Introdução
O que você precisa saber de cara
Fenda facial é uma abertura ou lacuna na face, ou uma malformação de uma parte do rosto. Fendas faciais é um termo coletivo para todos os tipos de fendas. Todas as estruturas, como osso, tecido mole, pele, etc., podem ser afetadas. As fendas faciais são anomalias congênitas extremamente raras. Existem muitas variações de um tipo de fenda e classificações são necessárias para descrever e classificar todos os tipos de fendas.
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Entender a doença
Do básico ao detalhe, leia no seu ritmo
Preparando trilha educativa...
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 5 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
Nenhum ensaio clínico registrado para esta condição.
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.
True Median Cleft With Sinus Tract in the Nasal Septum: A Case Report.
A true median cleft is an extremely rare congenital anomaly characterized by a midline vertical cleft and various deformities, also known as Tessier number 0 cleft. Here we report a case of a 5-year-old Asian boy with true median cleft associated with sinus tracts in the nasal septum, a phenomenon not previously reported in the literature. The tracts were identified as the cause of recurrent infections around the oral vestibule and upper lip. The tracts were resected successfully, and postoperative progress has been satisfactory. We discuss the importance of preoperative image evaluations and the selection of a suitable surgical approach.
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.
Bilateral Tessier Craniofacial Cleft Associated to Cleft Palate.
This case report presents the rare association of Tessier number 4 and 5 facial clefts with a palatal cleft in a 4-month-old infant, encountered during a humanitarian mission in Sabou, Burkina Faso. The patient, born with bilateral facial clefts and right anophthalmia, was planned for a 2-step surgical approach. The first surgery addressed the facial clefts, including a modified unilateral cleft lip repair and Z-plasty techniques for the left Tessier number 5 cleft. The second surgery, palatoplasty will be performed in the next mission. Follow-up assessments showed excellent outcomes, including improved facial aesthetics and ocular protection. The etiology of Tessier clefts remains uncertain, with multiple contributing factors such as genetic predisposition, craniofacial embryogenesis, and environmental influences. This case highlights the challenges of managing complex craniofacial deformities in resource-limited settings and underscores the importance of humanitarian missions in providing life-changing care to patients with limited access to specialized treatments.
Reconstruction of Congenital Alar Rim Defects (Tessier Number 1 Cleft) Using Laterally Based Alar Subunit Rotation Advancement Flap.
Alar rim defects develop in most cases as a result of burns, trauma, or tumor excision. Congenital alar rim defects are rare, with an incidence of 1 in 20,000 to 40,000 live births. Tessier classification is the most commonly used classification system for craniofacial defects. Facial clefts involving the nose are categorized as types 0, 1, 2, and 3, whereas cranial clefts with nose lesions are categorized as types 11, 12, 13, and 14. The patterns of nasal clefts are extremely variable, ranging from a simple notch of alar margin to complex craniofacial cleft involving the lip, nose, eyelid, brow, forehead, and underlying bone. This study was conducted at Fayoum University Hospital on 8 male patients who present with congenital alar rim defects (Tessier number 1 cleft). Surgeries were performed under general anesthesia with orotracheal intubation. A full-thickness incision was created along the whole alar subunit, keeping the ala attached only laterally (laterally based alar full-thickness flap). The alar flap was advanced and rotated medially and inferiorly and sutured to the anatomical place in 2 layers, skin and mucosa. Follow-up was done at least 1.5 years postoperatively. The study was conducted on 8 male patients presenting with congenital alar rim defects (Tessier number 1 cleft). The mean age of patients was 14.2 years, and the cleft was unilateral in 4 patients (50%) and bilateral in 4 patients (50%). The patients were assessed both aesthetically and functionally and followed at 1.5 years postoperatively. The aesthetic outcome was assessed according to both patients' and author's evaluations of the scar appearance, alar symmetry, nostril symmetry, and general satisfaction. Laterally based alar subunit rotation advancement flap is a reliable option for reconstruction of congenital alar rim defects (Tessier number 1 cleft) with the advantages of being an easy single-stage procedure with good tissue matching and nostril symmetry and only limitation of extremely wide defects with deficient tissues between the ala and tip.
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.
Reconstruction of Congenital Alar Rim Defects (Tessier Number 1 Cleft) Using Laterally Based Alar Subunit Rotation Advancement Flap.
Surgical Management of Median Cleft Lip Extending as Far as Alveolus Using Bone Grafting.
Lateral Canthal Clefts of the Eyelid.
Complete bilateral Tessier's facial cleft number 5: surgical strategy for a rare case report.
📚 EuropePMC2 artigos no totalmostrando 13
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 openClinical 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 surgeryBilateral Tessier Craniofacial Cleft Associated to Cleft Palate.
The Journal of craniofacial surgeryTrue Median Cleft With Sinus Tract in the Nasal Septum: A Case Report.
The Cleft palate-craniofacial journal : official publication of the American Cleft Palate-Craniofacial AssociationReconstruction of Congenital Alar Rim Defects (Tessier Number 1 Cleft) Using Laterally Based Alar Subunit Rotation Advancement Flap.
Annals of plastic 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 CollegeLateral Canthal Clefts of the Eyelid.
Ophthalmic plastic and reconstructive surgeryComplete bilateral Tessier's facial cleft number 5: surgical strategy for a rare case report.
Surgical and radiologic anatomy : SRAPhenotypic spectrum of Tessier facial cleft number 5.
Journal of cranio-maxillo-facial surgery : official publication of the European Association for Cranio-Maxillo-Facial SurgeryManifestation and grading of ocular involvement in patients with Tessier number 10 clefts.
Eye (London, England)Oblique 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
Organizações que acompanham esta doença — pra ter apoio e orientação
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Comunidades
Grupos ativos de quem convive com esta doença aqui no Raras
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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.
- True Median Cleft With Sinus Tract in the Nasal Septum: A Case Report.The Cleft palate-craniofacial journal : official publication of the American Cleft Palate-Craniofacial Association· 2026· PMID 39539147mais citado
- Clinical Manifestations of and Surgical Treatment Strategies for Tessier No. 3 and No. 4 Clefts in Chinese Patients: A Large Retrospective Case Series.
- Bilateral Tessier Craniofacial Cleft Associated to Cleft Palate.
- Reconstruction of Congenital Alar Rim Defects (Tessier Number 1 Cleft) Using Laterally Based Alar Subunit Rotation Advancement Flap.
- Surgical Management of Median Cleft Lip Extending as Far as Alveolus Using Bone Grafting.
- Lateral Canthal Clefts of the Eyelid.
- Complete bilateral Tessier's facial cleft number 5: surgical strategy for a rare case report.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:141261(Orphanet)
- MONDO:0015416(MONDO)
- GARD:19968(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Q55785462(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
