Raras
Buscar doenças, sintomas, genes...
Malformação das vias aéreas pulmonares congênita
ORPHA:2444CID-10 · Q33.0CID-11 · LA75.4DOENÇA RARA

Um problema no desenvolvimento do pulmão que se caracteriza por uma massa cheia de cistos (bolhas). Isso ocorre devido a um crescimento anormal e exagerado das células que revestem os bronquíolos terminais (os pequenos tubos que levam o ar para dentro dos pulmões). Por causa desse crescimento, há uma diminuição na quantidade de alvéolos pulmonares (que são as pequenas bolsas de ar). Essa alteração é dividida em três tipos, de acordo com o tamanho dos cistos.

Mantido por Agente Raras·Colaborar como especialista →

Introdução

O que você precisa saber de cara

📋

Um problema no desenvolvimento do pulmão que se caracteriza por uma massa cheia de cistos (bolhas). Isso ocorre devido a um crescimento anormal e exagerado das células que revestem os bronquíolos terminais (os pequenos tubos que levam o ar para dentro dos pulmões). Por causa desse crescimento, há uma diminuição na quantidade de alvéolos pulmonares (que são as pequenas bolsas de ar). Essa alteração é dividida em três tipos, de acordo com o tamanho dos cistos.

Pesquisas ativas
2 ensaios
8 total registrados no ClinicalTrials.gov
Publicações científicas
523 artigos
Último publicado: 2026 Apr 15

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
Unknown
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.0
Europe
Início
Adolescent
+ adult, antenatal, neonatal
🏥
SUS: Cobertura mínimaScore: 35%
Centros em: PA, PE, BA, CE, PB +10CID-10: Q33.0
🇧🇷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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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

Partes do corpo afetadas

🫁
Pulmão
2 sintomas
🦴
Ossos e articulações
1 sintomas

+ 2 sintomas em outras categorias

Características mais comuns

90%prev.
Anormalidade do sistema respiratório
Muito frequente (99-80%)
17%prev.
Nascimento prematuro
Ocasional (29-5%)
17%prev.
Polidrâmnio
Ocasional (29-5%)
17%prev.
Insuficiência respiratória
Ocasional (29-5%)
17%prev.
Morfologia anormal da pleura
Ocasional (29-5%)
5sintomas
Muito frequente (1)
Ocasional (4)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 5 características clínicas mais associadas, ordenadas por frequência.

Anormalidade do sistema respiratórioAbnormality of the respiratory system
Muito frequente (99-80%)90%
Nascimento prematuroPremature birth
Ocasional (29-5%)17%
PolidrâmnioPolyhydramnios
Ocasional (29-5%)17%
Insuficiência respiratóriaRespiratory insufficiency
Ocasional (29-5%)17%
Morfologia anormal da pleuraAbnormality of the pleura
Ocasional (29-5%)17%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico523PubMed
Últimos 10 anos200publicações
Pico202578 papers
Linha do tempo
2026Hoje · 2026🧪 1999Primeiro ensaio clínico📈 2025Ano de pico
Publicações por ano (últimos 10 anos)

Encontrou um erro ou informação desatualizada? Sugira uma correção →

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

Carregando...

Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
Pipeline regulatório — de medicamentos já aprovados a drogas em pesquisa exploratória.
·Pré-clínico2
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 2 ensaios
Carregando informações de tratamento...

Onde tratar no SUS

Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)

🇧🇷 Atendimento SUS — Malformação das vias aéreas pulmonares congênita

Centros de Referência SUS

24 centros habilitados pelo SUS para Malformação das vias aéreas pulmonares congênita

Centros para Malformação das vias aéreas pulmonares congênita

Detalhes dos centros

Hospital Universitário Prof. Edgard Santos (HUPES)

R. Dr. Augusto Viana, s/n - Canela, Salvador - BA, 40110-060 · CNES 0003808

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do Metabolismo

Hospital Infantil Albert Sabin

R. Tertuliano Sales, 544 - Vila União, Fortaleza - CE, 60410-794 · CNES 2407876

Serviço de Referência

Rota
Anomalias CongênitasDeficiência Intelectual

Hospital de Apoio de Brasília (HAB)

AENW 3 Lote A Setor Noroeste - Plano Piloto, Brasília - DF, 70684-831 · CNES 0010456

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

Hospital Estadual Infantil e Maternidade Alzir Bernardino Alves (HIABA)

Av. Min. Salgado Filho, 918 - Soteco, Vila Velha - ES, 29106-010 · CNES 6631207

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

Hospital das Clínicas da UFG

Rua 235 QD. 68 Lote Área, Nº 285, s/nº - Setor Leste Universitário, Goiânia - GO, 74605-050 · CNES 2338424

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do Metabolismo

Hospital Universitário da UFJF

R. Catulo Breviglieri, Bairro - s/n - Santa Catarina, Juiz de Fora - MG, 36036-110 · CNES 2297442

Atenção Especializada

Rota
Anomalias Congênitas

Hospital das Clínicas da UFMG

Av. Prof. Alfredo Balena, 110 - Santa Efigênia, Belo Horizonte - MG, 30130-100 · CNES 2280167

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

Hospital Universitário Julio Müller (HUJM)

R. Luis Philippe Pereira Leite, s/n - Alvorada, Cuiabá - MT, 78048-902 · CNES 2726092

Atenção Especializada

Rota
Anomalias Congênitas

Hospital Universitário João de Barros Barreto

R. dos Mundurucus, 4487 - Guamá, Belém - PA, 66073-000 · CNES 2337878

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

Hospital Universitário Lauro Wanderley (HULW)

R. Tabeliao Estanislau Eloy, 585 - Castelo Branco, João Pessoa - PB, 58050-585 · CNES 0002470

Atenção Especializada

Rota
Anomalias Congênitas

Instituto de Medicina Integral Prof. Fernando Figueira (IMIP)

R. dos Coelhos, 300 - Boa Vista, Recife - PE, 50070-902 · CNES 0000647

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

Hospital Pequeno Príncipe

R. Des. Motta, 1070 - Água Verde, Curitiba - PR, 80250-060 · CNES 3143805

Serviço de Referência

Rota
Anomalias CongênitasDeficiência Intelectual

Hospital Universitário Regional de Maringá (HUM)

Av. Mandacaru, 1590 - Parque das Laranjeiras, Maringá - PR, 87083-240 · CNES 2216108

Atenção Especializada

Rota
Anomalias Congênitas

Hospital de Clínicas da UFPR

R. Gen. Carneiro, 181 - Alto da Glória, Curitiba - PR, 80060-900 · CNES 2364980

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

Hospital Universitário Pedro Ernesto (HUPE-UERJ)

Blvd. 28 de Setembro, 77 - Vila Isabel, Rio de Janeiro - RJ, 20551-030 · CNES 2280221

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do Metabolismo

Instituto Nacional de Saúde da Mulher, da Criança e do Adolescente Fernandes Figueira (IFF/Fiocruz)

Av. Rui Barbosa, 716 - Flamengo, Rio de Janeiro - RJ, 22250-020 · CNES 2269988

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

Hospital São Lucas da PUCRS

Av. Ipiranga, 6690 - Jardim Botânico, Porto Alegre - RS, 90610-000 · CNES 2232928

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do Metabolismo

Hospital de Clínicas de Porto Alegre (HCPA)

Rua Ramiro Barcelos, 2350 Bloco A - Av. Protásio Alves, 211 - Bloco B e C - Santa Cecília, Porto Alegre - RS, 90035-903 · CNES 2237601

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

Hospital Universitário da UFSC (HU-UFSC)

R. Profa. Maria Flora Pausewang - Trindade, Florianópolis - SC, 88036-800 · CNES 2560356

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do Metabolismo

Hospital das Clínicas da FMUSP

R. Dr. Ovídio Pires de Campos, 225 - Cerqueira César, São Paulo - SP, 05403-010 · CNES 2077485

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

Hospital de Base de São José do Rio Preto

Av. Brg. Faria Lima, 5544 - Vila Sao Jose, São José do Rio Preto - SP, 15090-000 · CNES 2079798

Atenção Especializada

Rota
Anomalias Congênitas

Hospital de Clínicas da UNICAMP

R. Vital Brasil, 251 - Cidade Universitária, Campinas - SP, 13083-888 · CNES 2748223

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

Hospital de Clínicas de Ribeirão Preto (HCRP-USP)

R. Ten. Catão Roxo, 3900 - Vila Monte Alegre, Ribeirão Preto - SP, 14015-010 · CNES 2082187

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

UNIFESP / Hospital São Paulo

R. Napoleão de Barros, 715 - Vila Clementino, São Paulo - SP, 04024-002 · CNES 2688689

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do Metabolismo
Sobre os centros SUS: Estes centros são habilitados pelo Ministério da Saúde como Serviços de Referência em Doenças Raras ou Serviços de Atenção Especializada. O atendimento é pelo SUS, com encaminhamento da rede de atenção básica.

Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.

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Publicações mais relevantes

Timeline de publicações
459 papers (10 anos)
#1

Loss of CA12 drive proliferation and fibrosis in type i congenital pulmonary airway malformation via promoting abnormal activation of SHH signaling.

Cell communication and signaling : CCS2026 Mar 18
#2

Anesthesia for thoracic surgery in infants with congenital lung malformations.

World journal of pediatric surgery2026

Congenital lung malformations (CLMs) are disorders arising from defective embryonic development of the airways, lung parenchyma, and pulmonary vasculature, often presenting as heterogeneous lesions like congenital pulmonary airway malformation (CPAM), pulmonary sequestration (PS), and congenital lobar overinflation (CLO). CLMs in infants present unique anesthetic challenges -particularly the risk of progressive distention in cystic areas and the anatomical difficulty of achieving stable lung isolation- but institutional rarity limits clinical experience and consensus-driven practices. This review examines recent clinical anesthesia practices for infants with CLMs undergoing thoracic surgery, offering comprehensive guidance on perioperative anesthesia management, with an emphasis on ventilation management. Crucially, we evaluate the selection and precise positioning of lung isolation devices, such as left endobronchial intubation and bronchial blockers. Furthermore, it provides a structured management strategy for intraoperative hypoxemia and outlines lung-protective ventilation frameworks-incorporating low tidal volumes and personalized PEEP-to consolidate contemporary evidence and optimize the perioperative anesthetic approach for this vulnerable population.

#3

Pulmonary Malignancies in Adults With Congenital Lung Malformations: A Scoping Review.

JTO clinical and research reports2026 Apr

We aimed to synthesize current knowledge on pulmonary malignancies in adults with congenital lung malformations (CLMs), focusing on clinical presentation, treatment approaches, and outcomes. We performed a scoping review according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses extension for scoping reviews. A systematic search was conducted in January 2025 across three databases. References were included if they described patients aged 17 years or older with a CLM and a coexisting pulmonary malignancy. Data were extracted on patient demographics, CLM and malignancy type, symptoms, treatment, and outcomes. Findings were summarized using descriptive statistics and narrative synthesis. A total of 65 references met the inclusion criteria, comprising 85 adult patients with CLM and pulmonary malignancies. Pulmonary adenocarcinoma was most frequently observed (66%), particularly within congenital pulmonary airway malformations (89% of malignancies in that subgroup). The median age at lung cancer diagnosis was 43.5 years. Respiratory symptoms were the most common presentation (67%), with 18% of patients being asymptomatic at the time of pulmonary malignancy diagnosis. Surgical resection was performed in 94% of patients, with lobectomy being the most frequently performed surgery type (68% of operated patients). More than half of malignancies (57%) were definitively diagnosed postoperatively. Follow-up data were incomplete; however, most reported patients were alive without disease recurrence at last follow-up with a median follow-up time of 14 months. The young median age at cancer diagnosis, the presence of pulmonary malignancies in asymptomatic patients, and the high proportion of malignancy diagnoses established only postoperatively underscore the need for proactive and tailored surveillance strategies in adults with known CLM. A multidisciplinary approach with longitudinal follow-up is crucial to refine risk assessment and optimize long-term care.

#4

Non-Intubated Spontaneous Ventilation Versus Endotracheal Intubation Anesthesia for Pediatric Thoracoscopic Lung Resection: A Retrospective Propensity-Score-Matched Study.

Paediatric anaesthesia2026 Mar 13

Non-intubated spontaneous ventilation anesthesia is increasingly used in adult thoracoscopic surgery, but pediatric evidence remains limited. To compare perioperative outcomes between two anesthetic regimens in children undergoing thoracoscopic anatomical lung lesion resection: a laryngeal mask airway-assisted non-intubated spontaneous ventilation anesthesia and a conventional endotracheal intubation anesthesia. This single-center retrospective cohort study included children undergoing thoracoscopic anatomical lung lesion resection for pulmonary sequestration or congenital pulmonary airway malformation between April 2024 and May 2025. Children received either a conventional endotracheal intubation anesthesia or a laryngeal mask airway-assisted non-intubated spontaneous ventilation anesthesia. One-to-one propensity score matching was performed. The primary outcome was the overall incidence of postoperative pulmonary complications before discharge. Secondary outcomes were conversion to endotracheal intubation, intraoperative lowest peripheral oxygen saturation, highest end-tidal carbon dioxide, postanesthesia care unit length of stay, chest tube indwelling time, and postoperative length of hospital stay. Other perioperative variables were exploratory. Among 198 eligible children, 50 matched pairs were analyzed. Postoperative pulmonary complications occurred in 6 of 50 children (12.0%) in the endotracheal intubation group and in 0 of 50 children (0%) in the non-intubated spontaneous ventilation group (absolute risk reduction 12.0%, 95% confidence interval 3.0-21.0; p = 0.027). In a sensitivity analysis using Firth's logistic regression model adjusting for thoracic paravertebral block, the non-intubated regimen remained associated with a lower observed incidence of postoperative pulmonary complications (adjusted odds ratio 0.06, 95% confidence interval 0.0004-0.76; p = 0.029). No child in the non-intubated spontaneous ventilation group required conversion to endotracheal intubation. Secondary outcomes showed modest between-group differences in unadjusted analyses. However, after Holm-Bonferroni adjustment, adjusted p values for secondary outcomes ranged from 0.060 to 0.208. In this retrospective propensity-score-matched pediatric cohort, a laryngeal mask airway-assisted non-intubated spontaneous ventilation anesthesia was feasible and was not associated with worse perioperative outcomes than a conventional endotracheal intubation anesthesia. A lower observed incidence of postoperative pulmonary complications was noted. Given regimen-level differences, prospective studies with standardized analgesia are needed to confirm safety and potential benefits.

#5

Antenatal Diagnosis and Management of Congenital Pulmonary Airway Malformation: A Case Report.

Cureus2026 Feb

Congenital pulmonary airway malformation (CPAM) is a rare developmental lung anomaly that can be identified on prenatal ultrasonography. We report a case of antenatally diagnosed CPAM with a progressively increasing CPAM volume ratio (CVR) on serial foetal imaging. Antenatal corticosteroids were administered, and a male neonate was delivered by elective lower-segment caesarean section at 33 weeks of gestation. Postnatal imaging confirmed CPAM involving the right middle lobe. The infant underwent right middle lobectomy on postnatal day nine with an uneventful postoperative course. This case highlights the importance of serial CVR monitoring, antenatal planning, and timely surgical management in achieving favourable outcomes in high-risk CPAM.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC253 artigos no totalmostrando 199

2026

Anesthesia for thoracic surgery in infants with congenital lung malformations.

World journal of pediatric surgery
2026

Pulmonary Malignancies in Adults With Congenital Lung Malformations: A Scoping Review.

JTO clinical and research reports
2026

Loss of CA12 drive proliferation and fibrosis in type i congenital pulmonary airway malformation via promoting abnormal activation of SHH signaling.

Cell communication and signaling : CCS
2026

Non-Intubated Spontaneous Ventilation Versus Endotracheal Intubation Anesthesia for Pediatric Thoracoscopic Lung Resection: A Retrospective Propensity-Score-Matched Study.

Paediatric anaesthesia
2026

Tiny lungs, big challenges: Anaesthetic management of neonatal lobectomy for congenital pulmonary airway malformation - A case series.

Indian journal of anaesthesia
2026

Antenatal Diagnosis and Management of Congenital Pulmonary Airway Malformation: A Case Report.

Cureus
2026

Unicentric report on thoracoscopic surgery 7 cases for congenital pulmonary airway malformation combined with ipsilateral mediastinal bronchogenic cyst in children.

BMC surgery
2026

Methicillin Resistant Staphylococcus Aureus Colonization, The Tip of the Iceberg for Congenital Pulmonary Airway Malformation. A Case Report.

La Clinica terapeutica
2026

Type 1 Congenital Pulmonary Airway Malformation: Diagnostic Accuracy of Radiological Features.

Pediatric pulmonology
2026

Surgical Management of Congenital Lung Malformations in Children-A Single-Center Analysis of 25 Years of Experience.

The clinical respiratory journal
2026

A case of Kabuki syndrome with congenital pulmonary airway malformation.

Journal of investigative medicine : the official publication of the American Federation for Clinical Research
2026

Perioperative Care for Pediatric Patients Undergoing Lung Surgery: Retrospective Single Center Review.

Paediatric anaesthesia
2026

The Safety of Conservative Management of Asymptomatic Congenital Pulmonary Airway Malformations (CPAMs) in Children: A Systematic Review.

Journal of pediatric surgery
2026

[Congenital airway malformation and recurrent epistaxis].

Ugeskrift for laeger
2026

Fetal thoracoamniotic shunting for severe macrocystic congenital pulmonary airway malformation with the Somatex® intrauterine shunt: intrauterine course and postnatal outcome.

Archives of gynecology and obstetrics
2026

Radiofrequency ablation for giant congenital lung malformations complicated by fetal hydrops: a retrospective case series.

Archives of gynecology and obstetrics
2026

Prenatal characteristics and prognosis of congenital pulmonary airway malformation: a retrospective multicenter cohort study.

Scientific reports
2025

Prenatal diagnosis and postnatal outcomes in fetuses diagnosed with congenital pulmonary airway malformations.

BMJ case reports
2025

Enhanced Recovery After Surgery for Pediatric Lung Resection: Effects of a New Protocol.

Children (Basel, Switzerland)
2025

Learning Curve and Early Outcomes of Thoracoscopic Anatomical Lesion Resection for Congenital Pulmonary Airway Malformation in Children: A Single-surgeon Experience.

European journal of pediatric surgery : official journal of Austrian Association of Pediatric Surgery ... [et al] = Zeitschrift fur Kinderchirurgie
2026

Intra-diaphragmatic extralobar pulmonary sequestration: Surgical approaches and outcome.

Journal of pediatric surgery
2025

Character and Frequency of Early Postnatal Resection in Prenatally Diagnosed Congenital Lung Malformations.

Fetal diagnosis and therapy
2026

Spontaneous pneumothorax as the initial presentation of congenital pulmonary airway malformation in a teenager without prior symptoms.

Radiology case reports
2025

Late Onset Congenital Cystic Adenomatoid Pulmonary Malformation.

Journal of Nepal Health Research Council
2025

Delayed thoracoscopic lobectomy in symptomatic newborns with congenital pulmonary airway malformation.

Journal of cardiothoracic surgery
2025

Adverse events associated with ustekinumab in Crohn's disease treatment: an analysis based on the FAERS database.

Frontiers in medicine
2025

The essential role of cytoskeleton and ciliary abnormalities in the development of congenital pulmonary airway malformations.

Pediatric surgery international
2025

Systemic Arterial Supply to a Congenital Pulmonary Airway Malformation: Imaging of a rare Hybrid BPS/CPAM Lesion.

Portuguese journal of cardiac thoracic and vascular surgery
2025

A Case of Type 1 Congenital Pulmonary Airway Malformation (CPAM) in an Eight-Year-Old Girl Without a History of Recurrent Pneumonia or Respiratory Failure, Presenting With a Pulmonary Abscess.

Cureus
2025

Maternal Health Literacy and Prenatal Learning Experiences Related to Diagnosis and Surgical Management: A Qualitative Study of Women With Pregnancies Associated With Surgically Correctable Congenital Anomalies.

Prenatal diagnosis
2025

Synergistic exposure to endocrine-disrupting chemicals in pediatric lungs: Epidemiological drivers and disease-associated variations.

Environmental pollution (Barking, Essex : 1987)
2025

Virtual reality-assisted preoperative planning for pediatric thoracoscopic segmentectomy: a retrospective study.

BMC pediatrics
2025

Prenatal diagnosis and outcome of meso/dextrocardia: a single-center report of 29 cases.

Journal of medical ultrasonics (2001)
2025

The role of thoracic ultrasound in a rare combination of lung abscess and congenital pulmonary airway malformation (CPAM): case report and brief review.

Italian journal of pediatrics
2026

Congenital Lung Malformations: A Comprehensive Overview of Current Knowledge-Narrative Review.

Archivos de bronconeumologia
2025

Type III Congenital Pulmonary Airway Malformation: A Case Report.

Iranian journal of pathology
2025

Pediatric necrotizing pneumonia case report: clinical features, treatment experience, and prospects.

Frontiers in medicine
2025

Clinical Impact of Patient-Specific 3D Models in Neonatal Surgery: A Case-Based Review of Applications and Future Directions.

Children (Basel, Switzerland)
2025

Neonatal Congenital Pulmonary Airway Malformation Causing Respiratory Failure Managed With Early Surgical Resection: A Case Report.

British journal of hospital medicine (London, England : 2005)
2025

Type 2 Congenital Pulmonary Airway Malformation in Tetralogy of Fallot with Absent Pulmonary Valve Syndrome.

The Indian journal of radiology &amp; imaging
2025

Prenatal trio-based whole exome sequencing in fetuses with congenital pulmonary airway malformation.

Translational pediatrics
2025

Timing of surgery and outcomes in patients with congenital pulmonary airway malformation: a national inpatient database study.

Pediatric surgery international
2025

Delayed Diagnosis of Primary Pulmonary Sarcoma Arising from a Pulmonary Cyst in an Adult: A Case Report.

Annals of thoracic and cardiovascular surgery : official journal of the Association of Thoracic and Cardiovascular Surgeons of Asia
2026

Prenatal Intervention in High-Risk CPAM: Postnatal Outcomes After Fetal versus Standard Surgery: A Propensity Score Matched Study.

European journal of pediatric surgery : official journal of Austrian Association of Pediatric Surgery ... [et al] = Zeitschrift fur Kinderchirurgie
2025

A Case of Hybrid of Congenital Pulmonary Airway Malformation and Anomalous Systemic Blood Supply Presenting Elevated Serum Levels of Carbohydrate Antigen 19-9.

Respirology case reports
2025

Multiple congenital pulmonary malformations in a black-capped Bolivian squirrel monkey (Saimiri boliviensis boliviensis).

Journal of comparative pathology
2025

Maternal corticosteroids for fetal congenital pulmonary airway malformation: a systematic review.

American journal of obstetrics &amp; gynecology MFM
2025

Effect analysis of flexible bronchoscopy in thoracoscopic surgery for congenital pulmonary airway malformation.

Medicine
2025

"Phenotypic and genotypic insights, counseling strategies, and follow-up in 24 individuals with filamin a deficiency: findings from a retrospective cohort study".

Acta neurologica Belgica
2025

Technique for Consistent Selective Left Mainstem Intubation in Neonatal and Infantile Surgery.

The American surgeon
2025

A diagnostic challenge: The unusual combination of Kommerell's diverticulum and pulmonary sequestration due to type 1 congenital pulmonary airway malformation in a young patient.

Radiology case reports
2025

Computed tomography (CT)-based classification and management pathways for hybrid lesions in children: a comparative analysis of congenital pulmonary airway malformation (CPAM) and bronchopulmonary sequestration (BPS).

Clinical radiology
2025

GPC3: a novel mutated gene in pleuropulmonary blastoma.

Discover oncology
2025

Critical Care Dilemma: Complex Congenital Pulmonary Airway Malformation With Pulmonary Hypertension in a Neonate.

Cureus
2025

Congenital pulmonary airway malformation type 2 associated with intralobar pulmonary sequestration in an adult patient: a case report.

Future science OA
2025

Understanding and manipulating morphogenetic processes to generate in vitro models of airways.

American journal of physiology. Lung cellular and molecular physiology
2025

Postnatal Outcomes and Surgical Implications of Somatex™ Thoracoamniotic Shunting for CPAM: A Multicenter Experience.

European journal of pediatric surgery : official journal of Austrian Association of Pediatric Surgery ... [et al] = Zeitschrift fur Kinderchirurgie
2025

Continuation of Pregnancy After Donor Twin Demise Following Fetoscopic Laser Photocoagulation to Treat Twin-Twin Transfusion Syndrome.

Cureus
2025

Single-cell sequencing reveals cellular differences and potential mechanisms in congenital pulmonary airway malformation.

Frontiers in medicine
2025

Congenital Chylothorax and Congenital Pulmonary Airway Malformation: Case Report and Literature Review.

Respirology case reports
2025

Congenital Pulmonary Airway Malformation with Pulmonary Arteriovenous Malformation in Adulthood: A Case Report.

Surgical case reports
2025

Analysis of anatomical characteristics of congenital pulmonary airway malformation lesions based on CT images.

Frontiers in pediatrics
2025

Comprehensive morphological, immunohistochemical, and molecular characterization of congenital pulmonary airway malformation (CPAM).

Virchows Archiv : an international journal of pathology
2025

Key ultrasound predictors in the prenatal assessment of congenital pulmonary airway malformation: a single-center experience.

Frontiers in pediatrics
2025

Long-Term Pulmonary Function Outcomes in Children with Pulmonary Hypoplasia.

The Journal of pediatrics
2025

Radiofrequency ablation for massive congenital pulmonary airway malformation complicated with fetal hydrops.

European journal of obstetrics, gynecology, and reproductive biology
2025

Congenital Cystic Lung Lesions: An Update on Clinical and Pathologic Correlates.

Surgical pathology clinics
2025

Congenital lung malformations: Do preoperative symptoms have an impact in surgical outcomes?

Anales de pediatria
2025

Pneumatoceles or Not? Case Reports on Pulmonary Cystic Lesions in Infants.

Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society
2025

Congenital Thoracic Malformations: A Single-Center Retrospective Study From Hong Kong.

Cureus
2025

Prenatal diagnostic accuracy and epidemiology of congenital lung malformations: A retrospective review of cases in a tertiary referral center in northern Finland in 2010-2020.

Acta obstetricia et gynecologica Scandinavica
2025

Single-Port-Plus-One Thoracoscopic Lobectomy for Infants with Congenital Pulmonary Airway Malformation: Initial Experience.

Journal of laparoendoscopic &amp; advanced surgical techniques. Part A
2025

A unique case of pulmonary minimally invasive mucinous adenocarcinoma arising from atypical goblet cell hyperplasia in the bronchial epithelium of a 9-year-old girl.

BMC pediatrics
2025

Return of the Disruption Score: Fetal Surgery in the Spotlight (1975-2021).

Journal of pediatric surgery
2025

Characterization of 45 cases of congenital lung malformation and association of inflammation with symptomatology.

Scientific reports
2025

Unveiling the Unexpected: An Intriguing Case of Neonatal Nocardial Pneumonia.

Journal of Indian Association of Pediatric Surgeons
2025

Risk of malignant transformation and infections in congenital lung malformations in adults: a systematic review.

European respiratory review : an official journal of the European Respiratory Society
2025

Novel compound heterozygous OBSCN variants in Chinese children with congenital pulmonary airway malformation.

Italian journal of pediatrics
2025

Impact of pulmonary infection on thoracoscopic surgery outcomes in children with CPAM: a retrospective study.

BMC surgery
2025

Congenital bronchogenic cyst: A case study on early detection and surgical intervention.

International journal of surgery case reports
2025

Neonatal Surgery for Congenital Lung Malformations: Indications, Outcomes and Association With Malignancy.

Journal of pediatric surgery
2025

Clinical outcomes after surgical resection in asymptomatic and symptomatic children with congenital lung malformations.

Journal of cardiothoracic surgery
2025

Enhanced recovery after minimally invasive pneumonectomies in children.

Pediatric surgery international
2025

Pneumothorax associated with a displaced thoracoamniotic Somatex shunt in an infant with congenital pulmonary airway malformation: a case report.

Hong Kong medical journal = Xianggang yi xue za zhi
2025

Mid-trimester resolution of marked dextroposition of the fetal heart preceding regression of extensive left fetal lung lesion consisting of hybrid congenital pulmonary airway malformation (CPAM) and bronchopulmonary sequestration (BPS).

Radiology case reports
2025

Congenital Pulmonary Hybrid Lesions: Clinical Presentation, Surgical Management, and Outcomes.

Indian journal of pediatrics
2025

Atypical mediastinal mass in the fetus: a review of the literature.

Archives of gynecology and obstetrics
2024

Congenital Pulmonary Airway Malformation Associated With Papillary Adenocarcinoma.

Annals of thoracic surgery short reports
2025

Cystic masses of the pediatric lung: update on congenital pulmonary airway malformation and its differential diagnosis.

Virchows Archiv : an international journal of pathology
2025

Clinical features of atypical solid-type congenital pulmonary airway malformation (CPAM) type 1.

Pediatrics and neonatology
2024

Congenital pulmonary airway malformation presenting as a solid mass: a description of two cases and a literature analysis.

Quantitative imaging in medicine and surgery
2025

Respiratory Morbidity Among Children Undergoing Surgical Resection for a Congenital Lung Malformation.

Pediatric pulmonology
2024

Posterior mediastinal extralobar pulmonary sequestration in a neonate with pulmonary artery supply: a case report.

Frontiers in medicine
2025

Precise Thoracoscopic Pneumonectomy Using Fluorescence Imaging After Aerosolized Indocyanine Green Inhalation: A Novel Strategy for Treating Congenital Pulmonary Airway Malformation.

Journal of pediatric surgery
2025

KLF5 Shapes Developing Respiratory Tubules by Inhibiting Actin Asymmetry in Epithelial Cells.

American journal of respiratory cell and molecular biology
2025

Congenital pulmonary airway malformation in a cat.

Journal of veterinary diagnostic investigation : official publication of the American Association of Veterinary Laboratory Diagnosticians, Inc
2024

A case of cytomegalovirus cystic lung disease and review of literature.

Respirology case reports
2024

Silver hair in a neonate: a tale of 2 fatal cases.

Oxford medical case reports
2024

Operative Timing for Elective Thoracoscopic Lobectomy for CPAM and Sequestration: A NSQIP-Pediatric Analysis 2017-2021.

Journal of pediatric surgery
2024

Invasive Mucinous Adenocarcinoma in a Newborn With Antenatally Diagnosed Congenital Pulmonary Airway Malformation: A Case Report.

Cureus
2024

Association of Prenatal Congenital Lung Malformation Volume Ratio to Postnatal Computed Tomography Characteristics.

The Journal of surgical research
2024

Congenital Lung Anomalies in Adults.

Radiographics : a review publication of the Radiological Society of North America, Inc
2024

Congenital Pulmonary Airway Malformation in Preterm Infants: A Case Report and Review of the Literature.

Life (Basel, Switzerland)
2024

The role of 3-dimensional reconstruction imaging in bronchopulmonary sequestration.

Multimedia manual of cardiothoracic surgery : MMCTS
2024

Imaging Three-Dimensional Airway Morphology in Congenital Pulmonary Airway Malformation Using Hierarchical Phase-Contrast Tomography.

American journal of respiratory and critical care medicine
2024

Integrative analysis of bulk and single-cell RNA sequencing reveals the gene expression profile and the critical signaling pathways of type II CPAM.

Cell &amp; bioscience
2025

Congenital Pulmonary Airway Malformation with Suspected Nontuberculous Mycobacterium Infection.

Internal medicine (Tokyo, Japan)
2024

Surgical Management Of Congenital Thoracic Disorders: A 15-Year Center Experience.

Portuguese journal of cardiac thoracic and vascular surgery
2025

Retrospective evaluation of neonates with fatal congenital lung malformation: A single center 15-year forensic autopsy experience.

Forensic science, medicine, and pathology
2024

A case of bronchopulmonary sequestration combined with congenital pulmonary airway malformation prenatally diagnosed as having two aberrant arteries originating from the celiac artery.

Journal of clinical ultrasound : JCU
2024

Thoracoscopic surgery for rare congenital pulmonary airway malformations in adults: a decade of retrospective study.

Journal of thoracic disease
2024

Defective mesenchymal Bmpr1a-mediated BMP signaling causes congenital pulmonary cysts.

eLife
2025

Needle Fetal Thoracoscopy: A Technique to Assist with Ultrasound-Guided Placement of Challenging Thoracoamniotic Shunts.

Fetal diagnosis and therapy
2024

Comparison of ICG-Guided Near-Infrared Fluorescence Imaging and Modified Inflation-Deflation Method in Identifying the Intersegmental Plane During Lung Segmentectomy of Infants.

Journal of pediatric surgery
2024

Lung Function Evaluated By Structured Light Plethysmography in Children After Lung Surgery: A Preliminary Analysis.

Pediatric allergy, immunology, and pulmonology
2025

The Diagnostics and Management of Bronchopulmonary Sequestration: An International Survey among Specialized Caregivers.

European journal of pediatric surgery : official journal of Austrian Association of Pediatric Surgery ... [et al] = Zeitschrift fur Kinderchirurgie
2024

Complicated Congenital Pulmonary Adenomatoid Malformation Type I following Right Lower Lobectomy: A Case Report.

JNMA; journal of the Nepal Medical Association
2023

[Prenatal presentation of pleuropulmonary blastoma associated to DICER1 syndrome: differential diagnosis of congenital pulmonary malformation].

Andes pediatrica : revista Chilena de pediatria
2024

Prognosis of infants with congenital pulmonary airway malformations after surgery: a short and mid-term evaluation.

American journal of translational research
2024

Surgical Management of Congenital Pulmonary Airway Malformations (CPAM) in an Infant and a Toddler: Case Report Depicting Two Distinct Surgical Techniques With Successful Outcomes.

Cureus
2024

Prenatal features of congenital peribronchial myofibroblastic tumor.

Radiology case reports
2024

Assessment of mediastinal shift angles in congenital pulmonary airway malformation: a new fetal magnetic resonance imaging indicator of congenital lung disease.

Pediatric radiology
2023

Experience of video-assisted thoracic surgery treatment of congenital pulmonary airway malformation in infants less than 3 months of age.

Translational pediatrics
2023

Thoracoscopic Resection of Pulmonary Lesions in Israel: The Mentorship Approach.

Journal of Indian Association of Pediatric Surgeons
2023

A missed foreign body aspiration masquerading as congenital pulmonary airway malformation in a nine-year-old boy: A case report and literature review.

Respiratory medicine case reports
2023

Selection of potential targets for stratifying congenital pulmonary airway malformation patients with molecular imaging: is MUC1 the one?

European respiratory review : an official journal of the European Respiratory Society
2024

Antenatal diagnosis of bronchopulmonary sequestration: A case report and review of the literature.

Radiology case reports
2024

Management and Outcomes of Patients With High-Risk (Congenital Lung Malformation Volume Ratio≥ 1.6) Congenital Lung Malformations.

The Journal of surgical research
2023

Rare presentation of pneumothorax in a young woman with underlying congenital pulmonary airway malformation.

BMJ case reports
2023

Lymphangioleiomyomatosis With Atypical Presentation Following Pneumothorax: A Case Report.

Cureus
2024

The Effect of Steroids on Prenatally Diagnosed Lung Lesions.

Journal of pediatric surgery
2023

Treatment of congenital pulmonary airway malformation with rare high cystic volume ratio: A case report and literature review.

Medicine
2024

No Pathogenic DICER1 Gene Variants in a Cohort Study of 28 Children With Congenital Pulmonary Airway Malformation.

Journal of pediatric surgery
2024

Cytomorphologic and immunocytochemical characterization of pediatric pleuropulmonary blastoma with a comprehensive review of the literature.

Diagnostic cytopathology
2023

Intralobar Pulmonary Sequestration: A Case Report.

Cureus
2024

Near-Infrared Fluorescence Imaging with Intravenous Indocyanine Green Method in Segmentectomy for Infants with Congenital Pulmonary Airway Malformation.

European journal of pediatric surgery : official journal of Austrian Association of Pediatric Surgery ... [et al] = Zeitschrift fur Kinderchirurgie
2023

Infantile type I pleuropulmonary blastoma presenting with dyspnea due to compression by pneumothorax and an occupying tumor: a case report.

Surgical case reports
2024

Pulmonary invasive mucinous adenocarcinoma.

Histopathology
2024

Prenatal Measurements of Congenital Lung Malformations: Can They Predict Postnatal Size?

The Journal of surgical research
2023

Prenatal Array-CGH Detection of 3q26.32q26.33 Interstitial Deletion Encompassing the SOX2 Gene: Ultrasound, Pathological, and Cytogenetic Findings.

Fetal and pediatric pathology
2023

Neonate with congenital pulmonary airway malformation concurrent with enteric duplication cyst: a case report of a rare anomaly.

Journal of surgical case reports
2023

Congenital pulmonary airway malformation complicated by aspergilloma: A rare adulthood presentation - Case report.

Medical mycology case reports
2023

Anesthetic management of scapular Y-osteotomy using a combination of suprascapular nerve block and erector spinae plane block for Sprengel deformity associated with Klippel-Feil syndrome: a case report.

JA clinical reports
2023

Congenital pulmonary airway malformation in a 5 month old boy, complicated by pneumonia.

Indian journal of thoracic and cardiovascular surgery
2024

Uniportal video-assisted thoracoscopic surgery for pediatric cases.

General thoracic and cardiovascular surgery
2023

Multidisciplinary management of a large microcystic congenital pulmonary airway malformation: case report and literature review.

The journal of maternal-fetal &amp; neonatal medicine : the official journal of the European Association of Perinatal Medicine, the Federation of Asia and Oceania Perinatal Societies, the International Society of Perinatal Obstetricians
2023

Serial lung mass volume ratios as prognostic indicators of neonatal respiratory morbidity in fetal pulmonary malformations.

American journal of obstetrics &amp; gynecology MFM
2024

Association of prenatal thoracic ultrasound abnormalities with copy number variants at a single Chinese tertiary center.

International journal of gynaecology and obstetrics: the official organ of the International Federation of Gynaecology and Obstetrics
2023

A tale of two techniques-thoracoscopic and robotic surgery for congenital lung lesions.

Journal of thoracic disease
2023

Congenital pulmonary airway malformation (CPAM): A case report and review of the literature.

Radiology case reports
2023

Combined Pre- and Postnatal Minimally Invasive Approach to a Complex Symptomatic Congenital Pulmonary Airway Malformation.

European journal of pediatric surgery reports
2023

Type III congenital pulmonary airway malformation associated with oesophageal atresia and tracheoesophageal fistula. A case report and review of literature.

African journal of paediatric surgery : AJPS
2023

A pleuropulmonary blastoma type III in a 4-month-old infant: First case report in Syria.

International journal of surgery case reports
2023

Congenital pulmonary airway malformation (CPAM) presenting as spontaneous pneumothorax in young boy.

Lung India : official organ of Indian Chest Society
2023

Congenital pulmonary airway malformation manifesting with severe respiratory distress and pneumothorax in a neonate.

Indian journal of thoracic and cardiovascular surgery
2023

Congenital Pulmonary Airway Malformations With a Reconsideration and Current Perspective on the Stocker Classification.

Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society
2023

Intradiaphragmatic pulmonary sequestrations: a surgical challenge. Case series.

Frontiers in surgery
2023

Congenital pulmonary airway malformation.

The American journal of the medical sciences
2023

Uniportal video-assisted thoracoscopic anatomical resection of the right anterior pulmonary segment in a 10-year-old child with congenital pulmonary airway malformation.

Journal of cardiothoracic surgery
2023

The upper airway parameters: the potential diagnostic clues for congenital intrathoracic lesions.

BMC pregnancy and childbirth
2025

Thoracoscopic lobectomy for congenital pulmonary airway malformations before or after 5 months of age: evaluation of pulmonary function.

Minerva pediatrics
2023

Stapleless thoracoscopic lobectomy for congenital pulmonary airway malformation: a simple technique in thoracoscopic surgery.

Journal of surgical case reports
2023

Early resection of a rare congenital pulmonary airway malformation causing severe progressive respiratory distress in a preterm neonate: a case report and review of the literature.

BMC pediatrics
2023

Prenatal ultrasound, magnetic resonance imaging and therapeutic options for fetal thoracic anomalies: a pictorial essay.

Pediatric radiology
2023

Integrated bulk and single-cell RNA-sequencing reveals SPOCK2 as a novel biomarker gene in the development of congenital pulmonary airway malformation.

Respiratory research
2023

Transthoracic puncture for asymptomatic congenital pulmonary airway malformation during anesthesia.

Pediatric pulmonology
2023

Single-direction thoracoscopic lobectomy for incomplete pulmonary fissure in children with congenital pulmonary airway malformation: initial experience and propensity score-matched analysis.

Pediatric surgery international
2023

Neonatal COVID-19 with Atypical Lung Cystic Lesions Mimicking Congenital Pulmonary Airway Malformation - A Diagnostic Dilemma.

International medical case reports journal
2023

Learning curve for total thoracoscopic segmentectomy in treating pediatric patients with congenital lung malformation.

Surgical endoscopy
2023

COllaborative Neonatal Network for the first European CPAM Trial (CONNECT): a study protocol for a randomised controlled trial.

BMJ open
2023

Congenital pleuropulmonary blastoma in a newborn with a variant of uncertain significance in DICER1 evaluated by RNA-sequencing.

Maternal health, neonatology and perinatology
2023

Challenges in DICER1-Associated Lung Disease.

Journal of clinical medicine
2023

The Role of Bone Morphogenetic Protein 4 in Lung Diseases.

Current molecular medicine
2023

The Role of the miR-548au-3p/CA12 Axis in Tracheal Chondrogenesis in Congenital Pulmonary Airway Malformations.

Oxidative medicine and cellular longevity
2022

A case report of misdiagnosed fetal lung mass and review of the literature.

Frontiers in pediatrics
2023

An unusual site of a pericardial cyst in a child: A case report.

Radiology case reports
2023

Morbidity and Mortality in Neonates with Symptomatic Congenital Lung Malformation.

Journal of the American College of Surgeons
2023

Clinical Symptoms Affect Treatment and Prognosis in Pediatric Patients with Congenital Pulmonary Airway Malformation: A Propensity Score Matching Retrospective Cohort Study.

Journal of pediatric surgery
2023

Can we predict the need for postnatal surgery in patients with prenatal fetal lung masses detected by CVR value?

Journal of gynecology obstetrics and human reproduction
2022

Congenital Lung Malformations: Clinical and Functional Respiratory Outcomes after Surgery.

Children (Basel, Switzerland)
2022

Congenital Cystic Adenomatoid Malformation (CCAM) Type II: A Rare Case of Sudden Infant Death.

Children (Basel, Switzerland)
2022

Post Congenital Pulmonary Airway Malformation Excision Thymic Growth: An Unusual Postoperative Sequel.

Journal of Indian Association of Pediatric Surgeons
2022

Perinatal outcomes of prenatal diagnosis of congenital pulmonary airway malformation: an experience.

Revista da Associacao Medica Brasileira (1992)
2022

Application of second-generation sequencing in congenital pulmonary airway malformations.

Scientific reports
2022

Congenital lung malformations: Dysregulated lung developmental processes and altered signaling pathways.

Seminars in pediatric surgery
2022

Robotic approach for pediatric pulmonary resection: preliminary investigation and comparative study with thoracoscopic approach.

Journal of thoracic disease
2023

The long-term outcome following thoraco-amniotic shunting for congenital lung malformations.

Journal of pediatric surgery
2022

SOX2 and SOX21 in Lung Epithelial Differentiation and Repair.

International journal of molecular sciences
2022

Management of Congenital Lung Malformations.

Clinics in perinatology
2022

Bilateral congenital pulmonary airway malformation presenting in adulthood with a review of the literature.

Medical journal, Armed Forces India
2022

Thoracoscopic Clockwise Lobectomy May Be a Stylized Procedure for Treating Children with Congenital Lung Malformations.

Journal of laparoendoscopic &amp; advanced surgical techniques. Part A
2023

Prenatal ultrasound-and MRI-based imaging predictors of respiratory symptoms at birth for congenital lung malformations.

Journal of pediatric surgery
2022

A rare case of congenital pulmonary airway malformation presenting with chest pain and dyspnoea in an adult.

British journal of hospital medicine (London, England : 2005)
2023

Type II congenital pulmonary airway malformation with primary ciliary dyskinesia in a 4-year-old child: A case report.

Pediatric pulmonology
2022

A novel Mecom gene mutation associated with amegakaryocytic thrombocytopenia in a premature infant.

The Turkish journal of pediatrics
2022

Modified thoracoscopic wedge resection of limited peripheral lesions in S10 for children with congenital pulmonary airway malformation: Initial single-center experience.

Frontiers in pediatrics
2022

Maternal Steroids in High-Risk Congenital Lung Malformations.

The Journal of surgical research
2022

The Management of Asymptomatic Congenital Pulmonary Airway Malformation: Results of a European Delphi Survey.

Children (Basel, Switzerland)
2022

Fetal imaging of congenital lung lesions with postnatal correlation.

Pediatric radiology
Ver todos os 253 no EuropePMC

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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. Loss of CA12 drive proliferation and fibrosis in type i congenital pulmonary airway malformation via promoting abnormal activation of SHH signaling.
    Cell communication and signaling : CCS· 2026· PMID 41851891mais citado
  2. Anesthesia for thoracic surgery in infants with congenital lung malformations.
    World journal of pediatric surgery· 2026· PMID 41853389mais citado
  3. Pulmonary Malignancies in Adults With Congenital Lung Malformations: A Scoping Review.
    JTO clinical and research reports· 2026· PMID 41852937mais citado
  4. Non-Intubated Spontaneous Ventilation Versus Endotracheal Intubation Anesthesia for Pediatric Thoracoscopic Lung Resection: A Retrospective Propensity-Score-Matched Study.
    Paediatric anaesthesia· 2026· PMID 41823064mais citado
  5. Antenatal Diagnosis and Management of Congenital Pulmonary Airway Malformation: A Case Report.
    Cureus· 2026· PMID 41798503mais citado
  6. Delayed surgical management of extensive bilateral multilobar congenital pulmonary airway malformation.
    BMJ Case Rep· 2026· PMID 41986066recente
  7. Congenital pulmonary airway malformation or pulmonary sequestration with ipsilateral foregut cyst-inevitable association or coincidence?
    J Pediatr Surg· 2026· PMID 41933808recente
  8. Diagnostic Challenges in a Hybrid Congenital Pulmonary Airway Malformation: A Case Report.
    Cureus· 2026· PMID 41909389recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:2444(Orphanet)
  2. MONDO:0016580(MONDO)
  3. GARD:18772(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. Artigo Wikipedia(Wikipedia)
  6. Q246040(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

Malformação das vias aéreas pulmonares congênita
Compêndio · Raras BR

Malformação das vias aéreas pulmonares congênita

ORPHA:2444 · MONDO:0016580
Prevalência
Unknown
Herança
Not applicable
CID-10
Q33.0 · Pulmão cístico congênito
CID-11
Ensaios
2 ativos
Início
Adolescent, Adult, Antenatal, Neonatal
Prevalência
0.0 (Europe)
MedGen
UMLS
C0010668
EuropePMC
Wikidata
Wikipedia
Papers 10a
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