Two Rare Cases Of Rupture Type B Aortic Dissection With Right Hemothorax

Case Report

Two Rare Cases Of Rupture Type B Aortic Dissection With Right Hemothorax

*Corresponding author: Dr. Chih-Chien Yen, Division of Cardiovascular surgery, Department of Surgery, Taoyuan Armed Forces General Hospital, Taiwan, No. 168, Zhongxing Road, Longtan Dist, Taoyuan City, Taiwan. Tel: 886-3-4799595; E-mail: jordan721102@gmail.com

Abstract

This paper presents two cases of ruptured type B aortic dissection with right hemothorax. Closed pneumothorax refers to cases in which the chest wall is intact, such that a rupture of the lung and visceral pleura (or airway) allows air into the pleural space. Patients presenting spontaneous breathing can have an equilibration of pressures across the collapsed lung. In this situation, the patient may present symtpoms; however, the condition tends not to be life threatening.Open pneumothorax refers to an opening in the chest wall (with or without lung puncture), which allows atmospheric air to enter the pleural space. This situation is commonly encountered with penetrating trauma, such as stab wounds, gunshots, or impalement. Pressure changes in the chest associated with breathing cause air to move in and out of the chest through the opening in the chest wall. This situation can look very bad and sound even worse; however, the opening acts as a vent, which limits the accumulation of undue pressure from air trapped in the chest.

Hemothorax refers to the presence of blood in the pleural space. The source of blood may be the chest wall, lung parenchyma, heart, or great vessels. Not all cases of hemothorax require drainage. A failure to prepare for high-volume blood transfusion, particularly in cases of great vessel rupture (ruptured type B aortic dissection into the right hemithorax), can exacerbate negative pressure in the chest. When dealing with these cases, it is important to follow best practices, which means that the attending physician must make an effort to avoid profiling the patient and be prepared to reassess cases with acute complaints.

In this paper, we report on two patients with spontaneous right hemithorax. Many of these cases are silent presentations of ruptured of type B aortic dissection. Not all cases require drainage, and chest tube thoracostomy can be life threatening. Posterioranterior chest X-ray should be used to check for ruptured type B aortic dissection into the right hemithorax. Despite the infrequency of this condition, it should be considered by ER staff. The prompt diagnosis of right hemothorax due to ruptured type B aortic dissection and management of aortic dissection are essential to reducing morbidity and mortality.

Keywords: type B aortic dissection; hemothorax

Introduction

Cases of trauma-related hemothorax are frequently encountered in emergency departments; however, cases of non-traumatic or spontaneous hemothorax are encountered infrequently. Potentially fatal intrathoracic bleeding can be caused by aortic dissection, vascular malformations, oncological diseases, and abnormalities in connective tissue. In rare instances, it can be caused by extramedullary hematopoiesis, ectopic pregnancy, congenital heart defects, amyloidosis, or parasitic disease. Etiologic heterogeneity can impede diagnosis post-mortem, particularly when dealing with cases of hemothorax on the right side. Physicians must remember that hemothorax on the right side (even after low-energy trauma), does not necessarily indicate traumatic hemorrhage [1]. Trauma teams and clinicians should be aware of the possibility of ruptured type B aortic dissection giving rise to fatal hemothorax on the right side.

Hemothorax is seldom caused by the rupture of a large vessel in the chest. Generally, this condition is caused by the rupture of an aneurysm or aortic dissection with flow into the pleural cavity. The rarity of this complication often delays diagnosis, resulting in high mortality [2]. Aortic dissection is very rare in young children [3]; however, it is not unknown, particularly in those with congenital heart disease or connective tissue disorders as well as those subjected to severe trauma [4]. Early diagnosis and treatment is crucial [5]. Aortic dissection Type B is defined as the appearance of a false lumen distal to the outlet of the left subclavian artery. In some cases, this type of dissection extends toward the descending thoracic aorta and abdominal aorta. Fatal complications of type B aortic dissection include rupturing of the thoracic aorta, leg ischemia, visceral ischemia, and renal failure [3-6]. The most frequent complaint is severe pain, and the migration of pain is the most important clue for diagnosis [7].

Case Reports

Case 1

A 45-year-old woman with a history of hypertension and chronic renal failure was admitted to the emergency department of our regional hospital. The patient presented sudden pain onset in the interscapular region suggestive of aortic rupture. Upon admission, the patient scored 15 on the Glasgow Coma Scale (GCS) (E4M6V5) with the following vital signs: blood pressure (160/100 mmHg); heart rate (100 beats/min); respiratory rate (20 breaths/min); and body temperature (36.0 °C). The physical appearance of the patient was characterized by conjunctival palor, shortness of breath, and pain in the interscapular region. During emergency room evaluation, the patient complained of severe pain on the right side of the chest and the development of abdominal pain. Suspecting aortic dissection, the attending physician (the author) obtained computed tomography (CT) scans of the chest and abdomen, which supported the diagnosis of aortic dissection type B.

Figure 1: Posterior-anterior chest X-ray of a 45-year-old woman showing opacification of the right hemopneumothorax with widening of the aortic contour and mediastinum

Figure 1 presents a chest X-ray showing the area affected by spontaneous right hemithorax.  The enhanced CT scan in Fig. 2 shows mediastinal hematoma and what appears to be aortic dissection in the aortic arch and descending aorta, which was diagnosed as acute Stanford type B dissection. Following chest tube intubation for the removal of massive blood clots, the patient was prepared for surgical intervention. One hour after admission (while still in the ER), the patient began experiencing difficulty breathing and notable oxygen desaturation. A subsequent CT scan of the chest and abdomen revealed ruptured type B aortic dissection presenting with right hemothorax and massive hemomediastinum.

Fig. 2. Non-contrast axial CT scan showing acute intramural haematoma (IMH) type B, involving ascending, arch, descending aorta, Hemomediastinum. Acute intramural haematomas appear as crescentic, high-attenuating (60-70 HU) regions as a thickening of the aortic wall.

Fig. 3. Post-contrast CT scan in which lesion exhibit low attenuation in relation to the aortic lumen; Contrast enhanced axial CT scan shows the hyperattenuating right pleural effusion, hyperattenuating mediastinal hemorrhage, and type B aortic dissection.  

Case 2

In the following, we present the case of a 14-year-old boy referred to the emergency department after presenting with sudden onset severe chest pain, palpitations, cold sweating, and an inability to stand on his own. The sudden onset of chest pain was first reported early that morning. The patient denied any contributory medical, surgical, or medication history, previous musculoskeletal trauma, or neurologic symptoms. The patient also denied the presence of cardiovascular disease or risk factors such as hypertension, dyslipidemia, or diabetes. Upon arrival, the patient scored 15 on the Glasgow Coma Scale (GCS) (E4M6V5), and the vital signs were as follows: blood pressure (151/94 mmHg); heart rate (126 beats/min); respiratory rate (20 breaths/min); and body temperature (36.0 °C). The physical appearance of the patient was characterized by conjunctival palor, chest pain, shortness of breath, and diaphoresis. While undergoing examination in the ER, the patient complained of increasingly severe chest pain and newly developed abdominal pain. Suspecting aortic dissection, the attending physician (the author) obtained computed tomography (CT) scans of the chest and abdomen, which showed massive hemomediastinum, right hemothorax, and aortic dissection. Under the assumption of aortic dissection with aortic perforation at carinal level, the patient was prepared for an emergency for endovascular thoraco-abdominal aortic dissection repair and circumstances urgent thoracentesis was performed, resulting in over 5000 mL of hematic fluid. The surgeon encountered a massive right pneumothorax including massive pleural effusion on the right side with underlying parenchymal alterations. Following a rupture of the aorta during the operation, the patient suffered cardio-respiratory arrest and died (23 Dec 2015; 18:27).

The X- ray in Fig. 3

shows a right pleural effusion without deviation of mediastinum. The thoracic CT scan in Fig. 4 shows a thoracic aorta dissection with intimal flap, which was deemed responsible for the hemothorax.

Fig. 4. Posterior-anterior chest X-ray of 14-year-old boy presenting with hemopneumothorax on the right side

Fig. 5. Non-contrast axial CT scan showing acute intramural hematoma (IMH) type B, involving ascending, arch, descending aorta, hemomediastinum.

Fig. 6. Contrast enhanced axial CT scan showing hyperattenuating right pleural effusion, hyperattenuating mediastinal hemorrhage, and type B aortic dissection.

Discussion

Not all cases of pneumothorace require the insertion of a chest drain when they arrive at the ER. Most cases of primary spontaneous hemopneumothorax (in patients under the age of 50 without history of underlying heart or lung disease) can initially be treated via simple aspiration. The two patients in this paper presented with similar symptoms; however Case 1 survived whereas Case 2 died. Thinking and plan to the value of endovascular thoracoabdominal aortic dissection repair and thoracotomy before also need prepare a lots of blood transfusion ; It is not uncommon for blood loss to exceed the amount gained through blood transfusion, particularly in cases involving the rupture of great vessels. ( ruptured type B aortic dissection into the right hemithorax ) in negative pressure in chest that will get worse at all. Be careful in ruptured type B aortic dissection presenting with right hemothorax to accentuate keep in mind in Emergency Department.

 These two cases clearly illustrate the silent clinical presentation of right hemithorax resulting from ruptured type B aortic dissection. These cases also illustrate how physicians must try to avoid profiling the patient and be prepared to reassess cases with acute complaints. We report a patient with spontaneous right hemithorax which often have a silent clinical presentation and ruptured of type B aortic dissection; ruptured type B aortic dissection into the right hemithorax to call attention to this infrequent presentation and to accentuate keep in mind in emergency department. Hemothorax is generally the result of closed chest trauma; however, a number of cases referred to as spontaneous hemothorax can also be caused by various malignancies, anticoagulation therapy, or the rupture of an aneurysm.

Rupturing of an aortic dissection is relatively uncommon, but it is the most common non-traumatic cause of hemothorax in deaths, which presents with severe chest pain and hemodynamic compromise [8]. Cases of painless dissection are uncommon (6.4 percent); however, the associated risk of mortality is higher [9]. The incidence of aortic dissection ranges from 2.6 to 3.5 per 100,000 person-year. It is generally seen in elderly males (60 to 80 years) [10]; however, the 2 patients described in this study were much younger. Hemothorax occurs in approximately 10% of cases involving rupture of the descending aorta. They are usually located on the left side [11]; however, they can also occur on the right, as in Case 2 in this paper.

Once identified, hemothorax should be characterized according to vascular etiology as a dissecting aneurysm, arteriovenous malformation, arteriovenous fistula, vascular tumor, coagulopathy, neurofibromatosis, or exostoses [12]. The medical history of the patient and coagulation parameters should be considered, and contrast enhanced thoracic CT can yield crucial information pertaining to etiology. CT is the most common diagnostic test initially performed in cases where acute aortic dissection is suspected. CT scans make it possible to detect acute aortic dissection with a sensitivity and specificity of nearly 100%. As shown in Fig. 1, CT scans reveal an intimal flap in roughly 70% of cases involving acute dissection. This can be helpful in identifying the site and extent of dissection [13].

Management depends on the cause. In cases of anti-coagulant induced bleeding, correction of the coagulopathy may be all that is required, as in Case 1 in this paper [8]. Thoracostomy drainage is generally the first step for stable patients; however, an early surgical approach with ongoing resuscitation is preferred for patients presenting hemodynamic instability [14].  The descending aorta is close to the left pleural cavity; however, aortic dissection with rupture is a potentially fatal condition that can cause right hemothorax, albeit only rarely. Nonetheless, this unusual condition should always be considered in cases of hemothorax. Identification of the rupture site and accompanying complications is crucial for planning any surgical intervention; therefore, CT scans should be the first diagnostic test performed in such cases.  It is crucial that ER staff and clinicians be aware of all conditions that could potentially give rise to fatal hemothorax on the right side.

Conclusion

The clinical diagnosis of aortic dissection depends upon the characteristics of pain; however, the symptoms are not necessarily remarkable in children. Therefore, in cases of atypical ruptured type B aortic dissection presenting with right hemothorax, aortic dissection should be ruled in for its life-threatening outcomes. Hemothorax refers to the presence of blood in the pleural space. The source of the blood may be the chest wall, lung parenchyma, heart, or great vessels. A failure to prepare for blood transfusion, particularly in cases of great vessel rupture (ruptured type B aortic dissection into the right hemithorax), can exacerbate negative pressure in the chest. Not all cases require drainage. Physicians working in an acute care setting and particularly in the ER should be aware of disorders predisposing patients to acute aortic dissection [14].

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