Journal of Pediatric Surgery 49 (2014) 503–507
Contents lists available at ScienceDirect
Journal of Pediatric Surgery journal homepage: www.elsevier.com/locate/jpedsurg
Original Articles
Congenital Morgagni's hernia: A national multicenter study Ahmed H. Al-Salem a,⁎, Mohammed Zamakhshary b, Mohammed Al Mohaidly c, Aayed Al-Qahtani d, Mohamed Ramadan Abdulla a, Mohamed Ibrahim Naga a a
Department of Pediatric Surgery, Maternity and Children Hospital, Dammam, Saudi Arabia Pediatric Surgery and Public Health, National Guard Hospital, Riyadh, Saudi Arabia and Alfaisal University, Emory University, and Ministry of Health, Riyadh, Saudi Arabia Department of pediatric Surgery, Military Hospital, Riyadh, Saudi Arabia d Department of pediatric Surgery, King Khalid University Hospital, Riyadh, Saudi Arabia b c
a r t i c l e
i n f o
Article history: Received 25 June 2013 Received in revised form 11 August 2013 Accepted 20 August 2013 Key words: Congenital diaphragmatic hernia Congenital Morgagni's hernia Associated anomalies Treatment
a b s t r a c t Background: Congenital Morgagni's hernia (CMH) is rare and represents less than 5% of all congenital diaphragmatic hernias. This is a national review of our experience with CMH outlining clinical presentation, methods of diagnosis, associated anomalies, treatment, and outcome. Patients and methods: The medical records of all patients with the diagnosis of CMH treated at four pediatric surgery units in Saudi Arabia were retrospectively reviewed for age at diagnosis, sex, presenting symptoms, associated anomalies, diagnosis, operative findings, treatment, and outcome. Results: During a 20-year period (January 1990–December 2010), 53 infants and children with CMH were treated. There were 38 males and 15 females. Their age at diagnosis ranged from 1 month to 9 years (mean 22.2 months). Forty-three (81%) presented with recurrent chest infection. Twenty-two (44.5%) had right CMH, 15 (28.3%) had left-sided hernia and 16 (30.2%) had bilateral hernia. In 7, the diagnosis of bilaterality was made at the time of surgery. Associated anomalies were seen in 38 (71.7%). Twenty-one (39.6%) had congenital heart disease, 8 (15%) had malrotation, and 15 (28.3%) had Down syndrome. All were operated on. Twenty-nine (54.7%) underwent repair via an open approach. The remaining 24 (45.3%) underwent repair using minimal invasive surgery, laparoscopic-assisted hernia repair (19 patients) or totally laparoscopic approach (5 patients). At the time of surgery, the hernia sac content included the colon in 33 (62.3%), part of the left lobe of the liver in 13 (24.5%), the small intestines in 11 (20.75%), the omentum in 5 (9.4%), and the stomach in 4 (7.5%). In 12 (22.6%), the hernia sac was empty. When compared to the open repair, the laparoscopic-assisted approach was associated with a shorter operative time, an earlier commencement of feeds, less requirement for postoperative analgesia, a shorter hospital stay, and better cosmetic appearance. There was no mortality. On follow-up, 2 (7%) of the open surgical group developed recurrence. Conclusions: CMH is rare and in the pediatric age group commonly presents with recurrent chest infection and has a high incidence of associated anomalies, commonly congenital heart disease and Down syndrome. We advocate a laparoscopic-assisted approach to repair CMH. This is a simple technique that produces a sound repair, and when compared with the open approach it takes less operative time, requires less analgesia, allows earlier commencement of feeds, is associated with a shorter hospital stay, and has a better cosmetic outcome. © 2014 Elsevier Inc. All rights reserved.
Congenital Morgagni's hernia was first described by Giovani Morgagni in 1761 [1,2]. It is a congenital herniation of abdominal contents into the thoracic cavity through a retrosternal diaphragmatic defect. CMH is relatively rare and make up about 1%–5% of all types of congenital diaphragmatic hernia, and although commonly diagnosed in the pediatric age group, it can remain asymptomatic till adulthood [3,4].
⁎ Corresponding author. Qatif 31911, Saudi Arabia. Fax: +966 38630009. E-mail addresses: [email protected] (A.H. Al-Salem), [email protected] (M. Zamakhshary), [email protected] (M. Al Mohaidly), [email protected] (A. Al-Qahtani), [email protected] (M.R. Abdulla), [email protected] (M.I. Naga). 0022-3468/$ – see front matter © 2014 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.jpedsurg.2013.08.029
In the pediatric age group, CMH has unique features in terms of clinical presentation, high incidence of bilaterality and associated anomalies. CMH commonly presents with repeated attacks of chest infection and rarely vague, nonspecific gastrointestinal symptoms but at times it remains asymptomatic or discovered accidently during evaluation of other nonrelated conditions. The rarity of CMH and the vague nonspecific symptoms are two important factors leading to delayed diagnosis. CMH nevertheless can cause significant morbidity [2]. This is specially so if the child is not adequately investigated. Awareness of this is of paramount importance. During infancy CMH however can lead to acute respiratory distress indistinguishable from that of Bochdalek hernia [5]. The treatment of CMH is surgical repair either conventionally by open abdominal or thoracic approaches, or more recently, by using
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A.H. Al-Salem et al. / Journal of Pediatric Surgery 49 (2014) 503–507
minimal invasive surgery [2,6–11]. This study is a national review of our experience with CMH highlighting aspects of presentation, diagnosis, associated anomalies and their influence on outcome. The methods of treatment are discussed and a comparison between the conventional open technique and the laparoscopic-assisted repair of CMH is presented. 1. Patients and methods Four pediatric surgery units in the kingdom of Saudi Arabia participated in this study. The medical records of all patients with the diagnosis of CMH during the study period from January 1990 to December 2010 were retrospectively reviewed and the following information was collected: age at diagnosis, sex, clinical presentation, associated anomalies, site of hernia, and methods of repair, postoperative complications and outcome. 2. Results During the study period, 53 infants and children with CMH were treated. There were 38 males and 15 females (M/F of 2.5:1). Their mean age at diagnosis was 22.2 months (1 month–120 months). The majority (81%) presented with recurrent chest infection (Table 1). Five had nonspecific upper respiratory tract symptoms and two were treated as gastroesophageal reflux disease prior to diagnosis. In one, the hernia was discovered incidentally. One of our patients had a road traffic accident with blunt abdominal trauma and an abdominal CT scan noted bilateral CMH. This was confirmed intraoperatively where bilateral CMH with hernia sacs were found containing intestines. The last patient had beta-thalassemia major and underwent splenectomy because of frequent blood transfusion. Following this and as a result of progressive liver enlargement, the left lobe of the liver herniated into an already existing left Morgagni's hernia. Twenty-two (41.5%) patients had right-sided CMH, 15 (28.3%) had left-sided hernia and 16 (30.2%) had bilateral hernia. In those with bilateral hernias, the diagnosis of bilaterality was made at the time of surgery in 7. Associated anomalies were seen in 38 (71.7%) as shown in Table 2. A single associated anomaly was seen in 17 while 21 had more than one anomaly. Congenital heart disease was the commonest associated anomaly seen in 21 patients (39.6%). Down syndrome was noted in 15 patients (28.3%) and all of them had congenital heart disease. Eight (15%) had associated inguinal hernia, 4 (7.5%) had umbilical hernia and 2 had hydrocephalus. In the majority (50 patients), plain chest radiographs (anteroposterior and lateral views) were diagnostic showing anterior herniation of bowel into the chest (Fig. 1). Additional diagnostic investigations included barium enema (12 patients), barium meal and followthrough (7 patients) and CT scan of chest (5 patients) (Figs. 2 and 3). All patients underwent operative repair. The method of repair changed over the years. In the initial stages of the study, 29 (54.7%) underwent repair via an open approach. The remaining 24 patients underwent repair using a minimally invasive approach with laparoscopic-assisted hernia repair (19 patients) or totally laparoscopic
Table 1 Clinical features of patients with congenital Morgagni's hernia. Clinical features
No. of patients %
Recurrent chest infection 43 Nonspecific upper respiratory tract symptoms 5 Gastroesophageal reflux disease 2 Discovered incidentally 1 Following road traffic accident with blunt abdominal 1 trauma Following splenectomy 1
81 9.4 3.8 1.9 1.9 1.9
Table 2 Associated anomalies. Associated anomalies
No. of patients
%
Congenital heart disease Down's syndrome Inguinal hernia Malrotation Umbilical hernia Hypospadias Anorectal malformation Hydrocephalus Undescended testes Hemangioma Scoliosis
21 15 8 8 4 4 2 2 1 2 1
39.6 28.3 15 15 7.5 7.5 3.8 3.8 1.9 3.8 1.9
approach (5 patients). A comparison between the open approach and the laparoscopic-assisted approach is shown in Table 3. At the time of surgery, the hernia sac content included the colon in 33 (62.3%), part of the left lobe of the liver in 13 (24.5%), the small intestines in 11 (20.75%), the omentum in 5 (9.4%) and the stomach in 4 (7.5%). In 12 (22.6%), the hernia sac was empty. In all patients who had open repair, the hernia sac was excised and the repair was done using nonabsorbable sutures. The hernia sac was not excised and plicated in those who had minimal invasive surgery. Postoperatively, all did well except two patients in the open-surgery group (7%) who developed recurrence and required reoperation. One of them also had incisional hernia which was repaired. Both of them had Down syndrome. 3. Discussion Congenital diaphragmatic hernia through the foramen of Morgagni is an interesting condition from several aspects including its rarity, high incidence of bilaterality, presentation, and high incidence of associated anomalies [12–15]. It is relatively rare comprising 3%–5% of all surgically treated congenital diaphragmatic hernias. Over a period of 40 years, Berman et al. [15] treated only 18 cases of CMH and Pokorney et al. [5] saw only 4 cases over a period of 25 years. Cigdem et al. [12] over a period of 23 years treated 16 cases of CMH. Al-Salem [13] over a period of 18 years treated 23 cases of CMH. The exact incidence of CMH in Saudi Arabia is not known. In a previous study, CMH made up about 11% of all types of congenital diaphragmatic hernia [16]. This is relatively high when compared to that reported internationally. One contributing factor for this is the high rate of consanguinity in this part of the world. The majority (90%) of CMH occur on the right side and 2% on the left side and 8% are bilateral. The rarity of CMH on the left side is caused by the pericardial attachment to the diaphragm which is more on the left side giving support and protection to that side. Twenty-two (41.5%) of our patients had right-sided hernia, 15 (28.3%) had leftsided hernia and 16 (30.2%) had bilateral hernia. The reason for the high frequency of bilateral hernias in our patients is not known. An interesting point in our series is the high frequency of bilateral hernias diagnosed intraoperatively. One reason for this is the presence of an empty sac at the time of radiological evaluation. This must be kept in mind and looked for intraoperatively. Another interesting point is the increased incidence of associated anomalies in patients with CMH but the incidence is variable ranging from 34% to 50% [5,12,13]. Thirty-eight (71.7%) of our patients had associated anomalies. This is higher than that reported internationally. Congenital heart disease continues to be the commonest associated anomaly. This was the case in our series as 21 (39.6 %) of our patients had congenital heart disease. The severity of associated congenital heart disease was variable but none of them had major lifethreatening defects and atrial septal defect and ventricular septal defect were the commonest associated heart defects. This however
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Fig. 2. Barium enema showing anterior herniation of colon into the chest.
between Down syndrome and CMH. The hypotonic muscle development in those with Down syndrome may play a role in the development of CMH and also may play a role in recurrence. In two of our patients there was recurrence and one of them had an incisional hernia. Both of them were Down syndrome patients. The association of CMH hernia and malrotation is well established. Eight (15%) of our patients had malrotation. This fact is of paramount importance when considering the operative approach to repair Morgagni's hernia. An interesting finding was the remarkably higher incidence of associated anomalies in those with bilateral CMH [14]. In our series, 23 (62.2%) of those with unilateral CMH had associated anomalies when compared to a 93.75% incidence in those with bilateral CMH. The reason for this variation is not known. The presentation of CMH is variable and nonspecific. Physicians caring for these patients should be aware of this and a high index of suspicion is important to obviate delay in diagnosis with its associated morbidity. CMH can be discovered incidentally or cause vague gastrointestinal complaints but more commonly it causes respiratory symptoms with repeated chest infections [2]. This was the case in our series as the majority of our patients presented with repeated attacks of chest infections necessitating several courses of antibiotics and hospitalizations in some of them. In the neonatal period, CMH can present acutely resembling that of Bochdalek hernia [5]. None of our
Fig. 1. Anteroposterior and lateral chest x-ray showing anterior herniation of bowel loops into the chest.
had no effect on the overall morbidity and final outcome. The high frequency of associated congenital heart defects calls for a thorough cardiac evaluation of these patients including a preoperative echocardiogram. The association of CMH and Down syndrome is interesting. In a collective series of 46 children with CMH, 16 (34.8%) of them had Down syndrome [17]. Cigdem et al. [12] in a series of 16 patients with CMH seen over a period of 23 years reported a 31.25% incidence of Down syndrome. In our series 15 (28.3%) of our patients had Down syndrome. A causal relationship has been suggested
Fig. 3. Abdominal CT scan showing anterior herniation of part of the liver into the chest (marked by arrows).
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Table 3 Comparison between laparoscopic-assisted and open-surgery repair for Morgagni's hernia in infants and children. Variable Total no. of patients Sex Site of hernia
Laparoscopic-assisted group
20 13 M/7 F Right: 8 Left: 9 Bilateral: 3 Median age (range) 15 months (8–42 months) Median operative time 40 minutes (range) (27–95 minutes) Median time to 24 hours full feed (range) (20–36 hours) Requirement for Only 1 required analgesia pethedine Median hospital stay 48 hours (range) (36–168 hours) Complications None
Open-surgery group 28 22 M/6 F Right: 9 Left: 6 Bilateral: 13 10 months (1–60 months) 90 minutes (60–120 minutes) 50 hours (40–75 hours) All required pethedine 120 hours (96–240 hours) 2 (7%) recurrence
p Value
b0.0001 b0.0001 – b0.0001 0.50
patients presented acutely and CMH was asymptomatic discovered incidentally in only one of our patients. In four of our patients, CMH was precipitated by an increase in intraabdominal pressure. In one this was the result of blunt abdominal trauma which resulted in herniation of intestines into an already existing bilateral CMH. Two patients had hydrocephalus with a ventriculoperitoneal shunt and we feel this contributed to the appearance of the hernia by increasing the intraabdominal pressure. The fourth patient is a case of betathalassemia major who underwent splenectomy which lead to progressive hepatomegaly and herniation of part of the left lobe of the liver. There is a general consensus that in symptomatic patients, CMH should be treated surgically. Opinions however differ as to the treatment of those with asymptomatic hernia. We like others advocate repair of asymptomatic patients. This is to obviate the potential complication of strangulation in spite of its low frequency [18–20]. A still controversial point in the management of patients with CMH is the surgical approach, whether transthoracic or transabdominal [2,7]. In the past, we advocated the transabdominal 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 corrects an associated malrotation if present. Such an approach is supported by the fact that in 7 of our 16 patients with bilateral CMH, the diagnosis of bilaterality was only made intraoperatively and 5 of our patients had associated malrotation that was corrected simultaneously. The recent advances in minimal invasive surgery have made laparoscopic repair of CMH safe and effective both in children and in adults. Georgacapulo et al. [10] in 1997 reported the first successful laparoscopic repair of CMH in a child. A variety of techniques to repair CMH laparoscopically have been described including primary closure with a continuous suture, interrupted suture or using a mesh [21–25]. The laparoscopic interrupted or continuous suture technique to repair CMH is not simple and is time consuming. Add to this the fact that the anterior edge of the defect is not well developed and sometimes absent. This makes direct closure of the defect weak and at risk to breakdown. One way to overcome this is to anchor the sutures to the back of the sternum and costal margin. This however is also technically difficult. A better and simpler approach is to take the sutures through the posterior edge of the hernia defect and incorporate the entire anterior abdominal wall and tie the knots extracorporealy in the subcutaneous tissues [24,26]. This technique was used in the majority of our patients treated laparoscopically and in none was it necessary to use a prosthetic patch. This technique is simple and when compared to the open repair, it is definitely better. It takes a shorter operative time, and produces a sound repair. Add to
this, earlier commencement of feeds, less requirement for postoperative analgesia, a shorter hospital stay and better cosmetic appearance [26]. Robotic-assisted laparoscopic repair of CMH was also reported to be safe and feasible in children but the set-up time prolongs the procedure and the overall operative time is longer than the laparoscopic-assisted repair [27,28]. Another controversial issue at the time of repair is whether to excise or leave the associated hernia sac [2,21,22,26,29]. In all our cases treated via the open approach, the hernia sac was excised. This however was not the case for those treated via the laparoscopicassisted approach as none of the hernia sacs were excised. On followup however, two of the open-surgery group developed hernia recurrence while none of the laparoscopic-assisted group recurred. CMH is a relatively rare type of diaphragmatic hernia that usually presents with recurrent attacks of chest infection. Its rarity and the vague nonspecific symptoms are two contributing factors for delayed diagnosis. 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, and among these congenital heart diseases is the commonest. Awareness of this is important and echocardiography should be part of the preoperative investigations. The reason for the increased number of bilateral hernias in our series is not known. CMH should be repaired surgically even in asymptomatic cases. We advocate repairing CMH using the laparoscopic-assisted approach. This is a simple technique that produces a sound repair and when compared with the open approach takes less operative time, requires less analgesia, allows early commencement of feeds, and results in a shorter hospital stay and better cosmesis.
References [1] Federico JA, Ponn RB. Foramen of Morgagni hernia. In: Shields TW, Lo Cicero III J, Ponn RB, editors. General thoracic surgery, 5th ed. Philadelphia: Lippincott Wilkins; 2000. p. 647–66. [2] Al-Salem AH. Congenital hernia of Morgagni in infants and children. J Pediatr Surg 2007;42:1539–43. [3] Cullen ML, Klein MD, Philippart AI. Congenital diaphragmatic hernia. Surg Clin N Amer 1985;65:1135–8. [4] Simson JNL, Eckstein HB. Congenital diaphragmatic hernia: a 20 year experience. Br J Surg 1985;72:733–6. [5] Pokorny WJ, McGill CW, Harberg FJ. Morgagni hernia during infancy: presentation and associated anomalies. J Pediatr Surg 1984;19:394–7. [6] Kurkcuoglu IC, Eroglu A, Karaoglanoglu N, et al. Diagnosis and surgical treatment of Morgagni hernia: report of three cases. Surg Today 2003;33:525–8. [7] Kilic D, Nadir A, Doner E, et al. Transthoracic approach in surgical management of Morgagni hernia. Eur J Cardiothorac Surg 2001;20:1016–9. [8] Vogelaere De, Kristel MD. Laparoscopic repair of Morgagni diaphragmatic hernia: a new case. Surg Laparosc Endosc Perc Techn 2003;13:401–3. [9] Lima M, Domini M, Libri M, et al. Laparoscopic repair of Morgagni–Larry hernia in a child. J Pediatr Surg 2000;35:1266–8. [10] Georgacapulo P, Franchella A, Mandridi G, et al. Morgagni–Larry hernia correction by laparoscopic surgery. Eur J Pediatr Surg 1997;7:241–2. [11] Rau HG, Schardley HM, Lang V. Laparoscopic repair of Morgagni hernia. Surg Endosc 1994;8:1439–42. [12] Cigdem MK, Onen A, Okur H, et al. Associated malformation in Morgagni hernia. Pediatr Surg Int 2007;23:1101–3. [13] Ahmed Al-Salem. Congenital Morgagni's hernia and associated anomalies. J Pediatr Surg Specialities 2009;3(3):173–85. [14] Al-Salem AH. Bilateral Journal of congenital Morgagni–Larrey's hernia. World J Pediatr 2010;6:76–80. [15] Berman L, Stringer DA, Ein SH, et al. The late presenting pediatric Morgagni hernia: a benign condition. J Pediatr Surg 1989;24:970–2. [16] Al-Salem AH, Nawaz A, Matta H, et al. Herniation through the foramen of Morgagni: early diagnosis and treatment. Pediatr Surg Inter 2002;18:93–7. [17] 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;13:600–1. [18] Gangopadhyaya AN, Upadhyaya VD, Gupta DK, et al. Obstructed Morgagni's hernia. Indian J Pediatr 2007;74(12):1109–10. [19] Vaos G, Skondras C. Colonic necrosis because of strangulated recurrent Morgagni's hernia in a child with Down's syndrome. J Pediatr Surg 2006;41:589–91. [20] Cakmak O, Pektas O, Baskin D. Retosternal hernia (Morgagni) with colonic perforation due to incarceration. Pediatr Surg Int 1990;5:274–5. [21] Huntington TR. Laparoscopic transabdominal pre-peritoneal repair of Morgagni. J Laparoendosc 1996;6:131–3.
A.H. Al-Salem et al. / Journal of Pediatric Surgery 49 (2014) 503–507 [22] Orita M, Okino M, Yamashita K, et al. Laparoscopic repair of a diaphragmatic hernia through the foramen of Morgagni. Surg Endosc 1997;11:668–70. [23] Bortul M, Calligaris L, Gheller P. Laparoscopic repair of a Morgagnilarrey hernia. J Laparoendosc Adv Surg Techn 1998;8:309–13. [24] Azzie G, Maoate K, Beasley S, et al. A simple technique of laparoscopic fullthickness anterior abdominal wall repair of retrosternal (Morgagni) hernia. J Pediatr Surg 2003;38:768–70. [25] Dutta S, Albanese CT. Use of a prosthetic patch for laparoscopic repair of Morgagni diaphragmatic hernia in children. J Laparoendosc Adv Surg Tech A 2007;17:391–4.
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[26] Alqahtani A, Al-Salem AH. Laparoscopic-assisted versus open repair of Morgagni hernia in infants and children. Surg Laparosc Endosc Perc Techn: 2011;21:46–9. [27] Knight CG, Gidell KM, Lanning D, et al. Laparoscopic Morgagni hernia repair in children using robotic instruments. J Laparosc Endosc Percut Techn 2005;15:482–6. [28] Anderberg M, Kockum CC, Arnbjornsson E. Morgagni hernia repair in a small child using da Vinci robotic instruments—a case report. Eur J Pediatr Surg 2009;19:110–2. [29] Rau HG, Schardey HM, Lange V. Laparoscopic repair of a Morgagni hernia. Surg Endosc 1994;8(8):1439–42.
Contents lists available at ScienceDirect
Journal of Pediatric Surgery journal homepage: www.elsevier.com/locate/jpedsurg
Original Articles
Congenital Morgagni's hernia: A national multicenter study Ahmed H. Al-Salem a,⁎, Mohammed Zamakhshary b, Mohammed Al Mohaidly c, Aayed Al-Qahtani d, Mohamed Ramadan Abdulla a, Mohamed Ibrahim Naga a a
Department of Pediatric Surgery, Maternity and Children Hospital, Dammam, Saudi Arabia Pediatric Surgery and Public Health, National Guard Hospital, Riyadh, Saudi Arabia and Alfaisal University, Emory University, and Ministry of Health, Riyadh, Saudi Arabia Department of pediatric Surgery, Military Hospital, Riyadh, Saudi Arabia d Department of pediatric Surgery, King Khalid University Hospital, Riyadh, Saudi Arabia b c
a r t i c l e
i n f o
Article history: Received 25 June 2013 Received in revised form 11 August 2013 Accepted 20 August 2013 Key words: Congenital diaphragmatic hernia Congenital Morgagni's hernia Associated anomalies Treatment
a b s t r a c t Background: Congenital Morgagni's hernia (CMH) is rare and represents less than 5% of all congenital diaphragmatic hernias. This is a national review of our experience with CMH outlining clinical presentation, methods of diagnosis, associated anomalies, treatment, and outcome. Patients and methods: The medical records of all patients with the diagnosis of CMH treated at four pediatric surgery units in Saudi Arabia were retrospectively reviewed for age at diagnosis, sex, presenting symptoms, associated anomalies, diagnosis, operative findings, treatment, and outcome. Results: During a 20-year period (January 1990–December 2010), 53 infants and children with CMH were treated. There were 38 males and 15 females. Their age at diagnosis ranged from 1 month to 9 years (mean 22.2 months). Forty-three (81%) presented with recurrent chest infection. Twenty-two (44.5%) had right CMH, 15 (28.3%) had left-sided hernia and 16 (30.2%) had bilateral hernia. In 7, the diagnosis of bilaterality was made at the time of surgery. Associated anomalies were seen in 38 (71.7%). Twenty-one (39.6%) had congenital heart disease, 8 (15%) had malrotation, and 15 (28.3%) had Down syndrome. All were operated on. Twenty-nine (54.7%) underwent repair via an open approach. The remaining 24 (45.3%) underwent repair using minimal invasive surgery, laparoscopic-assisted hernia repair (19 patients) or totally laparoscopic approach (5 patients). At the time of surgery, the hernia sac content included the colon in 33 (62.3%), part of the left lobe of the liver in 13 (24.5%), the small intestines in 11 (20.75%), the omentum in 5 (9.4%), and the stomach in 4 (7.5%). In 12 (22.6%), the hernia sac was empty. When compared to the open repair, the laparoscopic-assisted approach was associated with a shorter operative time, an earlier commencement of feeds, less requirement for postoperative analgesia, a shorter hospital stay, and better cosmetic appearance. There was no mortality. On follow-up, 2 (7%) of the open surgical group developed recurrence. Conclusions: CMH is rare and in the pediatric age group commonly presents with recurrent chest infection and has a high incidence of associated anomalies, commonly congenital heart disease and Down syndrome. We advocate a laparoscopic-assisted approach to repair CMH. This is a simple technique that produces a sound repair, and when compared with the open approach it takes less operative time, requires less analgesia, allows earlier commencement of feeds, is associated with a shorter hospital stay, and has a better cosmetic outcome. © 2014 Elsevier Inc. All rights reserved.
Congenital Morgagni's hernia was first described by Giovani Morgagni in 1761 [1,2]. It is a congenital herniation of abdominal contents into the thoracic cavity through a retrosternal diaphragmatic defect. CMH is relatively rare and make up about 1%–5% of all types of congenital diaphragmatic hernia, and although commonly diagnosed in the pediatric age group, it can remain asymptomatic till adulthood [3,4].
⁎ Corresponding author. Qatif 31911, Saudi Arabia. Fax: +966 38630009. E-mail addresses: [email protected] (A.H. Al-Salem), [email protected] (M. Zamakhshary), [email protected] (M. Al Mohaidly), [email protected] (A. Al-Qahtani), [email protected] (M.R. Abdulla), [email protected] (M.I. Naga). 0022-3468/$ – see front matter © 2014 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.jpedsurg.2013.08.029
In the pediatric age group, CMH has unique features in terms of clinical presentation, high incidence of bilaterality and associated anomalies. CMH commonly presents with repeated attacks of chest infection and rarely vague, nonspecific gastrointestinal symptoms but at times it remains asymptomatic or discovered accidently during evaluation of other nonrelated conditions. The rarity of CMH and the vague nonspecific symptoms are two important factors leading to delayed diagnosis. CMH nevertheless can cause significant morbidity [2]. This is specially so if the child is not adequately investigated. Awareness of this is of paramount importance. During infancy CMH however can lead to acute respiratory distress indistinguishable from that of Bochdalek hernia [5]. The treatment of CMH is surgical repair either conventionally by open abdominal or thoracic approaches, or more recently, by using
504
A.H. Al-Salem et al. / Journal of Pediatric Surgery 49 (2014) 503–507
minimal invasive surgery [2,6–11]. This study is a national review of our experience with CMH highlighting aspects of presentation, diagnosis, associated anomalies and their influence on outcome. The methods of treatment are discussed and a comparison between the conventional open technique and the laparoscopic-assisted repair of CMH is presented. 1. Patients and methods Four pediatric surgery units in the kingdom of Saudi Arabia participated in this study. The medical records of all patients with the diagnosis of CMH during the study period from January 1990 to December 2010 were retrospectively reviewed and the following information was collected: age at diagnosis, sex, clinical presentation, associated anomalies, site of hernia, and methods of repair, postoperative complications and outcome. 2. Results During the study period, 53 infants and children with CMH were treated. There were 38 males and 15 females (M/F of 2.5:1). Their mean age at diagnosis was 22.2 months (1 month–120 months). The majority (81%) presented with recurrent chest infection (Table 1). Five had nonspecific upper respiratory tract symptoms and two were treated as gastroesophageal reflux disease prior to diagnosis. In one, the hernia was discovered incidentally. One of our patients had a road traffic accident with blunt abdominal trauma and an abdominal CT scan noted bilateral CMH. This was confirmed intraoperatively where bilateral CMH with hernia sacs were found containing intestines. The last patient had beta-thalassemia major and underwent splenectomy because of frequent blood transfusion. Following this and as a result of progressive liver enlargement, the left lobe of the liver herniated into an already existing left Morgagni's hernia. Twenty-two (41.5%) patients had right-sided CMH, 15 (28.3%) had left-sided hernia and 16 (30.2%) had bilateral hernia. In those with bilateral hernias, the diagnosis of bilaterality was made at the time of surgery in 7. Associated anomalies were seen in 38 (71.7%) as shown in Table 2. A single associated anomaly was seen in 17 while 21 had more than one anomaly. Congenital heart disease was the commonest associated anomaly seen in 21 patients (39.6%). Down syndrome was noted in 15 patients (28.3%) and all of them had congenital heart disease. Eight (15%) had associated inguinal hernia, 4 (7.5%) had umbilical hernia and 2 had hydrocephalus. In the majority (50 patients), plain chest radiographs (anteroposterior and lateral views) were diagnostic showing anterior herniation of bowel into the chest (Fig. 1). Additional diagnostic investigations included barium enema (12 patients), barium meal and followthrough (7 patients) and CT scan of chest (5 patients) (Figs. 2 and 3). All patients underwent operative repair. The method of repair changed over the years. In the initial stages of the study, 29 (54.7%) underwent repair via an open approach. The remaining 24 patients underwent repair using a minimally invasive approach with laparoscopic-assisted hernia repair (19 patients) or totally laparoscopic
Table 1 Clinical features of patients with congenital Morgagni's hernia. Clinical features
No. of patients %
Recurrent chest infection 43 Nonspecific upper respiratory tract symptoms 5 Gastroesophageal reflux disease 2 Discovered incidentally 1 Following road traffic accident with blunt abdominal 1 trauma Following splenectomy 1
81 9.4 3.8 1.9 1.9 1.9
Table 2 Associated anomalies. Associated anomalies
No. of patients
%
Congenital heart disease Down's syndrome Inguinal hernia Malrotation Umbilical hernia Hypospadias Anorectal malformation Hydrocephalus Undescended testes Hemangioma Scoliosis
21 15 8 8 4 4 2 2 1 2 1
39.6 28.3 15 15 7.5 7.5 3.8 3.8 1.9 3.8 1.9
approach (5 patients). A comparison between the open approach and the laparoscopic-assisted approach is shown in Table 3. At the time of surgery, the hernia sac content included the colon in 33 (62.3%), part of the left lobe of the liver in 13 (24.5%), the small intestines in 11 (20.75%), the omentum in 5 (9.4%) and the stomach in 4 (7.5%). In 12 (22.6%), the hernia sac was empty. In all patients who had open repair, the hernia sac was excised and the repair was done using nonabsorbable sutures. The hernia sac was not excised and plicated in those who had minimal invasive surgery. Postoperatively, all did well except two patients in the open-surgery group (7%) who developed recurrence and required reoperation. One of them also had incisional hernia which was repaired. Both of them had Down syndrome. 3. Discussion Congenital diaphragmatic hernia through the foramen of Morgagni is an interesting condition from several aspects including its rarity, high incidence of bilaterality, presentation, and high incidence of associated anomalies [12–15]. It is relatively rare comprising 3%–5% of all surgically treated congenital diaphragmatic hernias. Over a period of 40 years, Berman et al. [15] treated only 18 cases of CMH and Pokorney et al. [5] saw only 4 cases over a period of 25 years. Cigdem et al. [12] over a period of 23 years treated 16 cases of CMH. Al-Salem [13] over a period of 18 years treated 23 cases of CMH. The exact incidence of CMH in Saudi Arabia is not known. In a previous study, CMH made up about 11% of all types of congenital diaphragmatic hernia [16]. This is relatively high when compared to that reported internationally. One contributing factor for this is the high rate of consanguinity in this part of the world. The majority (90%) of CMH occur on the right side and 2% on the left side and 8% are bilateral. The rarity of CMH on the left side is caused by the pericardial attachment to the diaphragm which is more on the left side giving support and protection to that side. Twenty-two (41.5%) of our patients had right-sided hernia, 15 (28.3%) had leftsided hernia and 16 (30.2%) had bilateral hernia. The reason for the high frequency of bilateral hernias in our patients is not known. An interesting point in our series is the high frequency of bilateral hernias diagnosed intraoperatively. One reason for this is the presence of an empty sac at the time of radiological evaluation. This must be kept in mind and looked for intraoperatively. Another interesting point is the increased incidence of associated anomalies in patients with CMH but the incidence is variable ranging from 34% to 50% [5,12,13]. Thirty-eight (71.7%) of our patients had associated anomalies. This is higher than that reported internationally. Congenital heart disease continues to be the commonest associated anomaly. This was the case in our series as 21 (39.6 %) of our patients had congenital heart disease. The severity of associated congenital heart disease was variable but none of them had major lifethreatening defects and atrial septal defect and ventricular septal defect were the commonest associated heart defects. This however
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Fig. 2. Barium enema showing anterior herniation of colon into the chest.
between Down syndrome and CMH. The hypotonic muscle development in those with Down syndrome may play a role in the development of CMH and also may play a role in recurrence. In two of our patients there was recurrence and one of them had an incisional hernia. Both of them were Down syndrome patients. The association of CMH hernia and malrotation is well established. Eight (15%) of our patients had malrotation. This fact is of paramount importance when considering the operative approach to repair Morgagni's hernia. An interesting finding was the remarkably higher incidence of associated anomalies in those with bilateral CMH [14]. In our series, 23 (62.2%) of those with unilateral CMH had associated anomalies when compared to a 93.75% incidence in those with bilateral CMH. The reason for this variation is not known. The presentation of CMH is variable and nonspecific. Physicians caring for these patients should be aware of this and a high index of suspicion is important to obviate delay in diagnosis with its associated morbidity. CMH can be discovered incidentally or cause vague gastrointestinal complaints but more commonly it causes respiratory symptoms with repeated chest infections [2]. This was the case in our series as the majority of our patients presented with repeated attacks of chest infections necessitating several courses of antibiotics and hospitalizations in some of them. In the neonatal period, CMH can present acutely resembling that of Bochdalek hernia [5]. None of our
Fig. 1. Anteroposterior and lateral chest x-ray showing anterior herniation of bowel loops into the chest.
had no effect on the overall morbidity and final outcome. The high frequency of associated congenital heart defects calls for a thorough cardiac evaluation of these patients including a preoperative echocardiogram. The association of CMH and Down syndrome is interesting. In a collective series of 46 children with CMH, 16 (34.8%) of them had Down syndrome [17]. Cigdem et al. [12] in a series of 16 patients with CMH seen over a period of 23 years reported a 31.25% incidence of Down syndrome. In our series 15 (28.3%) of our patients had Down syndrome. A causal relationship has been suggested
Fig. 3. Abdominal CT scan showing anterior herniation of part of the liver into the chest (marked by arrows).
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Table 3 Comparison between laparoscopic-assisted and open-surgery repair for Morgagni's hernia in infants and children. Variable Total no. of patients Sex Site of hernia
Laparoscopic-assisted group
20 13 M/7 F Right: 8 Left: 9 Bilateral: 3 Median age (range) 15 months (8–42 months) Median operative time 40 minutes (range) (27–95 minutes) Median time to 24 hours full feed (range) (20–36 hours) Requirement for Only 1 required analgesia pethedine Median hospital stay 48 hours (range) (36–168 hours) Complications None
Open-surgery group 28 22 M/6 F Right: 9 Left: 6 Bilateral: 13 10 months (1–60 months) 90 minutes (60–120 minutes) 50 hours (40–75 hours) All required pethedine 120 hours (96–240 hours) 2 (7%) recurrence
p Value
b0.0001 b0.0001 – b0.0001 0.50
patients presented acutely and CMH was asymptomatic discovered incidentally in only one of our patients. In four of our patients, CMH was precipitated by an increase in intraabdominal pressure. In one this was the result of blunt abdominal trauma which resulted in herniation of intestines into an already existing bilateral CMH. Two patients had hydrocephalus with a ventriculoperitoneal shunt and we feel this contributed to the appearance of the hernia by increasing the intraabdominal pressure. The fourth patient is a case of betathalassemia major who underwent splenectomy which lead to progressive hepatomegaly and herniation of part of the left lobe of the liver. There is a general consensus that in symptomatic patients, CMH should be treated surgically. Opinions however differ as to the treatment of those with asymptomatic hernia. We like others advocate repair of asymptomatic patients. This is to obviate the potential complication of strangulation in spite of its low frequency [18–20]. A still controversial point in the management of patients with CMH is the surgical approach, whether transthoracic or transabdominal [2,7]. In the past, we advocated the transabdominal 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 corrects an associated malrotation if present. Such an approach is supported by the fact that in 7 of our 16 patients with bilateral CMH, the diagnosis of bilaterality was only made intraoperatively and 5 of our patients had associated malrotation that was corrected simultaneously. The recent advances in minimal invasive surgery have made laparoscopic repair of CMH safe and effective both in children and in adults. Georgacapulo et al. [10] in 1997 reported the first successful laparoscopic repair of CMH in a child. A variety of techniques to repair CMH laparoscopically have been described including primary closure with a continuous suture, interrupted suture or using a mesh [21–25]. The laparoscopic interrupted or continuous suture technique to repair CMH is not simple and is time consuming. Add to this the fact that the anterior edge of the defect is not well developed and sometimes absent. This makes direct closure of the defect weak and at risk to breakdown. One way to overcome this is to anchor the sutures to the back of the sternum and costal margin. This however is also technically difficult. A better and simpler approach is to take the sutures through the posterior edge of the hernia defect and incorporate the entire anterior abdominal wall and tie the knots extracorporealy in the subcutaneous tissues [24,26]. This technique was used in the majority of our patients treated laparoscopically and in none was it necessary to use a prosthetic patch. This technique is simple and when compared to the open repair, it is definitely better. It takes a shorter operative time, and produces a sound repair. Add to
this, earlier commencement of feeds, less requirement for postoperative analgesia, a shorter hospital stay and better cosmetic appearance [26]. Robotic-assisted laparoscopic repair of CMH was also reported to be safe and feasible in children but the set-up time prolongs the procedure and the overall operative time is longer than the laparoscopic-assisted repair [27,28]. Another controversial issue at the time of repair is whether to excise or leave the associated hernia sac [2,21,22,26,29]. In all our cases treated via the open approach, the hernia sac was excised. This however was not the case for those treated via the laparoscopicassisted approach as none of the hernia sacs were excised. On followup however, two of the open-surgery group developed hernia recurrence while none of the laparoscopic-assisted group recurred. CMH is a relatively rare type of diaphragmatic hernia that usually presents with recurrent attacks of chest infection. Its rarity and the vague nonspecific symptoms are two contributing factors for delayed diagnosis. 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, and among these congenital heart diseases is the commonest. Awareness of this is important and echocardiography should be part of the preoperative investigations. The reason for the increased number of bilateral hernias in our series is not known. CMH should be repaired surgically even in asymptomatic cases. We advocate repairing CMH using the laparoscopic-assisted approach. This is a simple technique that produces a sound repair and when compared with the open approach takes less operative time, requires less analgesia, allows early commencement of feeds, and results in a shorter hospital stay and better cosmesis.
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