A 15-year review of secondary and tertiary optic nerve sheath fenestration for idiopathic intracranial hypertension.

PURPOSE: Optic nerve sheath fenestration (ONSF) is a common surgical option for patients with idiopathic intracranial hypertension (IIH) with vision loss refractory to medical management. Little is known about the visual benefit of repeated ONSF. The authors aimed to assess the efficacy of secondary and tertiary ONSF in patients with IIH. METHODS: A retrospective chart review was performed on all patients with repeat ONSF for IIH at Emory University from 1999 to 2016. Primary outcome measures included visual acuity, optic nerve head findings, and visual field results. RESULTS: A total of nine eyes in seven patients (five females and two males) with repeat ONSF were identified. Two of the seven patients had repeat ONSF in both eyes, while the remaining five patients had only one eye repeated. Five of seven patients (five eyes) improved or remained stable after the secondary ONSF. Two patients (three eyes) continued to worsen despite the secondary fenestration surgery and underwent tertiary ONSF at an average of 13.2 months (SD 5.5 months) after the failed secondary ONSF. Both patients that underwent the tertiary fenestration showed improvement. Six of the patients had either improvement or stability in their clinical findings at their last documented follow-up, but one continued to worsen despite intervention. CONCLUSIONS: This study suggests that secondary and tertiary nerve sheath fenestration is a viable management option for patients with progressive vision loss from IIH. Repeat ONSFs do not appear to have increased complication or failure rates compared to prior documented studies regarding primary fenestrations.

Nodular Fasciitis of the Orbit: A Case Report Confirmed by Molecular Cytogenetic Analysis.

Nodular fasciitis is a benign fibroblastic proliferation typically found in the subcutaneous tissue or superficial fascia of the extremities that is often confused for malignancy. These lesions rarely occur on the eyelids and ocular adnexa and are seldom analyzed by ophthalmic pathologists. USP6 gene rearrangement has been recently demonstrated in nodular fasciitis and this rearrangement may lead to the formation of a fusion gene MYH9-USP6 in some cases. Herein, the authors describe a 38-year-old woman with a 6-month history of a progressively enlarging mass beneath her right medial upper eyelid. Histopathologic analysis of the excisional biopsy confirmed classic features of nodular fasciitis. Molecular cytogenetic analysis revealed a rearrangement of the USP6 locus, confirming the diagnosis of benign nodular fasciitis.

A modification to the Hughes tarsoconjunctival flap for a challenging case of recurrent lower eyelid retraction.

PURPOSE: To present a modification of the Hughes tarsoconjunctival flap to correct a case of refractory lower eyelid retraction. METHODS: The authors report a 49-year-old man with cicatricial right lower eyelid retraction that failed multiple surgical interventions, including ear cartilage grafts to the posterior lamella, tarsal strip, alloplastic implant, and Sub-Orbicularis Oculi Fat (SOOF) lift. A modified Hughes tarsoconjunctival flap was used for repair of a nonmarginal defect of the lower eyelid. A marginal defect was purposely created with a full-thickness blepharotomy that compensated for the amount of lower eyelid retraction. The posterior lamellar defect was filled with the tarsoconjunctival-Muller's flap and the anterior lamella defect covered with a full-thickness retroauricular skin graft. Mueller's muscle was included in the flap to increase the vascular supply to the reconstructed eyelid. Likewise, the authors waited 9 weeks to divide the flap because of concerns that the vascular supply of the surrounding eyelid might be compromised from multiple previous surgeries. RESULTS: A single case of cicatricial right lower eyelid retraction status after multiple failed surgical interventions successfully completed a full-thickness blepharotomy with a modified Hughes tarsoconjunctival flap. Two months after the procedure, the patient had good cosmesis and function with resolution of his signs and symptoms of exposure keratopathy. CONCLUSIONS: The Hughes tarsoconjunctival flap combined with a full-thickness blepharotomy may be a useful technique in selected patients for repair of nonmarginal defects of the lower eyelid when other standard techniques have failed.