Introduction

Diaphragmatic hernia (DH) is a defect of the diaphragm which allows the passage of an organ, or part of it, into the thoracic cavity. DH can be congenital or acquired. Congenital diaphragmatic hernias (CDH) are rare. CDH prevalence ranges from 1.7 to 5.7 per 10,000 births [1] with a survival rate of 67% [2]. Normally, during the eighth week of gestation, the diaphragm formation divides the thoracic cavity from the peritoneal one. The premature bowel returns to the abdomen or the incomplete development of the diaphragm are the etiopathological factors of CDH [3]. Traumatic diaphragmatic hernia (TDH) occurs in about 1–5% of victims of road accidents and in 10–15% of penetrating traumas of the lower chest [4].

Complicated DH is a rare problem encountered by Emergency Department. The diagnosis and management of complicated DH can be a medical issue; the onset of symptoms is subsequent to the traumatic event. Often the symptoms may occur even months or years after the injury [5].

There is no consensus about the indications to surgery and the timing. This study aims to evaluate the surgical treatment options in emergency setting.

Methods

An extensive bibliographic research of literature according to PRISMA criteria was performed (Fig. 1). Medline and PubMed were consulted in order to identify articles reporting the item “emergency surgery” from 1983 up to May 2020, and then, it has been meshed using the Boolean operator “AND” and “OR” with the following mesh terms: “traumatic diaphragmatic rupture” and “congenital diaphragmatic hernia.” Additional articles were searched by manual identification from the key articles.

Fig. 1
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PRISMA flow diagram

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Inclusion criteria articles in English language, reporting “emergency surgery” for diaphragmatic hernia (congenital and traumatic). In case of multiple papers from the same group of authors, an effort was made to identify duplicate paper.

Exclusion criteria non-English papers, patients under 19 years, hiatal or paraesophageal hernias, procedures performed in “elective setting” have been excluded.

Several parameters were recorded and analyzed including: sex, mean age, etiology, diagnosis (chest X-ray, CT scan, barium studies, MRI and others), treatment (laparoscopic, laparotomic, thoracoscopic, thoracotomic, thoracoabdominal, robotic, damage control surgery), use of mesh, site (right or left) and herniated organs.

Results

In the present review, 146 articles were included (Fig. 1, Table 1).

Table 1 Papers included in literature systematic review
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Among the 1542 analyzed patients, 809 (52.4%) were male and 379 (24.5%) were female. In the remaining 354 (22.9%), sex was not specified. The average age was 47.7 (SD ± 18.3) years. Considering the etiology, the cause of hospitalization was trauma in 1261 patients (81.7%) and CDH in 113 (7.2%). Among these, 50 (3.2%) were Bochdalek's hernias (BH) and 54 (3.5%) were Morgagni's hernias (MH). Five patients had previously performed surgery, 5 were pregnant, and in 151 patients the etiological diagnosis was not reported.

Trauma patients were younger than patients who developed symptoms for a congenital diaphragmatic defect (45.1 vs. 53.1 years). Mean age of Bochdalek’s hernia patients was 47.2 ± 18.3 years while in patients with Morgagni’s hernia was 64.1 ± 19.2 years.

In 709 cases (45.9%), the defect was located on the left side of the diaphragm while in 273 cases (17.7%) on the right one. In only 10 cases (all traumas), the defect was bilateral, and in 550 patients the site of the herniation was not specified (Table 2). The left hemidiaphragm was more involved than the right one: trauma (644 vs. 207), Bochdalek (34 vs. 14), post-surgery DH (4 vs. 1), during pregnancy DH (4 vs. 1). Unlike the others, Morgagni’s hernias mainly occur on the right side compared to the left side (45 vs. 9) (Table 3).

Table 2 Demographic, and pathological features of the studied population
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Table 3 Defect’s localization in various etiologies
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Chest X-ray was the most common diagnostic test used in 697 patients (45.2%). CT scan also plays a major role in instrumental diagnostic methods and was used in 315 cases (20.4%). In our analysis, the other methods were much less used: barium studies in 15 cases, US in 15, gastrografin swallow in 5, MRI in 8, EGDS in 5 and manometry in only 1 case. Diagnosis was achieved intraoperatively 6 performed. Diagnostic tests were not performed in 12 patients because they were unstable. Diagnostic methods could not be deduced in 463 patients. Of the 1261 trauma patients, a delayed presentation (> 7 days) has been reported in 297 cases. Surgery was performed the same day of hospital admission in 47 trauma patients (interval time range: 1 to 9 h). Surgery was performed in the first week after trauma in 100 cases. Data about time interval from trauma to surgery have not been reported in 817 patients.

As far as surgical treatment is concerned, the open approach is the one widely used. Laparotomy, thoracotomy and thoracoabdominal approach were used in 907 cases (58.8%), 184 (11.9%) and 42 (2.7%) patients, respectively. Laparoscopic, thoracoscopic and robotic approaches were used in 103 (6.6%), 28 (1.8%) and 3 (0.1%) patients, respectively. A conservative management was chosen in 11 patients for contraindications to surgery or because they were unstable. In 22 patients, along with the repair of the diaphragmatic defect, another associated procedure was performed (mainly splenectomy, gastrectomy and colectomy). Damage control surgery (DCS) was performed in only 12 patients. In one patient, a colopexy was performed to cover, without repairing, a massive diaphragmatic defect.

The use of meshes has been observed only in 55 cases, 17 times with laparotomic approach and 35 times with laparoscopy.

The treatment for each category of diaphragmatic hernias is summarized in Table 4.

Table 4 Surgical approach: differentiated analysis by etiology
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The stomach was the most common herniated organ in 176 cases, followed by the colon, in 125 cases, the omentum (90 cases), the small bowel (95 cases) and the spleen (44 cases). Liver and kidney involvement were observed in 32 and 5 patients, respectively. In 68 cases, there was no finding of abdominal organs in the chest, while for 906 patients, this information was not reported.

Discussion

The symptomatology of complicated diaphragmatic hernias can vary greatly depending also on their etiology. In CDH, the symptoms can be varied and occur at different times. Symptomatic CDH in the childhood derives from pulmonary hypoplasia because the herniation of the organs in the chest during prenatal period prevents the development of the lungs [6]. In spite of our series does not suggest it clearly, literature reports BH as the most frequent among the CDH [7, 8]. The MH are rarer. MH have an anterior development and derive from a closure defect of the sternal part of the diaphragm with the seventh chondrocostal arch. They can remain asymptomatic, and often, the diagnosis is an incidental finding during other instrumental test (chest X-ray) [9, 10]. CDH in the adulthood can present with non-specific respiratory and gastrointestinal disorders. Gastrointestinal problems at the diagnosis can be more common in the left-sided hernias, where the absence of the liver allows the migration of the abdominal organs into the thorax, sometimes causing mild (dyspepsia, recurrent or non-specific abdominal pain) or acute (obstructions or flies) abdominal symptoms. In right-side CDH, respiratory symptoms are predominant [11, 12]. TDH presentation symptoms may vary depending on the type of trauma (blunt or penetrating), on the amount of energy absorbed by the body and on the involved side. The most common cause of TDH is a traumatic event that creates an increase gradient between the abdominal and thoracic compartment with a rupture mainly at the level of the embryonic melting points. Penetrating traumas are the most frequent, but the diaphragmatic defects are generally smaller than the blunt ones [13]. Small chronic traumatic events such as coughing or obesity acting over time may cause the exhaustion of already existing hiatuses or the rupture of weaknesses. In fact, in some cases the symptoms may not be present for many months or years after the trauma [5]. Pregnancy also plays a fundamental role in this context; increased abdominal pressure that occurs in this period contributes to the rupture of the diaphragm in its weaknesses point or can unmask congenital diaphragmatic defects. It is a rare situation, and in our series, it occurred in 5 cases (0,5%), but in some cases, it may endanger the life of the fetus; therefore, it must not be ignored [14,15,16,17].

CDH have a different rupture site’s prevalence depending on the type of considered hernia. BH occur more frequently on the left side (80%), [18] MH mainly develop on the right side, but sometimes it can be bilateral or develops on the left side [3]. Our analysis also follows the literature, indeed 68% of BH developed on the left side (34 vs. 14), while almost all MH are on the right one (83%, 45/54). Considering TDH, in our series as well as in literature, the left hemidiaphragm is more commonly involved in blunt or penetrating injuries. This is probably due to the protective effect of the liver for the right hemidiaphragm on the blunt trauma, and the fact that most people use their right hand on the penetrating trauma [19]. The great variability in the etiology of trauma also determines the possibility the defect develops in both hemidiaphragms; in our review, there are only 6 bilateral herniations and all have traumatic origin [20,21,22].

In a “border” pathology between the thorax and the abdomen, a correct preoperative diagnostic work-up is essential. A precise diagnosis inevitably influences the surgeon in choosing the most correct approach to the patient with complicated diaphragmatic hernia. The most used diagnostic test in both our series and literature is chest X-ray. It allows to show an opaque hemithorax with deviation of the mediastinum and when the nature of the thoracic contents is uncertain, nasogastric tube’s course can help in the diagnosis. Soft opacity in the thorax with or without gas can be a sign of hernial sac, and in larger hernias, loops of bowel or the transverse, can also be visualized in the chest [3]. CT scan, with a sensitivity and specificity of 14–82% and 87%, respectively, is considered the diagnostic gold standard [23, 24]. Unlike chest X-ray, which can be normal in case of intermittent herniation, CT scan determines the presence, location and size of the diaphragmatic defect. CT scan can evaluate the intrathoracic herniation of abdominal contents and related complications [25]. Even in one case, in our unit the diagnostic accuracy of CT scan was fundamental to recognize some ischemia signs, such as the forward displacement of the gastric bubble, the missing of the gastric folds and the absence of gastric walls contrast enhancement (Fig. 2). The other diagnostic tests, barium studies, gastrografin swallow, EGDS, US and manometry are much less used in the considered articles. Barium studies can help in revealing barium filling stomach or bowels within the thorax with the strict segment of intestine at hernia site of the diaphragm, while MRI, not performable in emergency, may be used in selected patients (pregnant) [17, 26] in the study of the herniated structures and associated abdominal organ’s injuries [3].

Fig. 2
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CT scan shows ischemia signs (the forward displacement of the gastric bubble, the missing of the gastric folds and the absence of gastric walls contrast enhancement)

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Although there is no consensus on the indications and timing of surgery. Surgery seems to be the treatment of choice for complicated diaphragmatic hernia, both congenital and traumatic. Hernias, especially congenital and accidentally diagnosed, should be corrected even if the patient is asymptomatic because the risk of strangulation or incarceration [10, 27]. In case of complications, surgery is mandatory [23]. Smaller diaphragmatic defects can be primarily closed with a non-absorbable suture [28, 29] while for larger defects, where the primary suture would develop excessive tension due to the considerable loss of tissue, or also in order to reinforce the suture, meshes should be used [30, 31]. The biologic mesh represents an alternative to the synthetic one due to its lower rate of hernia recurrence, higher resistance to infections and lower risk of displacement [23, 32, 33]. The surgical approach can be either thoracotomic or laparotomic depending on the diagnostic investigation’s result and on the surgeon's preferences and skills. The thoracotomic approach, with the addition of a separate laparotomy when indicated, can be recommended especially in chronic herniation in order to reduce visceral-pleural adhesions and to avoid intra-thoracic visceral perforation [34]. Sometimes, thoracoabdominal approach may be necessary in emergency setting, when it is difficult to identify visceral abdominal lesions or to exclude bilaterality [35,36,37].

Recently, laparoscopic or thoracoscopic approach is becoming more feasible and safer allowing a lower hospital length stay and a lower morbidity rate [31, 38,39,40]. Despite this, our analysis reveals that open approaches are still predominant. This could be related to the majority of trauma hernias in which the laparoscopic approach is still very limited. A differentiated analysis of the etiology shows that most of the minimally invasive approaches have been used in repair of complicated CDH, while almost all of the complicated TDH have been approached with laparotomy. A further and even more recent approach is the robotic one, which allows a detailed anatomical visualization and a more precise dissection, but literature findings are poor. In our series, the robotic approach has been used only in 3 patients, [41, 42] and this can be determined by the high costs and the nature of the pathology that often occurs in “emergency setting” compared to “elective setting.” Damage control surgery (DCS) can be an advantageous/rescue alternative in emergency management of the patient with complicated DH although there is no general consensus on its use mainly due to the intra-abdominal hypertension and the abdominal compartment syndrome that may result [43]. DCS can be useful especially in complicated TDH. In unstable patients or damaged/bleeding organs, a second look may be required. The re-exploration of the abdomen 24/48 h later can help surgeon in recognizing the vital/non-vital areas of an ischemic organ leading him in resection [44].

Conclusion

Complicated CDH and TDH have different etiology but similar management. Surgery is the treatment of choice and is strongly influenced by the preoperative setting, performed mainly with chest X-ray and CT scan. DCS can be considered especially in traumas and can offer an advantage in management of the compromised patients. Minimally invasive approach is safe and feasible and offers advantages in terms of hospitalization and lower morbidity rate but is highly dependent on the surgeon's expertise, especially in emergency setting.