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Sequência de Pierre Robin
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A Sequência de Pierre Robin (SPR) é uma anomalia congénita caraterizada pela presença de uma combinação de hipoplasia mandibular (micrognatia), glossoptose (retrusão da língua para a via faríngea) levando a obstrução das vias aéreas, e frequentemente a fenda palatina, tipicamente em forma de U. O objetivo desta dissertação é fazer uma revisão da literatura acerca do diagnóstico, etiopatogénese, apresentação clínica, modalidades de tratamento e crescimento facial dos pacientes com SPR. Uma pesquisa da bibliografia disponível foi realizada até Junho de 2015 nas bases de dados MEDLINE e LILACS, sendo incluídos os seguintes tipos de artigos: ensaios clínicos randomizados, meta-análises, revisões sistemáticas e literárias, guidelines, ensaios clínicos, estudos coorte e caso-controlo. O diagnóstico da SPR é tipicamente feito ao nascimento, através de exame clínico, endoscopia, polissonografia, estudos laboratoriais e exame radiológico. A causa exata e a fisiopatologia da SPR ainda são desconhecidas, contudo existem duas formas de apresentação da condição, em que um defeito de crescimento inerente pode estar isolado ou a mandíbula pequena pode ser parte de uma Síndrome craniofacial. Em termos de tratamento, pode-se optar por uma abordagem não-invasiva ou invasiva. Como modalidades não-invasivas, verificam-se o posicionamento, a Intubação nasofaríngea (INF), o CPAP e o PBEP; como terapias invasivas, têm-se a Adesão lábio-língua (ALL), a Libertação subperióstea do pavimento da boca (LSPB), a Distração óssea mandibular (DOM) e a Traqueotomia. A aparência facial de recém-nascidos com SPR é caraterizada por um mento severamente retrusivo. O restante desenvolvimento da face é controverso na literatura. Há uma discussão frequente se a mandíbula tem um potencial normal de crescimento anterior para compensar parcial ou completamente o défice neonatal. De um modo geral, os pacientes com SPR na sua forma isolada, apresentam um prognóstico mais favorável, seja qual for a modalidade terapêutica abordada e em termos de crescimento facial, comparativamente aos que se apresentam com uma Síndrome associada.
Pierre Robin Sequence is a congenital anomaly characterized by the conjunction of mandibular hypoplasia (micrognathia), glossoptosis (tongue retrusion to the pharyngeal airway) leading to airway obstruction, and commonly cleft palate, typically U-shaped. The goal of this dissertation is to review literature about diagnosis, etiopathogenesis, clinical presentation, management and facial growth in patients with Pierre Robin Sequenece. A research of available literature was performed until June 2015 in databases MEDLINE and LILACS, including the following article types: randomized controlled trials, meta-analysis, systematic and literature reviews, guidelines, clinical trials, and cohort and case-control studies. Diagnosis of Pierre Robin Sequence is usually made at birth, through clinical examination, endoscopy, polysomnography, laboratory studies and radiology. Exact cause and pathophysiology are still unknown, however there are two presentation forms of the condition, in which a inherent growth defect may be isolated or a small mandible may be part of a craniofacial Syndrome. About treatment, it is possible to opt by a non-invasive or invasive approach. As non-invasive modalities, there are positioning, nasopharyngeal intubation, CPAP and PEBP; as invasive therapies, there are Tongue-lip adhesion, Subperiosteal release of the floor of the mouth, Mandibular Distraction Osteogenesis and Tracheostomy. Facial aspect of a newborn with Pierre Robin Sequence is characterized by a severely retrusive chin. The remaining facial development is controversial in literature. There is an usual discussion if mandible has a normal potential of anterior growth to compensate partially or entirelly the neonatal deficit. In general, patients with isolated form present a better prognosis than those with an associated Syndrome, whatever adressed therapeutic modality and in terms of facial growth.
Pierre Robin Sequence is a congenital anomaly characterized by the conjunction of mandibular hypoplasia (micrognathia), glossoptosis (tongue retrusion to the pharyngeal airway) leading to airway obstruction, and commonly cleft palate, typically U-shaped. The goal of this dissertation is to review literature about diagnosis, etiopathogenesis, clinical presentation, management and facial growth in patients with Pierre Robin Sequenece. A research of available literature was performed until June 2015 in databases MEDLINE and LILACS, including the following article types: randomized controlled trials, meta-analysis, systematic and literature reviews, guidelines, clinical trials, and cohort and case-control studies. Diagnosis of Pierre Robin Sequence is usually made at birth, through clinical examination, endoscopy, polysomnography, laboratory studies and radiology. Exact cause and pathophysiology are still unknown, however there are two presentation forms of the condition, in which a inherent growth defect may be isolated or a small mandible may be part of a craniofacial Syndrome. About treatment, it is possible to opt by a non-invasive or invasive approach. As non-invasive modalities, there are positioning, nasopharyngeal intubation, CPAP and PEBP; as invasive therapies, there are Tongue-lip adhesion, Subperiosteal release of the floor of the mouth, Mandibular Distraction Osteogenesis and Tracheostomy. Facial aspect of a newborn with Pierre Robin Sequence is characterized by a severely retrusive chin. The remaining facial development is controversial in literature. There is an usual discussion if mandible has a normal potential of anterior growth to compensate partially or entirelly the neonatal deficit. In general, patients with isolated form present a better prognosis than those with an associated Syndrome, whatever adressed therapeutic modality and in terms of facial growth.
Descrição
Tese de mestrado, Medicina Dentária, Universidade de Lisboa, Faculdade de Medicina Dentária, 2015
Palavras-chave
Odontologia Saúde oral Teses de mestrado - 2015