Introdução
O que você precisa saber de cara
Uma fissura facial é uma abertura ou lacuna na face, ou uma malformação de uma parte do rosto. Fissuras faciais é um termo coletivo para todos os tipos de fissuras. Todas as estruturas, como osso, tecido mole, pele, etc., podem ser afetadas. As fissuras faciais são anomalias congênitas extremamente raras. Existem muitas variações de um tipo de fissura e classificações são necessárias para descrever e classificar todos os tipos de fissuras.
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Entender a doença
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Linha do tempo da pesquisa
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Nenhum gene associado encontrado
Os dados genéticos desta condição ainda estão sendo catalogados.
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
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Fenda facial número 6 de Tessier
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Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.
Pesquisa ativa
Ensaios clínicos abertos e novidades científicas recentes
Pesquisa e ensaios clínicos
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Publicações mais relevantes
Transverse facial cleft repair: Preventing pigmentation around the postoperative scar by excising the pigmented white lip at the cleft margin-A retrospective case series of Japanese patients.
Numerous transverse facial cleft repair techniques and designs have been described; however, a complication of surgical reconstruction is scar formation with brown discoloration. This study investigated the histological basis of the darker white lip at the cleft margin, its role in postoperative brown discoloration, and the potential esthetic benefits of extending the excision. In this retrospective analysis, patients with transverse facial cleft were divided into limited and extended excision groups. Histological assessment of melanin pigment was performed using Fontana-Masson staining, and postoperative buccal scars were evaluated from photographs using the Stony Brook Scar Evaluation Scale. The Mann-Whitney U test was used for statistical analysis. We divided 16 Japanese patients (4 boys and 12 girls) into limited (n = 9) and extended excision groups (n = 7). The cleft length was 8.6 ± 2.1 and 8.9 ± 3.3 mm in the limited and extended excision groups, respectively, with unilateral/bilateral laterality being 9/0 and 6/1, respectively. Initial surgeries for both groups were performed at 11.4 ± 8.4 and 8.3 ± 7.7 months, and scar evaluations were based on photographs obtained at 47.8 ± 13.5 and 27.1 ± 14.2 months, respectively. Histology showed increased melanin in the darker area of the white lip at the cleft margin, with markedly reduced pigment density in the normal-toned area. The extended excision group had less colored scar than the limited excision group (p < 0.05). Incorporating pericleft pigmentation excision into surgical design may reduce postoperative periscar hyperpigmentation and improve esthetic outcomes for several years.
Facial Bone Defects Associated with Lateral Facial Clefts Tessier Type 6, 7 and 8 in Syndromic Neurocristopathies: A Detailed Micro-CT Analysis on Historical Museum Specimens.
Lateral facial clefts are rare and often part of more complex syndromic neurocristopathies. According to Tessier's classification, they correspond to facial cleft numbers 6, 7 and 8. Using micro-computer tomography (micro-CT), we analyzed their underlying bone defects (resolution 50 and 55 µm/voxel) in the context of the known syndrome-specific genetic background. Lateral facial clefts were diagnosed in three severely affected museum specimens representing mandibulofacial dysostosis type Treacher Collins syndrome (TCS), acrofacial dysostosis syndrome of Rodriguez (AFD-Rod) and tetra-amelia syndrome (TETAMS). The TCS specimen mainly showed an absence of the zygomatic bones and most of the lateral maxilla. The AFD-Rod specimen showed an extensive defect of the lateral maxilla, zygomatic bones, and mandible. The TETAMS specimen showed almost isolated agnathia. Possible relationships are discussed between the diverse facial bone defects due to apoptosis of neural crest-derived cells, known to be associated with ribosomopathies and spliceosomopathies, such as TCS and AFD-Rod, and the more targeted bone defects due to genetic variants known to cause TETAMS.
Clinical Manifestations of and Surgical Treatment Strategies for Tessier No. 3 and No. 4 Clefts in Chinese Patients: A Large Retrospective Case Series.
Tessier no. 3 and no. 4 clefts are rare and difficult-to-treat orbitofacial malformations. The aim of the present study is to describe the clinical manifestations of these rare clefts observed in 21 patients treated with surgery over the past 15 years. The clinical data of all patients with Tessier no. 3 and no. 4 clefts treated between 2009 and 2024 by a single senior surgeon were reviewed. The patient's demographic data and associated clinical findings, including eyelid, lacrimal system, and eyeball anomalies, were recorded. The reconstruction methods used for each patient were noted and evaluated retrospectively. This study included 21 eyes of 21 patients with Tessier no. 3 and no. 4 clefts who underwent plastic surgery. Eight males and thirteen females were identified, and the age at initial treatment ranged from 1 to 20 years. A lower eyelid coloboma was observed in most patients (14 patients); 12 of whom presented with medial canthus dystopia. Seven patients presented with isolated medial canthus dystopia. Thirteen patients had lacrimal system anomalies. Microphthalmia and ipsilateral anophthalmia were detected in 5 and 3 patients, respectively. Satisfactory esthetic and functional outcomes were achieved in all patients owing to the use of traditional techniques and techniques modified by the authors. The clinical manifestations of Tessier no. 3 and no. 4 facial clefts vary, thus treatment plans and techniques must be personalized to meet the needs for each patient.
Tessier Craniofacial Clefts.
Craniofacial clefts are extremely rare. In 1976, Paul Tessier developed a comprehensive numerical classification system to categorize craniofacial clefts based on precise anatomic location. In order to comprehend craniofacial cleft development, it is crucial to understand embryologic development of the face. Craniofacial clefts can be classified into 4 main categories: oral-nasal (number 0-3), oral-ocular (number 4-6), lateral face (number 7-9), and cranial (number 10-14). Treatment goals include restoring oral competence and function, achieving nasal symmetry, establishing separation of anatomically distinct cavities, addressing orbital asymmetries, hypotelorism, or hypertelorism, and reconstructing cranial defects caused from encephaloceles.
Comprehensive surgical reconstruction of Tessier number 7 congenital craniofacial cleft. A rare case report.
Introduction and importance Tessier 7 craniofacial congenital cleft is a rare anomaly, occurring in about 1 in 80,000 to 1 in 300,000 live births, comprising 0.3% to 1.0% of total cleft cases. A total of 24 cases have been reported since 2000. This case is the 25th instance and possibly the first reported in Nepal. A 3-year-old child, accompanied by parents, presented at the Department of Oral and Maxillofacial Surgery with complaints of feeding difficulties, speech impediment, and aesthetic concerns. Diagnosis revealed Tessier number 7 congenital cleft. Surgical intervention successfully repaired the cleft, involving straight-line closure of mucosa and skin, suturing of perioral muscles to establish a new modiolus and formation of a new commissure. Postoperative follow-up over 6 months demonstrated excellent functional and aesthetic results without any complications. Tessier 7 congenital cleft arises from anomalous fetal development, stemming from incomplete fusion of the maxillary and mandibular processes of the first pharyngeal arch. Surgical correction poses challenges due to atypical anatomical positioning and cleft appearance. The repair involves layered closure, linear mucosal closure, perioral muscle reorganization to establish a new modiolus, skin closure via straight-line or z-plasty techniques, culminating in the creation of a new commissure. Given its rarity, surgeons must be well-versed in the intricate surgical protocol for Tessier 7 cleft treatment. Early intervention is crucial for optimal functional and cosmetic results. Key steps encompass establishing a new modiolus, forming a new commissure, and achieving effective skin closure.
Publicações recentes
Clinical Manifestations of and Surgical Treatment Strategies for Tessier No. 3 and No. 4 Clefts in Chinese Patients: A Large Retrospective Case Series.
Facial cleft presenting as a congenital facial papule.
A Rare Patient With Midline Facial Cleft.
Surgical Management of Median Cleft Lip Extending as Far as Alveolus Using Bone Grafting.
Craniofacial Cleft: A Case of Tessier 10 Cleft With a Novel Method to Repair Large Bilateral Colobomas.
📚 EuropePMCmostrando 18
Transverse facial cleft repair: Preventing pigmentation around the postoperative scar by excising the pigmented white lip at the cleft margin-A retrospective case series of Japanese patients.
JPRAS openFacial Bone Defects Associated with Lateral Facial Clefts Tessier Type 6, 7 and 8 in Syndromic Neurocristopathies: A Detailed Micro-CT Analysis on Historical Museum Specimens.
BiologyClinical Manifestations of and Surgical Treatment Strategies for Tessier No. 3 and No. 4 Clefts in Chinese Patients: A Large Retrospective Case Series.
The Journal of craniofacial surgeryTessier Craniofacial Clefts.
Clinics in plastic surgeryComprehensive surgical reconstruction of Tessier number 7 congenital craniofacial cleft. A rare case report.
International journal of surgery case reportsFacial cleft presenting as a congenital facial papule.
Pediatric dermatologyTessier's Cleft Number 6 Revisited: A Series of 26 new Cases and Literature Review of 44.
The Cleft palate-craniofacial journal : official publication of the American Cleft Palate-Craniofacial AssociationPresentation and Management of Atypical Orofacial Clefts: A Single-Institution Experience for 13 Year Period.
The Cleft palate-craniofacial journal : official publication of the American Cleft Palate-Craniofacial AssociationA Rare Patient With Midline Facial Cleft.
The Journal of craniofacial surgeryRare Facial Cleft: Surgical Treatment and Middle-Term Follow-up During Charity Operation.
Craniomaxillofacial trauma & reconstructionSurgical Management of Median Cleft Lip Extending as Far as Alveolus Using Bone Grafting.
The Bulletin of Tokyo Dental CollegeCraniofacial Cleft: A Case of Tessier 10 Cleft With a Novel Method to Repair Large Bilateral Colobomas.
The Cleft palate-craniofacial journal : official publication of the American Cleft Palate-Craniofacial AssociationLateral Canthal Clefts of the Eyelid.
Ophthalmic plastic and reconstructive surgeryRepairing a Facial Cleft by Polyether-Ether-Ketone Implant Combined With Titanium Mesh.
The Journal of craniofacial surgeryTessier Number 30 Median Mandibular Cleft With Congenital Heart Anomalies in Qena, Egypt.
The Cleft palate-craniofacial journal : official publication of the American Cleft Palate-Craniofacial AssociationThe "Double" Tessier 7 Cleft: An Unusual Presentation of a Transverse Facial Cleft.
The Cleft palate-craniofacial journal : official publication of the American Cleft Palate-Craniofacial AssociationOblique facial clefts in Johanson-Blizzard syndrome.
American journal of medical genetics. Part AOrbital soft tissue surgery for patients with Treacher-Collins or Nager syndrome. A new surgical approach with early correction of soft tissue: prospective study.
The British journal of oral & maxillofacial surgeryAssociações
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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.
- Transverse facial cleft repair: Preventing pigmentation around the postoperative scar by excising the pigmented white lip at the cleft margin-A retrospective case series of Japanese patients.
- Facial Bone Defects Associated with Lateral Facial Clefts Tessier Type 6, 7 and 8 in Syndromic Neurocristopathies: A Detailed Micro-CT Analysis on Historical Museum Specimens.
- Clinical Manifestations of and Surgical Treatment Strategies for Tessier No. 3 and No. 4 Clefts in Chinese Patients: A Large Retrospective Case Series.
- Tessier Craniofacial Clefts.
- Comprehensive surgical reconstruction of Tessier number 7 congenital craniofacial cleft. A rare case report.
- Facial cleft presenting as a congenital facial papule.
- A Rare Patient With Midline Facial Cleft.
- Surgical Management of Median Cleft Lip Extending as Far as Alveolus Using Bone Grafting.
- Craniofacial Cleft: A Case of Tessier 10 Cleft With a Novel Method to Repair Large Bilateral Colobomas.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:141265(Orphanet)
- MONDO:0015417(MONDO)
- GARD:19969(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Q55785463(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
