Fissura lábio-palatina é um grupo de condições em que se inclui a fissura labial, a fenda palatina e ambas. Uma fenda labial representa uma fenda no lábio superior que pode se prolongar até ao nariz. A fenda pode situar-se em um dos lados, em ambos os lados ou ao centro. Uma fenda palatina ocorre quando o céu da boca apresenta uma fenda que se prolonga até à cavidade nasal. Estas malformações podem causar problemas na alimentação, na fala e na audição e resultam em infeções do ouvido frequentes. Em menos de metade dos casos a condição está associada a outras condições.
Introdução
O que você precisa saber de cara
A fenda nasal paramediana é uma anomalia congênita rara caracterizada por uma fenda unilateral ou bilateral no nariz, geralmente localizada ao lado da linha média. Pode variar em gravidade, afetando a cartilagem, osso e tecidos moles do nariz, impactando a estética e a função respiratória.
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Entender a doença
Do básico ao detalhe, leia no seu ritmo
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Linha do tempo da pesquisa
Triagem neonatal (Teste do Pezinho)
A triagem neonatal permite diagnóstico precoce e início imediato do tratamento.
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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 nasal
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Pesquisa ativa
Ensaios clínicos abertos e novidades científicas recentes
Pesquisa e ensaios clínicos
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Publicações mais relevantes
Infected type 1 congenital upper lip sinus in 8 years child: A rare case report.
A congenital lip sinus is a rare condition, also known as a lip pit or dimples. The lower lip sinus is more frequently observed with a prevalence of approximately 0.001% in general populations, while the upper lip sinus is even rarer with not more than 70 documented cases in English medical literature. These sinuses can be located at various positions on the lip, including midline, paramedian, or lateral, on either the upper or lower lip. In some cases, lip pits are associated with other congenital anomalies such as cleft lip or cleft palate or both. In this particular case, an 8-year-old girl from Nepal presented with pain, swelling, and a yellowish discharge from a pit located in the midline of her upper lip, just below the columella of the nose at the philtrum. She was diagnosed with a Type 1 congenital upper lip sinus. Flap-based tissue transfer, commonly involving skin or mucosa, is a key method in reconstructive surgery for achieving wound closure. Flaps are especially useful when closure through secondary intention, primary linear closure, or skin grafting is either not feasible or would result in suboptimal functional or aesthetic outcomes. Flaps are classified primarily by their blood supply. Axial flaps derive their blood supply from a specific, named artery running longitudinally through both the flap and its pedicle. In contrast, random pattern flaps depend on the dermal and subcutaneous vascular plexus for perfusion and venous drainage. Flaps may also be grouped based on the relationship between the harvest site and the defect. Free flaps involve harvesting tissue from a distant site, transferring it to the wound, and revascularizing it through microsurgical anastomoses. Regional flaps move nonadjacent tissue near the defect into the wound while maintaining a vascular pedicle for perfusion. Local flaps transfer tissue adjacent to the defect to repair the wound. Generally, free and regional flaps are supplied by axial blood vessels, while local flaps are typically perfused through random pattern blood vessels. Local flaps are further subdivided based on their primary movement. Advancement and rotation flaps, also known as sliding flaps, recruit adjacent tissue and move in a linear or arced motion, respectively, to fill the primary defect. Transposition and interpolation flaps, also referred to as lifting flaps, involve incising and shifting noncontiguous donor tissue to replace intact tissue, thereby closing a wound. Common transposition flaps in cutaneous surgery include bilobed, rhombic, nasolabial (melolabial) flaps, and Z-plasties. These flaps are particularly useful for defects near the medial and lateral canthi, cheeks, and the lateral upper 2/3 of the nose, but they also have a well-defined role in lateral forehead, temple, perioral, inferior chin, and dorsal hand defects. Interpolation flaps, such as the paramedian forehead flap, involve transferring nonadjacent tissue across or underneath intact tissue that remains undisturbed by the procedure. Due to this topology, interpolation flaps are usually more accurately classified as regional flaps. Ultimately, the exact nomenclature of a local flap is less important than its topology and physiology. Additionally, many of the same principles applied to the design of local flaps may also be applied to larger, regional flaps with axial blood supplies. Examples include propeller transposition flaps in the extremities, omental transposition flaps in the abdomen, and muscular transposition flaps in the pelvis and breast. Many application-specific, named transposition flaps exist, such as the Estlander lip-switch flap for lip reconstruction, the Furlow double-opposing Z-plasty for cleft palate closure, and the Fricke lid-switch flap for eyelid repair. This article will focus on the more generally applicable types of local transposition flaps as they pertain to facial surgery.
Anthropometric Change After Primary Repair of Rare Craniofacial Clefts.
This study aimed to investigate the clinical characteristics according to Tessier classification and evaluate the surgical outcomes in patients with rare craniofacial cleft (RCC) primary repair. A retrospective study on 30 patients with RCC was conducted at the Department of Craniofacial and Plastic Surgery of the Vietnam National Hospital of Pediatrics. Rare craniofacial cleft was recorded according to Tessier's classification and was analyzed for gender, affected side, clinical characteristics, and associated abnormalities. Primary surgical correction was based on the concept of aesthetic units with multiple Z-plasty. Preoperative deformities and postoperative outcomes were evaluated with anthropometric measurements using the Versnel scoring system. The most common type was Tessier 7 cleft (T7), followed by T0. There was no difference between the frequency of males and females. Patients with unilateral cleft accounted for a larger proportion than bilateral cleft (76.5% versus 23.5%; P = 0.029 <0.05). The median and paramedian cleft groups (T0, T1, T30) affected orbit 22.2%-nose 77.8%-mouth 44.4%. The oblique clefts (T3, T4, T5, T11) affected orbit 100%-nose 50%-mouth 50%. The transverse cleft group (T6, T7, T8) affected mouth 94.1%-ear 29.4%. Rare craniofacial cleft may present alone or in a syndrome (Treacher Collin, Goldenhar, Hemifacial atrophy), or in combination with other abnormalities. Repairing RCC with aesthetic units with multiple Z-plasty has improved facial balance and restored key landmarks with acceptable scar position. The Versnel scoring system can serve as an objective instrument to measure the surgical outcomes of RCC repair and can be used to evaluate the influence of growth.
A Rare Presentation of Synchronous Thyroglossal Cyst and Branchial Cyst in an Adult Male Patient.
The most common congenital cervical masses are thyroglossal cysts followed by branchial cleft anomalies. However, their synchronous presentation is uncommon. A man in his early thirties visited our ear, nose, and throat (ENT) outpatient department (OPD) with complaints of a three-month history of right-side neck swelling. On examination, a 6 cm x 4 cm non-tender, oval-shaped cystic swelling was noted towards the right side of the neck, extending obliquely to the midline. Incidentally, another swelling measuring 2 cm x 1 cm towards the left paramedian of the neck was well-defined, non-tender, cystic in consistency, and moved with deglutition and protrusion of the tongue. Investigations such as fine needle aspiration cytology, ultrasonography, and MRI neck plain and contrast confirmed the diagnosis of a right-sided type 2 branchial cyst with a coexisting thyroglossal cyst. Right branchial cyst excision with Sistrunk procedure was done under general anesthesia. This rare occurrence requires the surgeon to know the embryological basis of such cervical anomalies. We herein report a case of unusual co-occurrence of thyroglossal cyst and branchial cyst in an adult male patient.
A Clinical Report of the Complete Nasal Agenesis: Reconstruction of Congenital Arhinia and Review of the Literature.
Congenital absence of the nose or arhinia is an exceptionally rare craniofacial malformation, and the pathophysiology of the arhinia is still unknown. Most arhinia patients can have difficulties with breathing and feeding due to the absence of the nose, nasal cavities, and associated problems. A 38-day-old female patient was referred to our clinic with arhinia. Physical examination revealed the complete agenesis of nasal structures as the nasal bones and vestibulum nasi. The region of the absent nose was flat and firm at palpation. Congenital arhinia may occur with other associated malformations such as ocular, ear, palate, and gonadal. Therefore, it is recommended to evaluate computed tomography/magnetic resonance imaging in the postnatal period. Additionally, a radiological evaluation will help nasal reconstruction by documenting changes in nasal and maxillary anatomy over time. Due to the limited number of arhinia cases presented, the surgical management of this condition has not been standardized. We presented the pyramid-shaped cartilage grafts for the nasal framework and an expanded paramedian forehead flap for the skin coverage for reconstruction of arhinia.
Secondary Treatment of Cleft Lip Correction Sequelae With Percutaneous Needleotomy, Autologous Fat Grafting, and Local Flaps: An Integrated Approach.
Cleft lip and/or palate (CL/P) is the most common congenital craniofacial malformation. The severity of the anatomic deformity is highly variable and different techniques can be employed depending on whether the defect is unilateral or bilateral, complete or incomplete. Patients usually undergo multiple steps of reconstruction throughout childhood and adolescence and, for this reason, secondary deformities of the nasolabial region of the midface may ensue. Considering our experience in the treatment of scars, we opted to correct the cleft lip surgery sequelae not only with local flaps/scar revisions, but we decided to add the use of the autologous fat grafting (AFG) to induce a regenerative effect in these young patients. In this study we present the results obtained from a series of 12 consecutive Caucasian patients aged from 23 to 44 years with widened paramedian lip scars secondary to cleft lip reconstruction surgery, treated with a combination of needle-induced lysis of the scar tissue and AFG from January 2013 to December 2018. After the procedure we observed an overall improvement in the quality of the scar tissue, in the function of mimic features, in the symmetry and cosmetics of the lower third of the nose. Patients' satisfaction was excellent. Results were long lasting and remained virtually unchanged after 1 year of follow-up. Scar release by needle, AFG and local flaps are excellent tools for the treatment of cleft lip correction sequelae, but the combination of these surgical techniques can lead to even better results.Level of Evidence: Level V.
Publicações recentes
Nasolabial Flap Variations and Alternatives in Nasal Ala Reconstruction.
Combination of Paramedian Dorsal Component Excision and Low Septal Strip Septoplasty: A Hybrid Rhinoplasty Technique with Endonasal Approach.
Anatomical Variations in Keystone Area in Nepalese Noses: A Descriptive Cross-sectional Study.
Endoscopic strategies for giant V2 schwannoma: A step-by-step guide to the combined endonasal and pre lacrimal approaches.
A Case of Nasoseptal Flap Reconstruction for Refractory Medial Canthal Fistula.
📚 EuropePMCmostrando 8
Infected type 1 congenital upper lip sinus in 8 years child: A rare case report.
Radiology case reportsAnthropometric Change After Primary Repair of Rare Craniofacial Clefts.
The Journal of craniofacial surgeryA Rare Presentation of Synchronous Thyroglossal Cyst and Branchial Cyst in an Adult Male Patient.
CureusA Clinical Report of the Complete Nasal Agenesis: Reconstruction of Congenital Arhinia and Review of the Literature.
The Cleft palate-craniofacial journal : official publication of the American Cleft Palate-Craniofacial AssociationSecondary Treatment of Cleft Lip Correction Sequelae With Percutaneous Needleotomy, Autologous Fat Grafting, and Local Flaps: An Integrated Approach.
The Journal of craniofacial surgeryManagement of median and paramedian craniofacial clefts.
Journal of plastic, reconstructive & aesthetic surgery : JPRASDouble limbal dermoid with Tessier's paramedian facial cleft.
Journal of AAPOS : the official publication of the American Association for Pediatric Ophthalmology and StrabismusPatterns of orofacial clefting in the facial morphology of bats: a possible naturally occurring model of cleft palate.
Journal of anatomyAssociações
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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.
- Infected type 1 congenital upper lip sinus in 8 years child: A rare case report.
- Anthropometric Change After Primary Repair of Rare Craniofacial Clefts.
- A Rare Presentation of Synchronous Thyroglossal Cyst and Branchial Cyst in an Adult Male Patient.
- A Clinical Report of the Complete Nasal Agenesis: Reconstruction of Congenital Arhinia and Review of the Literature.The Cleft palate-craniofacial journal : official publication of the American Cleft Palate-Craniofacial Association· 2023· PMID 35068218mais citado
- Secondary Treatment of Cleft Lip Correction Sequelae With Percutaneous Needleotomy, Autologous Fat Grafting, and Local Flaps: An Integrated Approach.
- Nasolabial Flap Variations and Alternatives in Nasal Ala Reconstruction.
- Combination of Paramedian Dorsal Component Excision and Low Septal Strip Septoplasty: A Hybrid Rhinoplasty Technique with Endonasal Approach.
- Anatomical Variations in Keystone Area in Nepalese Noses: A Descriptive Cross-sectional Study.
- Endoscopic strategies for giant V2 schwannoma: A step-by-step guide to the combined endonasal and pre lacrimal approaches.
- A Case of Nasoseptal Flap Reconstruction for Refractory Medial Canthal Fistula.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:141242(Orphanet)
- MONDO:0015414(MONDO)
- GARD:16973(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Q56013719(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
