Abstract

Tracheomalacia is a rare congenital condition that results from improper formation of the cartilaginous tracheal rings, but is often seen in children who have esophageal atresia with/without trachea-esophageal fistula. This causes the trachea, which is the main airway, to be floppy, and as the child grows, can result in breathing difficulties. These difficulties are usually manifested as breathing noises that may change with position and improve during sleep, or that get worse with coughing, crying, or feeding. Upper respiratory infections can also be more common. While most cases of tracheomalacia resolve by 18 to 24 months of age, a small percentage either continue or cause such severe breathing or feeding issues that surgical intervention is warranted. In cases where the innominate artery is the cause of compression of the weakened trachea, an aortopexy to elevate the vessel up to the sternum and away from the trachea is performed.

Case Overview

Focused History

Patients with tracheomalacia present primarily with breathing difficulties, the most common of which is stridor. Many times this occurs in the setting of prior esophageal atresia-tracheoesophageal fistula repair, with the symptoms beginning only after repair of the atresia. Care should be taken to elicit symptoms consistent with cyanosis or feeding difficulties, as these can indicate a more severe problem than that indicated by the physical examination. Since most cases of tracheomalacia resolve without intervention, it is crucial to take into account the age of the patient and the overall clinical course up to the present when making a decision for surgery.

Physical Findings

Findings consistent with airway compromise guide management. Specifically, breathing noises that may change with position and improve during sleep, or that get worse with coughing, crying, or feeding. Upper respiratory infections can also be more common, along with high-pitched breathing and rattling or noisy breaths. Expiratory stridor at rest, biphasic stridor and cyanosis are findings that are indicative of severe airway compromise. In addition, care should be taken to rule out gastroesophageal reflux disease and, in patients with prior repair, recurrent tracheoesophageal fistula, as these latter two conditions can cause the same symptoms, or even co-exist with tracheomalacia.

Imaging studies

The diagnosis of tracheomalacia is confirmed by tracheobronchoscopy, or direct visualization of the trachea, at which time a recurrent fistula should also be ruled out in those patients with prior EA-TEF repair. Ancillary studies include CT angiogram of the chest to help document compression due to the innominate artery, as well as a barium swallow to rule out gastroesophageal reflux disease in those children where presence of reflux is suspected.

Natural history

Most cases of tracheomalacia resolve with time, with the more mature airways proving more resilience to compression due to increasing diameter of the trachea and maturation of the cartilaginous rings. However, in patients with severe respiratory compromise due to tracheomalacia, progressive pulmonary illness with severe infection and ultimately loss of life can be expected.

Options for treatment

Other than supportive care, including treatment of upper respiratory tract infections, medical management for tracheomalacia includes use of ipratropium bromide and, in severe cases, continuous positive airway pressure, or CPAP

Rationale for treatment in this case

The rationale for aortopexy resides in the severity of the condition being treated. In children with severe respiratory compromise due to compression of the trachea by the innominate artery, aortopexy is the only viable treatment option. Only after the child has failed more conservative measures is aortopexy recommended. While prior attempts have been made at endobronchial stenting, the high complication rate associated with that procedure has caused it to fall into disuse.

Special consideration

Obstruction of the main bronchus by the innominate artery must be documented to ensure that the appropriate patients are being selected for surgery. Prior cases of tracheoesophageal repair require documentation that the fistula has not recurred.

Discussion

Tracheomalacia may be subdivided into primary and secondary classifications. Primary tracheomalacia is a congenital absence of the trachea-supporting cartilage normally present, while secondary tracheomalacia is a result of some external insult to the trachea, such as the previously mentioned tracheoesophageal fistula. In addition, vascular rings and an aberrant innominate artery are also causes of secondary tracheomalacia. While classification is helpful in understanding the disease process, a successful aortopexy relies on the diagnosis of an obstructing innominate artery, regardless of the classification. The first description of the diagnosis and treatment of compression of the trachea due to an anomalous innominate artery was published over sixty years ago¹. Subsequently, tracheomalacia associated with tracheoesophageal fistula^2,3 has been added as an etiology of tracheomalacia treatable with aortopexy. The traditional approach to aortopexy has been via a thoracotomy incision, where access to the mediastinum is gained by incising the pleura, done either through the left or right chest. Multiple other approaches have been described in addition to the thoracotomy⁵, including thoracoscopic and partial sternotomy, with no approach having a clearly better outcome with respect to the others, although true comparison is difficult due to the rarity of the disease and the procedure. Due to its high natural resolution rate, breathing difficulties due to tracheomalacia should be treated surgically only in very select patients, after all non-operative options have been explored and/or exhausted. The recovery from the procedure itself is relatively rapid, and other than failure of the surgery to correct the respiratory compromise, long-term complications are few, but tend to be musculoskeletal in nature and more commonly associated with the thoracotomy approach⁶.

Equipment

A pediatric chest wall retractor is needed, as well as an infant bronchoscopy set.

Disclosures

The authors have no disclosures.

Statement of Consent

The patient referred to in this video article has given their informed consent to be filmed and is aware that information and images will be published on-line.