Primary Tracheomalacia

doi:10.1016/S0003-4975(10)60430-4

The Annals of Thoracic Surgery

Volume 35, Issue 5, May 1983, Pages 538-541

https://doi.org/10.1016/S0003-4975(10)60430-4 Get rights and content

Abstract

Tracheomalacia is a rare congenital malformation of the tracheobronchial cartilages in which the supporting cartilaginous rings permit expiratory collapse of the airway. The condition is usually mild and self-limited. There is a severe variant, however, that is life-threatening and warrants separate categorization. Four children with severe primary tracheomalacia were treated recently. The clinical symptoms, diagnostic findings, and eventual treatment of these patients were highly distinctive and almost identical in all 4, permitting us to make the following observations: (1) primary severe tracheomalacia must be suspected in infants with unexplained respiratory distress manifested by stridor and cyanosis; (2) symptoms are not present at birth but appear insidiously after the first weeks of life, are markedly aggravated by respiratory tract infections, and are made worse by agitation; (3) bronchoscopy is essential for definitive diagnosis and should be employed early in the diagnostic process; (4) tracheostomy is probably essential in most instances; and (5) resolution, although spontaneous, does not occur until after 2 years of age.

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Cited by (38)

Tracheobronchial stenting for airway malacia
2018, Paediatric Respiratory Reviews
Citation Excerpt :
Signs include cyanosis, a prolonged expiratory phase, stridor (which can be bi-phasic) respiratory distress and signs of infection [5,16,17]. Sternal or intercostal retraction may be apparent when TBM is severe [16,18,19]. Respiratory distress may manifest as grunting, open mouth breathing and head retraction [20].
Tracheobronchomalacia is a rare but clinically troublesome condition in paediatrics. The softening of the major airways – which can include some or all of the tracheobronchial tree can lead to symptoms ranging from the minor (harsh barking cough, recurrent chest infections) to severe respiratory difficulties including prolonged ventilator support and ‘near death attacks’. The causes are broadly divided into intrinsic softening of the airway wall which is considered a primary defect (e.g. syndromes; post tracheo-oesophageal fistula repair; extreme prematurity) or secondary malacia due to external compression from vascular structures or cardiac components. These secondary changes can persist even when the external compression is relieved, for example, following the repair of a pulmonary artery sling or double aortic arch. For children with severe clinical symptoms attributed to malacia, consideration is given to possible surgical remedies such as an aortopexy for short limited areas of malacia, or long term positive pressure support with CPAP either by non invasive or tracheostomy interface. More recently the role of stenting in children is receiving attention, especially with the development of newer techniques such as bioabsorbable stents which buy time for a natural history of improvement in the malacia to occur. This paper reviews the stents available and discusses the pros and cons of stenting in paediatric airway malacia.
External stenting: A reliable technique to relieve airway obstruction in small children
2017, Journal of Thoracic and Cardiovascular Surgery
Citation Excerpt :
The mean percent diameter was 88.5% ± 13.7% for the bronchus and 94.5% ± 8.2% for the T (Figure 7). An airway obstruction in children may result from a cartilage ring,1 congenital TBM,2 vascular compression,3 and other reasons. Presently, sliding plasty is the gold standard for a tracheal ring.7,8
Airway obstruction in children may be caused by conditions such as vascular compression and congenital tracheobronchomalacia. Obstructive pulmonary vascular disease may be a detrimental sequel for patients with congenital heart disease. We evaluate our own original external stenting technique as a treatment option for these patients.
Ninety-eight patients underwent external stenting (1997-2015). Cardiovascular anomalies were noted in 82 (83.7%). Nine patients had hypoplastic left heart syndrome and 6 had other types of single-ventricular hearts.
The median age at the first operation was 7.2 months (range, 1.0-77.1 months). The mechanisms were tracheobronchomalacia with (n = 46) or without (n = 52) vascular compression. Patients underwent 127 external stentings for 139 obstruction sites (62 trachea, 55 left bronchus, and 22 right bronchus). The stent sizes varied from 12 to 16 mm. There were 14 (8 in the hospital and 6 after discharge) mortality cases. Nine required reoperation for restenosis and 3 required stent removal for infection. The actuarial freedom from mortality and any kind of reoperation was 74.7% ± 4.6% after 2.8 years. The negative pressure threshold to induce airway collapse for congenital malacia (n = 58) improved from −15.9 to −116.0 cmH₂O. A follow-up computed tomography scan (>2.0 years interval from the operation; n = 23) showed the mean diameter of the stented segment at 88.5% ± 13.7% (bronchus) and 94.5% ± 8.2% (trachea) of the reference.
External stenting is a reliable method to relieve airway compression for small children, allowing an age-proportional growth of the airway.
Controlled trachea suspension for tracheomalacia after resection of large anterior mediastinal mass
2015, Annals of Thoracic Surgery
Tracheomalacia is a disorder of the large airways that is often caused by a large anterior mediastinal mass. This study describes 7 patients who underwent controlled trachea suspension as a surgical intervention to prevent severe tracheomalacia and provide potent relief of airway symptoms. All patients recovered well. The results demonstrate this procedure may be safe and effective for resection of a large mediastinal mass compressing the trachea with collapsed segments.
Tracheomalacia secondary to obstructive sleep apnea
2005, American Journal of Otolaryngology - Head and Neck Medicine and Surgery
Tracheomalacia (TM) is abnormal flaccidity of the trachea caused by inadequate cartilaginous support by the C-shaped tracheal rings. This defect generally results in various degrees of dynamic narrowing of the airway lumen. TM usually presents with expiratory stridor, wheeze, and respiratory obstruction. TM is usually associated with tracheoesophageal fistula and esophageal atresia or with other thoracic lesions of vascular rings and tumors. We report a well-documented case of a 20-month-old boy who presented to our institution with a severe history of obstructive sleep apnea and adenotonsillar hypertrophy. On direct laryngoscopy/bronchoscopy, severe TM was noted. Tonsillectomy and adenoidectomy was performed, and by the 37th postoperative day, the TM had completely resolved. We review the literature, discuss the differential diagnosis and clinical evaluation, and propose a new pathophysiological mechanism by which obstructive sleep apnea causes TM.
Tracheomalacia and tracheobronchomalacia in children and adults: An in-depth review
2005, Chest
Citation Excerpt :
In the majority of healthy and even premature infants, primary TM is a self-limiting disease. Most infants outgrow the condition by the age of 2 years.17,21,36 In the patients with connective tissue disorders and congenital syndromes, the effects of TM often persist and may even be fatal.26,37
Tracheomalacia and tracheobronchomalacia are disorders that are encountered in both pediatric and adult medicine. Despite increasing recognition of these disease processes, there remains some uncertainty regarding their identification, causes, and treatment. This article is intended to be a comprehensive review of both the adult and pediatric forms of the diseases, and includes sections on the historical aspects of the disorders, and their classification, associated conditions, histopathology, and natural history. We also review the various modalities that are used for diagnosis as well as the state of the art of treatment, including airway stent placement and surgical intervention
Anomalies of the Airways, Mediastinum, and Lung Parenchyma
2005, Avery's Diseases of the Newborn

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Primary Tracheomalacia

Abstract

Chest

J Thorac Cardiovasc Surg

Ann Thorac Surg

J Thorac Cardiovasc Surg

Tracheomalacia

Ann Otol Rhinol Laryngol

Generalized familial bronchomalacia

Acta Paediatr Scand