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1.
Zhonghua Er Ke Za Zhi ; 58(1): 19-24, 2020 Jan 02.
Artículo en Chino | MEDLINE | ID: mdl-31905471

RESUMEN

Objective: To summarize the diagnosis, clinical manifestations, treatment and prognosis of congenital cystic lung lesions. Methods: A retrospective study described the clinical course of 96 patients (46 female and 50 male) diagnosed with congenital cystic lung lesions treated at the Tianjin Children's Hospital from January 2010 to March 2019. The clinical findings, imaging examinations, pathological findings, treatment and follow-up were analyzed. Results: Totally 96 patients (aged from 4 days to 13 years) with congenital cystic lung lesions were included in this study. Eighty-six patients (90%) were diagnosed when they had cough and fever symptoms. Forty (42%) patients exhibited congenital cystic adenomatoid malformation, 30 underwent surgical excision, two were at emergency operations and one dead. There were 12 (13%) patients with pulmonary sequestration and four were surgical treated. Twelve (13%) patients with bronchogenic cyst were included and 4 were surgically treated. There were 3 (3%) patients with congenital lobar emphysema and one was surgically treated. Another patient with pneumothorax was operated in other hospital 2 months after discharge. Twenty-nine (30%) patients with unclassified congenital cystic lung lesions could not be definitively diagnosed by CT. Some of them were difficult to be distinguished from necrotizing pneumonia. Finally, 2 patients were diagnosed as necrotizing pneumonia after 6, 10 months follow-up. After operation 37 out of 39 patients recovered well. Conclusions: The diagnosis of congenital pulmonary cystic disease depend on imaging and pathological examination. Most patients are diagnosed when they have respiratory tract infection. The main clinical manifestations are cough and fever. The prognosis of operative management is good.


Asunto(s)
Quiste Broncogénico/diagnóstico , Quiste Broncogénico/cirugía , Malformación Adenomatoide Quística Congénita del Pulmón/diagnóstico , Enfisema Pulmonar/patología , Adolescente , Quiste Broncogénico/congénito , Secuestro Broncopulmonar/diagnóstico , Secuestro Broncopulmonar/cirugía , Niño , Preescolar , Malformación Adenomatoide Quística Congénita del Pulmón/cirugía , Femenino , Humanos , Lactante , Masculino , Neumonectomía , Enfisema Pulmonar/cirugía , Estudios Retrospectivos , Resultado del Tratamiento
3.
Tuberk Toraks ; 67(1): 71-76, 2019 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-31130138

RESUMEN

Congenital pulmonary airway malformation (CPAM), is a rare disease known as a developmental abnormality of the lower respiratory tract. It may occur in the neonatal period due to respiratory distress and patients usually die in the first few months of life. Rarely, it may remain asymptomatic until adulthood. In this study we describe an adult case and present a review of the literature. A 19-year-old male with no relevant medical history was admitted to our clinic with cough and wheeze. The patient had a history of frequent lower respiratory tract infection during childhood. Chest radiograph revealed a diffuse opacity and volume loss in the right hemithorax. High resolution tomography showed hypoplasia of the right hemithorax, multiple cysts in all of the lobes and segments on the right side, ground glass opacity and interlobular septal thickening of the whole right lung parenchyma. Right pneumonectomy was performed with the pre-diagnosis of congenital pulmonary airway malformation and the pathological examination was compatible with CPAM. CPAM is a rare disease in adulthood. We should consider CPAM in the differential diagnosis of patients with frequent recurrent pulmonary infection and cystic lung lesions. In order to prevent infections and to eliminate the risk of malignancy, surgical treatment should be applied for definite diagnosis and treatment.


Asunto(s)
Broncoscopía/métodos , Malformación Adenomatoide Quística Congénita del Pulmón/diagnóstico , Pulmón/diagnóstico por imagen , Neumonectomía/métodos , Enfermedades Raras , Tomografía Computarizada por Rayos X/métodos , Malformación Adenomatoide Quística Congénita del Pulmón/cirugía , Diagnóstico Diferencial , Humanos , Masculino , Adulto Joven
4.
Interact Cardiovasc Thorac Surg ; 29(1): 154-156, 2019 07 01.
Artículo en Inglés | MEDLINE | ID: mdl-30844060

RESUMEN

This is a case of type 1 congenital pulmonary airway malformation in the left lower lobe of a 26-year-old male. At the age of 1 year, he developed a pulmonary cystic lesion, which was considered to be bronchopulmonary sequestration. He grew up healthy and showed no impairment during exercise; however, giant bullous lesion development along with compressed left upper lobe and mediastinum was recently noted; consequently, the patient was referred to our hospital for further examination. We diagnosed congenital pulmonary airway malformation and performed left lower lobectomy. Postoperative course was uneventful, but a restrictive change on pulmonary function test did not improve. This unusual course of congenital pulmonary airway malformation with bullous changes suggests the importance of early-stage resection.


Asunto(s)
Malformación Adenomatoide Quística Congénita del Pulmón/diagnóstico , Pulmón/diagnóstico por imagen , Neumonectomía/métodos , Adulto , Malformación Adenomatoide Quística Congénita del Pulmón/cirugía , Humanos , Pulmón/cirugía , Masculino , Tomografía Computarizada por Rayos X
6.
Ann Diagn Pathol ; 39: 78-85, 2019 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-30798075

RESUMEN

Pulmonary malformations are rare disorders, with cystic and pseudocystic pulmonary malformations (CPPM) the most frequent, and constitute the first cause of lobectomy in children <1 year of age. Morphological overlap of congenital cystic pulmonary lesions might correspond to a spectrum of lesions in which bronchial atresia is a common etiopathogenetic mechanism. We aimed to report the frequency of CPPM resected in a tertiary-level hospital and to evaluate the degree of agreement between presurgical and anatomopathological diagnoses. We studied 44 surgical pieces with a diagnosis of CPPM received at the Pathology Service from 2009 to 2014, resected from 39 patients, 51.3 % males, with a median age of 16.8 months. Up to 69.2% of the patients had adenomatoid malformation of pulmonary airway (AMPA), with type 2 the most frequent (55.5%). Pulmonary sequestration was present in 15.4% of patients; in two cases the diagnosis was an incidental finding during surgery for the repair of a diaphragmatic hernia. Congenital lobar hyperinflation (CLH) occurred in 7.6% cases. Bronchogenic cyst (BC) was present in 7.6% cases. Presurgical and anatomopathological diagnoses in all patients coincided in 71.8% of cases. Kappa coefficient was 0.56 for global concordance in patients with AMPA, and 0.72, 0.64, 0.37 and 0.33 for CLH, BC, and types 1 and 2 AMPA, respectively. This relatively low interobserver agreement could reflect the low reproducibility of diagnoses used in the current nomenclature. Thus, the new nomenclature must be promoted in order to allow for better reproducibility and greater clinico-pathological concordance. The anatomopathological analysis must include the intentional search for bronchial atresia.


Asunto(s)
Procedimientos Quirúrgicos Pulmonares/métodos , Anomalías del Sistema Respiratorio/diagnóstico , Anomalías del Sistema Respiratorio/cirugía , Adolescente , Quiste Broncogénico/diagnóstico , Quiste Broncogénico/patología , Quiste Broncogénico/cirugía , Secuestro Broncopulmonar/diagnóstico , Secuestro Broncopulmonar/patología , Secuestro Broncopulmonar/cirugía , Niño , Preescolar , Estudios Transversales , Malformación Adenomatoide Quística Congénita del Pulmón/diagnóstico , Malformación Adenomatoide Quística Congénita del Pulmón/patología , Malformación Adenomatoide Quística Congénita del Pulmón/cirugía , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Variaciones Dependientes del Observador , Enfisema Pulmonar/congénito , Enfisema Pulmonar/diagnóstico , Enfisema Pulmonar/patología , Enfisema Pulmonar/cirugía , Anomalías del Sistema Respiratorio/patología , Estudios Retrospectivos , Centros de Atención Terciaria
7.
J Matern Fetal Neonatal Med ; 32(21): 3531-3536, 2019 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-29720002

RESUMEN

Objectives: To evaluate and describe the spectrum and rate of congenital thoracic malformations (CTMs) diagnosed by early prenatal sonography (gestational age (GA) less than 16 weeks). Methods: A retrospective, cross-sectional analysis of prenatal ultrasound screening tests in a community-based clinic. Results: In 2001-2017, 31 261 prenatal ultrasound tests detected 31 CTMs at a gestational age of 15.2 (range, 11.6-16.0) weeks. The most common malformation was congenital pleural effusion (CPE) (15 fetuses, 0.48/1000), followed by congenital diaphragmatic hernia (CDH) (10 fetuses, 0.32/1000). Pulmonary hypoplasia (PH), congenital pulmonary airway malformation and broncho-pulmonary sequestration appeared in much smaller proportions (three, two and one fetuses, respectively). Most CTMs were associated with additional fetal lesions (15 fetuses, 48%). All early CDH (10 fetuses) and PH (three fetuses) and 6/15 with CPE had termination of pregnancy or missed abortions. Conclusions: Prenatal ultrasound before 16 GA was able to detect CTMs in 0.99/1000 of screening ultrasound (US) performed. Most CTMs tended to appear with multiple lesions and were associated with unfavorable outcomes. Earlier prenatal diagnosis may enable early termination of pregnancy in fetuses with lethal malformations.


Asunto(s)
Edad Gestacional , Anomalías del Sistema Respiratorio/diagnóstico , Anomalías del Sistema Respiratorio/epidemiología , Ultrasonografía Prenatal , Estudios Transversales , Malformación Adenomatoide Quística Congénita del Pulmón/diagnóstico , Malformación Adenomatoide Quística Congénita del Pulmón/epidemiología , Diagnóstico Precoz , Femenino , Hernias Diafragmáticas Congénitas/diagnóstico , Hernias Diafragmáticas Congénitas/epidemiología , Humanos , Derrame Pleural/congénito , Derrame Pleural/diagnóstico , Derrame Pleural/epidemiología , Valor Predictivo de las Pruebas , Embarazo , Estudios Retrospectivos , Ultrasonografía Prenatal/estadística & datos numéricos
8.
Fetal Pediatr Pathol ; 37(5): 377-386, 2018 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-30358469

RESUMEN

BACKGROUND: Pleuropulmonary blastoma (PPB) is a childhood malignancy known to be associated with congenital pulmonary airway malformation (CPAM). CASE REPORT: An 18 months boy presented with respiratory distress. Computed tomography (CT) scans revealed a large right-sided lung mass. Fine needle aspiration cytology (FNAC) showed sheets and clusters of small round to oval cells with scanty cytoplasm. The possibility of PPB was suggested. Trucut biopsy from the mass confirmed the diagnosis of PPB, of at least type II. The child had earlier been diagnosed as CPAM for which he had undergone lobectomy at six months, which on review was diagnosed as PPB I. CONCLUSION: We describe the cytological and histological findings of a case of PPBII/III evolving from a PPB I originally thought to be a CPAM type IV. This supports the theory that PPB I may progress to a more aggressive type II with time, and highlights the importance of the adequately treating the PPB I to prevent this transformation.


Asunto(s)
Blastoma Pulmonar/patología , Malformación Adenomatoide Quística Congénita del Pulmón/diagnóstico , Malformación Adenomatoide Quística Congénita del Pulmón/patología , Errores Diagnósticos , Humanos , Lactante , Masculino , Blastoma Pulmonar/diagnóstico
9.
J Pediatr Surg ; 53(12): 2390-2393, 2018 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-30227995

RESUMEN

BACKGROUND/PURPOSE: Differences in clinical features between congenital pulmonary airway malformation (CPAM) and bronchial atresia (BA) have not yet been clearly described. METHODS: We retrospectively reviewed 112 patients with a pathological diagnosis of CPAM or BA. The clinical parameters were statistically analyzed between these diseases. RESULTS: Seventy-one patients received prenatal diagnosis and 41 received postnatal diagnosis. The percentage of prenatal diagnosis was significantly higher in CPAM patients (84% vs 50%, p < 0.001). Among patients with prenatal diagnosis, the backgrounds were not different between the two diseases except for the number of Caesarean sections (81% vs 9%, p < 0.0001). The numbers of patients that underwent fetal interventions and emergent neonatal surgery were higher in CPAM (51% vs 15%, p < 0.01 and 76% vs 12%, p < 0.0001), although there was no statistical difference in survival rate (86% vs 97%, p = 0.2). In patients receiving postnatal diagnosis, pneumonia was the primary symptom in most BA patients, whereas respiratory distress was the major symptom in patients with CPAM. Age at presentation of the primary symptom was significantly older in BA patients (4.2 years vs 1.2 years, p < 0.005). CONCLUSION: CPAM and BA have distinct clinical features in terms of therapeutic and natural history. Careful imaging evaluation and pathological analysis can lead to an accurate diagnosis of BA. TYPE OF STUDY: Prognostic study. LEVEL OF EVIDENCE: Level II. This study is categorized as a "Prognostic Study" with LEVEL III of Evidence.


Asunto(s)
Enfermedades Bronquiales/diagnóstico , Malformación Adenomatoide Quística Congénita del Pulmón/diagnóstico , Enfermedades Bronquiales/congénito , Enfermedades Bronquiales/terapia , Malformación Adenomatoide Quística Congénita del Pulmón/terapia , Femenino , Humanos , Recién Nacido , Masculino , Embarazo , Diagnóstico Prenatal/estadística & datos numéricos , Pronóstico , Estudios Retrospectivos
11.
J Coll Physicians Surg Pak ; 28(9): S201-S203, 2018 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-30173698

RESUMEN

Hybrid congenital cystic lesions of lung comprising of both congenital cystic adenomatoid malformation (CCAM) and broncho-pulmonary sequestration (BPS) are very rare congenital malformations of the lung. We present a case of a newborn who presented to us with severe respiratory distress and later on found to have a hybrid lesion of the lung. It is a very rare occurrence and no such case has been reported from Pakistan so far.


Asunto(s)
Secuestro Broncopulmonar/diagnóstico , Malformación Adenomatoide Quística Congénita del Pulmón/diagnóstico , Pulmón/diagnóstico por imagen , Síndrome de Dificultad Respiratoria del Recién Nacido/etiología , Ecocardiografía , Humanos , Recién Nacido , Pulmón/patología , Masculino
12.
J Pediatr Surg ; 53(12): 2386-2389, 2018 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-30244937

RESUMEN

PURPOSE: The purpose of this study was to clarify the relationship between congenital pulmonary airway malformation volume ratio (CVR) of bronchial atresia (BA), CVR of congenital cystic adenomatoid malformations (CCAM), and time of surgery after birth. METHOD: We retrospectively analyzed data of 36 BA and CCAM cases, prenatally diagnosed as CPAM from 2009 through 2014. RESULTS: Within 2 h after birth, 12 neonatal patients underwent emergent (EMG) lobectomy. Five cases of lobectomy were performed urgently (UG) from 12 to 48 h after birth. Four cases of lobectomy were required within 30 days after birth (early = EAG). We performed lobectomy in 15 other patients at 11 months after birth (late = LG). Of the EMG cases, 11 were macrotype CCAM (maximal CVR >2.0), and 4 of 5 UG cases were microtype CCAM (CVR >2.0). Of the EAG cases, 3 of 4 were macrotype CCAM with CVR of <1.5. Of 15 LG, 13 were BA and showed a CVR of 0.13-3.0 (median, 0.78). The CVR of the cases operated on within 48 h after birth was significantly larger than that of the cases operated on after 2 weeks (p = 0.001). CONCLUSION: EMG or UG lobectomy was usually required after birth in CCAM, indicating maximal CVR >2.0. By contrast, elective surgery was performed in most BA cases. LEVEL OF EVIDENCE: IV.


Asunto(s)
Enfermedades Bronquiales/cirugía , Malformación Adenomatoide Quística Congénita del Pulmón/cirugía , Neumonectomía/métodos , Medición de Riesgo/métodos , Enfermedades Bronquiales/congénito , Enfermedades Bronquiales/diagnóstico , Malformación Adenomatoide Quística Congénita del Pulmón/diagnóstico , Tratamiento de Urgencia/métodos , Humanos , Recién Nacido , Neumonectomía/estadística & datos numéricos , Diagnóstico Prenatal/métodos , Estudios Retrospectivos , Factores de Tiempo
13.
Autops. Case Rep ; 8(2): e2018022, Apr.-May 2018. ilus
Artículo en Inglés | LILACS | ID: biblio-905523

RESUMEN

Congenital pulmonary airway malformation (CPAM) is a developmental malformation of the lower respiratory tract. We report the case of a male newborn diagnosed with cystic lung disease during prenatal ultrasound. A cesarean section was performed at the 32nd gestational week because of premature rupture of the membranes, and soon after the delivery the newborn developed respiratory failure and died. The aim of this study is to report an autopsy case because of its rarity, and to briefly discuss the CPAM subtypes and differential diagnosis of cystic lung diseases of childhood.


Asunto(s)
Humanos , Femenino , Embarazo , Recién Nacido , Malformación Adenomatoide Quística Congénita del Pulmón/diagnóstico , Diagnóstico Prenatal/estadística & datos numéricos , Aborto , Autopsia , Malformación Adenomatoide Quística Congénita del Pulmón/patología , Diagnóstico Diferencial , Resultado Fatal , Recien Nacido Prematuro , Síndrome de Dificultad Respiratoria del Recién Nacido/etiología
14.
Am J Perinatol ; 35(12): 1222-1227, 2018 10.
Artículo en Inglés | MEDLINE | ID: mdl-29715700

RESUMEN

BACKGROUND AND OBJECTIVE: Congenital pulmonary airway malformation (CPAM) is a group of rare congenital malformations of the lung and airways. Lung ultrasound (LU) is increasingly used to diagnose neonatal respiratory diseases since it is quick, easy to learn, and radiation-free, but no formal data exist for congenital lung malformations. We aimed to describe LU findings in CPAM neonates needing neonatal intensive care unit (NICU) admission and to compare them with a control population. METHODS: A retrospective review of CPAM cases from three tertiary academic NICUs over 3 years (2014-2016) identified five patients with CPAM who had undergone LU examination. LU was compared with chest radiograms and computed tomography (CT) scans that were used as references. RESULTS: CPAM lesions were easily identified and corresponded well with CT scans; they varied from a single large cystic lesion, multiple hypoechoic lesions, and/or consolidation. The first two LU findings have not been described in other respiratory conditions and were not found in controls. CONCLUSION: We provide the first description of LU findings in neonates with CPAM. LU may be used to confirm antenatally diagnosed CPAM and to suspect CPAM in infants with respiratory distress if cystic lung lesions are revealed. Further studies are necessary to define the place of LU in the management of CPAM.


Asunto(s)
Malformación Adenomatoide Quística Congénita del Pulmón/diagnóstico por imagen , Pulmón/diagnóstico por imagen , Ultrasonografía , Malformación Adenomatoide Quística Congénita del Pulmón/diagnóstico , Femenino , Francia , Humanos , Recién Nacido , Unidades de Cuidado Intensivo Neonatal , Italia , Masculino , Radiografía Torácica , Estudios Retrospectivos , Tomografía Computarizada por Rayos X
15.
Pediatr Surg Int ; 34(6): 613-620, 2018 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-29632964

RESUMEN

PURPOSE: For the approximately three quarters of patients with a prenatal diagnosis of congenital pulmonary airway malformation (CPAM) who are asymptomatic at birth, the chance of eventually developing symptoms is unknown. We sought to explore the natural history of asymptomatic CPAM. METHODS: We searched EMBASE, MEDLINE, and the first 50 results from Google Scholar. Studies describing the natural history of prenatally diagnosed, initially asymptomatic CPAM were included. For asymptomatic patients initially managed non-surgically, we tabulated the proportion who went on to develop symptoms as well as the median age at symptom development. RESULTS: We included data from 19 retrospective studies on 353 patients. Of the 128 patients managed expectantly, 31 (24.2%) developed symptoms requiring surgical intervention. The median age at symptom development was 7.5 months (range 15 days-5 years). CONCLUSION: The risk for developing respiratory symptoms exists with originally asymptomatic CPAM patients, but the exact risk is difficult to predict. Parents may be given the value of approximately 1 in 4 as an estimate of the proportion of asymptomatic CPAM patients who go on to develop symptoms, which will help them make an informed decision regarding the option of elective surgery.


Asunto(s)
Enfermedades Asintomáticas , Malformación Adenomatoide Quística Congénita del Pulmón/terapia , Progresión de la Enfermedad , Tratamiento Conservador , Malformación Adenomatoide Quística Congénita del Pulmón/complicaciones , Malformación Adenomatoide Quística Congénita del Pulmón/diagnóstico , Procedimientos Quirúrgicos Electivos , Femenino , Humanos , Embarazo , Diagnóstico Prenatal
17.
Eur J Pediatr Surg ; 28(1): 1-5, 2018 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-28709163

RESUMEN

AIM: To define current management of congenital pulmonary airway malformation (CPAM). METHODS: A total of 181 European Pediatric Surgeons' Association members (91% senior) from 48 countries completed an online questionnaire. MAIN RESULTS: Prenatal: 93% respondents work in centers with prenatal diagnosis facilities, and 27% in centers offering in utero surgery. Prenatal counseling is performed by 86% respondents, 22% of whom see >10 cases per year. Risk of single pre-/postnatal complications is deemed low (<5%) by more than 60% of respondents. Eighty-six percent respondents do not offer pregnancy termination for prenatally diagnosed CPAM. Fetal hydrops is the most frequent indication for termination (87%), followed by parental willingness (52%). Prenatal surgery is an option for 44% respondents, preferring thoracoamniotic shunt (82%).Postnatal: 75% respondents operate on asymptomatic patients, 18% before 6 months of age, 62% between 6 and 12 months of age, and 20% after 12 months of age. Risk of infection (86%), cancer (63%), and symptoms development (62%) are indications for surgery in asymptomatic CPAM. Sixty-three percent prefer a thoracotomy. Lobectomy is the preferred procedure (58% respondents). Motivations against surgery include lesion <1 cm (64%), risk of postoperative complications (37%), and lack of evidence favoring surgery (27%). Seventeen percent respondents have seen at least one patient with CPAM with lung cancer, in 89% of the cases within the CPAM. Of all the respondents, 83% and 22% offered dedicated follow-up and genetic screening, respectively. CONCLUSION: Current pre- and postnatal management of CPAM lacks uniformity, particularly for surgical indication, timing, and approach. Efforts should be made toward standardization. Risk of CPAM-associated cancer is not clear.


Asunto(s)
Malformación Adenomatoide Quística Congénita del Pulmón/diagnóstico , Malformación Adenomatoide Quística Congénita del Pulmón/terapia , Pautas de la Práctica en Medicina/estadística & datos numéricos , Europa (Continente) , Femenino , Terapias Fetales/métodos , Terapias Fetales/estadística & datos numéricos , Encuestas de Atención de la Salud , Humanos , Recién Nacido , Atención Posnatal/métodos , Atención Posnatal/estadística & datos numéricos , Embarazo , Atención Prenatal/métodos , Atención Prenatal/estadística & datos numéricos , Diagnóstico Prenatal/estadística & datos numéricos
18.
Semin Pediatr Surg ; 26(5): 311-316, 2017 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-29110827

RESUMEN

Congenital lung malformations comprise a group of anatomical abnormalities of the respiratory tree including congenital cystic malformations, bronchopulmonary sequestrations, bronchogenic cyst, bronchial atresia, and congenital lobar emphysema. These anomalies are detected with increasing frequency by pre-natal sonography, but may also present for the first time with symptoms in childhood or later life. When symptomatic, there is little controversy that resection is indicated, which is usually curative. When a lesion is asymptomatic there is greater debate regarding the benefit of resection versus continued observation. This article provides an overview of the spectrum of disorders, the management options available and the long-term outcomes associated with each treatment option.


Asunto(s)
Secuestro Broncopulmonar , Malformación Adenomatoide Quística Congénita del Pulmón , Enfisema Pulmonar/congénito , Secuestro Broncopulmonar/complicaciones , Secuestro Broncopulmonar/diagnóstico , Secuestro Broncopulmonar/terapia , Malformación Adenomatoide Quística Congénita del Pulmón/complicaciones , Malformación Adenomatoide Quística Congénita del Pulmón/diagnóstico , Malformación Adenomatoide Quística Congénita del Pulmón/terapia , Humanos , Pulmón/anomalías , Pulmón/embriología , Pulmón/fisiopatología , Neumonectomía , Enfisema Pulmonar/complicaciones , Enfisema Pulmonar/diagnóstico , Enfisema Pulmonar/terapia , Resultado del Tratamiento
19.
Eur J Pediatr ; 176(12): 1559-1571, 2017 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-29046943

RESUMEN

Congenital pulmonary airway malformations or CPAM are rare developmental lung malformations, leading to cystic and/or adenomatous pulmonary areas. Nowadays, CPAM are diagnosed prenatally, improving the prenatal and immediate postnatal care and ultimately the knowledge on CPAM pathophysiology. CPAM natural evolution can lead to infections or malignancies, whose exact prevalence is still difficult to assess. The aim of this "state-of-the-art" review is to cover the recently published literature on CPAM management whether the pulmonary lesion was detected during pregnancy or after birth, the current indications of surgery or surveillance and finally its potential evolution to pleuro-pulmonary blastoma. CONCLUSION: Surgery remains the cornerstone treatment of symptomatic lesions but the postnatal management of asymptomatic CPAM remains controversial. There are pros and cons of surgical resection, as increasing rate of infections over time renders the surgery more difficult after months or years of evolution, as well as risk of malignancy, though exact incidence is still unknown. What is known: • Congenital pulmonary airway malformations (CPAM) are rare developmental lung malformations mainly antenatally diagnosed. • While the neonatal management of symptomatic CPAM is clear and includes prompt surgery, controversies remain for asymptomatic CPAM due to risk of infections and malignancies. What is new: • Increased rate of infection over time renders the surgery more difficult after months or years of evolution and pushes for recommendation of early elective surgery. • New molecular or pathological pathways may help in the distinction of type 4 CPAM from type I pleuropulmonary blastoma.


Asunto(s)
Malformación Adenomatoide Quística Congénita del Pulmón , Malformación Adenomatoide Quística Congénita del Pulmón/diagnóstico , Malformación Adenomatoide Quística Congénita del Pulmón/etiología , Malformación Adenomatoide Quística Congénita del Pulmón/patología , Malformación Adenomatoide Quística Congénita del Pulmón/terapia , Progresión de la Enfermedad , Femenino , Predisposición Genética a la Enfermedad , Humanos , Pulmón/anomalías , Pulmón/embriología , Neoplasias Pulmonares/diagnóstico , Neoplasias Pulmonares/etiología , Neoplasias Pulmonares/terapia , Pediatría , Embarazo , Diagnóstico Prenatal , Blastoma Pulmonar/diagnóstico , Blastoma Pulmonar/etiología , Blastoma Pulmonar/terapia
20.
J Med Case Rep ; 11(1): 212, 2017 Aug 04.
Artículo en Inglés | MEDLINE | ID: mdl-28774324

RESUMEN

BACKGROUND: Congenital cystic adenomatoid malformation is a rare pulmonary malformation, but is the most common lung malformation observed in children. In developing countries, such as Morocco, prenatal diagnosis is missing. Congenital cystic adenomatoid malformation may occur after birth in the presence of complications and needs a computed tomography scan for confirmation. However, our lack of awareness of this malformation has been responsible for a late and wrong diagnosis along with therapeutic errors. We report the first case in Morocco where diagnosis is confirmed by histology after death. CASE PRESENTATION: A 10-month-old Arab boy was prescribed various antibiotics (including anti-staphylococcal) and endured repeated chest drainages, leading to his death just after radiological diagnosis and instant surgery. CONCLUSIONS: The goal of this case report is to firmly express the need for both pediatricians and radiologists to enlarge diagnosis investigations, especially of congenital or constitutional entities in children, as soon as recurrence of respiratory distress and pulmonary infections are manifested. We also emphasize this important entity because of its frequency, to avoid the eventual therapeutic errors.


Asunto(s)
Antibacterianos/administración & dosificación , Malformación Adenomatoide Quística Congénita del Pulmón/diagnóstico , Hipoxia/mortalidad , Pulmón/patología , Neumonectomía/mortalidad , Complicaciones Posoperatorias/mortalidad , Infecciones del Sistema Respiratorio/patología , Tomografía Computarizada por Rayos X , Malformación Adenomatoide Quística Congénita del Pulmón/fisiopatología , Malformación Adenomatoide Quística Congénita del Pulmón/terapia , Errores Diagnósticos , Resultado Fatal , Humanos , Lactante , Pulmón/diagnóstico por imagen , Masculino , Infecciones del Sistema Respiratorio/diagnóstico por imagen , Infecciones del Sistema Respiratorio/tratamiento farmacológico
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