Download A CASE REPORT Dr. Ka - international journal of research in dentistry

Dr kalpna chaudhry IJRID Volume 4 Issue 4 Jul.-Aug.2014
Available online atwww.ordoneardentistrylibrary.org
ISSN 2249-488X
Case-report
INTERNATIONAL JOURNAL OF RESEARCH IN DENTISTRY
OLIGODONTIA ASSOCIATED WITH HYDROTIC ECTODERMAL DYSPLASIA: A CASE
REPORT
Dr. Kalpna Chaudhry, Dr. Chanchal Singh
Department of Pedodontics, K.D.Dental College, NH 2 , Mathura
Received: 21 June. 2014; Revised: 12Jul 2014; Accepted: 26 Aug. 2014; Available online: 5 Sep 2014
ABSTRACT
Oligodontia and associated conditions like hydrotic Ectodermal Dysplasia (HED) markedly influence on physical,
functional and psychosocial maturation of the affected individuals. Thorough evaluation, proper counselling and
careful treatment planning employing a multidisciplinary approach are keys to a successful, long-term
management. This report describes the management of a patient with oligodontia including ten permanent teeth
and six primary teeth. Adhesive techniques and new restorative techniques represent current options in the
management of the dental rehabilitation of young patients with Oligodontia. Restorative treatment was provided
for the psychosocial comfort of the young patient in this case.
Keywords: Oligodontia, hypodontia, ectodermal dysplasia.
INTRODUCTION
Congenitally missing teeth can occur in two variations, hypodontia and oligodontia. Hypodontia is
characterized by the absence of six or fewer permanent teeth, while oligodontia is characterized by the absence
of more than six permanent teeth. Congenital lack of one or more teeth, excluding the third molars is relatively
common, but oligodontia (six or more missing teeth) is rarer and may be associated with a syndrome such as
ectodermal dysplasia. Although, both environmental and genetic factors can cause failure of tooth development,
in the majority of cases, hypodontia has genetic bases. An isolated hypodontia/Oligodontia is inherited as an
autosomal dominant trait with incomplete penetration and variable expression1. Ectodermal Dysplasia (ED) is a
hereditary congenital disease that effects several ectodermal structures. Although the existence of an autosomal
recessive inheritance is reported , the disease is usually transmitted as an X - linked recessive trait . ED patients
require dental treatment primarily because of other dental needs and not always for good appearance.2-5HED
shows clinical manifestations of hypodontia, hypohidrosis, hypotrichosis and a highly characteristic facial
physiognomy, usually referred to as “fairy-like appearance”.6
The purpose of this article is to present a female patient with ED associated with numerous missing primary and
permanent teeth including all permanent canines.
75
Dr kalpna chaudhry IJRID Volume 4 Issue 4 Jul.-Aug.2014
Dr kalpna chaudhry IJRID Volume 4 Issue 4 Jul.-Aug.2014
Case Report
A 6½-year-old female child was brought to the Department of Pedodontics, seema Dental College,
rishikesh,Uttaranchal ,with chief complaint of decayed teeth and missing teeth in lower front region. The patient
was elder of the two siblings born of a non-consanguineous marriage at full term by normal delivery. Her
parents and brother appeared normal. Family history did not reveal any similar complaint from her immediate
or distant relatives. The Patient was moderately built; she was a class one student in a nearby rural school.
Extra oral examination revealed wet face appearance and glaucoma in right eye as the main findings
(Fig.1).Oral and radiographic examination showed mixed dentition with numerous carious teeth and missing
mandibular primary anterior teeth. She had no systemic disorders and also no medicine intake regularly. She
was on treatment for glaucoma by her paediatrician. No contributory family history and similar dental
manifestations were found from her parents. Radiographic examination showed, except for the third molars, 18
permanent teeth were in place, and 10 permanent teeth were absent (Fig.2). Missing teeth were 12, 13, 22, 23,
31, 32, 33, 41, 42, 43, numbers teeth. Teeth numbered 71, 72, 73,81,82,83 were also congenitally missing.
Consultation was established with the Paediatrician for the diagnosis of the case. The case was described as
Autosomal Recessive, Hydrotic Ectodermal Dysplasia. Intraoral examination revealed multiple carious
deciduous teeth including 53, 54, and 62, 63, 64, 74, 75, 84, 85, (Fig-3, 5,7). The oral hygiene was poor.
Treatment Planning: After the permission was received from the parents for the presentation of the child, full
history with clinical and radiological examination were obtained and primary carious molar teeth were treated
with glass ionomer restorations. Pulpally involved teeth i.e. 53, 64, 84 were treated with endodontic treatment.
64, 84 were obturated with resorbable paste Metapex as the succedaneous teeth were present beneath them,
whereas in 53 MTA was used at the apex and later the tooth was obturated with nonresorbable material (gutta
percha). 84 was later restored with stainless steel crown.(Fig-4,6,8) Lastly the removable partial denture was
given to replace missing teeth till the completion of the eruption of the remaining permanent teeth which would
be replaced by fixed prosthesis or implant later on (Fig -8).
Discussion :
A tooth is defined to be congenitally missing if it has not erupted in the oral cavity and is not visible in a
radiography.7In this case the absence of ten permanent teeth together with six deciduous was observed.HED and
oligodontia, though not a life-threatening condition, can have great impact on the physical, intellectual and
psychological maturation of the patient. In general hypodontia is the term most frequently used when describing
the phenomenon of congenitally missing teeth.8 Hypodontia is an anomaly that may result in dental
malpositioning, periodontal damage, lack of development of maxillary and mandibular bone height and has
76
Dr kalpna chaudhry IJRID Volume 4 Issue 4 Jul.-Aug.2014
Dr kalpna chaudhry IJRID Volume 4 Issue 4 Jul.-Aug.2014
significant psychological, aesthetic and functional consequences.9Good communication between health
professionals’ interdisciplinary team (Paediatric Dentists, Orthodontists and Prosthodontists) and also with
patient/parents/care-takers is crucial for successful outcome for these young patients. Many studies have
reported that the prevalence of congenital absence of permanent teeth varies from 3% to 11% among European
and Asian populations and it reaches 20% if the third molars are considered. The prevalence in primary
dentition is lower, ranging between 0.5% and 0.9%.10,11Although tooth agenesis is occasionally caused by
environmental factors, in the majority of cases hypodontia has a genetic basis. Developing teeth are affected by
environmental factors such as multiagent chemotherapy, radiation therapy, fractures, surgical procedures on the
jaws, extraction of the preceding primary teeth, and lack of necessary space imposed by malformed jaws12.
Patients with Oligodontia as a part of syndrome may have abnormalities in other parts the body, such as the
skin, ears, eyes, and skeleton.12Treatment of patients with Oligodontia generally requires a multidisciplinary
approach. Restoration with a removable partial denture, conventional fixed partial denture, an implant retained
prosthesis and adhesive restorative techniques, or a combination of these therapies are the treatment options.13
However, the expectation s for treatment by the patient and his parents was to achieve an esthetical result at a
low cost; a long-term treatment plan was rejected. In conclusion, missing teeth, abnormal occlusions or altered
facial appearance may cause some patients psychological distress. If the extent of hypodontia is mild, the
associated changes may likewise be slight and manageable by orthodontists. In more severe cases restorative
implants and prosthetic procedures can be undertaken. Thus, the concept of early diagnosis of these patients
becomes more important. When a case of tooth agenesis is seen, the presence of the anomaly should be
recorded with a complete clinical history including panoramic radiograms and models, for proper phenotype
characterization prior to any surgical or orthodontic treatment.
Conclusions: Congenital lack of one or more teeth, excluding the third molars is relatively common, but
oligodontia (six or more missing teeth) is rarer and may be associated with a syndrome such as ectodermal
dysplasia. Although, both environmental and genetic factors can cause failure of tooth development, in the
majority of cases, hypodontia has genetic bases. An isolated hypodontia/oligodontia is inherited as an autosomal
dominant trait with incomplete penetration and variable expression. Careful diagnosis and multidisciplinary
treatment approach can be performed as early as possible in order to prevent aesthetic and functional problems
in dentition.
References:
1. Binali Cakur, Saadettin Dagistan, Özkan Miloglu, Murat Bilge: Nonsyndromic Oligodontia in
Permanent Dentition: Three Siblings. The Internet Journal of Dental Science. 2006. Volume 3 Number
2.
2. Kupietzky A, Houpt M. Hypohidrotic ectodermal dysplasia: Characteristics and treatment. Quintessence
Int26:285 -91, 1995.
77
Dr kalpna chaudhry IJRID Volume 4 Issue 4 Jul.-Aug.2014
Dr kalpna chaudhry IJRID Volume 4 Issue 4 Jul.-Aug.2014
3. Wiedemann MD, Kunze MD, GrosseFR, Dibbern H. Ectodermal dysplasia with hair anomalies and
syndactyly. Anatlas of Clinical Syndromes. Wolfe publishing Ltd, London, England,page: 458, 1992.
4. Sabah E, Çalıskan MK, Aydyn Z. Üçolgu nedeniyle ektodermal displazinin endodontik ve protetik
tedavisi. EDFD 9 (3): 45-54, 1988.
5. Grinberg S. Ectodermal dysplasia: Report of two female cases. J Dent Child 47:193-5, 1980.
6. Bilal Ahmed and Nazia Yazdanie. Hypodontia and microdontia associated with hereditary ectodermal
dysplasia. Journal of the College of Physicians and Surgeons Pakistan 2009, Vol. 19 (3): 192-194
7. White SC, Pharoah MJ. Oral radiology principles and interpretation. In: White SC (ed). Dental
anomalies. St. Louis, PA: Mosby, 2000, pp 305-306.
8. Arte S. Phenotypic and genotypic features of familial hypodontia. Academic Dissertation. Institute of
dentistry, Universty of Helsinki, 2001.
9. Silva Meza R. Radiographic assessment of congenitally missing teeth in orthodontic patients. Int J
Paediatr Dent 2003; 13: 112-116.
10. Vastardis H. The genetics of human tooth agenesis: new discoveries for understanding dental anomalies.
Am J Orthod Dentofacial Orthop 2000; 117: 650-656.
11. Zarrinnia K, Bassiouny MA. Combined aplasia of maxillary first molars and lateral incisors: a case
report and management. J Clin Pediatr Dent 2003; 27: 127-132.
12. Schalk-van der Weide Y, Beemer FA. Symtomatology of patients with oligodontia. J Oral Rehabil 1994;
21:247–261.
13. Wagenberg BD, Spitzer DA. Therapy for patient with oligodontia: case report. J Periodontol
2000;71:510–516.
Legends For Figures :
Fig 1 Wet Face Appearance And Glaucoma In Right Eye.
78
Dr kalpna chaudhry IJRID Volume 4 Issue 4 Jul.-Aug.2014
Dr kalpna chaudhry IJRID Volume 4 Issue 4 Jul.-Aug.2014
Fig 2 Orthopentomograph (Pre-Operative)
Fig 3 Mandibular Arch (Pre-Operative)
Fig 4 Mandibular Arch (Post-Operative)
79
Dr kalpna chaudhry IJRID Volume 4 Issue 4 Jul.-Aug.2014
Dr kalpna chaudhry IJRID Volume 4 Issue 4 Jul.-Aug.2014
Fig 5 Maxillary Arch (Pre-Operative)
Fig 6 Maxillary Arch (Post-Operative)
Fig 7 Occlusal View ( Pre-Operative)
80
Dr kalpna chaudhry IJRID Volume 4 Issue 4 Jul.-Aug.2014
Dr kalpna chaudhry IJRID Volume 4 Issue 4 Jul.-Aug.2014
Fig 8 Occlusal View (Post-Operative)
Fig 9 Orthopentomograph (Post-Operative)
81
Dr kalpna chaudhry IJRID Volume 4 Issue 4 Jul.-Aug.2014