Surviving a delayed trans-diaphragmatic hepatic rupture complicated by an acute superior vena cava and thoracic compartment syndromes.

We describe the first reported survivor of a delayed trans-diaphragmatic hepatic rupture complicated by acute superior vena cava (SVCS) and thoracic compartment syndromes (TCS). A thirty one year old male was involved in a boating accident. The patient was diagnosed with a grade IV liver laceration, which was initially managed with both angio-embolization and open surgical repair. Exactly one month from admission, the patient presented with an abrupt cardiac arrest, which was further complicated by a SVCS and TCS. The SVCS was managed with bilateral thoracostomies which revealed a delayed trans-diaphragmatic hepatic rupture into the right chest cavity. The TCS was managed with a decompressive thoraco-abdominal incision. The patient survived and is now leading a normal life. Our success was largely due to an integrated trauma system of physicians, nurses and technicians that prompted the early recognition of two potentially life threatening complications of a delayed trans-diaphragmatic hepatic rupture.

INTRODUCTION

Rupture of the diaphragm in itself is a rare complication of blunt thoraco-abdominal trauma, occurring in up to 5% of cases.[1] Right-sided diaphragmatic rupture is even rarer, comprising only 5-35% of all diaphragmatic ruptures.[23] Of those cases, only 19% involve herniation of intra-abdominal organs into the pleural cavity.[3] In this article, we present a case that defied the odds while presenting late in the hospital course, further complicating the diagnosis.

CASE REPORT

A thirty one year old male was involved in a boating accident in which he suffered a severe right-sided thoraco-abdominal trauma. His past medical history of significance was bilateral ankle surgery several years ago. The patient was airlifted to our regional trauma center located in Florida, where a grade IV liver laceration was diagnosed. The patient was initially managed with both angio-embolization and open surgical repair. The rest of the abdominal organs, including bilateral diaphragms, were all manually inspected during the initial laparotomy and no additional injuries were found.

Exactly one month from admission, patient presented with an abrupt cardiac arrest in the ICU following an episode of melenotic stool. During heroic maneuvers of resuscitation, a total of four units of packed red blood cells and four units of fresh frozen plasma were transfused. The patient's precarious situation was complicated further by the acute presentation of a superior vena cava (SVCS) and thoracic compartment syndromes (TCS). SVCS was diagnosed clinically by the acute presentation of profound head, neck, and upper torso cyanosis. This was managed with bilateral thoracostomies which revealed the etiology of the patient's cardiac arrest: A delayed trans-diaphragmatic hepatic rupture into the right chest cavity. TCS was clinically diagnosed by the acute onset of “stiff lung,” which was relieved with a decompressive thoraco-abdominal incision. The patient was taken back to the operating room 24 h later for definitive repair of his delayed hepatic rupture by performing a subtotal right hepatectomy and cholecystectomy, as well as an abdominal wall and right thoracotomy closure. The patient survived and was discharged on hospital day sixty-five. He had monthly follow up in the trauma clinic for eight months, and is now leading a normal life.

DISCUSSION

A high clinical index of suspicion is needed to diagnose and effectively manage diaphragmatic ruptures even with a remote history of high-velocity injury, particularly true when other signs of severe trauma are present.[1] Diaphragmatic rupture in blunt trauma is thought to result from the transmission of forces through the abdominal viscera to the diaphragm. The incidence of rupture of the diaphragm secondary to blunt or penetrating trauma of the chest or abdomen is 4.5-5.8% and up to 30% of them present late. Ruptures of the right hemi-diaphragm account for 13-50% of diaphragmatic injuries secondary to blunt trauma. Earlier series are remarkable for a preponderance of left-sided injuries and some have proposed that the liver protects the right hemi-diaphragm.[2] Although autopsy studies have revealed equal incidence of right and left diaphragmatic ruptures, ante-mortum study reports suggest 88-95% of diaphragmatic ruptures occurred on the left side. Right sided ruptures are associated with high mortality and morbidity and thus the under diagnosis of right sided injuries may be due to greater pre-hospital mortality.[1]

To reduce the morbidity and mortality of ruptured diaphragm, it must be suspected and looked for in all cases of blunt trauma. Two pathophysiological explanations of delayed rupture have been discussed in the literature. The first, and most widely accepted, mechanism describes the progressive widening of initially occult diaphragmatic lacerations.[3] The second mechanism describes the role of an inflammatory response and the gradual weakening of the diaphragm until it is unable to maintain its own integrity.[4] The injured liver may regenerate with fibrosis or undergo further ischemic necrosis with abscess formation, rupture and/or hemorrhage. It is most likely in our case that a combination of these two mechanisms led to the eventual diaphragmatic rupture.

Rupture of a hepatic subcapsular hematoma is a relatively rare cause of sudden death following minor blunt abdominal trauma.[2] Death may be delayed several days to weeks as the liver undergoes necrosis that some describe as traumatic hepatic sequestrum.[5] The abdominal hemorrhage can present exclusively in the chest in the presence of an associated diaphragmatic rent, as evidenced by this case.

Malignancies cause 70-97% of the cases of SVCS. Typically, the onset of symptoms in SVCS occurs gradually over weeks to months. The development of acute SVCS in the immediate peri-operative period is rare.[6] In this case, an acute SVCS developed instantaneously as a result of a massive right-sided tension hemothorax from a delayed transdiaphragmatic hepatic rupture. The clinical diagnosis of the SVCS was based on the acute presentation of a profound cyanosis of the patient's upper body. The acute SVCS cyanosis is a clinical sign that results from the effective obstruction of its blood.[7]

Compartment syndrome occurs when compartment pressure exceeds tissue perfusion pressure. This syndrome can, however, occur in a circumscribed body cavity such as the abdomen or the thorax. TCS is well-known in the cardiac literature but is still developing in the non-cardiac thoracic/trauma literature. Keys to the diagnosis and treatment of this syndrome include the early recognition of the increasing airway pressures, falling cardiac output, and worsening acidosis. Treatment should include immediate decompression of the chest and maintenance of the open chest until the patient demonstrates correction of the previous hemodynamic instability.[8-10] TCS was managed in this case with a decompressive thoraco-abdominal incision as part of his damage control surgical management.

CONCLUSION

Our success was largely due to an integrated trauma system of physicians, nurses and technicians that prompted the early recognition of two potentially life threatening complications of a delayed trans-diaphragmatic hepatic rupture. The trauma systems key interventions began with the first responders being able to quickly determine the severity of injury and request air transport. The nursing staffs’ prompt recognition of the patient's downturn in the intensive care unit and the anesthesiologist's awareness to communicate with the trauma surgeon regarding the inability to ventilate during the aggressive resuscitation were vital to the patient's positive outcome.