James A. Garrity

Orbital lymphoma: radiotherapy outcome and complications

BACKGROUND AND PURPOSE Orbital non-Hodgkins lymphomas (NHL) have traditionally been treated with radiation. Forty-eight patients presenting with orbital NHL were treated with radiation and were evaluated for local control, overall survival, cause-specific survival, and complications. MATERIALS AND METHODS Forty-five patients had low-grade and 3 patients had intermediate-grade histologic findings. Orbit-only disease occurred in 22 patients, the conjunctiva in 16, both in five, and lacrimal gland only in five. Patient age ranged from 35 to 94 years (median, 68). Ann Arbor stages were cIEA (34), cIIEA (six), cIIIEA (two), and cIVEA (six). Radiation doses ranged between 15 and 53.8 Gy (median, 27.5 Gy). RESULTS Follow-up ranged from 0.14 to 18.23 years (median, 5.35). Median overall survival and cause-specific survival were 6.5 and 15.5 years, respectively. Patients with clinical stage I or II disease had significantly better overall and cause-specific survival than patients with stage III or IV disease. Ten-year relapse-free survival in 41 patients with stage I or II disease was 66%. However, there was continued downward pressure on relapse-free survival out to 18 years. One local failure occurred. Twenty-five patients sustained acute complications. There were 17 minor and four major late complications. All major late complications occurred with doses more than 35 Gy. CONCLUSIONS Excellent local control with radiation doses ranging from 15 to 30 Gy is achieved. Patients with stage I or II disease have better overall and cause-specific survival than patients with stage III or IV disease. Late relapse occurs in sites other than the treated orbit, even in patients with early-stage disease. Doses 35 Gy or higher result in significant late complications and are therefore not indicated for patients with low-grade tumors.

Idiopathic Intracranial Hypertension

OBJECTIVE This review was undertaken to summarize the significant progress that has been made in the epidemiology of idiopathic intracranial hypertension (IIH), the changing nosology, and the diagnostic criteria and therapeutic strategy. DESIGN We reviewed the pertinent literature on IIH published in major English-language journals for the last 20 years. MATERIAL AND METHODS The diagnosis, epidemiology, pathophysiology, complications, and treatment of IIH are described. RESULTS IIH occurs at an average annual incidence rate per 100,000 persons of 1 to 2 for the total population and 19 to 21 in obese females of the reproductive age-group. IIH is a recognized cause of reversible vision loss in adult obese women. The diagnosis of IIH requires a documented elevation of intracranial pressure, normal cerebrospinal fluid (CSF) composition, and normal findings on neuroimaging studies. At least two primary mechanisms for the development of increased CSF pressure in IIH have been postulated and supported by experimental data: vasogenic extracellular brain edema and a low conductance of CSF outflow at the arachnoid villi. This condition may be secondarily exacerbated by compression of intracranial venous sinuses by the increased intracranial pressure, resulting in a further reduction of flow across the arachnoid villi. The therapeutic strategy is determined by visual status, which needs close monitoring. CONCLUSION Although medical measures to lower the intracranial hypertension are successful in the majority of patients, surgical therapy should not be delayed in those with significant or progressive vision loss. Based on recent advances, a practical approach has been described for the diagnosis, early detection of visual impairment, and targeting therapy to preserve the vision or to reverse recent loss of vision in patients with IIH.

The frequency of perioperative vision loss

The frequency of perioperative vision loss, especially for spinal surgery, has been increasing recently. We undertook a retrospective study to determine the frequency of this outcome in a large surgical population receiving general or central neuraxis regional anesthesia for noncardiac procedures from 1986 to 1998. Specific criteria were used to separate cases in which the surgical procedure likely directly contributed to the vision loss. Vision loss was present if any part of the visual field was affected. Initial database screening found 405 cases of new-onset vision loss or visual changes in 410,189 patients who underwent 501,342 anesthetics and who survived at least 30 days after their final procedures. Two hundred sixteen of these patients regained full vision or acuity within 30 days. Of the 189 patients who developed vision deficits for longer than 30 days, 185 underwent ophthalmologic or neurologic procedures in which ocular or cerebral tissues were surgically damaged or resected. The remaining 4 patients (1 per 125,234 overall; 0.0008%) developed prolonged vision loss without direct surgical trauma to optic or cerebral tissues. In this large study population of noncardiac surgical patients, including those who underwent spinal surgical procedures, the frequency of perioperative vision loss persisting for longer than 30 days was very small.

Orbital Inflammation With IgG4-Positive Plasma Cells: Manifestation of IgG4 Systemic Disease

OBJECTIVE To describe clinical, radiographic, and morphologic findings in patients with IgG4-positive cells present on orbital biopsy specimens. DESIGN Retrospective review (from January 1, 1993, through December 31, 2006) of patients with orbital biopsy specimens that excluded lymphoma; comparison of patients with and without IgG4-positive cells on immunostaining. RESULTS Of 21 patients, 11 had increased IgG4-positive cells (defined as >10 cells on biopsy). Symptoms included eyelid or periocular swelling (8 patients) or proptosis (3 patients), with bilateral involvement in 6 patients. Computed tomographic imaging displayed lacrimal gland mass in 10 patients; 6 patients had lesions in other organs. Two patients had increased serum IgG4 levels. In 10 patients without IgG4-positive cells (≤10 cells on biopsy), 6 had proptosis, 1 had eyelid swelling, 2 had eyelid mass, and 1 had diplopia, all unilateral. None had systemic symptoms. Patients with IgG4-positive cells had longer symptom duration, and their biopsy specimens showed more background fibrosis, lymphoid hyperplasia, plasma cells, and eosinophils. CONCLUSIONS The clinical appearance, high incidence of bilateral disease, association with lesions in other organs, and increased IgG4 serum levels in some patients-with an increased number of IgG4-positive cells in the biopsy specimen, which shows more background fibrosis, lymphoid hyperplasia, plasma cells, and eosinophils-indicate that these patients have an orbital manifestation of IgG4-associated systemic disease.

Stereotactic radiosurgery and particulate embolization for cavernous sinus dural arteriovenous fistulae.

OBJECTIVE To evaluate the safety and efficacy of stereotactic radiosurgery, either with or without transarterial embolization, in the treatment of patients with dural arteriovenous fistulae (DAVFs) of the cavernous sinus. METHODS We reviewed the findings, from a prospectively established database, for 20 patients with cavernous sinus DAVFs who were treated with either radiosurgery alone (n = 7) or radiosurgery and transarterial embolization (n = 13) in a 7-year period. The median follow-up period after radiosurgery was 36 months (range, 4-59 mo). RESULTS Nineteen of 20 patients (95%) experienced improvement of their clinical symptoms. Fourteen of 15 patients (93%) experienced either total (n = 13) or nearly total (n = 1) obliteration of their DAVFs, as documented by angiography performed a median of 12 months after radiosurgery. No patient experienced a recurrence of symptoms after angiography showed DAVF obliteration. Two patients developed new neurological deficits after embolization procedures. One patient exhibited temporary aphasia secondary to a venous infarction; another patient exhibited permanent VIth cranial nerve weakness related to acute cavernous sinus thrombosis. Two patients experienced recurrent symptoms and underwent repeat transarterial embolization at 7 and 12 months; both patients achieved clinical and angiographic cures (5 and 10 mo later, respectively). One patient experienced recurrent visual symptoms and underwent transvenous embolization 4 months after radiosurgery. CONCLUSION Staged radiosurgery and transarterial embolization provided both rapid symptom relief and long-term cures for patients with cavernous sinus DAVFs. Radiosurgery alone was effective for patients with DAVFs whose arterial supply was not accessible via a transarterial approach, although the time course of symptom improvement was longer, compared with patients who also underwent embolization.

Diagnostic Value and Limitations of Orbital Biopsy in Wegener's Granutomatosis

Patients with Wegeners granulomatosis may present with ophthalmic involvement Pin either the classic or limited forms. Although the overall clinical picture and serologic testing for antineutrophil cytoplasmic antibody (ANCA) are important, biopsy is often necessary for a definitive diagnosis. Accurate interpretation of these orbital biopsies is essential. The authors reviewed the histopathologic features of 13 orbital biopsies in patients with well-documented Wegeners granulomatosis. The classic triad of vasculitis, tissue necrosis, and granulomatous inflammation was seen in 7 of 13 biopsies (54%). Vasculitis in combination with other microscopic findings was seen in 4 of 13 (31%) biopsies. Giant cells were seldom seen. Two biopsies showed only perivascular infiltrates. Based on this study, it appears that a spectrum of histopathologic features can be seen in orbital biopsies in Wegeners granulomatosis. This study underscores the importance of clinical correlation, the application of the ANCA test, and comparison with extraorbital biopsies, if available, when interpreting orbital biopsies in the diagnosis of Wegeners granulomatosis.

Risk factors for ischemic optic neuropathy after cardiopulmonary bypass: A matched case/control study

Visual loss (acuity or field) secondary to ischemic optic neuropathy (ION) is a rare but devastating complication of cardiac surgery involving cardiopulmonary bypass (CPB). We determined clinical features and risk factors for ION by a retrospective time-matched, case-control study. ION was identified in 17 (0.06%) patients out of 27,915 patients who underwent CPB between January 1, 1976, and December 31, 1994. For each ION patient, two patients who underwent CPB exactly 2 wk before the ION patient were selected as controls. Data were analyzed by using conditional logistic regression with the 1:2 matched-set feature of 17 cases and 34 controls. Two-tailed P values ≤0.05 were considered significant. From bivariate analysis, smaller minimum postoperative hemoglobin concentration (odds ratio [OR] = 1.9, P = 0.047) and the presence of atherosclerotic vascular disease (OR = 7.0, P = 0.026) were found to be independently associated with ION after CPB, as were smaller minimum postoperative hemoglobin concentration (OR = 2.2, P = 0.027) and preoperative angiogram within 48 h of surgery (OR = 7.2, P = 0.042). In ION patients, 13 (76.5%) of 17 experienced a minimum postoperative hemoglobin value of <8.5 g/dL, whereas only 14 (41.2%) of 34 control patients experienced values <8.5 g/dL.

Orbital Exenteration at the Mayo Clinic: 1967–1986

Orbital exenteration was performed in 102 patients at the Mayo Clinic during the 20-year period from 1967 through 1986. The surgical procedure was performed for mucormycosis in one patient and for pain and deformity after a severe facial burn in another; in the remaining 100 patients, exenteration was used to treat a neoplastic disorder. Although 19 different neoplasms were encountered, squamous cell carcinoma, basal cell carcinoma, and melanoma constituted 70% of the total. In 82 patients with no known residual tumor or metastases at operation, the 1-year survival rate was 88.6%, the 5-year rate was 56.8%, and the 5-year rate free of recurrence or metastases was 48.3%. In 18 patients with known residual tumor or metastases at exenteration, 55.0% were alive 1 year postoperatively, and the 5-year survival rate was 25.8%. Unusual findings in this series included two patients with metastatic basal cell carcinoma and one patient with a metastatic thyroid Hürthle cell carcinoma.

Local control and complications after radiation therapy for primary orbital lymphoma: A case for low-dose treatment

Orbital involvement at the time of initial presentation is unusual in non-Hodgkins lymphoma. In an effort to identify potential ways of improving the radiotherapeutic management of this disease, the records of 22 patients were reviewed retrospectively. All had biopsy-proven orbital non-Hodgkins lymphoma, and the minimal, median, and maximal durations of follow-up in surviving patients were 4.8 years, 7.0 years, and 17.4 years, respectively. Permanent local control was achieved in 21 of the 22 patients (96%). Complications were scored according to a grading scheme in which grade 1 was the least significant complication and grade 4 was blindness as a result of radiation therapy. Of the 12 patients who received a radiation dose less than 35 Gy, 6 developed a grade 1 or grade 2 complication. Of the 10 patients treated with greater than or equal to 35 Gy, 6 experienced a complication, 1 of whom had a grade 4 complication resulting in blindness and another who developed a severe keratitis, which was scored as a grade 3 complication resulting in decreased visual acuity. At last follow-up, 10 patients were alive at 4.8 to 17.4 years after completion of radiation therapy, 4 had died of intercurrent disease at 3 months to 10.6 years, and 8 had died of disease at 3 months to 15.8 years. Actuarial survival for the entire group was 75% at 5 years and 48% at 10 years. Survival in patients with Stage I AE disease (lymphoma confined to orbit) at presentation was 87% at 5 years and 50% at 10 years, and survival in patients with Stage II A through Stage IV disease was 36% at 5 years and at 10 years. Primary orbital lymphoma is an indolent disease characterized by prolonged survival after radiation therapy. Excellent local control can be achieved with radiation doses of 20 Gy to 35 Gy. Higher doses may result in an increased risk of complications.

IgG4-positive plasma cells in granulomatosis with polyangiitis (Wegener's): a clinicopathologic and immunohistochemical study on 43 granulomatosis with polyangiitis and 20 control cases ☆

Granulomatosis with polyangiitis (GPA) (Wegeners) may mimic IgG4-related disease (IgG4-RD) on histologic examination of some biopsies, especially those from head and neck sites. IgG4 immunostaining is often performed in this context for differential diagnosis with IgG4-RD. Herein, we report the results of IgG4-positive (IgG4+) cells in 43 cases of GPA including 26 previously published cases as well as the newly added cases from the lung and kidney. We also included 20 control cases without any clinical evidence of GPA or IgG4-RD that consisted of chalazion (n = 8), chronic sinusitis (n = 8), and chronic tonsillitis (n = 4). Forty-three biopsies diagnosed as GPA were from sinonasal mucosa/oral cavity/nasopharynx (n = 14), orbit/periorbital tissue (n = 7), lung/pleura (n = 14), kidney (n = 4), skin (n = 3), and dura (n = 1). Of 43 biopsies, 8 (18.6%) revealed increased IgG4+ cells (>30 per high-power field and >40% in IgG4+/IgG+ ratio) and originated from sinonasal (n = 4) or orbital/periorbital (n = 4) regions. The IgG4+ cells and IgG4+/IgG+ ratio in these cases ranged from 37 to 139 per high-power field and 44% to 83%, respectively. None of the control cases had increased IgG4+ cells. In conclusion, increased IgG4+ cells can be seen in sinonasal or orbital/periorbital biopsies of GPA, which could pose as a pitfall in the diagnosis of IgG4-RD. However, GPA in other organs and controls did not show increased IgG4+ cells when using the above threshold. The biologic or clinical importance of increased IgG4+ cells in GPA cases involving head and neck region is uncertain, and a further study might be warranted to address the potential pathogenic relationship between IgG4-RD and GPA in those cases.