13TH march 2013

FIXED PROSTHODONTIC
REHABILITATION IN A
WEAR PATIENT WITH
FABRY`S DISEASE

Avinash S. Bidra
Journal of prosthodontics: 2011,S2-S8.

DR. P.AKANSHA
M.D.S. 1ST YEAR
DEPARTMENT OF PROSTHODONTIC
CONTENT
 Introduction

 Clinical
report
 Discussion

 Reference
INTRODUCTION
 Fabry`s disease is a rare X-linked recessive,
lysosomal storage metabolic disorder described by
FABRY AND ANDERSON in 1898.
 A mutation in the gene that controls the
Galactosidase A enzyme causes insufficient
breakdown of lipids that builts up harmful levels of
glycosphingolipids in the kidneys, eyes ,autonomus
nervous system, cardiovascular system.
 The incidence has been estimated at 1: 40,000 to
1:117,000 people worldwide, but absolute numbers
are unavailable.
Systemic manifestation-
 Cardiovasular disease with susceptible to stroke at
young age,
 Renal dysfunction,

 Corneal dystrophy,

 Cutaneous angiokeratomas,

 Pain in the extremities(acroparasthesias),

 GI disturbance- most common symptoms including

nausea, vomiting, regurgitation and abdominal pain.
Medical management -
 As there is no known cure for this disease, so
treatment mainly direct towards management of
symptoms such as Kidney transplantation .
Dental manifestations –
 Baccaglini et al reported

1. Perioral angiokeratomas,

2. Telangiactasias of labial mucosa and soft palate,

3. Cysts/pseudocysts of maxillary sinus,

4. Diastema in the anterior region,

5. Bimaxillary prognathism,

6. Xerostomia.
Management –
 Patients with fabry`s disease requires special
considerations as they already medically
compromised one.
 Prosthodontic considerations includes the
Management of diastema for esthetic purpose and
optimal occlusal scheme,
 Management of bilateraly prognathism and
accompanying “gummy smile”.
 Several reports described fixed prosthodontic
management of patients with generalized wear of
dentition.
 The purpose of the report is to describe the
prosthodontic management of an American College
of Prosthodontists Prosthodontic diagnostic
index(ACP PDI) class IV dentate patient with
fabry`s disease.
 Criterias-
 Severely compromised tooth condition
• Insufficient tooth structure to retain or support
intracoronal or extracoronal restorations—in three
or more sextants.
• Pathology that affects the coronal morphology of
four or more teeth in all sextants.
• Teeth require localized adjunctive therapy, i.e.,
periodontal, endodontic or orthodontic procedure
for teeth in three or more sextants.
 Severely compromised occlusal scheme
• Occlusal scheme requires major therapy to
reestablish the entire occlusal scheme including
any changes in the occlusal vertical dimension.
 Other Class IV characteristics
• Severe manifestations of local or systemic disease,
including the sequelae from oncologic treatment.
• Maxillomandibular dyskensia and/or ataxia
• A refractory patient—a patient who presents with
chronic complaints following appropriate treatment.
CLINICAL REPORT

Patient History -

41 year caucasian man, reported to improve esthetics

& structure of teeth he recognized as being
compromised.
MEDICAL HISTORY
 Fabry`s disease
 Hypertension

 Bilateral renal faliure.

 GI disturbances- nausea, vomiting, regurgitation .

Medication –
 Immunosuppressants

 Antihypertensives

 Drugs for enzyme replacement therapy- administered

by intravenous , every 15 days. Doses are variable
depending on the preparation: 0.2 mg/kg/dose of
alpha algasidase .
DENTAL HISTORY
 Extaorally- multiple angiokeratomas in perioral
region including lips.
 Intraorally –
1) Soft tissue ---
 Telangiectasias

 Gingiva –

minimal inflammation
maximum probing
depth of 3 mm.
 Xerostomia .
2) Hard tisssue –---
 Diastema in maxillary and mandibular anterior
region of tooth.
 Generalized sever attrition -occlusal surfaces of
posteriors .
 Generalized sever erosion on :

Lingual surface of maxillary anteriors & premolars.

Facial surfaces of mandibular premolars.
 Minimal wear-

mandibular anteriors compered to canine .

 Multiple amalgam restorations in posteriors with
loss of marginal integrity and inadequate.
 All teeth respond positively to vitality testing of pulp.

 5mm vertical overlap with the mandibular incisors

contacting the palatal tissues.
clinically crowns
appears short due to combination of :
1. Wear

2. Altered passive eruption

3. Large jaw size

 Angles class – I molar relation.

 Bruxisum – multiple latrerotrusive & mediotrusive .

 On the basis of clinical assessment , esthetics,&

available freeway space – no loss in the patient`s
occlusal vertical dimension (OVD).
 Radiographic examination showed acceptable
crown- root ratios on all teeth and no bone loss.
TREATMENT PLAN
1. Medical –
 closely monitored by nephrologist during entire
course of treatment.
 2 gm of amoxicillin

 Avoidance of epinephrine – containing local

anesthetics to prevent changes in hypertension.
 Avoidance of all forms of nonsteroidal anti
inflammatory drugs.
2. Dental –
 Mounted diagnostic casts - ACP PDI class IV .

 Multidisciplinary team approach .

 Complete crowns on all teeth except mandibular

incisors –
1. Metal ceramic restorations except molars.

2. Complete cast crowns made of high noble alloy.

 Gingiva – crown – lengthening procedures for all
teeth except mandibular incisors.
 Elective endodontic treatment planned for maxillary
rt. 2nd premolar & mandibular left 2nd molar.
PREPROSTHETIC TREATMENT
 Existing incisal positions excepted to patient and
clinician.
 Maxillary incisal edge position assesed by 5 factors.

 A Lucia-type of acrylic resin jig fabricated over the

mandibular incisors on the cast and adjusted to
2mm increased OVD.
 Using this jig, a new centric relation record
obtained , and casts remounted on a semi-
adjustable articulator ( hanau wide vue arcon)at
planned OVD.
 The Lucia jig is a technique that promotes
neuromuscular reprogramming of the masticatory
system and allows the stabilization of the mandible
without the interference of dental contacts,
maintaining the mandible position in harmonic
condition with the musculature in normal subjects or
in patients with temporomandibular dysfunction .
 The Lucia jig is a device made with a chemically
activated acrylic resin (self-polymerized) placed
between the upper and lower anterior incisors to
de-program the proprioceptive patterns of habitual
contact between teeth, thus changing the
mandibular closure pattern.
 Diagnostic waxing accomplished on the mounted
casts.

 Patient wants closure of diastema in maxillary

anteriors in the final restoration, accordingly waxing
done.

 A partial group function occlusal scheme designed

for definitive restorations.
 Group function defined as “ multiple contact relation
between maxillary and mandibular teeth in lateral
movements on working side whereby simultaneous
contact of several teeth acts as a group to distribute
occlusal force.

 After patient exceptance of the diagnostic waxing,

crown-lengthning procedures accomplised, over a
series of 4 appointments.
 A scalloped vaccum formed matrices prepared
during the diagnostic waxing used as guides for all
crown-lengthening procedures.

 During the healing period, endodontic treatment on

2 planned teeth accomplished. i.e. maxillary rt. 2nd
premolar and mandibular left 2nd molar .
PROSTHODONTIC TREATMENT
 After 12 weeks of healing from crown-lengthening ,
initial tooth preparations performed & followed by the
cementation of interim prosthesis.
 The acrylic resin jig fabricated over the unprepared
mandibular incisors acted as an aid to maintain the
desired OVD (2mm).
 Cast dowel and core fabricated from high noble alloy
and cemented on the maxillary rt. 2nd premolar.
 The core of mandibular left 2nd molar made by using
silver amalgam material, engaging undercuts in the
pulp chamber & protruding 2 mm into the root canal.
 For the remaining tooth preparations
all previous restorations were removed and not
replaced.
Presence of caries and demineralized dentine
checked with slow-speed round bur and a caries-
detecting dye.
Special attention paid to ensure no undercuts existed
in the tooth preparations.
Grooves incorporated to augment resistance form
due to short height of prepared teeth.
 After refined final tooth preparations, the interim
prosthesis relined and cemented accordingly.
 The interim prosthesis had a partial group function
occlusion scheme, where guidance provided by the
canines and both premolars in laterotrusive
movements.

 Patient gets opportunity to experience the interim

prosthesis for 5 months for thorough evaluation of
his esthetics, function, comfort and allowed the
clinician a satisfactory assessment of patients
tolerance to the 2mm OVD increase.
 Appropriate health of soft tissues ensure prior to
making final impressions .

 Complete arch impressions of all tooth preparations

made by using polyether impression material for
both the arches .
 The master cast and dies prepared from these
impressions to proceed with fabrications of the
restorations.
 The definitive restorations then cemented with resin
– modified glass ionomer cement.
 The partial group function scheme of the interim
prostheses emulated in the definitive restorations
as well.
POSTTREATMENT THERAPY
 After final cementation, new diagnostic impressions
made.
 An occlusal device fabricated , and the patient
instructed to wear it at night.
 The patient given detailed oral hygiene instructions
and adviced to continue using the 1.1% sodium
flouride dentrifices for rest of his life , due to
predisposition for xerostomia.
 A set of new diagnostic cast provided to patient for
future monitoring of wear, especially on his
unrestored mandibular incisors and canines.
 Patient recall for maintenance after 6 months.
 After 3.5 -year recall, definitive restorations and
health of the soft tissues remained stable.
DISSCUSSION
 Important factors in the management of this patient
with fabry`s disease were:
1. Complex medical history
2. Multiple medications taken by patients.
3. Limited choice of anesthetics and analgesics,
4. Appointment durations and schedule,
5. Management of anterior diastema for esthetics,
6. Partial group function occlusal scheme,
7. Patients predisposition to xerostomia.
Choice of restorative materials for this patient based
on a confluence of factor.
 Complete gold crowns cast in high noble alloy -
molars due habit of bruxism.
 Advantage –

1. Lesser chance of fracture

2. Gold crowns are simple monolithic restorations

that required conservative tooth preparations.
 Metal ceramic crowns used as because of long
successful clinical track records .
 Unique feature related to this patient`s
prosthodontic treatment included management of
anterior diastema and use of partial group function
occlusion scheme.
REFERENCE
 Thomas J. McGarry et al:Classification System for
the Completely Dentate Patient. Journal of
Prosthodontics 2004,vol.13,issue 2, 73–82.
 Marcelo Palinkas et al:The effect of a Lucia jig for
30 minutes on neuromuscular re-programming, in
normal subjects.Braz. oral res. 2012,26,6 .
 SHUBHA R. PHADKE, KAUSIK MANDAL, K. M.
GIRISHA :Fabry disease: A treatable lysosomal
storage disorder. Natl Med J India 2009;22:20–22.
 Pastores GM, Lien YH. Biochemical and molecular genetic
basis of Fabry disease. J Am Soc Nephrol. 2002;13:S130-3
 Mehta A, Ricci R, Widmer U, Dehout F, Garcia de
Lorenzo A, Kampmann C, et al.Fabry disease
defined: Baseline clinical manifestations of 366
patients in the Fabry Outcome Survey. Eur J Clin
Invest 2004;34:236–42.