Bladder duplication in infant girls: role of imaging in two rare cases with variants of a complete sagittal septum.

Bladder duplication is an extremely rare congenital anomaly of the urinary system that is more frequent in boys; the literature is limited to case reports and case series. We describe two cases of bladder duplication in two infant girls with an uncommon variant of complete sagittal septum not included in the Abrahamson classification. The diagnosis was made using magnetic resonance urography, combining excellent anatomical information and static and dynamic evaluation of the urinary tract. The diagnostic information provided by MR-urography was confirmed on surgical exploration. These cases provide an opportunity for paediatric radiologists and urologists to learn more about bladder duplication and improve their diagnosis of this rare condition.

Up-to-date imaging review of paediatric soft tissue vascular masses, focusing on sonography.

The purpose of this article is to provide an up-to-date overview on imaging of paediatric vascular soft tissue masses, including both neoplastic and non-neoplastic lesions. We describe the-often challenging-imaging diagnosis-mainly performed by ultrasound (and secondarily by MRI) and differential diagnosis of vascular soft tissue lesions in children. We underline how important it is to determine whether a vascular anomaly has a regional vascular origin, or if there are other entities, ranging from benign to malignant lesions, which have flow-signal or blood degradation products. Even though clinical examination and patient's history are the first and indispensable steps in the initial diagnosis, the role of imaging is crucial, not only to determine whether a mass represents a true tumour/pseudo-tumour, but also to achieve a more correct diagnosis and determine the extension of the tumour/pseudo-tumour and its relation with the nearby anatomic structures.

Diagnostic Imaging in pediatric thoracic trauma.

Thoracic trauma accounts for approximately 14% of blunt force traumatic deaths, second only to head injuries. Chest trauma can be blunt (90% of cases) or penetrating. In young patients, between 60 and 80% of chest injuries result from blunt trauma, with over half as a consequence of impact with motor vehicles, whereas in adolescents and adults, penetrating trauma has a statistically more prominent role. Pulmonary contusions and rib fractures are the most frequent injuries occurring. Chest X-ray is the first imaging modality of choice to identify patients presenting with life-threatening conditions (i.e., tension pneumothorax, huge hemothorax, and mediastinal hematoma) and those who require a CT examination. Multi-Slice Computed Tomography is the gold standard to evaluate chest injuries. In fact, the high spatial resolution, along with multiplanar reformation and three-dimensional (3D) reconstructions, makes MDCT the ideal imaging method to recognize several chest injuries such as rib fractures, pneumothorax, hemothorax, lung contusions and lacerations, diaphragmatic rupture, and aortic injuries. Nevertheless, when imaging a young patient, one should always keep into account the ALARA concept, to balance an appropriate and low-dose technique with imaging quality and to reduce the amount of ionizing radiation exposure. According to this concept, in the recent years, the current trends in pediatric imaging support the rising use of alternative imaging modalities, such as US and MRI, to decrease radiation exposure and to answer specific clinical questions and during the observation period also. As an example, ultrasound is the first technique of choice for the diagnosis and treatment of pleural and pericardial effusion; its emerging indications include the evaluation of pneumothoraces, costocondral and rib fractures, and even pulmonary contusions.

M-mode ultrasound for assessment of the "tethered fat sign" in children: an easily performed way to certify a dynamic process as a still picture.

Background: Omental infarction is a rare pediatric disease. Ultrasound is a useful modality for a non-invasive pre-operative differential diagnosis between inflammatory conditions (as appendicitis) and omental infarction, especially by detecting immobility of the omentum adhered to the abdominal wall ("tetherd fat sign"). However, this is a dynamic sign that cannot be documented in a static image with B-mode technique. The goal of this work is to incorporate the versatile function of motion mode (M-mode) into omental infarction diagnosis to describe how the M-mode is useful in the evaluation of fat motion in children suspected of having omental infarction. In 2019 we suggested a new Ultrasound sign named "tethered fat sign" for an accurate non-invasive diagnosis of omental infarction in children. This finding was observed in 6 of the 234 seen children of our previous study with 4 laparoscopic confirmed diagnosis. Methods: From January 2019 to July 2021, we evaluated 195 children (91 boys and 104 girls, from 3 to 15 years) admitted to our Santobono-Pausilipon Children Hospital with acute right-sided abdominal pain. Abdominal ultrasound was performed to all the patients and the investigation of "tethered fat sign" was always included. Results: In 7 patients ultrasound showed the presence of a hyperechoic oval mass localized in the right upper abdominal quadrant and in 2 of these M-mode documented a normal subhepatic fat moving during respiratory movements in relation with the abdominal wall. The remaining 5 patients had an omental infarction showed as a subhepatic motionless mass tethered to the abdominal wall on M-mode. In these patients, a sonographic follow-up was performed every 15 d for 2 months showing a progressive reduction in size of the right-sided hyperechoic mass. Conclusions: In the evaluation of all children who showed the presence of the "tethered fat sign" the use of M-mode provide a certified image in diagnostic ultrasound.

Volvulus of a wandering spleen in a pediatric patient.

Wandering spleen is a rare condition in children that is often caused by the loss or weakening of the splenic ligaments. Its clinical presentation is variable; 64% of children with wandering spleen have splenic torsion as a complication. A 13-year-old boy who had been showing abdominal pain in the hypogastric region accompanied by vomit and an enormous tumefaction in the suprapubic region came to our observation. Considering the ovoid morphology at ultrasound exam, the echostructure and the marked reduction of parenchymal vascularization, suspicion for torsion of an ectopic spleen arose. Ultrasound evaluation has a primary role in the diagnosis of a suspected wandering spleen and, to avoid potentially life-threatening complications, immediate surgery is often times required.

Ultrasound findings in early congenital syphilis: Two case reports and literature review.

Syphilis is caused by treponema pallidum. If untreated, or inadequately treated, during pregnancy, it can result in congenital syphilis (CS), which is classified as early and late. Early CS displays before 2 years of age. We herein describe 2 cases of early CS, whose clinical onset included liver failure, edema, organomegaly, and respiratory distress. We focus on liver, intestinal, and brain ultrasound (US) and other peculiar radiological findings. To date, there are no scientific data on intestinal and brain US findings in patients with early CS whereas data on abdominal US are scarce. Increasing knowledge about US findings in early CS could be useful to improve the diagnostic and therapeutic approach to these patients.

Congenital tibial pseudarthrosis: A challenge in pediatric radiology.

Congenital pseudarthrosis of the tibia (CPT) is a rare disorder affecting the skeletal system in pediatric population with an estimated incidence of 1:140,000 to 1:250,000 newborns. It is characterized by deformity of the tibia, including anterolateral bowing of the bone diaphysis and/or narrowing of the medullary canal, leading to instability or fracture. CPT can be either idiopathic or associated with underlying conditions such as type 1 neurofibromatosis (NF1), fibrous dysplasia, or Campanacci's osteofibrous dysplasia. Diagnosis is based on clinical and imaging findings, using conventional radiography and magnetic resonance imaging (MRI). The disorder is characterized by recurrent pathological fractures of the tibia or fibula during childhood, often beginning by the age of 2 years. Treatment options include surgical and nonsurgical management.

Hepatic hemangiomas in childhood: the spectrum of radiologic findings. A pictorial essay.

Hepatic hemangiomas (HHs) are the most common benign liver tumors in infancy, but despite their frequent occurrence, their radiologic appearance may be particularly challenging to assess, owing to unique anatomical and development features that distinguish their subtypes in the pediatric population. There are 3 main patterns of the lesions recognized in HHs: focal, multifocal and diffuse. Medical care strategies range from simple observation to surgery, and a timely diagnosis is crucial to establish the most appropriated clinical management and therapy. Ultrasound (US) is typically the first level examination of pediatric vascular anomalies, able to meet a satisfactory diagnosis and has proved very helpful to assess for involution through serial imaging. CT and MRI are second-level methods seldom performed, offering an anatomical panoramic view and clarification when US is non-conclusive. Histologic confirmation is rarely required. HHs show a variable sonographic appearance, with hypoechoic (mostly) or hyperechoic solitary\multifocal mass and wide features. Hepatic vessels assessment reveals an abnormal flow, enlarged vasa, decreased resistive index (RI) values, scarce blood supply distally to lesions and hyper vascular regions inside them. In this pictorial essay, we review HHs subtypes in further details, illustrate US, CT and MRI findings and clarify a recurrent dispute over the existing terminology.

Multimodal imaging approach in hyponatremic hypertensive syndrome. A rare case of pediatric unilateral hypoplasia of the main renal artery combined itself with stenosis and review of literature.

Renal artery stenosis (RAS) accounts for approximately 5%-10% of secondary renovascular hypertension in the pediatric population. It can occur as an isolated entity, or as a hypoplasia combined itself with stenosis. Hypoplasia, or long-segment developmental narrowing, is a rare cause of renovascular hypertension. Hyponatremic hypertensive syndrome (HHS) is a malignant complication of unilateral RAS and/or renal artery hypoplasia. Hyponatremia, hypokalemic hypochloremic metabolic alkalosis, nephrotic range proteinuria, polyuria, polydipsia, and weight loss are the most common findings. In particular, hypertension remains refractory despite aggressive antihypertensive therapy. Laboratory findings of elevated plasma levels of renin in most case suggest that the stimulation of renin release from the ischemic kidney plays an important pathophysiologic role. HHS is a diagnostic and therapeutic challenge in children. We report a case of a unilateral right renal artery hypoplasia, complicated by a segmental narrowing, in a 17-month-old male, clinically symptomatic for hypertension. We emphasize the role of ultrasound, computed tomography, and digital subtraction angiography that should be planned as reliable and non-invasive multimodal imaging approach.

Para-oesophageal hernia in 4-month-old girl: possible role of ultrasound-case report and literature review.

Hiatal hernia is the passage of digestive tract portions into the posterior mediastinum through a defect in the esophageal diaphragmatic hiatus. By guidelines, the diagnosis uses first-level radiographic investigations such as chest X-ray and contrastographic methods. As of today, use of ultrasonography in the suspicion/diagnosis of hiatal hernia is not standardized although it shows advantages such as not using ionizing radiation, which is essential in the small pediatric patient. We report the case of a little 4-month-old patient who came to our attention for dysphagia, vomiting and borborygmus to whom Type II hiatal hernia was suspected by ultrasound investigation, later confirmed by guidelines approved methods.

Osteofibrous dysplasia: A rare case in 3-day-old female.

Osteofibrous dysplasia (OFD) is a nonneoplastic tumor-like lesion, made up of fibrous matrix with immature bone tissue surrounded by osteoblasts, occurring usually in the cortex of tibial diaphysis. OFD is usually seen in the first decade of life and, according to literature, it is rarely seen in the newborn period. Diagnosis of congenital OFD in the newborn is challenging because it is uncommon in this age group and can be confused with other bone benign or malignant lesions. Imaging plays an important role in diagnosis, although histological confirmation is often required. Our report presents a rare case of pathologically confirmed congenital OFD in 3-day-old female which presented with a swelling of her right leg. We will focus on imaging findings of OFD and main differential diagnosis of this lesion in neonatal age.

Pseudopapillary solid tumour of the pancreas in paediatric age: description of a case report and review of the literature.

Pseudopapillary solid tumour of the pancreas is a rare neoplasm that mainly affects young women in the second and third decade of life and less frequently children; originates from the exocrine component of the pancreas; and is characterized by slow growth, low potential for malignancy, and excellent prognosis following complete surgical resection. The tumour often presents as an asymptomatic abdominal mass that is accidentally detected during radiological investigations performed for other reasons. In this article, we report the clinical case of a 10-year-old girl who came to our observation for pain in the left hypochondrium, which had arisen for a week following a trauma; the imaging methods revealed a voluminous expansive pancreatic formation in the abdomen; on histological examination, the mass was a solid pseudopapillary tumour. Furthermore, we present a review of the literature aimed at highlighting the salient features of this neoplasm in paediatric age.

Early diagnosis of Meigs syndrome in children A case report and a review of the literature.

Meigs syndrome is a rare disease defined by the coexistence of benign ovarian neoplasm, ascites and hydrothorax, which mainly affects women over the age of 30. This clinical condition refers only to cases in which the ovarian neoformation is a fibroid, a thecoma, a granulosa cell tumor or a Brenner tumor with disappearance of symptoms and effusions after removal of the neoplasm. Meigs syndrome is most frequently characterized by the presence of an ovarian fibroid, which in childhood is very rare and not commonly associated with the disease. In this article we report the case of an 11- year-old girl who came to our observation for a high fever for five days accompanied by cough and abdominal pain; imaging methods revealed bilateral hydrothorax, ascites, and a voluminous expansive right ovarian formation. On histological examination, the mass showed a cellular fibroid and the diagnosis of Meigs syndrome was made. Furthermore, we present a review of the literature aimed at detecting the state of knowledge on this disease in pediatric age, giving particular emphasis to the condition for which, in the presence of pleural effusion and ascites, an ovarian neoformation is not necessarily malignant. KEY WORDS: CT, Meigs syndrome, Pediatric, Pelvic mass, Ultrasounds.

Duodenal hematoma in pediatric age: a rare case report.

In pediatric age, duodenal hematoma is rare and generally occurs following a closed abdominal trauma due to the crushing of the duodenum against the rigid plane of the spine; it rarely follows anticoagulant therapy, pancreatitis, bleeding disorders, vasculitis, tumors or upper digestive endoscopy. Duodenal hematoma is a rare cause of obstruction of the upper gastrointestinal tract and acute pancreatitis, and the diagnosis is sometimes difficult and late. On the other hand, the identification of the pathology in its initial stages allows the young patients to be subjected to a conservative treatment that resolves the issue most of the time, thus avoiding surgery. In this article we describe an unusual case of duodenal hematoma, following esophagus-gastro-duodenoscopy, in a 12-year-old boy with Di George syndrome.

Midsternal bump: an infrequent localization of dermoid cysts.

A 5-month-old boy was evaluated for an unusually large presternal bump present since birth. The ultrasound examination revealed a well-defined soft tissue mass with an oval shape. The lesion demonstrated a regular and well-demarcated outline, with an upper margin that was thinned and inserted into the upper skin plane; the content was anechoic with a small echogenic formation, mobile with changes in the patient's decubitus. The histologic diagnosis was dermoid cyst. Although dermoid cysts are commonly seen in the midline, the midsternal location, found in our patient, is rare. Dermoid cysts can have ultrasonographic features similar to those of other subcutaneous cystic masses. However, if an anechoic cyst with an internal well-circumscribed echogenic ball-like formation is seen within the presternal subcutaneous fat layer, as in our patient, dermoid cyst should be considered in the differential diagnosis of subcutaneous cystic masses.

Pneumatosis cystoides intestinalis, a rare case in a pediatric patient following allogeneic hematopoietic stem cell transplantation: CT findings and literature review.

Pneumatosis cystoides intestinalis (PCI) is a rare condition characterized by the presence of gas-filled cysts in the subserosa or submucosa of the bowel wall. It is associated with various disorders including chronic obstructive pulmonary diseases, autoimmune disorders, and organ transplantation. PCI has also been observed following Hematopoietic Stem Cell Transplantation (HSCT), associated with chemotherapy, acute Graft versus Host Disease (GvHD), immunosuppression, and infections. Computed tomography (CT) provides an easy diagnosis because it highlights the presence of air bubbles in the intestinal wall and possible pneumoperitoneum. We report the case of a patient with severe acquired medullary aplasia undergoing allogeneic HSCT with subsequent development of cutaneous GvHD and an incidental finding of PCI during a CT scan of the chest in absence of gastrointestinal symptoms. Our work aims at clarifying a possible complication in pediatric patients undergoing HSCT to guide young or non-pediatric radiologists in the identification of this rare condition, helping the clinician in the correct conservative management of these patients and reserving the surgical treatment only to specific complications.

An unusual onset of pediatric acute lymphoblastic leukemia.

Acute lymphoblastic leukemia is the most frequent cancer in children: it represents 80% of leukemias and about 24% of all neoplasms diagnosed between 0 and 14 years. Acute lymphoblastic leukemia mainly affects children between 2 and 5 years old and in this age group the incidence is about 80-90 cases per million per year. In acute lymphoblastic leukemia, cancer cells multiply rapidly and accumulate in the bone marrow and subsequently invade the blood. However, at the time of diagnosis, leukemia rarely occurs outside the bone marrow or blood vessels and the extramedullary involvement happens mostly in patients with refractory or relapsing disease. In this article, we report an unusual clinical presentation of acute B cell lymphoblastic leukemia with intestinal and ovarian localizations in a 5-year-old girl.

Mediastinal thymoma: A difficult diagnosis in the pediatric age.

Thymoma is a rare neoplasm of the anterior mediastinum, which originates from the epithelium of the thymic gland; it occurs mainly in middle-aged adults and is much less common in children. The tumor has slow growth and is asymptomatic in most pediatric cases, thus resulting in an accidental discovery; one-third of the young patient presents symptoms related to the compression of the tumor mass on the surrounding anatomic structures and/or related to paraneoplastic syndromes. Surgery is the treatment of choice and complete resection of the thymoma achieves excellent long-term results in terms of disease-free survival. In this article, we report the clinical case of a 21-month-old girl who came to our observation for persistent cough for over a month investigated with a chest X-ray, performed in another hospital. The X-ray showed an extensive opacification of the left hemithorax with contralateral dislocation of the mediastinum. The instrumental investigations carried out in our hospital (ultrasound, computed tomography, and magnetic resonance of the chest) showed a voluminous expansive mass of the left antero-superior mediastinum, which occupied the entire ipsilateral hemithorax and not dissociable from the thymus. At the histologic examination, the mass resulted to be a B1 thymoma with a low degree of malignancy according to the histologic classification of thymic tumors of the World Health Organization.

Type II pleuropulmonary blastoma in a 3-years-old female with dyspnea: a case report and review of literature.

Pleuropulmonary blastoma (PPB) is a rare but aggressive pediatric tumor originates from either lung or pleura. It was recently linked to the DICER I mutation as a part of predisposition syndrome for different type of tumor. It is characterized histologically by a primitive, variably mixed blastomatous and sarcomatous tissue. PPB is classified into four subtypes: cystic (type I and type Ir); cystic and solid (type II); solid (type III). PPB has no characteristic imaging findings. Integrated imaging can help to make a differential diagnosis and to recognize the subtypes in order to set up therapy. An early recognition and differentiation from congenital airway malformations and other benign cysts are very important. The treatment consists in a multimodal therapy including surgery and chemoterapy. We report a case of 3 years old female admitted at our hospital with fever, non productive cough and dyspnea, who was diagnosed with type II PPB.