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Management of ectodermal
dysplasia with tooth-supported
computer-engineered complete
overdentures: A clinical report
Amit Punj, and Mathew T. Kattadiyil- Journal of
prosthetic dentistry(2018)
Presented by:
Dr. Areeba Zainab
Mds 1st year.
Contents:
• Introduction
• Clinical manifestations.
• Etiology.
• Types.
• Difference between hypohidrotic and hidrotic
• Hypohidrotic ED
• Prosthodontic management
• Fixed prosthodontics
• Removable prosthodontics
• Implant prosthosontics
• Master article
• Clinical report
• Technique
• Discussion
• Critical analysis
• References.
Ectodermal dysplasia
• INTRODUCTION :
• Ectodermal dysplasias are a large group of heritable
conditions characterized by congenital defects of one
or more ectodermal structures and their appendages:
• hair (hypotrichosis, partial, or total alopecia),
• nails (dystrophic, hypertrophic, abnormally
keratinized),
• teeth (enamel defect or absent), and
• sweat glands (hypoplastic or aplastic).
Itin, P. H., & Fistarol, S. K. (2004). Ectodermal dysplasias. American Journal of Medical Genetics,
131C(1), 45–51.
Clinical Manifestations in the
Ectodermal Dysplasias
Skin alteration
• Superficial dry scaling skin at birth
• Eczema
• Skin erosions
• Dark colored skin around eyes, elbows
or knees
eczema
Dry skin
Skin erosions Dark skin
under eyes
Itin, P. H., & Fistarol, S. K. (2004). Ectodermal dysplasias. American Journal of Medical Genetics,
131C(1), 45–51.
Impaired sweat gland function
Absence or reduction of
sweating
Hyperthermia under warm
condition
Abnormalities in hair follicles
Flushed body due to absent sweat glands
Sparse, curly, and fair hair
Alopecia
Eyebrows or eyelashes
absent/sparse or malformed
Thin sparse hair Absent eyebrows
and eyelashes
Itin, P. H., & Fistarol, S. K. (2004). Ectodermal dysplasias. American Journal of Medical Genetics,
131C(1), 45–51.
Nail changes:
• Abnormally
keratinised
• Thick or thin nails
• Brittle with ridges
• Abnormally curved
nails
Thick nails, abnormally curved
• Facial changes
• Dysmorphic
features
• Numerous facial
malformations Thin, brittle nails, with ridges/lines.
Dental changes
Hypodontia or anodontia
Hypodontia with peg shaped
Malformed teeth with cone- or
teeth
pegshaped aspect
Eye abnormalities
Corneal dysplasias
Cataract
Defective or decreased
lacrimation
Dye to check dry eye syndrome
Itin, P. H., & Fistarol, S. K. (2004). Ectodermal dysplasias. American Journal of Medical Genetics,
131C(1), 45–51.
ETIOLOGY:
• Ectodermal dysplasias are genetic disorders, which means that they
can be passed on from affected people to their children. They are
caused by mutations in various genes; the mutations may be
inherited from a parent, or normal genes may become mutated at
the time of egg or sperm formation, or after fertilization.
The different types of inheritance patterns that occur depending on
the specific type of ectodermal dysplasia in the family include x-
linked recessive, autosomal dominant, and autosomal recessive.
Ectodermal dysplasia- genetic and rare diseases information center
TYPES:
• Pinheiro and Freire-Maia,extensively reviewed EDs from a
clinical point of view. They encompass two groups.
Group ‘B’ includes the conditions
Group ‘A’ includes the conditions with defects in only one of the four
with defects in at least two of the above mentioned structures, plus
‘classic’ epidermal appendages at least another ectodermal defect.
with or without other The authors also distinguished
malformations or defects. ‘pure EDs’, which are exclusively
By definition, in their current characterised by ectodermal signs
classification, and ‘ED malformation syndromes
‘1’ indicates hair dysplasia, or ED syndromes’ in which
‘2’ stands for teeth dysplasia, ectodermal derivatives defects are
‘3’ nail dysplasia and associated with other
‘4’ sweat gland dysplasia. malformations in different organs
or tissues.
Pinheiro M, Freire-Maia N. Ectodermal dysplasias: a clinical classification and a causal review. Am J Med Genet 1994: 53:
153–162.
• There are mainly two forms of ED. the hidrotic and the
hypohidrotic. The teeth and the hair are affected similarly in both
forms, but the manifestations in nails and sweat glands and the
hereditary patterns tend to differ.
Bonilla ED, Guerra L, Luna O. Overdenture prosthesis for oral rehabilitation of hypohidrotic
features hypohidrotic hidrotic
Generic X-linked Autosomal dominant
transmission recessive
Facial The skull resembles Depression in the
an inverted pyramid. nasal bone.
Frontal bossing. Pronounced Prominent lips.
supraorbital Protruding ears.
Depressed nasal
ridge. Protuberant
everted lips. Fine,
linear wrinkles.
Pigmentation around
eyes and mouth.
Skin Dry. thin, soft. Dry, rough, with
smooth crusty texture.
Hyperpigmentation
in some areas
Sweat glands Severely decreased. Normal
or absent
Bonilla ED, Guerra L, Luna O. Overdenture prosthesis for oral rehabilitation of hypohidrotic
Hair Fine, scanty and Dry. Fine, usually
light-colored hypotrichosis
Nails Normal or Short, thick;
spoonshaped elevated tip
Oral manifestations Anodontia or Occasional
oligodontia. hypodontia,
Peg-shaped teeth. Anodontia,
Poor development of Widely spaced teeth.
alveolar process. Natal teeth.
Both dentitions Carious teeth
affected. Retarded
tooth eruption,
Aplasia of labial and
buccal mucous glands.
Oral mucosa appears
sticky and dry
Bonilla ED, Guerra L, Luna O. Overdenture prosthesis for oral rehabilitation of hypohidrotic
HYPOHIDROTIC ECTODERMAL DYSPLASIA
• Hypohidrotic ectodermal dysplasia (HED) is an X
linked condition. The phenotype includes sparse
scalp hair, largely absent body hair, deficiency of the
eccrine sweat glands, and anodontia or oligodontia
with conical teeth.
• There is often a distinctive facies, with prominence of
the forehead, a depressed nasal bridge, prominent
lips, and periorbital wrinkling and pigmentation.
• Subcutaneous fat is often diminished or absent, as
are the mucous glands in the respiratory tree and the
gastrointestinal tract.
Clarke A. Hypohidrotic ectodermal dysplasia. DarwinJ Med Genet. 1987 Nov;24(11):659-63.
Prosthodontic management:
• ED requires the clinician to be knowledgeable in growth and
development, behavioral management, techniques in the
fabrication of a prosthesis, the modification of existing teeth
utilizing composite resins, the ability to motivate the patient and
parent in the use of the prosthesis, and the long-term follow-up for
the modification and/or replacement of the prosthesis.
Prosthodontic management of ectodermal dysplasia: A review of the [Link] A. Pigno,
Ronald B. Blackman, Robert J. Cronin, and Edmund Cavazos. (J Prosthet Dent 1996;76:541-5.)
• There is not a definitive time to begin treatment, but Till and
Marquesz recommend that an initial prosthesis be delivered before
the child begins school so that the child has a normal appearance and
time to adapt to the prosthesis.
• Prosthetic treatment of ED may include :
Removable,Fixed or Implant-supported prostheses. These treatment
approaches can be used individually or in combination to achieve
optimal results.
Till MJ, Marques AP. Ectodermal dysplasia: treatment considerations and case reports.
Northwest Dent 1992;71:25-8.
Fixed prosthetic treatment:
• Fixed prosthodontic treatment is seldom used in these cases,
primarily because many afflicted individuals have a minimal number
of teeth. In addition, ED patients are often quite young when they are
first treated, and fixed partial dentures (FPDs) with rigid connectors
should be avoided in young, actively growing patients.
• Individual crown restorations have no age restrictions related to jaw
growth, but larger pulp sizes and shorter crown heights may cause
concerns.
Prosthodontic management of ectodermal dysplasia: A review of the [Link] A. Pigno,
Ronald B. Blackman, Robert J. Cronin, and Edmund Cavazos. (J Prosthet Dent 1996;76:541-5.)
6 maxillary PFM crowns on natural teeth, two maxillary implant cantilever FPDs,
and a mandibular five implant fixed metal-acylic prosthesis (hybrid) delivered to
a patient with ectodermal dysplasia
BEFORE AFTER
Removable prosthethic treatment:
• Removable prosthodontics is the most frequently reported
treatment modality for the dental management of ED.‘s. Because
anodontia or hypodontia is typical in individuals with this condition,
complete dentures, partial dentures, or overdentures are often part
of the treatment provided.
• Although complete dentures can provide an acceptable esthetic
when there are teeth present for support, overdentures are a
desirable treatment option for these patients.
Prosthodontic management of ectodermal dysplasia: A review of the [Link] A. Pigno,
Ronald B. Blackman, Robert J. Cronin, and Edmund Cavazos. (J Prosthet Dent 1996;76:541-5.)
• Periodic recalls of young ED patients is also important because
prosthesis modification or replacement will be needed as a result of
continuing growth and development in addition to adjustments related
to fit, the occlusion of a prosthesis must be monitored for changes
because of jaw growth. Other problems related to removable
prostheses are speech difficulties, dietary limitations, eruption of teeth
and loss of the prosthesis.
A B
REMOVABLE PROSTHESIS DELIVERED TO A PATIENT WITH
ECTODERMAL DYSPLASIA, WITH EXPANSION SCREWS IN THE MIDLINE,
TO COMPENSATE FOR THE GROWING JAWS
A. MANDIBULAR PROSTHESIS B. MAXILLARY PROSTHESIS
Implant prosthodontics:
• The literature indicates implants being used more widely in the
prosthodontic management of ED patients.
• reason to consider dental implants in the treatment of ED is the
beneficial impact they could possibly have on the preservation of
alveolar bone.
Prosthodontic management of ectodermal dysplasia: A review of the [Link] A. Pigno,
Ronald B. Blackman, Robert J. Cronin, and Edmund Cavazos. (J Prosthet Dent 1996;76:541-5.)
• Concern has been raised against the placement of osseointegrated
implants in a growing jaw. Guckes et al recommended that implant
placement be postponed until age 13 because of possible implant
movement caused by jaw growth.
M Machadoa, C Wallacea, B Austin, S Deshpande, A Laib, T Whittlea, I Klineberga . Rehabilitation of ectodermal dysplasia patients presenting with
hypodontia: outcomes of implant rehabilitation part 1 . J prosthet dent 2018
FOUR IMPLANT-SUPPORTED PROSTHESIS IN MANDIBLE DELIVERED TO A PATIENT WITH
ECTODERMAL DYSPLASIA
Prosthodontic management of ectodermal dysplasia: A review of the [Link] A. Pigno,
Ronald B. Blackman, Robert J. Cronin, and Edmund Cavazos. (J Prosthet Dent 1996;76:541-5.)
• According to Cronin et al and Oesterle et al, possible
consequences of early implant placement include:
• Implant submergence because of jaw growth,
• implant exposure because of bone resorption associated
with jaw growth,
• implant movement because of jaw growth, and
• limitation of jaw growth if the implants are connected by a
rigid prosthesis that crosses the midline.
Prosthodontic management of ectodermal dysplasia: A review of the [Link] A. Pigno,
Ronald B. Blackman, Robert J. Cronin, and Edmund Cavazos. (J Prosthet Dent 1996;76:541-5.)
A CASE REPORT OF ORTHODONTIC AND
PROSTHODONTIC TREATMENT FOR ED
The two conical anterior teeth in Elastics were attached between the
the right maxilla buttons to apply a 60-gram force
M. Yenisey,A. Guler and U. [Link] and prosthodontic treatment of
ectodermal dysplasia-a case [Link] bristish dental journal.2004,196(11)-677-9
Cemented copings showing the
Appearance of the other conical
retention grooves on the mesial
anterior teeth in the left maxilla
aspects of the incisors
after orthodontic treatment
M. Yenisey,A. Guler and U. [Link] and prosthodontic treatment of
ectodermal dysplasia-a case [Link] bristish dental journal.2004,196(11)-677-9
Appearance of the clasps
in the upper overdenture
Finished prostheses in the
patient’s mouth
M. Yenisey,A. Guler and U. [Link] and prosthodontic treatment of
ectodermal dysplasia-a case [Link] bristish dental journal.2004,196(11)-677-9
MASTER ARTICLE
CLINICAL REPORT:
• A 20-year-old woman presented to the Faculty Dental
Practice at Oregon Health and Science University for an
evaluation and consultation to improve her mastication
ability and appearance.
• She had a mild form of the condition as there were no abnormalities
with her skin, hair, or nails.
• The patient had limited financial means and was unwilling to have her
teeth extracted.
• A complete prosthodontic examination was performed which revealed
a short upper lip, well-defined mentolabial fold, and reduced OVD.
Initial presentation. A, Maximal intercuspal position frontal view. B,
Maxillary arch occlusal view. C, Mandibular arch occlusal view. D,
Panoramic radiograph
TECHNIQUE:
Irreversible hydrocolloid impressions were made of both arches by using
stock trays, and a custom tray was fabricated
Border molding was accomplished with polyvinyl siloxane (PVS) putty,
and definitive impressions were made using light-body PVS
Definitive impressions were scanned using a desk-top scanner to create a
digital file. Definitive casts were then fabricated by using dowel pins and
autopolymerizing acrylic resin in the location of the teeth, and the
impressions were poured in Type III gypsum
On the definitive casts, the remaining teeth were blocked out, and
occlusion rims were fabricated . The maxillary occlusion rim was adjusted
for esthetics, and a facebow record was made. A centric relation (CR)
record was made by using a PVS material at the restored OVD.
The dental laboratory scanned the CR record and created another
digital fileDigital files. From the definitive impressions and
maxillomandibular relation records were merged, and the denture
was designed.
Anatomic teeth were selected to achieve bilateral balanced occlusion on a
virtual articulator programmed with average values.
Maxillomandibular relation record on
definitive casts.
Virtual design images of intaglio surfaces. A, Maxillary overdenture. B, Mandibular
overdenture.
Poly(methyl methacrylate) milled trial dentures were fabricated and
returned by the dental laboratory. The trial dentures were delivered and
adjustments were made for comfort, function, and esthetics
The patient took the trial dentures home for 2 weeks to evaluate
function and comfort.
The trial dentures were then sent back to the laboratory and scanned.
The definitive monolithic dentures were made .
Follow-up appointments were made at 1 and 3 weeks and 3 months. The
patient reported being very satisfied with the outcome.
Trial dentures.
Definitive treatment. Frontal smile view.
Definitive dentures
DISCUSSION:
• Advantages of the approach included the following:
• 1. Increased strength:In this protocol, monolithic dentures
milled from a single resin block that does not rely on
individual denture teeth bonding to the denture base
were used.
• 2. Easy block out and reduced finishing time.
• 3. Better fit.
• 4. Easy duplication.
• 5. Two-week trial placement period.
CRITICAL ANALYSIS:
• No information regarding the tooth preperation steps given.
• No photographs of prelimenary steps.
• Maxillomandibular relationship not explained properly.
REFERENCES:
• Itin, P. H., & Fistarol, S. K. (2004). Ectodermal dysplasias.
American Journal of Medical Genetics, 131C(1), 45–51
• Ectodermal dysplasia- genetic and rare diseases
information center
• Priolo, M., Silengo, M., Lerone, M., & Ravazzolo, R.
(2008). Ectodermal dysplasias: not only “skin” deep.
Clinical Genetics, 58(6), 415–430
• M Machadoa, C Wallacea, B Austin, S Deshpande, A Laib,
T Whittlea, I Klineberga . Rehabilitation of ectodermal
dysplasia patients presenting with hypodontia: outcomes
of implant rehabilitation part 1. J prosthet dent 2018
• Bonilla ED, Guerra L, Luna O. Overdenture prosthesis for
oral rehabilitation of hypohidrotic ectodermal dysplasia: a
case report. Quintessence Int. 1997 Oct;28(10):657-65
• Clarke A. Hypohidrotic ectodermal dysplasia. DarwinJ Med
Genet. 1987 Nov;24(11):659-63.
• Prosthodontic management of ectodermal dysplasia: A
review of the [Link] A. Pigno, Ronald B. Blackman,
Robert J. Cronin, and Edmund Cavazos. (J Prosthet Dent
1996;76:541-5.)
• M. Yenisey,A. Guler and U. [Link] and
prosthodontic treatment of ectodermal dysplasia-a case
[Link] bristish dental journal.2004,196(11)-677-9
THANK YOU.
