A ten-year review of congenital pulmonary airway malformation cases in a pediatric tertiary hospital

Objective@#A retrospective study of the demographic, clinical and diagnostic profile, intervention and outcomes of children with Congenital Pulmonary Airway Malformation (CPAM) in Philippine Children’s Medical Center (PCMC) from January 2011 to December 2021 was presented.@*Methodology@#Medical charts of identified patients were reviewed. Data obtained included demographic profile, clinical history, diagnostic procedures, intervention, and outcomes. The findings were analyzed and correlated with the synthesized findings from relevant studies about CPAM.@*Results@#Twenty-three cases (n=23) were included in the study. Most of the patients were diagnosed at 1 to 11 months of age, accounting for 43.48% while there is a minimal disparity in terms of gender distribution. Seventy-five percent of neonates presented with respiratory distress while recurrent pneumonia occurred more frequently beyond the neonatal period. Four patients at 17.39% had incidental findings of CPAM on prenatal ultrasound. All cases were confirmed using a Chest CT scan and only six patients at 26.09% had Chest X-ray results consistent with CPAM. Eight cases were confirmed using biopsy wherein results were mainly Type I seen in 87.5% of cases. Lobectomy is the procedure of choice with 92.86% success rate. Overall, patients who underwent surgical intervention had a low complication rate at 6.25%. @*Conclusion@#CPAM is most common in patients aged 1 to 11 months and has no gender predilection. Neonates often present with respiratory distress while recurrent pneumonia is the most common clinical manifestation beyond neonatal period. CPAM can be detected using ultrasound prenatally and CT scan can confirm CPAM postnatally. Surgical intervention particularly lobectomy is the preferred option over conservative management which showed a favorable outcome. CPAM has an overall good prognosis. Findings of this research may guide clinicians in the diagnosis and management of CPAM in the Philippines.

Malformación congénita de la vía aérea pulmonar: a propósito de un caso clínico / Congenital pulmonary airway malformation: a clinical case study / Malformação congênita das vias aéreas pulmonares: relato de caso

La malformación congénita de la vía aérea pulmonar (MVAP), antes llamada malformación adenomatoidea quística pulmonar, es una rara anormalidad del desarrollo de las vías respiratorias terminales. Las lesiones son de distribución y tamaños variables, usualmente unilaterales. El diagnóstico puede realizarse desde el período prenatal mediante ecografía gestacional, encontrándose, en ocasiones, graves repercusiones fetales. En los recién nacidos la enfermedad puede manifestarse con dificultad respiratoria aguda. En niños y adultos puede diagnosticarse ante infecciones pulmonares recurrentes u otras complicaciones. En pacientes sintomáticos está indicado el tratamiento quirúrgico para prevenir infecciones y la transformación neoplásica; sin embargo, sigue siendo controversial el tratamiento profiláctico frente al tratamiento expectante en pacientes asintomáticos. Se presenta el caso clínico de una lactante de 2 meses, que en el curso de una bronquiolitis se realizó una radiografía de tórax que evidenció una imagen radiolúcida del lóbulo medio. La tomografía computada visualizó gran imagen quística en pulmón derecho, que podría corresponder a una MVAP. Se decidió tratamiento quirúrgico coordinado. Se realizó una segmentectomía, confirmándose con anatomía patológica una MVAP tipo IV. Evolucionó favorablemente.

Congenital pulmonary airway malformation (CPAM), formerly called pulmonary cystic adenomatoid malformation, is a rare developmental abnormality of the terminal airways. Lesions are of variable size and distribution, usually unilateral. The diagnosis can be made from the prenatal period by means of gestational ultrasound, occasionally causing serious fetal repercussions. In newborns, the disease may manifest itself through acute respiratory distress. In children and adults it can be diagnosed through recurrent lung infections or other complications. In symptomatic patients, surgical treatment is indicated to prevent infections and neoplastic transformation. However, prophylactic versus expectant management in asymptomatic patients remains controversial. We present the clinical case of a 2-month-old infant who, during the course of bronchiolitis underwent a chest X-ray that revealed a radiolucent image of the middle lobe. The computed tomography scan showed a large cystic image in the right lung, which could be linked to an CPAM. The surgical treatment was carried out and the segmentectomy confirmed a pathological type IV CPAM. The patient evolved favorably.

A malformação congênita das vias aéreas pulmonares (MVAP), anteriormente chamada de malformação adenomatoide cística pulmonar, é uma anormalidade rara do desenvolvimento das vias aéreas terminais. As lesões são de tamanho e distribuição variáveis, geralmente unilaterais. O diagnóstico pode ser feito desde o pré-natal por meio da ultrassonografia gestacional, podendo encontrar repercussões fetais graves. Em recém-nascidos, a doença pode se manifestar com dificuldade respiratória aguda. Em crianças e adultos, pode ser diagnosticada com infecções pulmonares recorrentes ou outras complicações. Em pacientes sintomáticos, o tratamento cirúrgico é indicado para prevenir infecções e transformações neoplásicas; no entanto, o tratamento profilático versus expectante em pacientes assintomáticos permanece controverso. Apresentamos o caso clínico de uma criança de 2 meses que foi submetida a radiografia de tórax durante bronquiolite, que revelou imagem radiotransparente do lobo médio. A tomografia computadorizada mostrou grande imagem cística em pulmão direito, que poderia corresponder a uma MVAP. O tratamento cirúrgico foi realizado e a segmentectomia confirmou um tipo IV de MVAP com patologia. A criança evoluiu favoravelmente.

Use of Corticosteroids in Prenatal Treatment of Congenital Pulmonary Adenomatoid Malformation: Integrative Review / Uso de corticosteroides no tratamento pré-natal da malformação adenomatoide pulmonar congênita: Revisão integrativa

Abstract Objective To review data on the use of corticosteroids for the treatment of fetuses with high-risk congenital pulmonary adenomatoid malformation (CPAM). Methods Integrative review based on the literature available onMEDLINE and LILACS, including articles published until November, 2020. Results The initial search resulted in 87 articles, 4 of which were selected for analysis, with all of them being retrospective descriptive observational studies. In the group of fetuses that received only a single corticosteroid cycle, the hydrops resolution rate was 70%, and the survival rate was 83.8%. In fetuses treated with 2 or more cycles of corticosteroids, there was an improvement in the condition of hydrops or edema in a single body compartment in 47%, and survival of 81.8% of the fetuses. Conclusion The use of corticosteroids for the prenatal treatment of high-risk CPAM appears to be associated with an improvement in perinatal outcomes.

Resumo Objetivo Revisar os dados sobre o uso de corticoide no tratamento de fetos com malformação adenomatoide pulmonar congênita (MAPC) de alto risco. Métodos Revisão integrativa com base na literatura disponível no MEDLINE e LILACS, incluindo artigos publicados até novembro de 2020. Resultados A busca inicial resultou em 87 artigos, dos quais 4 foram selecionados para análise, todos tratando-se de estudos observacionais descritivos retrospectivos. No grupo de fetos que recebeu apenas um único ciclo de corticosteroide, a taxa de resolução da hidropsia foi de 70% e a taxa de sobrevida de 83,8%. Emfetos tratados com 2 ou mais ciclos de corticosteroides, houve melhora do quadro de hidropsia ou edema em um único compartimento corporal em 47% dos fetos e taxa de sobrevida de 81,8%. Conclusão O uso de corticosteroides para o tratamento pré-natal da MAPC de alto risco parece estar associado à melhora dos resultados perinatais.

Retrospective evaluation of inpatients admitted to a tertiary hospital in Somalia for Pediatric surgery

Objective: The aim is to reflect on the epidemiology of the patient population at a tertiary hospital for pediatric surgery, diagnostic pattern, and mortality in Somalia retrospectively. Methods: In this study, 163 patient who were hospitalized to Pediatric Surgery Clinic of Mogadishu Somalia Turkey Recep Tayyip Erdogan Training and Research Hospital in 2018 were included. Data regarding age, gender, diagnosis, surgical condition, mortality rate and cause of the death were recorded from the patient charts and the institutional digital database Results: Of 163 patients 47 were female (28.8%) and 116 were male (71.2%). The mean age of the patients was 6.4 ± 4.8 years. The main diagnoses were congenital malformation (34.4%), acute abdomen (25.8%), traumatic injury (23.3%), infection (9.8%) and neoplasm (6.1%). Mortality rate was 9.8% and the leading cause of death was sepsis by 87.5%. Perforated appendicitis, intestinal obstruction and intussusception were creating the 68.7% of the diseases that result in death. Conclusions: Our results show that two-thirds of the surgical deaths could be prevented with timely presentation. We think that the health policymakers in Somalia should focus on how to improve the access to surgical care, patient transfer, timely presentation, and training of pediatric surgeons and to overcome the poor surgical outcomes

Perioperative anesthetic care of children with Congenital Cystic Adenomatous Malformation: A report of 3 cases

@#<p style="text-align: justify;">Congenital cystic adenomatoid malformation (CCAM) is a rare developmental entity. Symptomatic patients must undergo thoracic surgery for lobectomy. Perioperative anesthetic management is challenging because it involves<br />thoracotomy in a young patient that may lead to hemodynamic compromise and inadequate ventilation. We present 3 cases of pediatric patients (6 months - 2 years old) with CCAM who underwent lobectomy.</p>

Actualización y controversias en el tratamiento de las malformaciones congénitas de la vía aérea pulmonar / Update and disagreements in the treatment of congenital pulmonary airway malformations

Congenital pulmonary airway malformations (CPAM) are rare and occur due to a failure in lung embryological development. They are classified according to their pathological characteristics and their anatomical origin. They can occur from the antenatal period to adulthood, can be associated with hydrops fetalis, respiratory distress, recurrent infections, or in an otherwise asymptomatic patient. In this article we carry out a scope review of the literature to answer frequent questions of the clinical teams in charge of patients with CPAM, such as the antenatal approach, the different types of surgeries and the management of asymptomatic patients. Although the indication for surgery is clear in symptomatic patients, little is known about its natural history of this condition, including the possibility of spontaneous resolution and the development of complications or neoplasm. So, the treatment of asymptomatic patients continues to be controversial. Therapeutic decisions must be made by multidisciplinary teams with the informed participation of parents and patients. In our opinion, considering the excellent results of minimally invasive surgery, its low incidence of complications, and practically zero mortality when performed by experienced groups, it seems reasonable to consider elective resection of a MCVAP in asymptomatic patients.

Las malformaciones congénitas de la vía aérea pulmonar (MCVAP) son infrecuentes y ocurren debido a una falla en el desarrollo embriológico pulmonar. Se clasifican de acuerdo con sus características patológicas y a su origen anatómico. Se pueden presentar desde el periodo antenatal hasta la adultez, asociarse a cuadros de hidrops fetal, distrés respiratorio, infecciones recurrentes, o como un hallazgo en pacientes asintomáticos. En este artículo realizamos una revisión bibliográfica exploratoria para responder dudas frecuentes de los equipos clínicos a cargo de pacientes con MCVAP, como el enfrentamiento antenatal, los distintos tipos de cirugía y su abordaje, y el manejo de pacientes asintomáticos. Si bien la indicación de cirugía es clara en pacientes sintomáticos, poco se conoce acerca de su historia natural, incluyendo la posibilidad de resolverse de forma espontánea, de complicarse o de evolucionar hacia el desarrollo de una neoplasia, por lo que el tratamiento de pacientes asintomáticos continúa siendo controversial. Las decisiones terapéuticas deben ser tomadas por equipos multidisciplinarios con la participación informada de los padres y de los pacientes. En nuestra opinión, considerando los excelentes resultados de la cirugía mínimamente invasiva, su baja incidencia de complicaciones y prácticamente nula mortalidad, al ser realizada por grupos con experiencia, nos parece razonable plantear la resección electiva de una MCVAP en un paciente asintomático.

Malformación congénita de la vía aérea pulmonar. Reporte de un caso / Congenital malformation of the pulmonary airway. Report of a case

RESUMEN El objetivo de este estudio es realizar una revisión de la Malformación Congénita Pulmonar a propósito del caso de un feto de una paciente de 34 años que cursaba su segunda gesta, cuyo diagnóstico se realizó a la semana 30 de gestación. Además se realizó una revisión bibliográfica de la patología y de casos similares.

ABSTRACT The objective of this study is to perform a review of Congenital Pulmonary Malformation regarding a case of a fetus in a 34-year-old woman who was in her second pregnancy, whose diagnosis was made at week 30 of gestation. In addition, a bibliographic review of the pathology and similar cases was carried out

Outcomes of cases of prenatally-diagnosed congenital pulmonary airway malformation / Desfechos de casos de malformação das vias respiratórias diagnosticados no pré-natal

Abstract Objective To evaluate the outcomes of cases of prenatally-diagnosed congenital pulmonary airway malformation (CPAM). Methods We retrospectively evaluated cases of prenatally-diagnosed CPAM between 2004 and 2018. Ultrasonographic features such as visualization of a fetal lung mass and heterogeneous pulmonary parenchyma were used for CPAM diagnosis. Prenatal and postnatal findings were compared in terms of accuracy regarding the CPAM diagnosis. Results The sample consisted of 27 cases. There were four cases in which the patients opted for the termination of pregnancy due to the severity of the lesion. A total of 23 neonates were delivered, and CPAM was confirmed in 15 cases. Themedian gestational age at delivery was 37 weeks (28-40 weeks) and the mean birth weight was 2,776 g. There were two neonatal deaths, one due to pneumothorax, and the other due to hypoplastic left heart syndrome (HLHS). A total of five patients with respiratory problems were operated in the postpartum period. There were eight misdiagnosis: bronchopulmonary sequestration (five cases), congenital lobar emphysema (two cases), and congenital diaphragm hernia (one case). Conclusion A precise postnatal diagnosis is very important to organize the proper management of the pregnancies with fetuses with CPAM. The positive predictive value of the prenatal diagnosis of CPAM via ultrasonography is of 70.3%. The differential diagnosis of CPAM may be prolonged to the postpartum period in some cases.

Utilidad de la quimioterapia con corticoides en la reducción de volumen de las lesiones en fetos con malformación congénita de la vía aérea pulmonar (MCVAP): serie de casos / Usefulness of chemotherapy with corticosteroids in reducing the volume of the lesions in fetuses with congenital pulmonary airway malformation (MCVAP): case series

RESUMEN OBJETIVO: el propósito es describir el efecto de los corticoides prenatales en la malformación congénita de la vía aérea pulmonar (MCVAP) mediante la evaluación secuencial del CVR y mostrar la experiencia en nuestro centro de terapia fetal. MATERIALES Y MÉTODOS: serie de casos en la cual se incluyeron ocho pacientes del Hospital de San José; con diagnóstico de MCVAP; se evaluó edad gestacional, el CVR (longitud x largo x ancho, en cms de la masa) x 0.523/circunferencia cefálica) como factor pronóstico, la desviación mediastínica, la presencia de hidrops, el tipo de MCVAP y la localización. Se aplicó betametasona a dosis de inducción de madurez pulmonar fetal. Se realizó la medición del CVR pre y pos tratamiento. Los datos obtenidos fueron analizados mediante el software estadístico SPSS (Versión 11.0 SPSS Inc. Chicago, IL). Se utilizaron la prueba Wilcoxon y el índice de correlación de Spearman según el caso. Un valor de p<0.05 se consideró significativo. RESULTADOS: ocho pacientes fueron incluidas. La mediana de la edad gestacional fue 30.5 semanas. Cuatro casos fueron tipo I, dos tipo II y dos tipo tres. Cuatro casos presentaron desviación mediastinal. Cinco casos fueron derechos y uno bilateral. Ningún caso presentó hidrops. El CVR pre tratamiento fue 0,93(RIQ:0,17-2,1) y pos tratamiento 0,55(RIQ:0,07-1,39). Se encontró una disminución del CVR pos tratamiento en todos los casos (p 0.0117) e índice de correlación de Spearman 0.9524 CONCLUSIÓN: Después de la aplicación de corticoides encontramos una disminución del CVR en todos los casos descritos. El uso de corticoides podría ser parte de la terapia prenatal con miras a mejorar el pronóstico.

ABSTRACT OBJECTIVE: The purpose of this study is to describe the effect of prenatal corticosteroids in the CPAM by sequential evaluation of the CVR, and to show the experience in our fetal therapy center. MATERIALS AND METHODS: Series of cases in which eight patients from the Hospital of San José were included; with diagnosis of CPAM; gestational age was evaluated, CVR (CVR = length x length x width cm, mass) x 0.523 / head circumference) as a prognostic factor, mediastinal shift, hydrops, type of CPAM, location. Betamethasone was applied to fetal lung maturation dose. In all patients underwent CVR measurement pre and post treatment. The data were analyzed using SPSS statistical software (Version 11.0 SPSS Inc. Chicago, IL). The Wilcoxon test and the Spearman correlation index were used according to the case. A value of p <0.05 was considered significant. RESULTS: eight patients were included. The median of gestational age was 30.5 weeks. Four cases were type I, two type II and two type three. Four cases were mediastinal shift. Five cases were rights and one bilateral. No case presented hydrops. The pretreatment CVR was 0,93(RIQ: 0,17-2,1) and post-treatment 0,55(RIQ: 0,07-1,39). We found a decrease in CVR after treatment in all cases (p 0.0117) and Spearman correlation index 0.9524 CONCLUSION: After application of corticosteroids are a reduction in CVR in all the cases described. The use of corticosteroids may be part of prenatal therapy to improve prognosis.

Evaluation of Newborn Infants with Prenatally Diagnosed Congenital Pulmonary Airway Malformation: A Single-Center Experience

PURPOSE: Congenital pulmonary airway malformation (CPAM)—a rare developmental anomaly—affects the lower respiratory tract in newborns. By comparing the reliability of diagnostic tools and identifying predictive factors for symptoms, we provide comprehensive clinical data for the proper management of CPAM. METHODS: We reviewed the medical records of 66 patients with prenatally diagnosed CPAM delivered at Severance Children's Hospital between January 2005 and July 2017. RESULTS: We enrolled 33 boys and 33 girls. Their mean gestational age and birth weight were 38.8 weeks and 3,050 g, respectively. Prenatal ultrasonography and postnatal radiography, lung ultrasonography, and chest computed tomography (CT) showed inconsistent findings. Chest CT showed superior sensitivity (100%) and positive predictive value (90%). Among the 66 patients, 59 had postnatally confirmed CPAM, three had pulmonary sequestration, one had cystic teratoma, and one had a normal lung. Of the 59 patients with CPAM, 21 (35%; mean age, 23.4 months) underwent surgery, including 15 who underwent video-assisted thoracoscopy. Twenty-five and 12 patients exhibited respiratory symptoms at birth and during infancy, respectively. Apgar scores and mediastinal shift on radiography were significantly associated with respiratory symptoms at birth. However, none of the factors could predict respiratory symptoms during infancy. CONCLUSION: Radiography or ultrasonography combined with chest CT can confirm an unclear or inconsistent lesion. Apgar scores and mediastinal shift on radiography can predict respiratory symptoms at birth. However, symptoms during infancy are not associated with prenatal and postnatal factors. Chest CT combined with periodic symptom monitoring is important for diagnosing and managing patients with prenatally diagnosed CPAM and to guide appropriate timing of surgery.

Congenital pulmonary airway malformation

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.

Diagnóstico ecográfico prenatal y resultados neonatales de la malformación adenomatoidea quística pulmonar / Antenatal ultrasound diagnosis and neonatal results of the congenital cystic adenomatoid malformation of the lung

INTRODUCCIÓN: La malformación adenomatoidea quística (MAQ) es una patología congénita del pulmón poco frecuente y su diagnóstico prenatal es factible en la mayoría de los casos. Existen discrepancias en cuanto al manejo prenatal y tratamiento postparto. OBJETIVO: Analizar una serie de casos de MAQ diagnosticados prenatalmente, evaluando los hallazgos ecográficos y la evolución fetal y post natal. PACIENTES Y MÉTODO: Estudio retrospectivo de todos los casos diagnosticados prenatalmente por ecografìa entre 2005 y 2016 en dos hospitales de referencia. Los exámenes de ultrasonido fueron realizados utilizando ecógrafos de alta resolución, Toshiba Xario y Voluson 730 Expert Pro, con seguimiento desde el diagnóstico hasta el parto. Las variables analizadas incluyeron la edad gestacional en el momento del diagnóstico, las características de la lesión pulmonar, las malformaciones asocia das, el estudio citogenético, la evolución del embarazo, el tipo de parto, presencia de distrés respira torio, necesidad de pruebas de imagen complementarias, evolución clínica pediátrica y tratamientos postnatales necesarios. Se consideró resolución la desaparición total de la lesión ecográfica prenatal o que la radiografía torácica postnatal no mostrara lesión alguna. RESULTADOS: Se identificaron pre natalmente 17 casos. La evolución varía desde la resolución prenatal de la lesión hasta la persistencia de la misma tras el nacimiento. Tres pacientes decidieron abortar voluntariamente por hallazgos ecográficos de mal pronóstico. De los catorce casos restantes no hubo ningún caso de muerte fetal ni neonatal, un caso requirió cirugía tras el nacimiento y cuatro pacientes presentaron sintomatología leve durante el primer año de vida. Se ha reportado un caso de falso negativo con muerte neonatal, que la necropsia informó como MAQ tipo 0. CONCLUSIONES: Esta malformación pulmonar presenta buen pronóstico, excluyendo los casos con hidrops fetal. La ecografía bidimensional suele ser suficiente para el diagnóstico y el seguimiento. La tomografía computarizada es la técnica de elección para confirmar la resolución de las lesiones tras el nacimiento. El tratamiento quirúrgico es preferible sobre el manejo conservador, aunque se desconoce si las complicaciones potenciales de esta patología, aun siendo asintomática, justifican la morbilidad quirúrgica.

INTRODUCTION: Congenital cystic adenomatoid malformation (CCAM) is a rare congenital lung di sease, and in the most of cases, prenatal diagnosis is feasible. There are discrepancies regarding pre natal management and postpartum treatment. OBJECTIVE: To analyze prenatally diagnosed CCAM in our hospitals, in order to evaluate ultrasound findings with fetal and postnatal evolution. PATIENTS AND METHOD: Retrospective study of all cases diagnosed prenatally by ultrasound between 2005 and 2016 in two reference hospitals. The ultrasounds were performed using high-resolution ultrasound scanners, Toshiba Xario and Voluson 730 Expert Pro, with follow-up from diagnosis to delivery. The variables analyzed included gestational age at diagnosis, the characteristics of the lung lesion, associated malformations, cytogenetic study, the evolution of pregnancy, type of delivery, presence of respiratory distress, need for complementary imaging tests, pediatric clinical course, and necessary postnatal treatments. It was considered a resolution the total disappearance of the lesion in the pre natal ultrasound or that the postnatal chest X-ray showed no lesion. RESULTS: 17 cases were prenatally diagnosed. The evolution ranges from the prenatal resolution of the lesion to the persistence after bir th. Three patients voluntarily decided to have an abortion due to ultrasound findings of poor progno sis. Of the fourteen remaining cases there were no cases of fetal or neonatal deaths, one case required surgery after birth and four patients had mild symptoms during the first year of life. One case of false negative with neonatal death has been reported which necropsy reported as CCAM type 0. CONCLUSIONS: This pulmonary malformation presents good prognosis, excluding cases with fetal hydrops. Two-dimensional ultrasound is usually enough for diagnosis and follow-up. Computed tomography is the technique of choice to confirm the resolution of lesions after birth. Surgical treatment is pre ferable over conservative management, although it is unknown if the potential complications of this disease, even when asymptomatic, justify surgical morbidity.

Case of mucinous adenocarcinoma of the lung associated with congenital pulmonary airway malformation in a neonate / 소아과

Congenital pulmonary airway malformation (CPAM), previously known as congenital cystic adenomatoid malformation, is a rare developmental lung abnormality associated with rhabdomyosarcoma, pleuropulmonary blastoma, and mucinous adenocarcinoma of the lung. We report an unusual case of a 10-day-old male newborn with a left lower lobe pulmonary cyst who underwent lobectomy, which revealed type II CPAM complicated by multifocal mucinous adenocarcinoma. KRAS sequencing revealed a somatic mutation in Codon12 (GGT → GAT), suggesting the development of a mucinous adenocarcinoma in the background of mucinous metaplasia. Mucinous adenocarcinoma is the most common lung tumor associated with CPAM, but it generally occurs in older children and adults. Further, all cases in the literature are of type I CPAM. This case in a neonate indicates that malignant transformation can occur very early in type II CPAM.

Malformación adenomatoidea quística y riesgo de tumores malignos pulmonares / Adenomatoid lung malformation and malignant tumors

Cystic adenomatoid malformation is the most frequent congenital pulmonary malformation. The usual treatment is surgical resection. However there is controversy over management in asymptomatic patients. The possible malignization would justify surgery of cystic lesions. Relation with pleuropulmonary blastoma has been described, however it is not clear whether this is a primary tumor or cyst malignization. Cystic adenomatoid malformation also has association with adenocarcinoma and rhabdomyosarcoma. Currently available evidence suggests surgical resection, despite the natural course of congenital lung cystic lesions is uncertain

La malformación adenomatoidea quística (MAQ) es la anomalía del desarrollo pulmonar más frecuente. El tratamiento habitual es la resección quirúrgica, no obstante existe controversia sobre el manejo en pacientes asintomáticos. La posible malignización de las lesiones quísticas es uno de los argumentos que justifican la cirugía en estos pacientes. Se ha descrito relación con blastoma pleuropulmonar, sin embargo no está claro si se trataría de una lesión quística que se maligniza o es una entidad diferente. También hay asociación con adenocarcinoma y rabdomiosarcoma . Actualmente se sugiere la resección quirúrgica como el tratamiento más adecuado, sin embargo la evolución natural de las lesiones quísticas pulmonares congénitas es incierta

Minimally invasive surgery for congenital cystic adenomatoid malformations - early experience

PURPOSE: The aim of this study is to present our experience with minimally invasive surgery (MIS) for congenital cystic adenomatoid malformations (CCAMs). METHODS: The medical records of infants under 2 years of age who underwent operation for a CCAM from 2009 to 2014 were retrospectively reviewed. RESULTS: MIS (9 of thoracoscopy and 1 of laparoscopy) was performed for 10 infants (male:female = 7:3) with CCAM. CCAM were discovered prenatally around gestational age of 24.7 weeks. The median gestational age was 38.6 weeks, and the median body weight was 2,817.5 g. None had respiratory distress after birth. The median age at the time of operation was 0.94 years (range: 8 days-1.66 years). Two underwent the operation during the neonatal period; one because of a coexisting large esophageal duplication cyst and the other due to diagnostic uncertainty. While awaiting operation, 5 of CCAM had grown without respiratory symptoms, and 2 infants had experienced pneumonia. The mean operative time was 98 minutes (range: 70-227 minutes), and there were no conversions or perioperative complications. The infants resumed enteral feeding within 2 days and were discharged within 7 days, except for 1 infant who underwent esophageal duplication cyst excision. During the follow-up period, there were no cases of either remnant lesions or respiratory symptoms. CONCLUSION: MIS for CCAMs is safe and feasible, with excellent cosmesis and short hospital stays. Increasing experience with various MIS procedures will widen the indications for MIS in lung pathology.

Pulmonary Arteriovenous Fistula: Clinical and Histologic Spectrum of Four Cases

Pulmonary arteriovenous fistula (PAVF) is abnormally dilated vessels that provide a right-to-left shunt between pulmonary artery and pulmonary vein and is clinically divided into simple and complex type. Here, we report four cases of surgically resected sporadic PAVFs presenting various clinical and histologic spectrums. Cases 1 (a 57-old-female) and 2 (a 54-old-female) presented as incidentally identified single aneurysmal fistulas and the lesions were surgically removed without complication. On the other hand, case 3 (an 11-old-male) showed diffuse dilated vascular sacs involving both lungs and caused severe hemodynamic and pulmonary dysfunction. Embolization and surgical resection of the main lesion failed to relieve the symptoms. Case 4 (a 36-old-male) had a localized multiloculated cyst clinically mimicking congenital cystic adenomatoid malformation. Microscopically, the lesion consisted of dilated thick vessels, consistent with the diagnosis of fistulous arteriovenous malformation/hemangioma.

Life-Threatening Congenital Cystic Adenomatoid Malformation in the Premature Neonate

Congenital cystic adenomatoid malformation is a rare, but well-known disease. It can be managed conservatively in patients without symptoms or require surgical removal when symptomatic. The surgical option of choice is en bloc resection of the affected lesion. We report an experience of life-threatening congenital cystic adenoid malformation in a low-birth-weight (1,590 g) premature neonate who was successfully treated with a lobectomy of the lung.

Malformación congénita de la vía aérea pulmonar o malformación adenomatoidea quística congénita: Serie clínica de 16 pacientes / Congenital pulmonary airway malformation or congenital cystic adenomatoid malformation

Objective: To describe 16 patients treated for a congenital pulmonary anomaly named congenital pulmonary airway malformation (CPAM) or congenital cystic adenomatoid malformation (CCAM) in Roberto del Río children S Hospital. Methods: We retrospectively analyzed the medical records of patienes who had had surgery for CPAM/CCAM from January 2000 to July 2014. Results: We found 16 cases, 9 of them were males. Nine had surgery within the first year of life (5 of them within 3 weeks of life). According to Stocker 's classification CPAM/CCAM was type 1 in 9 patients, and type 2 in 7. Diagnosis was based on antenatal ultrasonography s (n = 10), persistent abnormalities in Chest X-Ray (n = 4), recurrent pneumonia (n = 1) and incidental radiological findings (n = 1). Computed Tomography (CT) was performed in 13 patients, CT angiography in 2 and only chest x-ray in one. The lesions were located in right upper lobe (3), right middle lobe (2), right lower lobe (4), left upper lobe (4) and left lower lobe (3). One patient had pectus excavatum and another one had ventricuar septal defect. They all had open thoracotomy with lobectomy of the affected area. Postoperatively was uneventful except for transient lobar atelectasis in 2 patients. There was no mortality. Conclusions: We emphasize the early diagnosis and good outcome of our patients treated which this disease. Our findings are consistent with those that has been reported in the literature.

Objetivo: Describir los pacientes tratados por malformación congénita de la vía aérea pulmonar (MCVAP) o malformación adenomatoidea quística congénita (MAQC) en el Hospital de niños Roberto del Río desde el año 2000 hasta el segundo semestre de 2014. Métodos: Revisión retrospectiva de fichas clínicas. Resultados: 16pacientes, 9 hombres. Se operaron antes del año de vida 9 pacientes (5 de ellos antes de las 3 semanas de vida). Tipo de MCVAP (según clasificación de Stocker) tipo 1 fue 9/16 y tipo 2 fueron 7/16. La sospecha diagnóstica fue en base a diagnóstico prenatal 10/16, imagen radiológica persistente 4/16, neumonías repetidas 1/16 y hallazgo radiológico 1/16. Estudio imagenológico: Tomografia computada (TC) 13/16, Angio-TC 2/16y radiografía de tórax 1/16. Ubicación: lóbulo superior derecho 3/16, lóbulo medio 2/16, lóbulo inferior derecho 4/16, lóbulo superior izquierdo 4/16, lóbulo inferior izquierdo 3/16. Comorbilidad: ninguna 14/16, pectus excavatum 1/16, comunicación interventricular 1/16. Tratamiento quirúrgico: en todos los pacientes se realizó lobectomía total del lóbulo afectado por vía abierta. Evolución postoperatoria: Buena 14/16, atelectasia 2/16. No se registró mortalidad. Conclusiones: Se destaca el diagnóstico precoz y buena evolución de nuestros pacientes concordantes con la literatura.

The Clinical Courses of Patients with Congenital Cystic Adenomatoid Malformation Complicated by Pneumonia

PURPOSE: We evaluated the clinical characteristics and courses of patients with congenital cystic adenomatoid malformation (CCAM) complicated by pneumonia. MATERIALS AND METHODS: We retrospectively reviewed the records of 19 adult patients with surgically confirmed CCAM between March 2005 and July 2013. RESULTS: Eighteen of nineteen patients presented with acute pneumonia symptoms and signs, and inflammatory markers were elevated. On chest computed tomography, all 18 patients had parenchymal infiltration around cystic lesions, 17 (94%) had an air-fluid level, and 2 (11%) had pleural effusion. After antibiotics treatment for a median of 22 days prior to surgery, all acute pneumonia symptoms and signs disappeared in 17 (94%) patients at a median of 10 days. Improvements and normalization of inflammatory marker levels, occurred in 17 (94%) and 9 (50%) patients at medians of 8 and 17 days, respectively. Radiological improvement was evident in 11 (61%) patients, at a median of 18 days, of these patients, partial radiological improvement occurred in 10 (56%) and complete radiological resolution in only 1 (6%). One patient (6%) did not improve in terms of clinical, laboratory, or radiological findings despite antibiotic treatment for 13 days. Consequently, after 17 (94%) elective and 1 (6%) emergency surgeries, all patients improved without development of complications. CONCLUSION: We described the clinical characteristics and courses of patients with CCAM complicated by pneumonia, and showed that surgery may be performed safely after clinicolaboratory improvement is attained upon antibiotic treatment, even in the absence of complete radiological resolution.