RESUMO
BACKGROUND: Foetal thoracic lesions are uncommon, with the incidence of 1 in 15,000 live births. Antenatal monitoring of these lesions is required to prognosticate the parents about the postnatal outcome of the lesions and about the well-being of the baby. Foetal ultrasound and magnetic resonance imaging (MRI) are the modalities to detect these lesions and follow-up during pregnancy and postnatally. Congenital pulmonary adenomatoid malformations (CPAM), congenital diaphragmatic hernia (CDH) and bronchopulmonary sequestrations (BPS) are the commonly detected foetal thoracic lesions. Parameters such as congenital cystic adenomatoid malformation (CCAM) volume ratio (CVR) and lung-to-head ratio (LHR) determine the prognosis of these lesions. With this background, we planned to study the prognosis and outcome of antenatally detected thoracic lesions. MATERIALS AND METHODS: This was a prospective study carried out for 2 years (January 2017 to December 2018). Pregnant females with foetuses diagnosed to have thoracic lesions on the second- and third-trimester ultrasound and foetal MRI were enrolled for the study. CVR and LHR were noted. Outcomes were analysed in terms of the need of termination of pregnancy, foetal or neonatal demise and need of surgery or conservative management. RESULTS: Of a total of 521 pregnancies, thoracic lesions were detected in 22 (4.22%) cases. Individually, the incidence of each lesion was: CPAM-10 (45.45%), BPS-5 (22.73%), CDH-5 (22.73%) and congenital higher airway obstruction-2 (9.1%). Chromosomal screening was normal in 100% of cases. Follow-up was done for 2 years. Termination of pregnancy was required in 3 (13.64%) cases, postnatal death occurred in 4 (18.2%) cases, surgery was required in three cases of CDH soon after birth and two cases of extralobar BPS at 2 years of age. 10 (45.45%) cases were managed conservatively, who are doing well at 2-year follow-up. CONCLUSION: CPAMs, BPSs and CDH are the commonly detected antenatal thoracic lesions. Ultrasonography and MRI help detect and prognosticate the parents about the outcomes of these lesions. CVR and LHR with associated congenital malformations are important prognostic markers for these lesions. There is a need of long-term prospective studies to delineate the natural history of these lesions.
Assuntos
Sequestro Broncopulmonar , Malformação Adenomatoide Cística Congênita do Pulmão , Hérnias Diafragmáticas Congênitas , Lactente , Recém-Nascido , Gravidez , Humanos , Feminino , Estudos Prospectivos , Pulmão/diagnóstico por imagem , Pulmão/patologia , Pulmão/cirurgia , Prognóstico , Malformação Adenomatoide Cística Congênita do Pulmão/diagnóstico por imagem , Malformação Adenomatoide Cística Congênita do Pulmão/terapia , Ultrassonografia Pré-Natal , Sequestro Broncopulmonar/patologia , Sequestro Broncopulmonar/cirurgia , Estudos RetrospectivosRESUMO
Congenital cystic pulmonary lesions (CCPLs) are represented by the following entities: congenital pulmonary airway malformation (CPAM), formerly congenital cystic adenomatoid malformation, extra- and intralobar sequestration (EIS), congenital lobar emphysema (overexpansion), and bronchogenic cyst. The developmental model of CPAM histogenesis by Stocker proposed perturbations designated as CPAM type 0 to type 4 without known or specific pathogenetic mechanisms along the airway from the bronchus to the alveolus. This review highlights mutational events either at the somatic level in KRAS (CPAM types 1 and possibly 3) or germline variants in congenital acinar dysplasia, formerly CPAM type 0, and pleuropulmonary blastoma (PPB), type I, formerly CPAM type 4. The potential for overt malignant progression exists in the case of PPB type I and CPAM type 1 in some cases to well-differentiated mucinous adenocarcinoma. On the other hand, CPAM type 2 is an acquired lesion resulting from interruption in lung development secondary to bronchial atresia. The latter is also regarded as the etiology of EIS whose pathologic features are similar, if not identical, to CPAM type 2. These observations have provided important insights into the pathogenetic mechanisms in the development of the CPAMs since the Stocker classification.
Assuntos
Sequestro Broncopulmonar , Malformação Adenomatoide Cística Congênita do Pulmão , Neoplasias Pulmonares , Blastoma Pulmonar , Anormalidades do Sistema Respiratório , Humanos , Malformação Adenomatoide Cística Congênita do Pulmão/diagnóstico , Malformação Adenomatoide Cística Congênita do Pulmão/genética , Pulmão/patologia , Blastoma Pulmonar/diagnóstico , Blastoma Pulmonar/genética , Neoplasias Pulmonares/congênito , Anormalidades do Sistema Respiratório/diagnóstico , Anormalidades do Sistema Respiratório/genética , Sequestro Broncopulmonar/patologiaRESUMO
Congenital pulmonary airway malformations (CPAMs) have a range of morphologies with varying cyst sizes and histologic features (types 1 to 3). Evidence suggested they arise secondary to bronchial atresia, however, we recently showed that cases with type 1 and 3 morphology are driven by mosaic KRAS mutations. We hypothesized that 2 distinct mechanisms account for most CPAMs: one subset is secondary to KRAS mosaicism and another is due to bronchial atresia. Cases with type 2 histology, similar to sequestrations, would be related to obstruction and therefore negative for KRAS mutations regardless of cyst size. We sequenced KRAS exon 2 in type 2 CPAMs, cystic intralobar and extralobar sequestrations, and intrapulmonary bronchogenic cysts. All were negative. Most sequestrations had a large airway in the subpleural parenchyma adjacent to the systemic vessel, anatomically confirming bronchial obstruction. We compared morphology to type 1 and 3 CPAMs. On average, type 1 CPAMs had significantly larger cysts, but there remained substantial size overlap between KRAS mutant and wild-type lesions. Features of mucostasis were frequent in sequestrations and type 2 CPAMs, while their cysts were generally simple and round with flat epithelium. Features of cyst architectural and epithelial complexity were more common in type 1 and 3 CPAMs, which rarely showed mucostasis. Similarity in histologic features among cases that are negative for KRAS mutation support the hypothesis that, like sequestrations, the malformation of type 2 CPAMs is related to obstruction during development. A mechanistic approach to classification may improve existing subjective morphologic methods.
Assuntos
Sequestro Broncopulmonar , Malformação Adenomatoide Cística Congênita do Pulmão , Cistos , Humanos , Sequestro Broncopulmonar/patologia , Proteínas Proto-Oncogênicas p21(ras) , Malformação Adenomatoide Cística Congênita do Pulmão/genética , Malformação Adenomatoide Cística Congênita do Pulmão/patologia , Cistos/patologia , Aberrações Cromossômicas , Pulmão/patologiaRESUMO
Background: Extralobar pulmonary sequestration (ELS) is a malformation composed of bronchopulmonary tissue outside the lungs that is discontinuous from the main tracheobronchial tree. ELS can present as a suprarenal space occupying lesion. Case Report: A 1-day old girl presented with a right supra-adrenal mass, first detected in-utero at 34 weeks. The differential included congenital neuroblastoma, but the urinary VMA was normal. At resection, there was lung tissue composed of dilated and tortuous bronchioles lined by columnar epithelium present in a back- to- back arrangement along with thick-walled vessels, features of an ELS with congenital pulmonary airway malformation type II. Conclusion: ELS enters the differential diagnosis of neonatal suprarenal masses with normal urinary catecholamines, and can have the morphology of a type II congenital pulmonary airway malformation.
Assuntos
Neoplasias das Glândulas Suprarrenais , Sequestro Broncopulmonar , Malformação Adenomatoide Cística Congênita do Pulmão , Neuroblastoma , Recém-Nascido , Feminino , Humanos , Sequestro Broncopulmonar/diagnóstico , Sequestro Broncopulmonar/patologia , Sequestro Broncopulmonar/cirurgia , Pulmão , Neoplasias das Glândulas Suprarrenais/diagnóstico , Malformação Adenomatoide Cística Congênita do Pulmão/patologia , Neuroblastoma/diagnóstico , Neuroblastoma/patologiaRESUMO
Congenital lung lesions are a rare group of developmental pulmonary abnormalities that are often first identified prenatally on routine second-trimester US. Congenital pulmonary airway malformation (CPAM) is the most common anomaly while others include bronchopulmonary sequestration, congenital lobar overinflation, bronchogenic cyst and bronchial atresia. Clinical presentation is highly variable, ranging from apparent in utero resolution to severe mass effect with resultant hydrops fetalis and fetal demise. Differentiation among these lesions can be challenging because overlapping imaging features are often present. The roles of the radiologist are to identify key imaging findings that help in diagnosing congenital lung lesions and to recognize any ominous features that might require prenatal or perinatal intervention. High-resolution US and complementary rapid-acquisition fetal MRI provide valuable information necessary for lesion characterization. Postnatal US and CT angiography are helpful for lesion evaluation and for possible surgical planning. This article reviews the embryology of the lungs, the normal prenatal imaging appearance of the thorax and its contents, and the prenatal and neonatal imaging characteristics, prognosis and management of various congenital lung lesions.
Assuntos
Sequestro Broncopulmonar , Malformação Adenomatoide Cística Congênita do Pulmão , Pneumonia , Anormalidades do Sistema Respiratório , Sequestro Broncopulmonar/diagnóstico por imagem , Sequestro Broncopulmonar/patologia , Malformação Adenomatoide Cística Congênita do Pulmão/diagnóstico , Malformação Adenomatoide Cística Congênita do Pulmão/patologia , Malformação Adenomatoide Cística Congênita do Pulmão/cirurgia , Feminino , Humanos , Recém-Nascido , Pulmão/diagnóstico por imagem , Pulmão/patologia , Gravidez , Anormalidades do Sistema Respiratório/diagnóstico por imagem , Anormalidades do Sistema Respiratório/patologia , Ultrassonografia Pré-Natal/métodosRESUMO
RATIONALE: Pulmonary sequestration, which can be divided into 2 main types: intralobar pulmonary sequestration (IPS) and extralobar pulmonary sequestration, is an uncommon congenital condition for which surgical resection is usually indicated. Video-assisted thoracoscopic surgery, as compared with open thoracotomy, has increasingly become the preferred operative procedure in the treatment of PS, owing to less postoperative pain and faster recovery. This report describes a rare and challenging case with a giant IPS undergoing video-assisted thoracic lobectomy. PATIENT CONCERNS: A 39-year old woman suffered from recurrent pneumonia for nearly 3 years. An enhanced computed tomography scan performed in our hospital revealed a 12.0 cm × 10.0 cm-sized IPS in the left lower lobe, supplied by an 8-mm aberrant artery originating from the descending thoracic aorta. DIAGNOSIS: Histology of the resected lobe confirmed the diagnosis of giant intralobar pulmonary sequestration associated with infection. INTERVENTIONS: Thoracoscopic left lower lobectomy was performed. OUTCOMES: The patient has been discharged from the hospital on the ninth day after surgery with an uneventful recovery, she was in good health after a 1-year follow-up. LESSONS: Although full of challenges, thoracoscopic lobectomy for giant IPS is a safe and feasible surgical procedure associated with reduced surgical trauma and postoperative pain as well as improved cosmetic results compared with traditional thoracotomy.
Assuntos
Sequestro Broncopulmonar , Adulto , Sequestro Broncopulmonar/diagnóstico por imagem , Sequestro Broncopulmonar/patologia , Sequestro Broncopulmonar/cirurgia , Feminino , Humanos , Dor Pós-Operatória/cirurgia , Pneumonectomia/métodos , Cirurgia Torácica Vídeoassistida/métodos , ToracotomiaRESUMO
Background: Idiopathic lobar emphysema (ILE) and bronchopulmonary sequestration (BPS) are two of the well-characterized pulmonary malformations. Case report: An antenatally detected case of a left bronchopulmonary malformation (BPM) was clinicoradiologically diagnosed to be a left upper lobar emphysema with isolated dextrocardia in the neonatal period. Besides the emphysematous left upper lobe, an accessory lobe akin to an extra lobar BPS was an operative surprise. Histopathological examination of both excised lobes led to a revised diagnosis of a 'hybrid' malformation comprising lobar emphysema and extra lobar BPS. The postoperative recovery was uneventful. Discussion/conclusion: The observations suggest that BPS and ILE may be interim entities in a continuum of abnormal embryogenesis. Such a hybrid malformation has not been hitherto reported.
Assuntos
Sequestro Broncopulmonar , Enfisema , Enfisema Pulmonar , Brônquios/patologia , Sequestro Broncopulmonar/diagnóstico , Sequestro Broncopulmonar/patologia , Sequestro Broncopulmonar/cirurgia , Humanos , Recém-Nascido , Pulmão/anormalidades , Enfisema Pulmonar/diagnóstico , Enfisema Pulmonar/patologiaRESUMO
INTRODUCTION: We describe the rare case of endobronchial tuberculosis (EBTB) and chronic pulmonary atelectasis with mediastinal distortion. Finding of the concomitant venous anomaly of inferior vena cava revealed the diagnosis of bronchopulmonary sequestration. CASE REPORT: A 22-year-old Caucasian woman presented with a history of chronic cough, initially treated as bronchial asthma for a year. Chest X-ray showed fibrocaseous cavernous tuberculosis on the right lung. Acid Fast Bacilli (AFB) were found in sputum samples. Patient was treated for 6 months with usual antituberculous regiment. Control chest X-ray showed subatelectasis of the upper right lobe. Six months later the first thorax computed tomography (CT) showed complete atelectasis of the right lung. Patient was admitted to the hospital again after 6 years due to the persistent fever and cough. Endoscopic finding and histopathological analysis confirmed EBTB. Thoracic CT scan revealed duplication of inferior vena cava which led to profound vascular analysis and aberrant arterial vascularization of aortic origin that contributed to the diagnosis of bronchopulmonary sequestrations. Antituberculous treatment was initiated (streptomycin, isoniazid, rifampicin, ethambutol and pyrazinamide) and lasted for 8 months. After 8 months a follow-up fiberoptic bronchoscopy showed the progression of endoscopic finding with 60-70% tracheal stenosis. Histopathological finding of the mid-trachea showed non-specific granulations. During 7 years of follow-up repeated bronchoscopy and thoracic CT scans were unchanged and patient was well-shaped. CONCLUSIONS: The clinician should consider bronchopulmonary sequestration in the cases of recurrent EBTB.
Assuntos
Sequestro Broncopulmonar/complicações , Tuberculose Pulmonar/complicações , Adulto , Sequestro Broncopulmonar/diagnóstico por imagem , Sequestro Broncopulmonar/patologia , Diagnóstico Tardio , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Recidiva , Tomografia Computadorizada por Raios X , Tuberculose Pulmonar/diagnóstico por imagem , Tuberculose Pulmonar/patologia , Adulto JovemAssuntos
Infecções Bacterianas/diagnóstico , Infecções Bacterianas/patologia , Sequestro Broncopulmonar/complicações , Sequestro Broncopulmonar/diagnóstico , Pneumopatias/diagnóstico , Adulto , Antibacterianos/uso terapêutico , Infecções Bacterianas/tratamento farmacológico , Sequestro Broncopulmonar/patologia , Sequestro Broncopulmonar/terapia , Humanos , Ligadura , Pneumopatias/microbiologia , Pneumopatias/patologia , Masculino , Combinação Piperacilina e Tazobactam/uso terapêutico , ToracoscopiaRESUMO
RATIONALE: Pulmonary sequestration (PS) presenting with elevated serum tumor markers is rare, and it might be misdiagnosed as malignancy. PATIENT CONCERNS: A 26-year-old asymptomatic male patient was admitted because the x-ray showed an intrathoracic lesion. Meanwhile, the serum neuron-specific enolase (NSE) was elevated. Three-dimensional computed tomography angiography revealed an isolated feeding vessel arising from the aorta. DIAGNOSES: Extralobular PS was confirmed by computed tomography angiography and postoperative pathological staining. INTERVENTIONS: Two-port thoracoscopic resection of the sequestrated lobe was performed. OUTCOMES: The serum NSE decreased to within the normal range and persisted during the follow up of 10 months. LESSONS: A thorough work-up should be considered for the PS patients presenting with abnormal serum NSE. Detailed knowledge regarding the relationship between NSE and PS necessitates further studies.
Assuntos
Sequestro Broncopulmonar/diagnóstico por imagem , Angiografia por Tomografia Computadorizada/métodos , Fosfopiruvato Hidratase/sangue , Adulto , Assistência ao Convalescente , Aorta/anormalidades , Aorta/diagnóstico por imagem , Sequestro Broncopulmonar/patologia , Sequestro Broncopulmonar/cirurgia , Humanos , Masculino , Cirurgia Torácica Vídeoassistida/métodos , Resultado do TratamentoAssuntos
Biomarcadores Tumorais/sangue , Sequestro Broncopulmonar/sangue , Antígeno Ca-125/sangue , Neoplasias Pulmonares/sangue , Pneumonectomia/métodos , Adulto , Sequestro Broncopulmonar/diagnóstico por imagem , Sequestro Broncopulmonar/patologia , Sequestro Broncopulmonar/cirurgia , Antígeno CA-19-9/sangue , Células Epiteliais/metabolismo , Células Epiteliais/patologia , Humanos , Neoplasias Pulmonares/diagnóstico por imagem , Neoplasias Pulmonares/patologia , Neoplasias Pulmonares/cirurgia , Masculino , Tomografia Computadorizada por Raios XRESUMO
In intralobar pulmonary sequestrations, vascular changes similar to those in pulmonary hypertension (PH) are generally observed, such as intimal proliferation and plexiform lesions. However, to our knowledge, a sequestrated lung manifesting vascular changes with both arteritis and a plexiform lesion has never been reported. A 25-year-old man was diagnosed with intralobar pulmonary sequestration. Pathologically, both arteritis and a plexiform lesion were observed in the sequestrated lung. Systemic vasculitis syndrome was clinically excluded, and the pathological findings appeared to be associated with local PH. Arteritis is an extremely rare finding; only one case of arteritis associated with local PH has been reported in intralobar sequestration. In this case, the artery near the plexiform lesion had milder inflammation and fibrosis, suggesting that the arteritis formed prior to the plexiform lesion. This is the first case of arteritis and a plexiform lesion co-occurring in intralobar pulmonary sequestration associated with local PH. This case may shed light on the formation of plexiform lesions and their association with arteritis.
Assuntos
Arterite/patologia , Sequestro Broncopulmonar/patologia , Hipertensão Pulmonar , Adulto , Sequestro Broncopulmonar/complicações , Humanos , Hipertensão Pulmonar/etiologia , MasculinoAssuntos
Sequestro Broncopulmonar/diagnóstico por imagem , Cavidade Abdominal/diagnóstico por imagem , Cavidade Abdominal/patologia , Idoso , Sequestro Broncopulmonar/patologia , Sequestro Broncopulmonar/cirurgia , Diagnóstico Diferencial , Aspiração por Agulha Fina Guiada por Ultrassom Endoscópico , Feminino , Humanos , Tomografia Computadorizada por Raios XRESUMO
This study aimed to evaluate the prognosis of pulmonary sequestration (PS) by measuring congenital cystic adenomatoid malformation volume ratio (CVR) value in fetal congenital PS. The fetal CVR in 49 cases of fetal PS diagnosed by prenatal ultrasound in Xiangyang No. 1 People's Hospital from March 2010 to June 2017 were measured, and the clinical outcomes were observed. According to the prenatal ultrasound CVR value, 49 fetuses diagnosed with PS were divided into 2 groups: group 1 with CVR≥1.26, and group 2 with CVR<1.26. The incidence rate of fetal edema, respiratory distress symptoms and survival rate were compared between the two groups. The risk factors of the fetal PS were evaluated by single and multiple Logistic regression analysis. The correlation between CVR and fetal prognosis was analyzed. Of the 49 fetuses, there were 34 cases of PS (ILS) type (69.39%, 34/49), 10 cases of PS (ELS) type I (20.41%, 10/49) and 5 cases of PS (ELS) type II (10.20%, 5/49). Forty-six cases (93.88%, 46/49) were born alive, there was 1 case (CVR ≥1.26) (2.04%, 1/49) of induced abortion, and 2 cases (CVR ≥1.26) (4.08%, 2/49) of stillbirths. In group 1 (n=24), 21 cases were born alive, and the incidence rate of newborn respiratory distress and fetal edema was 100% (21/21) and 79.17% (19/24) respectively. In group 2 (n=25), there were 3 cases (12%,3/25) of newborn respiratory distress, 3 cases (12%, 3/25) of fetal edema, and the rate of live birth was 100%. There were statistically significant differences between the two groups in the incidence of fetal edema, postpartum respiratory symptoms and survival rate. CVR was a risk factor for PS and was associated with fetal prognosis. CVR in the midtrimester of pregnancy is an effective index to evaluate the prognosis of fetal PS. CVR ≥1.26 is associated with an increased risk of fetal edema, infant respiratory distress and intrauterine or postnatal death.
Assuntos
Sequestro Broncopulmonar/diagnóstico , Malformação Adenomatoide Cística Congênita do Pulmão/diagnóstico por imagem , Feto/diagnóstico por imagem , Pulmão/diagnóstico por imagem , Sequestro Broncopulmonar/diagnóstico por imagem , Sequestro Broncopulmonar/patologia , Malformação Adenomatoide Cística Congênita do Pulmão/fisiopatologia , Feminino , Feto/fisiopatologia , Humanos , Recém-Nascido , Nascido Vivo , Pulmão/fisiopatologia , Gravidez , Prognóstico , Resultado do TratamentoRESUMO
BACKGROUND: The potential complications of pulmonary sequestration are serious and may include recurrent pulmonary infection, hemoptysis, and tumorigenesis. Therefore, the gold standard of treatment has been surgery. Although an adequate boundary between the nonfunctional lung and normal lung is required for the resection of pulmonary sequestration, the boundaries have been conventionally identified intraoperatively with inflation/deflation of the target segment by clamping and unclamping the relevant bronchus. The technique of visualizing the demarcation line based on near-infrared fluorescence imaging with indocyanine green was recently developed. CASE PRESENTATION: A 42-year-old Japanese woman with right Pryce III intralobar sequestration was admitted to our hospital. We planned video-assisted thoracoscopic wedge resection of the right sequestration using near-infrared fluorescence imaging with indocyanine green because of the small volume of the nonfunctional region. The aberrant artery was recognized in the pulmonary ligament; the artery was cut off after ligation. Indocyanine green at 5 mg/body was rapidly injected into the peripheral vein, and the boundary of the sequestration was clearly identified under near-infrared fluorescence imaging. CONCLUSION: Near-infrared fluorescence imaging with indocyanine green is safe and useful for the identification of the boundary of a pulmonary sequestration.
Assuntos
Sequestro Broncopulmonar/cirurgia , Pneumonectomia/métodos , Cirurgia Torácica Vídeoassistida/métodos , Sequestro Broncopulmonar/diagnóstico por imagem , Sequestro Broncopulmonar/patologia , Corantes/administração & dosagem , Feminino , Humanos , Verde de Indocianina/administração & dosagem , Margens de Excisão , Pessoa de Meia-Idade , Tomografia Computadorizada por Raios XRESUMO
Pulmonary malformations are rare disorders, with cystic and pseudocystic pulmonary malformations (CPPM) the most frequent, and constitute the first cause of lobectomy in children <1â¯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 16.8â¯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.
Assuntos
Procedimentos Cirúrgicos Pulmonares/métodos , Anormalidades do Sistema Respiratório/diagnóstico , Anormalidades do Sistema Respiratório/cirurgia , Adolescente , Cisto Broncogênico/diagnóstico , Cisto Broncogênico/patologia , Cisto Broncogênico/cirurgia , Sequestro Broncopulmonar/diagnóstico , Sequestro Broncopulmonar/patologia , Sequestro Broncopulmonar/cirurgia , Criança , Pré-Escolar , Estudos Transversais , Malformação Adenomatoide Cística Congênita do Pulmão/diagnóstico , Malformação Adenomatoide Cística Congênita do Pulmão/patologia , Malformação Adenomatoide Cística Congênita do Pulmão/cirurgia , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Variações Dependentes do Observador , Enfisema Pulmonar/congênito , Enfisema Pulmonar/diagnóstico , Enfisema Pulmonar/patologia , Enfisema Pulmonar/cirurgia , Anormalidades do Sistema Respiratório/patologia , Estudos Retrospectivos , Centros de Atenção TerciáriaRESUMO
INTRODUCTION: Rhabdomyomatous dysplasia (RD) is a pathologic finding in CPAMs that was incorrectly attributed to their malignant potential. The increasing recognition of extrathoracic (intradiaphragmatic and intraabdominal) congenital pulmonary airway malformations (CPAMs) offers a clue to the origin of RD. We hypothesize that the presence of RD is related to the CPAM's anatomic location. MATERIALS AND METHODS: Retrospective review was performed of all children who underwent resection of a CPAM during a 10-year period. The age at the time of operation, location of the CPAM, and pathologic findings were collected. Peridiaphragmatic location was defined as within the inferior pulmonary ligament, deep to the diaphragmatic portion of the parietal pleura ("intradiaphragmatic") or adjacent to the abdominal side of the diaphragm. Statistical analysis was performed using Fisher's exact test for 2 × 2 tables. RESULTS: Twenty-six patients with CPAM were identified. Preoperative imaging was performed by computed tomography (CT) scan (16/26), ultrasound (5/26), magnetic resonance imaging (MRI) (1/26), and chest radiograph (4/26). The median age at resection was 15 months. Of these, 16 were pure cystic adenomatoid malformations, 4 were extralobar sequestrations, 4 were intralobar sequestrations, and 2 were bronchogenic cysts. Nine lesions were peridiaphragmatic with four being intradiaphragmatic (44%). Eight of the nine resected peridiaphragmatic lesions contained histologic evidence of rhabdomyomatous changes (89%, confidence interval [CI] 52-99%). None of the other lesions contained RD (CI 0-19%, p < 0.001). CONCLUSION: RD was seen exclusively, and in virtually all peridiaphragmatic CPAMs. While the exact significance of RD remains unclear, it may represent incorporation of striated muscle tissue associated with the developing diaphragm.