Congenital diaphragmatic eventration associated with massive hiatal hernia
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《美国医学杂志》
Division of Pediatric Surgery, College of Medicine, King Faisal University, Dammam, Saudi Arabia
Abstract
Congenital eventration of the diaphragm is uncommon. Its association with a hiatal hernia has not been reported earlier. We report a case of such an association in a 2-month-old boy who presented with tachypnea aggravated by feeding and recurrent vomiting. Diagnostic uncertainty and a unique course of postoperative recovery complicated by gastric volvulus are described. The patient underwent surgical plication of the right diaphragm, followed by an emergency hiatal repair on the first postoperative day. Timely recognition, and repair of the hiatal hernia averted a fatal complication.
Keywords: Diaphragmatic eventration; Hiatal hernia; Massive hiatal hernia; Gastric volvulus
Diaphragmatic eventration is an abnormal elevation of the diaphragm attributable to phrenic nerve injury or congenital muscular hypoplasia.[1] It is classified into (a) complete or partial, depending on the extent of diaphragmatic involvement, (b) congenital or acquired, (c) uni- or bilateral, and (d) adult or pediatric type.[2],[3] Diaphragmatic eventration has been reported in association with recognized and unrecognized patterns of malformation,[4],[5] but as far as the authors are aware, association of this entity with a hiatal hernia has not been reported earlier.
We report a 2 month-old-boy with partial eventration of the right diaphragm associated with massive hiatal hernia and gastric volvulus in a unique presentation and complete recovery.
Case report
A 2-month-old boy, a product of first degree consanguineous marriage, was born by spontaneous vaginal delivery after 38 weeks gestation. He weighed 2580g and Apgar scores were 9 and 10 at 1 and 5 minutes respectively. He was discharged home on the second day after delivery.
The baby was brough back to the hospital for vomiting. He was healthy apart from tachypnea which was aggravated by feeding and was associated with vomiting. There was no cough, cyanosis or history of upper respiratory tract infections.
On examination, the boy was found to be activeand weighed 3810g. Sleeping respiratory rate was 24/minute. Besides decreased air entry in the right lower zone with some coarse crepitation over the left middle zone, general examination was non- contributory.
Chest radiography showed a shadow in the right lower zone with mediastinal shift to the left raising suspicion of polycystic lung. Congenital lung sequestration, emphysema and diaphragmatic hernia were also discussed Figure1. An emergency computer tomogram was in favour of right congenital diaphragmatic hernia. The baby was operated upon the next day after admission through a thoractomy incision. The right lung was normal. The posterior portion of the right diaphragmatic dome was hypoplastic and elevated, while the anterior portion was normal. The diagnosis was revised to eventration, and, surgical plication of the affected part was performed using 2-0 silk.
In the early hours of the first postoperative day, coffee ground material was aspirated from the nasogastric tube, followed by vomiting. Anteroposterior and lateral chest radiograms showed gas shadow in the lower posterior mediastinum. Contrast study revealed gastric volvulus Figure2. An emergency laparotomy through an upper transverse abdominal incision was performed. A massive hiatal hernia with gastric volvulus was reduced. A hiatal repair with a modified Thal fundoplication was performed. The closure of the abdominal wall was under tension. The boy was, therefore, ventilated for 3 days and extubated on the 4th day. He was covered postoperatively with ampicillin, gentamicin and metronidazole. His recovery was uneventful.
Discussion
Diaphragmatic eventration is rare with an incidence of roughly 10% of all congenital diaphragmatic defects[4] The association of an eventration with a hiatal hernia has, to the best of our knowledge, not been reported earlier. Eventration presents with gastrointestinal and/or respiratory symptoms, but, the severity is diverse ranging from mild ro life-threatening.[6],[7]
Plain chest radiograph is usually diagnostic for eventration. Visualization of intact dome of the diaphragm is the most important criterium. Other indicative signs are mediastinal shift and presence of air filled bowel loops in the thorax. The condition can be indistinguishable from a diaphragmatic hernia, pleural effusion as well as spleen, liver and lung pathology.[1],[5] In some cases the use of other imaging diagnostic modalities such as ultrasonography, fluoroscopy, barium meal, computerized tomography, and magnetic resonance imaging have been indicated. At times, diagnosis is made only at explorative thoracotomy.[1],[8]
Associated pulmponary, cardiac, gastrointestinal, renal and musculoskeletal anomalies, with recognized or unrecognized pattern of malformation, were reported in 3% to 40% of cases,[4],[5] which have significant effect on the outcome. This underscores the necessity to perform echocardiography, and, in selected cases karyotyping for accurate assessment and more meaningful management.[4],[5]
Due to a capacious abdominal cavity created by the eventrated diaphragm, the hiatal hernia remained quiescent in this case only to refill symptomatically shortly after diaphragmatic plication had reduced the capacity of abdomen.
The natural history of conservatively managed eventration harbours potential gastric, and less frequently, sigmoid volvulus as complications as a result of increased intra-abdominal space created by the elevated diaphragm.[6],[8] The volvulus observed in this case was certainly the result of an excessive mobility of the stomach within a massive hiatal hernia sac.[9] Immediate surgical repair of all congenital diaphragmatic eventrations is essential because of the potential complications,and frequent illness, and the fact that pulmonary pathological changes become more diffuse with age.[10]
In contrast, the management of the acquired type of diaphragmatic eventration due to phrenic nerve paralysis remains controversial, as spontaneous recovery is expected within 2-6 weeks.[11],[12] During this time, mechanical ventilation may be needed in symptomatic patients. Some prefer immediate surgical plication while others recommend surgical intervention when ventilation is required for longer than two weeks. There is, however, general aggrement that immediate surgical plication shortens stay in intensive care unit, rapidly improves clinical symptoms, and, reduces mortality rate.
Surgical treatment through thoracotomy is the procedure of choice. Some authors recommend the excision of the central thin portion in congenital eventration in order to bring tendino-muscular tissues together, while others recommend plication. Postoperative course is usually uneventful. The diversity of associated congenital anomalies calls for alertness and high index of suspicion. Furthermore, prompt diagnostic action is indispensable, should unforeseen complications, arise these need to be addressed.
References
1. Green DW. Neonatal radiology case book. Neonatal eventration of the diaphragm. J Perinatol 1991; 11: 394-5.
2. Nath J, Jain VK, Mohan S. Eventration of diaphragm with pleural effusion. J Indian Med Assoc 1979; 73: 39-40.
3. Doehler R, Heinemann G: Eventration of the diaphragm with associated accessory lung and intestinal non-roration. Z Kinderchir 1978; 25: 258-62.
4. Cunniff C, Jones KL, Jones MC. Pattern of malformation in children with congenital diaphragmatic defects. J Pediatr 1990; 116: 258-61.
5. Bonham-Carter RE, Waterston DJ, Aberdeen E. Hernia and eventration of the diaphragm in childhood. Lancet 1962; 1: 656-659.
6. Tanner NC. Chronic and recurrent volvulus of the stomach, with late results of "colonic displacement". Am J Surg 1968; 115: 505-515.
7. Malone PS, Brain AJ, Kiely EM, Spitz L. Congenital diaphragmatic defects that present late. Arch Dis Child 1989; 64: 1542-1544.
8. Tsunoda A, Shibusawa M and Koike T. Volvulus of the sigmoid colon associated with eventration of the diaphragm. Am J Gastroenteroll 1992; 87: 1682-1683.
9. Al-Afraj A, Khwaja MS, Updhayaya P. Massive hiatal hernia in children. Eur J Surg 1991; 157: 465-468.
10. Obara H, Hosima H, Iwai S, Ito H, Hisano K. Eventration of the diaphragm in Infants and Children. Acta Pediatric Scand 1987; 76: 654-658.
11. Aldrich TK, Herman JH, Rochester DF. Bilateral diaphragmatic paralysis in the newborn infant. J Pediatr 1980; 97: 988-991.
12. Haller JA, Pickard LR, Tepas JJ, Rogers MC, Robothan JL, Shorter N et al. Management of diaphragmatic paralysis in infants with special emphasis on selection of patients for operative plication. J Pediatr Surg 1979; 14: 779-784.(Al-Arfaj Abdullatif A, Ch)
Abstract
Congenital eventration of the diaphragm is uncommon. Its association with a hiatal hernia has not been reported earlier. We report a case of such an association in a 2-month-old boy who presented with tachypnea aggravated by feeding and recurrent vomiting. Diagnostic uncertainty and a unique course of postoperative recovery complicated by gastric volvulus are described. The patient underwent surgical plication of the right diaphragm, followed by an emergency hiatal repair on the first postoperative day. Timely recognition, and repair of the hiatal hernia averted a fatal complication.
Keywords: Diaphragmatic eventration; Hiatal hernia; Massive hiatal hernia; Gastric volvulus
Diaphragmatic eventration is an abnormal elevation of the diaphragm attributable to phrenic nerve injury or congenital muscular hypoplasia.[1] It is classified into (a) complete or partial, depending on the extent of diaphragmatic involvement, (b) congenital or acquired, (c) uni- or bilateral, and (d) adult or pediatric type.[2],[3] Diaphragmatic eventration has been reported in association with recognized and unrecognized patterns of malformation,[4],[5] but as far as the authors are aware, association of this entity with a hiatal hernia has not been reported earlier.
We report a 2 month-old-boy with partial eventration of the right diaphragm associated with massive hiatal hernia and gastric volvulus in a unique presentation and complete recovery.
Case report
A 2-month-old boy, a product of first degree consanguineous marriage, was born by spontaneous vaginal delivery after 38 weeks gestation. He weighed 2580g and Apgar scores were 9 and 10 at 1 and 5 minutes respectively. He was discharged home on the second day after delivery.
The baby was brough back to the hospital for vomiting. He was healthy apart from tachypnea which was aggravated by feeding and was associated with vomiting. There was no cough, cyanosis or history of upper respiratory tract infections.
On examination, the boy was found to be activeand weighed 3810g. Sleeping respiratory rate was 24/minute. Besides decreased air entry in the right lower zone with some coarse crepitation over the left middle zone, general examination was non- contributory.
Chest radiography showed a shadow in the right lower zone with mediastinal shift to the left raising suspicion of polycystic lung. Congenital lung sequestration, emphysema and diaphragmatic hernia were also discussed Figure1. An emergency computer tomogram was in favour of right congenital diaphragmatic hernia. The baby was operated upon the next day after admission through a thoractomy incision. The right lung was normal. The posterior portion of the right diaphragmatic dome was hypoplastic and elevated, while the anterior portion was normal. The diagnosis was revised to eventration, and, surgical plication of the affected part was performed using 2-0 silk.
In the early hours of the first postoperative day, coffee ground material was aspirated from the nasogastric tube, followed by vomiting. Anteroposterior and lateral chest radiograms showed gas shadow in the lower posterior mediastinum. Contrast study revealed gastric volvulus Figure2. An emergency laparotomy through an upper transverse abdominal incision was performed. A massive hiatal hernia with gastric volvulus was reduced. A hiatal repair with a modified Thal fundoplication was performed. The closure of the abdominal wall was under tension. The boy was, therefore, ventilated for 3 days and extubated on the 4th day. He was covered postoperatively with ampicillin, gentamicin and metronidazole. His recovery was uneventful.
Discussion
Diaphragmatic eventration is rare with an incidence of roughly 10% of all congenital diaphragmatic defects[4] The association of an eventration with a hiatal hernia has, to the best of our knowledge, not been reported earlier. Eventration presents with gastrointestinal and/or respiratory symptoms, but, the severity is diverse ranging from mild ro life-threatening.[6],[7]
Plain chest radiograph is usually diagnostic for eventration. Visualization of intact dome of the diaphragm is the most important criterium. Other indicative signs are mediastinal shift and presence of air filled bowel loops in the thorax. The condition can be indistinguishable from a diaphragmatic hernia, pleural effusion as well as spleen, liver and lung pathology.[1],[5] In some cases the use of other imaging diagnostic modalities such as ultrasonography, fluoroscopy, barium meal, computerized tomography, and magnetic resonance imaging have been indicated. At times, diagnosis is made only at explorative thoracotomy.[1],[8]
Associated pulmponary, cardiac, gastrointestinal, renal and musculoskeletal anomalies, with recognized or unrecognized pattern of malformation, were reported in 3% to 40% of cases,[4],[5] which have significant effect on the outcome. This underscores the necessity to perform echocardiography, and, in selected cases karyotyping for accurate assessment and more meaningful management.[4],[5]
Due to a capacious abdominal cavity created by the eventrated diaphragm, the hiatal hernia remained quiescent in this case only to refill symptomatically shortly after diaphragmatic plication had reduced the capacity of abdomen.
The natural history of conservatively managed eventration harbours potential gastric, and less frequently, sigmoid volvulus as complications as a result of increased intra-abdominal space created by the elevated diaphragm.[6],[8] The volvulus observed in this case was certainly the result of an excessive mobility of the stomach within a massive hiatal hernia sac.[9] Immediate surgical repair of all congenital diaphragmatic eventrations is essential because of the potential complications,and frequent illness, and the fact that pulmonary pathological changes become more diffuse with age.[10]
In contrast, the management of the acquired type of diaphragmatic eventration due to phrenic nerve paralysis remains controversial, as spontaneous recovery is expected within 2-6 weeks.[11],[12] During this time, mechanical ventilation may be needed in symptomatic patients. Some prefer immediate surgical plication while others recommend surgical intervention when ventilation is required for longer than two weeks. There is, however, general aggrement that immediate surgical plication shortens stay in intensive care unit, rapidly improves clinical symptoms, and, reduces mortality rate.
Surgical treatment through thoracotomy is the procedure of choice. Some authors recommend the excision of the central thin portion in congenital eventration in order to bring tendino-muscular tissues together, while others recommend plication. Postoperative course is usually uneventful. The diversity of associated congenital anomalies calls for alertness and high index of suspicion. Furthermore, prompt diagnostic action is indispensable, should unforeseen complications, arise these need to be addressed.
References
1. Green DW. Neonatal radiology case book. Neonatal eventration of the diaphragm. J Perinatol 1991; 11: 394-5.
2. Nath J, Jain VK, Mohan S. Eventration of diaphragm with pleural effusion. J Indian Med Assoc 1979; 73: 39-40.
3. Doehler R, Heinemann G: Eventration of the diaphragm with associated accessory lung and intestinal non-roration. Z Kinderchir 1978; 25: 258-62.
4. Cunniff C, Jones KL, Jones MC. Pattern of malformation in children with congenital diaphragmatic defects. J Pediatr 1990; 116: 258-61.
5. Bonham-Carter RE, Waterston DJ, Aberdeen E. Hernia and eventration of the diaphragm in childhood. Lancet 1962; 1: 656-659.
6. Tanner NC. Chronic and recurrent volvulus of the stomach, with late results of "colonic displacement". Am J Surg 1968; 115: 505-515.
7. Malone PS, Brain AJ, Kiely EM, Spitz L. Congenital diaphragmatic defects that present late. Arch Dis Child 1989; 64: 1542-1544.
8. Tsunoda A, Shibusawa M and Koike T. Volvulus of the sigmoid colon associated with eventration of the diaphragm. Am J Gastroenteroll 1992; 87: 1682-1683.
9. Al-Afraj A, Khwaja MS, Updhayaya P. Massive hiatal hernia in children. Eur J Surg 1991; 157: 465-468.
10. Obara H, Hosima H, Iwai S, Ito H, Hisano K. Eventration of the diaphragm in Infants and Children. Acta Pediatric Scand 1987; 76: 654-658.
11. Aldrich TK, Herman JH, Rochester DF. Bilateral diaphragmatic paralysis in the newborn infant. J Pediatr 1980; 97: 988-991.
12. Haller JA, Pickard LR, Tepas JJ, Rogers MC, Robothan JL, Shorter N et al. Management of diaphragmatic paralysis in infants with special emphasis on selection of patients for operative plication. J Pediatr Surg 1979; 14: 779-784.(Al-Arfaj Abdullatif A, Ch)