Spontaneous rupture of the pancreatic arcade artery caused by neurofibromatosis type 1 successfully treated using emergency transcatheter arterial embolization, partial intra-aortic balloon occlusion, and stent graft placement: a case report and review of the literature

Abstract

Background: Vascular abnormalities in neurofibromatosis type 1 (NF1) are rare, but are the second leading cause of death in persons with NF1. In NF1 vasculopathy (NF-V), fatal bleeding due to a spontaneous arterial rupture sometimes occurs. Ruptured extracranial arteries in patients with NF1 often involve thoracic vessels, such as the intercostal and subclavian arteries; very few reports exist regarding the abdominal region. Herein, we present the first case of intraperitoneal bleeding due to spontaneous pancreatic arcade artery (PAA) rupture associated with NF1, successfully treated by transcatheter arterial embolization (TAE) combined with stent-graft placement and partial intra-aortic balloon occlusion (IABO). Case presentation: A 40-year-old woman complained of back and abdominal pain. Upon admission, her blood pressure was 85/41 mmHg and heart rate was 129 beats/min. Computed tomography (CT) showed large intraperitoneal bleeding due to PAA rupture. After CT scanning, her systolic blood pressure decreased to 50 mmHg. Therefore, we performed emergency TAE with partial IABO. She was treated by TAE of the anterior superior pancreaticoduodenal artery, anterior inferior pancreaticoduodenal artery, and inferior pancreaticoduodenal artery. However, even after TAE, minor extravasation around the superior mesenteric artery continued, and her vital signs remained unstable. Stent-graft placement was selected to stop the haemorrhage, preserving normal blood flow of the superior mesenteric artery trunk. Excellent patency of the stent graft was confirmed on follow-up CT, and she was discharged on postoperative day 56.

A case of hemorrhagic shock in a patient with neurofibromatosis type 1

“…Surgical hemostasis is reported to be difficult due to vascular fragility. In cases of hemodynamic instability, IVR can be safely performed with REBOA, as in this case 3 …”

“…In cases of hemodynamic instability, IVR can be safely performed with REBOA, as in this case. 3 Vascular disorders are second only to malignancy in terms of cause of death in NF-1 patients. Since multiple vessels can be involved, it is important to systemically investigate for vascular involvement to prevent fatal hemorrhage.…”

Endovascular embolization and needle aspiration of a life-threatening cervical hematoma due to a neurofibromatosis type 1–associated arteriovenous fistula: illustrative case

“… 1–7 As previously reported, an open surgical approach could risk uncontrollable bleeding from the vascular wall infiltration by a neurofibroma. 3 , 4 , 6 , 7 Therefore, in cases in which the bleeding source is considered strongly related to a neurofibroma, treatment strategies avoiding unnecessary damage of vascular structures should be considered.…”

Parkinsonism-hyperpyrexia Syndrome After Amantadine Withdrawal

“…One rare complication related to treatment alterations is a parkinsonismhyperpyrexia syndrome (PHS), a disorder first described in 1981 in a patient with PD that presented with acute symptoms that resembled that of the neuroleptic malignant syndrome (NMS) after the abrupt discontinuation of antiparkinsonian agents. 2 Patients with PHS may present with signs of central dopaminergic depletion related such as hyperthermia, rigidity, mental status reduction or fluctuations, elevated serum creatine kinase (CK), diaphoresis, urinary incontinence, tachycardia, or blood pressure elevation or fluctuations. 3 The authors report 1 case of PHS following the abrupt discontinuation of amantadine, an antiparkinsonian medication with scarcely reported association with this syndrome.…”

“…There are many therapeutic options for treating PD and, because of side effects profile or uncontrolled symptoms, changes in treatment regimens are common. One rare complication related to treatment alterations is a parkinsonism-hyperpyrexia syndrome (PHS), a disorder first described in 1981 in a patient with PD that presented with acute symptoms that resembled that of the neuroleptic malignant syndrome (NMS) after the abrupt discontinuation of antiparkinsonian agents 2. Patients with PHS may present with signs of central dopaminergic depletion related such as hyperthermia, rigidity, mental status reduction or fluctuations, elevated serum creatine kinase (CK), diaphoresis, urinary incontinence, tachycardia, or blood pressure elevation or fluctuations 3.…”