Congenital lobar hyperinflation is the currently recommended term, as it involves pathologically. Differential diagnosis of the disease remains challenging in an infant with acute respiratory distress. Emphysema, congenital lobar nord national organization for. This case is a bout a neonate with respiratory distress and primary diagnosis of pneumothorax who had congenital lobar emphysema. Congenital lobar overinflation should be considered in cases of prenatal echogenic lung lesions without macrocysts or classic findings of bronchial atresia. Pdf congenital lobar emphysema concurrent with pneumothorax. Congenital lobar emphysema cle is a rare developmental lung malformation. We are reporting a neonate admitted in the pediatric emergency ward with the respiratory distress since 16th day of life.
Congenital lobar emphysema cle is a rare clinical entity that usually presents as acute respiratory distress. Postnatally, cle is caused by air trapping of the obstructed bronchus. Pdf case of congenital lobar lung emphysema herbert. We report here the case of a 4monthold infant who at two months suddenly presented upper respiratory infections, treated symptomatically. One such patient, a 3monthold child who underwent successful. Congenital lobar overinflation radiology reference article. Congenital lobar emphysema used to be treated surgically. Although congenital obstructive lobar emphysema is a comparatively rare condition, it is one which taxes the skill and experience of both the medical and nursing staff in coping with a child acutely breathless and dangerously ill. Prenatal diagnosis and management of congenital lobar. A major cause of pulmonary insufficiency in chronic cigarette smokers, emphysema is a progressive disease that commonly occurs in conjunction with chronic bronchitis. It may be diagnosed without much difficulty and treated by lobectomy with gratifying success.
The routine use of prenatal ultrasonography has resulted in the early identification and serial evaluation of. Current operative management of congenital lobar emphysema. The routine use of prenatal ultrasonography has resulted in the early identification and serial evaluation of congenital lung lesions. Abnormality of bronchial cartilage is one of the suspected aetiological factors.
The examination findings with oxygen desaturations and a rightsided mediastinal shift on chest xray led. The ages of the patients at diagnosis ranged from 2 weeks to 10 years. A retrospective study was made of 9 patients with congenital lobar emphysema who presented over the past years. Anomalies are infrequent and usually present at birth. Congenital lobar emphysema and tension pneumothorax in a dog gopakumar gopalakrishnan, gregory w. Congenital lobar emphysema cle, also known as congenital lobar overinflation and infantile lobar emphysema, is a neonatal condition associated with enlarged air spaces in the lungs of newborn infants. Cureus congenital lobar overinflation presenting with. Congenital lobar emphysema is characterized by 1 difficulty in breathing or very rapid respiration respiratory distress in infancy, 2 an enlarged chest due to overinflation of at least one lobe of the lung, 3 compressed normal lung tissue in the section of the lung nearest to the diseased lobe, 4 bluish color of the skin due to a lack. We report a case of a 3weekold female infant with a weight of 2. Congenital lobar emphysema and tension pneumothorax in a. Investigation revealed the overexpansion of the left upper lobe with mediastinal herniation. It is diagnosed around the time of birth or in the first 6 months of life, occurring more often in boys than girls. Lobectomy can be difficult because of the hyperinflated lobe and limited space to carry out surgery. Description congenital lobar emphysema, also called infantile lobar emphysema, is a respiratory disease that occurs in infants when air enters the lungs but cannot leave easily.
Cle presents with emphysema cle was made and posterolateral overexpansion of a pulmonary lobe and resultant thoracotomy planned with left upper lobectomy. Congenital lobar emphysema is defined by hyperinflation of 1 or more lung lobes in the absence of extrinsic bronchial obstruction. Pdf congenital lobar emphysema in infants samasca gabriel. Congenital lobar emphysema cle is a rare developmental lung anomaly, characterized by hyperinflation of one lobe most commonly the left upper lobe. Congenital lobar emphysema cle is a rare lung condition present at birth that can result in the lungs overinflating when air becomes trapped in one of the lungs lobes. Definition congenital lobar emphysema is a chronic disease that causes respiratory distress in infants. Congenital lobar emphysema cle is a rare pulmonary malformation whose main cause is probably developmental anomalies of the bronchial cartilage. Pdf congenital lobar emphysema cle is a rare but serious developmental lung malformation. Congenital lobar emphysema is a rare respiratory disorder in which air can enter the lungs but cannot escape, causing overinflation hyperinflation of the lobes of the lung. During the third week of gestation, the development of the respiratory system begins and aberrations in this developmental stage may cause parenchymal lung malformations. Congenital lobar emphysema cle is a com mon cause of severe respiratory distress in the first few months of life. 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. Congenital lobar emphysema prof eisa osman eiamin mbbs, dtch, frcp introduction congenital lobar emphysema cle is a rare disorder of lung development that calls for urgent diagnosis and management 1.
Other terms for cle include congenital lobar overinflation and infantile lobar emphysema. Congenital lobar emphysema can be distinguished from other lesions on ultrasonography by differences in echogenicity and reflectivity. Congenital lobar emphysema is a rare neonatal respiratory tract pathology that may cause an emergent clinical picture requiring urgent surgical intervention. Congenital lobar emphysema cle is a rare lung congenital malformation.
Signs and symptoms may include difficulty breathing and respiratory distress in infancy, an enlarged chest. It is congenital or present at birth, and in severe cases may. Congenital lobar emphysema definition of congenital lobar. Generally it is encountered in infants in the first year of life, but occasionally clamant symptoms may not appear until the second year. Congenital lobar emphysema cle is a rare anomaly of lung development that usually presents in the neonatal period with respiratory distress and pulmonary lobar hyperinflation.
Sep 12, 2018 congenital lobar overinflation should be considered in cases of prenatal echogenic lung lesions without macrocysts or classic findings of bronchial atresia. Management of congenital lobar emphysema with endobronchial. The clinical features of congenital lobar emphysema were first described in 1932 by nelson. A 4weekold female patient presented with severe respiratory distress, hypoxia and apnoeic episodes on a background of a fewday history of coryza and cough. The earlier the presentation, the more severe were the symptoms. Congenital lobar emphysema, a lifethreatening respiratory disorder presenting in infancy is best diagnosed by chest xray. This disease is characterized by hyperinflation of one or more lobes of the lung, leading to compression atelectasis on the ipsilateral or contralateral side and mediastinal shift. Chest radiographs demonstrates increased translucency of the left lung with mild mediastinal shift to the right side. Congenital lobar emphysema cle, also known as congenital lobar overinflation, is a rare but well described congenital lung malformation clm known to cause progressive hyperinflation and air trapping within one or more pulmonary lobes. It is quite possible for some infants with this condition to do extremely well, while prognosis for more severe cases can vary based on complications. Williamsbeuren syndrome and congenital lobar emphysema. Congenital lobar emphysema sudanese journal of paediatrics.
Jan 20, 2009 congenital lobar emphysema is a rare variety of congenital malformation of lung characterized by over distension of a lobe of a lung due to partial obstruction of the bronchus. We are reporting a neonate admitted in the pediatric emergency ward with the. Congenital lobar emphysema definition of congenital. Congenital lobar emphysema is a surgical emergency of infancy. Congenital lobar emphysema concurrent with pneumothorax. Current operative management of congenital lobar emphysema in. Left upper lobectomy for congenital lobar emphysema in a. Congenital lobar emphysema cle and tension pneumothorax tpt are rarely reported in dogs. Although most times, emphysema is thought of as an adult lifestyle disease frequently cause by smoking, cle, also called infantile emphysema, is very different. Thoracic ultrasound revealed left upper lobe hyperinflation causing mediastinal displacement to the right, a slightly reduced blood supply at this level, and a lobar. Congenital lobar emphysema is a rare variety of congenital malformation of lung characterized by over distension of a lobe of a lung due to partial obstruction of the bronchus. The exact nature of the congenital defect in the bronchus should be revealed by a more thorough study of similar cases. Congenital lobar overinflation clo, previously known as congenital lobar emphysema, is a rare neonatal disease, with a prevalence of 1 in 20,00030,000.
Cle has a wide range of presentations, and it poses a diagnostic and therapeutic dilemma. It is a very rare condition with an incidence of 1 in 20,000 to. Congenital lobar emphysema article about congenital. Congenital lobar emphysema american academy of pediatrics. Left upper lobectomy for congenital lobar emphysema in a low. The pathology is usually due to deficiency in cartilage of the bronchial wall im2m3. Congenital lobar emphysema cle and williamsbeuren syndrome are two rare conditions that have only been reported together in a single case study. Abstract the authors describe a case of congenital lobar emphysema in child at the age of 45 days and discuss the clinical manifestations as well as the features of histological diagnostic of the disease. Congenital lobar emphysema and tension pneumothorax in a dog. This pdf download is supported by maquet the makers of flowi.
Congenital lobar emphysema with pulmonary extramedullary. We report another case of a male caucasian newborn with nonspecific initial respiratory distress, with detection of cle on repeat chest xray on day 25 of life and concurrent ventricular septal defect. Congenital lobar emphysema cle, a rare condition that usually presents in the neonatal period, can be a diagnostic and therapeutic challenge for the treating clinician. The patient may also present with a radioopaque mass on chest xray because of delayed clearance of lung fluid of the. Oct 10, 2011 congenital lobar emphysema is a rare respiratory disorder in which air can enter the lungs but cannot escape, causing overinflation hyperinflation of the lobes of the lung. There was significantly reduced air entry on the left side and a displacement of the apex beat to the right of the chest. Prenatal diagnosis and management of congenital lobar emphysema. Congenital lobar emphysema associated with congenital heart.
Congenital lobar emphysema genetic and rare diseases. Congenital lobar emphysema is most often detected in neonates or identified during in utero ultrasound. Congenital lobar emphysema cle is a potentially reversible, though possibly. Hypervascularity may be an important imaging feature of a subset of clo. Clinical material nine patients with congenital lobar emphysema 5 boys and4girls were diagnosed between 1970 and. It is most often detected in newborns or young infants, but some cases do not become apparent until adulthood.
R upper lobe bronchus showing stenosis of bronchus with valvelike fold of mucosa directed peripherally. Congenital lobar emphysema cle is a rare developmental malformation of the lung but can be associated with high morbidity and mortality. Congenital lobar emphysema in infants is a disorder that is detected most often in newborns or young infants. The diagnosis of probable congenital lobar emphysema of the right lung was entertained, but she was dis charged because of lack of symptoms. People with congenital lobar emphysema will also need to be followed by a pulmonologist, and length of time between doctor visits will depend on its severity. Congenital lobar emphysema is a rare disorder characterized by air trapping in a segmental or lobar distribution, which causes local mass effect 1, but does not destroy the tissue. Such diverse conditions as congenital atel ectasis, agenesisof one lung, congenital cyst, an inhaled foreign body, and lastly inter stitial emphysema may on occasion produce an identical radiologic picture to lobar em physema.
Congenital lobar overinflation clo, previously called congenital lobar emphysema, is a congenital lung abnormality that results in progressive overinflation of one or more lobes of a neonates lung. We are reporting a neonate admitted in the pediatric emergency ward with the respiratory distress since 16 th day of life. Congenital lobar emphysema cle is a life threatening yet potentially reversible cause of respiratory distress in the neonate. Lobar distention can be visible during in utero ultrasound as an overinflated, fluidfilled lobe. A case of cle of the right middle lung lobe predisposing to air trapping, alveolar hyperinflation, and. On imaging, it classically presents on chest radiographs as a hyperlucent lung segment with overinflation and contralateral mediastinal shift. If the condition is not suspected, it might readily be missed at routine autopsy, and consequently may occur more. Most cases become less conspicuous, decrease in size without overt hydrops, and are asymptomatic postnatally. Congenital lobar emphysema cle is a rare anomaly of lung development, which often presents in the neonatal period with hyperinflation of one or more pulmonary lobes. Although congenital lobar emphysema is a rare lung disease, it can cause severe respiratory distress in the newborn.
Omer faruk demir, 1 melih hangul, 2 mehmet kose 2 1 department of thoracic surgery, erciyes university, kayseri, turkey. We describe a case of cle in a 3monthold boy who was initially treated for suspected aspiration pneumonia at the referring hospital. On the lateral projection the hyperlucency is confined to the left upper lobe, shifting the oblique fissure posteroinferiorly. Congenital lobar emphysema cle is a well recognized yet uncommon clinical entity in human infants, which has uncommonly been described in dogs. Left upper lobectomy was performed and she had an uneventful recovery.
543 862 180 277 909 712 412 293 838 689 24 907 938 24 852 108 567 1488 1209 1235 516 364 1430 964 199 1174 1216 68 647 120 126 611 99 924 872 652 846 416 421