-__-Fur .l Cardio-thorac
Surg (1990) 4: 403-406
Congenital cystic adenomatoid malformation of the lung M. Ribet ‘, E R. Pruvot ‘, J. P. Dubos 2, J. Remy 3, M. C. Sault 4, B. Gosselin4, and J. L. Linder ’ ’ Clinique Chirurgicale
Quest. CHU. Lille. Departments
of ‘Pediatry,
Abstract. Twenty cases of cystic adenomatoid malformation of the lung were observed: 2 had died in utero; the diagnosis was made at birth in 13 infants of which 3 were premature. A Bochdalek’s hernia had been diagnosed before birth in 2 cases by echo-tomography. The correct antenatal diagnosis had been made in 2 cases. Two infants had no symptoms, 3 were dyspneic, 8 were in respiratory distress and had to be intubated and ventilated. Two had a prune belly syndrome. Eight infants had a thoracotomy during their first week of life. Pulmonary resections concerning those 13 patients comprised 10 lower lobectomies, 1 of which was associated with a lingulectomy, and 2 upper lobectomies, 3 of which was associated with a middle lobe resection. Five patients were diagnosed and operated upon between 10 months and 8 years of age; 4 had recurrent bronchitis and 1 was diagnosed during the treatment of a gastroenteritis. They had 3 lower and 2 upper lobectomies. Recovery was uneventful in all patients except for 1 who was reoperated upon for intestinal obstruction. Antenatal diagnosis of cystic adenomatoid malformation should become standard. The malformation may be mistaken for a pulmonary sequestration or bronchogenic cyst. Differential diagnosis of a congenitial hernia is important. [Eur J Cardio-thorac Surg (1990) 4: 403 -4061 Key words: Lung Congenital malformation adenomatoid malformation .- Surgery
~ Cystic
Congenital cystic adenomatoid malformation of the lung was first described in still-born infants by Ch’in and Tang in 1949 [3]. Its cystic character was confirmed by Craig, Kirkpatrick and Neuhauser [4]. Kwitken and Reiner in 1962 gave the first pathological description [8]: proliferation of the terminal bronchioles, with cysts lined by cuboi’dal or columnar epithelium and containing muscle Read at the 3rd Annual Meeting of the European Association Cardio-thoracic Surgery, Munich. October 9- 1I. 1989
for
’ Radiology.
and ’ Pathology.
Hopital Calmcttc.
Lillc. France
and elastic fibres with no cartilage. In 1977. Stocker, Madewell and Drake studied 38 cases and considered three types: type I, several cysts larger than 2 cm in diameter and lined with columnar cells; type II. numerous cysts less than 1 cm in diameter and lined with cuboi’dal cells, having a pseudo-bronchiolar structure and often associated with other malformations; type III, solid mass of bronchiolar microcysts with cubo’idal epithelium [2]. In 1978, Ostor and Fortune, estimating that the distinction between types I and II was not always easy, stated that only type III was a true adenomatoid malformation and considered only two types [lo], but the former classiGcation prevailed: both were based on the size, number and aspect of the cysts (Table 1). In 1985, Esposito, Deluca, Cicliano, Ascione and Ditooro found 279 published cases [6]. The malformation is probably more common than was thought. It can cause death at birth. respiratory distress in the newborn or may be diagnosed at a later age. Nowadays, antenatal echo-tomography can establish the diagnosis.
Material and methods Between 1970 and 1989,20 cases of congenital adenomatoid malformations of the lung were observed: 2 were discovered at autopsy of still-born infants, were type III and will not be discussed further. Two patients were diagnosed during pregnancy (type I). 11 at birth and were of type L in 5 cases and of type II in 5, 1 case remaining undefined. Five patients were discovered at ages varying from 10 months to 8 years and were of type I in 3 and of type II in 2 cases. The 18 patients were treated and cured by excision of the lesion.
Tabele I. Stocker’s classification
Type 1 (75%) Type II (lo- 15%) Type III
Several cysts>2 cm muscular and elastic libres. columnar cells Numerous cysts< 1cm. cuboid cells. pseudo-bronchiolar structure, associated malformations Solid mass, bronchiolar microcysts. cuhoid epithehum, death in utero
404
20 Cases
Late diagnosis: 5 patients (1 male, 4 females)
2 still-born infants (Type III) (2 antenatal
diagnosis) \
/ Neonatal forms: 13 (6 M. -7 E) Acute respiratory distress: I: 4, II: 4 (3 premature) Pneumopathy: 3 I: 1, II: 1. ?: 1 Other presentation: 2 I: 2
Late diagnosis: (1 M.p4 F.)
5
8 I: 3, II: 2 Infection: 4 No symptom:
These cases showed 3 type I and 2 type II anomalies. The ages varied from 10 months to 8 years and the diagnosis had not been established previously. Four children suffered from “chronic bronchitis” and recurrent lung infections; 1 was asymptomatic. Their chest films showed a multi-cavitated opacity in 3, aerated cavities in 2, with some degree of compression and displacement of the lung and mediastinum. Three patients underwent right lower and 2 left upper lobectomy, respectively.
1
Fig. 1. Clinical aspects and types of the malformation: no patient with type III malformation survived. Types II and I were equally divided in neonatal forms and late diagnosis. There were 8 cases of acute respiratory distress in the neonatal forms
There were 6 males and 12 females. The clinical presentation of the population could be classified into 4 groups; 3 concerning newborn infants, and 1 concerning older babies and children (Fig. 1).
Acute respiratory distress in the newborn. 8 patients (3 males, 5 females) The types of malformation were I and II in 4 cases each; 1 type II patient had an associated prune belly syndrome. Three infants were premature (31, 34, and 35 weeks). One type I malformation was diagnosed before birth and was operated upon on the 2nd day of life. The 8 patients were ventilated at birth and 6 were operated upon between the 1st and 7th day. One was operated upon on the 22nd day and 1 in the 3rd month: these 2 infants had first been weaned from the ventilator and had again become ventilator-dependent. Four patients had a left lower lobectomy, 1 had a left lower lobectomy associated with lingulectomy, 2 had a right lower lobectomy and 1, operated upon at 3 months, had a right upper lobectomy associated with a middle lobectomy. One of the patients first had a laparotomy with an erroneous diagnosis of Bochdalek’s hernia and then, an immediate left lower lobectomy.
Pneumopathy in the newborn: 3 patients (2 males, 1 female) The types were I, II and undefined in the last case; type I was associated with a prune belly syndrome. One baby was dyspneic and was operated upon on the 9th day of life; 1 suffered from dyspnea and cyanosis while feeding and was operated upon on the 36th day; the last case had recurrent lung infections and was operated upon during the 14th week. They had 2 left lower lobectomies and 1 right lower lobectomy, respectively.
Other clinical aspects in the newborn: 2 patients (1 male, 1 female) Both were of type I. One baby was born asymptomatic with an abnormal aspect of the right hemithorax which was diagnosed as a minor form of Bochdalek’s hernia. On his 1st day of life, he underwent thoracotomy and right lower lobectomy. The second patient was also asymptomatic after antenatal diagnosis. He was observed for 18 weeks and then underwent right lower lobectomy.
Results
There was no mortality; all patients were weaned from the ventilator within the 1st postoperative week. The patient who had both a laparotomy and a thoracotomy on her 2nd day of life was reoperated upon a month later for intestinal obstruction. None of the children had further pulmonary symptoms. The 2 associated prune belly syndromes were minor, without any urological malformation, and therefore did not need repair. All patients grew up normally with no further respiratory symptoms and no thoracic abnormality.
Discussion
Cystic adenomatoid malformation is observed with equal frequency in boys and girls, does not appear to be hereditary and is one of the lung anomalies which may cause respiratory distress and the need for ventilation at birth or soon after. Air trapping occurs in the cystic areas which may contain mucus and which frequently communicate with the bronchial tree, as the cysts are not supported by cartilage. The lesions are confined to one lobe and seem to be predominant in the lower lobes; they may or may not occupy the whole lobe. We observed that their limits are not well defined and in particular are not marked by a segmental plane. Sublobar resection is not to be recommended [9]. The lobe may increase in volume and weight after birth [2, 111, which may be an explanation for the late diagnosis. It previously had been thought that a localized cystic adenomatoid malformation may even appear only after birth. The diagnosis of cystic adenomatoid malformation can be made before birth as in 2 of our late cases. Death in utero is very frequent in type III and is observed in 10% of all cases. Radiologically, the lesion may be opaque at birth due to alveolar fluid, and then clear with the appearance of fluid levels. Pneumothorax and atelectasis may also occur. At birth, placing a tube into the oesophagus and stomach, opacification of these organs and an attentive examination of the chest and abdominal films should avoid the confusion of the anomaly with Bochdalek’s hernia, which indeed occurred once in our series, as the abdominal air spaces are normal in size, shape and number in this pulmonary malformation. This is especially true for the left side; on the right, the diagnosis may be more difficult. The confusion with Bochdalek’s hernia is probably less important, as thoracotomy provides a
405
This synthetic view of pulmonary malformations explains the increasing number of published cases and the variety of their clinical expressions, ranging from death in utero to respiratory distress at birth as well as the absence or the presence of pulmonary symptoms. Antenatal diagnosis allows preparation for immediate surgery if necessary.
Fig. 2. Topography of the malformation. Resection of middle lobe and lingula. which where atelectatic could have been avoided
approach for both lobectomy and diaphragmatic repair on that side. Giant lobar emphysema is easy to distinguish. In case of a large cavity, a bulla may be considered: 2 of the newborn patients had a decompressive transthoracic tap before they came to surgery and had to be drained for a persisting air leak before thoracotomy was performed [I 31. Respiratory distress is considered to occur in 30% of the cases. Subsequently, pulmonary symptoms, pain, infection, or retarded growth call for the differential diagnosis of post-infectious pneumatocele, pulmonary sequestration, cystic hygroma or a thymic cyst. Sometimes the malformation remains symptomless (3 patients in our series). The diseased lobe appears as both heavy and cystic. with a differing balance of solid and aerated areas according to the type of the anomaly. The hilum may contain inflammatory lymph nodes. We have never found any anomaly of bronchial or vascular distribution, nor have we observed an abnormal systemic vessel. In 2 cases undergoing lobectomy plus lingulectomy and a bi-lobectomy, respectively, there was no congenital anomaly in the adjacent segment or lobe which were compressed and atelectatic and probably should have been preserved (Fig. 2). The malformation is considered to be due to a mismatch of bronchiolar and alveolar development. Type III probably occurs before the 26th day of embryonic life, while types II and I develop later. There are no fundamental embryological division lines between these malformations, pulmonary sequestration and bronchogenic cysts, the differences stemming from the time of their appearance during embryonic development. Associated anomalies have been described [l , 51. The term “bronchiolar congenital malformation” had been preferred for that reason, while the term “cystic adenomatoid malformation” is more global, including the different components of the pathological appearance. good
References 1. Bale PM (1979) Congenital
2.
3.
4.
5.
6.
7.
8. 9.
10. 11. 12.
13.
cystic malformation of the lung. A form of congenital bronchiolar (“adenomatoid”) malformation. Am J Clin Pathol 71:41 l-430 Buntain WL. lsaacs H. Payne VC. Lindesmith GG. Rosenkrantz JG (1974) Lobar emphysema. cystic adenomatoid malformation. pulmonary sequestration and bronchogenic cyst in infancy and childhood: a clinical group. J Pediatr Surg 9: 8593 Ch’in KY. Tang MY (1949) Congenital adenomatoid malformation of one lobe of a lung with general anasarca. Arch Pathol 48: 221-229 Craig JM. Kirkpatrick J, Neuhauser EBD (1956) Congenital cystic adenomatoid malformation of the lung in infants. AJR 76:516-526 Demos NJ, Teresi A (1975) Congenital lung malformations. A unified concept and a case report. J Thorac Cardiovasc Surg 70:260-264 Esposito G. Deluca U, Cicliano B. Ascione G. Ditooro A (1985) La malformation adinomateuse kystique congknitale du poumon. Chir Pediatr 26: 321-327 Gilly J. Bouvier R, Bcrger R (1981) Malformation adknomato’ide pulmonaire. A propos de 13 observations. Arch Anat Cytol Path01 29: 16-22 Kwittken J, Reiner L (1962) Congenital cystic adenomatoid malformation of the lung. Pediatrics 30: 759-768 Olson JL. Mendelsohn G (1978) Congenital cystic adenomatoid malformation of the lung. Arch Pathol Lab Med 102: 24X-251 Ostor AG. Fortune DW (1978) Congenital cystic adenomatoid malformation of the lung. Am J Clin Pathol 70: 595-604 Shamji FM. Sachs HJ. Perkins DG (1988) Cystic disease of the lung. Surg Clin North Am 68: 581-620 Stocker JT. Madewell JE, Drake RM (1977) Congenital cystic adenomatoid malformation of the lung. Classification and morphological spectrum. Hum Pathol 8: 156- 171 Wesley JR, Heidelberger KP, Dipietro MA, Cho KJ, Coran AG (lY86) Diagnosis and management of congenital cystic disease of the lung in children. J Pediatr Surg 21: 202 207
Dr. M. Ribet S&vice de Chirurgie Hbpital Calmette Boulevard du Prof. Leclercq F-59037 Lille France
406
Discussion UK). I would like to comment that in our practice, this malformation is sometimes diagnosed by referring physicians as obstructive lobar emphysema. I would like to ask whether the authors have had a similar experience. We sometimes admit babies with cystic malformation or with obstructive lobar emphysema in whom referring physicians placed a chest drain. In some of them, tension pneumothorax was diag-
nosed on admission and the patients were in a very poor general condition. I therefore feel that we still have to do a lot of education in this area.
Mr. J. F. Stark (London,
Dr. Ribet: That’s right, it may be associated with lobar emphysema. It may cause pneumothorax. I had no time to say it, but two of our patients came to the hospital with tubes in their chests and a leak; the leak continued until we got the lobe out. Whatever the lung malformation, a certain percentage of babies come with a tube in their chest.
Surg (1990) 4: 403-406
Congenital cystic adenomatoid malformation of the lung M. Ribet ‘, E R. Pruvot ‘, J. P. Dubos 2, J. Remy 3, M. C. Sault 4, B. Gosselin4, and J. L. Linder ’ ’ Clinique Chirurgicale
Quest. CHU. Lille. Departments
of ‘Pediatry,
Abstract. Twenty cases of cystic adenomatoid malformation of the lung were observed: 2 had died in utero; the diagnosis was made at birth in 13 infants of which 3 were premature. A Bochdalek’s hernia had been diagnosed before birth in 2 cases by echo-tomography. The correct antenatal diagnosis had been made in 2 cases. Two infants had no symptoms, 3 were dyspneic, 8 were in respiratory distress and had to be intubated and ventilated. Two had a prune belly syndrome. Eight infants had a thoracotomy during their first week of life. Pulmonary resections concerning those 13 patients comprised 10 lower lobectomies, 1 of which was associated with a lingulectomy, and 2 upper lobectomies, 3 of which was associated with a middle lobe resection. Five patients were diagnosed and operated upon between 10 months and 8 years of age; 4 had recurrent bronchitis and 1 was diagnosed during the treatment of a gastroenteritis. They had 3 lower and 2 upper lobectomies. Recovery was uneventful in all patients except for 1 who was reoperated upon for intestinal obstruction. Antenatal diagnosis of cystic adenomatoid malformation should become standard. The malformation may be mistaken for a pulmonary sequestration or bronchogenic cyst. Differential diagnosis of a congenitial hernia is important. [Eur J Cardio-thorac Surg (1990) 4: 403 -4061 Key words: Lung Congenital malformation adenomatoid malformation .- Surgery
~ Cystic
Congenital cystic adenomatoid malformation of the lung was first described in still-born infants by Ch’in and Tang in 1949 [3]. Its cystic character was confirmed by Craig, Kirkpatrick and Neuhauser [4]. Kwitken and Reiner in 1962 gave the first pathological description [8]: proliferation of the terminal bronchioles, with cysts lined by cuboi’dal or columnar epithelium and containing muscle Read at the 3rd Annual Meeting of the European Association Cardio-thoracic Surgery, Munich. October 9- 1I. 1989
for
’ Radiology.
and ’ Pathology.
Hopital Calmcttc.
Lillc. France
and elastic fibres with no cartilage. In 1977. Stocker, Madewell and Drake studied 38 cases and considered three types: type I, several cysts larger than 2 cm in diameter and lined with columnar cells; type II. numerous cysts less than 1 cm in diameter and lined with cuboi’dal cells, having a pseudo-bronchiolar structure and often associated with other malformations; type III, solid mass of bronchiolar microcysts with cubo’idal epithelium [2]. In 1978, Ostor and Fortune, estimating that the distinction between types I and II was not always easy, stated that only type III was a true adenomatoid malformation and considered only two types [lo], but the former classiGcation prevailed: both were based on the size, number and aspect of the cysts (Table 1). In 1985, Esposito, Deluca, Cicliano, Ascione and Ditooro found 279 published cases [6]. The malformation is probably more common than was thought. It can cause death at birth. respiratory distress in the newborn or may be diagnosed at a later age. Nowadays, antenatal echo-tomography can establish the diagnosis.
Material and methods Between 1970 and 1989,20 cases of congenital adenomatoid malformations of the lung were observed: 2 were discovered at autopsy of still-born infants, were type III and will not be discussed further. Two patients were diagnosed during pregnancy (type I). 11 at birth and were of type L in 5 cases and of type II in 5, 1 case remaining undefined. Five patients were discovered at ages varying from 10 months to 8 years and were of type I in 3 and of type II in 2 cases. The 18 patients were treated and cured by excision of the lesion.
Tabele I. Stocker’s classification
Type 1 (75%) Type II (lo- 15%) Type III
Several cysts>2 cm muscular and elastic libres. columnar cells Numerous cysts< 1cm. cuboid cells. pseudo-bronchiolar structure, associated malformations Solid mass, bronchiolar microcysts. cuhoid epithehum, death in utero
404
20 Cases
Late diagnosis: 5 patients (1 male, 4 females)
2 still-born infants (Type III) (2 antenatal
diagnosis) \
/ Neonatal forms: 13 (6 M. -7 E) Acute respiratory distress: I: 4, II: 4 (3 premature) Pneumopathy: 3 I: 1, II: 1. ?: 1 Other presentation: 2 I: 2
Late diagnosis: (1 M.p4 F.)
5
8 I: 3, II: 2 Infection: 4 No symptom:
These cases showed 3 type I and 2 type II anomalies. The ages varied from 10 months to 8 years and the diagnosis had not been established previously. Four children suffered from “chronic bronchitis” and recurrent lung infections; 1 was asymptomatic. Their chest films showed a multi-cavitated opacity in 3, aerated cavities in 2, with some degree of compression and displacement of the lung and mediastinum. Three patients underwent right lower and 2 left upper lobectomy, respectively.
1
Fig. 1. Clinical aspects and types of the malformation: no patient with type III malformation survived. Types II and I were equally divided in neonatal forms and late diagnosis. There were 8 cases of acute respiratory distress in the neonatal forms
There were 6 males and 12 females. The clinical presentation of the population could be classified into 4 groups; 3 concerning newborn infants, and 1 concerning older babies and children (Fig. 1).
Acute respiratory distress in the newborn. 8 patients (3 males, 5 females) The types of malformation were I and II in 4 cases each; 1 type II patient had an associated prune belly syndrome. Three infants were premature (31, 34, and 35 weeks). One type I malformation was diagnosed before birth and was operated upon on the 2nd day of life. The 8 patients were ventilated at birth and 6 were operated upon between the 1st and 7th day. One was operated upon on the 22nd day and 1 in the 3rd month: these 2 infants had first been weaned from the ventilator and had again become ventilator-dependent. Four patients had a left lower lobectomy, 1 had a left lower lobectomy associated with lingulectomy, 2 had a right lower lobectomy and 1, operated upon at 3 months, had a right upper lobectomy associated with a middle lobectomy. One of the patients first had a laparotomy with an erroneous diagnosis of Bochdalek’s hernia and then, an immediate left lower lobectomy.
Pneumopathy in the newborn: 3 patients (2 males, 1 female) The types were I, II and undefined in the last case; type I was associated with a prune belly syndrome. One baby was dyspneic and was operated upon on the 9th day of life; 1 suffered from dyspnea and cyanosis while feeding and was operated upon on the 36th day; the last case had recurrent lung infections and was operated upon during the 14th week. They had 2 left lower lobectomies and 1 right lower lobectomy, respectively.
Other clinical aspects in the newborn: 2 patients (1 male, 1 female) Both were of type I. One baby was born asymptomatic with an abnormal aspect of the right hemithorax which was diagnosed as a minor form of Bochdalek’s hernia. On his 1st day of life, he underwent thoracotomy and right lower lobectomy. The second patient was also asymptomatic after antenatal diagnosis. He was observed for 18 weeks and then underwent right lower lobectomy.
Results
There was no mortality; all patients were weaned from the ventilator within the 1st postoperative week. The patient who had both a laparotomy and a thoracotomy on her 2nd day of life was reoperated upon a month later for intestinal obstruction. None of the children had further pulmonary symptoms. The 2 associated prune belly syndromes were minor, without any urological malformation, and therefore did not need repair. All patients grew up normally with no further respiratory symptoms and no thoracic abnormality.
Discussion
Cystic adenomatoid malformation is observed with equal frequency in boys and girls, does not appear to be hereditary and is one of the lung anomalies which may cause respiratory distress and the need for ventilation at birth or soon after. Air trapping occurs in the cystic areas which may contain mucus and which frequently communicate with the bronchial tree, as the cysts are not supported by cartilage. The lesions are confined to one lobe and seem to be predominant in the lower lobes; they may or may not occupy the whole lobe. We observed that their limits are not well defined and in particular are not marked by a segmental plane. Sublobar resection is not to be recommended [9]. The lobe may increase in volume and weight after birth [2, 111, which may be an explanation for the late diagnosis. It previously had been thought that a localized cystic adenomatoid malformation may even appear only after birth. The diagnosis of cystic adenomatoid malformation can be made before birth as in 2 of our late cases. Death in utero is very frequent in type III and is observed in 10% of all cases. Radiologically, the lesion may be opaque at birth due to alveolar fluid, and then clear with the appearance of fluid levels. Pneumothorax and atelectasis may also occur. At birth, placing a tube into the oesophagus and stomach, opacification of these organs and an attentive examination of the chest and abdominal films should avoid the confusion of the anomaly with Bochdalek’s hernia, which indeed occurred once in our series, as the abdominal air spaces are normal in size, shape and number in this pulmonary malformation. This is especially true for the left side; on the right, the diagnosis may be more difficult. The confusion with Bochdalek’s hernia is probably less important, as thoracotomy provides a
405
This synthetic view of pulmonary malformations explains the increasing number of published cases and the variety of their clinical expressions, ranging from death in utero to respiratory distress at birth as well as the absence or the presence of pulmonary symptoms. Antenatal diagnosis allows preparation for immediate surgery if necessary.
Fig. 2. Topography of the malformation. Resection of middle lobe and lingula. which where atelectatic could have been avoided
approach for both lobectomy and diaphragmatic repair on that side. Giant lobar emphysema is easy to distinguish. In case of a large cavity, a bulla may be considered: 2 of the newborn patients had a decompressive transthoracic tap before they came to surgery and had to be drained for a persisting air leak before thoracotomy was performed [I 31. Respiratory distress is considered to occur in 30% of the cases. Subsequently, pulmonary symptoms, pain, infection, or retarded growth call for the differential diagnosis of post-infectious pneumatocele, pulmonary sequestration, cystic hygroma or a thymic cyst. Sometimes the malformation remains symptomless (3 patients in our series). The diseased lobe appears as both heavy and cystic. with a differing balance of solid and aerated areas according to the type of the anomaly. The hilum may contain inflammatory lymph nodes. We have never found any anomaly of bronchial or vascular distribution, nor have we observed an abnormal systemic vessel. In 2 cases undergoing lobectomy plus lingulectomy and a bi-lobectomy, respectively, there was no congenital anomaly in the adjacent segment or lobe which were compressed and atelectatic and probably should have been preserved (Fig. 2). The malformation is considered to be due to a mismatch of bronchiolar and alveolar development. Type III probably occurs before the 26th day of embryonic life, while types II and I develop later. There are no fundamental embryological division lines between these malformations, pulmonary sequestration and bronchogenic cysts, the differences stemming from the time of their appearance during embryonic development. Associated anomalies have been described [l , 51. The term “bronchiolar congenital malformation” had been preferred for that reason, while the term “cystic adenomatoid malformation” is more global, including the different components of the pathological appearance. good
References 1. Bale PM (1979) Congenital
2.
3.
4.
5.
6.
7.
8. 9.
10. 11. 12.
13.
cystic malformation of the lung. A form of congenital bronchiolar (“adenomatoid”) malformation. Am J Clin Pathol 71:41 l-430 Buntain WL. lsaacs H. Payne VC. Lindesmith GG. Rosenkrantz JG (1974) Lobar emphysema. cystic adenomatoid malformation. pulmonary sequestration and bronchogenic cyst in infancy and childhood: a clinical group. J Pediatr Surg 9: 8593 Ch’in KY. Tang MY (1949) Congenital adenomatoid malformation of one lobe of a lung with general anasarca. Arch Pathol 48: 221-229 Craig JM. Kirkpatrick J, Neuhauser EBD (1956) Congenital cystic adenomatoid malformation of the lung in infants. AJR 76:516-526 Demos NJ, Teresi A (1975) Congenital lung malformations. A unified concept and a case report. J Thorac Cardiovasc Surg 70:260-264 Esposito G. Deluca U, Cicliano B. Ascione G. Ditooro A (1985) La malformation adinomateuse kystique congknitale du poumon. Chir Pediatr 26: 321-327 Gilly J. Bouvier R, Bcrger R (1981) Malformation adknomato’ide pulmonaire. A propos de 13 observations. Arch Anat Cytol Path01 29: 16-22 Kwittken J, Reiner L (1962) Congenital cystic adenomatoid malformation of the lung. Pediatrics 30: 759-768 Olson JL. Mendelsohn G (1978) Congenital cystic adenomatoid malformation of the lung. Arch Pathol Lab Med 102: 24X-251 Ostor AG. Fortune DW (1978) Congenital cystic adenomatoid malformation of the lung. Am J Clin Pathol 70: 595-604 Shamji FM. Sachs HJ. Perkins DG (1988) Cystic disease of the lung. Surg Clin North Am 68: 581-620 Stocker JT. Madewell JE, Drake RM (1977) Congenital cystic adenomatoid malformation of the lung. Classification and morphological spectrum. Hum Pathol 8: 156- 171 Wesley JR, Heidelberger KP, Dipietro MA, Cho KJ, Coran AG (lY86) Diagnosis and management of congenital cystic disease of the lung in children. J Pediatr Surg 21: 202 207
Dr. M. Ribet S&vice de Chirurgie Hbpital Calmette Boulevard du Prof. Leclercq F-59037 Lille France
406
Discussion UK). I would like to comment that in our practice, this malformation is sometimes diagnosed by referring physicians as obstructive lobar emphysema. I would like to ask whether the authors have had a similar experience. We sometimes admit babies with cystic malformation or with obstructive lobar emphysema in whom referring physicians placed a chest drain. In some of them, tension pneumothorax was diag-
nosed on admission and the patients were in a very poor general condition. I therefore feel that we still have to do a lot of education in this area.
Mr. J. F. Stark (London,
Dr. Ribet: That’s right, it may be associated with lobar emphysema. It may cause pneumothorax. I had no time to say it, but two of our patients came to the hospital with tubes in their chests and a leak; the leak continued until we got the lobe out. Whatever the lung malformation, a certain percentage of babies come with a tube in their chest.
