Near-complete tracheal ring deformity: a case report.

Abstract

Long-segment near-complete tracheal ring deformity is a rare condition with few documented cases. We present the case of a 7-week-old male with total anomalous pulmonary venous return and long-segment near-complete tracheal rings. We discuss the presentation, evaluation, and management of near-complete and complete tracheal rings.

Introduction

Complete and near-complete tracheal ring (NCTR) formations are rare anomalies first described in 1897. (1) They are often described in conjunction with tracheal stenosis and are rarely depicted as an isolated presentation. Faust et al were among the first to describe a case of long-segment NCTR that was associated with Pfeiffer syndrome. (1)

Most patients with tracheal abnormalities will present within the first 2 years of life with episodes of respiratory distress. (1) Other common symptoms include stridor, apnea, failure to thrive, cough, and tachypnea. (2) Some of these abnormalities maybe discovered after unsuccessful attempts at intubation or inability to achieve weaning from ventilation after surgery. (3) Furthermore, complete tracheal rings and NCTRs should be considered if an older child with asthma fails to respond to medical management. (4)

Case report

A 7-week old Hispanic male presented with episodes of respiratory distress beginning at birth. Immediate endotracheal intubation was determined to be necessary and was achieved uneventfully with a 3.5-mm endotracheal tube. Total anomalous pulmonary venous return was diagnosed, necessitating immediate cardiac surgery. The patient was placed on extracorporeal membrane oxygenation postoperatively until day of life 6 but was not extubated until day of life 11.

The patient did well extubated until 1 month of age, at which time he developed recurrent episodes of respiratory distress with sternal retraction, nasal flaring, oxygen desaturation, and little evidence of air movement. In addition, the patient was determined to be unable to take food or liquids orally because of his severe oromotor dysphagia and evidence of aspiration.

Physical examination revealed mild inspiratory stridor with oxygen saturation of 100% on room air. Flexible nasopharyngoscopy with a 2.7-mm scope demonstrated mild to moderate laryngomalacia, which prevented assessment of vocal fold mobility. Because of concerns about distal lesions in this complicated patient, he was taken to the operating room for direct laryngoscopy and bronchoscopy. Laryngoscopy demonstrated normal true vocal fold mobility and confirmed the previously visualized laryngomalacia. Bronchoscopy demonstrated a normal-caliber cricoid with NCTRs from the cricoid to the carina (figure).

Despite the abnormal shape, the tracheal diameter was within normal limits and the bronchi were normal in configuration and shape. The patient underwent a laparoscopic Nissen funoplication and gastrostomy tube placement secondary to aspiration and gastroesophageal reflux disease.

Postoperatively, the patient did well clinically, likely because of the gastrostomy tube placement and fundoplication. Despite the NCTRs, the absence of tracheal narrowing allowed for clinical observation of this patient.

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Melissa L. Somers, MD; Dana L. Suskind, MD

From the Department of Otolaryngology-Head and Neck Surgery, Henry Ford Health System, Detroit (Dr. Somers), and the Section of Otolaryngology-Head and Neck Surgery, Pritzker School of Medicine, University of Chicago (Dr. Suskind).

Corresponding author: Dr. Dana Suskind, Section of Otolaryngology-Head and Neck Surgery, University of Chicago, 5841 S. Maryland Ave., MC1035, Chicago, IL 60637. Phone: [phone]; fax: [phone]; e-mail: [e-mail]

Discussion

Tracheal development begins during the fourth week of life, when the respiratory diverticulum branches off of the foregut mesenchyme. (5) Evidence of tracheal cartilage development can be noted during weeks 9 to 10. (1) Holinger et al hypothesized that vascular compromise at this point may lead to abnormal formation of the trachea, with resulting stenosis. (6) The normal trachea is composed of G-shaped cartilage, with an average cartilage-to-posterior membrane ratio of 4.5:1. (6) With near-complete and complete tracheal ring formation, this ratio is increased as a result of the more complete O-shaped piece of cartilage.

Chen and Holinger have postulated that the altered ring shape may be caused by an overgrowth of the cartilage during week 10, with a resulting decrease in the amount of posterior membranacea. (7) The altered ring structure can cause the trachea to become less flexible, and often can cause the lumen diameter to be decreased. As a result, near-complete and complete tracheal rings are often the etiologies of tracheal stenosis.

Near-complete and complete tracheal rings have been shown to have an increased association with other anomalies. For example, there has been a documented increase in occurrence with cardiovascular abnormalities, including vascular slings and intracardiac lesions. (1) An increased incidence has also been observed with tracheoesophageal fistulas and esophageal atresia. (l) Some cases have been linked with genetic disorders such as Down syndrome, Pfeiffer syndrome, and Pierre Robin syndrome. (1,8)

Endoscopy is considered to be the gold standard for assessing tracheal abnormalities. (9) Contrast bronchography may also be used to assess the length of tracheal involvement if there is coexisting tracheal stenosis. (5) Echocardiogram or arteriography can be used to assess the possibility of concurrent cardiovascular anomalies.

Conservative medical management is chosen for children with complete tracheal rings who are asymptomatic or mildly symptomatic. Our patient was managed conservatively because of his mild symptoms, which were likely attributable to laryngomalacia. Rutter et al estimated that 10% of patients could be managed medically with regular endoscopic follow-up. (10)

For those patients whose respiratory distress is more severe, two surgical approaches can be used to correct the trachea: endoscopic and open. Endoscopic options include dilation, stenting, and laser therapy. (9) Othersen et al described a procedure combining laser and balloon dilation to assist with the treatment of complete tracheal rings.(11) Under direct vision with an endoscope, they were able to make an incision in the posterior midline of the rings using a KTP laser. This was combined with balloon dilation and postoperative endotracheal stenting. A layer developed in the divided posterior region that was similar to the absent posterior membranacea.

Open surgical approaches are usually reserved for patients with coexisting tracheal stenosis. Resection with end-to-end anastomosis is used for short-segment stenosis. (9) The reconstruction surgeries that can be performed on long-segment stenosis are slide tracheoplasty, (12,13) pericardial patch tracheoplasty, (14) and costal cartilage tracheoplasty. (15)

Conclusion

Near-complete and complete tracheal rings are rare anomalies that form during weeks 9 to 10 of development and often cause congenital tracheal stenosis. Patients usually present in the first few years of life with respiratory distress and stridor. There is a remarkable increased association between NCTRs and cardiovascular, esophageal, and genetic abnormalities.

Endoscopy is the gold standard for diagnosis of near-complete and complete tracheal rings. Ten percent of the patients can be managed medically, with surgical correction typically being reserved for patients with concurrent stenosis. Surgical approaches range from dilation and stenting to resection and reconstructive surgery.

References

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