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Sclerosing cervicitis: An unusual cause of syncope
Sclerosing Cervicitis: An Unusual Cause of Syncope LOUIS G. PORTUGAL, PAUL W. BIDDINGER,
MD, JACK L. GLUCKMAN, MD
(Editorial Comment: Sclerosing cervicitis is part of a spectrum of histologically benign idiopathic fibrosclerotic syndromes. Intractable syncopal episodes were successfully treated with resection and reconstruction of the involved carotid artery. This represents a highly unique solution to a severe and debilitating problem.) Sclerosing cervicitis is a rare fibroinflammatory disease process which involves the vascular compartment and surrounding soft tissues of the neck. It is considered part of the spectrum of idiopathic fibrosclerotic syndromes that includes retroperitoneal fibrosis, sclerosing mediastinitis, sclerosing cholangitis, orbital pseudotumor, and Reidel’s thyroiditis. Sclerosing cervicitis, like other fibrosclerotic lesions, behaves clinically like a malignancy, usually presenting as a firm, immobile mass that grossly invades the surrounding soft tissue. The histologic appearance, however, is that of a benign fibroinflammatory process. We report an unusual case of sclerosing cervicitis involving the carotid artery which presented with syncopal episodes. The literature is reviewed and controversial aspects regarding diagnosis and management are highlighted.
CASE REPORT A 44-year-old man presented to the University of Cincinnati Medical Center (Cincinnati, OH) with a s-month history of episodes of syncope precipitated by head turning and palpation in the region of the left carotid artery. These episodes became progressively worse, severely limiting his daily activities and eventually leading to a head injury from a syncope-related fall.
From the Departments of Otolaryngology-Head and Neck Surgery and Pathology and Laboratory Medicine, University of Cincinnati Medical Center, Cincinnati, OH. Address reorint requests to Jack L. Gluckman, MD, Department bf Otolajngology-Head and Neck Suraerv. Universitv of Cincinnati Medical Center, 231 Be
American
Journal
of Otolaryngology,
MD, AND
Initial physical examination revealed a firm, nontender, fixed mass anterior to the left sternocleidomastoid and extending from the midcervical area to the angle of the mandible. During examination, the patient complained of lightheadedness accompanied by bradycardia induced by palpation of the mass. These episodes were self-limiting and disappeared upon cessation of palpation of the mass. The remainder of the examination of the head and neck was normal including full neurologic and cardiovascular examination. Specifically, no arrhythmias or carotid bruits were present. Magnetic resonance imaging revealed a mass encompassing the carotid bifurcation and extending from the common carotid to just below the skull base superiorly (Fig 1). Angiography demonstrated that the lesion was nonvascular with mild circumferential compression of the common carotid artery. Other investigations including complete blood count and chest x-ray were all normal. The presumptive diagnosis was one of a malignancy; to obtain pathologic diagnosis an open exploration was performed. Findings at the time of surgery demonstrated a fibrotic mass encasing the entire carotid artery extending from 3 cm below the carotid bifurcation superiorly along the internal carotid to approximately 1.5 cm below the skull base. Cranial nerves X, XI, and XII were trapped within the mass. Multiple biopsies revealed chronic inflammatory infiltrates and fibrosis with no evidence of malignancy. Because of the history of debilitating syncope and the presence of an infiltrating lesion, resection of the mass was undertaken, including resection of the involved carotid artery together with cranial nerves X, XI, and XII. An anterior mandibulotomy was performed to gain access to the superior extent of the lesion. Reconstruction using saphenous vein graft was performed. Postoperatively the patient complained of mild hoarseness and shoulder weakness. However, he was able to swallow well with no aspiration. At 6 months, he was doing well with no evidence of recurrent disease and complete resolution of his syncopal episodes. The pathologic findings were consistent with sclerosing cervicitis. Gross examination revealed a firm-mass lesion encasing the carotid artery and involving the surrounding soft tissues (Fig 2). Histologically, the lesion consisted of very dense chronic inflammatory infiltrates admixed with areas of fibrosis that resembled relatively acellular scar tissue (Fig 3). The inflammatory cells and fibrous tissue surrounded and infiltrated nerve bun-
Vol 14, No 5 (September-October),
1993: pp 356-362
SCLEROSING
CERVICITIS
Fig 1. Magnatic resonance imaging demonstrating the lesion of sclerosing cervicitis (black arrowhead) encasing the left carotid sinus (white arrow).
dles and adipose tissue. Small caliber arteries exhibited medial thickening with marked luminal stenosis. No microorganisms, foci of necrosis, granulomas, or malignant changes were identified (Fig 4). DISCUSSION Sclerosing cervicitis is included among several fibrosclerotic disorders that behave clinically like a malignancy, but are histologically benign. These lesions include retroperitoneal fibrosis, sclerosing mediastinitis, sclerosing cholangitis, orbital pseudotumor, and Reidel’s thyroiditis. Because of their similar histologic appearance and tendency for multifocal behavior, these lesions have been regarded as different manifestations of the same disease
Fig 2. Gross specimen photograph showing encasement the carotid artery in the region of the bifurcation.
of
pr0cess.l Fibrosclerotic lesions, in general, are uncommon occurrences, but have been well reviewed in the literature. Retroperitoneal fibrosis and sclerosing mediastinitis have received the majority of this attention and provide much of our present understanding of this disease. The agent responsible for inciting the fibrosclerotic process is presently not known. Trauma, infection, lymphatic obstruction, vitamin E deficiency, localized inflammation of fibroadipose tissue, toxoplasmosis, and leakage from vessels have all been considered, but remain unsubstantiated as causative agents.’ The possibility that a malignant neoplasm can initiate this process is supported by reports of lymphoma involving paraaortic nodes in cases of retroperitoneal fibrosis.3 In addition, there are case reports associating methysergide and methyldopa ingestion with fibrosclerotic lesions.4V5Neither malignancy nor medication could be implicated as a causative agent in our patient. Whatever the etiologic agent may be, it appears that these fibrosclerotic lesions represent an exaggerated response to tissue injury.2’3 The histopathologic pattern is that of a chronic inflammatory infiltrate and varying degrees of fibrosis involving soft tissues. Areas of fibrosis can be relatively acellular, hyalinized, and focal calcification may occur. At the periphery of the fibroblastic process, foci of lymphocytes and plasma cells are observed invading adjacent soft tissue. Perivascular and perineural infiltration by inflammatory cells and fibrous tissue is seen causing encasement and at times complete occlusion of small vessels. The extensive fibrotic process eventually leads to symptoms that are characteristically obstructive and often quite devastating. The symptoms of retroperitoneal fibrosis are related to ureteral and inferior vena caval obstruction resulting in anuria with hydronephrosis and lower extremity edemaa Pain secondary to lumbar nerve compression is frequently seen, and as the disease progresses gastrointestinal symptoms may occur related to involvement of the duodenum, biliary system, or mesenteric arteries. Findings related to mediastinal fibrosis may vary from upper extremity edema and high esophageal varices
360
PORTUGAL, GLUCKMAN. AND BIDDINGER
Fig 3. Photomicrograph showing chronic inflammation, fibrosis and encasement of nervas (arrows; hematoxylineosin stain, magnification x64).
caused by superior vena cava obstruction to the more serious cardiopulmonary compromise related to fibrosis of bronchi and coronary arteries.7 In Reidel’s thyroiditis, the majority of cases have a relatively benign, selflimited course. However, a lethal case of Reidel’s thyroiditis causing irreversible tracheal compression has recently been reported.* Sclerosing cervicitis is a rare form of an already uncommon group of fibrosclerotic diseases. Seven cases have been reported in the literature, consisting of three isolated reportsg-I’ as well as four patients identified at
the Mayo Clinic (Rochester, Minn) among a group of 12 patients with fibrosclerotic lesions of the head and neck diagnosed since 1970.12All three of the isolated case reports presented with vascular involvement, one associated with coexisting retroperitoneal fibrosis.lO All four cases identified at the Mayo Clinic demonstrated coexisting fibrosclerotic lesions elsewhere including the face, parotid, and retroperitoneum. This multifocal behavior was not demonstrated in our patient. Among the reported cases of sclerosing cervicitis, the consistent clinical presentation is that of a slowly enlarging neck mass. Symp-
Photomicrograph Fig 4. showing dense chronic inflammatory infiltrates and scar-like fibrous tissue Ihematoxylineosin, magnification x1981.
SCLEROSING
CERVICITIS
toms of pain varied, the majority of patients presenting with a nontender mass. Hoarseness and shoulder droop related to vagus and accessory nerve involvement were also presenting complaints in one patient9 In a different patient, progressive superior vena caval obstruction occurred as a result of disease extension into the mediastinum.ll Additional symptoms of abdominal pain, parotid and orbital swelling, eustachian tube dysfunction, dysphagia, and trismus were also present and appeared secondary to coexisting lesions elsewhere in the retroperitoneum or head and neck. Our patient presented with an enlarging neck mass, but lacked symptoms related to venous obstruction or nerve deficits. The unique symptom of syncope precipitated by turning of the head or palpation of the mass was not observed in previously reported cases of sclerosing cervicitis. Review of the history and physical examination in our patient suggests that the syncopal episodes were due to a hypersensitive carotid sinus created by the involvement of the carotid bifurcation by sclerosing cervicitis. Syncope related to a hypersensitive carotid sinus is part of a very long differential diagnosis for syncope of which the more common causes include vaso-vagal syncope, situational syncope (including micturition and cough syncope), orthostatic hypotension, drug-induced syncope, and syncope due to cardiac diseaseal Carotid sinus syncope is differentiated from other such causes of syncope by the presence of one or more classic trigger mechanisms such as forced head turning, neck hyperextension, constricting neck wear, and shaving.14 Increased carotid sinus sensitivity to mechanical stimulation is commonly seen in the elderly but may also be associated with frequently used drugs such as digitalis, methyldopa, and propranolol. More unusual are the reported cases of carotid sinus syncope caused purely by pathologic changes adjacent to the carotid sinus such as thyroid tumors, carotid body tumors, malignant neoplasms, and large cervical nodes.‘4-1” Our case is, to the best of our knowledge, the first reported case of carotid sinus syncope resulting from sclerosing cervicitis involving the carotid bifurcation. The clinical picture of sclerosing cervicitis may mimic the behavior of more common be-
361
nign and malignant diseases. The presence of a firm, immobile mass with invasive characteristics suggests the possibility of a malignancy or benign neoplasm including carotid body tumor or neurogenic tumor, eg, schwannoma. The macroscopic fibrotic changes seen with sclerosing cervicitis may, of course, also be caused by an infectious process such as tuberculosis or actinomycosis. The histopathologic findings may be confused with other more commonly occurring fibrotic lesions of the head and neck, including nodular fasciitis and aggressive fibromatosis. Unlike sclerosing cervicitis, lesions of nodular fasciitis are selflimiting inflammatory reactions that may be differentiated histologically by the increased mitotic activity, scant inflammatory infiltrate, looser fibroblastic tissue, and matrix rich in mucopolysaccharidesl’ Fibromatosis, on the other hand, invades adjacent structures in a manner similar to sclerosing cervicitis, but is histologically more cellular and lacks the inflammation seen in fibrosclerotic lesions.18 Although fine needle aspiration may be helpful in identifying a malignancy, it is usually unable to distinguish the subtle histologic differences that exist between these fibrotic lesions. Overall, definitive diagnosis of sclerosing cervicitis depends ultimately on open bioPsY. With regard to treatment, experience with retroperitoneal fibrosis has shown that steroids may produce a dramatic response particularly if the lesion is in its more inflammatory, less fibrotic stages.lg Surgical treatment serves largely to improve symptoms caused by obstruction, although combination therapy of prolonged steroid use following palliative surgery can potentially control the disease process. Unfortunately, because of the relative paucity of cases, experience with treatment of sclerosing cervicitis is limited. Review of the literature reveals that in one case low-dose irradiation achieved a partial response with cessation of progression of the diseases9 Of the 12 patients with fibroinflammatory lesions of the head and neck presented by Olsen and colleagues, seven patients were treated with primary surgical excision, while five received steroids? Of the surgically treated patients, five demonstrated no evidence of recurrence. Among those treated with steroids, two experienced complete resolution after high-dose
362
steroids, while another achieved control of residual disease with long-term doses. The four patients who failed initial treatment with either surgery or steroids underwent salvage treatment consisting of steroids or surgery, respectively. Three patients achieved control of disease and the fourth continued to have nrogression of disease despite aggressive trkatment. Potential treatment strategies in our case varied from a conservative approach using steroids to a more aggressive surgical approach. Either way, treatment was warranted because of the progressive nature of untreated sclerosing cervicitis as noted in one case where progressive involvement of the mediastinum occurred.ll In our case, surgical exploration was necessary to establish the diagnosis, but once we knew what we were dealing with, the decision-making process became a choice between treating the patient with steroids or performing a complete resection including excision of the carotid artery. The issue of carotid resection for sclerosing cervicitis is not addressed in any of the previously reported cases; however, if the technical expertise is available, there is no reason why this should not be performed.” Nevertheless, it is reasonable to conclude that patients not medically fit for a major vascular procedure should be treated with steroids. Overall, in our case, a highly satisfactory result was achieved. It is quite possible that initial use of steroids may have resulted in complete resolution of the mass and cessation of the syncopal episodes, although this remains a matter of speculation. REFERENCES 1. Comings DE, Skubi KB, Van Eyes J, et al: Familial multifocal fibrosclerosis: Findings suggesting that retroperitoneal fibrosis, mediastinal fibrosis, sclerosing cholangitis, Reidel’s thyroiditis, and pseudotumor of the orbit
PORTUGAL,
GLUCKMAN,
AND BIDDINGER
may be different manifestations of a single disease. Ann Intern Med 66:884-892, 1967 2. Mitchinson MJ: The pathology of idiopathic retroperitoneal fibrosis. 1 Clin Path01 23:681-689, 1970 _ 3. Buckberg GD, Dilley RB, Longmire WP Jr: The protean manifestations of sclerosing fibrosis. Surg Gynecol Obstet 123:729-736, 1966 4. Graham JR, Suby HI, LeCompte PR, et al: Fibrotic disorders associated with methvsergide theraov for headache. N Engl J Med 274:359-368, 1966 *” 5. Iversen BM, Johannesen JW, Nordahl E, et al: Retroperitoneal fibrosis during treatment with methyldopa. Lancet 2:302-304. 1975 6. Packham DA, Yates-Bell JG: The symptomatology and diagnosis of retroperitoneal fibrosis-A review of case reports. Br J Urol 40:207-222, 1986 7. Light AM: Idiopathic fibrosis of mediastinum: A discussion of three cases and review of the literature. J Clin Path01 31:78-88, 1978 8. Girod DA, Bigler SA, Coltrera MD: Reidel’s thyroiditis: Report of a lethal case and review of the literature. Otolaryngol Head Neck Surg 107:591-595, 1992 9. Rice DH, Batsakis JG, Coulthard SW: Sclerosing cervicitis: Homologue of sclerosing retroperitonitis and mediastinitis. Arch Surg llO:l20-122, 1975 10. Husband P, Knudsen A: Idiopathic cervical and retroperitoneal fibrosis: Report of a case treated with steroids. Postgrad Med J 52:788-793, 1976 11. Esdaile J, Murray D, Hawkins D, et al: Idiopathic fibrosis of the lateral compartment of the neck. Arch Intern Med 140:1386-1387, 1980 12. Olsen KD, DeSanto LW, Wold LE, et al: Tumefactive fibroinflammatory lesions of the head and neck. Laryngoscope 96:940-944, 1986 13. Kappor WN: Evaluation and management of the patient with syncope. JAMA 268:2553-2560, 1992 14. Strasberg B, Sagie A, Erdman S, et al: Carotid sinus hypersensitivity and the carotid sinus syndrome. Prog Cardiovasc Dis 31:379-391, 1989 15. McSwain B, Spencer FL: Carotid body tumor in
association with carotid sinus syndrome: cases. Surgery 22:22-229, 1947
Report of two
16. Frank JI, Ropper AH, Zuniga G: Vasodepressor carotid sinus syncope associated with a neck mass. Neurology 42:1194-1197, 1992 17. Enzinger FM, Weiss SW: Soft Tissue Tumors (ed 2). St Louis, MO, Mosby, 1988 18. Conley J, Healy WV, Stout AP: Fibromatosis of the head and neck. Am J Surg 112:609-614, 1966 19. Longmire WP Jr, Goodwin WE, Buckberg GD: Management of sclerosing fibrosis of the mediastinal and retroneritoneal areas. Ann Surg 165:1013-1021, 1967
20. Smith LL, Field FI: Management of uncommon lesions affecting the extracranial vessels, in Rutherford RB (ed): Vascular Surgery, ~012. Philadelphia, PA, Saunders, 1989, pp 1441-1450
MD, JACK L. GLUCKMAN, MD
(Editorial Comment: Sclerosing cervicitis is part of a spectrum of histologically benign idiopathic fibrosclerotic syndromes. Intractable syncopal episodes were successfully treated with resection and reconstruction of the involved carotid artery. This represents a highly unique solution to a severe and debilitating problem.) Sclerosing cervicitis is a rare fibroinflammatory disease process which involves the vascular compartment and surrounding soft tissues of the neck. It is considered part of the spectrum of idiopathic fibrosclerotic syndromes that includes retroperitoneal fibrosis, sclerosing mediastinitis, sclerosing cholangitis, orbital pseudotumor, and Reidel’s thyroiditis. Sclerosing cervicitis, like other fibrosclerotic lesions, behaves clinically like a malignancy, usually presenting as a firm, immobile mass that grossly invades the surrounding soft tissue. The histologic appearance, however, is that of a benign fibroinflammatory process. We report an unusual case of sclerosing cervicitis involving the carotid artery which presented with syncopal episodes. The literature is reviewed and controversial aspects regarding diagnosis and management are highlighted.
CASE REPORT A 44-year-old man presented to the University of Cincinnati Medical Center (Cincinnati, OH) with a s-month history of episodes of syncope precipitated by head turning and palpation in the region of the left carotid artery. These episodes became progressively worse, severely limiting his daily activities and eventually leading to a head injury from a syncope-related fall.
From the Departments of Otolaryngology-Head and Neck Surgery and Pathology and Laboratory Medicine, University of Cincinnati Medical Center, Cincinnati, OH. Address reorint requests to Jack L. Gluckman, MD, Department bf Otolajngology-Head and Neck Suraerv. Universitv of Cincinnati Medical Center, 231 Be
American
Journal
of Otolaryngology,
MD, AND
Initial physical examination revealed a firm, nontender, fixed mass anterior to the left sternocleidomastoid and extending from the midcervical area to the angle of the mandible. During examination, the patient complained of lightheadedness accompanied by bradycardia induced by palpation of the mass. These episodes were self-limiting and disappeared upon cessation of palpation of the mass. The remainder of the examination of the head and neck was normal including full neurologic and cardiovascular examination. Specifically, no arrhythmias or carotid bruits were present. Magnetic resonance imaging revealed a mass encompassing the carotid bifurcation and extending from the common carotid to just below the skull base superiorly (Fig 1). Angiography demonstrated that the lesion was nonvascular with mild circumferential compression of the common carotid artery. Other investigations including complete blood count and chest x-ray were all normal. The presumptive diagnosis was one of a malignancy; to obtain pathologic diagnosis an open exploration was performed. Findings at the time of surgery demonstrated a fibrotic mass encasing the entire carotid artery extending from 3 cm below the carotid bifurcation superiorly along the internal carotid to approximately 1.5 cm below the skull base. Cranial nerves X, XI, and XII were trapped within the mass. Multiple biopsies revealed chronic inflammatory infiltrates and fibrosis with no evidence of malignancy. Because of the history of debilitating syncope and the presence of an infiltrating lesion, resection of the mass was undertaken, including resection of the involved carotid artery together with cranial nerves X, XI, and XII. An anterior mandibulotomy was performed to gain access to the superior extent of the lesion. Reconstruction using saphenous vein graft was performed. Postoperatively the patient complained of mild hoarseness and shoulder weakness. However, he was able to swallow well with no aspiration. At 6 months, he was doing well with no evidence of recurrent disease and complete resolution of his syncopal episodes. The pathologic findings were consistent with sclerosing cervicitis. Gross examination revealed a firm-mass lesion encasing the carotid artery and involving the surrounding soft tissues (Fig 2). Histologically, the lesion consisted of very dense chronic inflammatory infiltrates admixed with areas of fibrosis that resembled relatively acellular scar tissue (Fig 3). The inflammatory cells and fibrous tissue surrounded and infiltrated nerve bun-
Vol 14, No 5 (September-October),
1993: pp 356-362
SCLEROSING
CERVICITIS
Fig 1. Magnatic resonance imaging demonstrating the lesion of sclerosing cervicitis (black arrowhead) encasing the left carotid sinus (white arrow).
dles and adipose tissue. Small caliber arteries exhibited medial thickening with marked luminal stenosis. No microorganisms, foci of necrosis, granulomas, or malignant changes were identified (Fig 4). DISCUSSION Sclerosing cervicitis is included among several fibrosclerotic disorders that behave clinically like a malignancy, but are histologically benign. These lesions include retroperitoneal fibrosis, sclerosing mediastinitis, sclerosing cholangitis, orbital pseudotumor, and Reidel’s thyroiditis. Because of their similar histologic appearance and tendency for multifocal behavior, these lesions have been regarded as different manifestations of the same disease
Fig 2. Gross specimen photograph showing encasement the carotid artery in the region of the bifurcation.
of
pr0cess.l Fibrosclerotic lesions, in general, are uncommon occurrences, but have been well reviewed in the literature. Retroperitoneal fibrosis and sclerosing mediastinitis have received the majority of this attention and provide much of our present understanding of this disease. The agent responsible for inciting the fibrosclerotic process is presently not known. Trauma, infection, lymphatic obstruction, vitamin E deficiency, localized inflammation of fibroadipose tissue, toxoplasmosis, and leakage from vessels have all been considered, but remain unsubstantiated as causative agents.’ The possibility that a malignant neoplasm can initiate this process is supported by reports of lymphoma involving paraaortic nodes in cases of retroperitoneal fibrosis.3 In addition, there are case reports associating methysergide and methyldopa ingestion with fibrosclerotic lesions.4V5Neither malignancy nor medication could be implicated as a causative agent in our patient. Whatever the etiologic agent may be, it appears that these fibrosclerotic lesions represent an exaggerated response to tissue injury.2’3 The histopathologic pattern is that of a chronic inflammatory infiltrate and varying degrees of fibrosis involving soft tissues. Areas of fibrosis can be relatively acellular, hyalinized, and focal calcification may occur. At the periphery of the fibroblastic process, foci of lymphocytes and plasma cells are observed invading adjacent soft tissue. Perivascular and perineural infiltration by inflammatory cells and fibrous tissue is seen causing encasement and at times complete occlusion of small vessels. The extensive fibrotic process eventually leads to symptoms that are characteristically obstructive and often quite devastating. The symptoms of retroperitoneal fibrosis are related to ureteral and inferior vena caval obstruction resulting in anuria with hydronephrosis and lower extremity edemaa Pain secondary to lumbar nerve compression is frequently seen, and as the disease progresses gastrointestinal symptoms may occur related to involvement of the duodenum, biliary system, or mesenteric arteries. Findings related to mediastinal fibrosis may vary from upper extremity edema and high esophageal varices
360
PORTUGAL, GLUCKMAN. AND BIDDINGER
Fig 3. Photomicrograph showing chronic inflammation, fibrosis and encasement of nervas (arrows; hematoxylineosin stain, magnification x64).
caused by superior vena cava obstruction to the more serious cardiopulmonary compromise related to fibrosis of bronchi and coronary arteries.7 In Reidel’s thyroiditis, the majority of cases have a relatively benign, selflimited course. However, a lethal case of Reidel’s thyroiditis causing irreversible tracheal compression has recently been reported.* Sclerosing cervicitis is a rare form of an already uncommon group of fibrosclerotic diseases. Seven cases have been reported in the literature, consisting of three isolated reportsg-I’ as well as four patients identified at
the Mayo Clinic (Rochester, Minn) among a group of 12 patients with fibrosclerotic lesions of the head and neck diagnosed since 1970.12All three of the isolated case reports presented with vascular involvement, one associated with coexisting retroperitoneal fibrosis.lO All four cases identified at the Mayo Clinic demonstrated coexisting fibrosclerotic lesions elsewhere including the face, parotid, and retroperitoneum. This multifocal behavior was not demonstrated in our patient. Among the reported cases of sclerosing cervicitis, the consistent clinical presentation is that of a slowly enlarging neck mass. Symp-
Photomicrograph Fig 4. showing dense chronic inflammatory infiltrates and scar-like fibrous tissue Ihematoxylineosin, magnification x1981.
SCLEROSING
CERVICITIS
toms of pain varied, the majority of patients presenting with a nontender mass. Hoarseness and shoulder droop related to vagus and accessory nerve involvement were also presenting complaints in one patient9 In a different patient, progressive superior vena caval obstruction occurred as a result of disease extension into the mediastinum.ll Additional symptoms of abdominal pain, parotid and orbital swelling, eustachian tube dysfunction, dysphagia, and trismus were also present and appeared secondary to coexisting lesions elsewhere in the retroperitoneum or head and neck. Our patient presented with an enlarging neck mass, but lacked symptoms related to venous obstruction or nerve deficits. The unique symptom of syncope precipitated by turning of the head or palpation of the mass was not observed in previously reported cases of sclerosing cervicitis. Review of the history and physical examination in our patient suggests that the syncopal episodes were due to a hypersensitive carotid sinus created by the involvement of the carotid bifurcation by sclerosing cervicitis. Syncope related to a hypersensitive carotid sinus is part of a very long differential diagnosis for syncope of which the more common causes include vaso-vagal syncope, situational syncope (including micturition and cough syncope), orthostatic hypotension, drug-induced syncope, and syncope due to cardiac diseaseal Carotid sinus syncope is differentiated from other such causes of syncope by the presence of one or more classic trigger mechanisms such as forced head turning, neck hyperextension, constricting neck wear, and shaving.14 Increased carotid sinus sensitivity to mechanical stimulation is commonly seen in the elderly but may also be associated with frequently used drugs such as digitalis, methyldopa, and propranolol. More unusual are the reported cases of carotid sinus syncope caused purely by pathologic changes adjacent to the carotid sinus such as thyroid tumors, carotid body tumors, malignant neoplasms, and large cervical nodes.‘4-1” Our case is, to the best of our knowledge, the first reported case of carotid sinus syncope resulting from sclerosing cervicitis involving the carotid bifurcation. The clinical picture of sclerosing cervicitis may mimic the behavior of more common be-
361
nign and malignant diseases. The presence of a firm, immobile mass with invasive characteristics suggests the possibility of a malignancy or benign neoplasm including carotid body tumor or neurogenic tumor, eg, schwannoma. The macroscopic fibrotic changes seen with sclerosing cervicitis may, of course, also be caused by an infectious process such as tuberculosis or actinomycosis. The histopathologic findings may be confused with other more commonly occurring fibrotic lesions of the head and neck, including nodular fasciitis and aggressive fibromatosis. Unlike sclerosing cervicitis, lesions of nodular fasciitis are selflimiting inflammatory reactions that may be differentiated histologically by the increased mitotic activity, scant inflammatory infiltrate, looser fibroblastic tissue, and matrix rich in mucopolysaccharidesl’ Fibromatosis, on the other hand, invades adjacent structures in a manner similar to sclerosing cervicitis, but is histologically more cellular and lacks the inflammation seen in fibrosclerotic lesions.18 Although fine needle aspiration may be helpful in identifying a malignancy, it is usually unable to distinguish the subtle histologic differences that exist between these fibrotic lesions. Overall, definitive diagnosis of sclerosing cervicitis depends ultimately on open bioPsY. With regard to treatment, experience with retroperitoneal fibrosis has shown that steroids may produce a dramatic response particularly if the lesion is in its more inflammatory, less fibrotic stages.lg Surgical treatment serves largely to improve symptoms caused by obstruction, although combination therapy of prolonged steroid use following palliative surgery can potentially control the disease process. Unfortunately, because of the relative paucity of cases, experience with treatment of sclerosing cervicitis is limited. Review of the literature reveals that in one case low-dose irradiation achieved a partial response with cessation of progression of the diseases9 Of the 12 patients with fibroinflammatory lesions of the head and neck presented by Olsen and colleagues, seven patients were treated with primary surgical excision, while five received steroids? Of the surgically treated patients, five demonstrated no evidence of recurrence. Among those treated with steroids, two experienced complete resolution after high-dose
362
steroids, while another achieved control of residual disease with long-term doses. The four patients who failed initial treatment with either surgery or steroids underwent salvage treatment consisting of steroids or surgery, respectively. Three patients achieved control of disease and the fourth continued to have nrogression of disease despite aggressive trkatment. Potential treatment strategies in our case varied from a conservative approach using steroids to a more aggressive surgical approach. Either way, treatment was warranted because of the progressive nature of untreated sclerosing cervicitis as noted in one case where progressive involvement of the mediastinum occurred.ll In our case, surgical exploration was necessary to establish the diagnosis, but once we knew what we were dealing with, the decision-making process became a choice between treating the patient with steroids or performing a complete resection including excision of the carotid artery. The issue of carotid resection for sclerosing cervicitis is not addressed in any of the previously reported cases; however, if the technical expertise is available, there is no reason why this should not be performed.” Nevertheless, it is reasonable to conclude that patients not medically fit for a major vascular procedure should be treated with steroids. Overall, in our case, a highly satisfactory result was achieved. It is quite possible that initial use of steroids may have resulted in complete resolution of the mass and cessation of the syncopal episodes, although this remains a matter of speculation. REFERENCES 1. Comings DE, Skubi KB, Van Eyes J, et al: Familial multifocal fibrosclerosis: Findings suggesting that retroperitoneal fibrosis, mediastinal fibrosis, sclerosing cholangitis, Reidel’s thyroiditis, and pseudotumor of the orbit
PORTUGAL,
GLUCKMAN,
AND BIDDINGER
may be different manifestations of a single disease. Ann Intern Med 66:884-892, 1967 2. Mitchinson MJ: The pathology of idiopathic retroperitoneal fibrosis. 1 Clin Path01 23:681-689, 1970 _ 3. Buckberg GD, Dilley RB, Longmire WP Jr: The protean manifestations of sclerosing fibrosis. Surg Gynecol Obstet 123:729-736, 1966 4. Graham JR, Suby HI, LeCompte PR, et al: Fibrotic disorders associated with methvsergide theraov for headache. N Engl J Med 274:359-368, 1966 *” 5. Iversen BM, Johannesen JW, Nordahl E, et al: Retroperitoneal fibrosis during treatment with methyldopa. Lancet 2:302-304. 1975 6. Packham DA, Yates-Bell JG: The symptomatology and diagnosis of retroperitoneal fibrosis-A review of case reports. Br J Urol 40:207-222, 1986 7. Light AM: Idiopathic fibrosis of mediastinum: A discussion of three cases and review of the literature. J Clin Path01 31:78-88, 1978 8. Girod DA, Bigler SA, Coltrera MD: Reidel’s thyroiditis: Report of a lethal case and review of the literature. Otolaryngol Head Neck Surg 107:591-595, 1992 9. Rice DH, Batsakis JG, Coulthard SW: Sclerosing cervicitis: Homologue of sclerosing retroperitonitis and mediastinitis. Arch Surg llO:l20-122, 1975 10. Husband P, Knudsen A: Idiopathic cervical and retroperitoneal fibrosis: Report of a case treated with steroids. Postgrad Med J 52:788-793, 1976 11. Esdaile J, Murray D, Hawkins D, et al: Idiopathic fibrosis of the lateral compartment of the neck. Arch Intern Med 140:1386-1387, 1980 12. Olsen KD, DeSanto LW, Wold LE, et al: Tumefactive fibroinflammatory lesions of the head and neck. Laryngoscope 96:940-944, 1986 13. Kappor WN: Evaluation and management of the patient with syncope. JAMA 268:2553-2560, 1992 14. Strasberg B, Sagie A, Erdman S, et al: Carotid sinus hypersensitivity and the carotid sinus syndrome. Prog Cardiovasc Dis 31:379-391, 1989 15. McSwain B, Spencer FL: Carotid body tumor in
association with carotid sinus syndrome: cases. Surgery 22:22-229, 1947
Report of two
16. Frank JI, Ropper AH, Zuniga G: Vasodepressor carotid sinus syncope associated with a neck mass. Neurology 42:1194-1197, 1992 17. Enzinger FM, Weiss SW: Soft Tissue Tumors (ed 2). St Louis, MO, Mosby, 1988 18. Conley J, Healy WV, Stout AP: Fibromatosis of the head and neck. Am J Surg 112:609-614, 1966 19. Longmire WP Jr, Goodwin WE, Buckberg GD: Management of sclerosing fibrosis of the mediastinal and retroneritoneal areas. Ann Surg 165:1013-1021, 1967
20. Smith LL, Field FI: Management of uncommon lesions affecting the extracranial vessels, in Rutherford RB (ed): Vascular Surgery, ~012. Philadelphia, PA, Saunders, 1989, pp 1441-1450