Orbital Lymphangioma

Orbital Lymphangioma is an intra-orbital, non-encapsulated, congenital vascular tumour with a propensity for recurrent haemorrhage. Lymphangioma is a benign vascular hamartoma commonly noticed in head and neck area. Vascular mesenchyme that has differentiated abnormaly into isolated lymphatic- like spaces with no connection to the systemic circulation,may be the cause. Lymphangioma is a common vascular tumour in children and commonly presents in age group less than ten years. However, it is, sometimes detected later in life following trauma and intralesional haemorrhage. There are two growth patterns of lymphangioma (slow or rapidly explosive). Slow enlargement takes place over decades, producing progressive proptosis. In contrast, explosive growth results from intralesional haemorrhage arising from intrinsic nutrient vessels, or in association with an upper respiratory tract infection with proliferation of lymphoid tissue. In contrast to capillary haemangioma, lymphangiomas do not regress spontaneously. These tumours may become encapsulated due to normal bodily reaction to prevent expansion.

Lymphangiomas do not show any connection to the venous system, as has been studied by arteriography or venography. This is in contrast to orbital varix, which usually show connections to the venous system.

Like haemangiomas, Lymphangiomas may present in both superficial and deep orbital spaces. It consists of dilated, thin walled vascular chambers lined with endothelium. Fibrous stroma supports these and contain proteinaceous material reminiscent of lymph. There are micro-cystic, macro-cystic, or mixed types of Lymphangiomas.

 

References

Shields Jerry A, Shields Carol L. Eyelid, Conjunctival, and Orbital Tumors- An Atlas and Textbook Second Edition. Lippincott Williams & Wilkins, a Wolters Kluwer business 2008. P 534.

Karcioglu Zeynel A. Orbital Tumors – Diagnosis and Treatment Second Edition. Springer Science + Business Media New York 2015. P 155- 166.

Roy Hampton, Fraunfelder Frederick W, Fraunfelder Frederick T, Tindall Renee, Jensvold Bree. Roy and Fraunfelder’s Current Ocular Therapy Sixth Edition. Elsevier Inc. 2008. P 250 – 251.

Midyett F Allan, Mukherji Suresh K. Orbital Imaging. Saunders , an imprint of Elsevier Inc. 2015. P 143- 145.

Mcgregor Mary Lou, Stanton Bonita F. Pediatric Ophthalmology – Pediatric Clinics of North America Volume 61, Number 3. Elsevier Inc. 2014. P 548- 553.

Singh Arun D, Hayden Brandy C. Ophthalmic Ultrasonography. Elsevier Saunders 2012. P 171.

Bowling Brad. Kanski’s Clinical Ophthalmology – A Systematic Approach Eighth Edition. Elsevier Limited 2016. P 98- 100.

Guthoff RF, Katowitz JA. Oculoplastics and Orbit. Springer – Verlag Berlin Heidelberg 2007. P 113 – 121.

Martin Richard J, Fanaroff Avroy A, Walsh Michele C. Fanaroff & Martin’s Neonatal – Perinatal Medicine, Diseases of the Fetus and Infant Tenth Edition Volume one. Saunders, an imprint of Elsevier Inc. 2015. P 1763.

https://www.aao.org/eyenet/article/novel-approaches-to-orbital-lymphangioma

https://bjo.bmj.com/content/83/1/76

http://iv.iiarjournals.org/content/31/2/263.full

http://www.djo.org.in/articles/23/3/sclerosing-agents-in-ophthalmology.html

 

Patient may present with symptoms such as

  • Proptosis, which may be slowly progressive or sudden and painful
  • Cutaneous blood filled cysts in superficial lesions
  • Conjunctival haemorrhage or chemosis, in superficially located tumours
  • Ptosis (drooping of upper eyelid)
  • Strabismus, mainly if tumour is large and infiltrative
  • Optic neuropathy
  • Vascular lesions in oral mucosa, in deeply situated lesions with frequent intracranial component.

 

Lymphangioma is likely to be derived from vascular mesenchyme that has differentiated abnormaly into isolated lymphatic- like spaces with no connection to the systemic circulation. They are benign hamartomatous vascular tumours, which differ from haemangiomas in that they do not display proliferating endothelial cells.

Lymphangiomas are usually located in the head and neck region and may involve eyelid, conjunctiva, and orbit.

Some reports suggest a slight female preponderance.

Clinically, the tumours may be located superficially in the orbit and are cystic. More commonly, these are infiltrative and diffuse in the orbit. Lymphangioma, located apically or with intracranial extension, is more likely to be associated with sub-mucosal oral lesions and intracranial vascular anomalies.

Histopathology:

Lymphangioma is a histologically benign but clinically aggressive tumour. Histopathologically, there are non-encapsulated, thin walled, endothelium lined channels and cystic vascular spaces which may contain fluid resembling lymph. Scattered lymphoid tissue aggregates or haemorrhage may be present.

Imaging studies:

  • Ultrasonography: Ultrasonography lacks specificity and soft tissue details. It identifies soft tissue and cystic components of the lesion.
  • Computed tomography: Computed tomography best delineates bony deformity produced by long-standing lesions. This technique delineates well soft tissue details in the lesion. However, like ultrasonography, it also lacks specificity.
  • Magnetic resonance imaging: Magnetic resonance imaging provides superior quality soft tissue details. Identification of cystic structures and blood serum interfaces are diagnostic of the lesion. This technique helps in surgical planning of a case.

Differential diagnosis:

Differential diagnosis of lymphangioma includes

  • Orbital Varix
  • Orbital cellulitis
  • Orbital haemorrhage
  • Rhabdomyosarcoma
  • Neuroblastoma

 

Management includes

Supportive therapy

Since management of lymphangioma is difficult, it is advisable to observe it initially. Await for spontaneous resolution of haemorrhagic cyst or lymphoid hyperplasia associated with upper respiratory tract infection. Lymphangioma leading to compressive optic neuropathy, should be treated.

Medical therapy

Oral corticosteroids may help in the acute phase to resolve the lymphoid infiltration.

Surgical therapy

Surgery is, generally avoided since manipulation of the tumour may promote further spontaneous haemorrhage. Moreover, complete surgical excision is difficult due to infiltrative nature of the tumour and risk of damage to surrounding structures.  Recurrence rate is high.

Indications for surgery are

  • Compressive optic neuropathy
  • Diplopia
  • Disfigurement

Surgical procedures are

  • Cyst drainage: Cyst drainage may be accomplished under ultrasonography, computed tomography,or open orbitotomy.
  • De-bulking of tumour: De-bulking of tumour, often with multiple partial resections, may provide satisfactory results.
  • Carbon dioxide laser: Carbon dioxide laser may be a useful adjuvant for more solid vascularised variant.

 

Prognosis:

The majority of patients present in the first decade of life. It shows either slow or explosive growth patterns. Slow growth occurs over decades. Explosive growth results from intralesional haemorrhage.

Lymphangioma may present in childhood, when it may adversely affect vision. In most cases, it cannot be safely removed and may result in lifelong series of haemorrhages that threaten vision, produce pain, and may result in disfigurement.

Sequlae includes

  • Spontaneous resolution of mass with resolution of proptosis, usually over a period of weeks to months
  • Persistence of proptosis
  • Cutaneous disfigurement
  • Compressive optic neuropathy
  • Strabismus

 

 

Complications may be

  • Disfigurement
  • Optic neuropathy due to pressure by the tumour or haemorrhagic cyst.
  • Strabismus
  • Ptosis
  • Orbital bone asymmetry due to pressure by the tumour.
  • Amblyopia

 

  • PUBLISHED DATE : Aug 01, 2018
  • PUBLISHED BY : NHP Admin
  • CREATED / VALIDATED BY : Dr. S. C. Gupta
  • LAST UPDATED ON : Aug 01, 2018

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