Rehabilitación inicial de un niño con anodoncia: presentación de una situación clínica / Initial rehabilitation of a child with anodontia: report of a clinical situation

El objetivo de este trabajo es describir la rehabilitación inicial de un niño preescolar con displasia ectodérmica (DE) y anodoncia. Realizó la primera consulta a los 30 meses con ambos rebordes desdentados y requerimientos familiares de estética y función acorde a su edad. Requirió un amplio abordaje interdisciplinario (odontopediatra, psicóloga, fonoaudióloga y especialista en prótesis). Se instalaron prótesis totales removibles (PTR) en ambos maxilares rebasadas con material resiliente de larga duración, que cumplieron los requisitos de retención, soporte, estabilidad y confort para el niño.Conclusión: los pacientes con anodoncia requieren rehabilitación a edades tempranas, siendo necesaria la participación de equipos interdisciplinarios que aportan diferentes recursos para mejorar la estética, favorecer la función y la inserción en el contexto psicosocial acompañando el crecimiento y desarrollo (AU)

Dental implants for severely atrophied jaws due to ectodermal dysplasia.

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.

Clouston syndrome associated with eccrine syringofibroadenoma

Eccrine syringofibroadenoma is a rare benign neoplasia derived from acrosyryngium cells of the eccrine sudoriferous glands. It affects the extremities of elderly individuals as solitary tumors, or may also present as multiple lesions. There are controversies about the pathogenesis and differentiation of the tumor. Eccrine syringofibroadenoma has been associated with subjacent conditions, such as for example, hypohidrotic ectodermic dysplasias. The authors describe a case report of a patient with Clouston Syndrome, who presented papules and nodules in extremities, clinically and histologically compatible with eccrine syringofibroadenoma. There are only three cases described in the literature, associated with Clouston Syndrome, and this is the fourth case.

Aplasia cutis congénita: serie de casos de ubicación y asociación particular / Aplasia cutis congenita: a case series

La aplasia cutis congénita (ACC) es un trastorno poco frecuente del desarrollo cutáneo, presentándose la mayoría como defectos aislados, pero pueden asociarse a otras malformaciones y síndromes genéticos. En relación a un caso de ACC extensa asociada a Síndrome de Adams-Oliver (SAO) se analizaron 10 casos de ACC con ubicación especial o asociadas a otras malformaciones diagnosticadas en forma clínica o por ecografía. Se confeccionó una tabla clasificando cada caso según Frieden, describiendo la ubicación de la aplasia y las asociaciones encontradas.

Aplasia cutis congenita (ACC) is a rare disorder of skin development, appearing mostly as isolated defects, but may be associated with other malformations and genetic syndromes. In relation to a case of extensive associated ACC Adams-Oliver Syndrome (AOS) were analyzed 10 cases of ACC with special location or associated with other malformations diagnosed clinically or by ultrasound. We made a table classifying each case as Frieden`s classification, describing the location of the aplasia and the associations found.

Displasia ectodérmica: caso clínico / Ectodermal dysplasia: a clinical case

Las displasias ectodérmicas son un grupo extenso de enfermedades en las que se encuentra alterado el desarrollo de dos o más tejidos derivados del ectodermo. Es una patología no progresiva, difusa, genodermatosis congénita, caracterizada por la falta o escasez de pelo, dientes, uñas y glándulas sudoríparas. El paciente presenta maxilar inferior edéntulo. Se introduce una breve revisión de la literatura, se discute la terapéutica actual y se muestra un caso como ejemplo. El propósito del presente trabajo es describir los hallazgos clínicos en un paciente de 5 años con displasia ectodérmica hipohidrótica y su tratamiento.

Prosthodontic management of anhidrotic ectodermal dysplasia.

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.

Prosthetic rehabilitation for a patient with hypohidrotic ectodermal dysplasia: a clinical case

Hypohidrotic ectodermal dysplasia (HED) is a hereditary syndrome, characterized by a classictriad of hypotrichosis, hypodontia and hypohidrosis. The case of an 8-year-old girl with HEDpresenting oligodontia and marked resorption of maxillary and mandibular alveolar ridges is reported. A specially designed aluminum wire tray was used for taking impressions of both dental arches. Prosthetic rehabilitation included a removable maxillary over denture and a mandibular conventional acrylic resin denture made tissue-compatible by means of a soft denture liner attached to denture base. The proposed treatment plan provided improved esthetics, function,and quality of life.

Ectodermal dysplasia with alopecia, onychodysplasia, hypohidrosis, keratoderma, abnormal teeth and deafness.

The ectodermal dysplasias are a heterogeneous group of disorders with primary defect in hair, teeth, nail and sweat gland function. Numerous types have been described and several classifications exist. Here, we present a patient with ectodermal dysplasia with alopecia, dysplastic nails, hypohidrosis, sensorineural deafness, palmoplantar keratoderma, abnormal teeth and dry skin. To our knowledge, combination of all these features in ectodermal dysplasia has not been reported in the past. The etiology is unknown, but consanguinity of parents points to an autosomal recessive inheritance.

Aplasia cutis congenita of the scalp: therapeutic modalities.

Agenesis of scalp is an uncommon but well-recognized clinical entity. Congenital scalp and skull defects can be either obvious or occult; over 300 cases have been reported in literature. Aplasia cutis congenita (ACC) is recognized as a heterogeneous disorder, all characterized by focal absence of the epidermis, dermis and sometimes the calvarium and/or dura. We present a case of ACC in an infant whose mother was exposed to a teratogenic drug (Methimazole--an antithyroid drug) during pregnancy. This case report is presented to highlight the steps to successful management. Definitive full thickness scalp cover at the earliest avoids secondary infection, eschar formation and exsanguination.

Prosthetic rehabilitation of individuals with ectodermal dysplasia.

Ectodermal dysplasia is a genetic disorder commonly affecting the development of teeth. The quality of life of the affected individuals can be improved by a multidisciplinary approach to the treatment plan. The treatment may include removable, fixed, implant-supported prostheses, or a combination of these options. This case report describes in detail the diagnosis and treatment of two such individuals using removable overdentures.

Congenital pyloric atresia: Isolated and associated with aplasia cutis congenita

Two cases of congenital pyloric atresia [CPA] are, hereby, reported. One was suspected on antenatal ultrasound and turned out to be an isolated anomaly. Other patient had a rare association of aplasia cutis congenita with congenital pyloric atresia. The lesions of aplasia cutis congenita were multiple while congenital pyloric atresia was of type II. The patient with an isolated lesion survived following surgery while the other baby died of sepsis in postoperative period

Johanson--blizzard syndrome.

Johanson-Blizzard syndrome is an extremely rare ectodermal dysplastic disorder characterized by aplasia or hypoplasia of alae nasi, midline scalp defects, growth retardation, varying degrees of mental retardation, hypothyroidism, exocrine pancreatic insufficiency and congenital deafness. This condition is supposed to be an autosomal recessive disorder. We are reporting a female neonate with the characteristic features and an uncommon less emphasized feature viz. cafe-au-lait spots.

Ectrodactilia ectodérmica displásica: plano de tratamento. Parte 2 / Ectrodactyly ectodermal dysplasia: treatment plan. Part 2

A Síndrome da Ectrodactilia Ectodérmica Displásica com Fenda Labiopalatina, Síndrome EEC (Ectrodactyly Ectodermal Dysplasia Clefting) ou Síndrome da Garra de Lagosta, compõem-se de ectrodactilia associada à displasia ectodérmica e às fendas faciais. A etiopatogenia é genética, com transmissão autossômica dominante e expressividade variável. A sintomatologia inclui alterações na pele, como hiperceratose e xerodermia, deformidades peculiares nas mãos ou pés (como garras de lagosta), fissuras labiais, cabelos escassos e finos, microdontia, amelogênese imperfeita, hipodontia e oligodontia. A relevância deste artigo é relatar o tratamento odontológico proposto em um caso de Síndrome da Ectrodactilia Ectodérmica Displásica com Fenda Labiopalatina, a fim de restabalecer os fatores estéticos, funcionais e psicossociais da paciente

Ectodermal dysplasia with total anodontia: rehabilitation of a seven year old child.

Total anodontia in a paediatric patient is a difficult prosthetic problem. Upper and lower complete dentures were fabricated for a 7 year old patient with anodontia. The problems encountered on account of the age of the child, the absence of alveolar processes, the tooth size and acceptability of the dentures are discussed herein.

Síndrome de ectrodactilia-displasia ectodérmica-hendidura (EEC): revisión de la literatura; reporte de un caso / Ectrodactyly-ectodermal dysplasia-fissure syndrome (EEC): a review of the literature; report of a case

Este síndrome se caracteriza por la presencia de la tríada ectrodactilia-displasia ectodérmica-hendidura. Esta asociación muchas veces es confundida con otro tipo de entidades, quedando así erróneamente diagnosticada, por lo cual se plantean varios diagnóstico diferenciales del mismo. El siguiente trabajo presenta un caso clínico de un paciente masculino de 5 años de edad con diagnóstico presuntivo de displasia condroectodérmica y el cual cursaba con hendidura labiopalatina completa bilateral asociada a ectrodactilia de manos y pies, polidactilia postaxial, trastornos dentarios y obstrucción de ambos conductos lagrimales. Para tales efectos, se realizó un examen genético, clínico, radiografía panorámica y de tórax y una evaluación oftalmológica y neurológica. De estos exámenes se pudo concluir que en base a las características genéticas y clínicas representaba un caso de síndrome EEC. Estos resultados sugieren que el diagnóstico de estas entidades clínico-patológicas debe realizarse en conjunto con un grupo de especialistas para de esta forma establecer un plan de tratamiento adecuado para el paciente. Finalmente se describen el diagnóstico y plan de tratamiento dental instaurado en el paciente

Aplasia cutis congénita asociada a feto papiráceo / Aplasia cutis congenita associated to fetus papiraceous

La aplasia cutis congénita es un heterogéneo grupo de enfermedades caracterizado por la ausencia localizada o extendida de áreas de piel al momento del nacimiento, que en ocasiones es una manifestación de complejos síndromes malformativos. Presentamos una niña de 2 días de vida, con extensa aplasia cutis de tronco y miembros, sin malformaciones asociadas, producto de un embarazo gemelar. En el examen placentario se encontró feto muerto retenido (feto papiráceo)

Orodental abnormalities in lobster claw syndrome (a type of syndactyly).

Lobster Claw Syndrome is a type of syndactyly, where abnormalities in the hand-foot region as well as the orodental region occur. A case of a 14-year old boy with this syndrome is presented here.

Hypohidrotic ectodermal dysplasia--a case report.

A case presented here is that of a nine year old male patient with total anodontia. Findings of this case as regards to the orofacial, radiographic and other general manifestations were suggestive of hypohidrotic ectodermal dysplasia. The dental problems were best managed by prosthetic replacement of dentition taking into consideration a design which would provide adequate relief for the preservation of the ridges which were thin and underdeveloped to the absence of teeth.