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Incarcerated congenital diaphragmatic hernia
Incarcerated Congenital Diaphragmatic Hernia BZRNARD J. FICARRA,M.D., Rosly n Helgbts, New York °.
Front The Department oJ Surgery, RosIyn Park Hospital, Roslyn Helgbts, New York. T has been truly said that one may profit more by failure than by success. In surgery .we are more boastful of our accomplishments and try to hide our poor results, yet both are important if the failure prevents a similar error, or in some way teaches us a lesson. I should like to report a case in which operation was successful. However, the end result must be labelled a failure because the patient died.
occasions Mien he vomited part of his feeding. This was overcome when the infant was placed in a high Fowler's position. The parents were informed of the seriousness of the situation and that surgery was necessary. After two days of procrastination the parents consented to surgery. An abdominal operation was planned for this infant in order to ascertain the presence of any additional anomalies which might be corrected surgically. The thoracic disorder was shown by x-ray, and it was believed that CASE REPORT the diaphragmatic defect could be repaired abdominally. This plan was carried out with On September 9, x954, a healthy twentygreat difficulty because of tile persistent nine year old housewife (gravida n, para i) cyanosis, which continued even after the gave birth to' a 9 pound, 2 ounce baby boy, anesthetist had passed an intratracheal tube. who was normal by all external appearances However, when the abdomen was entered the except for hypospadias. The baby cried immediately and spontaneously after delivery. How- • incarcerated viable small intestine was liberated and returned to the peritoneal cavity. ever, respirations were rapid and tile infant was slightly cyanotic. Because of the cyanosis : Only one-third o f the left diaphragm was he was placed in an incubator: constant admin- , present due to a congenital absence of tissue in the central area. There was no other intraistration of oxygen was necessary. Thorough abdominal anomaly or disorder present. As far physical examination revealed a displacement as could be visualized or palpated, the left lung of the heart into the right chest with an absence was collapsed in tile apex of the left thorax. of breath sounds in the left chest. "Rumbles" Tile heart was completely displaced into the were heard in the left thorax and an x-ray was right thorax with partial collapse of the right taken. (Fig. x.) lung. At this point the anesthetist reported The x-ray revealed that the entire left hemithat tile infant was in distress; the heart fibrilthorax was filled with coils of intestine. No lated. Intravenous novocain® was adminislung tissue was evident in the left chest. The tered. The surgeon displaced the spleen into heart and mediastinum were shifted into the right chest. The diagnosis was incarcerated the absent diaphragmatic space and forced i t snugly so that it was fixed therein. It was Ieft diaphragmatic hernia due to a congenital foolish to believe this would stay in situ. Howdefect. A surgical consultation was obtained and it ever, at the time the major consideration was was agreed that surgery was indicated. It was to keep the patient alive and perhaps operate realized that ahhough the small intestine was again at a later date. The abdomen was closed incarcerated in the thorax, the bowel was not and the infant was returned to his incubator. strangulated. The problem confronting us was A postoperative x-ray taken on September 17, that the infant was constantly cyanotic and I954 (several days after surgery), demonbecame more deeply cyanotic when he was fed. strated " t h e heart and mediastinum to b e His formula was retained except for a few grossly unchanged compared to the previous American Journal of Surgery. Volume 92. September. ,9~6 472
I
Incarcerated Congenital Diaphragmatic Hernia
FIG. I. X-ray taken soon after birth showing coils of small intestine filling the entire left thorax. No lung tissue is visible in the left chest. The heart is.dlsplaced into the right chest. The roentgenologlst's diagnosis is that of an incarcerated congenital diaphragmatic hernia. The arrow marks the cardiac displacement. Note that there is non-vlsuallzatlon of the left diaphragm due to its congenital absence and the presence of the distended bowel. examination (displaced into the right hemithorax). T h e left lung is aerated and has expanded to the left fourth rib posteriorly. A solid intra-abdominal organ (spleen) lies between the aerated left lung and the coils of intestine which lie in tile left upper quadrant of the abdomen." (Fig. a.) This situation gave us a sense of false hope and security. T h e infant was no longer deeply cyanotic even when he was taking his formula. He no longer needed continuous oxygen. T h e infant was removed from the incubator for long intervals of time. T h e nurse would feed him outside the incubator on m a n y occasions, without signs of distress. On September 27, I954, the infant suddenly became deeply cyanotic. Our fear was t h a t the small bowel had again herniated into the thorax and t h a t the spleen had fallen, and no longer acted as a "plug." An x-ray was taken confirming t h e suspicion. Multiple coils of small bowel had re-entered the thorax. (Fig. 3.) The father and mother were informed of this 473
FIG. 2. X-ray taken after the first operation. This film reveals the heart and mediastlnum to be unchanged as compared to the previous x-ray examination, i.e., displacement into the right hemlthorax. The left lung is aerated and has expanded to the fourth rib. A solid intra-abdominal organ (spleen) lies between the aerated left lung and the coils of intestine which are in the left upper quadrant of the abdomen. The top arrow marks the expanded lung tissue. The center arrow indicates the solid organ between the lung and the intestine. The lowest arrow points to the small bowel idefftified by the gas in the lumen of the intestine. sudden change for the worse. T h e y f e i t t h a t if the boy were going to die, they would want him to d i e at home, this despite the patient's need for oxygen which was emphasized. T h e parents were allowed to have one of the hospital incubators, in which the infant was taken home. On December i5, I 9 5 4 , the parents were alarmed when the infant became deeply cyanotic during the course of feeding. (The baby had been home since September.) At the suggestion of the family doctor, t h e infant was returned to the hospital and t h e surgical department was again consulted. Another x-ray was taken which was similar in appearance to t h a t described previously showing an incarcerated diaphragmatic hernia. (Fig. 3.) T h e parents were again advised as to the seriousness of the situation. T h e surgical department agreed to reoperate. T h e r e w a s no enthusiasm for this operation as the infant was cyanotic even under constant oxygen.
Ficarra
FIG. 3- X-ray taken sixteen days following surgery. Muhlple coils of small intestine have re-entered the left hemithorax, indicating that the spleen has slipped back into the abdomen and that the herniation has recurred. Prior to operation alI conceivable consultations were obtained. All agreed that tile prognosis was grave and that surgical intervention was imperative even in t h e face of an operative mortality. Surgery was performed through tile thorax on December I8, x954. Tile left chest was filled with small bowel which was redeposited into the peritoneal cavity. The missing dlaphragm was again visualized. It could not be approximated because of excessive tension on the sutures. The diaphragmatic leaves were divided in their anteroposterior directions forming sliding grafts. This allowed an approxi~ mation of the diaphragm with black silk and thus the hernial hiatus was closed. During the course of surgery, cardiac arrest occurred for ten minutes. Manual resuscitation returned the heart beat. Fibrillation was controlled via intracardiac novocain solution. A catheter was placed in the left chest and double-bottle, water-sealed suction applied. The left lung did not expand completely and the heart and medlastinum remained in the right chest. The child was placed in the in474
Fx~. 4- Postoperative x-ray (second operation). This shows the gastrointestinal tract to be below the diaphragm. The diaphragm itself has a normal appearance. Pneumothorax with a drainage tube in situ is noted. The left lung is collapsed70 per cent. The heart and right lung are unchanged as compared to previous x-rays. eubator. In spite of surgery, intravenous fluids, oxygen and exeelIent nursing care, the baby died on the first postoperative day. (Fig. 4.) The autopsy report is summarized herewith (only the pertinent positive findings are recorded). There was a moderate amount of postmortem lividity noted in the dependent portions of the body. This lividity Was most marked in tile cervical region and the head. When the abdomen was opened there was only about I co. of hemorrhagic fluid in the peritoneal cavity. There were several blaek silk sutures in the Ieft Ieaflet of the diaphragm which closed the diaphragm completely. The stomach was adherent to the flyer. The small intestines were collapsed and the eolon slightly dilated in relative comparison. The colonic distention was due to feces and gas. All of the intestines were in the peritoneal cavity. The mesentery of the small bowel was abnormally long. The cecum was freely mobile and was found in the umbilical region. There were a few intra-abdominal adhesions among
Incarcerated Congenital D i a p h r a g m a t i c Hernia the loops of smalI boweI. Tile spleen was in its usuaI location and showed only a slight congestion when sectioned. The other abdominal and retroperitoneaI organs were normaI. The thoracic organs presented the following pathoIogy. Tile heart was a classic pleture of dextrocardia, but otherwise was normal. In the left pleural cavity about 5 ee. of hemorrhagic fluid were found. There was no fluid in the right pleural cavity. The heart was in tlle right chest with the thymus gland overlying the superior aspect of the heart. Tile right lung was retracted posteriorly. The left chest was empty except for the fluid previously mentioned and the left lung which was found in the upper posterior portion of the pleural cavity with the apex rotated inwardly. The trachea and bronchi contained grayish brown, mueoid material. The right middle Iobe was cmphysematous. The upper and lower lobes showed patchy areas of congestion. The left lung was heavier than normal lung tissue should be. The upper lobe was tan in color with visible decreased aeration. The Ieft lower Iobe was similar in type but demonstrated areas o f congestion. Sections from all five lobes floated when placed in water. Microscopic study of the lung tissue (right middle lobe) revealed marked emphysema. The remaining four lobes showed partial to eompIete atelectasis in the major portions of tile Iung parencyma. When this was not revealed, the remaining Iung tissue consisted of normal or slightly emphysematous alveoli. Histologic studies of tile other organs demonstrated nothing significant. The cause of death was attributed to puhnonary insufficiency due to congenital abnormalities of the lung. COMMENTS
After undergoing two surgical procedures the infant died. The details of the operations and the autopsy findings have been presented. The problem to be solved involved the absence of adequate diaphragmatic tissue and how to manage it. Our efforts of using the spleen as .a tampon was not expected to "work," but it was so used because of the urgent necessity of ending the operation in order to keep the infant alive. At the second operation the diaphragmatic defect was closed, but the patient died. Apparently tile lung tissue was not adequately expanded to maintain life. It has been suggested by European authors that large diaphragmatic defects could be repaired with preserved fascial grafts? This suggestion is worthy of note and perhaps a graft of this type would have expedited the surgical procedure. Be that as it may, the belief of almost everyone concerned was that the situation was hopeless from the beginning and that surgery merely delayed the inevitable outcome. Perhaps, in retrospect, it would have been wiser to have performed the first procedure via tile thorax and explore tile abdomen through the thoracic incision since the diaphragm was an avenue of approach due to the congenital disorder. Then, with a fascial graft, the absent diaphragmatic tissue could have been replaced. Surgically speaking, this would be sound, but the problem of cardiac displacement and the congenital partial to complete atelectasis of four pulmonary lobes remained. Perhaps we should evaluate our limitations and realize that we can only take tile ordinary means of preserving life and that extraordinary means are usually futile. SUMMARY
Tile complete life history of a three month old baby boy is presented for critical review. This infant was born with a congenital absence of almost two-thirds of the left diaphragm, which predisposed to herniation and incarceration of the entire small bowel in. the left thoracic cavity. When this occurred the child was cyanotic and required continuous oxygen. At the time of birth the left lung was completely collapsed, the heart was displaced into the right thorax and the right lung was only partially aerated, primarily because of congenital atelectasis and secondarily because of the cardiac dispIacement and compression.
i. The life history of a three month old baby boy with a congenital diaphragmatic hernia is presented. 2. The salient features of the problem were incarceration of the small bowel, absence of diaphragmatic tissue, cardiac displacement and pulmonary collapse of four lobes. 3. After two attempts at surgical repair of the hernia, the baby died. REFERENCE I. ESPOSITO,G., INFr.ANZl,A. and Rmclo, A. Repair of large diaphragmatic tissue with preserved fascia grafts. Gior. ital. cbir., Io: 8o4-8x6, 1954.
475
Front The Department oJ Surgery, RosIyn Park Hospital, Roslyn Helgbts, New York. T has been truly said that one may profit more by failure than by success. In surgery .we are more boastful of our accomplishments and try to hide our poor results, yet both are important if the failure prevents a similar error, or in some way teaches us a lesson. I should like to report a case in which operation was successful. However, the end result must be labelled a failure because the patient died.
occasions Mien he vomited part of his feeding. This was overcome when the infant was placed in a high Fowler's position. The parents were informed of the seriousness of the situation and that surgery was necessary. After two days of procrastination the parents consented to surgery. An abdominal operation was planned for this infant in order to ascertain the presence of any additional anomalies which might be corrected surgically. The thoracic disorder was shown by x-ray, and it was believed that CASE REPORT the diaphragmatic defect could be repaired abdominally. This plan was carried out with On September 9, x954, a healthy twentygreat difficulty because of tile persistent nine year old housewife (gravida n, para i) cyanosis, which continued even after the gave birth to' a 9 pound, 2 ounce baby boy, anesthetist had passed an intratracheal tube. who was normal by all external appearances However, when the abdomen was entered the except for hypospadias. The baby cried immediately and spontaneously after delivery. How- • incarcerated viable small intestine was liberated and returned to the peritoneal cavity. ever, respirations were rapid and tile infant was slightly cyanotic. Because of the cyanosis : Only one-third o f the left diaphragm was he was placed in an incubator: constant admin- , present due to a congenital absence of tissue in the central area. There was no other intraistration of oxygen was necessary. Thorough abdominal anomaly or disorder present. As far physical examination revealed a displacement as could be visualized or palpated, the left lung of the heart into the right chest with an absence was collapsed in tile apex of the left thorax. of breath sounds in the left chest. "Rumbles" Tile heart was completely displaced into the were heard in the left thorax and an x-ray was right thorax with partial collapse of the right taken. (Fig. x.) lung. At this point the anesthetist reported The x-ray revealed that the entire left hemithat tile infant was in distress; the heart fibrilthorax was filled with coils of intestine. No lated. Intravenous novocain® was adminislung tissue was evident in the left chest. The tered. The surgeon displaced the spleen into heart and mediastinum were shifted into the right chest. The diagnosis was incarcerated the absent diaphragmatic space and forced i t snugly so that it was fixed therein. It was Ieft diaphragmatic hernia due to a congenital foolish to believe this would stay in situ. Howdefect. A surgical consultation was obtained and it ever, at the time the major consideration was was agreed that surgery was indicated. It was to keep the patient alive and perhaps operate realized that ahhough the small intestine was again at a later date. The abdomen was closed incarcerated in the thorax, the bowel was not and the infant was returned to his incubator. strangulated. The problem confronting us was A postoperative x-ray taken on September 17, that the infant was constantly cyanotic and I954 (several days after surgery), demonbecame more deeply cyanotic when he was fed. strated " t h e heart and mediastinum to b e His formula was retained except for a few grossly unchanged compared to the previous American Journal of Surgery. Volume 92. September. ,9~6 472
I
Incarcerated Congenital Diaphragmatic Hernia
FIG. I. X-ray taken soon after birth showing coils of small intestine filling the entire left thorax. No lung tissue is visible in the left chest. The heart is.dlsplaced into the right chest. The roentgenologlst's diagnosis is that of an incarcerated congenital diaphragmatic hernia. The arrow marks the cardiac displacement. Note that there is non-vlsuallzatlon of the left diaphragm due to its congenital absence and the presence of the distended bowel. examination (displaced into the right hemithorax). T h e left lung is aerated and has expanded to the left fourth rib posteriorly. A solid intra-abdominal organ (spleen) lies between the aerated left lung and the coils of intestine which lie in tile left upper quadrant of the abdomen." (Fig. a.) This situation gave us a sense of false hope and security. T h e infant was no longer deeply cyanotic even when he was taking his formula. He no longer needed continuous oxygen. T h e infant was removed from the incubator for long intervals of time. T h e nurse would feed him outside the incubator on m a n y occasions, without signs of distress. On September 27, I954, the infant suddenly became deeply cyanotic. Our fear was t h a t the small bowel had again herniated into the thorax and t h a t the spleen had fallen, and no longer acted as a "plug." An x-ray was taken confirming t h e suspicion. Multiple coils of small bowel had re-entered the thorax. (Fig. 3.) The father and mother were informed of this 473
FIG. 2. X-ray taken after the first operation. This film reveals the heart and mediastlnum to be unchanged as compared to the previous x-ray examination, i.e., displacement into the right hemlthorax. The left lung is aerated and has expanded to the fourth rib. A solid intra-abdominal organ (spleen) lies between the aerated left lung and the coils of intestine which are in the left upper quadrant of the abdomen. The top arrow marks the expanded lung tissue. The center arrow indicates the solid organ between the lung and the intestine. The lowest arrow points to the small bowel idefftified by the gas in the lumen of the intestine. sudden change for the worse. T h e y f e i t t h a t if the boy were going to die, they would want him to d i e at home, this despite the patient's need for oxygen which was emphasized. T h e parents were allowed to have one of the hospital incubators, in which the infant was taken home. On December i5, I 9 5 4 , the parents were alarmed when the infant became deeply cyanotic during the course of feeding. (The baby had been home since September.) At the suggestion of the family doctor, t h e infant was returned to the hospital and t h e surgical department was again consulted. Another x-ray was taken which was similar in appearance to t h a t described previously showing an incarcerated diaphragmatic hernia. (Fig. 3.) T h e parents were again advised as to the seriousness of the situation. T h e surgical department agreed to reoperate. T h e r e w a s no enthusiasm for this operation as the infant was cyanotic even under constant oxygen.
Ficarra
FIG. 3- X-ray taken sixteen days following surgery. Muhlple coils of small intestine have re-entered the left hemithorax, indicating that the spleen has slipped back into the abdomen and that the herniation has recurred. Prior to operation alI conceivable consultations were obtained. All agreed that tile prognosis was grave and that surgical intervention was imperative even in t h e face of an operative mortality. Surgery was performed through tile thorax on December I8, x954. Tile left chest was filled with small bowel which was redeposited into the peritoneal cavity. The missing dlaphragm was again visualized. It could not be approximated because of excessive tension on the sutures. The diaphragmatic leaves were divided in their anteroposterior directions forming sliding grafts. This allowed an approxi~ mation of the diaphragm with black silk and thus the hernial hiatus was closed. During the course of surgery, cardiac arrest occurred for ten minutes. Manual resuscitation returned the heart beat. Fibrillation was controlled via intracardiac novocain solution. A catheter was placed in the left chest and double-bottle, water-sealed suction applied. The left lung did not expand completely and the heart and medlastinum remained in the right chest. The child was placed in the in474
Fx~. 4- Postoperative x-ray (second operation). This shows the gastrointestinal tract to be below the diaphragm. The diaphragm itself has a normal appearance. Pneumothorax with a drainage tube in situ is noted. The left lung is collapsed70 per cent. The heart and right lung are unchanged as compared to previous x-rays. eubator. In spite of surgery, intravenous fluids, oxygen and exeelIent nursing care, the baby died on the first postoperative day. (Fig. 4.) The autopsy report is summarized herewith (only the pertinent positive findings are recorded). There was a moderate amount of postmortem lividity noted in the dependent portions of the body. This lividity Was most marked in tile cervical region and the head. When the abdomen was opened there was only about I co. of hemorrhagic fluid in the peritoneal cavity. There were several blaek silk sutures in the Ieft Ieaflet of the diaphragm which closed the diaphragm completely. The stomach was adherent to the flyer. The small intestines were collapsed and the eolon slightly dilated in relative comparison. The colonic distention was due to feces and gas. All of the intestines were in the peritoneal cavity. The mesentery of the small bowel was abnormally long. The cecum was freely mobile and was found in the umbilical region. There were a few intra-abdominal adhesions among
Incarcerated Congenital D i a p h r a g m a t i c Hernia the loops of smalI boweI. Tile spleen was in its usuaI location and showed only a slight congestion when sectioned. The other abdominal and retroperitoneaI organs were normaI. The thoracic organs presented the following pathoIogy. Tile heart was a classic pleture of dextrocardia, but otherwise was normal. In the left pleural cavity about 5 ee. of hemorrhagic fluid were found. There was no fluid in the right pleural cavity. The heart was in tlle right chest with the thymus gland overlying the superior aspect of the heart. Tile right lung was retracted posteriorly. The left chest was empty except for the fluid previously mentioned and the left lung which was found in the upper posterior portion of the pleural cavity with the apex rotated inwardly. The trachea and bronchi contained grayish brown, mueoid material. The right middle Iobe was cmphysematous. The upper and lower lobes showed patchy areas of congestion. The left lung was heavier than normal lung tissue should be. The upper lobe was tan in color with visible decreased aeration. The Ieft lower Iobe was similar in type but demonstrated areas o f congestion. Sections from all five lobes floated when placed in water. Microscopic study of the lung tissue (right middle lobe) revealed marked emphysema. The remaining four lobes showed partial to eompIete atelectasis in the major portions of tile Iung parencyma. When this was not revealed, the remaining Iung tissue consisted of normal or slightly emphysematous alveoli. Histologic studies of tile other organs demonstrated nothing significant. The cause of death was attributed to puhnonary insufficiency due to congenital abnormalities of the lung. COMMENTS
After undergoing two surgical procedures the infant died. The details of the operations and the autopsy findings have been presented. The problem to be solved involved the absence of adequate diaphragmatic tissue and how to manage it. Our efforts of using the spleen as .a tampon was not expected to "work," but it was so used because of the urgent necessity of ending the operation in order to keep the infant alive. At the second operation the diaphragmatic defect was closed, but the patient died. Apparently tile lung tissue was not adequately expanded to maintain life. It has been suggested by European authors that large diaphragmatic defects could be repaired with preserved fascial grafts? This suggestion is worthy of note and perhaps a graft of this type would have expedited the surgical procedure. Be that as it may, the belief of almost everyone concerned was that the situation was hopeless from the beginning and that surgery merely delayed the inevitable outcome. Perhaps, in retrospect, it would have been wiser to have performed the first procedure via tile thorax and explore tile abdomen through the thoracic incision since the diaphragm was an avenue of approach due to the congenital disorder. Then, with a fascial graft, the absent diaphragmatic tissue could have been replaced. Surgically speaking, this would be sound, but the problem of cardiac displacement and the congenital partial to complete atelectasis of four pulmonary lobes remained. Perhaps we should evaluate our limitations and realize that we can only take tile ordinary means of preserving life and that extraordinary means are usually futile. SUMMARY
Tile complete life history of a three month old baby boy is presented for critical review. This infant was born with a congenital absence of almost two-thirds of the left diaphragm, which predisposed to herniation and incarceration of the entire small bowel in. the left thoracic cavity. When this occurred the child was cyanotic and required continuous oxygen. At the time of birth the left lung was completely collapsed, the heart was displaced into the right thorax and the right lung was only partially aerated, primarily because of congenital atelectasis and secondarily because of the cardiac dispIacement and compression.
i. The life history of a three month old baby boy with a congenital diaphragmatic hernia is presented. 2. The salient features of the problem were incarceration of the small bowel, absence of diaphragmatic tissue, cardiac displacement and pulmonary collapse of four lobes. 3. After two attempts at surgical repair of the hernia, the baby died. REFERENCE I. ESPOSITO,G., INFr.ANZl,A. and Rmclo, A. Repair of large diaphragmatic tissue with preserved fascia grafts. Gior. ital. cbir., Io: 8o4-8x6, 1954.
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