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Primary intraosseous venous malformation(VMOS; VMPI)

MedGen UID:
376071
Concept ID:
C1847197
Disease or Syndrome
Synonyms: HEMANGIOMA, INTRAOSSEOUS; VASCULAR MALFORMATION OSSEOUS; Vascular malformation, primary intraosseous
SNOMED CT: Intraosseous hemangioma (764100007); Osseous venous malformation (764100007); Primary intraosseous venous malformation (764100007)
Modes of inheritance:
Autosomal recessive inheritance
MedGen UID:
141025
Concept ID:
C0441748
Intellectual Product
Source: Orphanet
A mode of inheritance that is observed for traits related to a gene encoded on one of the autosomes (i.e., the human chromosomes 1-22) in which a trait manifests in individuals with two pathogenic alleles, either homozygotes (two copies of the same mutant allele) or compound heterozygotes (whereby each copy of a gene has a distinct mutant allele).
 
Gene (location): ELMO2 (20q13.12)
 
Monarch Initiative: MONDO:0011744
OMIM®: 606893
Orphanet: ORPHA140436

Definition

Primary intraosseous vascular malformation (VMPI), previously called intraosseous hemangioma, is a rare malformation that usually involves the vertebral column and the skull. The most commonly affected bones in the skull are the mandible and the maxilla, and life-threatening bleeding after a simple tooth extraction is frequent (Vargel et al., 2002). [from OMIM]

Clinical features

From HPO
Increased intracranial pressure
MedGen UID:
56241
Concept ID:
C0151740
Finding
An increase of the pressure inside the cranium (skull) and thereby in the brain tissue and cerebrospinal fluid.
Hypochromic anemia
MedGen UID:
8065
Concept ID:
C0002884
Disease or Syndrome
A type of anemia characterized by an abnormally low concentration of hemoglobin in the erythrocytes.
Gingival bleeding
MedGen UID:
42218
Concept ID:
C0017565
Pathologic Function
Hemorrhage affecting the gingiva.
Umbilical hernia
MedGen UID:
9232
Concept ID:
C0019322
Anatomical Abnormality
Protrusion of abdominal contents through a defect in the abdominal wall musculature around the umbilicus. Skin and subcutaneous tissue overlie the defect.
Diastasis recti
MedGen UID:
113171
Concept ID:
C0221766
Disease or Syndrome
A separation of the rectus abdominis muscle into right and left halves (which are normally joined at the midline at the linea alba).
Supraumbilical raphe
MedGen UID:
1615232
Concept ID:
C4539078
Anatomical Abnormality
An abnormality of the sternum that presents at birth as a ventral sternal non-union defect, due to an abnormality of the fusion of the layers of the skin. It presents as a scar-like line that extends upward from the umbilicus (belly button).
Elevated circulating alkaline phosphatase concentration
MedGen UID:
727252
Concept ID:
C1314665
Finding
Abnormally increased serum levels of alkaline phosphatase activity.
Ectopic tooth eruption
MedGen UID:
21206
Concept ID:
C0040438
Disease or Syndrome
A condition in which the permanent teeth, because of deficiency of growth in the jaw or segment of jaw, assume a path of eruption that intercepts a primary tooth, causes its premature loss and produces a consequent malposition of the permanent tooth.
Facial asymmetry
MedGen UID:
266298
Concept ID:
C1306710
Finding
An abnormal difference between the left and right sides of the face.
Proptosis
MedGen UID:
41917
Concept ID:
C0015300
Disease or Syndrome
An eye that is protruding anterior to the plane of the face to a greater extent than is typical.
Visual loss
MedGen UID:
784038
Concept ID:
C3665386
Finding
Loss of visual acuity (implying that vision was better at a certain time point in life). Otherwise the term reduced visual acuity should be used (or a subclass of that).

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPrimary intraosseous venous malformation
Follow this link to review classifications for Primary intraosseous venous malformation in Orphanet.

Professional guidelines

PubMed

Lee A, Driscoll D, Gloviczki P, Clay R, Shaughnessy W, Stans A
Pediatrics 2005 Mar;115(3):744-9. doi: 10.1542/peds.2004-0446. PMID: 15741381

Recent clinical studies

Etiology

Bhattacharjee K, Rehman O, Venkatraman V, Das D, Mohapatra SSD, Gogoi R, Soni D
Orbit 2024 Jun;43(3):316-328. Epub 2024 Jan 23 doi: 10.1080/01676830.2024.2303761. PMID: 38261337
Kurokawa R, Kurokawa M, Holmes A, Baba A, Bapuraj J, Capizzano A, Kim J, Srinivasan A, Moritani T
J Neuroimaging 2022 Nov;32(6):1170-1176. Epub 2022 Aug 3 doi: 10.1111/jon.13034. PMID: 35922879Free PMC Article
Antúnez-Conde R, Salmerón JI, Navarro C
J Craniofac Surg 2021 Nov-Dec 01;32(8):e757-e759. doi: 10.1097/SCS.0000000000007751. PMID: 34191769
Kadlub N, Dainese L, Coulomb-L'Hermine A, Galmiche L, Soupre V, Lepointe HD, Vazquez MP, Picard A
Int J Oral Maxillofac Surg 2015 Jun;44(6):718-24. Epub 2015 Feb 19 doi: 10.1016/j.ijom.2015.01.025. PMID: 25703595
Colletti G, Frigerio A, Giovanditto F, Biglioli F, Chiapasco M, Grimmer JF
J Oral Maxillofac Surg 2014 Jul;72(7):1326.e1-18. Epub 2014 Feb 15 doi: 10.1016/j.joms.2014.02.012. PMID: 24768420

Diagnosis

Kurokawa R, Kurokawa M, Holmes A, Baba A, Bapuraj J, Capizzano A, Kim J, Srinivasan A, Moritani T
J Neuroimaging 2022 Nov;32(6):1170-1176. Epub 2022 Aug 3 doi: 10.1111/jon.13034. PMID: 35922879Free PMC Article
Antúnez-Conde R, Salmerón JI, Navarro C
J Craniofac Surg 2021 Nov-Dec 01;32(8):e757-e759. doi: 10.1097/SCS.0000000000007751. PMID: 34191769
Fábián Z, Szabó G, Petrovan C, Horváth KU, Babicsák B, Hüttl K, Németh Z, Bogdán S
Oral Maxillofac Surg 2018 Jun;22(2):241-247. Epub 2018 Apr 13 doi: 10.1007/s10006-018-0691-0. PMID: 29651653
Kadlub N, Dainese L, Coulomb-L'Hermine A, Galmiche L, Soupre V, Lepointe HD, Vazquez MP, Picard A
Int J Oral Maxillofac Surg 2015 Jun;44(6):718-24. Epub 2015 Feb 19 doi: 10.1016/j.ijom.2015.01.025. PMID: 25703595
Defazio MV, Kassira W, Camison L, Meshkov L, Robinson PG, Kawamoto HK, Thaller SR
Ann Plast Surg 2014 Mar;72(3):323-7. doi: 10.1097/SAP.0b013e3182605690. PMID: 23241780

Therapy

Lambot-Juhan K, Pannier S, Grévent D, Péjin Z, Breton S, Berteloot L, Emond-Gonsard S, Boddaert N, Glorion C, Brunelle F
Pediatr Radiol 2012 May;42(5):599-605. Epub 2012 Jan 12 doi: 10.1007/s00247-011-2312-z. PMID: 22237479
Lee A, Driscoll D, Gloviczki P, Clay R, Shaughnessy W, Stans A
Pediatrics 2005 Mar;115(3):744-9. doi: 10.1542/peds.2004-0446. PMID: 15741381

Prognosis

Bhattacharjee K, Rehman O, Venkatraman V, Das D, Mohapatra SSD, Gogoi R, Soni D
Orbit 2024 Jun;43(3):316-328. Epub 2024 Jan 23 doi: 10.1080/01676830.2024.2303761. PMID: 38261337
Kadlub N, Dainese L, Coulomb-L'Hermine A, Galmiche L, Soupre V, Lepointe HD, Vazquez MP, Picard A
Int J Oral Maxillofac Surg 2015 Jun;44(6):718-24. Epub 2015 Feb 19 doi: 10.1016/j.ijom.2015.01.025. PMID: 25703595
Defazio MV, Kassira W, Camison L, Meshkov L, Robinson PG, Kawamoto HK, Thaller SR
Ann Plast Surg 2014 Mar;72(3):323-7. doi: 10.1097/SAP.0b013e3182605690. PMID: 23241780

Clinical prediction guides

Kadlub N, Dainese L, Coulomb-L'Hermine A, Galmiche L, Soupre V, Lepointe HD, Vazquez MP, Picard A
Int J Oral Maxillofac Surg 2015 Jun;44(6):718-24. Epub 2015 Feb 19 doi: 10.1016/j.ijom.2015.01.025. PMID: 25703595
Werdich XQ, Jakobiec FA, Curtin HD, Fay A
Am J Ophthalmol 2014 Oct;158(4):816-826.e1. Epub 2014 Jul 15 doi: 10.1016/j.ajo.2014.07.004. PMID: 25034115
Lee A, Driscoll D, Gloviczki P, Clay R, Shaughnessy W, Stans A
Pediatrics 2005 Mar;115(3):744-9. doi: 10.1542/peds.2004-0446. PMID: 15741381
Vargel I, Cil BE, Er N, Ruacan S, Akarsu AN, Erk Y
Am J Med Genet 2002 Apr 15;109(1):22-35. doi: 10.1002/ajmg.10282. PMID: 11932989

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