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Complex care by multiple medical and dental specialists of a patient with aggressive Gorlin-Goltz syndrome

Identifieur interne : 000354 ( PascalFrancis/Curation ); précédent : 000353; suivant : 000355

Complex care by multiple medical and dental specialists of a patient with aggressive Gorlin-Goltz syndrome

Auteurs : K. Nagy [Hongrie] ; E. Kiss ; C. Erdei [Hongrie] ; F. Oberna [Hongrie] ; P. Fejerdy [Hongrie] ; K. Marton [Hongrie] ; Z. Vajo [Hongrie]

Source :

RBID : Pascal:08-0375122

Descripteurs français

English descriptors

Abstract

Gorlin-Goltz syndrome is a genetically predisposed disease characterised by multiple basal cell carcinomas, odontogenic keratocysts and ectopic calcifications. The aim of this study was to show successful treatment of a 37-year-old male patient by cooperation between different dental and medical specialists. Because of the recurrence of a large basal cell carcinoma after multiple operations and a total dose telecobalt irradiation of 66 Gy, the patient's nose was ablated, with resection of the upper lip and part of the maxilla. The intraoral prosthetic treatment helped to restore the subtotal edentulousness. In order to enhance the application of the dental prosthesis, an Abbe plasty was performed at the second stage of surgery. As previous irradiation of the area precluded the use of facial implants immediately after the intraoral treatment, a temporary artificial nose prosthesis was created. The patient tolerated the procedures well and is completely disease-free 12 months after the surgery. Besides presenting a rare and complicated case of Gorlin-Goltz syndrome, the main purpose of this report is to show that, if different specialists in a dental-clinical team (maxillofacial surgeon, oncologist, radiation oncologist, prosthodontist and psychiatrist) combine their skills and expertise, successful management is possible even in a challenging complex case.
pA  
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A03   1    @0 Postgrad. med. j.
A05       @2 84
A06       @2 992
A08 01  1  ENG  @1 Complex care by multiple medical and dental specialists of a patient with aggressive Gorlin-Goltz syndrome
A11 01  1    @1 NAGY (K.)
A11 02  1    @1 KISS (E.)
A11 03  1    @1 ERDEI (C.)
A11 04  1    @1 OBERNA (F.)
A11 05  1    @1 FEJERDY (P.)
A11 06  1    @1 MARTON (K.)
A11 07  1    @1 VAJO (Z.)
A14 01      @1 Department of Oral and Maxillofacial Surgery, University of Szeged @2 Szeged @3 HUN @Z 1 aut. @Z 7 aut.
A14 02      @1 Department of Prosthodontics, Semmelweis University Medical School, Faculty of Dentistry @2 Budapest @3 HUN @Z 3 aut. @Z 5 aut. @Z 6 aut.
A14 03      @1 Department of Maxillofacial Surgery, Saint Rokus Hospital @2 Budapest @3 HUN @Z 4 aut.
A20       @1 330-332
A21       @1 2008
A23 01      @0 ENG
A43 01      @1 INIST @2 9614 @5 354000197361990120
A44       @0 0000 @1 © 2008 INIST-CNRS. All rights reserved.
A45       @0 9 ref.
A47 01  1    @0 08-0375122
A60       @1 P @3 EC
A61       @0 A
A64 01  1    @0 Postgraduate medical journal
A66 01      @0 GBR
C01 01    ENG  @0 Gorlin-Goltz syndrome is a genetically predisposed disease characterised by multiple basal cell carcinomas, odontogenic keratocysts and ectopic calcifications. The aim of this study was to show successful treatment of a 37-year-old male patient by cooperation between different dental and medical specialists. Because of the recurrence of a large basal cell carcinoma after multiple operations and a total dose telecobalt irradiation of 66 Gy, the patient's nose was ablated, with resection of the upper lip and part of the maxilla. The intraoral prosthetic treatment helped to restore the subtotal edentulousness. In order to enhance the application of the dental prosthesis, an Abbe plasty was performed at the second stage of surgery. As previous irradiation of the area precluded the use of facial implants immediately after the intraoral treatment, a temporary artificial nose prosthesis was created. The patient tolerated the procedures well and is completely disease-free 12 months after the surgery. Besides presenting a rare and complicated case of Gorlin-Goltz syndrome, the main purpose of this report is to show that, if different specialists in a dental-clinical team (maxillofacial surgeon, oncologist, radiation oncologist, prosthodontist and psychiatrist) combine their skills and expertise, successful management is possible even in a challenging complex case.
C02 01  X    @0 002B01
C02 02  X    @0 002B08A
C03 01  X  FRE  @0 Syndrome du naevus basocellulaire @5 01
C03 01  X  ENG  @0 Basal cell nevus syndrome @5 01
C03 01  X  SPA  @0 Nevo basocelular síndrome @5 01
C03 02  X  FRE  @0 Complexité @5 02
C03 02  X  ENG  @0 Complexity @5 02
C03 02  X  SPA  @0 Complejidad @5 02
C03 03  X  FRE  @0 Complexe @2 NA @5 03
C03 03  X  ENG  @0 Complexes @2 NA @5 03
C03 03  X  SPA  @0 Complejo @2 NA @5 03
C03 04  X  FRE  @0 Soin @5 05
C03 04  X  ENG  @0 Care @5 05
C03 04  X  SPA  @0 Cuidado @5 05
C03 05  X  FRE  @0 Multiple @5 06
C03 05  X  ENG  @0 Multiple @5 06
C03 05  X  SPA  @0 Múltiple @5 06
C03 06  X  FRE  @0 Médecine @5 08
C03 06  X  ENG  @0 Medicine @5 08
C03 06  X  SPA  @0 Medicina @5 08
C03 07  X  FRE  @0 Dent @5 09
C03 07  X  ENG  @0 Tooth @5 09
C03 07  X  SPA  @0 Diente @5 09
C03 08  X  FRE  @0 Homme @5 11
C03 08  X  ENG  @0 Human @5 11
C03 08  X  SPA  @0 Hombre @5 11
C03 09  X  FRE  @0 Malade @5 12
C03 09  X  ENG  @0 Patient @5 12
C03 09  X  SPA  @0 Enfermo @5 12
C03 10  X  FRE  @0 Haute malignité @5 14
C03 10  X  ENG  @0 High malignancy @5 14
C03 10  X  SPA  @0 Alta malignidad @5 14
C07 01  X  FRE  @0 Maladie héréditaire @5 37
C07 01  X  ENG  @0 Genetic disease @5 37
C07 01  X  SPA  @0 Enfermedad hereditaria @5 37
C07 02  X  FRE  @0 Pathologie de la peau @5 38
C07 02  X  ENG  @0 Skin disease @5 38
C07 02  X  SPA  @0 Piel patología @5 38
N21       @1 238
N44 01      @1 OTO
N82       @1 OTO

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Pascal:08-0375122

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<div type="abstract" xml:lang="en">Gorlin-Goltz syndrome is a genetically predisposed disease characterised by multiple basal cell carcinomas, odontogenic keratocysts and ectopic calcifications. The aim of this study was to show successful treatment of a 37-year-old male patient by cooperation between different dental and medical specialists. Because of the recurrence of a large basal cell carcinoma after multiple operations and a total dose telecobalt irradiation of 66 Gy, the patient's nose was ablated, with resection of the upper lip and part of the maxilla. The intraoral prosthetic treatment helped to restore the subtotal edentulousness. In order to enhance the application of the dental prosthesis, an Abbe plasty was performed at the second stage of surgery. As previous irradiation of the area precluded the use of facial implants immediately after the intraoral treatment, a temporary artificial nose prosthesis was created. The patient tolerated the procedures well and is completely disease-free 12 months after the surgery. Besides presenting a rare and complicated case of Gorlin-Goltz syndrome, the main purpose of this report is to show that, if different specialists in a dental-clinical team (maxillofacial surgeon, oncologist, radiation oncologist, prosthodontist and psychiatrist) combine their skills and expertise, successful management is possible even in a challenging complex case.</div>
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<s0>Dent</s0>
<s5>09</s5>
</fC03>
<fC03 i1="07" i2="X" l="ENG">
<s0>Tooth</s0>
<s5>09</s5>
</fC03>
<fC03 i1="07" i2="X" l="SPA">
<s0>Diente</s0>
<s5>09</s5>
</fC03>
<fC03 i1="08" i2="X" l="FRE">
<s0>Homme</s0>
<s5>11</s5>
</fC03>
<fC03 i1="08" i2="X" l="ENG">
<s0>Human</s0>
<s5>11</s5>
</fC03>
<fC03 i1="08" i2="X" l="SPA">
<s0>Hombre</s0>
<s5>11</s5>
</fC03>
<fC03 i1="09" i2="X" l="FRE">
<s0>Malade</s0>
<s5>12</s5>
</fC03>
<fC03 i1="09" i2="X" l="ENG">
<s0>Patient</s0>
<s5>12</s5>
</fC03>
<fC03 i1="09" i2="X" l="SPA">
<s0>Enfermo</s0>
<s5>12</s5>
</fC03>
<fC03 i1="10" i2="X" l="FRE">
<s0>Haute malignité</s0>
<s5>14</s5>
</fC03>
<fC03 i1="10" i2="X" l="ENG">
<s0>High malignancy</s0>
<s5>14</s5>
</fC03>
<fC03 i1="10" i2="X" l="SPA">
<s0>Alta malignidad</s0>
<s5>14</s5>
</fC03>
<fC07 i1="01" i2="X" l="FRE">
<s0>Maladie héréditaire</s0>
<s5>37</s5>
</fC07>
<fC07 i1="01" i2="X" l="ENG">
<s0>Genetic disease</s0>
<s5>37</s5>
</fC07>
<fC07 i1="01" i2="X" l="SPA">
<s0>Enfermedad hereditaria</s0>
<s5>37</s5>
</fC07>
<fC07 i1="02" i2="X" l="FRE">
<s0>Pathologie de la peau</s0>
<s5>38</s5>
</fC07>
<fC07 i1="02" i2="X" l="ENG">
<s0>Skin disease</s0>
<s5>38</s5>
</fC07>
<fC07 i1="02" i2="X" l="SPA">
<s0>Piel patología</s0>
<s5>38</s5>
</fC07>
<fN21>
<s1>238</s1>
</fN21>
<fN44 i1="01">
<s1>OTO</s1>
</fN44>
<fN82>
<s1>OTO</s1>
</fN82>
</pA>
</standard>
</inist>
</record>

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