a 50-year - old man with multiple caf - au - lait spots on his body was transferred to the emergency department of kyungpook national university hospital after the sudden onset of severe dyspnea .
we were aware that he had type 1 neurofibromatosis ( nf-1 ) from a previous visit to our hospital 27 years previously , but he had not undergone any treatment . on arrival ,
his mental status was alert and he had a blood pressure of 119/87 mmhg , but he also exhibited tachycardia , with a heart rate of 130 beats per minute .
his initial hemoglobin level was 8.2 g / dl , his hematocrit value was 25.3% , and his platelet count was 6710/l .
a chest x - ray showed a massive opacification in the right lung field with a tracheal deviation to the left side ( fig .
a chest computed tomography ( ct ) scan with medium contrast confirmed a large right hemothorax and extravasation of the contrast medium from an internal mammary artery ( i m a ) aneurysm ( fig
after closed thoracostomy with a chest tube insertion , we removed approximately 1,000 ml of blood .
we planned two - stage management for the patient , using endovascular embolization for the proximal portion of the i m a , followed by surgery to evacuate the hemothorax and clip the distal portion of the i m a . immediately after starting the angiogram , the patient suffered cardiac arrest from hypovolemic shock . during cardiopulmonary cerebral resuscitation with volume replacement , we planned to perform emergency embolization through the right common femoral artery , and emergency angiography revealed a ruptured aneurysm in the right i m a .
the interventional radiology team occluded the proximal portion of the i m a with two 5-mm tornado embolization microcoils ( cook medical inc . ,
subsequently , superselection of the distal i m a was attempted . however , since the i m a was made discontinuous by an aneurysmal segment , the microcatheter could not reach the distal i m a , making distal embolization impossible .
nonetheless , the patient s hemodynamic status recovered , although his mental state could not be determined at that time . after confirming complete occlusion of the proximal portion of the i m a
, the patient was moved to the intensive care unit ( icu ) and hypothermic therapy was performed there . in the icu ,
the patient was semicomatose and a small amount of bleeding through the chest tube persisted .
serial chest radiographs revealed an increasing pleural effusion that was suspicious for blood in the right lung field .
three days later , the patient was taken to the operating room to drain the pleural effusion and to clip the distal portion of the i m a . through a video - assisted thoracic surgery ( vats )
a ruptured mediastinal pleura was observed in the thoracic inlet and a small amount of blood was found to be leaking through the distal portion of the i m a .
we clamped the distal portion of the i m a with two hemostatic clips ( fig .
after six months of follow - up , the patient has remained clinically asymptomatic without recurrent hemothorax .
spontaneous hemothorax caused by a ruptured aneurysm in nf-1 patients is very rare , but can be fatal .
vascular lesions can occur in any vessels , but in the thoracic cavity , ruptures of aneurysms in the intercostal arteries , subclavian artery , and internal mammary artery have been reported in patients with nf-1 . the incidence of vascular lesions in patients with nf-1 has been reported to be 3.6% .
reported that the most common location of artery rupture was the subclavian and intercostal arteries .
spontaneous hemothorax caused by aneurysmal rupture in nf-1 is very rare , but may be critical , especially if it occurs in a large artery .
chest pain , dyspnea , and syncope are the presenting symptoms of arterial aneurysmal rupture in the thoracic cavity . in our case ,
the diagnosis should be confirmed by selective angiography in order to plan an optimal therapeutic intervention .
an aneurysmal rupture in a patient with neurofibromatosis should not be corrected by vessel reconstruction .
surgical management is more aggressive and complex , and artery reconstruction is limited by arterial fragility [ 25 ] . in the case of the rupture of
an i m a aneurysm , the treatment depends on the patient s hemodynamic condition . in unstable conditions ,
surgical exploration includes removing the hematoma , surgical ligation for bleeding , and packing to achieve hemostasis .
however , this surgery is aggressive and problematic due to difficulties in finding the source of bleeding and performing surgical ligation associated with the fragile nature of vascular tissue .
miura et al .   reported that they failed to identify the cause of bleeding during surgery , so they terminated the procedure in a patient with an intercostal aneurysmal rupture . in patients who are hemodynamically stable , endovascular treatment is the best choice for arterial rupture in nf-1 patients .
coil embolization or stent grafts are often used in these cases because these treatments are safer and less complicated than surgery .
however , fatal rebleeding from the aneurysm or due to backflow from collateral branches has been identified as a possible life - threatening complication after an endovascular procedure .
a subsequent vats procedure may be indicated for cases of aneurysmal rupture in the thoracic cavity to prevent rebleeding and to improve the patient s symptoms . in our case ,
regardless of whether the patient had been hemodynamically unstable on arrival , we planned a two - stage management strategy that combined endovascular treatment and a subsequent vats procedure .
the interventional radiology team was already prepared when the patient was transferred to our hospital , and we were concerned about possible difficulties in finding the bleeding site through surgery .
first , coil embolization of the proximal rupture of the i m a was undertaken , while the distal portion of the i m a was later clipped using two hemostatic clips via vats .
a large arterial rupture in a patient with nf-1 is a life - threatening condition that can result in hypovolemic shock and severe dyspnea .
therefore , when encountering an nf-1 patient with a massive hemothorax , early recognition and treatment are necessary . in conclusion , we planned a two - stage management strategy to treat an i m a aneurysmal rupture associated with nf-1 .
although the patient required cardiopulmonary cerebral resuscitation , successful results were obtained by coil embolization and a subsequent vats procedure .
 the rupture of an internal mammary artery ( i m a ) aneurysm in a patient with type 1 neurofibromatosis ( nf-1 ) is a rare but life - threatening complication requiring emergency management . 
 a 50-year - old man with nf-1 was transferred to the emergency department of kyungpook national university hospital , where an i m a aneurysmal rupture and hemothorax were diagnosed and drained . 
 the i m a aneurysmal rupture and hemothorax were successfully repaired by staged management combining endovascular treatment and subsequent video - assisted thoracoscopic surgery ( vats ) . 
 the patient required cardiopulmonary cerebral resuscitation , the staged management of coil embolization , and a subsequent vats procedure . 
 this staged approach may be an effective therapeutic strategy in cases of i m a aneurysmal rupture . 
CASE REPORT
DISCUSSION