[Downloaded free from http://www.jisppd.com on Tuesday, July 17, 2018, IP: 180.253.103.
29]
Case Report
Dentin dysplasia type 1 ‑ clinical management
dilemmas: A case report of first‑generation
sufferers
Navneet Grewal, Samita Gumber, Anupam Kaur1, Nirapjeet Kaur
Department of Pedodontics and Preventive Dentistry, GDC, 1Department of Genetics, Guru Nanak Dev University, Amritsar, Punjab,
India
ABSTRACT
Dentine Dysplasia is a rare genetic condition. The
treatment options and dilemmas associated with the
condition remain undiscovered so far. This article
highlights the variations in traits and challenges
faced in the treatment of the cases.
KEYWORDS: Clinical management, dentin dysplasia,
dilemmas
Introduction
Dentin dysplasia is a rare developmental disorder
affecting the dentin formation. Due to the rare
occurrence with the prevalence rate of 1:100,000[1,2] and
lack of literature, the dilemmas to treat such cases
are still unfolded which makes this condition a real
challenge for dentist to treat.[3‑7] The undermentioned
cases highlight the variations in clinical and
radiographic features and management dilemmas
faced in treating such patients.
Case Reports
Case 1 (proband)
A patient aged 17  years/male reported   to the
Department of Pedodontics and Preventive Dentistry,
GDC, Amritsar, with the chief complaint of unesthetic
appearance and history of early loss of primary
teeth and rapid attrition of permanent teeth. Family
history revealed that three more children in the family
were affected with the similar condition. Intraoral
examination of proband revealed oligodontia, attrition
of 13, 12, 11, 21, 22, 23, 33, 32, 31, 41, 42, and 43, and
mobility of 12.41  (Miller’s Grade  1). Radiographic
examination  (orthopantomogram  [OPG])  [Figure  1]
revealed multiple unerupted teeth and wide variations
in root morphology. Multiple periapical radiolucencies
were present with respect to 12, 21, 23, 31, 32, and 41.
No extra oral abnormality was detected.
© 2018 Journal of Indian Society of Pedodontics and Preventive Dentistry | Published by Wolters Kluwer - Medknow
Address for correspondence:
Dr. Samita Gumber,
Department of Pedodontics and Preventive Dentistry, GDC, S. S.
S. S. Chownk, Amritsar ‑ 143 001, Punjab, India.
E‑mail: samitagumber@gmail.com
Access this article online
Website:
Qucik response code
www.jisppd.com
DOI:
10.4103/JISPPD.JISPPD_1127_17
PMID:
******
Case 2 ‑ sibling (16 years/female)
The case presented with similar features as proband.
Hypodontia (16, 21, 26, 31, 32, 36, 37, 41, 42, 45, 46, 47,
53, 54), attrition  (16, 53, 54, 63, 65, 26, 31, 32, 36, 41,
42, 46), and an intraoral sinus with respect to 21 were
present intraorally. OPG [Figure 2] revealed tooth buds
without root formation with respect to all unerupted
teeth and periapical radiolucencies with respect to 21,
31, 32, 41 and 42.
Case 3 ‑ sibling (14 years/male)
This case presented with wider variations.
Hypodontia  (16, 11, 26, 36, 32, 32, 41, 42, 46, 53, 55,
and 63), attrited (55, 53, 63, 31, 32, 41, and 42), Angle’s
This is an open access journal, and articles are distributed under the terms
of the Creative Commons Attribution‑NonCommercial‑ShareAlike 4.0
License, which allows others to remix, tweak, and build upon the work
non‑commercially, as long as appropriate credit is given and the new
creations are licensed under the identical terms.
For reprints contact: reprints@medknow.com
How to cite this article: Grewal N, Gumber S, Kaur A, Kaur N.
Dentin dysplasia type 1 - clinical management dilemmas: A case
report of first-generation sufferers. J Indian Soc Pedod Prev Dent
2018;36:213-5.
213
[Downloaded free from http://www.jisppd.com on Tuesday, July 17, 2018, IP: 180.253.103.29]
Grewal, et al.: Dentine dysplasia and dilemmas in its management
Class  3 molar relation with collapsed anterior bite,
and severe bone resorption in mandibular posterior
region were present. OPG  [Figure  3] revealed tooth
buds present with respect to all unerupted teeth and
multiple periapical radiolucencies.
Case 4 ‑ cousin (14 years/female)
Hypodontia 16, 11, 21, 26, 31, 32, 36, 41, 42, 43, 46, 64,
74, and 75, attrition (11, 31, 32, 41, 42, and 43), Angle’s
Class  3 molar relation with collapsed anterior bite,
and severe bone resorption in mandibular posterior
region. OPG  [Figure  4] revealed tooth buds present
with respect to all unerupted teeth and complete root
formation was seen with respect to all erupted teeth
which increased the prognosis of the treatment. Rapid
changes in the eruption of tooth buds were seen in this
case.
Patients were referred to orthopedician, endocrinologist,
and a physician to rule out any syndromic association. No
positive findings were reported by any of the specialists.
A pedigree chart was prepared with the help of a
geneticist which revealed that the patients were
maternal as well as paternal cousins and no other family
member of the earlier generation was affected. Hence,
the patients became the “first‑generation sufferers.”
Chromosomal analysis
No abnormality in the size, shape, and count of
chromosomes was reported by the geneticist.
Figure 1: Orthopantomogram showing the radiographic findings of
Case 1
Figure 3: Orthopantomogram showing the radiographic findings of
Case 3
214 Journal of Indian Society of Pedodontics and Preventive Dentistry | Volume 36 | Issue 2 | April-June 2018
Histopathological analysis
Histopathological analysis of ground sections of
extracted permanent teeth revealed the presence
of patchy stained dentin with irregularly formed
dentinal tubules and osteodentin with entrapped
odontoblasts [Figure 5].
Based on clinical, radiographic, histological, karyotype
findings and pedigree analysis, the condition was
diagnosed as dentin dysplasia Type 1.
Treatment and dilemmas
The ultimate treatment in such cases will be implants.
However, the option of implants was restricted due
to the age of the patients. Multiple interim treatments
were performed so as to preserve the bone as much
as possible and hence attempts were made to preserve
the natural teeth but the teeth with poor prognosis
were extracted. Root canal treatments and interim
restorations were done. The biomechanical preparation
had to be done with great caution to avoid iatrogenic
errors of roots with abnormal morphology. Crown
lengthening was done in mandibualr anteriors as their
crowns were severally attrited. Since their prognosis
could not be predicted, acrylic crowns were chosen
as an interim treatment. Stainless steel crowns were
cemented to prevent further attrition of mandibular
molars followed by interim conventional removable
partial dentures with respect to unerupted premolars.
Figure 2: Orthopantomogram showing the radiographic findings of
Case 2
Figure 4: Orthopantomogram showing the radiographic findings of
Case 4
[Downloaded free from http://www.jisppd.com on Tuesday, July 17, 2018, IP: 180.253.103.29]
Figure 5: Histopathological picture of the affected permanent
tooth (21) of Case 2
The erratic eruption of teeth was unpredictable. The
conventional dentures created interference in the path
of eruption of teeth and the bony bulges created by the
erupting teeth lead to pain in the insertion of dentures.
Hence, new interim flexible removable partial dentures
were made for the patients later on.
Discussion
Dentin dysplasia may vary in clinical expression,
either in isolation or with other congenital anomalies,
presented in such severe form as in this case report
is a challenge to maintain the form and function of
teeth and meet long‑term need of patients. Morris
and Augsburger described dentin dysplasia  (type  1)
to be associated with sclerotic bone which could be an
explanation to the delayed eruption of the tooth buds.
However, the erratic eruption of these buds could only
be explained on the basis of early loss of gubernacular
cord and any other physiological guidance to these
buds.
All the cases are on a constant follow‑up of every
3 months for the adjustment of dentures according to
the eruption status of teeth in the interim period. Root
Journal of Indian Society of Pedodontics and Preventive Dentistry | Volume 36 | Issue 2 | April-June 2018
Grewal, et al.: Dentine dysplasia and dilemmas in its management
canal treated teeth and prosthesis are clinically and
radiographically asymptomatic till date. Under the
limitations of the treatment provided to the patients,
more options will be explored such as whole ridge
augmentation with bone grafts followed by implants
after completion of their growth.
Declaration of patient consent
The authors certify that they have obtained all
appropriate patient consent forms. In the form the
patient(s) has/have given his/her/their consent for
his/her/their images and other clinical information
to be reported in the journal. The patients understand
that their names and initials will not be published and
due efforts will be made to conceal their identity, but
anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
References
1. Kim JW, Simmer JP. Hereditary dentin defects. J Dent Res
2007;86:392‑9.
2. Rushton MA. A case of dentinal dysplasia. Guys Hosp Rep
1939;89:369‑73.
3. Steidler NE, Radden BG, Reade PC. Dentinal dysplasia:
A clinicopathological study of eight cases and review of the
literature. Br J Oral Maxillofac Surg 1984;22:274‑86.
4. Witkop CJ Jr. Hereditary defects of dentin. Dent Clin North
Am 1975;19:25‑45.
5. Logan J, Becks H, Silverman S Jr., Pindborg JJ. Dentinal
dysplasia. Oral Surg Oral Med Oral Pathol 1962;15:317‑33.
6. Sauk JJ Jr., Lyon HW, Trowbridge HO, Witkop CJ Jr. An
electron optic analysis and explanation for the etiology
of dentinal dysplasia. Oral Surg Oral Med Oral Pathol
1972;33:763‑71.
7. Wesley RK, Wysoki GP, Mintz SM, Jackson J. Dentin
dysplasia type I. Clinical, morphologic, and genetic studies of
a case. Oral Surg Oral Med Oral Pathol 1976;41:516‑24.
215