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Congenital anomalies of the diaphragm
Congenital Anomalies of the Diaphragm Personal Experience with Thirty-Five Consecutive Cases Franklin J. Harberg, MD, Houston, Texas David Meagher, MD, Houston, Texas Stewart Wetchler, BS, Houston, Texas Frederick Harris, MD,* Houston, Texas
Congenital anomalies of the diaphragm remain a difficult problem in diagnosis and management for those caring for the neonate and infant. An especially high mortality is encountered when symptoms occur during the first 24 hours of life. With the improvement of overall infant care as a result of regional perinatal units and increased availability of neonatal intensive care units, more of these children can be expected to survive long enough to be diagnosed and treated by the neonatologist and the pediatric surgeon. This report describes our experience in the operative management of thirty-five consecutive patients with congenital anomalies of the diaphragm treated during a sixteen year period at the Texas Children’s Hospital in Houston, Texas. Material and Methods All patients with posterolateral diaphragmatic defects (Bochdalek hernia), eventration of the diaphragm, and esophageal hiatal hernia who were operated on by our service from 1959 to 1975 are considered. Of the thirty-five patients, twenty were male and fifteen female, thirty-two were white and three black, and thirty-two were full-term infants and three premature. Virtually all of the patients presented with symptoms of respiratory distress with the exception of three patients with hiatal hernias who presented with vomiting. The diagnosis and management of each type of diaphragmatic anomaly will be considered separately. Bochdalek Hernia. There were twenty-five patients with posterolateral defects. Of these, twenty were left-sided and five were right-sided hernias. At the time of diagnosis, twenty-three were less than 24 hours old, one was 35 hours old, and one was four and a half months old. Only two were premature infants. Respiratory distress was the presenting
From the Department of Surgery, Baylor College of Medicine. and General Surgery Service, Texas Chit&en’s Hospital, Houston, Texas. Reprint requests should be addressed to Franklin J. Harberg, MD, Doctors Center Professional Bldg. 7000 Fannin, Suite 1400. Houston. Texas 77030. Presented af the Twenty-Eighth Annual Meeting of the Southwestern Surgical Congress, Houston, Texas, May 3-6, 1976. * present &dress: C/O 0. G. Rii, 421 Nulh Weber, Aberdeen, South Dakota 5740 1.
Vofuma 132, DecomMw 1976
symptom in all but one patient, and diagnosis was made by x-ray examination. In the remaining patient, the parents insisted on x-ray examination as they had lost a previous child with a diaphragmatic hernia. Other clinical signs were: decreased breath sounds on the involved side (21 patients); scaphoid abdomen (16); cyanosis (13); costosternal retraction (9); and dextrocardia (12 of the 20 with left-sided defects). The only diagnostic problem was in distinguishing hernia or eventration from lung malformation. A small (15 to 20 cc) pneumoperitoneum with upright chest film was used in two such questionable cases. All twenty-five of these patients underwent surgical repair. Four of five right-sided defects were approached thoracically, whereas nineteen of twenty left-sided defects were approached abdominally. Although all of the diaphragmatic hernias were primarily repaired, prosthetic material was used in two patients to allow repair of the defect. Three ventral hernias were electively created, leaving the abdominal muscle wall open with only a skin closure. This decreased pressure on the diaphragmatic repair permitted greater respiratory movement and allowed the abdominal cavity to hold the abdominal viscera. Fourteen of the patients with Bochdalek hernias died postoperatively: of the five with right-sided defects, three died, and of the twenty with left-sided defects, eleven died. Other anomalies were frequently reported at autopsy. Besides hypoplastic lung on the side of the lesion, these included: ventricular septal defect with and without pulmonary atresia; atrial septal defect; patent ductus arteriosus; bronchoalveolar dysplasia; Arnold-Chiara malformation; hydrocephalus; male pseudohermaphroditism; undescended testicle; hypospadias; and fusion of the right lung bud into the liver. Eventration of the Diaphragm. There were seven patients with eventration: three had right-sided involvement, three had left-sided involvement, and in one patient the eventration was bilateral. Three patients were symptomatic within 24 hours of birth, and the others were not symptomatic until seven days to two months of age. All of the patients had respiratory distress, three had cyanosis, three had costosternal retraction, two had scaphoid abdomens, and one had cardiac shift. In all patients chest x-ray examination was performed and confirmatory studies included fluoroscopic examination (2 patients), intrave-
747
Harberg et al
nous pyelogram (1 patient), and barium enema (1 patient). The surgical approach used was primarily thoracic. Of eight repairs (on 7 patients), only one patient (with left eventration) had an abdominal incision. In all cases the diaphragm was plicated with two rows of 3-O silk suture, and an underwater-seal chest tube was used postoperatively. No prosthetic material was required. Only one patient with eventration died, and this patient had multiple congenital anomalies. There have been no recurrences. Esophageal Hiatal Hernia. Three patients with esophageal hiatal hernia were managed. They were 24 hours, two years, and six years of age when symptoms began, and eleven days, four years, and seven years of age, respectively, at the time of surgery. All of these children presented with vomiting. Diagnosis was made by chest x-ray examination and an upper gastrointestinal series. Surgical therapy for the younger two patients was a left thoracic approach and modified Allison procedure, whereas for the older child an abdominal approach was used with
Nissen fundoplication. All three of these patients have done well. Overall mortality was 43 per cent (15 of the 35 patients). Of those who died, fourteen presented with acute respiratory symptoms at less than 24 hours of age, and only one who presented seven days later died postoperatively. A total of twenty-seven patients presented in the first 24 hours after birth, and eight presented at 24 hours to six years. Fourteen (51 per cent) of the first group and one (12.5 per cent) of the second group died. Comments
Development of the diaphragm is complex and all factors and details are as yet not known. Mechanism of hernia development is postulated to involve failure of migration, failure of fusion, or failure of development of precursor elements. Failure of migration by the septum transversum and by the esophageal elements may account for esophageal hiatal hernia. Late or defective fusion of the septum transversum with pleuroperitoneal elements may account for posterolateral and substernal anomalies as may arrest of lateral migration of the septum transversum. Phrenic nerve injury or hypoplasia is postulated to account for eventration of the diaphragm. Diagnosis of diaphragmatic anomalies depends on their recogmtion as a cause of neonatal respiratory distress, especially in a full-term baby. In virtually all patients, chest x-ray examination will be diagnostic. It is important to know whether the defect is actually a hernia or eventration as opposed to lung abnormality, and a small pneumoperitoneum made by injecting 15 to 20 cc of air into the right lower quadrant followed by upright chest film will demonstrate the diaphragm if it is present. Although it
74%
is rarely helpful to know what abdominal organs are in the chest, contrast dye and barium studies can be performed if necessary. The three important aspects of treatment are adequate preparation for surgery, surgery itself, and postoperative care. Management of patients in a neonatal intensive care unit is essential. Mechanical maintenance of respiration and oxygenation, body temperature control, decompression of the gastrointestinal tract, correction of acidosis by the use of NaHCOs, and more recently the attempt to diminish pulmonary hypertension pharmacologically all must precede surgery. Specific surgical approaches have already been discussed. In general, it takes two to four days for the compromised lung to expand after surgery. During the immediate postoperative period the same requirement for intensive supportive management continues. It is apparent from the mortality figures that the challenge lies in patients who have respiratory symptoms at less than 24 hours of age. Early diagnosis and adequate preoperative preparation, appropriate surgery, and continued postoperative care may improve the survival rate for these infants despite their severly compromised condition. Summary
A personal experience of thirty-five consecutive patients with congenital anomalies of the diaphragm is reported. The anomalies included twenty-five Bochdalek hernias, seven diaphragmatic eventrations, and three esophageal hiatal hernias. Except for the patients with esophageal hiatal hernias, virtually all patients presented with respiratory embarrassment, twenty-seven of the thirty-five within 24 hours of birth. An especially high mortality is associated with this early onset of symptoms. The treatment was surgical with a thoracic approach used for right Bochdalek defects and eventrations, and an abdominal approach used for left Bochdalek hernias. The importance of proper preoperative and postoperative management in a well equipped neonatal intensive care unit is emphasized. References 1. McNamara J, Eraklis J, Gross BE: Congenital posteriolateral diaphragmatic hernia in the newborn. J Thorac Cardiovasc Surg 55: 55, 1966. 2. Boles BT, Schiller M, Weinberger M: Improved management of neonates with congenital diaphragmatic hernia. Arch Surg 103: 3441971. 3. Dibbins AW, Wiener ES: Mortality from neonatal diaphragmatic hernia. J PediatrSurg 9: 653; 1974. 4. Dibbins AW: Neonatal diaphragmatic hernia: a physiologic challenge. Am J Surg 131: 406, 1976.
The American
Journalot Surgery
Congenital anomalies of the diaphragm remain a difficult problem in diagnosis and management for those caring for the neonate and infant. An especially high mortality is encountered when symptoms occur during the first 24 hours of life. With the improvement of overall infant care as a result of regional perinatal units and increased availability of neonatal intensive care units, more of these children can be expected to survive long enough to be diagnosed and treated by the neonatologist and the pediatric surgeon. This report describes our experience in the operative management of thirty-five consecutive patients with congenital anomalies of the diaphragm treated during a sixteen year period at the Texas Children’s Hospital in Houston, Texas. Material and Methods All patients with posterolateral diaphragmatic defects (Bochdalek hernia), eventration of the diaphragm, and esophageal hiatal hernia who were operated on by our service from 1959 to 1975 are considered. Of the thirty-five patients, twenty were male and fifteen female, thirty-two were white and three black, and thirty-two were full-term infants and three premature. Virtually all of the patients presented with symptoms of respiratory distress with the exception of three patients with hiatal hernias who presented with vomiting. The diagnosis and management of each type of diaphragmatic anomaly will be considered separately. Bochdalek Hernia. There were twenty-five patients with posterolateral defects. Of these, twenty were left-sided and five were right-sided hernias. At the time of diagnosis, twenty-three were less than 24 hours old, one was 35 hours old, and one was four and a half months old. Only two were premature infants. Respiratory distress was the presenting
From the Department of Surgery, Baylor College of Medicine. and General Surgery Service, Texas Chit&en’s Hospital, Houston, Texas. Reprint requests should be addressed to Franklin J. Harberg, MD, Doctors Center Professional Bldg. 7000 Fannin, Suite 1400. Houston. Texas 77030. Presented af the Twenty-Eighth Annual Meeting of the Southwestern Surgical Congress, Houston, Texas, May 3-6, 1976. * present &dress: C/O 0. G. Rii, 421 Nulh Weber, Aberdeen, South Dakota 5740 1.
Vofuma 132, DecomMw 1976
symptom in all but one patient, and diagnosis was made by x-ray examination. In the remaining patient, the parents insisted on x-ray examination as they had lost a previous child with a diaphragmatic hernia. Other clinical signs were: decreased breath sounds on the involved side (21 patients); scaphoid abdomen (16); cyanosis (13); costosternal retraction (9); and dextrocardia (12 of the 20 with left-sided defects). The only diagnostic problem was in distinguishing hernia or eventration from lung malformation. A small (15 to 20 cc) pneumoperitoneum with upright chest film was used in two such questionable cases. All twenty-five of these patients underwent surgical repair. Four of five right-sided defects were approached thoracically, whereas nineteen of twenty left-sided defects were approached abdominally. Although all of the diaphragmatic hernias were primarily repaired, prosthetic material was used in two patients to allow repair of the defect. Three ventral hernias were electively created, leaving the abdominal muscle wall open with only a skin closure. This decreased pressure on the diaphragmatic repair permitted greater respiratory movement and allowed the abdominal cavity to hold the abdominal viscera. Fourteen of the patients with Bochdalek hernias died postoperatively: of the five with right-sided defects, three died, and of the twenty with left-sided defects, eleven died. Other anomalies were frequently reported at autopsy. Besides hypoplastic lung on the side of the lesion, these included: ventricular septal defect with and without pulmonary atresia; atrial septal defect; patent ductus arteriosus; bronchoalveolar dysplasia; Arnold-Chiara malformation; hydrocephalus; male pseudohermaphroditism; undescended testicle; hypospadias; and fusion of the right lung bud into the liver. Eventration of the Diaphragm. There were seven patients with eventration: three had right-sided involvement, three had left-sided involvement, and in one patient the eventration was bilateral. Three patients were symptomatic within 24 hours of birth, and the others were not symptomatic until seven days to two months of age. All of the patients had respiratory distress, three had cyanosis, three had costosternal retraction, two had scaphoid abdomens, and one had cardiac shift. In all patients chest x-ray examination was performed and confirmatory studies included fluoroscopic examination (2 patients), intrave-
747
Harberg et al
nous pyelogram (1 patient), and barium enema (1 patient). The surgical approach used was primarily thoracic. Of eight repairs (on 7 patients), only one patient (with left eventration) had an abdominal incision. In all cases the diaphragm was plicated with two rows of 3-O silk suture, and an underwater-seal chest tube was used postoperatively. No prosthetic material was required. Only one patient with eventration died, and this patient had multiple congenital anomalies. There have been no recurrences. Esophageal Hiatal Hernia. Three patients with esophageal hiatal hernia were managed. They were 24 hours, two years, and six years of age when symptoms began, and eleven days, four years, and seven years of age, respectively, at the time of surgery. All of these children presented with vomiting. Diagnosis was made by chest x-ray examination and an upper gastrointestinal series. Surgical therapy for the younger two patients was a left thoracic approach and modified Allison procedure, whereas for the older child an abdominal approach was used with
Nissen fundoplication. All three of these patients have done well. Overall mortality was 43 per cent (15 of the 35 patients). Of those who died, fourteen presented with acute respiratory symptoms at less than 24 hours of age, and only one who presented seven days later died postoperatively. A total of twenty-seven patients presented in the first 24 hours after birth, and eight presented at 24 hours to six years. Fourteen (51 per cent) of the first group and one (12.5 per cent) of the second group died. Comments
Development of the diaphragm is complex and all factors and details are as yet not known. Mechanism of hernia development is postulated to involve failure of migration, failure of fusion, or failure of development of precursor elements. Failure of migration by the septum transversum and by the esophageal elements may account for esophageal hiatal hernia. Late or defective fusion of the septum transversum with pleuroperitoneal elements may account for posterolateral and substernal anomalies as may arrest of lateral migration of the septum transversum. Phrenic nerve injury or hypoplasia is postulated to account for eventration of the diaphragm. Diagnosis of diaphragmatic anomalies depends on their recogmtion as a cause of neonatal respiratory distress, especially in a full-term baby. In virtually all patients, chest x-ray examination will be diagnostic. It is important to know whether the defect is actually a hernia or eventration as opposed to lung abnormality, and a small pneumoperitoneum made by injecting 15 to 20 cc of air into the right lower quadrant followed by upright chest film will demonstrate the diaphragm if it is present. Although it
74%
is rarely helpful to know what abdominal organs are in the chest, contrast dye and barium studies can be performed if necessary. The three important aspects of treatment are adequate preparation for surgery, surgery itself, and postoperative care. Management of patients in a neonatal intensive care unit is essential. Mechanical maintenance of respiration and oxygenation, body temperature control, decompression of the gastrointestinal tract, correction of acidosis by the use of NaHCOs, and more recently the attempt to diminish pulmonary hypertension pharmacologically all must precede surgery. Specific surgical approaches have already been discussed. In general, it takes two to four days for the compromised lung to expand after surgery. During the immediate postoperative period the same requirement for intensive supportive management continues. It is apparent from the mortality figures that the challenge lies in patients who have respiratory symptoms at less than 24 hours of age. Early diagnosis and adequate preoperative preparation, appropriate surgery, and continued postoperative care may improve the survival rate for these infants despite their severly compromised condition. Summary
A personal experience of thirty-five consecutive patients with congenital anomalies of the diaphragm is reported. The anomalies included twenty-five Bochdalek hernias, seven diaphragmatic eventrations, and three esophageal hiatal hernias. Except for the patients with esophageal hiatal hernias, virtually all patients presented with respiratory embarrassment, twenty-seven of the thirty-five within 24 hours of birth. An especially high mortality is associated with this early onset of symptoms. The treatment was surgical with a thoracic approach used for right Bochdalek defects and eventrations, and an abdominal approach used for left Bochdalek hernias. The importance of proper preoperative and postoperative management in a well equipped neonatal intensive care unit is emphasized. References 1. McNamara J, Eraklis J, Gross BE: Congenital posteriolateral diaphragmatic hernia in the newborn. J Thorac Cardiovasc Surg 55: 55, 1966. 2. Boles BT, Schiller M, Weinberger M: Improved management of neonates with congenital diaphragmatic hernia. Arch Surg 103: 3441971. 3. Dibbins AW, Wiener ES: Mortality from neonatal diaphragmatic hernia. J PediatrSurg 9: 653; 1974. 4. Dibbins AW: Neonatal diaphragmatic hernia: a physiologic challenge. Am J Surg 131: 406, 1976.
The American
Journalot Surgery