Invasive Fungal Disease in Pediatric Acute Leukemia in the Nontransplant Setting: 8 Years' Experience From a Tertiary Care Center in North India.

BACKGROUND/AIM: The aim of this article is to study the spectrum, changing prevalence, and predictors for mortality of invasive fungal disease (IFD) in pediatric leukemia in a resource-limited setting. OBSERVATIONS: Prevalence was 7% (proven, 69%; probable, 16.4%; possible, 14.6%) and did not differ between acute lymphoblastic leukemia and acute myeloid leukemia. Lungs were frequently involved (46%). Aspergillus was the commonest fungus (47%). Visceral abscesses were frequent with candidiasis as compared with invasive molds (P=0.016). IFD resulted in a prolonged admission (mean, 12.6±2 d; P=0.014) and death (44%) (Aspergillus, 50%; Candida, 50%; Mucor, 34%). Diagnosis of acute myeloid leukemia predicted mortality (P=0.03). CONCLUSIONS: IFD was an important cause of treatment related mortality in pediatric leukemia (odds ratio, 8.39). Protocolled use of computed tomography-chest and galactomannan-assay aided diagnosis (P<0.05).

Pediatric Rhabdomyosarcoma in India: A Single-center Experience.

OBJECTIVE: Analyze the profile and outcome of children with rhabdomyosarcoma from a pediatric-oncology unit. DESIGN: Retrospective analysis of case records over 23 years (1990-2012). SETTING: Government-run, tertiary-care, university hospital in Northern India. PARTICIPANTS: 159 children (<12-years) with a diagnosis of rhabdomyosarcoma were enrolled. The median age was 4 years; 13% were infants. MAIN OUTCOME MEASURE: Five-year event free survival. RESULTS: The median symptom interval was 2-months. Head and neck region was the most frequent site (44%), followed by tumors in the extremity (15.7%). The majority (67%) of the tumors were located at 'unfavorable' sites; 68% were >5 cm in size. The most frequent (58%) pathological subtype was embryonal. Treatment was based on the 'Intergroup Rhabdomyosarcoma Study (IRS) Group' risk-stratification. 33% were 'low-risk' children, 11% were 'high-risk'. Treatment-refusal (18%) and abandonment (33%) were major impediments. The median ± SE five-year event free survival of those taking treatment was 43.6 ± 6%. CONCLUSION: Large sized tumors, tumors at unfavorable locations, and treatment refusal/abandonment contributed to inferior outcome in children with rhabdomyosarcoma.

Liver acoustic radiation force impulse (ARFI) in childhood obesity: comparison and correlation with biochemical markers.

PURPOSE: To compare and correlate the diagnostic efficiency of acoustic radiation force impulse (ARFI) elastography with biochemical markers for assessing hepatic changes in overweight and obese children. METHODS: This prospective study was approved by the institutional ethics committee. It included 54 overweight and obese children and 50 normal children (as a control group) in the age range 5-18 years. For all children, we performed grayscale ultrasonography to diagnose fatty liver, ARFI elastography to measure liver stiffness, and biochemical evaluation for aspartate aminotransferase (AST), alanine aminotransferase (ALT), and serum triglyceride (TG) levels. RESULTS: Of the 54 obese children, AST was elevated in 13 (24.1%) and ALT was elevated in 16 (29.6%); however, only 4 (25%) of these 16 obese children with abnormal aminotransferase levels had an AST/ALT ratio >0.8. Furthermore, all children with abnormal aminotransferase levels with AST/ALT ratio >0.8 also had abnormal readings of ARFI elastography. The TG was elevated (>150 mg/dL) in 2 out of 54 (3.7%) obese children. None of the normal children showed abnormal levels of aminotransferase and TG. Three out of 54 (5.6%) obese children did not show fatty liver changes, while 29 (53.7%) showed grade-I fatty liver changes, and 22 (40.7%) showed grade-II fatty liver changes. The mean (SD) ARFI value was 1.13 m/s (SD 0.199) for obese children and 1.02 m/s (SD 0.11) for children in the control group. Of the 54 obese children, 49 (90.7%) showed ARFI values of <1.19 m/s (normal), 4 (7.4%) had ARFI values from >1.19 to <1.75 m/s, and 1 (1.9%) had an ARFI value >1.75 m/s. Four children with an increased ARFI value also had an AST/ALT ratio >0.8. However, one obese child with a raised ARFI value did not have an elevated AST/ALT ratio, and none of his aminotransferase levels were abnormal. All normal children had ARFI values <1.19 m/s. CONCLUSION: ARFI elastography shows excellent correlation with AST/ALT ratios in obese children and may be used as a noninvasive tool to detect nonalcoholic fatty liver disease (NAFLD) and associated hepatic changes, especially in pediatric patients, for whom liver biopsy is not always feasible.

Omental infarction: An unusual cause of right iliac fossa pain in children.

Omental infarction is an uncommon cause of acute abdomen in the pediatric population. We report a case of a 4-year-old male child with right iliac fossa pain. The final diagnosis was made on ultrasound and computed tomography findings. This entity needs to be differentiated from acute conditions like appendicitis, avoiding surgery.

Comparative analysis of spherical and fusiform choledochal cyst based on three-dimensional magnetic resonance cholangiopancreatography, biliary amylase, and histopathological examination.

AIMS: The aim was to compare biliary amylase, common channel, and gall bladder/liver histopathology between spherical and fusiform choledochal cysts. MATERIALS AND METHODS: Children undergoing cyst excision with Roux-en-Y hepatico-jejunostomy over a 17 months period were prospectively studied. The common channel was assessed by three-dimensional (3D) magnetic resonance cholangiopancreatography (MRCP). RESULTS: Among 22 patients (spherical = 10, fusiform = 12), there was a higher incidence of spherical cysts in infants (5/7-71.4%) and fusiform cysts in older children (10/15-66.7%) (P = 0.09). Common channel identified in 14 (64%) cases was long (>10 mm) in 5 (38.2%) (one spherical, four fusiform [P = 0.5]) with associated high biliary amylase levels (>500 IU/L) in four (one spherical, three fusiform) (P = 0.05). Exact point of junction of common bile duct with pancreatic duct was visible with coronal half-Fourier-acquisition single-shot turbo spin-echo and 3D sampling perfection with application with optimize contrast using different flip angle evolution sequence using a reformatted plane of axis, particularly coronal, and coronal oblique orientation. Biliary amylase was raised (>100 IU/L) in 4 (40%) in the spherical group, compared to 8 (67%) in the fusiform group. Mean biliary amylase was similar in gallbladder and cyst in spherical but higher in gallbladder in fusiform cysts. Mean biliary amylase was <500 IU/L in 85.7% infants. Five out of 7 infants had liver fibrosis or cirrhosis on histopathology (P = 0.05). There were no dysplastic changes in the gallbladder epithelium. CONCLUSION: Three-dimensional MRCP delineated the common channel in two-third cases especially in coronal and coronal oblique orientation. The long common channel may have an etiological role in fusiform cysts. Spherical cysts, especially in infants, have a higher incidence of obstructive cholangiopathy.

Clinical application of 'Justification' and 'Optimization' principle of ALARA in pediatric CT imaging: "How many children can be protected from unnecessary radiation?".

RATIONALE AND OBJECTIVES: Practice of ALARA (as low as reasonably achievable) principle in the developed world is currently well established. However, there is striking lack of published data regarding such experience in the developing countries. Therefore, the goal of this study is to prospectively evaluate CT request forms to assess how many children could be protected from harmful radiation exposure if 'Justification' and 'Optimization' principles of ALARA are applied before obtaining CT imaging in a developing country. This can save children from potential radiation risks including development of brain cancer and leukemia. MATERIAL AND METHODS: Consecutive CT request forms over a six month study period (May 16, 2013 to November 15, 2013) in a tertiary pediatric children's hospital in India were prospectively reviewed by two pediatric radiologists before obtaining CT imaging. First, 'Justification' of CT was evaluated and then 'Optimization' was applied for evaluation of appropriateness of the requested CT studies. The number (and percentage) of CT studies avoided by applying 'Justification' and 'Optimization' principle of ALARA were calculated. The difference in number of declined and optimized CT requests between CT requests from inpatient and outpatient departments was compared using Chi-Square test. RESULTS: A total of 1302 consecutive CT request forms were received during the study period. Some of the request forms (n=86; 6.61%) had requests for more than one (multiple) anatomical regions, hence, a total of 1392 different anatomical CT requests were received. Based on evaluation of the CT request forms for 'Justification' and 'Optimization' principle of ALARA by pediatric radiology reviewers, 111 individual anatomic part CT requests from 105 pediatric patients were avoided. Therefore, 8.06% (105 out of 1302 pediatric patients) were protected from unnecessary or additional radiation exposure.The rates of declined or optimized CT requests from inpatient department was significantly higher than that from outpatient departments (p<0.05). CONCLUSIONS: A substantial number of pediatric patients, particularly coming from outpatient departments, can be protected from unnecessary or additional radiation exposure from CT imaging when 'Justification' and 'Optimization' principle of ALARA are applied before obtaining CT imaging in a developing country.

Hydronephrosis: Comparison of extrinsic vessel versus intrinsic ureteropelvic junction obstruction groups and a plea against the vascular hitch procedure.

INTRODUCTION: Pediatric ureteropelvic junction obstruction (UPJO) due to an extrinsic crossing vessel (CV) is rare and often remains undiagnosed preoperatively. Vascular hitch procedures are often performed as associated intrinsic obstruction is not expected. We compared data and intravenous urography (IVU) findings of patients with aberrant CV versus those with intrinsic UPJO, all undergoing open dismembered pyeloplasty. PRIMARY OBJECTIVE: Is accurate pre-operative diagnosis of aberrant CV causing extrinsic UPJO possible? SECONDARY OBJECTIVE: To assess differences in the demographic, clinical, radiological, intra-operative features and postoperative improvement after pyeloplasty between patients with a CV and those with only intrinsic UPJO. PATIENTS AND METHODS: Prospective study of all children below 12 years with UPJO presenting to a tertiary referral centre and who underwent open Anderson - Hynes dismembered pyeloplasty between 2003 and 2013 was conducted. Pre-operative investigations included serial ultrasonography, renal dynamic [ethylene di-cysteine (EC)] scan and IVU. These were repeated 3 months after pyeloplasty. Pre-operative IVUs of children with CV were compared with the IVUs of an equal number of similar aged children, randomly selected from the intrinsic obstruction group. RESULTS: Pyeloplasty was performed in 643 children during the study period. Data of 33 children with aberrant CVs (mean age 6.99 years) were compared with the remaining 610 children (mean age 3.27 years) with only intrinsic obstruction. Highly significant associations of those with CV included age above 2 years, female gender, associated anomalies, abdominal pain in those above 2 years and poor preoperative function on IVU. Specific IVU features which were statistically highly significant in favor of presence of CV were small, intrarenal and globular flat bottomed pelvis. (Figure) Calyceal dilatation was also more prominent in the CV group. A funnel shaped, extrarenal pelvis was highly significant in favor of intrinsic obstruction. There was associated intrinsic obstruction in addition to CV obstruction in 8 children. All children symptomatically improved after pyeloplasty and did well on long term follow up. The majority showed improvement or stabilization of function on EC scan. DISCUSSION: With the advent of antenatal ultrasonography, most children with UPJO are detected early. Children with CV tend to present later. This is often detected during surgery. Color Doppler is useful but is operator dependant and not performed routinely. In this study, IVU showed the presence of obstruction and loss of function unlike color Doppler, but also revealed specific diagnostic features not previously reported in literature. This can help in accurate preoperative prediction and avoid endopyelotomy, or a dorsal lumbotomy/retroperitoneal approach. Renal function in CVs is expected to be good as the obstruction is thought to be intermittent. However, we noted delayed contrast uptake on IVU in 60.6% and differential renal function on EC scan below 40% in 17 patients (56.6%). These indicate the effect of the obstruction on the renal parenchyma and the importance of early detection. Higher association with other anomalies and higher incidence in females has also not been emphasized in the literature so far. We noted associated intrinsic obstruction in 24.24% patients which is highly significant. This category of patients is likely to be missed and inappropriately treated if a "vascular hitch procedure" is performed. None of our patients had postoperative complications. CONCLUSIONS: Characteristic features were seen on IVU helping in preoperative diagnosis which can be extrapolated to magnetic resonance urography. There is a higher association of CV in age above 2 years, females, associated congenital anomalies, delayed uptake on IVU and differential renal function below 40% compared to intrinsic obstruction. Associated intrinsic obstruction in 24% with no postoperative complications indicates the superiority of dismembered pyeloplasty over vasculopexy procedures.

Infracardiac total anomalous pulmonary venous return: an unusual cause of neonatal portal vein enlargement.

We present an unusual cause of aneurysmal dilatation of portal vein in a neonate in a case of infracardiac total anomalous pulmonary venous return. The common descending pulmonary vein was seen joining below the diaphragm with the left branch of portal vein, which was terminating as a blind-ending, aneurysmally dilated vascular channel.

Intravenous contrast enhanced computed tomography colonoscopy in children with suspected colonic polyps.

OBJECTIVE: The purpose of this study was to evaluate the diagnostic performance of intravenous contrast enhanced computed tomographic colonoscopy (IVCTC) in the diagnosis of clinically suspected colorectal polyps in children, using conventional colonoscopy (CC) as the gold standard. METHODS: This was a prospective study conducted between July 2008 and June 2010. 30 pediatric patients with history of rectal bleeding and clinically suspected to have colorectal polyps were enrolled. All of the patients underwent IVCTC followed by CC. 30 IVCTC and 31 CC were performed in 30 patients. The findings of IVCTC were compared with those of CC. Statistical analysis was performed to obtain diagnostic performance values of IVCTC on per polyp (sensitivity and positive predictive value) and per patient (sensitivity, specificity, positive predictive value and negative predictive value) basis. RESULTS: By IVCTC, 63 polyps were detected in 28 patients of which 53 polyps were eligible for inclusion in the statistical analysis. 60 polyps were detected by CC in 28 patients of which 50 polyps were eligible for inclusion in the statistical analysis. The per polyp sensitivity and positive predictive values were 94% and 88.6% respectively. The per patient sensitivity, specificity, positive predictive value, and negative predictive values were 96.4, 50, 96.4, and 50% respectively. Twenty polyps, in 10 patients, were visualized only after intravenous contrast administration of which 5 polyps, in 5 patients, were likely to have been missed in the absence of the intravenous contrast injection as these polyps were submerged in fluid. Four patients would have had a false negative CTC examination if the intravenous contrast had not been injected; while in another patient, the number of polyps would have been underestimated. CONCLUSION: CTC is capable of serving as a safe and efficient non-invasive tool for evaluating children with clinically suspected colorectal polyps. Administration of intravenous contrast improves the sensitivity of polyp detection on CTC.

Role of abdominal sonography in the preoperative diagnosis of extrahepatic biliary atresia in infants younger than 90 days.

OBJECTIVE: The objective of our study was to prospectively assess the role of abdominal sonography in the preoperative diagnosis of extrahepatic biliary atresia (EHBA) in infants younger than 90 days. SUBJECTS AND METHODS: Sonography was performed in 99 infants younger than 90 days with conjugated hyperbilirubinemia (total bilirubin > 3 mg/dL, conjugated bilirubin > 20% of total) after 4 hours of fasting. They were evaluated for the "triangular cord" sign, the presence and morphology of the gallbladder, gallbladder contraction after oral feeding, the presence and diameter of the common bile duct (CBD), liver size and echotexture, spleen size, caliber of the right branch of the hepatic artery, and caliber of the right branch of the portal vein. The final diagnosis of EHBA was made on basis of surgery. The performance of sonography in the diagnosis of EHBA was evaluated. RESULTS: The study group was composed of 68 boys and 31 girls (age range, 13-89 days); of the 99 infants, 30 had EHBA. The triangular cord sign had a sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) of 23.3%, 97.1%, 77.8%, and 74.4%, respectively. The gallbladder was not visualized in seven infants, all of whom had EHBA. The sensitivity, specificity, PPV, and NPV of an abnormal gallbladder were 83.3%%, 82.6%, 67.6%, and 91.9%, respectively, and for noncontraction of the gallbladder were 87%, 72.5%, 51.3%, and 94.3%, respectively. A nonvisualized CBD had a sensitivity, specificity, PPV, and NPV of 93.3%, 47.8%, 43.8%, and 94.3%, respectively. A negative triangular cord sign with normal gallbladder morphology had an NPV of 91.9% for excluding EHBA. CONCLUSION: Comprehensive sonographic evaluation can help in segregating infants at high risk of EHBA from those at low risk.

Does all small bowel intussusception need exploration?

Small bowel intussusception (SBI) in infants and children are ususally associated with a lead point. With increased use of radiological investigations, more idiopathic SBIs are identified. As reduction by hydrostatic or air enema are less successful in these cases, most of them require surgical exploration in children. However, now many cases of SBI have been found to reduce spontaneously. We report two cases of SBI with spontaneous reduction and review the literature for the management guidelines.

Role of tissue harmonic imaging in characterization of cystic renal lesions.

OBJECTIVE: To determine the utility of tissue harmonic imaging in evaluating cystic renal lesions and to compare these findings with conventional ultrasound guidance (USG) and CT. METHODS: Thirty patients, detected with cystic renal lesions on routine USG (over a period of 18 months from July 2004 to December 2005 at the Postgraduate Institute of Medical Education and Research Chandigarh, Chandigarh, India) were included in this study. All patients underwent a conventional gray scale ultrasound study (GSI), followed by tissue harmonic imaging (THI) sonography on the same machine (advance technology limited high definition imaging 5000). Computed tomography of abdomen was carried out within one week of the ultrasound examinations. All images were evaluated for size, number, and location of lesions. The findings of THI sonography, conventional USG and CT of abdomen were recorded in their respective proformas. The images obtained by GSI, THI, and contrast enhanced CT were also evaluated for image quality, lesion conspicuity, and fluid-solid differentiation. RESULTS: Tissue harmonic imaging showed better image quality in 27 of 34 lesions, improvement in lesion conspicuity was found in 27 of 34 cystic lesions, and an improved solid-fluid differentiation in 30 of 34 lesions when compared to GSI. The THI provided additional information as compared to GSI in 8 patients. The grading of CT scan was significantly higher in overall image quality (p=0.007) and lesion conspicuity (p=0.004), but was non-significant for fluid-solid differentiation (p=0.23). CONCLUSION: Tissue harmonic imaging provides better image quality, lesion delineation, and superior characterization than conventional gray scale sonography.