TRPV6 gene mutation associated with giant pancreatic pseudocyst with mediastinal extension in a pediatric patient: Lessons learned.

Pancreatic pseudocyst is a common complication secondary to acute or chronic pancreatitis. The extension of a pseudocyst into the mediastinum is extremely rare, with notably few reported cases in the pediatric age group found in the literature. This report presents a giant pancreatic pseudocyst with mediastinal extension that developed secondary to TRPV6 gene mutation in a 12-year-old male patient with no previously known disease and normal laboratory values.

Shear wave elastography evaluation in predicting the success of ultrasound-guided saline enema hydrostatic reduction technique in ileocolic intussusception.

BACKGROUND: Intussusception is the invagination of the proximal intestinal segment into the distal portion. Reduction procedures with fluid or air have been used as the primary treatment of choice in clinically stable children. PURPOSE: To evaluate the role of intestinal wall elasticity measurements by shear wave elastography (SWE) to predict the success of ultrasound-guided saline enema (USGSE) reduction. METHODS: USGSE was performed, if not contraindicated otherwise, after the diagnosis of ileocecal intussusception via the ultrasound (US). The length and diameter of the intussusception and the median stiffness of the intestine were measured before USGSE. RESULTS: Seventeen children were diagnosed with ileocolic intussusception via grayscale US assessment. Two children whose SWE images became artifacts due to inadaptability were excluded from the study. Thus, the study involved 15 patients (9 boys, 6 girls; age range = 11-48 months). There was no statistically significant association between age and median stiffness measurement in kilopascal (kPa). (P > 0.05). A moderate positive correlation was observed between the median stiffness measurement (kPa) and the length of intussusception (r = 0.547; P = 0.035). There was no statistically significant relationship between median stiffness measurement (kPa) and short-axis diameter of intussusception (P > 0.05). CONCLUSIONS: Stiffness assessment of the intestinal wall in ileocolic intussusception during the US examination, which is the gold standard in the intussusception assessment, can be used as a new criterion for predicting the performance of the USGSE technique and might be useful in making decisions regarding the clinical management of ileocolic intussusception.

Posterior fossa horns; a new calvarial finding of mucopolysaccharidoses with well-known cranial MRI features

Background/aim: Mucopolysaccharidoses (MPS) are a group of hereditary metabolic diseases. The aim of this study was to share the previously unreported calvarial finding of internal hypertrophy of the occipitomastoid sutures (IHOMS) together with some other well-known cranial MRI findings in this patient series. Materials and methods: A retrospective evaluation was conducted of 80 cranial MRIs of patients who had been diagnosed and followed up with MPS from 2008 to 2019 in our center. Of these patients, 11 had Hurler, 14 had Hunter, 24 had Sanfilippo, 15 had Morquio, 14 had Maroteaux­Lamy, and 2 had Sly disease. The cranial MRIs were assessed in two main groups as parenchymal intradural cranial MRI findings and extradural calvarial findings. Results: The most common parenchymal intradural cranial MRI findings were white matter signal alterations (n = 51, 63%) and perivascular space enlargements (n = 39, 48%). The most common extradural calvarial findings were J-shaped sella (n = 45, 56%) and tympanic effusion (n = 44, 55%). Although IHOMS was defined in a relatively small number of the patients (n = 12, 15%), the prevalence rate was high in MPS type I (n = 6, 54%). Conclusion: The abnormal cranial MRI findings of the MPS patients, including the newly identified IHOMS, may provide diagnostic clues to differentiate the type of the disease in radiological imaging.

Hepatic Complications of Umbilical Venous Catheters in the Neonatal Period: The Ultrasound Spectrum.

OBJECTIVES: Umbilical venous catheterization is commonly used in the neonatal period; however, it has some complications. In this study, we evaluated neonates who underwent umbilical venous catheterization and developed hepatic complications. Furthermore, we aimed to define all of the possible lesions and to clarify the imaging findings of umbilical venous catheter-induced hepatic injury. METHODS: Two hundred forty-four neonates who underwent umbilical venous catheterization between March 2013 and September 2015 in a single tertiary care referral center were included in this study. To determine whether they had any hepatic complications, all patients underwent abdominal grayscale and Doppler ultrasound examinations, and their clinical data were recorded. RESULTS: The frequency of liver-related complications from umbilical venous catheterization was 33.6% (82 of 244). Air in the portal venous system was the most frequent complication (20.1% [49 of 244]). Left portal venous thrombosis was noted in 6.1% (15 of 244). Parenchymal lesions in the liver related to umbilical venous catheterization were seen in 7.4% of patients (18 of 244) as follows: single nodular echogenic lesions (4.1% [10 of 244]), branching small nodular echogenic lesions (2.1% [5 of 244]), and large irregular heterogeneous lesions with laceration and perihepatic fluid (1.2% [3 of 244]). There was no statistical significance for any type of complication according to the gestational age (P > .05). CONCLUSIONS: Hepatic complications due to umbilical venous catheters are not uncommon in the neonatal period. Ultrasound is the best imaging modality for confirming the diagnosis and for follow-up.

Diaphragmatic Mesothelial Cyst: Radiological Appearance and Follow-Up Results During Childhood.

ABSTRACT: To identify radiological findings of diaphragmatic mesothelial cysts (DMC) in the pediatric age group and to assess follow-up outcomes.In this study, 27 pediatric age group patients were evaluated with ultrasonography (US), computed tomography (CT), or magnetic resonance imaging due to various clinical indications and diagnosed with DMC from May 2014 to September 2018. Age, sex, imaging indications and DMC localization, volumes in the first diagnosis, and follow-ups were retrospectively evaluated. Descriptive statistics were used for age, sex, imaging indications, and volume are presented as numbers and percentages.Ages range from 5 months to 13 years. Nine girls and 18 boys included in this study. The most common imaging indications were abdominal pain, diarrhea, and obesity. The mean volume of DMC was at first 2.62 and 2.45 mL during the follow-ups. There was volume reduction in 24 cases, and no change in 3 cases. Mean follow-up duration was 22.4 months. The US imaging findings were similar for all cases, bilobular cystic lesion with fat indentation between the cyst and liver parenchyma.The typical localization and lateral fat sign are useful in differential diagnosis of DMC from cystic lesions of liver. The US is a very effective and beneficial radiological method for diagnosis and follow-up. Routine clinical and sonographic follow-ups may be sufficient for asymptomatic patients with stable cyst volume.

Optic Canal Diameters According to Age in the Pediatric Population.

PURPOSE: To determine the normal range of optic canal diameters in the Turkish pediatric population. METHODS: Brain, orbital, and maxillofacial computed tomography examinations were evaluated retrospectively. Children with cranial bone disorders affecting the bone structure of the optic canal were excluded from the study. Oblique axial and oblique sagittal multiplanar reformatted images were created in accordance with the axis of the optic canal on both sides, and measurements were taken from the shortest transverse and craniocaudal diameters of the optic canal in these images. RESULTS: Two hundred computed tomography examinations were evaluated. One hundred two of the patients were female and the rest were male. Patient ages ranged from 1 to 211 months (mean ± standard deviation: 86.42 ± 65.39 months). There was no significant difference between the transverse and craniocaudal optic canal diameters between sexes (P > .05). Therefore, the analyses were reevaluated in the entire patient series, regardless of sex. No significant correlation was found in the correlation test performed between optic canal diameters according to the age of the patients. No statistically significant difference was observed between the right and left optic canal diameters. CONCLUSIONS: The determination of normal values of tissues, structures, and organs that differs with age has an important role in pediatric radiology. The authors believe that the determination of normal optic canal diameters according to certain age groups will meet the needs of daily practice. [J Pediatr Ophthalmol Strabismus. 2021;58(5):319-323.].

Congenital cricopharyngeal achalasia: a rare cause of dysphagia in an infant.

Dysphagia secondary to congenital cricopharyngeal achalasia (CCA) is a rare condition in pediatric patients. We report a case of CCA in a 10-month-old boy presented with dysphagia, choking and nasal reflux. The diagnosis was made by barium studies. The patient was successfully treated by cricopharyngeal myotomy.

Relation of behavior problems with findings of cranial diffusion tensor MRI and MR spectroscopy in autistic children.

PURPOSE: To investigate any relation of behavior problems with cranial Diffusion Tensor Imaging (DTI) and Magnetic Resonance Spectroscopy (MRS) findings in autism spectrum disorders. MATERIALS AND METHODS: A total of 20 males children (12 autistic patients and 8 healthy controls) was examined by cranial DTI and MRS. The Aberrant Behavior Checklist (ABC) was used to calculate the irritability, lethargy-social withdrawal, stereotypic behavior, hyperactivity, and speech disorder scores for each patient. The results of MRS and DTI were evaluated together with the ABC scores. RESULTS: Fractional anisotropy (FA) values demonstrated significant decreases in the left frontoparietal white matter, anterior limb of the right internal capsule, and left middle cerebellar peduncle as the behavior problem scores elevated (P < 0.05). With the exception of social withdrawal, as the behavior problem scores increased, metabolite levels increased, as well. CONCLUSION: The positive correlation between the MRS findings, behavior problem scores, and metabolite levels suggests the presence of a dysfunction leading to hypo and hyper neuronal function in various locations. Reduced FA values in DTI and negative correlation of behavior problems with FA values in the contralateral hemisphere, may indicate reduced myelination and abnormal axonal organization.

Comparing brain magnetic resonance spectroscopy findings of pediatric treatment-naive obsessive-compulsive disorder patients with healthy controls.

OBJECTIVE: It is believed that biochemical alterations in different brain regions are involved in the pathophysiology of obsessive-compulsive disorder (OCD) in children and adolescents. The aim of this study was to explore possible metabolic variations between pediatric OCD cases and healthy controls in brain regions which were implicated in OCD pathophysiology. METHOD: Children and adolescents between 8 and 16 years of age with OCD (n:15) and case matched healthy controls (n:15) were recruited for the study. After detailed clinical and neuropsychological evaluations, all subjects underwent the multiregional magnetic resonance spectroscopy (MRS) procedure with a long echo time (TE:135). RESULTS: Significantly lower n-acetylaspartate (NAA) ratios in the left inferior frontal gyrus, right occipital grey matter, left anterior cingulate cortex and lower choline (Cho) ratios in right and left anterior cingulate cortex and higher Cho ratio in left lenticular nucleus was observed in the OCD group. Also we found a negative correlation between OCD duration and left insular cortex NAA/Cho ratio. CONCLUSION: We found significant metabolic alterations in the brain regions which were implicated in OCD pathophysiology. Lower NAA and Cho ratios in anterior cingulate cortex and lower NAA ratios in the left inferior fronyal gyrus containing lateral orbitofrontal cortex can be possibly related to higher activation in OCD patients. Also further studies of the occipital lobes and insula should be continued in OCD.