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Postpericardiotomy syndrome and chylopericardium: Two unusual complications after aortopexy for tracheomalacia
Postpericardiotomy Syndrome and Chylopericardium: Two Unusual Complications After Aortopexy for Tracheomalacia ByErik
D. Skarsgard,
Robert M. Filler, and Riccardo A. Superina
Toronto, Ontario l In two boys (aged 10 years and 7 months), large symptomatic pericardial effusions developed after aortopexy for tracheomalacia. Both patients underwent percutaneous pericardial drainage. The delayed presentation (3 weeks postoperatively) and associated findings in the 10 year old were typical of postpericardiotomy syndrome, and he had an uneventful recovery after a course of aspirin therapy. The 7 month old had chylopericardium and was treated with pericardial drainage and a low-fat, medium-chain triglyceride diet. Both cases represent previously unreported complications of aortopexy. Copyri9ht o 7994 by W.B. Saunders Compuny INDEX WORDS: Tracheomalacia; aortopexy; postpericardiotomy syndrome; chylopericardium; pericardial effusion.
P
OSTPERICARDIOTOMY syndrome (PPS) and chylopericardium are infrequently recognized complications of cardiac surgery; both are characterized by the development of a pericardial effusion. We describe two pediatric patients (aged 10 years and 7 months) who had these unusual complications after aortopexy for tracheomalacia (TM). CASE REPORTS
Case 1 A lo-year-old boy presented with a 9-year history of recurrent respiratory tract infections. His history also included esophageal atresia and distal tracheoesophageal fistula (TEF), repaired at birth, and a Nissen fundoplication at age 2 months for gastroesophageal reflux (GER). An upper gastrointestinal (GI) series showed moderately severe TM in lateral projections, but no GER. Rigid bronchoscopy showed TM, with complete luminal obliteration of the trachea and both mainstem bronchi with coughing. Aortopexy was performed using the ventilating bronchoscope, and correction of TM was observed. The patient’s hospital course was uneventful, and to he was discharged on the eighth postoperative day. He returned on the 24th postoperative day with a fever, dyspnea, and mild chest pain. His jugular venous pulsations were elevated, his heart sounds were diminished, and he had a pulsus paradoxus of 10 mm Hg. A chest x-ray showed markedly increased heart size and a left pleural effusion. Electrocardiography showed nonspecific STand T-wave changes. Echocardiography showed a large pericardial effusion with cardiac tamponade. Pericardiocentesis was performed on 360 mL of serosanguinous fluid, and a pericardial drain was left in for 2 days. The pericardial fluid was sterile. The patient was treated with aspirin (60 mglkgld) for 1 month and was
From the Department of Surgery, The Hospital for Sick Children, Toronto, Ontario. Address reprint requests to Robe)?M. Filler, MD, 5.55 UniversityAve, Toronto, Ontario, Canada M5G 1X8. Copyright o 1994 by W.B. Saunders Company 0022-3468/94/2912-0010$03.00/0 1534
hospitalized for 9 days. Follow-up echocardiograms (at 4 days and 1 month postpericardiocentesis) showed no recurrent effusion.
Case 2 A 1.4-kg twin boy was born by cesarean section at 33 weeks’ gestation. Esophageal atresia with distal TEF was diagnosed, and repair was performed on the fourth day of life. An upper GI series on the seventh postoperative day showed an intact esophageal anastomosis; however, both GER and TM were noted. The infant vomited all oral feedings and was treated by nasojejunal feeding and prokinetics, but had poor weight gain. A second upper GI series showed massive GER, and a Nissen fundoplication and feeding gastrostomy were performed at 6 weeks of age. The infant progressed to full oral feeding, and the gastrostomy tube was removed at 4 months of age. He was readmitted at age 5% months because of blue spells during feeding. A chest x-ray showed right middle lobe consolidation, and he was treated for pneumonia. An upper GI series showed tracheomalacia but no GER. Subsequent blue spells during feeding were observed in the hospital, and rigid bronchoscopy confirmed tracheomalacia. Aortopexy also was performed at this time. The patient was well for the first postoperative week, then progressive dyspnea developed. A chest x-ray showed right upper lobe atelectasis and a large cardiac silhouette (Fig 1), and a pericardial effusion was confirmed by echocardiographic examination. Ultrasound-guided pericardiocentesis yielded 100 mL of milky fluid. Fluid analysis showed the presence of chylomicrons and of 36,000 white blood cells per microliter, 96% of which were lymphocytes, A pericardial drain was left in (Fig 2) and portagen feeding was begun. Chylous pericardial drainage decreased daily, and the catheter was removed after 7 days. Follow-up ultrasound examinations showed no recurrence of effusion, and normal oral feeding was resumed after 3 weeks of portagen feeding. Surgical technique. The technique of aortopexy was described previously.’ Under general anaesthesia, maintained by a ventilating bronchoscope, the patient is positioned supine, with the left chest region elevated. The aorta is approached through the left third intercostal space via a left inframammary skin incision. A left thymic lobectomy is performed, and the pericardium is opened longitudinally between traction sutures, taking care to avoid injury to the left phrenic nerve. Three or four interrupted nonabsorbable sutures are placed through the adventitia of the ascending aorta, up to the origin of the innominate artery. The sutures are delivered through the sternum, into a subcutaneous pocket, where application of anterior traction allows bronchoscopic assessment of the adequacy of the procedure. The sutures are tied and the incisions closed without mediastinal drainage. DISCUSSION
PPS and chylopericardium are rare complications of surgical procedures in which the pericardium is opened. The onset of fever, chest pain, pericardial and pleural effusion, and occasionally cardiac tamponade weeks to months after cardiac surgery, was first enJournalof Pediatric Surgery, Vol29, No 12 (December), 1994: pp 1534-1536
1535
PERICARDIAL EFFUSION AFTER AORTOPEXY
Fig 1. Chest radiograph taken 10 days after aortopexy. Note the right upper lobe atelectasis and the enlarged cardiac silhouette. Echocardiography confirmed the presence of a large pericardial effusion.
countered in the early 1950s in relation to rheumatic mitral valve surgery.2 Initially, the symptoms of this “postcommissurotomy” or “postvalvotomy” syndrome were attributed to a reactivation of rheumatic fever.3 In 1958, Ito et al described an identical postoperative condition in patients who underwent surgery for nonrheumatic heart disease.4 They proposed that the common feature in all patients was a wide incision of the pericardium and designated this condition as postpericardiotomy syndrome (PPS). It appears that this condition has an immunologic etiology because it has been shown that cardiac-
Fig 2. Same case as In Fig 1, after ultrasound-guided tesh and pericardlal drain placement.
pericardiocen-
specific antibodies and antibodies to a number of viruses are present in higher titer among cardiac surgical patients who manifest the syndrome, versus those who do not. These investigators concluded that concurrent viral illness initiates or mediates an immune response that is responsible for the development of the syndrome.5 The latency of the syndrome (usually it develops 3 weeks to 6 months after surgery) distinguishes it from the commonly observed, self-limited, and unexplained fever with transient pericardial friction rub, seen early in the postoperative course of patients undergoing pericardiotomy. Treatment for PPS consists of careful observation for pericardial fluid accumulation, and institution of aspirin or indomethacin therapy.6 In a small number of patients, pericardial inflammation is refractory and requires systemic corticosteroids. Recurrence is common, especially after steroid withdrawal, and may persist for years. Chylopericardium is a rare condition that can be congenital in origin or occur secondary to thoracic trauma (operative and nonsurgical), mediastinal tumors (usually lymphangiomas), or irradiation. It was first reported in reIation to cardiac surgery in 1971, by Thomas and McGoon.’ Since then, there have been several case reports, most of which involved children who underwent surgery for congenital heart disease.8-12The exact etiology of chylopericardium is not known. Cardiac lymphatics form a tributary that crosses under the arch formed by the left and main pulmonary arteries, then travels to the left of the aorta, and joins the mediastinal lymphatics. The pericardial lymphatic plexus drains either superiorly into the paraesophageal lymphatics or inferiorly into the diaphragmatic lymphatics. AI1 lymphatic plexuses drain into the thoracic duct. These lymphatics are prone to injury in any procedure (including aortopexy) that involves pericardiotomy and dissection around the origin of the great vessels. Another observation has been the association of left subclavian vein thrombosis with chylopericardium.13 It may be that the combination of elevated thoracic duct pressures from venous thrombosis plus injury to the pericardial or cardiac lymphatics is required for the development of chylopericardium. Our patient had had vascular access through both the right and left internal jugular veins, although there was no clinical evidence of central venous thrombosis at the time the chylopericardium developed. The diagnosis of chylopericardium is made from milky fluid aspirated from the pericardial sac. The fluid has high concentrations of triglycerides and
1536
SKARSGARD,
protein, and the diagnosis is confirmed microscopically by demonstration of chylomicrons and a high concentration of leukocytes, almost all of which are lymphocytes. Treatment of chylopericardium involves prevention of tamponade. and avoidance of the nutritional and immunologic sequelae caused by a significant lymph leak. The role of dietary manipulation is to reduce chyle production and hence flow within the thoracic lymphatics. This is accomplished with either intravenous hyperalimentation or high-protein, lowfat, medium-chain triglyceride enteral formulas, both of which allow direct absorption of fat into the portal venous system. Pericardial drainage by pericardiocen-
FILLER, AND SUPERINA
tesis or tube pericardiostomy, in combination with dietary manipulation, is usually successful. Surgical procedures including thoracic duct and tributary lymphatic ligation, pericardiectomy, and pericardialperitoneal shunting are reserved for cases in which nonoperative therapy fails.14 Both cases presented herein illustrate uncommon complications of pericardiotomy, and neither PPS nor chylopericardium had been described in relation to aortopexy. Prompt recognition of a postoperative pericardial effusion, consideration of the diagnostic possibilities, and diagnostic/therapeutic pericardiocentesis (when indicated) should result in the appropriate diagnosis and treatment.
REFERENCES 1. Blair GK, Cohen R, Filler RM: Treatment of tracheomalacia: Eight years’ experience. J Pediatr Surg 21:781-7851986 2. Janton OH, Glover RP, O’Neil TJ, et al: Results of the surgical treatment of mitral stenosis. Circulation 6:321-333, 1952 3. Soloff LA, Zatuchni J, Janton OH, et al: Reactivation’ of rheumatic fever following mitral commissurotomy. Circulation 8:481-493,1953 4. Ito T, Engle MA, Goldberg HP: Postpericardiotomy syndrome following surgery for nonrheumatic heart disease. Circulation 17:549-556,1958 5. Engle MA, Zabriskie JB, Sentertit LB, et al: Viral illness and the postpericardiotomy syndrome: A prospective study in children. Circulation 62:1151-1158,198O 6. Shabetai R: Diseases of the pericardium, in Hurst JW (ed): The Heart, Arteries and Veins (ed 6): New York, NY, McGrawHill, 1986, pp 1249-1275 (chap 59) 7. Thomas CS Jr, McGooe DC: Isolated massive chylopericardium following cardiopulmonary bypass. J Thorac Cardiovasc Surg 61:945-948,197l 8. Hawker RE, Cardmill TB, Celermajer JM, et al: Chylous
pericardial effision complicating aorta-right pulmonary artery anastomosis. J Thorac Cardiovasc Surg 63:491-494,1972 9. Jacob T, deLeva1 M, Stark J, et al: Chylopericardium as a complication of aortopulmonary shunt. Arch Surg 108:870-871, 1974 10. Hargus EP, Carson SD, McGarth RL, et al: Chylothorax and chylopericardial tamponade following Blalock-Taussig anastomosis. J Thorac Cardiovasc Surg 75:642-645,1978 11. Pugliese P, Santi C, Eufrate S: Isolated chylopericardium after successful correction of total anomalous pulmonary venous drainage. J Cardiovasc Surg 25175-77,1984 12. Pierse PM, Byrne PJ, Coe JY, et al: Isolated chylopericardium after repair of coarctation of the aorta. J Thorac Cardiovasc Surg 94:307-310,1987 13. Rose DM, Colvin SB, Danilowicz D, et al: Cardiac tamponade secondary to chylopericardium following cardiac surgery: Case report and review of the literature. Ann Thorac Surg 34:333-336, 1982 14. Chan BB, Murphy MC, Rodgers BM: Management of chylopericardium. J Pediatr Surg 25:1185-1189,199O
D. Skarsgard,
Robert M. Filler, and Riccardo A. Superina
Toronto, Ontario l In two boys (aged 10 years and 7 months), large symptomatic pericardial effusions developed after aortopexy for tracheomalacia. Both patients underwent percutaneous pericardial drainage. The delayed presentation (3 weeks postoperatively) and associated findings in the 10 year old were typical of postpericardiotomy syndrome, and he had an uneventful recovery after a course of aspirin therapy. The 7 month old had chylopericardium and was treated with pericardial drainage and a low-fat, medium-chain triglyceride diet. Both cases represent previously unreported complications of aortopexy. Copyri9ht o 7994 by W.B. Saunders Compuny INDEX WORDS: Tracheomalacia; aortopexy; postpericardiotomy syndrome; chylopericardium; pericardial effusion.
P
OSTPERICARDIOTOMY syndrome (PPS) and chylopericardium are infrequently recognized complications of cardiac surgery; both are characterized by the development of a pericardial effusion. We describe two pediatric patients (aged 10 years and 7 months) who had these unusual complications after aortopexy for tracheomalacia (TM). CASE REPORTS
Case 1 A lo-year-old boy presented with a 9-year history of recurrent respiratory tract infections. His history also included esophageal atresia and distal tracheoesophageal fistula (TEF), repaired at birth, and a Nissen fundoplication at age 2 months for gastroesophageal reflux (GER). An upper gastrointestinal (GI) series showed moderately severe TM in lateral projections, but no GER. Rigid bronchoscopy showed TM, with complete luminal obliteration of the trachea and both mainstem bronchi with coughing. Aortopexy was performed using the ventilating bronchoscope, and correction of TM was observed. The patient’s hospital course was uneventful, and to he was discharged on the eighth postoperative day. He returned on the 24th postoperative day with a fever, dyspnea, and mild chest pain. His jugular venous pulsations were elevated, his heart sounds were diminished, and he had a pulsus paradoxus of 10 mm Hg. A chest x-ray showed markedly increased heart size and a left pleural effusion. Electrocardiography showed nonspecific STand T-wave changes. Echocardiography showed a large pericardial effusion with cardiac tamponade. Pericardiocentesis was performed on 360 mL of serosanguinous fluid, and a pericardial drain was left in for 2 days. The pericardial fluid was sterile. The patient was treated with aspirin (60 mglkgld) for 1 month and was
From the Department of Surgery, The Hospital for Sick Children, Toronto, Ontario. Address reprint requests to Robe)?M. Filler, MD, 5.55 UniversityAve, Toronto, Ontario, Canada M5G 1X8. Copyright o 1994 by W.B. Saunders Company 0022-3468/94/2912-0010$03.00/0 1534
hospitalized for 9 days. Follow-up echocardiograms (at 4 days and 1 month postpericardiocentesis) showed no recurrent effusion.
Case 2 A 1.4-kg twin boy was born by cesarean section at 33 weeks’ gestation. Esophageal atresia with distal TEF was diagnosed, and repair was performed on the fourth day of life. An upper GI series on the seventh postoperative day showed an intact esophageal anastomosis; however, both GER and TM were noted. The infant vomited all oral feedings and was treated by nasojejunal feeding and prokinetics, but had poor weight gain. A second upper GI series showed massive GER, and a Nissen fundoplication and feeding gastrostomy were performed at 6 weeks of age. The infant progressed to full oral feeding, and the gastrostomy tube was removed at 4 months of age. He was readmitted at age 5% months because of blue spells during feeding. A chest x-ray showed right middle lobe consolidation, and he was treated for pneumonia. An upper GI series showed tracheomalacia but no GER. Subsequent blue spells during feeding were observed in the hospital, and rigid bronchoscopy confirmed tracheomalacia. Aortopexy also was performed at this time. The patient was well for the first postoperative week, then progressive dyspnea developed. A chest x-ray showed right upper lobe atelectasis and a large cardiac silhouette (Fig 1), and a pericardial effusion was confirmed by echocardiographic examination. Ultrasound-guided pericardiocentesis yielded 100 mL of milky fluid. Fluid analysis showed the presence of chylomicrons and of 36,000 white blood cells per microliter, 96% of which were lymphocytes, A pericardial drain was left in (Fig 2) and portagen feeding was begun. Chylous pericardial drainage decreased daily, and the catheter was removed after 7 days. Follow-up ultrasound examinations showed no recurrence of effusion, and normal oral feeding was resumed after 3 weeks of portagen feeding. Surgical technique. The technique of aortopexy was described previously.’ Under general anaesthesia, maintained by a ventilating bronchoscope, the patient is positioned supine, with the left chest region elevated. The aorta is approached through the left third intercostal space via a left inframammary skin incision. A left thymic lobectomy is performed, and the pericardium is opened longitudinally between traction sutures, taking care to avoid injury to the left phrenic nerve. Three or four interrupted nonabsorbable sutures are placed through the adventitia of the ascending aorta, up to the origin of the innominate artery. The sutures are delivered through the sternum, into a subcutaneous pocket, where application of anterior traction allows bronchoscopic assessment of the adequacy of the procedure. The sutures are tied and the incisions closed without mediastinal drainage. DISCUSSION
PPS and chylopericardium are rare complications of surgical procedures in which the pericardium is opened. The onset of fever, chest pain, pericardial and pleural effusion, and occasionally cardiac tamponade weeks to months after cardiac surgery, was first enJournalof Pediatric Surgery, Vol29, No 12 (December), 1994: pp 1534-1536
1535
PERICARDIAL EFFUSION AFTER AORTOPEXY
Fig 1. Chest radiograph taken 10 days after aortopexy. Note the right upper lobe atelectasis and the enlarged cardiac silhouette. Echocardiography confirmed the presence of a large pericardial effusion.
countered in the early 1950s in relation to rheumatic mitral valve surgery.2 Initially, the symptoms of this “postcommissurotomy” or “postvalvotomy” syndrome were attributed to a reactivation of rheumatic fever.3 In 1958, Ito et al described an identical postoperative condition in patients who underwent surgery for nonrheumatic heart disease.4 They proposed that the common feature in all patients was a wide incision of the pericardium and designated this condition as postpericardiotomy syndrome (PPS). It appears that this condition has an immunologic etiology because it has been shown that cardiac-
Fig 2. Same case as In Fig 1, after ultrasound-guided tesh and pericardlal drain placement.
pericardiocen-
specific antibodies and antibodies to a number of viruses are present in higher titer among cardiac surgical patients who manifest the syndrome, versus those who do not. These investigators concluded that concurrent viral illness initiates or mediates an immune response that is responsible for the development of the syndrome.5 The latency of the syndrome (usually it develops 3 weeks to 6 months after surgery) distinguishes it from the commonly observed, self-limited, and unexplained fever with transient pericardial friction rub, seen early in the postoperative course of patients undergoing pericardiotomy. Treatment for PPS consists of careful observation for pericardial fluid accumulation, and institution of aspirin or indomethacin therapy.6 In a small number of patients, pericardial inflammation is refractory and requires systemic corticosteroids. Recurrence is common, especially after steroid withdrawal, and may persist for years. Chylopericardium is a rare condition that can be congenital in origin or occur secondary to thoracic trauma (operative and nonsurgical), mediastinal tumors (usually lymphangiomas), or irradiation. It was first reported in reIation to cardiac surgery in 1971, by Thomas and McGoon.’ Since then, there have been several case reports, most of which involved children who underwent surgery for congenital heart disease.8-12The exact etiology of chylopericardium is not known. Cardiac lymphatics form a tributary that crosses under the arch formed by the left and main pulmonary arteries, then travels to the left of the aorta, and joins the mediastinal lymphatics. The pericardial lymphatic plexus drains either superiorly into the paraesophageal lymphatics or inferiorly into the diaphragmatic lymphatics. AI1 lymphatic plexuses drain into the thoracic duct. These lymphatics are prone to injury in any procedure (including aortopexy) that involves pericardiotomy and dissection around the origin of the great vessels. Another observation has been the association of left subclavian vein thrombosis with chylopericardium.13 It may be that the combination of elevated thoracic duct pressures from venous thrombosis plus injury to the pericardial or cardiac lymphatics is required for the development of chylopericardium. Our patient had had vascular access through both the right and left internal jugular veins, although there was no clinical evidence of central venous thrombosis at the time the chylopericardium developed. The diagnosis of chylopericardium is made from milky fluid aspirated from the pericardial sac. The fluid has high concentrations of triglycerides and
1536
SKARSGARD,
protein, and the diagnosis is confirmed microscopically by demonstration of chylomicrons and a high concentration of leukocytes, almost all of which are lymphocytes. Treatment of chylopericardium involves prevention of tamponade. and avoidance of the nutritional and immunologic sequelae caused by a significant lymph leak. The role of dietary manipulation is to reduce chyle production and hence flow within the thoracic lymphatics. This is accomplished with either intravenous hyperalimentation or high-protein, lowfat, medium-chain triglyceride enteral formulas, both of which allow direct absorption of fat into the portal venous system. Pericardial drainage by pericardiocen-
FILLER, AND SUPERINA
tesis or tube pericardiostomy, in combination with dietary manipulation, is usually successful. Surgical procedures including thoracic duct and tributary lymphatic ligation, pericardiectomy, and pericardialperitoneal shunting are reserved for cases in which nonoperative therapy fails.14 Both cases presented herein illustrate uncommon complications of pericardiotomy, and neither PPS nor chylopericardium had been described in relation to aortopexy. Prompt recognition of a postoperative pericardial effusion, consideration of the diagnostic possibilities, and diagnostic/therapeutic pericardiocentesis (when indicated) should result in the appropriate diagnosis and treatment.
REFERENCES 1. Blair GK, Cohen R, Filler RM: Treatment of tracheomalacia: Eight years’ experience. J Pediatr Surg 21:781-7851986 2. Janton OH, Glover RP, O’Neil TJ, et al: Results of the surgical treatment of mitral stenosis. Circulation 6:321-333, 1952 3. Soloff LA, Zatuchni J, Janton OH, et al: Reactivation’ of rheumatic fever following mitral commissurotomy. Circulation 8:481-493,1953 4. Ito T, Engle MA, Goldberg HP: Postpericardiotomy syndrome following surgery for nonrheumatic heart disease. Circulation 17:549-556,1958 5. Engle MA, Zabriskie JB, Sentertit LB, et al: Viral illness and the postpericardiotomy syndrome: A prospective study in children. Circulation 62:1151-1158,198O 6. Shabetai R: Diseases of the pericardium, in Hurst JW (ed): The Heart, Arteries and Veins (ed 6): New York, NY, McGrawHill, 1986, pp 1249-1275 (chap 59) 7. Thomas CS Jr, McGooe DC: Isolated massive chylopericardium following cardiopulmonary bypass. J Thorac Cardiovasc Surg 61:945-948,197l 8. Hawker RE, Cardmill TB, Celermajer JM, et al: Chylous
pericardial effision complicating aorta-right pulmonary artery anastomosis. J Thorac Cardiovasc Surg 63:491-494,1972 9. Jacob T, deLeva1 M, Stark J, et al: Chylopericardium as a complication of aortopulmonary shunt. Arch Surg 108:870-871, 1974 10. Hargus EP, Carson SD, McGarth RL, et al: Chylothorax and chylopericardial tamponade following Blalock-Taussig anastomosis. J Thorac Cardiovasc Surg 75:642-645,1978 11. Pugliese P, Santi C, Eufrate S: Isolated chylopericardium after successful correction of total anomalous pulmonary venous drainage. J Cardiovasc Surg 25175-77,1984 12. Pierse PM, Byrne PJ, Coe JY, et al: Isolated chylopericardium after repair of coarctation of the aorta. J Thorac Cardiovasc Surg 94:307-310,1987 13. Rose DM, Colvin SB, Danilowicz D, et al: Cardiac tamponade secondary to chylopericardium following cardiac surgery: Case report and review of the literature. Ann Thorac Surg 34:333-336, 1982 14. Chan BB, Murphy MC, Rodgers BM: Management of chylopericardium. J Pediatr Surg 25:1185-1189,199O