La hernia diafragmática congénita de Bochdalek se manifiesta frecuentemente como un distrés respiratorio grave del recién nacido y constituye una urgencia. The Congenital Diaphragmatic Hernia (CDH) is an anatomical structural defect, Download full-text PDF hernia diafragmática congénita y su evolución. A diaphragmatic hernia is a birth defect, which is an abnormality that occurs as a fetus is forming in the mother's uterus. An opening is present in the diaphragm (the muscle that separates the chest cavity from the abdominal cavity). A diaphragmatic hernia allows abdominal organs.
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¿Qué es una hernia diafragmática? Una hernia diafragmática es un defecto congénito, una anomalía que se produce cuando el feto se está formando en el. Morgagni-Larrey Hernia: A Rare Cause of Pericardial Effusion. Hernia diafragmática de Morgagni-Larrey: una rara causa de derrame Download PDF. Pseudodextrocardia Secondary to Diaphragmatic Hernia. Seudodextrocardia secundaria a hernia diafragmática. Visits. Download PDF. Miguel A. Ariasa .
The defect can be so serious that it is incompatible with life 7. Surgical care is based on the reduction of the herniated viscera and closure of the diaphragmatic defect with sutures or, depending on the size, placement of a patch or an abdominal wall flap. There are intrauterine strategies aimed at improving the development of the lungs before birth.
One of these strategies is the fetoscopic pulmonary occlusion that stimulates the proliferation of the distal airway and improves the prognosis in these patients. However there are risks involved such as premature membrane disruption and pre-term delivery 14, Excessive hyperventilation should be avoided in the post-natal period.
The actual recommendation is "gentle ventilation" as described by Wung et al. The newborn must be intubated immediately after birth, avoiding a facemask positive pressure during pre-oxygenation, which causes gastric distension and worsens the pulmonary compression.
The stomach has to be decompressed using a continuous suction nasogas-tric tube. La hipercapnia permisiva ha mostrado incrementar la supervivencia 12, La asistencia respiratoria de alta frecuencia se reserva para aquellos pacientes que no responden a la convencional y persisten con hipoxia e hipercap-nia. The mean blood pressure should be kept above 50mm Hg to reduce the right to left shunt. This goal is achieved with a conscientious input of isotonic fluids, and if needed, administering inotropic agents such as dopamine and dobutamine.
Conventional, pressure-controlled respiratory support is recommended, with a respiratory rate between 30 to breaths per minute, a peak inspiratory pressure between 20 to 25 cm of H2O and physiological PEEP.
Permissive hypercap-nia has shown to increase survival 12, The respiratory support parameters are adjusted according to the arterial gases report and oxygen weaning should be done with extreme care to prevent pulmonary hypertension.
High frequency respiratory support is reserved for those patients that fail to respond to the conventional therapy and have persistent hypoxia and hypercapnia. There are some reports of improved survival in these patients with this type of respiratory support ECMO is indicated for patients that fail to respond to other treatment strategies and it is a useful approach to reduce the risk of barotrau-ma and in patients with severe pulmonary hypertension.
This latter condition has also ben-efitted from the use of prostaglandins, milrinone and nitric oxide.
The ELSO guidelines define the use of Extra-Corporeal Life Support ECLS as the use of mechanical devices to support the cardiopulmonary function partially or totally on a temporary basis days to months during cardiopulmonary failure, giving some time for recovery of the patient or organ transplant.
There are two types of ECMO: venoarterial VA , that replaces the heart and lung function either partially or totally and venousvenous VV , when the heart output depend entirely on the function of the native heart. ECMO is better when it is administered during the first seven days of mechanical assisted ventilation. Some contraindications include: lethal chromosomal disorders, conditions incompatible with normal life if the patient recovers, pre-existing conditions that impact the quality of life CNS status, terminal malignancy, risk of systemic bleeding with anticoagulation , age and size of the patient and patients who are too sick, have been in conventional therapy for too long or have a fatal diagnosis.
Intracranial hemorrhage or postsurgical conditions that increase the risk of bleeding neurosurgery or intracranial procedures; surgery or trauma are also excluded. Extracorporeal Circuit Blood flow for cardiac support. Blood flow and gas exchange for respiratory failure.
The pulmonary membrane and the blood flow must meet the normal metabolic needs. If the circuit is partial and programmed only for removing CO2, the access may be ve-nousvenous, venousarterial or arteriovenous. In neonates with respiratory failure, vascular access is through the neck, with catheterization of the internal jugular artery and the common carotid artery. The circuit includes a blood pump, a pulmonary membrane, tubes and ducts. Estas variables determinan el tipo de membrana. Se agregan cristaloides al circuito.
Es necesario un intercambiador de calor. The pulmonary membrane is a porous silicon or polymethyl-pentene membrane and the gas exchange capacity is determined by the flow rate and the maximum oxygen output. The flow rate refers to the venous blood that will be totally saturated when the blood exits the pulmonary membrane. The maximum oxygen output is the volume of oxygen released per minute, calculated as the difference between the oxygen content going in and out of the membrane.
These variables determine the type of membrane. It is important to maintain a higher pressure on the arterial side than on the venous side to prevent the development of gas embolism and to keep the pump below the patient's level.
Crystalloids are added to the circuit. You may also add A heat exchanger should be available. The blood flow, the blood pressures, the O2 saturation are all measured before and after the membrane and the blood gases. During ECMO, special attention should be given to the blood flow circuit pressures, air, clots , the oxygenation, CO2 elimination, activated clotting time values and timetable for adjusting the clotting time to 1,5 of the normal during the heparin infusion, the number of platelets and fibrinogen levels.
The ideal timing to repair a congenital diaphragmatic hernia in patients with extracorporeal membrane oxygenation differs among the various institutions. Experienced surgeons must carry out the procedure, with particular emphasis on hemostasis when anticoagulation is used.
It is recommended to follow the patients closely to identify any neurological development problems. The mode of delivery of choice for infants with CDH is unknown. However, this was not a randomized trial There are some conditions that seem to impact survival rates in cases of congenital diaphragmatic hernia. From the clinical perspective, respiratory distress in the first six hours after birth reflects a more severe lung involvement and relates to higher mortality rates.
It is very important to ensure adequate analgesia and sedation, in addition to the most stringent control of the acid-base balance. Its clinical presentation ranges from mild symptoms to symptoms incompatible with life, depending on the severity of the pulmonary hy-poplasia and on concomitant pathologies. Prenatal diagnosis is key to develop a suitable management plan that includes taking care of the patient at a specialized institution and support by a multidisciplinary team.
A comprehensive evaluation should be done in an attempt to identify any cardiopulmonary disorders, hydro-electrolytic and acid-base imbalances that may further aggravate the poor pulmonary function. One of the main issues related to the management of anesthesia in these patients is ventilation. Depending on the severity of the case, the patient may receive assisted pressure control ventilation , high frequency assisted oscillatory ventilation and extracorporeal membrane oxygenation.
The prognosis of this particular case was gloomy because of a bilateral pulmonary involvement. An interdisciplinary evaluation with the participation of the group of anesthesiologists of the institution from the prenatal stage would have been helpful in planning the postnatal surgical management.
Basic monitoring was provided for preventing hypoxic crisis, which in fact occurred twice while the patient was being transferred. These crises were detected and managed promptly. Respiratory management was extremely helpful. We used assisted pressure controlled ventilation in an attempt to secure the lowest possible airway pressures, administering inotropic agents and indodilators i.
The postoperative outcome was consistent with the prognosis. Hartnett KS. Congenital diaphragmatic hernia: advanced physiology and care concepts. Adv Neonat Care.
En: Miller R. Anestesia de Miller. Madrid: Elsevier; Steinhorn RH. Congenital diaphragmatic hernia [Internet]. Omaha: E-medicine; Genetic factors in congenital diaphragmatic hernia. Am J Hum Genet.
Moderate anterior basal pericardial effusion. Figure 2. Chest x-ray. The posteroanterior projection PA demonstrates the presence of air obscuring the internal cardiac border and linear limit at its apex. The lateral projection Lat confirms the basically anterior position, with images of air that present portions of the intestine in their interior.
Although cases have been reported in fetuses and newborn infants, it is generally produced as a consequence of acquired mechanisms that increase the intraabdominal pressure, resulting in herniation of the abdominal organs into the thoracic cavity through a congenital anatomical defect in the left sternocostal triangle of the diaphragm.
In adults, it usually goes undetected and the diagnosis is incidental, whereas in children, it frequently produces respiratory symptoms dyspnea, tachypnea, and recurrent pneumonia. However, the slow development of the effusion due to the direct irritation of the pericardial sac can result in the accumulation of a significant amount of fluid with no hemodynamic impact.
Thus, it would appear to be reasonable to adopt an initial conservative approach and perform definitive surgical treatment electively. They also found a 14 per cent possibility of bilaterality.
We report three cases of BH, two on the right side and one bilateral in which the largest hernia is on the right side. The size of a BH is usually highly variable, from a few millimeters to occupying most of the thorax, and it is not necessarily related to the size of the diaphragmatic defect 5,11,20 ; 20 per cent of BHs are contained by a sac, and the remainder show direct communication between thorax and abdominal cavity BHs typically contain omental fat, which can be accompanied by the stomach, spleen, colon, and even the small intestine 10,11,16,20,22, The involvement of the liver, gallbladder, pancreas, kidney, or retroperitoneal fat is rare 5 , and there has been no report of a lung component in adult BH In all our cases, the content was omental fat alone.
However, in one of them, the right kidney was adjacent to the diaphragmatic defect but inside the abdomen Figs. In newborns, the protrusion of the liver inside the right hemithorax is typically due to a CDH.
In these cases, the liver will cause a mass effect and displace the mediastinum towards the left. However, if the mediastinum is not displaced, other less common causes for intrathoracic liver should be considered, such as primary right pulmonary hypoplasia e.
Most congenital BHs are associated with a respiratory insufficiency 21 that becomes evident during the first weeks of life, and they are among the most frequent causes of respiratory distress in neonates. In adults, most BHs are usually asymptomatic 16 and their detection is incidental, as in our three patients. Symptoms, if any, are typically imprecise.
Patients usually report chest pain or gastrointestinal symptoms 5,7, A case was reported of a yr-old woman in the second trimester of pregnancy with symptoms of retching, oliguria, and shortness of breath that were initially attributed to the pregnancy itself but finally proved to be caused by a left BH with acute gastric volvulus One of our patients was hospitalized for intense epigastric pain, but its relationship with the BH could not be demonstrated.
There have been rare reports of late presenting BH with gastric volvulus, spleen rupture, obstruction, and gastrointestinal perforation 23,25, Congenital BHs can be diagnosed by ultrasound, even in the prenatal period, as a displacement of the mediastinum and a mass in the thorax of the fetus 2. Chest-abdominal X-rays of a neonate with BH and respiratory distress show a soft tissue mass in the thorax that reveals gas or hydro-air levels when the neonate swallows air.
X-rays with radiopaque contrast may be useful before surgery to assess the herniated intestinal loops and their possible malrotation 10, Occasionally, a BH can appear as a lesion with soft tissue density in the posterior area of the pulmonary base in lateral chest X-rays 6,11 , which may be confused with a pulmonary lymph node adjacent to the diaphragm 27 or, as in one of our cases, with a posterior diaphragmatic lobulation Figs.
Using CT, however, a BH can be readily differentiated from a diaphragmatic eventration or lobulation "bosselation" by examining the continuity of the diaphragm muscle itself. In BH, the muscle always shows a break or defect, whereas it is always whole, though thinned, in the other two conditions In two of our patients, with known malignant disease, the predominant clinical indication for CT was to rule out metastatic disease, as reported by other authors 5. Examination by multi-slice CT, with the possibility of coronal and sagittal reconstruction, should be considered the standard method to diagnose BH 5.
Some authors recommended replacing conventional axial images with coronal reformatted images, because there are fewer images and less time is required for interpretation However, it has been verified that the lack of familiarity of observers with coronal visualization may contribute to detection errors and the missing of defects CT and MRI studies are highly valuable for the assessment of fat-containing thoracic lesions 6 , detecting, localizing, and identifying lesions and markedly reducing the time required for the differential diagnosis Figs.
CT or MRI diagnosis can be definitive. In BHs, sagittal and coronal reformatted images can reveal the diaphragmatic defects and hernia contents Figs. The treatment of incidental BH remains controversial 5. All DH types are assumed to have a congenital origin, but the clinical presentation, management, complications, and prognosis markedly differ between neonate and adult cases. The prognosis for late-presenting BH is usually more favorable 13,16 , as in the present patients, who have shown no clinical or imaging changes after a follow-up of up to six years.
Despite the generally symptom-free nature of incidental BH in adults, some authors recommend surgery, including hernia reduction and defect closure, arguing that the risk of tissue strangulation and even death warrants an aggressive approach 7.
The diagnosis of BH as an incidental finding in adulthood appears to be increasingly frequent. Given the growing prevalence of obesity in our society, there may be a connection between these phenomena, which would have public health implications 7.
The incidental finding of BH in asymptomatic adults appears to be increasing, largely due to the application of new imaging techniques. The use of CT or MRI offers greater precision in the diagnosis, localization and characterization of the hernia, facilitating its management and the choice of treatment. References 1. Diferencial diagnosis of fat-containing lesions with abdominal and pelvis CT.
RadioGraphics ; Imaging of abdominal hernias. CT and MR imaging of extrahepatic fatty masses of the abdomen and pelvis: techniques, diagnosis, differential diagnosis, and pitfalls. Radiographics ;