Spontaneous superior ophthalmic vein thrombosis: a rare entity with potentially devastating consequences.

PURPOSE: Spontaneous superior ophthalmic vein thrombosis (SOVT) is a rare entity. We describe three patients with spontaneous ophthalmic vein thrombosis, each with various risk factors. PATIENTS AND METHODS: A retrospective review of three patients with a diagnosis of superior ophthalmic vein thrombosis. Clinical characteristics, radiographic features, management techniques and outcomes are described. RESULTS: All patients presented with unilateral painful proptosis. Two patients had intact light perception, whereas one patient presented with absent light perception. All patients had identifiable risk factors for thrombosis, which included sickle cell trait, hereditary hemorrhagic telangectasia and colon cancer with recurrent deep vein thrombosis. Anticoagulation was initiated in two patients. Resolution of proptosis was seen in all patients, with no recovery of vision in one patient. CONCLUSIONS: Risk factors for spontaneous superior ophthalmic vein thrombosis are multifactorial. MRI and MRV confirm the diagnosis of SOVT. Despite urgent intervention devastating visual loss may occur.

Increased elastin expression in astrocytes of the lamina cribrosa in response to elevated intraocular pressure.

PURPOSE: To determine whether abnormal elastin synthesis in the glaucomatous optic nerve head and lamina cribrosa is due to elevated intraocular pressure (IOP) or secondary to axonal injury, monkeys with elevated IOP and with optic nerve transection were compared. METHODS: Unilateral, chronic elevated IOP was induced in 11 rhesus monkeys by laser scarification of the trabecular meshwork. IOP was monitored weekly and maintained within 25 to 45 mm Hg for 7 to 36 weeks. In 6 monkeys, unilateral, optic nerve transection was performed, and monkeys were killed after 4 weeks. Optic nerve damage was assessed by stereoscopic slit-lamp biomicroscopy and fundus photography and by confocal scanning laser ophthalmoscopy. The eyes were enucleated and processed for immunohistochemistry and in situ hybridization and for electron microscopic immunogold detection of elastin. Axonal loss was evaluated in cross sections of the optic nerve stained with phenylenediamine. RESULTS: Compared with normal contralateral controls, the lamina cribrosa of eyes with elevated IOP exhibited markedly increased elastin and the presence of elastotic aggregates in the extracellular matrix and upregulation of elastin mRNA in the astrocytes. In transected eyes, elastin appeared as fine fibers in the lamina cribrosa, without elastotic aggregates, and without new synthesis or abnormal deposition of elastin. At the transected site, new synthesis of elastin was present in the pia mater but not in astrocytes in the glial scar. CONCLUSIONS: This study demonstrates that abnormal elastin synthesis in experimental glaucomatous optic neuropathy in the monkey is specific to elevated IOP and not secondary to axonal loss. The mechanisms by which elevated IOP induces enhanced elastin synthesis in laminar astrocytes are unknown but differ from those involved in acute axonal injury such as transection, where inflammation and breakdown of the blood-nerve barrier occur.

Canthus-sparing ectropion repair.

PURPOSE: To describe a novel surgical technique for lower eyelid ectropion repair that avoids canthotomy and cantholysis and can be used in combination with external levator repair and/or in combination with blepharoplasty. METHODS: A retrospective analysis of lower eyelid procedures with the use of the canthus-sparing technique between January 1, 1998, and December 31, 1999, was performed. The canthus-sparing approach was used in 198 eyelid procedures for the correction of lower eyelid ectropion. Seventy-four (37.4%) procedures involved the correction of lower eyelid ectropion alone and 25 (12.6%) procedures involved the correction of lower eyelid ectropion during upper eyelid small-incision external levator repair. In these cases, an incision was made lateral to the lateral canthus and a periosteal flap was created at the lateral orbital rim. The inferior crus of the lateral canthal tendon was then attached to this full-thickness elevated periosteum. Twenty (10.1%) procedures involved the correction of ectropion during upper blepharoplasty and 79 (39.9%) procedures involved the correction of ectropion during combined upper eyelid ptosis repair and blepharoplasty. In these cases, the inferior crus of the lateral canthal tendon was attached to a periosteal flap created through the lateral portion of the blepharoplasty incision. RESULTS: The mean age of patients undergoing ectropion repair was 74.3+/-9.3 years (range, 42-93 years). The average duration of symptoms (most commonly tearing and/or ocular irritation) was 20+/-14 months (range, 3-84 months). Recurrences of lower eyelid ectropion or symptoms occurred in 4 (2%) eyelids. The average follow-up interval was 54+/-65 days (range, 3-330 days). CONCLUSIONS: The canthus-sparing approach to ectropion repair promotes a secure adhesion to the lateral orbital wall with minimal violation of normal anatomic structures and relations. It is time-efficient and reduces postoperative morbidity.

Primary and secondary transconjunctival involutional entropion repair.

PURPOSE: Lower eyelid involutional entropion is a significant disorder of the aging population resulting from horizontal eyelid laxity, overriding orbicularis oculi muscle, and attenuation of the lower eyelid retractors. The purpose of this study is to describe the long-term results of transconjunctival entropion repair. DESIGN: Interventional noncomparative case series. PARTICIPANTS: Thirty-six eyelids in 31 patients. METHODS: Charts were reviewed of all transconjunctival entropion repairs, which included myectomy, retractor fixation, and horizontal shortening performed by three oculoplastic surgeons between January 1993 and January 1999. Cases with less than 12 months follow-up were excluded. MAIN OUTCOME MEASURES: Entropion recurrence. RESULTS: Thirty-six lids in 31 patients were followed for mean of 31.5 months (12.5-79). Six of 36 lids (16.7%) had postoperative complications. Recurrent entropion occurred in 3 of 36 lids (8.3%) an average 16.3 months (7-35) after surgery. An average of 6 trichiasis lashes (1-10) occurred in 4 of 36 lids (11.1%) at a mean of 2.25 months (1-4) after surgery. There were no overcorrections. Three of 36 lids (8.3%) required additional surgery. CONCLUSIONS: Entropion recurrence after three-step transconjunctival repair is within the 0% to 30% reported recurrence for other repair techniques but more frequent than reported for a similar transcutaneous procedure. The 8.3% recurrence rate might have resulted from inadequate myectomy, inadequate retractor fixation, cicatricial changes directly related to the transconjunctival incision, or progressive involutional changes. Trichiasis was the most frequent complication. Transconjunctival entropion repair may be slightly less effective than transcutaneous repair.

Microcystic adnexal carcinoma: report of 13 cases and review of the literature.

BACKGROUND: Microcystic adnexal carcinoma (MAC) is a rare tumor of the skin. Clinically it often masquerades as a firm, subcutaneous nodule on the head and neck regions. Microscopically it extends far beyond assessed clinical margins spreading locally in the dermal, subcutaneous, and perineural tissue planes. The local recurrence rate by standard excision is about 50%. Recent preliminary reports indicate more favorable cure rates with Mohs micrographic surgery (MMS). OBJECTIVE: To present our data on 13 cases (12 patients) of MAC treated by MMS. In addition, we reviewed the medical literature to summarize the accumulated experience of MMS treatment in the management of MAC. We also present a case of bilateral MAC of the face and describe a renal transplant recipient on immunosuppressive therapy who developed MAC of the nasal bridge. METHODS: We reviewed and updated our series of MAC cases treated by MMS over the last 9 years. A total of 13 cases of MAC are reviewed. We also searched the literature for MAC treated by MMS with a follow-up of more than 2-years. RESULTS: One patient had bilateral MAC of the nose and cheek. Another patient developed a MAC of the nasal bridge 20 years after renal transplantation. In this patient predisposing factors were radiation for teenage acne and immunosuppression therapy. A total of 13 cases of MAC were treated by MMS with no recurrences, with a mean follow-up of 5.0 years (range 1.1-8.0 years). CONCLUSION: We update the medical literature with 13 MAC cases treated by MMS. To our knowledge there have been 148 cases of MAC reported in the world literature. Including our series, there have been 73 cases of MAC treated with MMS. There were only four treatment failures. Regional and/or distant metastasis from MAC is rare, with only one reported death. Following MMS, the 2-year success rate was 89.7% (35 of 39). The accumulated data continue to confirm that when MAC is discovered early and is readily accessible to excision by MMS and other subspecialty support, a favorable outcome can be expected.

Depressor supercilii muscle: anatomy, histology, and cosmetic implications.

PURPOSE: To describe the gross and microscopic anatomy of the depressor supercilii muscle and to discuss its cosmetic implications. METHODS: The depressor supercilii muscle was studied in detail with the use of gross anatomic dissections carried out on eight sides of four fresh cadaver heads and ten sides of five preserved cadaver heads. Histological analysis was performed on parasagittal sections of one side of a preserved cadaver head. Measurements were taken on cadaver specimens to determine the insertion point of the depressor supercilii muscle on the undersurface of the skin. RESULTS: The depressor supercilii muscle is distinct from the corrugator supercilii muscle and the medial head of the orbital portion of the orbicularis oculi muscle. The depressor supercilii muscle was noted to be superior in orientation and redder in color than the orbicularis oculi muscle. The depressor supercilii muscle arose from the frontal process of the maxilla approximately 1 cm above the medial canthal tendon and appeared to originate from two distinct heads in most specimens, a novel finding. In specimens containing two heads of the depressor supercilii muscle, the angular vessels passed between the two muscle heads. In specimens containing one muscle head, the angular vessels were found anterior to the muscle. The insertion of the depressor supercilii muscle in the dermis lay approximately 13 to 14 mm superior to the medial canthal tendon. CONCLUSIONS: The origin, insertion, and anatomy of the depressor supercilii muscle help it to act as a depressor of the eyebrow. Histologically, the depressor supercilii muscle arises distinctly from bone and has a unique insertion. The depressor supercilii muscle appears to be distinct from the corrugator supercilii and the orbicularis oculi muscles.

Protection of ganglion cells in experimental glaucoma by retinal laser photocoagulation.

OBJECTIVE: To test a hypothesis of photoreceptor involvement in retinal ganglion cell (RGC) death in chronic glaucoma. METHODS: Laser spots were applied to 6 eyes of 3 rhesus monkeys, causing focal destruction of the outer retina, including the photoreceptors. After 3 to 4 weeks, experimental glaucoma was induced in the right eyes of each monkey using argon laser trabecular destruction (ALTD). The intraocular pressures in these eyes were elevated for 3 to 7 months. As a control, 1 additional monkey underwent retinal laser photocoagulation followed by optic nerve transection instead of ALTD. Following enucleation, the retinas were embedded and sectioned for histologic evaluation. RESULTS: There was extensive loss of RGCs in the eyes with ALTD except over the large retinal laser spots, where there was an increased survival of RGCs. The RGC protection was not observed in the monkey that had undergone optic nerve transection. CONCLUSION: Photocoagulation of the outer retina that completely destroys the photoreceptors results in survival of the overlying RGCs in experimental glaucoma in monkey eyes. CLINICAL RELEVANCE: Although this is an experimental model and not a therapeutic option, these results suggest that treatments other than lowering intraocular pressure may be potential therapies for preventing RGC death in glaucomatous eyes. Arch Ophthalmol. 2000;118:1242-1250

The anatomy of midfacial ptosis.

PURPOSE: To investigate the anatomic and histologic changes present in midfacial ptosis. METHODS: Experimental study applying gross anatomic and histologic techniques to formalin-preserved and fresh-frozen cadaver heads with and without midfacial ptosis. High-resolution surface coil magnetic resonance imaging (MRI) was performed to obtain radiologic correlations. RESULTS: The orbitomalar ligament was further characterized by identification of a well-developed lateral component in the sub-superficial musculoaponeurotic plane; abnormalities of this important supporting structure were present in the subcutaneous plane in 8 of 10 specimens with midfacial ptosis. The zygomatic and masseteric cutaneous ligaments also were further characterized on a gross anatomic level, and histologic evidence of these two structures was produced. The subcutaneous components of the zygomatic and masseteric cutaneous ligaments were attenuated or not identifiable in 40% and 30% of specimens with midfacial ptosis, respectively. High-resolution surface coil MRI provided exquisite correlations of midfacial anatomy. CONCLUSIONS: The lateral component of the orbitomalar ligament provides major osteocutaneous midfacial support. Subcutaneous attenuation of the orbitomalar, masseteric cutaneous, and zygomatic ligaments was associated with midfacial ptosis.

Management of moderate-to-severe Marcus-Gunn jaw-winking ptosis.

OBJECTIVE: To report the results of levator excision and frontalis suspension for moderate-to-severe Marcus-Gunn jaw-winking ptosis. DESIGN: A retrospective noncomparative case series. PARTICIPANTS: Twenty-four patients with moderate-to-severe Marcus-Gunn jaw-winking ptosis (21 unilateral and 3 bilateral) were treated surgically between 1978 and 1997 by one surgeon. INTERVENTION: Levator excision either in the involved eyelid or in both eyelids, followed by bilateral frontalis suspension, was performed. MAIN OUTCOME MEASURES: Postoperative improvement of jaw-winking was determined. The surgical results of ptosis surgery were assessed as good, fair, or poor based on habitual upper eyelid heights and symmetry. RESULTS: Postoperative follow-up periods ranged from 6 months to 153 months, with an average of 36.9 months. After levator excision in a total of 27 eyelids exhibiting jaw-winking, 10 eyelids (37.0%) showed complete resolution of jaw-winking, and 13 eyelids (48.2%) showed mild winking (1 mm or less) on the lateral jaw movement only (functionally and cosmetically not a problem). In four eyelids (14.8%), these results were not recorded. In the group of five patients undergoing bilateral frontalis suspension and levator excision only on the involved side, final results were good in two patients (40%) and poor in three (60%). Of the 19 patients who underwent bilateral levator excision, final results were good in 13 (68.4%) and fair in 6 (31.6%). CONCLUSIONS: For moderate-to-severe jaw-winking ptosis, bilateral frontalis suspension after bilateral levator excision generally provided satisfactory correction of both jaw-winking and ptosis.

Small incision external levator repair: technique and early results.

PURPOSE: To describe a new surgical technique and early results of external levator repair performed through a small skin incision. METHODS: A chart review of consecutive patients undergoing small incision external levator repair was conducted. This modified external levator repair was performed through an 8-mm eyelid crease incision. Patients with unilateral or bilateral aponeurogenic blepharoptosis were candidates for the technique. Patients with excessive horizontal upper eyelid laxity and those requiring blepharoplasty in addition to blepharoptosis surgery did not undergo this technique. Patients who underwent previous upper eyelid surgery or concurrent brow surgery were excluded from the review. Preoperative measurements included upper eyelid margin reflex distance, levator function, and degree of dermatochalasis, as well as Goldmann visual field results. Outcome measures included incidence and type of intraoperative complications, postoperative upper eyelid position (including margin reflex distance, eyelid contour, and symmetry), incidence and type of postoperative complications, and revisions or additional necessary surgery. RESULTS: Twenty-eight eyelids of 17 patients met study inclusion criteria. Preoperative margin reflex distance +/- SD averaged 0.8 +/- 0.4 mm. Average length of follow-up was 28 +/- 5 weeks (range, 15 to 52 weeks). No significant intraoperative complications occurred. Postoperative margin reflex distance averaged 3.7 +/- 0.3 mm. Two eyelids were mildly undercorrected, and one demonstrated moderately peaked contour postoperatively. Satisfactory eyelid position and contour were achieved in 25 of 28 treated eyelids. No patient elected reoperation. CONCLUSIONS: Early results demonstrated that small incision levator repair is safe and generally effective. This minimally invasive external levator repair is useful for a carefully selected subset of patients with aponeurogenic blepharoptosis.

Primary orbital Ewing sarcoma in a middle-aged woman.

A 43-year-old woman had unilateral exophthalmos caused by primary orbital Ewing sarcoma. Specialized immunohistochemical stains, primarily MIC-2 (CD99), aided in the diagnosis of Ewing sarcoma. Twenty-two months after radiotherapy and multiagent chemotherapy, the patient remained tumor free. To our knowledge, this is the first reported case of orbital Ewing sarcoma to present in an adult beyond the fourth decade of life.

Ancient schwannoma of the orbit.

The ancient schwannoma is a rare variant of a neurilemoma with a course typical of a slow-growing benign neoplasm. Histologically, it can be confused with a malignant mesenchymal tumor because of increased cellularity, nuclear pleomorphism, and hyperchromatism. Despite the degree of nuclear atypia, mitotic figures are absent. We describe the clinical and histopathologic features of an ancient schwannoma of the orbit.

Traumatic hyphema: outcomes of outpatient management.

OBJECTIVE: This study aimed to compare the outcomes of outpatient and inpatient management of layered hyphema. DESIGN: The charts of all patients with traumatic layered hyphema treated in the Massachusetts Eye and Ear Infirmary Emergency Ward between January 1991 and November 1995 were analyzed retrospectively. Patients with a diagnosis of microscopic hyphema, ruptured globe, or posterior segment injury other than commotio retinae on their initial emergency department visit were excluded. The study patients were compared with an historic control group of patients with hyphema who had been treated at the same institution from July 1986 to February 1989. PARTICIPANTS: A total of 154 patients met the study criteria. These were compared with 119 patients in the historic control group. INTERVENTION: Of the study patients, 5% were admitted on the day of presentation, 95% were treated initially as outpatients, and 4% subsequently were admitted. All of the patients in the historic control group were treated with initial hospital admission. MAIN OUTCOME MEASURES: The rebleed rates of the study and control groups were compared. The final recorded visual acuity and causes of best-corrected visual acuity worse than 20/30 were analyzed for the study group. RESULTS: The rebleed rates of the study group and the historic control group were 4.5% and 5.0%, respectively (P > 0.05). The rebleed rates of the study patients initially treated as outpatients and the historic control group were 3.4% and 5%, respectively (P > 0.05). The rebleed rates of study patients who did not receive aminocaproic acid and the subset of historic control patients who received aminocaproic acid were 3.3% and 4.8%, respectively (P > 0.05). Ninety-six percent of study patients achieved a final best-corrected visual acuity of 20/30 or better. Causes of a final documented visual acuity worse than 20/30 included loss of patient follow-up before resolution of the hyphema, traumatic cataract, macular hole, and macular degeneration. CONCLUSIONS: In the authors' predominantly white patient population, close outpatient follow-up of traumatic hyphemas appears to be safe and effective. Hospitalization for hyphema does not appear to decrease the rate of rebleeding. Decreased vision in the setting of traumatic hyphema generally results from comorbidities not affected by inpatient management.

Monopolar electrosurgical flash fire.

A 78-year-old man underwent ectropion repair with a monopolar electrosurgical unit. A flash fire occurred, resulting in a loss of eyelashes of the left upper and lower eyelids. A retrospective analysis of the case was conducted, including a review of the relevant literature. Although rare, the possibility of a flash fire should be considered when performing surgery with an electrosurgical unit. Minimizing supplemental oxygen and electrosurgical power settings may help to avoid such an incident.

Optic nerve avulsion.

OBJECTIVE: To characterize the presentation, role of diagnostic imaging, and course in patients with optic nerve avulsion. METHODS: A retrospective review of medical records of all 6 patients with optic nerve avulsion who were seen at the Massachusetts Eye and Ear Infirmary, Boston, from January 1, 1991, to July 31, 1995. RESULTS: The initial visual acuity ranged from 20/100 to no light perception. All 6 patients underwent neuroimaging, including computed tomography, magnetic resonance imaging, or both. B-scan ultrasonography was performed on 4 patients, and the condition of 1 patient was evaluated with color Doppler ultrasonography to assess the optic nerve vasculature. In 1 patient, a computed tomographic scan was suggestive of an optic nerve avulsion. Neuroimaging in the other 5 patients, including 2 patients who underwent magnetic resonance imaging, failed to demonstrate an avulsion. During a follow-up period of up to 25 months, 4 patients showed no improvement in visual acuity, 1 patient improved from no light perception to bare light perception, and 1 patient improved from 20/100 to 20/25. CONCLUSIONS: These data suggest that final visual outcome was dependent on initial postinjury visual acuity. Neuroimaging, B-scans, and Doppler ultrasonography were usually not helpful in establishing the presence of optic nerve avulsion, although they may be useful in evaluating comorbid conditions.

Optic nerve decompression for compressive neuropathy secondary to neoplasia.

OBJECTIVE: To evaluate the outcome of extracranial optic nerve decompression in patients with compressive optic neuropathy secondary to intracranial, paranasal sinus, or orbital neoplastic processes. DESIGN: A retrospective chart review and clinical follow-up of patients who underwent optic nerve decompression. SETTING: Tertiary care referral center. PATIENTS: During an 8-year period, 95 extracranial optic nerve decompressions were performed by one of us (M.P.J.) for optic neuropathy resulting from traumatic, inflammatory, infectious, iatrogenic, neoplastic, and idiopathic processes. Thirty patients with compressive optic neuropathy secondary to histopathologically confirmed tumors were identified. INTERVENTION: Optic nerve decompression via external ethmoidectomy approach. RESULTS: Twenty (67%) of 30 patients showed improvement in vision. Improvement in 17 of the 20 patients has been stable. Seven patients (23%) showed no improvement but there was no further worsening of vision after surgery. In 3 patients (10%) vision deteriorated following surgery. CONCLUSION: Extracranial optic nerve decompression may be considered for the preservation or improvement of vision in selected patients with compressive optic neuropathy from neoplasms.

Hydrops and spontaneous perforation in pellucid marginal corneal degeneration.

PURPOSE: Pellucid marginal degeneration of the cornea (1,2) is an idiopathic condition characterized by noninflammatory, nonulcerative thinning of the inferior, peripheral cornea. Occasionally the condition is complicated by acute corneal hydrops (3). We describe a patient with findings suggestive of pellucid marginal corneal degeneration who experienced acute hydrops and spontaneous perforation of the cornea. METHOD: A retrospective analysis of the case was conducted. RESULTS: A 56-year-old woman was seen with decreased vision in the left eye. Clinical findings, including computerized corneal topography, were suggestive of pellucid marginal degeneration. Two weeks later, the patient had hydrops and spontaneous perforation of the left cornea. Medical workup of the patient and histopathologic and microbiologic studies of the corneal specimen revealed no contributory disease process. The patient underwent emergency tectonic lamellar keratoplasty and is doing well after subsequent optical penetrating keratoplasty. CONCLUSION: We believe this is the first report of a case of pellucid marginal degeneration of the cornea complicated by hydrops and perforation.