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Diaphragmatic eventration in infants and children: Is conservative treatment justified?
Diaphragmatic Eventration in Infants and Children: Is Conservative Treatment Justified? By
Chikara
Tsugawa,
Ken
Kimura,
Eiji
Nishijima,
Toshihiro
Muraji,
and
Masahiro
Yamaguchi
Kobe, Japan
furpose:The purpose of this study is to examine the justification of diaphragmatic plication to treat diaphragmatic eventration. A retrospective review of 50 patients who underwent diaphragmatic plication for phrenic nerve injury (PNI) or congenital muscular deficiency (CMD) of the diaphragm was conducted. Methods: During the last 26 years, 50 patients, aged 4 days to 7 years, were surgically treated for diaphragmatic eventration. Twenty-five patients had iatrogenic PNI and another 25 had CMD. Respiratory distress developed in all patients who had PNI and 10 required mechanical ventilatory support for 13 to 78 days (mean, 41 days) before operation. Respiratory symptoms developed in 17 of 25 patients who had CMD, and four required ventilatory support. In those who were asymptomatic, we justified surgical repair to optimize future lung growth. All patients underwent diaphragmatic plication by a thoracic approach. Reefing mattress sutures on pledgets were used for the plication.
D
IAPHRAGMATIC EVENTRATION is caused by phrenic nerve injury (PNI) or congenital muscular deficiency (CMD) of the diaphragm. Clinical manifestations in infants and children who have diaphragmatic eventration are diverse, ranging from mild gastrointestinal symptoms such as failure to thrive, tachypnea, nausea and vomiting, to life-threatening respiratory distress requiring mechanical ventilatory support. Diaphragmatic plication dramatically improves these symptoms. However, the indication for this procedure remains controversial because some patients are asymptomatic.1-7 This fact motivated us to complete a retrospective review of our patients who had diaphragmatic eventration to determine appropriate indications for diaphragmatic plication. MATERIALS
AND
METHODS
Patients During the period from 197 1 to 1996,50 patients, ranging in age from 4 days to 7 years, were treated for diaphragmatic eventration by diaphragmatic plication at the Kobe Children’s Hospital. Twenty five patients were considered to have PNI as a result of birth trauma (n = lo), surgical injury (n = 12), motor neuron disease (n = 2), and unknown causes (n = 1). The associated anomalies in this group in whom surgery might have caused the PNI were congenital cardiac defects (n = 7), mediastinal tumor (n = 3), esophageal atresia (n = l), and others (n = 1). Respiratory distress developed in all patients of this group after phrenic nerve injury. Ten of these patients needed ventilatory support for 13 to 78 days (mean 41 days), and seven patients required oxygen inhalation for 14 to 90 days before operation. Journal
of Pediatric
Surgery,
Vol32,
No 11 (November),
1997: pp 1643-1644
Results: In patients be discontinued operation, with a status. Two patients tion was not tight but none because Conclusion: who have immediately respiratory J Pediatr Saunders
INDEX injury, tion.
who had PNI, ventilatory support could within 0 to 6 days (mean, 3 days) after dramatic improvement in their respiratory required reoperation because the plicaenough. Seven patients died in this series, of the diaphragmatic plication.
This study suggests that symptomatic patients diaphragmatic eventration should be operated on with an expected dramatic resolution of their problems. Surg 3.21643-1644. Copyright o 7997 by W.B.
Company.
WORDS: Diaphragmatic diaphragmatic muscular
eventration, deficiency,
phrenic surgical
nerve indica-
The age range at operation in this group was 16 days to 5 years (mean, 8 months). The other 25 patients belonged to the group who had CMD of the diaphragm. In this group of patients, the diaphragmatic muscle was either uniformly thin with only its outer edges being normal, or the diaphragmatic muscle fibers were nonresponsive to electric stimulation. The left side of the diaphragm was involved in 13 patients, and the right side in 12 patients. Respiratory distress developed in 17 patients. Four of these patients needed ventilatory support from 3 1 to 210 days (mean, 120 days), and five patients required oxygen inhalation for 4 days to 4 months before operation. Three of seven patients who were free of respiratory distress acquired gastrointestinal symptoms such as vomiting, feeding difficulties, and poor weight gain since birth. The remaining four patients were asymptomatic. The age range at operation was 19 days to 7 years.
Operative
Technique
The diaphragm is approached through a sixth or seventh intercostal space thoracotomy.* In patients who have phrenic nerve injury, diaphragmatic plication is performed using several traction sutures to find the appropriate tension on the diaphragm that is required to eliminate
From the Departments of Surgery and Cardiothoracic Surgery, Kobe Children’s Hospital, Kobe, Japan. Presented at the 30th Annual Meeting of the PaciJc Association of Pediatric Surgeons, Phoenix, Arizona, May 9-13, 1997. Address reprint requests to Chikara Tsugawa, MD, Department of Surgery, Kobe Children 5 Hospital, l-l-l, Takakuradai, Suma-Ku, Kobe 654, Japan. Copyright o 1997 by WE. Saunders Company 0022-3468/97/3211-0030$03.00/O 1643
1644
TSUGAWA
paradoxical motion of the diaphragm. When the appropriate suture placement has been found, tension is released on these sutures. An appropriate number of nonabsorbable sutures on pledgets are placed in a reefing fashion. When these sutures are pulled up for tying, the diaphragm is reefed together in an accordion fashion. In patients who have congenital muscular defects, the electric stimulation is used to demarcate the muscular defect. Several traction sutures are placed at the muscular margin to test the tension on the sutures when the margins are approximated. The same suture materials and suturing technique were used both for patients who had PNI and CMD.
RESULTS
Of the 25 patients who had PNI, 10 patients who had been on mechanical ventilation with endotracheal intubation and seven patients with oxygen inhalation had ventilatory support removed within 0 to 6 days (mean, 3.0 days) after diaphragmatic plication. However, two patients in whom plication was not adequate to eliminate paradoxical respiration relapsed into respiratory distress. In these patients, replication of the diaphragm was performed 3 months and 16 days after the initial operation. The first patient died of respiratory failure from pulmonary damage caused by prolonged mechanical ventilation, and the second recovered. In one patient, the small bowel was injured during plication, and the patient subsequently required a laparotomy. Ultimately, 5 of the 25 patients in this group died: two of cardiac anomalies, two of congenital motor neuron disease, and one of pulmonary failure. Twenty survivors have been followed-up for 1 to 25 years. The involved diaphragm of these patients appeared normal on the chest x-ray films, and none of them has had respiratory distress. Of the 25 patients who had CMD, four who previously required ventilatory support were released from mechanical ventilation within 1 to 61 days (mean, 29 days) after plication. Recurrence of eventration developed in none. Two patients died of cardiac disease. Twenty-three survivors are followed-up radiologically annually to demonstrate synchronous movement of the involved side of the diaphragm. DISCUSSION
For many decades, there has been much controversy regarding conservative versus surgical treatment for diaphragmatic eventration.lm7 Because of the possibility
ET AL
of spontaneous resolution occurring within several months in patients who have PNI, previous reports support conservative management of respiratory distress even in symptomatic patients requiring mechanical ventilatory s~pport.*,~,~Our early patients were treated according to this principle with prolonged preoperative ventilatory support. However, an immediate and dramatic improvement in the respiratory status in patients who underwent a diaphragmatic plication encouraged us to operate on all patients with diaphragmatic eventration. Diaphragmatic plication is technically simple, easy and straightforward with a very low complication rate when carefully performed. 3,6,8A thoracic approach is mandatory for optimal access. Substantial tension to immobilize the plicated diaphragm avoids remaining flail, which reduces the tidal volume. To avoid injury of the intraabdominal organs, a shallow scoop of the diaphragm during placement of plication sutures is important. A retrospective review of our patients indicates that all patients were successfully weaned from ventilatory support within a few days of surgery with remarkable improvement in their respiratory status. In addition, gastrointestinal symptoms disappeared immediately. It is clear from our experience that diaphragmatic plication can eliminate the serious complications that result from both prolonged ventilatory support and malnutrition. In patients suffering from severe cyanosis or cardiac failure caused by associated congenital cardiac defects, reduction in tidal volume induced by diaphragmatic paralysis can be intolerable and fatal. Surgical intervention for asymptomatic patients or patients who have congenital diaphragmatic muscular defect is not recommended in the literature to date.2,4,7 However, when it is considered that the lungs continue to grow until the age of nearly 10 years9 it seems reasonable to provide space for future pulmonary development, although no comparative data on this subject are available as yet. When considering the cost-effectiveness issues, which are important in the era of managed care, prolonged hospitalization only for ventilatory support without providing an added reason for an end point is difficult to rationalize.
REFERENCES 1. Bishop HC, Koop CE: Acquired eventration of the diaphragm in infancy. Pediatrics 22:1088-1096, 1958 2. Wayne ER, Campbell JB, Burrington JD, et al: Eventration of the diaphragm. J Pediatr Surg 9:643-65 1, 1974 3. Schwartz MZ, Filler RM: Plication of diaphragm for symptomatic phrenic nerve paralysis. J Pediatr Surg 13:259-263, 1978 4. Mickell JJ, Oh KS, Siewers RD, et al: Clinical implications of postoperative unilateral phrenic nerve paralysis. J Thorac Cardiovasc Surg 76:297-304, 1978 5. Haller JA Jr, Pickard LR, Tepas JJ, et al: Management of
diaphragmatic paralysis in infants with special emphasis on selection of patients for operative plication. .I Pediatr Surg 14:779-785, 1979 6. Shoemaker R, Palmer G, Brown JW, et al: Aggressive treatment of acquired phrenic nerve paralysis in infants and small children. Ann Thorac Surg 32:251-259, 1981 7. Watanabe T, Tmsler GA, Williams WG, et ah Phrenic nerve paralysis after pediatric cardiac surgery. J Thorac Cardiovasc Surg 94:383-388,1987 8. Kimura K, Tsugawa C, Matsumoto Y, et al: Use of pledget in the repair of diaphragmatic anomalies. J Pediatr Surg 26:84-86, 1991 9. Inselman LS, Mellins RB: Growth and development of the lung. J Pediatr 98:1-15, 1981
Chikara
Tsugawa,
Ken
Kimura,
Eiji
Nishijima,
Toshihiro
Muraji,
and
Masahiro
Yamaguchi
Kobe, Japan
furpose:The purpose of this study is to examine the justification of diaphragmatic plication to treat diaphragmatic eventration. A retrospective review of 50 patients who underwent diaphragmatic plication for phrenic nerve injury (PNI) or congenital muscular deficiency (CMD) of the diaphragm was conducted. Methods: During the last 26 years, 50 patients, aged 4 days to 7 years, were surgically treated for diaphragmatic eventration. Twenty-five patients had iatrogenic PNI and another 25 had CMD. Respiratory distress developed in all patients who had PNI and 10 required mechanical ventilatory support for 13 to 78 days (mean, 41 days) before operation. Respiratory symptoms developed in 17 of 25 patients who had CMD, and four required ventilatory support. In those who were asymptomatic, we justified surgical repair to optimize future lung growth. All patients underwent diaphragmatic plication by a thoracic approach. Reefing mattress sutures on pledgets were used for the plication.
D
IAPHRAGMATIC EVENTRATION is caused by phrenic nerve injury (PNI) or congenital muscular deficiency (CMD) of the diaphragm. Clinical manifestations in infants and children who have diaphragmatic eventration are diverse, ranging from mild gastrointestinal symptoms such as failure to thrive, tachypnea, nausea and vomiting, to life-threatening respiratory distress requiring mechanical ventilatory support. Diaphragmatic plication dramatically improves these symptoms. However, the indication for this procedure remains controversial because some patients are asymptomatic.1-7 This fact motivated us to complete a retrospective review of our patients who had diaphragmatic eventration to determine appropriate indications for diaphragmatic plication. MATERIALS
AND
METHODS
Patients During the period from 197 1 to 1996,50 patients, ranging in age from 4 days to 7 years, were treated for diaphragmatic eventration by diaphragmatic plication at the Kobe Children’s Hospital. Twenty five patients were considered to have PNI as a result of birth trauma (n = lo), surgical injury (n = 12), motor neuron disease (n = 2), and unknown causes (n = 1). The associated anomalies in this group in whom surgery might have caused the PNI were congenital cardiac defects (n = 7), mediastinal tumor (n = 3), esophageal atresia (n = l), and others (n = 1). Respiratory distress developed in all patients of this group after phrenic nerve injury. Ten of these patients needed ventilatory support for 13 to 78 days (mean 41 days), and seven patients required oxygen inhalation for 14 to 90 days before operation. Journal
of Pediatric
Surgery,
Vol32,
No 11 (November),
1997: pp 1643-1644
Results: In patients be discontinued operation, with a status. Two patients tion was not tight but none because Conclusion: who have immediately respiratory J Pediatr Saunders
INDEX injury, tion.
who had PNI, ventilatory support could within 0 to 6 days (mean, 3 days) after dramatic improvement in their respiratory required reoperation because the plicaenough. Seven patients died in this series, of the diaphragmatic plication.
This study suggests that symptomatic patients diaphragmatic eventration should be operated on with an expected dramatic resolution of their problems. Surg 3.21643-1644. Copyright o 7997 by W.B.
Company.
WORDS: Diaphragmatic diaphragmatic muscular
eventration, deficiency,
phrenic surgical
nerve indica-
The age range at operation in this group was 16 days to 5 years (mean, 8 months). The other 25 patients belonged to the group who had CMD of the diaphragm. In this group of patients, the diaphragmatic muscle was either uniformly thin with only its outer edges being normal, or the diaphragmatic muscle fibers were nonresponsive to electric stimulation. The left side of the diaphragm was involved in 13 patients, and the right side in 12 patients. Respiratory distress developed in 17 patients. Four of these patients needed ventilatory support from 3 1 to 210 days (mean, 120 days), and five patients required oxygen inhalation for 4 days to 4 months before operation. Three of seven patients who were free of respiratory distress acquired gastrointestinal symptoms such as vomiting, feeding difficulties, and poor weight gain since birth. The remaining four patients were asymptomatic. The age range at operation was 19 days to 7 years.
Operative
Technique
The diaphragm is approached through a sixth or seventh intercostal space thoracotomy.* In patients who have phrenic nerve injury, diaphragmatic plication is performed using several traction sutures to find the appropriate tension on the diaphragm that is required to eliminate
From the Departments of Surgery and Cardiothoracic Surgery, Kobe Children’s Hospital, Kobe, Japan. Presented at the 30th Annual Meeting of the PaciJc Association of Pediatric Surgeons, Phoenix, Arizona, May 9-13, 1997. Address reprint requests to Chikara Tsugawa, MD, Department of Surgery, Kobe Children 5 Hospital, l-l-l, Takakuradai, Suma-Ku, Kobe 654, Japan. Copyright o 1997 by WE. Saunders Company 0022-3468/97/3211-0030$03.00/O 1643
1644
TSUGAWA
paradoxical motion of the diaphragm. When the appropriate suture placement has been found, tension is released on these sutures. An appropriate number of nonabsorbable sutures on pledgets are placed in a reefing fashion. When these sutures are pulled up for tying, the diaphragm is reefed together in an accordion fashion. In patients who have congenital muscular defects, the electric stimulation is used to demarcate the muscular defect. Several traction sutures are placed at the muscular margin to test the tension on the sutures when the margins are approximated. The same suture materials and suturing technique were used both for patients who had PNI and CMD.
RESULTS
Of the 25 patients who had PNI, 10 patients who had been on mechanical ventilation with endotracheal intubation and seven patients with oxygen inhalation had ventilatory support removed within 0 to 6 days (mean, 3.0 days) after diaphragmatic plication. However, two patients in whom plication was not adequate to eliminate paradoxical respiration relapsed into respiratory distress. In these patients, replication of the diaphragm was performed 3 months and 16 days after the initial operation. The first patient died of respiratory failure from pulmonary damage caused by prolonged mechanical ventilation, and the second recovered. In one patient, the small bowel was injured during plication, and the patient subsequently required a laparotomy. Ultimately, 5 of the 25 patients in this group died: two of cardiac anomalies, two of congenital motor neuron disease, and one of pulmonary failure. Twenty survivors have been followed-up for 1 to 25 years. The involved diaphragm of these patients appeared normal on the chest x-ray films, and none of them has had respiratory distress. Of the 25 patients who had CMD, four who previously required ventilatory support were released from mechanical ventilation within 1 to 61 days (mean, 29 days) after plication. Recurrence of eventration developed in none. Two patients died of cardiac disease. Twenty-three survivors are followed-up radiologically annually to demonstrate synchronous movement of the involved side of the diaphragm. DISCUSSION
For many decades, there has been much controversy regarding conservative versus surgical treatment for diaphragmatic eventration.lm7 Because of the possibility
ET AL
of spontaneous resolution occurring within several months in patients who have PNI, previous reports support conservative management of respiratory distress even in symptomatic patients requiring mechanical ventilatory s~pport.*,~,~Our early patients were treated according to this principle with prolonged preoperative ventilatory support. However, an immediate and dramatic improvement in the respiratory status in patients who underwent a diaphragmatic plication encouraged us to operate on all patients with diaphragmatic eventration. Diaphragmatic plication is technically simple, easy and straightforward with a very low complication rate when carefully performed. 3,6,8A thoracic approach is mandatory for optimal access. Substantial tension to immobilize the plicated diaphragm avoids remaining flail, which reduces the tidal volume. To avoid injury of the intraabdominal organs, a shallow scoop of the diaphragm during placement of plication sutures is important. A retrospective review of our patients indicates that all patients were successfully weaned from ventilatory support within a few days of surgery with remarkable improvement in their respiratory status. In addition, gastrointestinal symptoms disappeared immediately. It is clear from our experience that diaphragmatic plication can eliminate the serious complications that result from both prolonged ventilatory support and malnutrition. In patients suffering from severe cyanosis or cardiac failure caused by associated congenital cardiac defects, reduction in tidal volume induced by diaphragmatic paralysis can be intolerable and fatal. Surgical intervention for asymptomatic patients or patients who have congenital diaphragmatic muscular defect is not recommended in the literature to date.2,4,7 However, when it is considered that the lungs continue to grow until the age of nearly 10 years9 it seems reasonable to provide space for future pulmonary development, although no comparative data on this subject are available as yet. When considering the cost-effectiveness issues, which are important in the era of managed care, prolonged hospitalization only for ventilatory support without providing an added reason for an end point is difficult to rationalize.
REFERENCES 1. Bishop HC, Koop CE: Acquired eventration of the diaphragm in infancy. Pediatrics 22:1088-1096, 1958 2. Wayne ER, Campbell JB, Burrington JD, et al: Eventration of the diaphragm. J Pediatr Surg 9:643-65 1, 1974 3. Schwartz MZ, Filler RM: Plication of diaphragm for symptomatic phrenic nerve paralysis. J Pediatr Surg 13:259-263, 1978 4. Mickell JJ, Oh KS, Siewers RD, et al: Clinical implications of postoperative unilateral phrenic nerve paralysis. J Thorac Cardiovasc Surg 76:297-304, 1978 5. Haller JA Jr, Pickard LR, Tepas JJ, et al: Management of
diaphragmatic paralysis in infants with special emphasis on selection of patients for operative plication. .I Pediatr Surg 14:779-785, 1979 6. Shoemaker R, Palmer G, Brown JW, et al: Aggressive treatment of acquired phrenic nerve paralysis in infants and small children. Ann Thorac Surg 32:251-259, 1981 7. Watanabe T, Tmsler GA, Williams WG, et ah Phrenic nerve paralysis after pediatric cardiac surgery. J Thorac Cardiovasc Surg 94:383-388,1987 8. Kimura K, Tsugawa C, Matsumoto Y, et al: Use of pledget in the repair of diaphragmatic anomalies. J Pediatr Surg 26:84-86, 1991 9. Inselman LS, Mellins RB: Growth and development of the lung. J Pediatr 98:1-15, 1981