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Case presentation

A 56-year-old Hispanic male with a previous history of extra-laryngeal tuberculosis requiring tracheostomy, presented for evaluation for decannulation. Over the past several months, the patient had noted increasing difficulty breathing from his nasal air passage when occluding his tracheostomy. He did not note any difficulty swallowing solids or liquids. Fiber optic examination in the office revealed an inability to pass the fiber optic laryngoscope through the nasal cavity into the larynx due to presumed scarring in the nasopharyngeal region as well as persistent pharyngoepiglottic scarring and epiglottic granulation tissue. After a thorough discussion with the patient, decision was made to proceed to the operating room for a direct laryngoscopy with possible intervention to recanalize the nasopharyngeal airway and lyse laryngeal scar tissue.

Direct laryngoscopy in the operating room revealed fusion of the soft palate with the posterior pharyngeal wall occluding the nasopharyngeal airway ( Fig. 1 ). The oropharyngeal airway, despite the presence of fibrous tissue, was noted to be patent. While probing the extensive scar tissue, one small opening was appreciated providing a communication from the nasopharynx to the oropharynx, and a clear tissue plane of scar separation.

Complete nasopharyngeal stenosis with pin-point opening into the oropharynx.

The posterior extension of the soft palate was divided from the posterior pharyngeal wall with sharp dissection. Following release of this tissue, the nasopharyngeal air passage was noted to regain patency. A substantial amount of fibrous tissue was resected from the pharyngeal wall, followed by debridement of the anterior and posterior tonsillar pillars to demucosalize the mucosal surfaces.

The posterior aspect of the soft palate was then denuded of mucosa on the oral surface, and plicated onto itself to appose the raw mucosal surfaces ( Fig. 2 ). After the palatoplasty was completed, the anterior and posterior tonsillar pillars were sutured together to complete the modified palatopharyngoplasty. At the completion of the procedure, the nasopharyngeal airway was patent and continuous with the oropharyngeal airway. One nasal trumpet was placed to prevent restenosis during the immediate post-operative period ( Fig. 3 ).

During intervention, the pin-point opening previously noted was enlarged, revealing a pathway between the nasopharynx and oropharynx. As shown in the above photo, mucosa from the soft palate, posterior pharyngeal wall, and tonsillar pillars was denuded.

Following recanalization of the nasopharynx with modified palatopharyngoplasty, a nasal trumpet was placed to act as a stent.

In the post-anesthesia care unit, the patient was able to breathe comfortably through his nasal airway. Post-operatively, the patient did well without any complications. At one month following surgery, the patient was safely decannulated in the clinic and noted to have a well-healed surgical site without evidence of restenosis ( Fig. 4 ). Three months following surgery, the patient had a well-healed tracheostomy stoma and continued to breathe comfortably through his nasal airway. He was phonating well with return of his pre-tracheostomy voice, and had no evidence of nasal regurgitation.

Well healed surgical site during the post-operative follow-up without evidence of restenosis.

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Discussion

Nasopharyngeal stenosis (NPS) is an infrequently encountered entity characterized by obliteration of the natural pathway between the nasopharynx and the oropharynx. Obstruction ranges from a thin membranous diaphragm to a mass of dense fibrotic tissue obliterating the nasopharynx. Historically, it was caused by a host of infectious agents such as tuberculosis (TB), syphilis, diphtheria, and rhinoscleroma; however, since the advent of antibiotics, the etiology is predominantly iatrogenic . Overresection of mucosa during surgery of the naso/oropharynx, particularly uvulopalatopharyngoplasty, and irradiation of nasopharyngeal tumors comprise the majority of current day cases. Rarely, granulomatous disease such as sarcoidosis, or the autoimmune disease cicatricial pemphigoid, has been found to be causative. Ultimately, it is inflammation of the opposing pharyngeal soft tissues that gives way to cicatricial scar formation and fusion of soft palate and tonsillar pillars to the posterior pharyngeal wall .

Tuberculous pharyngitis, which may affect the nasopharynx, oropharynx, and hypopharynx, is rarely seen today. The most common manifestation of TB in the head and neck is cervical lymphadenopathy (95%) followed by laryngeal TB (2%). Other head and neck sub-sites such as the middle ear, pharynx, and sinonasal tract, are less frequently affected . Sore throat, dysphagia, odynophagia, and non-healing ulcerations are reported by patients more commonly than systemic symptoms such as weight loss, fever, or night sweats . The most important diagnostic tool is histopathologic analysis, showing caseating granulomas on light microscopy and positive acid fast staining. There are no studies on the optimal treatment of pharyngeal tuberculosis; however, the CDC recommends the same treatment principles as those for pulmonary TB, typically centered on a 4-drug regimen of 6–9 months duration. Concomitant surgical intervention is sometimes required due to abscess formation, fistula formation, or luminal obstruction, as was the case in the present case report .

Acquired NPS is associated with the morbidities of nasal obstruction, sleep apnea, hyponasal speech, dysphagia, rhinorrhea, and Eustachian tube dysfunction. Unfortunately, the treatment of NPS is plagued by high recurrence rates and the frequent need for revision surgeries leading to a long and varied list of proposed treatment schemes. Reported treatments include cold knife excision, laser excision, skin grafts, local flaps, regional flaps, microvascular free flaps, nasal stenting, balloon dilation, intralesional injections, and combinations thereof . Comparative studies are nonexistent and clinical series are usually limited to just a few patients.

The choice between methods is influenced by many factors including the severity of the stenosis, the quality of the surrounding mucosa, the surgeon’s experience, and the sequelae of the individual methods. Techniques vary considerably with respect to the manner in which epithelized lining is provided for reconstruction. Skin grafts in this region are technically difficult to secure with pressure dressings, leading to poor graft take, and are prone to contracture and therefore recurrent stenosis. Regional flaps and free flaps may be necessary to provide adequate epithelial coverage in cases of severe stenosis; however, the increased donor site morbidity and unwanted bulk limits their application . Local flaps, which are based off either the soft palate or pharyngeal walls, are relatively simple to raise and have minimal donor site morbidity, making them a primary means of providing epithelial coverage after scar excision.

In cases of NPS arising from a suspected inflammatory condition of the pharynx, as in the present case, the soft palate may offer a greater degree of healthy tissue for transposition than the fibrosed pharyngeal wall. Several soft palatal flaps have previously been described for the treatment of NPS . The present case report introduces the novel application of a palatal flap originally described in the context of uvulopalatopharyngoplasty (UPPP) known as the extended uvulopalatal flap (EUPF) . This flap involves stripping the mucosa and adipose tissue off the posterior aspect of the oral soft palate and making releasing incisions above the bilateral tonsillar fossa to permit imbrication of the denuded flap to the proximal soft palate. The suture closure of the tonsillar fossa, as routinely preformed in UPPP, enlarges the velopharyngeal inlet laterally, while the resection and elevation the inferior margin of the uvulopalatal complex by way of EUPF increases the anteroposterior dimension. Of most particular importance in this technique is the preserved mucosal edge of the soft palate, which may serve to decrease the chance of re-stenosis.

Our case demonstrates the successful initial management of acquired NPS in the setting of TB using the tenets of UPPP with the EUPF modification. Pharyngeal tuberculosis resulting in NPS is exceedingly rare in the modern era, and the current case serves to remind practitioners of the diverse otolaryngologic manifestations of TB and the need to maintain a level of clinical suspicion to ensure prompt diagnosis and to limit unnecessary exposures. The surgical treatment of NPS remains a challenge considering the notable absence of a standardized treatment scheme. The modified palatopharyngoplasty represents a novel method in the armamentarium of the surgeon managing acquired NPS.