Extralobar pulmonary sequestration in neonates: The natural course and predictive factors associated with spontaneous regression.

Purpose: To describe the natural course of extralobar pulmonary sequestration (EPS) and identify factors associated with spontaneous regression of EPS.

Materials And Methods: We retrospectively searched for patients diagnosed with EPS on initial contrast CT scan within 1 month after birth and had a follow-up CT scan without treatment. Spontaneous regression of EPS was assessed by percentage decrease in volume (PDV) and percentage decrease in sum of the diameter of systemic feeding arteries (PDD) by comparing initial and follow-up CT scans.

Cranial Nerve Disorders in Children: MR Imaging Findings.

Cranial nerve disorders are uncommon disease conditions encountered in pediatric patients, and can be categorized as congenital, inflammatory, traumatic, or tumorous conditions that involve the cranial nerve itself or propagation of the disorder from adjacent organs. However, determination of the normal course, as well as abnormalities, of cranial nerves in pediatric patients is challenging because of the small caliber of the cranial nerve, as well as the small intracranial and skull base structures. With the help of recently developed magnetic resonance (MR) imaging techniques that provide higher spatial resolution and fast imaging techniques including three-dimensional MR images with or without the use of gadolinium contrast agent, radiologists can more easily diagnose disease conditions that involve the small cranial nerves, such as the oculomotor, abducens, facial, and hypoglossal nerves, as well as normal radiologic anatomy, even in very young children.

Cholangiographic characteristics of common bile duct dilatation in children.

Aim: To investigate whether children with congenital common bile duct dilatation (CBDD) differ from children with obstructive CBDD in cholangiographic characteristics.

Methods: In this retrospective cohort study, the baseline data and the results of imaging analyses were reviewed among children who had endoscopic retrograde cholangiopancreatography (ERCP) due to CBDD. ERCP was performed on all pediatric patients by experienced pediatric endoscopists.

Humidifier disinfectant-associated children's interstitial lung disease: Computed tomographic features, histopathologic correlation and comparison between survivors and non-survivors.

Objectives: To report radiologic findings with histopathologic correlations of humidifier disinfectant-associated children's interstitial lung disease (HD-chILD) and to compare computed tomography (CT) findings between survivors and non-survivors.

Methods: Forty-seven children with HD-chILD (27.4 ± 12.

Transperineal ultrasonography for evaluation of the perianal fistula and abscess in pediatric Crohn disease: preliminary study.

Purpose: To assess the feasibility and effectiveness of transperineal ultrasonography (TPUS) for the evaluation of perianal Crohn disease (PCD) in pediatric patients.

Methods: Between September 2010 and August 2013, 64 TPUS examinations were performed in 43 patients (34 males and 9 females; mean age±standard deviation, 13.3±2.

Improved outcomes in liver transplantation in children with acute liver failure.

Objective: The aim of our study was to review the experiences of a living donor-dominant transplantation program for children with acute liver failure (ALF).

Methods: Data were derived from the retrospective chart review of 50 children with ALF in a major liver center in the Republic of Korea.

Results: A total of 50 children with ALF underwent 47 (94%) primary living donor liver transplantations and 3 (6%) cadaveric liver transplantations.

A case of campomelic dysplasia without sex reversal.

Campomelic dysplasia (CD; OMIM #114290), a rare form of congenital short-limbed dwarfism, is due to mutations in SOX9, a member of the SOX (SRY-related HMG box) gene family. Multiparous mother at 38 weeks' gestation delivered a 3,272 g baby boy with characteristic phenotypes including bowing of the lower limbs, a narrow thoracic cage, 11 pairs of ribs, hypoplastic scapulae, macrocephaly, flattened supraorbital ridges and nasal bridge, cleft palate, and micrognathia. He underwent a tracheostomy at the age of three months for severe laryngomalacia after a number of repeated hospitalizations due to respiratory problems and died at the age of four months from progressive respiratory failure.

An 18-year experience of tracheoesophageal fistula and esophageal atresia.

Purpose: To determine the clinical manifestations and outcomes of patients with tracheoesophageal fistula (TEF) and esophageal atresia (EA) born at a single neonatal intensive care unit.

Methods: A retrospective analysis was conducted for 97 patients with confirmed TEF and EA who were admitted to the neonatal intensive care unit between 1990 and 2007.

Results: The rate of prenatal diagnosis was 12%.

Long-term outcomes of pediatric living donor liver transplantation at a single institution.

There have only been a few studies on the long-term outcomes and prognostic factors after pediatric LDLT. We conducted a retrospective, single-center assessment of the outcomes as well as the demographic and clinical factors that influenced the poor outcomes in 113 children aged <16 (median age 21 months; 6 months-15.5 yr) who underwent 115 LDLTs, predominantly for biliary atresia (60.

Endoscopic retrograde cholangiopancreatography in pancreatic and biliary tract disease in Korean children.

Aim: To assess the indications, findings, therapeutic procedures, safety, and complications of endoscopic retrograde cholangiopancreatography (ERCP) performed in Korean children.

Methods: The demographic characteristics, indications for ERCP, findings, therapeutic procedures, and complications of 122 pediatric patients who underwent 245 ERCPs in the Asan Medical Center between June 1994 and March 2008 were investigated.

Results: The mean age of the 122 patients was 8.

Characteristics and trends in the incidence of inflammatory bowel disease in Korean children: a single-center experience.

Background: Inflammatory bowel disease (IBD) is rare in Asian children and few reports on pediatric IBD have appeared.

Aims: We, therefore, investigated the incidence trends and clinical characteristics of pediatric IBD in Korea.

Methods: We enrolled 48 children with Crohn's disease (CD) and 14 children with ulcerative colitis (UC) from 1996 to 2007.

Neonatal pulmonary sequestration: clinical experience with transumbilical arterial embolization.

Pulmonary sequestration (PS) is a rare congenital malformation of the lower respiratory tract. The exact natural course of PS is not well understood and there are no well-established treatment guidelines for antenatally diagnosed PS. The aim of this study was to describe clinical outcomes in neonates with PS and to evaluate the efficacy of transumbilical arterial embolization (TUE).

Comparison of the clinical manifestations, brain MRI and prognosis between neuroBeçhet's disease and neuropsychiatric lupus.

Background: Neuropsychiatric systemic lupus erythematosus (NPSLE) shows some similarities to neuroBeçhet disease (NBD) in that both conditions have some analogous clinical features and they are both pathologically associated cerebral vasculopathy. This study compared the clinical manifestations, brain MRI findings and prognosis of NPSLE and NBD patients.

Methods: Forty three patients with NPSLE (n = 25) or NBD (n = 18), who were monitored at a single center, were enrolled in this study.

Cytomegalovirus retinitis in a patient with dermatomyositis.

Cytomegalovirus (CMV) retinitis rarely occurs in patients with connective tissue disease. We present a rare case of CMV retinitis in a patient with dermatomyositis. A 38-year-old man was admitted due to blurred vision and floaters of right eye.

Implication of genetic variations in congenital obstructive nephropathy.

The renin-angiotensin system (RAS) has long been implicated in kidney development, and it has been reported that disruption of angiotensin type 2 receptor (AGTR2) results in a wide range of congenital anomalies of the kidney and urinary tract. We investigated the allele frequencies of the AGTR2 and other RAS genes in Korean patients with ureteropelvic junction obstruction, multicystic dysplastic kidney (MCDK), and unilateral renal agenesis (RA). Fifty-three Korean children were enrolled: 37 boys and 16 girls, 27 with hydronephrosis, 23 with MCDK, and 3 with RA.

Multi-slice spiral CT of living-related liver transplantation in children: pictorial essay.

In pediatric living-related liver transplantation, preoperative evaluation of the recipient is important for surgical planning, while the accurate diagnosis of postoperative complications is essential for graft salvage. Multiplanar and three-dimensional imaging using multi-slice spiral CT can be used for preoperative vascular imaging, as well as for evaluating postoperative complications. In this essay, we describe the usefulness of multi-slice CT, combined with a variety of different reconstruction techniques, for the preoperative evaluation of transplant recipients.

Congenital intrahepatic arterioportal and portosystemic venous fistulae with jejunal arteriovenous malformation depicted on multislice spiral CT.

We report a symptomatic infant with very rare congenital arterioportal and portosystemic venous fistulae in the liver. Multislice CT after partial transcatheter embolisation revealed not only the complicated vascular architecture of the lesion, but also an incidental jejunal arteriovenous malformation which explained the patient's melena. The patient underwent ligation of the hepatic artery and resection of the jejunal arteriovenous malformation.

CT of congenital heart disease: normal anatomy and typical pathologic conditions.

Computed tomography (CT) plays an important supplementary role in the evaluation of patients with congenital heart disease (CHD). Fast multisection spiral CT can be used to obtain isotropic volume data, and high-quality two- and three-dimensional multiplanar reformatted images can be created to accurately and systematically delineate the normal and pathologic morphologic features of the cardiovascular system. CT may be technically challenging and demanding in uncooperative young children.

Acute pancreatitis associated with biliary disease in children.

Background And Aims: Biliary disease is one of the most common causes of acute pancreatitis in adults; however, this cause and outcome in children have rarely been described in the literature. Therefore, the present study was conducted to evaluate the role of biliary disease as a cause of acute pancreatitis in children.

Methods: The present study included 56 children with acute pancreatitis, of which 16 (29%) cases were associated with biliary disease.

Value of supranormal function and renogram patterns on 99mTc-mercaptoacetyltriglycine scintigraphy in relation to the extent of hydronephrosis for predicting ureteropelvic junction obstruction in the newborn.

Unlabelled: We investigated the value of supranormal function and renogram patterns on (99m)Tc-mercaptoacetyltriglycine ((99m)Tc-MAG3) scintigraphy in relation to the extent of hydronephrosis for predicting ureteropelvic junction (UPJ) obstruction in the newborn.

Methods: We studied 48 patients with prenatally diagnosed unilateral hydronephrosis that was confirmed on postnatal ultrasonography. The anteroposterior pelvic diameter (APPD) and the Society for Fetal Urology (SFU) grade were measured on 1-mo ultrasonography to assess the extent of hydronephrosis.

Intra-abdominal desmoplastic small round-cell tumour: multiphase CT findings in two children.

We report the multiphase CT findings of intra-abdominal desmoplastic small round-cell tumour (DSRCT) in two children. CT showed a huge heterogeneous intraperitoneal mass with or without direct invasion into solid organs such as liver or kidney, extensive intraperitoneal seeding, intratumoural calcification, ascites, and lymphadenopathy. DSRCT should be included in the differential diagnosis of malignant intraperitoneal neoplasm in children.

Acute necrotizing encephalopathy: diffusion MR imaging and localized proton MR spectroscopic findings in two infants.

In this report, we describe the findings of diffusion MR imaging and proton MR spectroscopy in two infants with acute necrotizing encephalopathy in which there was characteristic symmetrical involvement of the thalami. Diffusion MR images of the lesions showed that the observed apparent diffusion coefficient (ADC) decrease was more prominent in the first patient, who had more severe brain damage and a poorer clinical outcome, than in the second. Proton MR spectroscopy detected an increase in the glutamate/glutamine complex and mobile lipids in the first case but only a small increase of lactate in the second.

Transcatheter arterial embolization of pulmonary sequestration in neonates: long-term follow-up results.

The purposes of this study are to describe experience with the safety and long-term efficacy of transcatheter arterial embolization (TAE) of pulmonary sequestration in neonates and to introduce a new technique of embolization by an umbilical-artery route. TAE was performed in five neonates, via the femoral artery in one and the umbilical artery in four. Complete regression was achieved in four cases and partial regression (>90%) was obtained in one.

Lateral decubitus HRCT: a simple technique to replace expiratory CT in children with air trapping.

Objective: To evaluate the effectiveness of lateral decubitus high-resolution CT (HRCT) in detecting air trapping in children.

Materials And Methods: HRCT scans of 21 children with heterogeneous lung attenuation caused by air trapping (n = 10) or with infiltrative lung disease (n = 11) were reviewed retrospectively. The air-trapping disease included bronchiolitis obliterans (n = 7), bronchial obstruction due to mediastinal lymphoma (n = 1), endobronchial haemangioma (n = 1) and foreign body aspiration (n = 1).