Blue Nevi of the Ocular Surface: Clinical Characteristics, Pathologic Features, and Clinical Course.

PURPOSE: Blue nevus is a melanocytic tumor that is commonly found in the skin. Extracutaneous presentations, including the ocular surface, are rare. As such, the purpose of this study was to characterize the clinical features and clinical course of congenital melanocytic tumor (blue nevus) of the conjunctiva. DESIGN: Retrospective, noncomparative case series. PARTICIPANTS: Twenty-one patients with 23 blue nevi of the ocular surface that were excised surgically between 2000 and 2016. METHODS: Chart review of patients identified from a database search of the Florida Lions Ocular Pathology Laboratory records. Pathologic diagnoses were confirmed by 2 pathologists (S.R.D. and G.E.). All specimens were bleached and, tissue permitting, stained using SOX10 (MilliporeSigma, Darmstadt, Germany) and CD68 (Leica Biosystems, Nussloch, Germany). MAIN OUTCOME MEASURES: Clinical characteristics, pathologic features, and clinical course. RESULTS: Mean age of the population was 55±15 years; 71.4% (n = 15) were white and 57.1% (n = 12) were men. One patient had 3 lesions, for a total of 23 lesions examined. Clinically, 13 lesions were on the bulbar conjunctiva, 3 were on the tarsal conjunctiva, 3 were in the fornix, 2 were caruncular, 1 was episcleral, and 1 was at the limbus. Before excision, 8 patients were thought to have primary acquired melanosis, 4 with concern for primary conjunctival melanoma, and 1 thought to have metastatic disease from a plantar melanoma. Five lesions were thought to be benign, and in 8 patients, the lesions were identified incidentally after other ocular surgeries, with no diagnosis of the lesions before excision. Pathologic features were consistent with simple blue nevi in 21 lesions and cellular blue nevus in 2 lesions. No malignant transformations were noted in any patient over the mean 20.2-month follow-up period (range, 2 weeks-103 months). CONCLUSIONS: Blue nevus is a rare deeply pigmented congenital melanocytic lesion with a benign clinical course that can appear clinically similar to primary acquired melanosis or melanoma.

Congenital capillary hemangioma arising from palpebral conjunctiva of a neonate.

Capillary hemangioma is the most common benign vascular eyelid tumor in childhood. The periocular lesion appears within the first few weeks after birth and usually has superficial or deep components. Primary conjunctival capillary hemangiomas are rarely reported. We present the case of a 2-day-old child with a pedunculated capillary hemangioma arising from superior palpebral conjunctiva. A complete surgical excision was performed under general anesthesia, and the child was asymptomatic at follow-up of two months.

[Benign conjunctival tumors]. / Tumeurs conjonctivales bénignes.

Benign conjunctival tumors include congenital, melanocytic, epithelial and stromal lesions. Clinical observation often fails to make the diagnosis, and complete excisional biopsy must be performed as soon as growth is suspected. Histology remains the best way to exclude malignancy or lesions with a risk of transformation, because malignant lesions carry a poor prognosis and require specific therapy and follow-up. Onco-ophthalmologists must have a perfect knowledge of benign conjunctival tumors, since during adulthood, nevi can be confused with melanomas, and epithelial or stromal lesions with carcinomas. For children and teenagers, surgery is only recommended when growth or functional problems are observed.

Congenital smooth muscle hamartoma of the palpebral conjunctiva.

Smooth muscle hamartoma is defined as a disorganized focus or an overgrowth of mature smooth muscle, generally with low capacity of autonomous growth and benign behavior. The implicated tissues are mature and proliferate in a disorganized fashion. A healthy 5-day-old Mexican boy was referred to the authors' hospital in México city for evaluation of a "cystic" lesion of the right eye that had been noted since birth. The pregnancy and delivery were unremarkable. On physical examination, there was a reddish-pink soft lesion with a tender "cystic" appearance, which was probably emerging from the upper eyelid conjunctiva, which measured 2.7 cm in its widest diameter and transilluminated. Ultrasound imaging revealed an anterior "cystic" lesion with normally formed phakic eye. An excisional biopsy was performed, and the lesion was dissected from the upper tarsal subconjunctival space. Subsequent histologic and immunohistochemical findings were consistent with the diagnosis of congenital smooth muscle hamartoma (CSMH) of the tarsal conjunctiva. The authors' research revealed that only one case of CSMH localized in the conjunctiva (Roper GJ, Smith MS, Lueder GT. Congenital smooth muscle hamartoma of the conjunctival fornix. Am J Ophthalmol. 1999;128:643-4) has been reported to date in the literature. To the best of the authors' knowledge, this current case would be the second case reported of CSMH in this anatomic location. Therefore, the authors' recommendation is to include CSMH in the differential diagnosis of a cystic mass that presents in the fornix and palpebral conjunctiva.

Solitary lacrimal gland choristoma of the limbal conjunctiva.

Lacrimal gland choristoma occurs in cases with a failure in migration of any portion of the lacrimal secretory apparatus. It is a rare conjunctival tumor. The authors report the clinical and histopathologic features of a conjunctival lacrimal gland choristoma. A 34-year-old woman presented with a pinkish conjunctival mass in the left eye that had been present since birth. The lesion was removed by excisional biopsy and the histopathologic findings were consistent with conjunctival lacrimal gland choristoma. Clinically, these tumors resemble conjunctival hemangioblastoma, lymphangioma, or a lymphoid infiltrate. Its presence since birth, temporal epibulbar location, and pink solid appearance can help in the differential diagnosis.

Total forehead flap: hemicraniofacial giant congenital melanocytic nevus.

Being non-hair-bearing and relatively thin and having similar color and texture of the skin of the forehead provide an excellent characteristic not only for nasal reconstruction but also in other facial areas. A 28-year-old man presented in the Department of Plastic Surgery, Argerich Hospital, Buenos Aires, Argentina, with a giant congenital melanocytic nevus extended on complete left cheek, total nose, both upper and lower lids, ocular globe, and temporoparietal region. Tumor resection and reconstructive aspect were planned in 3 stages. (1) Excision of the tumor located in the cheek, nose, and both upper and lower left lids. The reconstruction of the cheek was made with a total forehead flap. The nose was resurfaced by means of a total skin graft. (2) resection of the temporoparietal nevus. (3) Treatment of the tumor involving the ocular structures. Total forehead flap was outlined, permitting carryout of skin of the total forehead area, and the blood supply was originated from the frontal and parietal branches of the temporal superficial artery and secondarily by anastomosis with the branches arising from both homolateral auricular and occipital vessels. Postoperative control 1 year after surgery showed an excellent aesthetic and functional result not only in the recipient area but also in the donor area.

[Two cases of dermolipoma].

BACKGROUND: Dermolipoma is an uncommon benign tumor, congenitally occurring on the conjunctiva, and may be present at other sites. The appearance of dermolipoma closely resembles orbital fat prolapse and limbal dermoid, and therefore it is necessary to take this into account in diagnosis. Postoperative complications such as blepharoptosis, diplopia, or keratoconjunctivitis sicca can develop. CASE REPORT: The first case was a 52-year-old female patient referred for the treatment of an orbital fat prolapse in her right eye. Another case, an 8-year-old female patient, was referred for the treatment of a bulbar conjunctival tumor in her left eye. In both cases, dermolipoma was suspected because the tumor did not show in the limbus and it did not change in size even when the eyeball was pressed. There was no external ear anomaly, facial dissymmetry, or cervical abnormality. The tumors were surgically removed without any conjunctival loss. Neither case had any postoperative complications. CONCLUSIONS: If the special features of dermolipoma are understood, it is easy to distinguish this tumor from other disorders. Most of the postsurgical complications are caused by excessive conjunctival loss resulting in symblepharon. Avoiding harm to the conjunctiva is important to prevent such complications.

Benign neonatal hemangiomatosis with conjunctival involvement. Report of a case and review of the literature.

Hemangiomas are the most common tumours of infancy. When limited to the skin, multiple lesions have a benign course and excellent prognosis but in cases of visceral involvement, the morbidity and mortality rates are high. We report a rare case of a female infant with benign neonatal hemangiomatosis who had dramatic conjunctival involvement. The spectrum of neonatal hemangiomatosis is reviewed, highlighting the importance of differentiation of the two extremes of this disorder.

Congenital smooth muscle hamartoma of the conjunctival fornix.

PURPOSE: Congenital smooth muscle hamartomas are benign tumors composed of proliferating smooth muscle cells. They are usually seen as abnormal patches of skin. Ocular involvement of congenital smooth muscle hamartomas is unusual, with rare reports of patients with external eyelid involvement or proptosis resulting from orbital tumors. We describe a patient with a congenital smooth muscle hamartoma that involved the tarsal conjunctival fornix. METHODS: Review of the patient's medical records, including the results of ophthalmologic, radiologic, and histologic examinations. RESULTS: A healthy 2-year-old boy was initially seen with a conjunctival mass. He had a discrete, gray, cystic-appearing lesion in the inferior fornix of the left eye. A magnetic resonance imaging study revealed no signs of extension of the lesion into the orbit. The lesion was surgically excised. Histologic sections showed large bundles of smooth muscle with a fibrotic background and interdigitating fat, consistent with a diagnosis of a congenital smooth muscle hamartoma. CONCLUSION: To our knowledge, this is the first report of a patient with a congenital smooth muscle hamartoma arising from the conjunctival fornix. It presumably originated from either the smooth muscle of the vascular endothelium or from the capsulopapebral muscle. Congenital smooth muscle hamartoma should be considered in the differential diagnosis of cystic-appearing conjunctival fornix lesions.

[Epithelial-cystic nevus of the conjunctiva (Parinaud's dermo-epithelioma)]. / Nevul epitelio-chistic al conjunctivei (dermo-epiteliomul Parinaud).

The epithelio-cystic nevus of the conjunctiva, initially improperly denominated by Parinaud dermo-epithelioma, is a dysplasia circumscribed at the level of bulbar conjunctiva, characterized by its forming of epithelial cysts which develop in a base layer made of nevoid cells. This affection evolves by forming epithelial cysts mostly muciparus ones, when the tumor is sited rather far from the limbus, where multiple chaliced cells are to be found in the epithelium, and as simple epithelial cysts, mostly in the epidermis, when it is sited near the limbus where chaliced cells are scarcer. In this condition the setting of the epithelial compound takes the form of full lobules. The nevoid compound represents the capital element which establishes the diagnosis. The epitheliocystic nevus of conjunctiva is a benign dysembryo with excessive development of epithelial cells in a basic layer of nevoid cells. This affection is benign, but its morphologic diversity and its many denominations give rise to periodical debates.

Dermolipoma ocular: relato de caso / Ocular dermolipoma: a case report

Dermolipoma e um tumor conjuntival benigno, congenito, usualmente bilateral, lentamente progressivo, que pode determinar prejuizos funcionais a motilidade ocular, com diplopia e desconforto. Entretnto, alteracoes cosmeticas sao geralmente os principais motivos de procura do oftalmologista. O tratamento e microcirurgico, devendo-se evitar lesoes nos musculos extra-oculares, ductos e glandula lacrimal, mesmo as custas de uma remocao apenas paarcial do dermolipoma. Quando pequenos e assintomaticos a conduta e essencialmente conservadora e expectante, para evitar riscos de lesoes operatorias. Dermolipomas oculares, embora raros, sao ocasionalmente relatados na literatura medica; este caso foi o unico encontrado em cerca de 7000 pacientes atendidos pela autora nos ultimos anos

Epibulbar complex choristoma involving the bulbar conjunctiva and cornea.

A male infant was born with a fleshy mass between the temporal bulbar conjunctiva and cornea in the right eye. Four weeks after birth the patient underwent excision of the epibulbar tumor and a lamellar keratoplasty. Histopathologic examination of the excised tumor revealed cartilage, lobules of lacrimal gland, and adipose tissue, indicating an epibulbar complex choristoma. Although the graft remained almost completely transparent, right amblyopia ensued. Epibulbar complex choristoma involving the conjunctiva and cornea is a rare condition.

Benign cystic nevus of the conjunctiva.

A 51-year-old white man presented with a large, cystic conjunctival mass along the inferotemporal limbus which was obscuring his vision. Differential diagnosis included benign cystic nevus of the conjunctiva, lymphangioma, lymphangiectasia, and multiple conjunctival inclusion cysts. Biopsy revealed a large cystic nevus of the conjunctiva.

[Oto-cranio-facial syndromes with asymmetrical involvement of the face]. / Síndromes otocraneofaciales con afectación asimétrica de la cara.

Four cases otocraniumfacial syndromes with asymmetrical affectation of the face are presented. Clinic, radiologic and genetic studies are made. Authors comment the difference between Goldenhar's syndrome and hemifacial microsomia. Finally the etiopathogenic thesis, differential diagnostics and treatment are commented.