[Occlusal reconstruction of a patient with hypohidrotic ectodermal dysplasia].
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Prosthodontic treatment for patient with hypohidrotic ectodermal dysplasia (HED) is difficult to manage because of the loss of occlusal vertical dimension, abnormal alveolar ridge and anodontia A 20-year-old case with HED was reported. The patient was treated with occlusal reconstruction by a maxillary fixed prosthesis and mandibular telescopic prosthesis. At one-year's follow-up, dentures were well used and occlusal function of the patient returned to normal.Keywords:
Hypohidrotic ectodermal dysplasia
Anodontia
Alveolar ridge
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Prosthodontic treatment for patient with hypohidrotic ectodermal dysplasia (HED) is difficult to manage because of the loss of occlusal vertical dimension, abnormal alveolar ridge and anodontia A 20-year-old case with HED was reported. The patient was treated with occlusal reconstruction by a maxillary fixed prosthesis and mandibular telescopic prosthesis. At one-year's follow-up, dentures were well used and occlusal function of the patient returned to normal.
Hypohidrotic ectodermal dysplasia
Anodontia
Alveolar ridge
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Ectodermal dysplasia is a hereditary disease characterized by congenital dysplasia of one or more ectodermal structures. Intraorally, common findings are anodontia or oligodontia, conical teeth, and, consequently, generalized spacing. This case presented the oral rehabilitation of a child with hypohidrotic ectodermal dysplasia. Oral rehabilitation is important from functional, esthetic, and psychologic perspectives. Due to the absence of teeth, the volume of alveolar bone and its growth are decreased, resulting in a loss of vertical dimension and protuberant lips. The treatment involved increasing the patient's vertical dimension of occlusion, fabricating a maxillary partial denture, and using magnets to help retain the mandibular partial denture. A 5-year 7-month old Korean boy was referred to the pediatric department for examination, evaluation and treatment of his disorder. we used magnets on '73 and '83 for enhanced retention of a mandibular overdenture. The magnet used in this case was the Magfit system(GC Co., Japan).
Hypohidrotic ectodermal dysplasia
Oligodontia
Anodontia
Retainer
Removable partial denture
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Ectodermal dysplasia is characterized by the absence or defects of two or more ectodermally derived structures. It is commonly inherited as X-linked recessive disorder. Males are most commonly affected and females are usually carriers. In very rare cases it is inherited as autosomal recessive form and in such cases females can manifest complete syndrome. 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. A case of a 8-year-old female child with anhidrotic ectodermal dysplasia with complete anodontia is presented. Dental, oral, and physical features were taken into consideration in diagnosis and treatment for this patient. Clinical management consisted of removable complete dentures.
Hypohidrotic ectodermal dysplasia
Hypodontia
Anodontia
X-linked recessive inheritance
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Ectodermal dysplasias are a complex group of genetically determined disorders clinically characterized by congenital alterations of the structures derived from the ectoderm. Even though ectodermal dysplasia with partial anodontia is common, this condition with total anodontia is not. We describe features of hypohidrotic ectodermal dysplasia with complete anodontia in a 6-year-old boy. In order to improve esthetics, speech and mastication, the child was provided with upper and lower complete dentures.
Hypohidrotic ectodermal dysplasia
Anodontia
Hypodontia
Hypotrichosis
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Prosthetic Rehabilitation of a Child with Ectodermal Dysplasia and Partial Anodontia - A Case Report
Background: Ectodermal dysplasia is a rare congenital disease that affects several ectodermal structures like skin, hair, nails, teeth, or sweat glands. Children with ectodermal dysplasia may have various manifestations of the disease, which differ in severity and may involve teeth, nails, skin, hair, sweat glands and sebaceous glands. The hypohidrotic ectodermal dysplasia is most common form of the ectodermal dysplasia syndrome and is inherited as an X-linked recessive trait. This article reports the dental rehabilitation of an 8-year-old male child with ectodermal dysplasia. Patient was clinically managed by fabricating maxillary and mandibular partial dentures to help in psychosocial development and to restore the vertical dimension, aesthetics and functions of his stomatognathic system.
Hypohidrotic ectodermal dysplasia
Anodontia
Stomatognathic system
Hypodontia
Hypotrichosis
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Ectodermal dysplasia is a large complex group of disorders resulting in abnormal development of two or more ectodermal structures. Oral manifestations include partial or complete anodontia and poor bony bases affecting aesthetics and function. Dental management in such patients is difficult and requires intervention throughout the growth period of the patient, until a definitive treatment is rendered. This case report describes the prosthetic rehabilitation of a young patient with total anodontia, which is a rare event in contrast to partial anodontia. A unique approach was devised to improve the retention and stability of compromised ridges. Conventional dentures were delivered to the patient with like-pole magnets incorporated in the occlusal surface of posterior denture teeth of upper and lower denture facing correspondingly which resulted in repulsive force. This enhanced retention and stability of the dentures.
Hypohidrotic ectodermal dysplasia
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Summary. A patient with hypohidrotic ectodermal dysplasia and anodontia was studied clinically and cephalometrically from 6 to 18 years of age. This case is exceptional in that the patient was a young girl. Five sets of complete dentures were fitted during this period. Periodic recall examination and adjustment maintained good oral function and aesthetics. Serial cephalometric analysis of craniofacial development revealed that growth of the maxilla was slightly reduced, but there was no detectable effect in the mandible. Three‐dimensional analysis of the alveolar ridge form from serial casts showed that increase of the anteroposterior arch dimension was larger than that of the lateral arch dimension in both jaws. There was a lateral expansion in the anterior and premolar regions and a considerable height increase in the posterior tooth region of the upper alveolar ridge. Our findings support those of Sarnart et al. (1953) in showing that lack of teeth did not affect growth of the jaws. Résumé. Un patient souffrant de dysplasie ectodermique hypohhidrotique avec anodontie a été étudié cliniquement et par examen céphalométriques de l'âge de 6 à 18 ans. Ce cas est exceptionnel du fait du jeune âge de la patiente. Cinq jeux de prothéses complétes ont été préparé pendant cette période. Des consultations systématiques de contrôle ont permis de maintenir une bonne fonction et une bonne esthétique. Une analyse céphalométrique par série du développement cranio‐facial a montré que la croissance du maxillaire était légérement diminuée mais semblait normale à la mandibule. L'analyse tridimensionalle de la crête alvéolaire, à partir d'une série de moulages, a montré que l'augmentation de l'arc antéro‐postérieur était plus importante que celle de l'arc antérieur dans les deux maxillaires. Ont été noté une une augmentation transversale dans le secteur antérieur et des prémolaires et une augmentation importante de hauteur des procés alvéolaires dans la région molaire supérieure. Nos résultats confortent ceux de SARNAT et co (1953) montrant que l'absence de dents est sans conséquences sur la croissance des maxillaires. Zusammenfassung. Ein Patient mit der Diagnose einer Hypohidrotic Ectodermalen Dysplasie und einer Anodontia wurde klinisch und mit Fernröntenbilder vom 6–18. Lebensjahr überwacht. Der Fall bildete eine Ausnahme hiermit dass der Patient ein Mädchen war. Während dieser Periode wurden 5 Paare totaler Prothese angefertigt. Periodische Recalls ermöglichten dem Patienten eine gute orale Funktion und Aesthetik. Die F.R.‐Röntgenserie zeigten, dass das Wachstum des Oberkiefers etwas retardiert war. Das Eachstum des Unterkiefers war normal. Die dreidimensionale Analyse des Alveolarkammes anhand der Modelle zeigte, das die Zunahme der anteroposterioren Bogendimension grösser war als diejenige der lateralen Bogendimension beider Kiefer. Eine laterale Vergrösserung in der Front und prämolaren Gegend und eine merkliche Höhe‐Zunahme in der posterioren Region des oberen Kammes, wurde festgestellt. Unsere Befunde bestätigen die Resultate vor Sarnat et al. (1953) dass das Fehlen von Zähnen nicht das Wachsturn der Kiefer beeinflusst. Resumen. Un paciente con displasia ectodérmica hipohidrótica y anodoncia fue estudiado clínica y cefalográficamente de 6 a 18 años de edad. El caso es excepcional porque el paciente fue niña. Cinco dentaduras completas fueron realizadas durante este período. Se realizaron exámenes periódicos los cuales aseguraron buena función oral y estética. Análisis cefalométricos seriados del desarrollo craneofacial mostraron que el crecimiento de la maxila fue ligeramente reducido, pero no se detectó ningun efecto en la mandíbula. Análisis tridimensionales del reborde alveolar de los medelos seriados demostraron que el aumento de la dimensión anteroposterior del arco fue mayor que la de la dimension lateral del arco. Hubo una expansión lateral en las regiones anterior y premolar y un considerable aumento en la altura en la región denaria posterior en el reborde alveolar superior. Nuestros resultados dan apoyo a los de Sarnat et al. (1953) al demostrar que la falta de dientes no afectó el crecimiento de los maxilares.
Anodontia
Hypohidrotic ectodermal dysplasia
Premolar
Mandible (arthropod mouthpart)
Alveolar ridge
Hypodontia
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A five-year-old Japanese girl was referred to our clinic for non-eruption of the teeth. Panoramic radiographs revealed absence of all teeth of both primary and permanent dentitions. She showed hypotricosis, hypohidrosis, and anodontia. In this article, the reported cases of anodontia were reviewed and the fabricating procedure of full dentures for a young child was described.
Hypohidrotic ectodermal dysplasia
Anodontia
Girl
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The purpose of this paper was to report the case of a 6-year-old male patient who was clinically diagnosed as having Rapp-Hodgkin syndrome-a variant of ectodermal dysplasia characterized by dysplastic disorders of the skin, hair, and nails associated with cleft lip and/or palate. The patient exhibited only the primary maxillary and mandibular canines bilaterally. Restorative treatment was performed on all primary canines, followed by aquisition of maxillary and mandibular impressions for fabrication of acrylic removable partial dentures with circumferential clasps on the primary canines. The dentures allowed recovery of the vertical dimension and excellent esthetics. Hypodontia of several teeth associated with ectodermal dysplasia, as in this case involving Rapp-Hodgkin syndrome, causes several functional and esthetic alterations. These disorders should be diagnosed and treated as early as possible to restore the normal function, allow adequate mastication and speech, enhance esthetics, and, consequently, promote the patient's self-esteem and social integration.
Mastication
Hypodontia
Hypohidrotic ectodermal dysplasia
Anodontia
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Individuals affected by ectodermal dysplasia syndromes have abnormalities of the glands, tooth buds, hair follicles, and nail development. Oral finding in ectodermal dysplasia patient are significant and can include multiple abnormalities of the dentition such as anodontia, hy-podontia or malformed and widely spaced peg like teeth, loss of occlusal vertical dimension, protuberant lips and lack of normal alveolar ridge development. This clinical report describes a combined surgical, pedodontic, and prosthodontic approach for the treatment of a patient with hypohidrotic ectodermal dysplasia.
Hypohidrotic ectodermal dysplasia
Anodontia
Permanent dentition
Alveolar ridge
Hypodontia
Electronic journal
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