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Congenital cystic adenomatoid malformation of the lung: Prenatal management and prognosis
Congenital By Y. Revillon,
Cystic Adenomatoid Malformation of the Lung: Prenatal Management and Prognosis
D. Jan, V. Plattner,
P. Sonigo, M. Dommergues,
L. Mandelbrot, Y. Dumez, and C. Nihoul-F&c%&
Paris, France l Thirty-two cases of congenital cystic adenomatoid malformation of the lung diagnosed antenatally are reported. Antenatal diagnosis has made it possible to document the progress of the condition in utero and the postnatal prognosis, It has been possible to advise on termination and intrauterine intervention, to counsel the parents, and to plan arrangements for delivery and postnatal care among obstetricians, neonatologists, and pediatric surgeons. According to Stocker’s classification there were 12 cases of type 1. 15 of type II, and 5 of type Ill. Five pregnancies were terminated. Antenatal drainage of a cyst was performed in four patients with two survivors. Thirteen babies showed relative regression of the cyst as pregnancy progressed. After delivery the extent of the cystic malformation was assessed by chest x-rays and computed tomography scanning in 25 and angiography in 6. Treatment consisted of observation in 4, emboliration in 2, operation as an emergency in 3, and electively around 4 months in 15. Copyright o 1993 by W.6. Saunders Company INDEX WORDS: Congenital cystic adenomatoid tion, lung, antenatal diagnosis.
malforma-
P
RENATAL diagnosis has become an invaluable aid for the detection and recording of the progress of congenital malformations. This information enables obstetricians, neonatologists, and paediatric surgeons to plan management and counsel parents. We have studied the in utero evolution and postnatal progress of 32 cases of congenital cystic adenomatoid malformation (CCAM). Our aim was to establish the prognosis and best methods of treatment before and after birth. MATERIAL AND METHODS Between 1987 and 1992. 32 patients with fetal lung malformations were diagnosed antenatally as having CCAM. The gestational age at diagnosis ranged from 16 to 38 weeks with most patients presenting between 23 and 28 weeks. Lesions were classified according to the criteria proposed by Stocker’: type I macrocystic, multiple large cysts: fype 2 polymicrocystic, variable numbers of small cysts; type III homogenous echogenic mass. Sixteen cases were right sided, 15 left sided, and 1 was bilateral. The extent of pulmonary involvement was recorded as less than 50% of the thorax when the lesion was unilateral and ipsilateral lung could be identified: 50% where no normal lung was seen on the affected side: more than 50% when a mediastinal shift was observed; and 100% when no normal pulmonary parenchyma was seen. The potential prognostic indicators examined were: presence or absence of hydrops fetalis, polyhydramnios, and mediastinal shift. Fetal karyotyping was performed on all cases. In 4 cases, in utero drainage was performed because of developing hydrops fetalis and in the hope of avoiding pulmonary hypoplasia. This was performed by passing a double pigtail catheter into JournalofPediarric Surgery, Vol28, No 8 (August), 1993: pp
1009-1011
the amniotic cavity. The fetus was paralysed with vecuronium bromide, and a metal cannula was inserted through the amniotic cavity and through the fetal chest wall, draining the largest cysts. Cesarean section was not routinely performed. Postnatally, the assessment included routine chest x-rays, computed axial tomography, and in 6 cases angiography. The routine plan for intervention was to operate within the first four months. The diagnosis was confirmed histologically in all cases on whom resections were performed or following autopsy. RESULTS
Twelve fetuses had CCAM type I, 15 had type II, and 5 had type III. Severe hydrops was present in 6. Polyhydramnios was present in 16: in 4 cases this was severe. The prenatal assessment of the extent of the CCAM mass by ultrasound was less than 50% in 17, 50% in 6, more than 50% in 8, and 100% in 1. The karyotype was normal in all except one. No cardiac abnormalities were identified. The cerebral cortex was diffusely hypoechogenic in 2. Outcome and Management
Five pregnancies were terminated electively in accordance with French law (Table 1). In three cases the indication was severe hydrops, in one an abnormal karyotype 47,XXY and one was terminated at another institution against our advice. Of the 27 remaining pregnancies, intrauterine intervention was performed for hydrops or polyhydramnios in four instances. Two cases died: one aborted and one died at birth. In one case, 3 cysts one containing 50 mls of fluid were drained with a single catheter at 27 weeks. The lung expanded rapidly and the polyhydramnios resolved within 3 days. Nine weeks later the shunt became displaced resulting in immediate recurrence of polyhydramnios. The baby was delivered and operated on early. One further case (in which the hydrops decreased after drainage) awaits delivery. In 13 cases (3 type I, 8 type II, 2 type III) the size of the mass decreased spontaneously in utero. After
From the Service de Chirwgie Pediatrique and the Service de Radiologie Pediatrique, Hopital des Enfants Maludes. and the Clinique Universitaire de Port Royal-Hopital Cochin, Paris, France. Presented at the 39th Annual International Congress of the British Association of Paediattic Surgeons, Leeds, England. July 15-18, 1992. Address reprint requests to Y Revillon, Senice de Chirurgie Pediatrique, H6pital des Enfants Malades, Paris. France. Copyright 8 I993 by W.B. Saunders Company 0022.346819312808-0009$03.0010 1009
1010
REVILLON ET AL
Table 1. Outcome No. of Patients Termination of pregnancy
5
Death at birth
1
On-going
1
Operated
18
Embolization
2
Spontaneous regression
4
Waiting
1
birth the remaining 25 babies were assessed by chest x-ray and CT scan, and angiography in 6 cases. In four cases, the malformation was small and no operation has been performed. After more than 1 year of follow-up, they are progressing satisfactorily. In two cases the assessment showed a small lesion with a vascular pedicle. Embolization interrupted the vascular flow. Postoperatively the lung x-ray returned to normal. The follow-up on these is only 4 months. Eighteen babies have had operations. Three babies had immediate operation because of respiratory distress. In 15 cases the operation was performed near the fourth month of life. The operation involved lobectomy in 12 and segmentectomy in 6. The postoperative course was uneventful in 16. In 2 cases after emergency operation, cysts remained. One required reoperation 6 weeks later. The other awaits operation. One pregnancy is ongoing. The histological studies in all these cases confirmed the diagnosis of cystic adenomatoid malformation of the lung. In 5 cases it was associated with pulmonary sequestration. DISCUSSION
The CCAMs are rare2 but they represent 25% of the congenital lung malformations. Although the cause is unknown, a cessation of bronchiolar maturation with mesenchymal overgrowth is known to occur in the late fifth to early sixth week of gestation. This malformation is generally not associated with cardiac abnormality. In our series only one patient had an associated chromosomal abnormality, 47,XXY. Prenatal diagnosis is possible after the 16th week.3-6 The differential diagnoses on prenatal ultrasound include diaphragmatic hernia and rare thoracic masses such as bronchogenic or enteric cysts, mediastinal cystic teratoma, and pulmonary sequestration. There is an association between CCAM and pulmonary sequestration. Polyhydramnios is common presum-
ably due to fetal swallowing being decreased by esophageal compression. Polyhydramnios does not appear to be a prognostic indicator. Fetal hydrops secondary to the mediastinal shift caused by the malformation resulting in vena caval obstruction and cardiac compression appears to be the most important prognostic indicator. A prenatal diagnosis of type 1 generally indicates a good prognosis and type III, a poor prognosis.7-9 Two cases of type I were severe. One pregnancy was terminated and one baby died at birth. Three type I cases developed respiratory distress after birth. Previous reports have stated that type II is commonly associated with other malformations. In our series there were none. In 13 patients we observed a spontaneous reduction of the size of the cysts during the last quarter of the pregnancy.‘O-l2 This suggests that the focal anomaly does not tend to grow while the surrounding normal lung tissue continues to develop. Therefore the advantages and the hazards of invasive management should be carefully balanced with regard to gestational age and the risk of premature labor. In our successful case, drainage at 28 weeks appeared to be useful since the severe polyhydramnios resolved but recurred at 35 weeks after the shunt became displaced. Harrison et all3 have recently operated on such babies through a hysterotomy. Four of the babies presented with respiratory distress. Three were cured by removal of the affected segment of lung. One premature infant (31 weeks gestation) died. Detailed assessment using CT scanning was possible for the others. The pulmonary resection can and should be limited to the area of the malformation. In CCAM termination of pregnancy is indicated for children with hydrops, severe associated malformations, and chromosomal abnormalities. If the malformation is extensive intervention should be delayed as spontaneous regression is frequent. In utero drainage is recommended only in cases of hydrops. We have no experience of open prenatal surgery. The paediatric surgeon should be involved in the prenatal counselling of patients with CCAM. Usually it will be possible to give a good prognosis and relieve anxiety. The lesions can regress during the last quarter of pregnancy. Respiratory distress at birth is uncommon. Pulmonary resection can easily be limited to affected lung and after operation the prognosis is good.
REFERENCES 1. Stocker JT, Madewell JE, Drake RM: Congenital adenomatoid malformation of the lung: Classification and logic spectrum. Hum Pathol8:155-171, 1977
cystic
morfo-
2. Rosado adenomatoid 3. Adzick
de Christenson ML, Stocker JT: Congenital cystic malformation. Radiographics 11:865-886, 1991 NS, Harrison MR, Glick PL, et al: Fetal cystic
CCAM: PRENATAL
MANAGEMENT
AND PROGNOSIS
adenomatoid malformation: Prenatal diagnosis and natural history. J Pediatr Surg 20:483-488, 1985 4. Claiborne AK, Martin CM, McAlister WH, et al: Antenatal diagnosis of cystic adenomatoid malformation: Effect on patient management. Pediatr Radio1 15:337-339, 1985 5. Deacon CS, Smart PJ, Rimmer S: The antenatal diagnosis of congenital cystic adenomatoid malformation of the lung. Br J Radio1 63:968-970, 1990 6. Morcos SF, Lobb MO: The antenatal diagnosis by ultrasonography of type III congenital cystic adenomatoid malformation of the lung. Br J Obstet Gynecol93:1002-1005. 1986 7. Morris E, Constantine G, McHugo J: Cystic adenomatoid malformation of the lung: An obstetric and ultrasound perspective. Eur J Obstet Gynecol Reprod Biol40:11-15.1991 8. Nielson IR. Russo P, Laberge JM, et al: Congenital adenomatoid malformation of the lung: Current management and prognosis. J Pediatr Surg 26:975-981. 1991
1011
9. Ribet M, Pruvot FR, Dubos JP, et al: Congenital cystic adenomatoid malformation of the lung. Eur J Cardiothorac Surg 4:403-406, 1990 10. Glaves J, Baker JL: Spontaneous resolution of maternal hydramnios in coigenital adenomatoid malformation of the lung. Antenatal ultrasound features. Br J Obstet Gynecol90:1065-1068. 1983 11. Fine C, Adzick NS, Doubilet PM: Decreasing size of a congenital cystic adenomatoid malformation in utero. J Ultrasound Med 7:405-408, 1988 12. Sonek JD, Foley MR. Iams JD: Spontaneous regression of a large intrathoracic fetal lesion before birth. Am J Perinatol8:41-43. 1991 13. Harrison MR, Adzick NS, Jennings RW. et al: Antenatal intervention for congenital cystic adenomatoid malformation. Lancet 336:965-967. 3990
Cystic Adenomatoid Malformation of the Lung: Prenatal Management and Prognosis
D. Jan, V. Plattner,
P. Sonigo, M. Dommergues,
L. Mandelbrot, Y. Dumez, and C. Nihoul-F&c%&
Paris, France l Thirty-two cases of congenital cystic adenomatoid malformation of the lung diagnosed antenatally are reported. Antenatal diagnosis has made it possible to document the progress of the condition in utero and the postnatal prognosis, It has been possible to advise on termination and intrauterine intervention, to counsel the parents, and to plan arrangements for delivery and postnatal care among obstetricians, neonatologists, and pediatric surgeons. According to Stocker’s classification there were 12 cases of type 1. 15 of type II, and 5 of type Ill. Five pregnancies were terminated. Antenatal drainage of a cyst was performed in four patients with two survivors. Thirteen babies showed relative regression of the cyst as pregnancy progressed. After delivery the extent of the cystic malformation was assessed by chest x-rays and computed tomography scanning in 25 and angiography in 6. Treatment consisted of observation in 4, emboliration in 2, operation as an emergency in 3, and electively around 4 months in 15. Copyright o 1993 by W.6. Saunders Company INDEX WORDS: Congenital cystic adenomatoid tion, lung, antenatal diagnosis.
malforma-
P
RENATAL diagnosis has become an invaluable aid for the detection and recording of the progress of congenital malformations. This information enables obstetricians, neonatologists, and paediatric surgeons to plan management and counsel parents. We have studied the in utero evolution and postnatal progress of 32 cases of congenital cystic adenomatoid malformation (CCAM). Our aim was to establish the prognosis and best methods of treatment before and after birth. MATERIAL AND METHODS Between 1987 and 1992. 32 patients with fetal lung malformations were diagnosed antenatally as having CCAM. The gestational age at diagnosis ranged from 16 to 38 weeks with most patients presenting between 23 and 28 weeks. Lesions were classified according to the criteria proposed by Stocker’: type I macrocystic, multiple large cysts: fype 2 polymicrocystic, variable numbers of small cysts; type III homogenous echogenic mass. Sixteen cases were right sided, 15 left sided, and 1 was bilateral. The extent of pulmonary involvement was recorded as less than 50% of the thorax when the lesion was unilateral and ipsilateral lung could be identified: 50% where no normal lung was seen on the affected side: more than 50% when a mediastinal shift was observed; and 100% when no normal pulmonary parenchyma was seen. The potential prognostic indicators examined were: presence or absence of hydrops fetalis, polyhydramnios, and mediastinal shift. Fetal karyotyping was performed on all cases. In 4 cases, in utero drainage was performed because of developing hydrops fetalis and in the hope of avoiding pulmonary hypoplasia. This was performed by passing a double pigtail catheter into JournalofPediarric Surgery, Vol28, No 8 (August), 1993: pp
1009-1011
the amniotic cavity. The fetus was paralysed with vecuronium bromide, and a metal cannula was inserted through the amniotic cavity and through the fetal chest wall, draining the largest cysts. Cesarean section was not routinely performed. Postnatally, the assessment included routine chest x-rays, computed axial tomography, and in 6 cases angiography. The routine plan for intervention was to operate within the first four months. The diagnosis was confirmed histologically in all cases on whom resections were performed or following autopsy. RESULTS
Twelve fetuses had CCAM type I, 15 had type II, and 5 had type III. Severe hydrops was present in 6. Polyhydramnios was present in 16: in 4 cases this was severe. The prenatal assessment of the extent of the CCAM mass by ultrasound was less than 50% in 17, 50% in 6, more than 50% in 8, and 100% in 1. The karyotype was normal in all except one. No cardiac abnormalities were identified. The cerebral cortex was diffusely hypoechogenic in 2. Outcome and Management
Five pregnancies were terminated electively in accordance with French law (Table 1). In three cases the indication was severe hydrops, in one an abnormal karyotype 47,XXY and one was terminated at another institution against our advice. Of the 27 remaining pregnancies, intrauterine intervention was performed for hydrops or polyhydramnios in four instances. Two cases died: one aborted and one died at birth. In one case, 3 cysts one containing 50 mls of fluid were drained with a single catheter at 27 weeks. The lung expanded rapidly and the polyhydramnios resolved within 3 days. Nine weeks later the shunt became displaced resulting in immediate recurrence of polyhydramnios. The baby was delivered and operated on early. One further case (in which the hydrops decreased after drainage) awaits delivery. In 13 cases (3 type I, 8 type II, 2 type III) the size of the mass decreased spontaneously in utero. After
From the Service de Chirwgie Pediatrique and the Service de Radiologie Pediatrique, Hopital des Enfants Maludes. and the Clinique Universitaire de Port Royal-Hopital Cochin, Paris, France. Presented at the 39th Annual International Congress of the British Association of Paediattic Surgeons, Leeds, England. July 15-18, 1992. Address reprint requests to Y Revillon, Senice de Chirurgie Pediatrique, H6pital des Enfants Malades, Paris. France. Copyright 8 I993 by W.B. Saunders Company 0022.346819312808-0009$03.0010 1009
1010
REVILLON ET AL
Table 1. Outcome No. of Patients Termination of pregnancy
5
Death at birth
1
On-going
1
Operated
18
Embolization
2
Spontaneous regression
4
Waiting
1
birth the remaining 25 babies were assessed by chest x-ray and CT scan, and angiography in 6 cases. In four cases, the malformation was small and no operation has been performed. After more than 1 year of follow-up, they are progressing satisfactorily. In two cases the assessment showed a small lesion with a vascular pedicle. Embolization interrupted the vascular flow. Postoperatively the lung x-ray returned to normal. The follow-up on these is only 4 months. Eighteen babies have had operations. Three babies had immediate operation because of respiratory distress. In 15 cases the operation was performed near the fourth month of life. The operation involved lobectomy in 12 and segmentectomy in 6. The postoperative course was uneventful in 16. In 2 cases after emergency operation, cysts remained. One required reoperation 6 weeks later. The other awaits operation. One pregnancy is ongoing. The histological studies in all these cases confirmed the diagnosis of cystic adenomatoid malformation of the lung. In 5 cases it was associated with pulmonary sequestration. DISCUSSION
The CCAMs are rare2 but they represent 25% of the congenital lung malformations. Although the cause is unknown, a cessation of bronchiolar maturation with mesenchymal overgrowth is known to occur in the late fifth to early sixth week of gestation. This malformation is generally not associated with cardiac abnormality. In our series only one patient had an associated chromosomal abnormality, 47,XXY. Prenatal diagnosis is possible after the 16th week.3-6 The differential diagnoses on prenatal ultrasound include diaphragmatic hernia and rare thoracic masses such as bronchogenic or enteric cysts, mediastinal cystic teratoma, and pulmonary sequestration. There is an association between CCAM and pulmonary sequestration. Polyhydramnios is common presum-
ably due to fetal swallowing being decreased by esophageal compression. Polyhydramnios does not appear to be a prognostic indicator. Fetal hydrops secondary to the mediastinal shift caused by the malformation resulting in vena caval obstruction and cardiac compression appears to be the most important prognostic indicator. A prenatal diagnosis of type 1 generally indicates a good prognosis and type III, a poor prognosis.7-9 Two cases of type I were severe. One pregnancy was terminated and one baby died at birth. Three type I cases developed respiratory distress after birth. Previous reports have stated that type II is commonly associated with other malformations. In our series there were none. In 13 patients we observed a spontaneous reduction of the size of the cysts during the last quarter of the pregnancy.‘O-l2 This suggests that the focal anomaly does not tend to grow while the surrounding normal lung tissue continues to develop. Therefore the advantages and the hazards of invasive management should be carefully balanced with regard to gestational age and the risk of premature labor. In our successful case, drainage at 28 weeks appeared to be useful since the severe polyhydramnios resolved but recurred at 35 weeks after the shunt became displaced. Harrison et all3 have recently operated on such babies through a hysterotomy. Four of the babies presented with respiratory distress. Three were cured by removal of the affected segment of lung. One premature infant (31 weeks gestation) died. Detailed assessment using CT scanning was possible for the others. The pulmonary resection can and should be limited to the area of the malformation. In CCAM termination of pregnancy is indicated for children with hydrops, severe associated malformations, and chromosomal abnormalities. If the malformation is extensive intervention should be delayed as spontaneous regression is frequent. In utero drainage is recommended only in cases of hydrops. We have no experience of open prenatal surgery. The paediatric surgeon should be involved in the prenatal counselling of patients with CCAM. Usually it will be possible to give a good prognosis and relieve anxiety. The lesions can regress during the last quarter of pregnancy. Respiratory distress at birth is uncommon. Pulmonary resection can easily be limited to affected lung and after operation the prognosis is good.
REFERENCES 1. Stocker JT, Madewell JE, Drake RM: Congenital adenomatoid malformation of the lung: Classification and logic spectrum. Hum Pathol8:155-171, 1977
cystic
morfo-
2. Rosado adenomatoid 3. Adzick
de Christenson ML, Stocker JT: Congenital cystic malformation. Radiographics 11:865-886, 1991 NS, Harrison MR, Glick PL, et al: Fetal cystic
CCAM: PRENATAL
MANAGEMENT
AND PROGNOSIS
adenomatoid malformation: Prenatal diagnosis and natural history. J Pediatr Surg 20:483-488, 1985 4. Claiborne AK, Martin CM, McAlister WH, et al: Antenatal diagnosis of cystic adenomatoid malformation: Effect on patient management. Pediatr Radio1 15:337-339, 1985 5. Deacon CS, Smart PJ, Rimmer S: The antenatal diagnosis of congenital cystic adenomatoid malformation of the lung. Br J Radio1 63:968-970, 1990 6. Morcos SF, Lobb MO: The antenatal diagnosis by ultrasonography of type III congenital cystic adenomatoid malformation of the lung. Br J Obstet Gynecol93:1002-1005. 1986 7. Morris E, Constantine G, McHugo J: Cystic adenomatoid malformation of the lung: An obstetric and ultrasound perspective. Eur J Obstet Gynecol Reprod Biol40:11-15.1991 8. Nielson IR. Russo P, Laberge JM, et al: Congenital adenomatoid malformation of the lung: Current management and prognosis. J Pediatr Surg 26:975-981. 1991
1011
9. Ribet M, Pruvot FR, Dubos JP, et al: Congenital cystic adenomatoid malformation of the lung. Eur J Cardiothorac Surg 4:403-406, 1990 10. Glaves J, Baker JL: Spontaneous resolution of maternal hydramnios in coigenital adenomatoid malformation of the lung. Antenatal ultrasound features. Br J Obstet Gynecol90:1065-1068. 1983 11. Fine C, Adzick NS, Doubilet PM: Decreasing size of a congenital cystic adenomatoid malformation in utero. J Ultrasound Med 7:405-408, 1988 12. Sonek JD, Foley MR. Iams JD: Spontaneous regression of a large intrathoracic fetal lesion before birth. Am J Perinatol8:41-43. 1991 13. Harrison MR, Adzick NS, Jennings RW. et al: Antenatal intervention for congenital cystic adenomatoid malformation. Lancet 336:965-967. 3990