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Boyden's nomenclature, which was based on postmortem specimens and published in 1955 prior to the advent of computed tomography (CT), is commonly used to describe the normal segmental bronchial anatomy and various abnormalities. However, several additional anomalies have been recognized since that time, and there is some confusion over the names used to describe these anomalies. Several congenital branching anomalies affecting the trachea, main bronchi, and intermediate bronchus have been reported, all of which can be recognized at chest CT but are often overlooked. These anomalies, which probably occur early in fetal life, can be either supernumerary, with defects occurring at 29-30 days gestation, or displaced, with defects occurring later. Tracheobronchial positional anomalies are often associated with other congenital abnormalities but may be isolated. They often are asymptomatic but can be responsible for pulmonary symptoms such as dyspnea, recurrent pneumonia, and hemoptysis. It is essential that these anomalies are recognized prior to lung resection to avoid complications, especially when video-assisted thoracoscopic surgery is performed. In addition, bronchoscopists should be aware of these anomalies before performing diagnostic or therapeutic bronchoscopic procedures. Awareness of a few key bronchial anatomic principles and use of a lobe-based classification scheme will facilitate recognition of tracheobronchial positional anomalies.

IMPORTANCESirolimus is increasingly being used to treat various vascular anomalies, although evidence of its efficacy is lacking.OBJECTIVE To assess the efficacy and safety of sirolimus for children with slow-flow vascular malformations to better delineate the indications for treatment. DESIGN, SETTING AND PARTICIPANTSThis multicenter, open-label, observational-phase randomized clinical trial included 59 children aged 6 to 18 years with a slow-flow vascular malformation who were recruited between September 28, 2015, and March 22, 2018, in 11 French tertiary hospital centers. Statistical analysis was performed on an intent-to-treat basis from December 4, 2019, to November 10, 2020.INTERVENTIONS Patients underwent an observational period, then switched to an interventional period when they received oral sirolimus (target serum levels, 4-12 ng/mL). The switch time was randomized from month 4 to month 8, and the whole study period lasted 12 months for each patient. MAIN OUTCOMES AND MEASURESThe primary outcome was change in the volume of vascular malformations detected on magnetic resonance imaging scan (with centralized interpretation) per unit of time (ie, between the interventional period and the observational period). Secondary outcomes included subjective end points: pain, bleeding, oozing, quality of life, and safety. RESULTS Among the participants (35 girls [59.3%]; mean [SD] age, 11.6 [3.8] years), 22 (37.3%) had a pure venous malformation, 18 (30.5%) had a cystic lymphatic malformation, and 19 (32.2%) had a combined malformation, including syndromic forms. Variations in the volume of vascular malformations detected on magnetic resonance imaging scans associated with the duration period were not overall significantly different between the interventional period and the observational period (all vascular malformations: mean [SD] difference, -0.001 [0.007]; venous malformations: mean [SD] difference, 0.001 [0.004]; combined malformations: mean [SD] difference, 0.001 [0.009]). However, a significant decrease in volume was observed for children with pure lymphatic malformations (mean [SD] difference, -0.005 [0.005]). Overall, sirolimus had positive effects on pain, especially for combined malformations, and on bleeding, oozing, self-assessed efficacy, and quality of life. During sirolimus treatment, 56 patients experienced 231 adverse events (5 serious adverse events, none life-threatening). The most frequent adverse event was an oral ulcer (29 patients [49.2%]).CONCLUSIONS AND RELEVANCE This observational-phase randomized clinical trial allows for clarifying the goals of patients and families when starting sirolimus therapy for children older than 6 years. Pure lymphatic malformations seem to be the best indication for sirolimus therapy because evidence of decreasing lymphatic malformation volume per unit of time, oozing, and bleeding and increasing quality of life was found. In combined malformations, sirolimus significantly reduced pain, oozing, and bleeding. Benefits seemed lower for pure venous malformation...

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