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Neuro-Ophthalmologic Variability in Presentation of Genetically Confirmed Wolfram Syndrome: A Case Series and Review [Case Report]

Jauregui, Ruben; Abreu, Nicolas J; Golan, Shani; Panarelli, Joseph F; Sigireddi, Meenakshi; Nayak, Gopi K; Gold, Doria M; Rucker, Janet C; Galetta, Steven L; Grossman, Scott N
Wolfram syndrome is a neurodegenerative disorder caused by pathogenic variants in the genes WFS1 or CISD2. Clinically, the classic phenotype is composed of optic atrophy, diabetes mellitus type 1, diabetes insipidus, and deafness. Wolfram syndrome, however, is phenotypically heterogenous with variable clinical manifestations and age of onset. We describe four cases of genetically confirmed Wolfram syndrome with variable presentations, including acute-on-chronic vision loss, dyschromatopsia, and tonic pupils. All patients had optic atrophy, only three had diabetes, and none exhibited the classic Wolfram phenotype. MRI revealed a varying degree of the classical features associated with the syndrome, including optic nerve, cerebellar, and brainstem atrophy. The cohort's genotype and presentation supported the reported phenotype-genotype correlations for Wolfram, where missense variants lead to milder, later-onset presentation of the Wolfram syndrome spectrum. When early onset optic atrophy and/or diabetes mellitus are present in a patient, a diagnosis of Wolfram syndrome should be considered, as early diagnosis is crucial for the appropriate referrals and management of the associated conditions. Nevertheless, the condition should also be considered in otherwise unexplained, later-onset optic atrophy, given the phenotypic spectrum.
PMID: 37508961
ISSN: 2076-3425
CID: 5593192

The Association between Needle Size and Waste Product and Its Effect on Cost-Effectiveness of Botulinum Toxin Injections?

Golan, Shani; Pena, John; Moore, Jeffrey; Tandalam, Sangeetha; Lelli, Gary
Clostridium botulinum toxin (BT) must be administered intramuscularly with a syringe, but dead space in the syringe-needle complex may cause product waste and result in cost implications for the patient and provider. Syringe dead space is the volume of residual fluid that remains within the syringe after the plunger is fully depressed during medication injection. We calculated the average volume of remaining product in a syringe-needle complex and cost loss implication of this volume of BT. This is a single-center, analytical study using saline and four different sized needles for analytics of waste product and cost-effectiveness. Syringes of 1 mL with attached 18, 21, 30, and 32-gauge (G) needles, respectively, were compared. The syringe-needle complex was weighed before drawing 0.05 mL of saline. The fluid was then discarded with the appropriate syringe and then weighed again. This procedure was repeated for the four needle types and the average difference in weight of the syringe-needle complex before and after saline waste was measured. The volume was converted to units of BT used in clinical practice and the cost of waste product evaluated. The mean difference in needle-syringe complex weight before and after intervention was 0.068, 0.056, 0.04, and 0.026 g for the 18, 21, 30, and 32G needles, respectively. We found a statistically significant difference comparing the 18G with the 30 and 32G (0.02 and 0.0007, respectively) and comparing the 21G with the 30 and 32G (0.0042 and 0.00002, respectively). When we extrapolated the data to BT units (4U/0.1 mL), we found that theoretically 2.72, 2.24, 1.6, and 1.04 units of BT are left in the syringe-needle complex for the 18, 21, 30, and 32G syringes, respectively. At a cost of $6.01/U of onabotulinum toxin A, we then calculated a provider loss of a gross average (mean) revenue of $96 and 62.4 per 10 syringes used with 30 and 32G needles. Needle size used for drawing up and administering BT has an effect on the amount of waste product and subsequently on cost-effectiveness.
PMID: 32688397
ISSN: 1098-8793
CID: 4531962

Lacrimal tissue resection in Fasanella Servat operation and the correlation to dry eye

Golan, Shani; Olson, Luke C; Patel, Hency H; Pinchover, Shulamit; Magro, Cynthia M; Levine, Benjamin; Lelli, Gary J
Purpose: Fasanella-Servat operation (FSO) was previously reported to be associated with post-operative dry eyes due to accessory lacrimal gland resection during the surgery. We performed a retrospective, cohort study to determine the frequency of lacrimal tissue resection during FSO and its correlation with post-operative eye dryness and keratopathy. Methods: Review of all patients who underwent FSO at New York-Presbyterian Weill Cornell Hospital over a two-year period (2013-2015). Patients were included only if they had adequate histopathological specimens of the resected tissue obtained during surgery. Outcomes included the study of the pathological specimen for the presence of lacrimal tissue; Post-operative dry eye symptoms and pre- and post-operative corneal epitheliopathy. Results: 46 patients with a total of 58 eyelid resections were studied. Eight eyelids (13.7%) were found to have lacrimal tissue present in the pathology specimens. Postoperatively, nine patients reported some symptoms of dry eye and new-onset keratopathy was noted in four eyes (6.8%), only one of which had lacrimal tissue present in histopathology specimen obtained from surgery. Discussion: Previous studies found lacrimal tissue present in up to 43% of specimens resected during FSO. Our data found a lower rate of lacrimal tissue resection during FSO, and did not find an association between lacrimal tissue resection and post-operative dryness or epitheliopathy. Conclusion: Our study is one of few to examine histopathological resections from the FSO. We found that lacrimal tissue is not frequently resected during FSO, and when it is resected, there is no increased incidence of post-operative dryness or keratopathy.
PMID: 31393212
ISSN: 1744-5108
CID: 4033422

Involutional entropion repair combining the modified Bick quick strip procedure with Quickert rotational sutures

Golan, Shani; J Lelli, Gary
PURPOSE/OBJECTIVE:The purpose of the study was to determine the results of involutional entropion repair by combining the modified Bick strip procedure with Quickert rotational sutures. METHODS:A retrospective review of consecutive patients with involutional entropion who underwent repair by combined Bick and Quickert technique from January 2013 to December 2017 was performed. RESULTS:A total of 43 cases of involutional entropion repair were preformed in this time period. Fourteen eyelids of 12 patients had the combined procedure. There were no failures with a median follow-up of 29 months. Mean operative time, recorded in 10 patients, was 15 ± 2.2 minutes. Complications were minimal. CONCLUSIONS:The combination of the modified Bick quick strip procedure and Quickert sutures requires minimal surgical dissection and provides excellent results for the treatment of involutional entropion.
PMID: 30024812
ISSN: 1744-5108
CID: 3962722

Ocular protrusion in sitting and supine position [Letter]

Golan, Shani; Rootman, Daniel B; Lambros, Val; Goldberg, Robert A
PMID: 29430800
ISSN: 1442-9071
CID: 3962692

Does long-term success from endoscopic DCR correlate with early post-operative reduction in tearing?

Golan, Shani; Chen, Yuefan; Levine, Benjamin; Pearlman, Aaron N; Levinger, Joshua I; Tabaee, Abtin; Kacker, Ashutosh; Lelli, Gary J
PURPOSE/OBJECTIVE:The purpose of the study was to determine the association between early post-operative improvement in tearing and the long-term success rate of endoscopic dacryocystorhinostomy (eDCR). METHODS:A retrospective review of consecutive patients who underwent eDCR and were followed up for at least 6 months at our institution from January 2010 to December 2017 was performed. RESULTS:47 cases(39 patients) of eDCR met the inclusion and exclusion criteria during this time period. Mean follow up after the surgery was 12.5 ± 8 months. In 45 out of 47 (96%) cases post-operative improvement in epiphora within 2 weeks of surgery, or lack thereof, correlated with long-term success or failure of the procedure. There were only 2 cases in which the patients felt improvement in tearing at the initial post-operative visit and the tearing recurred in the late post-operative period (>6 months). CONCLUSIONS:There is a strong association between the early initial post-operative assessment of tearing resolution and the long-term result of eDCR.
PMID: 30017375
ISSN: 1532-818x
CID: 3962712

Abducens nerve palsy after orbital decompression [Case Report]

Golan, Shani; Goldberg, Robert A
INTRODUCTION/BACKGROUND:Double vision after decompression surgery for Thyroid Eye Disease (TED) is well described in the literature and the incidence ranges from 0 to 64%. The Mechanisms for new onset diplopia after orbital decompression are poorly understood. Common theories include: Fibrosis of muscles, displacement of the muscle cone, and reactivation of the TED. AIM/OBJECTIVE:We present two cases with Abducens nerve palsy after uncomplicated secondary orbital decompression surgery. RESULTS:Two patients with inactive TED, who were followed for an average of 2 years prior to uneventful secondary decompression surgery, presented at the first postoperative visit with double vision and limitation of abduction in the recently operated eye. Magnetic resonance imaging(MRI) was done in both cases and revealed no abnormal bleeding or scaring. DISCUSSION/CONCLUSIONS:Our two cases of Abducens palsy following reoperative orbital decompression may be due to ischemic neuropathy caused by postoperative hemorrhage or inflammation.
PMID: 29058517
ISSN: 1744-5108
CID: 3962682

Reply re: "Postoperative Changes in Strabismus, Ductions, Exophthalmometry, and Eyelid Retraction After Orbital Decompression for Thyroid Orbitopathy" [Comment]

Rootman, Daniel B; Golan, Shani; Pavlovich, Peter; Rootman, Jack
PMID: 29517606
ISSN: 1537-2677
CID: 3962702

Double Vision After Minimally Invasive Orbital Decompression

Golan, Shani; Gupta, Adit; Goldberg, Robert A
INTRODUCTION/BACKGROUND:New onset Diplopia following orbital decompression in thyroid eye disease patients is estimated to occur in up to 30% to 40% of decompression patients, mostly related to deep lateral and medial wall decompressions. METHODS:A retrospective chart review of all minimally invasive (fat and minimal bone orbital decompression performed at the UCLA Stein Eye Institute between 2005 and 2015. Inclusion criteria were thyroid eye disease patients older than 18 years undergoing fat only orbital decompression with no previous muscle surgery. RESULTS:The chart review revealed only 5 patients with new onset diplopia after this surgery. The cases are discussed and a possible mechanism for the diplopia is proposed. DISCUSSION/CONCLUSIONS:Double vision following minimally invasive orbital decompression is rare and the mechanisms are poorly understood.
PMID: 28665847
ISSN: 1536-3732
CID: 3962672

Postoperative Changes in Strabismus, Ductions, Exophthalmometry, and Eyelid Retraction After Orbital Decompression for Thyroid Orbitopathy

Rootman, Daniel B; Golan, Shani; Pavlovich, Peter; Rootman, Jack
INTRODUCTION/BACKGROUND:Surgical rehabilitation of thyroid orbitopathy involves reducing proptosis, treating strabismus, lengthening the eyelids, and managing aesthetic changes. Not all are necessary in each patient; however, they often are. The current investigation intends to describe postdecompression changes that may influence the staging of these procedures. METHODS:In this retrospective cohort study, records of 169 patients who underwent orbital decompression between 1983 and 2001 were reviewed. A single orbital specialist confirmed all measurements. Time to follow up was defined as the most recent follow up after decompression and prior to any secondary procedures. No strabismus or eyelid surgery was performed at the time of decompression. Strabismus was measured with alternating prism cover test. Ductions were estimated utilizing Hirschberg's method. Exophthalmometry was measured with Hertel. Eyelid positions were defined relative to the pupillary light reflex. Strabismus data were analyzed within eye pairs. Ductions, exophthalmometry and eyelid position were analyzed for each eye. T-test for paired data was utilized to compare means pre- and postoperatively. RESULTS:The study population was on average 45 years old and 73.4% women. Average length of follow up was 1.2 years. Esotropia was significantly increased after decompression by an average of 8.1 prism diopters (p < 0.01). Exotropia and vertical deviations were not significantly altered. Ductions decreased by >5 degrees in at least one meridian for 68.1% of the population. Upper eyelid retraction remained unchanged; however, lower eyelid retraction improved by 50% from 1.4 mm to 0.7 mm (p < 0.01). Exophthalmometry improved from 23.5 mm to 19.7 mm (p < 0.01), and this result was correlated with the number of walls removed (Pearson r = -0.302, p < 0.01). CONCLUSIONS:On average, esotropia and ductions tend to worsen with decompression surgery. This result supports the clinical dictum to avoid strabismus surgery until after decompression. The improvement in lower eyelid retraction suggests that at least lower eyelid-lengthening surgery should be reserved for after decompression, as there may be significant spontaneous improvement, while the same may not be true for upper eyelid retraction, which does not tend to change with decompression.
PMID: 27487726
ISSN: 1537-2677
CID: 3962632