crouzon syndrome: case report

1
Cowdens syndrome is a rare autosomal-dominant condition characterized by the presence of multiple mucocutaneous hamartomas, gastrointestinal polyps, and breast neoplasms. This report describes a case in which oral lesions were responsible for the diagnosis. Woman, 34, had a history of benign nodules of the thyroid and breast and came for an evaluation of her oral lesions. Cutaneous examination revealed papules in the nasal region, verrucous growths in the axillary region, and palmoplantar keratosis. Intraoral examination showed several papules in the gingiva and tongue. Histological analysis of the oral lesions was compatible with broepithelial hyperplasias. The diagnosis of Cowdens syndrome was reached after analyzing the intraoral ndings, cutaneous involvement and previous medical history. This case highlights the importance of the stomatologist in diagnosing this syndrome, which is characterized by a high prevalence of malignant tumors, requiring routine follow-up examinations of the affected patients. PE-096 - CRANIOFACIAL FIBROUS DYSPLASIA IN PE- DIATRIC PATIENT. MAIZA LUIZA VIEIRA SILVA, TÂNIA MARA PIMENTA AMARAL, MARIA BEATRIZ PIRES DE MAGALHÃES, HENRIQUE PRETTI, RICARDO ALVES MESQUITA. FACULDADE DE ODONTOLOGIA - UNI- VERSIDADE FEDERAL DE MINAS GERAIS. Fibrous dysplasia (FD) is a non-neoplastic condition in which normal bone marrow is replaced by bro-osseous tissue. Caucasian girl, 8, was referred to School of Dentistry of UFMG for evaluation of swelling on her face developing over a period of 4 years. Clinical examination found extraoral facial asymmetry. Imaging showed a radiopaque image with ground glass; displacement of the roots of teeth #43, #44, and #45; and expansion of cortical bone lingually and buccally. The diagnosis based on the clinical-radio- graphic ndings was FD, which was conrmed on histopathologic analysis. Cone-beam computed tomography revealed involvement of the mandible, zygomatic bone, and sphenoid bone. Serum phosphorus level was increased. The denitive diagnosis was craniofacial FD. The patient is undergoing treatment by her physician and orthodontist. The diagnosis and management of pediatric patients with DF are important to their quality of life (CNPq #309209/2010-2; 472045/2011-3, FAPEMIG). PE-097 - CROUZON SYNDROME: CASE REPORT. CAROLINE FARIAS LEMOS, NAYANE CARVALHO CHAGAS, BERNARDO FERREIRA BRASILEIRO, LUIZ CARLOS FERREIRA DA SILVA, MARTA RABELLO PIVA. UNIVERSIDADE FEDERAL DE SERGIPE. Crouzon syndrome is a rare condition that affects the devel- opment of the craniofacial skeleton and, although uncommon, has a 50% risk of transmission when one parent has the disease. Man, 19, was diagnosed with SC at age 12 years, but refused surgical treatment. He was returning with complaints of tooth pain and difculty opening the mouth. Physical examination revealed exophthalmos, attening of the occipital protuberance of the frontal and occipital bones, prognathism, and deviated septum. Clinical examination noted intraoral malocclusion, narrowing of the hard palate, limited mouth opening, and several carious lesions. Treatment will require a multidisciplinary approach, including physiotherapy, TMA surgery, and dental procedures. PE-098 - DELAYED INTRAORAL FOREIGN BODY REAC- TION TO POLYMETHYLMETHACRYLATE. STEPHANIE DE ALMEIDA, FRANCISCO SAMUEL RODRIGUES CARVALHO, FILIPE NOBRE CHAVES, EVELINE TURATTI, THYCIANA RODRIGUES RIBEIRO, KARUZA MARIA ALVES PEREIRA, FÁBIO WILDSON GURGEL COSTA. UNIVERSIDADE FEDERAL DO CEARÁ - CAMPUS SOBRAL. Facial rejuvenation aims to restore facial volume and contour through the use of dermal ller materials. Patient, 57, complained of facial swelling followed by the rapid development of bilateral non-movable nodules in the oral mucosa. During the history, the patient reported silicone dermal ller application (poly- methylmethacrylate) next to the oral commissure 9 years prior to this visit. Incisional biopsy was performed, and oral corticosteroid therapy instituted. Histopathological analysis revealed fragments of tissue showing vacuoles, consistent with exogenous material, permeated by mononuclear inammatory inltrate. A clinical approach without surgical intervention was chosen. Because adverse reactions may occur after a long period, it is necessary to carry out long-term monitoring and a meticulous history acqui- sition, especially because patients may be averse to reporting the use of aesthetic treatment modalities. PE-099 - DENTAL CARE OF PATIENTS WITH EXTRA- NODAL NASAL LYMPHOMA CELL T/NK TYPE DURING ANTICANCER TREATMENT. DAIANE TENOR LOPES, LILIANE CRISTINE FERREIRA DE SOUZA, PERLA PORTO LEITE SHITARA, TOMIO IWATA, LUIS ANTÔNIO PIRES, GILSON LUCHEZI DELGADO, MARCO VINICIUS CHAUD. UNIVERSIDADE DE SOROCABA (UNISO). Neoplasms of mature T cells and NK cells are relatively uncommon and have unknown causes. Extranodal lymphoma of the nasal type is characterized by broad-spectrum morphological angiocentric inltration, vascular destruction, and necrosis and is more common in adult men. Patient, 42, had diabetes mellitus type I and was a former smoker undergoing anticancer treatment due to T-cell lymphoma/nasal-type extranodal NK refractory to anticancer treatment. The patient was referred with symptoms of oral mucositis to the service of Dentistry Special Patients of the Hospital of Sorocaba. A circular lesion with raised edges and hardened and painful symptoms in the apex of the tongue were noted. Histopathological analysis showed granulation tissue and atypical lymphatic inltration. Immunohistochemical analysis diagnosed lymphocytic inltrate with a predominance of small lymphocytes and plasma cells. The multidisciplinary interaction allowed the patient to be treated with medical and dental support and achieve improved quality of life. PE-100 - DENTAL MANAGEMENT OF PATIENTS WITH LIVER DISEASES: CASE REPORT. HELOÍSA LAÍS ROSARIO DOS SANTOS, DAVI SILVA CARVALHO CURI, THAMIRES SILVA SOUZA, ANDRÉ LUCAS DALMEIDA LYRIO DOS SANTOS, ANTÔNIO FERNANDO PEREIRA FALCÃO, VIVIANE ALMEIDA SARMENTO, PATRÍCIA LEITE RIBEIRO LAMBERTI. FEDERAL UNIVERSITY OF BAHIA. The liver is responsible for multiple functions, such as the synthesis of coagulation factors and drug metabolism. These features should be considered when planning the dental treatment of patients with liver dysfunction. This study reports the dental management of a patient with liver cirrhosis who required mul- tiple extractions. After evaluation and discussing the case with the medical staff, the surgical procedure was performed in two stages using fresh frozen plasma, local hemostatic measures, and anti- biotic prophylaxis. The dental management in these situations is discussed. OOOO ABSTRACTS Volume 117, Number 2 Abstracts e151

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Page 1: Crouzon Syndrome: Case Report

OOOO ABSTRACTS

Volume 117, Number 2 Abstracts e151

Cowden’s syndrome is a rare autosomal-dominant conditioncharacterized by the presence of multiple mucocutaneoushamartomas, gastrointestinal polyps, and breast neoplasms. Thisreport describes a case in which oral lesions were responsible forthe diagnosis. Woman, 34, had a history of benign nodules of thethyroid and breast and came for an evaluation of her oral lesions.Cutaneous examination revealed papules in the nasal region,verrucous growths in the axillary region, and palmoplantarkeratosis. Intraoral examination showed several papules in thegingiva and tongue. Histological analysis of the oral lesions wascompatible with fibroepithelial hyperplasias. The diagnosis ofCowden’s syndrome was reached after analyzing the intraoralfindings, cutaneous involvement and previous medical history.This case highlights the importance of the stomatologist indiagnosing this syndrome, which is characterized by a highprevalence of malignant tumors, requiring routine follow-upexaminations of the affected patients.

PE-096 - CRANIOFACIAL FIBROUS DYSPLASIA IN PE-DIATRIC PATIENT. MAIZA LUIZA VIEIRA SILVA, TÂNIAMARA PIMENTA AMARAL, MARIA BEATRIZ PIRES DEMAGALHÃES, HENRIQUE PRETTI, RICARDO ALVESMESQUITA. FACULDADE DE ODONTOLOGIA - UNI-VERSIDADE FEDERAL DE MINAS GERAIS.

Fibrous dysplasia (FD) is a non-neoplastic condition in whichnormal bone marrow is replaced by fibro-osseous tissue. Caucasiangirl, 8, was referred to School of Dentistry of UFMG for evaluationof swelling on her face developing over a period of 4 years.Clinical examination found extraoral facial asymmetry. Imagingshowed a radiopaque image with “ground glass”; displacement ofthe roots of teeth #43, #44, and #45; and expansion of cortical bonelingually and buccally. The diagnosis based on the clinical-radio-graphic findings was FD, which was confirmed on histopathologicanalysis. Cone-beam computed tomography revealed involvementof the mandible, zygomatic bone, and sphenoid bone. Serumphosphorus level was increased. The definitive diagnosis wascraniofacial FD. The patient is undergoing treatment by herphysician and orthodontist. The diagnosis and management ofpediatric patients with DF are important to their quality of life(CNPq #309209/2010-2; 472045/2011-3, FAPEMIG).

PE-097 - CROUZON SYNDROME: CASE REPORT.CAROLINE FARIAS LEMOS, NAYANE CARVALHOCHAGAS, BERNARDO FERREIRA BRASILEIRO, LUIZCARLOS FERREIRA DA SILVA, MARTA RABELLO PIVA.UNIVERSIDADE FEDERAL DE SERGIPE.

Crouzon syndrome is a rare condition that affects the devel-opment of the craniofacial skeleton and, although uncommon, hasa 50% risk of transmission when one parent has the disease. Man,19, was diagnosed with SC at age 12 years, but refused surgicaltreatment. He was returning with complaints of tooth pain anddifficulty opening the mouth. Physical examination revealedexophthalmos, flattening of the occipital protuberance of thefrontal and occipital bones, prognathism, and deviated septum.Clinical examination noted intraoral malocclusion, narrowing ofthe hard palate, limited mouth opening, and several carious lesions.Treatment will require a multidisciplinary approach, includingphysiotherapy, TMA surgery, and dental procedures.

PE-098 - DELAYED INTRAORAL FOREIGN BODY REAC-TION TO POLYMETHYLMETHACRYLATE. STEPHANIEDE ALMEIDA, FRANCISCO SAMUEL RODRIGUES

CARVALHO, FILIPE NOBRE CHAVES, EVELINE TURATTI,THYCIANA RODRIGUES RIBEIRO, KARUZA MARIAALVES PEREIRA, FÁBIO WILDSON GURGEL COSTA.UNIVERSIDADE FEDERAL DO CEARÁ - CAMPUS SOBRAL.

Facial rejuvenation aims to restore facial volume and contourthrough the use of dermal filler materials. Patient, 57, complainedof facial swelling followed by the rapid development of bilateralnon-movable nodules in the oral mucosa. During the history, thepatient reported silicone dermal filler application (poly-methylmethacrylate) next to the oral commissure 9 years prior tothis visit. Incisional biopsy was performed, and oral corticosteroidtherapy instituted. Histopathological analysis revealed fragmentsof tissue showing vacuoles, consistent with exogenous material,permeated by mononuclear inflammatory infiltrate. A clinicalapproach without surgical intervention was chosen. Becauseadverse reactions may occur after a long period, it is necessary tocarry out long-term monitoring and a meticulous history acqui-sition, especially because patients may be averse to reporting theuse of aesthetic treatment modalities.

PE-099 - DENTAL CARE OF PATIENTS WITH EXTRA-NODAL NASAL LYMPHOMA CELL T/NK TYPE DURINGANTICANCER TREATMENT. DAIANE TENOR LOPES,LILIANE CRISTINE FERREIRA DE SOUZA, PERLAPORTO LEITE SHITARA, TOMIO IWATA, LUIS ANTÔNIOPIRES, GILSON LUCHEZI DELGADO, MARCO VINICIUSCHAUD. UNIVERSIDADE DE SOROCABA (UNISO).

Neoplasms of mature T cells and NK cells are relativelyuncommon and have unknown causes. Extranodal lymphoma ofthe nasal type is characterized by broad-spectrum morphologicalangiocentric infiltration, vascular destruction, and necrosis and ismore common in adult men. Patient, 42, had diabetes mellitustype I and was a former smoker undergoing anticancer treatmentdue to T-cell lymphoma/nasal-type extranodal NK refractory toanticancer treatment. The patient was referred with symptoms oforal mucositis to the service of Dentistry Special Patients of theHospital of Sorocaba. A circular lesion with raised edges andhardened and painful symptoms in the apex of the tongue werenoted. Histopathological analysis showed granulation tissue andatypical lymphatic infiltration. Immunohistochemical analysisdiagnosed lymphocytic infiltrate with a predominance of smalllymphocytes and plasma cells. The multidisciplinary interactionallowed the patient to be treated with medical and dental supportand achieve improved quality of life.

PE-100 - DENTAL MANAGEMENT OF PATIENTS WITHLIVER DISEASES: CASE REPORT. HELOÍSA LAÍSROSARIO DOS SANTOS, DAVI SILVA CARVALHO CURI,THAMIRES SILVA SOUZA, ANDRÉ LUCAS D’ALMEIDALYRIO DOS SANTOS, ANTÔNIO FERNANDO PEREIRAFALCÃO, VIVIANE ALMEIDA SARMENTO, PATRÍCIA LEITERIBEIRO LAMBERTI. FEDERAL UNIVERSITY OF BAHIA.

The liver is responsible for multiple functions, such as thesynthesis of coagulation factors and drug metabolism. Thesefeatures should be considered when planning the dental treatmentof patients with liver dysfunction. This study reports the dentalmanagement of a patient with liver cirrhosis who required mul-tiple extractions. After evaluation and discussing the case with themedical staff, the surgical procedure was performed in two stagesusing fresh frozen plasma, local hemostatic measures, and anti-biotic prophylaxis. The dental management in these situations isdiscussed.