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.

A case report of congenital lobar emphysema

Congenital lobar emphysema (CLE), also known as congenital alveolar overdistension, is a developmental anomaly of the lower respiratory tract that is characterized by hyperinflation of one or more of the pulmonary lobes. CLE is a rare congenital malformation with a prevalence of 1 in 20,000 to 1 in 30,000. We are reporting a 4-month-old boy presented with complaints of cough and cold for 3 days with history of similar complaints in the past at age of 1month and 2 months. Investigations revealed hyperlucency of left upper zone with tracheal shift and mild shift of the heart to the right. Elective Left Upper Lung Lobectomy was done and Appropriate supportive therapy given and child recovered well.

Congenital lobar emphysema: a rare cause of respiratory distress in a neonate

Congenital lobar emphysema (CLE) is a congenital condition characterized by distension and air trapping of the affected lobe of the lung. It is one of the causes of infantile respiratory distress, which may require surgical resection of affected lobe. Case characteristics: 3-day-old neonate with ventilation refractory respiratory distress. Imaging was suggestive of decreased lung tissue on the right side with ipsilateral mediastinal shift. Intervention/ outcome: Early surgical lobectomy was done to improve lung functions and the child improved dur to early intervention. Message: An early diagnosis with high index of suspicion helps patients with this rare congenital anomaly. Early intervention is the key to good long-term outcome. More awareness about the entity and treatment options available would greatly help improving the outcome and disease burden.

Congenital lung abnormalities / 中华实用儿科临床杂志

Congenital lung anomalies (CLA) are a group of malformations that include bronchopulmonary anomalies,vascular anomalies,and combined lung and vascular anomalies.With the advances in fetal ultrasonography and magnetic resonance imaging,and multi-detector computer tomography,CLA are increasingly being detected during both antenatal and postnatal periods,in turn influence patient counselling and management stratification.This paper focus on congenital lung abnormalities and congenital lobar emphysema.

Malformaciones pulmonares congénitas / Congenital lung malformations

Congenital lung malformations (CLM) comprise a heterogeneous group of lung diseases. They vary widely in their clinical presentation and severity, depending on the degree of lung involvement and their location in the thoracic cavity. They can manifest at any age and can be the source of significant morbidity and mortality in infants and children. Patients with CLM can present respiratory symptoms at birth or can remain asymptomatic for long periods. There has been an increase in early diagnosis of CLM attributable to the routine use of prenatal ultrasound. Management of these lesions depends on the type of malformation and symptoms. Treatment of asymptomatic patients is controversial, because the prognosis of these diseases is unpredictable. Because of the risk of complications, most authors recommend resection of the lesion at the time of diagnosis. This review describes the principal CLM, their diagnosis, and the controversies regarding treatment.

Las malformaciones pulmonares congénitas (MPC) constituyen un grupo heterogéneo de enfermedades. Varían en su presentación clínica y gravedad en función al grado de afectación pulmonar y a su ubicación en tórax. Se pueden manifestar a cualquier edad y pueden condicionar morbilidad y mortalidad significativa en lactantes y niños. Los pacientes pueden presentar síntomas respiratorios desde el nacimiento o permanecer asintomáticos durante largos períodos. Se ha observado un aumento en el diagnóstico precoz de las MPC atribuible al uso rutinario de la ecografía prenatal. El manejo de estas lesiones depende del tipo de malformación y de los síntomas. El tratamiento de pacientes asintomáticos es controvertido debido a que el pronóstico de estas enfermedades es desconocido. La mayoría de los autores recomiendan la resección de la lesión en el momento del diagnóstico debido al riesgo de complicaciones. Esta revisión describe las principales malformaciones congénitas del pulmón, su diagnóstico y las estrategias de tratamiento.

Enfisema Lobar Congénito, reporte de dos casos / Congenital Lobar Emphysema, Two cases report

El enfisema lobar congénito es una entidad poco común. No existe una etiología clara y se ha descrito que la causa más común es la displasia del cartílago bronquial. El diagnóstico clínico es difícil de realizar en tanto que la tomografía multidetector, así como el estudio histopatológico pueden definir el diagnóstico. Presentamos los casos clínicos de dos neonatos de sexo masculino con clínica similar a la de una neumonía.Describimos el proceso con el cual llegamos a la conclusión diagnóstica, su manejo, pronóstico y una breve revisión de la literatura.

Congenital lobar emphysema is a rare entity; there is no clear etiology and it has been described that the most common cause is the bronchial cartilage dysplasia. Clinical diagnosis is difficult to perform and multidetector computed tomography either the histopathological study can define the diagnosis. We present two cases of two male neonates with similar symptomatology than pneumonia. We describe the process that lead us to the diagnostic conclusion, management, prognostic and we present a brief review of the literature.

Conducta anestésica en un lactante con enfisema lobar congénito / Behavior of anesthesia in an infant with congenital lobar emphysema

Se presenta el caso clínico de un lactante con enfisema lobar congénito, tratado en el Hospital Infantil Sur de Santiago de Cuba con antecedentes de cuadros respiratorios recurrentes y varias hospitalizaciones previas. Inicialmente ingresó con bronconeumonía y disnea, por lo que se sospechó la presencia de un neumotórax, pero mediante estudios radiológicos se obtuvo el diagnóstico definitivo y se decidió aplicarle tratamiento quirúrgico. En el período intraoperatorio tuvo una complicación cardiovascular (bradicardia extrema), que fue tratada con las medidas adecuadas (oxígeno a 100 %, masaje cardíaco directo y sulfato de atropina), las cuales permitieron una evolución posoperatoria satisfactoria en la Unidad de Cuidados Intensivos Pediátricos.

The case of an infant with congenital lobar emphysema is reported, who was treated in the Southern Children Hospital of Santiago de Cuba with history of recurrent respiratory symptoms and several previous hospitalizations. Initially he was admitted with bronchopneumonia and dyspnea, and the presence of pneumothorax was suspected, but a definitive diagnosis was obtained by radiological studies and surgical treatment was decided. In the intraoperative period he had a cardiovascular complication (extreme bradycardia), which was treated with appropriate measures (100 % oxygen, direct heart massage and atropine sulfate), which allowed a satisfactory postoperative course in the Pediatric Intensive Care Unit.

Enfisema lobar congénito en los lóbulos superior y medio del pulmón derecho en una paciente de 2 meses de edad / Congenital lobar emphysema in upper and middle lobes of right lung in a 2-month-old female patient

Introducción. Las malformaciones broncopulmonares son infrecuentes, esporádicas y pueden asociarse con malformaciones congénitas en otros sistemas. Dentro de este grupo de malformaciones se encuentra el enfisema lobar congénito: una anomalía del desarrollo del tracto respiratorio bajo que se manifiesta como la hiperinsuflación de uno o más lóbulos pulmonares. Entre sus causas se han propuesto el atrapamiento de aire por un mecanismo valvular en los bronquios o el gigantismo lobular por sobredesarrollo alveolar del parénquima pulmonar. Caso clínico. Se reporta el caso de una paciente de 2 meses de edad que desde el nacimiento cursó con una patología respiratoria catalogada como proceso neumónico; fue tratada con múltiples esquemas de antibióticos, así como con la colocación de sellos pleurales por complicaciones con aparente neumotorax y derrame pleural. Posteriormente se realizó el diagnóstico de enfisema lobar congénito derecho con el apoyo de estudios de gabinete. Se manejó quirúrgicamente y se encontró la afectación de los lóbulos medio y superior del pulmón derecho. Conclusiones. La paciente evolucionó favorablemente con una buena expansión del pulmón residual derecho y la corrección de las posiciones mediastínicas.

Background. Bronchopulmonary malformations demonstrate a low frequency in the population. Their presentation is sporadic and may be associated with malformations in other systems. Within this group is congenital lobar emphysema, which is a developmental anomaly of the lower respiratory tract manifesting as hyperinflation of one or more lung lobes. Among its causes, entrapment of air through a valve mechanism at the bronchus or gigantism by overdevelopment of lobular alveolar lung parenchyma has been proposed. Case report. We report the case of a 2-month-old female patient who from birth presented with respiratory disease classified as pneumonic process. The patient was treated with multiple antibiotic schemes as well as placement of pleural seals complicated by pneumothorax and pleural effusion. Subsequently, diagnosis of congenital lobar emphysema was made and supported by laboratory studies. Surgery was carried out where involvement of the middle and upper lobes of the right lung was demonstrated. Conclusions. The patient presented a favorable evolution with a good expansion of residual right lung and correction of mediastinal positions.

Malformaciones pulmonares: enfisema lobar congénito / Lung malformations: lobar congenital emphysema

Congenital lobar emphysema (CLE) is a rare congenital lung anomaly. Clinical presentation varies from acute neonatal respiratory failure to recurrent episodes of tachypnea or infections. Chest x-ray is often enough to make the diagnosis in newborn, but a normal chest x-ray does not exclude the diagnosis and a CT scan must be done when there is a lung malformation suspect. The better antenatal ultrasound diagnosis has led to detection CLE in asymptomatic and mildly symptomatic infants, and involution of CLE has been demonstrated in some cases. Lobectomy is the treatment of choice for very symptomatic children, but could be expectant in the less symptomatic ones.

El enfisema lobar congénito (ELC), es una malformación pulmonar poco frecuente. Su presentación clínica varía desde la falla respiratoria en el recién nacido (RN) hasta episodios recurrentes de taquipnea o infecciones. La radiografía de tórax, muchas veces es suficiente para realizar el diagnóstico en el RN, pero ante una radiografía normal se debe realizar TAC de tórax si se sospecha malformación pulmonar. El aumento del diagnóstico por ecografía prenatal ha llevado a la pesquisa del ELC en lactantes asintomáticos o levemente sintomáticos, algunos de los cuales se ha demostrado involución de la malformación. El tratamiento de elección en niños francamente sintomáticos es la lobectomía, pudiendo ser conservador en el resto de los pacientes.

Congenital lobar emphysema: Pitfalls and management.

Congenital lobar emphysema is a rare entity presenting in the first month of life. It appears with varying degrees of respiratory distress, clinical and radiological evidence of over-aeration of the upper and middle lobes, mediastinal shift and hypoxia. Its early recognition and surgical intervention can be life-saving. Even today, despite advanced diagnostic techniques, pitfalls in diagnosis and management are not uncommon and the condition may be mistaken for pneumothorax or pneumonia. This report elucidates the anesthetic management of three such cases with a review of literature.

Bridging crossroads of congenital lobar emphysema--Lung perfusion scintigraphy: A guide towards the right path

Congenital lobar emphysema (CLE) is a life-threatening but potentially reversible lung abnormality of unknown etiology that can present a diagnostic dilemma. Imaging procedures are helpful for early diagnosis. This paper aims to present the scintigraphic features of CLE in a two-year-old male in correlation with other diagnostic modalities. Lung perfusion scintigraphy revealed globally diminished perfusion to the left lung. High Resolution Computed Tomography (HRCT) of the chest showed intrinsic narrowing of the distal left mainstem bronchus. CT scan of the Pulmonary Arteries (CTPA) showed a small left main pulmonary artery. Lung perfusion scintigraphy in CLE is characterized by markedly attenuated vascularity of the involved lobe with consequent decreased perfusion, and increased to normal perfusion in the unaffected lobe. It plays a vital role in the early diagnosis and management of pediatric pulmonary diseases presenting with a diagnostic dilemma, particularly in cases where it can preclude more invasive diagnostic procedures.

Congenital cystic lung lesions / 临床儿科杂志

Congenital cystic lung lesions are a group of congenital lung diseases with low incidence. These include congenital cystic adenomatoid malformation,bronchogenic cyst,congenital lobar emphysema and pulmonary sequestration. These malformations occur during the period of lung development stimulated by various factors. Manifestations of the diseases are very similar,but the pathogenesis and pathology are very different. Congenital cystic adenomatoid malformations are thought to be the results of the cessation of bronchiolar maturation with overgrowth of mesenchymal elements and without development of alveoli. There are 5 pathological types of congenital cystic adenomatoid malformations. Bronchogenic cysts are the results of abnormal budding from a segment of the tracheobronchial tree during embryo development,and the buds with no communication with normal tracheobronchial tree. Congenital lobar emphysema is a term reserved for hyperinflation of alveoli from idiopathic reasons or extrinsic compression,as well as pathological changes of the bronchial wall. Pulmonary sequestrations account for parts of nonfunctioning lung tissue that mostly do not communicate with normal bronchoalveolar tree and vascularized by a systemic artery,two types(intralobar and extralobar sequestration)are described.

Enfisema lobar congénito coexistente con pecho excavado. Presentación de un caso clínico radiológico / Congenital lobar emphysema associated witn pectus excavatum. A clinical radiological case

El enfisema lobar congénito es la hiperinsuflación con atrapamiento de aire de uno o más lóbulos pulmonares histológicamente normales, por un mecanismo valvular; produce compresión del parénquima normal y desplazamiento del mediastino. Se presenta el caso de un niño de 45 días con dificultad respiratoria in específica y pectum excavatum. La radiografía de tórax mostró hiperclaridad del pulmón derecho y desplazamiento del mediastino. Se realizó lobectomía, que confirmó el enfisema lobar congénito. Continúa evolución favorable 29 meses después.

Congenital lobar emphysema results from hyperinsufflation and air trapping in an otherwise histologically normal lobe, secondary to a bronchial valvular mechanism. It induces compression of normal lung and mediastinal shift. We present the case of a 45 day old male infant seen due to nonspecific respiratory difficulty and pectus excavatum; the chest X-ray showed a hyperlucent right lung and mediastinal shift. The resected middle lobe showed lobar emphysema. He remains in good condition 29 months later.

A Case of Prenatally Detected Congenital Lobar Emphysema

Congenital lobar emphysema (CLE) is an overinflation of infantile pulmonary lobe caused by air trapping within the affected area. This is usually diagnosed on radiological findings postnatally. Most cases present in the neonatal period with signs of acute or chronic respiratory distress. There are only a few reports of CLE in the literature with prenatal sonographic feature documented. The routine use of prenatal sonography has helped to find fetal anomalies, such as fetal lung mass, that pose a challenge to early diagnosis, appropriate counselling, postnatal follow-up, and better outcome. A boy with CLE was detected by prenatal sonogram. He had a uniformly echogenic right lung with mediastinal shift to the left which was suspected type III congenital cystic adenomatoid malformation prenatally. He showed no evidence of respiratory distress at birth, but experienced progressive tachypnea and chest retraction. He had right middle lobectomy on the seventh postnatal day and pathologic diagnosis of CLE had been made. We report this rare case with brief review of literature. This is the first domestic case which was detected prenatally and confirmed pathologically after lobectomy.

High Frequency Oscillation Ventilation for Resection of Congenital Lobar Emphysema: A case report / 대한마취과학회지

Congenital lobar emphysema (CLE) is a rare entity of unknown incidence. The main signs and symptoms are tachypnea, tachycardia, cyanosis, retractions, wheezing, tympanic chest percussion, asymetric breath sounds, or displaced cardiac tones within the first month of life. Occasionally a superimposed pulmonary infection exacerbates the condition, prompting hospital admission and medical treatment of the pneumonia followed by surgical treatment of the CLE. We now report a case of left upper CLE in a 15-day-old infant. Anesthesia was induced with thiopental sodium and maintained with intravenous fentanyl and midazolam. Endotracheal intubation was uneventful. Muscle relaxation was done with rocuronium. Gentle manual ventilation with a Mapleson D circuit was begun and changed to intermittent mandatory ventilation with an infant ventilator. During the left upper lobe resection, we used high frequency oscillation ventilation (HFOV) at FIO2 1.0, 12 Hz frequency, 12 cmH2O amplitude for 25 minutes. The minimal lung movement during HFOV was found to provide excellent operating conditions for the surgeons and adequate oxygenation without cardiovascular compromise. The PaCO2 was increased to 71 mmHg 25 minutes after the start of HFOV, and returned to normal value with intermittent mandatory ventilation. The remainder of the operation and anesthesia were uneventful. The patient was transferred to the neonatal intensive care unit.

Flexible Bronchoscopic Finding of Congenital Lobar Emphysema

Congenital lobar emphysema is a rare disease and a cause of respiratory distress in early infancy. The most common location of pulmonary involvement is the left upper lobe. We diagnosed a case of congenital lobar emphysema in a 5-month-old female infant, who had a complex heart disease using flexible bronchoscopy. In spite of palliative heart surgery, respiratory difficulty and cyanosis did not improve and weaning from artificial ventilation was not possible. Chest X-ray and CT scan showed persistent hyperinflation in the left upper lobe. So, we performed flexible bronchoscopy. The findings showed no cartilage and patent bronchus during inspiration, but complete collapse during expiration due to a check valve effect in the left upper lobar bronchus. After excision of the involved lobe, the patient improved from respiratory distress. The observed airway patency during inspiration, and dynamic airway collapse on expiration suggest that bronchomalacia contributed to lung overinflation in this case.

A Case of Congenital Lobar Emphysema on Right Lower Lobe

Congenital lobar emphysema is a rare disease and a cause of respiratory disturbance in newborns and infancts. The common locations of pulmonary involvement are the left upper lobe, right middle and upper lobe. We experienced a case of congenital lobar emphysema in a 9-month-old male infant. Chief complaints were dyspnea and tachypnea. Chest X-ray or CT scan revealed a hyperinflated right lower lobe, mediastinal shift to the left side and retrosternal herniaton of right lung. There was no evidence of infection and foreign body. This patient was successfully treated with right lower lobectomy. We report this case with a brief review of related literatures.

A Case of Congenital Lobar Emphysema on Right Lower Lobe

Congenital lobar emphysema is a rare disease and a cause of respiratory disturbance in newborns and infancts. The common locations of pulmonary involvement are the left upper lobe, right middle and upper lobe. We experienced a case of congenital lobar emphysema in a 9-month-old male infant. Chief complaints were dyspnea and tachypnea. Chest X-ray or CT scan revealed a hyperinflated right lower lobe, mediastinal shift to the left side and retrosternal herniaton of right lung. There was no evidence of infection and foreign body. This patient was successfully treated with right lower lobectomy. We report this case with a brief review of related literatures.

Congenital cystic diseases of the lung

No abstract available.