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Antenatal ultrasound diagnosis in cystic adenomatoid malformation of the lung—Case report
Antenatal Ultrasound Diagnosis In Cystic Adenomatoid Malformation of the Lung--Case Report By Urs G. Stauffer, Guido Savoldelli, and Dieter Mieth Zfrich, Switzerland 9 Antenatal ultrasound diagnosis of cystic adenomatoid malformation was made in a fetus of 27 weeks gestation, The child was born at term and successfully operated upon 3 0 h o u r s later. Early recognition by ultrasound allowed for adequate planning and preparation of m a n a g e m e n t b e t w e e n obstetricians, neonatologists, and pediatric s u r geons. The present case appears to be the f o u r t h one reported with antenatal ultrasound diagnosis and the first one with successful operation, longt e r m survival and cure. INDEX W O R D S : Cystic adenomatoid malformation (CAM}, maternal ultrasound.
23 year-old primigravida had a normal ultrasound of a single fetus at 22 weeks of gestation. At 27 weeks a repeated sonogram showed a multicystic, partially solid tumor in the :left hemithorax (Fig. 1A, 1B). The mass was distinct from the fetal heart and nonpulsatile. The diagnosis of cystic adenomatoid malforma-
A
Fig. 1. Ultrasound findings at 27 weeks of gestation. (A) Longitudinal section. (B) Cross-section. 1 normal lung tissue, 2 CAM affected lung, 3 displaced heart, 4 vertebra/ spine. Journal of Pediatric Surgery, Vol. 19, No. 2 (April), 1984
tion (CAM) was strongly suspected. No other congenital anomalies could be found and the size of the fetus was otherwise normal. In a team discussion between obstetricians, neonatologists and paediatric surgeons, it was decided to let pregnancy continue normally to term. Repeated sonograms showed that the tumour in the left chest grew in proportion with the child. At term it was decided to wait, i f possible, for normal delivery. A neonatologist and a pediatric surgical team were on call around the clock. At 40 6/7th weeks a normal 2630-g crying female infant was delivered. Postnatal adaptation with 80% oxygen was surprisingly good with Apgar Scores 7 / 9 / 9 . The initial x-ray immediately after birth of chest and abdomen (Fig. 2) showed a large multicystic, partially solid mass filling the left hemithorax, with massive displacement of the heart and mediastinum to the right and compression of the right lung. The abdomen was filled with intestinal loops. The diagnosis of CAM, type 1, according to Madewell ~ was confirmed. During the following hours, blood gas analyses and transcutaneous Po2 measurements were surprisingly normal. However, there was an increasing tachypnea from 78 to 110/min. within 24 hours. At 30 hours of age the child underwent surgical expl Oration. We found a partially solid, partially multicystic mass in the left hemithorax, confined to the left lower lobe which was resected. The left upper lobe was compressed but otherwise normal. The postoperative course was completely uneventful. The residual lobe distended slowly, extubation was possible after 48 hours, the pleural drain was removed 4 days after operation. The x-ray 1 year after surgery was normal. The child is now 2 years old and has a normal physical and psychomotoric development so far.
From the Division of Pediatric Surgery and Neonatology, University Children's Hospital, Zfirich, and the Department of Obstetrics, University Hospitals, Zfirich, Switzerland. Address reprint requests to Prof. U.G. Stauffer, Department of Pediatric Surgery, University Children's Hospital, Ziirich, Switzerland. 9 1984 by Grune & Stratton, Inc. 0022-3468/84/1902~9005501.00/0 141
142
STAUFFER, SAVOLDELLI, AND MIETH
DISCUSSION
Fig. 2. X-ray immediately after birth. Multicystic lesion filling the left hemi-thorax with massive displacement of the heart and the mediastinum to the right and compression of the right lung. Abdomen filled with gas-containing loops.
The resected lobe weighed 57 g. On section the normal lung parenchyma was replaced by multiple oval or polycyclic cysts with a diameter between 0.5 and 2.5 cm. Histological examination confirmed the diagnosis of CAM, type 1.
Cystic adenomatoid malformation of the lung is a rare congenital anomaly. The malformation is usually confined to one lobe; only in two of 31 cases collected by Madewell ~ two lobes were affected: Antenatal diagnosis allowed in our case for early recognition and adequate planning of management in a team approach between obstetricians, neonatologists and pediatric surgeons. The state of maturation of residual lung tissue is an important factor for ultimate survival in neonates undergoing lung resections. We decided, therefore, that pregnancy should be allowed to go to term. Approximately 30% of the alveolar fluid is expressed through the bronchotracheooral route as the fetal chest is compressed in passing through the birth canal. 2 This natural mechanical expression of alveolar fluid is of some importance for immediate postnatal respiratory adaptation. A normal vaginal delivery presented therefore some advantages in comparison to a caesarean section. On the other hand, surgical decompression might have been necessary immediately after birth. During the last 3 weeks of pregnancy, a pediatric surgical team was therefore always on call for this patient. When labor started, an operating room was reserved for immediate surgery and the neonatologist and an anesthesia team were present at birth. Because of a surprisingly good adaptation, the child underwent finally surgery on the second day of life. Antenatal diagnosis of C A M allows for better planning of the management of these patients and might save especially infants with development of severe respiratory distress syndrome immediately after birth which otherwise could be fatal. Our case seems to be the fourth one reported with antenatal diagnosis 3 and the first with successful operation and normal long-term survival. The child is now 2 years old and shows a completely normal physical and psychomotorical development.
REFERENCES
1. Madewell JE, Stocker JT, Korsower JM: Cystic A d e nomatoid Malformation of the Lung. Am J Roentgenol 124:436-448, 1975 2. Tucker TT, Smith WL, Smith JA: Fluid-Filled Cystic
AdenomatoidMalformation. Am J Roentgenol 129:323325, 1977 3. Donn SM, Martin JN, White SJ: Antenatal Ultrasound Findings in Cystic Adenomatoid Malformation. Pediatr Radiol 10:180~182, 1981
23 year-old primigravida had a normal ultrasound of a single fetus at 22 weeks of gestation. At 27 weeks a repeated sonogram showed a multicystic, partially solid tumor in the :left hemithorax (Fig. 1A, 1B). The mass was distinct from the fetal heart and nonpulsatile. The diagnosis of cystic adenomatoid malforma-
A
Fig. 1. Ultrasound findings at 27 weeks of gestation. (A) Longitudinal section. (B) Cross-section. 1 normal lung tissue, 2 CAM affected lung, 3 displaced heart, 4 vertebra/ spine. Journal of Pediatric Surgery, Vol. 19, No. 2 (April), 1984
tion (CAM) was strongly suspected. No other congenital anomalies could be found and the size of the fetus was otherwise normal. In a team discussion between obstetricians, neonatologists and paediatric surgeons, it was decided to let pregnancy continue normally to term. Repeated sonograms showed that the tumour in the left chest grew in proportion with the child. At term it was decided to wait, i f possible, for normal delivery. A neonatologist and a pediatric surgical team were on call around the clock. At 40 6/7th weeks a normal 2630-g crying female infant was delivered. Postnatal adaptation with 80% oxygen was surprisingly good with Apgar Scores 7 / 9 / 9 . The initial x-ray immediately after birth of chest and abdomen (Fig. 2) showed a large multicystic, partially solid mass filling the left hemithorax, with massive displacement of the heart and mediastinum to the right and compression of the right lung. The abdomen was filled with intestinal loops. The diagnosis of CAM, type 1, according to Madewell ~ was confirmed. During the following hours, blood gas analyses and transcutaneous Po2 measurements were surprisingly normal. However, there was an increasing tachypnea from 78 to 110/min. within 24 hours. At 30 hours of age the child underwent surgical expl Oration. We found a partially solid, partially multicystic mass in the left hemithorax, confined to the left lower lobe which was resected. The left upper lobe was compressed but otherwise normal. The postoperative course was completely uneventful. The residual lobe distended slowly, extubation was possible after 48 hours, the pleural drain was removed 4 days after operation. The x-ray 1 year after surgery was normal. The child is now 2 years old and has a normal physical and psychomotoric development so far.
From the Division of Pediatric Surgery and Neonatology, University Children's Hospital, Zfirich, and the Department of Obstetrics, University Hospitals, Zfirich, Switzerland. Address reprint requests to Prof. U.G. Stauffer, Department of Pediatric Surgery, University Children's Hospital, Ziirich, Switzerland. 9 1984 by Grune & Stratton, Inc. 0022-3468/84/1902~9005501.00/0 141
142
STAUFFER, SAVOLDELLI, AND MIETH
DISCUSSION
Fig. 2. X-ray immediately after birth. Multicystic lesion filling the left hemi-thorax with massive displacement of the heart and the mediastinum to the right and compression of the right lung. Abdomen filled with gas-containing loops.
The resected lobe weighed 57 g. On section the normal lung parenchyma was replaced by multiple oval or polycyclic cysts with a diameter between 0.5 and 2.5 cm. Histological examination confirmed the diagnosis of CAM, type 1.
Cystic adenomatoid malformation of the lung is a rare congenital anomaly. The malformation is usually confined to one lobe; only in two of 31 cases collected by Madewell ~ two lobes were affected: Antenatal diagnosis allowed in our case for early recognition and adequate planning of management in a team approach between obstetricians, neonatologists and pediatric surgeons. The state of maturation of residual lung tissue is an important factor for ultimate survival in neonates undergoing lung resections. We decided, therefore, that pregnancy should be allowed to go to term. Approximately 30% of the alveolar fluid is expressed through the bronchotracheooral route as the fetal chest is compressed in passing through the birth canal. 2 This natural mechanical expression of alveolar fluid is of some importance for immediate postnatal respiratory adaptation. A normal vaginal delivery presented therefore some advantages in comparison to a caesarean section. On the other hand, surgical decompression might have been necessary immediately after birth. During the last 3 weeks of pregnancy, a pediatric surgical team was therefore always on call for this patient. When labor started, an operating room was reserved for immediate surgery and the neonatologist and an anesthesia team were present at birth. Because of a surprisingly good adaptation, the child underwent finally surgery on the second day of life. Antenatal diagnosis of C A M allows for better planning of the management of these patients and might save especially infants with development of severe respiratory distress syndrome immediately after birth which otherwise could be fatal. Our case seems to be the fourth one reported with antenatal diagnosis 3 and the first with successful operation and normal long-term survival. The child is now 2 years old and shows a completely normal physical and psychomotorical development.
REFERENCES
1. Madewell JE, Stocker JT, Korsower JM: Cystic A d e nomatoid Malformation of the Lung. Am J Roentgenol 124:436-448, 1975 2. Tucker TT, Smith WL, Smith JA: Fluid-Filled Cystic
AdenomatoidMalformation. Am J Roentgenol 129:323325, 1977 3. Donn SM, Martin JN, White SJ: Antenatal Ultrasound Findings in Cystic Adenomatoid Malformation. Pediatr Radiol 10:180~182, 1981