Understanding the diagnosis of superior mesenteric artery syndrome: analysis of the location of duodenal impression on upper gastrointestinal studies.

BACKGROUND: Upper gastrointestinal (GI) contrast studies are frequently requested to aid superior mesenteric artery syndrome diagnosis, a rare entity. Compression of the third duodenal part is expected to be mid-to-left of the midline where the superior mesenteric artery arises from the aorta; however, a duodenal impression to the right of the midline due to normal anatomic impression by the inferior vena cava (IVC) is often encountered and frequently misdiagnosed. OBJECTIVE: The purpose of this study was to determine the frequencies of (1) normal right-of-midline duodenal impressions and (2) mid-to-left of midline compressions in upper GI studies in a tertiary pediatric referral center. MATERIALS AND METHODS: All upper GI studies performed at our institution over 2 years were retrospectively evaluated to determine whether the duodenum had vertical duodenal impression to the right of the vertebral midline, mid-to-left of the vertebral midline, or no identifiable duodenal impression at all. RESULTS: In total, 538 upper GI studies were included in this analysis. A total of 275 male and 247 female patients between 0 and 17 years of age (median: 6 years, range: 1 month-17 years) were included. Of 538 total upper GI studies, there were 240 studies (44.6%) with a right-of-midline impression. There were only 10 studies (1.9%) with a mid-to-left of midline compression, and 9/10 also showed a concurrent right-sided impression sign. CONCLUSION: Right-of-midline duodenal impression is a normal anatomic finding caused by the IVC and should not be confused with superior mesenteric artery syndrome. In the presence of an appropriate clinical context, proximal duodenal dilation, "to-and-fro" motion of contrast, and duodenal impression at mid-to-left of midline, a diagnosis of superior mesenteric artery syndrome should be considered.

Desmoplastic Small Round Cell Tumor With Ascending Intraspinal Metastasis at Recurrence: Case Report and Review of the Literature.

BACKGROUND: Desmoplastic small round cell tumor (DSRCT) is a rare and aggressive malignancy commonly involving the abdomen and/or pelvic peritoneum. Despite aggressive therapy, the prognosis remains poor. Central nervous system relapse is rare in abdominal/pelvic primary DSRCT. OBSERVATION: We report a case of a 10-year-old female with a large pelvic DSRCT and involvement of the rectosigmoid colon and liver. Following treatment with chemotherapy, and cytoreductive surgery with hyperthermic intraperitoneal chemotherapy an initial response was noted. With progressive lower limb weakness, recurrence with perineural invasion in the lumbosacral nerve root involving the conus was noted 2.5 years from diagnosis. Cerebrospinal fluid showed tumor cells with a molecular confirmation. CONCLUSIONS: Perineural invasion and ascending paralysis secondary to primary abdominal DSRCT has not been previously reported to our knowledge. We recommend a high index of suspicion for early and accurate diagnosis of this rare presentation.

Left-sided Poland's syndrome associated with dextrocardia.

Poland's syndrome is a rare congenital anomaly accompanied by the absence of the pectoralis major, and the ipsilateral upper-limb and chest wall deformities. Hypoplasia of the breast, agenesis of the ipsilateral rib cartilage, athelia, and ipsilateral developmental finger anomalies such as syndactyly can also be seen. In the literature, only 56 cases of dextrocardia and left-sided Poland's syndrome have been described. Herein, a case of left-sided Poland's syndrome coexisting with dextrocardia and nasal hemangioma was presented.

Evaluation of Ovarian Vascularity in Children by Using the "Superb Microvascular Imaging" Ultrasound Technique in Comparison With Conventional Doppler Ultrasound Techniques.

OBJECTIVES: The aim of this study was to assess the feasibility of the novel "superb microvascular imaging" (SMI) Doppler ultrasound technique to detect ovarian vascularity in healthy children and to compare it with the conventional Doppler ultrasound techniques. METHODS: This prospective study included 140 girls, aged 3 to 18 years, for which an abdominal ultrasound study was requested in the pediatrics outpatient clinics for suspicion of nonovarian pathologies. Nineteen individuals were excluded from the study due to incomplete ultrasound scanning. For the remaining 121 cases, gray scale ultrasound evaluation was performed followed by color Doppler imaging, power Doppler imaging, advanced dynamic flow, color SMI, and monochrome SMI to detect the presence and degree of vascularity in the ovaries. Ultrasound scanning was performed for all participants by the same doctor by using curvilinear probe via the suprapubic approach, and the images were independently evaluated by 3 different observers. The images were assessed with regard to the degree of vascularity and the presence of artifacts. RESULTS: A total of 146 ovaries (42 right, 54 left, and 25 bilateral) were examined by sonography. The mean interrater agreement regarding the detection of vascularity was moderate for the color Doppler imaging, power Doppler imaging, advanced dynamic flow, and monochrome SMI techniques (κ = 0.514-0.551) and substantial for the color SMI technique (κ = 0.636) (P < .001). The techniques were found to be statistically significant for detecting vascularity: monochrome SMI > color SMI > power Doppler imaging > color Doppler imaging > advanced dynamic flow (P < .001). CONCLUSION: SMI is a promising tool that can detect ovarian vascularity more effectively than the conventional Doppler ultrasound techniques.

Biliary Rhabdomyosarcoma in an Infant Male With Neurofibromatosis Type 1.

Different types of malignancies can be seen in patients with neurofibromatosis type 1 (NF-1). Herein we present a rare combination of NF-1 and biliary rhabdomyosarcoma in a male infant. An 11-month-old boy, who was recently diagnosed with NF-1, presented to the outpatient clinic with a 3-month history of prolonged jaundice, and failure to thrive. Clinical examination showed >20 café au let spots distributed mainly over the abdominal trunk. Hepatomegaly (4 cm below the costal margin) was additionally observed. His father was diagnosed with NF-1. Radiologic imaging studies showed a 6×5×5 cm in diameter cystic mass with multiple septations in the segment 4A of the liver. Surgical excision of the left hepatic lobe followed by hepatojejunostomy was further performed. Histopathology examination showed embryonal type rhabdomyosarcoma originating from the biliary duct. Chemotherapy regimen consisting of cyclophosphamide, actinomycin D, and vincristine, and radiotherapy were then initiated. This treatment led to a significant improvement in the patient's clinical status, and radiologic finding portrayed attainment of complete resolution. He is still in complete remission without any sequelae for 8 years.

Seeing the Unseen: Evaluating Testicular Vascularity in Neonates by Using the Superb Microvascular Imaging Ultrasound Technique.

OBJECTIVES: To evaluate testicular vascularity in neonates by using the novel Superb Microvascular Imaging (SMI; Toshiba Medical Systems Corporation, Tokyo, Japan) ultrasound (US) technique and to compare this technique to conventional Doppler US techniques. METHODS: A total of 140 healthy neonates between 2 and 60 days old who were admitted for routine screening were included in the study. However, in 9 cases, the Doppler study could not be completed. In the remaining 131 cases, color Doppler imaging (CDI), power Doppler imaging (PDI), Advanced Dynamic Flow (ADF; Toshiba Medical Systems), and monochrome SMI (mSMI) techniques were performed on a single representative testicle. Real-time cine images were captured for 5 to 10 seconds for each technique by using a high-frequency (7.2-14-MHz) linear transducer. The images were evaluated by 3 observers using a grading system to quantify the degree of vascularity and artifact interference. RESULTS: A total of 131 testicles (74 right and 57 left) were evaluated, and vascularity was shown in 110 (84%) testicles with CDI, 109 (83%) with PDI, 94 (72%) with ADF, and 128 (98%) with mSMI by the consensus of all observers. The Doppler techniques were ranked from highest to lowest in the following order based on the degree of vascularity detected: mSMI > PDI > CDI > ADF (P < .001). Based on the presence of artifacts, the Doppler techniques were ranked as follows from high to low: ADF > CDI > PDI > mSMI (P < .001). CONCLUSIONS: Superb Microvascular Imaging is a promising US technique that appears to detect testis vascularity better than conventional Doppler US techniques; however, further research is needed to support the results of this study.

Is real-time elastography helpful to differentiate acute from subacute deep venous thrombosis? A preliminary study.

PURPOSE: To detect and characterize changes in stiffness of thrombus in patients with acute and subacute deep venous thrombosis (DVT) by using real-time elastography (RTE). METHODS: Fifty-eight patients with acute or subacute DVT were prospectively evaluated by B-mode sonography (US), color Doppler US (CDUS), and RTE. Two radiologists evaluated the thrombus echogenicity, compressibility, and recanalization of the affected vein, and thrombus stiffness in consensus. The thrombi were classified into 3 groups as soft, intermediate, and hard on RTE images. RESULTS: The final study group consisted of 30 patients with acute DVT, among whom 10 were women (33%), and 19 patients with subacute DVT, among whom 6 were women (32%). The presence of hypoechoic thrombus, incompressible vein, and absence of recanalization on US and CDUS were significantly associated with acute DVT (P < .001 for all variables). The differences in elasticity pattern of the thrombi between acute and subacute DVT were not significant (P = .202). CONCLUSION: Venous thrombus hardens with age; however, elastography pattern on RTE, in its present form, may not be able to differentiate acute DVT from subacute DVT.

The utility of ADC measurement techniques for differentiation of low- and high-grade clear cell RCC.

PURPOSE: To evaluate the diffusion properties of clear cell renal cell carcinoma (ccRCC) on magnetic resonance imaging (MRI) concerning their Fuhrman nuclear grades and sizes, and to compare the diagnostic performance of two ROI placement techniques for apparent diffusion coefficient (ADC) measurement (entire mass vs. only the darkest region of the mass). MATERIAL AND METHODS: Fifty-one ccRCC were enrolled in the study and grouped into low-grade ccRCC (Fuhrman grade 1 and 2, n = 37) and high-grade ccRCC (Fuhrman grade 3 and 4, n = 14). Selective ADC (Sel-ADC) measurement was performed by placing a circular ROI that included the darkest region of the tumour on ADC map images. Extensive ADC (Ext-ADC) measurement was performed by drawing an ROI that covered the entire tumour. RESULTS: The Sel-ADC value was lower in high-grade ccRCC (p = 0.019), whereas the Ext-ADC value did not show a statistically significant difference (p = 0.42). Sel-ADC value of a ≤ 1.405 mm2/s has a sensitivity, specificity, positive predictive value, negative predictive value, and accuracy value of 78.6, 72.2, 73.87, 77.13, and 75.4, respectively, to differentiate high-grade from low-grade ccRCC. The size and Fuhrman grade of the ccRCC were inversely correlated with the Sel-ADC value; however, the correlations were weak (r = -0.322, p = 0.021 and r = -0.376, p = 0.006, respectively). There was no difference between ADC values of small (≤ 4 cm) and large (> 4 cm) ccRCCs. CONCLUSIONS: The ADC value of the darkest region in solid part of the ccRCC may play a role in predicting the nuclear grade of ccRCC.

Evaluation of aortic intima-media thickness in newborns with Down syndrome.

BACKGROUND: Health care programs for children with Down syndrome (DS) help improve the overall outcome and quality of life of these children. It is therefore very important to focus on the most common and serious problems of these patients, such as congenital heart defects and cardiac problems, and to keep medical guidelines updated with regard to these problems. OBJECTIVES: The aim of this study was to evaluate aortic intima-media thickness (aIMT), lipid profiles and blood pressure in DS patients in comparison with a control group of ageand gender-matched neonates without DS. MATERIAL AND METHODS: Serum concentrations of lipids (total cholesterol, LDL-cholesterol, HDL-cholesterol, and triglyceride), blood pressure and abdominal aIMT were measured in newborns with DS, and compared with the measurements from ageand gender-matched newborns without DS. RESULTS: No statistically significant differences between the 2 groups of newborns were detected with respect to aIMT, lipid levels or blood pressure. CONCLUSIONS: This study represents the first investigation of aIMT - one of the most important indicators of atherosclerosis - in DS patients, but neither a significantly increased aIMT, nor any significant changes in lipid profiles or blood pressure were detected in this group of patients. Whether aIMT differs according to the type of congenital heart defect (such as atrial septal defect, ventricular septal defect, patent ductus arteriosus, and atrioventricular septal defect) among DS patients remains to be determined in future studies.

Pediatric Hepatobiliary Neoplasms: An Overview and Update.

Recent developments regarding the treatment of pediatric liver tumors have significantly improved patient care. Stimulated by collaboration between international pediatric groups, advances have been made in surgical techniques, transplantation options, and chemotherapy schemas. In light of this progress, clear understanding of the state-of-the-art imaging evaluation of hepatobiliary tumors has become even more integral to the effective management of children with hepatic neoplasms. The unique imaging features of hepatic neoplasms in the pediatric population, when coupled with supportive demographic data and laboratory findings, can lead to accurate diagnosis and proper treatment of hepatobiliary tumors.

Pediatric Urinary System Neoplasms: An Overview and Update.

Pediatric urinary system neoplasms are a diverse group of tumors that frequently overlap in their clinical and radiologic features. By contrast, the histopathologic classification and treatment of these entities have become increasingly refined, resulting in improved outcomes, with the overall survival of Wilms tumors now exceeding 90%. Significantly, many contemporary protocols rely on radiologic diagnosis in the absence of tissue confirmation. This review article provides up-to-date clinical, epidemiologic, and imaging findings of pediatric urinary system neoplasms and their mimics frequently encountered in daily clinical practice.

Neurological Manifestations in Familial Mediterranean Fever: Results of 22 Children from a Reference Center in Kayseri, an Urban Area in Central Anatolia, Turkey.

Background Familial Mediterranean fever (FMF) is an inherited inflammatory disorder characterized by attacks of fever with polyserositis. Objective The purpose of this study was to evaluate pediatric patients with FMF who had central nervous system (CNS) findings. Materials and Methods Our medical records database for 2003 to 2014 was screened retrospectively. In total, 104 patients with FMF were identified, 22 of whom had undergone neurological examination for CNS symptoms. Results Neurological findings included headache in 16 patients (72.7%), epilepsy in 6 patients (27.3%), pseudotumor cerebri in 2 patients (9.1%), tremor in 2 patients (9.1%), and multiple sclerosis in 1 patient (4.5%). The most common MEFV gene mutation was homozygous M694V (40.9%). Conclusions Patients with FMF can present with various CNS manifestations. Further studies that include large populations are needed to elucidate the neurological manifestations of FMF.

Ileus and Intra-Abdominal Hypertension due to Phosphate- Containing Enema.

The most well known complications of fleet enema solution are cardiac insufficiency, renal failure, water-electrolyte imbalance, and ileus. A 7-y-old girl with phenylketonuria and long-term constipation was admitted to the emergency department with symptoms of seizure, vomiting and abdominal distention. Laboratory results revealed hypocalcemia and hyperphosphatemia. ECG findings showed normal sinus rhythm and prolonged QT interval. At the follow-up, the patient's abdominal distention was markedly increased. She was evaluated for a surgical pathology and, this was considered unlikely. Intra-abdominal pressure (IAP) was 19.5 mmHg. Gastric and colonic decompression, intravenous 10 % calcium gluconate were applied. After 2 d of treatment, the patient's condition became stable, and serum calcium and phosporus normalized to 8.8 mg/dl and 4.0 mg/dl, respectively. Abdominal distention regressed and the last IAP measurement was 3.5 mmHg. Thus, IAP measurements are a useful adjunct in clinical follow-up of patients with progressive abdominal distention due to phosphate enema use.

Imaging of childhood angiomatoid fibrous histiocytoma with pathological correlation.

BACKGROUND: Angiomatoid fibrous histiocytoma is a rare soft-tissue tumor that more often affects children and young adults. There is little information available regarding the imaging appearance of angiomatoid fibrous histiocytoma in children. OBJECTIVE: To describe the ultrasonographic (US) and magnetic resonance (MR) imaging findings of angiomatoid fibrous histiocytoma in children. MATERIALS AND METHODS: A retrospective analysis was done of US and MR imaging findings in children with angiomatoid fibrous histiocytoma. Clinical findings and histopathology with molecular analysis results were also collected. RESULTS: There were 7 children with angiomatoid fibrous histiocytoma with a median age of 6 years (age range: 16 months-14 years). Patients presented clinically with a soft-tissue mass in the extremities or in the trunk. Four children had anemia, and three of them had additional systemic symptoms. Two patients had US and three had MR imaging while the remaining two had both. Lesion size ranged from 1.3 cm to 7.2 cm. In four patients, angiomatoid fibrous histiocytoma presented as a nonspecific predominantly solid mass. The other three patients had a combination of the following imaging findings: intralesional blood-filled cystic spaces with fluid-fluid levels, enhancing fibrous pseudocapsule and hemosiderin deposition. These findings correlated well with histopathology. CONCLUSION: The imaging detection of intralesional blood-filled cystic spaces with fluid-fluid levels, enhancing fibrous pseudocapsule and hemosiderin deposition in a soft-tissue tumor in a child may suggest the diagnosis of angiomatoid fibrous histiocytoma. A history of systemic symptoms and anemia in the presence of a soft-tissue mass may also be a clue for the diagnosis of angiomatoid fibrous histiocytoma.

Quantitative assessment of hepatic blood flow in the diagnosis and management of necrotizing enterocolitis.

BACKGROUND: Necrotizing enterocolitis (NEC) is the most important gastrointestinal emergency in the neonatal period and early detection is very important for its management. Bowel ischemia-hypoperfusion is one of the main etiological factors. In the literature, a few studies have focused on arterial Doppler ultrasonography (DUS) features of splanchnic arteries; however, their clinical implications are not clear. OBJECTIVE: In this study, we aimed to quantitatively evaluate the blood flow features in the hepatic portal vein (PV) and hepatic veins (HVs) by using DUS in newborns with NEC. Patient-Method: Enrolled subjects were divided into two groups as patient (suspected/confirmed NEC, n = 24), and control group (n = 25). Daily serial DUS examinations were performed after the onset of the suspicion of NEC and continued until the initial day of the enteral feeding. Portal blood flow (PBF) and "hepatic blood flow ratio" (RHBF) were calculated manually by using DUS findings. Two groups were compared with respect to their PBF and RoHBF values. RESULTS: PBF and RHBF levels were significantly lower in patient group than those in control group. Clinical improvement in patients with NEC was associated with improvement in the PBF and RHBF. Cut-off level of the RHBF for the diagnosis of NEC was 0.66. CONCLUSION: DUS seems to be useful for the diagnosis and follow-up of NEC by providing quantitative information on liver blood flow. Daily measurements of the PBF and RoHBF in newborns with NEC may be beneficial to make the decision of starting enteral feeding.

Prospective evaluation of the impact of sonography on the management and surgical intervention of neonates with necrotizing enterocolitis.

BACKGROUND/AIM: Established indications for surgery in necrotizing enterocolitis (NEC) are pneumoperitoneum and failure to improve or clinical deterioration with medical treatment alone. It has been proposed that infants with intestinal necrosis may benefit from surgery in the absence of one of these indications yet the diagnosis of definitive intestinal necrosis is challenging. Recent data suggest that abdominal ultrasound (US) examination focused on the gastrointestinal tract and the peritoneal cavity may be of utility in this regard. The aim of this study was to evaluate the ability of abdominal US to detect intestinal necrosis in infants with radiographically confirmed NEC. METHODS: Twenty-six consecutive infants with Bell stage II or III NEC were prospectively included in the study between September 2013 and July 2014. Infants with a pre-existing indication for surgery were excluded. At least one abdominal US examination was performed in each patient using a standardized previously described method. Surgery was performed at the discretion of the attending surgeon based on clinical and imaging findings. Clinical, radiographic, US, and intra-operative data were recorded to allow comparison between US findings, surgical findings and outcome. RESULTS: US demonstrated signs of intestinal necrosis in 5 of the 26 patients. All of these five had laparotomy. Intestinal necrosis requiring resection was confirmed in four and the other was found to have NEC but no necrosis was identified. In 21 patients US did not suggest intestinal necrosis. Of these, only one had surgery in whom NEC but no necrosis was identified. The remaining 20 responded to medical treatment for NEC and were assumed not to have had intestinal necrosis based on improvement without surgical intervention. The sensitivity, specificity, positive predictive value and negative predictive values of US for the detection of bowel necrosis were calculated as 100, 95.4, 80.0, and 100%, respectively. CONCLUSION: Our prospective findings suggest that abdominal US can identify those infants with NEC who may need surgery by detecting bowel necrosis (prior to the development of perforation or medical deterioration) with high sensitivity and specificity. Early surgical intervention in the clinical pathway of NEC may lead to improved outcomes.

Unusual course of congenital hypothyroidism and route of the L-thyroxine treatment in a preterm newborn.

Congenital hypothyroidism (CH) is the most common endocrine pathology in neonates. Inappropriate treatment of CH is complicated by irreversible brain damage or low IQ score. Hormone replacement therapy with L-thyroxine (L-T4) is sufficient for a very large proportion of patients. However, during treatment, the patient needs to be carefully monitored for presence of factors which might affect the absorption or bio-availability of the drug as well as its dose. Herein, we report a preterm newborn with CH who presented with gastrointestinal problems mimicking necrotizing enterocolitis. The clinical course was also complicated by cholestasis. The L-T4 replacement treatment was switched from oral route to parenteral. After resolution of the cholestasis, L-T4 treatment was continued successfully by the oral route.

The comparison of thyroxine versus thyroxine plus oral iodine in the treatment of congenital hypothyroidism due to iodine deficiency.

AIM: Iodine deficiency is one of the most important causes of congenital hypothyroidism. In addition to thyroid hormone replacement, iodine supplementation is also given to newborns with congenital hypothyroidism due to iodine deficiency. We aimed to determine whether it is beneficial to administer iodine supplementation in addition to the L-thyroxine (L-T4) treatment of newborns with congenital hypothyroidism due to iodine deficiency. MATERIALS AND METHODS: Of 51 newborns, 26 who were diagnosed with congenital hypothyroidism due to iodine deficiency were treated with L-T4. The remaining 25 cases were given L-T4 plus 100 µg/day of oral iodine. Free triiodothyronine (fT3), free thyroxine (fT4), thyroid-stimulating hormone (TSH), thyroglobulin (TG), thyroid volume, urine iodine and breast milk iodine levels were measured in the first and third months of treatment, and the data were compared between the two groups. RESULTS: First- and third-month values of fT3, fT4, TSH, TG and thyroid volume for both groups were statistically similar. There was no significant difference between the two groups in respect to falling levels of fT3 and TSH, the rate of increase of fT4 levels or the shrinkage rate of thyroid volume. CONCLUSION: In this study, the addition of oral iodine to L-T4 treatment provided no benefit compared to treatment with L-T4 alone.