Novel application of lumen-apposing metal stent for management of malignant recto-sigmoid stricture with fistula.

Video 1Demonstration of novel application of lumen-apposing metal stent for management of a malignant recto-sigmoid junction stricture with fistula.

Magnetic resonance elastography (MRE) outperforms acoustic force radiation impulse (ARFI) in predicting oesophageal varices in obese NAFLD cirrhosis.

PURPOSE: Liver stiffness measurement (LSM) by transient elastography has been shown to underperform in high-risk varices (HRVs) prediction in obese non-alcoholic fatty liver disease (NAFLD) compensated cirrhosis (CC). LSM by magnetic resonance elastography (MRE) and acoustic force radiation impulse (ARFI) has been shown to be useful in prediction of oesophageal varices (EVs), but has limited evidence in obese NAFLD-CC. METHODS: Obese patients with NAFLD-CC who underwent MRE and ARFI for LSM and endoscopy for screening of varices were enrolled. Performance of MRE and ARFI for predicting EVs or HRVs was evaluated using area under receiver operating characteristics (AUROC) curves and regression analyses were performed for predictor variables. RESULTS: One hundred eight patients [mean age 54.7 ± 9.6 years, median BMI, 28.5 (26.4-30.0) kg/m2. 72.2% diabetics, 45.4% hypertensive] were enrolled. Fifty-two (48.1%) had no varices, while 29 (26.8%) and 27 (25%) had low-risk varices (LRVs) and HRVs, respectively. MRE-LSM was higher in patients with LRVs (p = 0.01) or HRVs (p = 0.001) against those without varices. ARFI-LSM did not differ significantly between those without and with LRVs or HRVs (p > 0.05 for all). There was a low correlation between ARFI-LSM and MRE-LSM in the overall cohort (r = 0.19). Only platelet count (PC) [0.98 (0.97-0.99)] and MRE-LSM [1.8 (1.26-2.79)] were predictors of HRVs. At a cut-off of 4.75, MRE showed a sensitivity of 96.3%. Model combining MRE-LSM with PC had a diagnostic AUROC of 0.77 and 0.76 for EVs and HRVs. CONCLUSION: In obese NAFLD-CC, MRE-LSM is significantly higher in patients with varices. MRE combined with PC predicts EVs and HRVs with better accuracy than ARFI.

Definitive Role of Contrast-enhanced Endoscopic Ultrasound (EUS) in the Diagnosis of Hepatocellular Carcinoma With Non-diagnostic Cross-sectional Imaging: A Case Report.

The diagnosis of hepatocellular carcinoma is usually centered around cross-sectional imaging (CSI) modalities. However, in some instances focal hepatic lesions may be missed on CSI. Endoscopic ultrasound (EUS) has an evolving role in hepatology and have been shown to be useful in diagnosing focal lesions with advantages of tissue acquisition. We report a case hepatitis B-related cirrhosis presenting with acute decompensation, wherein EUS was used to identify HCC and perform tissue acquisition as CSI was non-diagnostic.

Utilidade clínica dos escores de risco no sangramento varicoso / Clinical utility of risk scores in variceal bleeding

ABSTRACT BACKGROUND: Variceal bleeding remains important cause of upper gastrointestinal bleed. Various risk scores are used in risk stratification for non-variceal bleed. Their utility in variceal bleeding patients is not clear. This study aims to compare probability of these scores in predicting various outcomes in same population. OBJECTIVE: This study aims to compare probability of these scores in predicting various outcomes in same population. To study characteristics and validate AIMS65, Rockall, Glasgow Blatchford score(GBS), Progetto Nazionale Emorragia Digestiva (PNED) score in variceal Upper Gastrointestinal Bleed (UGIB) patients for predicting various outcomes in our population. METHODS: Three hundred subjects with UGIB were screened prospectively. Of these 141 patients with variceal bleeding were assessed with clinical, blood investigations and endoscopy and risk scores were calculated and compared to non-variceal cases. All cases were followed up for 30 days for mortality, rebleeding, requirement of blood transfusion and need of radiological or surgical intervention. RESULTS: Variceal bleeding (141) was more common than non variceal (134) and 25 had negative endoscopy. In variceal group, cirrhosis (85%) was most common etiology. Distribution of age and sex were similar in both groups. Presence of coffee coloured vomitus (P=0.002), painless bleed (P=0.001), edema (P=0.001), ascites (P=0.001), hemoglobin <7.5 gms (P<0.001), pH<7.35 (P<0.001), serum bicarbonate level <17.6 mmol/L (P<0.001), serum albumin<2.75 gms% (P<0.001), platelet count <1.2 lacs/µL (P<0.001), high INR 1.35 (P<0.001), BUN >25mmol/L (P<0.001), and ASA status (P<0.001), high lactate >2.85 mmol/L (P=0.001) were significant. However, no factor was found significant on multivariate analysis. Rockall was found to be significant in predicting mortality and rebleed. AIMS65 was also significant in predicting mortality. GBS was significant in predicting blood transfusion and need of intervention. PNED score was significant in all events except mortality. CONCLUSION: All four scores had lower predictive potential in predicting events in variceal bleed. However, AIMS65 & Rockall score were significant in predicting mortality, while GBS in predicting need of transfusion and intervention. PNED score was significant in all events except mortality.

RESUMO CONTEXTO: O sangramento varicoso permanece como importante causa de sangramento gastrointestinal superior. Vários escores são utilizados na estratificação do risco para sangramento não varicoso. Sua utilidade em pacientes de sangramento varicoso não é clara. OBJETIVO: Este estudo tem como objetivo comparar a probabilidade desses escores em prever vários desfechos na mesma população. Estudar característica e validar o AIMS65, o Rockall, a Pontuação de Glasgow Blatchford (GBS), o escore Progetto Nazionale Emorragia Digestiva (PNED), na pontuação em hemorragia gastrointestinal varicosa superior (UGIB) em pacientes para prever vários resultados em nossa população. MÉTODOS: Um total de 300 indivíduos com UGIB foram rastreados prospectivamente. Destes, 141 pacientes com sangramento varicoso foram submetidos à avaliação clínica, hematológica e endoscopia tendo seus escores de risco calculados e comparados aos casos não-varicosos. Todos os casos foram acompanhados por 30 dias para mortalidade, necessidade de transfusão sanguínea por ressangramento ou de necessidade de intervenção radiológica ou cirúrgica. RESULTADOS: O sangramento varicoso (141) foi mais comum do que não varicoso (134) e em 25 teve endoscopia negativa. No grupo varicoso, a cirrose foi a etiologia mais comum (85%). A distribuição da idade e do sexo foi semelhante em ambos os grupos. Presença de vômito colorido em borra de café (P=0,002), sangramento indolor (P=0,001), edema (P=0,001), ascite (P=0,001), hemoglobina <7,5 GMS (P<0,001), pH <7,35 (P<0,001), nível de bicarbonato sérico <17,6 mmol/L (P<0,001), albumina sérica <2,75 GMS% (P<0,001), contagem plaquetária <1,2 Lacs/μL (P<0,001), INR elevada 1,35 (P<0,001), Bun >25 mmol/L (P<0,001) e estado ASA (P<0,001), lactato elevado >2,85 mmol/L (P=0,001) foram significativos. Entretanto, nenhum fator foi encontrado como significativo na análise multivariada. Rockall foi significativo em prever a mortalidade e ressangrar. O AIMS65 também foi significante na predição da mortalidade. O GBS foi significativo na predição de transfusão sanguínea e necessidade de intervenção. O escore de PNED foi significante em todos os eventos, exceto mortalidade. CONCLUSÃO: Todos os quatro escores apresentaram menor potencial preditivo na predição de eventos em sangramento varicoso. Entretanto, o AIMS65 e o escore de Rockall foram significantes na predição da mortalidade, enquanto o GBS na predição da necessidade de transfusão e intervenção. O escore de PNED foi significante em todos os eventos, exceto mortalidade.

Massive Lower Gastrointestinal Bleeding Due to Fulminant Necrotizing Amebic Colitis: A Diagnostic and Therapeutic Challenge.

Acute fulminant necrotizing amebic colitis rarely presents with massive lifethreatening lower gastrointestinal bleeding without diarrhea. Diagnosis is difficult as colonoscopy is suboptimal due to active bleeding, stool testing is often negative and a positive serology cannot confirm the diagnosis. We herein report a case of a 39-year-old male who presented with profuse bleeding per rectum, without associated significant antecedent history of fever or diarrhea. Colonoscopy was inconclusive as active bleeding obscured the vision. Computed tomography of abdomen revealed non-specific thickening of the caecum. Emergency laparotomy with right hemicolectomy and temporary ileostomy was performed. Microscopic examination of colonic mucosa revealed Entamoeba histolytica trophozoites with erythrophagocytosis suggestive of fulminant amebic colitis. Intravenous metronidazole was given subsequently and patient recovered completely. Ileocolonic anastomosis was done after closing the ileostomy three months later. This case highlights this exceedingly rare presentation of fulminant amebic colitis which poses a diagnostic challenge and can be life threatening without early surgical intervention.

An unusual case of obstructive jaundice: ampullary Burkitt lymphoma.

Primary lymphomas of the digestive tract are uncommon heterogenous group of neoplasms that primarily affects stomach. Lymphomatous involvement of small intestine is amongst the rare lymphomas; ampullary involvement is even rarer. It is important to recognize this entity early as it mimics periampullary neoplasms and its management is different. We present the case of a 14-year-old male who presented with rapidly progressive obstructive jaundice and weight loss and ultimately was diagnosed to have ampullary Burkitt's lymphoma. Early diagnosis of this aggressive tumor and prompt induction of chemotherapy dramatically improved the patient's condition. It is crucial to consider Burkitt's lymphoma as a differential diagnosis of obstructive jaundice as both the treatment and prognosis are markedly different.

An Enigmatic Liver Mass in a Child.

Hepatic space occupying lesions in childhood are a diagnostic challenge, as they are caused by a variety of malignant and nonmalignant disorders with a different prognosis and, of course, treatment. They are often misdiagnosed or diagnosed only after surgical resection. A 14-year-old boy presented with abdominal pain, evening-rise fever with loss of appetite and weight. The patient also developed jaundice after 2 months of above symptoms. Ultrasound of the abdomen showed an irregular infiltrative mass in segment IV of the liver. Gadobenate disodium magnetic resonance imaging done showed T1 hypointense and T2 hyperintense lesions in segment VIII of the liver with extension into porta with delayed enhancement suggestive of fibrous tumor. Liver biopsy showed extensive liver parenchymal fibrosis with a mixed inflammatory infiltrate with eosinophils. Bacterial, tubercular, and fungal culture of liver biopsy were negative. Although serum IgG4 levels were 7.88 g/L (N =1.9 g/L), IgG4 staining of liver biopsy was negative. The patient was started on prednisolone 1 mg/kg considering the diagnosis of inflammatory pseudotumor (IPT). Twenty days after starting the steroid, mass lesions were converted into multicystic abscess requiring antibiotics and pigtail drainage. On follow-up, patient had improved symptoms with mass lesions turned into small-sized abscess cavity. Hepatic IPTs are difficult to differentiate from malignant tumors, as they are rare and can have variable imaging findings. To avoid inadvertent surgery, histological confirmation of the hepatic mass is essential. Steroids should be used with caution with close follow-up to prevent iatrogenic complications, such as a chronic liver abscess. HOW TO CITE THIS ARTICLE: Thanage R, Jain S, Sonthalia N, et al. An Enigmatic Liver Mass in a Child. Euroasian J Hepato-Gastroenterol 2019;9(2):104-107.

A Child with Debilitating Pruritus.

We describe a case of two-year-old boy presenting with debilitating pruritus, patchy alopecia and jaundice since the age of 6 months. On evaluation he had intrahepatic cholestasis with persistently raised serum alkaline phosphatase, normal Gamma glutamyl transferase and raised serum bile acid levels. His liver biopsy showed bland cholestasis and electron microscopy showed granular bile suggestive of progressive familial intrahepatic cholestasis type I. Medical therapy with ursodeoxycholic acid, cholestyramine, rifampicin with nutritional modification was successful in alleviating the symptoms and correcting the nutritional status. To our knowledge this is only the sixth case of progressive familial intrahepatic cholestasis type I reported from India. Herein we discuss the diagnostic and therapeutic hurdles that one encounters in managing progressive familial intrahepatic cholestasis and also review the literature regarding this rare disorder.

Congenital Esophageal Duplication Cyst: A Rare Cause of Dysphagia in an Adult.

Esophageal duplication cyst is a rare congenital embryonal gastrointestinal (GI) malformation which is diagnosed most commonly in childhood. In adults, they can present with a variety of symptoms ranging from dysphagia, chest pain, epigastric discomfort, and vomiting to more serious complications including infections, hemorrhage, and ulcerations. A 30-year-old male presented with gradually progressive dysphagia to solids for 4 months without significant weight loss. Clinical examination and routine laboratory examination were unrevealing. Upper GI endoscopy revealed a well-defined submucosal lesion bulging into the esophageal lumen involving the right antero-lateral wall of the distal esophagus. The overlying mucosa was normal with mild luminal narrowing but gastroscope could be negotiated across this narrowing. Differential diagnosis included lipoma, leiomyoma or GI stromal tumors. Contrast-enhanced computed tomography of thorax revealed a 3.5 × 2.3 × 3 cm well-defined homogenous hypodense lesion involving the right antero-lateral wall of the distal thoracic esophagus with likely possibility of submucosal or intramural lesion. Subsequently, endoscopic ultrasonography (EUS) revealed a 3.3 × 2.8 cm homogenous hypoechoic lesion without any vascularity involving the distal esophagus wall suggestive of duplication cyst. The lesion was intramural in location as muscularis propria was seen to go around the lesion. Bronchogenic cyst was excluded due to absence of cartilage and close proximity of the cyst to lumen. Fine-needle aspiration was not attempted in view of high risk of introducing infection. Being symptomatic, the patient underwent complete surgical excision of the cyst with exteriorization of the base which on histopathology confirmed duplication cyst. Esophageal duplication cysts are exceedingly rare congenital embryonal malformations with estimated prevalence of 0.0122% arising from aberration of posterior division of embryonic foregut at 3 - 4 weeks of gestation. This case shows that duplication cysts can rarely masquerade as submucosal tumor in adults and EUS is highly accurate in pre-operative diagnosis and therapeutic decision making. Literature search revealed only a handful of cases of EUS-guided diagnosis of esophageal duplication cyst reported in the literature.

Primary Esophageal Intramural Squamous Cell Carcinoma Masquerading as a Submucosal Tumor: A Rare Presentation of a Common Disease.

Esophageal squamous cell carcinoma (ESCC) is the commonest primary malignant esophageal tumor, which typically presents as endoscopically visible surface mucosal ulcerations, irregularities, or polyploidal masses. We here report a rare case of primary ESCC with completely intramural growth under a normal looking intact nondysplastic surface squamous epithelium disguising as a submucosal tumor. Upper gastrointestinal endoscopy-guided mucosal biopsy was negative for malignancy. Endoscopic ultrasound (EUS) revealed a heteroechoic solid mass originating from the muscularis propria of the distal esophagus. Cytological study of EUS-guided fine needle aspiration from the mass was suggestive of squamous cell carcinoma, which was confirmed on immunohistochemistry. There was no evidence of metastatic origin of this tumor or continuous cancer involvement from the surrounding structures, including the head, neck, and lungs on bronchoscopy, computed tomography scan, and positron emission tomography scan. Exclusive intramural squamous cell carcinoma with normal overlying mucosa is an exceedingly rare presentation of primary ESCC with only four cases reported in the literature so far. A high index of suspicion is required by the gastroenterologists and pathologists in diagnosing these cases as these tumors closely mimic the mesenchymal submucosal tumors such as lipoma, leiomyoma, and gastrointestinal stromal tumors. EUS is an indispensable tool in making a preoperative diagnosis and therapeutic decision making.

Primary hepatic amyloidosis: A case report and review of literature.

We describe a case of 42-year-old female presenting with abdominal pain associated with loss of weight and fever for 8 mo. On evaluation she had gross hepatomegaly with raised alkaline phosphatase and raised GGT levels with normal transaminases and bilirubin. On imaging she had diffuse enlargement of liver with heterogeneous contrast uptake in liver. Her viral marker and autoimmune markers were negative. Liver biopsy depicted massive deposition of amyloid in peri-sinusoidal spaces which revealed apple green birefringence on polarizing microscopy after Congo red staining. Cardiac and renal evaluation was unremarkable. Abdominal fat pad and rectum biopsy was negative for amyloid deposit. There was no evidence of primary amyloidosis as bone marrow examination was normal. Serum and urine immunofixation electrophoresis were normal. Immunoperoxidase staining for serum amyloid associated protein for secondary amyloidosis was negative from liver biopsy. We present this rare case of primary hepatic amyloidosis and review the literature regarding varied presentations of hepatic involvement in amyloidosis.

Cobb's collar occurring in two brothers in a family: A rare entity revisited.

Most urethral strictures arise from iatrogenic, traumatic, or inflammatory causes. We report the familial occurrence of a congenital bulbar urethral stricture in two brothers. Retrograde and voiding cystourethrography was performed. A Cobb's collar was diagnosed after radiological and endoscopic evaluation in both cases and was successfully managed with urethroplasty. Cobb's collar is a rarely recognized cause of a membranous stricture of bulbar urethra that can lead to several urinary problems. In cases of adolescent and young adults presenting with symptoms of progressive urinary obstruction and enuresis with or without urinary tract infection, Cobb's collar can be seen as a minor constriction in the bulbar urethra, but is not frequently symptomatic, and the familial occurrence of such a stricture is even rarer.

Fine needle aspiration diagnosis of isolated pancreatic tuberculosis: A case report.

Tuberculosis (TB) involving the pancreas are uncommon, especially when present in immunocompetent hosts. Pancreatic TB is more frequently associated with miliary TB or widely disseminated disease. Pancreatic TB may present as cystic or solid pancreatic masses, pancreatic abscess or acute or chronic pancreatitis. Majority of the cases are diagnosed after surgical exploration for presumed pancreatic malignancy and pre-operative diagnosis is quite difficult. However, improvement in imaging techniques and the resulting image-guided interventions gradually can obviate the need for more invasive diagnostic surgical procedures and expedite the planning of therapy. Herein, we report a rare case of isolated pancreatic TB which presented with pancreatic mass lesion in an immunocompetent host. Diagnosis was made by contrast enhanced computed tomography and guided fine needle aspiration of the pancreatic mass which revealed acid-fast bacilli on Ziehl-Neelsen stain. The case was treated successfully with antituberculous drugs. Pancreatic tuberculosis should be considered in the differential diagnosis of a pancreatic mass when the patient is young, residing in the endemic zone of tuberculosis. Every attempt should be made to diagnose the cases to prevent unnecessary operation.

Diagnosis and management of miliary tuberculosis: current state and future perspectives.

Tuberculosis (TB) remains one of the most important causes of death from an infectious disease, and it poses formidable challenges to global health at the public health, scientific, and political level. Miliary TB is a potentially fatal form of TB that results from massive lymphohematogenous dissemination of Mycobacterium tuberculosis bacilli. The epidemiology of miliary TB has been altered by the emergence of the human immunodeficiency virus (HIV) infection and widespread use of immunosuppressive drugs. Diagnosis of miliary TB is a challenge that can perplex even the most experienced clinicians. There are nonspecific clinical symptoms, and the chest radiographs do not always reveal classical miliary changes. Atypical presentations like cryptic miliary TB and acute respiratory distress syndrome often lead to delayed diagnosis. High-resolution computed tomography (HRCT) is relatively more sensitive and shows randomly distributed miliary nodules. In extrapulmonary locations, ultrasonography, CT, and magnetic resonance imaging are useful in discerning the extent of organ involvement by lesions of miliary TB. Recently, positron-emission tomographic CT has been investigated as a promising tool for evaluation of suspected TB. Fundus examination for choroid tubercles, histopathological examination of tissue biopsy specimens, and rapid culture methods for isolation of M. tuberculosis in sputum, body fluids, and other body tissues aid in confirming the diagnosis. Several novel diagnostic tests have recently become available for detecting active TB disease, screening for latent M. tuberculosis infection, and identifying drug-resistant strains of M. tuberculosis. However, progress toward a robust point-of-care test has been limited, and novel biomarker discovery remains challenging. A high index of clinical suspicion and early diagnosis and timely institution of antituberculosis treatment can be lifesaving. Response to first-line antituberculosis drugs is good, but drug-induced hepatotoxicity and drug-drug interactions in HIV/TB coinfected patients create significant problems during treatment. Data available from randomized controlled trials are insufficient to define the optimum regimen and duration of treatment in patients with drug-sensitive as well as drug-resistant miliary TB, including those with HIV/AIDS, and the role of adjunctive corticosteroid treatment has not been properly studied. Research is going on worldwide in an attempt to provide a more effective vaccine than bacille Calmette-Guérin. This review highlights the epidemiology and clinical manifestation of miliary TB, challenges, recent advances, needs, and opportunities related to TB diagnostics and treatment.

Treatment of hypertrophic osteoarthropathy in the case of pulmonary metastasis secondary-to-nasopharyngeal carcinoma with zoledronic acid: an enlightening experience.

We describe a case of 23-year-old man, who presented with painful hypertrophic pulmonary osteoarthropathy involving bilateral upper and lower extremities, resulting from intrathoracic metastasis. The patient had a history of undifferentiated nasopharyngeal carcinoma which was treated successfully 2 years ago. The painful osteoarthropathy had made the patient incapacitated. A single dose of 4 mg of intravenous zoledronic acid (ZA) was given which resulted in complete resolution of pain along with reduction of swelling. There was no recurrence on follow-up. Bisphosphonates by their action on bone metabolism might alleviate the symptoms and its use should be encouraged in cancer patients with debilitating arthropathies. This article aims at highlighting the role of bisphosphonates, particularly ZA in managing patients with hypertrophic osteoarthropathy (HOA) and sharing our experience with this drug because of the rarity of the condition and lack of sufficient data in the medical literature.

Syringomyelia, limb hypertrophy and sympathetic overactivity: a rare association.

A 32-year-old man presented with uniform enlargement of right upper limb for 6 years. Examination revealed painful disorganised elbow joint along with sensory impairment in the affected limb with weakness of small muscles. The patient was given empirical antileprotic therapy from outside without any benefit. Ultrasonography showed pan-hypertrophic nature of local tissues. Although axonal type of sensorimotor neuropathy involving right ulnar and median nerve was detected in nerve conduction velocity study, biopsy of the same failed to confirm any axonal degeneration or evidence of leprosy. Considering the nature of sensory abnormality MRI of cervical spine was done which revealed a syrinx extending from C3 till D2. Tests for vasomotor tone showed positive results on the affected limb. Syringomyelia can rarely give rise to pan-hypertrophy of limb due to sympathetic overactivity, which is infrequently reported in literatures and deficient in logical grounds.

Lambert-Eaton myasthenic syndrome and solitary cerebellar metastasis in a patient with occult small-cell lung cancer: a rare experience.

The authors describe a case of 60-year-old male patient presented with a 6 month history of progressive weakness of all the four limbs, ataxia, droopy eyelids and bulbar features. Further laboratory and electrodiagnostic studies confirmed the diagnosis of Lambert-Eaton myasthenic syndrome (LEMS). MRI of the brain showed a cerebellar tumour which, following surgery was revealed to be a metastatic small-cell lung carcinoma. Paraneoplastic LEMS together with solitary cerebellar metastasis was diagnosed but no evidence of primary malignancy was detected. An extensive search for related malignancies failed to get any clue. The patient underwent a total surgical excision of tumour and the histopathology revealed a metastatic small cell carcinoma. This case highlights that rarely both paraneoplastic LEMS and cerebellar mass can precede the primary malignancy causing them.