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.

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.

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.

Selective embolization and resection of a large noninvoluting congenital hemangioma of the lower eyelid.

PURPOSE: To present a combined interventional radiologic and resection technique for the management of a large amblyogenic, highly vascular lesion of the lower eyelid associated with severe mechanical ectropion, conjunctival pyogenic granuloma, and recurrent, spontaneous conjunctival bleeding. METHODS: Preoperative selective embolization and coil placement were followed by resection of the tumor. RESULTS: Embolization, coil placement, and resection were uneventful with cosmetic and functional visual improvement obtained. Pathology indicated a noninvoluting congenital hemangioma. CONCLUSIONS: To the authors' knowledge, this is the first surgical technique reported in the ophthalmic literature for the selective embolization of a noninvoluting congenital hemangioma prior to its resection. Such a technique should be considered in the management of large lesions of this type.

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.

The surgical treatment of congenital divided nevus of the eyelid: a case report and review of the literature.

We describe a 16-year-old male patient with congenital divided nevus of the eyelids who underwent surgical management using musculocutaneous and tarsoconjunctival flap, and discuss the alternative surgical approaches in the light of the literature. Musculocutaneous flap in conjunction with a tarsoconjunctical flap can be considered as a suitable surgical approach in the management of divided nevus. The patient's age, gender, the diameter of the lesion, and involvement degree of the entire tarsus, must be kept in mind before the surgery. If the surgical treatment method is tailored for the patient, a better cosmesis can be achieved.

Eyelash reconstruction with strip composite eyebrow graft.

Eyelash hairs have certain unique properties such as parallel direction, limited length, and tapering, which makes eyelash reconstruction considerably difficult. Several methods for eyelash reconstruction have been reported. Among them, strip composite eyebrow graft is the most suitable because the properties of eyebrow hairs are very similar to those of the original eyelash hairs. A rich blood supply in the eyelid ensures a good survival of the graft. Careful selection of the donor strip harvesting site is critical for preserving a good direction of hairs. The thickness of the graft strip should be determined considering the optimal thickness of hairs being obtained. When suturing the graft strip to the recipient bed, the burying method is suitable for preventing the protrusion of the graft. A well-performed surgical procedure results in a postoperative outcome that closely resembles the natural eyelashes.

[Kissing nevus, a rare form of palpebral nevus--case report]. / Kissing naevus (divided naevus) o forma rara de nev nevocelular situat pe pleoape--prezentare de caz.

Kissing naevus is a rare form of congenital nevocellular naevus. We present the cases of three young girls. One of them was 9 years old and had an asymptomatic homogeneously pigmented round-oval naevus on the right eye and two girls (12 and 20 years old) who had the same kind of naevus of different sizes on the left eye, symmetrically situated on the two eyelids. The lower part of the naevus were bigger than the upper part in all the three cases. Histological they are compound or dermal nevus As treatment, ones recommend early excision, others contraindicates any surgical interventions.

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.

Phakomatous choristoma of eyelid: electron microscopical confirmation of lenticular derivation.

This article presents the sixth recorded example of phakomatous choristoma of the eyelid. The tumor occurred in a 4-month-old boy and was located in the lower lid near the inner canthus. The findings from light microscopy included epithelial, tubular structures with associated patches of thick, irregular basement membrane. The results of electron microscopy (ie, epithelial cells with a scarcity of organelles, homogeneous type of basement membrane, cytoplasmic microfilaments, segments of fascia occludens, and degenerated cells with peculiar interdigitations of their plasmalemmas) are all consistent with lenticular derivation, as originally proposed by Zimmerman.

Congenital divided naevus of the eyelids.

A newborn infant presenting with a divided congenital melanocytic naevus of the eyelid is described. Because of the severe disfigurement, risk of later malignant change in the lesion, and the possibility of deprivation amblyopia, early surgical treatment is recommended for all medium and large congenital melanocytic naevi of the eyelid. Surgery in the first few months of life gives the best cosmetic results.

Disorders of the lids.

Included in the discussion is the management of anomalies of abnormal formation of the lid folds, disorders of differentiation of the lid margins, and disorders of tissue differentiation. Also discussed are lid involvement with bacterial lesions and viral diseases.

[Congenital ectropion of the iris epithelium and glaucoma]. / Ectropion congénital de l'épithélium irien et glaucome.

Two cases of unilateral ectropion uveae with ipsilateral juvenile glaucoma are reported including one with ipsilateral ptosis. This rare, non progressive anomaly, is characterized by the presence of iris pigment epithelium on the anterior surface of iris stroma, quite often associated with neurofibromatosis, ipsilateral ptosis or Rieger's syndrome. Congenital or juvenile glaucoma is almost systematically associated with the condition and must be evaluated and treated. Pathogeny of the anomaly has to be interpreted keeping in mind the concept of neurocristopathy, i.e. developmental anomalies of neural crest origin tissues.

[Isolated palpebral tumor revealing Von Recklinghausen's neurofibromatosis: a case report]. / Tumeur palpébrale isolée révélatrice de la neurofibromatose de Von Recklinghausen.

INTRODUCTION: Von Recklinghausen's neurofibromatosis is the most frequent of phakomatoses. Its incidence is 1/2,500-3,000 births. It is inherited through a dominant autosomal mode with a strong prevalence and changing expression. Eye and skin signs are essential to diagnosis and this case provided a noteworthy example of a unilateral upper palpebral tumor revealing this disease. CLINICAL CASE: We report a case of a 6-year-old boy whose original clinical manifestation was a tumor on the upper eyelid of the right eye, present at birth. According to the anamnesis, this palpebral tumor gradually increased in size, causing a full ptosis. A biopsy exeresis of the tumor was carried out with an anatomicopathological study of the biopsy specimen, confirming the diagnosis. CONCLUSION: The anatomicopathological study of the biopsy specimen enabled diagnosis of a neurofibromatosis revealed by this isolated palpebral localization responsible for significant aesthetic and functional damage.

[Congenital malformations of the eyelids]. / Les malformations congénitales des paupières.

Congenital malformations of the eyelids have been classified in many ways, considering their various origins. The author proposes hereby a simplified classification concentrated on the therapy: 1. isolated malformations of one or two eyelids 2. palpebral anomalies associated with a more extensive craniofacial malformative syndrome 3. congenital tumors On this basis the author examines the most frequent malformations: blepharoptosis, coloboma, epicanthus, blepharophimosis, microblepharism and some congenital tumors. For each of these lesions, a surgical treatment is proposed and illustrated.