CASE REPORT
Conservative Treatment and Implant Rehabilitation of the Mandible in a Case of Craniofacial Fibrous Dysplasia: A Case Report Marzia Petrocelli, MD,* and Winfried Kretschmer, MD, DDS, PhDy We present a case of implant rehabilitation of the mandible in a young patient affected by craniofacial fibrous dysplasia. A computed tomography scan showed involvement of the left orbital contour and left maxilla, with total involvement of the mandible. We performed conservative surgical treatment from 2004 to 2011 to reconstruct and recontour the orbital and mandible region. Next, we performed implant rehabilitation of the mandible. This case emphasizes the importance of conservative and nondemolition treatment to obtain excellent functional and esthetic results. Ó 2014 American Association of Oral and Maxillofacial Surgeons J Oral Maxillofac Surg 72:902.e1-902.e6, 2014 Fibrous dysplasia is a rare bone disease that can involve a single site (monostotic fibrous dysplasia [MFD]) or multiple sites (polyostotic fibrous dysplasia [PFD]) or multiple locations of the craniofacial region (craniofacial fibrous dysplasia [CFD]).1 The MFD has occurred equally in males and females, and PFD has predominantly occurred in females. It appears in children and young adults. Its occurrence has been related to alterations of the GNAS gene that encodes a protein that regulates the proliferation and differentiation of osteoblasts. Its histologic appearance will be characterized by deposits of fibrous tissue between irregular trabecular bone and woven bone. Fibrous dysplasia will stabilize with bone maturation.2 Fibrous dysplasia was described by von Recklinghausen in the 1891; he termed it ‘‘fibrous disseminated osteitis.’’ Albright, in the 1937, termed a syndromic condition as ‘‘Albright’s syndrome,’’ in which PFD is associated with endocrine disease, precocious puberty, premature skeleton ossification, and ‘‘cafe au lait’’ spots on the skin.3 Lichtenstein,4 in 1938, made a primary classification of monostotic and polyostotic dysplasia according to the number of bone districts
*Trainee/Surgeon, Department of Maxillo-Facial Surgery, Federico II University of Naples, Naples, Italy.
involved in the dysplasia. Thoma, in 1954, was the first to describe a ‘‘facial fibrous dysplasia.’’ Obwegeser, in 1973, introduced a classification according to the evolution of the disease, with a first form termed ‘‘juvenile,’’ with a regression after puberty, and a second form termed ‘‘persistent.’’3 Fibrous dysplasia has been considered a rare bone disease that represents 2.5% of all bone diseases and 7% of all benign bone tumors. In 70 to 80% of cases, it will occur as MFD, and the most commonly affected bones have been the long bones (femur, tibia, ribs). In 20 to 30% of the cases, it will occur as PFD, with craniofacial involvement in 50% of the patients.5 CFD is a fibrous dysplasia that involves the contiguous bones of the craniofacial skeleton. It cannot be strictly classified as monostotic because of the involvement of multiple adjacent bones of the craniofacial region. It also cannot be strictly classified as polyostotic, because no involvement of bones outside the craniofacial region will be present. This condition has a slight female predilection. Most cases of CFD occur between the first and third decades of life, with disease stabilization when the patient reaches skeletal maturity.6
Received November 4 2013 Accepted January 18 2014
ySurgeon, Department of Maxillofacial and Reconstructive Surgery, Paracelsus-Krankenhaus Ruit, Ostfildern, Germany. Address correspondence and reprint requests to Dr Petrocelli:
Ó 2014 American Association of Oral and Maxillofacial Surgeons 0278-2391/14/00144-X$36.00/0 http://dx.doi.org/10.1016/j.joms.2014.01.020
Department of Maxillo-Facial Surgery, Federico II University of Naples, Via Pansini, 5, Naples 80131, Italy; e-mail: marzia.petrocelli@ alice.it
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The serum alkaline phosphatase (ALP) concentration might be a marker to monitor CFD evolution and stabilization in a young patient. Its elevation in serum was correlated with disease recurrence by Park et al.7 CFD will involve the maxilla almost twice as often as the mandible, usually in the posterior part. Most of these lesions will be unilateral. The diagnosis of CFD should be determined from the clinical evidence, histopathologic analysis of the biopsy specimen, and radiologic findings. The present study reports the conservative8 and gradual surgical treatment of a case of polyostotic CFD with final implant rehabilitation of the mandible.
Case Report In February 2004, a 10-year-old girl presented at Marienhospital (Stuttgart, Germany) after a histopathologic diagnosis of CFD from a biopsy of the mandible region. The patient had swelling in the left side of her face, specifically in the left orbital region and left vestibular sulcus of the mandibular region. She had discrete orbital dystopia. The swelling was palpable during the intraoral examination in the left side of the mandible sulcus (Fig 1). The mobility of the teeth was palpable in the front quadrant of the mandible. The radiologic examination using computed tomography (CT) scans showed typical bone lesions in the infraorbital region of the left maxillary bone without involvement of the orbit and total involvement of the mandible with the alveolar process (Fig 2). The radiographs showed the specific signs of a ‘‘sclerotic type’’ (Fig 3), with a homogeneous appearance and a ground glass appearance. Conservative step-by-step treatment was planned. In the first procedure, in December 2004, we extracted the teeth in the mandible and
FIGURE 1. Intraoral examination showing, through the mirror, swelling in the left side of the mandible sulcus and mobility of the front teeth. Petrocelli and Kretschmer. Conservative Treatment and Implant Rehabilitation in CFD. J Oral Maxillofac Surg 2014.
recontoured the mandible. In May 2005, we performed bilateral mandible recontouring. In March 2007, we removed the tumor from the left orbital and maxillary regions and performed a reconstruction using a calvarian bone graft and its fixation by titanium plates and screws. In October 2010, we removed the tumor from the mandibular symphysis and reconstructed it with an iliac crest bone graft covered by titanium mesh. In April 2011, 6 dental implants (4.3 16 mm; CAMLOG Implant Systems, CAMLOG Biotechnologies, Basel, Switzerland) were placed using the submucosal technique with another iliac crest bone graft placed at the same time (Fig 4). In December 2011, the implants were uncovered, and submucosal vestibuloplasty surgery, without any soft tissue grafting, was performed (Fig 5). In August 2012, we removed the tumor from the right mandibular ramus and performed recontouring of the mandible. Finally, in November 2012, we removed the titanium mesh from the mandible. Every histopathologic examination performed at the removal of the tumorous part confirmed the primary diagnosis and showed the typical histologic signs of CFD, including the presence of benign fibroblastic tissue with irregular spicules of woven bone and osteoblastic rimming embedded in fibrous tissue (Fig 6). The last follow-up visit after implant surgery in May 2013 showed an optimal functional and esthetic rehabilitation of the mandible. Radiographs showed complete reossification of the defect bone and complete integration of the implants (Fig 7).
Discussion CFD is a rare bone disease that involves the craniofacial skeleton. About 70% of patients with PFD will have craniofacial involvement, but only about 10% with MFD will have craniofacial involvement.9 It has been seen more often in childhood and often stabilizes in adulthood, with a slight female predilection. The serum ALP concentration might be a marker to monitor CFD evolution and stabilization in a young patient. Its serum elevation correlated with disease regrowth in a study by Park et al.7 In the maxillofacial region, CFD has involved the maxilla 50% more often than the mandible, usually in the posterior region and usually as a unilateral lesion. CFD will also affect the calvaria, skull base, zygoma, orbits, frontal bone, and temporal bone.5,6 Chen and Noordhoff10 described CFD as a disease that affects patients between the second and third decades of life. They classified CFD according to the disease location and extension. According to the classification, ‘‘first zone’’ dysplasia will involve the maxilla without the alveolar process, orbits, zygoma, or frontal bone; ‘‘second zone’’ dysplasia will involve the parietaloccipital region with extension up to the temporal
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FIGURE 2. Radiologic examination—computed tomography scan showing typical bone lesions in the infraorbital region of the left maxillary bone without involvement of the orbit and total involvement of the mandible with the alveolar process. Petrocelli and Kretschmer. Conservative Treatment and Implant Rehabilitation in CFD. J Oral Maxillofac Surg 2014.
scale bone. The ‘‘third zone’’ dysplasia will involve the skull base, temporal petrous bone, pterygoid process, and mastoid bone. Finally, ‘‘fourth zone’’ dysplasia will
involve the maxilla and mandible separately (monostotic form) or together (polyostotic form) with the alveolar process.3,10
FIGURE 3. Radiologic examination—radiographic view showing typical bone lesions in the left maxillary bone and total involvement of the mandible with the alveolar process. The radiographs showed the specific signs of a ‘‘sclerotic type’’ with a ground glass appearance. Petrocelli and Kretschmer. Conservative Treatment and Implant Rehabilitation in CFD. J Oral Maxillofac Surg 2014.
PETROCELLI AND KRETSCHMER
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FIGURE 4. Intraoperative view of the implant placement. Six dental implants (4.3 16 mm, CAMLOG) were placed using the submucosal technique with another iliac crest bone graft placed concurrently. Petrocelli and Kretschmer. Conservative Treatment and Implant Rehabilitation in CFD. J Oral Maxillofac Surg 2014.
The etiology is probably a genetic predisposition with a mutation located in the GNAS1 gene on chromosome 20q13. This mutation activates adenylate cyclase and consequently increases the intracellular concentrations of cyclic adenosine monophosphate, resulting in abnormal osteoblast differentiation and production of dysplastic bone.2,11 The diagnosis of CFD should be determined from the clinical evidence, histopathologic analysis findings of biopsy specimens, and radiologic findings. The usual presentation of patients with CFD has been a diffuse swelling in the affected region, and disease progression can cause esthetic deformities (facial asymmetry) and clinical symptoms such as visual disturbance caused by compression of the orbital nerve, proptosis, orbital dystopia, diplopia, and strabismus. Nasal malfunction will result from involvement of the paranasal sinus. Dental problems will be caused by involvement of the alveolar process. Sensory disturbances (deficits of II, III, IV, V, VII, and VIII cranial nerves) will result from constriction of the cranial foramina or involvement of bony cavities.3
FIGURE 5. Intraoral view after implant rehabilitation showing the correct and symmetric position of 6 dental implants to provide optimal functional and esthetic results. Petrocelli and Kretschmer. Conservative Treatment and Implant Rehabilitation in CFD. J Oral Maxillofac Surg 2014.
FIGURE 6. Histopathologic image showing typical craniofacial fibrous dysplasia signs, including the presence of benign fibroblastic tissue with irregular spicules of woven bone and osteoblastic rimming embedded in fibrous tissue. Petrocelli and Kretschmer. Conservative Treatment and Implant Rehabilitation in CFD. J Oral Maxillofac Surg 2014.
The histopathologic analysis in cases of CFD will show the presence of benign fibroblastic tissue with irregular spicules of woven bone and osteoblastic rimming embedded in fibrous tissue. Sometimes, a cystic degeneration with hemorrhage and osteoclasts located around the cyst will be found. Degeneration into sarcoma is possible, but rare, with recurrence of less than 1%.12 The radiologic examinations should include standard radiography, magnetic resonance imaging, and/ or CT. The radiologic images of CFD will show a thin bony cortex with well-defined borders—a typical ‘‘ground glass’’ appearance. The images will be radiolucent in the early disease stage and radiopaque in later stages.13,14 Panda et al15 described 3 types of CT findings for CFD: the pagetoid type, characterized by bone expansion and islands of bone formation; the sclerotic type, characterized by a homogeneous appearance and a ground glass presentation; and the cystic type, characterized by well-defined lesions with a sclerotic margin.15 The surgical treatment for patients affected by CFD has been controversial. It can be conservative or radical, depending on the patient’s age, disease extension, and lesion localization. In the polyostotic form, for which radical treatment is impossible, conservative treatment will generally be used. Usually in young patients, surgical treatment will be performed at puberty to exclude irreversible effects to the bone growth and in the hope that the disease will stabilize with stabilization of the skeletal growth of the patient. The aim of surgical treatment is to reduce pain and bone deformities
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FIGURE 7. Radiograph at last follow-up visit in 2013 showing complete reossification of the defect bone and complete integration of the implants. Petrocelli and Kretschmer. Conservative Treatment and Implant Rehabilitation in CFD. J Oral Maxillofac Surg 2014.
and prevent pathologic fractures.8 Surgical treatment16-18 of CFD has ranged from biopsy to recontouring of bone lesions; remodeling the bone defects using bone grafts, using calvaria or iliac crest bone grafts; reconstruction of large bone defects with free flaps, and bimaxillary osteotomies, reported by Sachs in 1984.3
In our case, in accordance with the surgical protocol from Chen and Noordhoff10 regarding the management of CFD, we treated ‘‘zone 1’’ with radical excision and reconstruction. We removed the tumor from the left orbital and maxillary regions and performed reconstruction using a calvaria bone graft and its fixation by
FIGURE 8. Functional and esthetic results showing a young girl’s smile after a conservative approach to craniofacial fibrous dysplasia and dental implant rehabilitation. Petrocelli and Kretschmer. Conservative Treatment and Implant Rehabilitation in CFD. J Oral Maxillofac Surg 2014.
PETROCELLI AND KRETSCHMER
titanium plates and screws.19,20 Also, we treated ‘‘zone 4’’ using conservative surgical management. We performed a gradual excision of the tumor and reconstruction of the mandible with an iliac crest bone graft21,22 after skeletal growth and disease stabilization. We also decided to perform implant rehabilitation23 to provide good functional and esthetic results in our young patient. We achieved complete reossification of the defect bone and complete integration of the implants, in accordance with the studies by Samman, who, in 1991, demonstrated that the use of titanium materials will result in good integration and histologic adaptation in the dysplastic bone.3 In conclusion, CFD is a rare and singular bone disease, and its surgical treatment remains controversial. We have demonstrated that conservative recontouring of the mandible with gradual excision of the dysplastic bone, reconstruction using an iliac crest bone graft, and, finally, implant rehabilitation at the end of skeletal growth can provide optimal functional and esthetic results (Fig 8) in a young patient, avoiding radical and aggressive surgical treatment and its consequences.
References 1. Barnes L, Eveson JW, Reichart P, Sidranski D: Pathology and Genetics of Head and Neck Tumors: World Health Classification of Tumors. Lyon, IARC Publications, 2005 2. Cawson RA, Binnie WH, Speight PM, et al: Lucas’s Pathology of Tumors of the Oral Tissues. Churchill Livingstone, London, 1998 3. Italian Society of Maxillo Facial Surgery (SICMF). Trattato di patologia chirurgica maxillo-facciale. Torino, Italy, Minerva Medica, 2007, pp 538–543. 4. Lichtenstein L: Polyostotic fibrous dysplasia. Arch Surg 36:874, 1938 5. Ricalde P, Magliocca KR, Lee JS: Craniofacial fibrous dysplasia. Oral Maxillofac Surg Clin North Am 24:427, 2012 6. Menon S, Venkatswamy S, Ramu V, et al: Craniofacial fibrous dysplasia: Surgery and literature review. Ann Maxillofac Surg 3: 66, 2013 7. Park BY, Cheon YW, Kim YO, et al: Prognosis for craniofacial fibrous dysplasia after incomplete resection: Age and serum alkaline phosphatase. J Oral Maxillofac Surg 39:221, 2010
902.e6 8. Valentini V, Cassoni A, Marianetti TM, et al: Craniomaxillofacial fibrous dysplasia: Conservative treatment or radical surgery? A retrospective study on 68 patients. Plast Reconstr Surg 123: 653, 2009 9. Kim DD, Ghali GE, Wright JM, Edwards SP: Surgical treatment of giant fibrous dysplasia of the mandible with concomitant craniofacial involvement. J Oral Maxillofac Surg 70:102, 2012 10. Chen YR, Noordhoff MS: Treatment of craniomaxillofacial fibrous dysplasia: How early and how extensive. Plast Recontr Surg 87:799, 1991 11. Mandrioli S, Carinci F, Dallera V, Calura G: Fibrous dysplasia. The clinicotherapeutic picture and new data on its etiology: A review of the literature. Minerva Stomatol 47:37, 1998 12. Assaf AT, Benecke AW, Riecke B, et al: Craniofacial fibrous dysplasia (CFD) of the maxilla in an 11-year old boy: A case report. J Craniomaxillofac Surg 40:788, 2012 13. Shah ZK, Peh WCG, Koh WL, Shek TWH: Magnetic resonance imaging appearances of fibrous dysplasia. Br J Radiol 78:1104, 2005 14. Abdelkarim A, Green R, Startzell J, Preece J: Craniofacial polyostotic fibrous dysplasia: A case report and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 106: 49, 2008 15. Panda NK, Parida PK, Sharma R, et al: A clinicoradiologic analysis of symptomatic craniofacial fibro-osseous lesions. Otolaryngol Head Neck Surg 136:928, 2007 16. Kruse A, Pieles U, Riener MO, et al: Craniomaxillofacial fibrous dysplasia: A 10-year database 1996-2006. Br J Oral Maxillofac Surg 47:302, 2009 17. Bequignon E, Cardinne C, Lachiver X, et al: Craniofacial fibrous dysplasia surgery: A functional approach. Eur Ann Otorhinolaryngol Head Neck Dis Epub 2013 Jul 18 18. Vegas Bustamante E, Gargallo Albiol J, Berini Aytes L, Gay Escoda C: Benign fibro-osseous lesions of the maxillas: Analysis of 11 cases. Med Oral Patol Oral Cir Bucal 13:E653, 2008 19. Stoler A, Hill T: Part I: Reconstruction after total mandibulectomy with free cranial and microvascular iliac crest grafts as preparation for implants. J Oral Implantol 18:38, 1992 20. Stoler A, Hill T: Part II. Mandibular reconstruction: combined intra-oral and in vitro placement of osseointegrated implants into a free and vascularized bone graft. J Oral Implantol 18:45, 1992 21. Mendonc¸a Caridad JJ, Platas F Jr: Fibrous dysplasia of the mandible: Surgical treatment with platelet-rich plasma and a corticocancellous iliac crest graft—Report of a case. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 105:e12, 2008 22. Obiechina AE, Ogunlade SO, Fasola AO, Arotiba JT: Mandibular segmental reconstruction with iliac crest. West Afr J Med 22: 46, 2003 23. Listrom RD, Symington JM: Osseointegrated dental implants in conjunction with bone grafts. Int J Oral Maxillofac Surg 17: 116, 1988
Conservative Treatment and Implant Rehabilitation of the Mandible in a Case of Craniofacial Fibrous Dysplasia: A Case Report Marzia Petrocelli, MD,* and Winfried Kretschmer, MD, DDS, PhDy We present a case of implant rehabilitation of the mandible in a young patient affected by craniofacial fibrous dysplasia. A computed tomography scan showed involvement of the left orbital contour and left maxilla, with total involvement of the mandible. We performed conservative surgical treatment from 2004 to 2011 to reconstruct and recontour the orbital and mandible region. Next, we performed implant rehabilitation of the mandible. This case emphasizes the importance of conservative and nondemolition treatment to obtain excellent functional and esthetic results. Ó 2014 American Association of Oral and Maxillofacial Surgeons J Oral Maxillofac Surg 72:902.e1-902.e6, 2014 Fibrous dysplasia is a rare bone disease that can involve a single site (monostotic fibrous dysplasia [MFD]) or multiple sites (polyostotic fibrous dysplasia [PFD]) or multiple locations of the craniofacial region (craniofacial fibrous dysplasia [CFD]).1 The MFD has occurred equally in males and females, and PFD has predominantly occurred in females. It appears in children and young adults. Its occurrence has been related to alterations of the GNAS gene that encodes a protein that regulates the proliferation and differentiation of osteoblasts. Its histologic appearance will be characterized by deposits of fibrous tissue between irregular trabecular bone and woven bone. Fibrous dysplasia will stabilize with bone maturation.2 Fibrous dysplasia was described by von Recklinghausen in the 1891; he termed it ‘‘fibrous disseminated osteitis.’’ Albright, in the 1937, termed a syndromic condition as ‘‘Albright’s syndrome,’’ in which PFD is associated with endocrine disease, precocious puberty, premature skeleton ossification, and ‘‘cafe au lait’’ spots on the skin.3 Lichtenstein,4 in 1938, made a primary classification of monostotic and polyostotic dysplasia according to the number of bone districts
*Trainee/Surgeon, Department of Maxillo-Facial Surgery, Federico II University of Naples, Naples, Italy.
involved in the dysplasia. Thoma, in 1954, was the first to describe a ‘‘facial fibrous dysplasia.’’ Obwegeser, in 1973, introduced a classification according to the evolution of the disease, with a first form termed ‘‘juvenile,’’ with a regression after puberty, and a second form termed ‘‘persistent.’’3 Fibrous dysplasia has been considered a rare bone disease that represents 2.5% of all bone diseases and 7% of all benign bone tumors. In 70 to 80% of cases, it will occur as MFD, and the most commonly affected bones have been the long bones (femur, tibia, ribs). In 20 to 30% of the cases, it will occur as PFD, with craniofacial involvement in 50% of the patients.5 CFD is a fibrous dysplasia that involves the contiguous bones of the craniofacial skeleton. It cannot be strictly classified as monostotic because of the involvement of multiple adjacent bones of the craniofacial region. It also cannot be strictly classified as polyostotic, because no involvement of bones outside the craniofacial region will be present. This condition has a slight female predilection. Most cases of CFD occur between the first and third decades of life, with disease stabilization when the patient reaches skeletal maturity.6
Received November 4 2013 Accepted January 18 2014
ySurgeon, Department of Maxillofacial and Reconstructive Surgery, Paracelsus-Krankenhaus Ruit, Ostfildern, Germany. Address correspondence and reprint requests to Dr Petrocelli:
Ó 2014 American Association of Oral and Maxillofacial Surgeons 0278-2391/14/00144-X$36.00/0 http://dx.doi.org/10.1016/j.joms.2014.01.020
Department of Maxillo-Facial Surgery, Federico II University of Naples, Via Pansini, 5, Naples 80131, Italy; e-mail: marzia.petrocelli@ alice.it
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The serum alkaline phosphatase (ALP) concentration might be a marker to monitor CFD evolution and stabilization in a young patient. Its elevation in serum was correlated with disease recurrence by Park et al.7 CFD will involve the maxilla almost twice as often as the mandible, usually in the posterior part. Most of these lesions will be unilateral. The diagnosis of CFD should be determined from the clinical evidence, histopathologic analysis of the biopsy specimen, and radiologic findings. The present study reports the conservative8 and gradual surgical treatment of a case of polyostotic CFD with final implant rehabilitation of the mandible.
Case Report In February 2004, a 10-year-old girl presented at Marienhospital (Stuttgart, Germany) after a histopathologic diagnosis of CFD from a biopsy of the mandible region. The patient had swelling in the left side of her face, specifically in the left orbital region and left vestibular sulcus of the mandibular region. She had discrete orbital dystopia. The swelling was palpable during the intraoral examination in the left side of the mandible sulcus (Fig 1). The mobility of the teeth was palpable in the front quadrant of the mandible. The radiologic examination using computed tomography (CT) scans showed typical bone lesions in the infraorbital region of the left maxillary bone without involvement of the orbit and total involvement of the mandible with the alveolar process (Fig 2). The radiographs showed the specific signs of a ‘‘sclerotic type’’ (Fig 3), with a homogeneous appearance and a ground glass appearance. Conservative step-by-step treatment was planned. In the first procedure, in December 2004, we extracted the teeth in the mandible and
FIGURE 1. Intraoral examination showing, through the mirror, swelling in the left side of the mandible sulcus and mobility of the front teeth. Petrocelli and Kretschmer. Conservative Treatment and Implant Rehabilitation in CFD. J Oral Maxillofac Surg 2014.
recontoured the mandible. In May 2005, we performed bilateral mandible recontouring. In March 2007, we removed the tumor from the left orbital and maxillary regions and performed a reconstruction using a calvarian bone graft and its fixation by titanium plates and screws. In October 2010, we removed the tumor from the mandibular symphysis and reconstructed it with an iliac crest bone graft covered by titanium mesh. In April 2011, 6 dental implants (4.3 16 mm; CAMLOG Implant Systems, CAMLOG Biotechnologies, Basel, Switzerland) were placed using the submucosal technique with another iliac crest bone graft placed at the same time (Fig 4). In December 2011, the implants were uncovered, and submucosal vestibuloplasty surgery, without any soft tissue grafting, was performed (Fig 5). In August 2012, we removed the tumor from the right mandibular ramus and performed recontouring of the mandible. Finally, in November 2012, we removed the titanium mesh from the mandible. Every histopathologic examination performed at the removal of the tumorous part confirmed the primary diagnosis and showed the typical histologic signs of CFD, including the presence of benign fibroblastic tissue with irregular spicules of woven bone and osteoblastic rimming embedded in fibrous tissue (Fig 6). The last follow-up visit after implant surgery in May 2013 showed an optimal functional and esthetic rehabilitation of the mandible. Radiographs showed complete reossification of the defect bone and complete integration of the implants (Fig 7).
Discussion CFD is a rare bone disease that involves the craniofacial skeleton. About 70% of patients with PFD will have craniofacial involvement, but only about 10% with MFD will have craniofacial involvement.9 It has been seen more often in childhood and often stabilizes in adulthood, with a slight female predilection. The serum ALP concentration might be a marker to monitor CFD evolution and stabilization in a young patient. Its serum elevation correlated with disease regrowth in a study by Park et al.7 In the maxillofacial region, CFD has involved the maxilla 50% more often than the mandible, usually in the posterior region and usually as a unilateral lesion. CFD will also affect the calvaria, skull base, zygoma, orbits, frontal bone, and temporal bone.5,6 Chen and Noordhoff10 described CFD as a disease that affects patients between the second and third decades of life. They classified CFD according to the disease location and extension. According to the classification, ‘‘first zone’’ dysplasia will involve the maxilla without the alveolar process, orbits, zygoma, or frontal bone; ‘‘second zone’’ dysplasia will involve the parietaloccipital region with extension up to the temporal
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FIGURE 2. Radiologic examination—computed tomography scan showing typical bone lesions in the infraorbital region of the left maxillary bone without involvement of the orbit and total involvement of the mandible with the alveolar process. Petrocelli and Kretschmer. Conservative Treatment and Implant Rehabilitation in CFD. J Oral Maxillofac Surg 2014.
scale bone. The ‘‘third zone’’ dysplasia will involve the skull base, temporal petrous bone, pterygoid process, and mastoid bone. Finally, ‘‘fourth zone’’ dysplasia will
involve the maxilla and mandible separately (monostotic form) or together (polyostotic form) with the alveolar process.3,10
FIGURE 3. Radiologic examination—radiographic view showing typical bone lesions in the left maxillary bone and total involvement of the mandible with the alveolar process. The radiographs showed the specific signs of a ‘‘sclerotic type’’ with a ground glass appearance. Petrocelli and Kretschmer. Conservative Treatment and Implant Rehabilitation in CFD. J Oral Maxillofac Surg 2014.
PETROCELLI AND KRETSCHMER
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FIGURE 4. Intraoperative view of the implant placement. Six dental implants (4.3 16 mm, CAMLOG) were placed using the submucosal technique with another iliac crest bone graft placed concurrently. Petrocelli and Kretschmer. Conservative Treatment and Implant Rehabilitation in CFD. J Oral Maxillofac Surg 2014.
The etiology is probably a genetic predisposition with a mutation located in the GNAS1 gene on chromosome 20q13. This mutation activates adenylate cyclase and consequently increases the intracellular concentrations of cyclic adenosine monophosphate, resulting in abnormal osteoblast differentiation and production of dysplastic bone.2,11 The diagnosis of CFD should be determined from the clinical evidence, histopathologic analysis findings of biopsy specimens, and radiologic findings. The usual presentation of patients with CFD has been a diffuse swelling in the affected region, and disease progression can cause esthetic deformities (facial asymmetry) and clinical symptoms such as visual disturbance caused by compression of the orbital nerve, proptosis, orbital dystopia, diplopia, and strabismus. Nasal malfunction will result from involvement of the paranasal sinus. Dental problems will be caused by involvement of the alveolar process. Sensory disturbances (deficits of II, III, IV, V, VII, and VIII cranial nerves) will result from constriction of the cranial foramina or involvement of bony cavities.3
FIGURE 5. Intraoral view after implant rehabilitation showing the correct and symmetric position of 6 dental implants to provide optimal functional and esthetic results. Petrocelli and Kretschmer. Conservative Treatment and Implant Rehabilitation in CFD. J Oral Maxillofac Surg 2014.
FIGURE 6. Histopathologic image showing typical craniofacial fibrous dysplasia signs, including the presence of benign fibroblastic tissue with irregular spicules of woven bone and osteoblastic rimming embedded in fibrous tissue. Petrocelli and Kretschmer. Conservative Treatment and Implant Rehabilitation in CFD. J Oral Maxillofac Surg 2014.
The histopathologic analysis in cases of CFD will show the presence of benign fibroblastic tissue with irregular spicules of woven bone and osteoblastic rimming embedded in fibrous tissue. Sometimes, a cystic degeneration with hemorrhage and osteoclasts located around the cyst will be found. Degeneration into sarcoma is possible, but rare, with recurrence of less than 1%.12 The radiologic examinations should include standard radiography, magnetic resonance imaging, and/ or CT. The radiologic images of CFD will show a thin bony cortex with well-defined borders—a typical ‘‘ground glass’’ appearance. The images will be radiolucent in the early disease stage and radiopaque in later stages.13,14 Panda et al15 described 3 types of CT findings for CFD: the pagetoid type, characterized by bone expansion and islands of bone formation; the sclerotic type, characterized by a homogeneous appearance and a ground glass presentation; and the cystic type, characterized by well-defined lesions with a sclerotic margin.15 The surgical treatment for patients affected by CFD has been controversial. It can be conservative or radical, depending on the patient’s age, disease extension, and lesion localization. In the polyostotic form, for which radical treatment is impossible, conservative treatment will generally be used. Usually in young patients, surgical treatment will be performed at puberty to exclude irreversible effects to the bone growth and in the hope that the disease will stabilize with stabilization of the skeletal growth of the patient. The aim of surgical treatment is to reduce pain and bone deformities
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FIGURE 7. Radiograph at last follow-up visit in 2013 showing complete reossification of the defect bone and complete integration of the implants. Petrocelli and Kretschmer. Conservative Treatment and Implant Rehabilitation in CFD. J Oral Maxillofac Surg 2014.
and prevent pathologic fractures.8 Surgical treatment16-18 of CFD has ranged from biopsy to recontouring of bone lesions; remodeling the bone defects using bone grafts, using calvaria or iliac crest bone grafts; reconstruction of large bone defects with free flaps, and bimaxillary osteotomies, reported by Sachs in 1984.3
In our case, in accordance with the surgical protocol from Chen and Noordhoff10 regarding the management of CFD, we treated ‘‘zone 1’’ with radical excision and reconstruction. We removed the tumor from the left orbital and maxillary regions and performed reconstruction using a calvaria bone graft and its fixation by
FIGURE 8. Functional and esthetic results showing a young girl’s smile after a conservative approach to craniofacial fibrous dysplasia and dental implant rehabilitation. Petrocelli and Kretschmer. Conservative Treatment and Implant Rehabilitation in CFD. J Oral Maxillofac Surg 2014.
PETROCELLI AND KRETSCHMER
titanium plates and screws.19,20 Also, we treated ‘‘zone 4’’ using conservative surgical management. We performed a gradual excision of the tumor and reconstruction of the mandible with an iliac crest bone graft21,22 after skeletal growth and disease stabilization. We also decided to perform implant rehabilitation23 to provide good functional and esthetic results in our young patient. We achieved complete reossification of the defect bone and complete integration of the implants, in accordance with the studies by Samman, who, in 1991, demonstrated that the use of titanium materials will result in good integration and histologic adaptation in the dysplastic bone.3 In conclusion, CFD is a rare and singular bone disease, and its surgical treatment remains controversial. We have demonstrated that conservative recontouring of the mandible with gradual excision of the dysplastic bone, reconstruction using an iliac crest bone graft, and, finally, implant rehabilitation at the end of skeletal growth can provide optimal functional and esthetic results (Fig 8) in a young patient, avoiding radical and aggressive surgical treatment and its consequences.
References 1. Barnes L, Eveson JW, Reichart P, Sidranski D: Pathology and Genetics of Head and Neck Tumors: World Health Classification of Tumors. Lyon, IARC Publications, 2005 2. Cawson RA, Binnie WH, Speight PM, et al: Lucas’s Pathology of Tumors of the Oral Tissues. Churchill Livingstone, London, 1998 3. Italian Society of Maxillo Facial Surgery (SICMF). Trattato di patologia chirurgica maxillo-facciale. Torino, Italy, Minerva Medica, 2007, pp 538–543. 4. Lichtenstein L: Polyostotic fibrous dysplasia. Arch Surg 36:874, 1938 5. Ricalde P, Magliocca KR, Lee JS: Craniofacial fibrous dysplasia. Oral Maxillofac Surg Clin North Am 24:427, 2012 6. Menon S, Venkatswamy S, Ramu V, et al: Craniofacial fibrous dysplasia: Surgery and literature review. Ann Maxillofac Surg 3: 66, 2013 7. Park BY, Cheon YW, Kim YO, et al: Prognosis for craniofacial fibrous dysplasia after incomplete resection: Age and serum alkaline phosphatase. J Oral Maxillofac Surg 39:221, 2010
902.e6 8. Valentini V, Cassoni A, Marianetti TM, et al: Craniomaxillofacial fibrous dysplasia: Conservative treatment or radical surgery? A retrospective study on 68 patients. Plast Reconstr Surg 123: 653, 2009 9. Kim DD, Ghali GE, Wright JM, Edwards SP: Surgical treatment of giant fibrous dysplasia of the mandible with concomitant craniofacial involvement. J Oral Maxillofac Surg 70:102, 2012 10. Chen YR, Noordhoff MS: Treatment of craniomaxillofacial fibrous dysplasia: How early and how extensive. Plast Recontr Surg 87:799, 1991 11. Mandrioli S, Carinci F, Dallera V, Calura G: Fibrous dysplasia. The clinicotherapeutic picture and new data on its etiology: A review of the literature. Minerva Stomatol 47:37, 1998 12. Assaf AT, Benecke AW, Riecke B, et al: Craniofacial fibrous dysplasia (CFD) of the maxilla in an 11-year old boy: A case report. J Craniomaxillofac Surg 40:788, 2012 13. Shah ZK, Peh WCG, Koh WL, Shek TWH: Magnetic resonance imaging appearances of fibrous dysplasia. Br J Radiol 78:1104, 2005 14. Abdelkarim A, Green R, Startzell J, Preece J: Craniofacial polyostotic fibrous dysplasia: A case report and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 106: 49, 2008 15. Panda NK, Parida PK, Sharma R, et al: A clinicoradiologic analysis of symptomatic craniofacial fibro-osseous lesions. Otolaryngol Head Neck Surg 136:928, 2007 16. Kruse A, Pieles U, Riener MO, et al: Craniomaxillofacial fibrous dysplasia: A 10-year database 1996-2006. Br J Oral Maxillofac Surg 47:302, 2009 17. Bequignon E, Cardinne C, Lachiver X, et al: Craniofacial fibrous dysplasia surgery: A functional approach. Eur Ann Otorhinolaryngol Head Neck Dis Epub 2013 Jul 18 18. Vegas Bustamante E, Gargallo Albiol J, Berini Aytes L, Gay Escoda C: Benign fibro-osseous lesions of the maxillas: Analysis of 11 cases. Med Oral Patol Oral Cir Bucal 13:E653, 2008 19. Stoler A, Hill T: Part I: Reconstruction after total mandibulectomy with free cranial and microvascular iliac crest grafts as preparation for implants. J Oral Implantol 18:38, 1992 20. Stoler A, Hill T: Part II. Mandibular reconstruction: combined intra-oral and in vitro placement of osseointegrated implants into a free and vascularized bone graft. J Oral Implantol 18:45, 1992 21. Mendonc¸a Caridad JJ, Platas F Jr: Fibrous dysplasia of the mandible: Surgical treatment with platelet-rich plasma and a corticocancellous iliac crest graft—Report of a case. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 105:e12, 2008 22. Obiechina AE, Ogunlade SO, Fasola AO, Arotiba JT: Mandibular segmental reconstruction with iliac crest. West Afr J Med 22: 46, 2003 23. Listrom RD, Symington JM: Osseointegrated dental implants in conjunction with bone grafts. Int J Oral Maxillofac Surg 17: 116, 1988
