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In 2008, a partnership between the academic pediatric radiology department at the Children's Hospital of Philadelphia in the USA and the radiology department at Tikur Anbessa Specialized Hospital in Ethiopia, was established. The partnership aims to support pediatric radiology education during radiology residency and to establish pediatric radiology subspeciality training in Ethiopia. In this paper, we review the needs that elicit this type of partnership, its structure, achievements, challenges and future state as a successful roadmap to the establishment of a high-impact subspeciality program in a bilateral partnership.
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Internship and Residency , Radiology , Humans , Child , Hospitals , EthiopiaABSTRACT
Radiological imaging is a crucial diagnostic tool for the pediatric population. However, it is associated with several unique challenges in this age group compared to adults. These challenges mainly come from the fact that children are not small-sized adults and differ in development, anatomy, physiology, and pathology compared to adults. This paper reviews relevant articles published between January 2015 and October 2023 to analyze challenges associated with imaging technologies currently used in pediatric radiology, emerging technologies, and their role in resolving the challenges and future prospects of pediatric radiology. In recent decades, imaging technologies have advanced rapidly, developing advanced ultrasound, computed tomography, magnetic resonance, nuclear imaging, teleradiology, artificial intelligence, machine learning, three-dimensional printing, radiomics, and radiogenomics, among many others. By prioritizing the unique needs of pediatric patients while developing such technologies, we can significantly alleviate the challenges faced in pediatric radiology.
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Forecasting , Pediatrics , Humans , Child , Pediatrics/methods , Diagnostic Imaging/methods , Diagnostic Imaging/trends , Radiology/trends , Radiology/methods , Artificial IntelligenceABSTRACT
BACKGROUND: Encouraged by the previous success in malaria control and prevention strategies, the Ethiopian ministry of health launched malaria elimination with a stepwise approach by primarily targeting the low-transmission Districts and their adjacent areas/zones in order to shrink the country's malaria map progressively. Hence, this community survey was conducted to establish baseline malaria information at the preliminary phase of elimination at targeted settings. METHODS: A community-based cross-sectional survey was conducted at 20 malaria-elimination targeted Districts selected from five Regional states and one city administration in Ethiopia. The GPS-enabled smartphones programmed with Open Data Kit were used to enumerate 9326 study households and collect data from 29,993 residents. CareStart™ Malaria PAN (pLDH) Rapid Diagnostic Tests (RDTs) were used for blood testing at the field level. Armpit digital thermometers were used to measure axillary temperature. RESULT: Overall malaria prevalence by RDTs was 1.17% (339/28973). The prevalence at District levels ranged from 0.0 to 4.7%. The proportion of symptomatic cases (axillary temperature > 37.5oc) in the survey was 9.2% (2760/29993). Among the 2510 symptomatic individuals tested with RDTs, only 3.35% (84/2510) were malaria positive. The 75.2% (255/339) of all malaria positives were asymptomatic. Of the total asymptomatic malaria cases, 10.2% (26/255) were under-five children and 89.8% (229/255) were above 5 years of age. CONCLUSION: The study shows a decrease in malaria prevalence compared to the reports of previous malaria indicator surveys in the country. The finding can be used as a baseline for measuring the achievement of ongoing malaria elimination efforts. Particularly, the high prevalence of asymptomatic individuals (0.88%) in these transmission settings indicates there may be sustaining hidden transmission. Therefore, active case detection with more sensitive diagnostic techniques is suggested to know more real magnitude of residual malaria in the elimination-targeted areas.
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Malaria, Falciparum , Malaria , Child , Cross-Sectional Studies , Diagnostic Tests, Routine , Ethiopia/epidemiology , Humans , Malaria/diagnosis , Malaria/epidemiology , Malaria/prevention & control , PrevalenceABSTRACT
BACKGROUND: Health management information system (HMIS) is a system whereby health data are recorded, stored, retrieved and processed to improve decision-making. HMIS data quality should be monitored routinely as production of high quality statistics depends on assessment of data quality and actions taken to improve it. Thus, this study assessed accuracy of the routine HMIS data. METHODS: Facility based cross-sectional study was conducted in Southern Nations Nationalities and People's region in 2017. Document review was done in 163 facilities of different levels. Statistical Package for the Social Sciences (SPSS) for windows version 20 was used to perform data analysis. Data accuracy was presented in terms of mean and standard deviation of data verification factor. RESULTS: Though inaccuracy was noted for all data elements, 96.9 and 84.7% of facilities reported institutional maternal death and skilled birth attendance within acceptable range respectively while confirmed malaria (45.4%), antenatal care fourth visit (46.6%), postnatal care (55.2%), fully immunized (55.8%), severe acute malnutrition (54.6%) and total malaria (50.3%) were reported accurately only by about half of facilities. Antenatal care fourth visit was over reported by 24% while total malaria was under reported by 28%. Reasons for variations included technical, behavioral and organizational factors. CONCLUSIONS: Majority of facilities over reported services while under reporting diseases. Data quality should be monitored routinely against data quality parameters quantitatively and/or qualitatively to catch-up country's information revolution agenda.
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Data Accuracy , Decision Making, Computer-Assisted , Health Information Systems , Maternal Health Services/organization & administration , Cross-Sectional Studies , Delivery of Health Care , Ethiopia , Evidence-Based Practice , Female , Health Facilities , Health Information Management , Humans , Pregnancy , Vaccination/statistics & numerical dataABSTRACT
Kartagener's syndrome is a rare autosomal recessive genetic disorder that causes a defect in the action of the cilia lining the respiratory tract. Patients usually present with chronic recurrent rhinosinusitis, otitis media, pneumonia, and bronchiectasis caused by Pseudomonas infection. In this case report, we present a 12-year old female with Kartagener's syndrome from Tikur Anbessa Teaching Hospital which to our knowledge is the first of it's kind to be reported in Ethiopia. The clinical and imaging findings are discussed.
Subject(s)
Kartagener Syndrome , Child , Ethiopia , Female , Fingers/pathology , Humans , Liver/diagnostic imaging , Liver/pathology , Paranasal Sinuses/diagnostic imaging , Paranasal Sinuses/pathology , Radiography, ThoracicABSTRACT
Background: Accurate radiographic assessment is pivotal in evaluating trauma patients with suspected pelvic ring disruptions. The conventional approach of using anteroposterior, 45° inlet, and 45° outlet radiographs for the evaluation of pelvic injury may not consistently align with varying lumbopelvic anatomy. This study aimed to determine the ideal pelvic inlet and outlet radiographic angles when there is limited access to advanced imaging (e.g., computed tomography [CT]) for assessing clinically relevant pelvic osseous landmarks and to investigate variations based on age, sex, and sacral dysmorphism. Methods: This cross-sectional study investigated patients who were ≥18 years of age who had no traumatic injuries or pelvic ring pathology; we reviewed abdominopelvic CT scans that were obtained between January 1, 2023, and June 30, 2023. Midsagittal reconstruction and 3D rendering of 148 CT scans facilitated the measurement of pelvic inlet and outlet angles. Standard techniques that were based on previous studies were used to determine the ideal angles. Statistical analyses investigated mean pelvic inlet and outlet angles as well as correlations with age, sex, and sacral dysmorphism. Results: The mean pelvic inlet angle was 23.8° ± 8.4° (95% confidence interval [CI]: 22.4° to 25.2°), and the mean outlet angle was 40.1° ± 5.9° (95% CI: 39.2° to 41.1°). Male patients exhibited greater inlet angles (27° versus 20°), whereas female patients had greater outlet angles (41° versus 39°). Pelves with dysmorphism showed a 3.6° increase in outlet angles when compared with those with normal sacral anatomy. An inverse relationship between age and inlet angle was observed. Conclusions: This study highlights that the recommended 45° angle for pelvic inlet and outlet views may not optimally align with the anatomy of the Ethiopian population. The findings suggest that the ideal inlet and outlet angles for this population are 25° and 40°, respectively. Understanding these variations is crucial for optimizing pelvic radiographic views in trauma evaluation, potentially leading to more accurate assessments and improved patient care in this demographic. Level of Evidence: Diagnostic Level IV. See Instructions for Authors for a complete description of levels of evidence.
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Congenital lobar overinflation is a rare but well-recognized congenital cause of neonatal and infantile respiratory distress. At times, the condition can mimic other congenital or acquired diseases and have atypical distribution and imaging patterns. Lobectomy of the involved lobe(s) is curative. We present our experience with 3 surgically confirmed cases of congenital lobar overinflation. Referral papers, patient's charts, including operation notes, and radiographic records were reviewed. All of them were initially misdiagnosed or underdiagnosed based on the initial radiographic examination alone. All 3 were referred to our center with respiratory distress, and the first 2 were treated with antibiotics prior to the settlement of their diagnosis. Chest computed tomography was key in diagnosing all 3 cases. The first patient was a 10-day-old neonate diagnosed with bilateral congenital lobar overinflation. The second patient was a 2-month-old infant diagnosed with right middle lobe disease. In these 2 cases, the initial assessment of the vascularity was atypically excessive in the affected lobe(s). Eventually, correlation with typical concurrent imaging features and the clinical condition of the patients led to the correct diagnosis. The third case was a 4-month-old infant with left upper lobe congenital lobar overinflation. All cases underwent successful surgical treatment. Congenital lobar overinflation is a rare anomaly, and multiple-lobe involvement is even rarer. Vascularity within the affected lobes is a subjective assessment that can be overestimated, leading to confusion, and a feature that needs correlation with other common imaging features and the clinical course of patients.
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Introduction: Adamantinoma is an infrequent, low-grade malignant bone tumor, predominantly affecting the tibia and often presents diagnostic challenges due to its nonspecific radiographic characteristics. Case Presentation: A 55-year-old military personnel, with no history of trauma, who presented with a one-year history of right leg swelling and pain. Radiological examination showed right anterior tibial, mid diaphyseal lytic expansile lesion with internal trabeculations and excisional biopsy led to the diagnosis of adamantinoma. Below knee amputation was done as a definitive management. Conclusion: Despite diagnostic challenges adamantinoma presents, the patient was referred to oncology and underwent amputation, underscoring the importance of considering adamantinoma in differential diagnosis for persistent bone lesions.
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Ascaris-induced intestinal obstruction is a rare complication primarily seen in children in areas with a high prevalence of worm infestations. It can occur through 2 mechanisms: immune-mediated reactions releasing neurotoxins that cause contractions and inflammation in the small intestine (aperistalsis), or mechanical obstruction by adult worms, commonly at the ileocecal valve. Partial obstructions are managed conservatively, while complete obstructions often require surgical intervention. In a recent case, a 19-year-old male presented with persistent abdominal pain, vomiting, and inability to pass stools, and gas. Imaging revealed partial obstruction, and conservative management with fluids, a nasogastric tube, and antibiotics led to the spontaneous passage of worms, relieving symptoms. The patient was discharged with anthelmintics and advised on follow-up and sanitary measures. This case is notable for the uncommon occurrence of Ascaris-induced intestinal obstruction in adults and the successful conservative management resulting in early worm expulsion.
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Preiser's disease or idiopathic avascular necrosis of the scaphoid is a rare condition where ischemia and necrosis of the scaphoid bone occurs without previous fracture. It is thought to be caused by repetitive micro trauma or side effects of drugs (e.g., steroids or chemotherapy) in conjunction with existing defective vascular supply to the proximal pole of the scaphoid. Wrist radiography or CT coupled with MRI is the imaging modality of choice in the diagnosis of this rare entity. Here, we report a case of Preiser's disease of the left wrist in a 17-year-old female patient who presented with left wrist pain of 2 years duration in the absence of trauma history or causative drug use. The diagnosis was made by wrist X-ray and MRI. She was managed by Physiotherapy and wrist immobilization using wrist and forearm support as well as NSAIDS (Meloxicam).
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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.
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Tuberculosis is one of the most common pediatric problems, especially in the developing world. In spite of that, intraocular tuberculosis is a rare disease that can easily be confused with other noninfectious processes, even in regions where tuberculosis is rampant. Diagnosis is difficult, yet it is very important to provide effective antituberculosis treatment and avoid potentially sight-losing interventions. We present a case of a 2-year-old child with a positive contact history of tuberculosis who presented with progressively worsening seizures and constitutional symptoms for 6 months. Brain computed tomography revealed right frontotemporal region conglomerated ring-enhancing lesions with central necrosis consistent with tuberculosis. On the same scan, a calcified right retinal lesion with a contrast-enhancing soft tissue component was identified. A chest radiograph and abdominal sonography showed evidence of disseminated tuberculosis. Subsequently, antituberculosis treatment was initiated, and the right retinal lesion improved, thus leading to the imaging diagnosis of right intraocular tuberculosis. Early and accurate diagnosis of retinal tuberculosis is of paramount importance in avoiding potentially catastrophic interventions. Neuroimaging is a useful, noninvasive method to consider this difficult diagnosis and also for follow-up.
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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.
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BACKGROUND: Globally, fall-related injuries are a substantial problem, and 80% of fatal falls occur in low-income and middle-income countries. We aimed to measure time from injury to hip-fracture surgery in people aged 50 years or older living in low-income and middle-income regions, as well as to measure the proportion of patients with surgical stabilisation of their hip fracture within 72 h of admission to hospital and to identify risk factors associated with surgical delay. METHODS: For this secondary analysis, we analysed data collected from Africa, Latin America, China, India, and Asia (excluding China and India) for the International Orthopaedic Multicentre Study in Fracture Care (INORMUS) between March 29, 2014, and June 15, 2022. Patients from INORMUS were included in this analysis if they were aged 50 years or older and had an isolated, primary hip fracture sustained from a ground-level fall. Staff at participating hospitals identified patients with musculoskeletal injury and referred them for assessment of eligibility. We report time from injury to surgery as three distinct time periods: time from injury to hospital admission, time from admission to surgery, and a total time from injury to surgery. Date and time of injury were self-reported by patients at the time of study recruitment. If time to hospital admission after injury exceeded 24 h, patients reported the primary reason for delayed admission. Reasons for surgery, no surgery, and surgical delay were reported by the treating team. For patients undergoing surgery, multivariable regression analyses were used to identify risk factors for surgical delay. FINDINGS: 4486 adults aged 50 years or older with an isolated, primary hip fracture were enrolled in INORMUS from 55 hospitals in 24 countries. Countries were grouped into five regions: Africa (418 [9·3%] of 4486), Latin America (558 [12·4%]), China (1680 [37·4%]), India (1059 [23·6%]) and Asia (excluding China and India; 771 [17·2%]). Of 4486 patients, 3805 (84·8%) received surgery. The rate of surgery was similar in all regions except in Africa, where only 193 (46·3%) of 418 patients had surgery. Overall, 2791 (62·2%) of 4486 patients were admitted to hospital within 24 h of injury. However, 1019 (22·7%) of 4486 patients had delayed hospital admission of 72 h or more from injury. The two most common reasons for delayed admission of more than 24 h were transfer from another hospital (522 [36·2%] of 1441) and delayed care-seeking because patients thought the injury would heal on its own (480 [33·3%]). Once admitted to hospital, 1451 (38·1%) of 3805 patients who received surgery did so within 72 h (median 4·0 days [IQR 1·7-6·0]). Regional variation was seen in the proportion of patients receiving surgery within 72 h of hospital admission (92 [17·9%] of 514 in Latin America, 53 [27·5%] of 193 in Africa, 454 [30·9%] of 1471 in China, 318 [44·4%] of 716 in Asia [excluding China and India], and 534 [58·6%] of 911 in India). Of all 3805 patients who received operative treatment, 2353 (61·8%) waited 72 h or more from hospital admission. From time of injury, the proportion of patients who were surgically stabilised within 72 h was 889 (23·4%) of 3805 (50 [9·7%] of 517 in Latin America, 31 [16·1%] of 193 in Africa, 277 [18·8%] of 1471 in China, 189 [26·4%] of 716 in Asia [excluding China and India], and 342 [37·5%] of 911 in India). INTERPRETATION: Access to surgery within 72 h of hospital admission was poor, with factors that affected time to surgery varying by region. Data are necessary to understand existing pathways of hip-fracture care to inform the local development of quality-improvement initiatives. FUNDING: The National Health and Medical Research Council of Australia, the Canadian Institutes of Health Research, McMaster Surgical Associates, Hamilton Health Sciences, and the US National Institutes of Health.
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Hip Fractures , Humans , Hip Fractures/surgery , Female , Male , Aged , Middle Aged , Accidental Falls/statistics & numerical data , Time-to-Treatment/statistics & numerical data , Aged, 80 and over , Risk Factors , China/epidemiology , Latin America/epidemiology , Developing Countries , Asia/epidemiology , Poverty , Time Factors , Africa/epidemiologyABSTRACT
We examined the frequency of osteoporotic fractures among patients presenting to Tikur Anbessa Specialized Hospital. Osteoporotic fractures accounted for 10.4% of all fractures and 31.8% of those 40 years and older. In addition, hip fractures accounted for 60%. Therefore, devising strategies for preventing, treating, and rehabilitating osteoporotic fractures is critical. PURPOSE: Examine the frequency of osteoporotic fractures among patients presenting to Tikur Anbessa Specialized Hospital. METHODS: This is an observational study of prospectively collected data between January 2018 and December 2021. Patients were categorized as having osteoporotic fracture if they were 40 years or older, sustained a low-energy injury, and had characteristic fracture patterns to the hip, proximal humerus, distal radius, tibia (in females only), clavicle, and scapula. A descriptive analysis was carried out to assess patient demographics. Risk factors were then evaluated using a binary logistic regression model. RESULTS: A total of 4712 orthopedic injury patients presented to the emergency department with 4422 fracture cases. Of these, 461 fulfilled the diagnostic criteria for osteoporotic fractures. The overall rate of osteoporotic fractures was 10.4% of all patients with fractures and 31.8% of those 40 years or older. Overall, 63.3% were female. One in four females and 5% of males with musculoskeletal trauma had an osteoporotic fracture. Osteoporotic hip fractures made up 59.9% of osteoporotic fractures and 5.9% of all fractures, followed by distal radius (23%), tibia in females (8.2%), and proximal humerus (7.4%). Pelvis (2.6%), clavicle (0.9%), and scapula (0.2%) fractures were found to be rare. Among all patients with fractures following low-energy injuries, when patients were aged 50 years and older, there was a higher risk that the trauma resulted in an osteoporotic fracture. This figure was highest among those aged 80 years and older (odds ratio (OR), 11.88; 95% CI, 7.01-20.11). CONCLUSIONS: Further studies need to be done to show the prevalence of osteoporosis and osteoporotic fractures in Ethiopia and examine risk factors. Devising strategies for preventing, treating, and rehabilitating osteoporotic fractures is critical.
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Hip Fractures , Osteoporosis , Osteoporotic Fractures , Male , Humans , Female , Middle Aged , Aged , Osteoporotic Fractures/epidemiology , Ethiopia , Osteoporosis/epidemiology , Hip Fractures/epidemiology , Hip Fractures/therapy , HospitalsABSTRACT
Soil erosion is a vector of disturbances to agricultural productivity and economic development in the western highlands of Ethiopia. Yet, tough vegetation cover loss swapped to other land uses could have amplified the soil loss rate at which land cover change preceded, but little is known about their effects on soil loss in the Limu-Seqa watershed. This study was designed to evaluate the historical trends of the effects of land use-land cover change on soil erosion dynamics as a threshold for potential monitoring of soil loss. Satellite image data of 1987, 2002, 2021, and DEM-20 m resolution were used. The RUSLE model was applied with primary parameters to generate soil loss. Findings show that average annual soil loss increased from 4.5 in 1987 to 13.5 t ha-1 yr-1 in 2002 and surpassed to 45.35 t ha-1 yr-1 in 2021 as a result of LULC changes, particularly the transition of forest and overgrazed land to cropland (43.83%) and dense-forest to poor-open-up forest (6.92%) between 1987 and 2021. Soil loss during the recent study period was substantially affected by a substantial LULC change, from forest to cropland. The severe and very severe erosion risk categories jointly cover more than half of the entire catchment, which contributes to two-thirds of the total mean annual soil loss in the watershed, which is found to be over and above soil loss tolerance (SLT) in Ethiopia and tropical regions. Therefore, given the robust economic and political status of priority conservation measures, red hues areas are significant.
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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.
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Generalized arterial calcification of infancy (GACI) is a rare condition characterized by diffuse arterial calcification within the internal elastic lamina associated with intimal proliferation leading to stenosis of great and medium-sized vessels, which causes end-organ damage and loss of life during infancy. The clinical presentation of acute renal failure with normal cardiac function is rare. A 7-day-old female neonate was admitted with a clinical impression of late-onset neonatal sepsis, meningitis, and acute kidney injury after developing a high-grade fever, abnormal body movements, and vomiting of the ingested matter associated with decreased urinary output. On laboratory tests, she had abnormal urea and creatinine levels, multiple electrolyte abnormalities, and a negative septic workup. Ultrasonography revealed diffuse arterial calcification that also involved the renal arteries and renal parenchyma bilaterally. She was clinically diagnosed with GACI and initiated on supportive care including renal replacement therapy. However, she died at the age of 42 days. This case highlights that GACI can present as unexplained acute kidney injury associated with generalized arterial calcification. Ultrasound can be optimized to aid in diagnosis in resource-limited settings.
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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 .
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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.