Lung growth and airway function after lobectomy in infancy for congenital lobar emphysema.

To characterize the outcome of lobectomy in infancy and the low expiratory flows which persist after lobectomy for congenital lobar emphysema, 15 subjects with this history were studied at age 8-30 yr. Total lung capacity was normal in all, but higher values (P < 0.05) were observed in nine subjects with upper lobectomy than in five subjects with right middle lobectomy. Ratio of residual volume to total lung capacity was correlated (P < 0.05) with the amount of lung missing as estimated from normal relative weights of the respective lobes. Xe(133) radiospirometry in eight subjects showed that the operated and unoperated sides had nearly equal volumes at total lung capacity, but that the operated side was larger than the unoperated side at residual volume. Perfusion was equally distributed between the two sides. Similar findings were detected radiographically in four other subjects. Forced expiratory volume in 1 s and maximal midexpiratory flow rate averaged 72 and 45% of predicted, respectively. Low values of specific airway conductance and normal density dependence of maximal flows in 12 subjects suggested that obstruction was not limited to peripheral airways. Pathologic observations at the time of surgery and morphometry of the resected lobes were not correlated with any test of pulmonary function. These data show that lung volume can be completely recovered after lobectomy for congenital lobar emphysema in infancy. The volume increase occurs on the operated side, and probably represents tissue growth rather than simple distension. The response to resection is influenced by the particular lobe resected and may be associated with decreased lung recoil near residual volume. Low expiratory flows in these subjects could be explained by several mechanisms, among which a disproportion between airway and parenchymal growth in infancy (dysanaptic growth) is most compatible with our data.

Effects of therapeutic irradiation delivered in early childhood upon subsequent lung function.

To determine the long-term effects of therapeutic pulmonary irradiation and treatment with actinomycin D during a period of lung growth, 12 patients treated for Wilms' tumor metastatic to the lung and 8 patients treated for Wilms' tumor with no evidence of pulmonary metastases were studied 7 to 14 years after their initial tumor therapy. All patients had received irradiation to the tumor bed and treatment with actinomycin D. Group 1 had received a single course of bilateral pulmonary irradiation; group 2 had received additional pulmonary irradiation and/or thoracic surgery; group 3 had received no therapeutic irradiation directed primarily to the chest. Total lung capacity (TLC) averaged 71% of predicted value in group 1, 58% in group 2, and 94% in group 3. Diffusing capacity in groups 1 and 2 was reduced to the same extent as lung volume. Quasi-static pressure-volume relationships, studied in three of six patients in group 1, were within the normal range when lung volume was expressed as percentage of observed TLC. Airway resistance, evaluated by spirometry, maximum expiratory flow-volume curves, and resistance of the total respiratory system, was normal or reduced. The data support the hypothesis that therapeutic irradiation during a period of lung growth primarily affects the lung parenchyma and produces a decrease in subsequent size of both the lung and chest wall. No effect of actinomycin D alone upon the lung could be demonstrated.

Omphalocele and multiple severe congenital anomalies associated with osteodysplasty (Melnick-Needles syndrome).

Osteodysplasty (Melnick-Needles syndrome, MNS), a severe bone dysplasia with presumed autosomal dominant inheritance, has now been described in 24 individuals, with a predominance of females (21:3). We report an affected woman who gave birth to a male infant with omphalocele, hypoplastic kidneys, and the skeletal changes of this disorder; he died soon after birth. Histologic studies of the calvaria and long bones showed normal maturational sequences, but suggest that remodeling was not normal. This is the second known instance of a male infant with omphalocele and this skeletal dysplasia born to a woman with MNS. We suggest that the gene for the MNS may also cause a syndrome of multiple abnormalities that can be lethal and that this more severe phenotype in males may account for the altered sex ratio among reported cases. Both X-linked dominant and autosomal-dominant sex-limited inheritance are feasible interpretations of the existing information.

The pediatric tracheostomy: III). An appraisal of xerography.

Comparatively new in the roentgen evaluation of infants and children, xerography would appear to be ideal for portraying the pediatric airway. Since the radiation dose of xerography is higher than conventional radiography, its image quality is the subject of careful scrutiny in a pediatric setting here described in 23 infants and children with tracheostomies. In this appraisal three pediatric radiologists independently compared each child's xeroradiograph with an accepted control, conventional radiography. The principal conclusion: the xerographic image of the pediatric airway is slightly superior to standard radiography in most studies (40 of 47 paired studies, 85.1%). In all but two comparisons, however, the tracheal morphology is correctly perceived, described, and interpreted in both the standard radiograph and the xeroradiograph. The main role of xerography, then, is its verification of tracheal detail as first depicted by the preliminary radiography. This confirmation is important in this particular pediatric problem, the tracheostomy, which has inherent difficulties in correct roentgen diagnosis.

Prospective evaluation by computed tomography and pulmonary function tests of children with mediastinal masses.

BACKGROUND: Our ability to predict respiratory compromise during general anesthesia in a child with an anterior mediastinal mass is limited. Two prior reports have found a correlation between adequacy of ventilation during general anesthesia and the tracheal cross-sectional area obtained from computed tomograms (computed tomography [CT] scans). These and other reports have suggested that pulmonary function tests may provide additional information regarding anesthetic risks, but no studies have evaluated the extent of respiratory compromise in children with an anterior mediastinal mass. METHODS: We prospectively evaluated 31 children with mediastinal masses before 34 surgical procedures. At each evaluation the tracheal area (as a percent of the predicted area on the basis of age and gender) was determined by CT. Pulmonary function tests were performed in the sitting and supine positions. The eleven children with either a tracheal area or peak expiratory flow rate (PEFR) of less than 50% of predicted received only a local anesthetic; the majority of children above these levels (17 of 22) received a general anesthetic. RESULTS: Eleven of 31 patients had significant pulmonary restriction as defined by total lung capacity of less than 75% of predicted. Eight patients had a PEFR in the supine position of less than 50% of predicted. PEFR was lower in the supine than the upright position in all patients (median value of decrease, 12%). In 28 of 34 evaluations the child had a tracheal area greater than 50% of predicted, a criterion proposed for safe utilization of general anesthesia. This latter guideline, however, did not identify all patients with significant impairment of pulmonary function; five patients had a PEFR of less than 50% of predicted but tracheal areas of greater than 50% of predicted. All children were administered anesthetics uneventfully with these guidelines. CONCLUSIONS: Although the tracheal area can be accurately measured with the CT scan, this does not identify all children with mediastinal masses and abnormal pulmonary function. A large mass may produce significant restrictive impairment and hence reduction in PEFR by the intrathoracic volume it occupies and yet not cause tracheal compression. It may also reduce the PEFR by narrowing the bronchi distal to the carina. Currently no CT standards exist for measuring bronchial areas in children. Our study did not evaluate whether impaired pulmonary function as measured by PEFR would be predictive of respiratory collapse during general anesthesia because all were excluded and operated on under local anesthesia. General anesthesia was well tolerated in children with tracheal area and PEFR greater than 50% of predicted. Pulmonary function tests in children with anterior mediastinal masses may add valuable information to the anatomic evaluation obtained by CT scan.

Dependence of diaphragmatic length on lung volume and thoracoabdominal configuration.

Changes in lung volume can be partitioned into volume displacements of the rib cage and abdomen. Abdominal displacements are often used as estimates of diaphragmatic displacements and changes in lengthening of diaphragmatic muscle. We used X-rays, ultrasound, and linear measurements of thoracic and abdominal diameters to estimate relationships among lung volume, thoracoabdominal configuration and diaphragmatic length, and we found that diaphragmatic length was strongly dependent on rib cage as well as abdominal displacement. In three subjects, the diaphragm shortened 57-85% as much during a breath made without abdominal displacement as during a normal breath in which the abdominal wall moved outward with the rib cage. We conclude that changes in diaphragmatic length can be estimated from surface measurements without radiation and that the length of the diaphragm cannot be estimated from displacements of the abdominal wall alone.

Clue to diagnosis of neonatal urinary ascites. Relative radioluency of liver shadow.

About 25 per cent of neonatal ascites is caused by urinary tract disease. Opacification of the ascitic fluid by leakage of contrast material during cystography or intravenous urography may lead to striking relative radioluency of the liver. This phenomenon may be an important clue to the differential diagnosis of the etiology of neonatal ascites.

Diseases of the trachea, bronchi, and smaller airways.

Except in the fetus, life depends on the integrity of the airways. This article describes ways in which that integrity can be threatened in childhood.

Neonatal hydronephrosis: 146 cases.

Recognition and precise etiologic diagnosis of neonatal hydronephrosis is worthwhile since, although often severe, it is usually treatable with at least partial success. This relatively favorable prognosis, despite dilatation that may be marked, suggests a greater regenerative capacity at this age, or may be due to the relative infrequency or short duration of the infection.

Lower respiratory infections: how infants differ from adults.

Largely for anatomic reasons, the peripheral airways of infants are more susceptible to inflammatory narrowing than are those of adults. When infection occurs in the lower respiratory tract of an infant, the primary effect is likely to be on the smaller airways, not the alveoli. The results are airtrapping and atelectasis. This airway obstruction often causes severe respiratory embarrassment. It is recognized on chest films by generalized hyperinflation and irregularity of aeration. Small airway obstruction is a common and important manifestation of lower respiratory infection in infancy. True consolidative pneumonia is much less frequent.

Dimensions of the trachea to age 6 years related to height.

In an effort to establish normal values for both investigational and patient care purposes, computed tomography was used to determine the length, diameters, cross-sectional area, and volume of the tracheas of 34 children up to the age of 6 years. The measurements were taken when patients were asleep or resting quietly during tidal breathing, at perhaps 30-60% of total lung capacity. The results were related to body height (in infancy, to body length). There was virtually no difference between boys and girls. Each mean diameter correlated well with mean cross-sectional area. Tracheal diameters and area were reasonably constant over the length of individual tracheas. The slopes of the functions relating height to tracheal dimensions in these 34 infants and young children were slightly less steep than those previously measured near total lung capacity in 90 older children and adolescents.

Femoral anteversion.

Biplane roentgenography, axial roentgenography, and fluoroscopy are the usual roentgenographic methods of measuring femoral anteversion. These methods use a strict geometrical definition of anteversion. The computerized tomography method of measuring anteversion that was developed recently, and is now widely used, does not adhere to the accepted definition of anteversion and has not been tested for accuracy in a large series. In the present study, the widely used computerized-tomography method of measuring anteversion was tested on thirty-two femoral specimens. With that method, anteversion was consistently underestimated by an average of 10 degrees compared with direct measurements and was reproducible only to within +/- 3.6 degrees. Therefore, a new method of measuring anteversion using computerized tomography was developed. It was shown to be accurate to +/- 1 degree, as tested on the same specimens. This study demonstrated geometrically why the currently practiced computerized-tomography method of selecting the points that are used to define the axis of the femoral neck is not consistent with geometrical definitions of anteversion. A more accurate method for both defining the axis of the femoral neck and measuring femoral anteversion is described and recommended for clinical use.

CT quantitation of tracheal cross-sectional area as a guide to the surgical and anesthetic management of children with anterior mediastinal masses.

Pediatric patients presenting with anterior mediastinal masses between January 1980 and November 1988 were reviewed to assess the correlation between tracheal cross-sectional area and anesthetic risks. Forty-two patients had evaluable computed tomography (CT) scans and underwent a surgical procedure. Thirty-four patients had Hodgkin's disease, six had non-Hodgkin's lymphoma, and two had mediastinal teratoma. Tracheal cross-sectional areas were greater than 75% of expected in 19 cases, greater than 50% to 75% in 16 cases, greater than 25% to 50% in five cases, and less than or equal to 25% in two cases. The presence or extent of symptoms did not correlate well with the degree of tracheal narrowing shown by CT scan except for orthopnea. Local anesthesia was used primarily in patients with significant tracheal narrowing (tracheal size was less than or equal to 56% in 5 of 6 patients). General anesthesia with spontaneous ventilation by mask was performed in four patients with tracheal areas of 33%, 73%, 76%, and 98% of expected. General endotracheal anesthesia was utilized in the remaining 32 patients, only three of whom had tracheal areas of less than 50% of expected (down to 30%, 26%, and 24% of expected) and one received preoperative radiotherapy (26%). None of these 32 patients had symptoms of orthopnea or dyspnea at rest, and only one had dyspnea on exertion. All tolerated anesthesia without difficulty. No patient in this series suffered respiratory or cardiovascular collapse during surgery. Adequate biopsy material was obtained in all cases.(ABSTRACT TRUNCATED AT 250 WORDS)