Abstract
Background:
Morgagni hernia (MH), a rare type of congenital diaphragmatic hernia, does not have an established protocol for surgical repair.
Materials and Methods:
A MEDLINE search with terms related to various surgical approaches to repair MH in children was conducted. Articles comprising robotic-assisted surgery, laparoscopy, laparotomy, thoracoscopy, and thoracotomy over the last 20 years were assessed.
Results:
This narrative review provides an overview of MH in the pediatric population, covering the epidemiology, diagnosis, and management of this rare diaphragmatic hernia. We discuss various surgical techniques, including open and minimally invasive approaches, and compare their advantages and limitations in childhood MH repair. In addition, we address arguments for and against controversial topics such as hernia sac excision and patch reinforcement.
Conclusions:
Regarding MH in children, transabdominal repair is superior to transthoracic due to improved visualization of bilateral defects and easier reduction of the hernia contents. Laparoscopy has been reported as a popular approach. Single-site laparoscopy has gained attention due to better outcomes than standard three-port laparoscopy. Considering a limited number of children in the literature who underwent robotic MH repair, perioperative complications were reported to be minor.
Introduction
Morgagni hernia (MH) was initially defined in 1761 following a postmortem investigation by Giovanni Battista Morgagni, an anatomist and pathologist from Italy.1,2 MH, also termed Morgagni–Larrey, or anterior diaphragmatic hernia, is an uncommon condition with a prevalence of 3%−5% among operated congenital diaphragmatic hernias (CDH). It is defined by herniated abdominal contents through a retrosternal diaphragmatic defect called Morgagni or Larrey foramen into the chest cavity.3–5 Morgagni addresses diaphragmatic defects on the right side of the sternum and Larrey on the left. 6 However, Morgagni commonly refers to both. 7
Unspecific respiratory or gastrointestinal symptoms may be present, while many patients are asymptomatic, and MH might be found incidentally through a chest X-ray.8,9 Ultrasonography is recommended for screening of suspected cases. 10 Computed tomography (CT) scan or barium enema can be used to confirm the diagnosis.11,12 Surgical repair is recommended, even in asymptomatic cases to prevent later complications, such as obstruction or strangulation.11,13
Thoracotomy and laparotomy have been traditionally approached to treat MH.14,15 In 1997, the first pediatric patient underwent laparoscopic MH repair. 16 With advancements in technology, the first robotic correction of MH was performed on a 10-year-old child in 2003. 17 Regardless of all achievements, no definite surgical protocol has yet been established to reach a consensus on how to manage MH in the pediatric population. Therefore, we reviewed the most recent literature on repair options for children with MH.
Overview of MH in Pediatric Population: From Epidemiology to Management
Epidemiology and patient characteristics
It is estimated that 1 in 5000 neonates are born with CDH. 18 Three subtypes of CDH include Bochdalek hernia with a defect located on the posterolateral part of the diaphragm, MH with a defect of anterior diaphragmatic margin, and central hernia involving the central part of the diaphragm. 19 Among them, Morgagni or retrosternal hernia is the rarest.5,20,21 Considering MH, male patients have been reported more frequently than females in the literature.22,23 Children with MH may remain asymptomatic or present with unspecific respiratory or gastrointestinal symptoms due to herniated abdominal contents in the chest cavity.23,24 Many of the MHs in children are associated with other malformations such as Down syndrome and congenital heart disease.25–27 Authors have recommended to consider MH when a patient with Down syndrome presents with recurrent respiratory or gastrointestinal symptoms.22,28,29 In the literature, the majority of the MHs in children are located on the right side.30,31 Contrary to adult studies, 32 in pediatric studies bilateral and left hernias are also considerable.22,25 Several investigations have reported the midline or central location of the defect.26,27,33 However, it is still uncertain whether “bilateral” and “midline” describe the same anatomical position or not. 26
Diagnosis
The diagnosis is often missed until after birth to be suspected on a chest X-ray. 34 It also can be missed on X-ray in the case of an empty hernia sac or herniated solid organs such as the liver and omentum.3,29 A CT scan may confirm the diagnosis in these cases, due to a better demonstration of the herniated organs and sac expansion.11,27,35 Magnetic resonance imaging allows for a radiation-free evaluation of the defect, which distinguishes the thoracic and abdominal origin of the content, but the cost is excessive.32,36,37 Barium study also serves as a less expensive modality.11,12 A recent retrospective study revealed that ultrasonography can precisely identify the location, extension, and content of the MH. 10
Management
Numerous authors have advised to treatment of MH surgically, even in asymptomatic children, to avoid future complications such as volvulus, bowel perforation, and strangulation.38–41
Open Repair Approaches for MH
Thoracotomy
Thoracotomy for pediatric MH repair involves a posterolateral subcostal incision followed by suturing the defect primarily with interrupted or continuous non-absorbable sutures or using a mesh.11,42,43 The transthoracic method facilitates recognition and preservation of the phrenic nerve, dissection of adhesions, and excision of the hernia sac. 44 Thoracotomy used to be preferred when there was an unknown density in the chest radiograph, in the case of herniated omentum or liver, resembling a thoracic mass.42,45 There is evidence suggesting that thoracotomy in children may lead to potential thoracic wall deformities in the future.46,47
Moreover, thoracotomy should be considered for MH when there is no possibility of incarceration, strangulation, bilateral defect, or bowel malrotation.15,48 A three-year-old child died from strangulation of a herniated organ in the left-side defect which was missed after a successful right-side MH repair via thoracotomy. 49 Although thoracotomy is not recommended in conditions like intestinal obstruction, exceptions have been reported. For instance, a child with Down syndrome underwent MH repair at the age of one; six years later, reoperated with signs of strangulated recurrence of MH, starting with laparotomy, which converted to thoracotomy for reducing the herniated organs. 50
Laparotomy
Laparotomy was once the method of choice to repair MH in children. With a transabdominal approach, hernia contents can be smoothly brought down into the abdomen. 11 In contrast, Pokorny et al. encountered an insufficiency of laparotomy in reducing the left hepatic lobe, which seemed to adhere to the pericardium and had to be reduced by a right thoracotomy. 44
Laparotomy for MH repair is performed through a supraumbilical midline or transverse incision.9,50 The surgeon intracorporeally sutures the posterior defect margin to the back of the costal or sternal periosteum and/or posterior margin of the rectus sheath, which makes the procedure challenging, time-consuming, and prone to recurrence due to a weak closure.8,12,23 To ensure a secure closure, a patch is applied for tension-free repair. 38 It is worth mentioning that laparotomy is still practical for MH repair, especially in patients with large defects, 51 thick adhesions, severe scoliosis, 52 and tiny abdominal cavities which make the trocar placement hard. 53
Minimally Invasive Repair Approaches for MH
Thoracoscopy
In terms of retrosternal hernia, due to poor outcomes and a great possibility of surgical conversion, thoracoscopy is not prioritized by pediatric surgeons.33,54 Golden et al. described that of the 26 children with MH, just 1 of them underwent thoracoscopy, which was finally converted to laparotomy to explore the abdomen. 33 In addition, retrosternal suturing via thoracoscopy is a challenging task. 55 In 2008, Shah et al. reported a 14-month-old child with MH, who was initially approached by thoracoscopy. Eventually, the procedure was converted to laparoscopy, due to inaccessible angles and difficulties of the dissection. 56 In 2017, Alonso et al. reported a thoracoscopic attempt on a child with MH which was converted to laparotomy, because of technical hardship. 57 During thoracoscopy for MH, one lung ventilates and chest tube placement might be required in case of pleural violation; moreover, access to the herniated organs is restricted. 54
Laparoscopy
Shortly after the first laparoscopic treatment of a child with MH in 1997, this approach emerged as a safe and effective repair option in children. 23 Features of various surgical approaches for childhood MH are shown in Table 1.
Surgical Features of Laparoscopy, Laparotomy, Thoracotomy, and Thoracoscopy in Morgagni Hernia Repair
CHD, congenital heart disease; DS, Down syndrome; N, number of patients; SVC, superior vena cava.
The laparoscopic approach usually starts with the supine, reverse Trendelenburg positioning of the patient. The surgeon positions between the patient’s inferior limbs. One to four ports may be inserted into the abdominal cavity.59,64 For a three-port approach, a 5 mm or 10 mm camera port with a 0°−45° telescope is inserted through the umbilical incision, while two 3–5 mm working ports are placed along the right and left midclavicular lines.12,65 Single-site laparoscopic technique with percutaneous internal ring suturing, which has recently become popular for MH, decreases anesthesia time, cost of the operation, and blood loss, besides having satisfactory cosmetic results compared to the standard three-port laparoscopic technique.25,36,59,66,67 Considering single port repair, Okur et al. demonstrated that using a cystoscope with forceps to plicate and cauterize the hernia sac resulted in shorter surgery duration compared with using a laparoscopic scope without sac excision. This method also ensured a safe procedure by preventing damage to the intestine and liver. 58 Six to 12 mmHg of CO2 insufflation is usually applied.30,64 The falciform ligament is mostly cut to provide better visualization, although the operation can go well without dividing the ligament. 36 Using the single port technique, herniated organs can be reduced by maneuvering the endoscope. 58 Regardless of the number of ports that are inserted, three methods are utilized to close the defect primarily: (1) interrupted or running sutures passing through the anterior defect margin (intracorporeal knot tying), (2) interrupted sutures crossing abdominal full-thickness with knots tied in the subcutaneous tissue (extracorporeal knot tying), and (3) combination of both 20 (Fig. 1). Laparoscopic transparietal closure with extracorporeal placement of the knots is a relatively new method. There are two types of subcutaneous knot tying with statistically comparable results60: one involves separate 2 mm stab incisions along the defect margin,30,68,69 and the other one uses a single 1–2 cm incision in the sub-xiphoid region26,28,60 (Fig. 2). In any case, extracorporeal knot placement reduces the odds of perforating adjacent organs owing to limited needle movements inside the body. 30 The challenge of subcutaneous knot tying is to pull the needle out of the body from the entry point. Parlak et al. realized that among laparoscopic needle holder, 18-gauge catheter needle, and suture-passer forceps, the last one resulted in more accessible and faster needle removal from the same incision spot. 20

Intra- and extracorporeal suturing of the Morgagni hernia defect.

Two types of extracorporeal suturing for Morgagni hernia repair.
Absorbable vicryl threads and non-absorbable polypropylene, polyester, or silk threads in 2–0 or 3–0 sizes are used, although non-absorbable threads are favorable to reduce the chance of recurrence.63,64 Regarding non-absorbable sutures, surgeons mostly prefer polyester sutures for extracorporeal knot tying since propylene sutures may induce skin reactions, micro-abscesses, and patient discomfort.20,30
Robotic approach
The incorporation of robotic systems into pediatric surgery has been slower than in the adult population due to limited instrumental variety and proper size for smaller children. 70 Since the first pediatric MH repair with robotic devices in 2003, 17 the Zeus robot had been used until 2007. 71 However, the Da Vinci robot is still employed. 72 Our literature search found only case reports of children who have undergone robotic-assisted MH repair (Table 2). This approach has been found practical in the literature. To our information, no concomitant malformation, recurrence, complication, or death was reported,72–77 except for the first reported child who intraoperatively developed a pneumothorax. 17
Robotic-Assisted Morgagni Hernia Repair in Children
N, number of patients; NS, not specified.
After the placement of the sterile sheets (draping), the effective operation duration involves the placement of the trocars and docking of the robotic equipment, the console operating time, and the closure of the wound.72,78 Docking includes attachment of the robotic arms to the trocars and insertion of the camera and robotic instruments. 78 In the literature, robotic operative length (skin-to-skin) for MH repair has been indicated to vary from less than one hour to nearly four hours. Moreover, the presence of the hernia sac has been reported frequently while mostly left intact or incorporated into the sutures.17,72–77 Robot-assisted approach begins with the patient placed in a supine position and includes the insertion of a 5–12 mm 30° laparoscope along with at least two 5–10 mm trocars to serve as working ports.72,75,76 Three to five ports may be inserted.17,72 Anderberg et al., 75 who used four ports, reported that they prefer to use three ports in the future. Through robotic approach interrupted or running sutures with absorbable or non-absorbable threads can be used to repair the defect, employing intracorporeal knot tying.17,72–77 Robotic surgery might become a popular repair option for CDH, although it requires further evaluation. 74
A Comparison of Repair Approaches for MH
Regardless of the open or minimally invasive type of surgery, low recurrence rates, approximately 4%–5%, have been reported in the literature.22,51 However, the true recurrence rate may have been masked due to short-term follow-ups and publication bias. 52 Yet, it is unclear whether the etiology of the recurrences is related to the surgical technique, suturing material, sac resection, patch fixation, or patient malformation.52,63 It is believed that recurrence risk is increased in connective tissue diseases and Down syndrome with poor wound healing and altered immunity.25,27,60,79
The transabdominal approach is more applied than the transthoracic in the case of MH since many of the bilateral hernias and intestinal malrotations are diagnosed during the operation; therefore, may remain untreated by transthoracic surgery while untreated bowel malrotation can result in postoperative volvulus.23,80 Furthermore, laparoscopy is probably a better first choice than thoracoscopy due to improved visualization for bilateral defects and convenient reduction of the herniated contents.12,22
Comparing laparotomy and laparoscopy for MH in children, laparotomy is related to extended hospital stays, 38 increased operation time, prolonged postoperative time to feed, additional analgesic requirements, and poor cosmetic outcomes. 23 There are no significant differences related to postoperative complications or recurrences between the two approaches.33,59 Disadvantages of the laparotomy can be attributed to the fact that laparotomy is used mostly in acute cases, which makes laparotomies more engaged with incarceration, volvulus, perforation, and other surgical emergencies. 22
Surgeons find MH robotic repair more feasible than conventional laparoscopy due to more range of motion during adhesion release and suturing, tremor filtration, a high-resolution 3D view that provides visual depth, and the absence of opposite movements. 76 Conversely, robotic surgery prolongs the anesthesia duration due to the time needed to set up the robotic instruments. However, recent robotic-assisted surgeries take less time to finish.72,77 Previously, setting up the robot took 35 minutes, 75 now reduced to 15 minutes. 72 Iranmanesh et al. demonstrated that both the setting up (draping) and docking of the robot have a fast learning curve. Thus, in the hands of expert surgeons, it has minimal influence on total procedure length. 78 After all, the use of robotic systems is costly, needs special training, and can be challenging in smaller children. 73
Ongoing Debate in MH Repair
Apart from the surgical approach for MH repair, regarding hernia sac excision and mesh fixation controversies remain unresolved.
Excision of the hernia sac
The hernia sac may be excised or left intact while is cauterized on the edges,20,30 ligated, plicated, 36 or included in the sutures. 12 The potential to damage the pleura, phrenic nerve, and pericardium is the major drawback of the sac excision. 81 There are reports of mild pneumothorax after invading the pleura during the sac resection.6,34 Azzie et al. observed cardiac arrhythmia following the sac eversion. 82 Many authors suggested excising the sac once it is found safe and feasible.26,27 However, Celtik et al. detected only one recurrence among 19 patients who underwent either total (n = 15) or partial (n = 4) sac excision. Thus, they advised that when the complication risk is high, at least partially excise the sac to provide better tissue healing and decrease the chance of recurrence. 60
Bawazir and colleagues recommended that in case of fibrous adhesions or thickness of the hernia sac, the preferred option is to cauterize the margins of the sac and leave it in situ. 27 Karadage et al. cauterized sac edges and did not remove the sac in any of their 22 operations. They did not report any recurrence after long-term follow-up, although asymptomatic recurrences may have occurred. 30 In several cases, the fluid accumulated in the residual hernia sac was reabsorbed within two months in all of them.20,83 Cauterization, which stimulates scar tissue, has not been attributed to any recurrence in many of the studies.20,27,30,58,61,84 In addition, even after total sac resection, recurrences have been observed. 23
Usage of mesh
A patch is considered when the surgeon feels increased tension along the suture line or when the defect size is relatively large. 27 When tension is expected, a mesh can be sutured intracorporeally to the defect edges or the entire thickness of the abdominal wall.61,85 It might be fixed by sutures, pins and nails, 86 or spiral tacks. 85
Based on recent systematic reviews by Alqadi et al. and Tan et al., a patch has been used in 10% and 12% of the patients, respectively.22,51 To the best of our knowledge, regarding MH repair with the patch, even with an absorbable patch, 65 no recurrences have been reported in the literature of the pediatric population.85–87 Since MH repair with any type of patch carries a low probability of adverse outcomes, Tan et al. decided to utilize a patch for all but tiny defects. 51 Mesh is also suggested in redo surgeries for MH recurrences since elevated tension is the reason for recurrence in the first place. 52 In addition, connective tissue disorders and Down syndrome, with hypotonic muscles and impaired tissue healing,6,60 might be other indications of patch repair in MH. We found only one author who encountered bowel obstruction after using a patch to close the MH. 38 An adult study reported utilizing a peritoneal flap and hiding the mesh inside the peritoneal layers to prevent mesh-induced adhesion, erosion, and fistula. 88 Some authors avoid using a patch to prevent possible infection or interference with the growth of the diaphragm. 89 Mesh repair increases the cost, demand, and time of the surgery, whereas the cost and burden of the hernia recurrence have yet to be compared. 51
Conclusions
Regarding MH repair in children, the transabdominal approach is more advantageous than transthoracic repair in terms of better visualization of bilateral defects and easier reduction of the herniated organs. Considering transabdominal repair, laparoscopy has been widely used for pediatric MH repair. Recently, single-site laparoscopy has gained attention due to better outcomes than conventional three-port laparoscopy. Laparotomy is favorable for acute cases and challenging procedures. Robotic-assisted surgeries have been found to be feasible but need more evaluation. Regarding patch repair, no recurrences were reported; therefore, it might be suitable for conditions with high recurrence risk, such as Down syndrome, connective tissue disorders, and redo surgeries. Hernia sac cauterization has not been associated with any recurrences in many patients; thus, cauterization might be suitable rather than sac excision. Ultimately, studies with prolonged follow-ups are required to evaluate the long-term outcomes of MH repair in children.
Footnotes
Acknowledgments
The authors would like to thank Dr. Noosha Samieefar for her valuable comments on our article. Each author confirms that their research is supported by an institution that is primarily involved in education and research.
Authors’ Contributions
B.A. and D.S. had the idea for the article. Z.M. and F.G. performed the literature search. B.A., D.S., Z.M., and F.G. analyzed the data. D.S. and Z.M. drafted the article. B.A. and F.G. critically revised the article. All authors reviewed and approved the final version of the article.
Disclosure Statement
The authors declare that they have no conflict of interest.
Funding Information
The authors received no financial support for the research, authorship, and/or publication of this article.
