Pericardial patch tracheobronchoplasty for airway stenosis due to relapsing polychondritis

Abstract

Relapsing polychondritis is a rare autoimmune disorder characterized by recurrent inflammation in cartilaginous tissue, leading to progressive deformation and impaired function. Involvement of the laryngo-tracheobronchial tree is common and contributes significantly to the disease’s morbidity and mortality. Currently, medical management dominates, and bronchoscopic interventions offer limited satisfactory results. Notably, no surgical management approach for tracheobronchial tree involvement in relapsing polychondritis has been reported in the literature. In this paper, we present a case of sequential tracheoplasty and bilateral bronchoplasty as a novel surgical approach for managing relapsing polychondritis affecting the tracheobronchial tree.

Introduction

Relapsing polychondritis (RP) is a rare autoimmune disorder characterized by recurrent inflammation in cartilaginous tissue, leading to progressive deformation and impaired function. Involvement of the laryngo-tracheobronchial tree is common and contributes significantly to the disease’s morbidity and mortality. Currently, medical management dominates, and bronchoscopic interventions offer limited satisfactory results. Notably, no surgical management approach for tracheobronchial tree involvement in RP has been reported in the literature. In this paper, we present a case of sequential tracheoplasty and bilateral bronchoplasty as a novel surgical approach for managing RP affecting the tracheobronchial tree.

Case report

A 58-year-old female patient diagnosed with RP presented with progressive shortness of breath. The diagnosis of RP was confirmed based on the patient’s clinical presentation, imaging findings demonstrating cartilage involvement, including tracheobronchial tree, and laboratory results that supported an inflammatory etiology, while ruling out other differential diagnoses. Thorax computed tomography (CT) suggested a significant long segmental tracheal stenosis. Optimal medical support and several bronchoscopic dilatations failed to resolve the symptoms and she was referred to the department of thoracic surgery for a possible surgical intervention. On admission, further respiratory assessment could not be performed due to her general condition. Relying on bronchoscopic findings and her most recent CT scan (Fig. 1), we decided to perform surgical intervention.

Fig. 1.

Preoperative computed tomography scan (Numerous dilatations were performed; subclinical perforation might be present due to that reason.)

Among the various surgical techniques for the management of long segmental tracheal stenosis, patch tracheoplasty using autologous pericardium seemed the best option which was first reported by Idriss in 1984 [1]. In addition to the original technique, we decided to put a silicon stent peroperatively to secure a patent airway during the immediate postoperative course. On surgery, the anterior wall of the trachea was exposed from the cricoid cartilage to the carina via median sternotomy. After performing a median sternotomy, we mobilized the innominate vessels and opened the pericardium. The anterior trachea from the cricoid to the carina was then visible. A longitudinal cut was made along the stenotic segment including one inferior and superior ring to ensure total relief from obstruction. An appropriately sized raw autologous pericardium was freshly harvested. A “Y”-shaped tracheal stent was positioned in the lumen and the defect was repaired with pericardial patch using a continuous technique with 4/0 polypropylene suture along the tracheal edges with airway managed with controlled apnea, with intermittent ventilation resumed as needed to maintain adequate oxygenation. The patch was covered with mediastinal fatty tissue, some of which was used for the closure of the pericardial defect. The postoperative course was uneventful. The patient was discharged 12 days after the surgery. The stent was removed 6 months after the operation when adequate tracheal lumen was inspected on the control bronchoscopy. Moreover, autogenous pericardium is favorable as most patients do not require extended tracheal stenting. She was kept on routine follow-up without any respiratory problems.

She presented again with progressive shortness of breath 5 years after the surgery. Preoperative investigations including bronchoscopy revealed a patent tracheal lumen (Figs. 2A and 3), but bilateral segmental stenosis at both main bronchi (Fig. 2B, 2D). The excellent outcome 5 years after the initial operation led us to perform the same technique for bilateral main bronchi. Despite several attempts, it was not possible to document airway obstruction on spirometry preoperatively, since the patient could not cooperate. Therefore, the decision was mainly made with bronchoscopic findings. She underwent simultaneous bilateral pericardial patch bronchoplasty via anterior thoracotomy. The left main pulmonary artery was carefully dissected, and the ligamentum arteriosum was divided. By retracting the pulmonary artery superiorly, adequate exposure was achieved, allowing for sufficient exposure for a longitudinal incision and patch suturing and the technique described previously was undertaken for both sides. There was no concern for bulging of the patch during inspiration due to smaller dimensions. Thus, bronchial stenting was not performed.

Fig. 2.

A Trachea postoperatively. B Orifices of left and right main bronchi preoperatively. C Orifices of left and right main bronchi postoperatively. D Left main bronchi preoperatively, membranous parts bulging. E Left main bronchi postoperatively, patent lumen

Fig. 3.

Postoperative computed tomography scan

The postoperative course was uneventful, and she was discharged from the hospital 10 days after the surgery. A follow-up bronchoscopy was performed 2 years after her second operation when she was put on general anesthesia for laparoscopic cholecystectomy (Fig. 2C, E). She remains on clinical follow-up for 8 years following her initial operation.

Discussion

RP is a rare multi-system autoimmune disease characterized by the destruction of cartilaginous tissue. Matrilin-1 protein, a cartilage protein, is suspected in the pathogenesis of RP [2]. RP is mainly treated with medical therapy such as non-steroidal anti-inflammatory drugs, systemic corticosteroids, immunosuppressants, biologic agents, and monoclonal antibodies [3]. Laryngo-tracheobronchial involvement is usually not the initial symptom, but such symptoms develop in 20–50% of the patients [4]. Furthermore, airway involvement is one of the major causes of mortality and morbidity.

Management of airway involvement in RP is a challenging problem. In the reported series, bronchoscopic interventions including tracheotomy, dilatations, stents, and laser extirpations are only performed for the purpose of palliation. These procedures not only provide a limited benefit, but also may cause more complex airway problems. Surprisingly, none of the surgical procedures which can be undertaken for the management of long segmental stenosis has not been considered as a solution for this difficult problem [5]. For patients presenting with respiratory symptoms, we believe that it might be worthwhile to accept the risk of the surgery by taking into consideration the course of the disease in selected patients.

Even though slide tracheoplasty is an excellent technique for the management of long segmental stenosis, recurring inflammatory episodes continue progressively affecting the cartilages in RP and result in recurrent respiratory problems postoperatively. As a result, alternative surgical options, such as patch tracheoplasty using autogenous pericardium, have emerged as viable alternatives for the management of this condition. Patch tracheoplasty using autogenous pericardium has been used for many patients with acceptable outcomes since Idriss’ report in 1984. Number of reported cases revealed that airway mucosa covers the patch which provides a patent airway. This makes the patch less likely to be affected by recurring inflammation, thereby ensuring its long-term integrity. The use of the patient’s own tissue reduces the risk of complications such as rejection or infection, and therefore, this technique can be considered a safe and effective option for patients with long segmental tracheal stenosis. In this reported case, we speculated that the patch would not to be affected from the recurrent inflammation and remain intact in the long term.

Conclusion

Pericardial patch tracheobronchoplasty may be a reasonable option for the management of respiratory symptoms caused by RP and it may change its management in the future. Pulmonologists may consider directing these patients, whose symptoms do not resolve with optimal medical treatment, for possible surgical options. Long-term follow-up of this case suggests that the technique is not only beneficial for the quality of life, but also increases the survival rate in patients.

Author contribution

Yusuf Inanc: writing—original draft preparation.

Deniz Piyadeoglu: writing—original draft preparation.

Merih Kalamanoglu Balci: conceptualization.

Cemal Asim Kutlu: conceptualization, writing—review and editing.

Funding

None.

Declarations

Informed consent

Informed consent was collected from the patient.

Ethics committee approval

No IRB approval was deemed necessary as this is a case report.

Human and animal rights statement

Not applicable.

Conflict of interest

The authors have no conflicts of interest.