Childhood Congenital Diaphragmatic Hernia of Bochdalek Sanya Adeyemi-Doro, MB, BS St. Louis, Missouri
Congenital pleuroperitoneal diaphragmatic hernia usually presents in neonates. Children manifesting symptoms and signs after the neonatal period constitute a distinct, uncommon, and infrequently recognized group of patients. Occurrence in adults is also uncommon, with fewer than a hundred patients reported in the literature.
Case Report A two-year-old black female patient presented with a two-day history of mild epigastric pain and dyspnea which had been preceded by cough and mild fever for four days. The child had pneumonia at four months of age, but no history of trauma to chest or abdomen, and she was of normal growth and nutritional status. She was moderately dyspneic, with diminished vesicular breath sounds and audible bowel sounds in the left chest anteriorly and posteriorly. The abdomen was scaphoid and rather "empty." Pre-operative plain and meglucamine diatrizoate (Gastrografin) roentgenography (Figures 1 and 2) confirmed the diagnosis of a left diaphragmatic hernia with intrapleural evisceration of the bowels.
Requests for reprints should be addressed to Dr. Sanya Adeyemi-Doro, Department of Surgery, Homer G. Phillips Hospital, St. Louis, Mo 63113.
At laparotomy under general anesthesia and employing a left paramedian skin incision, the omentum, small bowel, caecum, appendix ascending, and the transverse colon were found eviscerated into the left pleural cavity. These were readily reduced after introducing a soft, red rubber catheter to fill the pleural space with air. Hence a thoracoabdominal incision was not needed. A 9 cm diameter congenital left pleuroperitoneal defect was found with a 1 cm posterior lip which was just adequate for a double layer repair with double 'O' nonabsorbable, ethiflex suture. There was no hernial sac, and other congenital abnormality was detected. The anterior abdominal wall was then manually stretched. This enabled the primary closure of the abdominal wall and the accommodation of the herniated viscera without undue tension. Post-operative pulmonary complications, including a left atelectasis, were satisfactorily managed with a volume respirator and assisted positive pressure respiration, maintenance of
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 69, NO. 2, 1977
fluid and electrolyte homeostasis, arterial blood gas monitor, and antibiotics. The left thoracostomy tube which had been connected to a water seal drainage was removed 48 hours postoperatively after satisfactory pulmonary expansion. The gastrostomy tube was removed on the eighth postoperative day. The patient was discharged on the 15th day after admission.
Discussion It is believed that closure of the pleuroperitoneal canal has been present since birth in late-onset cases of diaphragmatic hernia of Bochdalek. Delayed herniation of organs into the chest may be provoked by sudden increases in abdominal tension and in adults, by pregnancy and marked obesity. Eighty-five percent of congenital posterolateral diaphragmatic hernias occur on the left side.' The liver is thought to prevent herniation of viscera through the right pleuroperitoneal canal long enough for the defect to close.2 However, delayed manifestations in older infants occur more often on the right than left. The incidence of right-sided hernia in these older infants and children is twice that of neonates whose symptoms lead to operation in the first three days of life.3 However, in this patient, a relatively large congenital defect was found on the left side. There was no hernia sac, as 97
Figure 2. The same patient 15th post-operative day. Notice residual pneumonitis right-middle lobe.
Figure 1. Late-onset left pleuroperitoneal diaphragmatic hernia in a two-year-old girl At laparotomy, the omentum, small bowel, caecum, appendix, and colon were found eviscerated into the left pleural cavity. There was no hernia sac.
occurs in 85 to 90 percent of reported cases.5 The chest roentgenograms in the older children usually differ from the chest roentgenograms in the neonate with respect only to the lessened amount of mediastinal displacement and volume of herniated viscera.6'7 In this case, the mediastinal shift was obvious with multiple bowels in the chest (Figures 1 and 2). In contrast to the situation in neonates, ingestion of barium is useful for diagnostic confirmation and free from hazard in the absence of intestinal obstruction.3 The age of onset of symptoms is directly related to the success of surgical treatment. In neonates over 48 hours of age, the mortality is less than f ive percent. However, in those symptomatic within the first 48 hours of life, the operative mortality varies between 50 and 75 percent. ,4,9-19 Pulmonary insufficiency is not a problem post-operatively in infants with late manifestation3 because the 98
herniation was not present at birth but occurred sometime later.8 This fact accounts for the low operative mortality (one to three percent) and excellent results following operative repair in contrast to the high mortality rate in the neonates who suffer from pulmonary insufficiency.
Acknowledgement My gratitude goes to William F. Sasser, MD, cardiothoracic consultant, Homer G. Phillips Hospital, for invaluable discussions.
Literature Cited 1. Johnson DG, Deaver RM, Koop CE: Diaphragmatic hernia in infancy: Factors affecting mortal ity rate. Su rgery 62:1082-1086, 1967
2. Ban JL, Moore TC: Intrathoracic tension and incarceration of stomach and liver through right-sided cogenital posterolateral defect. J Thorac Cardiovasc Surg 66:969-975, 1973 3. Orringer MB, Kirsh MM, Sloan H: Congenital and traumatic diaphragmatic hernias exclusive of hiatus. Curr Prob Surg: 60-64, March, 1975 4. Baffes TG: Diaphragmatic hernia. In Mustard WT, Ravitch MM, Snyder WH, et al (eds): Pediatric Surgery. Chicago, Year Book Medical Publishers, 1969, pp 342-351 5. Gray SW, Scandalakes JE: The diaphragm. In Gray SW, Elias J (eds): Embryology for Surgeons. Philadelphia, WB Saunders, 1972, pp 359-374 6. Canino CW, Eichman J, Rominger CJ, et al: Congenital right diaphragmatic hernia. Radiology 82:249-253, 1964 7. Kirklin BR, Hodgson JR: Roentgenologic characteristics of a diaphragmatic hernia. Am J Roentgenol Radium Ther NucI Med 48:77-101, 1947 8. Forshall I: Less common herniae through the diaphragm of infants and children. Proc R Soc Med 59:212-214, 1966 9. Baumgartner CJ, Scott RF: Surgical emergency of diaphragmatic hernia in infancy. Arch Surg 61:170-182, 1950 10. Boles ET, Schiller M, Weinberger M: Improved management of neonates with congenital diaphragmatic hernia. Arch Surg 103:344-349, 1971 11. Bonham-Carter RE, Waterston DJ, Aberdeen E: Hernia and eventration of the diaphragm. Lancet 1:556-560, 1962 12. Brown JH, Davey RB: Congenital posterolateral diaphragmatic hernia. Aust NZ J Surg 40:30-33, 1970 13. Gravier L, Dorman GW, Votteler TP: Congenital diaphragmatic hernia in children. Surg Gynecol Obstet 132:408-413, 1971 14. Jackson TM: Congenital diaphragmatic hernia. Arch Surg 95:102-110,
1967 15. Livaditis A, Norstrand A: Congenital postereolateral diaphragmatic hernia in infants. Scan J Thorac Cardiovasc Surg 5:67-77, 1971 16. McNamara JJ, Erakles AJ, Gross RE: Congenital postereolateral diaphragmatic hernia in the newborn. J Thorac Cardiovasc Surg 55:55-59, 1968 17. Rickam PP, Johnston, JH: Neonatal Surgery. New York, Appleton-CenturyCrofts, 1969, pp 176-191 18. Snyder WH Jr, Greany EM Jr: Congenital diaphragmatic hernia: 77 consecutive cases. Surgery 57:576-588, 1965 19. Whittaker LD, Lynn HB, Dawson B, et al: Hernias of the Foramen of Bochdalek in children. Mayo Clin Proc 43: 580-582, 1968
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 69, NO. 2, 1977
Congenital pleuroperitoneal diaphragmatic hernia usually presents in neonates. Children manifesting symptoms and signs after the neonatal period constitute a distinct, uncommon, and infrequently recognized group of patients. Occurrence in adults is also uncommon, with fewer than a hundred patients reported in the literature.
Case Report A two-year-old black female patient presented with a two-day history of mild epigastric pain and dyspnea which had been preceded by cough and mild fever for four days. The child had pneumonia at four months of age, but no history of trauma to chest or abdomen, and she was of normal growth and nutritional status. She was moderately dyspneic, with diminished vesicular breath sounds and audible bowel sounds in the left chest anteriorly and posteriorly. The abdomen was scaphoid and rather "empty." Pre-operative plain and meglucamine diatrizoate (Gastrografin) roentgenography (Figures 1 and 2) confirmed the diagnosis of a left diaphragmatic hernia with intrapleural evisceration of the bowels.
Requests for reprints should be addressed to Dr. Sanya Adeyemi-Doro, Department of Surgery, Homer G. Phillips Hospital, St. Louis, Mo 63113.
At laparotomy under general anesthesia and employing a left paramedian skin incision, the omentum, small bowel, caecum, appendix ascending, and the transverse colon were found eviscerated into the left pleural cavity. These were readily reduced after introducing a soft, red rubber catheter to fill the pleural space with air. Hence a thoracoabdominal incision was not needed. A 9 cm diameter congenital left pleuroperitoneal defect was found with a 1 cm posterior lip which was just adequate for a double layer repair with double 'O' nonabsorbable, ethiflex suture. There was no hernial sac, and other congenital abnormality was detected. The anterior abdominal wall was then manually stretched. This enabled the primary closure of the abdominal wall and the accommodation of the herniated viscera without undue tension. Post-operative pulmonary complications, including a left atelectasis, were satisfactorily managed with a volume respirator and assisted positive pressure respiration, maintenance of
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 69, NO. 2, 1977
fluid and electrolyte homeostasis, arterial blood gas monitor, and antibiotics. The left thoracostomy tube which had been connected to a water seal drainage was removed 48 hours postoperatively after satisfactory pulmonary expansion. The gastrostomy tube was removed on the eighth postoperative day. The patient was discharged on the 15th day after admission.
Discussion It is believed that closure of the pleuroperitoneal canal has been present since birth in late-onset cases of diaphragmatic hernia of Bochdalek. Delayed herniation of organs into the chest may be provoked by sudden increases in abdominal tension and in adults, by pregnancy and marked obesity. Eighty-five percent of congenital posterolateral diaphragmatic hernias occur on the left side.' The liver is thought to prevent herniation of viscera through the right pleuroperitoneal canal long enough for the defect to close.2 However, delayed manifestations in older infants occur more often on the right than left. The incidence of right-sided hernia in these older infants and children is twice that of neonates whose symptoms lead to operation in the first three days of life.3 However, in this patient, a relatively large congenital defect was found on the left side. There was no hernia sac, as 97
Figure 2. The same patient 15th post-operative day. Notice residual pneumonitis right-middle lobe.
Figure 1. Late-onset left pleuroperitoneal diaphragmatic hernia in a two-year-old girl At laparotomy, the omentum, small bowel, caecum, appendix, and colon were found eviscerated into the left pleural cavity. There was no hernia sac.
occurs in 85 to 90 percent of reported cases.5 The chest roentgenograms in the older children usually differ from the chest roentgenograms in the neonate with respect only to the lessened amount of mediastinal displacement and volume of herniated viscera.6'7 In this case, the mediastinal shift was obvious with multiple bowels in the chest (Figures 1 and 2). In contrast to the situation in neonates, ingestion of barium is useful for diagnostic confirmation and free from hazard in the absence of intestinal obstruction.3 The age of onset of symptoms is directly related to the success of surgical treatment. In neonates over 48 hours of age, the mortality is less than f ive percent. However, in those symptomatic within the first 48 hours of life, the operative mortality varies between 50 and 75 percent. ,4,9-19 Pulmonary insufficiency is not a problem post-operatively in infants with late manifestation3 because the 98
herniation was not present at birth but occurred sometime later.8 This fact accounts for the low operative mortality (one to three percent) and excellent results following operative repair in contrast to the high mortality rate in the neonates who suffer from pulmonary insufficiency.
Acknowledgement My gratitude goes to William F. Sasser, MD, cardiothoracic consultant, Homer G. Phillips Hospital, for invaluable discussions.
Literature Cited 1. Johnson DG, Deaver RM, Koop CE: Diaphragmatic hernia in infancy: Factors affecting mortal ity rate. Su rgery 62:1082-1086, 1967
2. Ban JL, Moore TC: Intrathoracic tension and incarceration of stomach and liver through right-sided cogenital posterolateral defect. J Thorac Cardiovasc Surg 66:969-975, 1973 3. Orringer MB, Kirsh MM, Sloan H: Congenital and traumatic diaphragmatic hernias exclusive of hiatus. Curr Prob Surg: 60-64, March, 1975 4. Baffes TG: Diaphragmatic hernia. In Mustard WT, Ravitch MM, Snyder WH, et al (eds): Pediatric Surgery. Chicago, Year Book Medical Publishers, 1969, pp 342-351 5. Gray SW, Scandalakes JE: The diaphragm. In Gray SW, Elias J (eds): Embryology for Surgeons. Philadelphia, WB Saunders, 1972, pp 359-374 6. Canino CW, Eichman J, Rominger CJ, et al: Congenital right diaphragmatic hernia. Radiology 82:249-253, 1964 7. Kirklin BR, Hodgson JR: Roentgenologic characteristics of a diaphragmatic hernia. Am J Roentgenol Radium Ther NucI Med 48:77-101, 1947 8. Forshall I: Less common herniae through the diaphragm of infants and children. Proc R Soc Med 59:212-214, 1966 9. Baumgartner CJ, Scott RF: Surgical emergency of diaphragmatic hernia in infancy. Arch Surg 61:170-182, 1950 10. Boles ET, Schiller M, Weinberger M: Improved management of neonates with congenital diaphragmatic hernia. Arch Surg 103:344-349, 1971 11. Bonham-Carter RE, Waterston DJ, Aberdeen E: Hernia and eventration of the diaphragm. Lancet 1:556-560, 1962 12. Brown JH, Davey RB: Congenital posterolateral diaphragmatic hernia. Aust NZ J Surg 40:30-33, 1970 13. Gravier L, Dorman GW, Votteler TP: Congenital diaphragmatic hernia in children. Surg Gynecol Obstet 132:408-413, 1971 14. Jackson TM: Congenital diaphragmatic hernia. Arch Surg 95:102-110,
1967 15. Livaditis A, Norstrand A: Congenital postereolateral diaphragmatic hernia in infants. Scan J Thorac Cardiovasc Surg 5:67-77, 1971 16. McNamara JJ, Erakles AJ, Gross RE: Congenital postereolateral diaphragmatic hernia in the newborn. J Thorac Cardiovasc Surg 55:55-59, 1968 17. Rickam PP, Johnston, JH: Neonatal Surgery. New York, Appleton-CenturyCrofts, 1969, pp 176-191 18. Snyder WH Jr, Greany EM Jr: Congenital diaphragmatic hernia: 77 consecutive cases. Surgery 57:576-588, 1965 19. Whittaker LD, Lynn HB, Dawson B, et al: Hernias of the Foramen of Bochdalek in children. Mayo Clin Proc 43: 580-582, 1968
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 69, NO. 2, 1977
