Congenital Adenomatoid Malformation Research Articles

The use of “nonanatomic” pulmonary resection in infants with extensive congenital adenomatoid malformation of the lung : D. Browdie, D. Todd, R. Agnew, et al. J Thorac Cardiovasc Surg 105:732–736, (April), 1993

The use of “nonanatomic” pulmonary resection in infants with extensive congenital adenomatoid malformation of the lung : D. Browdie, D. Todd, R. Agnew, et al. J Thorac Cardiovasc Surg 105:732–736, (April), 1993

The use of “nonanatomic” pulmonary resection in infants with extensive congenital adenomatoid malformation of the lung

In three infants with extensive congenital adenomatoid malformation of the lung, a method of nonanatomic pulmonary resection permitted preservation of small superiorly positioned pulmonary remnants. These remnants then demonstrated a surprising capacity for expansion, growth, and function during follow-up periods of 5 years, 9 months; 7 years, 6 months; and 19 years. Thus far, this small experience indicates that this method of management does not appear to entail greater short- or long-term risks than the alternative procedure of pneumonectomy.

Prenatal management of congenital cystic adenomatoid malformation of the lung

In utero evolution and postnatal outcome were studied for 18 cases of congenital adenomatoid malformation (CAM) diagnosed by prenatal ultrasound. Five were macrocystic CAM, 9 were microcystic, and 4 were homogeneously hyperechogenic. Three fetuses presented with hydrops. Pulmonary amniotic shunting was performed in 3 patients. Outcome was the following: 4 were aborted, 1 died neonatally, and 13 survived. Four of these infants required no surgery in the neonatal or postneonatal period. In three of these, the size of the mass had decreased spontaneously in utero. Outcome did not appear to be related to the anatomic type of CAM nor to the presence of moderate polyhydramnios, but was related to the degree of mediastinal compression and to the existence of hydrops. A clearer understanding of the natural evolution of CAM is useful to determine the indications for in utero therapy.

Symptomatic cysts in otherwise normal lungs of children and adults

The clinical and pathological features of 28 lung cysts resected in the period 1980-1989, excluding those from patients with emphysema elsewhere in their lungs, have been reviewed. In 12 children aged 8 days to 17 years, five cysts were congenital adenomatoid malformations, three were bronchogenic cysts, two were intralobar sequestrations, one was a cystic haemangioma and one resembled the cysts excised from 16 adult patients. This latter group ranged in age from 20 to 62 years and included 11 cigarette smokers and five asthmatics. Twelve of these cysts were intralobar and four were attached by a pedicle to the pleural surface of the lung. All these cysts had a fibromuscular wall showing varying degrees of acute and chronic inflammation. The presence of at least a partial lining of epithelial cells in all the cysts was confirmed using an immunocytochemical marker. The surrounding lung did not show any significant pathology. These cysts are labelled as simple fibromuscular pulmonary cysts. In the childhood cases, a congenital cause could be established in the majority. The pathogenesis of the adult cysts remains unclear. The presence of inflammation in the cyst walls does not necessarily suggest a role for infection, as secondary infection of cysts cannot be ruled out. An aetiological role for local damage due to cigarette smoking or asthma must be taken into consideration.

Early prenatal diagnosis of congenital cystic adenomatoid malformation of the lung (Stocker's type I); a case report

A congenital cystic adenomatoid malformation was diagnosed by ultrasound examination at 20 week's gestation. The entire right lung was cystic and elements of poor prognosis such as hydrops fetalis and polyhydramnios were present. After verification of the karyotype, abortion was performed and autopsy confirmed prenatal findings and Stocker's type I. Cystic congenital adenomatoid malformation of the lung is a rare form of congenital pulmonary disease. Our case shows that this malformation can be accurately diagnosed during the midtrimester of pregnancy by ultrasound examination. Ultrasound examination permits an evaluation of the three types described by Stocker and may reveal certain lesions associated with poor prognosis, such as anasarca or polyhydramnios. Also, it offers the possibility to save some fetuses by surgical decompression in the immediate postnatal period, or to terminate earlier pregnancies by abortion.

Congenital adenomatoid malformation of the lung: Current management and prognosis

A retrospective 10-year review of congenital adenomatoid malformation (CAM) included 10 cases diagnosed in utero by ultrasound and 13 cases that presented postnatally. Two prenatally diagnosed cases were aborted because of associated lethal anomalies. All remaining patients underwent resection. Up to one third of all cases, whether diagnosed prenatally or postnatally, were asymptomatic. Resection is recommended to avoid respiratory distress, infection, or associated malignancy. There were 5 nonsurvivors, including 2 therapeutic abortions and 3 who died postoperatively. All had either polyhydramnios or ascites. All patients who died postoperatively had a respiratory arrest at birth and underwent immediate lobectomy. All died on the first day of life after a brief period and were found to have associated pulmonary hypoplasia. One had undergone a prenatal transthoracic cyst aspiration at 34 weeks gestation in an attempt to allow lung growth and prevent premature labor. Prenatal ultrasound findings suggestive of poor prognosis included polyhydramnios, ascites, mediastinal shift, and noncystic type III CAM. However, there is a spectrum of severity of CAM. The lesion can either regress and be asymptomatic at birth, or it can progress to cause either fetal death from hydrops or neonatal death caused by associated pulmonary hypoplasia. These findings should be considered in prenatal counseling for CAM.

SIX CASES OF PULMONARY SEQUESTRATION

Pulmonary sequestration is a relatively uncommon cystic lung disease. Six cases of the disease treated in our department up to now, are clinically studied. The patients were comprised of 1 nowborn, 1 infant. 2 schoolboy and girl, and 2 adults. Five cases had intralobar sequestration, and the remaining one, extralobar sequestration. Three cases were left sided, and 3, right sided.Initial manifestations by which the disease was found included infectious symptoms such as repeated pneumonia, hemosputum, and hemoptysis in 4 cases, and dyspnea in one, but one case was accidentally found by chest X-ray without any complaints. Correct preoperative diagnosis could be achieved in 2 out of 4, in which angiography was performed, but in the other 4 cases, differential diagnosis from other cystic lung diseases was difficult. All cases were operated on. Except a case of extralobar sequestration, lobectomy or segmentectomy was done. The extra lobar sequestration had a Congenital adenomatoid malformation (CCAM) in the same side, and sequestrated lobe was also CCAM.

Pränatale Diagnose der adenomatoiden Lungendysplasie Typ II

Congenital adenomatoid malformation of the lung is a rare form of lung disease, characterised by excessive overgrowth of terminal respiratory elements. From the histological as well as the sonographical point of view it can be classified into 3 subtypes. The prognosis depends on the type and the associated malformations. We report on two cases of congenital adenomatoid malformation of the lung type II, diagnosed by ultrasound before the 23rd week of gestation. Criteria that may be useful in the prenatal diagnosis of this malformation are described, and the clinical management is discussed.

Cartilaginous Variant of Congenital Adenomatoid Malformation of the Lung

We report the occurrence of an unusual variant of congenital adenomatoid malformation (CAM) of the lung in a 16-year-old male. Abundant cartilage in the walls of malformed bronchioles, an extremely rare finding in CAM, distinguishes this lesion from the usual forms. We present the roentgenographic, computed tomographic, and pathologic features of this lesion as well as its embryologic implications. In addition, two similar cases are reviewed, one of which has been briefly discussed in a previous publication. Cartilaginous CAM should be distinguished from another lesion with abundant cartilage, the so-called pulmonary hamartoma.

Diagnosis and management of congenital cystic disease of the lung in children

Pulmonary sequestration, congenital adenomatoid malformation (CAM), congenital lobar overinflation (CLO), and bronchogenic cysts are four congenital lesions that may present as abnormal cystic areas within the pleural cavity in early life. They share similar clinical and embryologic characteristics, are frequently difficult to diagnose, and all require surgical treatment. From December 1974 to January 1985, 22 patients were operated upon on the Pediatric Surgical Service for congenital cystic disease of the lung. There were eight females and 14 males, ranging in age from 1 day to 18 years; 12 were under 6 months of age, and 14 were under one year. There were 7 CAMs, 5 extralobar pulmonary sequestrations, 5 intralobar pulmonary sequestrations, 3 bronchogenic cysts, and 3 cystic lobes caused by CLO. One patient had bilateral pulmonary sequestrations, intralobar on one side and extralobar on the other, with both sharing a common systemic artery arising from the infradiaphragmatic aorta. All except two asymptomatic patients presented with either progressive respiratory distress or recurrent pulmonary infections, and had cystic changes noted on chest roentgenograms. The differentiation of these congenital lesions from resolvable inflammatory cysts is important. Diagnosis was aided by selective utilization of barium contrast studies, ultrasonography, computed tomography, and arteriography. Operation consisted of resection alone of the extralobar pulmonary sequestrations and bronchogenic cysts, and total lobectomy in CAM, intralobar sequestration, and CLO. One infant with CAM died 1 day postoperatively from bilateral hypoplastic lungs. The other 21 patients are alive and well with follow-up ranging from 1 month to 9 years, with a mean of 3.5 years.(ABSTRACT TRUNCATED AT 250 WORDS)

Extralobar Pulmonary Sequestration: Analysis of 15 Cases

Extralobar pulmonary sequestrations occurring in 15 patients were studied. Twelve of the lesions were discovered during the first day of life; all of these patients died. The lesions occurred more often in male patients (11 cases). Polyhydramnios was present in four infants, and these four plus an additional infant had localized or generalized edema. Associated congenital anomalies were present in ten patients, including three examples each of diaphragmatic hernia and bronchogenic cyst. In one case the extralobar pulmonary sequestration was composed entirely of a congenital cystic adenomatoid malformation. In one other case, congenital adenomatoid malformation was present in a nonsequestered portion of lung. Significant lymphatic dilatation was found in four cases, two of which resembled congenital lymphangiectasis. Hyaline membranes, present in the normal lungs of three preterm infants, were absent in extralobar pulmonary sequestrations.

Pleomorphic pulmonary hamartoma: An apparently unique variant of pulmonary hamartoma

Pulmonary hamartomas of limited variety have been described. Most present as asymptomatic coin lesions in adults and consist of mesenchymal tissue, usually cartilage, in combination with irregular spaces lined by epithelium. Another form is found in the neonate and involves large portions or all of a lung. This is associated with a developmental aberration and is best described as a “congenital adenomatoid malformation”. An apparently unique noninvasive tumor mass was resected from the lung of a middle aged man where it was associated with anomalous lung segmentation and the bronchial and blood supply to the lung. The tumor appeared to be undifferentiated by light microscopic criteria. Neurilemoma, leiomyoma, chordoid tumor, mixed tumor, and neurogenic sarcoma all entered the differential diagnosis. Ultrastructural examination demonstrated a highly complex and unique organization. Such a lesion, apparently developmental in this case, should be recognized and carefully distinguished from the malignant mesenchymal, neurogenic, and teratomatous lesions with which it may be confused. Electron microscopy may be helpful in this regard.

A congenital adenomatoid malformation of the lungs in a calf.

A 7-month-old fetal calf had a diffuse, solid adenomatoid malformation of the left diaphragmatic lobe of the lungs. The malformation consisted of a large white mass with a lobular pattern. The lobules had a sponge-like structure without any cysts and contained dilated bronchioles, lined by a single layer of epithelial cells and a layer of elastic fibres. There were neither cartilaginous rings nor muscle fibres. The anomaly was associated with generalized fetal hydrops, oedema of the fetal membranes, and indications of hydramnion or hydrallantois.

The various types of congenital adenomatoid malformation of the lung

The various types of congenital adenomatoid malformation of the lung

The opaque lung in lobar emphysema.

Two cases of lobar emphysema which exhibited overexpanded opaque lung fields as opposed to typical overdistended hyperlucent lung fields are reported. This phenomenon, termed the opaque lung of lobar is discussed with reference to its mechanism of development (probably fluid retention), roentgenographic features, and differential diagnosis.It is important to recognize opaque lung of congenital lobar emphysema, for roentgenographic findings may be misinterpreted as representative of some other condition, i.e., empyema, chylothorax, intrathoracic mass, congenital diaphragmatic hernia with no air, airless congenital adenomatoid malformation, etc. Diagnosis depends on being aware of this unusual configuration for congenital lobar emphysema, and appreciating rapidity with which it yields to more classic overdistended hyperlucent lung.

Five Cases of Lobar Tension Emphysema in Infancy; Importance of Bronchial Malformation and Value of Postoperative Steroid Therapy

Five cases of lobar emphysema are presented. We want to emphasize: a) the urgency of surgical intervention; b) the variability of the causative lesions, some being instances of congenital bronchomalacia of the bronchial root, others of congenital adenomatoid cystic malformation; and c) the value of intravenous steroid therapy in relieving postoperative pulmonary collapse or bronchospasm.

Congenital Adenomatoid Malformation of Lung with Pneumothorax

Congenital Adenomatoid Malformation of Lung with Pneumothorax

Fluoroscopy in pediatric radiology and the implications of image intensification.

Increasing Knowledge of the biologic effects of ionizing radiation necessitates constant reappraisal of radiologic technics. The aim of any diagnostic radiologic procedure is to establish an early and accurate diagnosis while exposing the patient to no more radiation than is necessary. Particularly in the pediatric age group, radiologic studies must be carefully selected so as to be informative and yet require a minimum of exposure to irradiation. Fluoroscopy is a potentially hazardous tool because of the amount of exposure which is involved, but it is the one method by which physiologic phenomena are readily observed. When properly employed, it represents an invaluable adjunct to other radiographic studies. It should not, however, supplant the latter, since the physical factors are such as to allow fewer details to be appreciated at comparable exposures and since it affords no permanent record for future reference. Although the limitations of fluoroscopy in infants and children parallel those in adults, the nature of certain disease processes in early life renders fluoroscopic examination of even greater value in this age group. Acute inflammatory disease of the lungs in an infant may be first manifested by obstructive emphysema, which is difficult or impossible to recognize on radiographic study. Moreover, early in life emphysema may be the only manifestation of cystic fibrosis of the pancreas. Disturbances in aeration of the lungs, which are best recognized fluoroscopically, often accompany congenital lesions of the tracheo-bronchial tree, of the pulmonary parenchyma, and of the heart and great vessels, e.g., congenital lobar emphysema, congenital adenomatoid malformations of the lungs, and vascular rings. Fluoroscopic observation in order to determine the size and nature of pulsation of the major vessels, the character of the intrapulmonary vasculature, and the configuration of the heart, is an integral part of the radiologic approach to congenital heart disease. Evaluation of esophageal activity in the infant who regurgitates may reveal a functional basis for such regurgitation. Under certain circumstances the diagnosis of pyloric stenosis and intussusception may be established by means of fluoroscopy and the diagnosis of aganglionic megacolon follows careful fluoroscopic study. Recognition of the value of fluoroscopy in pediatric radiology implies not only an appreciation of the functional manifestations of disease but also of the possibilities for teaching and for research which are inherent in the method. Therefore, a device that produces a fluoroscopic image one-thousand times brighter than that of the conventional screen, with significantly lower exposure to the patient, must be considered a major radiologic advance. An image intensifier of the electron optical type, manufactured by the North American Philips Company, Inc., has been in use for approximately one year at St. Christopher's Hospital for Children.