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Adenomatoid malformation of the lung in the new born infant
INTERNATIONAL
NEWBORN. J. Camamta. uary),
John
ABSTRACTS OF PEDIATRIC SURGERY
M.
Amer.
and
Samuel
Surg. 35:27-29
Salyer
(Jan-
1969.
Pneumothorax or pneumomediastinum occurs in 0.05 to 2.9 per cent newborn infants. This incidence will include the pneumothorax which occurs both before and after prompt postdelivery resuscitative measures and the differentiation between these two types of pneumothorax is clinically noteworthy, but not of great significance, since intercostal tube waterseal drainage, with few exceptions, should be the method of approach, regardless of the estimated extent or localization of the pneumothorax or pneumomediastinum. This report includes 40 newborn children who were diagnosed and treated for pneumothorax and/or pneumomediastinum during an eight-year period between 1969 and 1967. Thirty-six of the 40 children were treated by intercostal tube drainage without suction and there were no fatalities during hospitalization in any infant so treated. Two deaths resulted from bilateral tension pneumothorax which was not treated by tube drainage. A technic for the insertion of the intercostal tube is described, utilizing a #12 or #I4 catheter inserted through the third or fourth interspace at the anterior axillary line.-A. M. Sal&erg. ADENOMATOIDMALFORMATIONOFTHELUNC IN THE NEW BORN INFANT. Fr. Moyson, R. Poncelet, G. Carpent, N. Cremer, and Ph. Pourhaix. Ann. Chir. Inf. 9:275-288. 1968. Congenital cystic adenomatoid malformation of the lung, also known as pulmonary adenomatosis of the newborn, is a very rare disease. The authors could collect only 26 cases in the literature. They had the opportunity of observing a twenty-seventh case. Clinically, cyanosis and dyspnea were already present 2 hours after birth, as well as displacement of the heart to the right and on the plain x-ray of the chest a polycystic appearance of the left upper lobe, combined with emphysema and micronodular formations. Lobectomy, performed on the twenty-fifth day of life, was followed by recovery and cure. Microscopic examination of the specimen showed the typical adenomatous structure with tufts of very clear mucous cells here and there. Differential
381
diagnosis (hyaline membrane syndrome, syndrome of Wilson-Mikity, congenital pulmonary cystic lymphangiectasis, hamartoma of the lung, sequestration, congenital cysts, staphylococcic pneumatocele, and arteriovenous aneurysm) is discussed. Surgery is the only successful treatment. All cases in the literature who were not operated upon died.-M. Better SOME LATE SEQUELS OF CHILDHOOD PNEUMONECTOMY. Z. Sety, 1. Ressl, and J. Vyhnalek. Surgery 65:343-351 (February), 1968. Sixteen patients who had undergone pneumonectomy at 18 years of age or less were studied. Fifteen of the patients were less than 14 years old at the time of surgery. The youngest was 6 months old at the time of surgery. The most common indication for pneumonectomy was extensive suppurative lung disease. There were 3 cases of tuberculosis. Follow-up was from 3 to 17 years after the operation. All of the patients were doing well clinically and able to go about their usual activities in a normal manner. Substantial deformities of the chest and spine were observed in every case, but these were not readily apparent unless the patients were unclothed. No correlation was established between the degree of deformity and the functional capacity of the lungs. X-rays revealed an increase in volume of the remaining lung in all cases with herniation of varying degrees toward the opposite side. There were no symptoms relative to the esophagus, but esophagrams showed changes in the position and shape of the esophagus in every case. Electrocardiography showed marked vnriation in the electrical axis with no relationship to the side of the pneumonectomy. Electrocardiographic evidence of significant right ventricular hypertrophy was found in only 2 cases. Lung function tests showed reduction in maximum breathing capacity to a mean value of 48 per cent of the normal for both lungs. In some cases these values were less than expected for one lung. This was thought due to impairment of the mechanics of ventilation from anatomic alterations in the thoracic cage following surgery, and possibly from increased airway resistance. Thirteen patients had measurements of
382
INTERNATIONAL
the pulmonary artery pressure. The pressure rose markedly in each case from a mean resting value of 15 mm. Hg to 27 mm. Hg. In 10 cases the pressure rise exceeded the limits of normal. This significant evidence of pulmonary hypertension was considered a sign of latent car pulmonale and was the most important finding of the study. The authors suggest that the prognosis of these patients, although they all were clinically fairly normal at the time of study, must be guarded because of the significant pulmonary hypertension-D. T. Cloud.
of 84 patients with cystic fibrosis of the pancreas. Significant parenchymal destruction could be demonstrated in only 3 cases. In 29 of the older infants and children there was widespread respiratory bronchiole and alveolar duct dilatation. This widespread dilatation may be responsible for physiological and radiographic evidence of emphysema. It is suggested that the lobular dilatation in these young patients may be an antecedent of destructive emphysema in the adult--W. K. Sieber. TUMORS OF THE MEDIASTINUM IN CHILDREN. M. Jaubert de Beaujeu, P. Mollmd, and A. Campo-Paysaa. Ann. Chir. Inf. 9:177-191, 1968.
SURGICAL
RESULTS OF RESECTION OF THE LUNG IN CHILDHOOD. G. Brandesky, I. Geley, and R. Schindl. Kinderchir. 6:303-318, 1968. Sixty-six cases in which lung resection has been done in children over the past 12 years are reviewed and the early and late results are reported. There were 3 fatal cases. Postoperative results were excellent in 37 and good in 19. Forty-four of the children were between I and 12 years of age and they were re-investigated on the average of 5?4 years after operation. Generally the follow-up investigations showed excellent results, but it was demonstrated that segmental resection effects lung function less severely than more radical procedures. Complications are less commonly seen than in the adult.-S. Hofmann and H. B. Eckstein. PULMONARY CHANGE.YAND COP. PULMONALE IN Mucov~sc~~os~s. P. Wentworth, 1. Gough and J. E. Wentworth. Thorax 23: 582 ( November ) , 1968. The lungs of 8 patients, 19 days to 23 years of age, with cystic fibrosis were studied by special large section technic. In the older patients there was diffuse advanced bronchiectasis and luxuriant peribronchial granulation tissue with dilatation of the pulmonary arterial system. Distal air spaces were minimally distended except in the oldest patient where destructive emphysema was noted. Cor Pulmonale was present in 3 of these patients-w. K. Sieber. CYSTIC FIBROSISOF THE PANCREAS: STRUCTURAL CHANGES IN PERIPHERALAIRWAYS. J. R. Esterly and Ella H. Oppenheimm. Thorax 23: 670 ( November ), 1968. Pulmonary emphysema and bronchiolar obstruction were found at necropsy in 42
ABSTRACTS OF PEDIATRIC SURGERY
The authors observed and operated upon infants and children with mediastinal tumors. These tumors can be classified in 5 groups: 1. Tumors of the thymus (1 sarcoma, 6 hyperplasias, 6 cysts). 2. Heteroplastic dysembryomas (1 malignant teratoma, 4 benign dermoid cysts, 2 benign teratomas ). 3. Homoplastic dysembryomas (12 bronchogenic cysts, 2 enterogenic cysts and 1 nonclassified cyst which could have been a pulmonary sequestration). 4. Neurogenic tumors (4 neurofibroma in Recklinghausen disease, 3 ganglio-neuroblastomas, 3 neuroblastomas). 5. Miscellaneous 1 undifferentiated tumors (1 venoma, sarcoma, 1 fibromyxosarcoma, and 1 mesothelioma). The importance of early surgical treatment is stressed.-M. Bettex. 61
RADIOLOGICALFEATURESOF DIAPHRAGMATIC HERNIA IN INFANCY AND CHILDHOOD. Z. J. Rubinstein. S. Afr. Med. J. 42:10521056 (October), 1968. Sixty-nine patients under 8 years of age in whom diaphragmatic hemiation was demonstrated radiologically were studied. Fiftythree had sliding hiatus hernias; of these, persistent vomiting was the presenting complaint in 45 and iron deficiency anemia was present in 16. Three patients had paraesophageal hernias and all 3 had severe anemia. Of these two groups contrast studies were diagnostic in all instances. The authors stress the importance of upper G.I. barium study in infants and children with unexplained iron deficiency anemia whether vomiting is present or not. Twelve patients had pleuroperitoneal her-
NEWBORN. J. Camamta. uary),
John
ABSTRACTS OF PEDIATRIC SURGERY
M.
Amer.
and
Samuel
Surg. 35:27-29
Salyer
(Jan-
1969.
Pneumothorax or pneumomediastinum occurs in 0.05 to 2.9 per cent newborn infants. This incidence will include the pneumothorax which occurs both before and after prompt postdelivery resuscitative measures and the differentiation between these two types of pneumothorax is clinically noteworthy, but not of great significance, since intercostal tube waterseal drainage, with few exceptions, should be the method of approach, regardless of the estimated extent or localization of the pneumothorax or pneumomediastinum. This report includes 40 newborn children who were diagnosed and treated for pneumothorax and/or pneumomediastinum during an eight-year period between 1969 and 1967. Thirty-six of the 40 children were treated by intercostal tube drainage without suction and there were no fatalities during hospitalization in any infant so treated. Two deaths resulted from bilateral tension pneumothorax which was not treated by tube drainage. A technic for the insertion of the intercostal tube is described, utilizing a #12 or #I4 catheter inserted through the third or fourth interspace at the anterior axillary line.-A. M. Sal&erg. ADENOMATOIDMALFORMATIONOFTHELUNC IN THE NEW BORN INFANT. Fr. Moyson, R. Poncelet, G. Carpent, N. Cremer, and Ph. Pourhaix. Ann. Chir. Inf. 9:275-288. 1968. Congenital cystic adenomatoid malformation of the lung, also known as pulmonary adenomatosis of the newborn, is a very rare disease. The authors could collect only 26 cases in the literature. They had the opportunity of observing a twenty-seventh case. Clinically, cyanosis and dyspnea were already present 2 hours after birth, as well as displacement of the heart to the right and on the plain x-ray of the chest a polycystic appearance of the left upper lobe, combined with emphysema and micronodular formations. Lobectomy, performed on the twenty-fifth day of life, was followed by recovery and cure. Microscopic examination of the specimen showed the typical adenomatous structure with tufts of very clear mucous cells here and there. Differential
381
diagnosis (hyaline membrane syndrome, syndrome of Wilson-Mikity, congenital pulmonary cystic lymphangiectasis, hamartoma of the lung, sequestration, congenital cysts, staphylococcic pneumatocele, and arteriovenous aneurysm) is discussed. Surgery is the only successful treatment. All cases in the literature who were not operated upon died.-M. Better SOME LATE SEQUELS OF CHILDHOOD PNEUMONECTOMY. Z. Sety, 1. Ressl, and J. Vyhnalek. Surgery 65:343-351 (February), 1968. Sixteen patients who had undergone pneumonectomy at 18 years of age or less were studied. Fifteen of the patients were less than 14 years old at the time of surgery. The youngest was 6 months old at the time of surgery. The most common indication for pneumonectomy was extensive suppurative lung disease. There were 3 cases of tuberculosis. Follow-up was from 3 to 17 years after the operation. All of the patients were doing well clinically and able to go about their usual activities in a normal manner. Substantial deformities of the chest and spine were observed in every case, but these were not readily apparent unless the patients were unclothed. No correlation was established between the degree of deformity and the functional capacity of the lungs. X-rays revealed an increase in volume of the remaining lung in all cases with herniation of varying degrees toward the opposite side. There were no symptoms relative to the esophagus, but esophagrams showed changes in the position and shape of the esophagus in every case. Electrocardiography showed marked vnriation in the electrical axis with no relationship to the side of the pneumonectomy. Electrocardiographic evidence of significant right ventricular hypertrophy was found in only 2 cases. Lung function tests showed reduction in maximum breathing capacity to a mean value of 48 per cent of the normal for both lungs. In some cases these values were less than expected for one lung. This was thought due to impairment of the mechanics of ventilation from anatomic alterations in the thoracic cage following surgery, and possibly from increased airway resistance. Thirteen patients had measurements of
382
INTERNATIONAL
the pulmonary artery pressure. The pressure rose markedly in each case from a mean resting value of 15 mm. Hg to 27 mm. Hg. In 10 cases the pressure rise exceeded the limits of normal. This significant evidence of pulmonary hypertension was considered a sign of latent car pulmonale and was the most important finding of the study. The authors suggest that the prognosis of these patients, although they all were clinically fairly normal at the time of study, must be guarded because of the significant pulmonary hypertension-D. T. Cloud.
of 84 patients with cystic fibrosis of the pancreas. Significant parenchymal destruction could be demonstrated in only 3 cases. In 29 of the older infants and children there was widespread respiratory bronchiole and alveolar duct dilatation. This widespread dilatation may be responsible for physiological and radiographic evidence of emphysema. It is suggested that the lobular dilatation in these young patients may be an antecedent of destructive emphysema in the adult--W. K. Sieber. TUMORS OF THE MEDIASTINUM IN CHILDREN. M. Jaubert de Beaujeu, P. Mollmd, and A. Campo-Paysaa. Ann. Chir. Inf. 9:177-191, 1968.
SURGICAL
RESULTS OF RESECTION OF THE LUNG IN CHILDHOOD. G. Brandesky, I. Geley, and R. Schindl. Kinderchir. 6:303-318, 1968. Sixty-six cases in which lung resection has been done in children over the past 12 years are reviewed and the early and late results are reported. There were 3 fatal cases. Postoperative results were excellent in 37 and good in 19. Forty-four of the children were between I and 12 years of age and they were re-investigated on the average of 5?4 years after operation. Generally the follow-up investigations showed excellent results, but it was demonstrated that segmental resection effects lung function less severely than more radical procedures. Complications are less commonly seen than in the adult.-S. Hofmann and H. B. Eckstein. PULMONARY CHANGE.YAND COP. PULMONALE IN Mucov~sc~~os~s. P. Wentworth, 1. Gough and J. E. Wentworth. Thorax 23: 582 ( November ) , 1968. The lungs of 8 patients, 19 days to 23 years of age, with cystic fibrosis were studied by special large section technic. In the older patients there was diffuse advanced bronchiectasis and luxuriant peribronchial granulation tissue with dilatation of the pulmonary arterial system. Distal air spaces were minimally distended except in the oldest patient where destructive emphysema was noted. Cor Pulmonale was present in 3 of these patients-w. K. Sieber. CYSTIC FIBROSISOF THE PANCREAS: STRUCTURAL CHANGES IN PERIPHERALAIRWAYS. J. R. Esterly and Ella H. Oppenheimm. Thorax 23: 670 ( November ), 1968. Pulmonary emphysema and bronchiolar obstruction were found at necropsy in 42
ABSTRACTS OF PEDIATRIC SURGERY
The authors observed and operated upon infants and children with mediastinal tumors. These tumors can be classified in 5 groups: 1. Tumors of the thymus (1 sarcoma, 6 hyperplasias, 6 cysts). 2. Heteroplastic dysembryomas (1 malignant teratoma, 4 benign dermoid cysts, 2 benign teratomas ). 3. Homoplastic dysembryomas (12 bronchogenic cysts, 2 enterogenic cysts and 1 nonclassified cyst which could have been a pulmonary sequestration). 4. Neurogenic tumors (4 neurofibroma in Recklinghausen disease, 3 ganglio-neuroblastomas, 3 neuroblastomas). 5. Miscellaneous 1 undifferentiated tumors (1 venoma, sarcoma, 1 fibromyxosarcoma, and 1 mesothelioma). The importance of early surgical treatment is stressed.-M. Bettex. 61
RADIOLOGICALFEATURESOF DIAPHRAGMATIC HERNIA IN INFANCY AND CHILDHOOD. Z. J. Rubinstein. S. Afr. Med. J. 42:10521056 (October), 1968. Sixty-nine patients under 8 years of age in whom diaphragmatic hemiation was demonstrated radiologically were studied. Fiftythree had sliding hiatus hernias; of these, persistent vomiting was the presenting complaint in 45 and iron deficiency anemia was present in 16. Three patients had paraesophageal hernias and all 3 had severe anemia. Of these two groups contrast studies were diagnostic in all instances. The authors stress the importance of upper G.I. barium study in infants and children with unexplained iron deficiency anemia whether vomiting is present or not. Twelve patients had pleuroperitoneal her-