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A Rare Case of IgG4-Related Sclerosing Disease of the Maxillary Sinus Associated With Bone Destruction
2591
PACE AND WARD
J Oral Maxillofac Surg 68:2591-2593, 2010
A Rare Case of IgG4-Related Sclerosing Disease of the Maxillary Sinus Associated With Bone Destruction Clarence Pace, MD, BChD, MFDSRCS, MRCS,* and Sean Ward, FFDRCSI, FDSRCS(Eng), FRCS† Report of a Case A 73-year-old man presented to the accident and casualty department with a 2-week history of increasing rightsided facial swelling. This was associated with a lowgrade fever. Examination confirmed a 6-cm diameter swelling over the malar prominence. The overlying skin was normal and there was no paresthesia in the infraorbital nerve distribution. No trismus was evident. Intraoral examination revealed fullness in the upper buccal sulcus with almost complete effacement. Otherwise the examination was unremarkable. He was medically fit apart from benign prostatic hyperplasia. An orthopantomogram revealed a 2- ⫻ 2-cm “lozenge”shaped radiopacity in the right maxillary sinus. Magnetic resonance imaging (Fig 1) and computed tomographic scans clearly showed opacification of the right maxillary sinus and bone destruction. These demonstrated that the pathology extended into the infratemporal fossa. Magnetic resonance imaging of the abdomen revealed no evidence of retroperitoneal fibrosis. Several blood investigations were taken including a full blood cell count, urea, electrolytes, creatinine, C-reactive protein, liver function tests, immunoglobulin (Ig) G (including subclasses 1, 2, 3, and 4), IgA and IgM levels, a urinary sample for monoclonal free light chains, and blood viscosity. All results were within normal limits with the exception of slightly raised C-reactive protein and blood viscosity levels. Examination and biopsy were carried out under general anesthesia. These revealed a thickened buccal mucosa together with irregularity/erosion of the lateral wall of the right maxillary sinus. A small amount of pus was present in the buccal space and maxillary antrum.
Received from Oral and Maxillofacial Surgery, Charles Clifford Dental Hospital/Royal Hallamshire Hospital, Sheffield, United Kingdom. *Specialty Registrar. †Consultant, Oral and Maxillofacial Surgeon. Address correspondence and reprint requests to Mr Pace: Royal Hallamshire Hospital/Charles Clifford Dental Hospital, 76 Wellesley Road, Sheffield, United Kingdom; e-mail: [email protected] © 2010 American Association of Oral and Maxillofacial Surgeons
0278-2391/10/6810-0035$36.00/0 doi:10.1016/j.joms.2009.07.073
Subsequent histology revealed heavily, chronically inflamed collagenous connective tissue dominated by foci of cholesterol crystals. Dystrophic mineralization with new bone formation in the form of irregular trabeculae of woven bone was also evident. Immunohistochemistry was reported as showing numerous plasma cells of which more than 20% were of the IgG4 type. A diagnosis of IgG4-related sclerosing disease was subsequently made. A swab for culture and sensitivity was also sent, and the results were reported as negative to acid-alcohol fast bacilli, actinomyces-like organisms, and fungi. Gram-positive organisms were isolated. The patient was subsequently referred to the immunology department and was prescribed prednisolone 20 mg daily. Four weeks later, clinical examination of the right maxilla and retromaxillary area showed early signs of resolution. An interval magnetic resonance imaging scan 5 months after initiation of steroids revealed almost complete resolution (Fig 2). The patient ultimately went on to make a complete recovery.
Discussion IgG4-related sclerosing disease is a well-recognized chronic inflammatory disorder and mainly affects elderly men.1 It is a systemic disease that can affect various organs, the disorder being named according to the organ involved. It is characterized histologically by extensive infiltration of IgG4-positive plasma cells and T lymphocytes, associated with exuberant fibrosis. IgG4 is the rarest of the IgG subclasses to be expressed, accounting for only 3% to 6% of total IgG in normal serum. Retroperitoneal fibrosis is probably the most common example of the condition and is associated with increased serum IgG4 and an IgG4 –plasma cell infiltrate. Retroperitoneal fibrosis has many causes (drugs, notably methysergide; asbestos; and helminth infection) but in 70% of cases it is idiopathic.1 The condition is characterized by an inflammatory condition causing extensive fibrosis in the retroperitoneum. This can lead to ureteric obstruction with secondary renal failure.2 Hydroceles and unilateral lower limb edema
2592 secondary to encasement or entrapment of structures by the inflammatory mass are common presenting signs associated with retroperitoneal fibrosis. However, a few cases have been reported in which retroperitoneal fibrosis was closely associated with autoimmune pancreatitis,3 one of the characteristics of the latter being a raised IgG level. The formation of pseudotumors of the pancreatic head secondary to idiopathic retroperitoneal fibrosis has led to clinical and radiographic simulations of pancreatic carcinoma. Other organs that may be affected by this condition in the head and neck region include the salivary glands (chronic sclerosing sialadenitis, also known as Kuttner tumor),4 orbit, thyroid (Riedel thyroiditis), and pituitary gland.5 The kidneys, lungs (interstitial pneumonia), and prostate are also commonly involved. A review of the literature shows that, although pathology tends to be confined to 1 organ, cases have been reported where 3 or 4 organs were involved simultaneously. Most commonly patients present with signs and symptoms related to the organ affected. However, some patients may present with concomitant systemic symptoms such as fever as described in the present case.6 The differential diagnosis in this patient included infection, bacterial and fungal, and parasitic7 or malignant disease. Parasitic infections per se may
IgG4-RELATED SCLEROSIS OF MAXILLARY SINUS
FIGURE 2. Scan showing significant resolution of the retromaxillary pathology 5 months after corticosteroid treatment. Pace and Ward. IgG4-Related Sclerosis of Maxillary Sinus. J Oral Maxillofac Surg 2010.
be associated with high serum levels of IgG4.8 One of the preliminary diagnoses was cholesteatoma, which is primarily found in the middle ear. It has similar histologic features to those described in this report. However, cholesteatomas tend to have large foci of cholesterol associated with a proliferating squamous proliferating epithelial component, which was not evident in the definitive biopsy specimen. Although a single case of skeletal involvement has been previously reported,9 as far as the authors are aware, this is the first case in which IgG4-related sclerosing disease associated with bone destruction was found in the maxillary sinus. The diagnosis of IgG4-related sclerosing disease can be challenging, although once verified, clinical outcomes are very encouraging. Treatment with steroids before progression of the condition to dense fibrosis tissue formation is very effective. However, in those patients in whom significant fibrosis has occurred, surgical intervention may be necessary.10
References FIGURE 1. Scan before corticosteroid showing extensive pathology in the retromaxillary area and maxillary sinus. Pace and Ward. IgG4-Related Sclerosis of Maxillary Sinus. J Oral Maxillofac Surg 2010.
1. Neild GH, Rodriguez-Justo M, Wall C, et al: Hyper IgG4 disease: Report and characteristic of a new disease. BMC Med 4:23, 2006 2. Ormand JK: Bilateral ureteral obstruction due to envelopment and compression by an inflammatory retroperitoneal component. J Urol 59:950, 1948
2593
BAJ ET AL 3. Hamano H, Kawa S, Ochi Y, et al: Hydronephrosis associated with retroperitoneal fibrosis and sclerosing pancreatitis. Lancet 359:1403, 2002 4. Kamisawa T, Nakajima H, Hishima T: Close correlation between chronic sclerosing sialadenitis and immunoglobulin G4. Intern Med J 36:527, 2006 5. Grossman A, Gibson J, Stansfeld AG, et al: Pituitary and testicular fibrosis in association with retroperitoneal fibrosis. Clin Endocrinol 12:371, 2008 6. Petersdorf RG, Beeson PB: Fever of unexplained origin: Report on 100 cases. Med Baltimore 40:1, 1961
7. Robinson DS, Larche M, Durham SR: Tregs and allergic disease. J Clin Invest 114:1389, 2004 8. Jones CC, Hamilton RG, Jordon RE: Subclass distribution of human IgG autoantibodies in pemphigus. J Clin Immunol 8:43, 1988 9. Sozay S, Bayramoglu M, Karatas M, et al: Diffuse idiopathic skeletal hyperostosis in a patient with retroperitoneal fibrosis; a case report. Rheumatol Int 22:249, 2002 10. Uibu T, Oksa P, Auvinen A, et al: Asbestos exposure as a risk factor for retroperitoneal fibrosis. Lancet 363:1422, 2004 J Oral Maxillofac Surg 68:2593-2597, 2010
Anophthalmic Orbit Syndrome: A New Free Tissue Transfer Alessandro Baj, MD,* Francesco Laganà, MD,† Giada A. Beltramini, MD,‡ and Aldo B. Giannì, MD§ Report of a Case The anophthalmic socket is the space posterior to the eyelids and anterior to the muscle cone. It is created after enucleation or evisceration of an eye. Numerous distant and local flaps, grafts, and osteotomies have been used to improve these deformities.1-6 We describe our experience with socket contraction between April 2001 and July 2008, during which 36 patients underwent surgical treatment for contracted sockets. The indications for surgery included moderate socket contraction in 14 patients, empty socket contraction in 13, and a malignant anophthalmic socket in 9. In the case of moderate socket contraction, the solution is to replace the lost tissue with a tissue that is as similar to the original as possible. Thus the best tissue with which to correct the mucosal deficit of the fornices is the oral mucosa. The best option by which to manage empty socket contraction is a dermis fat graft. In the case of a malignant socket, free tissue transfer is preferred. An adequate socket will safely retain an ocular prosthesis with a good cosmetic appearance. In patients who have undergone radiotherapy after enucleation of the eye, bony growth retardation and
soft tissue retraction often cause severe hemifacial deformities. Moreover, so-called malignant contracture of the eye socket can develop at the site. Since 1990, different authors have proposed the use of free flaps to improve the results of this type of reconstruction, which remains challenging. The absence of an eye and the presence of hemifacial deformities should be treated with 2 goals in mind: 1) to obtain a socket sufficient to retain an ocular prosthesis and 2) to
Received from the Department of Maxillo-Facial Surgery, Istituto Ortopedico Galeazzi, University of Milan, Milan, Italy. *Chief Assistant. †Chief Assistant. ‡Resident. §Professor and Chief. Address correspondence and reprint requests to Dr Baj: Department of Maxillo-Facial Surgery, Istituto Ortopedico Galeazzi, University of Milan, via Riccardo Galeazzi 4, Milan, Italy; e-mail: [email protected] © 2010 American Association of Oral and Maxillofacial Surgeons
0278-2391/10/6810-0036$36.00/0 doi:10.1016/j.joms.2009.06.035
FIGURE 1. Vascular anatomy. Baj et al. Anophthalmic Orbit Syndrome. J Oral Maxillofac Surg 2010.
PACE AND WARD
J Oral Maxillofac Surg 68:2591-2593, 2010
A Rare Case of IgG4-Related Sclerosing Disease of the Maxillary Sinus Associated With Bone Destruction Clarence Pace, MD, BChD, MFDSRCS, MRCS,* and Sean Ward, FFDRCSI, FDSRCS(Eng), FRCS† Report of a Case A 73-year-old man presented to the accident and casualty department with a 2-week history of increasing rightsided facial swelling. This was associated with a lowgrade fever. Examination confirmed a 6-cm diameter swelling over the malar prominence. The overlying skin was normal and there was no paresthesia in the infraorbital nerve distribution. No trismus was evident. Intraoral examination revealed fullness in the upper buccal sulcus with almost complete effacement. Otherwise the examination was unremarkable. He was medically fit apart from benign prostatic hyperplasia. An orthopantomogram revealed a 2- ⫻ 2-cm “lozenge”shaped radiopacity in the right maxillary sinus. Magnetic resonance imaging (Fig 1) and computed tomographic scans clearly showed opacification of the right maxillary sinus and bone destruction. These demonstrated that the pathology extended into the infratemporal fossa. Magnetic resonance imaging of the abdomen revealed no evidence of retroperitoneal fibrosis. Several blood investigations were taken including a full blood cell count, urea, electrolytes, creatinine, C-reactive protein, liver function tests, immunoglobulin (Ig) G (including subclasses 1, 2, 3, and 4), IgA and IgM levels, a urinary sample for monoclonal free light chains, and blood viscosity. All results were within normal limits with the exception of slightly raised C-reactive protein and blood viscosity levels. Examination and biopsy were carried out under general anesthesia. These revealed a thickened buccal mucosa together with irregularity/erosion of the lateral wall of the right maxillary sinus. A small amount of pus was present in the buccal space and maxillary antrum.
Received from Oral and Maxillofacial Surgery, Charles Clifford Dental Hospital/Royal Hallamshire Hospital, Sheffield, United Kingdom. *Specialty Registrar. †Consultant, Oral and Maxillofacial Surgeon. Address correspondence and reprint requests to Mr Pace: Royal Hallamshire Hospital/Charles Clifford Dental Hospital, 76 Wellesley Road, Sheffield, United Kingdom; e-mail: [email protected] © 2010 American Association of Oral and Maxillofacial Surgeons
0278-2391/10/6810-0035$36.00/0 doi:10.1016/j.joms.2009.07.073
Subsequent histology revealed heavily, chronically inflamed collagenous connective tissue dominated by foci of cholesterol crystals. Dystrophic mineralization with new bone formation in the form of irregular trabeculae of woven bone was also evident. Immunohistochemistry was reported as showing numerous plasma cells of which more than 20% were of the IgG4 type. A diagnosis of IgG4-related sclerosing disease was subsequently made. A swab for culture and sensitivity was also sent, and the results were reported as negative to acid-alcohol fast bacilli, actinomyces-like organisms, and fungi. Gram-positive organisms were isolated. The patient was subsequently referred to the immunology department and was prescribed prednisolone 20 mg daily. Four weeks later, clinical examination of the right maxilla and retromaxillary area showed early signs of resolution. An interval magnetic resonance imaging scan 5 months after initiation of steroids revealed almost complete resolution (Fig 2). The patient ultimately went on to make a complete recovery.
Discussion IgG4-related sclerosing disease is a well-recognized chronic inflammatory disorder and mainly affects elderly men.1 It is a systemic disease that can affect various organs, the disorder being named according to the organ involved. It is characterized histologically by extensive infiltration of IgG4-positive plasma cells and T lymphocytes, associated with exuberant fibrosis. IgG4 is the rarest of the IgG subclasses to be expressed, accounting for only 3% to 6% of total IgG in normal serum. Retroperitoneal fibrosis is probably the most common example of the condition and is associated with increased serum IgG4 and an IgG4 –plasma cell infiltrate. Retroperitoneal fibrosis has many causes (drugs, notably methysergide; asbestos; and helminth infection) but in 70% of cases it is idiopathic.1 The condition is characterized by an inflammatory condition causing extensive fibrosis in the retroperitoneum. This can lead to ureteric obstruction with secondary renal failure.2 Hydroceles and unilateral lower limb edema
2592 secondary to encasement or entrapment of structures by the inflammatory mass are common presenting signs associated with retroperitoneal fibrosis. However, a few cases have been reported in which retroperitoneal fibrosis was closely associated with autoimmune pancreatitis,3 one of the characteristics of the latter being a raised IgG level. The formation of pseudotumors of the pancreatic head secondary to idiopathic retroperitoneal fibrosis has led to clinical and radiographic simulations of pancreatic carcinoma. Other organs that may be affected by this condition in the head and neck region include the salivary glands (chronic sclerosing sialadenitis, also known as Kuttner tumor),4 orbit, thyroid (Riedel thyroiditis), and pituitary gland.5 The kidneys, lungs (interstitial pneumonia), and prostate are also commonly involved. A review of the literature shows that, although pathology tends to be confined to 1 organ, cases have been reported where 3 or 4 organs were involved simultaneously. Most commonly patients present with signs and symptoms related to the organ affected. However, some patients may present with concomitant systemic symptoms such as fever as described in the present case.6 The differential diagnosis in this patient included infection, bacterial and fungal, and parasitic7 or malignant disease. Parasitic infections per se may
IgG4-RELATED SCLEROSIS OF MAXILLARY SINUS
FIGURE 2. Scan showing significant resolution of the retromaxillary pathology 5 months after corticosteroid treatment. Pace and Ward. IgG4-Related Sclerosis of Maxillary Sinus. J Oral Maxillofac Surg 2010.
be associated with high serum levels of IgG4.8 One of the preliminary diagnoses was cholesteatoma, which is primarily found in the middle ear. It has similar histologic features to those described in this report. However, cholesteatomas tend to have large foci of cholesterol associated with a proliferating squamous proliferating epithelial component, which was not evident in the definitive biopsy specimen. Although a single case of skeletal involvement has been previously reported,9 as far as the authors are aware, this is the first case in which IgG4-related sclerosing disease associated with bone destruction was found in the maxillary sinus. The diagnosis of IgG4-related sclerosing disease can be challenging, although once verified, clinical outcomes are very encouraging. Treatment with steroids before progression of the condition to dense fibrosis tissue formation is very effective. However, in those patients in whom significant fibrosis has occurred, surgical intervention may be necessary.10
References FIGURE 1. Scan before corticosteroid showing extensive pathology in the retromaxillary area and maxillary sinus. Pace and Ward. IgG4-Related Sclerosis of Maxillary Sinus. J Oral Maxillofac Surg 2010.
1. Neild GH, Rodriguez-Justo M, Wall C, et al: Hyper IgG4 disease: Report and characteristic of a new disease. BMC Med 4:23, 2006 2. Ormand JK: Bilateral ureteral obstruction due to envelopment and compression by an inflammatory retroperitoneal component. J Urol 59:950, 1948
2593
BAJ ET AL 3. Hamano H, Kawa S, Ochi Y, et al: Hydronephrosis associated with retroperitoneal fibrosis and sclerosing pancreatitis. Lancet 359:1403, 2002 4. Kamisawa T, Nakajima H, Hishima T: Close correlation between chronic sclerosing sialadenitis and immunoglobulin G4. Intern Med J 36:527, 2006 5. Grossman A, Gibson J, Stansfeld AG, et al: Pituitary and testicular fibrosis in association with retroperitoneal fibrosis. Clin Endocrinol 12:371, 2008 6. Petersdorf RG, Beeson PB: Fever of unexplained origin: Report on 100 cases. Med Baltimore 40:1, 1961
7. Robinson DS, Larche M, Durham SR: Tregs and allergic disease. J Clin Invest 114:1389, 2004 8. Jones CC, Hamilton RG, Jordon RE: Subclass distribution of human IgG autoantibodies in pemphigus. J Clin Immunol 8:43, 1988 9. Sozay S, Bayramoglu M, Karatas M, et al: Diffuse idiopathic skeletal hyperostosis in a patient with retroperitoneal fibrosis; a case report. Rheumatol Int 22:249, 2002 10. Uibu T, Oksa P, Auvinen A, et al: Asbestos exposure as a risk factor for retroperitoneal fibrosis. Lancet 363:1422, 2004 J Oral Maxillofac Surg 68:2593-2597, 2010
Anophthalmic Orbit Syndrome: A New Free Tissue Transfer Alessandro Baj, MD,* Francesco Laganà, MD,† Giada A. Beltramini, MD,‡ and Aldo B. Giannì, MD§ Report of a Case The anophthalmic socket is the space posterior to the eyelids and anterior to the muscle cone. It is created after enucleation or evisceration of an eye. Numerous distant and local flaps, grafts, and osteotomies have been used to improve these deformities.1-6 We describe our experience with socket contraction between April 2001 and July 2008, during which 36 patients underwent surgical treatment for contracted sockets. The indications for surgery included moderate socket contraction in 14 patients, empty socket contraction in 13, and a malignant anophthalmic socket in 9. In the case of moderate socket contraction, the solution is to replace the lost tissue with a tissue that is as similar to the original as possible. Thus the best tissue with which to correct the mucosal deficit of the fornices is the oral mucosa. The best option by which to manage empty socket contraction is a dermis fat graft. In the case of a malignant socket, free tissue transfer is preferred. An adequate socket will safely retain an ocular prosthesis with a good cosmetic appearance. In patients who have undergone radiotherapy after enucleation of the eye, bony growth retardation and
soft tissue retraction often cause severe hemifacial deformities. Moreover, so-called malignant contracture of the eye socket can develop at the site. Since 1990, different authors have proposed the use of free flaps to improve the results of this type of reconstruction, which remains challenging. The absence of an eye and the presence of hemifacial deformities should be treated with 2 goals in mind: 1) to obtain a socket sufficient to retain an ocular prosthesis and 2) to
Received from the Department of Maxillo-Facial Surgery, Istituto Ortopedico Galeazzi, University of Milan, Milan, Italy. *Chief Assistant. †Chief Assistant. ‡Resident. §Professor and Chief. Address correspondence and reprint requests to Dr Baj: Department of Maxillo-Facial Surgery, Istituto Ortopedico Galeazzi, University of Milan, via Riccardo Galeazzi 4, Milan, Italy; e-mail: [email protected] © 2010 American Association of Oral and Maxillofacial Surgeons
0278-2391/10/6810-0036$36.00/0 doi:10.1016/j.joms.2009.06.035
FIGURE 1. Vascular anatomy. Baj et al. Anophthalmic Orbit Syndrome. J Oral Maxillofac Surg 2010.