Bala, Madhu; Pathak, Anuradha
The hereditary condition known as ectodermal dysplasia is characterized by the absence or defect of two or more ectodermally derived structures. The most commonly observed forms of ectodermal dysplasia are the hidrotic and hypohidrotic types; discrimination is based on the absence or presence of sweat glands. A case of 8-year-old male child with hypohidrotic ectodermal dysplasia with complete anodontia of primary as well as secondary dentitions is presented. The child had a short stature, low intelligent quotient (I.Q.,), and was underweight. The patient experienced episodes of high fever, was intolerant to heat, and did not sweat. He exhibited smooth and dry skin, sparse light-colored eyebrows. Dental clinicians can be the first to diagnose ectodermal dysplasia due to the absence of teeth.
Consolaro, Alberto; Cardoso, Maurício Almeida; Consolaro, Renata Bianco
ABSTRACT The relationship between maxillary lateral incisor anodontia and the palatal displacement of unerupted maxillary canines cannot be considered as a multiple tooth abnormality with defined genetic etiology in order to be regarded as a “syndrome”. Neither were the involved genes identified and located in the human genome, nor was it presumed on which chromosome the responsible gene would be located. The palatal maxillary canine displacement in cases of partial anodontia of the maxillary lateral incisor is potentially associated with environmental changes caused by its absence in its place of formation and eruption, which would characterize an epigenetic etiology. The lack of the maxillary lateral incisor in the canine region means removing one of the reference guides for the eruptive trajectory of the maxillary canine, which would therefore, not erupt and /or impact on the palate. Consequently, and in sequence, it would lead to malocclusion, maxillary atresia, transposition, prolonged retention of the deciduous canine and resorption in the neighboring teeth. Thus, we can say that we are dealing with a set of anomalies and multiple sequential changes known as sequential development anomalies or, simply, sequence. Once the epigenetics and sequential condition is accepted for this clinical picture, it could be called “Maxillary Lateral Incisor Partial Anodontia Sequence.” PMID:29364376
Early functional, esthetic, and psychological rehabilitation of preschool child with nonsyndromic oligodontia and anodontia in mixed dentition stage through conservative systematic approach: A case report with 5-year follow-up
Full Text Available Missing teeth are a common developmental abnormality in humans. It may manifest as absence of varying numbers of primary and/or secondary teeth. Early treatment and follow-up are the key to successful rehabilitation of young patients with congenitally missing teeth. It is critical that oral rehabilitation is started early to maintain and correct the oral functions. Mucosa borne removable prostheses are the commonly selected treatment options for the young patients who present with oligodontia or anodontia. This clinical report describes esthetic, functional, and psychological rehabilitation of a young boy with severe oligodontia in maxillary arch and anodontia in mandibular arch. The individualized conservative graded approach in prosthetic rehabilitation with removable acrylic prosthesis helped to achieve esthetics, functionality, and psychological benefits.
Full Text Available Objective of this study was to determine reproductive performance of A. philippiana. Samples were collected from the oil affected mangrove mudﬂat in Pototan, Guimaras, Philippine on September 2007. A. philippiana were induced to spawn using the serotonin method. Only A. philippiana with shell length of approximately 4.0-5.5 cm were induced to spawn. Three pairs of one ripe female and one ripe male were chosen and placed in aquaria with 3 replicates. A 0.3 ml of 4 rnM serotonin solution (Gros et al., 1997 was injected into 1-2 mm of the gonad of both male and female clams using 0.65 x 25-mm bore hypodermic needle attached to a 5 ml plastic syringe during mid until late afternoon. Number of spawned eggs was calculated, and fertilization was conducted. At 47 h, the percentage of normal (D-larvae veliger relative to the initial number of eggs was calculated (Massapina et aL, 1999. Larvae from each spawner were reared separately in aquaria for several days without feeding in order to estimate survival rates. The number of larvae we re-estimated every 24-h intervals until total mortality. The decrease in the number of larvae per container we re-calculated as the proportion of live larvae from the initial number of larvae (extinction rate (Narvarte and Pascual, 2003. Result of this study are total Total spawned eggs (x10 (g m is 86.11±3.80, Fertilization rate (% is 83.01±3.13, and harching rate is 36.51+8.64, Length of newly hatched larvae (gm is 135.73±1.96, Number of days to total mortality (after hatching without feeding is 9-10 days.Keywords: reproductive, Anodotia philippiana
Shivayogi M Hugar
Full Text Available A case of prosthetic rehabilitation with complete dentures for a three year nine months old child is presented. Dental prosthesis are frequently used to avoid psychologic, speech, or swallowing problems in preschooler children. The case was followed up for a period of three years. These prosthesis were modified as the child grew and as the permanent teeth erupted into the oral cavity the dentures were trimmed from the areas of erupting teeth to facilitate their eruption.
Prasad Rao, V. Arun; Reddy, N.Venugopal; Krishnakumar, R.; Sugumaran, D.K.; Mohan, G.; Senthil Eagappan, A.R.
Dental anomalies of number and forms may occur in the primary and permanent dentition. Various terms have been used to describe dental twinning anomalies: gemination, fusion, concrescence, double teeth, cojoined teeth, twinned teeth, geminifusion and vicinifusion. Double tooth is a term used to describe connate tooth and includes both dental fusion and gemination. The phenomenon of gemination occurs when two teeth develop from one single bud leading to a larger...
Nádia Carolina Teixeira Marques
Full Text Available Complete and partial removable dentures have been used successfully in numerous patients with oligodontia and/or anodontia. However, there is little information in the literature regarding the principles and guidelines to prosthetic rehabilitation for growing children. This case report describes the management of a young child with oligodontia as well as the treatment planning and the prosthetic rehabilitation technique.
Teixeira Marques, Nádia Carolina; Gurgel, Carla Vecchione; Fernandes, Ana Paula; Lima, Marta Cunha; Machado, Maria Aparecida Andrade Moreira; Soares, Simone; Oliveira, Thais Marchini
Complete and partial removable dentures have been used successfully in numerous patients with oligodontia and/or anodontia. However, there is little information in the literature regarding the principles and guidelines to prosthetic rehabilitation for growing children. This case report describes the management of a young child with oligodontia as well as the treatment planning and the prosthetic rehabilitation technique. PMID:24175103
Items 1 - 35 of 35 ... Vol 7, No 1 (2017), A survey of self-reported oral health practices, behaviour and oral health status of pregnant women attending TBA ante-natal clinics in a ... Vol 3, No 1-2 (2006), Prosthetic management of an 11-year-old patient with hereditary ectodermal dysplasia and partial anodontia – a case report.
Full Text Available Ectodermal dysplasia is characterized by the absence or defects of two or more ectodermally derived structures. Anodontia or hypodontia is the most striking dental manifestation. In severe hypodontia, there is lack of alveolar development with consequent protrusion and eversion of the lips. Patients with anhidrotic forms suffer from heat intolerance due to lack of sweat glands and mild infections may lead to death in infancy from hyperthermia. A case of a 4-year-old child with anhidrotic ectodermal dysplasia with partial anodontia is presented. Dental, oral, and physical features were taken into consideration in diagnosis and treatment planning for this patient. Clinical management consisted of removable partial prosthesis in maxillary arch and complete denture prosthesis in mandibular arch. The main aim of the treatment was to improve psychological development and to promote better functioning of the stomatognathic system.
Full Text Available Christ-Siemens-Touraine (CST is a rare hereditary disorder of X-linked recessive trait, characterized by abnormal development of two or more structures or tissues of ectodermal origin. The common clinical findings include hypodontia, hypohydrosis, hypotrichosis, and onychodysplasia. Although hypodontia is common, anodontia is a rare feature. Most of the patients are suffering from social rejection and consequent psychological trauma because of the facial dysmorphism and absence of multiple teeth. Oral rehabilitation is of prime importance for such patients. This article presents a case in a 5½-year-old boy presenting with altered manifestations affecting almost all the ectodermal structures like skin, hair, nails, teeth, sebaceous glands, sweat glands, salivary glands, mammary glands, and tear glands. He also had complete anodontia and dry mouth. A multidisciplinary treatment was given to the patient with the collaboration of various health professionals. The child gained confidence and was relieved from the psychological impact following the prosthetic rehabilitation.
Retnakumari, N; Varghese, Manuja; Kannan, V P
Christ-Siemens-Touraine (CST) is a rare hereditary disorder of X-linked recessive trait, characterized by abnormal development of two or more structures or tissues of ectodermal origin. The common clinical findings include hypodontia, hypohydrosis, hypotrichosis, and onychodysplasia. Although hypodontia is common, anodontia is a rare feature. Most of the patients are suffering from social rejection and consequent psychological trauma because of the facial dysmorphism and absence of multiple teeth. Oral rehabilitation is of prime importance for such patients. This article presents a case in a 5½-year-old boy presenting with altered manifestations affecting almost all the ectodermal structures like skin, hair, nails, teeth, sebaceous glands, sweat glands, salivary glands, mammary glands, and tear glands. He also had complete anodontia and dry mouth. A multidisciplinary treatment was given to the patient with the collaboration of various health professionals. The child gained confidence and was relieved from the psychological impact following the prosthetic rehabilitation.
Santosh R Patil; Aileni Kaladhar Reddy
Human dentition is affected by a wide variety of abnormalities, which include variation in the number, morphology and eruption sequence. Absence of tooth development manifests as anodontia, hypodontia, and oligodontia. Taurodontism is a developmental anomaly affecting the teeth leading to minimal or no constriction cement enamel junction level manifesting as long pulp spaces and also the trunk of the roots is displaced toward apex giving a rectangular shape to the involved tooth. Its commonly...
Full Text Available Concomitant hypodontia and hyperdontia is a rare condition of unknown etiology. One such case of occurring in identical twins in mixed dentition is presented and discussed. A sibling, especially a twin of an affected patient, should be suspected of having a similar problem, even if he or she is asymptomatic. Genetic factors probably play an important etiological role in the co-occurence of partial anodontia and supernumerary teeth.
Full Text Available The ectodermal dysplasias are a heterogenous group of diseases, which have one or more anomalies of the hair, teeth, nails, and sweat glands. Hypohidrotic ectodermal dysplasia (HED is the most common type and is usually transmitted as an X-linked recessive trait. It is characterized by classical triad of hypotrichosis, anhidrosis/hypohidrosis, and hypodontia/anodontia. Here, we describe an Indian boy affected with HED and rare features including ankylosis of temporomandibular joint and cleft palate.
Full Text Available The aim was to present the successful esthetical and functional rehabilitation of partial anodontia in a case of severe ectodermal dysplasia with complete atrophy of the jaws. A 17-year-old male with Class III malocclusion with partial anodontia sought dental implant treatment. His expectation was that of Class I occlusion. The challenge in the case was to match the expectation, reality, and the clinical possibilities. Ridge augmentation was performed with a combination of rib graft and recombinant human bone morphogenetic protein-2. Simultaneously, 6 implants (Nobel Biocare™ - Tapered Groovy were placed in maxillary arch and 10 in the mandible. Simultaneous placement ensured faster and better osseointegration though a mild compromise of the primary stability was observed initially. After adequate healing, Customized Zirconia Procera™ system was used to build the framework. Zirconia crown was cemented to the framework. Radiological and clinical evidence of osseointegration was observed in all 16 dental implants. Successful conversion of Class III to Class I occlusion was achieved with the combination of preprosthetic alveolar ridge augmentation, Procera™ Implant Bridge system. Abnormal angulations and or placement of dental implants would result in failure of the implant. Hence conversion of Class III to Class I occlusion needs complete and complex treatment planning so that the entire masticatory apparatus is sufficiently remodeled. Planning should consider the resultant vectors that would otherwise result in failure of framework or compromise the secondary stability of the dental implant during function. A successful case of rehabilitation of complex partial anodontia is presented.
Full Text Available Congenitally missing teeth are the most commonly seen dental anomalies. Agenesis of the permanent first molar has the least frequency of all the tooth types, and it usually occurs in association with oligodontia or anodontia. Thus, agenesis of the bilateral maxillary first permanent molar is an extremely rare occurrence, and no such case has been reported in ethnic Saudi Arabian population. We hereby report a case of nonsyndromic bilateral congenitally missing maxillary first permanent molar in an eight-year-old Saudi female patient. Comprehensive oral rehabilitation was done for the patient. The implications of the tooth agenesis are also discussed. The prognosis of this case is presented.
Full Text Available Dental agenesis is the most common developmental anomaly in humans, which can be either in the form of anodontia, oligodontia, or hypodontia. Oligodontia can occur either as an isolated finding or as part of a syndrome. Nonsyndromic oligodontia is rare. Management requires integrated multidisciplinary approach. Prompt intervention improves quality of life. Here, we report a case of isolated, nonsyndromic oligodontia in a 13-year-old female who allegedly had complete set of primary teeth but failed to develop complete permanent dentition. No other clinical feature, except oligodontia, was present that could suggest any syndromic association.
Tarjan, Ildiko; Gabris, Katalin; Rozsa, Noemi
Hypohidrotic ectodermal dysplasia is a hereditary disorder of ectodermal origin. The early orthodontic treatment of 2 young boys suffering from hypohidrotic ectodermal dysplasia with partial maxillary and complete mandibular anodontia of the primary dentition is described. Both were treated with removable maxillary partial and mandibular complete dentures with individualized occlusion and age-appropriate artificial teeth. The results were significant improvements in speech, masticatory function, and facial esthetics, contributing to the development of normal dietary habits, and the improved and more rapid social integration of these children.
Full Text Available Patients with ectodermal dysplasia or having under‑ gone cleft surgery with anodontia or hypodontia, hypo‑ plastic conical teeth and patients with severely worn dentition are difficult to treat because of the poor remaining tooth structure. These patients often exhibit loss of vertical dimension of occlusion and aesthetic problems and usually need complex prosthetic treatments. Financial constraints or other priorities often restrict one from choosing the most desirable treatment. The overlay removable denture is a covering prosthesis partially supported by natural teeth, tooth roots, or dental implants, providing an efficient alter‑ native of treatment. Clinical reports describe the various applications of overlay dentures.
Full Text Available Hereditary hypohidrotic ectodermal dysplasia, also called the Christ-Siemens-Touraine Syndrome is characterized by congenital dysplasia of one or more ectodermal structures and is manifested by hypohidrosis, hypotrichosis and hypodontia. It is usually an X-linked recessive mendelian character which is rarely seen in males. It results from abnormal morphogenesis of cutaneous and oral embryonic ectoderm. Patients with this disorder exhibit smooth , thin and dry skin, fine and blond scanty hair. Intra-orally anodontia or hypodontia, with impaired development of alveolar process is seen. A case report of a rare case of this disorder in a female patient aged 18 years is hereby presented.
Nurul Hasyiqin Fauzi
Full Text Available Summary: Tooth agenesis in the reduction of tooth number which includes hypodontia, oligodontia and anodontia is caused by disturbances and gene mutations that occur during odontogenesis. To date, several genetic mutations that unlock the causes of non-syndromic tooth agenesis are being discovered; these have been associated with certain illnesses because tooth development involves the interaction of several genes for tooth epithelium and mesenchyme odontogenesis. Mutation of candidate genes PAX9 and MSX1 have been identified as the main causes of hypodontia and oligodontia; meanwhile, AXIN2 mutation is associated with anodontia. Previous study using animal models reported that PAX9-deficient knockout mice exhibit missing molars due to an arrest of tooth development at the bud stage. PAX9 frameshift, missense and nonsense mutations are reported to be responsible; however, the most severe condition showed by the phenotype is caused by haploinsufficiency. This suggests that PAX9 is dosage-sensitive. Understanding the mechanism of genetic mutations will benefit clinicians and human geneticists in future alternative treatment investigations. Keywords: Hypodontia, Oligodontia, Mutation, PAX9
Full Text Available Ectodermal dysplasia is a hereditary disease characterized by a congenital dysplasia of one or more ectodermal structure and their accessory appendeges. The main feature is hypohidrosis, hypotrichosis and hypodontia. Ectodermal dysplasia appear to be ingeritaed as an x-linked recessive trait. Common manifestations include frontal bossing with prominent supraorbital ridges, nasal bridge depression, protuberant lips and defective hair follicles and eyebrows. Intraorally, common findings are hypodontia or anodontia and conical teeth. The treatment is to improve the appearance and oral function included a removable prosthesis. A 5-year-old and 9-year-old male in the same family, described in the case report of ectodermal dysplasia with removable partial dentures
Santosh R Patil
Full Text Available Human dentition is affected by a wide variety of abnormalities, which include variation in the number, morphology and eruption sequence. Absence of tooth development manifests as anodontia, hypodontia, and oligodontia. Taurodontism is a developmental anomaly affecting the teeth leading to minimal or no constriction cement enamel junction level manifesting as long pulp spaces and also the trunk of the roots is displaced toward apex giving a rectangular shape to the involved tooth. Its commonly seen in permanent teeth is common and rarely in the deciduous dentition. Oligodontia is an uncommon genetic condition representing the congenital missing of six teeth or more in primary or permanent dentitions. This case report describes concomitant occurrence of tooth agenesis and taurodontism in a young Saudi girl and a brief review regarding the etiology, clinical features, and therapeutic aspects of have been mentioned.
Renkerová, M; Badura, S; Manicová, H; Jambor, J
The authors investigated the frequency of unfounded teeth in a selected cohort of jaw-orthopedic patients from the iZlina district at the age of seven to 12 years. From the total of 4,405 patients, a decreased number of teeth was found in 179 children (4%), 130 of them being girls and 69 boys. In all the 179 children, 410 teeth missing. Three teeth were missing per one child on the average. Patients with cleft disorders and evident displasiae were excluded from the investigation. The third molars were not considered in the evaluation. Most of the affected children live in localities where they are exposed to air pollution. These patients displayed various aplasiae of tooth groups, where event constant tooth groups (ocular teeth) were not exceptional. Recent data from the world literature present various patterns of partial anodontia as a result of numerous genetic-mutational interreactions of living environment.
Elif Oral Ahiskalioglu
Full Text Available ABSTRACT Ectodermal dysplasias are rare conditions with a triad of hypotrichosis, anodontia and anhidrosis. In literature review there have been only a few reports of anesthetic management of patients with ectodermal dysplasias. Hyperthermia is a very serious risk which may occur due to the defect of sweat glands. The present case involves a 10-year-old child with ectodermal dysplasia who presented with an acute abdomen and was considered for an emergency surgery. Our aim was to demonstrate the successful management of this case using a combination of general and epidural anesthesia. It is important for anesthesiologist to have information about this syndrome in case of emergency operations, since it can prevent serious complications and even save lives.
Marsillac, Mirian de Waele Souchois de; Andrade, Marcia Rejane Thomas; Fonseca, Raquel de Oliveira; Marcal, Sonia Lucia Macedo; Santos, Vera Lucia Campos
A panoramic radiograph is more likely utilized in children with high caries risk and mixed dentition, and it can be complemented by other X-rays (such as periapical and/or bitewings). This study analyzed 1359 panoramic radiographs taken over 33 years at the Pedodontics Clinic of the State University of Rio de Janeiro in order to determine the prevalence of dental anomalies in mixed dentition children. The population evaluated had 670 (49.3%) boys and 689 (50.7%) girls, ranging in age from 5-12 years, with a median age of 8 years. The total prevalence of anomalies detected was 11.72%; anodontia and supernumerary teeth were the most reported (4.63% and 3.31%, respectively). Statistical differences noted were in the presence of supernumary teeth in males (4.9%, P dentition, as well as complement the clinical examination at the first dental visit of a pediatric patient with a high risk for caries.
Karthik M. Sadashiva
Full Text Available Anhidrotic ectodermal dysplasia is a triad of hypodontia or anodontia, hypotrichosis, and hypohydrosis, associated with other problems that result from the defective development of structures of ectodermal origin (Freire-Maia, Pinheiro (1988. Early and extensive dental treatment is needed keeping in mind the effect on the craniofacial growth. Due to rapid growth of the jaws, the patients are rehabilitated using removable prostheses (Tarjan et al. (2005. Hence for a young patient in this case report, the placement of endosseous osseointegrated implants was delayed till adulthood. Finally a definitive fixed tooth-supported and osseointegrated implant supported fixed partial denture therapy was used to rehabilitate the patient satisfactorily after she had completed her growth (Sweeney et al. (2005. A review of the current literature relevant to several aspects of syndromic hypodontia, patient selection, and implant planning is discussed.
Toomarian, Lida; Ardakani, Mohammad Reza Talebi; Ramezani, Jamileh; Adli, Amin Rezaei; Tabari, Zahra Alizadeh
Ectodermal dysplasia (ED) is an inherited disorder that affects ectodermally derived organs, such as teeth. Pathogenesis is thought to involve an altered epithelium-mesenchymal interaction. ED patients have oligodontia (or sometimes anodontia) in addition to other abnormalities involving the skin, sweat glands, or hair. Many different subtypes have been introduced in the literature. This article describes the case of a 4-year-old patient who, after being diagnosed with ED, was put on a treatment plan that involved mandibular implants, reshaping of the maxillary primary central incisors, and prosthetic dental rehabilitation. Due to the child's rapid growth, both dentures were changed 9 months post-treatment. Two years post-treatment, the maxillary denture was changed again and the child was placed under close supervision.
Jain, Neha; Naitam, Dinesh; Wadkar, Arti; Nemane, Anuradha; Katoch, Shiva; Dewangan, Ashish
Hereditary ectodermal dysplasia is a rare group of inherited disorders characterized by aplasia or dysplasia of two or more tissues of ectodermal origin such as hair, nails, teeth, and skin. The dental characteristics of this syndrome include anodontia or hypodontia of the primary and/or permanent teeth, hypoplastic conical teeth, and underdevelopment of the alveolar ridges. The options for a definitive treatment plan include fixed, removable or implant-supported prostheses, singly or in combination. This clinical report describes the prosthetic rehabilitation of an 11-year-old boy with hereditary ectodermal dysplasia. Maxillary flexible removable partial denture and mandibular conventional complete denture were fabricated to establish an acceptable masticatory function, speech, and esthetics for the patient. PMID:23320200
Full Text Available Hereditary ectodermal dysplasia is a rare group of inherited disorders characterized by aplasia or dysplasia of two or more tissues of ectodermal origin such as hair, nails, teeth, and skin. The dental characteristics of this syndrome include anodontia or hypodontia of the primary and/or permanent teeth, hypoplastic conical teeth, and underdevelopment of the alveolar ridges. The options for a definitive treatment plan include fixed, removable or implant-supported prostheses, singly or in combination. This clinical report describes the prosthetic rehabilitation of an 11-year-old boy with hereditary ectodermal dysplasia. Maxillary flexible removable partial denture and mandibular conventional complete denture were fabricated to establish an acceptable masticatory function, speech, and esthetics for the patient.
Sainsbury, Anthony W; Kountouri, Amalia; DuBoulay, George; Kertesz, Peter
Ninety-one red squirrels (Sciurus vulgaris) found dead in the UK between January 1994 and August 1998 were necropsied at the Institute of Zoology (London, UK); their oral cavities were examined visually, and in eight cases, radiographically. Four red squirrels, which had evidence of oral disease when necropsied as part of a mortality survey, also were examined. A low prevalence (prevalence = 0.033, SE = 0.02, n = 91) of oral disease was found in free-living red squirrels. In only two cases was oral disease the probable cause of death. Attrition of the check teeth (three cases) and overgrowth of the incisors (four cases) were the most common lesions found. Partial anodontia was recorded in one squirrel.
Full Text Available Ectodermal dysplasia represents a rare syndrome affecting two or more ectodermally derived structures. The condition is thought to occur in approximately 1 in every 100,000 live births. It affects men more frequently and severely, while women being the carriers and heterozygote usually show minor defects. There are more than 150 different variants of ectodermal dysplasia (ED reported in the literature. Most commonly encountered among them is hypohidrotic ED which frequently exhibits the most severe dental anomalies like hypodontia or anodontia along with hypohidrosis and hypotrichosis. Here we make an attempt to collectively report and discuss eleven South Indian patients who reported to our department during the year 1998 to 2004. An added emphasis is laid on family history of consanguineous marriage among the parents of these patients.
Dharmil C Doshi
Full Text Available Oculodentodigital dysplasia is a rare, autosomal dominant disorder with high penetrance and variable expressivity, caused by mutations in the connexin 43 or gap junction protein alpha-1 gene. It has been diagnosed in fewer than 300 people worldwide with an incidence of around 1 in 10 million. It affects many parts of the body, particularly eyes (oculo, teeth (dento, and fingers and/or toes (digital. The common clinical features include facial dysmorphism with thin nose, microphthalmia, syndactyly, tooth anomalies such as enamel hypoplasia, anodontia, microdontia, early tooth loss and conductive deafness. Other less common features are abnormalities of the skin and its appendages, such as brittle nails, sparse hair, and neurological abnormalities. To prevent this syndrome from being overlooked, awareness of possible symptoms is necessary. Early recognition can prevent blindness, dental problems and learning disabilities. Described here is the case of a 21-year-old male who presented to the ophthalmology outpatient department with a complaint of bilateral progressive loss of vision since childhood.
Full Text Available Prosthodontic rehabilitation can be accomplished with fixed, overdenture, complete, or implant-retained prostheses. Dental treatment overcomes the patient’s functional, psychological, esthetic and phonation problems. Remaining healthy teeth may allow the dentist to fabrícate a removable partial overdenture, fixed partial prosthesis or implant - supported prosthesis. The retention of a number of abutments helps maintain a positive ridge form with greater height and volume of the alveolar bone, improving masticatory performance, as well as providing a more stable prostheses. Dental patients who have medical problems need many treatment procedures. Multidisciplinary treatment planning is invaluable for patient’s dental health. Progeria is a rare genetic condition where symptoms resembling aspects of aging are manifested at an early age. characteristic clinical findings of Progeria disease include abnormalities of the skin and hair in conjunction with char-acteristic facial features and skeletal abnormalities. The characteristic facies show protruding ears, beaked nose, thin lips with centrofacial cyanosis, prominent eyes, frontal and parietal bossing with pseudohydrocephaly, midface hypoplasia with micrognathia and large anterior fontanel. The other reported anomalies are dystrophic nails, hypertrophic scars and hypoplastic nipples. The findings that are nearly interested in dentistry are delayed dentition, anodontia, hypodontia, or crowding of teeth. This article presents the multidisciplinary dental treatment planning includes surgical, endodontic and prosthetic treatment of a patient with a history of progeria. In this case complete-arch fixed prostheses in both maxilla and mandible, supported by a combination of im-plants and teeth are reported.
Emilija Bajraktarova Valjakova
Full Text Available Introduction: Ectodermal dysplasia (ED is a hereditary disorder associated with developmental disorders of two or more structures of ectodermal embryonic origin. Hypodontia or anodontia of the primary and permanent dentition, poorly developed alveolar ridges and improper maxillo – mandibular relations, are the most common oral manifestations. Management of patients with ectodermal dysplasia requires a multidisciplinary team approach. Case presentation: A 6.5 year-old boy with hypohidrotic ectodermal dysplasia (HED presented in this article, had typical features of HED: hypohidrosis, hypotrichosis, severe hypodontia, atrophic alveolar ridges, old-looking facial expression. According to the patient’s age and clinical findings, removable complete over-denture prosthesis in both arches was the treatment of choice. Conclusion: In patients with ED, it is important to establish correct maxillo – mandibular relations and normal function of the dento-facial system (chewing, swallowing, and speaking. Prosthodontic treatment has a major impact on aesthetics and functions,facilitates psychological development and improves emotional condition and social life of the patient.
Mehndiratta, Sumit; Tyagi, Amita; Devgan, Veena
Ellis-van Creveld syndrome (EVC syndrome, MIM 225500) or chondroectodermal dysplasia is a rare, autosomal recessive disorder. This syndrome is characterized by a tetrad of chondrodystrophy, post axial polydactyly, and hidrotic ectodermal dysplasia, mostly involving teeth and nails and a high frequency of congenital cardiac anomalies, most frequently a common atrium. The genetic basis of this disorder has been identified as mutations in the Evc and Evc2 genes. We present a report of two affected siblings with features consistent with those of the syndrome. A 2-month-old child with features of lower respiratory tract infection was admitted to the pediatric emergency department. Detailed examination revealed skeletal anomalies such as limb shortening and polydactyly in both hands. On cardiac evaluation, ventricular septal defect was found. There were no neonatal teeth. A diagnosis of EVC syndrome was made based on the findings. Screening of family members revealed that the elder sibling had features consistent with those of EVC syndrome. He was 4 years old, yet undiagnosed with short bones, polydactyly, partial anodontia and ventricular septal defect. The third child and the parents were unaffected. The treatment of this disorder is primarily supportive particularly for associated cardio-respiratory problems. The parents were extensively counseled for regular follow-up. The diagnosis of this syndrome is based on clinical grounds supported by radiological evaluation. Prenatal diagnosis is possible by ultrasonography and genetic testing. Genetic counseling is required to make the parents aware of the risk of recurrences.
Sabbagh-Haddad, Aida; Haddad, Denise Sabbagh; Michel-Crosato, Edgard; Arita, Emiko Saito
The purpose of this study was to evaluate the dental radiographic characteristics as described in 40 records of patients with panoramic radiography. The patients were in the range of 6-17 years old, and were divided into two groups (20 subjects who were compatible with the normality standard and 20 individuals diagnosed with the FXS), which were matched for gender and age. Analysis of the panoramic radiographic examination involved the evaluation of dental mineralization stage, mandibular angle size, and presence of dental anomalies in both deciduous and permanent dentitions. The results of radiographic evaluation demonstrated that the chronology of tooth eruption of all third and second lower molars is anticipated in individuals with FXS (p<0.05). In this group, supernumerary deciduous teeth (2.83%), giroversion of permanent teeth (2.31%), and partial anodontia (1.82%) were the most frequent dental anomalies. In addition, an increase was observed in the mandibular angle size in the FXS group (p<0.05). We conclude that knowledge of dental radiographic changes is of great importance for dental surgeons to plan the treatment of these individuals.
Ceylan, Gözlem; Yılmaz, Nergiz; Şenyurt, Özgün; Kunt, Göknil Ergün
Prosthodontic rehabilitation can be accomplished with fixed, overdenture, complete, or implant-retained prostheses. Dental treatment overcomes the patient’s functional, psychological, esthetic and phonation problems. Remaining healthy teeth may allow the dentist to fabrícate a removable partial overdenture, fixed partial prosthesis or implant - supported prosthesis. The retention of a number of abutments helps maintain a positive ridge form with greater height and volume of the alveolar bone, improving masticatory performance, as well as providing a more stable prostheses. Dental patients who have medical problems need many treatment procedures. Multidisciplinary treatment planning is invaluable for patient’s dental health. Progeria is a rare genetic condition where symptoms resembling aspects of aging are manifested at an early age. characteristic clinical findings of Progeria disease include abnormalities of the skin and hair in conjunction with char-acteristic facial features and skeletal abnormalities. The characteristic facies show protruding ears, beaked nose, thin lips with centrofacial cyanosis, prominent eyes, frontal and parietal bossing with pseudohydrocephaly, midface hypoplasia with micrognathia and large anterior fontanel. The other reported anomalies are dystrophic nails, hypertrophic scars and hypoplastic nipples. The findings that are nearly interested in dentistry are delayed dentition, anodontia, hypodontia, or crowding of teeth. This article presents the multidisciplinary dental treatment planning includes surgical, endodontic and prosthetic treatment of a patient with a history of progeria. In this case complete-arch fixed prostheses in both maxilla and mandible, supported by a combination of im-plants and teeth are reported. PMID:19754475
Delarays Ossani Pérez Alfonso
Full Text Available Background: oligodontia, anodontia and hypodontia are terms used interchangeably to refer to the absence of one or more teeth. It is important to consider that it can bring clinical damages, whether functional, psychological and aesthetic.Objective: to characterize clinically and epidemiologically patients suffering from hypodontia treated at the dental clinic of "Guillermo Tejas" polyclinic in Las Tunas, from October, 2013 to July, 2015.Method: a descriptive study was carried out in patients suffering from hypodontia who went to the dental clinic during the aforementioned time period. The universe consisted of 180 patients who entered the service and the sample was composed of the 27 ones who were between 9 and 20 years old.Results: the prevalence of hypodontia was higher in white females; past health history showed prevalence of respiratory disease, inheritance factor and embryological cause. Hypodontia was more common in the maxillar; the most affected teeth were the third molar, the lateral incisor and bicuspids, in order of frequency, and the positive discrepancy bone-tooth prevailed.Conclusions: it was possible to characterize the patients of the sample by means of this study.
Full Text Available The function of dentition is not only for mastication, the other function is to coordinate the growth of maxilla, midface and mandible. This important function was done through occlusal contact during occlusion. The occlusal surfaces become a link of stomato-gnathic system. This case report showed the midface growth was affected by agenesis. The first case was 10-years old girl with congenital missing 16 and 26. The relationship of upper and lower arch erior was edge to edge and crossbite. In posterior, the occlusal contact were found between upper second primary molars and lower first permanent molars. The second and third cases were 5 and 9-years old boys. They were siblings with ectodermal dysplasias and partial anodontia. The facial profile of these children were concave. The relationship of maxilla and mandible was class III. The manifestation of growth retardation was shown by cephalometric measurement, SN, Ans-Pns, Ptm-A, N-Me, Ans-Me, S-Go were smaller than standard normal children.
Salvi, Alessandro; Giacopuzzi, Edoardo; Bardellini, Elena; Amadori, Francesca; Ferrari, Lia; De Petro, Giuseppina; Borsani, Giuseppe; Majorana, Alessandra
Dental agenesis is one of the most common congenital craniofacial abnormalities. Dental agenesis can be classified, relative to the number of missing teeth (excluding third molars), as hypodontia (1 to 5 missing teeth), oligodontia (6 or more missing teeth), or anodontia (lack of all teeth). Tooth agenesis may occur either in association with genetic syndromes, based on the presence of other inherited abnormalities, or as a non-syndromic trait, with both familiar and sporadic cases reported. In this study, we enrolled 16 individuals affected by tooth agenesis, prevalently hypodontia, and we carried out direct Sanger sequencing of paired box 9 (PAX9) and Msh homeobox 1 (MSX1) genes in 9 subjects. Since no mutations were identified, we performed whole exome sequencing (WES) in the members of 5 families to identify causative gene mutations either novel or previously described. Three individuals carried a known homozygous disease mutation in the Wnt family member 10A (WNT10A) gene (rs121908120). Interestingly, two of these individuals were siblings and also carried a heterozygous functional variant in EDAR-associated death domain (EDARADD) (rs114632254), another disease causing gene, generating a combination of genetic variants never described until now. The analysis of exome sequencing data in the members of other 3 families highlighted new candidate genes potentially involved in tooth agenesis and considered suitable for future studies. Overall, our study confirmed the major role played by WNT10A in tooth agenesis and the genetic heterogeneity of this disease. Moreover, as more genes are shown to be involved in tooth agenesis, WES analysis may be an effective approach to search for genetic variants in familiar or sporadic tooth agenesis, at least in more severe clinical manifestations.
Jaime Diez Betancourt
Full Text Available La displasia ectodérmica es una enfermedad genética muy rara, la cual se encuentra caracterizada por manifestaciones odontoestomatológicas imponentes; presenta aspectos interesantes desde el punto de vista del diagnóstico, así como la rehabilitación conjunta ortodóntica, protésica y estética. En el Servicio de Ortodoncia de la Clínica Estomatológica INPE concurrió una paciente de 9 años de edad con manifestaciones clínicas de displasia ectodérmica, principalmente anodoncia, hipoplasia de dientes anteriores y malposición dentaria, con dientes de aspecto de conos y anomalías de las cúspides, y con afectación sicológica de la paciente. Con un tratamiento conjunto de ortodoncia y del estomatólogo general integra,l se logró restituir la maloclusión, y al mismo tiempo, con el tratamiento estético, se devolvió a la paciente a la vida socialmente útil y afectiva en vísperas de cumplir los 15 años.The ectodermic dysplasia is a very rare genetic disease that is characterized by impressive odontostomatological manifestations. It presents interesting aspects regarding the diagnosis and the joined orthodontic, prosthetic and aesthetic rehabilitation. A 9-year-old patient with clinical manifestations of ectodermic dysplasia, mainly anodontia, hypoplasia of the anterior tooth and tooth malposition, with cone-like tooth, abnormalities of the cusps and psychological affectation, presented for care at the Orthodontics Service of the INPE Dental Clinic. By a joint treatment of orthdontics and the general stomatologist, it was possible to restore malocclusion and, at the same time, with the aesthetic treatment the patient was returned to the socially useful and affective life before being 15.
Full Text Available ABSTRACT Fragile X syndrome (FXS is a disorder linked to the chromosome X long arm (Xq27.3, which is identified by a constriction named fragile site. It determines various changes, such as behavioral or emotional problems, learning difficulties, and intellectual disabilities. Craniofacial abnormalities such as elongated and narrow face, prominent forehead, broad nose, large and prominent ear pavilions, strabismus, and myopia are frequent characteristics. Regarding the oral aspects, deep and high-arched palate, mandibular prognathism, and malocclusion are also observed. Objective: The purpose of this study was to evaluate the dental radiographic characteristics as described in 40 records of patients with panoramic radiography. Material and Methods: The patients were in the range of 6–17 years old, and were divided into two groups (20 subjects who were compatible with the normality standard and 20 individuals diagnosed with the FXS, which were matched for gender and age. Analysis of the panoramic radiographic examination involved the evaluation of dental mineralization stage, mandibular angle size, and presence of dental anomalies in both deciduous and permanent dentitions. Results: The results of radiographic evaluation demonstrated that the chronology of tooth eruption of all third and second lower molars is anticipated in individuals with FXS (p<0.05. In this group, supernumerary deciduous teeth (2.83%, giroversion of permanent teeth (2.31%, and partial anodontia (1.82% were the most frequent dental anomalies. In addition, an increase was observed in the mandibular angle size in the FXS group (p<0.05. Conclusion: We conclude that knowledge of dental radiographic changes is of great importance for dental surgeons to plan the treatment of these individuals.
Full Text Available We report new examples of Cenozoic cold-seep communities from Colombia, Cuba, the Dominican Republic, Trinidad, and Venezuela, and attempt to improve the stratigraphic dating of Cenozoic Caribbean seep communities using strontium isotope stratigraphy. Two seep faunas are distinguished in Barbados: the late Eocene mudstone-hosted 'Joes River fauna' consists mainly of large lucinid bivalves and tall abyssochrysoid gastropods, and the early Miocene carbonate-hosted 'Bath Cliffs fauna' containing the vesicomyid Pleurophopsis, the mytilid Bathymodiolus and small gastropods. Two new Oligocene seep communities from the Sinú River basin in Colombia consist of lucinid bivalves including Elongatolucina, thyasirid and solemyid bivalves, and Pleurophopsis. A new early Miocene seep community from Cuba includes Pleurophopsis and the large lucinid Meganodontia. Strontium isotope stratigraphy suggests an Eocene age for the Cuban Elmira asphalt mine seep community, making it the oldest in the Caribbean region. A new basal Pliocene seep fauna from the Dominican Republic is characterized by the large lucinid Anodontia (Pegophysema. In Trinidad we distinguish two types of seep faunas: the mudstone-hosted Godineau River fauna consisting mainly of lucinid bivalves, and the limestone-hosted Freeman's Bay fauna consisting chiefly of Pleurophopsis, Bathymodiolus, and small gastropods; they are all dated as late Miocene. Four new seep communities of Oligocene to Miocene age are reported from Venezuela. They consist mainly of large globular lucinid bivalves including Meganodontia, and moderately sized vesicomyid bivalves. After the late Miocene many large and typical 'Cenozoic' lucinid genera disappeared from the Caribbean seeps and are today known only from the central Indo-Pacific Ocean. We speculate that the increasingly oligotrophic conditions in the Caribbean Sea after the closure of the Isthmus of Panama in the Pliocene may have been unfavorable for such large
More, Chandramani B; Bhavsar, Khusbhu; Joshi, Jigar; Varma, Saurabh N; Tailor, Mansi
Ectodermal dysplasia (ED) is a group of rare, inherited disorders characterized by sparse hair, missing teeth and inability to sweat. To review and analyze cases of ED with an emphasis on clinical manifestations and parent's marriage history. The present retrospective study was conducted by assessing the clinical records of nineteen cases of ED, available in the archives of the department; for age, gender, family history of consanguineous marriage and clinical manifestations. It was observed that ED was more prevalent in males, with a ratio of 1.7:1. The hypohydrotic type was more common (78.95%) than hydrotic type (21.05%). The marriage history of parents revealed that 66.67% had consanguineous marriage and had 68.42% offspring's affected with ED; whereas 33.33% had history of non-consanguineous marriage and had 31.58% offspring's affected with ED. The clinical manifestations observed were- dry skin(94.74%); scaly skin(42.11%); sparse hair on scalp, eyebrows and eyelashes(100%); frontal bossing(63.18%); saddle nose (57.89%); hypertelorism (47.37%); nail abnormality(52.63%); normal sweat glands(21.05%); abnormal sweat glands(78.95%); hypoplastic maxilla(52.63%); protuberant lips (57.89%); palmo-plantar keratosis(21.05%); wrinkled & hyper pigmented facial skin(84.21%); partial anodontia(94.74%); conical shaped teeth(84.21%); high arched palate(68.42%); thin alveolar bone(100.00%); taurodontism(21.05%) and cleft lip & cleft palate(05.26%). The number of teeth present in all the cases ranged from 0 to 19. ED patients suffer from social problems and poor psychological and physiological development as a result of unacceptable esthetics and abnormal function of orofacial structures. Oral rehabilitation thus becomes mandatory, although it is often difficult; particularly in pediatric patients.
Full Text Available Background/Aim. Bupivacaine (Marcaine®, homologue of mepivacaine, chemically related to lidocaine, is used as a local anesthetic for local infiltration, peripheral nerve block, retrobulbar block, symphathetic block, and caudal and epidural anesthesia. The aim of this investigation was to determine and to compare clinical parameters of the local anesthetic effects of bupivacaine applied with and without a vasoconstrictor. Methods. This investigation included a total of 30 randomly selected patients, who ranged in age from 30−60 years, with partial or total anodontia in the molar region of the mandible. These patients with total or partial edentulous molar part of the mandible, scheduled for dental implantation placement, were asked to participate in the study. In the first phase of the investigation, the patients were subjected to local anesthesia with 3.5 cm3 of 0.5% bupivacaine with a vasoconstrictor (adrenalin, 1: 200 000 in the right side of the mandible. After administering local anesthesia, the placement of blade, cylindrical, transdental (B.C.T. implants was performed. In the second stage of the investigation, in 7−10 days period after the first oral surgery, the patients were subjected to local anesthesia with 3.5 cm3 of 0.5% bupivacaine, but without a vasoconstrictor, in the left side of the mandible. After administering local anesthesia, the placement of B.C.T. implants was performed. During the performance of both oral surgery procedures, the following clinical parameters of the local anesthetic effects were monitored: latent period, duration and the potency of anesthesia, and the evaluation of the postoperative pain level. Results. The latent period under local anesthesia with 3.5 cm3 of 0.5% bupivacaine and vasoconstrictor was statistically significantly shorter than without vasoconstrictor. The duration of local anesthesia was longer without vasoconstrictor. There was no difference in the potency of anesthesia with or without a