CONGENITAL DIAPHRAGMATIC HERNIA: A STUDY OF MORTALITY FACTORS R. J. Fitzgerald
Children's Research Centre, Our Lady's Hospital for Sick Children, Crumlin, DubEn 12. Summary /heTtPIS investigation sought to identify reasons for a high mortality (70 per cent) in 27 patients with congenital diaphragmatic hernia. The series has a higher incidence o1 extrapulmonary contributory causes of death than most published series from surgical units. These causes are analysed. Lung pathology, which accounted for the remaining 12 deaths, is reviewed and classified. The average age o1 the patients, who subsequent~y died, on arrival at our unit was five hours. Only two patients were older than 24 hours on ardvak This rapid tral~sfar of patients has meant that more of the kind of patients, which in some maternity units with less geographical proximity to their surgical unit would have died, are reaching us alive. The earlier the diagnosis is made the worse the prognosis Some of the recent literature concerning areas where improvement in results might be possible is discussed. Inlredur There has been a considerable variation in reported mortality in patients with diaphragmatic hernia (Adelman and Benson, 1976) The figures are not comparable since there are many variables particularly of referral from one unit to another. We were aware of a high mortality in our patients with diaphragmatic hernia and decided to investigate this and see if we could establish the reasons for our results.
Materials ~:nd Methods Our study is confined to those patients with a hernia o1 abdominal contents
through the cupola of the diaphragm due to the abnormally peTs]sting pleuroperitoneal canal. It is often named after Bochdalek (1848) although it may be more accurate to Ll~e the term 'congenital posterolateral diaphragmatic hernia' (White and Suzuki, 1972), Eeventration of the diaphragm, Morgagni hernia and hiatus hernia of all kinds ere excluded. The case notes of those patients presenting from 1968 to 1975 Were carefully scrutinised and supplemented by information from the referring maternity units. In addition to noting iqonpulmQnary abnormalities contributory to death special attention was paid to the lung pathology.
Results Of the 27 patients referred with Bochdalek hernia there were 15 males. Twenty five were situated in the left hemidiaphragm and two on the right. The mean weight of 17 patients was 3.19 kgm. Qne w~s over sfx months and was not therefore included il3 this calculation. Nineteen patients are d e a d All the children presented with severe respiratory distress. The average oxygen tension of ~rteria[ blood taken on arrival was 32 rnnrn. Hg. The average time from birth to arrival at our unit was five hours for these that died, while for the survivors the figure was 19 hours. The child that oresented at six months was excluded in establishing this figure and this patient and one other were the only ones to arrive older than 24 hours of age. Both of these survived It has been the policy of our unit in dealing with those babies in acute distress to operate immediately with a
CONGENITAL DIAPHRAGMATrC HERNIA m i n i m u m of preoperative preparation but c o n t i n u i n g resuscitation during surgery. it an endotracheal tube was not in situ on arrival t h o r one was passed without de~ay and positive pressure ventilation c o m m e n c e d immediately. A nasogastric tube was passed and intrav e n o u s therapy for metabolic acidosis, based on base excess estimations given. Two patients dfed iust after arrival at our unit.
Surgery Twenty-five out of 1he 27 patients reachee surgery but one of these died at the time el incision. An u p p e r abdominal transverse incision was used to approach the hernia. Four required a teflon or silastic sheet prosthesis to repair a large defect and all of these d i e d Nine patients had chest drains placed bi[aterally at the c o m p l e t i o n of surgery. One patient who ingiaHy had a drain placed only on the side of the hernia developed a contlalaferal lension p n e u m o t h o r a x but survived following l h e pi~ompt insertion of a drain, Three babies had gastrestomies in a bid to limit pressure on the diaphragm; all of these died. Of the 23 patients that left the operatina theatr~ alive, only two were not m e c h a n i c a l l y ventilated. A n y patient who require'd v e n t i l a t i c r for more than six days died and the m a x i m u m period was 16 d a y s T h e eight survivors have been fol !owed ub for a mean period of 30 weeks p~tope~'atively. Malrotation of the bowel is c o m m o n with B o c h d a ! e k hernia and in two s~rvivors Ladd's procedure was carried out at the initial operation. A M e c k e r s d i v e r t i c u l u m was f o u n d in one patient. A cervical m e n i n g o c e l e and chest cage deformity were present in one patient while oesophageal reflux was a m i n o r problem in another. T h e 19 patients who died were carefully analysed, since all had full pcst nlortem e x a m i n a t i c n , a n d were placed in o n e of two g r o u p s Significal'd nonp u l m o n a r y abnormalities were identified
281
in seven a n d these were placed in the first group (Table I). Of these seven, the three cardiac lesions were certainly significant in the cause of death. T h e c h i d with both atrial and v e n t d c u l a r defects also had hyaline m e m b r a n e disease ( H M D ) . Volvulus, with bowel strangulation was the cause of death in one TABLF 1 Exlrapulmonafy contributory causes of dealh Gioup 1. Tralqsposition of great vessels (PDA) Hypoplaslic left heart and patent ductus a[teriosus Atrial and ventricular sepia! defect Vol~ulus of bowel (comol~cation of malrotation) Cornelia de Lange syndrome Down's syndrome Prematurdy (34 weeks gestation) Total
1 1 1 1 1 1 1 ~-
p a t i e r t The baby with the Corne]ia de ! ange syndrome and the premature baby both had hyaline m e m b r a n e disease. T i e patient with Down's syndrome had a precipitate delivery and the mother had previously had an antepartum haemo r r h a g e Histology of this baby's lungs showed inhalation of amniotic contents ned haemorrhage. T h e remaining !2 patients were placed ill a second group dying of p u l m o n a r y causes (Table g ) Analysis of this group was d i f f i c u l t The p u l m o n a r y pathology was reviewed by a pathologist without the know~edge of the survival time of the c h i l d r e n He was able to state that the first six patients in Table II died shortly ~fter birth. The f i n d i n g s were of p u l m o n a r y hypog a s i a with ate[ectasis c o m p l i c a t e d by h a e m o r r h a g e The h a e m o r r h a g e was probably d u e to a n o x i a but may have in part been due to b a r o t r a u m a from mechanicaI ventgatiom These six children, who we classified as dying of the
V"
• Artificial ruplw€ of membranes.
'r Spontaneous rupture
(Labour4 hrs)
SilasliC glall 14hrs
Clf membranes
V"
V"
Ter/on graft
A.R.O.M.
3.07
"
12
16 days
4 days
Post mature 42/40 induced 39/40 S.R.O.M.t Post mature
y"
Died iust after arrival
1',
Silasllc graft
Yo,
V"
y"
y"
Died just alter arrival
3.92
'Smaff'
10
2 days
1st few
",
8hrs
3 days
1 day
10hrs
1-}days
No
y"
Surgery
Forceps FuU-term
Forceps Full·term poE toxaemia Hydramnios A.R.O,M.· 39/40 Forceps Maternity Hasp. unknown
Full-time preg. Mild hypertension LSC secUM 36/40 Forceps Full"lerm
11hrs
Hydrrs
Survival lime
Sigr,jficant obstetric hisloly
2.7D
'Well developed'
Not known
Not known
3,B6
Not kMwn
3.70
a,;?B
Not known
"
Weighl
TABLE: II Group 2.
Pulmonary infection
PUfm0
Children's Research Centre, Our Lady's Hospital for Sick Children, Crumlin, DubEn 12. Summary /heTtPIS investigation sought to identify reasons for a high mortality (70 per cent) in 27 patients with congenital diaphragmatic hernia. The series has a higher incidence o1 extrapulmonary contributory causes of death than most published series from surgical units. These causes are analysed. Lung pathology, which accounted for the remaining 12 deaths, is reviewed and classified. The average age o1 the patients, who subsequent~y died, on arrival at our unit was five hours. Only two patients were older than 24 hours on ardvak This rapid tral~sfar of patients has meant that more of the kind of patients, which in some maternity units with less geographical proximity to their surgical unit would have died, are reaching us alive. The earlier the diagnosis is made the worse the prognosis Some of the recent literature concerning areas where improvement in results might be possible is discussed. Inlredur There has been a considerable variation in reported mortality in patients with diaphragmatic hernia (Adelman and Benson, 1976) The figures are not comparable since there are many variables particularly of referral from one unit to another. We were aware of a high mortality in our patients with diaphragmatic hernia and decided to investigate this and see if we could establish the reasons for our results.
Materials ~:nd Methods Our study is confined to those patients with a hernia o1 abdominal contents
through the cupola of the diaphragm due to the abnormally peTs]sting pleuroperitoneal canal. It is often named after Bochdalek (1848) although it may be more accurate to Ll~e the term 'congenital posterolateral diaphragmatic hernia' (White and Suzuki, 1972), Eeventration of the diaphragm, Morgagni hernia and hiatus hernia of all kinds ere excluded. The case notes of those patients presenting from 1968 to 1975 Were carefully scrutinised and supplemented by information from the referring maternity units. In addition to noting iqonpulmQnary abnormalities contributory to death special attention was paid to the lung pathology.
Results Of the 27 patients referred with Bochdalek hernia there were 15 males. Twenty five were situated in the left hemidiaphragm and two on the right. The mean weight of 17 patients was 3.19 kgm. Qne w~s over sfx months and was not therefore included il3 this calculation. Nineteen patients are d e a d All the children presented with severe respiratory distress. The average oxygen tension of ~rteria[ blood taken on arrival was 32 rnnrn. Hg. The average time from birth to arrival at our unit was five hours for these that died, while for the survivors the figure was 19 hours. The child that oresented at six months was excluded in establishing this figure and this patient and one other were the only ones to arrive older than 24 hours of age. Both of these survived It has been the policy of our unit in dealing with those babies in acute distress to operate immediately with a
CONGENITAL DIAPHRAGMATrC HERNIA m i n i m u m of preoperative preparation but c o n t i n u i n g resuscitation during surgery. it an endotracheal tube was not in situ on arrival t h o r one was passed without de~ay and positive pressure ventilation c o m m e n c e d immediately. A nasogastric tube was passed and intrav e n o u s therapy for metabolic acidosis, based on base excess estimations given. Two patients dfed iust after arrival at our unit.
Surgery Twenty-five out of 1he 27 patients reachee surgery but one of these died at the time el incision. An u p p e r abdominal transverse incision was used to approach the hernia. Four required a teflon or silastic sheet prosthesis to repair a large defect and all of these d i e d Nine patients had chest drains placed bi[aterally at the c o m p l e t i o n of surgery. One patient who ingiaHy had a drain placed only on the side of the hernia developed a contlalaferal lension p n e u m o t h o r a x but survived following l h e pi~ompt insertion of a drain, Three babies had gastrestomies in a bid to limit pressure on the diaphragm; all of these died. Of the 23 patients that left the operatina theatr~ alive, only two were not m e c h a n i c a l l y ventilated. A n y patient who require'd v e n t i l a t i c r for more than six days died and the m a x i m u m period was 16 d a y s T h e eight survivors have been fol !owed ub for a mean period of 30 weeks p~tope~'atively. Malrotation of the bowel is c o m m o n with B o c h d a ! e k hernia and in two s~rvivors Ladd's procedure was carried out at the initial operation. A M e c k e r s d i v e r t i c u l u m was f o u n d in one patient. A cervical m e n i n g o c e l e and chest cage deformity were present in one patient while oesophageal reflux was a m i n o r problem in another. T h e 19 patients who died were carefully analysed, since all had full pcst nlortem e x a m i n a t i c n , a n d were placed in o n e of two g r o u p s Significal'd nonp u l m o n a r y abnormalities were identified
281
in seven a n d these were placed in the first group (Table I). Of these seven, the three cardiac lesions were certainly significant in the cause of death. T h e c h i d with both atrial and v e n t d c u l a r defects also had hyaline m e m b r a n e disease ( H M D ) . Volvulus, with bowel strangulation was the cause of death in one TABLF 1 Exlrapulmonafy contributory causes of dealh Gioup 1. Tralqsposition of great vessels (PDA) Hypoplaslic left heart and patent ductus a[teriosus Atrial and ventricular sepia! defect Vol~ulus of bowel (comol~cation of malrotation) Cornelia de Lange syndrome Down's syndrome Prematurdy (34 weeks gestation) Total
1 1 1 1 1 1 1 ~-
p a t i e r t The baby with the Corne]ia de ! ange syndrome and the premature baby both had hyaline m e m b r a n e disease. T i e patient with Down's syndrome had a precipitate delivery and the mother had previously had an antepartum haemo r r h a g e Histology of this baby's lungs showed inhalation of amniotic contents ned haemorrhage. T h e remaining !2 patients were placed ill a second group dying of p u l m o n a r y causes (Table g ) Analysis of this group was d i f f i c u l t The p u l m o n a r y pathology was reviewed by a pathologist without the know~edge of the survival time of the c h i l d r e n He was able to state that the first six patients in Table II died shortly ~fter birth. The f i n d i n g s were of p u l m o n a r y hypog a s i a with ate[ectasis c o m p l i c a t e d by h a e m o r r h a g e The h a e m o r r h a g e was probably d u e to a n o x i a but may have in part been due to b a r o t r a u m a from mechanicaI ventgatiom These six children, who we classified as dying of the
V"
• Artificial ruplw€ of membranes.
'r Spontaneous rupture
(Labour4 hrs)
SilasliC glall 14hrs
Clf membranes
V"
V"
Ter/on graft
A.R.O.M.
3.07
"
12
16 days
4 days
Post mature 42/40 induced 39/40 S.R.O.M.t Post mature
y"
Died iust after arrival
1',
Silasllc graft
Yo,
V"
y"
y"
Died just alter arrival
3.92
'Smaff'
10
2 days
1st few
",
8hrs
3 days
1 day
10hrs
1-}days
No
y"
Surgery
Forceps FuU-term
Forceps Full·term poE toxaemia Hydramnios A.R.O,M.· 39/40 Forceps Maternity Hasp. unknown
Full-time preg. Mild hypertension LSC secUM 36/40 Forceps Full"lerm
11hrs
Hydrrs
Survival lime
Sigr,jficant obstetric hisloly
2.7D
'Well developed'
Not known
Not known
3,B6
Not kMwn
3.70
a,;?B
Not known
"
Weighl
TABLE: II Group 2.
Pulmonary infection
PUfm0
