Congenital pulmonary malformations in children in a pediatric hospital in Peru, 2010-2020 / Malformaciones pulmonares congénitas en niños de un hospital pediátrico de Perú, 2010-2020

Abstract Background: Congenital pulmonary malformations (CPMs) are rare in children. This study aimed to describe the clinical, imaging characteristics, and treatment of patients with this pathology. Methods: We conducted a descriptive and retrospective study with data from patients with CPMs diagnosed at Instituto Nacional de Salud del Niño-Breña (Lima-Peru), from January 2010 to December 2020. We described CPM clinical and imaging characteristics, type and treatment. Results: The sample was formed of 70 patients. The median age was 29 months (range 15 days-14 years) and the male/female ratio was 1.4. The chest tomography found parenchymal involvement in 50 (71.4%) cases and mixed involvement (parenchymal and vascular) in 18 (25.7%) cases. Congenital malformation of the pulmonary airway was present in 39 (55.7%) cases, followed by bronchogenic cyst in 10 (14.3%), intralobar pulmonary sequestration in 9 (12.9%), and extralobar pulmonary sequestration in 7 (10%). Lobectomy was performed in 61 (87.1%) cases, cystectomy in 5 (7.1%), segmentectomy in 2 (2.9%), and embolization in 2 (2.9%). The most frequent post-operative complication was pneumonia, found in 9 (12.9%) cases. The median hospital stay was 26 days. No patient died during hospitalization. Conclusions: In our institution, the most frequent CPM was congenital malformation of the pulmonary airway, and lobectomy was the most frequently performed surgical procedure. CPMs represent a diverse group of disorders of lung development with varied imaging patterns and clinical manifestations.

Resumen Introducción: Las malformaciones pulmonares congénitas son poco frecuentes en niños. El objetivo de este estudio fue describir las características clínicas, imagenológicas y tratamiento de los pacientes con esta patología. Métodos: Se llevó a cabo un estudio descriptivo y retrospectivo con datos de los pacientes con malformaciones pulmonares congénitas diagnosticados en el Instituto Nacional de Salud del Niño-Breña (Lima-Perú) entre enero 2010 y diciembre 2020. Se describieron las características clínicas, imagenológicas, el tipo de malformación pulmonar congénita y el tratamiento. Resultados: La muestra fue de 70 pacientes. La mediana de edad fue 29 meses, la relación masculino/femenino fue 1.4. En la tomografía de tórax se encontró compromiso parenquimal en 50 (71.4%) casos, y compromiso mixto (parenquimal y vascular) en 18 (25.7%). La malformación congénita de la vía aérea pulmonar se observó en 39 (55.7%) casos, seguida del quiste broncogénico en 10 (14.3%), secuestro pulmonar intralobar en 9 (12.9%) y secuestro pulmonar extralobar en 7 (10%). La lobectomía fue realizada en 61 (87.1%) casos, la quistectomía en 5 (7.1%), segmentectomía en 2 (2.9%) y embolización en 2 (2.9%). La complicación posquirúrgica más frecuente fue la neumonía en 9 (12.9%) casos. La mediana de estancia hospitalaria fue de 26 días. Ningún paciente falleció durante la hospitalización. Conclusiones: En nuestra institución, la malformación pulmonar congénita más frecuente fue la malformación congénita de la vía aérea pulmonar, y la lobectomía el procedimiento quirúrgico más comúnmente realizado. Las malformaciones pulmonares congénitas representan un grupo diverso de trastornos del desarrollo pulmonar con variados patrones imagenológicos y manifestaciones clínicas.

20 cases of robotic assisted thoracoscopic surgery for the treatment of pulmonary sequestration in children / 中华胸心血管外科杂志

Objective:To analyze the application of robot-assisted thoracoscopic surgery in the treatment of pulmonary sequestration in children.Methods:The clinical data of 20 children with pulmonary sequestration admitted to the Children's Hospital Zhejiang University School of Medicine from May to November 2020 were analyzed retrospectively. There were 13 males and 7 females, ages ranged from 6 months to 5 years old, with median age of 10 months. Body weight ranged from 7.5 to 18.0 kg, with mean weight of(9.95±2.46)kg. Abnormal blood supply vessels in pulmonary sequestration were found by chest enhanced CT and were further confirmed during surgery. All the other 19 cases were found to have pulmonary lesions by prenatal ultrasound except 1 case due to repeated infection. The lesions were located in left lung in 15 cases and right lung in 5 cases.Results:1 case was converted to thoracotomy due to failure of intraoperative single lung ventilation and inability of artificial pneumothorax to collapse the lung lobe, and other 19 cases were successfully completed by robot-assisted thoracoscopic surgery. The operation time ranged from 40 to 270 min, mean(88.25±55.68) min. All 10 patients with extralobar sequestration underwent simple pneumonectomy, including 2 patients with intra-diaphragmatic pulmonary sequestration. In 10 cases of intralobar sequestration, 2 cases underwent wedge resection, 2 cases underwent segmental resection, and 6 cases underwent lobectomy. No operative death occurred. The postoperative hospital time ranged from 3 to 10 days, mean(5.00±1.89) days. All patients recovered well and no complications such as pleural effusion and atelectasis were observed during 1-6 months follow-up.Conclusion:The robotic surgical system is safe and effective for the treatment of pulmonary sequestration in children.

Secuestro pulmonar intralobar / Intralobar Pulmonary Sequestration

Introducción: El secuestro pulmonar está representado por masas de tejido pulmonar displásico, no funcionante, sin comunicación con el árbol bronquial. Objetivo: Presentar tres casos de secuestro pulmonar intralobar tratados por el autor. Caso clínico: Se presentan tres pacientes tratados entre 2013 y 2018 con diagnóstico de secuestro pulmonar. Dos fueron del sexo masculino (44 y 60 años de edad) y una del femenino (20 años de edad). Los síntomas fueron dolor torácico (1) y cuadro de infección pulmonar grave (2). En dos enfermos el secuestro se localizó en el lóbulo inferior derecho y en uno en el inferior izquierdo. Las operaciones fueron lobectomías inferiores derecha (1) e izquierda (1) y bilobectomía inferior y media derechas por afectación del lóbulo medio. Dos tuvieron una evolución satisfactoria y uno presentó un empiema pleural que resolvió con tratamiento antibiótico. Conclusiones: Los secuestros pulmonares son raros y los síntomas suelen asociarse con infección pulmonar. En la mayoría de los casos es necesario realizar una lobectomía. La evolución postoperatoria suele ser buena(AU)

Introduction: Pulmonary sequestration is characterized by masses of dysplastic, nonfunctioning lung tissue, without any communication with the bronchial tree. Objective: To present three cases of intralobar pulmonary sequestration treated by the author. Clinical case: The respective cases are presented of three patients with a diagnosis of pulmonary sequestration treated between 2013 and 2018. Two were male (44 and 60 years old, respectively) and one was female (20 years old). Their symptoms were chest pain (1) and severe lung infection (2). In two patients, the sequestration was located in the lower right lobe, while, in one, it was in the lower left lobe. The operations were right (1) and left (1) lower lobectomies and right lower and middle bilobectomy due to middle lobe involvement. Two had a satisfactory evolution, while one presented a pleural empyema healed with antibiotic treatment. Conclusions: Pulmonary sequestration is rare and their symptoms are usually associated with pulmonary infection. In most cases, a lobectomy is necessary. The postoperative evolution is usually good(AU)

Estenosis congénita de venas pulmonares y malformación vascular broncopulmonar / Congenital pulmonary vein stenosis and bronchopulmonary vascular malformation

Resumen El objetivo es mostrar el diagnóstico y la evolución de una paciente con estenosis de venas pulmonares y secuestro pulmonar. Se trata de una niña de 1 año de edad, con bronconeumonías de repetición, acrocianosis, 2R intenso, cardiomegalia, hipertensión venocapilar pulmonar, con diagnóstico clínico de comunicación interauricular. El ecocardiograma mostró estenosis de venas pulmonares izquierdas. El cateterismo cardiaco detectó fístulas arteriovenosas en la región apical del pulmón derecho. La imagen de resonancia magnética y la angiografía mostraron un vaso arterial aberrante paralelo a la aorta abdominal y con flujo dirigido al lóbulo pulmonar derecho. La angiotomografía reportó confluencia de las venas pulmonares del lado derecho. Se realizó lobectomía derecha. La paciente falleció en el posoperatorio debido a una hemorragia masiva pulmonar. Esta paciente es la primera descrita en la literatura con estenosis de venas pulmonares congénita asociada a secuestro pulmonar. La ecocardiografía es el estudio diagnóstico ideal inicial en los pacientes con estenosis congénita de venas pulmonares.

Abstract The objective is demonstrate the diagnostic process and evolution of a patient with a diagnosis of congenital pulmonary vein stenosis and broncho-pulmonary vascular malformation. One year old female patient with repeated bronchopneumonia, acrocyanosis, split S2, cardiomegaly, pulmonary hypertension, with a clinical diagnosis of atrial septal defect. The echocardiogram demonstrated left sided vein pulmonary stenosis. The cardiac catheterization demonstrated arterial-venous fistulas apical on the right lung. Magnetic Resonance image and angiography showed an aberrant arterial vessel parallel to the abdominal aorta which flow the right pulmonary lobe. The cardiac tomography angiography reported confluence of right-sided pulmonary veins. A lobectomy is performed. Patient died in post-operative due to massive pulmonary hemorrhaging. This is the first patient mentioned in written literature with pulmonary vein stenosis associated with pulmonary sequestration, with normal venous connection. Echocardiography represents the specific standard study ideal for initial diagnostic for patients with pulmonary vein stenosis.

Secuestro pulmonar: caracterización y tratamiento en pacientes adultos y pediátricos / Pulmonary sequestration: characteristics and treatment in adult and pediatric patients

Resumen Introducción: El secuestro pulmonar (SP) es una malformación congénita caracterizada por tejido pulmonar con vascularización de una arteria sistémica anómala. Objetivo: Analizar las características y tratamiento de pacientes adultos y pediátricos con secuestro pulmonar. Materiales y Método: Estudio descriptivo transversal. Periodo: enero de 1988 a diciembre de 2018. La información se obtuvo de fichas clínicas y registros de anatomía patológica. Se describen edad, sexo, características clínicas, diagnóstico, tratamiento quirúrgico y hallazgos anatomopatológicos. Se realizó análisis estadístico mediante SPSS25® y se usó la prueba Mann-Whitney y X2, considerándose significativo p < 0,05. Resultados: Total 33 pacientes, 25 (75,8%) mujeres. Edad promedio 30,2 años, rango: 0-68. Adultos 23 (69,7%) pacientes y pediátricos (< 15 años) 10 (30,3%) pacientes. La presentación clínica fue sintomatología pulmonar en 23 (69,7%) casos y 9 (27,3%) eran asintomáticos. Tres (9,1%) presentaron malformación congénita asociada. Diagnóstico preoperatorio en 15 (45,5%) pacientes. La ubicación más frecuente fue lóbulo inferior izquierdo. El tipo intralobar fue el más frecuente en 23 (69,7%) casos. La cirugía más frecuente fue la lobectomía con identificación y ligadura del vaso sistémico. El vaso aberrante se originó en aorta torácica en 27 (81,8%) casos e infradiafragmático (no precisado) en 3 (9,1%) casos. Vaso único en 26 (78,8%) y doble en 5 (15,2%) casos. No hubo mortalidad. Existen diferencias en las características entre los secuestros en pacientes adultos y pediátricos. Discusión y Conclusión: Los SP son infrecuentes, se presentan principalmente en adultos jóvenes como neumopatías a repetición, se distinguen diferencias en las características entre los pacientes adultos y pediátricos, y tienen excelente pronóstico posoperatorio.

Background: Pulmonary sequestration (PS) is a congenital malformation characterized by lung tissue with vascularization from anomalous systemic arteries. Aim: To analyze characteristics and treatment of adult and pediatric patients with pulmonary sequestration. Materials and Method: Transversal descriptive study. Period: January-1988 to December-2018. Information was obtained from clinical files and pathological anatomy records. Age, sex, clinical characteristics, diagnosis, surgical treatment and pathological findings are described. Statistical analysis was performed using SPSS25® and the Mann-Whitney and Chi square test were used, considering p < 0.05 to be significant. Results: Total 33 patients, 25 (75.8%) women. Average age 30.2 years, range: 0-68. Adults 23 (69.7%) patients and pediatric (< 15 years) 10 (30.3%) patients. The clinical presentation was pulmonary symptoms in 23 (69.7%) cases and 9 (27.3%) were asymptomatic. Three (9.1%) presented another congenital malformation. Preoperative diagnosis in 15 (48.4%) patients. The most frequent location was the left lower lobe. The intralobar type was the most frequent: 23 (69.7%) cases. The most frequent surgery was lobectomy with identification and ligation of the systemic vessel. The systemic vessel originated in the thoracic aorta in 27 (81.8%) cases and infradiaphragmatic (not specified) in 3 (9.1%) cases. Single vessel in 26 (78.8%) and double in 5 (15.2%) cases. There was no mortality. Differences were found in characteristics between adult and pediatric patients. Conclusion: SP are infrequent, they mostly appear in young adults as recurrent lung diseases, differences in characteristics are distinguished between adult and pediatric patients and they have an excellent postoperative prognosis.

Secuestro pulmonar intralobar / Intralobar pulmonary sequestration

El secuestro pulmonar es una malformación congénita del tracto respiratorio inferior, rara e importante. En niños y adultos suele presentarse con infecciones pulmonares a repetición o crónicas severas. Se describió un caso de una paciente de 19 años de edad que tuvo un cuadro de sepsis respiratoria bajas a repetición. En los estudios de imágenes de tórax se evidenció opacidad homogénea en hemitórax derecho. Fue intervenida quirúrgicamente; se le realizó una lobectomía inferior derecha la cual fue diagnóstica para secuestro pulmonar, con evolución satisfactoria(AU)

Pulmonary sequestration is a rare and important congenital malformation of the lower respiratory tract. In children and adults, it usually presents with severe chronic or repeated lung infections. A case of a female 19-year-old patient who had recurrent lower respiratory sepsis is reported. Homogeneous opacity was evident in the chest imaging studies in the right hemithorax. She underwent surgery. A lower right lobectomy was performed, which was diagnostic for pulmonary sequestration, with satisfactory evolution(AU)

Secuestro pulmonar: a propósito de un caso / Pulmonary sequestration: a case report

Resumen Introducción El secuestro pulmonar es un segmento anormal de tejido pulmonar sin comunicación con el árbol traqueobronquial e irrigado por una arteria aberrante de origen sistémico. El secuestro pulmonar puede ser intralobar o extralobar. Caso Clínico Presentamos el caso de un neonato con dificultad respiratoria debido a secuestro pulmonar. El tratamiento quirúrgico consiste en la resección del segmento pulmonar con la ligadura y corte de su arteria. Se describe el manejo realizado y la revisión de la literatura indexada.

Introduction Pulmonary sequestration is an abnormal segment of lung tissue without communication with the tracheobronchial tree and irrigated by an aberrant artery of systemic origin. Can be intralobar or extralobar. Case report We present the case of a neonate with respiratory distress due to pulmonary sequestration. Surgical treatment consists in the resection of the lung segment with the ligature and cut of its artery. Is describe the management carried out and the review of the indexed literature.

Report of 15 cases of single-port video-assisted thoracoscopic surgery for pulmonary sequestration / 上海交通大学学报（医学版）

Objective: To investigate the feasibility of single-port video-assisted thoracoscopic surgery (VATS) for pulmonary sequestration. Methods: Fifteen cases with intralobar pulmonary sequestration in Renji Hospital, Shanghai Jiao Tong University School of Medicine from January 2014 to December 2018 were reviewed. All the cases had underwent lobectomy by single-port VATS, including 13 cases of left lower lobectomy and 2 cases of right lower lobectomy. Results: Pleural adhesion was found in 11 of the 15 cases. Lobectomy was successfully performed by single-port VATS in 12 cases. Three cases were transferred to multi-port VATS because of severe pleural adhesion. There was no case conversed to thoracotomy in the study. There was no mortality and complication during the perioperative period. For the 15 cases, the operation time was (96.7±27.8) min, the bleeding volume was (75.3±45.5) mL, the postoperative intubation was (5.2±2.0) d and the postoperative hospital stay was (6.2±2.2) d. Conclusion: Single-port VATS treatment for pulmonary sequestration is safe, effective and feasible.

Diagnóstico diferencial y cirugía mínimamente invasiva de un tumor suprarrenal de hallazgo antenatal / Differential diagnosis and minimally invasive surgery of an antenatal adrenal mass

Resumen: Introducción: Las masas suprarrenales en recién nacidos son infrecuentes. El diagnóstico diferen cial incluye masas benignas (hemorragia suprarrenal o secuestro pulmonar extralobar) y malignas (neuroblastoma), y pueden ser un hallazgo durante la ecografía obstétrica. El uso de imágenes com plementarias en el periodo postnatal permite una mejor aproximación diagnóstica, con implicancias en el manejo de estos pacientes. Objetivos: comunicar el caso de una recién nacida portadora de una masa suprarrenal, discutir los diagnósticos diferenciales y el manejo de lesiones suprarrenales en recién nacidos. Caso Clínico: Lactante de 2 meses de edad, derivada para estudio de tumor supra rrenal de diagnóstico antenatal a las 22 semanas de edad gestacional. El estudio imagenológico con ecografía postnatal mostró un tumor compatible con neuroblastoma. Paciente asintomática, estudios de laboratorios sin hallazgos relevantes. Se realizó resección laparoscópica de la lesión. El estudio histológico confirmó un secuestro pulmonar. Conclusión: El secuestro pulmonar extralobar debe ser considerado en el diagnóstico diferencial de una masa suprarenal del recién nacido. La cirugía mínimamente invasiva debiera considerarse como el abordaje de elección en casos como este, donde existe factibilidad técnica y beneficios en la recuperación y secuelas cosméticas del paciente.

Abstract: Introduction: Adrenal masses are uncommon in newborns. The differential diagnosis includes be nign masses (adrenal hemorrhage, extralobar pulmonary sequestration) and malignant ones (neuro blastoma) that may be a finding during an obstetric ultrasound. The use of complementary imaging methods allows a better diagnosis approach during the postnatal period, with implications for the management of these patients. Objective: To report the case of a female newborn with diagnosis of an adrenal mass, and to discuss differential diagnoses and management alternatives of adrenal lesions in newborns. Case report: Two-month-old female infant, referred for adrenal tumor study diagnosed at 22 weeks gestational age. Postnatal ultrasound showed a tumor compatible with neuroblastoma. The patient was asymptomatic, and the laboratory studies showed no relevant findings. The lesion was excised by laparoscopy. A histological study confirmed pulmonary sequestration. Conclusions: Extralobar pulmonary sequestration should be considered in the differential diagnosis of an adrenal mass in the newborn. Minimally invasive surgery should be the preferred surgical technique choice in these cases, given the technical feasibility and benefits in the recovery and cosmetic issues of the patient.

Sequestro pulmonar e tratamento endovascular: um relato de caso / Pulmonary sequestration and endovascular treatment: a case report

O sequestro pulmonar refere-se à anomalia congênita definida por massa de parênquima pulmonar não funcionante. É dividido pela existência de envoltório pleural próprio em intralobar, representando cerca de 75% dos casos, ou extralobar, responsável pelos 25% restantes. O diagnóstico é feito através de radiografia e confirmado com tomografia computadorizada, ressonância nuclear magnética e arteriografia. Tradicionalmente, o tratamento proposto é cirúrgico, mas a técnica endovascular tem apresentado bons resultados. É relatado o caso de uma mulher, 29 anos, com quadro clínico de pneumonias de repetição desde os 5 anos. A tomografia de tórax revelou malformação vascular em região inferior de pulmão direito. O tratamento vascular foi realizado através de embolização do ramo anômalo

Pulmonary sequestration is a congenital anomaly defined as a nonfunctioning mass of lung parenchyma. Presence of an independent pleural envelope classifies it as intralobar, accounting for approximately 75% of the cases, while absence classifies cases as extralobar, accounting for the remaining 25%. Diagnosis is made through radiography and confirmed by computed tomography, magnetic resonance, or angiography. The traditional treatment is open surgical repair, but endovascular techniques have been used, with good results. We report the case of a 29-year-old-woman presenting with recurrent pneumonia for 5 years. A CT scan of the chest revealed poor vascular formation in the lower region of the right lung. The pulmonary sequestration was treated by embolization of the anomalous branch

Secuestro pulmonar asociado a una malformación congénita de la vía aérea pulmonar / Pulmonary sequestration associated with congenital pulmonary airway malformation

Resumen Introducción: Las malformaciones pulmonares congénitas son una causa poco frecuente de morbilidad neonatal. Algunas de ellas tienen un origen común, lo que permite identificar lesiones combinadas. Su diagnóstico puede realizarse de forma prenatal mediante ultrasonido, con las limitaciones de que solo se realiza en centros especializados y que depende de la pericia del operador. La asociación entre el secuestro pulmonar y la malformación congénita de la vía aérea se ha descrito aproximadamente en 40-60 casos desde 1949, cuando se observó por primera vez. Muchas lesiones no son perceptibles en la vida intrauterina. Sin embargo, en el periodo neonatal se presentan síntomas respiratorios recurrentes que en algunos casos están asociados con una malformación pulmonar. Caso clínico: Se presenta el caso de una lactante diagnosticada con secuestro pulmonar a las 24 semanas de edad gestacional. Recibió tratamiento quirúrgico intrauterino con reporte de resolución completa de la malformación en ultrasonidos posteriores. Fue valorada por neumología pediátrica a los 4 meses de edad. Se realizó una angiotomografía y se confirmó la presencia de secuestro pulmonar, por lo que se realizó una lobectomía. El estudio histopatológico reportó secuestro pulmonar extralobar con malformación congénita de la vía aérea pulmonar tipo 2. Estas lesiones combinadas se identificaron mediante un estudio histopatológico. El tratamiento de elección fue quirúrgico. Conclusiones: Ante la confirmación de una malformación, destaca la importancia de realizar la búsqueda de otras malformaciones que pudieran estar asociadas.

Abstract Introduction: Congenital pulmonary malformations are a rare cause of neonatal morbidity. Some of them have a common origin, which allows the identification of combined lesions. Its diagnosis can be made prenatally by ultrasound, with the limitation that this study is performed in specialized centers and depends on the expertise of the operator. The association of pulmonary sequestration and congenital malformation of the airway has been described in approximately 40-60 cases since its first description in 1949. Many lesions are not perceptible in intrauterine life and in the neonatal period there are recurrent respiratory symptoms that in some cases are associated with a congenital pulmonary malformation. Case report: We report the case of a young infant, who was diagnosed with pulmonary sequestration at 24 weeks of gestational age, undergoing intrauterine surgical treatment with a report of complete resolution of the malformation in posterior ultrasounds. She was valued by pediatric pneumology at 4 months of age, where angiotomography was performed and the presence of pulmonary sequestration was confirmed by lobectomy. The histopathological study reported extralobar pulmonary sequestration with congenital malformation of the pulmonary airway type 2. These combined lesions were identified by histopathological study. The treatment of choice was surgical. Conclusions: Upon the confirmation of a malformation, we emphasize the importance of performing a screening in order to search for other that could be associated.

The relationship between intralobar pulmonary sequestration syndrome with recurrent infection and basal stem cell proliferation / 天津医药

Objective To investigate the proliferation potential of the basal stem cells in intralobar pulmonary sequestration syndrome (ILS) for revealing the pathogenesis of ILS. Methods In this study, lung tissue samples were collected from healthy control subjects(n=4)and abnormal lung lobes of ILS patients(n=4).The pathological changes were compared by HE staining between the two groups.The proportion of goblet cells was compared by PAS staining between the two groups.The expression and secretion of MUC5AC and MUC5B were compared by immunofluorescence staining and real-time PCR between the two groups. The distribution of ciliated cells and the proliferation of basal cells were compared by immunofluorescence staining between the two groups.Results The abnormal lobe of ILS group was filled with inflammatory cells, and the airway epithelium was disrupted. The airway goblet cells of ILS were obviously hyperplastic. The mucin proteins of MUC5AC and MUC5B were hypersecretion in the abnormal lobe of ILS patients.KRT5-positive basal stem cells proliferated only slightly in ILS patients, although there was no significant difference in KRT5 expression between two groups. Conclusion These data suggest that the pathogenesis of ILS may be associated with defects in basal stem cell function. Restoring airway integrity by targeting epithelial regeneration can be a future non-surgical treatment for patients with ILS.

Prenatal diagnosis of intraabdominal extralobar pulmonary sequestration by ultrasound / 中华超声影像学杂志

Objective To describe the diagnostic approach and clinical outcomes of fetal intraabdominal extralobar pulmonary sequestration (IEPS).Methods Three cases of IEPS were diagnosed prenatally by ultrasound in our center.The data of these 3 cases and 43 cases in prior studies from 1986 to 2016 were retrospectively reviewed.The prenatal sonographic features,treatment,and outcomes were collected.Results The gestational age of diagnosis IEPS was (24.5±5.9)weeks,the male-to-female ratio was 3-4∶1,78.3% (36/46) of IEPS masses located on the left side,47.8% (22/46) of lesions were homogenous and hyperechoic,8.7% (4/46) of cases associated anomalies,78.3% (36/46) of patients were preformed resection and postoperative recovery and subsequent course were uncomplicated.Feeding arteries were detected in 15.2% (7/46) cases and confirmed by surgery or autopsy.Conclusions Prenatal ultrasound has the diagnostic accuracy of IEPS.Feeding arteries noted on color Doppler are strongly indicators of IEPS.Postsurgical outcomes are favorable.

Secuestro pulmonar intralobar: hallazgo en el intraoperatorio / lntralobar pulmonary sequestration: an intraoperative finding

Reportamos un caso de secuestro pulmonar intralobar ubicado en el lóbulo inferior del pulmón izquierdo en una paciente de sexo femenino de 3 años de edad, diagnosticado en el intraoperatorio. El secuestro pulmonar es una malformación pulmonar congénita muy rara, caracterizada por una masa de tejido pulmonar no funcionante,que recibe irrigación anómala de una arteria sistémica, generalmente, procedente de la aorta descendente; su variedad intralobar usualmente se manifiesta con neumonías recurrentes. El diagnóstico definitivo es hecho al identificar la arteria anómala hacia el pulmón mediante estudios por imágenes (TEM con contraste y ecografía Doppler).No hay estudios de su incidencia a nivel nacional. El presente caso es el primero reportado en la ciudad de Huacho, Perú

The case of a 3 year-old female patient, who was intraoperative diagnosed with intralobar pulmonary sequestration located in the lower lobe of the left lung, is reported. Pulmonary sequestration is a rarecongenital pulmonary malformation characterized by a non-functionalmass of lung tissue, which is irrigatedby an abnormalsystemic artery, general!y originated in the descending aorta;its intralobar variety manifests as recurrent pneumonia. Definitive diagnosis is made after identifying the abnormal artery with imaging techniques (MSCT with contrast and Doppler echocardiography). There is no national incidence study on this condition. The following case is the first pulmonary sequestration case reported in the city of Huacho, Perú

Evaluation of pulmonary sequestration with multidetector computed tomography angiography in a select cohort of patients: A retrospective study

OBJECTIVES: This study aimed to evaluate the role of multidetector computed tomography angiography in diagnosing patients with pulmonary sequestration. METHODS: We retrospectively analyzed the computed tomography studies and clinical materials of 43 patients who had undergone preoperative multidetector computed tomography angiography in our hospital and had pathologically proven pulmonary sequestration. Each examination of pulmonary sequestration was reviewed for type, location, parenchymal changes, arterial supply and venous drainage on two-dimensional and three-dimensional computed tomography images. RESULTS: Multidetector computed tomography successfully detected all pulmonary sequestrations in the 43 patients (100%). This included 40 patients (93.0%) with intralobar sequestration and 3 patients (7.0%) with extralobar sequestration. The locations of pulmonary sequestration were left lower lobe (28 cases, 70% of intralobar sequestrations), right lower lobe (12 cases, 30% of intralobar sequestrations) and costodiaphragmatic sulcus (3 cases). Cases of sequestered lung presented as mass lesions (37.2%), cystic lesions (32.6%), pneumonic lesions (16.3%), cavitary lesions (9.3%) and bronchiectasis (4.6%). The angioarchitecture of pulmonary sequestration, including feeding arteries from the thoracic aorta (86.1%), celiac truck (9.3%), abdominal aorta (2.3%) and left gastric artery (2.3%) and venous drainage into inferior pulmonary veins (86.0%) and the azygos vein system (14.0%), was visualized on multidetector computed tomography. Finally, the multidetector computed tomography angiography results of the sequestered lungs and angioarchitectures were surgically confirmed in all the patients. CONCLUSIONS: As a noninvasive modality, multidetector computed tomography angiography is helpful for making diagnostic decisions regarding pulmonary sequestration with high confidence and for visualizing the related parenchymal characteristics, arterial supply, and venous drainage features to help plan surgical strategies.

PULMONARY SEQUESTRATION / Мэс засал

Introduction: Pulmonary sequestration(PS) is a cystic or solid mass composed ofnonfunctioning primitive tissue that doesnot communicate with the trachea-bronchialtree and has anomalous systemic bloodsupply. Pulmonary sequestration is a raredisease, of unknown etiology, representing0.1-6% of all structural lung diseases anddevelopmental malformations. We describea case of girl with pulmonary sequestration.Result: She has been admitted in ourhospital with left lower lobe mass. Fordifferential diagnosis we did X-ray, CTscanning, MRI of chest and angiography.In the X-ray had been detected retrocardiactriangular mass. CT scanning shown us massin the left lower lobe. Due to angiography wecan’t seen arterial supply. MRI demonstratedas like as X-ray, retrocardiac triangular mass.She had been gone under the electivesurgery: Left thoracotomy, resection ofpulmonary sequestration, drainage of pleuralcavity.During and after the surgery had notcomplication and she had been dischargedafter few days of post-operative treatment.She had been fully recovered.Conclusions: Pulmonary sequestrationis rare anormaly with multiple theoreticaletiologies. Due to high technological imaginginvestigations can take right diagnosis.The patient can be fully recovered afterthe surgical treatment. Intralobular typeis more difficult to resect the pulmonarysequestration.

Diagnosis and surgical treatment of pulmonary sequestration in adults / 局解手术学杂志

Objective To explore the diagnosis and surgical treatment of pulmonary sequestration in adults. Methods Clinical data of 21 cases of pulmonary sequestration whose diagnosis was confirmed by surgical biopsy in our department from March 2009 to February 2016 were retrospectively analyzed. Divided the patients into the thoracotomy group (n=9) and the thoracoscope group (n=12) according to dif-ferent surgical methods, and compared the diagnosis and surgery of the two groups. Results Among the patients, 8 cases were diagnosed as pulmonary sequestration and the remaining 13 cases were misdiagnosed,with the misdiagnosis rate of 61. 9%. Intraoperative exploration dem-onstrated that the abnormal blood vessels were originated from thoracic aorta (n=14,66. 7%),abdominal aorta (n=4,19%),phrenic artery (n=3,14. 3%) and aortic arch (n=1,4. 8%), and there were 20 cases (95. 2%) of intralobar sequestration and 1 case (4. 8%) of ex-tralobar sequestration. Patients underwent thoracotomy and patients underwent video-assisted thoracoscopic surgery were of no significant differences in operative time (P=0. 104),blood loss (P=0. 209),chest tube duration (P=0. 511),drainage volume (P=0. 135) and postoperative hospital stay (P=0. 450). All the patients recovered well after surgery. Conclusion As pulmonary sequestration lacks specific clinical manifestations,missed diagnosis and misdiagnosis are very common in patients. Chest enhanced CT and CT angiography are effective diagnostic methods at present. Both thoracotomy and VATS can achieve good therapeutic effects.

Effects of two different venous drainage patterns on the prognosis of fetal pulmonary sequestration / 中华超声影像学杂志

Objective To evaluate the effect of two different venous drainage patterns on the prognosis of fetal pulmonary sequestration( PS) . Methods Sixty cases of fetal PS with confirmed venous drainage diagnosed by prenatal ultrasound were retrospectively analyzed . Changes of the volumes of PS lesions and the clinical outcomes were compared between two different venous drainage patterns . Results Among the total 64 cases ,34 cases were pulmonary venous drainage and 30 cases were systemic venous drainage . There was no case combined with any abnormality in pulmonary venous drainage group;whereas , 6 cases combined with other abnormalities in systemic venous drainage group ,between which significant difference was noted( P =0 .02) . In pulmonary venous drainage group ,there was no significant difference in the volumes of PS lesions between at 20-24 weeks′gestational age(WGA) and at 24+1 -30 WGA( P >0 .05) ;but not between at 24+1 -30 WGA or at 20 -24 WGA and at 30+1 -39 WGA ( P 0 .05) . Postnatal respiratory symptoms and postnatal surgery rates were similar between the two groups( P > 0 .05) . Conclusions PS with systemic venous drainage is more likely combined with other abnormalities than PS with pulmonary venous drainage . The lesion volumes of PS with pulmonary venous drainage decreas remarkably during the middle‐late pregnancy . Nevertheless ,the clinical postnatal outcomes are both favorable in the two groups .

Secuestro pulmonar en el adulto / Pulmonary sequestration in adults

Introducción: el secuestro pulmonar es una malformación congénita inusual que consiste en segmentos pulmonares afuncionales sin comunicación con el árbol traqueobronquial y un aporte sanguíneo a través de la circulación sistémica. Caso Clínico: nosotros presentamos el caso de una mujer con infecciones pulmonares a repetición en el lóbulo inferior izquierdo. Debido a múltiples infecciones en el mismo sitio y con la sospecha de secuestro pulmonar se realiza angiotomografía en donde se observa una arteria aberrante que proviene de la aorta torácica, confirmando el diagnóstico; posteriormente es resuelto quirúrgicamente. Conclusión: el secuestro pulmonar es una patología rara con mayor incidencia en la infancia sin embargo algunas variantes se pueden presentar en el adulto y la sintomatología de infecciones pulmonares a repetición nos hace sospechar en esta enfermedad.

Introduction: pulmonary sequestration is an unusual malformation consisting of isolated nonfunctioning lung segments lacking communication with functional tracheobronchial trees, with blood fow through the systemic circulation. Case report: we present the case of a woman with recurrent pulmonary infections in the left lower lobe. Because multiple infections in the same place, and with the suspicion of pulmonary sequestration, we perform a pulmonary angio tomography where aberrant artery that comes from the thoracic aorta is observed, confrming the diagnosis; which was then surgically resolved. Conclusion: pulmonary sequestration is a rare disease with highest incidence in childhood, but some variants may be present in adults and symptoms of recurrent pulmonary infections should make us suspicious of the disease.

Intralobar Pulmonary Sequestration Presenting as Chronic Non-productive Cough.

We report a case of a 60-year-old female who was known to have intralobar pulmonary sequestration and her only symptom was chronic cough. She had no history of infections and surgical resection led to complete resolution of her chronic cough.