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                    D e n t a l    F o l l i c l e               

             The        Monthly     E-newsletter                   Vol - I I      Number-  II        July  2007

In this Issue:

  • Editorial

  • News

  • Laughter - The best Medicine

  • Radiotherapy induced osteosarcoma of the orbit in a 60-year old; a case report following choroidal melanoma treatment without retinoblastoma oncogenesis or Paget’s disease predisposition. - S.Arun ,R.E.Weir
     

  • Papillon-Lefevre syndrome: A case report with a new laser assisted treatment method - Maziar MIR

  • INTERNATIONAL STUDENT EXCHANGE PROGRAMME (ISEP) of IADS - Magdalena Maciejowska

     

Editorial :

      Dear Fellow Dentist,

                  Neuromuscular Dentistry is the science of dentistry that embodies accepted scientific principles of patho-physiology, anatomy, form and function. Neuromuscular Dentistry objectively evaluates the complex relationship between teeth, temporomandibular joints and the masticatory muscles in order to achieve an occlusion that is based on the optimal relationship between the mandible and the skull — Neuromuscular Occlusion.  The goal of the Neuromuscular Dentist is to relax the muscles controlling jaw position to establish a true physiological rest position upon which treatment considerations are based. 

           In summary, Neuromuscular Dentistry is the science of occlusion encompassing not only the teeth, but the objective evaluation of the status and function of the jaw muscles and joints — before, during and after treatment — to achieve the optimal result.I am sure more advancements in Neuromuscular Dentistry will take Dentistry to a new Horizon.

             I hope all of you enjoy this issue.

                      

 

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Yours truly

Dr. S . Nabeel

Editor of Dental Follicle & WebMaster www.DentistryUnited.com

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News :

         

                       

Laughter - The Best Medicine :

Patient: Doctor, I have yellow teeth, what do I do? Dentist: Wear a brown tie...

                                         

Radiotherapy induced osteosarcoma of the orbit in a 60-year old; a case report following choroidal melanoma treatment without retinoblastoma oncogenesis or Paget’s disease predisposition.

S.Arun ,R.E.Weir

Osteogenic sarcoma (OS) is a rare malignant bone tumour occurring in 1:100,000 people per year, with around 6% to 13% of these tumours affecting the craniofacial bones 1-4. Approximately 25% of osteosarcoma involving the head and neck in one series occurred following previous head and neck radiotherapy 5. Review of the literature of post radiotherapy induced osteosarcoma reveals 12 cases following orbital radiotherapy for retinoblastoma, a tumour of childhood. We report the clinical presentation of a 60-year-old woman and the clinical course of this rare occurrence after choroidal melanoma treatment. 

Case Report

A 60-year-old lady presented with a numb, swollen left periorbital region ten years after radiation for a recurrence of choroidal melanoma. On examination she had a fungating mass visible within the left orbit invading through the socket and displacing her shell prosthesis.  

At the age of nineteen she was treated with enucleation of the left globe for choroidal malignant melanoma extending into the orbit. 30 years later she presented with an ill fitting ocular shell prosthesis. CT scan confirmed an orbital mass displacing the ocular shell prosthesis. Biopsy confirmed a recurrence of a malignant melanoma, which was treated with radiotherapy shrinkage and surgical resection. In total 4,800 centigray in 12 fractions was administered over four weeks using 4 MeV x-rays in a two field planned technique. Radiotherapy was well tolerated. 

Over the next ten years, she attended routine review periodically with the ophthalmology and radiotherapy teams without any signs of recurrence. Ten years following her radiotherapy course she presented to the ophthalmologist with an inflamed orbit with a six month history of left midfacial numbness. On examination she had a left orbital nodular mass involving the periorbital structures. CT and MRI scans revealed a large mass extending through the lateral orbital wall into temporal, infratemporal and pterygopalatine fossae (figures 1 and 2). Biopsy confirmed osteoblastic osteosarcoma.  

                       

The osteosarcoma grew rapidly over the next four weeks. Repeat CT scans revealed a very large mass in the left temporal fossa further extending into the left frontal lobe, the floor and roof of orbit and extending posteriorly to the zygomatic arch, greater wing of sphenoid, petrous bone and mastoid region.  

The patient was managed palliatively for two half years after presentation before dying of osteosarcoma related complications at 62 years of age. 

 

Comment

Osteosarcoma of craniofacial bones is rare and represents 2% of all osteosarcomas 6.  

In one series of 66 patients, the presentation of radiotherapy induced osteoscarcoma is described with an age range of 3.5 to 33 years and a median latent period following radiotherapy of 10.5 years 7.  However reported post radiotherapy craniofacial cases usually follow retinoblastoma or Paget’s disease. In one series, 2 orbital cases out of 27 post radiotherapy induced osteosarcomas of skull and maxilla, followed retinoblastoma 2 with another case predisposed by Paget’s disease 5. It is very unusual to follow choroidal melanoma radiotherapy treatment and predictably occuring at an older age than retinoblastoma radiotherapy treated patients. 

Interestingly, there is another case report in a similarly aged 59 year old lady following radiotherapy of an unknown orbital primary tumour in an enucleated socket. The patient having previously undergone enucleation after trauma and then subsequently developing an orbital tumour, without having histology prior to radiotherapy in Vietnam. Histology of the traumatised enucleated eye is not described in the case report and may have been unavailable to the authors but she is the age at which choroidal melanoma is feasible 8.  

Identified predisposing factors for osteosarcoma include retinoblastoma, Li-Fraumeni syndrome, Paget disease and radiation exposure 5. Mutation of the RB1 & TP53 genes are hypothesised to both be needed to predispose to osteosarcoma 9.   

Craniofacial osteosarcoma has a much worse prognosis than long bone osteosarcomas and retinoblastoma cases of post radiation induced osteosarcoma having a similar prognosis to other cases. Survival rates in craniofacial post radiation induce osteosarcomas overall being reported as 66% at two years and 55% at five years 5.

Survival of this post choroidal melanoma case is similar to the published series of radiation induced tumours of other origins.  

Patients managed with radiotherapy for choroidal melanoma are at very small risk of developing osteosarcoma of the orbit as a rare complication ten years after radiotherapy exposure. Surgeons and physicians reviewing patients previously treated by radiotherapy for this condition should be aware of this occurrence as an unusual complication 10 years after radiotherapy. The time-to-presentation being similar to other craniofacial tumours treated with radiotherapy but in an older age group.

 

References

 

1.Huvos,AG.Bone Tumors: Diagnosis,Treatment, and Prognosis.2nd edn.Philadelhia: WB Saunders; 1991:88-156 

2.Caron AS,Hajdu SI,Strong EW.Osteogenic sarcoma of the facial and cranial bone: a review of forty-three cases.Am J Surg 1971;122:719-725. 

3.Clark.JL,Unni KK,Dahlin DC,Devine KD.Osteosarcoma of the jaw.Cancer 1983;51:2311-2316. 

4.Garrington GE,Scofield HH,Cornyn J,Hooker SP.Osteosacrcoma of the jaws:analysis of 56 cases.Cancer 1967;20:377-391. 

5.Ha,Patric K.BA;Eisele,David W.MD;Frassica,Frank J.MD;Zahurak,Marianna L.MS; McCarthy,Edward F.MD : Osteosarcoma of the head & neck review: A review of the Johns Hopkins experience The American Laryngological,Rhinological & otalogical Society,Inc.Volume109(6) June 1999,pp 964-969 

6.Nora FE, Unni KK, Pritchard DJ, Dahlin DC (1983) Osteosarcoma of extragnathic craniofacial bones. Mayo Clin Proc 58:268-272 

7.Huvos AG, Woodward HQ, Cahan WG, Higinbotham NL, Stewart FW, Butler A, Bretsky SS (1985) Postradiation osteogenic sarcoma of bone and soft tissues, a clinicopathologic study of 66 patients. Cancer 55:1244-1255 

8.Michael Marks, Susan Marks, Hervey D Seagall, C.P.Schwinn and Deborah M.Forrester Case report 420 Skeletal Radiol (1987) 16:246-251. 

9.Knudson AG.Genetics of tumors of the head and neck.Arch Otolaryngol Head Neck Surg.1993;119:735-737.

 

         

                

Papillon-Lefevre syndrome: A case report with a new laser assisted treatment method
 

Maziar MIR,
Assistant Prof,
RWTH Hospitl, Germany

 

Abstract
   Background: A 3.5 year old girl patient presented with 10 missing and   6 mobile primary teeth
   in April 1998. Physical examinations revealed  palmar and plantar hyperkeratosis. No other
   physical, mental or   laboratory disorder was found. Dental examinations showed severe  
   generalized gingival attachment loss in both dental arches. There was   a root exposure around
   all present teeth.
  
   Methods: Clinical, radiographic, histopathological and  microbiological   examinations were 
   carried out. Blood was taken for mutation analysis   of the cathepsin C gene. Based on the 
   outcome of the microbiological   test the patient was treated with daily chlorhexidine mountrinse.
   All   primary teeth were extracted to prevent re-infection. A diode laser   (970 nm, 2 W, 20 Hz) 
   was selected for de-epithelialization of free   gingival margins additional to the sulcus 
   decontamination.
 
   Results: The patient was treated successfully and no signs of relapse   were present during
   the last recall.. Mutation analysis of the  cathepsin C gene confirmed that the patient described 
   in this paper  is   a Papillon-Lefevre syndrome patient. Mutation analysis revealed a   novel 
   mutation(1212 A G;405 His Arg) in the cathepsin C gene. The   laser treatment additional to
   periodical scaling and root planning is   successful to save the 12 erupted permanent teeth
   till the age of 12  while the patient was last time visited on 27th July 2007.
 
  Conclusions: Careful screening for the appropriate antibiotic and   excellent oral hygiene 
  successfully lead to the preservation of the  permanent dentition.
  
KEY WORDS
 
INTRODUCTION
 
  Papillon-Lefevre syndrome (PLS) is a rare autosomal recessive   disorder. Its reported
  incidence is 1-4 per million and both the  sexes  are equally affected 1. PLS is characterised 
  by palmo-plantar  hyperkeratosis, periodontopathy and premature loss of deciduous as  well as
  permanent dentition. It manifests between 1-5 year of life and  the patient becomes edentulous
  in the early teens. Another component  of PLS is asymptomatic ectopic calcification in choroid 
  plexus and  tentorium. About 20% of these patients also show an increased  susceptibility to 
  infections probably due to dysfunction of  lymphocytes and leukocytes  2. PLS is diagnosed mainly 
  clinical.  PLS patients usually have very complex subgingival flora which  includes the presence of 
  Actinobacillus actinomycetemcomitans,  capnophilic and Capnocytophaga spp  3. In a PCR study the  
  Bacteroides, in particular Bacteroides forsythus were associated with  different types of 
  periodontitis  4. It was mentioned by Kabashima et  al  5 that IL-8, IL-1alpha and IL-1beta
  cytokines may be responsible  for modulating the process of rapidly progressive periodontitis in a
  patient with PLS  5 .  Papillon-Lefevre syndrome is caused by mutations in the gene encoding
  cathepsin C. This gene is located on chromosome 11. Up to now 43  different mutations have been
  described in PLS patients.  The conventional mechanical treatment of Papillon-Lefèvre syndrome 
  periodontitis has a poor prognosis. Up to now, nearly no successful  treatment that saved the 
  permanent dentition in PLS patients has been  described. The most optimistic papers described an 
  extraction of all  the deciduous teeth followed by a period of edentulousness. The  edentulous period
  may explain the fact that there has been no  recurrent attachment loss in the permanent teeth up to 
  age 17 8.  After this age all the treatments are shifting to use of dental  implants and complete
  dentures as the best solution of this problem.9
 
  In this study a complete clinical, radiological, pathological and   genetic diagnosis is performed on 
  a PLS patient.  An excellent oral hygiene, professional periodontal treatment and  selective antibiotic 
  therapy were used to treat the patient. The  patient was successfully treated and the permanent dentition 
  of this  patient could be preserved. Currently, Cobb concluded from the   literature some clinical evidence 
  that shows some wave lengths of  laser could be helpful for periodontal sockets decontamination.12   
  Ishikawa and Sculean published a review article on 2007 showing the   successful results of diode laser 
  assisted de-epithelializing and  sulcus decontamination therapies. 13  
 
 MATERIALS AND METHODS
  Clinical Findings
  A 3.5 year old girl patient presented with 10 missing and 6 mobile   primary teeth in April 1998. Physical
  examinations revealed palmar  and   plantar hyperkeratosis. No other physical, mental or laboratory   disorder 
  was found. Dental examinations showed severe generalized   gingival attachment loss in both dental arches. 
  There was a root   exposure around all present teeth.   Radiographic Findings   Severe bone loss was evident in 
  panoramic and occlusal radiographs.
  
  Microbiological Findings
  The early antibiogram detection showed cephalexine as the antibiotic   of choice for
  the disease. The result of cultures revealed the   predominant presence of Bacterioids. Histopathologhy: 
  Hypercementosis   and inflamatory reactive (fibrosis) hyperplasia was observed in the   slides of the 
  involved teeth   and surronding tissues, respectively.
  
  Genetic Analysis
  By the use of PCR we amplified the 7 exons of cathepsin C by using  the   primers previously described by 
  other groups. After the PCR process  we  confirmed the presence of the PCR product by 2% agarose gel 
  electrophoresis. The PCR products were purified by using columns and   the concentration of the DNA was 
  determined spectophotometrically. For  the sequence reaction we used the same primers as for the PCR  reaction
  and the reaction was carried out using the BigDye Terminator   mix. The data were automatically collected and
  analysed by the  software of the Sequencer. 
  The sequences were compared with the  published cathepsin C sequence. A nucleotide 1212 A G mutation in the  
  cathepsin C gene was found, which was predicted to result in an amino  acid 405His Arg mutation. The mutation was 
  confirmed by the use of  restriction enzyme analysis performed on exon 7. The nucleotide   mutation has not been 
  reported previously. The alteration of the  Histidine at position 405 has been demonstrated in a Pakistani 
  family  with PLS.
  
 
  Treatments and Follow-Ups
  The patient was treated with a daily chlorhexidine mouth rinse. To  eliminate the source of infection all primary 
  teeth were extracted in   June 1998.  Early antibiogram to select the best antibiotic for recommending  after  
  extraction of teeth prevents the furture infection and need to  antibiotic therapy before the eruption 
  of permanent teeth.
 
  Follow-up
  The infection was successfully controlled. The patient was   reevaluated clinically and paraclinically 
  and no future antibiotic  therapy was needed. The permanent incisors and first molars have  erupted under good oral 
  hygiene care. During the last recall   (November 2003) no significant finding were reported in the panoramic  X-ray.
 
  We conclude that the microbiological tests may be a powerful tool to   select the proper antibiotic for a successful 
  treatment of a   Papillon-Lefevre syndrome patient. Also, as the last laser assisted  treatment on 27th
  July 2007 showed successful acceptance of patient,  this new method as assistant to routine scaling and 
  root planning is   recommended strongly.
 
  DISCUSSION
  Laser could be used as a tool which shows unique effects on   micro/organisms especiallz in deep periodontiom
  infections. Removing   some layers of epithelium at the orifices of deep pocketes will  prevent the growth of
  these layers toward the sulcus and help the   attachment and healing of periodontitis. In this case this effect
  was  obviously seen and this method would be repeated till the end of  eruption of all permanent teeth.
 
 
 
 
  References:
 
  1. Griffiths WAD, Judge MR, Leigh IM. Disorders of keratinization. In:  Champion RH, Burton JL, Burns DA, 
  Breathnach SM editors. Textbook of   Dermatology, 6th edn. Oxford: Blackwell Scientific Publications;
  1998.pp. 1569-1571.
 
  2. Bergman R, Friedman-Birnbaum R. Papillon- Lefevre syndrome a study   of the long term clinical course
  of recurrent pyogenic infections and   the effects of etretinate treatment. Br J Dermatol 1998; 119: 131-136.
  
  3. Robertson KL, Drucker DB, James J, Blinkhorn AS, Hamlet S, Bird  PS.  A microbiological study of 
  Papillon-Lefevre syndrome in two patients.  J Clin Pathol. 2001 May;54(5):371-6.
  
  4. Y. Huang, M. Umeda, Y. Takeuchi, M. Ishizuka, K. Yano-Higuchi and   I. Ishikawa. Distribution of Bacteroides 
  forsythus genotypes in a   Japanese periodontitis population. Oral Microbiol Immunol. 2003   Aug;18(4):208-14.
  
  5. Hiroaki Kabashima, Masahiro Yoneda, Kengo Nagata, Kazuaki Nonaka,  Takao Hirofuji and Katsumasa Maeda. 
  THE PRESENCE OF CYTOKINE (IL-8,  IL-1 alpha , IL-1beta)-PRODUCING CELLS IN INFLAMED GINGIVAL TISSUE
  FROM A PATIENT MANIFESTING PAPILLON-LEFEVRE SYNDROME (PLS). Cytokine.  2002 May 7;18(3):121-6.
  
  6. Hart TC, Hart PS, Bawden DW, Michalec MD, Callison SA, Walker SJ,  et al. Mutations of the cathepsin C gene 
  are responsible for  Papillon-Lefevre syndrome. J Med Genet 1999; 36: 881-887. 
 
  7. Pacheco JJ, Coelho C, Salazar F, Contreras A, Slots J, Velazco CH.  Treatment of Papillon-Lefevre syndrome 
  periodontitis. J Clin   Periodontol. 2002 Apr;29(4):370-4.
 
  8. Wiebe CB, Hakkinen L, Putnins EE, Walsh P, Larjava HS. Successful   periodontal maintenance of a case with 
  Papillon-Lefevre syndrome:  12-year follow-up and review of the literature.  J Periodontol. 2001 Jun;72(6):824-30.
  Review.
  
  9. Woo I, Brunner DP, Yamashita DD, Le BT. Dental implants in a young   patient with Papillon-Lefevre syndrome: 
  a case report. Implant Dent.  2003;12(2):140-4.
 
  10. Eickholz P, Kugel B, Pohl S, Naher H, Staehle HJ. A  microbiological study of Papillon-Lefevre syndrome in 
two patients. J  Clin Pathol. 2001 May;54(5):371-6.
 
  11. Battino M, Ferreiro MS, Bompadre S, Leone L, Mosca F, Bullon P.  Elevated hydroperoxide levels 
and antioxidant patterns in  Papillon-Lefevre syndrome. J Periodontol. 2001 Dec;72(12):1760-6.
 
  12. Cobb CM. Lasers in periodontics: a review of the literature. J   Periodontol. 2006 Apr;77(4):545-64. Review.
  
  13. Ishikawa I and Sculean A. Lasers in periodontics in: Gutknecht N   et al. 1st proceeding of international 
workshop of evidence based  laser dentistry. Quintessence Ltd, 2007.

 

 

 

 

 

IADS Projects

       


 

INTERNATIONAL STUDENT EXCHANGE PROGRAMME (ISEP)

 

             International Student Exchange Programme is one of the most popular and important activities, which IADS organizes for members.

ISEP gives the students the opportunity to spend some time , usually it is between 2-6 weeks, at a dental school in another part of the world.

          This scheme allows students to experience the foreign systems of dentistry, new culture, language. Moreover it is another great opportunity to meet        new   people and their social life.

 

Exchanges are possible within the IADS member countries only. At the moment members of IADS are: Bosnia, Croatia, Czech Republic, Denmark, Egypt, Germany, Georgia, Ghana, Hungary, Italy, Kuwait, Macedonia, Malta, Poland,  Romania, Russia, Serbia, Slovenia, Sudan, Turkey. But each IADS meeting brings us new countries that join our family, what gives our students other new  possibilities to do an exchange.

 

 

VOLUNTARY WORK PROJECTS (VWP)                      

 

Voluntary Work Abroad is another undertaking for IADS members, as a result of a cooperation with international organizations who provide dental health care in different parts of the world.

This project is addressed to dentists and students with clinical experience- they can work under the supervision of dentists.

Some of projects are organized by local university or hospital and health organizations.

Depending on the place, it is simply oral hygiene promotion, preventive dentistry or simple treatment carried out.

By taking part in this project volunteers not only can gather more experience but also get to know closer new people from different cultures; their behaviors, traditions, ways of life.

 

You have to remember that participating in VWP you bring people in need hope and joy. Your experience and knowledge are very important. Patients trust you and you have to take full responsibility for them and their treatment!

 

At the moment IADS can offer projects in following countries:

  

·        Guatemala 

There are about 100 Guatemalan workers and between 8-15 volunteers working and living within the project. Volunteers have a place to live in a small space in a gallery building overlooking the river, or in a small Bungalow type house at the back of the project. There is no electricity in the bungalows so be prepared to use candles or lanterns for light at night to see by. The project will provide you with a foam mattress to sleep on.  There is also ultra-violet water filter in the clinic so there is safe water to drink.

Life and accommodations are simple. The experience is very enriching and the area is beautiful. 

Ak’ Tenamit is a project in Guatemala, where dentists are needed. Dentists or dental students – if there are any volunteers – work on a boat. Main treatments: Amalgam and Composite fillings, extractions (no x-ray, no prosthetics).

 

  •  Cambodia:

 

You can go to work in the „Angkor Hospital for children“.

                                                                     

·        Kenya:

 THIS works with Hospitals, Health and Medical Centers where volunteers work
through Workcamp or MLTV programme. Some of these Centers have dental units
that would be of great help to IADS members.

A volunteer wishing to come to work/serve in our partner dental unit need to have a good command/knowledge and skills in dental profession and practice (both for dentists and students), though others like nurses for support work at the unit.
 

·        Sudan, MEDICAL FIELD TRIP: 

During post- Congress in Sudan in 2006 participants had the chance to partake in this project. We had great opportunity to work and travel with dental students and dentists from University of Khartoum. IADS visitors carried out simply treatment at departments of conservative dentistry, oral surgery, periodontology which, for time of treatment, were located in small rooms. Simply prosthetic work was also possible as there was area with small very basic laboratory. We were working in different villages. Patients seemed to be glad about us and we were happy seeing smile on their faces.

This incredible experience will stay forever in our memory. 

·        Tanzania:  

Volunteers may stay in government clinic or may rotate through various clinics throughout the country. Volunteers have to spend a minimum of 1 week in any one clinic but can transfer from clinic to clinic. Nowadays at Sumbawanga Regional Hospital works U.S. dentist. 

For more information please visit  www.iads_web.9x.pl/voluntary.php.

 

You can all make a difference!

 

 

 

Magdalena Maciejowska

IEO