Sine Scleroderma.

Systemic sclerosis (SSc) is a connective tissue disorder of unknown aetiology. A small subset (10%) of patients with limited systemic sclerosis have all other features of the disease without any skin involvement and is known as systemic sclerosis sine scleroderma (ssSSc). Severe Critical Limb Ischaemia is rare in sine scleroderma.The present case showed severe critical limb ischaemia with severe PAH,Esophageal dysmotility,Glomerulonephritis(a rare association) with hypertension. Although skin thickening is considered as a hallmark of systemic sclerosis, there should be a high index of clinical suspicion in patients presenting with possible manifestations of systemic sclerosis without sclerodermatous cutaneous involvement because early diagnosis and treatment can reduce the morbidity and mortality in it.

MEN Type I (Wermer Syndrome).

MEN I inherited as an autosomal dominant disorder leads to hyperplastic/ neoplastic changes in parathyroid, pituitary and endocrine pancreas along with other characteristic tumours. Hyperparathyroidism is the most common manifestation of MEN I. Our case was a female patient aged 42 years who was diagnosed with parathyroid adenoma, coincident with pancreatic neoplasm and adrenal adenoma. Hyperparathyroidism was noted initially and hemiparathyroidectomy was performed. Though adrenal adenoma and pancreatic neoplasm were detected on CECT, patient was symptom free from them and thus steps were taken to treat the chief complaint of presentation which was multiple bone pains. Post-operatively patient's serum Ca levels, serum PTH levels dropped drastically to normal ranges and there was remarkable improvement in complaints of patient. A multidisciplinary approach involving physicians, endocrinologists, oncologists, ENT surgeons and radiologists is pivotal for optimizing patient treatment. Treatment consists of surgery and drug therapy, often in association with radiotherapy or chemotherapy.

Clinical Outcomes and Cost Analysis of PreserFlo versus Trabeculectomy for Glaucoma Management in the United Kingdom.

PURPOSE: Clinical evaluation and cost analysis of mitomycin-C-augmented PreserFlo MicroShunt versus trabeculectomy. DESIGN: Retrospective cohort study across 3 teaching hospitals. PARTICIPANTS: A total of 134 consecutive eyes of 129 patients (70 undergoing MicroShunt, 64 trabeculectomy). METHODS: Primary and secondary glaucoma cases with uncontrolled intraocular pressure (IOP) were included. Neovascular glaucoma and surgery combined with cataract extraction were excluded. The cost analysis used results from the clinical study to estimate operative costs (equipment and staff costs) and postoperative costs (follow-up visits, nonglaucoma medications, and postoperative procedures) per eye for PreserFlo and trabeculectomy. MAIN OUTCOME MEASURES: The primary clinical outcome measure was surgical failure (defined as IOP > 21 mmHg or < 20% reduction from baseline, IOP ≤ 5 mmHg, reoperation, or loss of light perception) or qualified and complete success (with or without medication) at 18 months. Secondary measures were IOP, glaucoma medications, visual acuity, mean deviation, time to cessation of steroid drops, complications, surgical time, follow-up visits, postoperative interventions, and reoperations. The cost analysis evaluated costs of PreserFlo compared with trabeculectomy. RESULTS: Baseline characteristics were similar, except for more non-White patients in the trabeculectomy group (51% Black and Asian vs. 32% MicroShunt, P = 0.02) and more cases with prior ab externo glaucoma surgery in the MicroShunt group (19% vs. 3% in the trabeculectomy group, P = 0.004). Overall, 59% of eyes had primary open-angle glaucoma. Mean follow-up was 19.9 months for both groups. At 18 months, surgical failure was 25% for MicroShunt compared with 35% for trabeculectomy (P = 0.18). Failure in MicroShunt cases was due to inadequate IOP reduction (84%) or reoperation for glaucoma (16%). Failure in trabeculectomy cases was due to inadequate IOP reduction (58%), persistent hypotony (29%), or reoperation for glaucoma (13%). Combined blebitis and endophthalmitis rate was 1.4% for MicroShunt and 3.1% for trabeculectomy. Cost analysis showed a savings of £245 to £566 per eye in the MicroShunt group, driven mostly by reduced postoperative procedures and follow-up visits. This is in contrast to prior randomized controlled trial data reporting the incremental cost of $2058 of PreserFlo over trabeculectomy. CONCLUSIONS: Our experience of introducing PreserFlo MicroShunt surgery showed it was safer than trabeculectomy and is a cost-saving and effective option that offers potential to free up highly limited National Health Service resources. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found after the references.

One-year surgical outcomes of the PreserFlo MicroShunt in glaucoma: a multicentre analysis.

BACKGROUND/AIMS: To evaluate the efficacy and safety of the PreserFlo MicroShunt glaucoma device in a multicentre cohort study. METHODS: All consecutive patients who received the microshunt with mitomycin-C (MMC) 0.4 mg/mL from May 2019 to September 2020 in three UK tertiary centres. Primary outcome at 1 year was a complete success, with failure defined as intraocular pressure (IOP) >21 mmHg or <20% reduction, IOP≤5 mmHg with any decreased vision on two consecutive visits, reoperation or loss of light perception vision. Secondary outcomes were IOP, best-corrected visual acuity, medications, complications, interventions and reoperations. We also performed subgroup analyses for severe glaucoma and assessed risk factors for failure. RESULTS: 104 eyes had 1-year follow-up. Complete and qualified success at 1 year were achieved in 51.9% (N=54) and 16.4% (N=17), respectively, and failure occurred in 31.7% (N=33). There was a significant reduction in IOP (mmHg) from preoperatively (23.4±0.8, N=104) to 12 months (14.7±0.6, N=104) (p<0.0001). Antiglaucoma medications also decreased from preoperatively (3.4±0.1, N=104) to 12 months (0.7±0.1, N=104) (p<0.0001). Multivariate analyses showed an association between higher mean deviation and failure (HR 1.055, 95% CI 1.0075 to 1.11, p=0.0227). Complications were hypotony (19.2%; N=20), choroidal detachments (10.6%; N=11), hyphaema (5.8%; N=6) and bleb leak (5.8%; N=6). Needling and 5-fluorouracil injections were performed in 12.5% (N=13) and 33.7% (N=35), respectively, and 11.5% (N=12) required revision surgery. CONCLUSION: The PreserFlo MicroShunt with MMC 0.4 mg/mL showed an overall success rate of 68.3% at 1 year, and led to significant IOP and medication reduction with a low rate of adverse effects.

Long-term outcomes in Primary congenital glaucoma, aniridia and anterior segment dysgenesis.

AIM: To determine the long-term outcomes of a cohort of complex patients with primary congenital glaucoma, aniridia and anterior segment dysgenesis. METHODS: Retrospective consecutive series between 1990-2021 in two UK tertiary centres: Guy's and St Thomas' NHS Foundation Trust and King's College Hospital NHS Foundation Trust. We recorded the number and types of surgical and laser treatments along with preoperative and postoperative data, including intraocular pressures (IOP) and anti-glaucoma medications. RESULTS: A total of 41 eyes of 21 patients were included. Primary diagnoses were primary congenital glaucoma in 16 eyes (39.0%), aniridia in 14 eyes (34.2%), and anterior segment dysgenesis in 8 eyes (19.5%). Sixteen eyes (39.0%) had one or more glaucoma surgery or laser procedures for advanced glaucoma, and the long-term follow-up was 12.8 ± 3.6 years. There was a significant decrease in postoperative IOP (mmHg) at 3 months (16.5 ± 1.6; p = 0.0067), 6 months (18.7 ± 2.1; p = 0.0386), 12 months (18.6 ± 1.7; p = 0.0229), 3 years (14.7 ± 1.2; p = 0.0126), 5 years (15.5 ± 1.8; p = 0.0330) and 10 years (15.4 ± 2.3; p = 0.7780), compared to preoperatively (24.1 ± 2.6). Surgical success (complete and qualified) was 62.5%, 50.0%, 43.8%, 46.2%, 45.5% and 28.6% at 3 months, 6 months, 12 months, 3 years, 5 years and 10 years, respectively. There was no significant change in the number of anti-glaucoma drugs postoperatively (p > 0.05). Four eyes (25.0%) had postoperative complications (hyphaema, hypotony) that resolved after conservative management. CONCLUSIONS: Surgical management of these complex eyes with advanced glaucoma is challenging. Overall, the cohort had good surgical outcomes with a significant decrease in IOP by 36.1% after long-term follow-up.

Long-term refractive stability following combined astigmatic keratotomy and phakoemulsification.

BACKGROUND: To determine whether combined astigmatic keratotomy and cataract surgery induces a sustained reduction in astigmatism. METHODS: Patients who had previously undergone combined cataract surgery and astigmatic keratotomy for naturally occurring astigmatism were identified and their notes reviewed. Mean final follow-up was 34 months (30-42 months). Fourteen eyes of 11 patients were eligible for study. Arcuate corneal incisions had been employed using the Buzard nomogram. Using power vector analysis, the post-operative refractive change at 6 months and final follow-up was determined. RESULTS: The mean preoperative manifest cylindrical refractive error was 2.88 D (1.50-5.50 D). The mean postoperative manifest cylindrical refractive error was 1.89 and 1.79 D at 6 months and final follow-up, respectively. Power vector analysis demonstrated a significant postoperative reduction in refractive blur at 6 months and final follow-up (P = 0.006, P = 0.004, respectively): mean preoperative blur strength was 2.90 D and mean postoperative blur strength was 1.33 and 1.25 D at 6 months and final follow-up, respectively. Sustained postoperative reduction in astigmatism was noted in 12 of 14 eyes at final follow-up. CONCLUSIONS: Astigmatic keratotomy is a well-established treatment for astigmatism. In this series, the refractive benefit from combined astigmatic keratotomy and cataract surgery is maintained for several years. No surgical complications were observed.

Effectiveness of iron-fortified infant cereals on hemoglobin levels of children aged 12-24 months: A cross-sectional study from New Delhi, India.

INTRODUCTION: Iron deficiency anemia represents 3rd largest disease burden, with an estimated 6.9 billion disability-adjusted life years. Iron-fortified cereals (IFIC) can contribute substantially in preventing iron deficiency anemia and maintaining an adequate body iron status. The aim of this study is to assess the effectiveness of IFIC intake along with other complementary food/s on the hemoglobin (hb) level of children from 12 to 24 months of age. MATERIALS AND METHODS: A cross-sectional study was conducted from November 2015 to February 2016 in three pediatric outpatient clinics of New Delhi, India. A predesigned questionnaire was used to elicit information on socio-demography, complementary feeding, and intake of IFIC from 66 mother and child pairs. Child's anthropometric measurement and hb levels were recorded by the pediatrician. Chi-square and Student's t-tests were used to compare the key study variables between IFIC (minimum 1-2 serving/day) and non-IFIC groups. Multiple logistic regression analysis was applied to explore the independent correlates of anemia in the study groups. RESULTS: Out of 66 children, 60.6% (n = 40) of children were boys. The prevalence of anemia (hb% <11 g/dl) was 42.4% (95% confidence interval (CI): 30.5%-55.2%, n = 28). Multiple logistic regression analysis revealed that the children in IFIC group were unlikely to be anemic (adjusted odds ratio (OR): 0.007, 95% CI: 0.001-0.079, P < 0.001). On the contrary, boys (adjusted OR: 11.6, 95% CI: 1.23-108.9, P = 0.032) and children with low birth weight (adjusted OR: 11.7, 95% CI: 1.23-111.76, P = 0.032) were associated with anemic status. CONCLUSION: Intake of IFIC (minimum 1-2 serving/day) was associated with the lesser chance of anemia in children of 12-24 months. However, gender and low birth weight were also associated with anemia. IFIC may have a role in mass fortification programs. However, further larger and controlled studies are recommended to test this hypothesis.

Aneurysmal bone cyst of the pubis: a case report.

An aneurysmal bone cyst is considered as a locally aggressive benign tumour. Intra-lesional extended curettage and bone-grafting is the mainstay of the treatment for aneurysmal bone cysts. Grafting is used usually in cases where the lesion compromises the mechanical strength of the bone. However, the massive size of the highly vascular tumour and the relative inaccessibility of its deeper extensions into the femoral vessels and the intra-abdominal structures, especially the urinary bladder, make it a relatively challenging case to perform excision and curettage.Presenting a case of a 15 years old male patient with the complaint of a right inguinal swelling since the past eight months. The swelling had started growing since the past two months and it was associated with pain. X-ray showed a lytic blowout legion of the entire right pubic ramus. An intra-lesional curettage was done. Complete tumour excision which was done by intra-lesional curettage and biopsy yielded satisfactory results with low complications and low recurrence of aneurysmal bone cyst of the superior ramus of the pubis.

Giant cell tumor of lower end of tibia.

Introduction. Giant cell tumor of bones is an unusual neoplasm that accounts for 4% of all primary tumors of bone, and it represents about 10% of malignant primary bone tumors with its different grades from borderline to high grade malignancy. Case Report. A 35-year-old patient presented with complains of pain and swelling in left ankle since 1 year following a twisting injury to his left ankle. On examination, swelling was present over the distal and anterior part of leg and movements of ankle joint were normal. All routine blood investigations were normal. X-ray and CT ankle showed morphology of subarticular well-defined expansile lytic lesion in lower end of left tibia suggestive of giant cell tumor. Histopathology of the tissue shows multinucleated giant cells with uniform vesicular nucleus and mononuclear cells which are spindle shaped with uniform vesicular nucleus suggestive of GCT. The patient was treated by excision, curettage, and bone cement to fill the defect. Conclusion. The patient at 12-month followup is doing well and walking without any pain comfortably and with full range of motion at ankle joint with articular congruity maintained and no signs of recurrences.

Intravitreal bevacizumab in refractory neovascular glaucoma: a prospective, observational case series.

OBJECTIVE: To examine the efficacy of intravitreal bevacizumab for pain relief in eyes with refractory neovascular glaucoma. METHODS: In this prospective case series, 52 eyes with neovascular glaucoma were administered intravitreal bevacizumab, 1.25 mg, and monitored for 6 months. The primary outcome measure was change in subjective pain score. Intraocular pressure and iris neovascularization were evaluated at each visit. Surgical intervention for control of intraocular pressure was performed according to clinical need. RESULTS: Forty-two patients (44 eyes) completed the 6-month follow-up. Subjective pain score was reduced significantly 1 week after intravitreal bevacizumab injection and lasted throughout the follow-up period (median [interquartile range]: baseline, 3 [0-6]; week 1, 1 [0-3]; month 1, 0 [0-1]; month 3, 0 [0-1]; and month 6, 0 [0-0]; Kruskal-Wallis χ(2) 31.03; P < .001). A rapid, yet relatively transient, reduction in iris neovascularization was also noted (iris neovascularization grade at baseline, 4.0 [3-4]; week 1, 2.5 [1-4]; month 1, 2.0 [1-4]; month 3, 3.0 [2-4]; and month 6, 3.0 [2-4], χ(2) 23.33; P < .001). Four eyes (8%) required more than 1 injection to facilitate further intraocular surgery. CONCLUSIONS: Intravitreal bevacizumab is a useful adjunct in the management of refractory neovascular glaucoma, producing rapid relief of pain. However, we found no evidence to suggest that intravitreal bevacizumab lowers intraocular pressure in eyes with angle closure; conventional medical, laser, and surgical treatment are still needed in these eyes.