Congenital lobar emphysema: experience with 21 cases

A retrospective study of all cases (n=21) of congenital lobar emphysema (CLE) treated at the Royal Hospital, Muscat, from September 1988 to August 1999 was performed. The presentation, diagnosis, treatment, and outcome are reviewed. All 21 patients were non-white. Thirteen had left-upper-lobe, 7 right-middle-lobe, and 1 right-upper-lobe involvement. Of the 14 upper-lobe cases, 10 had severe symptoms whereas only 2 of the 7 middle-lobe cases had severe symptoms. Mildly symptomatic cases on conservative treatment invariably needed surgery if infected. It is concluded that CLE is not limited to white infants, upper-lobe disease is more severe than middle-lobe disease, and nonoperative measures are likely to be useful in mildly symptomatic cases involving the middle lobe. Accepted: 12 May 2000     

Congenital lobar emphysema: Differential diagnosis and therapeutic approach

Background: Congenital lobar emphysema (CLE) is a rare anomaly of lung development that usually presents in the neonatal period with respirator distress and pulmonary lobar hyperinflation. It is commonly confused with pneumothorax. The aim of the present paper was to review the authors’ experience in order to emphasize the importance of differential diagnosis with pneumothorax. Methods: Children with CLE treatment at Department of Thoracic Surgery, Dicle University School of Medicine, Turkey, between January 1993 and June 2004, were reviewed. Results: Ten children consisting of six boys and four girls (age range, 6 h–12 months) had CLE. Major presenting symptoms were tachypnea(n = 100%) and respiratory distress in (n = 80%). On chest radiograph, emphysema was seen in all patients, and shift‐herniation to the opposite lung, atelectasis were observed. Computed tomography was performed in all patients, which indicated emphysema in the affected lobes in all cases. Pulmonary perfusion scan was performed in two patients, showing loss of perfusion in the affected lobe. The most common affected lobe was the left upper lobe (50%). In the present series, three patients were mistakenly diagnosed as pneumothorax and intercostal drains were inserted in the emergency department. Eight patients underwent lobectomy, and postoperative course was uneventful. Two patients were followed conservatively. Emphysema was detected in all pathological specimens. One patient was lost to follow up. Mean follow‐up duration of all patients was 26.8 ± 29.24 months (range, 1–89 months). Conclusions: CLE is established on combined clinical, radiological and scintigraphic imaging. Surgical excision of the affected lobe is the appropriate treatment. Particularly, differential diagnosis should be made between CLE and pneumothorax.     

Progressive interstitial pulmonary lobar emphysema

Progressive pulmonary interstitial lobar emphysema is a complication of artificial ventilation in premature infants with RDS. Three cases are presented who developed a progressive form of PIPE. It is demonstrated that PIPE is caused by air escaping through alveolar leaks into the pulmonary lymphatic capillary system causing dilation of lymphatic channels. Because of its increasing compressive effect on adjacent lung areas PIPE requires rapid and effective therapy. Although several types of conservative approach are suggested, lobectomy appears to be the method of choice. Lobectomy is not indicated when prolonged ventilation with high pressures and high concentrations of oxygen have caused severe bronchopulmonary dysplasia.     

Giant lobar emphysema — Neonatal diagnosis

Giant emphysema of a lung lobe has distinctive features. Classically there is overdistension of the affected lung lobe, with one lobe only being involved, and, 50% of cases occur in the newborn infant [1, 4, 8].The authors describe a particularly severe example with marked mediastinal shift and initially the hemithorax on the side of the lesion was opaque. Angiography was carried out and followed by resection when the infant was 4 months old.     

Ball valve obstruction of a bronchus causing lobar emphysema in a neonate

Lobar emphysema in the neonate is usually congenital, resulting from cartilage deficiency causing bronchomalacia and distal air trapping. Acquired forms are usually associated with chronic lung disease or endobronchial obstruction such as mucus plugging. We report a pedunculated endobronchial polyp in a 2-month old ex-premature infant causing intermittent hyperinflation of the right middle lobe. The polyp was seen prolapsing in and out of the bronchus intermedius at bronchoscopy. Possible aetiological links with mechanical ventilation are discussed. We also emphasise the value of bronchoscopy prior to lobectomy in cases of congenital lobar emphysema (CLE).     

The value of cardiac catheterization and cineangiography in infantile lobar emphysema

Lobar emphysema is an uncommon cause of respiratory distress in infancy. Congenital heart disease is seen in about 20% of the patients with infantile (congenital) lobar emphysema. We described six infants with lobar emphysema. In three of them a congenital heart disease was demonstrated by cardiac catheterization and cineangiography; two had a tetralogy of Fallot with right aortic arch and the third infant a ventricular septal defect. The pulmonary angiography showed stretching of the arteries with very poor filling of the peripheral arteries and a characteristic smaller pulmonary vein in the affected lobe. In all the six patients the pulmonary artery pressure was normal. All the patients underwent lobectomy with good results. We feel that a preoperative cardiac catheterization and cineangiography is of value in this very sick group of infants.     

Congenital cystic lesions of lung in the paediatric population: A 5-year single institutional study with review of literature

Background:

The aim was to evaluate the clinical presentation, investigation modalities, operative management, pathology, outcome (morbidity and mortality) and short term follow-up of congenital cystic lesions of the lung.

Materials and Methods:

This is a retrospective study. Study period was 5 years (December 2008-November 2013) in the Department of paediatric surgery, Nil Ratan Sircar Medical College. Study population: Total number = 10 patients. Age range: 2 days-7 years. (Neonate-4). Male and female ratio = 1:1.

Result:

Among 10 cases of lung cyst four having congenital lobar emphysema, four having congenital pulmonary airway malformation, one sequestration and one teratoma. All patients have undergone surgical excision in terms of lobectomy or excision of the lesion. Post-operative histopathology confirmed the diagnosis. Recovery was uneventful.

Conclusion:

Although our experience is limited, operative management of lung cysts seems to be safe with rewarding results. However we are yet to encounter many of the other varieties of the cysts found in the lung, which may be associated with other congenital anomalies and have an impact on prognosis.     

Lobar emphysema complicating hyaline membrane disease

A pre-term infant with severe hyaline membrane disease (HMD), requiring intubation and mechanical ventilation, who developed bronchopulmonary dysplasia (BPD), which was followed by lobar emphysema in the right lower lobe is presented. Surgical removal of the right lower lobe led to prompt improvement in the oxygen requirements and subsequent recovery of the infant's respiratory distress. Pathology revealed marked stenosis of the right lower lobe bronchus and air-trapping, without destruction of alveoli. The lobar bronchus contained adequate cartilage, but deficient muscle at the site of stenosis. It seems likely that this bronchial stenosis, and resultant lobar emphysema was not congenital, but was a complication of HMD, BPD or therapy.     

The CT appearances of delayed amniotic fluid clearance from the lungs in an infant with absent pulmonary valve and congenital lobar emphysema

Congenital lobar emphysema (CLE) is a cause of severe neonatal respiratory distress. Overexpansion of the affected pulmonary lobe in the fetus is due to narrowing of the airway, with a resultant ball-valve effect. At birth, there may be delayed clearance of fetal lung fluid. Early chest radiographs show opacification of the hyperexpanded lobe. The CT findings in the immediate neonatal period have not been previously reported. We describe the imaging in a neonate with tetralogy of Fallot and absent pulmonary valve with secondary CLE. CT demonstrates the hyperexpanded lobe with initial thickening of the interlobular septa and alveolar ground glass attenuation, with subsequent clearing. This resorption of fetal lung fluid via the pulmonary interstitium should not be confused with interstitial lung disease.     

Pulmonary interstitial emphysema due to respiratory syncytial virus infection

Pulmonary interstitial emphysema (PIE) primarily affects premature infants on positive pressure ventilation. PIE is rarely reported in infants and children in the absence of mechanical ventilation and/or associated respiratory infection. We report a case of PIE in a 22‐month‐old girl who had severe respiratory distress due to respiratory syncytial virus infection. Chest computed tomography showed cystic lung lesions mimicking congenital cystic adenomatoid malformation. The cystic lesions spontaneously resolved after conservative treatment. Based on the clinical course and the chronological changes on imaging, the cystic lung lesions were diagnosed as localized persistent PIE.     

Outcome of congenital lung abnormalities detected antenatally

To determine the outcome of congenital lung abnormalities, data were collected retrospectively between January 1991 and December 1996 on any foetus found to have a lung lesion on antenatal ultrasound. A total of 23 foetuses had lung lesions on antenatal ultrasound. In two foetuses the antenatal ultrasound showed bilateral enlarged "bright" echogenic lungs with evidence of hydrops. Both pregnancies were terminated and tracheal atresia was confirmed. In 15 foetuses the antenatal ultrasound appearance was of a unilateral "bright" echogenic lung. There was one case of bronchial atresia and two cases of congenital lobar emphysema, which all had surgery. In nine cases there was a reduction in the size of the lesion on serial antenatal ultrasounds and no lesion was detected after birth. In three cases a small lesion was present after birth on chest radiography. In six foetuses the antenatal ultrasound appearance was of unilateral cystic or mixed cystic and echogenic lung lesions. Two pregnancies were terminated; both had congenital cystic adenomatoid malformation. Four pregnancies were continued and three infants had surgery soon after birth and were confirmed to have had congenital cystic adenomatoid malformation. One infant has been managed conservatively. In conclusion, a definitive diagnosis cannot usually be made antenatally. A large lesion on initial scan does not necessarily predict a poor outcome. The natural history of small asymptomatic postnatal lesions is unknown and a long-term prospective study is needed to determine the outcome of these lesions.     

An unusual late presentation of a congenital diaphragmatic hernia

An unusual and late-presenting case of left Bochdalek diaphragmatic hernia is presented to highlight the fact that the timing and mechanism of presentation of the hernia remains obscure. In this case there were two previous admissions, initially for a left upper lobe lobectomy for congenital lobar emphysema and a subsequent admission for bronchiolitis. He then presented at the age of nine months, with acute respiratory distress and was diagnosed to have a left diaphragmatic hernia. This is the first case of a late- presenting congenital diaphragmatic hernia reported in association with a previous ipsilateral lobectomy for congenital lobar emphysema. A brief review of late -presenting congenital diaphragmatic hernias is given and an alternative theory is proposed for the mechanism of late presentation.     

Selective intubation in pulmonary interstitial emphysema: experience in five patients

Abstract Selective bronchial intubation can be useful in the management of severe localized pulmonary interstitial emphysema (PIE). Five infants between 9 and 20 days old with severe left sided PIE had the right main bronchus intubated for between 4 to 30 h. Four patients showed permanent and one patient temporary improvement and the experience from these cases suggests that prolonging the procedure after obtaining radiological evidence of improvement may not be necessary. There were no serious complications although right upper lobe atelectasis occurred in three cases and could not be prevented by using an endotracheal tube with a side hole. Nevertheless, active management should only be considered in PIE causing respiratory failure or pressure effects on surrounding structures.     

Congenital lobar emphysema: a clinicopathologic evaluation of 14 cases.

Controversy still exists concerning the diagnosis and treatment of congenital lobar emphysema (CLE). Although surgical removal of the affected lobe is the most commonly accepted form of treatment, detection of milder or even asymptomatic cases is usually followed by a more conservative management of patients, i. e. non-surgical treatment and follow-up. We therefore decided to evaluate our patients with CLE, placing special emphasis on treatment and diagnostic techniques. We also evaluated quantitative analyses of alveolar diameters. Fourteen children with CLE were analysed retrospectively, including age, sex, clinical picture, localisation, diagnostic and surgical modalities and histopathologic diagnosis. The alveolar diameters of affected lobes were compared with those of the lobectomised patients with other non-obstructive respiratory diseases. All children but one had severe respiratory distress as an initial symptom. All patients, except newborns, had a history of pulmonary infection. All cases underwent thoracic CT examination as the main radiologic method. In all of the patients, only one lobe was affected. We found an obvious mediastinal shift and atelectasis of adjacent lobes due to compression of the affected lobe. The affected lobe was therefore surgically removed in all of the children. In one case, we had to carry out a partial lobectomy to reduce the duration of the operation, due to an intraoperative fall of oxygen saturation. All of the children had an uneventful clinical course postoperatively. The alveolar diameters of the cases with CLE were significantly greater than those of the control patients. We think that the majority of cases with CLE have too severe respiratory distress to avoid surgical removal of affected lobe. Conservative management should be reserved only for patients with milder symptoms or no distress at all.     

Congenital lobar emphysema : A review of 10 cases

We reviewed the clinical features and management of 10 patients with congenital lobar emphysema (CLE) treated in our institution over a period of seven years, from September 1988 to September 1995. The age of onset of symptoms ranged from few days from birth to six months. Correct diagnosis was made in only 4 patients before referral, even though diagnosis could have been arrived at from the chest radiograph in all of them. Lobectomy was done in seven patients, while one patient had vascular ring resected. Of the three patients managed conservatively, in one lobectomy was performed at the end of 5 months conservative course as her clinical condition deteriorated during an intercurrent chest infection. Of the remaining two, one has mild episodic reactive air way disease and the other patient is asymptomatic.     

大叶性肺炎形成支气管黏液栓高危因素分析

目的 探讨大叶性肺炎形成支气管黏液栓的临床特征和高危因素.方法 选取2018年1月至2020年3月收治的大叶性肺炎患儿临床资料.根据纤维支气管镜下情况分为黏液栓组和非黏液栓组,比较两组患儿临床特征和实验室检查结果.结果 共收治大叶性肺炎患儿935例.根据纤维支气管镜检查结果纳入黏液栓组135例,男73例、女62例,中位...