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Congenital Morgagni's hernia and associated anomalies

Ahmed H. Al-Salem
Department of Pediatric Surgery, Maternity and Children Hospital, Dammam, Saudi Arabia

 

 

Correspondence

Dr. Ahmed H. Al-Salem FRCSI, FACS, FICS
P. O. BOX 61015
Qatif 31911
Saudi Arabia
FAX: 966 3 8630009
e-mail: This email address is being protected from spambots. You need JavaScript enabled to view it.

 

Abstract

Background: Congenital Morgagni’s hernia (CMH) is very rare. In the pediatric age group, CMH commonly causes recurrent chest infection which can be severe and has a high incidence of associated anomalies.

Patient and method: The medical records of all patients with the diagnosis of CMH treated at our hospital were retrospectively reviewed for: age at diagnosis, sex, presenting symptoms, associated anomalies, diagnosis, operative findings, treatment and outcome.

Results: During an 18 year period (January 1989 - December 2007), 23 cases with the diagnosis of CMH were treated at our hospital. There were 16 males and 7 females. Their age at time of diagnosis ranged from 28 days to 9 years (mean 14 months) and the majority of them (70%) were below 1 year of age. Ninteen of them (82.6%) presented with recurrent chest infection which was since early infancy in the majority of them. Of the 23 patients, 9 had right-sided Morgagni’s hernia, 6 were left-sided and 8 had bilateral hernias. In 5 of those with bilateral hernia, the diagnosis of bilaterality was made at the time of surgery. Associated anomalies were seen in 18 (78.3%) of our patients. Eight (34.8%) had congenital heart disease, 5 (21.7%) had malrotation of bowel and 6 (26%) had Down’s syndrome. All our patients were operated on transabdominally (14 upper midline, 7 upper transverse) except two. One had a right thoracotomy while the other underwent laparoscopic repair. In all there was a hernial sac which was excised and the defect was repaired using non absorbable sutures. The contents of the hernial sac included colon, small intestines, part of the liver and omentum with the colon being the commonest (82.6%). There was no mortality and post-operatively all patients did well. One of our patients required postoperative ventilation for 5 days because of associated lung hypoplasia. On follow-up, 2 patients (8.7%) developed recurrence and one of them developed incisional hernia.

Conclusions: CMH is very rare. In the pediatric age group the presentation is commonly recurrent chest infection. Physicians caring for these patients should be aware of this and a high index of suspicion is recommended to obviate delay in diagnosis with its associated morbidity. CMH has a high incidence of associated anomalies, namely congenital heart disease, malrotation and Down’s syndrome. Down’s syndrome may have a negative influence on the final outcome in the form of increased recurrence. We advocate a transabdominal approach either via an upper midline or an upper transverse incision. This allows easily reduction and inspection of contents, allows access and repair of bilateral hernias and correct an associated malrotation if present.

Key words: congenital diaphragmatic hernia, congenital Morgagni’s hernia, associated anomalies, treatment.

  

Introduction:

Congenital Morgagni’s hernia (CMH) is relatively rare when compared with other types of congenital diaphragmatic hernia and although commonly diagnosed in the pediatric age group, it can remain asymptomatic till adulthood. In the pediatric age group, CMH comprises about 1% - 5% of all types of congenital diaphragmatic hernia and can be discovered incidentally or cause vague gastrointestinal symptoms but more commonly it causes respiratory symptoms which can be severe especially during infancy (1,2,3). An interesting feature of CMH is the high incidence of associated anomalies (3,4,5). This as well as its occurrence in identical twins raised the possibility that it may result from an inherited defect (6). This is a review of our experience with CMH highlighting associated anomalies and their influence on outcome.

Patient and method

This is a retrospective review of all infants and children admitted to our hospital with the diagnosis of CMH. Their medical charts were reviewed for: age at diagnosis, sex, side of hernia, presenting symptoms and signs, associated anomalies, radiological features, intraoperative findings, postoperative complications, and outcome were recorded.

Results

Over a period of 18 years from January, 1989 to December, 2007, 23 infants and children with the diagnosis of CHM were treated at our hospital. There were 16 males and 7 females (M: F 2.3:1). Their age at diagnosis ranged from 28 days to 9 years (mean 14 months) and the majority of them (70%) were below 1 year of age. Nineteen of them (82.6%) presented with recurrent chest infection which was since early infancy in the majority of them. One of them presented at the age of 28 days with acute respiratory distress, poor feeding, tachycardia and vomiting. In the other 4 patients the diagnosis of CHM was an incidental finding on chest x-ray. This was following a road traffic accident in one, following splenectomy in one, following a ventriculo-peritoneal shunt in one and an incidental finding on chest x-ray in the fourth. Of the 23 patients, 9 had right-sided CMH, 6 were leftsided and 8 (34.8%) had bilateral hernias. In 5 (62.5%) of those with bilateral hernia, the diagnosis of bilaterality was made at the time of surgery. In all our patients with bowel herniation into the chest, the diagnosis was suspected on chest x-ray especially a lateral chest x-ray and the diagnosis was confirmed by barium enema or meal and follow-through ( fig 1).

morgagni hernia 1 morgagni hernia 2

Fig. 1. Barium enema showing herniation of large intestines into the chest. 

In those with herniation of solid contents namely part of the liver or omentum, the diagnosis was made on CT-scan of the chest ( fig 2). In one of our patients, the diagnosis was made intraoperatively. He had a right-sided soft tissue density mass in the right cardiophrenic angle, and via a right thoracotomy, he was found to have a right CMH with herniation of part of the left lobe of liver. Associated anomalies were seen in 18 (78.3%) of our patients as shown in table 1. Eight (34.8%) had congenital heart disease, 5 (21.7%) had malrotation of bowel and 6 (26%) had Down’s syndrome. All those with bilateral CMH had associated anomalies when compared with those with bilateral CMH as only 10 (66.7%) of them had associated anomalies as shown in table 2.

morgagni hernia 3

Fig. 2. CT-scan showing herniation of part of the liver through a Morgagni’s hernia. 

Table 1. Associated anomalies

 No.  ASSOCIATED ANOMALIES  NO. OF PATIENTS
 1  CONGENITAL HEART DISEASE
ATRIAL SEPTAL DEFECT
VENTRICULAR SEPTAL DEFECT
PULMONARY STENOSIS
PATENT DUCTUS ARTERIOSUS

8
6

2

1

34.8
26
8.7
4.3
4.3 
 2  MALROTATION 21.7 
 3  DOWN’S SYNDROME  6  26
 4  INGUINAL HERNIA 21.7 
 5 BETA-THALASSEMIA MAJOR  4.3 
 6  HYDROCEPHALUS 4.3 
 7  UMBILICAL HERNIA  1  4.3
 8  PYLORIC STENOSIS  1  4.3
 9  HEMANGIOMA  1  4.3
 10  HYPOSPADIAS  1  4.3
 11  ANORECTAL MALFORMATION  1  4.3

 

Table 2. Associated anomalies in those with unilateral cmh and those with bilateral CMH

ASSOCIATED ANOMALY BILATERAL CMH (8) % UNILATERAL CMH
(15)
%
Overall 8 100 10 66.7
Congenital heart disease 5 62.5 3 20
Down’s syndrome 4 50 2 13.3
Malrotation 3 37.5 2 13.3

One of our patients had hypoplasia of the left lung on a lung perfusion scan and another had deformity of the anterior chest wall. In both, this was secondary to a large CMH. All our patients were operated on transabdominally (14 upper midline, 7 upper transverse) except two. One had a right thoracotomy while the other underwent laparoscopic repair. In all, there was a hernial sac which was excised and the defect was repaired using non absorbable sutures. The contents of the hernial sac included the colon, part of the liver, omentum, stomach and small intestines, with the colon being the commonest (82.6%). There was no mortality and post-operatively all patients did well. The patient with associated lung hypoplasia required postoperative ventilation for 5 days. On follow-up, two (8.6%) of our patients with Down’s syndrome developed recurrence of the hernia. These were subsequently repaired. One of them also developed an incisional hernia.

Discussion

Anterior congenital diaphragmatic hernia through the foramen of Morgagni which was first described by Giovani Morgagni in 1761 is very rare comprising 3%-5% of all surgically treated congenital diaphragmatic hernias [1, 2, and 7]. Over a period of 40 years, Berman et al [5] treated only 18 cases of CMH and Pokorny et al [3] saw only 4 cases of CMH over a period of 25 years. Cigdem et al over a period of 23 years treated 16 cases [3].Over a period of 18 years, we treated 23 cases of CMH.

The majority (90%) of CMH occur on the right side, 2% on the left side and 8% are bilateral [7]. The rarity of CMH on the left side is due to the pericardial attachment to the diaphragm which is more on the left side giving support and protection to that side. Of interest was the finding of 34.8 % with bilateral hernias in our series. The reason for the high incidence of bilaterality in our series is not known. In the pediatric age group, the presentation of CMH is variable and non-specific. Commonly, the presentation is that of recurrent chest infection and rarely may present with gastrointestinal symptoms. Physicians (pediatricians and general practitioners) caring for these patients should be aware of this and a high index of suspicion is recommended to obviate delay in diagnosis with its associated morbidity. The majority of our patients suffered repeated attacks of chest infection, received several courses of antibiotics and some of them were hospitalized several times.

There is an increased risk of associated anomalies with Morgagni’s hernia but the incidence is variable ranging from 34% to 50% [5]. Eighteen (78.3%) of our patients had associated anomalies. Congenital heart disease continues to be the commonest associated anomaly. This was the case in our series as 34.8 % of our patients had congenital heart disease with an overall 45.5 % of heart defects. The severity of congenital heart disease was variable but none of them had major life threatening defects and ASD and VSD were the commonest associated heart defects. This however did not influence the final outcome and had no effect on the overall morbidity. The high frequency of associated congenital heart defects calls for a thorough cardiac evaluation of these patients including a preoperative echocardiogram. An interesting association was that of CMH and Down’s syndrome. In a collective series of 46 children with Morgagni’s hernia, 16 (34.8%) of them had Down’s syndrome [8]. Cigden et al in a series of 16 patients with CMH seen over a period of 23 years reported a 31.25% incidence of Down’s syndrome [4]. Six (26%) of our patients had Down’s syndrome. The association of Down’s syndrome and CMH is interesting. Infants with Down’s syndrome are known to have a high frequency of associated congenital malformations which generally occur with a much higher frequency than in the general population.

A causal relationship may exist between Down’s syndrome and other congenital malformations including esophageal atresia and anorectal malformations. This may be the case also with Down’s syndrome and CMH [6]. The hypotonic muscle development which is a well established developmental defect in those with Down’s syndrome may play a role in the development of CMH. This association definitely affected the final outcome and the poor hypotonic muscle development in them has resulted in recurrence in two of our patients and an incisional hernia in one of them. The association of CMH and malrotation is well established. Five of our patients (21.7%) of our patients had malrotation. This fact is of paramount importance when considering the operative approach to repair CMH. An interesting finding was the remarkably high incidence of associated anomalies in those with bilateral CMH. In our series, only 10 (66.7%) of those with unilateral CMH had associated anomalies when compared to a 100% incidence in those with bilateral CMH. The reason for this variation is not known.

There is a general consensus that in symptomatic patients, CMH should be treated surgically. Opinions however differ as to the best surgical approach, whether trans-thoracic or trans-abdominal [3, 9, 10]. We advocate a trans-abdominal approach either via an upper midline or an upper transverse incision. This will make reduction and inspection of contents easy, allows access and repair of bilateral hernias simultaneously and correct an associated malrotation if present. Such an approach is supported by the fact that in 5 of our 8 patients with bilateral CMH, the diagnosis of bilaterality was only made intraoperatively and 4 of our patients had associated malrotation that was corrected simultaneously. The recent advances in minimal invasive surgery has made laparoscopic repair of CMH safe and effective in adults and recently in children [11, 12, 13, 14, 15]. Only one of our patients (the last patient seen) underwent laparoscopic repair.

 

 

 

REFERENCES 

  1. Cullen ML, Klein MD, Philippart AI: Congenital diaphragmatic hernia. Surg Clin North America 1985, 65: 1135-1138
  2. Simson JNL, Eckstein HB: Congenital diaphragmatic hernia: A 20 years experience. Br J Surg 1985, 72: 733-736
  3. Pokorny WJ, McGill CW, Harberg FJ: Morgagni hernia during infancy: presentation and associated anomalies. J Pediatr Surg 1984, 19: 394-397
  4. Cigdem MK, Onen A, Okur H, Otcu S: Associated malformation in Morgagni hernia. Pediatr Surg Int 2007, 23:1101-1103
  5. Berman L, Stringer DA, Ein SH, Shandling B: The late presenting pediatric Morgagni hernia: a benign condition. J Pediatr Surg 1989, 24: 970-972
  6. Harris GJ, Soper RT, Kimura KK: Foramen of Morgagni hernia in identical twins: Is this an inheritable defect? J Pediatr Surg 1993, 28: 177-178
  7. Federico JA, Ponn RB: Foramen of Morgagni hernia. In Shields TW, LoCicero III J, Ponn RB, eds. General thoracic Surgery. 5th ed. Philadelphia: Lippincott Williams and Wilkins, 2000: 647-660
  8. Kubiak R, Platen C, Schmid E, et al: Delayed appearance of bilateral Morgagni’s hernia in a patient with Down’s syndrome. Pediatr Surg Int 1998,13: 600-601
  9. Kurkcuoglu IC, Eroglu A, Karaoglanoglu N, et al: Diagnosis and surgical treatment of Morgagni hernia: report of three cases. Surg Today 2003, 33: 525-528
  10. Kilic D, Nadir A, Doner E, et al: Transthoracic approach in surgical management of Morgagni hernia. Eur J Cardiothoracic Surg 2001, 20: 1016-1019
  11. De Vogelaere, Kristel MD: Laparoscopic repair of Morgagni diaphragmatic hernia: A new case. Surgical Laparoscopy, endoscopy and percutaneous techniques 2003, 13(6): 401-403
  12. Lima M, Domini M, Libri M, et al: Laparoscopic repair of Morgagni- Larry hernia in a child. J Pediatr Surg 2000, 35:1266-1268
  13. Georgacapulo P, Franchella A, Mandridi G, et al: Morgagni-Larry hernia correction by Laparoscopic surg. Eur J Pediatr Surg 1997, 7: 241- 242
  14. Rau HG, Schardley HM, Lang V: Laparoscopic repair of Morgagni hernia. Surg Endosc 1994, 8: 1439-1442
  15. Rau HG, Schardley HM, Lang V: Laparoscopic repair of Morgagni hernia. Surg Endosc 1994, 8: 1439-1442

 

Table 1. Associated anomalies