Diagnostic accuracy of barium enema versus full-thickness rectal biopsy in children with clinically suspected Hirschsprung's disease: A comparative cross-sectional study.

Background and Aims: Hirschsprung's disease (HSD) remains a common cause of pediatric intestinal obstruction. Barium contrast enema (BE) is the primary imaging modality for the evaluation of clinically suspected cases. Here, we aimed to assess the diagnostic accuracy of BE in children with clinically suspected HSD when compared to a gold standard full-thickness rectal biopsy (FTRB). Methods: We recruited and consecutively enrolled children with clinically suspected HSD at two tertiary teaching hospitals. Participants underwent BE imaging and two radiologists interpreted the findings independently. Participants further underwent FTRB by pediatric surgeons as the confirmatory test. Sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and receiver operating characteristics (ROC) with the area under the curve (AUC) were calculated on Stata version 14.2, taking FTRB as the standard. Results: We enrolled 55 cases, of which 49 completed the evaluation and were included in the final analysis. The median age was 9.4 months (interquartile range: 2-24], with a male-to-female ratio of 4.4:1. The sensitivity, specificity, PPV, and NPV of BE were 0.95 (95% confidence interval [CI] [0.81-0.99]), 0.73 (95% CI [0.39-0.94]), 0.92 (95% CI [0.82-0.97]), and 0.80 (95% CI [0.50-0.94]), respectively. On AUC, the diagnostic accuracy of BE compared to the confirmatory FTRB was 0.84 (95% CI [0.69-0.98]). The diagnostic accuracy was higher in neonates (ROC: 1.00) when compared to infants (ROC: 0.83) or those above 1 year of age (ROC: 0.798). HSD-suggestive BE findings were associated with absence of ganglion cells on FTRB (χ 2 = 23.301, p < 0.001). Inverted rectosigmoid ratio and transition zone were more sensitive in detecting HSD of 0.92 (95% CI [0.74-0.98]) and 0.81 (95% CI [0.63-0.92]), respectively. Conclusion: BE is sufficiently accurate in the diagnosis of children with HSD, suggesting BE would likely be used to inform surgical management in settings where confirmatory biopsy is lacking. However, clinical judgment is warranted in interpreting negative BE findings.

Effects of sedatives on radiologic enema reduction in children with ileocolic intussusception: A systematic review and meta-analysis.

BACKGROUND: In children with ileocolic intussusception, sedatives such as midazolam, ketamine and propofol may facilitate radiologic enema reduction, but studies on their separate and joint effects remain controversial. OBJECTIVES: We aimed to systematically analyze studies for the effects of sedatives on the radiologic reduction of ileocolic intussusception in children. METHODS: We searched PubMed, EMBASE, CINAHL, Scopus and Web of Science from database inception through March 2023 for articles that enrolled children with ileocolic intussusception who underwent non-operative pneumatic or hydrostatic enema reduction under ultrasound or fluoroscopic guidance with or without the use of sedatives. The primary and secondary outcomes were success rate in radiologic reduction of ileocolic intussusception and risk of perforation, respectively. Effect estimates from the individual studies were extracted and combined using the Hartung-Knapp-Sidik-Jonkman log-odds random-effects model. Heterogeneity between studies was checked using Cochran's Q test and the I2 statistic. RESULTS: A total of 17 studies with 2094 participants were included in the final review, of which 15 were included in the meta-analysis. Nine studies reported on the success rate of radiologic reduction performed under sedation in all participants, while six studies compared the success rate in two patient groups undergoing the procedure with or without sedation. The pooled success rate of non-operative reduction under sedation was 87 % (95 % CI: 80-95 %), P = 0.000 with considerable heterogeneity (I2 = 85 %). A higher success rate of 94 % (95 % CI: 88-99 %) and homogeneity (I2 = 12 %) were found in studies with pneumatic enema reduction. Among comparative studies, the odds of success of non-operative reduction were increased when the procedure was performed under sedation, with a pooled odds ratio of 2.41 (95 % CI: 1.27-4.57), P = 0.010 and moderate heterogeneity (I2 = 60 %). In a sensitivity analysis, homogeneity was found between analyzed studies when two outliers were excluded (I2 = 0.73 %). The risk of perforation was not significantly different (OR 1.52, 95 % CI: 0.09-23.34), P = 0.764 indicating small study effects. No publication, bias was detected on visual inspection of the funnel plots or the Begg's and Egger's bias tests. Most studies were categorized as having a low risk of bias using Joanna Briggs Institute checklists. CONCLUSIONS: In selected patient groups, sedation can increase the success rate of radiologic enema reduction in children with ileocolic intussusception without evidence of increased risk of perforation. Systematic review protocol registration: PROSPERO CRD42023404887.

Jarcho-Levin syndrome with diastematomyelia and a dorsal dermal sinus.

Jarcho-Levin syndrome (JLS) is a congenital dysostosis characterized by multiple vertebral and intrinsic rib abnormalities. JLS and neural tube abnormalities rarely occur together. There have been few cases of JLS associated with a split spinal cord malformation (diastematomyelia). A dorsal dermal sinus is a tract from the skin that may end in soft tissue, epidural space, or most commonly intradural. We report the case of a 5-day-old male neonate with JLS who presented with respiratory distress immediately after birth. A chest radiograph revealed multiple bilateral asymmetric rib deformities and irregular rib fusions, multi-level segmentation defects of the thoracic vertebrae, and associated dextroconvex scoliosis of the thoracic spine. He was subsequently diagnosed with diastematomyelia, a dorsal dermal sinus, and tethered cord on ultrasound. The infant succumbed to respiratory distress from superimposed pneumonia. JLS is rarely associated with distematomyelia, and there are only ten reports worldwide. We presented the eleventh case of JLS with type 2 diastematomyelia. In addition, this is the first reported case of co-occurrence with a dorsal dermal sinus.

Madura foot and a continued diagnostic enigma: Dot-in-circle sign on magnetic resonance imaging and ultrasound.

Madura foot is a chronic granulomatous disease of the skin and underlying tissues caused by fungi or bacteria. Early diagnosis is important to avoid disfiguring limb deformities. Low clinical suspicion, limited availability of diagnostic tools, and infection with fastidious organisms may lead to misdiagnosis and delayed treatment. Imaging tests can help to make a timely diagnosis in a non-invasive manner. Here we report two patients with a non-classical clinical presentation and a more favorable differential diagnoses who were correctly diagnosed as cases of Madura foot based on the ''dot-in-circle sign'', a specific finding on magnetic resonance imaging and ultrasound.

Beckwith-Wiedemann syndrome in a child with multifocal Wilms tumor and lateralized overgrowth: A case report.

Beckwith-Wiedemann syndrome (BWS) is a rare imprinting disorder and overgrowth syndrome with a prevalence of 1 in 10,000 live births. It is characterized by predilection for embryonal tumor growth, especially Wilms tumor (WT), and manifestations like lateralized overgrowth/hemihypertrophy, macroglossia, macrosomia, anterior abdominal wall defects, and hyperinsulinism. Our case is a 1 year of female child who presented with abdominal swelling and limb length discrepancies. A clinical diagnosis of BWS was made based on multifocal WT and hepatomegaly and nephromegaly detected on contrast-enhanced abdominal computed tomography and physical examination findings of lateralized overgrowth and umbilical hernia. A molecular genetic test was not available. The patient was started on preoperative chemotherapy with good tolerance. Clinical criteria can be used to diagnose WBS in a setting where confirmatory molecular testing is unavailable. This will considerably change approaches to management of presenting complications such as WT .

Congenital extrahepatic portosystemic shunt masquerading as chronic portal vein thrombosis: A case report.

INTRODUCTION AND IMPORTANCE: Congenital extra hepatic portosystemic shunt (CEPS) is a rare vascular malformation in which splanchnic and portal blood is shunted into the systemic circulation eluding the liver. Type 1 CEPS is sometimes difficult to differentiate from pathologies such as chronic portal veinthrombosis as the portal vein may not be visualized in either entities. CASE PRESENTATION: A 3-year-old male child with a week of abdominal pain was diagnosed with chronic portal vein thrombosis in an out-of-hospital setting. Repeat abdominal ultrasound was done at our institution and we were able to visualize termination of the portal vein to the suprarenal infra-hepatic inferior vena cava with an end to side pattern and a focal hypoechoic hepatic lesion at segment eight of the liver. There was no evidence of cavernous transformation or sign of portal hypertension. Subsequently, tri-phasic computed tomography revealed similar findings, with the portal vein terminating at the suprarenal inferior vena cava. The focal hepatic lesion showed peripheral contrast enhancement in the arterial phase and appeared as a central non-enhancing area with evidence of homogeneous enhancement on the subsequent sequences. CLINICAL DISCUSSION: Type 1 CEPS can be easily confused with chronic portal vein thrombosis as the portal vein may not be visible and the hepatic artery shows compensatory enlargement in both entities. However, portal vein thrombosis is usually associated with underlying predisposing factors and can result in the development of secondary signs of portal hypertension and cavernous transformation which are critical to distinguish it from CEPS. CONCLUSION: Chronic portal vein thrombosis is a great mimicker that should be distinguished from CEPS on ultrasound. A meticulous scan with color flow is helpful to scrutinize vascular anatomy, identify findings associated with CEPS such as hepatic lesions, and exclude signs of chronic portal vein thrombosis.

Chest X-ray predicts cases of pulmonary tuberculosis among women of reproductive age with acute respiratory symptoms: A multi-center cross-sectional study.

Background: Tuberculosis (TB) prevalence is increasing among women of reproductive age (WRA) in sub-Saharan Africa, yet undiagnosed and untreated cases remain rather high with serious health and socio-economic consequences. We aimed to assess the prevalence and predictors of TB in WRA seeking health care for acute respiratory symptoms. Methods: We consecutively enrolled outpatient WRA with acute respiratory symptoms seeking care at four healthcare facilities in Ethiopia between July 2019 and December 2020. Data on sociodemographic characteristics and clinical information were collected using a structured questionnaire administered by trained nurses. Posteroanterior chest X-ray was performed in non-pregnant WRA and interpreted independently by two radiologists. Sputum samples were collected from all patients and tested for pulmonary TB using Xpert MTB/RIF and/or smear microscopy. Predictors of bacteriologically confirmed TB cases were determined using binary logistic regression, with clinically relevant variables included in the final Firth's multivariate-penalized logistic regression model. Results: We enrolled 577 participants, of whom 95 (16%) were pregnant, 67 (12%) were living with HIV, 512 (89%) had cough of less than 2 weeks, and 56 (12%) had chest-x-ray findings suggestive of TB. The Overall prevalence of TB was 3% (95% CI: 1.8%-4.7%) with no significant difference observed between patient groups categorized by duration of cough or HIV serostatus (P-value = 0.9999). In multivariable analysis, TB-suggestive CXR abnormality (AOR 18.83 [95% CI, 6.20-57.18]) and history of weight loss (AOR 3.91 [95% CI, 1.25-12.29]) were associated with bacteriologically-confirmed TB cases. Conclusions: We found a high TB prevalence among low-risk women of reproductive age with acute respiratory symptoms. Routine CXR may improve early case detection and thereby TB treatment outcomes.

Giant pulmonary artery aneurysm in a child: Rare complication of congenital heart disease.

Key Clinical Message: This case report aims to increase awareness that pulmonary artery aneurysms may occur as a complication of neglected patent ductus arteriosus and should be sought in children with ill-treated congenital heart diseases. Abstract: Pulmonary artery aneurysm is a rare anomaly with an autopsy prevalence of 1:14,000. These aneurysms can arise secondary to various etiologies, with congenital causes identified in 25% of cases and congenital heart diseases (CHD) responsible for more than half of these cases. A 12-year-old boy with CHD in the form of patent ductus arteriosus (PDA) and irregular clinical follow-up presented with new onset fatigue of 3 months duration. A physical examination revealed anterior chest wall bulging and a continuous murmur. A chest radiograph showed a smooth left hilar region opacity that has a close relation with the left cardiac border. Transthoracic echocardiogram shows no progression from the previous one; there was a large PDA and pulmonary hypertension, but no further information was available. Computed tomography angiography revealed a giant aneurysm of the main pulmonary artery (PA), with a maximum diameter of 8.6 cm, and dilatation of its branches of 3.4 and 2.9 cm for the right and left PA, respectively.

Congenital extrahepatic Portosystemic shunt with hypoplasia of the intrahepatic inferior vena cava: A rare case report.

Congenital portosystemic shunt (CPS) is a rare vascular anomaly resulting in diversion of splanchnic or portal blood into the systemic circulation. Other vascular malformations associated with this entity are uncommon. A 4-year-old female child with a diagnosis of acute viral hepatitis had an incidental finding of extrahepatic CPS on a Doppler abdominal ultrasound. Contrast-enhanced computed tomography showed a dilated portal vein having H-type side-to-side communication with a hypoplastic intrahepatic portion of the inferior vena cava and a prominent dilated azygos vein. There was retroaortic left renal vein drained into the IVC which was visualized in its entirety. Echocardiography findings were normal and the patient was discharged after symptomatic treatment that achieved improvement. With the expansion of abdominal imaging, incidental cases of CPS are increasingly being diagnosed in children. Although vascular malformations associated with CPS are rare, early diagnosis of cases helps avoid complications during shunt closure.

Utility of chest imaging in the diagnosis and management of patients with visceral leishmaniasis: A systematic review.

Objectives: Visceral leishmaniasis remains a deadly parasitic disease with diagnostic complexities. Currently, point-of-care chest imaging is gaining momentum in the diagnosis of infectious diseases. Respiratory symptoms are common in visceral leishmaniasis. Here we aimed to systematically synthesize the evidence on the utility of chest imaging on the diagnosis and management of patients with visceral leishmaniasis. Methods: We searched PubMed, Scopus, Web of Science, ScienceDirect, and Google Scholar databases for studies reporting chest imaging findings in patients with visceral leishmaniasis, published in English from database inception to November 2022. We used the Joanna Briggs Institute checklists to evaluate the risk of bias. The protocol of this systematic review was registered with the Open Science Framework: https://doi.org/10.17605/OSF.IO/XP24W. Results: Of 1792 studies initially retrieved, 17 studies with 59 participants were included. Of the 59 patients, 51% (30) had respiratory symptoms and 20% (12) were human immunodeficiency virus co-infected. Chest X-ray, high-resolution computed tomography, and chest ultrasound findings were available for 95% (56), 93% (55), and 2% (1) of the patients, respectively. The most common findings were pleural effusion (20%; 12), reticular opacities (14%; 8), ground-glass opacities (12%; 7), and mediastinal lymphadenopathies (10%; 6). High-resolution computed tomography was more sensitive than chest X-ray and detected lesions that were lost on chest X-ray, 62% (37) versus 29% (17). In almost all cases, regression of the lesions was observed with treatment. Microscopy of pleural or lung biopsy detected amastigotes. Polymerase chain reaction yield was better in pleural and bronchoalveolar lavage fluids. A parasitological diagnosis from pleural and pericardial fluid was possible in AIDS patients. Overall, the risk of bias was low. Conclusions: Visceral leishmaniasis patients frequently had abnormal findings on high-resolution computed tomography. Chest ultrasound is a useful alternative in resource-limited settings to aid in diagnosis and subsequent treatment follow-up, especially when routine tests yield negative results despite clinical suspicion.

Patterns of childhood tuberculosis diagnosis in Ethiopia: A multicenter cross-sectional study.

Background: Children share 12% of the global 10 million people infected with tuberculosis (TB) each year. Closing case detection gap in children remains difficult, with 56% of all children and 65% under-five with TB missed each year. We aimed to assess the patterns of childhood TB diagnosis and underlying determinants in Ethiopia when different TB diagnostic platforms are applied. Methods: A multi-site, cross-sectional study was carried out in Ethiopia as part of the larger EXIT-TB study - evidence-based multiple focused integrated intensified TB screening package. Outpatient children aged ≤ 15 with cough of any duration seeking care at four healthcare facilities in Ethiopia were enrolled consecutively. Participants underwent sputum Xpert MTB/RIF and/or smear microscopy and posteroanterior chest X-ray (CXR), and their clinical and sociodemographic data were captured using a structured questionnaire. Data were analyzed using Stata version 23. Multiple regression model was computed to determine the factors that influence TB case detection, with a 95% confidence interval (CI) and p < 0.05 taken as statistically significant. Results: A total of 438 children were enrolled. Of these, 399 had CXR examination of which 55 (13.8%) were suggestive of TB, 270 had Xpert MTB/RIF testing of which 32 (11.9%) were positive, and AFB smear microscopy was done for 51 children of which 2 (3.9%) were positive. Febrile children were more likely to be diagnosed with pulmonary TB than those without fever [aPR = 1.3, 95% CI (1.1-1.4)], and those with a TB contact history were more likely to be diagnosed with pulmonary TB than those with no such contacts [aPR = 1.2, 95% CI (1.1-1.3)]. Children from rural residences were more likely to be diagnosed with TB than those from urban residences [aPR = 1.3, 95% CI (1.1-1.5)]. Conclusion: The findings showed that clinical diagnosis remains an important method of TB diagnosis in children and the preferred choice to avert underdiagnosis. A more sensitive TB diagnostic method for children was symptom screening, followed by CXR and Xpert MTB/RIF assay or smear microscopy. Hence, an algorithm that combines clinical, CXR, and microbiological confirmatory tests can improve the rate of pulmonary TB diagnosis in children till more accurate and cost-effective diagnostic tools are accessible. Fever, weight loss, and TB contact history are highly associated with TB positivity rates in children.