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Maxillofacial Implications of Scleroderma and Systemic Sclerosis: A Case Report and Literature Review
Maxillofacial Implications of Scleroderma and Systemic Sclerosis: A Case Report and Literature Review Charles Burchfield, DDS,* and John Vorrasi, DDSy Scleroderma and more generally systemic sclerosis comprise a group of autoimmune diseases characterized by increased collagen synthesis and deposition within connective tissue. The Scleroderma Foundation estimates that as many as 300,000 persons in the United States have scleroderma. Although the etiology of the disease is unknown, it is thought to be related to chronic inflammation secondary to autoimmune tissue damage. Scleroderma has variable presentations and may result in changes to the skin, blood vessels, muscles, and internal organs. Oral and maxillofacial findings can include limited mouth opening; xerostomia; periodontal disease; widening of the periodontal ligament space; zygomatic arch resorption; and resorption of the mandibular angle, coronoid process, and condyle. We present the case of a 26-yearold female patient with scleroderma who exhibited extreme maxillofacial manifestations of the disease. Ó 2019 American Association of Oral and Maxillofacial Surgeons J Oral Maxillofac Surg 77:1203-1208, 2019 Scleroderma is a group of autoimmune diseases characterized by increased collagen synthesis and deposition within connective tissue. The skin disease can be classified into 2 major groups: localized and systemic scleroderma. Localized scleroderma differs from systemic scleroderma in that it involves only the skin. Systemic scleroderma, also known as ‘‘systemic sclerosis,’’ may affect any organ system.1 Systemic sclerosis is often differentiated from localized scleroderma early by the development of Raynaud phenomenon or by paroxysmal vasospasm induced by cold or stress, which is the first sign in the vast majority of systemic sclerosis cases.2 Localized scleroderma can be further classified into 4 subtypes based on morphologic and clinical presentation: plaque, bulbous, linear, or deep. Systemic sclerosis also can be divided into 2 subtypes depending on the extent of skin involvement: cutaneous and limited. Some authors also have described a third subtype of systemic sclerosis, overlap
syndrome, which is diagnosed when a patient meets the criteria for more than 1 connective tissue disease, such as rheumatoid arthritis or systemic lupus erythematous.1 The Scleroderma Foundation estimates that as many as 300,000 persons in the United States have scleroderma, either localized or systemic. It is a disease of middle-aged women, with the average age at diagnosis being between 30 and 50 years3 and with a female-tomale predilection of 4:1.4 Although the etiology of the disease is unknown, it is thought to be related to chronic inflammation secondary to autoimmune tissue damage. Systemic sclerosis has variable presentations and may result in changes to the skin, blood vessels, muscles, and internal organs. Oral and maxillofacial findings can include limited mouth opening; xerostomia; periodontal disease; widening of the periodontal ligament (PDL) space; zygomatic arch resorption; and resorption of the mandibular angle, coronoid process, and condyle.5
Received from Department of Oral and Maxillofacial Surgery,
ter Medical Center, Eastman Institute for Oral Health, 601 Elmwood
University of Rochester Medical Center, Eastman Institute for Oral
Ave, PO Box 705, Rochester, NY 14642; e-mail: Charles_Burchfield@
Health, Rochester, NY. *Chief Resident.
urmc.rochester.edu Received December 17 2018
yAssistant Professor and Associate Program Director.
Accepted January 10 2019
Conflict of Interest Disclosures: None of the authors have any
Ó 2019 American Association of Oral and Maxillofacial Surgeons
relevant financial relationship(s) with a commercial interest.
0278-2391/19/30102-8
Address correspondence and reprint requests to Dr Burchfield:
https://doi.org/10.1016/j.joms.2019.01.027
Department of Oral and Maxillofacial Surgery, University of Roches-
1203
1204
SCLERODERMA AND SYSTEMIC SCLEROSIS
Case
and high risk of mandibular fracture, the patient was scheduled to undergo the procedure under general anesthesia in the operating room setting. A surgical airway was discussed with the patient with her lack of mouth opening. Nasal fiberoptic intubation was successful, but during the procedure, sufficient access for extraction of the mandibular left third molar was unable to be obtained. A left-sided oral commissurotomy was performed to increase mouth opening and facilitate extraction. The mandibular left third molar was surgically extracted with the use of a surgical handpiece, and no evidence of mandibular fracture was noted. The commissurotomy incision was closed primarily with No. 4-0 Vicryl sutures (Ethicon, Somerville, NJ) to close the orbicularis muscle, No. 3-0 chromic mucosal sutures, and No. 5-0 Prolene skin sutures (Ethicon). The patient presented for follow-up at 1 week and 9 weeks postoperatively with good healing, no evidence of fracture, and acceptable esthetics at the site of the left oral commissurotomy (Figs 7, 8). She has healed well without any consequences or deficits.
A 26-year-old female patient with a history of severe systemic sclerosis was referred to the Oral and Maxillofacial Surgery Department at Strong Memorial Hospital for evaluation of a symptomatic mandibular left third molar for extraction. The patient had been previously treated by the orthodontic department at the Eastman Institute for Oral Health (Figs 1, 2). Her clinical examination was relevant for microstomia, limited mouth opening with a maximum incisal opening of approximately 10 mm, a grossly carious mandibular left third molar, and generalized moderate to severe periodontal disease. The first documentation of the patient receiving a diagnosis of systemic sclerosis was at the age of 15 years. The patient’s mouth opening had severely deteriorated over the past 7 years since she underwent orthodontic treatment owing to perioral skin contracture (Fig 3). Cone beam computed tomography imaging was obtained, and a rendered panoramic view confirmed the presence of a grossly carious mandibular left third molar with roots extending to the inferior border of the mandible (Fig 4). Severe bone resorption of the mandibular angle, ramus, and coronoid process bilaterally also was noted (Figs 4-6). These findings were noted to be new in comparison to her panoramic radiograph obtained in 2011. On review of the patient’s health history, it was noted that although her disease manifestation was primarily cutaneous and muscular, she also had severe gastroesophageal reflux disease secondary to lower esophageal sphincter dysfunction, a common finding in systemic sclerosis. In addition, evidence of pulmonary fibrosis was noted on a recent computed tomography scan. The clinical significance of the fibrosis, however, was difficult to determine because pulmonary function tests could not be completed as her microstomia and limited mouth opening prevented adequate fitting of the spirometer mouthpiece. The findings of a recent echocardiogram were found to be essentially normal. It was determined that the mandibular left third molar required extraction, and because of the limited access
Discussion Patients with scleroderma, whether the localized or systemic form of the disease, can present with a multitude of different maxillofacial abnormalities. Microstomia is the most common orofacial clinical finding in scleroderma patients, and this is a direct result of perioral tissue sclerosis.5 The microstomia seen in scleroderma patients has multiple implications, including social concerns, difficulties with mastication, and limitations in maintaining oral hygiene. Furthermore, the decreased mouth opening can hinder access for dental and oral surgical procedures, as seen in our patient.6 Another common clinical finding is temporomandibular joint pain or dysfunction. The development of temporomandibular joint disorders is considerably more likely in patients with scleroderma than in the general population. This is likely secondary to
FIGURE 1. Facial assessment performed in orthodontic department in 2011. Burchfield and Vorrasi. Scleroderma and Systemic Sclerosis. J Oral Maxillofac Surg 2019.
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BURCHFIELD AND VORRASI
FIGURE 2. Panoramic radiograph obtained in 2011. L, left; R, right. Burchfield and Vorrasi. Scleroderma and Systemic Sclerosis. J Oral Maxillofac Surg 2019.
condylar resorption, musculature atrophy, and sclerosis, as well as synovial fibrosis.7 Scleroderma patients often present with a number of characteristic radiographic findings that can be seen on routine dentofacial imaging. These findings include widening of the PDL space, as well as maxillofacial bone resorption. PDL widening is often the first radiographic sign in scleroderma and is thought to be a result of increased collagen deposition within the periodontal ligament itself. As opposed to PDL widening seen in traumatic occlusion, the lamina dura remains intact in the case of scleroderma.8 Facial bone resorption seen in scleroderma predominantly occurs at areas of muscle attachment, such as the mandibular angle, coronoid process, mandibular condyle, and zygomatic arch. The current theory behind the etiology of bone resorption in scleroderma is multifactorial, including ischemia secondary to pressure necrosis from fibrosed and atrophic musculature, as well as decreased vascularity as a result of vessel wall sclerosis.9,10 As many as a third of patients with scleroderma show evidence of mandibular resorption on radiographic imaging.11 Our case report highlights an extreme deterioration and manifestation of the scleroderma process within the mandible and perioral soft tissue. It is important, as dental and oral surgical providers, to be aware of the orofacial manifestations of diseases such as scleroderma, as many of them can substantially impact oral and dental treatment. Furthermore, recognition of
clinical and radiographic findings may facilitate early identification of scleroderma patients and lead to earlier diagnosis, as many orofacial manifestations
FIGURE 3. Severe perioral contracture. Burchfield and Vorrasi. Scleroderma and Systemic Sclerosis. J Oral Maxillofac Surg 2019.
1206
SCLERODERMA AND SYSTEMIC SCLEROSIS
FIGURE 4. Panoramic radiograph obtained in 2018 with substantial mandibular deterioration and nonrestorable mandibular left third molar. L, left. Burchfield and Vorrasi. Scleroderma and Systemic Sclerosis. J Oral Maxillofac Surg 2019.
FIGURE 5. Rendered 3-dimensional cone beam computed tomography scan of left side. Burchfield and Vorrasi. Scleroderma and Systemic Sclerosis. J Oral Maxillofac Surg 2019.
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BURCHFIELD AND VORRASI
FIGURE 6. Rendered 3-dimensional cone beam computed tomography scan of right side. Burchfield and Vorrasi. Scleroderma and Systemic Sclerosis. J Oral Maxillofac Surg 2019.
FIGURE 7. Limited mouth opening.
FIGURE 8. Healed left commissurotomy scar.
Burchfield and Vorrasi. Scleroderma and Systemic Sclerosis. J Oral Maxillofac Surg 2019.
Burchfield and Vorrasi. Scleroderma and Systemic Sclerosis. J Oral Maxillofac Surg 2019.
1208 such as PDL space widening can precede any systemic symptoms.8
References 1. Balbir-Gurman A, Braun-Moscovici Y: Scleroderma overlap syndrome. Isr Med Assoc J 13:14, 2011 2. Chung L, Lin J, Furst DE, Fiorentino D: Systemic and localized scleroderma. Clin Dermatol 24:374, 2006 3. Nikpour M, Stevens WM, Herrick AL, Proudman SM: Epidemiology of systemic sclerosis. Best Pract Res Clin Rheumatol 24:857, 2010 4. Mayes MD, Lacey JV Jr, Beebe-Dimmer J, et al: Prevalence, incidence, survival, and disease characteristics of systemic sclerosis in a large US population. Arthritis Rheum 48:2246, 2003 5. Wood RE, Lee P: Analysis of the oral manifestations of systemic sclerosis (scleroderma). Oral Surg Oral Med Oral Pathol 65:172, 1988
SCLERODERMA AND SYSTEMIC SCLEROSIS 6. Silvestre-Rangil J, Martinez-Herrera M, Silvestre FJ: Dental management of patients with microstomia. A review of the literature and update on the treatment. J Oral Res 4:340, 2015 7. Crincoli V, Fatone L, Fanelli M, et al: Orofacial manifestations and temporomandibular disorders of systemic scleroderma: An observational study. Int J Mol Sci 17:E1189, 2016 8. Anbiaee N, Tafakhori Z: Early diagnosis of progressive systemic sclerosis (scleroderma) from a panoramic view: Report of three cases. Dentomaxillofac Radiol 40:457, 2011 9. Aliko A, Ciancaglini R, Alushi A, et al: Temporomandibular joint involvement in rheumatoid arthritis, systemic lupus erythematosus and systemic sclerosis. Int J Oral Maxillofac Surg 40:704, 2011 10. Auluck A: Widening of periodontal ligament space and mandibular resorption in patients with systemic sclerosis. Dentomaxillofac Radiol 36:441, 2007 11. Vincent C, Agard C, Barbarot S, et al: [Orofacial manifestations of systemic sclerosis: A study of 30 consecutive patients]. Rev Med Interne 30:5, 2009 (in French)
syndrome, which is diagnosed when a patient meets the criteria for more than 1 connective tissue disease, such as rheumatoid arthritis or systemic lupus erythematous.1 The Scleroderma Foundation estimates that as many as 300,000 persons in the United States have scleroderma, either localized or systemic. It is a disease of middle-aged women, with the average age at diagnosis being between 30 and 50 years3 and with a female-tomale predilection of 4:1.4 Although the etiology of the disease is unknown, it is thought to be related to chronic inflammation secondary to autoimmune tissue damage. Systemic sclerosis has variable presentations and may result in changes to the skin, blood vessels, muscles, and internal organs. Oral and maxillofacial findings can include limited mouth opening; xerostomia; periodontal disease; widening of the periodontal ligament (PDL) space; zygomatic arch resorption; and resorption of the mandibular angle, coronoid process, and condyle.5
Received from Department of Oral and Maxillofacial Surgery,
ter Medical Center, Eastman Institute for Oral Health, 601 Elmwood
University of Rochester Medical Center, Eastman Institute for Oral
Ave, PO Box 705, Rochester, NY 14642; e-mail: Charles_Burchfield@
Health, Rochester, NY. *Chief Resident.
urmc.rochester.edu Received December 17 2018
yAssistant Professor and Associate Program Director.
Accepted January 10 2019
Conflict of Interest Disclosures: None of the authors have any
Ó 2019 American Association of Oral and Maxillofacial Surgeons
relevant financial relationship(s) with a commercial interest.
0278-2391/19/30102-8
Address correspondence and reprint requests to Dr Burchfield:
https://doi.org/10.1016/j.joms.2019.01.027
Department of Oral and Maxillofacial Surgery, University of Roches-
1203
1204
SCLERODERMA AND SYSTEMIC SCLEROSIS
Case
and high risk of mandibular fracture, the patient was scheduled to undergo the procedure under general anesthesia in the operating room setting. A surgical airway was discussed with the patient with her lack of mouth opening. Nasal fiberoptic intubation was successful, but during the procedure, sufficient access for extraction of the mandibular left third molar was unable to be obtained. A left-sided oral commissurotomy was performed to increase mouth opening and facilitate extraction. The mandibular left third molar was surgically extracted with the use of a surgical handpiece, and no evidence of mandibular fracture was noted. The commissurotomy incision was closed primarily with No. 4-0 Vicryl sutures (Ethicon, Somerville, NJ) to close the orbicularis muscle, No. 3-0 chromic mucosal sutures, and No. 5-0 Prolene skin sutures (Ethicon). The patient presented for follow-up at 1 week and 9 weeks postoperatively with good healing, no evidence of fracture, and acceptable esthetics at the site of the left oral commissurotomy (Figs 7, 8). She has healed well without any consequences or deficits.
A 26-year-old female patient with a history of severe systemic sclerosis was referred to the Oral and Maxillofacial Surgery Department at Strong Memorial Hospital for evaluation of a symptomatic mandibular left third molar for extraction. The patient had been previously treated by the orthodontic department at the Eastman Institute for Oral Health (Figs 1, 2). Her clinical examination was relevant for microstomia, limited mouth opening with a maximum incisal opening of approximately 10 mm, a grossly carious mandibular left third molar, and generalized moderate to severe periodontal disease. The first documentation of the patient receiving a diagnosis of systemic sclerosis was at the age of 15 years. The patient’s mouth opening had severely deteriorated over the past 7 years since she underwent orthodontic treatment owing to perioral skin contracture (Fig 3). Cone beam computed tomography imaging was obtained, and a rendered panoramic view confirmed the presence of a grossly carious mandibular left third molar with roots extending to the inferior border of the mandible (Fig 4). Severe bone resorption of the mandibular angle, ramus, and coronoid process bilaterally also was noted (Figs 4-6). These findings were noted to be new in comparison to her panoramic radiograph obtained in 2011. On review of the patient’s health history, it was noted that although her disease manifestation was primarily cutaneous and muscular, she also had severe gastroesophageal reflux disease secondary to lower esophageal sphincter dysfunction, a common finding in systemic sclerosis. In addition, evidence of pulmonary fibrosis was noted on a recent computed tomography scan. The clinical significance of the fibrosis, however, was difficult to determine because pulmonary function tests could not be completed as her microstomia and limited mouth opening prevented adequate fitting of the spirometer mouthpiece. The findings of a recent echocardiogram were found to be essentially normal. It was determined that the mandibular left third molar required extraction, and because of the limited access
Discussion Patients with scleroderma, whether the localized or systemic form of the disease, can present with a multitude of different maxillofacial abnormalities. Microstomia is the most common orofacial clinical finding in scleroderma patients, and this is a direct result of perioral tissue sclerosis.5 The microstomia seen in scleroderma patients has multiple implications, including social concerns, difficulties with mastication, and limitations in maintaining oral hygiene. Furthermore, the decreased mouth opening can hinder access for dental and oral surgical procedures, as seen in our patient.6 Another common clinical finding is temporomandibular joint pain or dysfunction. The development of temporomandibular joint disorders is considerably more likely in patients with scleroderma than in the general population. This is likely secondary to
FIGURE 1. Facial assessment performed in orthodontic department in 2011. Burchfield and Vorrasi. Scleroderma and Systemic Sclerosis. J Oral Maxillofac Surg 2019.
1205
BURCHFIELD AND VORRASI
FIGURE 2. Panoramic radiograph obtained in 2011. L, left; R, right. Burchfield and Vorrasi. Scleroderma and Systemic Sclerosis. J Oral Maxillofac Surg 2019.
condylar resorption, musculature atrophy, and sclerosis, as well as synovial fibrosis.7 Scleroderma patients often present with a number of characteristic radiographic findings that can be seen on routine dentofacial imaging. These findings include widening of the PDL space, as well as maxillofacial bone resorption. PDL widening is often the first radiographic sign in scleroderma and is thought to be a result of increased collagen deposition within the periodontal ligament itself. As opposed to PDL widening seen in traumatic occlusion, the lamina dura remains intact in the case of scleroderma.8 Facial bone resorption seen in scleroderma predominantly occurs at areas of muscle attachment, such as the mandibular angle, coronoid process, mandibular condyle, and zygomatic arch. The current theory behind the etiology of bone resorption in scleroderma is multifactorial, including ischemia secondary to pressure necrosis from fibrosed and atrophic musculature, as well as decreased vascularity as a result of vessel wall sclerosis.9,10 As many as a third of patients with scleroderma show evidence of mandibular resorption on radiographic imaging.11 Our case report highlights an extreme deterioration and manifestation of the scleroderma process within the mandible and perioral soft tissue. It is important, as dental and oral surgical providers, to be aware of the orofacial manifestations of diseases such as scleroderma, as many of them can substantially impact oral and dental treatment. Furthermore, recognition of
clinical and radiographic findings may facilitate early identification of scleroderma patients and lead to earlier diagnosis, as many orofacial manifestations
FIGURE 3. Severe perioral contracture. Burchfield and Vorrasi. Scleroderma and Systemic Sclerosis. J Oral Maxillofac Surg 2019.
1206
SCLERODERMA AND SYSTEMIC SCLEROSIS
FIGURE 4. Panoramic radiograph obtained in 2018 with substantial mandibular deterioration and nonrestorable mandibular left third molar. L, left. Burchfield and Vorrasi. Scleroderma and Systemic Sclerosis. J Oral Maxillofac Surg 2019.
FIGURE 5. Rendered 3-dimensional cone beam computed tomography scan of left side. Burchfield and Vorrasi. Scleroderma and Systemic Sclerosis. J Oral Maxillofac Surg 2019.
1207
BURCHFIELD AND VORRASI
FIGURE 6. Rendered 3-dimensional cone beam computed tomography scan of right side. Burchfield and Vorrasi. Scleroderma and Systemic Sclerosis. J Oral Maxillofac Surg 2019.
FIGURE 7. Limited mouth opening.
FIGURE 8. Healed left commissurotomy scar.
Burchfield and Vorrasi. Scleroderma and Systemic Sclerosis. J Oral Maxillofac Surg 2019.
Burchfield and Vorrasi. Scleroderma and Systemic Sclerosis. J Oral Maxillofac Surg 2019.
1208 such as PDL space widening can precede any systemic symptoms.8
References 1. Balbir-Gurman A, Braun-Moscovici Y: Scleroderma overlap syndrome. Isr Med Assoc J 13:14, 2011 2. Chung L, Lin J, Furst DE, Fiorentino D: Systemic and localized scleroderma. Clin Dermatol 24:374, 2006 3. Nikpour M, Stevens WM, Herrick AL, Proudman SM: Epidemiology of systemic sclerosis. Best Pract Res Clin Rheumatol 24:857, 2010 4. Mayes MD, Lacey JV Jr, Beebe-Dimmer J, et al: Prevalence, incidence, survival, and disease characteristics of systemic sclerosis in a large US population. Arthritis Rheum 48:2246, 2003 5. Wood RE, Lee P: Analysis of the oral manifestations of systemic sclerosis (scleroderma). Oral Surg Oral Med Oral Pathol 65:172, 1988
SCLERODERMA AND SYSTEMIC SCLEROSIS 6. Silvestre-Rangil J, Martinez-Herrera M, Silvestre FJ: Dental management of patients with microstomia. A review of the literature and update on the treatment. J Oral Res 4:340, 2015 7. Crincoli V, Fatone L, Fanelli M, et al: Orofacial manifestations and temporomandibular disorders of systemic scleroderma: An observational study. Int J Mol Sci 17:E1189, 2016 8. Anbiaee N, Tafakhori Z: Early diagnosis of progressive systemic sclerosis (scleroderma) from a panoramic view: Report of three cases. Dentomaxillofac Radiol 40:457, 2011 9. Aliko A, Ciancaglini R, Alushi A, et al: Temporomandibular joint involvement in rheumatoid arthritis, systemic lupus erythematosus and systemic sclerosis. Int J Oral Maxillofac Surg 40:704, 2011 10. Auluck A: Widening of periodontal ligament space and mandibular resorption in patients with systemic sclerosis. Dentomaxillofac Radiol 36:441, 2007 11. Vincent C, Agard C, Barbarot S, et al: [Orofacial manifestations of systemic sclerosis: A study of 30 consecutive patients]. Rev Med Interne 30:5, 2009 (in French)