Tal J. Rubinstein

Desmoplastic melanoma of the eyelid and conjunctival melanoma in neurofibromatosis type 1: A clinical pathological correlation

A 56-year-old woman with neurofibromatosis type 1 (NF1) presented with a left upper eyelid amelanotic nodule with adjacent eyelid margin hyperpigmentation. Physical examination additionally revealed primary acquired melanosis (PAM) on the palpebral conjunctiva of the same eyelid. Full thickness eyelid excision and conjunctival map biopsy identified desmoplastic melanoma of the eyelid in addition to invasive conjunctival melanoma and conjunctival melanoma in situ. Sentinel lymph node biopsy was negative for metastasis. She was treated with surgical excision for the eyelid melanoma and topical mitomycin C for the conjunctival melanoma. We discuss the rare entity of desmoplastic melanoma of the eyelid and its possible association with NF1.

Anatomy and histology of the frontalis muscle.

Purpose: To determine the gross and histologic configurations of the medial and lateral frontalis muscle. Methods: After making a midcoronal incision and bluntly dissecting to the orbital rim, the frontalis muscle was marked and measured. A protractor was used to measure the frontalis–orbicularis angle (FOA) and, when present, the angle of central bifurcation (AOB). Three strips of full-thickness forehead soft tissue measuring 0.5 cm × 8 cm were excised 3, 4.5, and 6 cm above the supraorbital notch and analyzed histologically for the presence of skeletal muscle fibers. Data were analyzed using 2-sample t tests, paired t tests, Pearson correlations, and mixed effect models. A p value of ⩽ 0.05 was considered statistically significant. Results: Sixty-four hemifaces of 32 cadavers (16 males) were dissected. All specimens were Caucasian. The average age was 78.2 years (range, 56–102 years). The average FOA was 88.7° (13.0°), and the average AOB was 90.0° (26.4°). A visible midline bifurcation occurred in 28 of 32 subjects (88%) at an average height of 4.7 cm (range, 2.4–7.2 cm) superior to the supraorbital notch. Continuous skeletal muscle fibers were present within the midline bifurcation histologically in 89%, 75%, and 11% of specimens 3.5, 5.0, and 6.5 cm above the supraorbital notch, respectively. In 46% of individuals, skeletal muscle fibers were continuously present microscopically within the gross bifurcation. Conclusion: While a medial frontalis muscle bifurcation occurs grossly in most senescent Caucasians, muscle fibers exist microscopically within this zone in nearly half of individuals.

Orbital and Intraocular Myofibroblastoma

Abstract A 66-year-old woman presented with a blind, painful, hypertensive, and proptotic left eye. Computed tomographic imaging revealed a well-circumscribed mass involving the left orbit and globe. Metastatic work-up failed to reveal extraorbital lesions and the tumor was removed in toto via an evisceration approach orbitotomy. Histopathology and immunohistochemistry were most consistent with mammary-type myofibroblastoma with fascicles of bland, uniform spindle cells that stained positive for desmin and CD34. We are not aware of previous reports of orbital or ocular myofibroblastoma. This neoplasm has not been shown to recur, undergo malignant transformation, or metastasize. Familiarity with its clinical, histopathologic, and immunohistochemical features may improve diagnostic accuracy and treatment decisions for patients presenting with similar findings.

Orbital Xanthogranuloma in an Adult Patient With Xanthelasma Palpebrarum and Hypercholesterolemia

A 44-year-old man with a history of hypercholesterolemia presented with eyelid lesions and a separate orbital mass. Pathology of eyelid lesions confirmed xanthelasma palpebrarum, and pathology of the orbital mass showed a non-Langerhans cell xanthogranuloma, consistent with a lesion within the spectrum of adult orbital xanthogranulomatous disorders. While xanthelasma palpebrarum is associated with increased serum lipids, adult orbital xanthogranuloma does not share a clear association. Distinct, histology-proven xanthelasma palpebrarum and orbital xanthogranuloma rarely occur together in the literature. This case further represents a unique coexistence between these 2 lesions in an adult with hypercholesterolemia.

Molecular biology and genetics of embryonic eyelid development

ABSTRACT The embryology of the eyelid is a complex process that includes interactions between the surface ectoderm and mesenchymal tissues. In the mouse and human, the eyelids form and fuse before birth; they open prenatally in the human and postnatally in the mouse. In the mouse, cell migration is stimulated by different growth factors such as FGF10, TGF-α, Activin B, and HB-EGF. These growth factors modulate downstream BMP4 signaling, the ERK cascade, and JNK/c-JUN. Several mechanisms, such as the Wnt/β-catenin signaling pathway, may inhibit and regulate eyelid fusion. Eyelid opening, on the other hand, is driven by the BMP/Smad signaling system. Several human genetic disorders result from dysregulation of the above molecular pathways.

Eyebrow Position Following Upper Blepharoplasty

Abstract Purpose: To evaluate the effect of upper blepharoplasty on eyebrow height, accounting for ocular dominance, fat excision, change in MRD1, and degree of dermatochalasis. Methods: Retrospective review of patients undergoing upper blepharoplasty between January 2013 and July 2014. Patients with a prior history of ocular trauma, disease, and surgery were excluded. Digital photographs were analyzed using NIH ImageJ software to measure pre and postoperative eyebrow height at the medial, central, and lateral positions, MRD1, and dermatochalasis. Univariable comparisons of brow height and MRD1 were performed. A multivariate analysis was used to assess for the effect of percentage change in MRD1 and dermatochalasis and of ocular dominance and fat excision in mean percentage change of eyebrow height. Results: Charts of 19 patients were reviewed. Mean age was 73.2 years (SD = 8.86). There were 9 male (47.4%) and 10 female (52.6%) patients. There were 11 right eye dominant (57.9%) and 8 left eye dominant (42.1%) patients. Then 13 patients (68.4%) underwent fat removal. A univariable comparison found insufficient evidence to suggest a significant change from 0 postoperatively in brow height at all positions. A multivariable comparison found insufficient evidence to suggest MRD1, ocular dominance, or dermatochalasis were significantly associated with mean percentage change in brow height at all positions with or without fat excision. Conclusion: Upper blepharoplasty does not change eyebrow height at the medial, central, or lateral positions, after accounting for any impact of ocular dominance, fat excision, change in MRD1, or degree of dermatochalasis.

Dimensions and anatomic variations of the orbicularis oculi muscle in nonpreserved, fresh-frozen human cadavers.

Purpose: To determine average dimensions of the orbicularis oculi muscle (OOM) from the orbital rim and to investigate polymorphic variations through anatomical dissection of nonpreserved, fresh-frozen human cadavers. Methods: The OOM was exposed using sharp and blunt dissection until its distal borders were identified. A metric ruler was used to measure the superior (S line), inferior (I line), and lateral (L line) dimensions of the OOM from the orbital rim. Data collection included age, gender, and race. Data were analyzed using 2-sample t tests, paired t tests, and mixed effect models. A p-value of ⩽0.05 was considered statistically significant. Results: A total of 40 hemifaces of 20 cadavers were dissected. All specimens were Caucasian. Ten specimens were men. Average age was 73.9 years (56–92 years). The overall S line was 1.4 cm (95% confidence interval [CI], 1.23–1.57), the I line was 1.2 cm (95% CI, 1.00–1.36), and the L line was 2.5 cm (95% CI, 2.27–2.68). Men had significantly larger average T, L, and S line values than women (p = 0.003, 0.005, 0.008, respectively). I lines did not differ significantly between genders (p = 0.28). Conclusions: In senescent Caucasians, the OOM extends approximately 1.4 cm superior, 1.2 cm inferior, and 2.5 cm lateral to the orbital rim. The muscle extends significantly further superiorly and laterally in Caucasian men than in women. Knowledge of the extent of the OOM should improve the understanding and the treatment of conditions affecting this region.

Indocyanine green-guided sentinel lymph node biopsy for periocular tumors.

Purpose: To compare the accuracy of indocyanine green (ICG)–guided sentinel lymph node biopsy to sentinel lymph node biopsy performed with technetium-99m in eyelid and in conjunctival malignancies. Methods: Review of a consecutive series of adult patients undergoing sentinel lymph node biopsy for eyelid and conjunctival malignancies between 2009 and 2013. Only patients undergoing both ICG-guided and technetium-99m–guided sentinel lymph node biopsies were included. Results: Five patients were identified: 3 women and 2 men. Four had conjunctival melanoma and 1 had eyelid melanoma. ICG aided in localization and confirmation of the sentinel nodes identified by technetium-99m, and all sentinel lymph nodes identified by technetium-99m were identified by ICG. All patients who underwent both sentinel lymph node modalities had negative lymph node biopsies for micrometastasis, but metastatic disease eventually developed in 1 patient. No safety concerns were identified with the use of ICG in the ocular adnexal region. Conclusions: For certain periocular malignancies, ICG-guided sentinel lymph node biopsy safely identifies sentinel lymph nodes intraoperatively possibly to a similar extent compared with technetium-99m–guided methods.

Frontalis Muscle Asymmetry and Lateral Landmarks.

Purpose: To investigate frontalis muscle asymmetry and characterize its lateral interdigitation with the orbicularis oculi muscle. Methods: After making a mid-coronal incision and bluntly dissecting to the orbital rim, the frontalis muscle was exposed, marked, and photographed. The right and left muscle bellies were analyzed and compared in both pixels and cm2 ratios generated with NIH ImageJ software. A ratio of ≥1.5 was considered significantly asymmetric. The lateral interdigitation of the frontalis and orbicularis oculi muscles was measured from the supraorbital notch with a metric ruler. Data were analyzed using 2-sample t tests, paired t tests, log scales, and nonparametric tests were performed for sensitivity analyses. A p value of ⩽0.05 was considered statistically significant. Results: Fifty-eight hemifaces of 29 Caucasian cadavers were studied for muscle belly asymmetry. Thirty-six hemifaces of 18 Caucasian cadavers (9 males) were dissected for lateral landmarks and average age of these specimens was 73 years (range: 35–91 years). Significant asymmetry in muscle belly area was found in 6/29 (20%) specimens, with the right muscle belly larger in all 6 specimens. On average, the right muscle belly area was 1.23 times that of the left (p = <0.001). The average frontalis-orbicularis interdigitation occurred 3.4 cm lateral to the supraorbital notch. Conclusions: Significant frontalis muscle belly asymmetry exists in 20% of Caucasians cadavers. The right muscle belly was larger on average and in all cases of significant asymmetry. The frontalis muscle interdigitates with the orbicularis oculi on average 3.4 cm lateral to the supraorbital notch.

Globe Loss From Intraocular Invasion of Miragel Scleral Buckle Components

Purpose: To describe intraocular invasion of MIRAgel scleral buckles requiring evisceration. Methods: This is an Institutional Review Board-approved retrospective consecutive case series of eyes requiring evisceration secondary to intraocular intrusion of MIRAgel implants performed at the Cole Eye Institute from 2000 to 2014. Charts were reviewed for age at surgery, gender, laterality, time between MIRAgel placement and evisceration, preoperative examination and imaging results, intraoperative findings, postoperative complications, and duration of follow up. Results: Five eyes of 5 patients underwent evisceration due to a blind, painful eye secondary to MIRAgel expansion. The mean time between MIRAgel placement and evisceration was 21 years (range: 17–30 years). Preoperative ultrasound identified intraocular MIRAgel in 3 of 5 cases; however, intraocular MIRAgel was identified during surgery in all 5 cases. A transocular-approach orbitotomy was performed at the time of evisceration in an effort to remove the MIRAgel. Postoperative complications included ptosis and inability to retain an ocular prosthesis. No cases of orbital implant extrusion occurred. Conclusion: Scleral invasion and intraocular penetration of MIRAgel may occur decades after placement. This may result in a blind, painful eye requiring evisceration and orbitotomy to remove residual material. Suspicion of intraocular penetration of implant should be high in blind, painful eyes. Surgical removal can be difficult due to MIRAgel fragmentation. Conjunctival insufficiency may result in the need for further surgery after evisceration.