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Lymphangioma of the orbit

MedGen UID:
734711
•Concept ID:
C1531935
•
Neoplastic Process
Synonym: Lymphangioma of orbit
SNOMED CT: Lymphangioma of orbit (414626005)
 
HPO: HP:0500091

Definition

A hamartoma of lymph vessels that usually presents in childhood. It tends to increase in size with head-down posture and with the Valsalva maneuver. Superficial lesions are visible as transilluminable cystic spaces of the lid or conjunctiva that may also contain blood. Deep lesions may cause gradual proptosis or present acutely with orbital pain and reduced vision due to hemorrhage. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVLymphangioma of the orbit

Professional guidelines

PubMed

Medical and sclerosing agents in the treatment of orbital lymphatic malformations: what's new?
Lam SC, Yuen HKL
Curr Opin Ophthalmol 2019 Sep;30(5):380-385. doi: 10.1097/ICU.0000000000000585. PMID: 31232717
Complications of intralesional bleomycin in the treatment of orbital lymphatic malformations.
MacIntosh PW, Yoon MK, Fay A
Semin Ophthalmol 2014 Sep-Nov;29(5-6):450-5. doi: 10.3109/08820538.2014.959617. PMID: 25325873
Angiography and differential diagnosis of orbital tumours.
Huber A
Trans Ophthalmol Soc U K (1962) 1972;92:35-50. PMID: 4515522

Recent clinical studies

Etiology

Interventions for orbital lymphangioma.
Patel SR, Rosenberg JB, Barmettler A
Cochrane Database Syst Rev 2019 May 15;5(5):CD013000. doi: 10.1002/14651858.CD013000.pub2. PMID: 31094450Free PMC Article
A prospective study of the role of intralesional bleomycin in orbital lymphangioma.
Raichura ND, Alam MS, Noronha VO, Mukherjee B
J AAPOS 2017 Apr;21(2):146-151. Epub 2017 Mar 12 doi: 10.1016/j.jaapos.2017.03.007. PMID: 28300684
Complications of intralesional bleomycin in the treatment of orbital lymphatic malformations.
MacIntosh PW, Yoon MK, Fay A
Semin Ophthalmol 2014 Sep-Nov;29(5-6):450-5. doi: 10.3109/08820538.2014.959617. PMID: 25325873
Orbital venous anomalies.
Wright JE, Sullivan TJ, Garner A, Wulc AE, Moseley IF
Ophthalmology 1997 Jun;104(6):905-13. doi: 10.1016/s0161-6420(97)30208-5. PMID: 9186428
Primary orbital tumors.
Adam YG, Farr HW
Am J Surg 1971 Dec;122(6):726-31. doi: 10.1016/0002-9610(71)90435-1. PMID: 4331407

Diagnosis

Tumor pathology of the orbit.
Héran F, Bergès O, Blustajn J, Boucenna M, Charbonneau F, Koskas P, Lafitte F, Nau E, Roux P, Sadik JC, Savatovsky J, Williams M
Diagn Interv Imaging 2014 Oct;95(10):933-44. Epub 2014 Sep 5 doi: 10.1016/j.diii.2014.08.002. PMID: 25195185
Standardized echography: basic principles, clinical applications, and results.
Ossoinig KC
Int Ophthalmol Clin 1979 Winter;19(4):127-210. PMID: 395120
Orbitocranial asymmetry.
Burrows EH
Br J Radiol 1978 Oct;51(610):771-81. doi: 10.1259/0007-1285-51-610-771. PMID: 101260
B-mode orbital ultrasonography.
Purnell EW
Int Ophthalmol Clin 1969 Fall;9(3):643-65. doi: 10.1097/00004397-196900930-00008. PMID: 5396776
CYSTIC LYMPHANGIOMA OF THE ORBIT.
Mortada A
Br J Ophthalmol 1962 Jan;46(1):45-50. doi: 10.1136/bjo.46.1.45. PMID: 18170753Free PMC Article

Therapy

Medical and sclerosing agents in the treatment of orbital lymphatic malformations: what's new?
Lam SC, Yuen HKL
Curr Opin Ophthalmol 2019 Sep;30(5):380-385. doi: 10.1097/ICU.0000000000000585. PMID: 31232717
Interventions for orbital lymphangioma.
Patel SR, Rosenberg JB, Barmettler A
Cochrane Database Syst Rev 2019 May 15;5(5):CD013000. doi: 10.1002/14651858.CD013000.pub2. PMID: 31094450Free PMC Article
Update on orbital lymphatic malformations.
Lally SE
Curr Opin Ophthalmol 2016 Sep;27(5):413-5. doi: 10.1097/ICU.0000000000000291. PMID: 27219273
Complications of intralesional bleomycin in the treatment of orbital lymphatic malformations.
MacIntosh PW, Yoon MK, Fay A
Semin Ophthalmol 2014 Sep-Nov;29(5-6):450-5. doi: 10.3109/08820538.2014.959617. PMID: 25325873
Conservative management of orbital lymphangioma.
Muallem MS, Garzozi HJ
J Pediatr Ophthalmol Strabismus 2000 Jan-Feb;37(1):41-3. doi: 10.3928/0191-3913-20000101-10. PMID: 10714694

Prognosis

Orbital Lymphangioma: Characteristics and Treatment Outcomes of 12 Cases.
Woo YJ, Kim CY, Sgrignoli B, Yoon JS
Korean J Ophthalmol 2017 Jun;31(3):194-201. Epub 2017 May 12 doi: 10.3341/kjo.2016.0034. PMID: 28534344Free PMC Article
A prospective study of the role of intralesional bleomycin in orbital lymphangioma.
Raichura ND, Alam MS, Noronha VO, Mukherjee B
J AAPOS 2017 Apr;21(2):146-151. Epub 2017 Mar 12 doi: 10.1016/j.jaapos.2017.03.007. PMID: 28300684
Giant facial lymphangioma.
Sanger C, Wong L, Wood J, David LR, Argenta LC
J Craniofac Surg 2011 Jul;22(4):1271-4. doi: 10.1097/SCS.0b013e31821c6cf1. PMID: 21772195
Vascular malformations of the orbit: hemodynamic concepts.
Rootman J
Orbit 2003 Jun;22(2):103-20. doi: 10.1076/orbi.22.2.103.14311. PMID: 12789590
Orbital venous anomalies.
Wright JE, Sullivan TJ, Garner A, Wulc AE, Moseley IF
Ophthalmology 1997 Jun;104(6):905-13. doi: 10.1016/s0161-6420(97)30208-5. PMID: 9186428

Clinical prediction guides

The Experience Of Intralesional Bleomycin For Orbital Vascular Anomalies.
Kamil Z, Qurban Q, Hassan Khan MT
J Ayub Med Coll Abbottabad 2021 Apr-Jun;33(2):240-243. PMID: 34137537
Interventions for orbital lymphangioma.
Patel SR, Rosenberg JB, Barmettler A
Cochrane Database Syst Rev 2019 May 15;5(5):CD013000. doi: 10.1002/14651858.CD013000.pub2. PMID: 31094450Free PMC Article
Giant facial lymphangioma.
Sanger C, Wong L, Wood J, David LR, Argenta LC
J Craniofac Surg 2011 Jul;22(4):1271-4. doi: 10.1097/SCS.0b013e31821c6cf1. PMID: 21772195
Orbital cysts of childhood--classification, clinical features, and management.
Shields JA, Shields CL
Surv Ophthalmol 2004 May-Jun;49(3):281-99. doi: 10.1016/j.survophthal.2004.02.001. PMID: 15110666
Orbital venous anomalies.
Wright JE, Sullivan TJ, Garner A, Wulc AE, Moseley IF
Ophthalmology 1997 Jun;104(6):905-13. doi: 10.1016/s0161-6420(97)30208-5. PMID: 9186428

Recent systematic reviews

Interventions for orbital lymphangioma.
Patel SR, Rosenberg JB, Barmettler A
Cochrane Database Syst Rev 2019 May 15;5(5):CD013000. doi: 10.1002/14651858.CD013000.pub2. PMID: 31094450Free PMC Article

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