Tracheal Compression in Marfan Syndrome

Kolilekas, Likurgos; Manali, Effrosyni D.; Kalomenidis, Ioannis; Mouchtouri, Ero; Spyrou, Konstantina; Papiris, Spyros

doi:10.2169/internalmedicine.47.0996

Cited by 7 publications

(4 citation statements)

References 3 publications

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“…Adjacent cardiovascular structures can then cause compression of the airway caliber. [11][12][13] The pathology of great-vessel anomalies was conceptualised from the theoretical embryogenesis of the aortic arch and pulmonary artery. 14 Aberrant embryonic development of great vessels prompts the simultaneous aberrant development of the neighbouring airway.…”

Section: Discussionmentioning

confidence: 99%

Cardiovascular-associated tracheobronchial obstruction in children

Lai

Liao²,

Wong

2012

Cardiol Young

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Tracheobronchial compression of cardiovascular origin is an uncommon and frequently unrecognised cause of respiratory distress in children. The compression may be due to encircling vessels or dilated neighbouring cardiovascular structures. Bronchoscopy and detailed radiography, especially computed tomography and magnetic resonance imaging, are among the most powerful diagnostic tools. Few previous reports have addressed the relationship between bronchoscopic findings and underlying cardiovascular anomalies. The objective of this study was to correlate bronchoscopic and radiographic findings in children with cardiovascular-associated airway obstruction. A total of 41 patients were recruited for the study. Patients with airway obstruction were stratified on the basis of the aetiology of the cardiovascular structures and haemodynamics into an anatomy-associated group and a haemodynamics-associated group. In the anatomy-associated group, stenosis and malacia were found with comparable frequency on bronchoscopy, and the airway obstructions were mostly found in the trachea (71% of patients). In the haemodynamics-associated group, malacia was the most common bronchoscopic finding (85% of patients), and nearly all locations of airway involvement were in the airway below the carina (90% of patients). The tracheal compression was usually caused by aberrant systemic branching arteries in the anatomy-associated group. In the haemodynamics-associated group, the causal relationships varied. Tracheal compression was often caused by lesions of the main pulmonary artery and aorta, whereas obstruction of the right main bronchus was caused by lesions of the main pulmonary artery and right pulmonary artery. The causes of left main bronchus compression were more diverse. In summary, the bronchoscopic presentations and locations are quite different between these two groups.

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Section: Discussionmentioning

confidence: 99%

Cardiovascular-associated tracheobronchial obstruction in children

Lai

Liao²,

Wong

2012

Cardiol Young

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“…Tracheomalacia is seen as one of the most common complications after laryngotracheal separation or scoliosis [6,7]. After laryngotracheal separation, excessive bending of the trachea would induce the narrowed tracheal lumen.…”

Section: Discussionmentioning

confidence: 99%

Surgical strategy for acquired tracheomalacia due to innominate artery compression of the trachea

Tatekawa

Muraji

2011

European Journal of Cardio-Thoracic Surgery

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We reviewed six cases demonstrating acquired tracheomalacia due to innominate artery compression of the trachea and investigated the benefit and effectiveness of a technique for correcting tracheomalacia. All the patients developed scoliosis and the innominate artery run over the trachea. Four cases had permanent neurologic impairment, whereas two patients developed acquired neurologic impairment. The surgical strategy for acquired tracheomalacia due to innominate artery compression of the trachea involved superior mediastinal exposure, external reinforcement with autologous cartilage graft, anterior sling of the innominate artery with a muscle sling, and tracheopexy. Our surgical procedure has been effective in maintaining the patency of the tracheal lumen in all cases but one. This patient suffered from straight back syndrome and developed recurrence of tracheomalacia owing to mucosal infolding secondary to the deformed spine in a supine position. The authors believe our surgical procedure is effective to relieve the symptoms of tracheomalacia, but it is important to select surgical interventions in accordance with the specific patient's condition.

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“…Pulmonary artery aneurysm [90,91] and pulmonary artery dilation [92][93][94] are risk factors of extrinsic vascular CAO, which usually occur below the carina. Besides, extremely abnormal thoracic anatomy has the potential to cause vascular tracheobronchial compression syndrome [95,96]. Severe kyphoscoliosis in Marfan syndrome causes trachea compression by the innominate artery anteriorly and thoracic spine posteriorly [96], which may be the result of defects in extracellular matrix composition, leading to the compromise of the structural integrity of the trachea, artery, and thoracic skeleton.…”

Section: Congenital Vascular Anomaliesmentioning

confidence: 99%

“…In this situation, pulmonary function is helpful to differential diagnosis. Flowvolume loop demonstrating a fixed obstruction pattern with flattening of expiratory portion indicates dynamic intrathoracic obstruction of CAO [1,8,40,96]. Obstructive pattern showing insignificant bronchodilator response is beneficial to exclude the possibilities of asthma [107].…”

Section: Clinical Manifestations Of Vascular Caomentioning

confidence: 99%

Airway management in vascular central airway obstruction: a literature review

Lee¹,

Chao²,

Hung³

et al. 2021

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Vascular central airway obstruction (CAO) is a rare cause of upper airway obstruction in adults. CAO occurs below the level where it is invisible in a laryngoscope. Doctors therefore should pay attention to the possibilities of vascular CAO when attempting to prevent and resolve catastrophic complications from upper airway obstruction such as cardiorespiratory collapse and hemoptysis, which requires a thoughtful preoperative planning of airway management before starting a surgical reconstruction.

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Tracheal Compression in Marfan Syndrome

Cited by 7 publications

References 3 publications

Cardiovascular-associated tracheobronchial obstruction in children

Cardiovascular-associated tracheobronchial obstruction in children

Surgical strategy for acquired tracheomalacia due to innominate artery compression of the trachea

Airway management in vascular central airway obstruction: a literature review

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