Chronic spontaneous diploic hematoma

Case report

Restricted access

The authors report the case of a 23-year-old woman with café-au-lait spots and axillary and inguinal freckling who presented with a diploic chronic spontaneous hematoma of the left parietal bone. To the authors' knowledge, this case represents the first description of a diploic hematoma in a patient with stigmata of neurofibromatosis Type 1 unrelated to head trauma. Plain skull radiography showed an osteolytic lesion with well-circumscribed margins, corresponding to the hematoma, together with exuberant perilesional vascular markings. Angiography demonstrated an incidental aneurysm of the left supraclinoidal internal carotid artery and an unusual cortical venous drainage toward the diploic vessels. The blood flow of these vessels on the right hemicranium was sluggish and exhibited enlarged diploic venous lacunas. The authors hypothesize that the hematoma was formed by both an abnormal venous drainage toward the diploic vascular net, together with a vasculopathy that caused stenosis and obstruction of the normal drainage pathways from these vessels.

Abbreviations used in this paper: AVF = arteriovenous fistula; NF = neurofibromatosis; NF1 = NF Type 1; NIH = National Institutes of Health.

Article Information

Address correspondence to: José González-Tortosa, M.D., Servicio Regional de Neurocirugía, Hospital Universitario Virgen de la Arrixaca, 30120 El Palmar, Murcia, Spain. email:

Please include this information when citing this paper: published online January 28, 2011; DOI: 10.3171/2010.12.JNS101589.

© AANS, except where prohibited by US copyright law.



  • View in gallery

    A: Axial T1-weighted MR image showing an extraaxial lesion of mixed densities that compresses the underlying brain. B: Axial T2-weighted MR image demonstrating the heterogeneous extraaxial tumor with a central hypointense zone. C: Coronal postcontrast MR image showing dural tail-like enhancement. D: Preoperative axial postcontrast MR image demonstrating irregular uptake, especially at the periphery. E: Postoperative postcontrast coronal bone-window CT scan depicting enlarged venous channels and lacunas. F: Postoperative 3D angiographic reconstruction illustrating the incidental finding of a supraclinoidal aneurysm of the left internal carotid artery.

  • View in gallery

    Left: Lateral radiograph showing a large osteolytic lesion with sclerotic margins and intracystic septi. Note the prominent diploic vascular marks. Right: Anteroposterior radiograph revealing the osteolytic lesion with diploic expansion.

  • View in gallery

    Left: Photograph of the outer aspect of the excised en bloc lesion showing a slight bone prominence of the thinned outer table with focal bluish discoloration. Right: Photograph of the inner aspect of the excised bone. Note the intense erosion of the inner cranial table with intraosseous cavities of varying size and numerous marked vascular marks surrounding the lesion.

  • View in gallery

    Histopathological examination of the excised lesion. Left: Walls of the lesion contents showing a concentric fibrous lining, within which there was no viable cellularity, that embeds fibrinous-hematic material with intense blue staining. Masson trichrome, original magnification × 4. Right: Thinning of the outer cranial table as illustrated in the macroscopic photograph. Note the presence of a fine osseous trabecula in the periphery of the collagen tissue. H & E, original magnification × 4.

  • View in gallery

    Right internal carotid artery angiograms in the venous phases. Left: Anteroposterior image showing blood drainage toward the diploic veins (black arrows) originating from frontal cortical veins. A cortical vein that does not drain into the sagittal sinus (white arrows) is clearly visible; rather, this vein drains into the diploic vessels. Right: Lateral image demonstrating ectasia of the diploic vein (black arrows). The contrast persisted within this vessel even when it had been completely washed out from the intracranial venous sinuses (image not shown). Note that the diploic vein does not drain in any of the intra- or extracranial venous networks.

  • View in gallery

    Postoperative left carotid artery angiograms in the venous phases. Left: Anteroposterior image revealing venous drainage toward the diploic veins (black arrows). Right: Oblique image showing a large intraosseous trajectory of the diploic vein (black arrows) that ends draining into the posterior cervical vascular network.


  • 1

    Brems HChmara MSahbatou MDenayer ETaniguchi KKato R: Germline loss-of-function mutations in SPRED1 cause a neurofibromatosis 1-like phenotype. Nat Genet 39:112011262007

  • 2

    Friedman JMArbiser JEpstein JAGutmann DHHuot SJLin AE: Cardiovascular disease in neurofibromatosis 1: report of the NF1 Cardiovascular Task Force. Genet Med 4:1051112002

  • 3

    Goel ADesai KPatil S: Unusual intradiploic hematoma. J Neurosurg 93:8958962000

  • 4

    Goel ANadkarni TMazumdar D: Intradiploic haematoma behaving as a progressive mass lesion. Br J Neurosurg 10:6116121996

  • 5

    Hamilton SJFriedman JM: Insights into the pathogenesis of neurofibromatosis 1 vasculopathy. Clin Genet 58:3413442000

  • 6

    Hinsch NKriener SRitter RGHolzer K: Fatal haemorrhage due to extensive fragility of medium- and large-sized arteries and veins in a young patient with neurofibromatosis 1. Cardiovasc Pathol 17:1081122008

  • 7

    Karadimas PHatzispasou EBouzas EA: Retinal vascular abnormalities in neurofibromatosis type 1. J Neuroophthalmol 23:2742752003

  • 8

    Messiaen LYao SBrems HCallens TSathienkijkanchai ADenayer E: Clinical and mutational spectrum of neurofibromatosis type 1-like syndrome. JAMA 302:211121182009

  • 9

    Mori FKawai MSato EIgarishi SHikichi TYoshida A: Branch retinal vein occlusion in a Japanese patient with neurofibromatosis 1. Jpn J Ophthalmol 45:6346352001

  • 10

    Muci-Mendoza RRamella MFuenmayor-Rivera D: Corkscrew retinal vessels in neurofibromatosis type 1: report of 12 cases. Br J Ophthalmol 86:2822842002

  • 11

    National Institutes of Health Consensus Development Conference: Neurofibromatosis. Conference statement Arch Neurol 45:5755781988

  • 12

    Nopajaroonsri CLurie AA: Venous aneurysm, arterial dysplasia, and near-fatal hemorrhages in neurofibromatosis type 1. Hum Pathol 27:9829851996

  • 13

    Oderich GSSullivan TMBower TCGloviczki PMiller DVBabovic-Vuksanovic D: Vascular abnormalities in patients with neurofibromatosis syndrome type I: clinical spectrum, management, and results. J Vasc Surg 46:4754842007

  • 14

    Präve FHach-Wunderle VHach W: Vascular manifestation of von Recklinghausen neurofibromatosis: case report of venous dysplasia. Vasa 32:36392003. (Ger)

  • 15

    Rosser TLVezina GPacker RJ: Cerebrovascular abnormalities in a population of children with neurofibromatosis type 1. Neurology 64:5535552005

  • 16

    Sobata EOhkuma HSuzuki S: Cerebrovascular disorders associated with von Recklinghausen's neurofibromatosis: a case report. Neurosurgery 22:5445491988

  • 17

    Spurlock GBennett EChuzhanova NThomas NJim HPSide L: SPRED1 mutations (Legius syndrome): another clinically useful genotype for dissecting the neurofibromatosis type 1 phenotype. J Med Genet 46:4314372009

  • 18

    Sun ZJZhao YFWang SPHe SG: Giant facial haematoma in neurofibromatosis type 1. Dentomaxillofac Radiol 37:52572008

  • 19

    Uemura KTsuboi KNose T: Intradiploic hematoma. AJNR Am J Neuroradiol 18:3973981997

  • 20

    Williams VCLucas JBabcock MAGutmann DHKorf BMaria BL: Neurofibromatosis type 1 revisited. Pediatrics 123:1241332009

  • 21

    Yücesoy KMertol TOzer HOzer E: An infantile intraosseous hematoma of the skull. Report of a case and review of the literature. Childs Nerv Syst 15:69721999

  • 22

    Zaret CRChoromokos EAMeisler DM: Cilio-optic vein associated with phakomatosis. Ophthalmology 87:3303361980




All Time Past Year Past 30 Days
Abstract Views 71 71 3
Full Text Views 61 61 1
PDF Downloads 76 76 1
EPUB Downloads 0 0 0


Google Scholar