Presentation
Incisors and canines are often conical-shaped while primarily second molars, if present, are mostly affected by taurodontism. [amsdottorato.unibo.it]
The enamel was present only in some areas of the tooth and absent in some. Generalized attrition was observed on the incisal and occlusal surfaces with open contacts. [ijdr.in]
Treatment is based on the signs and symptoms present in each person. MalaCards based summary : Teeth, Congenital Absence of, with Taurodontia and Sparse Hair, is also known as taurodontia, absent teeth, sparse hair syndrome. [malacards.org]
The number of teeth present in all the cases ranged from 0 to 19. [jnsbm.org]
Bilateral postaxial polydactyly of hands were present (Figure 2a). Hair was sparse especially in the eyebrows (Figure3). The left testis was not fully descended. [omicsonline.org]
Entire Body System
- Nail Abnormality
Nails: Most people with Ectodermal Dysplasia do not have nail abnormalities, although the nails are frequently dry and rough. [dentalimplants-usa.com]
Hypoplastic toenails Abnormal palate morphology Recurrent fractures Kyphoscoliosis Nail dysplasia Tetraplegia Abnormality of the skin Abnormality of skin pigmentation Retinal detachment Papule Pallor Skin rash Visual loss X-linked dominant inheritance [mendelian.co]
The disorder is characterized by sparse thin hair, multiple congenitally missing teeth, abnormal conically shaped teeth, lack of sweat pores and nail abnormalities. [thefreelibrary.com]
This form also affects teeth, nails and hair. But the hereditary patterns and nail and sweat gland manifestations tend to differ from hypohydrotic type. [jnsbm.org]
Abnormal nail phenotype was reported in two individuals: Individual IV who had absent nails at birth and individual VI who had mild hyponychia and brittle nails. None of the patients could be classified as a specific ED entity [ 22 ]. [bmcmedgenet.biomedcentral.com]
Eyes
- Visual Impairment
[…] loss X-linked dominant inheritance Optic atrophy Visual impairment Neoplasm Cataract Plantar hyperkeratosis Palmoplantar hyperkeratosis Oligodontia Metaphyseal spurs Therapeutic abortion Scarring Lymphedema Hypocupremia Breast hypoplasia Hyperpigmented [mendelian.co]
Skin
- Sparse Hair
Prevention - Taurodontia absent teeth sparse hair Not supplied. [checkorphan.org]
More Symptoms of Taurodontia, absent teeth, sparse hair syndrome » Causes See also causal information: Causes of Taurodontia Causes of Sparse hair Similar Topic Articles Taurodontia Absent teeth Sparse hair Names and Terminology References Source: GARD [familydiagnosis.com]
[…] unusually sparse, slow-growing hair. [malacards.org]
Name Teeth, Congenital Absence Of, With Taurodontia And Sparse Hair Synonyms TAURODONTIA, ABSENT TEETH, AND SPARSE HAIR Classification developmental, genetic, odontological, skin Phenotypes Autosomal recessive inheritance ; Oligodontia ; Sparse hair ; [mousephenotype.org]
Title Other Names: Teeth, congenital absence of, with taurodontia and sparse hair Categories: Taurodontia, absent teeth, sparse hair syndrome is a rare condition that, as the name suggests, is primarily characterized by malformations of the primary and [rarediseases.info.nih.gov]
- Alopecia
Researchers estimate that about 1 per 100 diagnoses of alopecia totalis are incorrect. See introduction to alopecia totalis. [checkorphan.org]
Ectodermal dysplasia alopecia preaxial polydactyly 0 *Alopecia *Ectodermal Dysplasia *Polydactyly *Facies. Ectodermal dysplasia adrenal cyst 0 *Adrenal Gland Diseases *Cysts *Ectodermal Dysplasia. [reference.md]
Clinical differential considerations include: alopecia areata aplasia cutis congenita focal dermal hypoplasia syndrome incontinentia pigmenti Naegeli-Franceschetti-Jadassohn syndrome pachyonychia congenita Promoted articles (advertising) [radiopaedia.org]
[…] contractures Partial agenesis of the corpus callosum Cortical gyral simplification Femoral bowing Preaxial polydactyly Diabetes insipidus Agenesis of cerebellar vermis Colpocephaly Absent facial hair Epidermal acanthosis Palmar hyperkeratosis Patchy alopecia [mendelian.co]
[…] of teeth-maxillary hypoplasia-genu valgum syndrome Oculocerebral hypopigmentation syndrome, Cross type Oculodental syndrome, Rutherfurd type Oculodentodigital dysplasia Oculofaciocardiodental syndrome Oculootodental syndrome Odonto-onycho dysplasia-alopecia [se-atlas.de]
- Sparse Fine Hair
The clinical features include sparse, fine hair, missing or conical-shaped teeth, decreased sweat and mucous glands, hypoplastic skin, and heat intolerance with exercise or increased ambient temperature. [amsdottorato.unibo.it]
- Dry Hair
Dry hair, severe hypodontia, smooth tongue, nail dysplaisa, hyperhidrosis of palms/soles, hyperkeratosis. Malformations in eye, umbilical and hypodontia. Affects both dentitions. MN 2nd premolars, then central incisors and MX 2nd premolars. [brainscape.com]
- Blonde Hair
Frontal bossing, depressed midface, prominent lips and depressed nasal bridge were typical features ( fi gure 1A ). 6 10 Almost all children showed thin, sparse and blond hair, with sparse eyelashes and eyebrows. [iedn.org]
Face, Head & Neck
- Facial Hemihypertrophy
Facial hemihypertrophy, otodental syndrome. Constriction of CEJ and elongated pulp chamber with apically displaced pulp. HYPODONTIA associated. Elongated pulp chamber with short stunted roots. Failure of Hertwig's epithelial root sheath. [brainscape.com]
Treatment
[…] space and advanced treatment strategy such as use of osseointegrated implants are the treatments of choice. [jidonline.com]
Treatment & prognosis Type I - short root s (early loss from periodontitis) Pulp channels close to DEJ ( restoratio n & endodontic treatment) Type II - permanent - endodontic treatment 143. [slideshare.net]
As in other genetic disorders the causes involve multiple factors and the treatment is only palliative. [nordimplant.com]
Treatment is based on the signs and symptoms present in each person. MalaCards based summary : Teeth, Congenital Absence of, with Taurodontia and Sparse Hair, is also known as taurodontia, absent teeth, sparse hair syndrome. [malacards.org]
You may also want to contact a university or tertiary medical center in your area, because these centers tend to see more complex cases and have the latest technology and treatments. [rarediseases.info.nih.gov]
Prognosis
Treatment and prognosis Done for aesthetic purpose Peg laterals- full size porcelain crowns. 14. In number of teeth 15. [slideshare.net]
Some of the overlooked hair/scalp conditions include cicatrial alopecia, traction folliculitis and sebhorrheic dermatitis Prognosis - Taurodontia absent teeth sparse hair Not supplied. Treatment - Taurodontia absent teeth sparse hair Not supplied. [checkorphan.org]
ED per se has not only a poor prognosis, but it also needs constant care for temperature and other problems associated with this condition. Genetic counseling is recommended for families who have family history of ED. [digitmedicine.com]
Diagnosis is also possible using chorionic villi or amniotic fluid using linked�microsatellite markers if a previously affected sibling has been identified [ 9 ].The prognosis of EvCsyndrome is linked to the presence of congenital heart defects and [omicsonline.org]
Thus, individuals with bi-allelic WNT10A mutations may be recognized early in life by combining information of both dentitions as well as other ectodermal features of importance for diagnosis, prognosis and counselling. [bmcmedgenet.biomedcentral.com]
Etiology
This article reports rare three cases of congenitally missing permanent teeth with over-retained deciduous dentition and review the literature citing its etiology, clinical implications, and management. [jidonline.com]
Taurodontism – A review on its etiology, prevalence and clinical considerations. J Clin Exp Dent 2010;2:187-90. 15. Rathee M, Mahesh G, Arun K, Richa M, Renu K. [jioh.org]
Hypodontia–a retrospective review of prevalence and etiology. Part I. Quintessence Int 2005 ; 36 : 263 – 70. 18. Lexner MO, B ardow A, Hertz JM, e t al. A nomalies of tooth formation in hypohidrotic ectodermal dysplasia. [iedn.org]
Etiology unknown. Possibly familial, common for more than 1 tooth. MN primary molars are the most common. Should exfoliate normally. Want to monitor and not extract as to not damage the successors. Minor eruption variation is normal. [brainscape.com]
Pathophysiology
Another pathophysiological method seen in the background of PSS is that of meagre craft vasculitis that can less commonly muddle PSS but all but certainly accounts benefit of some of the neurological manifestations, in particular mononeuropathy multiplex [sarcomacancer.org]
Prevention
Prevention - Taurodontia absent teeth sparse hair Not supplied. [checkorphan.org]
The goal of the treatment is aesthetic, preventive and restorative. Bonding may be done for aesthetic reasons. Crowns can restore the structure and function of the teeth as well as prevent further attrition. [thehealthsite.com]
Treatment & prognosis Enamel pearl – area of weak point of periodontal attachment. Oral hygiene maintained to prevent loss of periodontal support. Flattening / removal of enamel & furcation plasty. 80. [slideshare.net]
[…] people with hypodontia and so prevention of dental disease is paramount. [nature.com]
Parents were counseled regarding the disease and preventive care needed. [digitmedicine.com]