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Jornal Vascular Brasileiro

Print version ISSN 1677-5449On-line version ISSN 1677-7301

J. vasc. bras. vol.7 no.4 Porto Alegre Dec. 2008 



Spontaneous lumbar artery bleeding in patient with Von Recklinghausen’s disease: endovascular treatment



Felipe NasserI; Breno Boueri AffonsoI; Charles Edouard ZurstrassenI; Wilson de Oliveira Sousa JuniorI; Joaquim Mauricio da Motta Leal FilhoI; Fabio YamadaI; Paulo Henrique PetterleI; Francisco Cesar CarnevaleI

IInterventional Radiology and Endovascular Surgery Department, Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo (USP), São Paulo, SP, Brazil.





Retroperitoneal bleeding is mainly due to traumatic events with a high amount of kinetic energy, with few reported cases of spontaneous events in the literature. We report on a case of a pregnant woman with Von Recklinghausen’s disease and bulky retroperitoneal hematoma diagnosed during cesarean delivery secondary to spontaneous lumbar artery rupture. Von Recklinghausen’s disease has well-described vascular manifestations, mainly characterized by stenoses related to intramural tumors (Schwann cell proliferation) and rarely asymptomatic aneurysmal dilatations. In this case, aortography was performed with successful selective catheterization and embolization of the bleeding artery.

Keywords: Hematoma, von Recklinghausen’s disease, artery, therapeutic embolization.


Os sangramentos no retroperitônio são, em sua grande maioria, secundários a eventos traumáticos envolvendo grande energia cinética, com poucos relatos na literatura caracterizados como espontâneos. No presente relato, descrevemos paciente gestante, portadora de doença de Von Recklinghausen e com volumoso hematoma retroperitoneal diagnosticado durante o parto cesariano, secundário a ruptura espontânea de artéria lombar. A doença de Von Recklinghausen apresenta manifestações vasculares bem descritas, caracterizando-se principalmente por estenoses que são secundárias a tumores intramurais (proliferação das células de Schwann) e raramente dilatações aneurismáticas, assintomáticas em sua maioria. No presente caso, foi realizada a aortografia com cateterização seletiva e embolização da artéria sangrante com sucesso.

Palavras-chave: Hematoma, doença de Von Recklinghausen, artéria, embolização terapêutica.




Retroperitoneal bleeding in lumbar arteries is rare and mostly related to trauma1,2 involving large amount of kinetic energy with associated lesions of pelvis and/or vertebral column.1 Spontaneous bleeding of these arteries is rare and has few reports in the literature in English.1

Among causes of bleeding unrelated to trauma are structural changes of the arterial wall (aneurysms or pseudoaneurysms)3 or use of anticoagulant therapy.4 In this group there are patients with Von Recklinghausen disease, an autosomal dominant disease, whose incidence is 1:3,000 and in which arterial involvement is well known, although unusual. Stenotic lesions are predominant, especially in renal arteries, whereas aneurysmal degenerations are less frequent.5

In this report the patient had Von Recklinghausen disease and a voluminous retroperitoneal hematoma caused by spontaneous lumbar artery bleeding. She was successfully submitted to endovascular treatment with microcoil embolization.


Case report

A 36-year-old female patient with type I neurofibromatosis and 29-week pregnancy was admitted to the emergency room with hypertensive peak (blood pressure: 180/110 mmHg), and was hospitalized for control and evaluation of fetal vitality. On the fourth hospitalization day she started presenting intense right lumbar pain with fall in hemoglobin levels from 11.9 to 7.2 g% associated with fetal bradycardia. Diagnosis was of premature separation of placenta, and immediate interruption of pregnancy was indicated. During cesarean section a large retroperitoneal hematoma was observed on the right. After intraoperative assessment by the general and vascular team, and because there were no signs of expansion or hemodynamic instability, a conservative conduct was chosen, indicating angiographic tomography in the immediate postoperative period.

Abdominal tomography was performed immediately after surgical procedure, confirming a voluminous retroperitoneal hematoma displacing the right kidney and pancreas inferiorly and anteriorly (Figure 1), with sign of active bleeding compatible with lumbar artery pseudoaneurysm.



Interventional radiology assessment was requested. Abdominal aortography was performed with right femoral puncture using a 5 Fr cobra catheter selecting the lumbar arteries on the right. Bleeding origin was located on the second lumbar artery (Figure 2). The microwire (0.014) and microcatheter were advanced and a superselective embolization using microcoils was performed. Angiography showed control of bleeding with no contrast extravasation and occlusion of the corresponding branch (Figure 3).





The patient progressed uneventfully, receiving two units of concentrated red cells during cesarean section and was discharged on the 13th postoperative day. At the moment the patient has no bleeding and is well after a 12-month follow-up.



Retroperitoneal bleeding resulting from lumbar arteries are more frequently associated with traumatic events of large kinetic energy, spine surgical instrumentation, or vertebral biopsy.6,7 On the other hand, spontaneous bleeding in these arteries are rare and related to anticoagulant therapy, or even aneurysm rupture.8

In this report, the patient had a voluminous retroperitoneal hematoma, and aortography showed bleeding from a lumbar artery with active bleeding and no true aneurysmal dilatation, despite concomitance of neurofibromatosis diagnosis. Neurofibromatosis consists of a group of three genetic dysfunctions characterized by neural sheath tumors1,3,9: neurofibromatosis type I; type II and schwannomatosis, which differ in their mutations and clinical manifestations. Neurofibromatosis type I (Von Recklinghausen's disease), initially described in 1882 by pathologist Friedrich Von Recklinghausen, is characterized by varied signs and symptoms, secondary to involvement of many different organs, from skin with the classical pigmentation called café-au-lait macule, limbs, trunk with neuronal tumors (neurofibromas), and central nervous system (gliomas).5 Vascular clinical manifestations, found in only 3.6% of cases, are primarily due to involvement of the arterial bed5 as arterial stenoses secondary to intramural thickening (Schwann cell proliferation); more rarely, they may present aneurysmal dilatations, which are poststenotic dilatations.10 In order of frequency, the renal artery is the most affected site, but there are reports of lower limb arteries,5,10 intercostal arteries10 and visceral arteries.11 It is believed that all patients with this disease, in post-mortem evaluation, have some degree of arterial wall thickening with poststenotic stenoses and dilatations, but silent most of the times.11

The retroperitoneal regions, because it is irrigated by several arterial branches,2 is a challenge for an open surgical approach, since a single lumbar artery may communicate with many other arterial branches, such as intercostal or internal iliac artery branches, and that may maintain an active bleeding. Sclafani et al.12 reported how multiple collaterals could suppress the same site of arterial bleeding.12 Lumbar artery pairs (L1 - L4) originate from the posterior surface of the aorta at the level of transversal processes, and the fifth lumbar artery emerges from the lateral sacral artery, iliolumbar or, more rarely, median sacral artery.2 They all run along the vertebra before bifurcating in their anterior and posterior branches in the medial sheath of the psoas muscle, which irrigate the spine and back muscles, respectively.3

Another important concept is the origin of the anterior radiculomedular artery (T8 – L2) or Adamkiewicz artery, which accounts for irrigation of the thoracolumbar portion of the spinal cord. Inadvertent manipulation might cause medullary infarction and paralysis. In about 19% of times, its origin is in the first or second lumbar artery,13 which should be remembered during manipulation and treatment, always avoiding embolization from the lumbar artery origin.

Based on these data, treatment should be started with aortography and further selection of all branches in the bleeding area2 and, after identifying the bleeding focus, superselective embolization should be performed using micro-guide wire and microcatheters. By the end of the procedure, control arteriography should be performed to record interruption of active bleeding, in addition to identification of branches that lead to refilling of treated sites.

Endovascular approach is a minimally invasive therapeutic option that has significant advantages over conventional surgical approach: general anesthesia is avoided in patients with bleeding and hemodynamic instability; it reduced blood loss caused by surgical trauma and retroperitoneal dissection; safe occlusion of the lumbar artery after identification of the great radicular artery origin.3,9 Open surgical approach also involves direct manipulation on an arterial wall that is weakened by the chronic inflammatory process, with risk of direct lesion and consequent increased bleeding in a region of hard dissection and control.



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2. Sofocleous CT, Hinrichs CR, Hubbi B, Doddakashi S, Bahramipour P, Schubert J. Embolization of isolated lumbar artery injuries in trauma patients. Cardiovasc Intervent Radiol. 2005;28:730-5.         [ Links ]

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9. Theos A, Korf BR; American College of Physicians; American Physiological Society. Pathophysiology of the neurofibromatosis type I. Ann Intern Med. 2006;144:842.         [ Links ]

10. Kipfer B, Lardinois D, Triller J, Carrel T. Embolization of a ruptured intercostal artery aneurysm in type I neurofibromatosis. Eur J Cardiothorac Surg. 2001;19:721-3.         [ Links ]

11. Riccardi V. Neurofibromatosis: phenotype, natural history, and pathogenesis. The Johns Hopkins University Press. 1992:124-7.         [ Links ]

12. Sclafani SJ, Florence LO, Phillips TF, et al. Lumbar arterial injury: radiologic diagnosis and management. Radiology. 1987;165:709-14.         [ Links ]

13. Takase K, Sawamura Y, Igarashi K, et al. Demonstration of the artery of Adamkiewicz at multi- detector row helical CT. Radiology. 2002;223:39-45.         [ Links ]



Wilson de Oliveira Sousa Junior
Rua Capote Valente, 189/1401, Pinheiros
CEP 05409-000 – São Paulo, SP, Brazil
Tel.: (11) 9449.1364

Manuscript received July 7, 2008, accepted October 14, 2008.



No conflicts of interest declared concerning the publication of this article.

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