Atsuhiko Handa

Scurvy: From a Selective Diet in Children with Developmental Delay.

A 5-year-old boy with developmental delay presented with painful knees and difficulty walking for a month. He had been diagnosed with autism spectrum disorder and had a selective diet, only consuming bread, snacks, and water. Radiograph of the knee demonstrated a radiodense band at the chondroosseous junction and a translucent band in the metaphysis of the distal femora and the proximal tibiae (Figure, A), which raised a possibility of leukemic bands. However, the child’s complete blood count result was unremarkable. Prior to the scheduled bone marrow biopsy, magnetic resonance imaging (MRI) was obtained, which demonstrated ill-defined T2 hyperintensities in the metaphyses and juxtaosseous soft tissue (Figure, B). The diaphysis was spared. These findings led us to another possible diagnosis of scurvy. His serum vitamin C level was undetectable (<0.2 mg/dL).Vitamin supplementation improved his symptoms over the next 2 months. Scurvy or vitamin C deficiency is an ancient condition that has been known since the “Age of Discovery.” In modern developed countries, scurvy has become rare; however, it can still be seen in children with a very selective diet. In particular, children with developmental delay are at an increased risk for selective diet, both from the children’s choice and from the parents’ offering a limited variety of food. Affected children may have only musculoskeletal symptoms without classical hemorrhagic diathesis. Radiography may show only some of the classic signs of scurvy (generalized osteoporosis with Wimberger ring, Pelkan spur, Frankel line, and Trummerfeld zone). Recently, several reports about MRI findings of scurvy have been published. They recapitulate classic histologic features of scurvy at bone ends, such as disruption of the chondroosseous junction and metaphyseal reparative changes. Diaphyseal involvement is less common.MRI usually, but not always, enables clinicians to distinguish scurvy from other bone marrow infiltrative disorders, such as leukemia or metastatic neuroblastoma. Clinicians should be aware of scurvy as a differential diagnosis of children with painful limbs, particularly those with developmental delay and selective diet. ■

National trend survey of hospitalized patients with febrile seizure in the United States

PURPOSE Several studies have reported the prevalence and incidence of febrile seizure (FS) among children in the USA and other countries. However, recent trends in FS among hospitalized children, hospital course, and risk factors for its severity remain unknown at a national level in the USA. METHOD Hospital discharge records of patients with FS aged <6years were obtained for the years 2003, 2006, 2009, and 2012 from the Kids Inpatient Database. Data were weighted to estimate the annual hospitalization rates with respect to gender and race/ethnicity in the United States. Multivariable logistic regression was conducted to ascertain factors associated with FS severity. RESULTS A decreasing trend in total annual hospitalization rates due to FS was observed, ranging from 59.0 per 100,000 children in 2003 to 40.8 per 100,000 children in 2012 (p < 0.001). Winter predominance of hospitalizations was observed (p = 0.001). Hispanic children and children admitted to hospitals in northeast region were less likely to be severely affected. Age, gender, health insurance status, and household income level were not associated with FS severity. CONCLUSIONS Total hospitalization rates due FS is decreasing, and race/ethnicity and geographic locations of the patients were associated with FS severity.

Radiological findings of perivascular epithelioid cell tumour (PEComa) of the falciform ligament.

Perivascular epithelioid cell tumour (PEComa) encompasses a group of mesenchymal tumours composed of histologically and immunohistochemically distinctive perivascular epithelioid cells. A subset of PEComa that typically arises from the falciform ligament and/or ligamentum teres is termed clear cell myomelanocytic tumour of the falciform ligament/ligamentum teres. To date, its imaging findings have not been described. Here, we report the first radiological description of a pathologically confirmed tumour. The patient was a 5‐year‐old girl with a palpable abdominal mass. US, CT, MR and FDG‐PET revealed a midline, well‐defined, solid anterior abdominal wall tumour below the rectus abdominis and contiguous with the umbilicus that was hypervascular and FDG avid. Awareness of these imaging findings facilitates the diagnosis of this distinctive tumour.

Acute chest syndrome among children hospitalized with vaso‐occlusive crisis: A nationwide study in the United States

Acute chest syndrome (ACS) is a common complication among pediatric inpatients with sickle cell disease and vaso‐occlusive crisis (VOC). However, little is known about the factors associated with ACS complication. The present study assessed the epidemiological features of children hospitalized with VOC and ascertained factors associated with ACS complication.

MR imaging findings in some rare neurological complications of paediatric cancer

Neurological complications of paediatric cancers are a substantial problem. Complications can be primary from central nervous system (CNS) spread or secondary from indirect or remote effects of cancer, as well as cancer treatments such as chemotherapy and radiation therapy. In this review, we present the clinical and imaging findings of rare but important neurological complications in paediatric patients with cancer. Neurological complications are classified into three phases: pre-treatment, treatment and post-remission. Paraneoplastic neurological syndromes, hyperviscosity syndrome, haemophagocytic lymphohistiocytosis and infection are found in the pre-treatment phase, while Trousseau’s syndrome, posterior reversible encephalopathy syndrome and methotrexate neurotoxicity are found in the treatment phase; though some complications overlap between the pre-treatment and treatment phases. Hippocampal sclerosis, radiation induced tumour, radiation induced focal haemosiderin deposition and radiation-induced white matter injury are found in the post-remission phase. With increasingly long survival after treatment, CNS complications have become more common. It is critical for radiologists to recognise neurological complications related to paediatric cancer or treatment. Magnetic resonance imaging (MRI) plays a significant role in the recognition and proper management of the neurological complications of paediatric cancer.Teaching Points• Neurological complications of paediatric cancer include various entities.• Neurological complications are classified into three phases: pre-treatment, treatment and post-remission.• Radiologists should be familiar with clinical and imaging findings of neurological complications.• MRI features may be characteristic and lead to early diagnosis and proper treatments.

Additional report on Moreno-Nishimura-Schmidt overgrowth syndrome

1MassGeneral Hospital for Children and Harvard Medical School, Boston, Massachusetts 2Department of Radiology, St. Luke’s International Hospital, Tokyo, Japan 3Department of Endocrinology and Metabolism, Kanagawa Children’s Medical Center, Yokohama, Japan 4Department of Pediatrics, Keio University School of Medicine, Tokyo, Japan 5Department of Radiology, Tokyo Metropolitan Children’s Medical Center, Tokyo, Japan

Insights and ImagesTracheal Tumor

A 1-year-old boy was referred for evaluation of persistent cough and wheezing. His respiratory symptoms had gradually increased over the prior 3 months, and he had experienced multiple episodes of pneumonia and/or atelectasis mainly in the left lung. Physical examination showed decreased breath sounds on the left side. Chest radiograph showed hyperinflation of the left lung (Figure 1). Computed tomography of the chest demonstrated a well-demarcated, intraluminal tracheal mass that originated from the posterior wall of the tracheal bifurcation that nearly occluded the left main bronchus (Figures 2 and 3; Figure 3 available at www. jpeds.com). He was scheduled for elective surgery, but he developed respiratory failure. Emergent endoscopic laser resection of the tumor was performed (Figure 4; available at www.jpeds.com). Histologic examination revealed a benign fibrous histiocytoma. He underwent endoscopic laser resections 3 times over the next 3 months due to difficulty in completely resecting the tumor. There is no recurrence on further follow-up. Persistent cough, a commonly encountered clinical condition, may be caused by a variety of conditions, such as congenital anomalies, infection, asthma, aspiration, environmental, and psychological causes. Following detailed history and physical examination, chest radiograph is the first diagnostic test for evaluation and may lead to specific diagnoses or help determine subsequent diagnostic tests.

Multiple migratory recurrence of Kikuchi-Fujimoto disease

Kikuchi–Fujimoto disease (KFD), or histiocytic necrotizing lymphadenitis, is a benign self-limiting disorder of unknown cause that manifests as prolonged fever and cervical lymphadenopathy. Recurrence of KFD or involvement of other lymph nodes is rare, but has been previously reported. Multiple recurrences, however, are extremely rare and there are no reports of migratory recurrence of KFD. Here, we report one such case that predominantly involved intra-abdominal lymph nodes in the third episode, showing characteristic computed tomography (CT) findings for KFD.