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P.032 The simultaneous removing of TMJ ankylosis and distraction osteogenesis correction of mandibular deformities in childhood
S176
Journal of Cranio-Maxillofacial Surgery 36(2008) Suppl. 1
5 cases, the asymmetry was in the vertical plan and in 2 cases the asymmetry was both transverse and vertical. The high condylectomy was performed in all cases. Five patients had acceptable facial symmetry and satisfactory occlusion after a mean follow up of 1 year. In the remaining cases, a residual deformity was observed needing a bimaxillary osteotomy following orthodontic treatment in 1 case and a genioplasty in the other case. Conclusions: Condylar hyperplasia is more prevalent in females and the age of onset is late puberty. The high condylectomy combined to orthodontic treatment can produce good aesthetic and functional results in AUCH. Orthognatic surgery is needed in severe cases. P.029 Congenital muscular torticollis: a study of 7 cases O. Monia, R. Moatemri, T. Tayeb, Z. Bellalah, Z. Mziou, S. Ayachi, A. Slama, H. Khochtali. Sehloul Hospital, Sousse, Tunisia Objective: Discussion of the therapeutic aspects of congenital muscular torticollis (CMT). Methods: This is a retrospective study of 7 CMT cases of patients (3 females and 4 males, aged 2 to 21 years) treated at the Maxillofacial Surgery Department of Sahloul in Tunisia from 2002 to 2007. A limitation in passive rotation of the affected side was more than 30º in 4 cases and between 15 and 30º in 3 cases. Surgical technique consists of bipolar tenotomy (3 cases) or lenthning myoplasty (4 cases). In post operatively period a collar was placed for 4 to 6 weeks in all cases and all patients were treated by physiotherapy. Results: A full passive motion of the neck was obtained in 5 cases in contrast with a persistant limitation less than 15º in 2 cases. A total correction of initial asymetry was seen in 6 cases. In terms of cosmesis, all our patients had acceptable fine scars. None of our patients showed a relapse of the torticollis during the follow up period. Conclusions: Physical examination and standard radiographs are sufficient to insure the diagnosis of CMT and for therapeutic planning. If it is diagnosed in early childhood a conservative treatment can be proposed. Patients over the age of 1 year and those with cord-like torticollis and rotational deficits in excess of 30º usually require surgical treatment. We prefer myoplasty lenthning technique because it preserves a good sternocleidomastoid muscular relief. P.030 Infiltrating lipomatosis of the face: report of three cases K. Dounia, D. Kamal, I. Tougui, P. Breton, P. Bouletreau. Department of Stomatology, Maxillo-FacialSurgery and Plastic Surgery of the Face, Centre Hospitalier Lyon-Sud, Lyon, France Objectives: Three new cases of hemifacial hypertrophy caused by congenital infiltrating lipomatosis of the face are reported. The literature on infiltrating Lipomatosis of the face is reviewed with an emphasis on accompanying anomalies and treatment strategies. Report of cases: In this study, the clinical features, radiographic findings, histopathology, and postoperative results were analyzed in 3 patients with facial infiltrating lipomatosis. The condition was diagnosed in infancy (one male subject, two female subjects) and characterized by enlargement of the cheek. Most patients had early eruption of ipsilateral deciduous and permanent teeth. Computed tomography and magnetic resonance imaging showed an infiltrated soft-tissue mass of fatty density and skeletal overgrowth. Multiple resections were performed on two patients. Discussion: Described first by Slavin and colleagues in 1989, Facial infiltrating lipomatosis is a rare congenital disorder in which mature lipocytes invade adjacent tissue. The phenotypic features include soft-tissue and skeletal hypertrophy, premature
Abstracts, EACMFS XIX Congress dental eruption, and regional macrodontia. Due to its diffuse infiltration and involvement of important facial structures, complete surgical excision is often impossible. The etiology, natural history, optimal management, and relationship to other disorders of fatty overgrowth are unclear. Because surgical removal of the mass is usually unsuccessful, specific management of this condition will require insight into its etiopathogenesis.
P.031 Strained-deformed mandible condition in partial resection O.P. Chudakov, S.A. Naumovich, F.G. Drik, V. Charanda. BSMU, Minsk, Belarus Objectives: to study the strained-deformed conditions of the mandible in case of resection of the part of its body. Materials and Methods: the method of laser holographic interferometry in counter-current bundles was used. Mandible defect was modeled by block resection of the body 3×4 cm (2/3 of its height) in the molar region. Results: In frontal loads resected mandible displays the orientation of stripes similar to the normal on the non-resected side. The frequency of stripes on the resected side is about 2 times higher, thus an asymmetry (i.e. a deformation warp) is evident. In lateral scanning the dynamics of the stripes system is significantly different. Dynamics in he front part and the remaining bone below the defect is similar to the condition of the integral mandible. The ramus and the angle of the mandible displays just some parallel strips displacement, with the most intensive displacement in the distal defect anle. In lateral loads on the overdenture both sides of the resecton defect stripe systems are orthogonal and show just some parallel displacement with fluent hange on the bone fragment below the defect. Discussion: This shows that pathologic weakening of the mandible structure caused the displacement of the rotation axis to this angle of the defect and the bone structure in this region carrries the highest deformation strains in functional loads.
P.032 The simultaneous removing of TMJ ankylosis and distraction osteogenesis correction of mandibular deformities in childhood A. Lopatin, S. Yasonov. Department for Craniomaxollo-Facial Surgery, All-Russian Children’s Hospital, Moscow, Russia Aims: to present the results of simultaneous removing of ankylosis of TMJ and distraction osteogenesis correction of mandibular deformities in childhood. Material and Methods: Eighteen patients aged 3-12 years with unilateral ankylosis of TMJ and related deformities of mandible underwent simultaneous surgical ankylosis removal and unilateral distraction osteogenesis of the vertical ramus of the mandible using the unidirectional intraoral device (KLS Martin). CT scans were analysed by the software Surgicase (Materialise) and determine the placement of the mandibular osteotomy and select the appropriate placement of distractor device. After latency period (3 days), distraction is begun following a standart protocoll until the desired length of distraction is obtained. Simultaneous with distraction was used the mechanotherapy to prevent of the rekankylosis. Six months after distraction the device is removed. The evaluation included 3D-CT, panoramic radiographs, dental models and facial photographs before and 6, 12 and 24 months after treatment. Results: Total distraction distances ranged from 10-15.5 mm with a mean lengthening distance of 12.4 mm. Radiografic and CT scan evaluation demonstrated progressive ossification of the distraction regenerate with no evidence of relapse. Excellent stability of distraction regenerate was noted at the time of device removal. The clinical investigation showed the full volume of mandibular movement is obtained.
Posters Conclusion: The simultaneous surgical TMJ ankylosis removing and mandibular distraction osteogenesis has proved to be excellent technique for the treatment of this group of patient. Careful treatment planning, control of vector of distraction, active mechanotherapy and orthodontic treatment during the consolidation period is the way to achive satisfactory stability results.
Benign lesions of the facial bones P.033 Aneurysmal bone cyst of the clivus. A midfacial translocation L. Garcia Monleon, J. Acero, M. Coll, V. Perez, S. Ochandiano, C. Navarro Vila. Hospital General Universitario Gregorio Mara˜no´ n, Madrid, Spain Introduction: Aneurysmal bone cyst (ABC) is an uncommon lesion that occurs mainly in long bones and vertebrae, being the location in the skull base extremely rare. Case report: A young man pressented with nasal obstruction, headache and nasal voice. A CT showed a massive tumor of heterogeneous nature occupying the nasopharynx and the anterior cranial base. The MR revealed a multicystic, infrasellar lesion, probably originated in the clivus. Angiography showed an avascular tumor. No endovascular embolization was performed because of the lack of predominant branches feeding the tumor. A biopsy of the sphenoid sinus was performed with the result of ABC. The tumor was excised through a bilateral midfacial translocation. Histological examination confirmed an ABC with no evidence of malignancy. Discussion: Aneurysmal bone cyst is a benign, non-neoplastic lesion that destroys the bony architecture. It represents 1−2% of all bone tumors. This type of lesions are very rare in the craniofacial region (3%). Histological examination shows blood-filled spaces surrounded by compressed fibrous tissue. Multinucleated giant cells of the osteoclast type are also common. Radiography of ABC usually shows an expansile cystic lesion with a honey comb appearance and a thin cortex of newly formed bone surrounding the lesion. Total resection of the lesion is associated with cure and an excellent long-term outcome. Radiotherapy is usually reserved for the recurrent cases, because of the risk of secondary sarcomas. Conclusion: ABC involving the clivus is a rare entity. Total surgical extirpation seems to be the best available treatment option with excellent long term results. A rational surgical approach is critical to achieve complete resection of the lesion. P.034 Diagnosis of intraosseous jaw lesions by aspirative cytology E. Iane¸s1 , F. Streian1 , L. Vasile2 . 1 Department of Oral & Maxillofacial Surgery, 2 Department of Histopathology and Cytology University of Medicine and Pharmacy Victor Babes Timisoara, Romania Purpose: The primary intraosseous jaw lesion are challenging because of their phenotypique diversity. Cytodiagnosis by fine needle aspiration (FNA) technique is a valuable evaluation method of the tumours vs. tumour-like conditions of intraosseous jaw lesions. Material and Method: Our study included 57 FNA of intraosseous jaw lesions performed at the Oral and Maxillofacial Clinic in Timi¸soara, between 2003–2007. Each case was evaluated for adequacy of sampled material, presence of malignant cells and correlation between cytodiagnosis and final histopathology. Cytodiagnosis was correlated with histopathology through usual and selective histochemical methods. Results: Aspirated samples were adequate for evaluation and allowed cytodiagnosis in 54 out of 57 cases. In 10 out of the 54 cases, malignant cells were found. Cytodiagnosis of
Benign lesions of the facial bones
S177
malignancy was confirmed by histopathology in all of these 10 cases (100% accuracy). Cytodiagnosis of benign lesions was confirmed in 29 out of 44 cases (65.90%). Diagnosis difficulties in distinguishing between benign and malign were met in odontogenic tumors. Diagnosis errors were related to material adequacy criteria, sampling technique and cytological interpretation. Conclusions: Differentiation is prerequisite to treatment because the procedure differs in each lesion. FNA is a useful technique in obtaining a cytodiagnosis which allows to distinguish between malignant and benign intraosseous jaw lesions. It is a simple and quick technique, suitable for outpatient procedure, in the initial diagnosis and the follow-up of the patients. P.035 Endoscopic treatment of odontogenic keratocystic tumour F. Giovannetti, A. Cassoni, T. Matteo Marianetti, P. Priore, V. Valentini. Maxillo-Facial Surgery Dept, Policlinico Umberto I, Sapienza University of Rome, Italy Objectives: The keratocyst is a jaw cyst now called more properly odontogenic keratocystic tumour because of his proclivity for local invasion and recurrence. The treatment of these lesions is controversial and includes conservative (marsupialization) and radical approaches. We present a case of keratocystic tumour involving the mandibular ramus and condyle treated with combined open and endoscopic approach. Methods: We propose a new surgical technique in which the use of angled endoscopic optiques and instrumentation can provide the complete enucleation of the tumour and the radicalization of the bone surrounding the tumour with angled drill. Results: A radical treatment can be obtained even in keratocystic tumours involving the mandibular ramus and condyle, in which most Authors prefer more conservative treatment because of poor intraoperative visibility and great difficulty to completely remove these lesions. Post-operative imaging showed no evidence of residual tumor. Conclusion: The endoscopy has modified several therapeutic approaches to maxillofacial pathologies. His application in keratocystic tumours in areas more difficult to reach can be very useful in order to obtain a radical treatment of the lesion. P.036 Fibrous dysplasia and tuberous sclerosis S. Gonzalez, L. Garcia Monleon, J. Lopez de Atalaya, J. Verdaguer, Jl. Barrios, C. Navarro Vila. Hospital General Universitario Gregorio Mara˜no´ n, Madrid, Spain Introduction: Tuberous sclerosis (TS) is a neurocutaneous syndrome classically characterized by a clinical triad of epileptic seizures, mental retardation, and skin hamartomas. Osseous manifestations are very frequent and several types of skull implication have been described, but its association with fibrous dysplasia (FD) is unusual. Case report: A 13-year-old patient with TS pressented with an enlarging mass in her left maxilomalar area. The CT revealed osseous abnormalities on the left maxilar and temporal bones with ground-glass appearance and cystic pattern, which are typical of FD. A biopsy of the maxilar bone confirmed this diagnosis. Because of the minimal clinical deformity and early age of the patient, a conservative attitude was taken. Discussion: TS is a neurocutaneous syndrome with frequent skeletal abnormalities (hyperostosis of the calvaria, osteoblastic or osteosclerotic changes in the spine, cystic changes in the phalanges). Our patient pressented with several clinical signs of TS (subcortical hamartomas, facial angiofibromas, epileptic seizures) and FD affecting the left maxilar and temporal bones. The FD is a developmental anomaly in which normal bone marrow is replaced by fibro-osseous tissue, usually affecting adolescents and young adults. FD may affect one bone (70%) or several bones (27%). The poliostotic variant is associated in 3%
Journal of Cranio-Maxillofacial Surgery 36(2008) Suppl. 1
5 cases, the asymmetry was in the vertical plan and in 2 cases the asymmetry was both transverse and vertical. The high condylectomy was performed in all cases. Five patients had acceptable facial symmetry and satisfactory occlusion after a mean follow up of 1 year. In the remaining cases, a residual deformity was observed needing a bimaxillary osteotomy following orthodontic treatment in 1 case and a genioplasty in the other case. Conclusions: Condylar hyperplasia is more prevalent in females and the age of onset is late puberty. The high condylectomy combined to orthodontic treatment can produce good aesthetic and functional results in AUCH. Orthognatic surgery is needed in severe cases. P.029 Congenital muscular torticollis: a study of 7 cases O. Monia, R. Moatemri, T. Tayeb, Z. Bellalah, Z. Mziou, S. Ayachi, A. Slama, H. Khochtali. Sehloul Hospital, Sousse, Tunisia Objective: Discussion of the therapeutic aspects of congenital muscular torticollis (CMT). Methods: This is a retrospective study of 7 CMT cases of patients (3 females and 4 males, aged 2 to 21 years) treated at the Maxillofacial Surgery Department of Sahloul in Tunisia from 2002 to 2007. A limitation in passive rotation of the affected side was more than 30º in 4 cases and between 15 and 30º in 3 cases. Surgical technique consists of bipolar tenotomy (3 cases) or lenthning myoplasty (4 cases). In post operatively period a collar was placed for 4 to 6 weeks in all cases and all patients were treated by physiotherapy. Results: A full passive motion of the neck was obtained in 5 cases in contrast with a persistant limitation less than 15º in 2 cases. A total correction of initial asymetry was seen in 6 cases. In terms of cosmesis, all our patients had acceptable fine scars. None of our patients showed a relapse of the torticollis during the follow up period. Conclusions: Physical examination and standard radiographs are sufficient to insure the diagnosis of CMT and for therapeutic planning. If it is diagnosed in early childhood a conservative treatment can be proposed. Patients over the age of 1 year and those with cord-like torticollis and rotational deficits in excess of 30º usually require surgical treatment. We prefer myoplasty lenthning technique because it preserves a good sternocleidomastoid muscular relief. P.030 Infiltrating lipomatosis of the face: report of three cases K. Dounia, D. Kamal, I. Tougui, P. Breton, P. Bouletreau. Department of Stomatology, Maxillo-FacialSurgery and Plastic Surgery of the Face, Centre Hospitalier Lyon-Sud, Lyon, France Objectives: Three new cases of hemifacial hypertrophy caused by congenital infiltrating lipomatosis of the face are reported. The literature on infiltrating Lipomatosis of the face is reviewed with an emphasis on accompanying anomalies and treatment strategies. Report of cases: In this study, the clinical features, radiographic findings, histopathology, and postoperative results were analyzed in 3 patients with facial infiltrating lipomatosis. The condition was diagnosed in infancy (one male subject, two female subjects) and characterized by enlargement of the cheek. Most patients had early eruption of ipsilateral deciduous and permanent teeth. Computed tomography and magnetic resonance imaging showed an infiltrated soft-tissue mass of fatty density and skeletal overgrowth. Multiple resections were performed on two patients. Discussion: Described first by Slavin and colleagues in 1989, Facial infiltrating lipomatosis is a rare congenital disorder in which mature lipocytes invade adjacent tissue. The phenotypic features include soft-tissue and skeletal hypertrophy, premature
Abstracts, EACMFS XIX Congress dental eruption, and regional macrodontia. Due to its diffuse infiltration and involvement of important facial structures, complete surgical excision is often impossible. The etiology, natural history, optimal management, and relationship to other disorders of fatty overgrowth are unclear. Because surgical removal of the mass is usually unsuccessful, specific management of this condition will require insight into its etiopathogenesis.
P.031 Strained-deformed mandible condition in partial resection O.P. Chudakov, S.A. Naumovich, F.G. Drik, V. Charanda. BSMU, Minsk, Belarus Objectives: to study the strained-deformed conditions of the mandible in case of resection of the part of its body. Materials and Methods: the method of laser holographic interferometry in counter-current bundles was used. Mandible defect was modeled by block resection of the body 3×4 cm (2/3 of its height) in the molar region. Results: In frontal loads resected mandible displays the orientation of stripes similar to the normal on the non-resected side. The frequency of stripes on the resected side is about 2 times higher, thus an asymmetry (i.e. a deformation warp) is evident. In lateral scanning the dynamics of the stripes system is significantly different. Dynamics in he front part and the remaining bone below the defect is similar to the condition of the integral mandible. The ramus and the angle of the mandible displays just some parallel strips displacement, with the most intensive displacement in the distal defect anle. In lateral loads on the overdenture both sides of the resecton defect stripe systems are orthogonal and show just some parallel displacement with fluent hange on the bone fragment below the defect. Discussion: This shows that pathologic weakening of the mandible structure caused the displacement of the rotation axis to this angle of the defect and the bone structure in this region carrries the highest deformation strains in functional loads.
P.032 The simultaneous removing of TMJ ankylosis and distraction osteogenesis correction of mandibular deformities in childhood A. Lopatin, S. Yasonov. Department for Craniomaxollo-Facial Surgery, All-Russian Children’s Hospital, Moscow, Russia Aims: to present the results of simultaneous removing of ankylosis of TMJ and distraction osteogenesis correction of mandibular deformities in childhood. Material and Methods: Eighteen patients aged 3-12 years with unilateral ankylosis of TMJ and related deformities of mandible underwent simultaneous surgical ankylosis removal and unilateral distraction osteogenesis of the vertical ramus of the mandible using the unidirectional intraoral device (KLS Martin). CT scans were analysed by the software Surgicase (Materialise) and determine the placement of the mandibular osteotomy and select the appropriate placement of distractor device. After latency period (3 days), distraction is begun following a standart protocoll until the desired length of distraction is obtained. Simultaneous with distraction was used the mechanotherapy to prevent of the rekankylosis. Six months after distraction the device is removed. The evaluation included 3D-CT, panoramic radiographs, dental models and facial photographs before and 6, 12 and 24 months after treatment. Results: Total distraction distances ranged from 10-15.5 mm with a mean lengthening distance of 12.4 mm. Radiografic and CT scan evaluation demonstrated progressive ossification of the distraction regenerate with no evidence of relapse. Excellent stability of distraction regenerate was noted at the time of device removal. The clinical investigation showed the full volume of mandibular movement is obtained.
Posters Conclusion: The simultaneous surgical TMJ ankylosis removing and mandibular distraction osteogenesis has proved to be excellent technique for the treatment of this group of patient. Careful treatment planning, control of vector of distraction, active mechanotherapy and orthodontic treatment during the consolidation period is the way to achive satisfactory stability results.
Benign lesions of the facial bones P.033 Aneurysmal bone cyst of the clivus. A midfacial translocation L. Garcia Monleon, J. Acero, M. Coll, V. Perez, S. Ochandiano, C. Navarro Vila. Hospital General Universitario Gregorio Mara˜no´ n, Madrid, Spain Introduction: Aneurysmal bone cyst (ABC) is an uncommon lesion that occurs mainly in long bones and vertebrae, being the location in the skull base extremely rare. Case report: A young man pressented with nasal obstruction, headache and nasal voice. A CT showed a massive tumor of heterogeneous nature occupying the nasopharynx and the anterior cranial base. The MR revealed a multicystic, infrasellar lesion, probably originated in the clivus. Angiography showed an avascular tumor. No endovascular embolization was performed because of the lack of predominant branches feeding the tumor. A biopsy of the sphenoid sinus was performed with the result of ABC. The tumor was excised through a bilateral midfacial translocation. Histological examination confirmed an ABC with no evidence of malignancy. Discussion: Aneurysmal bone cyst is a benign, non-neoplastic lesion that destroys the bony architecture. It represents 1−2% of all bone tumors. This type of lesions are very rare in the craniofacial region (3%). Histological examination shows blood-filled spaces surrounded by compressed fibrous tissue. Multinucleated giant cells of the osteoclast type are also common. Radiography of ABC usually shows an expansile cystic lesion with a honey comb appearance and a thin cortex of newly formed bone surrounding the lesion. Total resection of the lesion is associated with cure and an excellent long-term outcome. Radiotherapy is usually reserved for the recurrent cases, because of the risk of secondary sarcomas. Conclusion: ABC involving the clivus is a rare entity. Total surgical extirpation seems to be the best available treatment option with excellent long term results. A rational surgical approach is critical to achieve complete resection of the lesion. P.034 Diagnosis of intraosseous jaw lesions by aspirative cytology E. Iane¸s1 , F. Streian1 , L. Vasile2 . 1 Department of Oral & Maxillofacial Surgery, 2 Department of Histopathology and Cytology University of Medicine and Pharmacy Victor Babes Timisoara, Romania Purpose: The primary intraosseous jaw lesion are challenging because of their phenotypique diversity. Cytodiagnosis by fine needle aspiration (FNA) technique is a valuable evaluation method of the tumours vs. tumour-like conditions of intraosseous jaw lesions. Material and Method: Our study included 57 FNA of intraosseous jaw lesions performed at the Oral and Maxillofacial Clinic in Timi¸soara, between 2003–2007. Each case was evaluated for adequacy of sampled material, presence of malignant cells and correlation between cytodiagnosis and final histopathology. Cytodiagnosis was correlated with histopathology through usual and selective histochemical methods. Results: Aspirated samples were adequate for evaluation and allowed cytodiagnosis in 54 out of 57 cases. In 10 out of the 54 cases, malignant cells were found. Cytodiagnosis of
Benign lesions of the facial bones
S177
malignancy was confirmed by histopathology in all of these 10 cases (100% accuracy). Cytodiagnosis of benign lesions was confirmed in 29 out of 44 cases (65.90%). Diagnosis difficulties in distinguishing between benign and malign were met in odontogenic tumors. Diagnosis errors were related to material adequacy criteria, sampling technique and cytological interpretation. Conclusions: Differentiation is prerequisite to treatment because the procedure differs in each lesion. FNA is a useful technique in obtaining a cytodiagnosis which allows to distinguish between malignant and benign intraosseous jaw lesions. It is a simple and quick technique, suitable for outpatient procedure, in the initial diagnosis and the follow-up of the patients. P.035 Endoscopic treatment of odontogenic keratocystic tumour F. Giovannetti, A. Cassoni, T. Matteo Marianetti, P. Priore, V. Valentini. Maxillo-Facial Surgery Dept, Policlinico Umberto I, Sapienza University of Rome, Italy Objectives: The keratocyst is a jaw cyst now called more properly odontogenic keratocystic tumour because of his proclivity for local invasion and recurrence. The treatment of these lesions is controversial and includes conservative (marsupialization) and radical approaches. We present a case of keratocystic tumour involving the mandibular ramus and condyle treated with combined open and endoscopic approach. Methods: We propose a new surgical technique in which the use of angled endoscopic optiques and instrumentation can provide the complete enucleation of the tumour and the radicalization of the bone surrounding the tumour with angled drill. Results: A radical treatment can be obtained even in keratocystic tumours involving the mandibular ramus and condyle, in which most Authors prefer more conservative treatment because of poor intraoperative visibility and great difficulty to completely remove these lesions. Post-operative imaging showed no evidence of residual tumor. Conclusion: The endoscopy has modified several therapeutic approaches to maxillofacial pathologies. His application in keratocystic tumours in areas more difficult to reach can be very useful in order to obtain a radical treatment of the lesion. P.036 Fibrous dysplasia and tuberous sclerosis S. Gonzalez, L. Garcia Monleon, J. Lopez de Atalaya, J. Verdaguer, Jl. Barrios, C. Navarro Vila. Hospital General Universitario Gregorio Mara˜no´ n, Madrid, Spain Introduction: Tuberous sclerosis (TS) is a neurocutaneous syndrome classically characterized by a clinical triad of epileptic seizures, mental retardation, and skin hamartomas. Osseous manifestations are very frequent and several types of skull implication have been described, but its association with fibrous dysplasia (FD) is unusual. Case report: A 13-year-old patient with TS pressented with an enlarging mass in her left maxilomalar area. The CT revealed osseous abnormalities on the left maxilar and temporal bones with ground-glass appearance and cystic pattern, which are typical of FD. A biopsy of the maxilar bone confirmed this diagnosis. Because of the minimal clinical deformity and early age of the patient, a conservative attitude was taken. Discussion: TS is a neurocutaneous syndrome with frequent skeletal abnormalities (hyperostosis of the calvaria, osteoblastic or osteosclerotic changes in the spine, cystic changes in the phalanges). Our patient pressented with several clinical signs of TS (subcortical hamartomas, facial angiofibromas, epileptic seizures) and FD affecting the left maxilar and temporal bones. The FD is a developmental anomaly in which normal bone marrow is replaced by fibro-osseous tissue, usually affecting adolescents and young adults. FD may affect one bone (70%) or several bones (27%). The poliostotic variant is associated in 3%