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Deficiency of adenosine deaminase 2 (DADA2) is a newly described entity of monogenic vasculitis with multisystem involvement and prominent neurological features. With this report, we are adding to the growing spectrum of cases of DADA2 with two adult cases of early-onset recurrent cerebrovascular events with multisystem involvement. These cases highlight the need for high suspicion of this diagnosis in adults presenting late with symptoms compatible with DADA2. We further report the futility and probable harm of antiplatelet agents in DADA2. Tumour necrosis factor inhibitors have been shown to be beneficial for the treatment and prevention of severe manifestations of this condition.

Introduction HLA-B*27 is strongly associated with Ankylosing spondylitis (AS). Its subtypes show considerable geographic and ethnic difference. The main aim of this study was to assess the frequency of subtypes of HLA-B*27 in the Indian Tamil AS patients. Methods and materials Adult AS patients positive for HLA-B*27 were considered for the study. The high-resolution typing to define HLA-B*27 subtypes were done using Invitrogen B kits from One Lambda (SeCore® Sequencing Kits, Thermo Fisher, United States). Results and conclusion Prevalence of subtypes identified were HLA-B*27:04 (52.2%), HLA-B*27:05 (41.6%), HLA-B*27:07 (3.5%) and HLA-B*27:02 (2.7%). All subtypes showed disease predisposition for males. The most common extra articular manifestation seen was enthesitis in HLA-B*27:04 and HLA-B*27:05. Uveitis was mainly associated with HLA-B*27:05 and dactylitis with HLA-B*27:04. A significant peripheral joints involvement for female and axial joint involvement for males was seen in HLA-B*27:04. Our study establishes the prevalence of HLA-B*27 subtypes and the associated clinical phenotypes among the Indian Tamil population. Considering the variability of presentation, organ involvement, and disease course in different subtypes and across ethnicities it is critical to define these associations in the ethnic populations we treat for their appropriate care considering the significant negative health and socioeconomic effects of AS.

Consensus on treatment of idiopathic inflammatory myositis (IIM), particularly with regard to flares and interstitial lung disease (ILD), does not exist. We studied the long-term outcome and treatment response in our large, retrospective cohort of adult South-Asian patients exclusively with IIM. Electronic records of IIM patients satisfying inclusion and exclusion criteria were studied longitudinally at presentation, at 3, 6, 12, 18 and 24 months and thereafter yearly till their last follow up (F/u) visit. Depending on clinical, imaging, and muscle enzyme profile during the F/u period, patients were categorised as complete (CR) and partial responders (PRs). Parameters favouring CR were assessed using multivariate logistic regression analysis. Outcome parameters and flares on immunosuppressants (IS) were then assessed in patients with/without ILD. Two hundred thirty-two patients with median F/u duration of 44.5 months (25–80.25) were included. ILD was seen in 40.1%. Patients with non-Jo1 anti-synthetase antibodies (n=26) were numerically more than those with Jo-1 antibody (n=24). CR status was attained by 50.9% patients. Absence of pericardial effusion (p=0.042, OR 4.223, 95% CI: 1.05–16.9) and presence of Gottron’s rash (p=0.044, OR 1.78, 95% CI 1.017–3.121) at baseline predicted CR by multivariate regression. Majority received mycophenolate during the entire F/u period. Discontinuation of steroids was feasible in 51.7% after a median duration of 24 months (18–42). After excluding patients with ILD, flares were numerically lesser in patients only on mycophenolate compared with those only on methotrexate (p=0.06). Further flares were curtailed when switched from other agents to mycophenolate. Mycophenolate is an effective treatment option in IIM patients with and without co-existing ILD. Presence of Gottron’s rash and absence of pericardial effusion were found to be predictors of favourable clinical outcome in this largest single-centre study.

Leprosy and sarcoidosis are chronic multisystem disorders, with clinical features overlapping with each other. The diagnosis requires a thorough clinical, laboratory, histopathological, and radiological examination. Although it is possible to conclude on one particular diagnosis most of the time, in some cases, even after extensive evaluation, it is difficult to differentiate between the two diseases. In such scenarios, treating both the conditions is warranted. Here, we present a case with a diagnostic dilemma between Hansen's disease and sarcoidosis and systematically discuss the specific pointers which can help to differentiate between the two diseases.

Objectives: The objective of this study is to study the role of urinary vascular cell adhesion molecule-1 (uVCAM-1) as a marker for disease activity in lupus nephritis (LN). Methodology: This was a diagnostic study where patients with active LN were taken as cases and those without LN were taken as disease controls. uVCAM-1 was correlated with the classes of LN and standard of care markers. Results: There was a significant difference between the uVCAM-1 values in cases (59.69 [range: 0.07–13752.5]) pg/mg and controls (2.11 [range − 0.11 − 1138.5] pg/mg [P = 0.02]). Maximum levels of VCAM 1 were seen in Class 4 and Class 5 LN (P = 0.96). Although higher renal systemic lupus erythematosus disease activity index score had a higher median value of uVCAM 1, the values were not statistically significant (P = 0.2). There is a positive correlation between the uVCAM1 and anti-double-stranded DNA (anti-dsDNA) levels (r = 0.38) and a negative correlation between VCAM 1 levels and C3 (r = −0.19).The sensitivity of uVCAM1 for determining the disease activity was 65.2%, and the specificity was 75% at a cutoff value of more than 23.8 pg/mg. Conclusions: uVCAM 1, although not in isolation, but along with the other standard of care markers may be useful in assessing the disease activity.

Background:. Subpectoral implant reconstruction (SIR) is associated with animation deformity and increased postoperative pain. The aim of our study was to compare the short- to medium-term outcome of prepectoral implant reconstruction (PIR) and SIR with acellular dermal matrix. Methods:. A review of prospectively collected data of patients who underwent PIR and SIR using biological mesh by a single surgeon between Nov 2016 and Nov 2020 was done. Presentation, smoking history, BMI, bra size, radiology size, pathology data, mastectomy weight, implant volume, radiation treatment, and outcomes were analyzed. For intergroup comparisons, the data were analyzed using Pearson chi-square test and the Student t-test. P values of less than 0.05 were considered statistically significant. Results:. Eighty-two patients had 109 implant reconstructions (85 PIR and 24 SIR). Median follow-up was 27 months (4–53). Twenty-five of PIR and six of SIR were risk reduction surgeries. Two groups were comparable except for significantly higher percentage of node positive (13/18, 72% versus 20/60, 33%; P = 0.003), Her 2-positive disease (7/18, 39% versus 9/60, 15%; P = 0.027), and radiotherapy (15/24, 63% versus 21/85, 25%; P = 0.001) in SIR. There was no statistically significant difference between the two groups with regard to hematoma, wound infection, red reaction, seroma needing aspiration, wound necrosis, and implant loss. Significantly more patients in the prepectoral group had rippling needing fat grafting (n = 13, 15% versus 0; P = 0.041) and significantly more patients in the subpectoral group had fat grafting for contour deformity (n = 6, 25% versus n = 6, 7%; P = 0.025). Conclusion:. PIR is comparable to conventional SIR with regard to most of the complications.

Chylothorax due to inadvertent thoracic duct injury after esophagectomy is a well-known complication and requires careful postoperative management and timely intervention to prevent potential morbidity and mortality. We present a case of high-output chylothorax after esophagectomy where the source of chyle leak was not in the thorax.

Esophageal leiomyosarcoma is the commonest of all esophageal sarcomas but yet has a very low incidence. These tumors have been resected by the open approach so far. We describe the steps and challenges involved in the thoracoscopic excision of a huge leiomyosarcoma of the esophagus.

Background & objectives: Fibromyalgia syndrome (FMS) is one of the most common chronic pain conditions of unknown aetiology. Mitochondrial dysfunction has been reported in FMS with some studies reporting the presence of mitochondrial mutation namely A3243G, which also causes mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes. This pilot study was conducted to assess this mutation and also detect large deletions in mitochondrial DNA (mtDNA) in patients with FMS. Methods: Thirty female patients with FMS participated and 30 matched controls were included. Genomic DNA was subjected to polymerase chain reaction (PCR) amplification using specific primers followed by restriction digestion with Apa I enzyme to detect the specific A3243G mtDNA mutation. Long-range PCR was done in two sets to detect the large deletions in the mtDNA. Biochemical parameters including thyroid-stimulating hormone and vitamin D levels were also looked at. Results: None of the patients were found to carry the common mutation or large deletions. Low vitamin D level was a common finding. Hypothyroidism was found in a few patients. Interpretation & conclusions: Although the common mutation or large mtDNA deletions were not detected in blood mtDNA in the FMS patients, mutations in the muscle and sequence variation in mtDNA remained a possibility. Future studies in both blood and muscle tissue including mtDNA sequencing are warranted in such patients to determine if a subset of FMS patients have mitochondrial myopathy.

Introduction: There is a paucity of real-world data on mycophenolate mofetil/mycophenolate sodium in systemic sclerosis-related interstitial lung disease. Aim: To study the efficacy of mycophenolate mofetil/ mycophenolate sodium in systemic sclerosis-related interstitial lung disease. Methods: In this single-centre study, clinical, laboratory and imaging details of consecutive patients with systemic sclerosis-related interstitial lung disease receiving mycophenolate mofetil/mycophenolate sodium from rheumatology and pulmonology clinics between January 2008 and March 2017 were retrospectively retrieved. The change in percentage of predicted normal forced vital capacity at last follow-up visit as compared with baseline was studied. In addition, high-resolution computed tomography scans at baseline and 2-year follow-up visit were scored as either stable/improved or worsened by experienced thoracic radiologists blinded to the clinical details of patients. Results: Altogether, 88 patients (85.2% females) with mean age (SD) of 33.8 years (± 11.3) and median (interquartile range) duration of disease since non-Raynaud’s symptoms of 36 months (13.5–60) were studied. Diffuse systemic sclerosis comprised 85.2% of them. The mean baseline forced vital capacity was 61.2 ± 17.9% and median scores for ground glass opacities and fibrosis in high-resolution computed tomography were 0.5 (0–1.3) and 1 (0–1.3), respectively. At a median follow-up duration of 30 months (interquartile range = 16.5–49), the percentage of forced vital capacity improved by 1.8% (–3.82 to 9.07) as compared with baseline visit ( p = 0.02). In the 2-year follow-up, the ground glass opacity and fibrosis scores in high-resolution computed tomography improved in 17.3% and 7.7% of patients and stabilized in 63.5% and 78.8% patients, respectively. Conclusion: Mycophenolate mofetil/mycophenolate sodium was efficacious in improving /stabilizing forced vital capacity irrespective of the baseline high-resolution computed tomography lung scores in our patients with systemic sclerosis-related interstitial lung disease during the ⩾ 2-year follow-up period.

Type I cryoglobulins are monoclonal immunoglobulins produced due to underlying hematological malignancy. Cryoglobulins spontaneously precipitate from serum and plasma at low temperatures and become soluble again on rewarming to 37 °C. Processing of blood at temperature lower than 37 °C in the laboratory may cause precipitation of cryoglobulins resulting in interferences in the automated cell counter analysis. We report three patients with cryoglobulinemic vasculitis wherein each case had different morphology of cryoglobulin precipitates on peripheral blood film, like needle shaped bluish-gray crystals, amorphous weakly basophilic extracellular deposits extraneously indenting red blood cells and basophilic neutrophilic inclusions respectively. The effect of cryoglobulins on two technologically different automated cell counters based on principles of impedance, Volume-Conductivity-Scatter (VCS) and fluorescence flow cytometry was assessed. This case series provides interesting insight into the varying morphological features of cryoglobulins on May-Grunwald-Giemsa stained blood films and interference caused by cryoglobulins in different automated cell counter analysis resulting in pseudo-leucocytosis, pseudo-thrombocytosis, abnormal histograms and scatterplots. Identification of these hematologic abnormalities and artifacts induced by cryoglobulins is necessary since it may be the first clue leading to the timely diagnosis of cryoglobulinemia and hence the underlying hematological malignancy, as in our cases.

Background: It remains clinically important to identify ER positive breast cancers likely to respond to tamoxifen (TAM) and so we aimed to select a group of biomarkers able to predict response. We also assessed whether data from different sample types [tumor microarrays (TMAs) and core biopsies] or tumor sites could be combined for biomarker studies. Methods: A total of 123 endocrine treatment naïve patients with known ER and HER2 status treated with TAM had paraffin-embedded tumor tissue available either as TMAs (n=102) or core biopsies (n=21). TMA cores were collected from three different tumor sites, two central and one peripheral. Ten biomarkers were evaluated by immunohistochemistry, for % positivity and/or H-Score, comprising: ER, HER2, Ki67, phosphorylated forms of ER (Ser118), IGF1R, PRAS40, Akt & MAPK (ERK1/2), and PTEN & androgen receptor expression (AR). Each tumor was analysed for Akt1 E17K somatic mutation using BEAMing technology. Patient outcome was assessed by clinical benefit (CB) rate & survival analyses [time to progression (TTP) and time to death (TTD)]. Results: There was no significant difference in % positivity or H-Score between central & peripheral tumor sites for all biomarkers examined. After False Discovery Rate (FDR) correction differences (P

Introduction Hereditary angioedema (HAE) is a clinical condition which could be fatal if not identified and managed appropriately. Knowledge of this condition is mostly confined to individual case reports and literature reviews in India. In this retrospective study we describe HAE cases which presented to a tertiary care centre in India over a period of three and half years. Materials and methods Clinical case records of forty-two HAE patients diagnosed based on clinical and laboratory features were accessed after due approval from the Ethics committee. C1 esterase inhibitor (C1 INH) and C4 levels were measured using nephelometry. All relevant data was entered into Microsoft EXCEL worksheet and analysed using simple statistical tools. Results Among the 42 patients diagnosed as having HAE, 37 had low C1INH levels and were diagnosed to have type 1 HAE. The remaining 5 had normal C1 INH levels and were considered probable HAE based on family history and response to HAE specific treatment. The median age of onset of symptoms was 15 years (range 5–49) and median age at diagnosis, 27.5 years (range 5–55). The median delay in diagnosis was 10 years (range 1–27 years). Family history of HAE was observed in 52.6% and 29% reported deaths in the family with HAE like disease. Low dose androgens or tranexamic acid or both were prescribed in 64.2% of the patients. Orofacial edema was the commonest clinical presentation (76%) followed by edema of the extremities (38%), GI tract symptoms (19%) and genital involvement (11.9%). Conclusion Many cases of HAE may be going undetected in India. There is a need for clinical awareness and laboratory means to accurately identify and administer appropriate treatment.

Background & objectives: The pathological hallmark of scrub typhus infection is focal or disseminated vasculitis. As with other infections, antinuclear antibodies (ANA) have been previously described in scrub typhus. However, the underlying mechanisms and implications of this immunological phenomenon is not well understood. In the present work it was assessed whether ANA is associated with illness severity and outcomes. Methods: In this prospective study spanning one year, patients fulfilling the diagnostic criteria for scrub typhus were recruited. Patients with other acute infective febrile illnesses were taken as controls. ANA positivity was compared between the cases and controls. ANA in scrub typhus was assessed for correlation with disease severity, organ dysfunction and outcomes. Results: The cohort comprised of 149 patients (scrub 89; controls 60) with mean age 46.5 (SD=16.9) yr; 48.3% were female. ANA was detected in 48 (53.9%) patients with scrub typhus and 9(15%) controls (p < 0.001). The ANA pattern was predominantly speckled (93.8%) in both scrub typhus patients and controls. In patients with scrub typhus, ANA positivity was associated with increasing APACHE-III score [Odds ratio (OR) 1.01; 95% CI 0.99–1.03; p = 0.09]. On bivariate analysis, ANA tended to be correlated with acute respiratory distress syndrome (OR 2.32; 95% CI 0.98–5.46; p = 0.06), hepatic dysfunction (OR 2.25; 95% CI 0.94–5.39, p = 0.06) and aseptic meningitis (OR 6.83; 95% CI 0.80–58.05, p = 0.08). The presence of these antibodies did not correlate with duration of hospitalization or mortality. Convalescent sera on 31 ANA positive scrub typhus patients demonstrated persistence of ANA in only 5 (16.1%) patients. Interpretation & conclusion: The disappearance of ANA during the convalescent phase suggests that ANA is expressed during the acute phase of scrub typhus infection. Its association with organ dysfunction warrants further study of the mechanisms and impact of autoantibody formation in scrub typhus.

Even though minor degrees of heterotrophic ossification are common in total knee arthroplasty, it is of little clinical significance. But severe degrees of heterotrophic ossification are very rare after total knee arthroplasty. Here we discuss about a 70 years old woman who initially had excellent post-operative range of movements after cemented total knee arthroplasty, but later presented with knee pain, swelling and loss of range of movements after 3 months. X ray showed severe heterotrophic ossification around knee near the quadriceps tendon. She was treated conservatively with non-steroidal anti inflammatory drugs and physiotherapy. After a period of 3 months of physiotherapy, patient regained the lost range of movements and is currently under follow up for the past 1 year. Hence this case instantiates that even in cases of severe Heterotrophic Ossification after total knee arthroplasty, non-operative treatments such as physiotherapy with anti-inflammatory drugs should be the primary option to treat the stiffness before considering surgery.

Mycophenolate mofetil (MMF) is an effective therapeutic agent with high safety profile in the management of lupus nephritis. This retrospective study was conducted to assess the efficacy and side effect profile of MMF as induction as well as maintenance therapeutic agent along with tapering steroids in neuropsychiatric lupus (NPSLE). Hospital electronic medical records of patients with SLE diagnosed by ACR 1990 and/or SLICC 2012 criteria between January 2005 and May 2015 were retrieved. Among them, patients fulfilling ACR 1999 criteria for NPSLE were identified. Data of NPSLE patients treated with MMF as upfront second line immunosuppressive agent, both for induction and maintenance, were analyzed. Of the 140 patients with NPSLE, 88 fulfilled the inclusion criteria. Mean age of the cohort was 25.51 ± 7.82 years with female to male ratio of 84:4. Median duration of follow-up was 33 months (3-129 months). Seizure was the most common NPSLE manifestation (n = 37, 42.05%). Of the 88 patients, 18 had NPSLE solely due to secondary antiphospholipid syndrome. Of the remaining 70 patients, 61 (87.1%) had improved, 7 remained unchanged with no worsening and 3 patients had worsening or developed new symptoms during follow up after 3 months from baseline. At last follow-up, 55 out of 57 patients (97.1%) with detailed data had improved, while 2 patients had relapsed. Side effects were significantly more common in patients on prednisolone as compared to those on deflazacort. In patients with NPSLE, MMF along with tapering steroids is an efficacious combo in inducing remission and preventing relapse of disease.

Background: Arterial inflammation Takayasu arteritis (TA) is an outcome of balance between pro- and anti-inflammatory cytokines. Comprehensive assessment of these cytokines is important for understanding pathogenesis and assessing disease activity. Objective: To study pro- and anti-inflammatory cytokines representing different T-helper cell pathway in serum samples of Asian Indian patients with TA and to assess their association with disease activity. Methods: Consecutive Indian patients with TA were assayed for serum interferon-γ, interleukin-6, interleukin-23, interleukin-17, interleukin-10 and transforming growth factor- β levels at baseline and follow up visit. Patients were grouped into active and stable disease based on Indian Takyasu Arteritis clinical Activity Score-2010. Serum levels of these cytokines between active and stable disease and between baseline and follow up visits were compared by non-parametric tests. Results: Among 32 patients enrolled, 15 were classified as active while 17 as stable disease at baseline. IFN-γ levels were significantly higher in active disease than stable disease (p=0.0129) while other cytokines did not differ significantly between 2 groups. Serum levels of none of the cytokines changed significantly over 2 visits in both responders and non-responders. IL23 levels positively correlate with disease duration ((r=0.999; p Conclusion: IFN-γ levels are raised in active disease in TA and correlates well with other biomarkers of disease activity and proinflammatory cytokines. There is also a direct correlation between Il-23 levels and disease duration.

In most developed nations, endometrial cancer is the most common gynecological malignancy. However, the endometrium is an extremely rare site for metastasis from an extragenital site. Also, the most common presentation of such metastasis is with uterine bleeding, similar to primary endometrial cancers. We present an unusual case of a 38-year-old female who presented with abdominal pain and distension. Evaluation revealed an endometrial mass lesion with bilateral adnexal masses and massive ascites. An endometrial biopsy was done, which revealed a mucinous adenocarcinoma with signet ring cells. Immunohistochemistry showed cells positive for CK20 and CDX2 and negative for CK7, which was in favor of a colonic primary. A colonoscopy was attempted, but failed due to extrinsic compression at the rectosigmoid junction. There was no evidence of supradiaphragmatic disease. She was started on palliative chemotherapy. This case report highlights the indispensability of histological correlation and immunohistochemistry in the diagnosis of pelvic malignancies. How to cite this article Komaranchath AS, Gopalakrishnan S, John S, Mahadevan P, Mathew J, Kumar L. Endometrial Metastasis from an Occult Colonic Primary presenting with Massive Ascites. J South Asian Feder Menopause Soc 2017;5(2):138-141.

Background: There are many clinical and laboratory parameters which have been proven to be associated with increased risk of pregnancy morbidity in patients with SLE and APS. This study was undertaken as there is a lacuna in explaining all the pregnancy related morbidity in this group Objectives: Primary: Correlation of IgA anticardiolipin antibody with the risk for pregnancy morbidity in patients with APS/SLE Secondary: Association of IgA Anticardiolipin antibody with the risk of thromboembolic events Methods: Patients diagnosed as SLE and/or Primary APS who are married and conceived atleast once were recruited and 3ml blood sample was obtained from them. Patients with history of recurrent infections, which could suggest probable IgA deficiency, were excluded. Demographic data including duration of illness, number of pregnancies, thromboembolic events, disease activity were noted. They were categorized into two different groups depending on whether they had a pregnancy morbidity or not. The blood samples were subjected to IgA Anticardiolipin assay by ELISA. Results: A total number of 186 patients were recruited with mean age 34.54(+/-7.32)years, mean duration of illness of 5.52(+/-4.31)yrs with a diagnosis of 76.88% SLE, 7.53% primary APS and 15.59% SLE with secondary APS. On assessment of disease activity, mean SLEDAI score was found to be 4.46+/-6.32. Out of the total, 14.71% patients had conceived once, 14.56% twice, 16.72% patients thrice and 18.41% had conceived more than three times. 36.56% patients had pregnancy morbidity (majority being pregnancy induced hypertension 14.52%, prematurity 11.29%, IUGR 10.22%, pre-ecclampsia in 3.76%, eclampsia 1.08%,), 19.35% patients had history of thromboembolic events (6.45% pulmonary embolism, 5.38% deep vein thrombosis, 3.76% visceral vessel thrombosis, 0.54% cortical vein thrombosis, 6.99% other vessel thrombosis). 33.87% patients had positivity for antiphospholipid antibodies with 27.82% lupus anticoagulant positive, 20.97% IgG Anticardiolipin positive and 17.35% anti-beta2 glycoprotein positive. IgA Anticardiolipin was found to be positive in 4.84%(n=9) out of which, 10.29% were in the group with pregnancy morbidity as against 1.69% without pregnancy morbidity. Out of the total positives titre in 37.5% were indeterminate, 25% low positive and 37.5% high positive. All patients who had high titre positivity had pregnancy morbidity. The positivity of IgA anticardiolipin in the patient group with thromboembolic events was 5.56%. Conclusion: Among patients with SLE, APS or SLE and APS, the overall prevalence of IgA anticardiolipin antibody was found to be about 4.84%, which is much less as compared to the percentages reported by other groups[1] IgA Anticardiolipin antibody positivity was more in patients with pregnancy morbidity compared to patients without a pregnancy morbidity. In this study however, statistical significance was not reached. Similar studies with larger number of patients are required. We also need to prospectively follow up such patients with IgA ACLA and evaluate for morbidity during pregnancy References [1] Clinical significance of IgA anticardiolipin and anti-beta2-GP1 antibodies in patients with systemic lupus erythematosus and primary antiphospholipid syndrome, Clin Rheumatol. 2006 Mar;25(2):199-204 Disclosure of Interests: None declared

Tumors of the accessory parotid gland are very rare. Surgical removal of an accessory parotid tumor is usually accomplished by superficial parotidectomy through an external neck incision. However, this procedure inevitably results in a neck scar. In this case, we performed complete excision of a parotid tumor via an endoscopic-assisted transoral approach. Resection of such benign tumors can be achieved with less morbidity by endoscope-assisted surgery with a nerve monitoring system. The field of transoral surgery will continue to expand with technological advancements.

Background: Thoracoscopic oesophageal mobilisation during a minimally invasive oesophagectomy (MIE) is most commonly performed with the patient placed in the lateral decubitus position (LDP). The prone position (PP) for thoracoscopic oesophageal mobilisation has been proposed as an alternative. Materials and Methods: This was a retrospective, comparative study designed to compare early outcomes following a minimally invasive thoracolaparoscopic oesophagectomy for oesophageal cancer in LDP and in PP. Results: During the study period, between January 2011 and February 2014, 104 patients underwent an oesophagectomy for cancer. Of these, 42 were open procedures (transhiatal and transthoracic oesophagectomy) and 62 were minimally invasive. The study group included patients who underwent thoracolaparoscopic oesophagectomy in LDP (n = 23) and in PP (n = 25). The median age of the study population was 55 (24-71) years, and there were 25 males. Twenty-one (21) patients had tumours in the middle third of the oesophagus, 24 in the lower third, and 3 arising from the gastro-oesophageal junction. The most common histology was squamous cell cancer (85.4%). The median duration of surgery was similar in the two groups; however, the estimated median intraoperative blood loss was less in the PP group [200 (50-400) mL vs 300 (100-600) mL; P = 0.01)]. In the post-operative period, 26.1% patients in the LDP group and 8% in the PP group (8%) developed respiratory complications. The incidence of other post-operative complications, including cervical oesophagogastric anastomosis, hoarseness of voice and chylothorax, was not different in the two groups. The T stage of the tumour was similar in the two groups, with the majority (37) having T3 disease. A mean of 8 lymph nodes (range 2-33) were retrieved in the LDP group, and 17.5 (range 5-41) lymph nodes were retrieved in the PP group (P = 0.0004). The number of patients with node-positive disease was also higher in the PP group (19 vs 10, P = 0.037). Conclusion: MIE in the PP is an effective alternative to LDP. The exposure obtained is excellent even without the need for a complete lung collapse, thereby obviating the need for a double-lumen endotracheal tube. A more meticulous dissection can be performed resulting in a higher lymph nodal yield.

A 12 year old boy presented initially with symptoms suggestive of urinary tract infection (UTI). Later, it turned out to be acute appendicitis with appendicular abscess. Pre-operative investigations revealed co-presentation of pheochromocytoma. Although this child was having no symptoms of pheochromocytoma, the presentation of phaechromocytoma with appendicular abscess needs to be dealt with extreme vigil as pheochromocytoma can result in severe sympathetic outburst storm. Laparoscopic appendicectomy was done and the child was discharged after complete recovery.

Background:Idiopathic inflammatory myositis (IIM) are a heterogeneous group of immune-mediated disorders with varied presentations and multiple organ involvement. Data on long term outcome among South Asian patients with IIM is sparse.Objectives:To study the long term clinical outcome, treatment responses and factors predicting outcome among adult patients with IIMMethods:Patients diagnosed as ‘Idiopathic Inflammatory Myositis’ under the department of Clinical Immunology and Rheumatology at CMC, Vellore, India were screened retrospectively. Patients aged 18 years and above, satisfying Bohan and Peter criteria, having follow up of one year or more with atleast two outpatient or inpatient visits between January 2010 and April 2019 were included in this study. Those patients with connective tissue disease associated myositis were not included. Details on muscle weakness, extramuscular involvement, muscle enzymes and treatment administered were recorded at baseline, 3, 6, 12, 18, 24 months and yearly thereafter. After assessing their cumulative response, categorization of patients into complete and partial responders was done. Complete responders were defined as patients with persistent muscle power of more than 4/5 and/or MMT 8 more than 76/80, complete resolution of skin, articular and lung involvement (if any) as well as muscle enzymes less than twice the upper limit of normal without any documented flares during the entire follow up period. Patients not satisfying the said criterias were grouped as Partial responders. Disease free survival duration was also analyzed.Results:Out of 310 patients of IIM identified, 187 (60.3%) patients satisfied the inclusion criteria. Women were 2.2 times more than men and mean age at symptom onset was 35.7±12.6 years. Dermatomyositis was the predominant myositis subtype seen. All patients were put on steroids with the mean dose being 45.9 ± 18.6 mg/day. At baseline, the key immunosuppressants used were methotrexate in 44.9% and mycophenolate in 37.6% patients. The median follow up duration was 48 (25-80) months. An associated malignancy was diagnosed in 3.2% after a median duration of 24.5 months. Five patients expired after a median duration of 80 months from diagnosis. Normal muscle power was attained in 76.1% patients and 88.6% were vocational by the last follow up visit. Steroids were discontinued in 56.7% patients after a median duration of 24 months (p=0.0002). Discontinuation of the immunosuppressant was feasible in 10.2% patients after a median duration of 44 months. Assessment of the cumulative responses revealed a relapsing and remitting course in 45.9%. Outcome predictors in univariate analysis were Jo-1 status, presence of arthritis, interstitial lung disease and pericardial effusion at baseline. On multivariate analysis, absence of pericardial effusion (p=0.011) and interstitial lung disease (p=0.067) at baseline were found to be predictors of complete response. Disease free survival probability estimated at 5 years and 10 years was 91.6% and 72.4% respectively. Estimating the probability gender wise, males achieved disease free status earlier than females.Conclusion:A favorable clinical and functional outcome was seen in a significant proportion of these patients with IIM on long term follow up. Pericardial effusion and ILD were identified as predictors of poor clinical outcome.References:[1]Taborda AL, Azevedo P, Isenberg DA. Retrospective analysis of the outcome of patients with idiopathic inflammatory myopathy: a long-term follow-up study. Clin Exp Rheumatol. 2014 Apr; 32(2):188–93.Acknowledgments:NilDisclosure of Interests:None declared

A 51-year-old female with mixed connective tissue disease presented with bilateral sensorineural hearing loss. The hearing deficit was gradually progressive over a period of 6 months. On evaluation, no obvious cause for hearing was evident. Due to ototoxic potential of hydroxychloroquine, we decided to stop the drug and observe for improvement. Over time, her hearing loss stabilized, with improvement in audiometric findings. Hydroxychloroquine induced auditory toxicity is rare. Increased awareness and early recognition may minimize damage.

Context: There is heterogeneity in the clinical behavior of breast carcinoma patients with node negativity. Studies have analyzed different factors influencing the outcome in such patients. It is suggested that the presence of nodal micrometastasis can act as a tool in predicting the aggressiveness of these tumors. Aims: The aim of this study is to assess the yield of micrometastasis/isolated tumor cell (ITC) by ultrastaging the morphologically negative axillary nodes and staining them with immunohistochemistry for epithelial membrane antigen. The association of such metastasis with the clinical outcome is determined. Settings and Design: This was a retrospective analytical study. One hundred cases of node-negative breast carcinoma patients who underwent surgery along with axillary lymph node dissection were selected. Materials and Methods: The largest node from the axillary dissection was selected and subjected to ultrastaging and immunohistochemical staining (as sentinel node dissection was not a routine practice at that time), to look for occult metastasis in the form of micrometastasis or ITCs. Statistical Analysis: Occurrence of events in the form of recurrence or death was noted. Association of the parameters was analyzed using Fisher's exact test. Results: Among the 100 cases, 79 patients were followed up for a minimum period of 5 years. Two cases had micromets in one node each. These two patients were among the eight, who developed events subsequently (25%). Hence, a statistically significant association was found between the presence of micromets with events. Conclusions: There is a statistically significant association between the presence of micromets and disease recurrence. Hence, we suggest that ultrastaging of the negative axillary node (now sentinel node, as it is being routinely done) might prove effective in predicting the events/prognosis in clinically and morphologically node-negative breast carcinoma patients

Antiphospholipid syndrome (APS) is the most common acquired pro-thrombotic disorder, also associated with obstetric complications. Phosphatidylserine/Prothrombin complex antibody (aPSPT) though associated with various APS manifestations, is not included in the revised Sapporo Criteria. To study the prevalence of aPSPT in Asian-Indian patients with suspected APS and compare its performance with the criteria anti-phospholipid antibodies (APLs). Electronic charts of 372 individuals whose sera was tested for aPSPT in suspected APS between June 2014 and May 2016 were retrieved and analyzed. aPSPT was assayed by ELISA. aPSPT tested individuals were categorized into cases—seropositive and seronegative APS (SNAPS) and controls. aPSPT was positive in 24/58 (41.3%) cases and 17/314 (5.4%) controls (p

The aim of this study is to analyze the outcomes of patients with chronic pancreatitis who underwent the Frey procedure and who had a histologic evidence of adenocarcinoma in the cored out specimen.The type of analysis is retrospective. Out of 523 patients who underwent Frey procedure for chronic pancreatitis, seven (five males and two females; age range 42 to 54 years) had histologically proven adenocarcinoma. In the first four cases, intraoperative frozen section was not done. The diagnosis was made on routine histopathology and only one out of four could undergo attempted curative therapy. In the remaining three cases, intraoperative frozen section confirmation was available, and curative resection performed. Only four out of seven had a clear-cut mass lesion: (a) cancer can occur in chronic pancreatitis in the absence of a mass lesion and (b) intraoperative frozen section of the cored specimen is crucial to exercising curative therapeutic options and must be performed routinely. If frozen section is reported as adenocarcinoma, a head resection with repeat frozen of the margins of resection is appropriate. If the adenocarcinoma is reported on regular histopathology after several days, then a total pancreatectomy may be more appropriate.

We report a case of a 63 years old man who presented with a profusely bleeding mass in the left external auditory canal. He had been diagnosed to have left skull base osteomyelitis and had undergone surgery twice for the same. The mass was diagnosed radiologically to be a pseudoaneurysm arising from the left retroauricular artery. He subsequently underwent embolization of the retroauricular branch of the left external carotid artery following which the mass subsided and bleeding from the ear stopped. How to cite this article John BT, Augustine AM, Lepcha A, Mathew J, Moses V. Bleeding Mass in the Ear: A Rare Etiology. Int J Otorhinolaryngol Clin 2015;7(3):144-146.

Background Surgeons and endoscopists welcome routine preoperative biliary drainage prior to pancreaticoduodenectomy despite evidence that it increases complications. Its effect on postoperative pancreatic fistula is variably reported in literature. Simultaneous biliary and pancreatic drainage is rarely performed for very selected indications and its effects on postoperative pancreatic fistula are largely unknown. Our aim was to analyze the same while eliminating confounding factors. Methods Retrospective single center cohort study of patients who underwent pancreaticoduodenectomy over the past 10 years for carcinoma obstructing the lower common bile duct. Patients who underwent biliary stenting alone, biliary and pancreatic stenting, and no stenting prior to pancreaticoduodenectomy were the three study cohort groups and their records were scrutinized for the incidence of postoperative pancreatic fistula. Results Sixty-two patients underwent biliary stenting alone, 5 patients underwent both biliary and pancreatic stenting, and 237 patients were not stented in the adenocarcinoma group without chronic pancreatitis. The pancreatic fistula rate was similar in the patients who underwent biliary stenting alone when compared with the group which was not stented. (24/62 versus 67/237, odds ratio [OR] =0.619, confidence interval (CI) =0.345–1.112, p = 0.121). However, the patients who underwent both biliary and pancreatic stenting had a significant increase in postoperative pancreatic fistula compared with the not stented (p = 0.003). By univariate and multivariate analysis using Firth logistic regression, pancreatic texture (OR = 1.205, CI = 0.103–2.476, p = 0.032) and the presence of a biliary and pancreatic stent (OR = 2.695, CI = 0.273–7.617, p = 0.027) were the significant factors affecting pancreatic fistula. Conclusion Preoperative biliary drainage alone has no significant influence on postoperative pancreatic fistula except when combined with pancreatic stenting. We need more such studies from other centers to confirm that the rare event of preoperative biliary and pancreatic stenting has indeed this harmful effect on healing of postoperative pancreatic anastomosis.

Background Rheumatoid vasculitis (RV) is a severe extra-articular manifestation of rheumatoid arthritis (RA), with high morbidity and mortality reported in literature Objectives To describe the Asian Indian perspective on RV patients, their clinico-laboratory features and their outcome along with the factors affecting them Methods A retrospective review of electronic medical records of 8984 RA patients from January 2007 to August 2016, was done for those satisfying Scott & Bacon criteria for RV 1 . Probable RV was defined as patients not satisfying Scott & Bacon Criteria, but were managed like RV after exclusion of alternate diagnosis. Birmingham Vasculitis activity score (BVAS) version 3 2 was used for monitoring activity of RV Results 63 patients of RV were identified, with a study period prevalence of 0.7%, in our RA cohort. 33 (52.4%) patients were female. Mean age of patients was 50.7±11.5 years with median duration of RA being 6 years. Involvement of Peripheral Nervous System (PNS) was the commonest manifestation of RV in 52/63 (82.5%) patients followed by skin in 34/63 (53.9%) patients. Rheumatoid Nodule was seen in 14/ 63 (22.2%) patients. Percentage of current and ex-smokers combined,was same as rheumatoid nodule prevalence. 52 (82.5%) patients had biopsy evidence of vasculitis.26/51 (50.9%)patients were started on mycophenolate mofetil, 13/51 (25.5%)patients on cyclophosphamide, 8/51 (15.7%) patients on azathioprine, 4/51 (7.8%) patients on Methotrexate as immunosuppressive (IS) agent along with mean dose of 46.6±13.7 (0.86±0.23mg/kg/day) prednisolone. Additionally, Rituximab & IVIg were used in 2 patients each respectively. 3 months after initiation of immunosuppression 26/50 (52%) patients on follow-up were in remission and 39/47 (82.9%) patients attained remission at 6 months. Mean time to achieve remission was 151.1±86.3 days. All IS agents were equally effective in inducing remission at 3 and 6 months and showed statistically similar BVAS reduction at 3 and 6 months from baseline (t test & chi-square test). 7 (11.2%) deaths noted in the cohort at their respective last visit during 195.3 patient years cumulative follow up. Multiple regression analysis showed that at baseline, presence of PNS involvement, eosinophilia, thrombocytosis, higher BVAS score and higher steroid requirement were predictors of persistently active vasculitis and absence of eye involvement and higher hemoglobin % at baseline were predictors for remission, at 3 months (p Conclusions Our cohort of Asian Indian RV was comparitively younger with lesser RA duration, less percentage of ever-smokers, lesser rheumatoid nodule prevalence, higher PNS involvement with better survival/mortality rates compared to published literature. All IS agents showed equal rates of BVAS remission & BVAS reduction at 3 and 6 months of treatment References Scott DG, Bacon PA. Intravenous cyclophosphamide plus methylprednisolone in treatment of systemic rheumatoid vasculitis. Am J Med. 1984 Mar;76(3):377–84. Mukhtyar C, Lee R, Brown D, Carruthers D, Dasgupta B, Dubey S, et al. Modification and validation of the Birmingham Vasculitis Activity Score (version 3). Ann Rheum Dis. 2009 Dec;68(12):1827–32. Disclosure of Interest None declared

Background and aims Currently we do not have a biomarker that can closely reflect the renal disease activity. So the aim of this study is to study the utility of urinary VCAM 1(Vascular cell adhesion molecule 1) in lupus nephritis. Methods It was a diagnostic case control study. The patients presenting to Rheumatology outpatient department were recruited. Patients were divided into 2 groups, SLE without active nephritis and SLE with active nephritis based on the renal SLEDAI. Urinary VCAM1 was tested in all patients using an early morning spot urine sample using ELISA. Renal biopsy was done in patients with active nephritis. VCAM1 levels were compared with the renal SLEDAI, renal biopsy disease activity (ISNRPS) and standard of care markers. The results were analysd using SPSS software version 16.The validity and predictive value statistics was presented with 95 percent confidence interval. Results Urinary VCAM 1 levels had significant correlation (p=0.01) with disease activity based on renal SLEDAI. However, the correlation between the biopsy findings and VCAM levels was not statistically significant. Class 4 and 5 lupus nephritis had higher VCAM level than the lower classes. A positive correlation (r=0.38) was found between VCAM 1 and double stranded DNA. There was a negative correlation between C3 value and VCAM (r=−0.19). The sensitivity and specificity of urinary VCAM 1 is 65.22% and 75% respectively. The cut off value of VCAM is 23.8 pg/mg of creatinine. Conclusions Urinary VCAM 1 may not independently, but combined with other markers may be a promising biomarker for disease activity in lupus nephritis.

Background and aims To explore utility of Antibodies to Phosphatidylserine/Prothrombin Complex (aPSPT), in Indian Anti phospholipid Syndrome (APS) patients. Methods Data of 372 subjects whose sera was tested for aPSPT by ELISA (AESUKU,GERMANY), was retrospectively analysed. Performance of various APLs was compared using non-parametric tests. To ascertain additional utility of aPSPT, patients with suspected APS were categorised into 2 groups i.e. i) those with any one of criteria antibodies positive ii) those with any one APL positivity including anti- PSPT positivity. Results In 46 patients of APS, 38 (82.60%) had thrombotic events (21 arterial , 26 venous) and 11 ( 23.91%) had obstetric events. Lupus anticoagulant was present in 37/46 (80.43%) ,anticardiolipin antibody in 19/46 (41.33%), aPSPT in 17/46 (36.95%), anti-β2glycoprotein1 (β2GP1) IgG and IgM in 13/46 (28.26%) and 7/46 (15.21%) patients of APS respectively. aPSPT tested positive in 9/60 (15%) patients with seronegative APS (SNAPS). aPSPT did not differ from other criteria APLs in any clinical parameter except compared to those with IgM B2GP-1 positivity. aPSPT was numerically more sensitive and specific than IgM B2GP1 for thrombotic, non-criteria events but less sensitive for detecting obstetric events (Table 2). Inclusion of aPSPT test in patients with suspected APS increased sensitivity marginally but with concomitant decrease in specificity. Conclusions In our study, aPSPT was third most common antibody in APS patients, displaying higher sensitivity and specificity in comparison to IgM anti-β2 GP1 for all APS manifestations except obstetric ones. SNAPS patients may test positive for aPSPT, thus endorsing its inclusion in classification criteria of APS.

Clinical diagnosis of autoinflammatory diseases requires a high degree of clinical suspicion and clinching molecular evidence to substantiate the diagnosis. This is more so in populations with low prevalence of these disorders. In this report, we describe the case of a young man from India with recurrent fever and persistent arthritis. The patient's forefathers were of Egyptian ancestry who practiced consanguinity. Molecular genetic analysis using whole-exome sequencing suggested the presence of variants c.443A>T:p.E148V and c.442G>C:p.E148Q in the MEFV gene, earlier independently shown to be associated with familial Mediterranean fever (FMF) in a compound heterozygous state. The variants were further confirmed by capillary sequencing. This report also highlights the application of whole exome sequencing to delineate the allelic differences in the variants apart from serving as a quick genetic screening approach for autoinflammatory diseases. To the best of our knowledge, this is the first report of a compound heterozygosity for the two well-characterized variants associated with atypical FMF in a patient.

Pancreatic cancer continues to be a life-threatening disease with poor long term outcomes despite various treatments. Progress has been slow, although results of surgery have improved, and mortality rates have fallen. Surgery is still the modality with the highest potential to cure pancreatic cancer. We examine some of the key issues relating to the treatment of pancreatic cancer, largely to the description of issues related to pancreatic head cancer.

Objective To determine the prevalence of laryngopharyngeal reflux (LPR) in patients with voice disorders and to find out the efficacy of reflux symptom index (RSI) and reflux finding score (RFS) in the diagnosis. Methods This prospective analytical study was conducted on 554 patients who presented with dysphonia and symptoms suspicious of LPR. They were then subjected to clinical work up based on symptomatic history based RSI and video stroboscopic RFS scoring criteria. The results were analyzed statistically. The differences between RSI and RFS were calculated by Fisher's two sided test and the comparison between the two is calculated using kappa statistics to check the strength of agreement. Results Among 554 patients, 457 (82.4%) patients were diagnosed to have LPR based on RSI score of more than 13. Among 448 (80.8%) patients RFS score was positive. Vocal nodule (23.64%), laryngitis (22.38%) and the vocal polyp (20.03%) constitute the majority, with a female preponderance in vocal nodule. Both RSI and RFS were poor or almost absent in conditions such as spasmodic dysphonia, tremors, vocal cord palsy, hemorrhage, functional aphonia and traumatic conditions. The Pearson correlation coefficient showed a good correlation and agreement between RSI and RFS was statistically significant. Conclusion The association of LPR in patients with voice disorders is significantly high. The RSI and RFS based clinical diagnosis is reliable and valid in the diagnosis of LPR as revealed in our study. How to cite this article Sebastian S, Kolethekkat AA, Mathew J, Gowri M. Laryngopharyngeal Reflux in Dysphonics— Understanding the Significance and the Efficacy of Clinical Diagnosis: A Case-based Study. Int J Phonosurg Laryngol 2014;4(1):5-9.

Context: Pancreatic necrosis is a local complication of acute pancreatitis. The development of secondary infection in pancreatic necrosis is associated with increased mortality. Pancreatic necrosectomy is the mainstay of invasive management. Aims: Surgical approach has significantly changed in the last several years with the advent of enhanced imaging techniques and minimally invasive surgery. However, there have been only a few case series related to laparoscopic approach, reported in literature to date. Herein, we present our experience with laparoscopic management of pancreatic necrosis in 28 patients. Materials And Methods: A retrospective study of 28 cases [20 men, 8 women] was carried out in our institution. The medical record of these patients including history, clinical examination, investigations, and operative notes were reviewed. The mean age was 47.8 years [range, 23-70 years]. Twenty-one patients were managed by transgastrocolic, four patients by transgastric, two patients by intra-cavitary, and one patient by transmesocolic approach. Results: The mean operating time was 100.8 min [range, 60-120 min]. The duration of hospital stay after the procedure was 10-18 days. Two cases were converted to open (7.1%) because of extensive dense adhesions. Pancreatic fistula was the most common complication (n = 8; 28.6%) followed by recollection (n = 3; 10.7%) and wound infection (n = 3; 10.7%). One patient [3.6%] died in postoperative period. Conclusions: Laparoscopic pancreatic necrosectomy is a promising and safe approach with all the benefits of minimally invasive surgery and is found to have reduced incidence of major complications and mortality.

IgG4-related disease (IgG4-RD) is a systemic fibro-inflammatory disease. This disease may be associated with elevated serum and tissue IgG4 levels. Early treatment prevents fibrosis and organ damage. We retrospectively studied the clinicopathologic correlation and outcome of treatment in IgG4-RD. This single-center retrospective study was done using electronic records of patients subjected to assay of serum IgG4 levels in our laboratory by nephelometry. There were 473 patients with suspected IgG4-RD. Of them, 41 patients fulfilled comprehensive diagnostic criteria for IgG4-RD and 432 had diseases other than IgG4-RD. Clinical and histopathological data including tissue IgG4/IgG ratio, other relevant laboratory findings as well as management data of 41 patients with IgG4-RD were analyzed. There were 29 males and 12 females with mean age of 44.1 ± 2.19 years. Thirteen patients had definite, 19 had probable and 9 had possible IgG4-RD. Male predominance, multiple organ involvement and IgG4 responder Index were significantly higher in definite IgG4-RD as compared to probable and possible IgG4-RD. Serum IgG4 level was elevated in 37 patients (90.2%). Glucocorticoids were used in 35 patients (85.4%) and second-line immunosuppressive agent in 23 patients (65.7%). Of the 21 patients on follow-up, 19 (90.7%) had clinical improvement at the first follow-up visit. Nine (90%) out of the ten patients who were assessed by IgG4 responder index, also had shown improved score with treatment. Patients with IgG4-RD in our series showed favorable responses to treatment with glucocorticoids and addition of steroid sparing immunosuppressive agents (mainly mycophenolate mofetil) helped successful tapering of steroids, while maintaining the improvement.

Introduction Throughout the world, laparoscopic cholecystectomy is a widely accepted surgical treatment for both acute and chronic cholecystitis. It provides total relief of pre-surgical symptoms in up to 85% of patients. However, about 5% of patients may experience severe episodes of upper abdominal pain similar to those that they had prior to cholecystectomy; this is known as post-cholecystectomy syndrome. Gallbladder remnant with calculi is one of the causative factors. However, there have been only a few case series related to this reported in literature to date. Herein, we present our experience with laparoscopic management of gallbladder remnant with calculi in 40 cases. Methods A retrospective study of 40 cases was carried out in our institution. All patients underwent open cholecystectomy at other centres, and their cases were managed by laparoscopic completion cholecystectomy. Results The mean operating time was 102.4 min (range, 60–120 min). The duration of hospital stay was 2–4 days. Two cases were converted to open surgery because of extensive dense adhesions. One case had minor a common bile duct injury, and another had port-site infection. There were no cases of mortality. Conclusion Gallbladder remnant containing stones may be the cause of otherwise unexplained postcholecystectomy pain. Completion cholecystectomy offers a definitive treatment for any residual gallbladder remnant and can be performed laparoscopically.

Pneumomediastinum has been described as a rare complication of connective tissue diseases. Here, we report four cases of pneumomediastinum: three of which are associated with dermatomyositis and one with mixed connective tissue disease. All our patients had interstitial lung disease. The first case of dermatomyositis described below was complicated by epidural emphysema (pneumorrhachis) in addition to pneumomediastinum. Pneumorrhachis is reported in many isolated case reports and series in the setting of asthma, pneumothorax, blunt chest trauma, etc. Less than 10% of pneumomediastinum cases develop this complication and vast majority of cases resolve spontaneously. The mechanism behind this has been postulated to be the passage of air through the intervertebral foramen. Others suggest entrapment of air which dissects between paraspinal soft tissues and along the vascular and nerve sheaths into the epidural space. This is the first ever reported case of epidural emphysema in connective tissue disease to the best of our knowledge.

Background: Sickle cell disease (SCD) is characterized by intermittent episodes of vascular occlusion and end-organ damage. Neurologic symptoms are frequent and auditory involvement is not unexpected. Aim: To study the prevalence and pattern of hearing loss in Omani patients with SCD. Methods: We conducted a prospective case control study on SCD patients attending the outpatient department. Age and sex matched normal volunteer blood donor controls were recruited after an informed consent and Medical Ethics Committee approval. Pure tone audiometry was performed in all cases studied. Results: Forty-six SCD patients (15 males, 32.6%) aged 16–45 years with a mean age of 26 years ± 6.9 and 29 controls (10 males, 34.4%) aged 16–39 years with a mean age of 25.24 ± 8.2 were enrolled in this study. The average hearing thresholds of SCD patients were consistently higher than controls in all frequencies tested in both ears. Of the 92 ears tested in SCD patients, 29.34% had SNHL. Ten patients had bilateral SNHL; whereas 3 and 4 cases had SNHL in left and right ears, respectively. All the control subjects had hearing thresholds within normal limits. Summary/Conclusions: The study reveals a significant incidence of sensorineural hearing loss (SNHL) in SCD patients (36.95%), although the patients were clinically asymptomatic. The hearing loss was worse in the right ears and had a female preponderance. Also, the hearing loss was more severe at the higher frequencies, 2,000–8,000 Hz in SCD patients. HbS, HbF, or low hemoglobin levels did not discriminate SCD patients with SNHL, and the role of hemoglobin F in the cochlea is still not clear. Regular audiometric assessment should therefore be recommended in SCD patients routinely. Laryngoscope, 2011

Thyroglossal duct carcinoma (TGDC) is a rare disease with few reported series. No clear consensus exists regarding further management after adequate excision of the cyst, especially the role of total thyroidectomy and postoperative radioiodine therapy. We review our experience of nine cases and the literature to clarify the management of this rare condition. A retrospective study over a period of 10 years was performed using the clinical records from Christian Medical College, Vellore to identify patients with TGDC and to assess the frequency of cases with concomitant papillary carcinoma of the thyroid. The clinical presentation, fine needle aspiration cytology (FNAC), imaging, treatment and follow-up were analyzed. There were a total of nine cases of TGDC with five males. Imaging available in six patients detected a suspicious lesion in four cysts and three thyroid glands. Preoperative FNAC detected atypical cells in two of five cases. Thyroid carcinoma was seen in four (44.4%) after histopathological evaluation. Ultrasound of the neck and image-guided FNAC of the cyst may be adequate initial investigation for thyroglossal cysts. FNAC by itself is not a good investigation to diagnose TGDC as rate of false-negatives and inadequate specimens were high. Solid components and calcification on imaging were predictive of carcinoma within a thyroglossal cyst in 100% cases. All patients with TGDC may be offered total thyroidectomy as a simultaneous or staged procedure to address the high incidence of concomitant thyroid foci of cancer. The role of adjuvant therapy is still debatable.

Background: Symptomatic hydrocephalus due to vertebrobasilar dolichoectasia is a rare occurrence. Case Description: We report a patient who presented with acute confusion and vomiting. Neuroimaging revealed elongated and tortuous basilar artery indenting and elevating the floor of third ventricle causing obstructive hydrocephalus. Initially, the patient was treated with external ventricular drain and then with ventriculo-peritoneal shunt. Conclusion: We suggest prompt surgical intervention upon diagnosis as a first choice of treatment in order to avoid further complications.

Mycophenolate mofetil (MMf) has recently been reported as a useful alternative immunosuppressive drug in autoimmune diseases. There is paucity of literature on its use in Takayasu's arteritis (TA). The aim of this study was to assess the safety and efficacy of MMf in Asian Indian patients with Takayasu's arteritis. Records of 21 consecutive patients with TA on treatment with oral MMF attending our centre from January 2005 to August 2008 were studied. The clinical, laboratory and angiography findings were noted and disease activity assessment was done using Indian Takayasu's arteritis activity score (ITAS) and physician's global observation score at baseline and last follow-up. Eleven patients were on steroids alone at baseline while ten patients had received azathioprine prior to administration of mycophenolate. The mean duration of follow-up on mycophenolate was 9.6 (±6.4) months. Nineteen patients (90%) received mycophenolate due to active disease, while in the other two patients, it was given to facilitate steroid tapering. Mycophenolate had to be discontinued in one patient due to skin rash. At the last visit, all the remaining 20 patients who continued mycophenolate had improvement in disease activity as evident by the drop in median ITAS [7 (range 0–19) versus 1 (range 0–7); p = 0.001]. A similar trend was noted in laboratory markers of inflammation with a reduction in mean Erythrocyte Sedimentation Rate (ESR) (68 ± 36.5 versus 43.2 ± 34 mm/first hour; p = 0.003) and mean C - Reactive protein (CRP) (31 ± 46.7 versus 17.3 ± 23.9 mg/L; p = 1.00). All patients received concomitant steroids, but there was a significant decrease in steroid dosage from 36 (±16) mg/day at baseline to 19 (±14) mg/day at last follow-up (p

Aim: The study aimed to examine the perceptions of illness in self and among others in patients with bipolar disorder in remission. The effect of a structured educational programme on the perceptions of illness was also tested. Method: We examined the perceptions of illness in self and in others (using a vignette) among patients with bipolar disorder in remission attending the Department of Psychiatry, Christian Medical College, Vellore, India. We also examined the effect of a structured educational programme on explanatory models in a randomized controlled trial. Explanatory models were assessed using the Tamil version of the Short Explanatory Model Interview. Results: Eighty two subjects were recruited for the trial; half of them received structured education while the other half received treatment as usual. There was agreement between perceptions related to the individual’s own illness and their opinion of illness in others as assessed using a vignette at baseline and at follow-up. There were no significant differences in explanatory models between patients who received education and those who did not. Conclusions: The results of this study show that during periods of remission, patients can clearly see the relationship between their own illness and that described in others, suggesting that insight is state dependent and may be related to psychopathology with good recovery of insight during periods of remission. These findings also argue for the fact that the current multi-dimensional models of insight which focus on biomedical explanations and treatments are not culturally sensitive. The assessment of insight demands universal conventions with comparison to the local cultural standards rather than universal defi nitions and yardsticks which employ Western and biomedical perspectives.

Relapsing polychondritis (RP) is a rare recurring inflammatory disorder with variable clinical course. It has been described mainly in Caucasian population. Reports from other ethnic groups are few. We report seven cases of relapsing polychondritis in south Indian population. In between 1995 and 2008, seven patients fulfilling the McAdam-Damiani-Levine criteria for diagnosis of relapsing polychondritis were identified. Records pertaining to these patients were studied and clinical presentation, course, and treatment offered were analyzed retrospectively. The female-to-male ratio in our series was 2.5:1. The age of onset of symptoms ranged from 28 to 54 years, with a mean of 40.2 years. An average of 20 months, ranging from 3 months to 6 years, elapsed before the patient presented to us seeking a diagnosis. Various structural involvement in our series were as follows: pinna in four (57%), nasal cartilage in five (71%), joints in three (43%), eyes in three (43%), laryngotracheal tree in three (43%), inner ear in one (14.3%), skin in one (14.3%), and heart in one (14.3%). Associated autoimmune diseases were present in four (57%) patients in the form of one of the following in each: vasculitis, autoimmune hemolytic anemia, hypothyroidism, and rheumatoid arthritis. All seven patients received prednisolone with three of them requiring additional immunosuppressants. There was no mortality amongst the four patients who had remained on follow-up at the time of this report. Although RP is an uncommon disorder, clinicians should be aware of the manifestations so as to initiate prompt treatment and prevent complications. Our series reports less frequent auricular cartilage and skin involvement and an exceptional case of basal cell carcinoma, although the other manifestations were similar to that seen in Caucasian and other Asian populations.

Idiopathic hypoparathyroidism can rarely present with extensive spondyloarthropathic changes that closely resemble ankylosing spondylitis, diffuse idiopathic skeletal hyperostosis, or fluorosis. Clinical presentation of the patient, typical laboratory parameters, and radiological findings aid in their differential diagnosis. Extensive spondyloarthropathy with normal sacroiliac joints in patients with neuromuscular symptoms like tetany, paresthesia, and hypocalcemia should raise the possibility of this entity.

For thousands of years, women simply tolerated the distressing symptoms generated by rectovaginal fistulas (RVFs). This is no longer necessary because most RVFs can be surgically corrected via a number of approaches. Most rectovaginal fistulas are acquired; obstetric injury alone accounts for nearly 88% of the cases. The high fistulas are repaired by abdominal approach, while middle or low fistulas are best approached perineally. There are only few case reports of laparoscopic RVF repair noted in literature till date. Laparoscopic repair of RVF is challenging and requires advanced laparoscopic skill. Laparoscopy is a better alternative in selected cases of RVF and yields faster recovery and good patient compliance. We present a case of high RVF managed laparoscopically by using stapler.