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Surgical management of nasal and sinus sarcoidosis
Surgical management of nasal and sinus sarcoidosis STEVEN C. MARKS, MD, FACS, and ROY S. GOODMAN, MD, Detroit, Michigan
S
arcoidosis is a systemic inflammatory condition of unknown cause. Sarcoidosis is known to occur in all ethnic populations, in both sexes, and over a wide range of ages; however, the predominant population at risk in the United States is young to middle-aged black women.1-4 This condition primarily affects the lungs and hilar lymph nodes but may also affect the liver, eyes, skin, central nervous system, and nose and sinuses.5,6 The hallmark of sarcoidosis is the histologic finding of noncaseating granuloma on standard hematoxylin and eosin preparations. To make the diagnosis of sarcoidosis, a tissue biopsy specimen must demonstrate these findings in the absence of other granulomatous disorders, including tuberculosis and fungal disease. Patterns of disease in sarcoidosis are variable, and spontaneous exacerbations and remissions are possible. Several variants of sarcoidosis have been well described, including uveoparotid fever (HeerfordtÕs disease), neurosarcoidosis, and lupus pernio.7-9 Sinonasal involvement in this disease is infrequent, and estimates of incidence range from 1% to 6% in large series.10-13 However, this estimate may be inaccurate because of a lack of recognition of the sinonasal manifestations of the disease, with the true incidence being much higher. Severe symptoms of nasal obstruction and rhinorrhea may develop from involvement of the nasal mucosa, especially the inferior turbinates. More chronic nasal involvement can lead to irregularity and nodularity of the mucosa, which can acquire a cobblestone appearance with severe crusting and friability. Sinusitis can occur by either direct involvement of the sinus mucosa or obstruction of the sinus outflow with secondary bacterial infection. Typically, patients have severe chronic sinusitis marked by headache and purulent postnasal discharge. However, exacerbations are common with patients who have classic signs and symptoms of acute sinusitis. Traditionally, sinonasal sarcoidosis has been treated medically. The cornerstone of therapy is topical nasal steroids with saline irrigations and periodic antibiotics as indicated. When symptoms increase, oral systemic steroids can be used, which usually results in a prompt but not durable response. The role of surgery has been questionable. Existing literature cautions against septoplasty and turbinectomy as leading
From Wayne State University College of Medicine. Reprint requests: Steven C. Marks, MD, Department of Otolaryngology, Grace Hospital, 6071 W. Outer Dr., Detroit, MI 48235. Otolaryngol Head Neck Surg 1998;118:856-8. Copyright © 1998 by the American Academy of OtolaryngologyÐ Head and Neck Surgery Foundation, Inc. 0194-5998/98/$5.00 + 0 23/4/78795 856
to a high incidence of complications, specifically septal perforation in the case of septoplasty.14-16 Sinus surgery has been used only in emergency situations. In this article the results of a series of patients treated by sinus or nasal surgery is presented to illustrate the indications and the potential pitfalls in surgery for this disease. RESULTS Six patients underwent surgery for sinonasal sarcoidosis (Table 1). All were young to middle-aged black women (aged 21 to 45 years). Five had active pulmonary disease and four had known preexisting sarcoidosis. All patients were followed up for a minimum of 2 years from the date of the first procedure, except one patient, who was lost to follow-up after 3 months. These six patients have undergone a total of 11 operative procedures. CASE REPORTS
Case 1. This 45-year-old black woman was admitted to the hospital with dyspnea, uveitis, and acute sinusitis. Chest x-ray and ophthalmologic examination findings were consistent with sarcoidosis, but the patient had no previous history. Head and neck examination revealed massive hypertrophy of the inferior turbinates, exquisite tenderness of the frontal sinuses, and purulent postnasal discharge. CT demonstrated bilateral pansinusitis with frontal air-fluid levels. After 48 hours of intravenous antibiotics and steroids, the patient showed no improvement and was taken to surgery for drainage. The nasal cavity was completely filled by the enlarged turbinates, which did not decongest with 300 mg of cocaine flakes, making an intranasal approach impossible. Therefore a bilateral external ethmoidectomy with frontal sinus trephination was performed. Pathologic analysis demonstrated diffuse sinus involvement by noncaseating granuloma. Cultures were positive for Staphylococcus and Streptococcus species. The patient recovered well after surgery and received oral steroids intermittently thereafter. After surgery, bilateral synechiae amenable to endoscopic lysis developed. Now 42 months after surgery, she is doing well and has symptoms of only chronic rhinitis when the steroids are tapered. Case 2. A 35-year-old black woman who had a known diagnosis of sarcoidosis and was taking a varying dose of oral steroids was seen with a dry nasal obstruction. Her inferior turbinates were severely enlarged and completely obstructed the nasal airway bilaterally. After 3 months of nasal beclomethasone four times per day, no improvement was noted. A bilateral lateral turbinoplasty preserving the entire medial mucosal surface was performed. She is presently doing well with moderate but tolerable turbinate enlargement 42 months after surgery.
Otolaryngology– Head and Neck Surgery Volume 118 Number 6
Case 3. A 30-year-old black woman with known sarcoidosis was seen with bilateral masses protruding from her nose. CT showed these to be hypertrophied inferior turbinates without sinus involvement. She started taking oral prednisone 60 mg/day and had a brisk response with regression of the turbinates to normal during a 2-week period. However, despite institution of nasal beclomethasone four times per day, on withdrawal of the oral steroids, the turbinate hypertrophy returned. This cycle was repeated with the same result. Bilateral lateral turbinoplasty was performed without difficulty. After surgery, the patient was given nasal beclomethasone, but recurrent hypertrophy of the turbinates developed, although not to the extent previously noted. She only intermittently had an adequate airway but did not have recurrence of the turbinate prolapse. Case 4. A previously healthy 22-year-old black woman was seen as an outpatient with chronic facial headaches and purulent postnasal discharge. After an extensive trial of antibiotics, decongestant/mucolytic, and nasal steroids, a CT scan was obtained and demonstrated bilateral ethmoid opacification with frontal sinus mucosal thickening but no unusual bony or soft tissue findings. An endoscopic ethmoidectomy was performed in routine fashion. Pathologic analysis demonstrated diffuse involvement of the mucosa by noncaseating granuloma. Cultures and stains for mycobacteria and fungi were negative. Chest radiograph findings were normal. After surgery, the patient did well initially, but acute frontal sinusitis developed about 1 month later. After treatment with intravenous antibiotics and methylprednisolone, the symptoms resolved, and a subsequent CT scan demonstrated an aerated frontal sinus without mucosal thickening. After withdrawal of the steroids, the frontal sinusitis relapsed. A second course of treatment again resulted in complete resolution. Endoscopic examination at this time revealed small synechiae from the middle turbinate to the lateral nasal wall, which were lysed in a second endoscopic procedure. The patient was subsequently treated with long-term lowdose steroids and did well for several months until she became pregnant and had to discontinue the steroids. Since that time she has remained symptomatic with chronic headaches, nasal congestion, and postnasal drainage. It has now been 30 months since her surgery. Case 5. A 25-year-old black woman was seen for chronic sinusitis with headache and postnasal discharge. She had a 5-year history of sarcoidosis that was maintained on variable doses of oral steroids with significant pulmonary and facial skin involvement. Nasal examination demonstrated the classic signs of sarcoidosis with mucosal cobblestoning, crusting, and friability. She had already been receiving nasal beclomethasone and had received several courses of oral antibiotics. CT demonstrated pansinus involvement with mucosal thickening and air-fluid levels in the sphenoid, maxillary, and ethmoid sinuses. She underwent a bilateral endoscopic sphenoethmoidectomy with preservation of the middle turbinates. Pathologic analysis revealed diffuse noncaseating granuloma. After surgery, healing was somewhat delayed and crusting
MARKS and GOODMAN 857
Table 1. Patients and procedures Patient no.
1
Age (yr)
Surgery date
45
5/5/92 6/25/92
2 3 4
35 30 22
5/28/92 10/29/92 9/30/93 1/13/94
5 6
25 35
10/14/93 1989 5/17/94 8/30/94 9/20/94 8/10/95
Surgical procedure
Bilateral Ex Eth, Fr Treph Endoscopic lysis of synechiae Lateral turbinoplasty Lateral turbinoplasty Bilateral ESS Endoscopic lysis of synechiae Bilateral ESS Septoplasty, bilateral turbinectomy Osteoplastic frontal sinusectomy Bilateral transantral sphenoethmoidectomy Endoscopic lysis of synechiae Sublabial excision mucocele
Ex Eth, External ethmoidectomy; Fr Treph, frontal sinus trephination; ESS, endoscopic sinus surgery.
increased, but after 6 weeks a well-mucosalized ethmoid cavity without synechiae formed. Her symptoms of headache and drainage improved, but the nasal crusting and obstruction from the inferior turbinate involvement were unchanged. The patient declined further follow-up after 3 months. Case 6. A 29-year-old black woman was initially seen for nasal obstruction. Examination at that time showed a deviated nasal septum and enlarged inferior turbinates. The turbinates failed to respond to intranasal steroids, so septoplasty and partial turbinectomy were performed. Pathologic analysis surprisingly indicated noncaseating granulomas of the bone and mucosa. She had no pulmonary symptoms at that time, but chest x-ray films and pulmonary function test results were abnormal. The patient did well for several years but then returned with sinusitis and nasal obstruction. She had severe frontal headaches, nasal obstruction, crusting, and purulent discharge. Nasal examination revealed severe mucosal changes with cobblestoning, crusting, and friability. Endoscopic examination was not possible even after meticulous cleaning and maximal decongestion. CT showed ethmoid, sphenoid, and frontal sinus opacification with normal maxillary sinuses. The patient began receiving oral steroids, antibiotics, and nasal irrigations. Despite this aggressive regimen, her headaches did not improve. An osteoplastic frontal sinusectomy with obliteration was performed. The operation and recovery were routine, and pathologic analysis demonstrated noncaseating granuloma and infection. The frontal headaches resolved completely after this procedure. However, the patient continued to have facial headaches over the ethmoid distribution. After several more months of unsuccessful therapy, surgery was considered. In the operating room extensive efforts were made at an endo-
858 MARKS and GOODMAN
scopic procedure, but it was technically not possible because of nasal involvement of the sarcoidosis. Therefore a bilateral transantral sphenoethmoidectomy with preservation of the middle turbinates was performed. Pathologic analysis was consistent with sarcoidosis. She had temporary improvement, but recurrent ethmoiditis developed after about 6 months. One year after the transantral procedure, a right facial mass developed, and on CT scan it was found to be a postoperative cyst protruding through the wall of the sinus. This was removed through a sublabial approach. Currently it has been 7 years since the initial operation and 20 months since the frontal sinus surgery; there is no evidence of recurrence in that location.
Otolaryngology– Head and Neck Surgery June 1998
crusting and obstruction. The risk of severe crusting or atrophic rhinitis would seem to outweigh the potential benefit of more aggressive turbinate reduction. The one instance of osteoplastic frontal sinusectomy with obliteration was successful, with resolution of frontal headache without recurrence after 20 months. There is a theoretic risk of invasion of the fat graft by the sarcoid process, but to date no sign of problems has been seen. The results of this study are encouraging in that surgery can be performed safely in this patient population. However, the results also point to the need to reserve surgical intervention as a last resort in known cases of sarcoidosis of the nose and sinuses because of the high risk of late symptomatic relapse.
DISCUSSION The usual treatment of sarcoidosis, whether in the sinuses, nasal cavity, or chest, is medical management. First-line therapy for nasal involvement includes topical nasal steroids, antibiotics, and decongestants, with systemic steroids held in reserve. With these combinations almost all patients can be treated adequately. In fact, a significant level of symptoms should be considered inevitable in this patient population. Circumstances do exist, though, in which symptoms are so severe that surgical intervention should be considered, even in cases of sarcoidosis. In this small series of patients, medical management failed. Each patient had prolonged, intense medical therapy before surgery was considered. The indications for surgery in this series were nasal obstruction in two cases, incapacitating headache in three patients, synechiae in two cases, mucocele in one case, and acute frontal sinusitis unresponsive to treatment in one case. In two cases sarcoidosis was not diagnosed until after surgery. The results indicate that sinus surgery can be successfully and safely performed in patients with sarcoidosis. Short-term results were excellent with symptomatic improvement in all patients after surgery. However, the long-term results are less encouraging. None of the patients was asymptomatic on last follow-up, and only two of the four patients with sinus surgery had long-term symptomatic improvement. Crusting and synechiae were also more of a problem in these patients. Sinus cavity crusting was temporary in all cases and severe in only one patient. However, final healing of the cavity was significantly delayed to approximately 6 weeks. Synechiae requiring formal lysis developed in three of the four sinus surgery patients, although all four patients were without significant synechiae in the final result. Recurrent nasal obstruction after conservative turbinoplasty developed in both patients with turbinate hypertrophy, although not to the extent present before surgery. In neither case, however, has atrophic rhinitis developed in more than 3 years of follow-up. More aggressive turbinate resection, as was seen in patient 6, may result in problems with severe
REFERENCES 1. Crystal RG. Sarcoidosis. In: Isselbacher KJ, Braunwald E, Wilson JD, Martin JB, Fauci AS, Kasper DL, editors. HarrisonÕs principles of internal medicine. 13th ed. New York: McGrawHill; 1995. p. 1679-84. 2. Grassi C, Rizzato G, Pozzi E, editors. Sarcoidosis and other granulomatous disorders. Proceedings of the XI World Congress. Amsterdam: Excerpta Medica; 1988. 3. Johns CJ. Sarcoidosis. In: Fishman AP, editor. Pulmonary diseases and disorders. New York: McGraw-Hill; 1998, p. 645-66. 4. Fanburg BL, Pitt EA. Sarcoidosis. In: Murrary JF, Nadel JA, editors. Textbook of respiratory medicine. Philadelphia: WB Saunders; 1988, p. 1486-500. 5. Vestbo J, Langer SW, Iversen ET, Viskum K. Long-term prognosis of pulmonary sarcoidosis. Cardiac sarcoidosis and other extrapulmonary manifestations. Ugeskr Laeger 1995;157: 2848-50. 6. Sulavik SB, Palestro CJ, Spencer RP, Swyer AJ, Goldsmith SJ, Tierstein AS. Extrapulmonary sites of radiogallium accumulation in sarcoidosis. Clin Nucl Med 1990;15:876-8. 7. Ray CG. Mumps. In: Isselbacher KJ, Braunwald E, Wilson JD, Martin JB, Fauci AS, Kasper DL, editors. HarrisonÕs principles of internal medicine. 13th ed. New York: McGraw-Hill; 1995. p. 830-2. 8. Veres L, Mihaescu T. Neurosarcoidosis. Comments on 2 cases. Rev Med Chir Soc Med Nat Iasi 1990;94:421-4. 9. Spiteri MA, Matthey F, Gordon T, Carstairs LS, James DG. Lupus pernio: a clinico-radiological study of thirty-five cases. Br J Dermatol 1985;112:315-22. 10. Wilson R, Lund V, Sweatman M, Mackay IS, Mitchell DN. Upper respiratory tract involvement in sarcoidosis and its management. Eur Respir J 1988;1:269-72. 11. James DG, Barter S. Jash D, MacKinnon DM, Carstairs LS. Sarcoidosis of the upper respiratory tract (SURT). J Laryngol Otol 1982;96:711-8. 12. McCaffrey TV, McDonald TJ. Sarcoidosis of the nose and paranasal sinuses. Laryngoscope 1983;93:1281-4. 13. Neville E, Mills RG, Jash KD, Mackinnon DM, Carstairs LS, James DG. Sarcoidosis of the upper respiratory tract and its association with lupus pernio. Thorax 1976;31:660-4. 14. Hammond BL, Kataria YP. Nasal sarcoidosis with septal perforation. J Otolaryngol 1980;9:31-4. 15. Maiwald J, Rauchfuss A. A rare case of maxillary sinus sarcoidosis. HNO 1981;29:98-101. 16. Scott PM, Morphopoulos G, Bleach N. Augmentation rhinoplasty in nasal sarcoidosis. J Laryngol Otol 1992;106:544-6.
S
arcoidosis is a systemic inflammatory condition of unknown cause. Sarcoidosis is known to occur in all ethnic populations, in both sexes, and over a wide range of ages; however, the predominant population at risk in the United States is young to middle-aged black women.1-4 This condition primarily affects the lungs and hilar lymph nodes but may also affect the liver, eyes, skin, central nervous system, and nose and sinuses.5,6 The hallmark of sarcoidosis is the histologic finding of noncaseating granuloma on standard hematoxylin and eosin preparations. To make the diagnosis of sarcoidosis, a tissue biopsy specimen must demonstrate these findings in the absence of other granulomatous disorders, including tuberculosis and fungal disease. Patterns of disease in sarcoidosis are variable, and spontaneous exacerbations and remissions are possible. Several variants of sarcoidosis have been well described, including uveoparotid fever (HeerfordtÕs disease), neurosarcoidosis, and lupus pernio.7-9 Sinonasal involvement in this disease is infrequent, and estimates of incidence range from 1% to 6% in large series.10-13 However, this estimate may be inaccurate because of a lack of recognition of the sinonasal manifestations of the disease, with the true incidence being much higher. Severe symptoms of nasal obstruction and rhinorrhea may develop from involvement of the nasal mucosa, especially the inferior turbinates. More chronic nasal involvement can lead to irregularity and nodularity of the mucosa, which can acquire a cobblestone appearance with severe crusting and friability. Sinusitis can occur by either direct involvement of the sinus mucosa or obstruction of the sinus outflow with secondary bacterial infection. Typically, patients have severe chronic sinusitis marked by headache and purulent postnasal discharge. However, exacerbations are common with patients who have classic signs and symptoms of acute sinusitis. Traditionally, sinonasal sarcoidosis has been treated medically. The cornerstone of therapy is topical nasal steroids with saline irrigations and periodic antibiotics as indicated. When symptoms increase, oral systemic steroids can be used, which usually results in a prompt but not durable response. The role of surgery has been questionable. Existing literature cautions against septoplasty and turbinectomy as leading
From Wayne State University College of Medicine. Reprint requests: Steven C. Marks, MD, Department of Otolaryngology, Grace Hospital, 6071 W. Outer Dr., Detroit, MI 48235. Otolaryngol Head Neck Surg 1998;118:856-8. Copyright © 1998 by the American Academy of OtolaryngologyÐ Head and Neck Surgery Foundation, Inc. 0194-5998/98/$5.00 + 0 23/4/78795 856
to a high incidence of complications, specifically septal perforation in the case of septoplasty.14-16 Sinus surgery has been used only in emergency situations. In this article the results of a series of patients treated by sinus or nasal surgery is presented to illustrate the indications and the potential pitfalls in surgery for this disease. RESULTS Six patients underwent surgery for sinonasal sarcoidosis (Table 1). All were young to middle-aged black women (aged 21 to 45 years). Five had active pulmonary disease and four had known preexisting sarcoidosis. All patients were followed up for a minimum of 2 years from the date of the first procedure, except one patient, who was lost to follow-up after 3 months. These six patients have undergone a total of 11 operative procedures. CASE REPORTS
Case 1. This 45-year-old black woman was admitted to the hospital with dyspnea, uveitis, and acute sinusitis. Chest x-ray and ophthalmologic examination findings were consistent with sarcoidosis, but the patient had no previous history. Head and neck examination revealed massive hypertrophy of the inferior turbinates, exquisite tenderness of the frontal sinuses, and purulent postnasal discharge. CT demonstrated bilateral pansinusitis with frontal air-fluid levels. After 48 hours of intravenous antibiotics and steroids, the patient showed no improvement and was taken to surgery for drainage. The nasal cavity was completely filled by the enlarged turbinates, which did not decongest with 300 mg of cocaine flakes, making an intranasal approach impossible. Therefore a bilateral external ethmoidectomy with frontal sinus trephination was performed. Pathologic analysis demonstrated diffuse sinus involvement by noncaseating granuloma. Cultures were positive for Staphylococcus and Streptococcus species. The patient recovered well after surgery and received oral steroids intermittently thereafter. After surgery, bilateral synechiae amenable to endoscopic lysis developed. Now 42 months after surgery, she is doing well and has symptoms of only chronic rhinitis when the steroids are tapered. Case 2. A 35-year-old black woman who had a known diagnosis of sarcoidosis and was taking a varying dose of oral steroids was seen with a dry nasal obstruction. Her inferior turbinates were severely enlarged and completely obstructed the nasal airway bilaterally. After 3 months of nasal beclomethasone four times per day, no improvement was noted. A bilateral lateral turbinoplasty preserving the entire medial mucosal surface was performed. She is presently doing well with moderate but tolerable turbinate enlargement 42 months after surgery.
Otolaryngology– Head and Neck Surgery Volume 118 Number 6
Case 3. A 30-year-old black woman with known sarcoidosis was seen with bilateral masses protruding from her nose. CT showed these to be hypertrophied inferior turbinates without sinus involvement. She started taking oral prednisone 60 mg/day and had a brisk response with regression of the turbinates to normal during a 2-week period. However, despite institution of nasal beclomethasone four times per day, on withdrawal of the oral steroids, the turbinate hypertrophy returned. This cycle was repeated with the same result. Bilateral lateral turbinoplasty was performed without difficulty. After surgery, the patient was given nasal beclomethasone, but recurrent hypertrophy of the turbinates developed, although not to the extent previously noted. She only intermittently had an adequate airway but did not have recurrence of the turbinate prolapse. Case 4. A previously healthy 22-year-old black woman was seen as an outpatient with chronic facial headaches and purulent postnasal discharge. After an extensive trial of antibiotics, decongestant/mucolytic, and nasal steroids, a CT scan was obtained and demonstrated bilateral ethmoid opacification with frontal sinus mucosal thickening but no unusual bony or soft tissue findings. An endoscopic ethmoidectomy was performed in routine fashion. Pathologic analysis demonstrated diffuse involvement of the mucosa by noncaseating granuloma. Cultures and stains for mycobacteria and fungi were negative. Chest radiograph findings were normal. After surgery, the patient did well initially, but acute frontal sinusitis developed about 1 month later. After treatment with intravenous antibiotics and methylprednisolone, the symptoms resolved, and a subsequent CT scan demonstrated an aerated frontal sinus without mucosal thickening. After withdrawal of the steroids, the frontal sinusitis relapsed. A second course of treatment again resulted in complete resolution. Endoscopic examination at this time revealed small synechiae from the middle turbinate to the lateral nasal wall, which were lysed in a second endoscopic procedure. The patient was subsequently treated with long-term lowdose steroids and did well for several months until she became pregnant and had to discontinue the steroids. Since that time she has remained symptomatic with chronic headaches, nasal congestion, and postnasal drainage. It has now been 30 months since her surgery. Case 5. A 25-year-old black woman was seen for chronic sinusitis with headache and postnasal discharge. She had a 5-year history of sarcoidosis that was maintained on variable doses of oral steroids with significant pulmonary and facial skin involvement. Nasal examination demonstrated the classic signs of sarcoidosis with mucosal cobblestoning, crusting, and friability. She had already been receiving nasal beclomethasone and had received several courses of oral antibiotics. CT demonstrated pansinus involvement with mucosal thickening and air-fluid levels in the sphenoid, maxillary, and ethmoid sinuses. She underwent a bilateral endoscopic sphenoethmoidectomy with preservation of the middle turbinates. Pathologic analysis revealed diffuse noncaseating granuloma. After surgery, healing was somewhat delayed and crusting
MARKS and GOODMAN 857
Table 1. Patients and procedures Patient no.
1
Age (yr)
Surgery date
45
5/5/92 6/25/92
2 3 4
35 30 22
5/28/92 10/29/92 9/30/93 1/13/94
5 6
25 35
10/14/93 1989 5/17/94 8/30/94 9/20/94 8/10/95
Surgical procedure
Bilateral Ex Eth, Fr Treph Endoscopic lysis of synechiae Lateral turbinoplasty Lateral turbinoplasty Bilateral ESS Endoscopic lysis of synechiae Bilateral ESS Septoplasty, bilateral turbinectomy Osteoplastic frontal sinusectomy Bilateral transantral sphenoethmoidectomy Endoscopic lysis of synechiae Sublabial excision mucocele
Ex Eth, External ethmoidectomy; Fr Treph, frontal sinus trephination; ESS, endoscopic sinus surgery.
increased, but after 6 weeks a well-mucosalized ethmoid cavity without synechiae formed. Her symptoms of headache and drainage improved, but the nasal crusting and obstruction from the inferior turbinate involvement were unchanged. The patient declined further follow-up after 3 months. Case 6. A 29-year-old black woman was initially seen for nasal obstruction. Examination at that time showed a deviated nasal septum and enlarged inferior turbinates. The turbinates failed to respond to intranasal steroids, so septoplasty and partial turbinectomy were performed. Pathologic analysis surprisingly indicated noncaseating granulomas of the bone and mucosa. She had no pulmonary symptoms at that time, but chest x-ray films and pulmonary function test results were abnormal. The patient did well for several years but then returned with sinusitis and nasal obstruction. She had severe frontal headaches, nasal obstruction, crusting, and purulent discharge. Nasal examination revealed severe mucosal changes with cobblestoning, crusting, and friability. Endoscopic examination was not possible even after meticulous cleaning and maximal decongestion. CT showed ethmoid, sphenoid, and frontal sinus opacification with normal maxillary sinuses. The patient began receiving oral steroids, antibiotics, and nasal irrigations. Despite this aggressive regimen, her headaches did not improve. An osteoplastic frontal sinusectomy with obliteration was performed. The operation and recovery were routine, and pathologic analysis demonstrated noncaseating granuloma and infection. The frontal headaches resolved completely after this procedure. However, the patient continued to have facial headaches over the ethmoid distribution. After several more months of unsuccessful therapy, surgery was considered. In the operating room extensive efforts were made at an endo-
858 MARKS and GOODMAN
scopic procedure, but it was technically not possible because of nasal involvement of the sarcoidosis. Therefore a bilateral transantral sphenoethmoidectomy with preservation of the middle turbinates was performed. Pathologic analysis was consistent with sarcoidosis. She had temporary improvement, but recurrent ethmoiditis developed after about 6 months. One year after the transantral procedure, a right facial mass developed, and on CT scan it was found to be a postoperative cyst protruding through the wall of the sinus. This was removed through a sublabial approach. Currently it has been 7 years since the initial operation and 20 months since the frontal sinus surgery; there is no evidence of recurrence in that location.
Otolaryngology– Head and Neck Surgery June 1998
crusting and obstruction. The risk of severe crusting or atrophic rhinitis would seem to outweigh the potential benefit of more aggressive turbinate reduction. The one instance of osteoplastic frontal sinusectomy with obliteration was successful, with resolution of frontal headache without recurrence after 20 months. There is a theoretic risk of invasion of the fat graft by the sarcoid process, but to date no sign of problems has been seen. The results of this study are encouraging in that surgery can be performed safely in this patient population. However, the results also point to the need to reserve surgical intervention as a last resort in known cases of sarcoidosis of the nose and sinuses because of the high risk of late symptomatic relapse.
DISCUSSION The usual treatment of sarcoidosis, whether in the sinuses, nasal cavity, or chest, is medical management. First-line therapy for nasal involvement includes topical nasal steroids, antibiotics, and decongestants, with systemic steroids held in reserve. With these combinations almost all patients can be treated adequately. In fact, a significant level of symptoms should be considered inevitable in this patient population. Circumstances do exist, though, in which symptoms are so severe that surgical intervention should be considered, even in cases of sarcoidosis. In this small series of patients, medical management failed. Each patient had prolonged, intense medical therapy before surgery was considered. The indications for surgery in this series were nasal obstruction in two cases, incapacitating headache in three patients, synechiae in two cases, mucocele in one case, and acute frontal sinusitis unresponsive to treatment in one case. In two cases sarcoidosis was not diagnosed until after surgery. The results indicate that sinus surgery can be successfully and safely performed in patients with sarcoidosis. Short-term results were excellent with symptomatic improvement in all patients after surgery. However, the long-term results are less encouraging. None of the patients was asymptomatic on last follow-up, and only two of the four patients with sinus surgery had long-term symptomatic improvement. Crusting and synechiae were also more of a problem in these patients. Sinus cavity crusting was temporary in all cases and severe in only one patient. However, final healing of the cavity was significantly delayed to approximately 6 weeks. Synechiae requiring formal lysis developed in three of the four sinus surgery patients, although all four patients were without significant synechiae in the final result. Recurrent nasal obstruction after conservative turbinoplasty developed in both patients with turbinate hypertrophy, although not to the extent present before surgery. In neither case, however, has atrophic rhinitis developed in more than 3 years of follow-up. More aggressive turbinate resection, as was seen in patient 6, may result in problems with severe
REFERENCES 1. Crystal RG. Sarcoidosis. In: Isselbacher KJ, Braunwald E, Wilson JD, Martin JB, Fauci AS, Kasper DL, editors. HarrisonÕs principles of internal medicine. 13th ed. New York: McGrawHill; 1995. p. 1679-84. 2. Grassi C, Rizzato G, Pozzi E, editors. Sarcoidosis and other granulomatous disorders. Proceedings of the XI World Congress. Amsterdam: Excerpta Medica; 1988. 3. Johns CJ. Sarcoidosis. In: Fishman AP, editor. Pulmonary diseases and disorders. New York: McGraw-Hill; 1998, p. 645-66. 4. Fanburg BL, Pitt EA. Sarcoidosis. In: Murrary JF, Nadel JA, editors. Textbook of respiratory medicine. Philadelphia: WB Saunders; 1988, p. 1486-500. 5. Vestbo J, Langer SW, Iversen ET, Viskum K. Long-term prognosis of pulmonary sarcoidosis. Cardiac sarcoidosis and other extrapulmonary manifestations. Ugeskr Laeger 1995;157: 2848-50. 6. Sulavik SB, Palestro CJ, Spencer RP, Swyer AJ, Goldsmith SJ, Tierstein AS. Extrapulmonary sites of radiogallium accumulation in sarcoidosis. Clin Nucl Med 1990;15:876-8. 7. Ray CG. Mumps. In: Isselbacher KJ, Braunwald E, Wilson JD, Martin JB, Fauci AS, Kasper DL, editors. HarrisonÕs principles of internal medicine. 13th ed. New York: McGraw-Hill; 1995. p. 830-2. 8. Veres L, Mihaescu T. Neurosarcoidosis. Comments on 2 cases. Rev Med Chir Soc Med Nat Iasi 1990;94:421-4. 9. Spiteri MA, Matthey F, Gordon T, Carstairs LS, James DG. Lupus pernio: a clinico-radiological study of thirty-five cases. Br J Dermatol 1985;112:315-22. 10. Wilson R, Lund V, Sweatman M, Mackay IS, Mitchell DN. Upper respiratory tract involvement in sarcoidosis and its management. Eur Respir J 1988;1:269-72. 11. James DG, Barter S. Jash D, MacKinnon DM, Carstairs LS. Sarcoidosis of the upper respiratory tract (SURT). J Laryngol Otol 1982;96:711-8. 12. McCaffrey TV, McDonald TJ. Sarcoidosis of the nose and paranasal sinuses. Laryngoscope 1983;93:1281-4. 13. Neville E, Mills RG, Jash KD, Mackinnon DM, Carstairs LS, James DG. Sarcoidosis of the upper respiratory tract and its association with lupus pernio. Thorax 1976;31:660-4. 14. Hammond BL, Kataria YP. Nasal sarcoidosis with septal perforation. J Otolaryngol 1980;9:31-4. 15. Maiwald J, Rauchfuss A. A rare case of maxillary sinus sarcoidosis. HNO 1981;29:98-101. 16. Scott PM, Morphopoulos G, Bleach N. Augmentation rhinoplasty in nasal sarcoidosis. J Laryngol Otol 1992;106:544-6.