Generality
A "diaphragmatic hernia is the protrusion, due to an" abnormal opening on the diaphragm, of one or more abdominal viscera into the thoracic cavity.
The "opening on the diaphragm can be a" congenital anomaly - therefore present from birth - or an "acquired anomaly - that is, developed in the course of life, following a specific causal event.
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The symptomatic picture generally consists of respiratory disorders and consequences deriving from these. Moreover, the abdominal organs that herniate occupy the space reserved for the lungs, compromising their growth (especially in children) and function.
To avoid complications, it is essential that diagnosis and therapy are timely; therapy consisting of surgical treatment ad hoc, aimed at relocating the abdominal viscera and repairing the diaphragm.
So "is a" hernia
A "hernia" is the leakage of a bowel and / or adjacent tissues (for example the surrounding adipose tissues) from the body cavity that normally contains them (N.B: the word bowel indicates a generic internal organ).
The spill can be total or partial.
What is a diaphragmatic hernia?
A "diaphragmatic hernia is the leakage of one or more viscera contained in the abdomen, through an abnormal opening in the diaphragm. Therefore, the aforementioned viscera protrude into the thoracic compartment, where the heart and lungs reside.
The opening on the diaphragm, which induces visceral leakage, can be a congenital anomaly - therefore present from birth - or acquired - that is, developed over the course of life.
Especially when the subjects involved are very young children, the diaphragmatic hernia represents a medical emergency to be treated promptly, to avoid the appearance of unpleasant complications.
POSITION AND FUNCTION OF THE DIAPHRAGM?
The diaphragm is the laminar-shaped muscle that resides on the lower edge of the rib cage and separates the thoracic cavity from the abdominal cavity.
In addition to keeping the organs of the chest separate from the organs of the abdomen, this particular laminar muscle plays a fundamental role during the breathing process:
- In the inhalation phase, he contracts, pushing the abdominal organs down and causing the ribs closest to him to rise. This expands the volume of the chest cavity and allows the lungs to take in the necessary air.
- In the exhalation phase, it is released, allowing the abdominal organs to rise again (N.B: this also happens thanks to the support of the abdominal muscles) and the lower ribs to return to their normal position.
In this phase, the chest volume is noticeably reduced.
WHICH ABDOMINAL VISCERAS ARE INVOLVED?
Position of the diaphragm
The abdominal viscera that can participate in the formation of a diaphragmatic hernia are: the stomach, intestines, spleen and liver.
ARE THE DIAPHRAGMATIC HERNIA AND THE HYATAL HERNIA THE SAME?
Many people believe that diaphragmatic hernia and hiatal hernia are synonymous and that the aforementioned terms refer to the same medical condition.
However, this is not the case.
The hiatal hernia, in fact, is a particular form of diaphragmatic hernia, as it consists in the specific protrusion of the stomach through the so-called esophageal diaphragmatic hiatus, that is the hole in the diaphragm into which the esophagus usually inserts.
Causes
As it is understood from the definition, a diaphragmatic hernia is formed due to an opening on the diaphragm.
The latter has different origins depending on whether it is congenital or acquired.
CONGENITAL DIAPHRAGMATIC HERNIA
Congenital openings on the diaphragm result from an error during fetal development, which compromises the normal formation of the laminar muscle interposed between the chest and abdomen.
With the protrusion of one or more abdominal viscera into the thoracic compartment, the organs enclosed in the rib cage (the lungs in particular) lack the right space to grow and develop properly. Moreover, to occupy the space reserved for them, are the organs of the "abdomen" wedged in the thorax.
When the opening on the diaphragm is congenital, the diaphragmatic hernia takes the name of congenital diaphragmatic hernia.
Doctors have identified at least three different types of congenital diaphragmatic hernia:
- Bochdalek's hernia. It is the most common type and accounts for 95% of clinical cases of congenital diaphragmatic hernia. The anomalous opening on the diaphragm is located in the posterolateral corners of this muscle. In 80-85% of patients, the posterolateral corner with the pathological opening is the left one.
- Morgagni's hernia. This type accounts for only 2% of clinical cases of congenital diaphragmatic hernia. The abnormal opening, from which the viscera emerge, resides on the anterior part of the diaphragm, just behind the xiphoid process of the sternum. The exact point is in correspondence with the foramen of Morgagni (hence the name of Morgagni's hernia).
- The eventration of the diaphragm. It is the rarest type. It consists of a permanent elevation of part or all of the diaphragm. This involves the abdominal organs moving upwards, occupying the place reserved for the anatomical elements of the thorax (lungs particularly).
Is the congenital diaphragmatic hernia the result of a genetic alteration?
Scientific studies have found that only 30% of cases of congenital diaphragmatic hernia result from an "alteration in the chromosomal makeup of patients.
For the remaining cases, the causes of the congenital opening on the diaphragm are completely unknown. It is for this reason that doctors refer to these circumstances with the term of idiopathic congenital diaphragmatic hernia, where idiopathic means the lack of a precise and identifiable triggering cause.
ACQUIRED DIAPHRAGMATIC HERNIA
Openings acquired in the diaphragm are generally the result of impact trauma or penetrating trauma located between the chest and abdomen.
Impact trauma is what a motorist involved in a road accident or an individual can suffer when falling down stairs, participating in a contact sport, etc.
A penetrating trauma, on the other hand, is that to which a subject victim of a stabbing or a shooting is subjected.
When the opening on the diaphragm is acquired, the diaphragmatic hernia takes the specific name of acquired diaphragmatic hernia.
Although very rarely, it is possible that surgical incisions, performed during operations on the abdomen or chest, are responsible for penetrating trauma to the diaphragm. Therefore, in fact, even abdominal / thoracic surgery represents a possible cause of diaphragmatic hernia. acquired.
To be more precise, doctors indicate this particular condition with the definition of iatrogenic diaphragmatic hernia.
EPIDEMIOLOGY
Studies relating to the mortality rate of congenital diaphragmatic hernia report that the latter is lethal in 40-60% of cases. This confirms what was said at the beginning of the article regarding the danger of diaphragmatic hernia in very young children.
Similar research, this time relating to the mortality rate of acquired diaphragmatic hernia, has shown that 17% of cases, in which the diaphragm suffers a serious rupture, is fatal.
Symptoms and Complications
Congenital diaphragmatic hernia and acquired diaphragmatic hernia share most of the symptoms and signs.
A typical symptom picture includes:
- Respiratory difficulties. In the presence of a "congenital diaphragmatic hernia, breathing difficulties are the result of insufficient (or abnormal) development of the lungs (pulmonary hypoplasia); in the presence of an" acquired diaphragmatic hernia, however, they are the result of inadequate functioning of the lungs . In this second circumstance, the functioning of the lungs is affected by the abdominal viscera which, being in the thoracic cavity, pushes the lungs and deprives them of the necessary space during the respiratory act.
- Cyanosis. Cyanosis is a typical consequence of an "insufficient quantity of oxygen in the blood. To determine the onset of this condition - which coincides with the presence of a bluish-purplish color of the skin - are the breathing difficulties, described in the previous point. Breathing, in fact, is the act that allows the blood to "charge" with oxygen, oxygen that the same blood carries to the various organs and tissues of the body.
- Tachypnea. It is the medical term for accelerated breathing or, rather, an increase in the number of breaths per minute.
- Tachycardia. It is the condition in which the resting heart rate exceeds the normal limits. In other words, it is when the resting heartbeat is accelerated.
In the case of diaphragmatic hernia (both congenital and acquired), it arises in response to breathing difficulties and the reduced presence of oxygen in the blood. In fact, as the heart rate increases, so does the blood flowing into the lungs, to "charge" with oxygen. Unfortunately, in patients with diaphragmatic hernia, the lungs function badly, so tachycardia is completely useless. - Decrease or total absence of respiratory sounds. These are two typical signs of congenital diaphragmatic hernia, as they appear when there is underdevelopment of the lungs (pulmonary hypoplasia).
- Perception of classic sounds and bowel movements at the level of the thoracic area. This is typical of patients with diaphragmatic hernia characterized by the protrusion of a part of the intestine into the thoracic cavity.
- Perception of an emptied abdomen. It is the consequence of the fact that one or more abdominal viscera have moved into the chest.
These symptoms vary in severity in relation to the causes: the more severe they are, the more the symptoms and signs can lead to the onset of unpleasant consequences.
OTHER INJURIES ASSOCIATED WITH "ACQUIRED DIAPHRAGMATIC HERNIA
Acquired diaphragmatic hernia is often associated with other conditions, which are also the result of impact or penetrating trauma.
These conditions include head injuries, aorta injuries, long bone or pelvic bone fractures, liver lacerations and spleen lacerations.
COMPLICATIONS
If not treated promptly, a diaphragmatic hernia can cause pulmonary hypertension and / or lung infections.
Diagnosis
Regarding congenital diaphragmatic hernias, doctors have the possibility to diagnose them even before birth, by means of a simple prenatal ultrasound examination (prenatal ultrasound). This should not come as a surprise, since, as we said in the chapter dedicated to the causes, the opening on the diaphragm is the result of an error occurring during fetal development.
Another sign of some diagnostic importance (always in the case of a possible congenital diaphragmatic hernia) is the presence of a "high quantity of amniotic fluid (ie the fluid that surrounds and protects the fetus, inside the" uterus).
As for acquired diaphragmatic hernias (and obviously congenital ones not diagnosed before birth), their identification is based on the execution of various diagnostic tests and examinations. The first step generally consists in the physical examination; the following in various instrumental evaluations, including: X-rays, thoraco-abdominal ultrasound, CT scan of the thoraco-abdominal compartment and arterial blood gas analysis.
OBJECTIVE EXAMINATION
During the physical examination, the doctor:
- Analyze the movements of the chest and evaluate the presence of any abnormalities
- Assess the presence and severity of breathing difficulties
- See if your lungs are making sounds. The absence of sound in one or both sides of the chest indicates an abnormality in one or both lungs.
- Check for chest sounds or bowel movements.
- Palpate the abdomen to see if any organs are missing inside it. If some organs have moved into the chest, the abdominal cavity will be less full than normal.
INSTRUMENTAL TESTS
X-rays, thoraco-abdominal ultrasound and thoraco-abdominal CT scan are three instrumental tests that provide fairly clear images of the internal organs and tissues, allowing the doctor to observe their state of health.
Arterial blood gas analysis, on the other hand, allows an evaluation of lung function, of the efficiency of gas exchanges inside the alveoli and of the oxygen levels circulating in the blood.
Treatment
Diaphragmatic hernia is a medical emergency that can only be treated by surgery.
When it is congenital, the ideal time to perform the surgery is 24-48 hours after birth.
WHAT DOES THE OPERATION CONSIST OF?
The surgery involves general anesthesia, incision of the chest (in the point where the diaphragm resides), the repositioning of the abdominal organs that have escaped into their natural cavity, repair of the diaphragm (in the point where it has the opening) and closure of the thoracic incision with sutures.
Awakening from general anesthesia occurs as soon as the anesthetist stops administering the anesthetic drugs.
SPECIAL FEATURES OF THE TREATMENT OF CONGENITAL DIAPHRAGMATIC HERNIA
Before each congenital diaphragmatic hernia surgery, surgeons subject young patients to a medical procedure known as extracorporeal membrane oxygenation (ECMO).
ECMO consists of a complex device, called membrane oxygenator, which replaces the function of the lungs and provides for the oxygenation of the blood of the individual to which the same device is connected.
The membrane oxygenator, in fact, is made in such a way that, once connected to the patient, it collects, oxygenates and reinserts the patient's blood into the circulation.
The connection to the ECMO can last for almost the entire hospitalization, as the lungs, especially in the first moments of the post-operative phase, need to rest and recover.
In fact, the ECMO is equivalent to a heart-lung machine for adults.
Prognosis
The prognosis of a "diaphragmatic hernia is affected by various factors, including in particular:
- The degree of involvement of the lungs. If lung health is severely compromised, the patient's life is in serious danger.
- The timeliness of the treatment. Late treatment, even if the diaphragmatic hernia is not serious, can compromise the patient's health and complicate the healing process.
- The presence of traumatic lesions in other anatomical sites. This factor specifically affects the prognosis of acquired diaphragmatic hernia of traumatic origin.
According to some US statistical studies, the current survival rate for treated cases of congenital diaphragmatic hernia is over 80%.
Prevention
If the acquired diaphragmatic hernia is, in some way, preventable by acting on the risk factors, the congenital hernia is a condition for which there is no preventive remedy.
