Patient Consent Forms Research Articles

Tuberculoid granuloma in bone marrow biopsy can be a rare cause of pancytopenia

Tuberculoid granuloma in bone marrow biopsy can be a rare cause of pancytopenia

A case of tumoral calcinosis in hemodialysis

Dear Editor, Tumoral calcinosis is a rare syndrome which presents as painless, firm, tumor-like masses around the joints leading to pain, and limitation of joint movements. Commonly affected joints are shoulders, elbows, and hip joints. Spinal, temporomandibular joint, metacarpals, and metatarsal involvement have also been described. A 50-year-old female on twice weekly maintenance hemodialysis for the last 3 years presented with complaints of pain and restriction of movements at right hip joint and knee. On examination, range of movements was restricted at the hip joint. On palpation, there were subcutaneous hard nodules in the right hip region. Plain radiographic images of the pelvis were obtained and they showed irregular periarticular masses [Figure 1a]. Plain radiographs of hand, skull, and shoulder joins showed similar lesions [refer to adjoining Figure 1b-d]. On metabolic work, she had parathyroid hormone level of 162 pg/mL, phosphorus of 8.5 mg/dL, calcium of 9.8 mg/dL, albumin of 3.5 g/dL, and Vitamin D level of 29 ng/mL. She was poorly compliant with dietary restrictions and taking phosphate-binding medications. Diagnosis of secondary tumoral calcinosis was made. The patient was managed with phosphate-restricted diet, phosphate-binding medication, and increasing the dialysis frequency from twice weekly to thrice weekly.Figure 1: X-ray images showing (a) irregular peri articular masses around the hip joint. Similar findings were seen in X-ray images of (b) hand (c) skull and (d) shoulder joints as wellTeutschlaender initially described this disease in his studies in 1930’s and it came to be known as Teutschlaender disease.[1] The term tumoral calcinosis was coined by Inclan in 1943.[2] Smack et al. retrospectively studied 122 cases of tumoral calcinosis and proposed a classification system.[3] They classified it as (1) primary normophosphatemic type, (2) primary hyperphosphatemic type, and (3) secondary tumoral calcinosis. Secondary causes include renal insufficiency, hyperparathyroidism, or hypervitaminosis D, with renal insufficiency being the most common identifiable condition. Even though soft-tissue calcifications are common in hemodialysis patients, the prevalence of periarticular masses is low. Most of cases in chronic kidney disease are thought to occur due to the presence of hyperparathyroidism although it may occur in its absence as well. The calcification process results from elevated calcium–phosphorus product with hyperphosphatemia being the most important component. Diagnosis is based on characteristic imaging findings. Imaging typically shows amorphous, cystic, and multi-lobulated deposits in periarticular areas.[4] Computed tomography may show cystic loculi with fluid levels giving rise to sedimentation signs. Treatment of secondary tumoral calcinosis in the setting of renal failure is predominantly medical therapy. Calcium- and phosphate-restricted diets and phosphate binders are used as first-line management. Intravenous pamidronate is also shown to be effective.[5] Partial or total parathyroidectomy can be considered in refractory cases in case hyperparathyroidism is the cause.[6] Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that her name and initial will not be published and due efforts will be made to conceal her identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

Dermoscopy of Cylindroma in Fitzpatrick Type IV Skin: A Case Report and Review of Literature.

Dermoscopy of Cylindroma in Fitzpatrick Type IV Skin: A Case Report and Review of Literature.

Rescue use of Pegylated Interferon in Dialysis Patient who Failed to Respond Sofosbuvir.

Rescue use of Pegylated Interferon in Dialysis Patient who Failed to Respond Sofosbuvir.

Dermoscopy of Crusted Scabies: Revisiting the New and Old Signs.

Dear Editor, Scabies is a common ectoparasitosis in India caused by the eight-legged mite, Sarcoptes scabiei var. hominis. Crusted scabies occurs predominantly in immunosuppressed individuals and presents with diffuse hyperkeratotic scales, crusts, or even erythroderma. It is highly contagious because crusted scabies patient may harbor millions of mites on their body in contrast to 10–12 mites found in classical scabies.[1] It may often be misdiagnosed due to the absence of typical nocturnal pruritus and atypical clinical presentations. Dermoscopy is extremely useful in the diagnosis of this uncommon variant of the common parasitosis. We present a case of crusted scabies showing the classical and newly described dermoscopic signs. A 38-year-old female, case of mixed connective tissue disease, on treatment with daily prednisolone (10 mg) along with azathioprine 100 mg and admitted to the medical ward due to cirrhosis and ascites, presented with skin rash of 1 month duration. On clinical examination, generalized erythematous rash with scaly papules and crusting was seen along with multiple dry crusted hyperkeratotic plaques (showing wet sand appearance) predominantly over the bony prominences, trunk, limbs, and scalp [Figure 1].Figure 1: (a) multiple dry crusted hyperkeratotic plaques with erythematous scaly papules and crusting. (b) scaly crusted plaque involving the scalp. (c) “wet sandpaper” appearance of crusted plaquesDermoscopic examination showed numerous burrows in a “noodle-like pattern” with multiple brownish triangular structures (delta glider sign) [Figure 2a]. These burrows showed the “gray-edged line sign”, which is a gray-brownish line on the outer edges of the burrow (Figure 2b) and wake sign [Figure 3]. Potassium hydroxide (KOH) mount of scales revealed numerous scabies mites and eggs [Figure 4]. On histopathology, hyperkeratosis, parakeratosis, acanthosis, and scabies mites in the stratum corneum were seen [Figure 5]. The patient was advised topical application of 5% permethrin cream every third day for 2 weeks along with oral ivermectin 12 mg to be taken on day 1, 2, and 8.Figure 2: Dermoscopy showing (a) “noodle-like pattern” consisting of accumulation of several curvilinear burrows displaying the “gray-edged line sign” [blue arrowhead (in fig b also)]. (Heine delta NC1, polarized, 10×) (b) brown-colored triangular structure (yellow arrow) that simulates a delta glider trailed by the jet contrail (inset). (Dinolite Videodermoscope polarized non-contact 50x)Figure 3: Wake sign—the burrow (black lines) and the wake-shaped scale (dashed line) together presenting a Y-shaped lesion. (Higher magnification Dinolite Videodermoscope polarized non-contact 50×)Figure 4: Potassium hydroxide mount showing numerous mites (red arrows), eggs (blue arrowhead), and scybala (yellow circles)Figure 5: Multiple scabies mites (black arrow) in stratum corneum (H & E 10x)Burrows are the pathognomonic lesions of scabies; however, they are difficult to be seen on naked eyes, especially in brown skin and in patients with excoriations. Various eponymous dermoscopic signs of scabies include “Delta sign”/“hang glider sign”/“triangle sign” (brownish triangular structures in the shape of hang glider) corresponding to the head and anterior legs of the mite and “Jetliner with contrail sign” (where the mite body appears translucent and air-filled burrow resembles a jetliner with contrail).[2] “Noodle pattern” or “millipede-like structure” (showing numerous burrows) as seen in our case is a characteristic dermoscopy sign of crusted scabies.[3] “Gray-edged line sign” on dermoscopy is a recently described sign where the dark line is often seen on the outer wall of the curved burrow and is supposed to form mainly due to the melanin containing fecal material of the mite, which gets deposited on the outer wall by the tail end of the mite as it crosses the curved burrow.[4] “Wake sign” is described as a pattern of scale reminiscent of the “wake” left on the water surface by a moving object.[5] All the dermoscopic signs were seen in our case. Thus dermoscopy is an extremely useful, rapid, and noninvasive in vivo tool for the diagnosis of crusted scabies. This case is interesting as the dermoscopic signs of noodle pattern and gray-edged line sign have not been previously described in Indian patients. Declaration of patients consent The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

THRIVE to the rescue.

Dear Editor, “Transnasal humidified rapid insufflation ventilator exchange (THRIVE)” works on the principle of ventilation without ventilator movements. With THRIVE, ventilation occurs with a noninvasive nasal cannula, with high flow humidified oxygen at 30–60 L/min. These high flows create a distending alveolar pressure or positive end-expiratory pressure (PEEP) up to 7 cm of water, which prevents airway collapse and atelectasis.[1,2] We describe two cases in which the use of THRIVE helped prevent reintubation postoperatively. Case 1: A 61-year-old male was operated for robotic radical cystectomy under general anesthesia in lithotomy and Trendelenburg position for 8 h. After extubation within a few minutes, he started to desaturate. Blood gas showed pH-7.33/pCO2-46.5/pO2-47.2/sat-76.4%. Bilateral lung basal atelectasis secondary to the position during the procedure was the probable diagnosis. In view of persistent hypoxia and the possibility of worsening oxygenation, the choice was noninvasive ventilation (NIV) or reintubation. However, NIV was logistically not possible in the OT. THRIVE (OptiFlow THRIVE system by Fisher and Paykel Healthcare Ltd, Auckland, New Zealand), was started at 30 L/min. Within few minutes, the saturation picked to 100%. Blood gas done after 30 min of THRIVE showed pH-7.38, pCO2-35, pO2-107.9, satulation-97.5%. As the patient was breathing comfortably and hemodynamically stable, he was shifted to the intensive care unit (ICU) with facemask oxygen for transfer. THRIVE was continued in the ICU for another 2 h and finally weaned off to a nasal cannula of 2 L/min. Case 2: A 61-year-old lady was operated for breast microdocectomy under general anesthesia, post-operatively the patient was extubated after which her respiratory rate was 36–38 per min with oxygen saturation of 88%–90% on 10 L oxygen non rebreathing mask and blood pressure of 213/102 mm Hg. Chest auscultation revealed bilateral crepts. Suspecting it to be pulmonary edema, injection furosemide IV was administered, and nitroglycerine (NTG) infusion was started. Her blood gas showed pH- 6.99, pCO2- 116, pO2- 85, pHCO3- 19, oxygen saturation-90%. After the initiation of THRIVE at 40 L/min over the next 10 min, her saturation started picking up to 97%–98%, and her respiratory rate settled to 18–20 per mi. Eventually her THRIVE flow was reduced to 20 L/min. Her blood gas was pH- 7.32, pCO2- 50.6, PO2 -140, HCO3-26 and was shifted to the ICU. After 2 h of THRIVE in the ICU, her blood gas was pH-7.3, pCO2- 41, pO2- 138, pHCO3- 25. Thrive delivers gases at a flow rate that exceeds the patient’s peak inspiratory flow rate.[3] It helps to recruit the lungs. It washes out the upper airway dead space, creating an oxygen reservoir and increasing the tidal volume, probably explaining the mechanism for decreasing the PCO2.[4] By increasing the end-inspiratory lung volume, it decreases the work of breathing.[5] The warm and humidified inspired gases decrease airway inflammation and improve drainage of respiratory secretions.[4] THRIVE can be an important additional tool to provide oxygen in the post-operative period to prevent reintubation. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

Diagnosis of renal amyloidosis by liquid chromatography-tandem mass spectrometry: experience from a single-center cohort study in China.

Diagnosis of renal amyloidosis by liquid chromatography-tandem mass spectrometry: experience from a single-center cohort study in China.

Peripheral smear reckons the nature of IgM cold agglutinins: A case‐based study asserting the role of thermal amplitude and clonality

Peripheral smear reckons the nature of IgM cold agglutinins: A case‐based study asserting the role of thermal amplitude and clonality

Salivary Gene Expression of RANK, RANKL, and OPG in Type 1 Diabetes Mellitus and Periodontal Disease Patients.

The relationship between type 1 diabetes mellitus (T1DM) and periodontal disease may exhibit by the alteration of bone metabolism. However, evidence for this relationship is scarce and inconclusive. Thus, the aims of the present study were to investigate salivary receptor activator of nuclear factor kappa-β (RANK), receptor activator of nuclear factor kappa-β ligand (RANKL), osteoprotegerin (OPG) gene expression and the RANKL:OPG ratio in T1DM and non-T1DM. Secondary objective was to determine the relationships of RANK, RANKL and OPG gene expression to clinical parameters of T1DM and periodontal disease. Twenty patients with T1DM and twenty age-matched non-T1DM were recruited. Clinical periodontal parameters were measured. Total RNA was isolated from non-stimulated saliva, and the relative gene expressions of RANK, RANKL, OPG and RANKL:OPG ratio were determined by quantitative real-time polymerase chain reaction. The T1DM group had significantly higher mean periodontal parameters than the non-T1DM group, while the mean plaque scores of both groups were not significantly different. There was a trend of higher relative gene expression of RANK, RANKL, and the RANKL:OPG ratio and lower expression of OPG in T1DM group but no statistic significant different when compared to non-T1DM. In the T1DM group, RANKL:OPG correlated with the percentage of bleeding sites, whereas RANK, RANKL, and HbA1c levels correlated with pocket depth. Bone metabolisms demonstrating by decreased OPG gene expression and upregulated of RANK, RANKL, RANKL:OPG with higher pocket depth and bleeding in T1DM may play an important role in periodontal destruction in T1DM.

A balloon-supported embolism protection technique during vertebral/subclavian artery angioplasty stenting.

A balloon-supported embolism protection technique during vertebral/subclavian artery angioplasty stenting.

Cough, Cough-Induced, and Primary Cough Headache

Dear Editor, We read the article entitled “Magnetic Resonance Imaging of Leptomeningeal Cyst with Atypical Presentation: Cough-Induced Headache” on the esteemed “Matrix Science Medica” with great interest. Dar et al. presented two adult patients with posttraumatic leptomeningeal cysts presenting as cough-induced headache. The headache worsened on forceful coughing in both individuals. In this context, the presence of growing skull fractures with headache and cough highlights the possible association between this type of headache and cerebrospinal fluid dynamic.[1] Headache associated with coughing is characterized by headaches triggered by a rapid increase in intra-abdominal pressure caused by coughing, sneezing, or straining. It is an uncommon finding with a lifetime prevalence of <1%. Interestingly, the syndrome of cough headache is symptomatic in about 40% of cases, and the majority of these patients have Arnold–Chiari malformation Type I.[2] Herein, we would like to discuss recent studies about this rare condition. Four main mechanisms could explain headaches triggered by cough, which are jugular vein stenosis, cerebrospinal fluid hypervolemia, infection altering vascular tone, and crowdedness of the posterior cranial fossa [Figure 1]. We believe that these mechanisms combined lead to the development of headaches, instead of being separated pathways.Figure 1: Pathophysiological mechanisms related to headache associated with cough. There are four main pathways that are jugular vein stenosis, cerebrospinal fluid hypervolemia, infection alters vascular tone, and crowdedness of posterior cranial fossa. The third version of the diagnostic criteria for primary cough headache of the international classification of headache disordersTakizawa et al. reported a case of an individual presenting with both primary cough headache and headache attributed to temporomandibular disorder. Their report was interesting because headaches associated with referred pain exhibit secondary central nervous system effects that can easily confound clinicians. It is worthy of mentioning that involuntary nonfunctional tooth contact is believed to be an important cause of both types of headaches.[2] Primary cough headaches are mainly observed in adult patients, but cough-induced headaches are potentially symptomatic in the pediatric population. Omata et al. described a 7-year-old boy presenting with powerful cough-induced headaches. Echo examination confirmed right internal jugular vein dilatation during a Valsalva maneuver, and the individual was diagnosed with primary cough headache with internal jugular phlebectasia. Their report brings a new idea about the pathophysiology of this type of headache, which may be associated with the morphology and function of the internal jugular vein.[3] Moreno-Ajona et al. reported a 53-year-old male diagnosed with primary cough headache using indomethacin, which was stopped due to gastroesophageal reflux disease. He was started on noninvasive vagal nerve stimulation (nVNS) using the gammaCore™ device. The individual showed improvement in his symptoms with nVNS, in particular, the parasympathetic reflex arc. This approach has advantages when compared to indomethacin such as good tolerability and safety of neuromodulation.[4] In a recent Chinese respiratory clinic, it was assessed the prevalence, predictive factors, and clinical characteristics of cough headache in 679 patients. The prevalence of cough headache was 18.0% in these coughing patients. The main risk factor for the development of cough headache was age between 31 and 50 years (odds ratio 2.0). Furthermore, cough headache was associated with cough severity. It is noteworthy that headache severity had a positive correlation with cough severity, age, and headache duration.[5] In sum, primary cough headaches are related to a mixture of pathological mechanisms. They are probably more common than we first believed. Moreover, when present in the pediatric population, further evaluation is needed. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initial s will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

Double-tunneled catheter method for continuous peripheral nerve block.

Dear Editor, Continuous peripheral nerve block provided by an infusion through a peripheral nerve catheter is an attractive option for both the patient and the anesthesiologist. One of the main problems is dislodgement of the catheter, which can occur in up to 10% of cases.[1] Methods used to prevent peripheral catheter displacement are dressings, tissue glue, suturing and tunneling. The benefit of a dressing probably decreases with time, as the dressing becomes less sticky with time, and is interfered with by sweat or by ooze from the catheter site. Additionally, the site of catheter, presence of hairy skin and type of dressing have a large impact on occurrence of catheter displacement. Tunneling has been described as a method of reducing catheter displacement and infection rates, and seems to have a high rate of success.[2,3] Byrne et al.[4] compared the force requirement for extraction for peripheral nerve catheters under three different situations, tunneled, tunneled and double-tunneled catheters in a porcine model. They concluded that double tunneled catheters were associated with nearly a fourfold increase in force required for displacement compared with single-tunneled catheters, and a 17-fold increase in force of displacement compared with tunneled catheters in porcine model. Double tunneling is not much tested in human subjects. It could be because of chances of kinking of catheter at the site of second tunnel, lack of enough space, requirement of special care at the time of removal, and increase in infection rate due to two skin punctures. Infection is unlikely to be a major problem due to its very low incidence in short-term peripheral nerve catheters.[5] Double-tunneling allows catheter to circle the bridge of skin created between two loops of catheter Figure 1. In our case, we double tunneled the continuous catheter in the infraclavicular region after its placement under ultrasound guidance. We did not face any technical difficulty while inserting it. Insertion required the help of an assistant to stabilize the catheter during the procedure. The catheter was maintained for 48 hours and was removed without any problems. The patient did not complain of any scar formation on successive follow up. Catheter dislodgement is prevented due to the tightening of the bridge of skin in case any untoward force pulls it. This benefit of tunneling is less affected by the site of the catheter or the nature of the surrounding skin, unlike dressings. We have successfully extended the use of this technique to supraclavicular continuous catheter placements. Double-tunneling is a new technique of peripheral nerve catheter fixation which provides more protection from catheter dislodgement. The incidence of infection and scar formation needs to be studied further.Figure 1: Steps of double tunneling peripheral nerve catheterDeclaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

Rare cause of intraoperative endotracheal cuff deflation.

Dear Editor, Manufacturing defects in endotracheal tubes (ETTs) can lead to cuff leaks, inadequate ventilation, hypoxemia, and risk of aspiration.[1] These defects may go unnoticed during preuse check of tube before intubating[2] and may lead to difficulty in tube exchange during head and neck surgeries or prone positioning. We report a case of trivial leak that went unnoticed until the intraoperative phase with a source of leak being only established after tube exchange. A 39-year-old female (43 kg), undergoing gastric pull-up surgery, was intubated with a 7.5-mm ID cuffed ETT (Portex®, Smiths Medical, Minneapolis, MN, USA) and a pilot balloon was filled with 4 ml of air at which there was no cuff leak. After around 30 min, a leak was visible on the ventilator with 150 ml of difference between the inspired and expired air. On inspection of the pilot balloon, it was found to be deflated. It was again filled with air resulting in correction of leak. As neck incision had been made with the face covered under the drapes with no leak, we decided to continue with the surgery. Thereafter, every 45–60 min, the tube developed a leak that was managed by filling air repeatedly. A syringe was attached at the valve to rule out valve incompetency, but it did not prevent leak and tube was exchanged. The surgery lasted for 5 h and the patient was extubated uneventfully. The tube was dipped in water and cuff did not show any bubbling. Then, the pilot balloon and valve were immersed in water, which showed slight bubbling at the junction of pilot balloon and valve assembly [Figure 1]. The leak was mild and clearly appreciable only when the tube cuff was pressurized by hand. These types of leaks were found in other cases in the same operating room complex as well implying a faulty manufacturing defect in the batch of ETT s.Figure 1: Site of leak in endotracheal tube. (a) Immersion in water showed bubbling. (b) Application of water showed bubble at junction of pilot balloon and one-way valveManufacturing defects in tube can lead to leaks present in the valve, pilot balloon, junction of cuff inflation line and ETT shaft, lumen of tube and the cuff.[3,4] Minute leaks may not be visible and missed during inspection of tube before intubating. These leaks can be hazardous in cases of head and neck surgery and difficult airway. Various methods of minimizing the leak have been described and include throat packing, injecting the cuff inflation line with lignocaine, continuous inflation, and blocking the one-way valve (in case of valve incompetency).[5] These methods are not foolproof and act as temporary measures. If a leak is detected, a plan must be formulated depending upon the surgery, airway, and amount of leak. Manufacturing defects must be reported to the manufacturer and stores department. The tubes of same batch must be inspected thoroughly as they may have the same defect and leak. If a trivial leak is present, it should always be looked for after extubating and immersing the tube in water. Intense vigilance toward monitoring difference in inspired and expired tidal volume can aid in detection of such minuscule defects. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

Inverse Gottron’s papules

A 40-year-old woman presented with facial edema and painful skin lesions involving the hands. On examination, heliotrope rash [Figure 1a], hyperkeratotic ulcerated papules on the knuckles [Figure 1b] and erythematous, flat-to-raised keratotic lesions involving the palmar aspect of the interphalangeal joints [Figure 1c] were noted. A diagnosis of dermatomyositis (DM) was made with clinical features and raised muscle enzymes.Figure 1: Clinical photograph showing heliotrope with periorbital edema (a) and Gottron’s sign over dorsal aspect of hand (b), flat-to-raised lesions involving palmar aspect of the fingers in a distribution “inverse” of the classic Gottron’s papules/sign (c, black arrow) and fingertip scars suggestive of healed ulcers (c, white arrow)Gottron’s papules are pathognomonic of DM. Inverse Gottron’s sign/papules [Figure 1c], due to vasculopathy are a rare but specific cutaneous finding in DM, usually associated with digital ulceration, interstitial lung disease, and anti-MDA5 autoantibodies.[1-3] Anti-MDA5-positive DM occurs in 7.2% of adults and 9.7% of children, respectively, in the Indian subcontinent. Cutaneous vasculopathic features such as cutaneous ulceration, and periorbital edema as seen in the index may indicate a worse poor prognosis in adults with anti-MDA-5 DM.[4] Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

It Melts Like Snow: Steroid Responsiveness of Immunoglobulin G4-Related Disease

Dear Editor, A 35-year-old male presented with insidious onset gradually progressive bilateral painless submandibular swelling, followed by periorbital swelling [Figure 1] for the past 7 years. He had no constitutional or sicca symptoms. He had no history of addiction or autoimmunity in the family. Systemic examination was unremarkable. On evaluation, he had raised inflammatory markers with a negative autoimmune panel. Magnetic resonance imaging orbit showed bilateral orbital extraconal soft-tissue masses with contrast enhancement. Incisional biopsy from the extraconal mass was suggestive of fibrocollagenous stroma with aggregation of lymphocytes, plasma cells, and histiocytes without any necrosis or granuloma. On further evaluation, submandibular gland biopsy showed chronic sialadenitis with immunoglobulin G4 (IgG4)-positive plasma cell >10/hpf and IgG4: IgG ratio >40% [Figure 2] without any major organ involvement on imaging. The diagnosis of IgG4-related disease was kept. He was started on oral steroid 0.5 mg/kg/day and observed a rapid clinical response within 15 days of steroid therapy. Later, he was started on azathioprine and continued with tapering dose of steroid. He was followed up every month for first 3 months and then every 3 monthly upto 16 months without any signs of recurrence. For a long-standing indolent disease of 7 years, drastic steroid response with resolution of clinical symptoms was quite evident in our case.Figure 1: (a) Pretreatment bilateral upper eyelid swelling, (b) pretreatment bilateral submandibular swelling, (c and d) during glucocorticoid therapy (day 15) resolution of orbital and submandibular swelling, respectivelyFigure 2: Salivary gland with seromucinous acini and ducts embedded in mildly fibrotic stroma with numerous lymphoid follicles with germinal centers (a and b: H and E). Clusters of plasma cells are highlighted with CD138 immunohistochemical stain (c). IgG4 positive cells, >10/hpf (d)IgG4-related disease (IgG4-RD) is a chronic, systemic, fibroinflammatory condition with variable organ manifestation. CD4+ T-cells and B-cells are central to the pathogenesis causing organ damage and fibrosis. Marked expansion of IgG4-secreting plasmablast in the blood is usually seen in active and untreated cases.[1] Key to diagnosis is the classical histopathological hallmarks of lymphoplasmacytic infiltrate, storiform fibrosis, and obliterative phlebitis. IgG4 RD usually has a good response to steroids but a variable relapse rate. Factors associated with lesser treatment response and relapse were well analyzed in a Japanese multicenter study. Multivariate analysis from the study revealed that intermediate steroid initial dose (0.4–0.69 mg/day), slow tapering regimen (<0.4 mg/day), and shorter disease duration were associated with a significantly lower rate of disease relapse.[2] The study of isolated IgG4 ophthalmic disease also echoed similar results regarding steroid response and relapse, with more relapse in those with elevated serum IgG4 levels and a shorter duration of initial steroid treatment (<5 months). Low-dose steroid maintenance should be considered to avoid recurrence in patients with elevated serum IgG4 levels.[3] Later on, rituximab therapy showed promising results in inducing remission[4] and steroid-free long-term maintenance.[5] Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that his name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

Familial Incomplete Hypertrophic Osteoarthropathy: A Rare Disorder

Dear Editor, Hypertrophic osteoarthropathy is a condition in which there is abnormal proliferation of skin and soft tissue, particularly around the periosteal tissues. It is a very rare condition which is mainly described as two types, in which the common form is secondary hypertrophic osteoarthropathy caused by underlying disease and the rare primary idiopathic form. Here, we present a case of incomplete familial form of hypertrophic osteoarthropathy where a young male presented with multiple joint pains and Grade 4 clubbing and large patella. A 24-year-old male presented with multiple joint pains predominantly involving both knees, elbows, and shoulders. There was no history of any joint swelling or morning stiffness. There was no other history suggestive of connective tissue disorder. On examination, Grade 4 clubbing was present [Figure 1]. Systemic examination was unremarkable. Musculoskeletal (MSK) examination revealed a significantly large patella on both sides [Figure 1]. There were no signs of inflammation. On further probing, the patient gave a history of similar clubbing and joint pains for his sister, brother, and grandfather. His father also has similar complaints but with less severity (pedigree charting below mentioned) [Figure 2]. On investigations, blood counts and liver and kidney function tests were normal. C-reactive protein was negative. Thyroid profile was normal. To rule out acromegaly, growth hormone levels and magnetic resonance imaging brain were done which were normal. X-ray hands and both knees were done suggestive of periostosis. MSK ultrasonography of the knees was done suggestive of zigzag appearance of the patella [Figure 3]. A diagnosis of incomplete familial hypertrophic osteoarthropathy was made as there was no skin thickening and no coarse facial features and no other systems such as hematological or endocrine are involved. He was prescribed etoricoxib, and on follow-up, he was symptomatically better. Genetic testing was not done in our patient as he is not willing for that.Figure 1: Digital clubbing with large patellaFigure 2: Pedigree chart showing affected individuals in three generationsFigure 3: X-ray showing periosteal reaction involving the terminal phalanges, both bones of forearm, and long bones of lower limbs. USG image showing Zigzag appearance of the patella. USG: UltrasonographyPrimary or idiopathic form of hypertrophic osteoarthropathy is very rare and constitutes only 2%–3% of cases. It is inherited as autosomal dominant or recessive disorder, and it was thought to be caused by impaired metabolism of prostaglandin E2.[1] Pachydermoperiostosis is an another name given to this disorder due to the presence of coarse facial features with underlying thickening and furrowing of skin.[2] If both skin involvement and periosteal components are present then it is called complete form. The other two forms are incomplete with prominent periosteal with less skin involvement (like our case) and fruste form with more skin features and less periosteal features. The diagnostic criteria for PDP (pachydermo-periostosis) include major criteria and minor criteria. Major consists of pachyderma, periostosis, and finger clubbing and minor criteria includes hyperhidrosis, arthralgia, gastric ulcer, cutis verticis gyrata, blepharoptosis, joint effusion, edema, seborrhea, acne, and flushing.[3] Endocrine disorders like acromegaly, hypothyroidism, acropathy and other conditions like multifocal periostitis to be ruled out before labeling this condition. The other mimic of this condition is psoriatic onycho-pachydermo-periostitis except for the presence of psoriatic lesions.[4] Nonsteroidal anti-inflammatory drugs are the main stay of treatment as they act by blocking prostaglandin synthesis. They can have a good prognosis and normal life span.[5] Other treatment modalities available are corticosteroids, colchicine, or bisphosphonates.[6] Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

What next and what to expect?

What next and what to expect? A 17-year-old female presented with painless progressive loss of vision in both eyes (OU) with multiple episodes of syncope and postural giddiness for 1 month. On ocular examination, best-corrected visual acuity (BCVA) was 1/60 in the right eye (OD) and finger counting close to the face in the left eye (OS); intraocular pressure was within normal limits in both the eyes. Slit-lamp examination revealed corkscrew conjunctival vessels [Fig. 1a), anterior chamber (AC) cells 1(+), and iris neovascularization. Pupils were mid-dilated and sluggishly reacting to light. Fundus examination revealed hyperemia and neovascularization of the disc, dilated retinal veins with a beaded appearance, patches of retinal whitening over the macula and peripapillary area, and arterio-venous (AV) anastomosis [Fig. 1b and 1c]. What is the Next Step? Systemic examination Computed tomography (CT)-angiography Fundus fluorescein angiography and optical coherence tomography All of the above Findings On systemic examination, the radial artery pulse could not be felt, and the carotid pulsations were felt very feebly on both sides. The pulse rate measured in the right popliteal artery was 80/min. Blood pressure (BP) was not recordable in the upper limbs. BP recorded in the right lower leg was 150/90 mm Hg. CT angiography revealed severe luminal narrowing involving the left common carotid artery and left subclavian artery at the ostial level, and juxta-ostial narrowing of the right brachiocephalic trunk with multiple enhancing collateral channels noted in the neck [Fig. 1d-f].Figure 1: (a) Slit-lamp photography showing corkscrew conjunctival vessels, (b) and (c) fundus images showing neovascularization of the disc, dilated retinal veins with beaded appearance, patches of retinal whitening over the macula and peripapillary area, and arterio-venous anastomosis, computed tomography (CT) angiography image showing (d) narrowing of the right brachiocephalic trunk (yellow arrow), (e) narrowing of the left common carotid artery (yellow arrow), (f) stenosed left subclavian artery, minimal contrast filled vessel seen (yellow arrow)Proliferative retinopathy in both eyes was treated with pan-retinal photocoagulation (PRP) and one intravitreal injection of bevacizumab (Avastin) in OS. She received oral prednisolone for the systemic condition. Subsequently, she underwent phacoemulsification with foldable intraocular lens implantation in OU. She had a BCVA of 20/120 in OD and 20/400 in OS at the last follow-up visit. Diagnosis: Takayasu arteritis (TA) with Takayasu retinopathy Correct Answer: B Discussion TA is a chronic inflammatory disease affecting the large and medium-sized vessels, predominantly in the aorta and its main branches leading to the arterial wall thickening, fibrosis, stenosis, and thrombus formation resulting in cerebral and ocular ischemia.[1] Ophthalmic features in TA result from either due to the obliteration of arteries causing ischemic ocular changes (hypotensive retinopathy) or due to renal artery stenosis causing uncontrolled hypertension (hypertensive retinopathy).[2] Manifestations due to retinal hypoxia are microaneurysms, AV anastomosis, retinal vein occlusion, proliferative retinopathy, cataract, neovascular glaucoma, ocular ischemic syndrome, ischemic optic neuropathy, optic atrophy, retinal detachment, etc.[3] Statement of ethics Written informed consent for publication (including the images) has been obtained from the parent of the patient. All procedures carried out were in accordance with the tenets of the Declaration of Helsinki. Institute Ethics Committee approval is not required for a case report according to Indian council of medical research guidelines. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

406 Appendicectomy: Consenting for a Normal Appendix

Abstract Aim To assess consent forms for appendicectomy and ensure that patients are consented for an intraoperative finding of a normal appendix. Method Retrospective audit assessing consent forms (for mention of Normal appendix finding) for patients who underwent appendicectomy over a 2-month period. Results A total of 68 patients underwent appendicectomy. The age range of these patients was 3 to 84 years old (median 20 years). 58 of these procedures were performed laparoscopically. 23 (33.8%) of the consent forms have mention of a normal appendix. The remaining 45 patients (66.2%) had no mention of normal appendix on the consent form. In analysis of the operative notes, 7 patients had no intraoperative finding of macroscopic inflammation. Of these 7 patients, only 1 was consented for a normal appendix finding. Conclusion During the consent process for an appendicectomy, it is important to warn the patient (or caregiver) about the possibility of finding a normal appendix. The appendix is routinely removed regardless of the intraoperative findings (normal or inflamed). The patient should be made aware, as the appendicectomy may not result in the resolution of their symptoms, or further procedures may be required. This audit found that there is currently a deficit in consenting patients, undergoing appendicectomy, about the possibility of finding a normal appendix intraoperatively. A quality improvement project is currently underway, wherein a combination of departmental education and patient information booklets aims to improve consenting practices.

Emergency decompressive craniotomy with scalp block and closed-loop TCI sedation for acute ischemic stroke in a patient with incidental diagnosed large left ventricular mass, presenting with severe LV dysfunction.

Dear Editor, An average built 35-year-old male presented to the emergency-department with a history of a sudden onset of left-sided weakness and bi-frontal headache, with a chronic history of ischemic cardiomyopathy, and acute myocardial infarction one year back, with current functional status New York Heart Association classification (NYHA) class III. Patient underwent thrombolytic therapy with 0.9 mg/kg alteplase (r-TPA) over 60 minutes after evaluating risk factors during the precious golden hour period. Over the next 12 hours, the GCS deteriorated. There was cerebral swelling with a midline shift of 8 to 12 mm, although the patient was not intubated or ventilated. In a repeat scan and the neurosurgeon planned to proceed with emergency decompressive craniotomy. Complete hemogram was normal, electrocardiogram revealed sinus tachycardia with left anterior hemiblock. Routine trans-thoracic echocardiogram suggested global left ventricular (LV) dysfunction with an ejection fraction of 20%, large left ventricular mass (query myxoma or thrombus attached to LV apex measuring 44 mm × 36 mm), and grade III mitral regurgitation with eccentric jet. [Figure 1] Computerized tomography showed Acute Ischemic Stroke (AIS) in the right middle cerebral artery territory. After explaining due appropriate risks and benefits of surgery to the patient relatives and interdisciplinary consensus, the team decided to proceed with craniotomy under Monitored Anesthesia Care using scalp block and manual closed-loop TCI Propofol sedation under spectral entropy monitoring.Figure 1: Echocardiography (ECHO) Revealing Large Left Ventricular Mass Attached LV Apex Measuring 44 Mm × 36 Mm (A) Parasternal Long Axis View, (B) Apical Four Chamber ViewBilateral scalp anesthesia was given using 24-gauge short bevel needle with 40 ml 0.2% Ropivacaine. After assessing for adequate block by pin prick method, entropy leads were attached and secured with 1-inch plaster tape. TCI sedation with Propofol was started with the initial effect site concentration of 1 microgram/ml, later escalated to 3 microgram/ml to maintain spectral entropy between 60-80 and hemodynamic parameters. Decompressive craniotomy was completed in 2 hours 10 minutes, with no complications; patient recovery was rapid and was obeying verbal comments when effect-site concentration reached 0.5 microgram/ml. After this emergency surgery, the patient was operated for his cardiac mass after 9 months and it was found to be thrombus on histopathological diagnosis. Thrombus, tumor, and vegetation are the most common causes of intracardiac mass and must be distinguished from normal variants such as papillary muscles, false tendons, sigmoid septum, and apical trabeculation.[1] Our patient was at high risk with associated large left ventricular mass, severe left ventricular dysfunction, and ischemic cardiomyopathy and grade III mitral regurgitation for emergency craniotomy. Anesthetic management in this clinical scenario could be associated with risk of complications ranging from peri-operative myocardial infarction, pulmonary edema, LV outlet obstruction and sudden cardiac arrest, new thrombo-embolic episodes, and hemorrhagic changes in infraction.[2] Scalp block with TCI Propofol sedation prevents hemodynamic perturbances associated with general anesthesia with intubation and cardio depressant effects of anesthetic agents. Manual closed loop TCI Propofol sedation with deep anesthesia control offers optimal titration with stable hemodynamic sedation and eliminates the need for unintended protection of the airway.[3] Considering the risk and benefit ratio, craniotomy with closed loop TCI Propofol sedation outweighed the risks of general anesthesia in our patient. Though awake craniotomy with TCI sedation is safe but has a few limitations like difficulty to alter ventilation in case of intraoperative cerebral edema and airway securement may be difficult in case requirement occur intraoperatively due to positioning of patient. In spite of appropriate anticoagulation, a thrombus traveling across the cardiac cycle has a high potential to embolize. It is imperative LV masses should be surgically removed as early as possible, owing to risk of recurrent thrombo-embolic phenomenon. We conclude that craniotomy under scalp block may be performed even in an emergency craniotomy in patients with high peri-operative risk. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

Type A Wellens’ syndrome - A critical electrocardiographic sign

Wellens’ syndrome was first described in 1982[1] and classified into type A and type B. Type A Wellens’ syndrome is characterized by biphasic T-waves in leads V2 and V3, and type B is characterized by deep symmetrical T-wave inversions in the same leads. The study reported that Wellens’ syndrome electrocardiographic (ECG) finding related to left anterior descending (LAD) total or near-total occlusion, which is also described as one of the ST-segment elevation myocardial infarction (STEMI) equivalent signs.[2] Cardiology consultation for possible coronary angiography is necessary for evaluating patients.[3] A 29-year-old male without underlying medical diseases was admitted to our emergency department due to intermittent retrosternal chest tightness for 1 week. His chest pain usually occurs after exertion, accompanied by nausea and dizziness, and subsides 10–15 min after rest. He did not notice other exacerbating or relieving factors. Thus, he visited our emergency department for a consultation. The patient is a nonsmoker with no family history of acute coronary syndrome. In the emergency department, his chest pain had already subsided. He was vitally stable. On physical examination, his cardiac auscultation revealed a regular heartbeat with no murmurs. His ECG performed in the emergency department showed sinus rhythm with biphasic T-waves in leads V2 and V3 without precordial Q waves [Figure 1], which is compatible with type A Wellens’ syndrome. As for his blood examination, his troponin I level is 3.2 ng/mL (normal range < 0.02 ng/mL) and creatine kinase-myocardial band mass is 8.5 ng/mL (normal range <5 ng/mL). He also had thrombocytosis with a platelet count of 993,000/μL.Figure 1: Patient’s initial ECG. ECG: ElectrocardiographicThe patient received aspirin, ticagrelor, and heparin for the myocardial infarction. He received urgent coronary angiography and the examination showed an 85% stenosis of the proximal LAD artery [Figure 2] that was successfully opened through angioplasty. Two drug-eluted stents were placed. After angioplasty, his ECG showed sinus rhythm with no Wellens’ pattern. In addition, he did not experience any chest pain attack after angioplasty. On admission, the patient was diagnosed with essential thrombocythemia through positive JAK-2 V617F mutation test and bone marrow biopsy.Figure 2: Patient’s coronary angiography showed LAD stenosis. (Left) Before the intervention. (Right) After the intervention. LAD: Left anterior descendingWellens’ syndrome is one of the STEMI equivalent ECG signs that need to be treated as acute myocardial infraction in the emergency department.[2] A previous study[1] showed that 75% of the patients with Wellens’ syndrome developed extensive anterior wall myocardial infarction a few weeks after admission. T-wave findings of Wellens’ syndrome are classified into two patterns. In type B, which comprises approximately 75% of cases, the T-wave is deeply inverted and the inverted T-wave is symmetric in contour. In type A, which comprises 25% of cases, the T-wave is biphasic in leads V2 and V3. Other ECG criteria included no loss of R rave or precordial Q waves. Considering noncoronary causes of T-wave inversions, the left ventricular hypertrophy and bundle branch block patterns should be readily recognized by their significant coexistent findings.[3] The characteristic ECG pattern often develops when the patient is not experiencing angina.[3] Patients with ET may present with vasomotor symptoms such as myocardial infarction or stroke. However, in this case, the patient was young and without any risk factors for myocardial infarction before diagnosis. Prompt recognition of the ECG pattern and early cardiology consultation are essential to prevent missing the diagnosis of myocardial infarction. Declaration of patient consent The authors certify that they have obtained appropriate patient consent form. In the form, the patient has given his consent for the images and other clinical information to be reported in the journal. The patient understands that name and initial will not be published and due efforts will be made to conceal the identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.