscopy was performed to complete airway assessment. If post-operative respiratory symptoms (noisy breathing, respiratory difficulty, failure to extubate, or difficulty feeding) were noted, a second laryngotracheoscopy was performed. Primary study outcome was to evaluate the prevalence of LTA in EA/TEF infants, characterizing of LTA, and their impact on early post-operative outcomes. Those primary study outcomes were planned before data collection began.Results: During the study period 207 patients with EA/TEF were treated. LTA had a period prevalence of 40.1% (83/207). Although no differences were recorded in terms of demographics and clinical presentation, LTA+ infants more frequently required tracheostomy (12/52, 23% vs. 0/124, 0%; p 0.0001) and were at increased risk of death (12/83, 14% vs. 5/124, 4%; p 0.009) in comparison with EA/TEF without LTA.Conclusions: Present data suggest a high prevalence of congenital LTA in patients affected by EA. Most of the abnormalities are congenital and a high proportion of patients with LTA require a tracheostomy. Mortality significantly correlates with the presence of LTA. Systematic airway endoscopic preoperative evaluation has to be pushed forward to minimize LTA-related morbidity and mortality.

Aim of the study: Lymphatic malformations (LMs) are rare entities, sometimes difficult to treat, that may be life-threatening when intricately connected to airway structures. Invasive treatments are occasionally required, with sclerotherapy considered the treatment of choice and surgery as a second-line approach. The aim of the present study was to evaluate our multidisciplinary team experience in treating newborns affected by LMs requiring operative management, while defining early outcomes.Methods: Retrospective review of all consecutive patients admitted for LMs requiring operative management between January 2000 and January 2019. Patients were mainly characterized based on anatomical district of the LM (and further stratified based on the development of respiratory distress), need for tracheostomy, number of sclerotherapies, indication for surgery, and residual disease beyond the 1st year. Morbidity and mortality were also evaluated. Fisher exact test and Mann–Whitney test were used as appropriate. Statistical significance was set at p < 0.05.Results: Fifty-seven patients were included in the study, 36 with cervicofacial and/or mediastinal LMs and 21 with LMs of other anatomical districts. Due to the risk of developing respiratory distress at birth, patients with cervicofacial and/or mediastinal LMs were divided into two groups (8/36 group A vs. 28/36 group B). Group A patients are at higher risk for tracheostomy (7/8 group A vs. 1/28 group B, p = 0.0001) and more often require surgical reduction of the residual lymphatic abnormality (5/8 group A vs. 4/28 group B, p = 0.013). They also require sclerotherapies more often, but the difference is not statistically significant (8/8 group A vs. 19/28 group B, p = 0.15). Patients with cervicofacial/mediastinal LMs frequently suffer from persistent residual disease beyond the 1st year of life, significantly more often in group A (7/8 group A vs. 12/28 group B, p = 0.043).Conclusion: LMs are rare conditions with potential life-threatening behavior. Their intrinsic clinical complexity requires a multidisciplinary approach to the affected patients. Planning a long-term follow-up is essential because of the late-term problems those patients may experience.

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In our series, ARMs were associated only with SD developing after primary neurulation, suggesting a single insult leading to both SD and the associated ARM. Neurosurgery is indicated less frequently in patients with ARM-associated SD, despite the similar preoperative clinical features.

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