315
Early Human Development, 29 (1992) 315-319 Elsevier Scientific Publishers Ireland Ltd.
EHD 01306
Prenatal diagnosis of congenital diaphragmatic hernia and perinatal care: assessmentof lung hypoplasia S. Kamata, T. Hasegawa,S. Ishikawa, N. Usui, H. Okuyama, H. Kawahara, A. Kubota, M. Fukuzawa, K. Imura and A. Okada Department
of Pediatric
Surgery,
Osaka
University
Medical
School,
Osaka
553 (Japan)
To assessthe severity of lung hypoplasia, we have attempted to measure the lung thorax transverse area ratio (L/T) by using ultrasonic echography and to select immediate surgery after delivery by caesarian section. The evaluation of L/T for an index of lung hypoplasia was made by arterial blood gas data and clinical courses. Of 14 fetuses diagnosed, 13 had left sided CDH and one right sided case, from 11 to 38 weeks of pregnancy, 10 cases survived. The L/T in 14 fetuses with CDH was from 0.08 to 0.36 (mean 0.2 f 0.073) and was significantly lower than that of the controls. L/T was correlated best with data of arterial pH, PCOZ,preductal A-aDO before operation and the duration of mechanical ventilation in survivors. Although L/T was also significantly low in the cases with severe grade, diaphragmatic patch closure and ECMO therapy, no significant differences were noted in LiT between survivors and non-survivors. These results indicated that L/T may predict the severity of lung hypoplasia in CDH and that the combination of perinatal management bases on prenatal diagnosis of CDH and ECMO support may improve the outcome of fatal CDH with severe lung hypoplasia. Key words: congenital diaphragmatic hernia; lung hypoplasia; fetus; ultrasound; extracorporeal membrane oxygenation
Correspondence
to:
S. Kamata, Department of Pediatric Surgery, Osaka University Medical School,
Osaka 553, Japan. 0378-3782/92.605.00 0 1992 Elsevier Scientific Publishers Ireland Ltd. Printed and Published in Ireland
316
Introduction The outcome for fetuses with congenital diaphragmatic hernia (CDH) has been reported to be fatal because of associated anomalies and the severity of lung hypoplasia. Extensive research has, however, been continued on the treatment of CDH. To the fetuses with CDH, several treatments may be applicable, which include fetal surgery, extracorporeal membrane oxygenation (ECMO), immediate surgery after delivery and delayed surgery. In considering for treatment, the severity of lung hypoplasia should be assessed As an index of lung hypoplasia, we attempted to measure the lung thorax transverse area ratio (L/T) by using ultrasonic echography and to select immediate surgery after delivery by caesarian section for fetuses with severe lung hypoplasia. Patients and Methods For measurements of L/T [l], the transverse section of the thorax was analyzed at the level of 4-chamber view of the heart in the end-diastolic phase by using Hitachi EUB-25M with 3.5 MHz probe. L/T was calculated as the area of bilateral lungs divided by the area of thorax, which was delineated by ribs an sternum for avoiding the influence of subcutaneous edema. Data of L/T were shown measured from 35 to 38 weeks. As a control value, 0.52 f 0.04 was measured from 81 normal fetuses ranging from 18 to 36 weeks. Along with measurements of L/T, measurements of circulatory index by Doppler examination and lecithin sphingomyeline ratio (L/S) and examination of blood type and karyotype were performed. The fetuses with severe brain damage, severe circulatory failure and chromosomal abnormalities were excluded from the study. Immediate surgery after delivery by caesarian section at 36 or 37 weeks of gestation was selected in fetuses with L/T less than 0.24; namely, immediately after delivery, the babies were transported to the next operating room and were intubated for resuscitation. After securing peripheral and central venous lines and preductal and postductal arterial lines, reduction of abdominal organs was performed through the subcostal incision. The severity of each patient was classified into four groups according to our criteria; namely, A-aDO in group A is not more than 500 mmHg before operation, preductal and postductal A-aD02 in group B is not more than 500 mmHg after operation, preductal A-aD02 in group C is not more than 500 mmHg but postductal A-aDO is more than 500 mmHg after operation and preductal and postductal A-aDO in group D is more than 500 mmHg even after operation. The evaluation was made by arterial blood gas data and their clinical courses. Results Of fourteen fetuses with left sided CDH, except one right sided case, cesarian section and immediate repair were performed in 12 cases. Operation was started at mean time 1.88 h after birth. Of three cases with L/T not less than 0.28, vaginal delivery was selected in one case. Two cases were delivered at 41 and 39 weeks of gestation and then transported to the operating room of our hospital 5 h after birth.
371 TABLE I Treatment and outcome Case no.
m
Delivery
Closure of diaphragm
MV (days)
Special treatment (days)
Outcome
1 2 9 3 14 8 5 11 4 IO 12 7 6 13
0.36 0.28 0.28 0.24 0.23 0.22 0.21 0.19 0.17 0.17 0.15 0.12 0.11 0.08
c/sa V c/s c/s c/s us cts c/s ClSb c/s c/s c/s c/s c/s
Direct Direct Direct Direct Direct Direct Patch Direct Patch Patch Patch Patch Patch Patch
5 8 15 7 21 9 12 34 16 19 13 19 18 28
-
Alive Alive Alive Alive Alive Alive Alive Alive Died Died Died Died Alive Alive
HFOV (4) HFOV HFOV HFOV HFOV HFOV HFOV HFOV HFOV HFOV ECMO
(2) (3) (3) (6) (5) (lo), ECMO (19) (I), ECMO (I), ECMO (5), ECMO (5)
(4) (13) (18) (5)
Abbreviations: L/T, lung thorax transverse area ratio; MV, mechanichal ventilation; C/S, caesarian section; HFOV, high frequency oscillatory ventilation; ECMO, extracorporeal membrane oxygenation. aFor a maternal reason. bEmergency operation for fetal distress.
Finally measured L/T from 35 to 38 weeks of gestation in 14 fetuses with CDH was ranged from 0.08 to 0.36 (mean 0.201 f 0.076) and was significantly lower than that of the controls. Significant correlation was observed between final L/T and gestational week at diagnosis. Amniotic fluid was examined at 35 weeks in 6 cases, in which L/S was normal except for one case. This case, however, showed positive gastric shake test after birth. Two fetuses showed abnormal data of Doppler examination. One fetus was complicated with DORV and coarctation of aorta. The other fetus developed fetal distress and caesarian section was emergently performed at 35 weeks. L/T was correlated with the preductal arterial pH under manual ventilation at birth. However, no correlation was observed under high frequency oscillatory ventilation (HFOV). WT was correlated with arterial PO* under manual ventilation and HFOV with pure oxygen. L/T ratio was also correlated with A-aD02 under manual ventilation and HFOV. After surgical reduction of abdominal organs, a significant elevation of preductal Pa02 was observed. In patients with a large defect, diaphragm was repaired with artificial patch. L/T in patients with patch closure was significantly lower than that in patients with direct closure. Patients with patch closure also showed poor prognosis. According to the severity classification, patients were divided into 3 groups. In group C and in group D, L/T was significantly lower than group A and B. L/T in group D was significantly lower than group C. Patients in group D also showed poor
378
prognosis. ECMO therapy was performed in live patients. In these patients, L/T was significantly lower than that in other patients. Significant correlation was also observed between L/T and the duration of mechanical ventilation in survivors. Although L/T correlated to the respiratory condition after birth, no significant difference was observed between L/T in survivors and non-survivors. Discussion As an index for lung hypoplasia, either chest circumference or the ratio of chest abdominal circumferences have been used in fetuses with oligohydramnion. However, these parameters are thought to be not applicable in fetuses with CDH. L/T well correlated to postnatal arterial blood gas deta and duration of mechanical ventilation in survivors and may predict type of diaphragmatic repair, involvement of PFC, necessity of HFOV and ECMO support. These may partially depend on the maturity of the lung, which may be thought to be accomplished by normal levels of L/S, shake test and term delivery. As a prognostic index in CDH, polyhydramnion has been mentioned. Indeed, our cases with polyhydramnion had decreased L/T due to a lung compression by intrathoracic dilated stomach. However, no significant difference was observed between L/T in fetuses with and without polyhydramnion. Measurements of L/T may predict lung hypoplasia due to compression by other organs such as liver and intestine and may afford more precise information of lung hypoplasia in fetuses with CDH. Immediate postnatal surgery fairly improved blood gas data in fetuses with CDH along with the use of HFOV. Minimizing the duration of acidosis and hypoxia by caesarian delivery and immediate operation may prevent involvement of PFC before operation and may improve prognosis. The fetuses with mildly decreased L/T more than 0.28 may, however, have no indication for immediate surgery following caesarian section. In fetuses with severely decreased LiT not more than 0.17, the effects of immediate surgery and HFOV could not be fully expected. Therefore, surgical stress and intraoperative fluid load may transiently deteriorate postoperative respiratory condition in these cases. Because these, however, eventually necessitated ECMO support, it should be considered which is preferable ECMO after diaphragmatic repair or the repair after and on ECMO. In our patients, early initiation of ECMO therapy may have releaved two cases. In other two patients, ECMO therapy failed because of coarctation of aorta and massive air way bleeding due to patent ductus. Early cardiovascular surgery should have been considered. As one of criteria for fetal surgery, liver and stomach in the chest have been reported. In this results, however, only fetuses with intrathoracic liver showed poor prognosis. The fact that two patients with minimum L/T survived indicated that the combination of perinatal management based on prenatal diagnosis of CDH and ECMO support may improve the outcome of fatal CDH with severe lung hypoplasia rather than fetal surgery. It has been reported that the mortality of CDH with ECMO depends on bleeding complications. Perinatal management based on
319
prenatal diagnosis of CDH may decrease predisposing factors of bleeding complications such as immaturity, hypoxia and acidosis. In summury, L/T correlated to the blood gas data after birth and the duration of mechanical ventilation in survivors. And also, significant differences were observed in L/T among type of closure, severity classification and use of ECMO therapy. In conclusion, along with an accurate diagnosis of combined anomalies, examination for confirming lung maturation and monitoring of circulation in the fetus, measurements of L/T may predict respiratory status in CDH after birth and combined ECMO therapy may improve the outcome of fatal CDH with severe lung hypoplasia. Reference 1 Hasegawa, T., Kamata, S. Imura, K. et al. (1990): Use of lung-thorax transverse area ratio in the antenatal evaluation of lung hypoplasia in congenital diaphragmatic hernia. J. Clin. Ultrasound, 18, 705-709.
Early Human Development, 29 (1992) 315-319 Elsevier Scientific Publishers Ireland Ltd.
EHD 01306
Prenatal diagnosis of congenital diaphragmatic hernia and perinatal care: assessmentof lung hypoplasia S. Kamata, T. Hasegawa,S. Ishikawa, N. Usui, H. Okuyama, H. Kawahara, A. Kubota, M. Fukuzawa, K. Imura and A. Okada Department
of Pediatric
Surgery,
Osaka
University
Medical
School,
Osaka
553 (Japan)
To assessthe severity of lung hypoplasia, we have attempted to measure the lung thorax transverse area ratio (L/T) by using ultrasonic echography and to select immediate surgery after delivery by caesarian section. The evaluation of L/T for an index of lung hypoplasia was made by arterial blood gas data and clinical courses. Of 14 fetuses diagnosed, 13 had left sided CDH and one right sided case, from 11 to 38 weeks of pregnancy, 10 cases survived. The L/T in 14 fetuses with CDH was from 0.08 to 0.36 (mean 0.2 f 0.073) and was significantly lower than that of the controls. L/T was correlated best with data of arterial pH, PCOZ,preductal A-aDO before operation and the duration of mechanical ventilation in survivors. Although L/T was also significantly low in the cases with severe grade, diaphragmatic patch closure and ECMO therapy, no significant differences were noted in LiT between survivors and non-survivors. These results indicated that L/T may predict the severity of lung hypoplasia in CDH and that the combination of perinatal management bases on prenatal diagnosis of CDH and ECMO support may improve the outcome of fatal CDH with severe lung hypoplasia. Key words: congenital diaphragmatic hernia; lung hypoplasia; fetus; ultrasound; extracorporeal membrane oxygenation
Correspondence
to:
S. Kamata, Department of Pediatric Surgery, Osaka University Medical School,
Osaka 553, Japan. 0378-3782/92.605.00 0 1992 Elsevier Scientific Publishers Ireland Ltd. Printed and Published in Ireland
316
Introduction The outcome for fetuses with congenital diaphragmatic hernia (CDH) has been reported to be fatal because of associated anomalies and the severity of lung hypoplasia. Extensive research has, however, been continued on the treatment of CDH. To the fetuses with CDH, several treatments may be applicable, which include fetal surgery, extracorporeal membrane oxygenation (ECMO), immediate surgery after delivery and delayed surgery. In considering for treatment, the severity of lung hypoplasia should be assessed As an index of lung hypoplasia, we attempted to measure the lung thorax transverse area ratio (L/T) by using ultrasonic echography and to select immediate surgery after delivery by caesarian section for fetuses with severe lung hypoplasia. Patients and Methods For measurements of L/T [l], the transverse section of the thorax was analyzed at the level of 4-chamber view of the heart in the end-diastolic phase by using Hitachi EUB-25M with 3.5 MHz probe. L/T was calculated as the area of bilateral lungs divided by the area of thorax, which was delineated by ribs an sternum for avoiding the influence of subcutaneous edema. Data of L/T were shown measured from 35 to 38 weeks. As a control value, 0.52 f 0.04 was measured from 81 normal fetuses ranging from 18 to 36 weeks. Along with measurements of L/T, measurements of circulatory index by Doppler examination and lecithin sphingomyeline ratio (L/S) and examination of blood type and karyotype were performed. The fetuses with severe brain damage, severe circulatory failure and chromosomal abnormalities were excluded from the study. Immediate surgery after delivery by caesarian section at 36 or 37 weeks of gestation was selected in fetuses with L/T less than 0.24; namely, immediately after delivery, the babies were transported to the next operating room and were intubated for resuscitation. After securing peripheral and central venous lines and preductal and postductal arterial lines, reduction of abdominal organs was performed through the subcostal incision. The severity of each patient was classified into four groups according to our criteria; namely, A-aDO in group A is not more than 500 mmHg before operation, preductal and postductal A-aD02 in group B is not more than 500 mmHg after operation, preductal A-aD02 in group C is not more than 500 mmHg but postductal A-aDO is more than 500 mmHg after operation and preductal and postductal A-aDO in group D is more than 500 mmHg even after operation. The evaluation was made by arterial blood gas data and their clinical courses. Results Of fourteen fetuses with left sided CDH, except one right sided case, cesarian section and immediate repair were performed in 12 cases. Operation was started at mean time 1.88 h after birth. Of three cases with L/T not less than 0.28, vaginal delivery was selected in one case. Two cases were delivered at 41 and 39 weeks of gestation and then transported to the operating room of our hospital 5 h after birth.
371 TABLE I Treatment and outcome Case no.
m
Delivery
Closure of diaphragm
MV (days)
Special treatment (days)
Outcome
1 2 9 3 14 8 5 11 4 IO 12 7 6 13
0.36 0.28 0.28 0.24 0.23 0.22 0.21 0.19 0.17 0.17 0.15 0.12 0.11 0.08
c/sa V c/s c/s c/s us cts c/s ClSb c/s c/s c/s c/s c/s
Direct Direct Direct Direct Direct Direct Patch Direct Patch Patch Patch Patch Patch Patch
5 8 15 7 21 9 12 34 16 19 13 19 18 28
-
Alive Alive Alive Alive Alive Alive Alive Alive Died Died Died Died Alive Alive
HFOV (4) HFOV HFOV HFOV HFOV HFOV HFOV HFOV HFOV HFOV ECMO
(2) (3) (3) (6) (5) (lo), ECMO (19) (I), ECMO (I), ECMO (5), ECMO (5)
(4) (13) (18) (5)
Abbreviations: L/T, lung thorax transverse area ratio; MV, mechanichal ventilation; C/S, caesarian section; HFOV, high frequency oscillatory ventilation; ECMO, extracorporeal membrane oxygenation. aFor a maternal reason. bEmergency operation for fetal distress.
Finally measured L/T from 35 to 38 weeks of gestation in 14 fetuses with CDH was ranged from 0.08 to 0.36 (mean 0.201 f 0.076) and was significantly lower than that of the controls. Significant correlation was observed between final L/T and gestational week at diagnosis. Amniotic fluid was examined at 35 weeks in 6 cases, in which L/S was normal except for one case. This case, however, showed positive gastric shake test after birth. Two fetuses showed abnormal data of Doppler examination. One fetus was complicated with DORV and coarctation of aorta. The other fetus developed fetal distress and caesarian section was emergently performed at 35 weeks. L/T was correlated with the preductal arterial pH under manual ventilation at birth. However, no correlation was observed under high frequency oscillatory ventilation (HFOV). WT was correlated with arterial PO* under manual ventilation and HFOV with pure oxygen. L/T ratio was also correlated with A-aD02 under manual ventilation and HFOV. After surgical reduction of abdominal organs, a significant elevation of preductal Pa02 was observed. In patients with a large defect, diaphragm was repaired with artificial patch. L/T in patients with patch closure was significantly lower than that in patients with direct closure. Patients with patch closure also showed poor prognosis. According to the severity classification, patients were divided into 3 groups. In group C and in group D, L/T was significantly lower than group A and B. L/T in group D was significantly lower than group C. Patients in group D also showed poor
378
prognosis. ECMO therapy was performed in live patients. In these patients, L/T was significantly lower than that in other patients. Significant correlation was also observed between L/T and the duration of mechanical ventilation in survivors. Although L/T correlated to the respiratory condition after birth, no significant difference was observed between L/T in survivors and non-survivors. Discussion As an index for lung hypoplasia, either chest circumference or the ratio of chest abdominal circumferences have been used in fetuses with oligohydramnion. However, these parameters are thought to be not applicable in fetuses with CDH. L/T well correlated to postnatal arterial blood gas deta and duration of mechanical ventilation in survivors and may predict type of diaphragmatic repair, involvement of PFC, necessity of HFOV and ECMO support. These may partially depend on the maturity of the lung, which may be thought to be accomplished by normal levels of L/S, shake test and term delivery. As a prognostic index in CDH, polyhydramnion has been mentioned. Indeed, our cases with polyhydramnion had decreased L/T due to a lung compression by intrathoracic dilated stomach. However, no significant difference was observed between L/T in fetuses with and without polyhydramnion. Measurements of L/T may predict lung hypoplasia due to compression by other organs such as liver and intestine and may afford more precise information of lung hypoplasia in fetuses with CDH. Immediate postnatal surgery fairly improved blood gas data in fetuses with CDH along with the use of HFOV. Minimizing the duration of acidosis and hypoxia by caesarian delivery and immediate operation may prevent involvement of PFC before operation and may improve prognosis. The fetuses with mildly decreased L/T more than 0.28 may, however, have no indication for immediate surgery following caesarian section. In fetuses with severely decreased LiT not more than 0.17, the effects of immediate surgery and HFOV could not be fully expected. Therefore, surgical stress and intraoperative fluid load may transiently deteriorate postoperative respiratory condition in these cases. Because these, however, eventually necessitated ECMO support, it should be considered which is preferable ECMO after diaphragmatic repair or the repair after and on ECMO. In our patients, early initiation of ECMO therapy may have releaved two cases. In other two patients, ECMO therapy failed because of coarctation of aorta and massive air way bleeding due to patent ductus. Early cardiovascular surgery should have been considered. As one of criteria for fetal surgery, liver and stomach in the chest have been reported. In this results, however, only fetuses with intrathoracic liver showed poor prognosis. The fact that two patients with minimum L/T survived indicated that the combination of perinatal management based on prenatal diagnosis of CDH and ECMO support may improve the outcome of fatal CDH with severe lung hypoplasia rather than fetal surgery. It has been reported that the mortality of CDH with ECMO depends on bleeding complications. Perinatal management based on
319
prenatal diagnosis of CDH may decrease predisposing factors of bleeding complications such as immaturity, hypoxia and acidosis. In summury, L/T correlated to the blood gas data after birth and the duration of mechanical ventilation in survivors. And also, significant differences were observed in L/T among type of closure, severity classification and use of ECMO therapy. In conclusion, along with an accurate diagnosis of combined anomalies, examination for confirming lung maturation and monitoring of circulation in the fetus, measurements of L/T may predict respiratory status in CDH after birth and combined ECMO therapy may improve the outcome of fatal CDH with severe lung hypoplasia. Reference 1 Hasegawa, T., Kamata, S. Imura, K. et al. (1990): Use of lung-thorax transverse area ratio in the antenatal evaluation of lung hypoplasia in congenital diaphragmatic hernia. J. Clin. Ultrasound, 18, 705-709.
