Lobular capillary hemangioma (pyogenic granuloma)

Risk factors for amblyopia in children with capillary hemangiomas of the eyelids and orbit.J AAPOS. 2006 Jun;10(3):262-8

INTRODUCTION: Capillary hemangiomas are the most common orbital tumors of childhood and can cause amblyopia secondary to occlusion of the pupil, anisometropia, or strabismus. We undertook this study to describe the clinical characteristics of children with capillary hemangiomas and to propose a classification system to guide clinical treatment decisions. METHODS: A retrospective review of the records of 129 patients with 132 capillary hemangiomas in two pediatric ophthalmology practices was conducted. Hemangiomas were classified based on size. Presence of aniosometropic astigmatism, ptosis, pupillary occlusion, lid margin change, proptosis, globe displacement, and strabismus was recorded. RESULTS: Thirty-one hemangiomas measured less than 1 cm in greatest dimension and were not associated with amblyogenic factors. Seventy-five patients had hemangiomas that measured greater than 1 cm, 40 of which were associated with amblyopia. Eighteen children had diffuse hemangiomas that could not be measured and 14 of these were associated with amblyopia. Five of seven hemangiomas in six patients with PHACES syndrome were associated with amblyopia. CONCLUSION: This study is the largest review of capillary hemangiomas of the orbit and eyelids. Our findings suggest that size greater than 1 cm in largest diameter is an important predictor of amblyogenic factors and approximately half of these patients will require treatment. Diffuse hemangiomas and hemangiomas in patients with PHACES syndrome will cause amblyopia in the majority of cases.

Infantile Hemangioma

Orbit and eyelid hemangiomas: is there a relationship between location and ocular problems?J Am Acad Dermatol. 2006 Oct;55(4):614-9. Epub 2006 Aug 2.

PURPOSE: Infantile hemangiomas of the orbit and eyelid can cause serious ocular problems, such as astigmatism and amblyopia. Magnetic resonance imaging and ultrasonography are the best ways to establish the diagnosis and to evaluate the extent of the lesion. Our goal was to correlate the lesion location with the visual impairment. MATERIAL AND METHODS: This was a retrospective study (1992-2004) of 63 cases of orbit and eyelid hemangiomas (13 male, 50 female patients; ages: 1 day old to 3 years old). Imaging methods were magnetic resonance imaging or computed tomographic-scanning and color Doppler ultrasonography. The anatomic definition was palpebral, extraconal, and extraconal with intraconal involvement. All patients had ophthalmologic evaluations. RESULTS: The hemangiomas were palpebral (n = 32) (normal vision: 19; amblyopia and/or astigmatism: 13), extraconal, and extraconal with intraconal involvement (n = 31) (normal vision: 4; amblyopia and/or astigmatism: 27). LIMITATIONS: This study was small; a prospective study is needed. CONCLUSION: Orbit and eyelid hemangiomas need to be carefully evaluated by an ophthalmologist. Extraconal and intraconal hemangiomas are more frequently associated with ocular involvement. However, ocular complications can occur in palpebral lesions.

Port wine stain (nevus flammeus)

Unilateral eyelid swelling in naevus flammeus faciei. Problems in differential diagnosis of unilateral eyelid swelling.Hautarzt. 1994 Nov;45(11):792-8. German

A 32-year-old white woman presented with persistent swelling of the right upper eyelid for diagnosis and therapy. History and physical examination revealed a faint nevus flammeus on the right side of her face and neck and bilateral blepharochalasis, both of which can produce unilateral periorbital swelling. A biopsy taken for histology was diagnostic in this case, since it showed lymphoedema secondary to lymphangiectasias associated with the patient's port-wine stain. Many other possible causes of unilateral lid swelling have to be considered and excluded before a definite diagnosis can be established. The differential diagnosis is discussed.

Bilateral diffuse choroidal hemangiomas with unilateral facial nevus flammeus in Sturge-Weber syndrome.Am J Ophthalmol. 2000 Sep;130(3):362-4

PURPOSE: To report bilateral choroidal hemangiomas associated with unilateral facial nevus flammeus in Sturge-Weber syndrome. METHODS: Case report. RESULTS: A 6-year-old male with a left facial nevus flammeus and a history of a left cerebral angioma had been followed 2 years for increasing esotropia. Examination demonstrated bilateral diffuse choroidal hemangiomas with overlying exudative retinal detachments. After bilateral external beam radiotherapy, the retinal detachments resolved and vision improved. CONCLUSION: Patients with Sturge-Weber syndrome and unilateral facial nevus flammeus may harbor bilateral choroidal hemangiomas. Clinical manifestations of the Sturge-Weber syndrome are characteristically unilateral and ipsilateral to the facial nevus flammeus. Bilateral choroidal hemangiomas associated with bilateral facial nevus flammeus are rare. 1,2 We report bilateral diffuse choroidal hemangiomas associated with unilateral facial nevus flammeus in Sturge-Weber syndrome.

Masson's Tumour (Intravascular papillary endothelial hyperplasia)

Intravascular papillary endothelial hyperplasia of the eyelid. Arch Ophthalmol.1983 Nov;101(11):1728-30

Intravascular papillary endothelial hyperplasia (IPEH), a benign lesion histopathologically characterized by intravascular fibrous papillary fronds covered by pleomorphic endothelium, is usually found in the subcutaneous tissues of the head and extremities. We describe the clinical and histologic findings of IPEH in the upper eyelid of a 20-year-old woman. Intravascular papillary endothelial hyperplasia has been confused with angiosarcoma. The differential diagnosis and key distinguishing features are discussed and the literature is reviewed.

Intravascular papillary endothelial hyperplasia of the orbit and ocular adnexa. A report of five cases.  Arch Ophthalmol.1983 Nov;101(11):1731-6

This is a clinicopathologic study of five patients with intravascular papillary endothelial hyperplasia involving the orbit and ocular adnexa. The median age of the patients was 45 years. Three lesions were located in the orbit, one in the eyelid, and one in the eyebrow. Four lesions were located within a distended vein and one involved an artery. The walls of the vessel corresponded to the "capsule" of the circumscribed mass. The lumen disclosed different stages of organization of a thrombus with prominent endothelial cell proliferation centered around cores of collagenous tissue and fibrin. Electron microscopy of one lesion displayed spindle-shaped cells with features of endothelial cells forming a polarized basement membrane with numerous micropinocytotic vesicles on their plasmalemma. Some cells showed ultrastructural features of pericytes. The lesion, which may be confused with angiosarcoma, represents an unusual exuberant proliferation of vascular endothelium as a cellular response to the organization of a thrombus.

Kaposi's sarcoma

Solitary eyelid Kaposi sarcoma in an HIV-negative patient.Cornea. 2006 May;25(4):490-2.

PURPOSE: To describe a case of localized Kaposi sarcoma (KS) of the eyelid in an HIV-seronegative patient. METHODS: An 80-year-old man developed an ulcerated nodular tumor-like mass that grew rapidly on his left upper eyelid. There were no similar lesions elsewhere. The eyelid lesion was completely excised and histopathologically examined. Serological analyses and molecular biologic techniques, including polymerase chain reaction, were used. RESULTS: Laboratory examinations were within normal limits, and serology for HIV was negative. Histological sections revealed a vascular proliferation composed predominantly of small slit-like blood vessels and epithelioid spindle cells, supporting the diagnosis of KS. Polymerase chain reaction was positive for human herpesvirus 8. During a 2-year follow-up, no recurrences, development of new lesions, or HIV seroconversions were observed. CONCLUSION: This is a classic KS involving only the eyelid in an HIV-negative patient. Location in the eyelid is a possible, albeit rare, initial solitary manifestation of KS in elderly HIV-negative patients. Surgery is both safe and effective.

Kimura's disease

Kimura disease of the eyelid: a clinicopathologic study with electron microscopic observations. Ophthal Plast Reconstr Surg. 2006 Nov-Dec;22(6):495-8

Kimura's disease of bilateral upper eyelids: a case report. Kaohsiung J Med Sci. 1999 Apr;15(4):239-43

Bilateral Kimura's disease of the eyelids. Br J Ophthalmol. 1992 Dec;76(12):755-7

Rhabdomyomatous Mesenchymal Hamartoma

Rhabdomyomatous mesenchymal hamartoma of the eyelid: report of a case and literature review.Ophthalmology. 2001 Apr;108(4):798-804.

PURPOSE: To report a rare case of rhabdomyomatous mesenchymal hamartoma and to compare its features with those cases previously reported. DESIGN: Interventional case report and literature review. INTERVENTION: Complete ophthalmologic and systemic examinations followed by excisional biopsy and histopathologic examination. MAIN OUTCOME MEASURES: Clinical examination features and histopathologic findings. RESULTS: A 6-month-old Latino male presented with a congenital, elevated, smooth, flesh-colored right lower eyelid lesion. An ipsilateral right limbal dermoid and an upper eyelid coloboma were also present. Excisional biopsy of the eyelid lesion revealed randomly oriented mature striated muscle tissue with associated adipose tissue, blood vessels, pilosebaceous units, and peripheral nerves, findings consistent with rhabdomyomatous mesenchymal hamartoma. Of the 24 reported cases (including the current case), eight had associated congenital anomalies. CONCLUSIONS: Although rhabdomyomatous mesenchymal hamartomas are rare and benign, they may be associated with other congenital anomalies and anomaly syndromes. As a result, we recommend systemic evaluation of patients diagnosed with this entity.

Neurofibroma

Unilateral facial and cerebral hyperplasia associated with neurofibromatosis type 1. Report of four patients. Rev Neurol. 2006 Sep 16-30;43(6):346-52

Eyelid neurofibroma affecting a young woman.J Fr Ophtalmol. 2002 Mar;25(3):333-6.

A 43-year-old female patient had an amelanotic single tumor thickening the upper right eyelid and observed since early childhood. Clinically, it appeared as a firm mass, without sharp margins, giving a thickened appearance to the superior eyelid. The ophthalmic clinical examination was normal but some subcutaneous nodules attested to the diffusion of the disease recognized as a limited form of neurofibromatosis 1. Although well tolerated, it gave rise to a cosmetic disturbance and the patient was willing to undergo an excision. A complete surgical excision was performed on the lesions and the pathological study disclosed a typical neurofibroma. Regular observation of the different cutaneous nodules was recommended.

Solitary circumscribed neuroma (palisaded encapsulated neuroma)

Palisaded encapsulated neuroma (solitary circumscribed neuroma of skin) of the eyelid: report of two cases and review of the literature.
Br J Ophthalmol. 2001 Aug;85(8):949-51

BACKGROUND/AIMS: Palisaded encapsulated neuroma of the skin (PEN) is an uncommon benign neural tumour that occurs on the head and neck, including the eyelid, but is unfamiliar to many ophthalmic clinicians. For the first time in the ophthalmic literature, the clinical and histopathological features of two patients with PEN of the eyelid are described. METHODS: Two cases of PEN were identified in the archives of the Department of Pathology, Institute of Ophthalmology, London. Clinical details were derived by a case note review and the histopathological features defined using standard methods including immunohistochemistry. RESULTS: Two cases of PEN are described, from the eyelids of patients aged 43 and 70 years. The tumours were virtually identical in nature, being painless solid masses composed of a mixture of S100 positive Schwann cells and scattered neurofilament positive nerve fibres. An incomplete thin fibrous capsule, which contains EMA positive perineural cells, partially surrounded each lesion. The lesions were distinguishable from other more common eyelid lesions only on histopathological examination. Both tumours were completely excised with no evidence of recurrence. Neither patient had signs of any associated systemic disease. CONCLUSION: PENs are usually solitary, firm, non-pigmented, dome-shaped lesions that are found on the face of patients usually over the age of 40 years. Most often these lesions are diagnosed clinically as basal cell carcinomas, dermal naevi, or skin adnexal tumours. Histologically, PENs must be differentiated from other neural tumours that may be associated with systemic syndromes, such as neurofibromas or mucosal neuromas.