Introduction
Total rhinectomy is a surgical procedure exclusively reserved for locally advanced, aggressive or recurrent malignancies of the nasal framework that are not amenable to limited resection or radiotherapy.Reference Stanley and Olsen1 The removal of the vast majority of the nasal framework and related soft tissues not only has disfiguring consequences, but also interferes with normal nasal function. Nevertheless, the functional sequelae of total rhinectomy are not well known.Reference Becker, Becker, Dahlem, Offergeld and Pfeiffer2
For years, uvulopalatopharyngoplasty (UPPP) was considered the standard surgical treatment for sleep-disordered breathing disorders, including obstructive sleep apnoea (OSA).Reference Fujita, Conway, Zorick and Roth3 However, the procedure has been frequently questioned for its limited effectiveness,Reference Caples, Rowley, Prinsell, Pallanch, Elamin and Katz4 serious complications,Reference Kezirian, Weaver, Yueh, Deyo, Khuri and Daley5 and long-term side effects, including dysphagia, globus sensation, velopharyngeal insufficiency and voice changes.Reference Tang, Salapatas, Bonzelaar and Friedman6 Thus, with the emergence of less invasive techniques that reposition rather than excise upper airway structures,Reference Vicini, Hendawy, Campanini, Eesa, Bahgat and AlGhamdi7–Reference Li and Lee9 the use of UPPP surgery has progressively declined in patients with OSA.
This case report presents the application of UPPP for the treatment of nasal blockage as a long-term side effect of total rhinectomy.
Case report
In April 1997, a 62-year-old male with no significant medical history underwent total rhinectomy for a nasal schwannoma at the level of the septum and right nostril without distant metastasis. This lifesaving surgical procedure consisted of a bilateral Weber–Ferguson approachReference Weber10,Reference Ferguson11 and complete resection of the nasal septum. Bone-anchored fixation screws were inserted for prosthetic rehabilitation.
Pathological investigation revealed a moderately differentiated peripheral schwannoma, grade II according to the grading system proposed by Coindre and colleagues,Reference Coindre, Trojani, Contesso, David, Rouesse and Bui12 with measurements of 1.8 × 1.1 × 1.2 cm. The excision was performed with free margins. Approximately five months after the procedure, the inferior nasal conchae were partially resected because of manifest hyperplasia of the nasal mucosa. The final result was both functionally and aesthetically satisfactory.
Twenty-one years later, from October 2018 onwards, the patient consulted our department with complaints of nasal blockage. On further enquiry, the patient stated that he used the nasal prothesis constantly during the daytime and at night-time without any issues. As clinical examination showed no major anomalies, conservative treatment with aerosol, nasal irrigation and ointments was initiated. However, during the following weeks, his symptoms worsened; he suffered from both diurnal and nocturnal dyspnoea, leading to dysphagia, insomnia and a general decline in physical activity. As his complaints mostly occurred during the day, no polysomnography was performed. Fibre-optic laryngoscopy revealed adhesion of the soft palate to the pharyngeal wall, causing significant narrowing at this level (Figure 1a). Computed tomography showed no causal anomalies. In May 2019, after a thorough discussion with the patient and his family, we decided to perform UPPP surgery in order to enlarge the retropalatal airway.Reference Fujita, Conway, Zorick and Roth3
The first step of the procedure consisted of bilateral tonsillectomy using cold instruments. Subsequently, both the anterior and posterior arches of the soft palate were extensively trimmed while preserving the muscular layer. The uvula was excised as well. Then, patency of the retropalatal airway was verified by inspecting the passage of light from the oropharynx to the nasopharynx. Finally, the mucosal edges of the anterior and posterior arches were reapproximated with interrupted resorbable sutures. Additionally, the upper airway was expanded in the lateral dimension by suturing both tonsillar pillars (Figure 1b).
The patient was discharged home the next day in a general good condition. Importantly, nasal blockage disappeared shortly after the procedure, leading to a significant improvement in quality of life for the patient. One month after surgery, we noted a well-healed surgical site and a patent nasopharyngeal airway (Figure 1c). Presently, one year after the procedure, the patient is satisfied with the results, despite experiencing limited nasal regurgitation of liquids and mucus production. The Nasal Obstruction Symptom Evaluation (‘NOSE’) surveyReference Stewart, Witsell, Smith, Weaver, Yueh and Hannley13 objectified the symptomatic improvement, with a pre-operative score of 19 out of 20, compared to 7 out of 20 one year post-operatively.
Discussion
A search of the literature was conducted in August 2019 using the PubMed search engine, with the following search terms: uvulopalatopharyngoplasty, pharyngoplasty, rhinectomy, velopharynx, nasal prosthesis and Ferguson Weber. Articles were selected based on title and abstract; in addition, the references of selected articles were screened.
As total rhinectomy is an uncommon procedure, only a few case series have been published on this topic.Reference Stanley and Olsen1,Reference Harrison14–Reference Becker, Kayser and Pfeiffer18 Most of these studies addressed surgical outcome in terms of survival and recurrence (Table 1). The largest cohort to date was described by Stanley and Olsen, who performed total rhinectomy in 51 patients.Reference Stanley and Olsen1 Squamous and basal cell carcinoma accounted for the majority (86 per cent) of the malignancies. Similar to other studies, total rhinectomy often served as an end-stage procedure following (multiple) unsuccessful attempts of more limited surgical excision.
Reconstruction of the surgical defect after total rhinectomy is an essential aspect of care. Both surgical reconstruction and a nasal prosthesis can be used for this purpose.Reference Miller19,Reference Mendelson, Masson, Arnold and Erich20 A nasal prosthesis is the cornerstone of rehabilitation, as autologous reconstruction of large defects is technically challenging and often unsatisfactory.Reference Stanley and Olsen1 The prothesis is made of medical grade silicon and is individually tailored to fit the nasal area. Osseous integrated magnetic abutments keep the prosthesis in place. The major advantage of a prosthesis is that it allows early rehabilitation, as well as regular inspection of the former tumour site for recurrence.
Obviously, however, a nasal prosthesis cannot replace normal nasal function. Becker et al. investigated the functional and aesthetic outcomes in patients with a nasal prosthesis.Reference Becker, Becker, Dahlem, Offergeld and Pfeiffer2 They found that patients were most satisfied about the appearance, overall function and fit of the prosthesis. However, there was wide variability in terms of the outcomes of breathing and smelling, possibly related to the extent of tumour excision. As the turbinates in particular affect nasal airflow and heat exchange, resection of these structures may lead to symptoms reminiscent of empty nose syndrome, such as crusting, dryness and blockage.Reference Becker, Becker, Dahlem, Offergeld and Pfeiffer2
Twenty-one years after total rhinectomy, our patient experienced progressive breathing difficulties without any local anomalies of the nasal cavity. This clinical report discusses the treatment of this long-term sequela using UPPP. The procedure remedied the retropalatal narrowing as observed endoscopically, and significantly improved nasal breathing. Thus, alterations in nasal heating, humidification and turbulence may not only disrupt normal nasal functions, but may also change the configuration of upper airway structures. The observed retropalatal narrowing is presumably due to persistent mucosal dryness, leading eventually to fixed adhesions between the soft palate and pharyngeal wall.
• Total rhinectomy is an uncommon procedure that is exclusively reserved for locally advanced, aggressive or recurrent malignancies of the nasal framework
• Total rhinectomy impairs the intranasal humidification and heating of inspired air
• Altered nasal function may cause mucosal dryness, pharyngeal structure adhesion, and ultimately upper airway narrowing or obstruction
• Use of uvulopalatopharyngoplasty for obstructive sleep apnoea has diminished since the emergence of less invasive procedures
• Uvulopalatopharyngoplasty may be indicated to treat selected patients with daytime breathing difficulties associated with velopharyngeal narrowing
Uvulopalatopharyngoplasty may prove more effective for this purpose than other, less invasive palatal techniques for OSA.Reference Vicini, Hendawy, Campanini, Eesa, Bahgat and AlGhamdi7–Reference Li and Lee9 Uvulopalatopharyngoplasty is an ablative technique and creates an optimal expansion of the retropalatal space in the anteroposterior direction.Reference Fujita, Conway, Zorick and Roth3 Thus, although it does not alleviate the causal mechanism of mucosal dryness, UPPP may be beneficial for these patients by restoring upper airway patency.
This application of UPPP may not be limited to velopharyngeal narrowing after total rhinectomy alone. Cranford et al. described a 56-year-old tracheostomised Hispanic male with extensive nasopharyngeal and pharyngoepiglottic scarring secondary to extra-laryngeal tuberculosis.Reference Cranford, Kadakia and Berzofsky21 This impaired decannulation, as occluding the stoma resulted in severe nasal breathing difficulties. After UPPP, the nasopharyngeal airway became patent again, leading to an uneventful decannulation.Reference Cranford, Kadakia and Berzofsky21 Hence, UPPP appears to be a valid option for people with retropalatal narrowing and breathing difficulties. As such, UPPP should at least be discussed within the multidisciplinary team as a potential option in these specific cases.
Conclusion
Total rhinectomy is an invasive procedure that impairs the intranasal humidification and heating of inspired air. This may cause mucosal dryness, adhesions between pharyngeal structures, and ultimately upper airway narrowing or obstruction. The use of UPPP for OSA has diminished over the past years as a result of the emergence of less invasive procedures. According to this case report, however, UPPP should be kept in the armamentarium of ENT surgeons to treat selected patients with daytime breathing difficulties associated with velopharyngeal narrowing.
Competing interests
None declared