cockayne syndrome: case report

1
ALMEIDA MONTENEGRO FURTADO, FILIPE NOBRE CHAVES, THYCIANA RODRIGUES RIBEIRO, KARUZA MARIA ALVES PEREIRA, FÁBIO WILDSON GURGEL COSTA. UNIVERSIDADE FEDERAL DO CEARÁ CAMPUS SOBRAL. Florid osseous dysplasia (FOD) is a benign process limited to gnathic bones. Woman, 76, had hypertension, diabetes, and car- diac problems. She was referred to the Stomatology clinic for evaluation of unspecic jaw pain. Physical examination revealed no disorders. Radiographic examination showed multiple radi- opaque lesions with well-dened borders surrounded by a thin cortical line in the apical region of teeth #33, #43, #44, and #45, as well as in the alveolar regions of teeth #25, #34, #35, #36, and #46. Based on the clinicoradiographic features a diagnosis of FOD was proposed. The clinical approach included drug therapy without surgical intervention because of the patients systemic condition and in light of the fact that the FOD did not require surgical removal. The patient was given analgesics and referred for dental treatment. At 6-month clinic-radiographic follow-up, no alterations were found. This case demonstrates the impor- tance of adequately managing medically compromised patients with FOD. PE-081 - CLINICAL ASPECTS AND CONSERVATIVE DENTAL MANAGEMENT OF A PATIENT WITH FIBRO- DYSPLASIA OSSIFICANS PROGRESSIVA. CLARISSA PESSOA FERNANDES, FRANCISCO ARTUR FORTE OLIVEIRA, MALENA REGINA FREITAS E SILVA, RENATA VERAS CARVALHO MOURÃO OKA, CAMILA CARVALHO DE OLIVEIRA, MÁRIO ROGÉRIO LIMA MOTA, ANA PAULA NEGREIROS NUNES ALVES. FEDERAL UNIVER- SITY OF CEARA. Fibrodysplasia ossicans progressiva (FOP) is a rare genetic disease characterized by skeletal malformations and ectopic os- sications in skeletal muscles, tendons, ligaments, and aponeu- rosis. Exacerbation of these ossications can be caused by dental treatment. Man, 26, with a diagnosis of FOP was referred for dental treatment. Conservative dental procedures, such as oral hygiene instructions and recurrent topical uoride applications, were performed in addition to endodontic and restorative treat- ments. Brief dental appointments were conducted without using regional anesthesia or dental dam clamps. The dental chair was positioned at 45 to provide more comfort and to avoid exacer- bating the disease. The patient has now completed 6 months of follow-up and is free of heterotopic ossications resulting from dental treatment. The dental treatment modications implemented for the present case were sufcient to establish good oral health and to prevent the formation of heterotopic ossications in the maxillofacial region. PE-082 - DENTAL TREATMENT OF PATIENTS PRE- SENTING WITH TOOTHACHE WHILE USING BISPHOSPHONATE: CLINICAL CASE REPORT. LILIANE CRISTINE FERREIRA DE SOUZA BORGES, JULIANA FERREIRA DE SOUZA, PERLA PORTO LEITE SHITARA, DAIANE TENOR LOPES, MARCO VINICIUS CHAUD, TOMIO IWATA. UNIVERSIDADE DE SOROCABA UNISO. Bisphosphonates (BPs) are inhibitors of bone resorption used in the treatment of bone diseases. These drugs have been linked to a debilitating complication, osteonecrosis of the jaws. This study reports dental care in the pre-, intra-, and postoperative period in patients treated with BPs. Woman, 52, complained of pain in the jaw. The patient reported receiving chemotherapy and using BPs (pamidronate sodium) 2 years previously to manage bone me- tastases. The panoramic radiograph revealed the need for extraction of tooth #47. During the procedure, excessive trauma to adjacent tissue was avoided and low-power laser was used as adjuvant therapy. The surgery plus laser therapy approach has been successful, with satisfactory healing. However, the patient must be followed-up closely because the risk of osteonecrosis continues indenitely. PE-083 - CLINICAL-IMAGING FEATURES AND SURGI- CAL CONSIDERATIONS IN A PEDIATRIC DENTAL PA- TIENT WITH OSTEOGENESIS IMPERFECTA TREATED WITH BISPHOSPHONATE. FILIPE NOBRE CHAVES, RODRIGO RODRIGUES RODRIGUES, FRANCISCO SAMUEL RODRIGUES CARVALHO, THYCIANA RODRIGUES RIBEIRO, MANUELA ALMEIDA MONTENEGRO FURTADO, KARUZA MARIA ALVES PEREIRA, FÁBIO WILDSON GURGEL COSTA. FEDERAL UNIVERSITY OF CEARA - CAMPUS SOBRAL. Osteogenesis imperfecta (OI) is a rare autosomal dominant disorder caused by mutations in the genes COL1A1 or COL1A2 associated with changes in type I collagen metabolism. Patients with OI may have blue sclerae, hearing loss, dentinogenesis imperfecta (DI), growth deciency, ligamentous laxity, or a combination of these. Bisphosphonate therapy is reported to improve mobility and bone density and to reduce pain and the incidence of fracture. However, it may also favor the develop- ment of maxillary osteonecrosis, especially after dental surgery. Male pediatric patient with OI but no family history of OI had previously been treated with intravenous bisphosphonate and now required dental surgery. Currently, after 18 months of follow-up, the patient shows no signs of osteonecrosis. The clinical and imaging ndings were discussed, along with a brief review of the literature on treatment and surgical indications in this patient population. PE-084 - COCKAYNE SYNDROME: CASE REPORT. KARIN SOARES GONÇALVES CUNHA, RAQUEL RICHELIEU LIMA DE ANDRADE PONTES, RAFAELA ELVIRA ROZZA DE MENEZES, ELOÁ BORGES LUNA, ARLEY SILVA JUNIOR, KARLA BIANCA FERNANDES DA COSTA FONTES, ALEXANDRE TRINDADE SIMÕES DA MOTTA. UNIVERSIDADE FEDERAL FLUMINENSE, NITERÓI-RJ. Cockayne syndrome (CS) is an autosomal recessive disorder characterized by several abnormalities, including oral ndings. A clinical case was reported to show to the dental community the characteristics of CS. Woman, 25, with CS who had consan- guineous parents presented to the diagnostic service complaining of diastema. She was 115 cm tall, weighed 32 kg, and had a circumference head of 51 cm. She suffered bilateral deafness, psychomotor and mental retardation, dysgeusia, and insomnia and presented a typical facial birdlike appearance. Intraoral ex- amination showed hygiene deciency, gingivitis, dental apposi- tion, microdontia, lower central incisor diastema, and the absence of teeth #16, #26, and #48 (conrmed by radiographic exami- nation). CS is a rare condition with several oral manifestations. The oral alterations reported herein are in accordance with reports in the literature. Knowing about CS is an important aid to dentists in formulating a diagnosis and providing adequate care and treatment. ORAL AND MAXILLOFACIAL PATHOLOGY OOOO e148 Abstracts February 2014

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Page 1: Cockayne Syndrome: Case Report

ORAL AND MAXILLOFACIAL PATHOLOGY OOOO

e148 Abstracts February 2014

ALMEIDA MONTENEGRO FURTADO, FILIPE NOBRECHAVES, THYCIANA RODRIGUES RIBEIRO, KARUZAMARIA ALVES PEREIRA, FÁBIO WILDSON GURGELCOSTA. UNIVERSIDADE FEDERAL DO CEARÁ CAMPUSSOBRAL.

Florid osseous dysplasia (FOD) is a benign process limited tognathic bones. Woman, 76, had hypertension, diabetes, and car-diac problems. She was referred to the Stomatology clinic forevaluation of unspecific jaw pain. Physical examination revealedno disorders. Radiographic examination showed multiple radi-opaque lesions with well-defined borders surrounded by a thincortical line in the apical region of teeth #33, #43, #44, and #45,as well as in the alveolar regions of teeth #25, #34, #35, #36, and#46. Based on the clinicoradiographic features a diagnosis ofFOD was proposed. The clinical approach included drug therapywithout surgical intervention because of the patient’s systemiccondition and in light of the fact that the FOD did not requiresurgical removal. The patient was given analgesics and referredfor dental treatment. At 6-month clinic-radiographic follow-up,no alterations were found. This case demonstrates the impor-tance of adequately managing medically compromised patientswith FOD.

PE-081 - CLINICAL ASPECTS AND CONSERVATIVEDENTAL MANAGEMENT OF A PATIENT WITH FIBRO-DYSPLASIA OSSIFICANS PROGRESSIVA. CLARISSAPESSOA FERNANDES, FRANCISCO ARTUR FORTEOLIVEIRA, MALENA REGINA FREITAS E SILVA, RENATAVERAS CARVALHO MOURÃO OKA, CAMILA CARVALHODE OLIVEIRA, MÁRIO ROGÉRIO LIMA MOTA, ANAPAULA NEGREIROS NUNES ALVES. FEDERAL UNIVER-SITY OF CEARA.

Fibrodysplasia ossificans progressiva (FOP) is a rare geneticdisease characterized by skeletal malformations and ectopic os-sifications in skeletal muscles, tendons, ligaments, and aponeu-rosis. Exacerbation of these ossifications can be caused by dentaltreatment. Man, 26, with a diagnosis of FOP was referred fordental treatment. Conservative dental procedures, such as oralhygiene instructions and recurrent topical fluoride applications,were performed in addition to endodontic and restorative treat-ments. Brief dental appointments were conducted without usingregional anesthesia or dental dam clamps. The dental chair waspositioned at 45� to provide more comfort and to avoid exacer-bating the disease. The patient has now completed 6 months offollow-up and is free of heterotopic ossifications resulting fromdental treatment. The dental treatment modifications implementedfor the present case were sufficient to establish good oral healthand to prevent the formation of heterotopic ossifications in themaxillofacial region.

PE-082 - DENTAL TREATMENT OF PATIENTS PRE-SENTING WITH TOOTHACHE WHILE USINGBISPHOSPHONATE: CLINICAL CASE REPORT. LILIANECRISTINE FERREIRA DE SOUZA BORGES, JULIANAFERREIRA DE SOUZA, PERLA PORTO LEITE SHITARA,DAIANE TENOR LOPES, MARCO VINICIUS CHAUD,TOMIO IWATA. UNIVERSIDADE DE SOROCABA UNISO.

Bisphosphonates (BPs) are inhibitors of bone resorption usedin the treatment of bone diseases. These drugs have been linked toa debilitating complication, osteonecrosis of the jaws. This studyreports dental care in the pre-, intra-, and postoperative period inpatients treated with BPs. Woman, 52, complained of pain in the

jaw. The patient reported receiving chemotherapy and using BPs(pamidronate sodium) 2 years previously to manage bone me-tastases. The panoramic radiograph revealed the need forextraction of tooth #47. During the procedure, excessive traumato adjacent tissue was avoided and low-power laser was used asadjuvant therapy. The surgery plus laser therapy approach hasbeen successful, with satisfactory healing. However, the patientmust be followed-up closely because the risk of osteonecrosiscontinues indefinitely.

PE-083 - CLINICAL-IMAGING FEATURES AND SURGI-CAL CONSIDERATIONS IN A PEDIATRIC DENTAL PA-TIENT WITH OSTEOGENESIS IMPERFECTA TREATEDWITH BISPHOSPHONATE. FILIPE NOBRE CHAVES,RODRIGO RODRIGUES RODRIGUES, FRANCISCOSAMUEL RODRIGUES CARVALHO, THYCIANARODRIGUES RIBEIRO, MANUELA ALMEIDAMONTENEGRO FURTADO, KARUZA MARIA ALVESPEREIRA, FÁBIO WILDSON GURGEL COSTA. FEDERALUNIVERSITY OF CEARA - CAMPUS SOBRAL.

Osteogenesis imperfecta (OI) is a rare autosomal dominantdisorder caused by mutations in the genes COL1A1 or COL1A2associated with changes in type I collagen metabolism. Patientswith OI may have blue sclerae, hearing loss, dentinogenesisimperfecta (DI), growth deficiency, ligamentous laxity, or acombination of these. Bisphosphonate therapy is reported toimprove mobility and bone density and to reduce pain and theincidence of fracture. However, it may also favor the develop-ment of maxillary osteonecrosis, especially after dental surgery.Male pediatric patient with OI but no family history of OI hadpreviously been treated with intravenous bisphosphonate and nowrequired dental surgery. Currently, after 18 months of follow-up,the patient shows no signs of osteonecrosis. The clinical andimaging findings were discussed, along with a brief review of theliterature on treatment and surgical indications in this patientpopulation.

PE-084 - COCKAYNE SYNDROME: CASE REPORT.KARIN SOARES GONÇALVES CUNHA, RAQUELRICHELIEU LIMA DE ANDRADE PONTES, RAFAELAELVIRA ROZZA DE MENEZES, ELOÁ BORGES LUNA,ARLEY SILVA JUNIOR, KARLA BIANCA FERNANDES DACOSTA FONTES, ALEXANDRE TRINDADE SIMÕES DAMOTTA. UNIVERSIDADE FEDERAL FLUMINENSE,NITERÓI-RJ.

Cockayne syndrome (CS) is an autosomal recessive disordercharacterized by several abnormalities, including oral findings. Aclinical case was reported to show to the dental community thecharacteristics of CS. Woman, 25, with CS who had consan-guineous parents presented to the diagnostic service complainingof diastema. She was 115 cm tall, weighed 32 kg, and had acircumference head of 51 cm. She suffered bilateral deafness,psychomotor and mental retardation, dysgeusia, and insomniaand presented a typical facial birdlike appearance. Intraoral ex-amination showed hygiene deficiency, gingivitis, dental apposi-tion, microdontia, lower central incisor diastema, and the absenceof teeth #16, #26, and #48 (confirmed by radiographic exami-nation). CS is a rare condition with several oral manifestations.The oral alterations reported herein are in accordance with reportsin the literature. Knowing about CS is an important aid to dentistsin formulating a diagnosis and providing adequate care andtreatment.