Multicentric myofibromatosis presenting as a large congenital eyelid myofibroma.

Infantile myofibromatosis is a rare mesenchymal neoplasm that commonly involves the head and neck but rarely the eyelid. We report the case of a newborn boy referred for evaluation of a left eyelid lesion that occluded the visual axis. Urgent biopsy was performed to evaluate for malignancy. Histopathologic analysis demonstrated myofibroma. Although these lesions have been reported to regress spontaneously, debulking surgery was performed to prevent sensory or anisometropic amblyopia. Follow-up systemic evaluation revealed numerous subcutaneous and deep soft tissue lesions. There was no visceral involvement.

Congenital Eyelid Rhabdomyosarcoma.

Rhabdomyosarcoma is the most common type of soft tissue sarcoma in children. The authors present a rare case of eyelid rhabdomyosarcoma in a newborn, who was found to have a reddish eyelid tumor in his OD. A mass with a clear margin, confined to the upper eyelid, was revealed using orbital MRI. Intralesional steroids were injected under the impression of a capillary hemangioma and the tumor shrank initially, but grew rapidly later. Therefore, a debulking surgery was performed and the final diagnosis was embryonal rhabdomyosarcoma. After the operation, metastases still occurred despite the treatment with chemotherapy and concurrent radiation. The patient expired at 6 months of age. In an autopsy, a neuroblastoma was incidentally found in his left adrenal gland. Early biopsy may help lead to an early correct diagnosis and avoid metastases in similar cases.

Divided nevus of the eyelid: review of embryology, pathology and treatment.

Divided nevus, which is also known as "kissing nevus," "split ocular nevus" and "panda nevus" is a rare congenital dermatological abnormality that occurs on opposing margins of upper and lower eyelids. There is a paucity of literature on this rare anomaly, with most knowledge from this disease process derived from isolated case reports and series. The purpose of this study is to report a new case of divided nevus of the eyelid and to discuss the unique embryology, pathology, and potential treatment options for this rare entity. A systematic review of literature was performed of the English literature on PubMed and Medline with just under 150 cases reported in the literature. The vast majority of the divided nevi seen in this review were medium sized and of the melanocytic intradermal type. There were no described cases of malignant transformation in any of the documented cases. Numerous methods for reconstruction were described including the entire reconstructive ladder with both one and two staged approaches. In this review, we present basic guidelines to the reconstruction of these complicated defects, although ultimate treatment should be individualized and dependent on surgeon comfort.

Surgical management of a congenital panda nevus with preexpanded triple forehead flaps and temporal island flap.

Congenital panda nevus or the divided nevus of the eyelids is a rare form of melanocytic nevus involving both upper and lower eyelids with the risk of future malignant transformation along with the patients' complaints about the cosmetic appearance. Delicate anatomic features of the eyelids and the limited skin redundancy of the periorbital region make the surgical removal and reconstruction difficult, even in mild cases with partial involvement of the eyelids. A case of congenital panda nevus involving upper and lower eyelids, the eyebrow, and part of the malar eminence and the nasal dorsum is presented. A multistaged surgical management of the lesion with staged excision and reconstruction with preexpanded forehead flaps, temporal island flap, and skin grafts is discussed.

Isolated infantile myofibromatosis of the upper eyelid: uncommon localization and long-term results after surgical management.

Myofibroma, the most common juvenile fibrous disorder in infancy, usually manifests as a solitary soft tissue tumor and less commonly as simultaneous multiple tumors in both soft tissue and bones. Infantile myofibromatosis is well described in the head and neck, but cutaneous lesions rarely occur in the periorbit and orbit, where fast growth and bone damage can mimic malignant tumors. We describe a case of a solitary periorbital myofibroma in a newborn. Treatment consisted of partial excision of the tumor. Histologic and immunohistochemistry analyses provided the diagnosis of infantile myofibromatosis. The patient remains disease-free at 10 years of age, but with some visual impairment. Infantile myofibromatosis is an uncommon tumor with exceptional periorbital involvement. Differential diagnosis can be difficult when it is solely based on histologic assessment. Immunohistochemistry evaluation demonstrating cytoplasmic actin filaments in neoplastic spindle cells confirms the diagnosis. As soon as the diagnosis is made, chest and abdominal imaging must be performed to evaluate the overall prognosis and direct treatment. The treatment of choice is early conservative surgery to minimize functional and/or esthetic damage. Complete tumor excision is not always possible. Lengthy ophthalmologic monitoring is required to detect the onset of amblyopia.

Massive congenital kaposiform hemangioendothelioma of the eyelid in a neonate.

BACKGROUND: Kaposiform hemangioendothelioma (KHE) is a rare and aggressive vascular tumor of infancy and childhood. It is associated with the development of Kasabach-Merritt syndrome, a life-threatening consumptive thrombocytopenia. We report an interesting case of a massive periorbital congenital KHE in a neonate to raise awareness of this aggressive diagnosis. METHODS: A male neonate presented with a large congenital mass of the lower eyelid. To prevent development of amblyopia, this mass was surgically excised on the sixth day of life. RESULTS: Histologic investigation demonstrated spindle-shaped endothelial cells with surrounding crescentic vessels, which were GLUT-1 receptor-negative and D2-40 receptor-positive, consistent with KHE. Surgical excision of the periorbital KHE successfully cleared the neonate's visual axis. At 1 year of follow-up, there was no evidence of tumor recurrence, and visual development was progressing normally. A pleasing surgical result was achieved without periorbital distortion. CONCLUSIONS: Pediatric vascular tumors have historically been wrought with diagnostic confusion. With recent advances in immunohistochemistry, this previously uncharacterized group of tumors has been differentiated into multiple distinct clinical entities. Accurate and timely diagnosis is paramount because these tumors vary greatly in their clinical behavior, prognosis, and recommended treatment. Surgical excision is preferred and necessary in a neonate with visual access obstruction to prevent amblyopia and irreversible blindness.

Congenital solitary eyelid trichoepithelioma.

Trichoepitheliomas are unusual, benign tumors of hair follicle origin. They may present in children and adults as acquired lesions on the body and face, but they rarely involve the eyelids. Solitary trichoepitheliomas have not previously been reported in infants. We present a first report of congenital solitary eyelid trichoepithelioma in an infant and review the classification of trichoepitheliomas.

One stage management of a giant kissing naevus.

We report a one stage management of a Giant Divided Naevus of the eyelids with excellent cosmetic results.

Visual development in infants: visual complications of periocular haemangiomas.

UNLABELLED: Periocular haemangioma of childhood can severely impact visual development. OBJECTIVE(S): To review our experience with 20 periocular haemangioma patients; to review infant ocular development in the context of periocular capillary haemangioma; to identify early clinical warning signs that may precede devastating visual outcomes in the absence of timely management and to review our experience with surgical debulking for the treatment of selected periocular haemangioma. DESIGN: Retrospective case series. INTERVENTIONS: Twenty children with congenital periocular haemangiomas received care by a multidisciplinary team consisting of doctors from the specialties ophthalmology, plastic surgery, paediatrics and dermatology. The patients were separated by age at presentation to our centre (1 year). Based on consensus amongst the team, certain patients were considered to be at high risk for development of amblyopia, permanent cortical visual change or blindness. These patients were scheduled for urgent surgical excision or debulking. The effect of treatment on visual development over time was evaluated. RESULTS: Patients presenting to our centre after 1 year of age were more likely to have amblyopia (75% vs. 0% if presenting at

Congenital melanocytic nevi of the eyelids and periorbital region.

BACKGROUND: Congenital melanocytic nevi of the eyelids and periorbital region are unusual. Although their malignant potential can be debated, they present a significant aesthetic concern and also disturb lid function. In this article, the authors present an expanded approach to evaluation and treatment of these patients. METHODS: Forty-four consecutive patients, aged 6 months to 18 years, were treated from 1980 to 2008. All patients had congenital nevi involving one or both eyelids, with or without extension into the surrounding periorbital area and face. Follow-up ranged from 6 months to 20 years. RESULTS: All patients were treated successfully with excision and reconstruction of their congenital eyelid and/or periorbital nevi. The involved ciliary border was preserved in all but one case, where the exophytic lesion presented function concerns. Complications included asymptomatic lateral ectropion in three patients. Asymmetry of the palpebral apertures, before treatment, was present in at least half of the patients with extensive facial nevi, and the abnormalities causing these differences may impact efforts to obtain final lid symmetry. A single patient died as a result of extensive metastatic melanoma from an extracutaneous site. CONCLUSIONS: Early evaluation and treatment of these nevi may help in preventing the aesthetic, functional, and health-related issues for the patients. Although the current group of infants and young children will not reach full facial growth for more than another decade and a half, and therefore await critical assessment of their long-term outcomes, the authors hope that the experience gained to date will assist surgeons in managing these complex reconstructions.

Unilateral eyelid angiofibroma with complete blepharoptosis as the presenting sign of tuberous sclerosis.

Tuberous sclerosis is a multisystem autosomal-dominant disease characterized by hamartomatous growths in the brain, skin, kidneys, eyes, and heart, but it may affect almost any organ. Retinal hamartomas are 1 of the major diagnostic criteria for tuberous sclerosis and occur in approximately 50% of patients. Nonretinal findings include angiofibromas of the eyelid, strabismus, and pseudo-colobomas of the lens and iris. We report a case of a newborn with congenital eyelid angiofibroma mimicking complete congenital blepharoptosis that was revealed by central nervous system imaging to be part of the tuberous sclerosis complex.

Congenital osteoma cutis of the lateral canthus.

Osteoma cutis is a rare condition involving the formation of bone in skin or subcutaneous tissue. This may be a primary event or, more often, secondary to an inflammatory, traumatic, or neoplastic process. There is little evidence in the literature of this condition involving the eye or ocular adnexal structures. The authors report two remarkably similar cases involving children with congenital isolated osteoma cutis involving the left lateral canthus.