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Long-term follow-up of patients with congenital diaphragmatic hernia
Long-Term Follow-Up of Patients With Congenital Diaphragmatic Hernia By lan S. Reid and R. J. Hutcherson
I
N 1971 C h a t r a t h et al. p u b l i s h e d a r e v i e w o f 14 c h i l d r e n w h o h a d h a d c o n g e n i t a l d i a p h r a g m a t i c h e r n i a s a n d h a d r e a c h e d t h e a g e s o f 6 - 1 2 yr. 1 T h e y s h o w e d t h a t , a l t h o u g h x - r a y f i n d i n g s w e r e n o r m a l in m o s t i n s t a n c e s , t h e r e w a s a r e s i d u a l d e f e c t in t h e v e n t i l a t o r y f u n c t i o n as s h o w n o n p u l m o n a r y f u n c t i o n tests. T h e y felt this d e f e c t c o u l d b e d u e to a p r o g r e s s i v e loss o f e l a s t i c fibers a n d c o n c l u d e d t h a t f o l l o w - u p s t u d i e s s h o u l d b e c a r r i e d o u t to a s c e r t a i n w h e t h e r f u r t h e r d e t e r i o r a t i o n will o c c u r . A t t h e R o y a l A l e x a n d r a H o s p i t a l f o r C h i l d r e n w e h a v e felt t h e s a m e c o n c e r n a n d in this s t u d y w e w e r e a b l e to r e v i e w p a t i e n t s u p t o 21 y r a f t e r t h e o r i g i n a l r e p a i r . MATERIALS A N D M E T H O D S
An attempt was made to locate all survivors admitted to the Royal Alexandra Hospital for Children with congenital diaphragmatic hernia excluding those with Morgagni hernias. Thirty patients were traced and came for the survey. From the original surgical notes, the hernias were classified as follows: Bochdalek, 25; eventration, 2; and others, 3.2,3 The three classified as "others" could not be placed in the Bochdalek group from information available from the surgical notes. However, since they were not Morgagni hernias, they will be included in this report. The patients' ages at follow-up are given in Table 1 together with the breakdown of neonatal presentation. Twenty-four of the hernias were on the left side, five on the right, and one patient had bilateral hernias. Of the 21 patients presenting in the neonatal period, 16 were less than 24 hr of age at operation. A clinical examination was carried out on each patient and this included the ability of the child to take part in sports and other recreations and some assessment of the child's vulnerability to respiratory tract infections. The general physique was noted. Each child had anteroposterior and lateral chest x-ray films. Pulmonary function tests were offered to all children over the age of 7 yr. Sixteen children had such tests. The tests consisted of ventilatory tests in all patients and lung volume estimations in the older patients. Lung scans were carried out on nine selected patients. RESULTS
G e n e r a l l y , t h e r a d i o l o g i c s t u d i e s r e v e a l e d n o r m a l l u n g fields w i t h s o m e distortion of the diaphragm. In some, there was evidence of decreased vascularity o n t h e side o f t h e h e r n i a s as c o m p a r e d w i t h t h e c o n t r a l a t e r a l side. O n e girl w h o h a d a left B o c h d a l e k h e r n i a r e p a i r e d o n t h e d a y o f b i r t h w a s r e v i e w e d at 14 y r o f age. T h e r e w a s h y p o p l a s i a o f t h e s o f t tissues o f t h e w h o l e o f t h e left c h e s t w a l l i n c l u d i n g t h e b r e a s t . L u n g s c a n a l s o r e v e a l e d h y p o p e r f u s i o n o f t h e left l u n g as c o m p a r e d w i t h t h e right.
From the Children's Medical Research Foundation, Royal Alexandra Hospital for Children, and the Royal Prince Alfred Hospital, Camperdown, N.S. W., Australia. Address for reprints: Dr. Ian S. Reid, Children's Medical Research Foundation, P.O. Box 61, Camperdown, N.S.W. 2050, Australia. 9 1976 by Grune & Stratton, Inc.
Journal of Pediatric Surgery, Vol. 11, No. 6 (December), 1976
939
940
REID AND HUTCHERSON
Table 1. Ages at Review and at Presentation of the 30 Patients Ages at Review
Neonatal Presentation
(Yr)
Yes
No
Total
>5 5-10 11-15 16-20 21
6 10 2 2 1
2 3 2 1 1
8 13 4 3 2
21
9
30
Of the 16 children who attempted pulmonary function tests, 3 were unable to cooperate and were, in fact, mentally retarded. Figure 1 summarizes the results of the pulmonary function tests in nine children--the oldest patients being 21 yr of age. The ages of the various patients are given on the abscissa, while the ordinate represents the percentage of the predicted reading for each of the tests. The overall pattern was one of low total lung capacity, low vital capacity, and high residual volume, giving a high residual volume to total lung capacity ratio. The readings of the 21-yr-old patient were doubly checked, since they showed a pattern seen in patients with airway obstruction with insipient or established 287~.279 260
240
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Fig. 1. G r a p h s h o w i n g results of pulmonary function tests of ten of the patients studied. The ordinate represents the result of each test as a percentage of that p r e d i c t e d for age. The abscissa gives the ages of the patients in years. VC, vital capacity; RV, residual volume; TLC, total lung capacity.
DIAPHRAGMATIC HERNIA
941
Fig. 2. Lung scan at 21 yr of age of patient operated on at 12 hr of life for left diaphragmatic hernia. Note areas of nonunified perfusion.
emphysema. However, this patient has never had any clinical evidence of obstructive airway disease or emphysema; on the contrary, she had been rem a r k a b l y free of respiratory illness during her lifetime. Her operation was performed at 12 hr of life and, following her survival, the surgeon r e c o m m e n d e d to the mother that the child learn singing to develop her lungs. She became a professional singer and has made a number of recordings that demonstrate her ability to hold a note with power. She has had one child and during the pregnancy had no problems with respiratory tract disease. The other 21-yr-old is an oyster farmer who is in extremely good physical condition; however, he probably has no p u l m o n a r y hypoplasia, since he presented with vomiting at the age of 7 mo and the hernia was found on investigating this. Generally, the lung scans showed hypoperfusion on the side of the hernia. This hypoperfusion was not always uniform but often a focal defect occurred. Usually, this focal defect was in the lower lobe and in most eases was probably due to irregularity of the diaphragm. This is illustrated in one child who had bilateral hernias. The left hernia was operated on at 2 mo of age and the right 1 m o later. The left lower lobe posteriorly was selectively hypoperfused. Since undergoing the scan, the child has been operated on for a recurrent hernia, which was obviously the reason for the area of hypoperfusion. It is therefore probable that these focal areas seen in the lower lobes m a y be due to diaphragmatic distortion. However, it is interesting to note that sequestration of lung was noted in association with the hernia at operation in three children with Bochdalek hernias, and one wonders whether focal areas of hypoperfusion m a y in some way be related to areas of sequestration. Figure 2 is the scan of the 21-yr-old singer who showed 5 0 ~ less perfusion of the left lung as c o m p a r e d with the right. However, in her case there were also some areas of nonunified perfusion in each l u n g - - a n appearance that is seen in patients with airway obstructive conditions such as asthma or e m p h y s e m a . The general functions of the patients were satisfactory. There was gross mental retardation requiring special schooling in three children, who represent
942
REID AND HUTCHERSON
1 0 ~ o f the c h i l d r e n s t u d i e d . A few c h i l d r e n were said to have h a d a s t h m a , b u t this was p r o b a b l y n o t o u t s i d e the i n c i d e n c e in t h e p o p u l a t i o n . A t review, these children did not exhibit any evidence of asthma clinically or radiologically. All c h i l d r e n c o p e d s a t i s f a c t o r i l y with o t h e r c h i l d r e n in s p o r t s , a l t h o u g h n o n e was o u t s t a n d i n g in a n y s p o r t . T h e r e w a s n o e v i d e n c e o f i n c r e a s e d s u s c e p t i b i l i t y to r e s p i r a t o r y i n f e c t i o n in the g r o u p . It w o u l d t h e r e f o r e a p p e a r t h a t c h i l d r e n w h o survive b i r t h a n d o p e r a t i v e r e p a i r go on to d e v e l o p e n o u g h lung f u n c t i o n to c o p e with n o r m a l activities at least t h r o u g h t h e t e e n a g e p e r i o d , b u t we h a v e little i n f o r m a t i o n b e y o n d this age. SUMMARY
A l o n g - t e r m f o l l o w - u p was c a r r i e d o u t on 30 p a t i e n t s w h o h a d c o n g e n i t a l d i a p h r a g m a t i c h e r n i a s o p e r a t e d on o v e r a 21-yr p e r i o d . 4'5 T h e review t o o k the f o r m o f clinical e x a m i n a t i o n , chest x-rays, p u l m o n a r y f u n c t i o n tests, a n d lung scans. L u n g s t h a t were h y p o p l a s t i c at b i r t h r e m a i n e d u n d e r d e v e l o p e d as j u d g e d b y p u l m o n a r y .function tests a n d h y p o p e r f u s i o n o n l u n g scan; h o w e v e r , c h i l d r e n w h o survived b i r t h 6 a n d o p e r a t i v e r e p a i r w e n t on to live n o r m a l lives t h r o u g h the p e r i o d studied. T h e r e was s o m e s u g g e s t i o n f r o m p u l m o n a r y f u n c t i o n tests a n d lung scans t h a t in the o l d e r g r o u p a p r e e m p h y s e m a t o u s state m a y exist b u t this was a s y m p t o m a t i c . T h e r e was n o evidence o f i n c r e a s e d s u s c e p t i b i l i t y to r e s p i r a t o r y i n f e c t i o n in the g r o u p . T e n p e r cent o f the c h i l d r e n were m e n t a l l y ret a r d e d , e v i d e n t l y f r o m h y p o x i a after b i r t h . ACKNOWLEDGMENT
We are grateful to Dr. John Brown, respiratory pediatrician of the Royal Alexandra Hospital for Children, for arranging and interpreting the pulmonary function tests. The cooperation of Dr. J. G. Morris, Director of the Department of Nuclear Medicine, Royal Prince Alfred Hospital, for making his staff and equipment available is gratefully recognized. The cooperation of the staff members of the Radiology Department of the Royal Alexandra Hospital for Children is also much appreciated. REFERENCES
1. Chatrath RR, El Shafie M, Jones RS: Fate of hypoplastic lungs after repair of congenital diaphragmatic hernia, Arch Dis Child 46:633, 1971 2. Snyder WH, Greaney EM: Congenital diaphragmatic hernia: 77 consecutive cases. Surgery 57:576, 1965 3. Carter REB, Waterston D J, Aberdeen E~ Hernia and eventration of the diaphragm in childhood. Lancet 1:659, 1962
4. Allen MS, Thomson SA: Congenital diaphragmatic hernia in children under one year of age: A 24 year review. J Pediatr Surg 1:157, 1966 5. Graivier L: Congenital diaphragmatic hernia. South Med J 67:59, 1974 6. Raphaely RC, Downes J J: Congenital diaphragmatic hernia: Prediction of survival. J Pediatr Surg 8:815, 1973
I
N 1971 C h a t r a t h et al. p u b l i s h e d a r e v i e w o f 14 c h i l d r e n w h o h a d h a d c o n g e n i t a l d i a p h r a g m a t i c h e r n i a s a n d h a d r e a c h e d t h e a g e s o f 6 - 1 2 yr. 1 T h e y s h o w e d t h a t , a l t h o u g h x - r a y f i n d i n g s w e r e n o r m a l in m o s t i n s t a n c e s , t h e r e w a s a r e s i d u a l d e f e c t in t h e v e n t i l a t o r y f u n c t i o n as s h o w n o n p u l m o n a r y f u n c t i o n tests. T h e y felt this d e f e c t c o u l d b e d u e to a p r o g r e s s i v e loss o f e l a s t i c fibers a n d c o n c l u d e d t h a t f o l l o w - u p s t u d i e s s h o u l d b e c a r r i e d o u t to a s c e r t a i n w h e t h e r f u r t h e r d e t e r i o r a t i o n will o c c u r . A t t h e R o y a l A l e x a n d r a H o s p i t a l f o r C h i l d r e n w e h a v e felt t h e s a m e c o n c e r n a n d in this s t u d y w e w e r e a b l e to r e v i e w p a t i e n t s u p t o 21 y r a f t e r t h e o r i g i n a l r e p a i r . MATERIALS A N D M E T H O D S
An attempt was made to locate all survivors admitted to the Royal Alexandra Hospital for Children with congenital diaphragmatic hernia excluding those with Morgagni hernias. Thirty patients were traced and came for the survey. From the original surgical notes, the hernias were classified as follows: Bochdalek, 25; eventration, 2; and others, 3.2,3 The three classified as "others" could not be placed in the Bochdalek group from information available from the surgical notes. However, since they were not Morgagni hernias, they will be included in this report. The patients' ages at follow-up are given in Table 1 together with the breakdown of neonatal presentation. Twenty-four of the hernias were on the left side, five on the right, and one patient had bilateral hernias. Of the 21 patients presenting in the neonatal period, 16 were less than 24 hr of age at operation. A clinical examination was carried out on each patient and this included the ability of the child to take part in sports and other recreations and some assessment of the child's vulnerability to respiratory tract infections. The general physique was noted. Each child had anteroposterior and lateral chest x-ray films. Pulmonary function tests were offered to all children over the age of 7 yr. Sixteen children had such tests. The tests consisted of ventilatory tests in all patients and lung volume estimations in the older patients. Lung scans were carried out on nine selected patients. RESULTS
G e n e r a l l y , t h e r a d i o l o g i c s t u d i e s r e v e a l e d n o r m a l l u n g fields w i t h s o m e distortion of the diaphragm. In some, there was evidence of decreased vascularity o n t h e side o f t h e h e r n i a s as c o m p a r e d w i t h t h e c o n t r a l a t e r a l side. O n e girl w h o h a d a left B o c h d a l e k h e r n i a r e p a i r e d o n t h e d a y o f b i r t h w a s r e v i e w e d at 14 y r o f age. T h e r e w a s h y p o p l a s i a o f t h e s o f t tissues o f t h e w h o l e o f t h e left c h e s t w a l l i n c l u d i n g t h e b r e a s t . L u n g s c a n a l s o r e v e a l e d h y p o p e r f u s i o n o f t h e left l u n g as c o m p a r e d w i t h t h e right.
From the Children's Medical Research Foundation, Royal Alexandra Hospital for Children, and the Royal Prince Alfred Hospital, Camperdown, N.S. W., Australia. Address for reprints: Dr. Ian S. Reid, Children's Medical Research Foundation, P.O. Box 61, Camperdown, N.S.W. 2050, Australia. 9 1976 by Grune & Stratton, Inc.
Journal of Pediatric Surgery, Vol. 11, No. 6 (December), 1976
939
940
REID AND HUTCHERSON
Table 1. Ages at Review and at Presentation of the 30 Patients Ages at Review
Neonatal Presentation
(Yr)
Yes
No
Total
>5 5-10 11-15 16-20 21
6 10 2 2 1
2 3 2 1 1
8 13 4 3 2
21
9
30
Of the 16 children who attempted pulmonary function tests, 3 were unable to cooperate and were, in fact, mentally retarded. Figure 1 summarizes the results of the pulmonary function tests in nine children--the oldest patients being 21 yr of age. The ages of the various patients are given on the abscissa, while the ordinate represents the percentage of the predicted reading for each of the tests. The overall pattern was one of low total lung capacity, low vital capacity, and high residual volume, giving a high residual volume to total lung capacity ratio. The readings of the 21-yr-old patient were doubly checked, since they showed a pattern seen in patients with airway obstruction with insipient or established 287~.279 260
240
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140
120
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0 O0 0 0
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Fig. 1. G r a p h s h o w i n g results of pulmonary function tests of ten of the patients studied. The ordinate represents the result of each test as a percentage of that p r e d i c t e d for age. The abscissa gives the ages of the patients in years. VC, vital capacity; RV, residual volume; TLC, total lung capacity.
DIAPHRAGMATIC HERNIA
941
Fig. 2. Lung scan at 21 yr of age of patient operated on at 12 hr of life for left diaphragmatic hernia. Note areas of nonunified perfusion.
emphysema. However, this patient has never had any clinical evidence of obstructive airway disease or emphysema; on the contrary, she had been rem a r k a b l y free of respiratory illness during her lifetime. Her operation was performed at 12 hr of life and, following her survival, the surgeon r e c o m m e n d e d to the mother that the child learn singing to develop her lungs. She became a professional singer and has made a number of recordings that demonstrate her ability to hold a note with power. She has had one child and during the pregnancy had no problems with respiratory tract disease. The other 21-yr-old is an oyster farmer who is in extremely good physical condition; however, he probably has no p u l m o n a r y hypoplasia, since he presented with vomiting at the age of 7 mo and the hernia was found on investigating this. Generally, the lung scans showed hypoperfusion on the side of the hernia. This hypoperfusion was not always uniform but often a focal defect occurred. Usually, this focal defect was in the lower lobe and in most eases was probably due to irregularity of the diaphragm. This is illustrated in one child who had bilateral hernias. The left hernia was operated on at 2 mo of age and the right 1 m o later. The left lower lobe posteriorly was selectively hypoperfused. Since undergoing the scan, the child has been operated on for a recurrent hernia, which was obviously the reason for the area of hypoperfusion. It is therefore probable that these focal areas seen in the lower lobes m a y be due to diaphragmatic distortion. However, it is interesting to note that sequestration of lung was noted in association with the hernia at operation in three children with Bochdalek hernias, and one wonders whether focal areas of hypoperfusion m a y in some way be related to areas of sequestration. Figure 2 is the scan of the 21-yr-old singer who showed 5 0 ~ less perfusion of the left lung as c o m p a r e d with the right. However, in her case there were also some areas of nonunified perfusion in each l u n g - - a n appearance that is seen in patients with airway obstructive conditions such as asthma or e m p h y s e m a . The general functions of the patients were satisfactory. There was gross mental retardation requiring special schooling in three children, who represent
942
REID AND HUTCHERSON
1 0 ~ o f the c h i l d r e n s t u d i e d . A few c h i l d r e n were said to have h a d a s t h m a , b u t this was p r o b a b l y n o t o u t s i d e the i n c i d e n c e in t h e p o p u l a t i o n . A t review, these children did not exhibit any evidence of asthma clinically or radiologically. All c h i l d r e n c o p e d s a t i s f a c t o r i l y with o t h e r c h i l d r e n in s p o r t s , a l t h o u g h n o n e was o u t s t a n d i n g in a n y s p o r t . T h e r e w a s n o e v i d e n c e o f i n c r e a s e d s u s c e p t i b i l i t y to r e s p i r a t o r y i n f e c t i o n in the g r o u p . It w o u l d t h e r e f o r e a p p e a r t h a t c h i l d r e n w h o survive b i r t h a n d o p e r a t i v e r e p a i r go on to d e v e l o p e n o u g h lung f u n c t i o n to c o p e with n o r m a l activities at least t h r o u g h t h e t e e n a g e p e r i o d , b u t we h a v e little i n f o r m a t i o n b e y o n d this age. SUMMARY
A l o n g - t e r m f o l l o w - u p was c a r r i e d o u t on 30 p a t i e n t s w h o h a d c o n g e n i t a l d i a p h r a g m a t i c h e r n i a s o p e r a t e d on o v e r a 21-yr p e r i o d . 4'5 T h e review t o o k the f o r m o f clinical e x a m i n a t i o n , chest x-rays, p u l m o n a r y f u n c t i o n tests, a n d lung scans. L u n g s t h a t were h y p o p l a s t i c at b i r t h r e m a i n e d u n d e r d e v e l o p e d as j u d g e d b y p u l m o n a r y .function tests a n d h y p o p e r f u s i o n o n l u n g scan; h o w e v e r , c h i l d r e n w h o survived b i r t h 6 a n d o p e r a t i v e r e p a i r w e n t on to live n o r m a l lives t h r o u g h the p e r i o d studied. T h e r e was s o m e s u g g e s t i o n f r o m p u l m o n a r y f u n c t i o n tests a n d lung scans t h a t in the o l d e r g r o u p a p r e e m p h y s e m a t o u s state m a y exist b u t this was a s y m p t o m a t i c . T h e r e was n o evidence o f i n c r e a s e d s u s c e p t i b i l i t y to r e s p i r a t o r y i n f e c t i o n in the g r o u p . T e n p e r cent o f the c h i l d r e n were m e n t a l l y ret a r d e d , e v i d e n t l y f r o m h y p o x i a after b i r t h . ACKNOWLEDGMENT
We are grateful to Dr. John Brown, respiratory pediatrician of the Royal Alexandra Hospital for Children, for arranging and interpreting the pulmonary function tests. The cooperation of Dr. J. G. Morris, Director of the Department of Nuclear Medicine, Royal Prince Alfred Hospital, for making his staff and equipment available is gratefully recognized. The cooperation of the staff members of the Radiology Department of the Royal Alexandra Hospital for Children is also much appreciated. REFERENCES
1. Chatrath RR, El Shafie M, Jones RS: Fate of hypoplastic lungs after repair of congenital diaphragmatic hernia, Arch Dis Child 46:633, 1971 2. Snyder WH, Greaney EM: Congenital diaphragmatic hernia: 77 consecutive cases. Surgery 57:576, 1965 3. Carter REB, Waterston D J, Aberdeen E~ Hernia and eventration of the diaphragm in childhood. Lancet 1:659, 1962
4. Allen MS, Thomson SA: Congenital diaphragmatic hernia in children under one year of age: A 24 year review. J Pediatr Surg 1:157, 1966 5. Graivier L: Congenital diaphragmatic hernia. South Med J 67:59, 1974 6. Raphaely RC, Downes J J: Congenital diaphragmatic hernia: Prediction of survival. J Pediatr Surg 8:815, 1973