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Malformação congênita das vias aéreas pulmonares, tipo 4
ORPHA:280854CID-10 · Q33.0CID-11 · LA75.4DOENÇA RARA

Em audiologia, perda auditiva periférica é um déficit auditivo de etiologia multifatorial que pode sofrer influência de fatores ambientais ou genéticos, que se manifesta em duas classificações principais, perda condutiva e perda neurosensorial, que tem como origem porções específicas do sistema audiorreceptor. Além disso, atualmente também adota-se a caracterização para a perda auditiva mista, perda auditiva funcional e por deficiência auditiva central.

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Introdução

O que você precisa saber de cara

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Malformação congênita rara das vias aéreas pulmonares, tipo 4, caracterizada por cistos múltiplos e difusos, frequentemente associados a anomalias vasculares e prognóstico reservado. Pode apresentar-se com dificuldade respiratória neonatal ou ser assintomática.

Publicações científicas
615 artigos
Último publicado: 2026 Apr
🏥
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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Anos de pesquisa1desde 2026
Total histórico615PubMed
Últimos 10 anos79publicações
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Genética e causas

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Onde tratar no SUS

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

🇧🇷 Atendimento SUS — Malformação congênita das vias aéreas pulmonares, tipo 4

Centros de Referência SUS

24 centros habilitados pelo SUS para Malformação congênita das vias aéreas pulmonares, tipo 4

Centros para Malformação congênita das vias aéreas pulmonares, tipo 4

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.

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Publicações mais relevantes

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Timeline de publicações
459 papers (10 anos)

Mostrando amostra de 79 publicações de um total de 459

#1

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.

#2

Challenges with Congenital Lung Cysts: When to Consider DICER1 Testing? A Narrative Review.

Acta medica portuguesa2026 Feb 20

Congenital pulmonary airway malformations (CPAMs), according to the Stocker classification, comprise five types, two of which are non-cystic in nature (types 0 and 3) whereas the others present as cystic lung lesions (types 1, 2 and 4). While there is consensus that symptomatic lesions should be managed surgically, the asymptomatic cases are more problematic in terms of therapeutic intervention. The dilemma is further complicated by CPAM types 1 and 4 and their preneoplastic potential. In the case of CPAM type 1, there are reports of lipidic adenocarcinoma arising from the mucinous component. Type 4 has been equated to type I or cystic pleuropulmonary blastoma (PPB), a proposition that has been challenged in the past. Pleuropulmonary blastoma is associated with a heterozygous germline or somatic variants in DICER1. It was recognized that the earliest stage of the sarcomatous progression of PPB was a multicystic lesion in the periphery of the lung whose architectural features were identical to CPAM type 4. This narrative review addresses key aspects of the pathogenetic and diagnostic issues of type I PPB/CPAM type 4, as well as the association of DICER1 and PPB, offering valuable insights for pediatricians and clinicians caring for young adults who are impacted by the presence of a germline DICER1 variant.

#3

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

Paediatric anaesthesia2026 Feb 11

Procedures involving lung surgery in the pediatric population are relatively uncommon and tend to be centralized in a limited number of institutions. Anesthesia literature is also sparse. To have a clear overview of frequency, underlying pathologies, ICU and hospital stay, anesthetic techniques, one lung ventilation, and perioperative analgesia. We conducted a retrospective review in a single-center tertiary hospital, from January 2014 to 2023. We included children aged 0-16 years who underwent major lung surgery and received anesthesia managed by the pediatric anesthesia team. Patients with congenital diaphragmatic hernia, esophageal atresia, or those undergoing surgery for pectus excavatum were excluded. Our main outcome measures include the type of underlying pathology and surgical procedure, ICU and hospital stay, methods of one-lung ventilation, source of perioperative analgesia, and the incidence of (postoperative) complications. We included 73 patients, 55% male and 45% female. The median age was 2.8 years and the median weight was 12.9 kg. Congenital pulmonary airway malformation was diagnosed in 43%, and 45% underwent a (partial) lobectomy. The proportion of video-assisted thoracoscopic surgery was comparable to that of open thoracotomy. One-lung ventilation (OLV) was used in 81%, primarily facilitated by a bronchial blocker. Epidural catheterization with ropivacaine for perioperative pain management was used in 71%. The proportion of patients receiving intravenous morphine on postoperative Days 1, 2, 3, 4, and 5 was 40%, 34%, 19%, 15%, and 11%, respectively. Insufficient pain control was reported in 14%. 70% were admitted to the ICU for one night. The average length of hospital stay was 8 days. We addressed the anesthetic care of pediatric lung surgery procedures. OLV was required in the majority of the population and a bronchial blocker was the preferred method. Epidural analgesia was the preferred choice to tackle perioperative pain.

#4

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

Archivos de bronconeumologia2026 Feb

Congenital lung malformations (CLMs) encompass a range of developmental anomalies, including congenital pulmonary airway malformation (CPAM), bronchopulmonary sequestration (BPS), congenital lobar overinflation (CLO), bronchogenic cysts (BC), and bronchial atresia (BA). These lesions present with varying severity, from incidental findings to severe neonatal respiratory distress. Advances in prenatal imaging, such as ultrasound and fetal magnetic resonance image (MRI), have significantly improved early detection, aiding in better management planning. CLMs arise from abnormalities during specific stages of lung development. CPAMs are categorized by cyst size and histological type, while BPS is characterized by systemic arterial supply. CLO and BA are associated with air trapping and hyperinflation, and BCs are typically fluid-filled, well defined, and compressive. Postnatal diagnosis is based on high-resolution computed tomography (CT) and occasionally MRI for detailed evaluation. Surgical resection is recommended for symptomatic lesions, while management of asymptomatic cases remains debated. Elective surgery may help prevent complications like infection or rare malignancy, particularly in CPAM Types 1 and 4 and BCs. However, some lesions remain stable or regress, supporting conservative management in selected cases. Long-term outcomes are generally positive, with children undergoing early resection often maintaining good lung function due to compensatory lung growth, though subtle functional deficits may persist. Risk stratification using imaging and genetic markers, such as DICER1 mutations, is gaining importance for guiding treatment decisions. Management should be individualized, involving a multidisciplinary approach and shared decision-making with families. Further research is needed to clarify the natural history of CLMs, optimize the timing of interventions, and standardize long-term surveillance strategies.

#5

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

Indian journal of pediatrics2025 Dec

Congenital pulmonary airway malformation (CPAM) lesion with an aberrant systemic blood supply is considered a "hybrid" lesion (HL). The aim of the study was to report authors' experience in the presentation, surgical management, and outcomes of HL. A retrospective cohort study of HL patients followed from 2000 to 2022 in a single institution was done. Clinical and radiographic data were collected from the medical records. Over the study period, 39 patients with HL were identified. HL with CPAM type 2 was more common than type 1 (74% vs. 26%), and intralobar sequestrations were more prevalent than extralobar sequestrations (ELS) (79.5% vs. 20.5%). HL with CPAM type 2 had higher rates of co-morbidities and higher likelihood to present with respiratory symptoms after birth. HL with ELS had smaller feeding vessels (all < 4 mm) which resulted in lower rates of open thoracotomy approach for resection. The variability in HL patient presentation and outcomes is correlated with the size and the hybrid composition.

Publicações recentes

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📚 EuropePMC253 artigos no totalmostrando 79

2026

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

JTO clinical and research reports
2026

Challenges with Congenital Lung Cysts: When to Consider DICER1 Testing? A Narrative Review.

Acta medica portuguesa
2026

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

Paediatric anaesthesia
2025

Laser therapy for type III fetal congenital cystic adenomatoid malformation.

Ceska gynekologie
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

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

The Indian journal of radiology &amp; imaging
2025

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

Journal of comparative pathology
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

Congenital Pulmonary Airway Malformations: Experience of a Tunisian Tertiary Referral Center Over a Five-Year Period.

Pediatric pulmonology
2025

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

Surgical case reports
2025

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

Anales de pediatria
2025

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

Scientific reports
2025

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

Journal of pediatric surgery
2025

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

Indian journal of pediatrics
2024

[Clinical and morphological characteristics of lung sequestration].

Arkhiv patologii
2024

The ten-year evaluation of clinical characteristics in congenital lung anomaly in pediatrics; a retrospective study in North of Iran.

BMC pediatrics
2024

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

JNMA; journal of the Nepal Medical Association
2024

[Clinicopathological and genetic characteristics of congenital cystic adenomatoid malformation of lung and its associated lung cancer in adults].

Zhonghua bing li xue za zhi = Chinese journal of pathology
2023

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

Translational pediatrics
2024

The Effect of Steroids on Prenatally Diagnosed Lung Lesions.

Journal of pediatric surgery
2023

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

Surgical case reports
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

Congenital pulmonary malformations in children in a pediatric hospital in Peru, 2010-2020.

Boletin medico del Hospital Infantil de Mexico
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 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

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

Maternal health, neonatology and perinatology
2023

The Role of Bone Morphogenetic Protein 4 in Lung Diseases.

Current molecular medicine
2023

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

Journal of pediatric surgery
2023

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

Pediatric pulmonology
2022

Congenital cystic adenomatoid malformations of the lung: a retrospective study of diagnosis, treatment strategy and postoperative morbidity in surgically treated patients.

European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery
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
2023

Thoracoscopic segmentectomy for congenital pulmonary airway malformation arising in a lung with an accessory fissure: A case report.

Asian journal of endoscopic surgery
2022

Vascular Anatomy in Congenital Lung Lesions-Description and Classification.

Frontiers in pediatrics
2021

Atypical Antenatal Presentation of an Unusual Nonmucinous Papillary Variant of Giant Congenital Pulmonary Airway Malformation Masquerading as Congenital Diaphragmatic Hernia with Volvulus.

Journal of medical ultrasound
2022

18FDG PET-Positive Congenital Pulmonary Airway Malformation Mimicking Lung Cancer.

Clinical nuclear medicine
2022

Neonatal congenital pulmonary airway malformation associated with mucinous adenocarcinoma and KRAS mutations.

Journal of pediatric surgery
2021

Congenital Pulmonary Airway Malformation - A Histomorphological Spectrum of 15 Cases: A 5-Year Study from a Tertiary Care Center.

Journal of Indian Association of Pediatric Surgeons
2021

Congenital pulmonary airway malformation (cpam) mimicking an spontaneous pneumothorax in a newborn.

Cirugia pediatrica : organo oficial de la Sociedad Espanola de Cirugia Pediatrica
2021

An adult with episodic retrosternal chest pain: an unusual presentation of congenital pulmonary airway malformation - case report.

Journal of cardiothoracic surgery
2021

Mucinous Adenocarcinoma With Intrapulmonary Metastasis Harboring KRAS and GNAS Mutations Arising in Congenital Pulmonary Airway Malformation.

American journal of clinical pathology
2020

Single-cell transcriptome profiling reveals the mechanism of abnormal proliferation of epithelial cells in congenital cystic adenomatoid malformation.

Experimental cell research
2021

Mucinous adenocarcinoma arising in congenital pulmonary airway malformation: clinicopathological analysis of 37 cases.

Histopathology
2020

Thoracoscopic Resection in the Treatment of Spontaneous Pneumothorax.

Sisli Etfal Hastanesi tip bulteni
2020

Pleuropulmonary blastoma type I and congenital pulmonary airway malformation type 4: distinct entities or sides of the same coin?

Virchows Archiv : an international journal of pathology
2020

Computed tomography features can distinguish type 4 congenital pulmonary airway malformation from other cystic congenital pulmonary airway malformations.

European journal of radiology
2020

Pleuropulmonary blastoma type I might arise in congenital pulmonary airway malformation type 4 by acquiring a Dicer 1 mutation.

Virchows Archiv : an international journal of pathology
2020

Analysis of Wnt7B and BMP4 expression patterns in congenital pulmonary airway malformation.

Pediatric pulmonology
2019

Congenital Cystic Adenomatoid Malformation of the Lung Tipe II: Three Cases Report.

Translational medicine @ UniSa
2021

Obstetric and neonatal outcomes in pregnancies complicated by fetal lung masses: does final histology matter?

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
2019

Type I pleuropulmonary blastoma presenting as congenital pulmonary airway malformation: A report of two cases.

Indian journal of pathology &amp; microbiology
2020

Value of routine ultrasound examination at 35-37 weeks' gestation in diagnosis of fetal abnormalities.

Ultrasound in obstetrics &amp; gynecology : the official journal of the International Society of Ultrasound in Obstetrics and Gynecology
2019

Congenital Cystic Adenomatoid Malformation Volume Ratio in Prenatal Assessment of Prognosis of Fetal Pulmonary Sequestrations.

Current medical science
2019

Esophageal duplication cyst presenting with stridor in a child with congenital pulmonary airway malformation: A case report and literature review.

Medicine
2019

Cystic and pseudocystic pulmonary malformations in children: Clinico-pathological correlation.

Annals of diagnostic pathology
2019

Early vs late resection of asymptomatic congenital lung malformations.

Journal of pediatric surgery
2018

Fetal Congenital Peripheral Bronchial Atresia Diagnosed by Magnetic Resonance Imaging: Two Case Reports.

AJP reports
2018

An investigation on clinical differences between congenital pulmonary airway malformation and bronchial atresia.

Journal of pediatric surgery
2018

[Pulmonary lobectomy in children: the sooner the better?].

Cirugia pediatrica : organo oficial de la Sociedad Espanola de Cirugia Pediatrica
2018

Congenital cystic adenomatoid malformation - diagnostic and therapeutic procedure: 8-year experience of one medical centre.

Kardiochirurgia i torakochirurgia polska = Polish journal of cardio-thoracic surgery
2018

Types II and III congenital pulmonary airway malformation with hydrops treated in utero with percutaneous sclerotherapy.

Prenatal diagnosis
2018

[Pulmonary sequestration associated with congenital pulmonary airway malformation].

Boletin medico del Hospital Infantil de Mexico
2017

Congenital pulmonary airway malformations: state-of-the-art review for pediatrician's use.

European journal of pediatrics
2017

An Atypical Presentation of Congenital Pulmonary Airway Malformation (CPAM): A Rare Case with Antenatal Ultrasound Findings and Review of Literature.

Polish journal of radiology
2016

Congenital Cystic Adenomatoid Malformation: A Tertiary Care Hospital Experience.

Journal of clinical and diagnostic research : JCDR
2016

Association between Congenital Lung Malformations and Lung Tumors in Children and Adults: A Systematic Review.

Journal of thoracic oncology : official publication of the International Association for the Study of Lung Cancer
2016

Pulmonary Kirsten Rat Sarcoma Virus Mutation Positive Mucinous Adenocarcinoma Arising in a Congenital Pulmonary Airway Malformation, Mixed Type 1 and 2.

The Annals of thoracic surgery
2016

Outcome of infants operated on for congenital pulmonary malformations.

Pediatric pulmonology
2016

Fetal MRI as Complementary Study of Congenital Cystic Adenomatoid Malformation During Pregnancy: A Single Case Report.

Cureus
2016

Pulmonary Arteriovenous Fistula: Clinical and Histologic Spectrum of Four Cases.

Journal of pathology and translational medicine
2016

Retrospective Evaluation of Children with Congenital Pulmonary Airway Malformation: A Single Center Experience of 20 Years.

Fetal and pediatric pathology
2016

Nasoethmoidal meningocele in a child presenting bilateral congenital cystic adenomatoid malformation: Evidence for a new entity or consequence of gestational exposures?

Birth defects research. Part A, Clinical and molecular teratology
2015

[Pediatric lung lesions: a clinicopathological study of 215 cases].

Zhonghua bing li xue za zhi = Chinese journal of pathology
2016

Can congenital pulmonary airway malformation be distinguished from Type I pleuropulmonary blastoma based on clinical and radiological features?

Journal of pediatric surgery
2015

Congenital Pulmonary Airway Malformation in an Adult Male: A Case Report with Literature Review.

Case reports in pulmonology
2015

Extralobar sequestration of lung associated with congenital diaphragmatic hernia and malrotation of gut.

Lung India : official organ of Indian Chest Society
2015

[Congenital pulmonary airway malformation of lung in fetus: a clinicopathological analysis].

Zhonghua bing li xue za zhi = Chinese journal of pathology
2015

Surgical management of bronchopulmonary malformations.

The Journal of surgical research
Ver todos os 253 no EuropePMC

Associações

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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Doenças relacionadas

Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico

Ainda não achamos doenças com sintomas parecidos o suficiente.

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. Pulmonary Malignancies in Adults With Congenital Lung Malformations: A Scoping Review.
    JTO clinical and research reports· 2026· PMID 41852937mais citado
  2. Challenges with Congenital Lung Cysts: When to Consider DICER1 Testing? A Narrative Review.
    Acta medica portuguesa· 2026· PMID 41719532mais citado
  3. Perioperative Care for Pediatric Patients Undergoing Lung Surgery: Retrospective Single Center Review.
    Paediatric anaesthesia· 2026· PMID 41670069mais citado
  4. Congenital Lung Malformations: A Comprehensive Overview of Current Knowledge-Narrative Review.
    Archivos de bronconeumologia· 2026· PMID 41109832mais citado
  5. Congenital Pulmonary Hybrid Lesions: Clinical Presentation, Surgical Management, and Outcomes.
    Indian journal of pediatrics· 2025· PMID 39833625mais citado
  6. Rising detection of congenital pulmonary airway malformations and a pragmatic institutional algorithm: a four-patient thoracoscopic case series.
    J Surg Case Rep· 2026· PMID 41993110recente
  7. Delayed surgical management of extensive bilateral multilobar congenital pulmonary airway malformation.
    BMJ Case Rep· 2026· PMID 41986066recente
  8. Lobectomy vs. sublobar resection for congenital pulmonary airway malformations: a 10-year NSQIP-pediatric review.
    Pediatr Surg Int· 2026· PMID 41945161recente
  9. Congenital pulmonary airway malformation or pulmonary sequestration with ipsilateral foregut cyst-inevitable association or coincidence?
    J Pediatr Surg· 2026· PMID 41933808recente
  10. 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:280854(Orphanet)
  2. MONDO:0017252(MONDO)
  3. GARD:21097(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. Q55786940(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 congênita das vias aéreas pulmonares, tipo 4
Compêndio · Raras BR

Malformação congênita das vias aéreas pulmonares, tipo 4

ORPHA:280854 · MONDO:0017252
CID-10
Q33.0 · Pulmão cístico congênito
CID-11
Início
Antenatal, Neonatal
MedGen
UMLS
C5437760
EuropePMC
Wikidata
Papers 10a
Evidência
🥉 Relato de caso
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