Sabah Servaes

Sonographic findings of ovarian torsion in children

BackgroundThe clinical diagnosis of ovarian torsion is challenging and findings on pelvic sonography can be pivotal in making the correct diagnosis.ObjectiveTo determine the sonographic characteristics in children of surgically and pathologically proven ovarian torsion.Material and methodsWe performed a retrospective review of the sonograms and medical records of 41 patients with surgically and pathologically proven ovarian torsion at a pediatric hospital between 1994 and 2005. All sonograms were reviewed retrospectively by two pediatric radiologists with attention to the size, echotexture, location, presence of peripheral round cysts, and evidence of flow on Doppler sonography within the torsed ovary. The amount of free pelvic fluid was also recorded.ResultsThe most common sonographic finding of ovarian torsion was an enlarged ovary/adnexal mass. All torsed adnexa were larger than the normal contralateral ovary, with the median volume 12 times that of the normal contralateral side. The majority (61%, n = 25) of the torsions occurred on the right. Color flow, either venous or arterial, was present in 62% (n = 21/34) of the torsed ovaries for which flow on Doppler sonography was documented. In 63% of the torsed ovaries (n = 26), the torsed adnexa appeared heterogeneous. Ovarian or para-ovarian pathology that may have acted as a potential lead point was present in 55% (n = 24) of torsed ovaries. The volume ratio of the torsed to normal ovary can predict the presence of an ovarian mass within the torsed ovary. In 70% of torsed ovaries with a volume ratio greater than 20, an ovarian mass was present, and in approximately 90% of those with a volume ratio less than 20, an internal mass was absent.ConclusionAn enlarged heterogeneous appearing ovary is the most common finding in ovarian torsion. The presence or absence of flow by Doppler sonography is not helpful in the diagnosis. The volume ratio of the torsed to the normal ovary can predict the presence of an internal mass within the torsed adnexa.

Current Imaging of Prenatally Diagnosed Congenital Lung Lesions

Congenital lung lesions refer to a spectrum of pulmonary developmental anomalies including, but not limited to, bronchial atresia, congenital pulmonary airway malformation (formerly known as congenital cystic adenomatoid malformation) and bronchopulmonary sequestration. These anomalies comprise about 90% of the anomalies seen in clinical practice. The advent of prenatal sonography and, more recently, fetal magnetic resonance imaging has changed our understanding and practice in the evaluation of congenital lung lesions. Postnatal imaging using low-dose computed tomography angiography (CTA) is extremely useful as it may provide information essential for differential diagnosis by allowing multiplanar reconstructions of the airway, lung parenchyma, and vasculature. The use of iodine in CTA permits the application of low-dose radiation protocols in these young patients. The purpose of this article is to emphasize the technical factors that may optimize low-dose CTA evaluation of these lesions. We also provide a description of prenatal imaging findings and helpful diagnostic clues that may be useful for the characterization of the most commonly encountered prenatally diagnosed pulmonary developmental anomalies.

Characterizing degree of lung injury in pediatric acute respiratory distress syndrome.

Objective: Although all definitions of acute respiratory distress syndrome use some measure of hypoxemia, neither the Berlin definition nor recently proposed pediatric-specific definitions proposed by the Pediatric Acute Lung Injury Consensus Conference utilizing oxygenation index specify which PaO2/FIO2 or oxygenation index best categorizes lung injury. We aimed to identify variables associated with mortality and ventilator-free days at 28 days in a large cohort of children with acute respiratory distress syndrome. Design: Prospective, observational, single-center study. Setting: Tertiary care, university-affiliated PICU. Patients: Two-hundred eighty-three invasively ventilated children with the Berlin-defined acute respiratory distress syndrome. Interventions: None. Measurements and Main Results: Between July 1, 2011, and June 30, 2014, 283 children had acute respiratory distress syndrome with 37 deaths (13%) at the Children’s Hospital of Philadelphia. Neither initial PaO2/FO2 nor oxygenation index at time of meeting acute respiratory distress syndrome criteria discriminated mortality. However, 24 hours after, both PaO2/FIO2 and oxygenation index discriminated mortality (area under receiver operating characteristic curve, 0.68 [0.59–0.77] and 0.66 [0.57–0.75]; p < 0.001). PaO2/FIO2 at 24 hours categorized severity of lung injury, with increasing mortality rates of 5% (PaO2/FIO2, > 300), 8% (PaO2/FIO2, 201–300), 18% (PaO2/FIO2, 101–200), and 37% (PaO2/FIO2, ⩽ 100) across worsening Berlin categories. This trend with 24-hour PaO2/FIO2 was seen for ventilator-free days (22, 19, 14, and 0 ventilator-free days across worsening Berlin categories; p < 0.001) and duration of ventilation in survivors (6, 9, 13, and 24 d across categories; p < 0.001). Similar results were obtained with 24-hour oxygenation index. Conclusions: PaO2/FIO2 and oxygenation index 24 hours after meeting acute respiratory distress syndrome criteria accurately stratified outcomes in children. Initial values were not helpful for prognostication. Definitions of acute respiratory distress syndrome may benefit from addressing timing of oxygenation metrics to stratify disease severity.

Imaging of pediatric ovarian neoplasms

We review the clinical and imaging characteristics of the most common ovarian neoplasms in children and adolescents. Because of the widespread use of diagnostic imaging, incidental ovarian neoplasms might be encountered during the evaluation of abdominal pain, trauma or other indications and might pose a diagnostic dilemma. Conducting adequate imaging studies under these conditions is important, as management strategies differ according to the size and appearance of the lesion as well as the age of the patient. US dominates in gynecological imaging because of its excellent visualization, absence of ionizing radiation and sedation risks and comparatively low cost. For further examination of indeterminate lesions found using US, MRI is being used more progressively in this field, particularly for the evaluation of complex pelvic masses with the aim of distinguishing benign and malignant conditions and conditions requiring surgical intervention. CT is reserved primarily for tumor staging and follow-up and for emergency situations.

Value of 18F-fluoro-2-deoxy-d-glucose positron emission tomography/computed tomography scan versus diagnostic contrast computed tomography in initial staging of pediatric patients with lymphoma

Abstract Our objective was to evaluate the value of 18F-fluoro-2-deoxy-d-glucose (FDG) positron emission tomography/computed tomography (PET/CT) versus diagnostic contrast CT in the initial staging of pediatric Hodgkin disease (HD) and non-Hodgkin lymphoma (NHL). Thirty patients with HD and 21 patients with NHL were included in this retrospective study. On nodal lesion analysis, PET/CT detected 94.8% (HD) and 88.3% (NHL) of total lesions, respectively, in contrast to 82.6% (HD) and 69.1% (NHL), respectively, for diagnostic contrast CT. PET/CT also detected more extranodal lesions. On the patient level, PET/CT detected additional lesions in 50% of patients with HD and in 42.9% of patients with NHL. In contrast, diagnostic contrast CT detected additional lesions in 16.7% (HD) and 23.8% (NHL) of total cases. FDG PET/CT led to upstaging in seven cases of HD and seven cases of NHL, while diagnostic contrast CT upstaged two cases of NHL. We conclude that FDG PET/CT outperforms diagnostic contrast CT in the initial staging of pediatric patients with lymphoma.

Prevalence of stress reaction in the pars interarticularis in pediatric patients with new-onset lower back pain.

Background Spondylolysis is a defect of the pars interarticularis and is most commonly encountered in the lower lumbar spine of pediatric patients. Stress reaction of the pars interarticularis was regarded as an early stage of this pathological diagnosis in which no obvious anatomical change is detected. Both SPECT with 99mTc MDP and CT of the lumbar spine have been used to evaluate the patients with low back pain. The purpose of this study was to determine the prevalence of stress reaction in pars interarticularis in pediatric patients presenting new-onset back pain using both bone SPECT and CT. Patients and Methods The records and images of 63 consecutive pediatric patients (mean age, 13.2 years) with new onset of back pain, who received both 99mTc MDP bone scintigraphy with SPECT of the lumbar spine and lumbar thin-slice CT were retrospectively analyzed. The results of bone SPECT and CT were retrospectively compared. Results Among the cohort of 63 patients who had both bone SPECT and thin-slice CT of the lumbar spine, there was a total of 56 positive SPECT results indicating stress injuries in the pars interarticularis. Spondylolysis was shown on both bone SPECT and thin-slice CT in 45 patients. In 11 patients, stress reaction was shown. In 7 patients, no abnormality was detected by either SPECT or thin-slice CT. Conclusions Our result showed that a significant portion (19.6%, 11 of 56) of the stress injuries in the pars interarticularis will be CT-negative stress reaction in pediatric patients with new-onset back pain.

Müllerian Duct and Related Anomalies in Children and Adolescents

Although many Müllerian duct anomalies do not require treatment, surgical intervention is sometimes necessary to enable sexual activity or to preserve fertility. The identification of these anomalies is important for optimal clinical management or surgical treatment. Magnetic resonance (MR) imaging is a robust method for adequately evaluating and characterizing uterine and vaginal anomalies. The information provided by MR imaging allows for a more complete understanding of the malformation, facilitating management decisions and potentially changing the outcome. In this article, the embryology, classification, and MR imaging findings of Müllerian duct and related anomalies in children and adolescents are reviewed.

I-131 MIBG post-therapy scan is more sensitive than I-123 MIBG pretherapy scan in the evaluation of metastatic neuroblastoma.

ObjectiveIodine-123 metaiodobenzylguanidine (I-123 MIBG) scintigraphy is gradually replacing I-131 MIBG scans in the diagnostic workup of neuroblastoma. High-dose I-131 MIBG, however, is commonly used for subsequent therapy in patients with proven MIBG-avid lesions. The objective of this study was to compare the sensitivities of pretherapy I-123 MIBG and post-therapy I-131 MIBG scans for detecting metastatic lesions of neuroblastoma and determine the suitability of post-therapy scans for detecting new metastases. Materials and methodsPretherapy I-123 MIBG scans and post-therapy I-131 MIBG scans of 126 patients with neuroblastoma were analyzed retrospectively and the number of detected lesions was compared. ResultsIn 70 patients (55.6% cases), the pretherapy and post-therapy scans were concordant, showing similar MIBG-avid foci. In the remaining 56 patients (44.4% cases), the post-therapy I-131 MIBG scans revealed additional lesions (i.e. a total of 716 lesions) compared with pretherapy I-123 MIBG scans (only 532 lesions). All lesions detected on pretherapy I-123 MIBG scans were revisualized on the post-therapy I-131 MIBG scans, the latter also revealing 184 new MIBG-avid lesions. ConclusionPost-therapy I-131 MIBG scans demonstrate new lesions in almost half of the patients when compared with pretherapy I-123 MIBG scans and therefore are essential to delineate the disease extent.

Contemporary pediatric gynecologic imaging.

Ultrasound is the primary screening modality for the evaluation of pediatric gynecologic maladies. Magnetic resonance imaging (MRI) is used progressively more in this field, particularly for the evaluation of complex pelvic masses and congenital anomalies. However, ultrasound remains the preferred modality due to the excellent visualization, the dynamic nature of the examination modality, lack of ionizing radiation and sedation risks, and comparatively lower cost. MRI is generally a second-line examination and is preferred over computed tomography as it does not involve the use of ionizing radiation. Additionally, visualization of the female reproductive system anatomy with MRI is superior to computed tomography, the latter being reserved generally for tumor staging. Fluoroscopic examinations and abdominal radiographs can provide additional information that may support a diagnosis.

Irinotecan–temozolomide with temsirolimus or dinutuximab in children with refractory or relapsed neuroblastoma (COG ANBL1221): an open-label, randomised, phase 2 trial

BACKGROUND Outcomes for children with relapsed and refractory neuroblastoma are dismal. The combination of irinotecan and temozolomide has activity in these patients, and its acceptable toxicity profile makes it an excellent backbone for study of new agents. We aimed to test the addition of temsirolimus or dinutuximab to irinotecan-temozolomide in patients with relapsed or refractory neuroblastoma. METHODS For this open-label, randomised, phase 2 selection design trial of the Childrens Oncology Group (COG; ANBL1221), patients had to have histological verification of neuroblastoma or ganglioneuroblastoma at diagnosis or have tumour cells in bone marrow with increased urinary catecholamine concentrations at diagnosis. Patients of any age were eligible at first designation of relapse or progression, or first designation of refractory disease, provided organ function requirements were met. Patients previously treated for refractory or relapsed disease were ineligible. Computer-based randomisation with sequence generation defined by permuted block randomisation (block size two) was used to randomly assign patients (1:1) to irinotecan and temozolomide plus either temsirolimus or dinutuximab, stratified by disease category, previous exposure to anti-GD2 antibody therapy, and tumour MYCN amplification status. Patients in both groups received oral temozolomide (100 mg/m2 per dose) and intravenous irinotecan (50 mg/m2 per dose) on days 1-5 of 21-day cycles. Patients in the temsirolimus group also received intravenous temsirolimus (35 mg/m2 per dose) on days 1 and 8, whereas those in the dinutuximab group received intravenous dinutuximab (17·5 mg/m2 per day or 25 mg/m2 per day) on days 2-5 plus granulocyte macrophage colony-stimulating factor (250 μg/m2 per dose) subcutaneously on days 6-12. Patients were given up to a maximum of 17 cycles of treatment. The primary endpoint was the proportion of patients achieving an objective (complete or partial) response by central review after six cycles of treatment, analysed by intention to treat. Patients, families, and those administering treatment were aware of group assignment. This study is registered with ClinicalTrials.gov, number NCT01767194, and follow-up of the initial cohort is ongoing. FINDINGS Between Feb 22, 2013, and March 23, 2015, 36 patients from 27 COG member institutions were enrolled on this groupwide study. One patient was ineligible (alanine aminotransferase concentration was above the required range). Of the remaining 35 patients, 18 were randomly assigned to irinotecan-temozolomide-temsirolimus and 17 to irinotecan-temozolomide-dinutuximab. Median follow-up was 1·26 years (IQR 0·68-1·61) among all eligible participants. Of the 18 patients assigned to irinotecan-temozolomide-temsirolimus, one patient (6%; 95% CI 0·0-16·1) achieved a partial response. Of the 17 patients assigned to irinotecan-temozolomide-dinutuximab, nine (53%; 95% CI 29·2-76·7) had objective responses, including four partial responses and five complete responses. The most common grade 3 or worse adverse events in the temsirolimus group were neutropenia (eight [44%] of 18 patients), anaemia (six [33%]), thrombocytopenia (five [28%]), increased alanine aminotransferase (five [28%]), and hypokalaemia (four [22%]). One of the 17 patients assigned to the dinutuximab group refused treatment after randomisation; the most common grade 3 or worse adverse events in the remaining 16 patients evaluable for safety were pain (seven [44%] of 16), hypokalaemia (six [38%]), neutropenia (four [25%]), thrombocytopenia (four [25%]), anaemia (four [25%]), fever and infection (four [25%]), and hypoxia (four [25%]); one patient had grade 4 hypoxia related to therapy that met protocol-defined criteria for unacceptable toxicity. No deaths attributed to protocol therapy occurred. INTERPRETATION Irinotecan-temozolomide-dinutuximab met protocol-defined criteria for selection as the combination meriting further study whereas irinotecan-temozolomide-temsirolimus did not. Irinotecan-temozolomide-dinutuximab shows notable anti-tumour activity in patients with relapsed or refractory neuroblastoma. Further evaluation of biomarkers in a larger cohort of patients might identify those most likely to respond to this chemoimmunotherapeutic regimen. FUNDING National Cancer Institute.