Maria M. Choudhary

The effects of liver transplantation on the clinical course of colitis in ulcerative colitis patients with primary sclerosing cholangitis

The course of ulcerative colitis (UC) following orthotopic liver transplantation (OLT) for primary sclerosing cholangitis (PSC) is unclear.

Gender and Ocular Manifestations of Connective Tissue Diseases and Systemic Vasculitides

Ocular manifestations are present in many connective tissue diseases which are characterized by an immune system that is directed against self. In this paper, we review the ocular findings in various connective tissue diseases and systemic vasculitides and highlight gender differences in each disease. In rheumatoid arthritis, we find that dry eyes affect women nine times more than men. The other extra-articular manifestations of rheumatoid arthritis affect women three times more commonly than men. Systemic lupus erythematosus can involve all ocular structures and women are nine times more affected than men. Systemic sclerosis is a rare disease but, again, it is more common in women with a female to male ratio of 8 : 1. Polymyositis and dermatomyositis also affect women more commonly than men but no gender differences have been found in the incidence or disease course in the systemic vasculitides associated with antineutrophil cytoplasmic antibody such as granulomatosis with polyangiitis (GPA, formerly known as Wegeners granulomatosis). Finally, Behcets disease is more common in males, and male gender is a risk factor for Behcets disease. There is a slight female preponderance in sarcoidosis with female gender carrying a worse prognosis in the outcome of ocular disease.

Elevated immunoglobulin G4 level is associated with reduced colectomy-free survival in patients with primary sclerosing cholangitis and ulcerative colitis.

BACKGROUND AND AIM Patients with primary sclerosing cholangitis (PSC) and elevated immunoglobulin (Ig) G4 have been shown to have more severe disease with a shorter time to orthotopic liver transplantation (OLT). The aim of the study was to investigate the clinical outcomes of PSC and UC in patients with elevated serum IgG4. METHODS We analyzed data from 50 patients with PSC and known serum levels of IgG4. They were divided into groups called high IgG4 (>112 IU/L; n = 10) or normal IgG4 (n = 40). We compared the requirement of OLT and colectomy between groups. RESULTS High IgG4 was found in 10 PSC patients (20%). UC was associated in 9/10 patients with high IgG4 vs. 32/40 patients with normal IgG4 (p=0.67). Patients with high IgG4 were younger at PSC diagnosis (28.1 ± 13.9 vs. 37.6 ± 13.4 years, P=0.04), more likely to have backwash ileitis (7/9 vs. 12/32, P < 0.001) and UC flares (median of 5.5 vs. 1.5, P = 0.02). Kaplan-Meier curve analysis showed that patients with elevated IgG4 had reduced colectomy-free survival than patients with normal IgG4 (Log Rank p < 0.001). The median time to colectomy was 5 years from UC diagnosis in high IgG4 group vs. 12 years in the normal IgG4 group (p = 0.01). CONCLUSIONS Elevated IgG4 was seen in a small number of PSC patients. Most of these patients had associated UC, were younger at the time of PSC diagnosis, more likely to have backwash ileitis and had reduced colectomy-free survival than patients with normal IgG4.

Hepatic Ultrasonography for Surveillance in Patients With Uveal Melanoma

IMPORTANCE There is a lack of information regarding the role of systemic surveillance in patients with primary uveal melanoma. OBJECTIVE To evaluate the utility of serial hepatic ultrasonography (USG) for detection of asymptomatic liver metastases in patients undergoing surveillance after primary treatment of uveal melanoma. DESIGN, SETTING, AND PARTICIPANTS Retrospective cohort study reviewing data from patients with primary uveal melanoma treated between October 2003 and October 2012 at a multispecialty tertiary care center. Patients were managed using a standardized protocol. Initial staging was done with contrast-enhanced computed tomography of the chest, abdomen, and pelvis. This was followed by periodic surveillance with hepatic USG and liver function tests scheduled every 6 months for the first 5 years and annually thereafter. Abnormal surveillance hepatic USG findings were categorized as (1) cyst or hemangioma, (2) indeterminate lesion, (3) suspicious for metastasis, or (4) consistent with metastasis. If indicated, hepatic USG abnormalities were confirmed by additional imaging modalities (confirmatory scans) such as computed tomography or magnetic resonance imaging. Liver biopsy was performed only if the confirmatory scan was positive. MAIN OUTCOMES AND MEASURES Sensitivity, specificity, and positive predictive value of hepatic USG for detecting asymptomatic liver metastases. RESULTS In 263 patients (121 men, 142 women; mean [SD] age at diagnosis, 61.1 [13.9] years), a total of 1390 hepatic USGs were performed, with a mean of 5.3 per patient (range, 1-17 per patient). Overall, 86 hepatic USGs of 71 patients (27%) were reported as abnormal. Of the 13 lesions identified as a cyst/hemangioma and 17 as indeterminate, 1 was found to be metastatic in each group (8% and 6%, respectively). Of 36 patients with findings suspicious for metastasis, 23 (64%) had metastasis confirmed. All 5 patients (100%) with findings consistent with metastasis had biopsy-proven metastasis. The sensitivity, specificity, and positive predictive value of hepatic USG for findings that were indeterminate or suspicious for metastasis were 96% (95% CI, 80%-99%), 88% (95% CI, 83%-91%), and 45% (95% CI, 33%-59%), respectively. Specificity of the confirmatory scan was greater than that of hepatic USG (93% [95% CI, 89%-96%] vs 88% [95% CI, 83%-91%], respectively; P < .001). Only 4 of 30 patients (13%) with metastasis had abnormal findings on simultaneous liver function tests. CONCLUSIONS AND RELEVANCE A stepwise surveillance protocol based on serial hepatic USGs followed by confirmatory scans offers high likelihood of detecting asymptomatic metastases in patients with primary uveal melanoma.

Uveal melanoma: evidence for adjuvant therapy.

Considerable progress has been made in identifying patients at risk for metastatic death. Uveal melanomas are characterized by mutations in G proteins (GNAQ and GNA11). Monosomy-3, loss of 6q, and gain of 8q in the primary tumor are associated with a worse prognosis. Gene expression profiling is being applied to tumors to classify tumor gene expression as ‘‘class 1,’’ associated with a low risk of metastasis, and ‘‘class 2,’’ associated with a high risk. Unfortunately, after development of metastatic disease there seems to be no significant survival difference between the treated and untreated patients. Given these findings, adjuvant therapy to prevent progression of subclinical microscopic metastatic disease to detectable macroscopic disease seems logical. We provide a summary of published, current, and future adjuvant therapy trials for uveal melanoma.

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.

Eyelid Contour Following Conjunctival Müllerectomy With or Without Tarsectomy Blepharoptosis Repair.

Purpose: To quantitatively determine how accurately conjunctival Müllerectomy with or without tarsectomy (CM ± T) blepharoptosis repair restores eyelid contour. Methods: The charts of all patients undergoing unilateral CM ± T blepharoptosis repair at the Cole Eye Institute between June 2012 and September 2014 were reviewed. Preoperative and postoperative digital images were used for eyelid contour analysis. Eyelid contour was measured according to a previously described technique measuring 13 radial mid-pupil eyelid distances (MPLDs) in pixels at 15° intervals from 0° to 180°. Eyelid contour was computed taking the ratio of the corresponding radial MPLD on either side of margin reflex distance (105/75, 120/60, 135/45, 150/30, 165/15, and 180/0) using ImageJ software (National Institutes of Health, Bethesda, MD). Ratios were compared between the preoperative and postoperative images using paired t test with statistical significance set at p < 0.05. Results: One hundred and six cases of unilateral CM ± T were performed during the study period and 20 cases met inclusion criteria. In the ptotic eyelid, the postoperative eyelid and the unoperated eyelid groups, the average temporal-to-nasal MPLD ratios at corresponding angles from the midline were closer to 1 from 45° to 135°. Then, further from the midline, at more obtuse angles, the temporal MPLD was greater than the nasal MPLD (i.e., the ratio was greater than 1) in all 3 groups. Conclusions: There was no significant difference in any corresponding MPLD or temporal/nasal MPLD ratio between the postoperative eyelid and the postoperative control eyelid. This technique for CM ± T blepharoptosis repair adequately restores eyelid contour.

Congenital Cranial Dysinnervation Disorders

This chapter describes the clinical features, diagnostic evaluation, and management options of patients with congenital fibrosis of the extraocular muscles (CFEOM) as well as those with other types of congenital cranial dysinnervation disorders (CCDD) such as Mobius syndrome, horizontal gaze palsy with progressive scoliosis, and synergistic horizontal deviation. These are rare and challenging strabismus cases to the most experienced pediatric ophthalmologists and strabismologists. The classification and genetics of CCDDs are briefly discussed, and two case presentations are given. Management strategies are discussed and a flowchart for the evaluation and management of CCDD cases is provided.

Re: “Comparison of Fasanella–Servat and Small-Incision Techniques for Involutional Ptosis Repair”

To the Editor: I read the article reporting quite a large number of cases of ptosis correction. Several criticisms of the study come to mind. Each surgical suite has its own procedures in patient preparation, “time-outs” for patient identification, monitoring systems, and postoperative care, but actual operating time should be counted from the time the surgeon marks and injects to cutting the final skin suture. A resident of mine did her first levator tuck in 13 minutes. The time for this procedure has never been longer than that required for the Fasanella-Servat operation. The Fasanella-Servat procedure excises normal structures, which should be avoided. (Neoplastic or infected or excess tissues should be removed.) The levator tuck with minimal dissection makes any subsequently needed procedure easy. The learning curve for the Fasanella procedure is longer and early results can be poor. The lining of the eyelid is often left irregular and one case I saw after a Fasanella by an experienced oculoplastic surgeon had an opening through the eyelid of 3 to 4 mm. The suture for a levator tuck must be passed through the tissue more than a single pass or else can migrate (“cheese wire”) and lose some of the beneficial effect. The anesthesia injection volume for the Fasanella is greater than for the levator tuck, which usually needs only 1 ml of 2% lidocaine. The levator tuck utilizes a single suture (5-0 Dacron R) in most cases to set the eyelid height, which is easily adjusted intraoperatively! Once that is established, if the eyelid contour is not as desired, placement of 1 or 2 sutures for adjustment takes only 2 or 3 minutes. With time and further aging, ptosis may again be a problem and with a levator tuck, reoperation is not at all difficult. After a Fasanella operation another procedure can be quite difficult. After many years of experience at a home for the aged and with a myasthenia gravis center, I feel qualified to express my opinion.