Long-term efficacy and safety of botulinum toxin A injections to treat blepharospasm and hemifacial spasm.

BACKGROUND: To evaluate efficacy and safety of botulinum toxin A injections after more than 10 consecutive years of treatment for benign essential blepharospasm and hemifacial spasm. DESIGN: Retrospective chart review at university-affiliated hospital. PARTICIPANTS: Study consisted of 64 patients treated with botulinum toxin A injections between October 2005 and May 2006. METHODS: Inclusion criteria included patients treated with at least one annual botulinum toxin-A injection for more than 10 consecutive years. Data collected included diagnoses and patient characteristics, injection dates, doses administered at each visit, response scores, duration of effect, and adverse events. MAIN OUTCOME MEASURES: Included changes in doses, response scores, duration of effects, and adverse events between the first and last botulinum toxin A injections. RESULTS: Thirty-two of 64 patients (mean age at first injection, 57.2 ± 12.4 years; 25 women) met the inclusion criteria. The mean duration of follow up was 14.1 ± 3.1 years (range 10-20 years; mean total visits 44.4 ± 19). A higher mean injection dose per visit was administered during the last year compared with the first year (26.8 ± 10.3 vs. 22.5 ± 7.5 units, respectively) (P = 0.003). The mean durations of effect during the first and last years were 12.4 ± 7.1 and 14.6 ± 7.0 weeks, respectively (P = 0.076). There were no significant differences between genders or between benign essential blepharospasm and hemifacial spasm subgroups. The most common adverse events were ptosis, lagophthalmos and dry eye. CONCLUSIONS: Botulinum toxin A is an effective, safe, long-term treatment for patients with benign essential blepharospasm and hemifacial spasm. Sustained treatment efficacy required higher doses; however, fewer adverse reactions developed.

Intraoperative predictability of temporal artery biopsy results.

PURPOSE: To determine whether inflammatory/granulomatous changes of giant cell arteritis can be grossly identified by the surgeon during temporal artery biopsy to allow institution or discontinuation of steroid therapy without delay. METHODS: The records of 113 consecutive patients operated on by the same surgeon (R.C.K.) between November 2002 and June 2007 were reviewed. Demographic characteristics, date and site of the surgeries, surgical complications, surgeon's intraoperative presumption about the outcome (based on his assessment of the thickness, nodularity, tortuosity, and color/pallor of the arterial wall, blood flow, and the lumen size), and histopathologic biopsy results were collected from the chart review. The main outcome measure was the correspondence of the surgeon's intraoperative impression expressed in the operative note to the ultimate histopathologic diagnosis. RESULTS: After exclusion of 5 cases with incomplete data, the study consisted of 108 patients (78 females and 30 males) with an average age of 72.4 +/- 11.4 years (range, 45-93 years). There were no complications during or after surgery. The surgeon's comment was "grossly positive" in 17 cases, and 15 of these cases were pathologically positive. For all of the cases that the surgeon's impression was negative, the pathology was negative as well (100% negative predictive value). Overall, the surgeon's intraoperative comments had a 100% sensitivity to detect giant cell arteritis. The specificity and accuracy values were 97.9% and 98.2%, respectively. CONCLUSION: The surgeon's intraoperative evaluation of the temporal artery may be extremely valuable in predicting the biopsy result and may allow the surgeon to determine whether steroids could be discontinued in patients in whom the clinical history is not highly suggestive of giant cell arteritis.

Surgical management of recurrent localized eyelid amyloidosis.

Amyloidosis is a disease of unknown origin characterized by the widespread or localized deposition of amorphous hyaline material, amyloid, in various body tissues, including the ocular and orbital structures. Primary advanced localized amyloidosis involving the eyelid and conjunctiva is a rare cause of diffuse eyelid thickening. Management of these cases is often unsatisfactory because of the diffuse deposition of amyloid tissue and the increased risk of intraoperative hemorrhage, due to the fragile vessels encountered in amyloidosis. The possibility of recurrence also presents challenging treatment dilemmas. Therapeutic options include en bloc removal of the involved eyelid tissue, radiation therapy or, in advanced cases, no treatment. We describe an effective surgical approach that offers a safe, effective, and, most notably, repeatable procedure for the treatment of advanced ocular adnexal amyloidosis.

Sclerosing idiopathic orbital inflammation.

A 5-year-old girl referred for orbital cellulitis was found to have a right orbital mass. Computed tomography revealed a mass occupying the inferotemporal orbit, extending into the maxillary sinus. Biopsy yielded a diagnosis of sclerosing idiopathic orbital inflammation. She was successfully treated with prednisone.

Isolated medial orbital wall fractures with medial rectus muscle incarceration.

PURPOSE: To retrospectively review and analyze cases of isolated medial orbital wall fractures with medial rectus muscle incarceration presenting to a tertiary ophthalmic plastic surgery practice from 1997 to 2005. METHODS: Retrospective chart review and literature review. RESULTS: Nine cases of isolated medial wall fracture with medial rectus muscle incarceration are presented. The most frequently encountered clinical feature was adduction deficit on the affected side. Extraocular motility improved in all patients who underwent surgery, and mean postoperative enophthalmos was minimal. CONCLUSIONS: Isolated medial orbital wall fractures with medial rectus muscle incarceration are rare. Ocular motility abnormalities were the only indication of underlying fracture in the majority of our cases. Clinicians should be alerted to the anticipated presentation of medial wall fractures with incarceration of the medial rectus muscle, including the possibility of a "white eye" and normal abduction of the traumatized eye.

A case of primary adenocarcinoma of the lacrimal sac.

PURPOSE: To present a rare case of adenocarcinoma arising from the lacrimal sac. DESIGN: Interventional case report. METHODS: Report of a new case of primary lacrimal sac adenocarcinoma. RESULTS: The clinical presentation, workup, surgical treatment and pathology of a case of adenocarcinoma of the lacrimal sac are described. CONCLUSION: Although very rare, these tumors should be in the differential diagnosis of any lacrimal sac mass, especially those presenting above the medial canthal tendon. Other common features include expressible blood from the punctum, lack of pain, and a history of chronic epiphora or intermittent dacryocystitis. Local destruction and metastatic spread can be prevented by a high index of suspicion and early intervention.

Unilateral frontalis sling for the surgical correction of unilateral poor-function ptosis.

PURPOSE: To evaluate the functional and cosmetic results after frontalis sling repair for unilateral ptosis associated with either poor levator function or synkinesis. METHODS: Preoperative and postoperative photographs and records of 127 patients who underwent unilateral frontalis sling ptosis repair were retrospectively reviewed. An eyelid crease incision was used in all cases, with suturing of the sling material directly to tarsus. RESULTS: Preoperative diagnosis for all patients was either unilateral poor-function blepharoptosis or ptosis associated with levator synkinesis. Underlying causes included 75 congenital, 13 posttraumatic, 11 congenital "jaw-winking," 10 cranial nerve III palsies, 9 myasthenia gravis, 5 chronic progressive external ophthalmoplegia, and 4 congenital "double-elevator" palsies. There was a mean follow-up of 11.6 months. Twenty-eight eyelids required reoperation: 11 for undercorrection, 6 for overcorrection with keratopathy, 2 for upper eyelid crease revision, 7 for correction of poor contour, 1 for a broken sling, and 1 for removal of an infected exposed polytetraflouroethylene sling. Lagophthalmos of greater than 2 mm was noted in 18 patients, 5 of whom had persistent keratopathy requiring reoperation. No other complications were reported, except for 1 suture granuloma. Good to excellent final postoperative eyelid height was achieved in 121 patients (95%) after all surgeries and with conscious recruitment of the frontalis muscle. A large majority of patients and/or parents expressed satisfaction with the final cosmetic result and were not bothered by any asymmetric lagophthalmos in downgaze or lack of a synchronous blink. However, 19 of 25 amblyopic patients were less satisfied with passive eyelid height as they failed to recruit the ipsilateral frontalis muscle to activate the sling during binocular viewing. In 17 of these 19 patients, good to excellent eyelid height could be achieved with conscious active brow elevation. CONCLUSIONS: Unilateral sling provides good to excellent functional and cosmetic results in unilateral poor-function ptosis. However, patients with amblyopia usually require conscious effort to activate the frontalis muscle to achieve satisfactory eyelid height.

Bowen disease of the eyelid successfully treated with imiquimod.

We present a case of Bowen disease affecting the eyelid that was successfully treated with imiquimod. The clinical presentation, histopathology, and treatment of this case are presented. The tumor was treated with imiquimod for 3 months, leading to complete resolution of the disease both clinically and histopathologically. There was no evidence of recurrence 5 months after treatment cessation. Imiquimod was safely used on the eyelid in this case and may avoid extensive periocular tumor excision and reconstruction in Bowen disease.

Dacryocystoceles in the aftermath of Stevens-Johnson syndrome.

Although Stevens-Johnson syndrome has been reported to result in canalicular and nasolacrimal duct obstruction, reports of dacryocystocele formation are lacking. We describe the occurrence and management of bilateral dacryocystoceles related to Stevens-Johnson syndrome. A 45-year-old man, recovered from Stevens-Johnson syndrome, presented with bilateral medial canthal masses and intermittent overlying facial cellulitis. Examination revealed severe keratitis sicca with extensive corneal scarring, cicatricial occlusion of all puncta, and palpable medial canthal masses bilaterally. Computed tomography demonstrated bilateral cystic masses consistent with dacryocystoceles. Bilateral lacrimal sac/cyst excision was attempted, but the left cyst recurred within 6 months after surgery. A dacryocystorhinostomy was then performed, effectively marsupializing the cyst. The puncta were not canalized and stents were not placed. The masses have not recurred with 2 years of follow-up. Dacryocystoceles may arise in the setting of Stevens-Johnson syndrome. Successful management may be achieved with marsupialization to the nasal cavity (dacryocystorhinostomy).

Effect of unilateral blepharoptosis repair on contralateral eyelid position.

PURPOSE: This study evaluates the effect of unilateral blepharoptosis repair on contralateral eyelid position and assesses the relation between preoperative eyelid height interdependence, consistent with Hering law, and surgical outcome. METHODS: The medical records of 54 patients (21 men, 33 women; mean age, 65 years) who underwent external levator advancement for unilateral aponeurotic blepharoptosis were reviewed for preoperative and postoperative margin reflex distance (MRD) of the nonoperated eye. To assess the relation between preoperative Hering dependence (mechanical elevation of the ptotic eyelid causing a decrease in contralateral eyelid height) and postoperative eyelid position, the change in MRD of the nonoperated eye was compared between subjects who on preoperative evaluation did (n=18) and did not (n=36) demonstrate eyelid height interdependence, using the 2-sample t test. RESULTS: After unilateral blepharoptosis repair, the mean (+/- SD) change in contralateral MRD was -0.2 +/- 0.8 mm. There was no significant difference in contralateral MRD change in subjects with and without preoperative Hering dependence (-0.3 +/- 0.8 mm versus -0.2 +/- 0.9 mm, respectively, p=0.78). Seventeen percent (9 of 54) of patients had a contralateral MRD decrease of more than 1 mm. Three patients (5.6%) required contralateral blepharoptosis repair within 1 year of initial surgery. CONCLUSIONS: After levator advancement for unilateral blepharoptosis, roughly 17% of patients will have a decrease in contralateral eyelid height of more than 1 mm, with 5% of patients requiring surgical repair during the first postoperative year. The degree of change in contralateral eyelid height cannot be reliably predicted by preoperative assessment of Hering dependence.

Primary Nocardia brasiliensis of the eyelid.

PURPOSE: To report a rare case of lymphocutaneous Nocardia brasiliensis originating in the eyelid. DESIGN: Observational case report. METHODS: The clinical presentation, workup, and treatment of a case of lymphocutaneous Nocardia brasiliensis originating in the eyelid are presented. RESULTS: The patient presented with a preseptal cellulitis from an abrasion of the eyelid that progressed to submandibular lymph node suppuration. Culture was performed, and a diagnosis of lymphocutaneous Nocardia brasiliensis was made. CONCLUSION: Nocardia brasiliensis may cause a lymphocutaneous infection of the face and must be considered in the differential diagnosis of preseptal cellulitis.

Enucleation versus preservation of blind eyes following plaque radiotherapy for choroidal melanoma.

BACKGROUND: Currently available information about patients with posterior uveal melanoma treated by plaque radiotherapy is insufficient to determine what to do about eyes that become blind as a consequence of the tumour and its treatment. Should they be enucleated, or is ocular preservation just as good in terms of survival? METHODS: We performed a retrospective survival analysis of secondary enucleation versus ocular preservation in patients with a posterior uveal melanoma treated by plaque radiotherapy whose irradiated eye became completely blind following that treatment. Of the 79 patients who fulfilled defined inclusion criteria, 25 underwent secondary enucleation of the blind eye, and 54 retained their irradiated blind eye. RESULTS: Most of the baseline demographic and tumour-related variables evaluated were similarly distributed between the subgroups. The 5-year, 10-year and 15-year all-cause death rates in the secondary enucleation subgroup were 24.7%, 51.5% and 52.0% respectively, and those in the ocular preservation subgroup were 7.4%, 32.9% and 48.1% respectively. In spite of the apparent slight difference between the curves, the difference was not statistically significant (p = 0.41, Mantel-Haenszel test). INTERPRETATION: Although a retrospective study of this type has several limitations, our results suggest that secondary enucleation is not likely to substantially improve survival of patients whose irradiated eye becomes totally blind following plaque radiotherapy for choroidal or ciliochoroidal melanoma.

Osteogenic sarcoma after orbital radiation rhabdomyosarcoma.

PURPOSE: We describe the occurrence of maxillary and orbital osteogenic sarcoma in a child after treatment of contralateral orbital rhabdomyosarcoma with external beam radiation and chemotherapy. DESIGN: Interventional case report. INTERVENTION: Treatment of a maxillary and orbital rhabdomyosarcoma with surgical resection, systemic chemotherapy, and local radiation. MAIN OUTCOME MEASURES: Occurrence and histology of secondary malignancy after orbital radiation. RESULTS: An eleven year-old male presented for evaluation of left facial swelling, occurring ten years after successful treatment of a right orbital embryonal rhabdomyosarcoma with chemotherapy and 5040 cGY of external beam radiation. Computerized tomography demonstrated a mass involving the left maxillary sinus and orbital floor. Biopsy demonstrated osteogenic sarcoma. Despite attempted total excision with radical maxillectomy, resection margins were found to have microscopic extension of the tumor. Postoperatively he was treated with systemic chemotherapy and local radiation. Eight months postoperatively he remains alive despite local progression. CONCLUSIONS: Osteogenic sarcoma can occur as a secondary malignancy years after the successful treatment of orbital rhabdomyosarcoma with external beam radiation and chemotherapy. After orbital radiation, subjects should undergo routine lifelong examinations.

Outcome and influencing factors of external levator palpebrae superioris aponeurosis advancement for blepharoptosis.

PURPOSE: To evaluate, in patients with acquired good-function blepharoptosis, levator advancement success and surgical failure risk factors. METHODS: This retrospective, case-cohort study was university based. An estimated 828 patients underwent levator advancement for acquired good-function blepharoptosis between January 1, 1990, and December 31, 1999. Seventy-two patients underwent reoperation during the first postoperative year. Of 125 randomly selected patients not undergoing reoperation, 106 met the desired outcome criteria: postoperative margin reflex distance (MRD) >/=2.0 mm and

Solitary fibrous tumor of the orbit: is it rare? Report of a case series and review of the literature.

PURPOSE: The real incidence of solitary fibrous tumor (SFT) of the orbit is unknown, but it seems that since it was first described in 1994, orbital SFT has been increasingly recognized. We believe that the orbital SFT is a relatively common tumor and that it should be considered in the differential diagnosis of any orbital tumor. DESIGN: Interventional case series. PARTICIPANTS: Four new cases of orbital SFT. METHODS: Four patients affected by solitary fibrous tumor of the orbit are described. One patient experienced a recurrent SFT shortly after initial surgical excision performed elsewhere. Thirty-eight cases have been reported in the literature in 7 years. RESULTS: The number of orbital SFTs reported has been increasing, reaching an average of more than five tumors reported per year. Since the first orbital SFT was described in 1994, 37 cases have been reported in the literature. We add four new cases in our series, including a recurrent tumor. A total of 42 cases have now been described, eight with recurrences. Malignant transformation occurred in one case. CONCLUSIONS: We believe that before 1994, the diagnosis orbital SFT was confused with other benign orbital tumors, such as fibrous histiocytoma and hemangiopericytoma because of a lack of use of immunohistochemical techniques. This entity should now be considered relatively common and should be included in the differential diagnosis of orbital tumors in any age group. Local recurrences of SFT are possible and usually follow an incomplete initial excision. Recurrent tumors in the orbit have shown the tendency to infiltrate the surrounding tissues and the bone, rendering complete secondary excision more difficult. Recurrent orbital SFT also has the potential for malignant transformation. The treatment of choice of orbital SFT is complete surgical excision and careful follow-up. Considering the more aggressive course followed by recurrent tumor, correct diagnosis and management is essential.

Proptosis after hair pulling.

We describe proptosis due to a subperiosteal orbital hematoma that originated from a subgaleal bleed caused by hair pulling. A 13-year-old boy presented with a one-week history of progressive proptosis after his older sister pulled his hair during an argument. Computerized tomography showed a subgaleal hematoma and a right superior subperiosteal orbital hematoma. Progressive proptosis and compressive optic neuropathy developed, necessitating surgical evacuation with a favorable outcome.Hair pulling can result in a subgaleal bleed with extension to the orbital subperiosteal space. When managing patients with a subgaleal hematoma, this vision-threatening complication should be considered and treated accordingly.

Malignant mesenchymoma of the orbit: case report and review of the literature.

OBJECTIVE: Malignant mesenchymoma are rare tumors of the orbit. From 1961 using English-language literature, we present the sixth such case and the first case with three malignant components. DESIGN: Interventional case report. METHODS: The clinical presentation, workup, surgical treatment, and pathology of a case of malignant mesenchymoma of the orbit are presented. RESULTS: Although very rare, these tumors should be in the differential diagnosis of any tumor of the orbit. The prognosis is unknown because of the lack of follow-up of reported patients. CONCLUSIONS: This malignant orbital mesenchymoma, an entity not accepted by all pathologists, was more complex than cases previously reported in the English literature in demonstrating rhabdomyosarcomatous, chondrosarcomatous, and osteogenic differentiation.

Monitored anesthesia care for enucleations and eviscerations.

OBJECTIVE: To report the technique and success of using monitored anesthesia care instead of general anesthesia for ocular enucleation and evisceration surgeries. DESIGN: Retrospective, noncomparative interventional case series. PARTICIPANTS: Twelve enucleated patients (Soparkar and Patrinely) and 146 eviscerated patients (Kulwin and Kersten). METHODS: Surgical logs of two oculoplastic practices were reviewed searching for cases of ocular enucleations and eviscerations performed under monitored anesthesia care between 1990 and 2001. Identified hospital and clinic charts were then reviewed. MAIN OUTCOME MEASURES: Monitored anesthesia care was deemed successful if (1) there were hemodynamic stability and complete analgesia intraoperatively; (2) there was absence of any chart documentation regarding patient or family psychological distress over the anesthesia method used; and (3) patients were discharged from the hospital without the need for observation or treatment > or = 23 hours. RESULTS: Between 1990 and 2001, 146 eviscerations were performed under local anesthesia with monitored anesthesia care by two surgeons (RCK, DRK) as their routine practice pattern. In 1996, Drs. Soparkar and Patrinely began performing enucleations under monitored anesthesia care in selected cases, and from 1996 to 2001, these surgeons enucleated 12 patients under monitored anesthesia care. Four of the enucleated patients requested surgery without general anesthesia. The remaining eight patients had been refused surgery by at least one other specialist because of the patient's perceived high medical risk for complications under general anesthesia. In all 158 patients, the procedures were deemed successful by the preceding criteria. CONCLUSIONS: This four-surgeon case series reports the successful use of local anesthesia with monitored care for ocular enucleation and evisceration procedures, offering several potential advantages over the traditional use of general anesthesia.