A síndrome da fenda palatina-sinéquia lateral (CPLS) é uma síndrome de malformação congênita caracterizada pela associação de fenda palatina e sinéquias laterais intra-orais conectando as bordas livres do palato e o assoalho da boca. Presume-se que o CPLS seja herdado de maneira autossômica dominante.
Introdução
O que você precisa saber de cara
A síndrome da fenda palatina-sinéquia lateral (CPLS) é uma síndrome de malformação congênita caracterizada pela associação de fenda palatina e sinéquias laterais intra-orais conectando as bordas livres do palato e o assoalho da boca. Presume-se que o CPLS seja herdado de maneira autossômica dominante.
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Genética e causas
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Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
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Publicações mais relevantes
Anaesthetic management of an infant with cleft palate lateral synechiae syndrome.
An infant with restricted mouth opening from birth had presented for cleft lip repair. He had an interalveolar gap of 6 mm and was diagnosed as a case of cleft palate lateral synechiae syndrome. Fibreoptic bronchoscope of appropriate size was not available at the time of the procedure, and we had to device an alternative plan. The case describes the common challenges that can arise while anaesthetising infants with this syndrome in a resource-limited setting and highlights the importance of adapting the protocol to the demands.
Early Surgical Intervention of Congenital Syngnathia.
Congenital syngnathia is a rarely reported malformation when there is a fusion between the maxilla and the mandible. It is necessary to modify it in childhood because congenital syngnathia causes incongruity in pronunciation, diet, and esthetics during the growth process. In this case report, 1 case of syngnathia, a rare craniofacial anomaly, is presented with a review of reports. Prompt diagnosis and surgery were performed right after birth for the present case. A partial limitation point was resolved for further growth. Herein, the authors present the case of a female infant (7 d after birth) diagnosed with congenital syngnathia and treated by early surgical intervention.
Application of Computer-Assisted Preoperative Planning in Treating Congenital Bilateral Zygomatico-Maxillo-Mandibular Fusion: A Case Report and Literature Review.
Congenital fusion of the jaws (syngnathia) is a rare facial malformation with an unknown etiology. This disease may vary in severity with adhesion of soft tissue and bony fusion. It can be anterior fusion, unilateral or bilateral fusion, and complete fusion. The main problem of these patients is the difficulty of airway maintenance and feeding, and the most common postoperative complication is the relapse of bony fusion. Here, we report a young male patient with bony syngnathia, involving bilateral fusion of the ascending ramus and body of the mandible with the maxillary complex. We performed bone isolation by computer-assisted preoperative planning and used an insertional temporalis flap to fix the wound surface to prevent refusion of bone.
Cleft palate-lateral synechiae syndrome: a case report.
Cleft palate-lateral synechiae syndrome (CPLSS) is an extremely rare congenital malformation syndrome with undetermined etiology, characterized by a cleft palate and lateral intraoral synechiae linking the free borders of the palate to the mouth floor. We report a case of a female neonate, admitted for suckling difficulties with a cleft lip and palate associated to multiple lateral intraoral synechiae. Resection of the synechiae allowed oral feeding. Cleft palate-lateral synechiae syndrome is an exceptional syndrome as only seventeen cases have been reported in the literature. Synechiae can be isolated or more frequently in association with other congenital anomalies such as cleft lip and/or palate. These synechiae can cause functional deficits, especially in the respiratory and feeding tracts, language disorders or recurrent otitis. Although it is exceptional, this malformative entity must be known by medical practitioners in order to set up a well-adapted therapeutic protocol.
A new surgical approach to treatment of bilateral syngnathia in a patient with popliteal pterygium-syndrome.
This case describes the treatment of syngnathia with a popliteal-pterygium syndrome. Although short-term successful surgical treatment has been described in literature, long-term follow up is essential, as the mouth opening limitations can be repetitively reoccurring. This requires new, individual treatment strategies as demonstrated in this case. A young patient is shown, who is successfully undergoing an alloplastic temporomandibular joint replacement to improve mouth opening, increasing the posterior airway space and facilitating the food intake.
Publicações recentes
Cleft palate lateral synechia syndrome in two patients and literature review.
Nonsyndromic palate Synechia with floor of mouth.
🥇 Revisão sistemáticaCongenital intra-oral adhesions: a surgical approach to cleft palate lateral synechia syndrome.
🥈 ObservacionalCleft palate lateral synechia syndrome.
Cleft palate lateral synechia syndrome: an opportunity for unique surgical closure.
📚 EuropePMC8 artigos no totalmostrando 17
Anaesthetic management of an infant with cleft palate lateral synechiae syndrome.
BMJ case reportsCleft palate-lateral synechiae syndrome: a case report.
The Pan African medical journalEarly Surgical Intervention of Congenital Syngnathia.
The Journal of craniofacial surgeryA new surgical approach to treatment of bilateral syngnathia in a patient with popliteal pterygium-syndrome.
Journal of stomatology, oral and maxillofacial surgeryFgf8 dosage regulates jaw shape and symmetry through pharyngeal-cardiac tissue relationships.
Developmental dynamics : an official publication of the American Association of AnatomistsApplication of Computer-Assisted Preoperative Planning in Treating Congenital Bilateral Zygomatico-Maxillo-Mandibular Fusion: A Case Report and Literature Review.
The Cleft palate-craniofacial journal : official publication of the American Cleft Palate-Craniofacial AssociationCleft palate lateral synechia syndrome in two patients and literature review.
International journal of oral and maxillofacial surgeryVertical Distraction for the Treatment of Congenital Syngnathia.
The Journal of craniofacial surgerySyngnathia in Spectrum of Oromandibular Limb Hypogenesis Syndrome.
The Journal of craniofacial surgeryCongenital Unilateral Zygomatico-Maxillo-Mandibular Fusion.
The Journal of craniofacial surgeryCongenital Synechia and Syngnathia: Two Case Reports.
Journal of the College of Physicians and Surgeons--Pakistan : JCPSPIRF6 and SPRY4 Signaling Interact in Periderm Development.
Journal of dental researchSyngnathia Between the Palate and Mouth Floor, Cleft Palate, and Funnel Chest.
The Journal of craniofacial surgeryAnesthesia-airway management in a newborn with syngnathia.
Journal of clinical anesthesiaNonsyndromic palate Synechia with floor of mouth.
Annals of maxillofacial surgeryIsolated congenital maxillomandibular synechiae.
American journal of otolaryngologyCongenital intra-oral adhesions: a surgical approach to cleft palate lateral synechia syndrome.
International journal of pediatric otorhinolaryngologyAssociações
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Comunidades
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Referências e fontes
Bases de dados externas citadas neste artigo
Publicações científicas
Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.
- Anaesthetic management of an infant with cleft palate lateral synechiae syndrome.
- Early Surgical Intervention of Congenital Syngnathia.
- Application of Computer-Assisted Preoperative Planning in Treating Congenital Bilateral Zygomatico-Maxillo-Mandibular Fusion: A Case Report and Literature Review.The Cleft palate-craniofacial journal : official publication of the American Cleft Palate-Craniofacial Association· 2023· PMID 35440222mais citado
- Cleft palate-lateral synechiae syndrome: a case report.
- A new surgical approach to treatment of bilateral syngnathia in a patient with popliteal pterygium-syndrome.
- Cleft palate lateral synechia syndrome in two patients and literature review.
- Nonsyndromic palate Synechia with floor of mouth.
- Congenital intra-oral adhesions: a surgical approach to cleft palate lateral synechia syndrome.
- Cleft palate lateral synechia syndrome.
- Cleft palate lateral synechia syndrome: an opportunity for unique surgical closure.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:2016(Orphanet)
- OMIM OMIM:119550(OMIM)
- MONDO:0007337(MONDO)
- GARD:1391(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Q55373613(Wikidata)
Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
