A Prospective Study of Prevalence and Impact of Sarcopenia on Short-term Mortality in Hospitalized Patients with Liver Cirrhosis.

Background: Sarcopenia is common in chronic advanced liver disease and is associated with poor prognosis. There is paucity of Indian data regarding sarcopenia in chronic advanced liver disease & its impact on prognosis. The aim of this study was to study the prevalence of sarcopenia in Indian patients with chronic advanced liver disease and its impact on morbidity and short-term mortality. Methods: Patients with chronic advanced liver disease were prospectively evaluated for the presence of sarcopenia using computerized tomography (CT) abdomen. The cross-sectional area of the right psoas muscle was measured at the third lumbar vertebra (L3) and the Psoas muscle index (PMI) was calculated. Sarcopenia was defined as PMI <295 mm2/m2 for females and <356 mm2/m2 for males. The normative values of PMI were obtained from patients undergoing CT scan for non-specific abdominal pain who had no confounding factor which could result in sarcopenia. All patients were followed up for 6 months or until death, whichever was earlier. The impact of sarcopenia on mortality and rate of readmission has been assessed at the end of 6 months. Results: Of the 156 patients with chronic advanced liver disease, 74 (47.4%) patients had sarcopenia. Sarcopenia was more commonly seen in males (M: F = 61:13) and in patients with alcohol-related liver disease. There was a linear correlation (negative) between the PMI and severity of liver disease as assessed by Child-Pugh and model for end-stage liver disease (MELD) scores (r = -0.591 and -0.465, respectively). Patients with encephalopathy, ascites, and coagulopathy had higher prevalence of sarcopenia. On six months follow-up, sarcopenic patients had higher readmission rates (74.3% vs. 22%; P = 0.0001) and higher mortality (24.3% vs. 3.7%; P = 0.002). MELD score and PMI were independent predictors of mortality. The cut-off value of PMI 305.9 mm2/m2 predicted mortality with a sensitivity of 76.2% and a false positivity of 22.2% (area under curve was 0.805; 95% confidence interval: 0.69-0.91, P = 0.001). Conclusion: Sarcopenia is seen in about half of the patients with chronic advanced liver disease. It is commoner in males, patients with alcoholic liver disease, and those with advanced liver disease. Patients with sarcopenia have worse prognosis, require more frequent hospitalization and it negatively impacts short-term survival.

Non-traditional tools for predicting coronary artery disease.

Background The traditional coronary calcium score (CCS) is a time-tested tool for the evaluation of coronary atherosclerosis and predictor of future cardiovascular events. Non-traditional tools can also have a value in predicting and detecting subclinical coronary artery disease (CAD). Methods We studied the role of CCS, the traditional CAD risk predictor, and the less-recognized, non-traditional risk factors, i.e. epicardial fat volume (EFV) and thoracic extracoronary calcium (ECC), to assess the degree of subclinical CAD. In this cross-sectional observational study, we included 950 Indian patients (suspected to have CAD). Coronary computed tomography angiography was performed. Estimation of CCS, EFV and thoracic ECC was done. Results A CCS of 0 was seen in 583 patients (61.4%). Of these, 492 patients had normal coronary angiogram but 91 patients had CAD. The median values of EFV were statistically significantly higher in the 'CAD present and CCS 0' group compared to the 'CAD absent and CCS 0' group (p<0.001). The presence of thoracic ECC involving at least a single site was seen in only 6 of these 91 patients. When both EFV and CCS were considered together for the detection of CAD, the sensitivity and negative predictive value (NPV) were improved compared to either of these in isolation. When ECC was taken together with CCS and EFV, no further improvement in sensitivity or NPV was observed. Conclusion The combined use of traditional CCS along with non-traditional EFV may guide us in better profiling cardiovascular risk and supplement the various traditional cardiovascular risk factors/scores.

Endometriosis and Its Myriad Presentations: Magnetic Resonance Imaging-Based Pictorial Review.

Endometriosis is a major cause of infertility and pain in females in the reproductive age group. It is a result of ectopic functional endometrial cells outside the uterus. It consists of a spectrum of findings from superficial to deep implants initiating a fibrotic response and resulting in adhesions. Diagnosis of endometriosis is based on clinical history, noninvasive and invasive techniques. The final diagnosis is based on laparoscopy with histopathological confirmation. Ultrasonography is the first line of investigation, followed by magnetic resonance imaging (MRI) in complex cases. MRI is a noninvasive, multiplanar technique that involves no radiation and provides excellent delineation of the disease process. As deep endometriosis has a similar low signal to adjacent normal organs, it can be easily overlooked by radiologists. They should be aware of the spectrum of diseases so as to provide a roadmap for the surgeons. A structured reporting system helps radiologists organize and standardize their reports.

Congenital Central Overinflation with Mainstem Bronchial Stenosis: Pre- and Postnatal Imaging and Outcome-Case Report and Brief Review of Literature.

Congenital overinflation of lung is underdiagnosed prenatally as the imaging features of the same are not well described. We describe a very rare case of central variety of congenital overinflation, secondary to right mainstem bronchial stenosis, not previously described in our knowledge, which presented as an enlarged homogenous echogenic/hyperintense lung with cardio-mediastinal shift and was misdiagnosed as congenital pulmonary airway malformation (CPAM). We reviewed imaging features helpful in prenatal diagnosis of this condition on ultrasound and magnetic resonance imaging and discussed an approach for differential diagnosis. The congenital central overinflation may be considered in prenatal detected echogenic lung lesions with the absence of cysts or systemic arterial supply and presence of hypervascularity or dilated bronchi within the lesion.

CT appearance of gastrointestinal tract mucormycosis.

Mucormycosis is a fungal infection caused by fungi of order mucorales. It is most commonly seen in patients with an impaired immune system due to any cause. Gastrointestinal mucormycosis is the least frequent type and may be a primary disease or a feature of generalized mucormycosis. Angioinvasion is the hallmark feature of mucormycosis, leading to bowel infarction which is the responsible for the most common clinical complaint of pain, and is also responsible for most of the imaging findings in this disease. The stomach is most commonly involved organ in the gastrointestinal tract and pneumatosis and lack of gastric wall enhancement are the most common imaging findings. Areas of bowel wall thickening and/ or lack of enhancement are seen in small bowel mucor and perforation can occur due to ischemia. Colonic mucor can present with mural thickening, or complete lack of definition or 'disappearance' of bowel wall with associated air containing collections. Mucormycosis affecting the bowel has a high mortality rate and early recognition and intervention may improve patient outcomes significantly. It should be suspected in immunosuppressed patients with imaging findings of unexplained bowel ischemia, infarction and/or pneumatosis without any obvious visible vascular thrombus.

Percutaneous Recanalization of Anastomotic Portal Vein in a Pediatric Patient After Liver Transplant Without Any Recognizable Portal Vein Remnant on Imaging.

In patients with complete portal vein thrombosis, the main portal vein is obstructed, resulting in development of hepatopetal collateral vessels. In cases of complete portal vein thrombosis, interventional procedures are challenging, with the greatest difficulty in the form of passing the guide wire across the level of obstruction. A recognizable main portal vein remnant has been deemed as a mandatory criterion in previous reports. Here, we report a case of cavernous trans-formation of the portal vein in a pediatric patient after liver transplant who had no obvious detectable portal vein remnant on radiologic imaging. Using digital subtraction angiography, we were successful in passing a guide wire through the level of obstruction and placing a stent, thus causing successful recanalization of the occluded segment.

Correlation of epicardial fat quantification with severity of coronary artery disease: A study in Indian population.

OBJECTIVE: We studied the correlation of quantified epicardial fat with severity of coronary artery disease in patients [suspected cases of coronary artery disease (CAD)] referred for computed tomography (CT) coronary angiography and established cutoffs for epicardial fat volume (EFV) for the presence of CAD and obstructive CAD. METHODS: A prospective cum retrospective cross-sectional observational study was carried out on 950 Indian subjects (suspected cases of CAD) who were referred for coronary CT in the year 2013-2016. EFV was quantified using semiautomatic technique on multidetector coronary CT angiography. The presence of atherosclerotic plaques and degree of stenosis was assessed on coronary CT angiography scans. The correlation between quantified EFV and degree of stenosis was assessed. Multivariate analysis was also performed. RESULTS: A higher quantity of epicardial fat is found in patients with increasing severity of coronary artery stenosis. The EFV cutoff for the presence of CAD and obstructive CAD are 49.75 and 67.69 mL with area under the curve, sensitivity, specificity, positive predictive value, negative predictive value, and diagnostic accuracy of 0.68, 81%, 45.9%,58.24%, 72.2%, and 62.84% and 0.709, 64.9%, 66.4%, 35.84%, 86.55%, and 66%, respectively. EFV correlates with age, weight, and body mass index (BMI). Multivariate analysis revealed EFV to be an independent risk factor for the presence of CAD. CONCLUSIONS: Higher quantities of EFV are found in patients with greater degree of coronary artery stenosis. EFV correlates with age, weight, and BMI. EFV is an independent risk factor for CAD.

The Abernethy malformation-myriad imaging manifestations of a single entity.

Abernethy malformation, also known as congenital extrahepatic portosystemic shunts (CEPS) is a rare clinical entity and manifests with different clinical symptoms. CEPS are abnormalities of vascular development where there is shunting of portal blood into the systemic venous system. Multidetector computed tomography (MDCT) is a fast and effective modality for evaluation of CEPS. CT displays all the information desired by the surgeon as well as the clinician including the anatomy of the splenic and superior mesenteric veins, size and site of the shunt, presence or absence of the portal vein radicles, and helps to plan the therapy and even the follow-up of these patients. Contrast-enhanced magnetic resonance imaging (MRI) has also emerged as a promising tool for the evaluation of liver lesions associated with the malformation. The Radiologist should be aware of the various imaging appearances of this entity including its complications. In this article, we describe the imaging appearances of CEPS, their complications, and their imaging appearances on CT and MRI. We have also described various associated anomalies.

Ectopic Pancreas in the Duodenojejunal Flexure-Computed Tomographic and Endoscopic Ultrasonographic Images.

Ectopic pancreas is a rare entity where the pancreatic tissue has no anatomic and vascular contact with the main body of the pancreas and has its own duct system and vascular supply. A detailed clinical report with contrast-enhanced computed tomography (CT) and endoscopic ultrasonography (EUS) imaging findings of a 40-year-old male came with vague symptoms. CT showed a well-defined homogeneously enhancing mass lesion in the duodenojejunal (DJ) flexure. EUS revealed a well-defined hypoechoic mass lesion in the submucosal layer of the DJ flexure. Surgical resection of the mass was performed, and histopathological examination of specimen confirmed the pancreatic tissues. Here, we have described the CT and EUS imaging features which can help to differentiate the ectopic pancreas from the gastrointestinal submucosal tumours.

Amoebic liver abscess with hepatic artery pseudoaneurysm: successful treatment by interventional radiology.

Amoebic liver abscess is most common extra-intestinal presentation of amoebiasis. It is rarely complicated with vascular involvement including thrombosis of hepatic vein or IVC and pseudo-aneurysm of hepatic artery. We describe a case of hepatic artery pseudo-aneurysm as a complication of amoebic liver abscess treated with percutaneous embolization.

Radiological assessment of the Indian children with congenital sensorineural hearing loss.

Introduction. Congenital sensorineural hearing loss is one of the most common birth defects with incidence of approximately 1 : 1000 live births. Imaging of cases of congenital sensorineural hearing loss is frequently performed in an attempt to determine the underlying pathology. There is a paucity of literature from India and for this reason we decided to conduct this study in Indian context to evaluate the various cochleovestibular bony and nerve anomalies by HRCT scan of temporal bone and MRI with 3D scan of inner ear in a tertiary care centre. Material and Methods. A total of 280 children with congenital deafness (158 males and 122 females), between January 2002 to June 2013 were included in the study and they were assessed radiologically by HRCT scan of temporal bone and MRI with 3D scan of inner ear. Results. In the present study we found various congenital anomalies of bony labyrinth and vestibulocochlear nerve. Out of 560 inner ears we found 78 anomalous inner ears. Out of these 78 inner ears 57 (73%) had cochlear anomaly, 68 (87.1%) had anomalous vestibule, 44 (56.4%) had abnormal vestibular aqueduct, 24 (30.7%) had anomalous IAC, and 23 (29.4%) had abnormal cochleovestibular nerves. Conclusion. In present study, we found lower incidences of congenital anomalies comparative to existing literature.

Coronary cameral fistula treated by the Amplatzer vascular plug.

Coronary artery anomalies occur in less than 1 % of the general population. With the advent of multidetector rows and 3D reconstruction, multidetector computed tomography has emerged as the modality of choice in the delineation of the complex coronary anatomy and diagnosis of coronary artery anomalies, helping in the institution of appropriate therapy. We report a case of coronary cameral fistula of the left anterior descending artery to the right ventricle, which was closed by an Amplatzer vascular plug. Coronary CT angiography was used to evaluate the patient prior to the procedure to locate the placement site for the vascular plug.

Severe combined immunodeficiency caused by a new homozygous RAG1 mutation with progressive encephalopathy.

We describe an unusual case of severe combined immunodeficiency (SCID) with neutropenia and central nervous system (CNS) manifestations in which a novel RAG1 mutation was identified. A 15-month-old boy presented with failure to thrive, neutropenia and recurrent infections. He was diagnosed with T-B-NK+ SCID. He subsequently developed right partial seizures with ipsilateral hemiparesis and became comatose. Magnetic resonance imaging (MRI) of the brain revealed an inflammatory lesion in the left thalamus which later progressed to diffuse meningo-encephalitis on serial imaging. No CNS infection was documented. Genetic work-up in the child revealed a novel homozygous deleterious mutation in the RAG1 gene (c:2881T>C; p:I794T), for which both parents were heterozygous. He underwent a haploidentical bone marrow transplant without conditioning and died on day +35 with no improvement in his neurological status. The features of neutropenia and progressive encephalopathy could be linked to the novel genetic defect but more data is required to establish this conclusively.

Unusual cause of massive upper gastrointestinal bleeding: a pancreatic arteriovenous malformation.

CONTEXT: Upper gastrointestinal bleeding is one of the most common emergencies in gastroenterology. The common causes of the upper gastrointestinal bleeding include peptic ulcer disease, gastric erosive mucosal disease and portal hypertension. Gastrointestinal arteriovenous malformation is a less common cause of gastrointestinal bleeding and these arteriovenous malformation are most commonly located in the large and small intestine. Pancreatic arteriovenous malformation is a rare condition in which there is tumor-like formation or vascular anomaly built up via an aberrant bypass anastomosis of the arterial and venous systems in the pancreas. Splenic artery is most commonly involved (42%), followed by gastroduodenal artery (22%) and small pancreatic arteries (25%). Clinically it may present as gastrointestinal hemorrhage which is occasionally fatal. Other presentations are abdominal pain, pancreatitis, duodenal ulcer, jaundice, and portal hypertension. CASE REPORT: We present a rare case of pancreatic arteriovenous malformation presenting as massive upper gastrointestinal bleeding. CONCLUSION: Since early surgery is a life saving treatment for such cases, hence, a high index of suspicion should be maintained especially when massive bleeding is detected from the medial wall of second part of duodenum.

Large leiomyoma in a woman with Mayer-Rokitansky-Kuster-Hauser syndrome.

Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is a rare congenital anomaly characterized as aplasia or hypoplasia of uterus and vagina in women with normal development of secondary sex characteristics. It affects 1 in 4000-5000 female births. Women with this syndrome present with primary amenorrhoea. MRKH syndrome may be associated with renal, skeletal, cardiac and auditory anomalies. Women with MRKH syndrome may develop leiomyoma from a rudimentary uterus, though very rare. Initial investigation in women having MRKH syndrome with leiomyoma is ultrasonography (USG). However, CT and MRI are more accurate to evaluate the pelvic anatomy and pathologies.

Living donor intestinal transplant using a standardized technique: first report from India.

AIM: We describe the first living donor intestinal transplant (LDIT) in India and discuss the indications and problems of this complex procedure. METHODS: A 43-year-old male patient required massive bowel resection for gangrene due to thrombosis of the superior mesenteric artery. He was maintained on parenteral nutrition but developed cholestasis and well as repeated catheter related infections with progressive loss of venous access due to thrombosis of central veins. A LDIT was performed using 200 cm of small intestine from the patient's son. The graft was based on the continuation of the superior mesenteric vessels beyond the ileocolic branch. The artery was anastomosed directly to the aorta and the vein to the venacava. RESULTS: The graft functioned well and he was weaned off parenteral nutrition. However, he later developed complications (wound dehiscence and enterocutaneous fistula) and developed sepsis. He succumbed to sepsis with a functioning graft 6 weeks after the transplant. The donor recovered uneventfully and was discharged on the 4th postoperative day. CONCLUSIONS: LDIT can be life saving in patients with intestinal failure and failure of parenteral nutrition. There is a need to introduce this modality in India. In a setting of scarcity of deceased donor organs the living donor option has advantages.