Ann Thorac Surg 2005;79:1676-1681
© 2005 The Society of Thoracic Surgeons
Original articles: General thoracic
Airway Obstruction Owing to Tracheopathia Osteoplastica: Treatment by Linear Tracheoplasty
Hermes C. Grillo, MD*,
Cameron D. Wright, MD
Thoracic Surgical Division, the Surgical Services, Massachusetts General Hospital, and the Department of Surgery, Harvard Medical School, Boston, Massachusetts
Accepted for publication October 12, 2004.
* Address reprint requests to Dr Grillo, Massachusetts General Hospital, Blake 1570, 55 Fruit St, Boston, MA 02114 (E-mail: pguerriero{at}partners.org).
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Abstract
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BACKGROUND: Tracheopathia osteoplastica is a rare disease that may involve the entire trachea and progress to critical airway obstruction. It is not dilatable and does not respond to laser therapy or bronchoscopic curettage. Stents usually cannot be inserted.
METHODS: Lack of involvement of the membranous wall by the disease allows tracheal widening after complete linear tracheoplasty. Opening is preserved during healing by prolonged stenting with a T or T-Y silicone tube.
RESULTS: Stent removal after firm healing produced long-term correction of stenosis in 3 of 4 patients, examined up to 12 years.
CONCLUSIONS: Severe, symptomatic tracheal obstruction by tracheopathia osteoplastica is definitively surgically correctible.
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Introduction
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Tracheopathia osteoplastica (TPO), or tracheobronchopathia osteochondroplastica, presents with submucosal nodules of hyaline cartilage and areas of lamellar bone overlying the cartilaginous tracheal rings, but sparing the membranous wall. Bony, cartilaginous, and fibrous tissues interconnect the osteocartilaginous nodules and the underlying tracheal rings to form a semicircular submucosal endotracheal structure. Pathologic findings were detailed by Young and associates [1]. The process usually involves the subglottic larynx, the trachea, and the main bronchi. Involvement varies from a cobblestone appearance caused by multiple small nodules to large, erratically located nodules. These may cause obstruction, especially because the trachea usually has a saber sheath cross-section in this disease. The degree of obstruction may vary from one part of the trachea to another. Less often, the disease may involve only a limited segment of the trachea when identified.
Tracheopathia osteoplastica has been described more often after 50 years of age, although this may be related to the number of cases discovered only at autopsy [1]. It seems to occur quite evenly in men and in women. Although often quite asymptomatic, it sometimes does produce wheezing, stridor, dyspnea (initially on exertion), cough, difficulty in raising viscous secretions, tracheobronchitis, pneumonia, and hemoptysis [2, 3]. In some patients, it is progressive and leads to severe airway obstruction. Nonspecificity of symptoms often results in earlier misdiagnosis. Difficulty in intubation may lead to its discovery. Appearance on bronchoscopy and on computed tomographic scan is diagnostic if the examiner is aware of this relatively rare condition (Figs 1, 2).
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Fig 1. Preoperative bronchoscopic observations. (A) Patient 1. Note narrowing as a result of tracheopathia osteoplastica nodules and saber sheath deformity. The membranous wall is unaffected. (B) Patient 3. Large and small nodules, distorted lumen.
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Fig 2. Tracheopathia osteoplastica. (A) Patient 1. Preoperative posteroanterior chest roentgenogram. Note marked narrowing of the trachea. Clips and sutures are from previous coronary artery surgery. (B) Patient 3. Three-dimensional computed tomographic reconstruction of the trachea, showing extreme deformity. (C) Patient 3. Computed tomographic scan of trachea. The nodularity, calcification, extreme saber sheath deformity, and narrowed lumen are diagnostic of tracheopathia osteoplastica.
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Few patients have symptoms severe enough to require surgical relief. We report 4 patients whose disease became severe enough to require mechanical amelioration. Dilation is usually impossible, so rigid is the "endoskeleton" within the saber sheath trachea. Indeed, passage of a rigid bronchoscope may be next to impossible. Bronchoscopic placement of stents is hence impossible. Effective bronchoscopic rongeuring of nodules is difficult and may be impossible. Laser treatment has been unsuccessful [4]. A laryngeal mask airway may be required to administer inhalation anesthesia effectively if satisfactory intubation is not possible.
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Patients and Methods
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Patients
Four patients, 3 men and 1 woman, 30 to 49 years of age, with histories of respiratory symptoms extending from several months to 20 years, presented with severe dyspnea on any effort, stridor, cough, bouts of pneumonia, viscid secretions that were difficult to raise, and choking spells. Details are listed in Table 1. These 4 patients had reached a point at which intervention was wholly necessary.
Abundant Pseudomonas aeruginosa was identified in the sputum of these patients, and other organisms such as Klebsiella ozaenae, Escherichia coli, and Enterobacter aerogenes were also reported. They received perioperative and postoperative antibiotics and were discharged on courses of antibiotics.
In 1 additional patient TPO was limited to the upper 4.5 cm of the trachea. Operation in this case was not for obstruction, but for removal of a focus of cytologically demonstrated and confirmed squamous cell carcinoma identified in the tracheal segment involved by TPO. This patient is noted in this report only to record an unusual presentation and its management in this rare condition.
Patients 1 and 3 were previously presented in clinicopathologic conferences [5, 6].
Operative Technique
In the first patient (Table 1, No 1) complete anterior vertical linear tracheotomy was performed from just below the cricoid cartilage to the carina, with the intent to introduce a long T or T-Y tube (Fig 3A), and, if necessary, to seal any resulting anterior gap in the tracheal wall with pedicled cervical strap muscles. Because the membranous wall of the trachea is uninvolved by the process, vertical anterior tracheal division allowed the two halves of the rigid cartilaginous walls to hinge outward from the anterior midline point of division laterally and posteriorly, stretching the membranous wall behind the splinting T tube. The anterior wall could then be resutured to itself over the T or T-Y tube (Fig 3B) [5, 6].
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Fig 3. Operative correction of obstructive tracheopathia osteoplastica. (A) The trachea is opened from the cricoid to the carina (dashed line), or also over the main bronchi (dotted line) if required. Exposure is described in text. (B) A previously prepared T or T-Y silicone rubber tube is placed in the lumen of the airway, and the tracheotomy (and any bronchotomies) are sutured closed. (C) The tracheal incisions are buttressed with pedicled sternohyoid muscles below the T sidearm and with sutured sternothyroid muscles above.
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For patients 1 and 4, individually designed silicone rubber T-Y tubes were prepared in advance (Hood Company, Pembroke, MA) to (1) provide sufficient length of vertical limb above the T sidearm to allow passage through the vocal cords if necessary; (2) allow the T sidearm to emerge at a predetermined location from the cervical trachea (approximately the second and third rings); (3) locate the carinal Y at an appropriate distance from the stoma; and (4) construct right and left main bronchial limbs at appropriate angles for the particular carina and of sufficient length. All lengths of T and Y elements could be shortened in the operating room as required and the ends sanded smooth. Templates for the T-Y tubes were drawn from linear tomograms of the trachea and from prior bronchoscopic measurements. Generally, two 12- and 14-mm-diameter tubes each were prepared, in case there should be intraoperative difficulty in fitting them. T tubes were of extralong design (Hood Company) to allow precise fit with respect to the desired stomal location and carinal configuration.
Approach is by means of a low collar incision, plus complete median sternotomy. The thyroid isthmus is divided in the midline and the thymus also. The pretracheal plane is dissected to the carina. In patients 1, 3, and 4, transpericardial exposure of the lower trachea was obtained between the superior vena cava and aortic arch, behind the brachiocephalic vein and artery. The trachea is incised vertically in the midline from just below the cricoid to the carina. A small divot of tracheal wall is removed at the point where the sidearm of the T tube will emerge, because of tracheal rigidity and also for pathologic examination. Stout scissors as well as scalpel may be needed. A T-Y tube (14 mm diameter) was used in patients 1, 2, and 4, and a T tube (14 mm) was used in patient 3. In patient 4, both main bronchi were also opened longitudinally because of severe nodular obstruction therein [6]. Numerous large critical nodules in trachea and bronchi were removed with a pituitary rongeur, markedly improving the lumen. In this patient the distal left main bronchus was accessed through a pericardiotomy lateral to the aortic arch, with division of the ligamentum arteriosum, carefully avoiding the left recurrent laryngeal nerve. The pleura remained intact. During various parts of the dissection, ventilation is managed across the operative field by direct placement of an endotracheal tube through the linear tracheotomy into the distal trachea or into the left main bronchus (or in patient 4 during left main bronchial debridement, in the right main bronchus).
We prefer 4-0 Vicryl sutures (Ethicon, Somerville, NJ), interrupted, for closure. All sutures are placed before inserting a T or T-Y tube. Ventilation is then administered by connection to the T sidearm, with care to avoid kinking of the flexible silicone sidearm. A Fogarty or Pruitt catheter introduced through the sidearm is used to occlude the proximal vertical arm of the T tube during ventilation.
The tracheal and bronchial walls approximate in the midline without difficulty as the sutures are tied and the membranous wall widens. The suture lines are tested for leak under saline. Suture lines were buttressed in all patients. In patients 1, 3, and 4, the left sternohyoid muscle was divided at the hyoid bone, brought beneath the brachiocephalic vessels, and pedicled over the lower tracheal suture line. The right sternohyoid muscle was also pedicled and passed beneath the vessels, to cover the tracheal incision below the stoma but above the left sternohyoid muscle. The sternothyroid muscles were approximated in the midline to cover the tracheal incision above the stoma (Fig 3C). All these muscles are also sutured to the tracheal wall. The two lobes of the thymus were pedicled to cover suture lines in patient 3. The pedicled left thymic lobe was used to cover the left main bronchus in patient 4, sealing a slight air leak. Steps in the correction are diagrammed in cross-section in Figure 4. A fifth patient with segmental disease underwent a standard 4.5-cm upper tracheal resection, including the lower border of cricoid cartilage, with primary anastomosis.
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Fig 4. Cross-sectional diagrams of the correction. (A) The initial configuration of the lumen. Dashed line indicates location of longitudinal tracheal incision. (B) Anterior incision allows the side walls to hinge open on the uninvolved membranous tracheal wall. (C) The rigid walls are sutured together anteriorly, stretching the membranous wall posteriorly, over the T tube. (D) After firm healing the T tube is removed, leaving a permanently enlarged lumen.
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Subsequent Management
T and T-Y tubes were removed after intervals believed to be long enough to allow firm healing of the midline in an open-book position (Fig 4D). A 6-month interval was arbitrarily planned, but actual periods depended on social needs of each patient. The actual intervals were, respectively in cases 1 to 4, 7 months, 6 months, 7.5 months, and 11 months. Patient 1 required later revision of tracheostomy with placement of a custom-made T-Y tube, which passed through the vocal cords. After removal of the T tubes, a small tracheostomy tube (No 3) was placed in patients 1, 2, and 4, and a Montgomery tracheal cannula in patient 3, for security, for varying lengths of time (chiefly because of patients' geographical locations at some distance from Boston). About a month seemed adequate to observe for possible postoperative stenosis.
During the period of T tube splinting, thick secretions continued to require variable attention, including suctioning, bronchoscopy, and antibiotics. After removal of the T tube, the patients were generally able to clear their now reduced secretions by cough alone.
One patient had a granuloma at the stomal site removed bronchoscopically 10 months postoperatively, and also needed a brief course of antibiotics for tracheitis 2.5 years after operation. Neither recurred.
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Results
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Respiratory obstruction was relieved, and patients were able to return to full activities. Patient 1 exhibits mild dyspnea on significant exertion but leads an otherwise normal life. Patients 3 and 4 are asymptomatic even on exercise (Table 1). Patient 1 has maintained this relief for 12 years and underwent repeat coronary artery bypass grafting successfully 3 years after tracheal surgery. Patients were followed clinically and by bronchoscopy (Fig 5) performed either at Massachusetts General Hospital or by referring pulmonologists. Six interval bronchoscopies in patient 1 (the last 3.5 years ago) showed a stable and functional airway. Patient 3, who is now 6 years after the operation, has returned to hunting in hilly country outside of Lisbon. Bronchoscopy 1.5 years after surgery showed a very satisfactory airway. Patient 4 has a good airway 3.5 years after reconstruction. The locations where nodules had been rongeured reepithelized well, and no obstructive nodules reformed. Peak expiratory and inspiratory flow rates increased in patients 1 and 3 (Table 1). Flow volume curves showed improvement (Fig 6). Computed tomographic scans confirm the repositioning of the nodular lateral walls in an open-book position (Fig 7). The lumen is markedly improved, but the lack of curvature of the walls owing to the saber sheath deformity prevents even wider opening.
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Fig 6. Flow–volume curves, preoperative (dotted lines) and postoperative (solid lines) in patient 3 (A) and patient 4 (B). Inspiratory loops were not determined satisfactorily in patient 4.
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Patient 2 failed, appearing with tracheal stenosis 1.5 years after operation. He managed to lead a satisfactory life with a T tube until last contact, 4 years after surgery. Social and economic factors precluded his return from California for possible resection or revision of a limited stenotic tracheal segment. He then returned to Mexico and could not be traced.
A fifth patient, resected for neoplasm, has remained asymptomatic and cancer-free for 8 years. His most recent bronchoscopy was in July 2001.
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Comment
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Most patients with TPO seem to have no symptoms or only moderately troublesome symptoms. Many reported cases were found at autopsy [1]. Many others probably go unidentified, either as a result of lack of symptoms and failure of diagnosis because of nonspecific symptoms or as a result of physicians' failure to recognize this rare condition. The disease is well described clinically, pathologically, and radiologically, but its origin remains unknown. Prakash and colleagues [4] described familial occurrence in a mother and daughter. Computed tomographic scans of the mother and son of patient 1 failed to show disease. We detected no suggestion of familial disease historically in our other patients.
The rare situation of incapacitating and life-endangering tracheal obstruction caused by TPO, which is not responsive to dilation and hence impossible to stent or to endobronchial clearing by curettage or laser therapy, can now be successfully relieved by linear anterior tracheoplasty. Subsequent tracheal remodeling is ensured by prolonged stenting, but with ultimate freedom from any appliance. The airway correction obtained has been long lasting, and probably is permanent. As expected, some mucosal secretions continue to form, but are usually cleared by cough.
Surgical excision of segmental disease as described in our fifth case was reported previously by Besso and colleagues [7] for TPO obstruction of upper trachea in conjunction with goiter in 1972, and by O'Reilly and Marty [8] in 1978. Kutlu and colleagues [9] adapted modified lateral slide tracheoplasty to segmental TPO in 1 patient.
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References
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- Young RH, Sandstrom RE, Mark GJ. Tracheopathia osteoplasticaClinical, radiologic, and pathological correlations. J Thorac Cardiovasc Surg 1980;79:537-541.[Abstract]
- Nienhuis DMJ, Prakash UBS, Edell ES. Tracheobronchopathia osteochondroplastica Ann Otol Rhinol Laryngol 1990;99:689-694.[Medline]
- Grillo HC. Infectious, inflammatory, infiltrative, idiopathic and miscellaneous tracheal lesionsIn: Grillo HC, editor. Surgery of the trachea and bronchi. Hamilton, Ontario: BC Decker; 2004. pp. 363-395.
- Prakash UBS, McCullough AE, Edell ES, Nienhuis DM. Tracheopathia osteoplastica: familial occurrence Mayo Clin Proc 1989;64:1091-1096.[Medline]
- [No authors listed] Case records of the Massachusetts General Hospital Weekly clinicopathological exercisesCase 46-1992. A 48-year-old woman with a narrowed trachea. N Engl J Med 1992;327:1512-1518.[Medline]
- [No authors listed] Case records of the Massachusetts General Hospital Weekly clinicopathological exercisesCase 32-1999. A 44-year-old man with tracheal narrowing and respiratory stridor. N Engl J Med 1999;341:1292-1299.[Free Full Text]
- Besso J-C, Eschapasse H, Bullinelli M, et al. Trachéopathie ostéoplastique à forme sténosante et asphyxiante opérée J Franc Med Chir Thor 1972;26:291-304.
- O'Reilly RR, Marty AT. Tracheopathia osteoplastica: case report Milit Med 1978;143:497-498.
- Kutlu CA, Yeginsu A, Ozalp T, Baran R. Modified slide tracheoplasty for the management of tracheobronchopathia osteochondroplastica Eur J Cardiothorac Surg 2002;21:140-142.[Abstract/Free Full Text]
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Abstract
Introduction
