Pujari, Amar; Bajaj, Mandeep S.; Shabeer, B.
A 9-month-old male child born out of nonconsanguineous marriage was brought with the history of abnormally bisected nose since birth (Fig. 1). On examination, the child was able to fix and follow the light in each eye separately, but because of the broad nasal bridge, there was pseudoesotropia. Anterior segment and fundus examination was essentially within normal limits. The external nasal openings were widely displaced on either side with a broad sheet of skin between them; nasal examination revealed patent nasal openings on each side, which were independently opening in the nasopharynx and the nasolacrimal system on each side was patent.
There are 4 groups of proboscis lateralis (proboscis is a blind ended tube-like structure located in the midface) according to Khoo. Group I affected individuals will have proboscis lateralis with a normal nose. Group II individuals will have proboscis lateralis with an ipsilateral deformed nose. Similarly, Group III patients will have proboscis lateralis with an ipsilateral deformed nose, eye, and ocular adnexa and Group IV patients have an ipsilateral deformed nose, eye, ocular adnexa, and cleft lip or palate.
Homer, Natalie; Freitag, Suzanne K.
A 90-year-old man had a 14-month history of an enlarging right cutaneous cheek and temple lesion with rolled, ulcerated borders and a hypopigmented necrotic center. The lesion was originally thought to be a malignancy and an outside physician attempted excision. The patient presented to our service after the lesion grew to involve the right facial nerve and upper and lower eyelids (Fig. 1). He developed significant exposure keratopathy due to a combination of paralytic and cicatricial ectropion despite lateral tarsorrhaphy. Further inquiry revealed a history of prior necrotic skin change of the anterior trunk, which had been previously determined to be pyoderma gangrenosum. Biopsy of the right temple lesion revealed a dermal neutrophil-rich infiltrate with negative microbial stains, consistent with pyoderma gangrenosum (Fig. 2). The patient underwent lysis of upper and lower eyelid cicatrix with placement of a permanent, nearly complete right tarsorrhaphy with symptomatic relief and resolution of exposure keratopathy. Stabilization of active skin ulceration was achieved with topical steroid therapy, and regular dermatologic monitoring has thus far been negative for recurrence.
Pyoderma gangrenosum is a rare neurophilic dermatosis that presents with painful ulcerative skin lesions and is often associated with systemic inflammatory disease. Although pathologic examination is useful in excluding other infectious and autoimmune etiologies, the diagnosis is ultimately made clinically. Treatment involves topical wound care and systemic immunosuppression. Expansion of ulceration after surgical debridement is common. Pyoderma gangrenosum should be considered in malignant-appearing facial lesions with mechanical and neurologic sequelae, and excision should be pursued with caution.
Jakobiec, Frederick A.; Zakka, Fouad R.; Lee, Nahyoung Grace
Most bony and cartilaginous lesions of the orbit and periorbital compartments are benign, grow endophytically, and are composed of dense lamellar bone (eburnated or ivory osteomas). An 87-year-old woman had a well-circumscribed, firm, round, and exophytic lesion of the brow region for at least 15 years. Excisional surgery disclosed an osteocartilaginous lesion with an enveloping pseudocapsule (periosteum/perichondrium) and a narrow stalk connecting it to the frontal bone. The periphery of the lesion displayed lamellar bone which appeared to be replacing a central cartilaginous zone. The adjacent deep preaponeurotic fat displayed nodules of collagen with myxoid change and occasional CD34+ spindle cells suggestive of a spindle cell lipoma. Because of the osteochondroma’s deep location in the preaponeurotic fat, the lesion differs from an osteoma cutis found in the dermis which fails to exhibit a cartilaginous component or a periosteum. Other clinically simulating lesions are described.
Ramesh, Sathyadeepak; Goldberg, Robert Alan; Rootman, Daniel Benson
Cretara, Elizabeth A.; Swan, Robert T.; Hill, Robert H
A 50-year-old male presented with bilateral cicatricial entropion with subepithelial fibrosis and fornix foreshortening. Conjunctival biopsy showed lymphocytic infiltrate along the interface of the epithelium and lamina propria with linear, shaggy fibrinogen deposition along the epithelial basement membrane zone, supporting a diagnosis of lichen planus. The patient was treated with commercially available topical cyclosporine for 5 months after which bilateral lower eyelid splitting procedure with recession of the anterior lamella and complete excision of the lower eyelid lashes was performed.
Mahatma, Gina; Sadaka, Ama; Berry, Shauna; Malik, Amina; Lee, Andrew G.
A 10-year-old boy with bilateral colobomatous cavitary disc anomalies presented with a 3-month history of vision loss in his right eye. MRI of the head and orbit revealed bilateral tubular cystic enlargement of the optic nerve/optic sheath complex with thickening of the optic nerves without inflammation or neoplasm, suggestive of bilateral optic nerve meningocele. An optic nerve sheath fenestration was performed OD, and he experienced an improvement and stabilization of vision in his right eye during a 1-year follow-up period. The authors recommend that surgical decompression, particularly optic nerve sheath fenestration, should be considered in cases with progressive vision loss due to optic nerve meningocele.
Choi, Catherine J.; Jakobiec, Frederick A.; Zakka, Fouad R.; Foster, C. Stephen; Chodosh, James; Freitag, Suzanne K
The purpose of this study was to describe a possible causal relationship between ocular cicatricial pemphigoid (OCP) and ocular surface squamous neoplasia. Two middle-aged female patients with already diagnosed OCP were studied in regard to the subsequent onset of conjunctival squamous neoplasia. Their clinical histories, ocular examinations, clinical photographs, and results of biopsies and any ancillary immunofluorescent laboratory evaluation studies were carefully reviewed. One patient had a preinvasive squamous dysplasia and the other an invasive squamous cell carcinoma, both in the unequivocal setting of OCP with bilateral conjunctivitis, symblephara, and forniceal foreshortening. The patients had been receiving intensive immunotherapy consisting of some combination of corticosteroids, rituximab, and interferon alpha. Both patients had a positive immunofluorescent study demonstrating immunoreactants at the level of the epithelial basement membrane. Each patient had 2 earlier negative immunofluorescent studies before a third was positive. While rare, there is 1 previous report of an association between OCP and conjunctival squamous neoplasia. The current report provides more data supporting the proposal that this conjunction is more than a random event. Repeat immunofluorescent studies after an initial negative result in a patient with strong clinical signs of OCP are imperative due to the frequency of false negative studies in the context of clinically persuasive disease.
Singh, Swati; Mittal, Ruchi; Rath, Suryasnata
A 64-year-old healthy female presented with a papillomatous limbal lesion involving 6 clock hours of superior limbus and diffuse involvement of upper tarsal conjunctiva of left eye of 6 months duration. Excision of superior limbal lesion on histopathology showed carcinoma in situ with focal breach in basement membrane and moderate degree of differentiation. The surgical base and margins were uninvolved. The tarsal lesion was initially treated with subconjunctival interferon alpha 2b (IFN alpha 2b) (3 cycles of IFN alpha 2b) with poor response. Subsequently treatment with topical mitomycin C 0.04% showed a dramatic response in the upper eyelid tarsal lesion. A small residual lesion needed excision. One year after treatment, she was completely tumor-free. In multifocal ocular surface squamous neoplasia, multimodal treatment with excision and topical mitomycin C may be effective in cases refractory to immunotherapy.
Alam, Md. Shahid; Vaidehi, D.; Therese, K. Lily; Ali, Mohammad Javed
Transient nasolacrimal duct obstruction secondary to a mass lesion compressing the sac or sac-duct junction is rare. The authors report a case of a young female who presented to the authors with complaints of watering and a mass lesion in the lacrimal sac region. An organized perilacrimal mass filled with thick pultaceous material was noted during surgery. Following the drainage of the mass lesion, irrigation of the lacrimal system was freely patent. Microbiological examination and DNA sequencing targeting interspacer region was suggestive of Pleurostomophora richardsiae species. To the best of the authors’ knowledge, this is only the second case report of a subcutaneous perilacrimal mass causing transient nasolacrimal duct obstruction by Pleurostomophora richardsiae.
Verma, Rohan; Lee, Bradford W.; Alameddine, Ramzi M.; Ko, Audrey C.; Khanna, Paritosh C.; Kikkawa, Don O.; Korn, Bobby S.
An 11-year-old female presented with orbital cellulitis, bacterial sinusitis, enlarged left superior ophthalmic vein, dural venous sinuses, and internal jugular vein. The patient underwent endoscopic sinus surgery and was started on intravenous antibiotics and anticoagulation with limited improvement in orbital signs and symptoms. A magnetic resonance imaging/magnetic resonance venography of the orbits and brain revealed a dilated left superior ophthalmic vein with absence of flow but no clearly discernible orbital abscess. Intravenous corticosteroids resulted in dramatic improvement of pain, hypoglobus, proptosis, and extraocular motility, all of which rapidly recurred on discontinuation. Serial imaging revealed progression of what eventually manifested as a well-defined, rim-enhancing peri-superior ophthalmic vein abscess, which was incised and drained with prompt resolution of orbital cellulitis and complete visual recovery.