Japanese consensus guidelines for pediatric nuclear medicine. Part 1: Pediatric radiopharmaceutical administered doses (JSNM pediatric dosage card). Part 2: Technical considerations for pediatric nuclear medicine imaging procedures.

The Japanese Society of Nuclear Medicine has recently published the consensus guidelines for pediatric nuclear medicine. This article is the English version of the guidelines. Part 1 proposes the dose optimization in pediatric nuclear medicine studies. Part 2 comprehensively discusses imaging techniques for the appropriate conduct of pediatric nuclear medicine procedures, considering the characteristics of imaging in children.

Guidelines for imaging and staging of neuroblastic tumors: consensus report from the International Neuroblastoma Risk Group Project.

Neuroblastoma is an enigmatic disease entity; some tumors disappear spontaneously without any therapy, while others progress with a fatal outcome despite the implementation of maximal modern therapy. However, strong prognostic factors can accurately predict whether children have "good" or "bad" disease at diagnosis, and the clinical stage is currently the most significant and clinically relevant prognostic factor. Therefore, for an individual patient, proper staging is of paramount importance for risk assessment and selection of optimal treatment. In 2009, the International Neuroblastoma Risk Group (INRG) Project proposed a new staging system designed for tumor staging before any treatment, including surgery. Compared with the focus of the International Neuroblastoma Staging System, which is currently the most used, the focus has now shifted from surgicopathologic findings to imaging findings. The new INRG Staging System includes two stages of localized disease, which are dependent on whether image-defined risk factors (IDRFs) are or are not present. IDRFs are features detected with imaging at the time of diagnosis. The present consensus report was written by the INRG Imaging Committee to optimize imaging and staging and reduce interobserver variability. The rationales for using imaging methods (ultrasonography, magnetic resonance imaging, computed tomography, and scintigraphy), as well as technical guidelines, are described. Definitions of the terms recommended for assessing IDRFs are provided with examples. It is anticipated that the use of standardized nomenclature will contribute substantially to more uniform staging and thereby facilitate comparisons of clinical trials conducted in different parts of the world.

Can biliary atresia be diagnosed by ultrasonography alone?

BACKGROUND/PURPOSE: The triangular cord sign (TCS) is a specific ultrasonographic finding, reflecting a fibrotic mass at the porta hepatis in biliary atresia (BA). We evaluated whether BA can be diagnosed by ultrasonography alone using 3 findings: TCS, gallbladder length (GBL), and gallbladder contractility (GBC). METHODS: Subjects comprised 85 infants (median age, 47 days; range, 4-144 days) with cholestatic jaundice who underwent ultrasonographic examination for diagnosis between May 1996 and June 2006. Medical records were reviewed with regard to TCS, GBL, and GBC. Positive findings for BA included TCS >or=3 mm, GBL <15 mm, and GBC <68% (for < 12 weeks) or <25% (for >or=12 weeks). RESULTS: Biliary atresia was diagnosed in 48 patients surgically, with other cholestatic diseases diagnosed in the remaining 37 patients. Triangular cord sign was positive in 41 of 48 infants with BA and negative in 35 of 37 infants without BA. The 7 patients with BA who displayed negative results for TCS displayed positive findings for GBL or GBC. Positive predictive value in the diagnosis of BA was 98% if positive TCS was combined with abnormal gallbladder findings, whereas negative predictive value in diagnosis of BA was 100% if negative TCS was combined with findings of a normal gallbladder. CONCLUSIONS: Biliary atresia can be accurately diagnosed by ultrasonography using the findings of TCS combined with GBL and GBC.

Development of acquired tracheal stenosis in premature infants due to prolonged endotracheal ventilation: etiological considerations and surgical management.

The aim of this study was to determine the etiology and appropriate surgical treatment for acquired tracheal stenosis that developed in patients who had undergone prolonged endotracheal mechanical ventilation as premature neonates. During the period 2000-2004, four patients aged 1-16 years were referred for tracheal stenosis characterized by stridor, choking, and recurrent pulmonary infection. All patients had undergone endotracheal mechanical ventilation for 2-5 months for respiratory distress related to prematurity (gestational age 25-29 weeks, birth weight 648-1,222 g). During this period, methicillin-resistant Staphylococcus aureus was predominantly cultured from the trachea. All patients exhibited a stenotic lesion encompassing 30-37% of the entire tracheal length on spiral CT. On palpation and inspection of the trachea during surgery, however, the stenotic segment appeared to encompass over 50% of the entire trachea. The carina was intact. Three patients underwent slide tracheoplasty with a tracheal resection and the other underwent resection and end-to-end anastomosis. Of the three patients treated by slide tracheoplasty, two are currently free of respiratory symptoms. However, one patient in this group required secondary resection of the remaining stenotic lesion with end-to-end anastomosis. This patient is currently asymptomatic. The remaining patient who underwent a resection and end-to-end anastomosis is doing well. The resected specimen showed fibrosis and degeneration of tracheal cartilage. A combination of prematurity, prolonged endotracheal mechanical ventilation and tracheal infection seem to be responsible for tracheal inflammation and stenosis. When considering surgical procedures for acquired tracheal stenosis, resection and end-to-end anastomosis are desirable. However, slide tracheoplasty with a partial tracheal resection is indicated for the treatment of stenosis involving a long tracheal segment.

Communicating bronchopulmonary foregut malformation: particular emphasis on concomitant congenital tracheobronchial stenosis.

A total of four patients with communicating bronchopulmonary foregut malformation were treated surgically at Kobe Children's Hospital between 1993 and 2004. Of these, three patients displayed congenital tracheobronchial stenosis and developed life-threatening respiratory distress soon after birth. In each case, anomalous bronchi arose from the lower portion of the esophagus and connected to the lower part of the ipsilateral lung. This anomaly involved the right lung in two patients, and the left lung in one patient. Tracheobronchial stenosis extended from the inlet of the thorax to the carina in one patient, and to the contralateral main stem bronchus in two patients. Surgical treatment included division of the esophageal bronchus and anastomosis of bronchus to the trachea in one patient. In the other patient, the ipsilateral lung was resected and the stenotic tracheobronchus was stented. The remaining patient underwent pneumonectomy of the ipsilateral lung. Details of this fatal anomaly and a discussion of appropriate surgical management are described herein.

Sonographic diagnosis of biliary atresia in pediatric patients using the "triangular cord" sign versus gallbladder length and contraction.

OBJECTIVE: A retrospective review was performed to evaluate the importance of the "triangular cord" sign in comparison with gallbladder length and contraction for the diagnosis of biliary atresia in pediatric patients. MATERIALS AND METHODS: Fifty-five fasting infants with cholestatic jaundice were examined on sonography. The examinations focused on the visualization of the triangular cord sign and assessment of gallbladder length and contraction. The diagnosis of neonatal hepatitis or of other causes of infantile cholestasis was made if symptom resolution occurred during follow-up. RESULTS: A triangular cord sign was found in 27 of 29 infants with biliary atresia and in one of 26 infants with neonatal hepatitis or other causes of infantile cholestasis. The diagnostic accuracy was 95%, sensitivity was 93%, and specificity was 96%. The gallbladder was thought to be abnormal if it was less than 1.5 cm long, was not detectable, or was detectable but had no lumen. The gallbladder was abnormal in 21 of 29 infants with biliary atresia, whereas it was abnormal in eight of 26 infants with neonatal hepatitis or other causes of infantile cholestasis. The diagnostic accuracy was 71%, sensitivity was 72%, and specificity was 69%. The gallbladder was detectable on sonography in 13 infants with biliary atresia and 26 infants with neonatal hepatitis or other causes of infantile cholestasis. Gallbladder contraction was not confirmed in 11 of 13 infants with biliary atresia and seven of 26 infants with neonatal hepatitis or other causes of infantile cholestasis. The diagnostic accuracy was 77%, sensitivity was 85%, and specificity was 73%. CONCLUSION: The triangular cord sign was a more useful sonographic finding for diagnosing biliary atresia than gallbladder length and contraction.

Congenital esophageal stenosis: Therapeutic strategy based on etiology.

BACKGROUND/PURPOSE: The diagnosis and treatment of congenital esophageal stenosis (CES) can be a vexing clinical problem. This study aims at determining the ideal therapeutic strategy for the management of CES. METHODS: Medical records of patients with CES were reviewed retrospectively with regard to diagnostic method, therapy, and outcome. RESULTS: During the last 29 years, 36 patients, aged 1 month to 9 years, were found to have CES. Diagnosis was made by fluoroscopy, esophagoscopy, endoscopic ultrasonography (EUS), and pathologic examination. Of the 36 patients, 15 had tracheobronchial remnants (TBR), 13 had fibromuscular stenosis (FMS), 5 had membranous stenosis (MS), and 3 had multiple stenoses (MPS). EUS was diagnostic in distinguishing TBR from FMS. All patients with TBR underwent operative repair in which 13 patients had resection and anastomosis with or without preoperative dilation. The 14th patient had enucleation and the 15th underwent myotomy. Postoperative dilation was needed 2.2 times for 2.1 months on average in this group of patients. Ten patients with FMS were treated by dilation only (a mean of 4.7 dilations was required for a duration of 2.6 years), whereas 3 patients underwent open surgical intervention. Most patients with MS or MPS were treated by dilation (with or without endoscopic resection of the membranes), whereas 2 patients had surgical intervention. All patients in this study are doing well after a mean follow-up period of 5.3 years (range, 0.5 to 25 years) without any further operative interventions. CONCLUSIONS: If the etiologic diagnosis of CES is unclear, EUS is useful in distinguishing TBR from FMS. This distinction is critical, because patients with TBR should undergo surgical resection, whereas most cases of FMS, MS, and MPS can be treated with esophageal dilation alone.