kapilläres Hämangiom der Orbita

Capillary hemangiomas of the orbit, also known as strawberry hemangiomas, on account of its coloring, or orbital infantile hemangiomas, are the most common orbital tumors of infancy, and unlike orbital cavernous hemangiomas, they are neoplasms rather than vascular malformations.

Clinical presentation

Usually present as a periorbital subcutaneous mass. In a minority of cases the tumor is deep to the skin overlying the orbit and has a more variable presentation, including :

• palpable lump
• proptosis
• diplopia
• ptosis
• optic atrophy and visual deterioration

Pathology

Capillary hemangiomas are usually located anterior to the globe, in the eyelid, and are present at birth or shortly thereafter . They may involve the extra-ocular muscles and lacrimal glands and may extend intracranially through the optic canal or superior orbital fissure . Only rarely are they associated with systemic hemangiomas or with other pathology such as PHACES syndrome.

Radiographic features

Radiology is usually only required when the diagnosis is unclear, and this is most frequent in lesions with a deep component.

Ultrasound

On ultrasound, capillary hemangioma characteristically is hyperechoic and compressible lesions  with high peak intra-tumoral on arterial shift. Ultrasound is mostly useful for smaller, limited lesions. At some institutions, other criteria such as >5-6 vessels/cm and a maximum Doppler systolic flow of >2 kHz are used .

CT

The CT appearance is that of a strongly enhancing lobulated mass. The enhancement is typically homogeneous. On imaging alone, it is difficult to differentiate these lesions from other vascular lesions of the orbit, thus relying on patient age and clinical appearance.

MRI

The MRI appearance of capillary hemangioma is usually slightly hypointense on T1, iso- to hyperintense on T2 with multiple serpiginous flow voids . Enhancement is homogenous with gadolinium with marked enhancement of intratumoral vessels. Its lobulated appearance with thin septa is characteristic.

Treatment and prognosis

During the first 3-6 months of life, these masses enlarge before gradually reducing in size from the age of 12-18 months. 30% have resolved by the age of 3 years and 75-90% by the age of 7 years . Injection of corticosteroids or systemic administration is effective but reserved for cases where vision is affected. Intra-tumoral laser therapy has also been used for larger lesions and, in recalcitrant cases, interferon or vincristine can be considered .

Differential diagnosis

Differential considerations include:

• other vascular malformations of the orbit
  • cavernous hemangioma of the orbit
  • orbital venous varix
  • orbital lymphangioma
• orbital tumors
  • retinoblastoma
  • orbital metastases
  • rhabdomyosarcoma
  • chloroma

Siehe auch:

• intraorbitale Raumforderungen
• Rhabdomyosarkom
• Metastasen in der Orbita
• Retinoblastom
• kavernöses Hämangiom der Orbita
• Varizen der Orbita
• kapilläres Hämangiom
• Hämangiom der Orbita
• Myelosarkom
• infantiles Hämangiom

und weiter:

• Hämangiom
• intraorbital capillary hemangioma: color and power Doppler findings