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Fenda facial número 5 de Tessier
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Introdução

O que você precisa saber de cara

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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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SUS: Cobertura mínimaScore: 15%
CID-10: Q18.8
🇧🇷Dados SUS / DATASUS
PROCEDIMENTOS SIGTAP (5)
0202010503
Cariótipo — bandas G, Q ou Rgenetic_test
0202010600
Pesquisa de microdeleções/microduplicações por FISHlab_test
0202010694
Sequenciamento completo do exoma (WES)rehabilitation
0202010260
Dosagem de alfa-fetoproteína
0301070040
Atendimento em reabilitação — doenças raras
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Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Últimos 10 anos13publicações
Pico20193 papers
Linha do tempo
2026Hoje · 2026📈 2019Ano de pico
Publicações por ano (últimos 10 anos)

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Genética e causas

O que está alterado no DNA e como passa nas famílias

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Diagnóstico

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Tratamento e manejo

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🇧🇷 Atendimento SUS — Fenda facial número 5 de Tessier

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Publicações mais relevantes

Timeline de publicações
0 papers (10 anos)
#1

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 open2026 May

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.

#2

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 Association2026 Jan

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.

#3

Clinical 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 surgery2025 Sep 01

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.

#4

Bilateral Tessier Craniofacial Cleft Associated to Cleft Palate.

The Journal of craniofacial surgery2025

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.

#5

Reconstruction of Congenital Alar Rim Defects (Tessier Number 1 Cleft) Using Laterally Based Alar Subunit Rotation Advancement Flap.

Annals of plastic surgery2021 Aug 01

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

Ver todas no PubMed

📚 EuropePMC2 artigos no totalmostrando 13

2026

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 open
2025

Clinical 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 surgery
2025

Bilateral Tessier Craniofacial Cleft Associated to Cleft Palate.

The Journal of craniofacial surgery
2026

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
2021

Reconstruction of Congenital Alar Rim Defects (Tessier Number 1 Cleft) Using Laterally Based Alar Subunit Rotation Advancement Flap.

Annals of plastic surgery
2020

Rare Facial Cleft: Surgical Treatment and Middle-Term Follow-up During Charity Operation.

Craniomaxillofacial trauma &amp; reconstruction
2019

Surgical Management of Median Cleft Lip Extending as Far as Alveolus Using Bone Grafting.

The Bulletin of Tokyo Dental College
2019

Lateral Canthal Clefts of the Eyelid.

Ophthalmic plastic and reconstructive surgery
2019

Complete bilateral Tessier's facial cleft number 5: surgical strategy for a rare case report.

Surgical and radiologic anatomy : SRA
2018

Phenotypic spectrum of Tessier facial cleft number 5.

Journal of cranio-maxillo-facial surgery : official publication of the European Association for Cranio-Maxillo-Facial Surgery
2017

Manifestation and grading of ocular involvement in patients with Tessier number 10 clefts.

Eye (London, England)
2016

Oblique facial clefts in Johanson-Blizzard syndrome.

American journal of medical genetics. Part A
2015

Orbital 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 &amp; maxillofacial surgery

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Doenças relacionadas

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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.

  1. 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 open· 2026· PMID 41799641mais citado
  2. 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
  3. Clinical 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 surgery· 2025· PMID 40193142mais citado
  4. Bilateral Tessier Craniofacial Cleft Associated to Cleft Palate.
    The Journal of craniofacial surgery· 2025· PMID 40043301mais citado
  5. Reconstruction of Congenital Alar Rim Defects (Tessier Number 1 Cleft) Using Laterally Based Alar Subunit Rotation Advancement Flap.
    Annals of plastic surgery· 2021· PMID 34253699mais citado
  6. Surgical Management of Median Cleft Lip Extending as Far as Alveolus Using Bone Grafting.
    Bull Tokyo Dent Coll· 2019· PMID 31761879recente
  7. Lateral Canthal Clefts of the Eyelid.
    Ophthalmic Plast Reconstr Surg· 2019· PMID 30865066recente
  8. Complete bilateral Tessier's facial cleft number 5: surgical strategy for a rare case report.
    Surg Radiol Anat· 2019· PMID 30656417recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:141261(Orphanet)
  2. MONDO:0015416(MONDO)
  3. GARD:19968(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. 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

Fenda facial número 5 de Tessier
Compêndio · Raras BR

Fenda facial número 5 de Tessier

ORPHA:141261 · MONDO:0015416
CID-10
Q18.8 · Outras malformações congênitas especificadas da face e do pescoço
CID-11
Início
Antenatal, Neonatal
MedGen
UMLS
C0432119
EuropePMC
Wikidata
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