Dan D. DeAngelis

American Joint Committee on Cancer Classification Predicts Outcome of Patients with Lacrimal Gland Adenoid Cystic Carcinoma

PURPOSE To investigate whether American Joint Committee on Cancer (AJCC) classification at initial diagnosis of lacrimal gland adenoid cystic carcinoma predicts outcome of treatment on local recurrence. DESIGN Retrospective chart review. PARTICIPANTS Consecutive patients with adenoid cystic carcinoma of the lacrimal gland treated at 8 institutions between January 1986 and December 2007. METHODS Clinical records, including pathology reports and imaging studies, were reviewed. MAIN OUTCOME MEASURES AJCC classification, histologic subtype, local recurrence rate, and survival. RESULTS AJCC classification at initial diagnosis was assessable for 53 patients and was as follows: T1N0M0, 7 patients; T2N0M0, 8 patients; T3aN0M0, 14 patients; T3aNxM0, 1 patient; T3aN0M1, 1 patient; T3bN0M0, 13 patients; T3bN0M1, 1 patient; T4aN0M0, 2 patients; T4bN0M0, 4 patients; T4bN0M1, 1 patient; and T4bNXM0, 1 patient. Thirty-eight (72%) of the 53 patients had >T3 tumors at presentation. Of the 38 patients with >T3 tumors, 20 were treated with orbital exenteration and postoperative adjuvant radiotherapy (RT), 6 were treated with orbital exenteration without RT, and 12 were treated with globe-preserving surgery (10 with RT and 2 without RT). Of the 15 patients with <T3 tumors, 6 were treated with globe-preserving surgery and RT, 2 were treated with globe-preserving surgery without RT, 6 were treated with orbital exenteration with bone removal and RT, and 1 was treated with orbital exenteration with bone removal without RT. Only 1 patient with a <T3 tumor, had local recurrence. Among patients with >T3 tumors, the risk of local recurrence (in the orbit or skull base) was higher in patients treated with conservative surgery as opposed to orbital exenteration and RT. Only 4 (20%) of the 20 patients treated with orbital exenteration and RT had local recurrence, compared with 3 (50%) of the 6 patients treated with orbital exenteration without RT and 8 (67%) of the 12 patients treated with globe-preserving surgery. Overall, 17 (45%) of the 38 patients with >T3 tumors and only 1 (7%) of the 15 patients with <T3 tumors died of disease during the study period. CONCLUSIONS In patients with lacrimal gland adenoid cystic carcinoma, AJCC >T3 disease at initial diagnosis correlates with worse outcomes than does AJCC <T3 disease. FINANCIAL DISCLOSURE(S) The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Outcome of lacrimal surgery in older patients

BACKGROUND Performance of lacrimal surgery under neuroleptic (local) anesthesia has greatly facilitated the procedure and decreased the associated morbidity. We reviewed the outcome of lacrimal surgery in older patients to determine whether such surgery can be performed safely in the outpatient setting in this group. METHODS Review of the office and hospital charts and the surgical and anesthetic records of 120 patients (84 women and 36 men) aged 70 to 90 years who underwent lacrimal drainage procedures (dacryocystorhinostomy [DCR], canaliculodacryocystorhinostomy, DCR with insertion of a Jones tube, or a revision endonasal procedure with probing and tube insertion) at a university-affiliated hospital in Toronto in 1996. The interval between surgery and data collection ranged from 10 to 22 months. RESULTS Of the 120 patients 65 were aged 70 to 75 years, 38 were 76 to 80 years, 11 were 81 to 85 years, and 6 were 86 to 90 years. Ninety-six patients had a unilateral procedure, and 24 (22 of whom were aged 70 to 80) had a bilateral procedure. Concomitant conditions, such as hypertension and cardiac disorders, were found in 104 patients (87%). Of the 120 patients 98 (82%) (including all those aged 81 to 90) had local anesthesia, and 22 (18%) had general anesthesia. In one case anesthesia had to be changed from local to general during the procedure because of noncompliance. A total of 112 patients (93%) whose surgery was planned as a day procedure were able to leave the hospital the same day. Three additional patients were admitted to hospital for an overnight stay because of increased bleeding at the time of surgery (one patient) or a history of cardiac problems (two patients). Five patients who had planned overnight stays because of cardiac problems did well during surgery and were discharged the same day, without consequence. None of the patients had to be readmitted at a later date for bleeding or health problems. In 109 patients (91%) the presenting symptom(s) was completely relieved. Overall, 116 patients (97%) had a totally open system with no reflux on syringing. INTERPRETATION The surgical goals and techniques of lacrimal surgery in older patients were not compromised by performing the surgery in the outpatient setting and under neuroleptic anesthesia in most cases.

Adrenergic receptors in the ptotic human eyelid: correlation with phenylephrine testing and surgical success in ptosis repair.

Purpose: To investigate the relationship between adrenergic receptors in Müller muscle and response to phenylephrine testing in patients undergoing ptosis surgery. This study also compares outcomes of Fasanella and Putterman approaches to posterior ptosis repair. Methods: Prospective analysis of 71 patients undergoing posterior ptosis surgery. Eyelid height was measured before and after phenylephrine. Müller muscle was examined for alpha-1D, alpha-2C, beta-1, and beta-2 receptors. Specimens were graded on receptor staining intensity. Patients were seen 1 week and 6 weeks following surgery. Surgical outcomes were scored on a scale of 1 (most favorable) to 3 (least favorable). Results: Adrenergic receptors were found in decreasing order: alpha-1D, beta-1, alpha-2C, and beta-2. Receptor grade significantly predicted eyelid height for alpha-2C receptors (p = .03). Mean outcome scores for 36 Putterman (1.10) and 35 Fasanella (1.27) procedures were not significantly different. Conclusions: Alpha 1D, alpha-2C, and beta-2 receptors are documented within human Müller muscle. Human eyelid elevation response to phenylephrine is inversely related to the amount of alpha-2C receptor staining in Müller muscle. Fasanella and Putterman procedures have equal outcomes, independent of adrenergic receptors.

The role of bacteriologic infection in the etiology of nasolacrimal duct obstruction

BACKGROUND In patients with nasolacrimal duct obstruction, the inflammation and fibrosis may be secondary to coexisting infectious colonization within the lumen of the lacrimal sac. We examined the bacterial flora within the lacrimal sac at the sac-duct junction to explore the possibility of a primary bacteriologic etiology of the inflammatory response. METHODS The study included 114 consecutive patients (132 sides) with epiphora undergoing routine external dacryocystorhinostomy (DCR) at a tertiary care hospital in Toronto between November 1999 and April 2000. A lacrimal sac tissue biopsy specimen was acquired intraoperatively from the inferior posterior lacrimal sac near the sac-duct junction. Microbiologic analysis was carried out to determine the presence and identification of infectious organisms. RESULTS Culture gave positive results in 51 patients (44.7%) (41.7% of specimens). A total of 65 isolates were cultured, of which 78.5% were gram-positive bacteria and 21.5% gram-negative bacteria. Of the gram-positive organisms 76.5% were Staphylococcus sp. The presence of a positive culture result was independent of a history of dacryocystitis or the presence of a mucocele. INTERPRETATION Using direct biopsy methods, we found culture-positive lacrimal sac specimens in a large proportion of patients undergoing DCR surgery. These organisms were found to be present in patients with and without a history of infection.

Cadaveric anatomical comparison of the lateral nasal wall after external and endonasal dacryocystorhinostomy.

Purpose: Much literature has accumulated espousing the relative merits of endonasal and external dacryocystorhinostomy (DCR). However, there is comparatively little information on the relative anatomic differences between these 2 approaches. The purpose of this study is to investigate the anatomic relationships of the lateral nasal wall for endonasal and external DCR. Methods: Ten cadaver half heads were used in this study. Half were subject to endonasal and half to external DCR procedures. The lateral nasal wall was then dissected and measurements were taken of ostium and anastomosis size and position relative to other landmarks on the lateral nasal wall. Relationships were compared between the 2 procedures. Results: The dimensions and area of the ostium and the anastomosis were similar between the 2 procedures. The lower portion of the ostium was located more inferiorly in endonasal DCR. Additionally, the ostium was more likely to be found lateral to the axilla of the middle turbinate in endonasal DCR, when compared with anterior for external. External DCR was also more likely to involve opening the anterior ethmoid air cells than endonasal approach. Conclusion: Endonasal and external DCR osteomies appear to be of similar size, with the endonasal opening being located slightly lower and more posterior on the lateral nasal wall.

Digital Photography as a Novel Technique of Measuring Ocular Surface Dimensions

Purpose: To introduce a novel technique for measuring ocular surface dimensions using digital photography and computerized image analysis in the context of ptosis repair surgery. Methods: Digital photographs and patient questionnaires on dry eye symptoms were obtained from 31 patients before and after ptosis repair. Patients were examined preoperatively and at 1 and 6 weeks postoperatively. Adobe Photoshop 7.0 (Adobe Systems Incorporated, 345 Parkl Avenue, San Jose, CA 95110-2704, USA) was used to digitally measure palpebral fissure height, fissure width, and ocular surface area (OSA). Similar digital measurements were obtained on 30 control subjects as well. Digital calculations of OSA for control, preoperative, and postoperative groups were compared with other published techniques. Results: Graphical comparison between our method of measuring OSA and the mathematical estimations proposed by previous studies suggests that our method is more precise in measuring OSA, and that it is also better able to identify individual variations of OSA from general population trends. Conclusion: Digital ocular photography combined with computerized image analysis is a fast, easy to use, and reliable method of measuring ocular surface dimensions. In addition to ptosis surgery, this method can be used in other ocular surface studies.

The pathogenesis and treatment of lacrimal obstruction: The value of lacrimal sac and bone analysis.

INTRODUCTION . The cause of primary acquired nasolacrimal duct obstruction (NLDO) has not been fully elucidated. In an attempt to determine the role of an inflammatory etiology, the pathology of nasolacrimal sac and bone specimens was assessed and correlated with clinical lacrimal variables. MATERIALS AND METHODS . Lacrimal sac and bone tissues from patients with known NLDO were sampled at the time of external dacryocystorhinostomy (DCR). Histopathological analysis was carried out to determine the presence and degree of inflammatory changes present in the tissues, and to correlate them with the clinical presentation. RESULTS . Of 104 cases analyzed, bony inflammatory changes were seen in 14% and lacrimal sac inflammatory changes in 94%. All cases of bony inflammation had accompanying lacrimal sac inflammation. The inflammatory changes were independent of the following variables: gender, duration of symptoms, a history of dacryocystitis, the presence of a lacrimal sac mucocele, the location of obstruction, and the presence of lacrimal sac calculi. CONCLUSIONS . Inflammatory changes are almost invariably present in all patients with NLDO. Its occurrence in bone is probably secondary to lacrimal sac inflammation. Although attempts are made to perform DCR surgery only in the absence of lacrimal sac inflammation, almost all cases exhibit subclinical inflammation. This may suggest that bypassing this ‘critical area’ of the sac-duct junction, as in a dacryocystorhinostomy, would be more reasonable than to re-canalize through an inflammatory obstruction.

Indications for orbital imaging by the oculoplastic surgeon.

Objective: To determine the indications for ordering orbital imaging and the indications for ordering CT versus MRI by oculoplastic surgeons and to assess the correlation between surgeons clinical indications for imaging and the radiologists diagnosis. Design: Retrospective review of imaging requisitions and radiology reports. Participants: Patients of 4 oculoplastic surgeons who required CT or MRI scans. Methods: Imaging requisitions and radiology reports of patients from 4 oculoplastic surgeons were reviewed to determine the indication for ordering a CT or MRI scan between March 2006 and March 2009. The indications were then compared with the radiologists diagnosis. Results: A total of 735 patients were included: 449 (61.1%) female and 286 (38.9%) male, with an average age of 50.1 years and an age range of 7 months to 93 years. Of these patients, a total of 632 CT and 223 MRI scans were ordered, 135 of which were follow-up scans. Conclusions: The most common indication for CT scan was thyroid disease, followed by orbital tumors and then inflammatory disease, while the most common indication for MRI scan was orbital tumors, followed by inflammatory disease and then thyroid disease. CT scans were more commonly ordered than MRI, largely for trauma and to rule out orbital foreign body.

Orbital wall infarction in sickle cell disease

Patients with sickle cell disease are prone to a variety of ocular manifestations, including comma-shaped conjunctival capillaries, iris atrophy, rubeosis, and nonproliferative and proliferative retinopathy.1 However, orbital involvement is rare. We report a case of sphenoid bone infarction in a patient with homozygous sickle cell disease. A 20-year-old man with a history of sickle cell disease presented to hospital with a 2-day history of fevers, diarrhea, knee pain, headaches, right eye swelling, and painful extraocular movements. There was no loss of vision or diplopia. The patient’s medical history was significant for multiple sickle cell crises, avascular necrosis of the right hip, osteomyelitis, asplenia, and anemia. Ophthalmic examination revealed visual acuity to be 20/25 OD and 20/20 OS. Pupils were equal, round, and reactive to light, with no afferent pupillary defect. Ishihara testing was unremarkable, and there was no red desaturation OD. Right eye motility was limited in all directions of gaze, while left eye motility was normal. Examination of the anterior segment indicated periorbital edema and chemosis in the right eye. Intraocular pressure was 16 mm Hg OD and 13 mm Hg OS. There were no abnormalities in the posterior segment. Computed tomography imaging of the orbits revealed extensive swelling of the right eyelid and subcutaneous tissues. An enlarged lacrimal gland and an adjacent soft tissue mass in the right lateral orbit, extending posterior to the lacrimal gland, were detected. At this point, the patient was given a diagnosis of sickle cell crisis with concomitant orbital cellulitis. Conservative treatment was initiated consisting of intravenous hydration with normal saline, supplemental oxygen to maintain saturations above 90%, analgesia, and intravenous vancomycin and ceftriaxone for presumed orbital cellulitis. Magnetic resonance imaging (MRI) indicated an elongated mass along the lateral aspect of the right orbit, adjacent to the sphenoid bone, which demonstrated bone marrow signal changes (Fig. 1). A second mass was noted in the middle cranial fossa, adjacent to the sphenoid bone.

Complete ophthalmoplegia in Ipilmumab and Nivolumab combination treatment for metastatic melanoma

ABSTRACT Ipilimumab and Nivolumab are novel monoclonal antibodies that have recently been used successfully for treatment of metastatic melanoma. Ipilimumab is a human monoclonal antibody against Cytotoxic T Lymphocyte Antigen 4 (CTLA4) receptor, which suppresses T-cell proliferation and stimulates an inflammatory response against cancer cells. Nivolumab is an IgG4 monoclonal antibody against the cytotoxic T lymphocyte associated programmed death 1 receptor (PD-1). Ipilimumab and Nivolumab combination treatment has been shown to induce remission and prolong survival in patients with metastatic melanoma. The side effect profile of these medications has not been well studied. One entity of the side effects reported in the literature is immune-related adverse events (irAEs). There have been few case reports where these events were serious and irreversible. In this case report, we describe a fatal and severe diffuse panmyositis that involved the cardiac, respiratory, and extraocular muscles in a patient with metastatic melanoma secondary to combination treatment with Ipilimumab/Nivolumab.