Figure 18-17 Axial CT image showing unifocal eosinophilic granuloma with partial destruction of the right posterior lateral orbital wall (arrow) in a 15-year-old boy, who presented with retrobulbar pain and mild edema and erythema of the right upper eyelid.

Multifocal eosinophilic granuloma of the bone is a disseminated and aggressive form of LCH. It usually presents between 2 and 5 years of age and may produce proptosis from involvement of the bony orbit. Diabetes insipidus is common. Chemotherapy is often required, but the prognosis is generally good.

Diffuse soft-tissue histiocytosis is the most severe form, usually affecting infants younger than 2 years. It is characterized by soft-tissue lesions of multiple viscera (liver, spleen) but rarely involves the eye.

Herwig MC, Wojno T, Zhang Q, Grossniklaus HE. Langerhans cell histiocytosis of the orbit: five clinicopathologic cases and review of the literature. Surv Ophthalmol. 2013;58(4):330–340.

Benign Tumors

Vascular lesions: hemangiomas

Advances in the biological characterization of vascular lesions have led to a revision of their classification. The current classification of vascular lesions establishes clear clinical, histologic, and prognostic differences between hemangiomas and vascular malformations. The older terms capillary and strawberry hemangioma should be replaced by the single term hemangioma. Cavernous hemangiomas, port-wine stains, and lymphangiomas all should be classified as “malformations.” This nomenclature has been incorporated into the medical literature but has not been used consistently in the ophthalmic literature.

Hemangiomas are hamartomatous growths composed of proliferating capillary endothelial cells. They grow rapidly in early infancy, with a subsequent period of regression and involution. Periocular hemangiomas can be classified as follows:

preseptal, involving the skin and preseptal orbit intraorbital, involving the postseptal orbit

compound/mixed, involving the preseptal and postseptal orbit

Hemangiomas occur in 1%–3% of term newborns and are more common in premature infants, in females, and after chorionic villus sampling. Most hemangiomas are clinically insignificant at birth; they can be inapparent or can appear as an erythematous macule or a telangiectasia. The natural history is one of rapid proliferation and growth over the first several months of life, rarely lasting beyond 1 year. During this phase, the lesion may ulcerate, hemorrhage, or cause amblyopia by inducing astigmatism or obstructing the visual axis. After the first year of life, the lesion usually begins to regress, although the rate and degree of involution vary.

Systemic disease associated with hemangiomas PHACE is an acronym for posterior fossa

malformations, hemangiomas, arterial lesions, and cardiac and eye anomalies. The eye abnormalities include increased retinal vascularity, microphthalmia, optic nerve hypoplasia, exophthalmos, choroidal hemangiomas, strabismus, colobomas, cataracts, and glaucoma. The PHACE syndrome should be considered in any infant presenting with a large, segmental, plaquelike facial hemangioma involving 1 or more dermatomes (Fig 18-18).

Figure 18-18 Plaque hemangioma in a child with PHACE syndrome. (Courtesy of Ken K. Nischal, MD.)

Kasabach-Merritt syndrome is a thrombocytopenic coagulopathy with a high mortality rate. It is caused by sequestration of platelets within a vascular lesion.

Diffuse neonatal hemangiomatosis is a potentially lethal condition that occurs in infants, with multiple small cutaneous hemangiomas associated with visceral lesions affecting the liver, gastrointestinal tract, and brain. These hemangiomas are initially asymptomatic but can lead to cardiac failure and death within weeks. Infants with more than 3 cutaneous lesions should be evaluated for visceral lesions.

Treatment of hemangiomas The diagnosis of hemangiomas is usually clear from the clinical presentation. If not, MRI or Doppler ultrasonography may be helpful in establishing the diagnosis and delineating the posterior extent of the lesion.

Observation is indicated when hemangiomas are small and there is no risk of amblyopia from either obstruction of the visual axis or induced astigmatism.

Propranolol induces involution of most hemangiomas. Since its use was first reported in 2008,

propranolol has rapidly become the treatment of choice for most cases requiring therapy. Risks of treatment with systemic β-blockers in infants include bradycardia, hypotension, hypoglycemia, and bronchospasm. Particular caution should be taken when propranolol is used in children with PHACE syndrome, because this drug may increase their risk of stroke. Timolol maleate solution applied topically has also shown efficacy in treating superficial hemangiomas.

Corticosteroids (including topical, intralesional, or systemic administration) were previously employed as the primary treatment of hemangioma but are now used infrequently because of the potential complications associated with any form of this treatment and because of the efficacy of propranolol. Interferon alfa-2a and vincristine may be used in severe cases. Pulsed-dye laser can treat superficial hemangiomas with few complications, but it has little effect on deeper components of the tumor.

Surgical excision of periocular hemangiomas is feasible for some well-localized lesions (Fig 1819). In other cases, surgery may be used as a reconstructive tool after medical treatment.

Figure 18-19 A, Five-month-old boy with well-circumscribed hemangioma in left upper eyelid. Preoperative refraction was – 6.00 +8.00 × 40º. B, Six months after the lesion was surgically excised, the induced astigmatism resolved and refraction was

–0.25 +0.25 × 80º. (Courtesy of David A. Plager, MD.)

Drolet BA, Frommelt PC, Chamlin SL, et al. Initiation and use of propranolol for infantile hemangioma: report of a consensus conference. Pediatrics. 2013;131(1):128–140. Epub 2012 Dec 24.

Léauté-Labrèze C, Dumas de la Roque E, Hubiche T, Boralevi F, Thambo JB, Taïeb A. Propranolol for severe hemangiomas of infancy. N Engl J Med. 2008;358(24):2649–2651.

Ni N, Langer P, Wagner R, Guo S. Topical timolol for periocular hemangioma: report of further study. Arch Ophthalmol. 2011;129(3):377–379.

Vascular lesions: malformations

Vascular malformations are developmental anomalies derived from capillary venous, arterial, or lymphatic vessels. In contrast to hemangiomas, vascular malformations remain relatively static, with growth correlating to growth of the child. The age and mode of clinical presentation vary, but in general, vascular malformations manifest later in life, although cutaneous vascular malformations such as port-wine stains are evident from birth.

Orbital lymphatic malformation Orbital lymphatic malformation (LM), also known as lymphangioma, may produce proptosis at birth or, more commonly, in the second or third decade of life. LM of the orbit is best managed conservatively. Exacerbations tend to occur during upper respiratory tract infections and may be managed with short-course systemic corticosteroids. Rapid expansion may be