Obi Chrystal,  Nadel Helen,  Seekins Jayne

Final Pr. ID: Poster #: EDU-015

To identify patterns of calcifications and location on abdominal radiography to help diagnose their disease processes. We retrospectively reviewed abdominal x-rays with abnormal calcifications and correlated the findings with additional imaging such as ultrasound, CT or MR. We grouped calcifications by quadrant or diffuse to correlate with the differential diagnosis and disease process. Assessing the location and characteristics of calcifications on abdominal radiography can be highly useful in aiding in the formation of a differential diagnosis and guide next imaging steps. Read More

Authors:  Obi Chrystal , Nadel Helen , Seekins Jayne

Keywords:  Abdominal, Calcification

Golden Eleza,  Simoneaux Stephen,  Dickson Paula

Final Pr. ID: Poster #: CR-049

To describe the radiographic appearance of subclinical calcified brown fat necrosis and to delineate the associated clinical and laboratory findings. While brown fat necrosis has been described in infants with underlying cardiac disease treated with prostaglandins, we emphasize hypotension from cardiac or respiratory arrest as a primary risk factor. Read More

Authors:  Golden Eleza , Simoneaux Stephen , Dickson Paula

Keywords:  fat necrosis, soft tissue calcification, congenital heart disease

Chopra Mark,  Barrett Hannah,  Hutchinson J.,  Kiho Liina,  Arthurs Owen

Final Pr. ID: Poster #: CR-012

Ductus Arteriosus calcification is a poorly comprehended pathology occasionally reported on chest radiographs, more commonly on CT. The purpose of this educational abstract is to present the concepts surrounding ductal arteriosus (DA) calcification and reviews the literature to date.
We retrospectively searched the main medical literature (PubMed, MEDLINE, CINAHL and Google scholar databases) using the following keywords: “ductus arteriosus”, “ligamentum arteriosum”, “calcification”, “ductal”, “patent ductus arteriosus”, “children”, “paediatric”. Articles regarding ductus arteriosus calcification were further evaluated for citations.

Sixteen articles were found which dated back over nearly a century. Of these eight concerned a paediatric population and very few had histological confirmation. The majority highlighted that ductal calcification is more prevalent than previously thought. Ductal calcification was initially reported on the chest roentgen-ray in 1931 by Weiss. The increasing prevalence has been compounded by the use of the greater spatial resolution offered by modern CT scanners. A study in 2012 showed up to a third of children have ductal calcification on CT, with peak incidence is towards the end of the first decade in girls. There is conflicting opinion regarding the pathophysiology of ductal calcification, which may be either ductal aneurysmal thrombus regression, or more likely ligamentum arteriosum intimal and medial wall calcification.

This literature review illustrates the general paucity of histological data and suggests that the autopsy evidence favours intimal and medial wall calcification. Read More

Authors:  Chopra Mark , Barrett Hannah , Hutchinson J. , Kiho Liina , Arthurs Owen

Keywords:  Ductus arteriosus calcification, ligamentum arteriosum

Chopra Mark,  Barrett Hannah,  Hutchinson J.,  Kiho Liina,  Arthurs Owen

Final Pr. ID: Poster #: CR-013

Ductus Arteriosus calcification is a relatively common, typically unreported feature on plain film radiography. The more recent literature quotes a prevalence on unenhanced CT of between 37-61% of children and a peak at the age of 6-10 years. Adult studies have shown that calcification of the ligamentum arteriosum can occur alone or be associated with atherosclerotic and/or granulomatous calcification. We report two cases in whom they were identified as incidental findings on forensic post mortem CT, for whom we obtained histological confirmation.

Case 1: An 8 month old child who died in non-suspicious circumstances had incidental ductal arteriosus calcification reported on post mortem CT, following unremarkable skeletal survey. Intimal mural calcification was reported on histology at autopsy.

Case 2: A 19 month old child who died of aspiration underwent full skeletal survey and PMCT as part of the pre-autopsy imaging assessment. The skeletal survey revealed incidental ductus arteriosus calcification, initially reported as PDA clip, with correlation at CT. Intimal and medial mural calcification was reported on histology at autopsy.

These cases showed that ductal calcification was intimal and medial in nature. This is consistent with the current literature regarding mural calcification rather than thrombus regression calcification. Read More

Authors:  Chopra Mark , Barrett Hannah , Hutchinson J. , Kiho Liina , Arthurs Owen

Keywords:  Ductus arteriosus calcification, Post mortem, Non-accidental injury

Fontalvo Herazo Lucia,  Putnins Rita,  Rotaru Carmen

Final Pr. ID: Poster #: CR-011

Idiopathic arterial calcification of infancy (IACI) is a rare condition, characterized by extensive depositions of hydroxyapatite in the internal elastic lamina of medium-sized and large arteries. It is usually diagnosed prenatally or in early infancy. Congenital intrahepatic portosystemic shunt is a persistent communication between the vitelline veins and sinus venosus, due to a focal absence of sinusoid formation. Infrequently they can present with signs of portal hypertension such as varices, ascites, and splenomegaly, but in most cases this feature usually indicates that the existing shunt is compensatory and not congenital.

We present a 29 week male neonate with a prenatal sonographic examination showing pericardial effusion, severe ascites and calcified cardiac outflow tract. After C section, the patient required resuscitation, intubation and abdominal drainage due to ascites in the NICU. Postnatal abdominal ultrasound confirmed abnormal calcification of the wall of the abdominal aorta, common iliac and origin of the superior mesenteric artery. Cranial ultrasound showed abnormal heterogeneous echotexture of the brain parenchyma with several linear parallel echogenicities, that may correspond to microcalcification of small caliber arteries. During the first few days of life the patient developed liver failure, severe cholestasis, coagulopathy and hypoalbuminemia. Follow up ultrasound done on day 12 showed hepatosplenomegaly, multiple hypoechoic focal liver lesions and an intrahepatic portosystemic shunt between the bifurcation of the main portal vein and the left hepatic vein. This finding was determined to be the primary cause of the ascites and progressive hepatosplenomegaly. By day 65 the portosystemic shunt had spontaneously resolved. Unfortunately the idiopathic arterial calcification had significantly progressed, despite bisphosphonate treatment. Multisystem organ failure had developed, the patient died on day 68.

DNA sequencing and high density target array analysis of ABCC6 and ENPP1 revealed no detectable mutation (anomaly most frequently related with IACI). Nevertheless, a c2320C>T transition in exon 23ENPP1 was identified. Finding been previously reported in a case of periarticular calcification.

This case illustrates two different and important pathologies that can be present in neonates. Each one has specific features and significant clinical relevance. This is a rare association as these two entities are usually seen separately. Read More

Authors:  Fontalvo Herazo Lucia , Putnins Rita , Rotaru Carmen

Keywords:  Idiopathic arterial calcification, Transient congenital porto systemic shunt, Peterme baby

Aslan Mine,  Kalyoncu Ucar Ayse,  Arioz Habibi Hatice,  Namdar Yesim,  Kurugoglu Sebuh,  Adaletli Ibrahim

Final Pr. ID: Poster #: CR-027

Wolman disease is a rare autosomal recessive inherited disease characterized by storage of cholesterol esters and triglycerides in lysosomes due to a deficiency of lysosomal acid lipase. Clinical signs such as persistent vomiting, diarrhea, hepatosplenomegaly, growth retardation and liver dysfunction occur in the first weeks of life. Most cases die in the first year of life. Here, we aim to present radiological findings of Wolman disease.
An asymptomatic girl two months of age was admitted to the pediatric genetic disease service with the history of Wolman disease in two brothers who had died in the neonatal period. Abdominal X-ray imaging displayed calcification of the bilateral adrenal gland regions. Hepatosplenomegaly and bilateral adrenal gland posterior acoustic shadowing due to calcifications were revealed by abdominal sonography. Magnetic resonance imaging showed enlarged hypointense adrenal glands in all sequences and hepatosplenomegaly. The lysosomal acid lipase levels were low compatible with Wolman disease.
Multimodality radiologic imaging methods should be performed to display hepatosplenomegaly, hepatosteatosis, bilateral adrenal gland enlargement and calcification in Wolman disease. Read More

Authors:  Aslan Mine , Kalyoncu Ucar Ayse , Arioz Habibi Hatice , Namdar Yesim , Kurugoglu Sebuh , Adaletli Ibrahim

Keywords:  Wolman Disease, Sonography, Adrenal gland, calcification, magnetic resonance imaging

Goncalves Fabricio,  Pinelli Lorenzo,  Mankad Kshitij,  Alves Cesar Augusto,  Teixeira Sara,  Andronikou Savvas,  Vossough Arastoo

Final Pr. ID: Poster #: EDU-050

Intracranial calcification (ICC) can be either physiological or pathological. Physiological ICC is not an expected neuroimaging finding in the neonatal period but can be seen as children grow older in the pineal gland, habenula, choroid plexus, and occasionally in the dura mater. Pathological ICC can be broadly divided into six groups: infectious, toxic, neurodegenerative, neoplastic, vascular, and syndromic. The first two groups are typically composed of diseases that more commonly result in static encephalopathies, whereas the last four groups are composed of diseases that tend to cause progressive encephalopathy.

Various neuroimaging modalities have distinct utilities and sensitivities in the depiction of ICC. Age at presentation, ICC location, and additional neuroimaging findings are useful information that may be useful to narrow down the differential diagnosis of ICC. Bilateral ICC is commonly due to congenital infections or due to neurodegenerative or infectious diseases. ICC involving the basal ganglia and thalami are commonly seen in neurodegenerative diseases. ICC can be seen in isolation or be associated with other neuroimaging features.

TORCH infections are the most common neonatal causes of ICC. ICC in congenital infections can be associated with clastic changes, hydrocephalus, chorioretinitis, white matter abnormalities, skull changes, and cortical development malformations. Specific non-infectious causes of ICC that mimic TORCH infections are known as pseudo-TORCH. Neurodegenerative diseases causing ICC are mainly due to parathyroid and thyroid hormone dysfunction and inborn errors of metabolism, such as MELAS, Kearns Sayre and Cockayne syndrome, interferonopathies syndrome, and Krabbe disease. Tumoral ICCs are more commonly seen in low-grade tumors. Arteriovenous malformations, arteriovenous fistulas, chronic venous hypertension, and cavernomas are also known causes of ICC. Other vascular causes of ICC include atherosclerosis, healed hematoma, radiotherapy treatment, old infarct, and disorders of the microvasculature such as COL4A1- and COL4A2-related diseases. Down syndrome and phakomatosis are also known causes of ICC.

Clinical information such as age at presentation; maternal exposure to teratogens, such as virus; in addition to the association with chromosomal abnormalities; genetic mutations and postnatal infections, facilitate in the differential diagnosis of the multiple causes of ICC. Read More

Authors:  Goncalves Fabricio , Pinelli Lorenzo , Mankad Kshitij , Alves Cesar Augusto , Teixeira Sara , Andronikou Savvas , Vossough Arastoo

Keywords:  Calcification, Computed Tomography, Magnetic Resonance

Donuru Achala,  Kandula Vinay,  Averill Lauren

Final Pr. ID: Poster #: EDU-073

The purpose of educational exhibit is to summarize the radiological appearances of various conditions causing basal ganglia (BG) calcification in children. The pathogenesis for symmetric BG calcification is diverse and ranges from benign physiological calcifications to a variety of pathological disorders including metabolic, infectious and genetic diseases. We present a practical approach to further narrow the differential diagnosis based on associated musculoskeletal imaging findings in patients with BG calcification.
Parathyroid disorders are the most common causes of pathological BG calcification. In hyperparathyroidism, distinctive subperiosteal bone resorption can be seen in phalanges on hand radiographs. Tapering of the clavicles, brown tumors and salt-and-pepper appearance of the skull are also classic radiographic features. In the Albright hereditary osteodystrophy phenotype of pseudohypoparathyroidism, shortening of the fourth and fifth metacarpals as well as advanced bone age can aid in diagnosis. Tumoral calcinosis is another radiographically distinct disease that can cause BG calcification, characterized in the extremities by periarticular calcific deposits. Cockayne syndrome is a rare autosomal recessive (AR) disorder with 4 overlapping subtypes, all with BG calcification as well as diffuse skeletal abnormalities that become apparent in the toddler years. Carbonic anhydrase deficiency type 2 is another rare AR disorder with intracranial calcification including the BG, as well as osteopetrosis and pathologic fractures. Coat’s plus syndrome has characteristic rock-like intracranial calcifications paired with leukodystrophy and brain cysts. It manifests skeletally with osteopenia, delayed fracture healing, bowing of long bones, scoliosis, midface hypoplasia and femoral head avascular necrosis. Other common conditions such a Down syndrome and HIV, and rare disorders such Fahr's disease, malignant phenylketonuria, and Aicardi-Goutieres syndrome are in the differential diagnosis of BG calcifications. Read More

Authors:  Donuru Achala , Kandula Vinay , Averill Lauren

Keywords:  basal ganglia, calcification, musculoskeletal

Burns Madisen,  Cao Joseph,  Carrico Caroline

Final Pr. ID: Poster #: CR-001

Aortic calcifications are an uncommon finding in the pediatric population and when present are often reflective of underlying congenital heart or metabolic disease. Intraluminal polypoid calcified lesions (coral reef aorta) causing flow limiting stenosis are exceedingly rare in even the congenital heart disease population.
A 15-year-old patient with a history of hypoplastic left heart syndrome (HLHS) with mitral and aortic atresia. She was found to have significant flow limiting stenosis of the augmented neo-aorta under catheter hemodynamic assessment. CT angiography was performed showing a 4.4 cm partially calcified mass in the ascending aorta extending into the proximal arch causing an 80% narrowing of the aortic lumen. The intrinsic multi-energy capabilities of the first-generation photon counting CT (PCCT) scanner used permitted retrospective material decomposition and thus more definitive characterization of the mass. The patient underwent open ascending aorta replacement and arch repair where densely calcified ascending aorta and base of the arch were removed en bloc along with a large intraluminal mass component without complication. Pathologic assessment revealed PTFE conduit material, thrombus, and extensive calcification with fibrous incorporation of graft and arterial wall. Read More

Authors:  Burns Madisen , Cao Joseph , Carrico Caroline

Keywords:  Aorta, Calcifications, Stenosis

Funes Poblete Flavia,  Mackintosh Cecilia,  Vargas Maria,  Gonzalez Veronica

Final Pr. ID: Poster #: CR-021

We report a case of 2-year-old female, twin, low birth weight (37 weeks, 1630 g - 3.59 lb), who was referred to our hospital to study an indurated nodular tumor involving her left leg, which extended from her pelvis to her left foot. Blood tests showed only an increase in alkaline phosphatase (862 mg/dl). Lower-limb X-ray was performed, which showed an extensive calcified mass with fibrillar pattern affecting dermis and deep connective tissue. MRI was also performed and showed an extensive signal alteration in muscle and dermis with hipointense signal in relation to heterotopic calcifications.
The differential diagnoses were Albright hereditary osteodystrophy (AHO), fibrodysplasia ossificans progressiva (FOP), and progressive osseous heteroplasia (POH).
AHO was ruled out because of the lack of compromise of muscle plane, and the absence of congenital malformations (such as brachydactyly, obesity, low height), hypocalcemia and hyperphosphatemia. Similarly, FOP was also ruled out since it is characterized by muscle ossification, with no dermal calcifications, and hallux malformation (also absent in this case).
The diagnosis reached was POH, which is a rare genetic (autosomal dominant inheritance) disorder characterized by progressive periarticular ossification of dermis and deep connective tissues (muscles, tendons and ligaments), with no congenital malformations. The diagnosis is built upon the clinical symptoms, imaging findings and genetic study (GNAS mutation). There is no effective treatment, with frequent progression to ankylosis. Read More

Authors:  Funes Poblete Flavia , Mackintosh Cecilia , Vargas Maria , Gonzalez Veronica

Keywords:  heteroplasia, dermal calcification, calcified mass