Diagnosis and treatment of 131 adult patients with bronchopulmonary sequestration: A retrospective analysis / 中国胸心血管外科临床杂志

@#Objective    To explore the safety and feasibility of uni-portal video-assisted thoracic surgery (VATS) for the treatment of bronchopulmonary sequestration (BPS). Methods    The clinical data of BPS patients with surgical resection in Shanghai Pulmonary Hospital from February 2010 to June 2021 were reviewed. The patients were divided into a VATS group and a thoracotomy group according to the operation method. The operation time, intraoperative blood loss, hospital stay and postoperative complication rate were compared between the two groups. The VATS group was subdivided into a uni-portal VATS group and a multi-portal VATS group for subgroup analysis. Results    Finally 131 patients were enrolled, including 62 males and 69 females with an average age of 39.3±13.2 years. There were 103 patients in the VATS group and 28 patients in the thoracotomy group. A total of 104 patients were diagnosed with left lower BPS, 26 with right lower BPS and 1 with bilateral lower BPS. The main symptom was cough (88 patients, 67.2%). There were 119 patients diagnosed by thoracic enhanced CT before operation. Compared with the thoracotomy group, the operation time was not statistically different (P=0.717), but the blood loss was less, the rate of postoperative complication was lower and hospital stay was shorter in the VATS group (P<0.05). The rate of conversion to open surgery in the uni-portal VATS group and multi-portal VATS group was 11.8% and 13.5%, respectively. Meanwhile, patients in the uni-portal VATS group had shorter operation time and postoperative hospital stay, less blood loss and lower postoperative complication rate than those in the multi-portal VATS group (P<0.05). Conclusion     In order to improve the rate of diagnosis, the lung enhanced CT scan should be selected as an optimal noninvasive method in adult suspected patients (especially those with solid cystic and solid lesions in the lower lobe). Uni-portal VATS is a safe and feasible method for BPS which can be widely promoted.

Congenital extralobar pulmonary sequestration supplied by pulmonary artery: a case report / 中华围产医学杂志

We report the clinical features of a case of congenital extralobar pulmonary sequestration in the left upper lobe supplied by the pulmonary artery. Prenatal ultrasound examination at 24 weeks of gestation revealed a high echogenic and uniform density mass in the fetal left thoracic cavity with the congenital pulmonary airway malformation volume vatio (CVR) of 1.16, which was supplied by pulmonary arterial vessels. MRI examination at 27 weeks indicated that the left lung volume increased to about 48.52 ml, while the right lung volume was about 8.56 ml giving the total lung volume of 57.08 ml. The congenital pulmonary airway malformation in the left upper lobe was suspected to be congenital bronchial atresia (CBA) or congenital lobar overinflation (CLO). The baby boy was born through vaginal delivery assisted by forceps at 38 +1 weeks without neonatal asphyxia. Postnatal CT and MRI were both indicated suspicious bronchial atresia in the left upper lobe. Bronchofibroscopy on postnatal day 2 excluded CBA or CLO and extralobar pulmonary sequestration was considered. Thoracoscopic surgery was performed due to continuous shortness of breath after birth, despite two-week conservative treatment including oxygenation, invasive and non-invasive mechanical ventilation,etc, and congenital extralobar sequestration was diagnosed. Blood supply from the left pulmonary artery was observed at the base of abnormal lung tissue. Resection of the pathogenic tissue of the left lung was performed thoracoscopically. The boy recovered and was discharged after the operation. Pulmonary sequestration was confirmed by histopathology.

Endovascular treatment of a right pulmonary sequestration supplied by an aneurysmal aberrant artery originating from the abdominal aorta / Tratamento endovascular de sequestro pulmonar direito irrigado por artéria aneurismática aberrante originária da aorta abdominal

Abstract Endovascular embolization of arteries feeding pulmonary sequestrations is a growing therapeutic option. A 51-year-old woman with chest pain and hemoptysis was admitted. During hospitalization she presented 150 mL hemoptysis, hypotension, and hematocrit fell to 23.3%. Contrast-enhanced computed tomography confirmed a pulmonary sequestration irrigated by an aneurysmal artery from the abdominal aorta. The patient underwent endovascular coil embolization of the artery feeding the aneurysm and an Amplatzer device was deployed in the proximal third of the sequestration artery. Subsequent contrast-enhanced computed tomography confirmed complete thrombosis of the aberrant artery feeding the aneurysm and absence of irrigation of the pulmonary sequestration. At 56 months follow-up the patient remains asymptomatic, tomography showed involution of the sequestration and complete thrombosis of the aberrant artery. The challenges presented by the different treatment alternatives are discussed.

Resumo A embolização endovascular das artérias que alimentam os sequestros pulmonares é uma opção terapêutica em crescimento. Uma mulher de 51 anos com dor torácica e hemoptise foi internada. Durante a internação, ela apresentou hemoptise de 150 mL, hipotensão e queda do hematócrito para 23,3%. A tomografia computadorizada com contraste confirmou um sequestro pulmonar irrigado por uma artéria aneurismática originária da aorta abdominal. A paciente foi submetida a embolização endovascular da artéria que alimentava o aneurisma com uso de coils e dispositivo Amplatzer no terço proximal da artéria sequestrante. A tomografia subsequente confirmou a trombose completa da artéria aberrante que alimentava o aneurisma e a ausência de irrigação dentro do sequestro pulmonar. No seguimento de 56 meses, a paciente permanecia assintomática, e a tomografia mostrou involução do sequestro e trombose completa da artéria aberrante. Os desafios apresentados pelas diferentes alternativas de tratamento são discutidos neste artigo.

Malformación adenomatoidea quística congénita asociada a secuestro broncopulmonar en un recién nacido / Congenital cystic adenomatoid malformation presenting with bronchopulmonary sequestration in a newborn

Resumen Antecedentes: La malformación adenomatoidea quística y el secuestro broncopulmonar son malformaciones que se pueden diagnosticar prenatalmente. Los adecuados controles prenatales ayudan a un diagnóstico temprano y su manejo. Objetivo: Presentar el caso de un recién nacido con diagnóstico parental de una malformación pulmonar y su desenlace posterior. Reporte de caso: Recién nacido a término con diagnóstico antenatal de malformación pulmonar, el cual se confirmó tras su nacimiento. Discusión: Estas dos malformaciones son entidades cuya incidencia viene aumentando, razón por la cual es importante el conocimiento del curso clínico, ayudas diagnósticas, complicaciones y las posibilidades de manejo con las que se cuenta.

Abstract Background: Cystic adenomatoid malformation and bronchopulmonary sequestration are malformations that can be diagnosed prenatally. Appropriate prenatal controls helps to early diagnosis and management. Objective: To present the case of a newborn with a parental diagnosis of a pulmonary malformation and its subsequent outcome. Case report: Newborn at term with antenatal diagnosis of pulmonary malformation which was confirmed postnatal. Discussion: These two malformations are entities whose incidence is increasing, which is why knowledge of the clinical course, diagnostic aids, complications, and the management possibilities that are available are important.

Secuestro pulmonar intrapulmonar / Intrapulmonary pulmonary sequestration

RESUMEN El secuestro pulmonar es una de las malformaciones pulmonares más comunes en el adulto joven. Este puede ser originado a partir de un brote pulmonar accesorio que migra junto al desarrollo del esófago, o puede ocurrir por infecciones pulmonares repetitivas; sus manifestaciones clínicas son muy variables y su detección es un hallazgo radiológico. Se presentó un paciente joven con ingresos múltiples por neumonía en el Hospital Universitario Dr. Celestino Hernández Robau. En la radiografía de tórax se apreció una imagen no homogénea en la base pulmonar derecha, que no mejoró a pesar de múltiples tratamientos antimicrobianos de amplio espectro. En una tomografía axial computarizada se observó un grueso vaso que partía de la aorta abdominal hasta la base pulmonar derecha. Se realizó reconstrucción de imágenes y con el diagnóstico de secuestro pulmonar se intervino quirúrgicamente. El paciente tuvo una evolución satisfactoria.

ABSTRACT Pulmonary sequestration is one of the most common lung malformations in young adults. This can be originated from an accessory lung bud that migrates along the development of the esophagus, or it can occur by repetitive pulmonary infections; its clinical manifestations are highly variable and its detection is a radiological finding. We present a young patient with multiple admissions for pneumonia at "Dr. Celestino Hernández Robau" University Hospital. Chest X-ray showed a non-homogeneous image in the right lung base, which did not improve despite several broad-spectrum antimicrobial treatments. A thick vessel that started from the abdominal aorta to the right pulmonary base was observed through a computerized axial tomography. Image reconstruction was performed and with the diagnosis of pulmonary sequestration, surgery was performed. The patient had a satisfactory evolution.

Secuestro pulmonar intralobar y aspergilosis pulmonar: reporte de un caso / Intralobar pulmonary sequestration and pulmonary aspergilosis: a case report

El secuestro pulmonar es un tipo de malformación congénita pulmonar poco frecuente, con presentación clínica variable y de usual diagnóstico durante la infancia. Puede coexistir con otras patologías pulmonares, principalmente de tipo infecciosas y su manejo definitivo es mediante un procedimiento quirúrgico. Se presenta el caso de una paciente adulta con secuestro pulmonar, asociado a una infección fúngica, manejada con cirugía mínimamente invasiva

Pulmonary sequestration is a rare type of congenital pulmonary malformation, with variable clinical presentation and usually diagnosed during childhood. It can coexist with other pulmonary pathologies, mainly infectious, and its definitive management is through a surgical procedure. We present the case of an adult patient with pulmonary sequestration associated with a fungal infection, managed with minimally invasive surgery

Clinical analysis of 96 cases of congenital cystic lung lesions in children / 中华儿科杂志

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.

Manejo quirúrgico del secuestro pulmonar complicado. Reporte de caso / Surgical management of the complicated pulmonary sequestration. A case report

Introducción: el secuestro pulmonar es una patología poco frecuente en la edad adulta, su mayor incidencia es en etapas tempranas de la vida. Se acompaña de infecciones pulmonares a repetición o crónicas severas, y puede asociarse a malformaciones congénitas. El diagnóstico se fundamenta en la detección de la irrigación anómala a través de un vaso de la circulación sistémica. Este caso nos permite plantear un diagnóstico diferencial en pacientes de edad adulta, aún cuando se trate de patología con mayor incidencia en niños y jóvenes, y poder orientar el manejo de acuerdo a esta posibilidad diagnóstica. Caso clínico: paciente femenino, de 19 años, con antecedente de asma e infecciones respiratorias a repetición de un mes de evolución. Ingresa con dificultad respiratoria, fiebre y tos productiva. Se realiza tomografía y Radiografía de tórax, en la que se evidencia imágenes compatibles con colección en campo pulmonar izquierdo y derrame pleural. Se realiza BAAR en esputo con resultado negativo para tuberculosis. Se realiza toracotomía posterolateral izquierda, resección de lóbulo inferior izquierdo, y se coloca dren pleural. Se identifica absceso pulmonar en lóbulo inferior izquierdo, y hallazgos anatómicos de secuestro pulmonar. Paciente presenta evolución favorable; el control clínico y radiográfico en el postoperatorio inmediato y mediato fue satisfactorio. Conclusión: presentamos el caso de edad adulta, donde un diagnóstico adecuado y un manejo multidisciplinario permiten una evolución satisfactoria de los pacientes.(AU)

Introduction: pulmonary sequestration is a rare disease in adulthood; its highest incidence is in early stages of life. It is accompanied by repeated or severe chronic lung infections, and may be associated with congenital malformations. The diagnosis is based on the detection of anomalous irrigation through a vessel of the systemic circulation.This case allows us to propose a differential diagnosis in patients of adulthood, even when it is pathology with higher incidence in children and young people, and guide the management according to this diagnostic possibility.Clinical case: female patient, 19 years old, with a history of asthma and respiratory infections after a month of evolution. The patient was admitted with respiratory distress, fever and productive cough.A tomography and chest X-ray are performed; that show images compatible with collection in the left pulmonary field and pleural effusion. BAAR is performed in sputum with a negative result for tuberculosis.Posterolateral left thoracotomy is performed, left lower lobe resection, and pleural drain is placed. Pulmonary abscess is identified in the left lower lobe, and findings of anatomical pulmonary sequestration. Patient presents positive evolution; The clinical and radiographic control in the immediate and intermediate postoperative period was satisfactory.Conclusion: we present the case of adulthood, where an adequate diagnosis and multidisciplinary management allows a satisfactory evolution of the patient.(AU)

Secuestro broncopulmonar. Diagnóstico prenatal, factores pronóstico y tratamiento por cirugía fetal / Bronchopulmonary Sequestration. Prenatal diagnosis, prognostic factors and treatment by fetal surgery

Resumen ANTECEDENTES: El secuestro broncopulmonar suele diagnosticarse antes del nacimiento como una masa sólida intratorácica, homogénea e hiperecogénica, casi siempre localizada en el lóbulo inferior pulmonar. Su signo patognomónico es la demostración con Doppler color de la arteria nutricia sistémica dentro de la masa pulmonar. La historia natural de la enfermedad ha demostrado que durante la vida fetal en la mayoría de los casos la masa puede tener regresión espontánea. Una proporción importante de casos tiene rápido crecimiento de la masa con hidrotórax, compresión pulmonar e hidrops. Estos casos son de mal pronóstico y elevada mortalidad perinatal y, en estas circunstancias, la cirugía fetal es la única opción que puede mejorar el pronóstico. Para este propósito se han intentado varias técnicas de cirugía fetal pero la ideal sigue siendo un tema de controversia. OBJETIVO: Describir los métodos diagnósticos, factores pronóstico y discutir las repercusiones perinatales de cada una de las diferentes técnicas de cirugía fetal descritas en la bibliografía para el tratamiento de fetos complicados con secuestro broncopulmonar. METODOLOGÍA: La búsqueda se efectuó en Medline y PubMed de todos los artículos en inglés y español publicados entre 1990 y 2019 que tuvieran las palabras clave "bronchopulmonary sequestration", "secuestro broncopulmonar", "fetal surgery" y "cirugía fetal". Se eligieron los que describían casos de secuestro broncopulmonar tratados con alguna técnica de cirugía fetal. RESULTADOS: Se encontraron 150 artículos pero solo se seleccionaron 29 estudios que describían casos con diagnóstico prenatal de secuestro broncopulmonar tratados con alguna técnica de cirugía fetal. Para tratar el secuestro broncopulmonar grave se describieron varias técnicas de intervención fetal, entre ellas: cirugía fetal abierta, colocación de catéter de derivación toraco-amniótica, oclusión del vaso nutricio sistémico mediante guía ecográfica con láser, radiofrecuencia, embolización o escleroterapia con inyección de alcohol intravascular e, incluso, broncoscopia fetal. CONCLUSIONES: Al parecer la coagulación láser del vaso nutricio es la técnica con mejores resultados perinatales que evita la muerte fetal, la morbilidad pulmonar y la necesidad de cirugía posnatal en prácticamente todos los casos tratados; se propone como el único tratamiento potencialmente curativo.

Abstract BACKGROUND: The bronchopulmonary sequestration (BPS) is usually diagnosed prenatally as an intrathoracic hyperechoic, homogeneous, solid mass usually located in the lower pulmonary lobe. The pathognomonic sign is the demonstration of its systemic feeding artery into the lung mass by using color Doppler ultrasound. Natural history data reports that a significant proportion of BPS cases usually regress in the intrauterine period. However, a proportion of cases showed a rapid and progressive growth with development of massive pleural effusion and hydrops. Such cases present the poorest prognosis and high perinatal mortality and therefore, fetal intervention should be considered to improve prognosis. Different fetal surgery procedures have been attempted for such purposes. However, the optimal fetal therapeutica strategy remains controversial. OBJECTIVE: In the present manuscript, we describe and discuss the perinatal outcome of the different published fetal interventions in fetuses complicated with BPS. METHODOLOGY: We search in the English and Spanish literature (Medline and PubMed) for cases complicated with BPS and treated with any fetal surgical intervention including the key words "broncopulmonary sequestration" and "fetal surgery" from 1990 to 2019. RESULTS: A total of 150 references were reviewed including only 26 studies describing pregnancies with prenatal diagnosis of bronchopulmonary sequestration that were treated with any fetal surgical intervention. Several fetal surgery procedures have been attempted for the management of complicated fetuses with severe BPS. These include open fetal surgery, placement of thoracoamniotic shunts, and occlusion of the feeding blood vessel by ultrasound-guided intrafetal laser coagulation, radiofrequency ablation, coil embolization, sclerotherapy with intravascular alcohol injection and fetal bronchoscopy. CONCLUSIONS: Laser coagulation of the feeding artery appears to be the best intervention, avoiding fetal death, neonatal pulmonary morbidity and the need for postnatal surgery in virtually all cases postulating itself as the only potential curative management.

Communicating Bronchopulmonary Foregut Malformation Type III with Pulmonary Sequestration Diagnosed in a Newborn: A Case Report

Communicating bronchopulmonary foregut malformation (CBPFM) is a communication between the respiratory and gastrointestinal tracts that can be difficult to differentiate from pulmonary sequestration or H-type tracheoesophageal fistula (TEF) because of the similarities in clinical features. A female neonate born at full term had been experiencing respiratory difficulty during feeding from the third day of life. The esophagography performed to rule out H-type TEF revealed that the esophageal bronchus directly communicated with the left lower lobe (LLL) of the lung. Lobectomy of the LLL, fistulectomy of the esophagobronchial fistula, and primary repair of the esophagus were performed. Finally, CBPFM type III with pulmonary sequestration was confirmed on the basis of the postoperative histopathological finding. We report the first newborn case of CBPFM type III with pulmonary sequestration in Korea.

Cirugía fetal en tumores pulmonares mediante escleroterapia

Microcystic fetal lung tumors or masses such as the congenital cystic adenomatoid malformation (CCAM) and bronchopulmonary sequestration (SBP) are rare congenital pathologies, diagnosed by ultrasound during prenatal assessment, with a high mortality rate of 95% as a consequence of prenatal complications due to hydrops and postnatal death from pulmonary hypoplasia. Minimally invasive therapies using sclerosing agents are positioned among the low cost and highly effective techniques for such pathologies. Methodology: We conducted a prospective, randomized, blind clinical study in 17 pregnant women with diagnosis of fetal microcystic CCAM or hybrid lesion (CCAM + SBP), who were treated with betamethasone or polidocanol. Alternative therapy was employed in case of failure of the initial procedure. Results: In four (44.4%) of the nine cases treated with betamethasone, the alternative therapy of sclerosis with polidocanol was required for treatment due to fetal compromise. Involution of the lung lesion and the hydropic condition was faster and progressive with percutaneous sclerotherapy compared to the maternal steroid effect. Perinatal results were also more satisfactory in the group with polidocanol (91.7%) than in the group with the steroid (60%). Conclusions: Percutaneous sclerotherapy with polidocanol was found efficacious for CCAM and hybrid lesion treatment, with faster and more efficient resolution of the pathology in cases resistant to previously administered steroid therapy.

Las tumoraciones o masas pulmonares fetales microquísticas, tales como la malformación adenomatoidea quística congénita (CCAM, por sus siglas en inglés) y el secuestro bronco pulmonar (SBP), representan patologías congénitas poco frecuentes, diagnosticadas por ultrasonido durante la evaluación prenatal, con un alto índice de mortalidad de 95% como consecuencia de las complicaciones prenatales por hidropesía y fallecimiento posnatal debido a hipoplasia pulmonar. Las terapias de mínima invasión usando agentes esclerosantes han tomado posicionamiento entre las técnicas de bajo costo y alta efectividad para dichas patologías. Metodología. Se realizó un estudio clínico prospectivo, aleatorio, ciego, en 17 embarazadas con diagnóstico de CCAM microquística o de lesión híbrida (CCAM +SBP) tratadas con betametasona o con polidocanol, optando por la terapia alternativa en caso de fallar la técnica inicial. Resultados. En 4/9 (44,4%) de los 9 casos tratados con betametasona se requirió la terapia alternativa de esclerosis con polidocanol para la resolución del cuadro, que comprometía el estado fetal. La involución de la lesión pulmonar y del cuadro hidrópico fue más rápida y progresiva con la escleroterapia percutánea en comparación con el efecto esteroideo materno. Los resultados perinatales fueron asimismo más satisfactorios en el grupo con polidocanol (91,7%) que en el grupo con el esteroide (60%). Conclusiones. Se comprobó la eficacia de la escleroterapia percutánea con polidocanol para el tratamiento de la CCAM y de lesión híbrida, con resolución más rápida y eficaz de la patología en los casos donde existía resistencia a la terapia esteroidea previamente administrada.

Secuestro pulmonar en adultos: reporte de tres casos y revisión de la literatura / Adult bronchopulmonary sequestration: report of three cases and review of the literature

Resumen El secuestro pulmonar es una enfermedad congénita infrecuente caracterizada por la presencia de una porción de parénquima pulmonar que recibe vascularización independiente de la circulación sistémica. Se presentan tres casos de secuestro pulmonar intralobares, de presentación tardía, uno de ellos asociado a una malformación adenomatosa quística y en los cuales el manejo se completó por videotoracoscopia: dos de ellos con lobectomía total y uno con lobectomía sublobar. Se trata de una enfermedad de presentación clínica variable y cuyo tratamiento es, en la mayoría de los casos, quirúrgico, requiriéndose usualmente resecciones lobares, las cuales pueden llevarse a cabo de manera eficaz y segura por videotoracoscopia.

Abstract Pulmonary sequestration is an infrequent congenital disease characterized by the presence of a portion of pulmonary parenchyma that receives vascularization independent of the systemic circulation. We present three cases of intralobar pulmonary sequestration of late presentation, one of them associated with a cystic adenomatous malformation and in which the management was completed by videothoracoscopy: two of them with total lobectomy and the other with sublobar lobectomy. It is a disease of variable clinical presentation and whose treatment is, in most cases, surgical, usually requiring lobar resections, which can be carried out effectively and safely by video-assisted thoracoscopy.

Dolor torácico y hemotórax como presentación atípica de secuestro pulmonar en adulto / Chest pain and hemothorax: an atypical presentation of a pulmonary sequestration

Resumen El secuestro pulmonar es una malformación pulmonar rara, presentándose generalmente en edades tempranas. Se presenta mayoritariamente con neumonías e infecciones repetidas, distrés respiratorio y falla cardíaca; raramente en pacientes de mayor edad se presenta con hemoptisis y dolor torácico. En este artículo se describe el caso clínico de un paciente de 60 años de edad que se presenta con un infarto de un secuestro pulmonar y hemotórax.

Bronchopulmonary sequestration is a rare pulmonary malformation, usually occurring at an early age. It presents mainly with pneumonia and repetitive infections, respiratory distress and heart failure; rarely in aged patients presents with hemoptysis and chest pain. This article describes the clinical case of a 60-year-old male patient who presented an ischemic pulmonary sequestration and hemothorax.

Ultrasound measurement of congenital cystic adenomatoid malformation volume ratio in prenatal evaluation on prognosis in fetus with congenital pulmonary sequestrations / 中国介入影像与治疗学

Objective To investigate the value of cystic adenomatoid malformation volume ratio (CVR) using prenatal ultrasound in evaluation of congenital pulmonary sequestrations (PS) prognosis in fetus.Methods Totally 88 cases of fetal PS diagnosed with prenatal ultrasound were enrolled.The fetal CVR were recorded,and the clinical outcomes were observed.The fetus were further divided into CVR≥1.6 group and CVR＜1.6 group,then the fetal hydrops rate,incidence of respiratory distress symptoms after birth and perinatal infant survival rate between two groups were compared.Results In 88 cases,prenatal ultrasound diagnosed intralobar pulmonary sequestration (ILS) type in 62 cases (62/88,70.45％),extralobar pulmonary sequestration (ELS) type I in 19 cases (19/88,21.59％),and ELS type Ⅱ in 7 cases (7/88,7.95％).There were 81 (81/88,92.05％) live births,5 (5/88,5.68％) of induction,and 2 (2/88,2.27％) of intrauterine fetal death,respectively.In 44 cases of CVR≥1.6 group,36 cases (36/44,81.82％) had respiratory symptoms after birth,and 39 (39/44,88.64％) had combined fetal hydrops.The live birth rate was 84.09％ (37/44).In 44 cases of CVR＜1.6 group,3 cases (3/44,6.82％) had respiratory symptoms after birth and 3 (3/44,6.82％) had combined fetal hydrops.The live birth rate was 100％ (44/44).The fetal hydrops rate,incidence of respiratory symptoms after birth and perinatal infant survival rate were statistically different between the two groups (all P＜0.05).Conclusion Prenatal ultrasound CVR is an effective index for screening and assessing the prognosis in fetus with PS.

Clinical characteristics of bronchopulmonary foregut malformation / 中华儿科杂志

Objective@#To analyze the clinical characteristics, diagnosis and treatment of bronchopulmonary foregut malformation(BPFM).@*Method@#The clinical manifestations, imaging findings and treatment of 8 patients with BPFM were analyzed retrospectively from January 2006 to May 2016 in Beijing Children′s Hospital.@*Result@#The age of children varied from 2 months to 7 years and 3 children were male while 5 female. Symptoms showed cough in 6 cases, fever in 4 cases, bucking when intaking of fluids or foods in 3 cases, tachypnea in 1 case, wheezing in 1 case, vomiting in 1 case, haematemesis in 1 case Pulmonary signs were decreased breath sounds in 4 cases, phlegm rale in 3 cases, shortness of breath in 2 cases, wheeze in 1 case, and retraction in 1 case. The upper gastrointestinal series showed abnormal fistulous tracts arising from the esophagus or the gastric fundus and extending into the mass in the lung. CT showed pulmonary sequestration and prompted the tube between lung and esophagus. Six children underwent pneumonectomy and esophageal fistula repair. They were discharged and their symptoms were improved. Two cases of children were discharged from a hospital without surgery.@*Conclusion@#Bronchopulmonary foregut malformation usually has its onset in early stage of life. The most common symptoms include recurrent pneumonia or bucking when intaking of fluids or foods. CT can demonstrate the bronchopulmonary sequestration and evaluate the communication with the gastrointestinal tract. The upper gastrointestinal series can demonstrate the abnormal tract directly. Pneumonectomy and esophageal fistula repair are the treatment of this disease.

Atypical presentation of intra-abdominal extralobar pulmonary sequestration detected in prenatal care: a case report / Apresentação atípica de sequestro pulmonar extralobar intra-abdominal detectado no pré-natal: relato de caso

Objective: To describe an unusual clinical presentation of intra-abdominal extralobar pulmonary sequestration in a 2-year, 9 month-old patient and assess diagnostic and treatment aspects of this pathology. Case description: An undefined intra-abdominal mass was identified in the right adrenal region in a male fetus. Postnatal evaluation with ultrasound images, computed tomography, magnetic resonance imaging and laboratory testing was insufficient to determine the nature of the lesion. After two years, laparoscopic resection of the mass and histopathological examination of the surgical specimen allowed to establish the diagnosis of intra-abdominal extralobar pulmonary sequestration. Comments: This malformation can be monitored clinically; however, surgical excision is often performed, probably due to the impossibility of attaining diagnosis with non-invasive methods, such as in the present case, in which the lesion appeared in an unusual position for intra-abdominal extralobar pulmonary sequestration. Therefore, the surgical approach seems to be the key to attain the diagnosis and establish the conduct for this type of congenital malformation.

Objetivo: Descrever apresentação clínica incomum de sequestro pulmonar extralobar intra-abdominal em um paciente de dois anos e nove meses e avaliar aspectos diagnósticos e de tratamento dessa patologia. Descrição do caso: Uma massa intra-abdominal indefinida em topografia suprarrenal direita de feto masculino. A avaliação pós-natal com imagens de ultrassom, tomografia computadorizada, ressonância magnética e testes laboratoriais não foi suficiente para determinar a natureza da lesão. Após dois anos, a resseção laparoscópica da massa e o exame histopatológico do espécime cirúrgico permitiram estabelecer o diagnóstico de sequestro pulmonar extralobar intra-abdominal. Comentários: Essa malformação pode ser monitorada clinicamente; entretanto, a excisão cirúrgica frequentemente é feita, provavelmente devido à impossibilidade de diagnóstico com métodos não invasivos, como ocorreu no presente caso, na qual a lesão apresentou-se em posição não habitual para sequestro pulmonar extralobar intra-abdominal. Desse modo, a abordagem cirúrgica parece ser a chave para o diagnóstico e a condução desde tipo de malformação congênita.

Clinical value of prenatal MRI in the diagnosis and differential diagnosis of fetal bronchopulmonary sequestration / 中华妇产科杂志

Objective To investigate the clinical value of prenatal MRI in the diagnosis and differential diagnosis of congenital bronchopulmonary sequestration (BPS). Methods From January 2009 to December 2014, 16 fetuses with BPS were diagnosed by fetal MRI in Huzhou Maternity and Child Care Hospital and Shanghai Children′s Medical Center Affiliated to Shanghai Jiaotong University School of Medicine. The clinical data of these cases were analyzed retrospectively. All were singleton pregnancy, and MRI was carried out within 24-48 hours after routine prenatal ultrasound. All the neonates underwent postnatal enhanced CT scan or surgical biopsy after birth, and the results were compared to prenatal MRI diagnosis. Results (1)With prenatal MRI, 16 cases were diagnosed BPS. The lesions located in left lung in 10 cases, and right lung in 6 cases. As the scope of the lesion, 3 cases located in the whole left lung, 6 cases limited to the left lower lobe, and 1 case was subdiaphragmatic on the left side. 2 cases located in the whole right lung and 4 cases limited to the right lower lobe. One case complicated oligoamnios, and one had pleural effusion. Supplying vessels could be found in 14 cases.(2)When the postnatal results were compared with prenatal MRI, 15 cases were comfirmed as BPS (15/16), including 10 intralobar cases 5 extralobar cases. One that was diagnosed as BPS by prenatal MRI was confirmed to be congenital cystic adenomatoid malformation (CCAM) by pathology. The accuracy of prenatal MRI diagnosis of BPS was 15/16. Prenatal ultrasound missed one case and misdiagnosed two cases, as one was mistakened as CCAM and the other as cystic teratoma. Conclusion Prenatal MRI has good clinical value in the diagnosis and differential diagnosis of fetal BPS.

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.

Elevated Carbohydrate Antigen 19-9 Level in a Patient with Horseshoe Type Pulmonary Sequestration

Elevated carbohydrate antigen (CA) 19-9 can indicate malignancies of the gastrointestinal, pancreatic, and biliary tracts, and be found in a pulmonary sequestration. A 30-year-old man visited Seoul National University Bundang Hospital due to elevated CA 19-9 levels, representing pulmonary sequestration of the bilateral lower lobes, which were connected with each other. We performed left lower lobectomy and division of the systemic arteries. After operation, CA 19-9 levels decreased to normal range, even though a small amount of sequestrated lung remained in the right lower lobe. It is not uncommon that presence of pulmonary sequestration might elevate serum CA 19-9 levels; however, horseshoe type bilateral pulmonary sequestration is very rare.

Aberrant Bronchial Artery to Non-Sequestrated Left Upper Lobe in Massive Hemoptysis / 결핵및호흡기질환

Systemic arterial supply from the descending thoracic aorta to the basal segment of the left lower lobe without a pulmonary arterial supply is a rare congenital anomaly within the spectrum of sequestration lung disease. The most common pattern of anomalous systemic artery to the lung arises from the descending thoracic aorta and feeds the basal segments of the left lower lobe. We report an extremely rare case of a 29-year-old woman who underwent a successful left upper lobectomy for the treatment of recurrent massive hemoptysis from anomalous bronchial arterial supply to the lingular segment of left upper lobe.