Tibial Adamantinoma: A Case Report from a Resource-Limited-Setting.

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.

Intraocular tuberculosis masquerading as ocular tumor: A case report.

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.

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.

Rare presentation of a rare disease: Bilateral congenital lobar overinflation.

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.

Establishing pediatric radiology in a low-income country: the Ethiopian partnership experience.

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.

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.

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.

Generalized arterial calcification of infancy in a neonate with acute kidney injury: A rare case report.

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.

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 .

Isolated renal hydatid cyst in a ten-year-old female child: A rare case report.

Hydatid cyst is a zoonotic disease caused by CestodaEchinococcus, especially Echinococcus granulosus. Isolated renal hydatidosis is an extremely rare clinical condition associated with nonspecific symptoms and physical findings. Diagnosis can be difficult and depends on imaging findings and histologic examination of excised tissue specimens. Here, we report a case of an isolated right renal hydatid cyst in a 10-year-old girl who presented with progressively worsening right flank pain. Diagnosis was established using multimodal imaging after which she underwent a successful cystectomy, had a smooth post-operative course, and was discharged with improvement and a continuation of albendazole.

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.

Dorsolumbar parasitic twin associated with lipomyelomeningocele: a case report from a tertiary teaching hospital, Ethiopia, East Africa.

A parasitic or heteropagus twin is a grossly defective fetus (or fetus part) attached externally, with or without internal connections and is dependent on the cardiovascular system of the other twin (autosite) for survival. The estimated incidence is approximately 1 per 1 million live births. To date according to the authors' knowledge; there are a few case reports published in the literature. Here we present a case of dorsolumbar parasitic twin with associated lipomyelomeningocele.

KARTAGENER'S SYNDROME: A CASE REPORT.

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.