- Email: [email protected]
Concha bullosa mucocele with invasion of the orbit
650
Otolaryngology– Head and Neck Surgery November 1999
ARMENGOT et al
Concha bullosa mucocele with invasion of the orbit MIGUEL ARMENGOT, MD, PhD, NURIA RUIZ, MD, CARMEN CARDA, MD, PhD, PACO HOSTALET, MD, and JORGE BASTERRA, MD, PhD,
Valencia, Spain
W
e describe the case of a patient with a large concha bullosa mucocele invading the orbit. The wall of the mucocele was lined by a well-preserved ciliary epithelium with a ciliary density similar to that of the healthy sinus mucosa. Ciliary ultrastructure was normal. Endonasal microsurgery yielded good results, and no recurrence has been observed after 18 months. The thickness of the middle turbinate is variable and often exhibits vacuolar structures. When pneumatized, this gives origin to a so-called concha bullosa.1 This lesion was first described by Zuckerlandl in 1893.2 For a number of years concha bullosa has been related to the pathogenesis of inflammatory disorders of the paranasal sinuses. Few cases have been reported dealing with concha bullosa as a cause of disease. In the past, a case of aspergilloma was reported within a concha bullosa3; however, a triple-index bibliographic search of the past 3 years yielded only a single case of a concha bullosa mucopyocele.4 This study reports a patient with a giant mucocele within a concha bullosa that destroyed the anterior ethmoid bone and invaded the orbit.
From the Services of Otolaryngology (Drs. Armengot, Ruiz, and Basterra) and Pathology (Drs. Carda and Hostalet), General and University Hospital, Valencia Medical School. Reprint requests: M. Armengot, MD, PhD, c/ Mediterrani, 17, 46134 Foios, Valencia, Spain. Otolaryngol Head Neck Surg 1999;121:650-2. Copyright © 1999 by the American Academy of Otolaryngology– Head and Neck Surgery Foundation, Inc. 0194-5998/99/$8.00 + 0 23/78/87695
CASE REPORT
A 65-year-old woman consulted an ophthalmologist for diplopia. Examination revealed left exophthalmos with bulging of the medial canthus of the homolateral eye. Nasal endoscopy of the left nasal fossa revealed an abnormally enlarged middle turbinate covered with normal mucosa. A CT scan showed a rounded, expansile lesion with a homogeneous structure of soft tissue density. It expanded against the neighboring bone, thinning it and invading the orbit (Fig 1). Endonasal microsurgery was performed, and the diagnosis was concha bullosa mucocele. Incision of the mucoperiosteum and underlying bone was followed by the outflow of abundant brownish fluid. The exophthalmos and bulging of the corresponding medial canthus immediately disappeared. Resection of the lateral and inferior walls of the middle turbinate was performed. Histologic examination of the surgical specimen showed the mucocele wall with a well-preserved ciliary respiratory epithelium (Fig 2). In the electron microscopy study, a high concentration of ciliary units was observed. The ciliary ultrastructure was normal as regards more than 95% of the ciliary axonemes (Fig 3). Compound cilia and numeric abnormalities of the peripheral microtubules were observed in 5% of the cilia. A follow-up CT scan 18 months after surgery showed an absence of disease (Fig 4). DISCUSSION Concha bullosa has been implicated in the cause of sinusal inflammatory disease. The prevalence of concha bullosa in patients with chronic sinusitis varies among authors but may
Otolaryngology– Head and Neck Surgery Volume 121 Number 5
ARMENGOT et al
651
Fig 3. High ciliary density with normal ciliary axonemes corresponding to the mucocele wall. (Transmission electron microscopy; original magnification ×75,000.)
Fig 1. CT scan shows expansive lesion in the left fossa. It expanded against the neighboring bone, thinning it and invading the orbit.
Fig 2. Histologic appearance of the mucocele wall with well-preserved ciliary respiratory epithelium. (Hematoxylin and eosin stain.)
Fig 4. CT scan 18 months after surgery shows absence of disease.
be estimated at about 30%.5-7 The pathophysiology of chronic or recurrent sinusitis caused by pneumatization of the middle turbinate is probably related to its pernicious effects on ventilation of the paranasal sinuses and mucociliary drainage of the middle meatus.6,8 In addition to this mechanical effect, an additional factor would be the release of substance P as a result of the contact between the mucosal surfaces; this fact would in turn produce mucosal edema and hence increased impairment of sinus drainage.9 The release of substance P has also been related to the development of headache in certain patients with pneumatization of both the middle and superior turbinates.9,10
Concha bullosa only rarely gives rise to pathologic entities. In this sense most mucoceles (more than 70%) are frontoethmoidal,12,13 and a mucocele as large as that reported in this study, arising within a concha bullosa with destruction of the anterior ethmoid bone and invasion of the orbit, is a very rare occurrence. The preservation of the epithelial wall of the mucocele and its high ciliary density (Figs 2 and 3) strongly suggest that the lesion did not expand under pressure; rather, its genesis appears to have been a dynamic process. Our observations seem to support the idea that mucoceles are a reactive response to different pathologic situations involving com-
652
ARMENGOT et al
bined sinus-ostium closure and inflammation.15 This vicious circle could in turn cause the epithelial cells to release cytokines (interleukin E2, α-tumor necrosis factor, prostaglandin E2, and vascular adhesion factors) that would stimulate osteoclastic bone resorption.16,17 These events could account for the observation that the bony walls surrounding the mucocele exhibit areas of both bone resorption and bone formation,12 where osteoclastic activity accompanied by osteogenesis and sclerosis alternates with areas of active bone destruction.13 The mucocele would thus grow by outward expansion rather than under the effects of internal pressure, thereby preserving the sinus mucosa, as occurred in our patient. The role of infection in the cause of mucocele is uncertain. However, infectious processes are known to be a potential triggering factor when they take place in the presence of sinus isolation as a result of ostium obstruction.13 Endoscopic surgery adopting an endonasal approach is an adequate management choice in patients with mucoceles.11 Concha bullosa mucoceles are no exception to this, and highly satisfactory results were obtained in our case with endonasal microsurgery (Fig 4). CONCLUSIONS Concha bullosa may give rise to large mucoceles, which in turn may even invade the orbit. The good preservation of the ciliary epithelial lining suggests that its development is the result of a dynamic process. Endonasal microsurgery is well indicated for the management of patients with concha bullosa mucoceles. REFERENCES 1. Guerrier Y, Rouvier P. Anatomie du nez et des fosses nasales. Encyl Med Chir (Paris) Oto-Rhino-Laryngologie 20265 A10, 411-04, 18p.
Otolaryngology– Head and Neck Surgery November 1999
2. Zuckerkandl E. Die untere sicbbeinmuschel (mitther Nasanmuschel), normale und pathologische Anatomie der nasenhohle und ihrer pneumatischen Anhange. Bd 1, Bd 2. Vienna and Leipzig; 1893. 3. Massegur H, Ademá JM, Lluansi J. El cornete medio como causa de patología sinusal. Acta Otorrinolaringol Esp 1995;46: 27-9. 4. Badía L, Parikh A, Brrokes GB. Pyocele of the middle turbinate. J Laryngol Otol 1994;108:783-4. 5. Zinreich SJ, Mattox DE, Kennedy DW, et al. Concha bullosa: CT evaluation. J Comput Assist Tomogr 1988;12:778-84. 6. Batman C, Biren T, Tutkun A, et al. CT evaluation of patients with chronic sinusitis. In: Tos M, Thomsen J, Balle V, editors. Rhinology: a state of the art. Amsterdam: Kugler Publications; 1995. p. 267-9. 7. Clark ST, Babin RW, Salazar J. The incidence of concha bullosa and its relationship to chronic sinonasal disease. Am J Rhinol 1989;3:11-2. 8. Zinreich SJ, Kennedy DW, Rosenbaum AE, et al. Paranasal sinuses: CT imaging requirements for endoscopic surgery. Radiology 1987;163:769-75. 9. Stammberger H, Wolf G. Headache and sinus disease: the endoscopic approach. Ann Otol Rhinol Laryngol 1988;97(Suppl 134):3-23. 10. Clerico DM. Pneumatized superior turbinate as a cause of referred migraine headache. Laryngoscope 1996;106:874-9. 11. Massegur H. Variaciones anatómicas. In: Ademá JM, Massegur H, Bernal M, et al., editors. Cirugía endoscópica nasosinusal. Madrid: Editorial Garsi, SA; 1994. p. 165-71. 12. Armengot M, Marco J, de la Fuente L, et al. Mucoceles: estudio retrospectivo y aportación de nuestra experiencia. Acta Otorrinolaringol Esp 1990;41:43-6. 13. Lund VJ. Physiopathologie des mucoceles. Cah Otorhinolaryngol 1996;21:407-11. 14. Canalis JG, Zajtchusk JT, Henkins HA. Ethmoid mucoceles. Arch Otolaryngol 1978;104:268-91. 15. Perrin CL. Mucoceles et pneumosinus dilatans. Encyl Med Chir (Paris) Oto-Rhino-Laryngologie 20465 A10 (12-1978). 16. Lund VJ, Harvey W, Meghji S, et al. Prostaglandin synthesis in the pathogenesis of fronto-ethmoidal mucoceles. Acta Otolaryngol (Stockh) 1993;113:540-6. 17. Lund VJ, Henderson B, Song Y. Involvement of cytokines and vascular adhesion receptors in the pathology of fronto-ethmoidal mucoceles. Acta Otolaryngol (Stockh) 1993;113:5406.
Otolaryngology– Head and Neck Surgery November 1999
ARMENGOT et al
Concha bullosa mucocele with invasion of the orbit MIGUEL ARMENGOT, MD, PhD, NURIA RUIZ, MD, CARMEN CARDA, MD, PhD, PACO HOSTALET, MD, and JORGE BASTERRA, MD, PhD,
Valencia, Spain
W
e describe the case of a patient with a large concha bullosa mucocele invading the orbit. The wall of the mucocele was lined by a well-preserved ciliary epithelium with a ciliary density similar to that of the healthy sinus mucosa. Ciliary ultrastructure was normal. Endonasal microsurgery yielded good results, and no recurrence has been observed after 18 months. The thickness of the middle turbinate is variable and often exhibits vacuolar structures. When pneumatized, this gives origin to a so-called concha bullosa.1 This lesion was first described by Zuckerlandl in 1893.2 For a number of years concha bullosa has been related to the pathogenesis of inflammatory disorders of the paranasal sinuses. Few cases have been reported dealing with concha bullosa as a cause of disease. In the past, a case of aspergilloma was reported within a concha bullosa3; however, a triple-index bibliographic search of the past 3 years yielded only a single case of a concha bullosa mucopyocele.4 This study reports a patient with a giant mucocele within a concha bullosa that destroyed the anterior ethmoid bone and invaded the orbit.
From the Services of Otolaryngology (Drs. Armengot, Ruiz, and Basterra) and Pathology (Drs. Carda and Hostalet), General and University Hospital, Valencia Medical School. Reprint requests: M. Armengot, MD, PhD, c/ Mediterrani, 17, 46134 Foios, Valencia, Spain. Otolaryngol Head Neck Surg 1999;121:650-2. Copyright © 1999 by the American Academy of Otolaryngology– Head and Neck Surgery Foundation, Inc. 0194-5998/99/$8.00 + 0 23/78/87695
CASE REPORT
A 65-year-old woman consulted an ophthalmologist for diplopia. Examination revealed left exophthalmos with bulging of the medial canthus of the homolateral eye. Nasal endoscopy of the left nasal fossa revealed an abnormally enlarged middle turbinate covered with normal mucosa. A CT scan showed a rounded, expansile lesion with a homogeneous structure of soft tissue density. It expanded against the neighboring bone, thinning it and invading the orbit (Fig 1). Endonasal microsurgery was performed, and the diagnosis was concha bullosa mucocele. Incision of the mucoperiosteum and underlying bone was followed by the outflow of abundant brownish fluid. The exophthalmos and bulging of the corresponding medial canthus immediately disappeared. Resection of the lateral and inferior walls of the middle turbinate was performed. Histologic examination of the surgical specimen showed the mucocele wall with a well-preserved ciliary respiratory epithelium (Fig 2). In the electron microscopy study, a high concentration of ciliary units was observed. The ciliary ultrastructure was normal as regards more than 95% of the ciliary axonemes (Fig 3). Compound cilia and numeric abnormalities of the peripheral microtubules were observed in 5% of the cilia. A follow-up CT scan 18 months after surgery showed an absence of disease (Fig 4). DISCUSSION Concha bullosa has been implicated in the cause of sinusal inflammatory disease. The prevalence of concha bullosa in patients with chronic sinusitis varies among authors but may
Otolaryngology– Head and Neck Surgery Volume 121 Number 5
ARMENGOT et al
651
Fig 3. High ciliary density with normal ciliary axonemes corresponding to the mucocele wall. (Transmission electron microscopy; original magnification ×75,000.)
Fig 1. CT scan shows expansive lesion in the left fossa. It expanded against the neighboring bone, thinning it and invading the orbit.
Fig 2. Histologic appearance of the mucocele wall with well-preserved ciliary respiratory epithelium. (Hematoxylin and eosin stain.)
Fig 4. CT scan 18 months after surgery shows absence of disease.
be estimated at about 30%.5-7 The pathophysiology of chronic or recurrent sinusitis caused by pneumatization of the middle turbinate is probably related to its pernicious effects on ventilation of the paranasal sinuses and mucociliary drainage of the middle meatus.6,8 In addition to this mechanical effect, an additional factor would be the release of substance P as a result of the contact between the mucosal surfaces; this fact would in turn produce mucosal edema and hence increased impairment of sinus drainage.9 The release of substance P has also been related to the development of headache in certain patients with pneumatization of both the middle and superior turbinates.9,10
Concha bullosa only rarely gives rise to pathologic entities. In this sense most mucoceles (more than 70%) are frontoethmoidal,12,13 and a mucocele as large as that reported in this study, arising within a concha bullosa with destruction of the anterior ethmoid bone and invasion of the orbit, is a very rare occurrence. The preservation of the epithelial wall of the mucocele and its high ciliary density (Figs 2 and 3) strongly suggest that the lesion did not expand under pressure; rather, its genesis appears to have been a dynamic process. Our observations seem to support the idea that mucoceles are a reactive response to different pathologic situations involving com-
652
ARMENGOT et al
bined sinus-ostium closure and inflammation.15 This vicious circle could in turn cause the epithelial cells to release cytokines (interleukin E2, α-tumor necrosis factor, prostaglandin E2, and vascular adhesion factors) that would stimulate osteoclastic bone resorption.16,17 These events could account for the observation that the bony walls surrounding the mucocele exhibit areas of both bone resorption and bone formation,12 where osteoclastic activity accompanied by osteogenesis and sclerosis alternates with areas of active bone destruction.13 The mucocele would thus grow by outward expansion rather than under the effects of internal pressure, thereby preserving the sinus mucosa, as occurred in our patient. The role of infection in the cause of mucocele is uncertain. However, infectious processes are known to be a potential triggering factor when they take place in the presence of sinus isolation as a result of ostium obstruction.13 Endoscopic surgery adopting an endonasal approach is an adequate management choice in patients with mucoceles.11 Concha bullosa mucoceles are no exception to this, and highly satisfactory results were obtained in our case with endonasal microsurgery (Fig 4). CONCLUSIONS Concha bullosa may give rise to large mucoceles, which in turn may even invade the orbit. The good preservation of the ciliary epithelial lining suggests that its development is the result of a dynamic process. Endonasal microsurgery is well indicated for the management of patients with concha bullosa mucoceles. REFERENCES 1. Guerrier Y, Rouvier P. Anatomie du nez et des fosses nasales. Encyl Med Chir (Paris) Oto-Rhino-Laryngologie 20265 A10, 411-04, 18p.
Otolaryngology– Head and Neck Surgery November 1999
2. Zuckerkandl E. Die untere sicbbeinmuschel (mitther Nasanmuschel), normale und pathologische Anatomie der nasenhohle und ihrer pneumatischen Anhange. Bd 1, Bd 2. Vienna and Leipzig; 1893. 3. Massegur H, Ademá JM, Lluansi J. El cornete medio como causa de patología sinusal. Acta Otorrinolaringol Esp 1995;46: 27-9. 4. Badía L, Parikh A, Brrokes GB. Pyocele of the middle turbinate. J Laryngol Otol 1994;108:783-4. 5. Zinreich SJ, Mattox DE, Kennedy DW, et al. Concha bullosa: CT evaluation. J Comput Assist Tomogr 1988;12:778-84. 6. Batman C, Biren T, Tutkun A, et al. CT evaluation of patients with chronic sinusitis. In: Tos M, Thomsen J, Balle V, editors. Rhinology: a state of the art. Amsterdam: Kugler Publications; 1995. p. 267-9. 7. Clark ST, Babin RW, Salazar J. The incidence of concha bullosa and its relationship to chronic sinonasal disease. Am J Rhinol 1989;3:11-2. 8. Zinreich SJ, Kennedy DW, Rosenbaum AE, et al. Paranasal sinuses: CT imaging requirements for endoscopic surgery. Radiology 1987;163:769-75. 9. Stammberger H, Wolf G. Headache and sinus disease: the endoscopic approach. Ann Otol Rhinol Laryngol 1988;97(Suppl 134):3-23. 10. Clerico DM. Pneumatized superior turbinate as a cause of referred migraine headache. Laryngoscope 1996;106:874-9. 11. Massegur H. Variaciones anatómicas. In: Ademá JM, Massegur H, Bernal M, et al., editors. Cirugía endoscópica nasosinusal. Madrid: Editorial Garsi, SA; 1994. p. 165-71. 12. Armengot M, Marco J, de la Fuente L, et al. Mucoceles: estudio retrospectivo y aportación de nuestra experiencia. Acta Otorrinolaringol Esp 1990;41:43-6. 13. Lund VJ. Physiopathologie des mucoceles. Cah Otorhinolaryngol 1996;21:407-11. 14. Canalis JG, Zajtchusk JT, Henkins HA. Ethmoid mucoceles. Arch Otolaryngol 1978;104:268-91. 15. Perrin CL. Mucoceles et pneumosinus dilatans. Encyl Med Chir (Paris) Oto-Rhino-Laryngologie 20465 A10 (12-1978). 16. Lund VJ, Harvey W, Meghji S, et al. Prostaglandin synthesis in the pathogenesis of fronto-ethmoidal mucoceles. Acta Otolaryngol (Stockh) 1993;113:540-6. 17. Lund VJ, Henderson B, Song Y. Involvement of cytokines and vascular adhesion receptors in the pathology of fronto-ethmoidal mucoceles. Acta Otolaryngol (Stockh) 1993;113:5406.