A Rare Cause of Respiratory Distress in Newborn: Huge Nasal Chondromesenchymal Hamartoma; Patient Report.

ABSTRACT: Nasal chondromesenchymal hamartoma is a rare benign tumor of the sinonasal tract in pediatrics and only few cases in infantile, early pediatric, and adolescent population have been reported. Nasal chondromesenchymal hamartoma commonly presents as respiratory difficulty, intranasal mass, or facial swelling and typically arises from the nasal septum or vestibule, lest frequently maxillary or ethmoid sinuses, orbit, nasopharynx, and oropharynx. The authors report a case of nasal chondromesench- ymal hamartoma that caused respiratory distress since birth, in a 4- week-old (28 days) infant which was arised from the medial aspect of the middle turbinate, an unexpected localization.

Comparison of posterior cranial fossa morphometric measurements in Chiari type I patients with and without syrinx cavity on magnetic resonance imaging.

Purpose: To compare the posterior fossa measurements of Chiari type I malformation (CHM1) patients with and without syrinx and with a control group. Material and methods: The patients with syrinx were divided into 2 groupd according to syrinx width/cord width (S/C) ratios: group 1 - S/C ratio < 50%; group 2 - S/C ratio > 50%. The length of the clivus, the AP length of the foramen magnum, the AP length of the posterior fossa, the perpendicular distance between the McRae line and (a) the splenium of corpus callosum, (b) the pons, and (c) the fastigium of the 160 patients and of the 160 control patients were statistically compared. In addition, the measurements of the patients with and without syrinx, according to the S/C ratio, were statistically compared. Results: Syrinx was present in 59 (36.8 %) of the 160 patients. The S/C ratio was < 50% in 30 (50.9 %) of them, and S/C ratio > 50% in 29 (49.1%) of them. All the measurements in the patient group, except of the AP length of the foramen magnum, were statistically significantly lower than in the control group (p = 0.001). There was no significant difference in the measurements of the patients with syrinx group 1 and the patients without syrinx, but the AP length of posterior fossa was statistically significantly lower in the patients with syrinx group 2 than the patients without syrinx (p = 0.03). Conclusion: The S/C ratio can be a guide to the underlying aetiology.

Investigation of the prognostic value of psoas muscle area measurement in pediatric patients before liver transplantation: A single-center retrospective study.

BACKGROUND: In this study, our aim is to investigate the prognostic value of psoas muscle area (PMA) evaluation before liver transplantation (LT) in pediatric patients. METHODS: Two hundred seventy-six patients under 18 years of age who underwent LT between January 2012 and December 2019 were included in the study. The patients' demographic, laboratory, clinical, and imaging data were scanned retrospectively. PMA was measured at the L4/5 level using computed tomography images. To determine reproducibility, a different radiologist evaluated 30 randomly selected patients. RESULTS: In patients with end-stage liver disease (ESLD), PMA was significantly lower than in patients with acute liver failure (P < .001). In patients with ESLD, a weakly significant correlation was found between PMA and 1-year survival after LT (r: .251, P = .030), hospitalization period (r: -.275, P = .039), and pediatric ESLD score (r: -.338, P < .001). Interobserver correlation was excellent (ICC: .941, 95% CI: .925, .971). CONCLUSION: In children with ESLD, PMA evaluated before LT can be used as a negative prognostic factor.

Comparison of Urinary Tract Dilatation and Society of Fetal Urology systems in the detection of vesicourethral reflux and renal scar.

PURPOSE: The presence and degree of hydronephrosis is very important in the management of many diseases of the urinary tract. In this study, we aim to compare the sensitivity and specificity of 2 classification systems that are used for hydro-nephrosis grading in ultrasound, for reflux and scar detection. The classification systems were the Society of Fetal Urology (SFU) and Urinary Tract Dilatation (UTD). MATERIAL AND METHODS: Ultrasounds and dimercaptosuccinic acid scintigraphies (DMSA) of all patients who underwent voiding cystourethrogram (VCUG) due to urinary tract infection were examined retrospectively. DMSA was accepted for scar detection and VCUG for reflux detection as reference methods. SFU classification was used for hydronephrosis in ultrasound reports, and UTD classification was made over the reports. Sensitivity, specificity, and positive and negative predictive values of UTD and SFU classification systems for reflux and scar detection were calculated, and these 2 systems were compared. RESULTS: 103 (39%) of the patients were male and 162 (61%) were female. Pathologies were detected in 192 (35%) of 530 kidneys in ultrasound. In 110 (42%) of the children, reflux was detected in VCUG. Scars in DMSA were detected in only 16% (44) of 266 kidneys. Sensitivity, positive and negative predictive values of the UTD classification system were statistically significantly higher than the SFU system for scar and reflux detection (p < 0.01). CONCLUSIONS: If we use the UTD system in ultrasounds of patients with urinary tract infections, children reported as UTD 0 may not need VCUG, which reduces radiation exposure to children and the cost of the diagnostic interventions.

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.

Analysis of magnetic resonance imaging findings of children with neurologic complications after liver transplantation.

OBJECTIVE: To analyze the magnetic resonance imaging findings in children diagnosed with neurologic complications after liver transplantation (LT). MATERIALS AND METHODS: A total of 39 patients diagnosed with neurologic complications following LT between 2010 and 2016. Neuroradiologic imaging was performed using cranial magnetic resonance imaging (MRI). Descriptive statistics regarding age, gender, type of complication, diagnostic and therapeutic modalities were calculated and presented as number and percentage. RESULTS: Our series consisted of 18 girls and 21 boys. Cryptogenic hepatitis (n = 13, 32%), metabolic diseases (Wilson's disease, tyrosinemia and glycogen storage disease) (n = 7, 18%) and fulminant toxic hepatitis (n = 4, 11%) constitute the most frequent indications for LT. The indications for neuroradiological imaging were convulsion and alteration of mental status. CONCLUSION: These central nervous system complications may present in a variable spectrum and convulsions and altered mental state were the most frequent clinical pictures. Imaging studies were normal in approximately one-third of cases; the most frequent pathologic findings were diffuse cerebral edema, atrophy, and PRES. Clinical history, careful examination and integrated analysis of radiologic data as well as close collaboration and multidisciplinary approach are of utmost importance for establishing the diagnosis rapidly and accurately.

Subgaleal and epidural metastases of the undifferentiated embryonal sarcoma of the liver.

Undifferentiated embryonal sarcoma of the liver (UESL) is very rare and has a very poor prognosis. UESL metastases have been reported in 5%-13% of the children with UESL and most metastases reported in the literature are present at diagnosis. Metastases reported in the literature belong to the lungs, pleura, and peritoneum. Radiological diagnosis of the UESL remains a poorly understood problem due to its rarity. Most of the reports published in the literature are also based on a relatively small number of patients. Approximately 200 cases have been reported regarding imaging features of this tumor. We reported a girl with UESL, who applied to the emergency department with abdominal pain. The lesion was solid and had cystic areas on ultrasound and there were peripherally enhanced serpenginous vessels in the lesion on Computed Tomography and MRI. Immunohistochemical diagnosis of the lesion was UESL. 26 months after surgery and adjuvant chemotherapy extradural and subcutaneus metastases were detected. These metastasis sites were first described for UESL.

Evaluation of Early and Late Complications of Pediatric Liver Transplantation with Multi-slice Computed Tomography: A High-Volume Transplant Single-Center Study.

BACKGROUND: To present abdominal multi-slice computed tomography (MSCT) results following transplantation in pediatric patients with a liver transplantation (LT), and to create awareness of early (<3 months) and late (>3 months) complications that may occur. METHODS: This retrospective study included 119 children with an LT performed in our hospital from 2014 to 2017. The descriptive statistics relating to patients' age, gender, transplantation indications, transplantation technique, and MSCT findings were calculated, and are presented as numbers and percentages. The complications were divided into 4 groups: vascular, biliary, parenchymal, and extraparenchymal. RESULTS: The LT procedures were performed with organs from living donors for 83 patients, and from deceased donors for 36 patients. Hepatic artery and portal vein complications were mostly seen in the early period (n = 18), and hepatic vein complications were also observed in the late period (n = 6). The most commonly encountered biliary complications were stenosis/stricture (n = 13) and bile leak/ bilioma (n = 9). Stenosis/stricture frequently occurred in the late period. The most common parenchymal complications were ischemic infarct (n = 8) in the early period, and abscess (n = 4) and recurrent hepatoblastoma (n = 2) in the late period. Hematoma (n = 7), intestinal perforation (n = 3), and focal spleen infarct (n = 3) were among the most commonly observed extraparenchymal abdominal complications. CONCLUSION: The complications occurring after pediatric LT varied according to the time after surgery and the transplantation technique used. Using MSCT, different abdominal complications can be assessed simultaneously, greatly contributing to diagnosis and treatment.

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.

Comprehensive Analysis of Long-term Splenic Volume Changes in Pediatric Liver Transplant Patients: Does It Correlate With Adverse Graft Outcomes?

OBJECTIVES: Splenomegaly and hypersplenism caused by liver failure increase the mortality and morbidity of patients even after liver transplantation if they do not regress. We evaluated the relation of splenic volume change and transplanted liver function. MATERIAL AND METHODS: A total of 59 of 207 pediatric patients who had liver transplantation between 2013 and 2018 in our institute were evaluated. The relation of spleen volume changes (splenic volume to standard splenic volume ratio [SV/SSV]) were measured at 0, 1, 6, 12, 24, and 36 months of follow-up by constructing electronic three-dimensional structure of the spleen at dynamic computed tomography (CT), and the course of liver functions were evaluated. RESULTS: The SV/SSV ratio decreases in the first postoperative 6 months. After 6 months, SV increases and SV/SSV increases gradually. In a normal functioning graft, SV/SSV significantly decreased in all time points (P < .001). In patients with adverse events, SV/SSV started to increase after 6 months. In patients with fulminant hepatic failure, SV/SSV started to increase after postoperative 6 months. Adverse events in patients with fulminant hepatic failure were more than the patients with chronic liver disease (58% vs 28%). There was an inverse correlation between SV/SSV and thrombocyte levels (P < .001). CONCLUSIONS: SV/SSV seems to be correlated to the adverse events (ie, rejection). Together with thrombocyte levels, it can be used as a noninvasive test for follow-up of transplant patients in terms of adverse events in graft function.

The Change in the Renal Pelvis Anterior-Posterior Diameter Between Prevoiding and Postvoiding Status, and Its Correlation With Vesicoureteral Reflux.

In this study, our aim was to evaluate the significance of the change in renal pelvis anterior-posterior diameter (RPAPD) before and after micturition between vesicoureteral reflux (VUR)-positive and -negative patients to whom had voiding cystourethrography (VCUG) was performed.In this study, 69 children, age ranging from 0 to 12 years, were included. Before the VCUG imaging, the RPAPD was measured first with a full bladder and then after urination via ultrasound (US). The differences between in RPAPD measurements were noted and values compared made among VUR-positive and -negative children. Data distribution was inhomogeneous, and the Wilcoxon Sign Rank test was utilized instead of Student t test. There was no statistically significant difference in prevoiding and postvoiding RPAPD in VUR (+) and VUR (-) patients (P = 0.672). There was no statistically significant relation between VUR and the presence of hydronephrosis (P = 0.126). Vesicoureteral reflux is more common in patients with urinary tract infections (UTI) (P = 0.001). There was no statistically significant relationship between prevoiding and postvoiding RPAPD change and VUR diagnosis (P = 0,164).Ultrasound is the modality of choice for urinary system evaluation. Diagnosis of hydronephrosis via US is not sufficient in predicting VUR; however, indirect findings may reveal the diagnosis. A decrease in RPAPD in postvoiding US evaluation may not rule out the VUR diagnosis for this reason further imaging modalities, such as VCUG, should be taken into consideration for the patients with clinical suspicion.

A Case of Cleidocranial Dysplasia with a Novel Mutation and Growth Velocity Gain with Growth Hormone Treatment

Cleidocranial dysplasia (CCD) is a rare congenital autosomal dominant skeletal disorder that is characterized by hypoplasia or aplasia of clavicles, failure of cranial suture closure, dental anomalies, short stature and other changes in skeletal patterning and growth. The gene responsible for pathogenesis has been mapped to the short arm of chromosome 6p21, core binding factor alpha-1 (CBFA1) or runt related transcription factor-2 (RUNX2). Here we describe a CCD patient with a novel mutation in the RUNX2 gene. A five-and-a-half year old girl presented with severe short stature, dysmorphic facial appearance (hypertelorism, prominent forehead, high palate, midfacial hypoplasia), macrocephaly, large anterior fontanelle, increased anteroposterior chest diameter. Her shoulders were close to each other and her bilateral clavicles appeared short on physical examination. Bilateral hypoplastic clavicles, coxa valga, hypoplasia of iliac bones, wide symphysis pubis and phalangeal dysplastic features were detected on her skeletal X-ray examination. She was diagnosed as having CCD. Molecular analysis detected a novel heterozygous mutation 'NM_001024630.3p.T155P(c.463A>C)' in the RUNX2 gene. At age seven years and two months old, because of her severe short stature, growth hormone (GH) treatment was started and she responded well to GH therapy with no adverse effects. In conclusion, hypoplasia or aplasia of the clavicles, failure of cranial suture closure, dental anomalies and short stature should bring CCD to mind. We present a novel mutation in the RUNX2 gene for CCD. We obtained growth velocity gain with GH treatment in our patient.

Advantages of early intervention with arterial embolization for intra-abdominal solid organ injuries in children.

PURPOSE: Active bleeding due to abdominal trauma is an important cause of mortality in childhood. The aim of this study is to demonstrate the advantages of early percutaneous transcatheter arterial embolization (PTAE) procedures in children with intra-abdominal hemorrhage due to blunt trauma. METHODS: Children with blunt abdominal trauma were retrospectively included. Two groups were identified for inclusion: patients with early embolization (EE group, n=10) and patients with late embolization (LE group, n=11). Both groups were investigated retrospectively and statistically analyzed with regard to lengths of stay in the intensive care unit and in the hospital, first enteral feeding after trauma, blood transfusion requirements, and cost. RESULTS: The duration of stay in the intensive care unit was greater in the LE group than in the EE group (4 days vs. 2 days, respectively). The duration of hospital stay was greater in the LE group than in the EE group (14 days vs. 6 days, respectively). Blood transfusion requirements (15 cc/kg of RBC packs) were greater in the LE group than in the EE group (3 vs. 1, respectively). The total hospital cost was higher in the LE group than in the EE group (4502 USD vs. 1371.5 USD, respectively). The time before starting enteral feeding after first admission was higher in the LE group than in the EE group (4 days vs. 1 day, respectively). CONCLUSION: Early embolization with PTAE results in shorter intensive care and hospitalization stays, earlier enteral feeding, and lower hospital costs for pediatric patients with intra-abdominal hemorrhage due to blunt trauma.

Diffusion-weighted imaging features of brain in obesity.

PURPOSE: Obesity is characterized by an altered distribution of body fluid. However, distribution of fluid (extracellular/intracellular) in brain tissues has not been studied in obese subjects yet. The purpose of this study was to detect possible brain diffusion changes especially in satiety and hunger related centers in obese subjects by diffusion weighted imaging (DWI). METHODS: Conventional MRI and DWI of the brain was obtained from 81 obese patients (obese=68, morbid obese=13) and 29 age-matched, nonobese. The apparent diffusion coefficient (ADC) values were calculated in hypothalamus; amygdala; hippocampal gyrus; thalamus; insula; cingulate gyrus; orbitofrontal, dorsomedial and dorsolateral frontal, middle temporal and occipital cortex; cerebellum; midbrain and corpus striatum. RESULTS: The ADC values of hypothalamus, hippocampal gyrus, amygdala, insula, cerebellum and midbrain were significantly increased in patients (n:81) when compared to nonobese subjects. The ADC values of thalamus, hippocampal gyrus, amygdala, orbitofrontal, occipital, dorsolateral and middle temporal cortex, insula and midbrain were significantly increased in morbid obese when compared to nonobese subjects. The ADC values of orbitofrontal and occipital cortex were significantly higher in morbid obese than the values in the obese. The body mass index positively correlated with ADC values of amygdala, insula, orbitofrontal and middle temporal cortex. CONCLUSION: We observed increased ADC values of distinct locations related to satiety and hunger that suggest altered fluid distribution and/or vasogenic edema in obese subjects. Awareness of this abnormalities in brain tissue composition/function in obesity may contribute to better understanding of the underlying mechanisms.

Morphometry of the Middle Cerebral Arteries: A Radio-Anatomical Study Based on Computed Tomography Angiography Findings / Morfometría de las Arterias Cerebrales Medias: Un Estudio Anátomo-Radiológico Basado en los Hallazgos de la Angiografía por Tomografía Computada

SUMMARY: Middle cerebral artery (MCA), which has the largest irrigation area of the arteries that feed the brain, is an important artery whose microanatomy should be well known because of its vascular variation. In pathologies which are known to affect the cerebrovascular system such as type 2 diabetes mellitus (T2DM) and hypertension, morphometric characteristics of MCA gain importance. The aim of this study is to compare the morphometric characteristics of M1 segment of MCA in T2DM and hypertensive patients with those of healthy control group by using computed tomographic angiography (CTA). The study was carried out with retrospective morphometric analysis of CTA images of 200 individuals between 40 and 65 years of age. The individuals were grouped in four as hypertensive patients (group 1), patients with T2DM (group 2), patients with hypertension and T2DM (group 3) and healthy control group (group 4). Length and diameter measurements of M1 segment were performed and recorded by using 3D CTA images. While statistically significant difference was found between bilateral M1 segment diameters of both women and men (p0.05). As a result of the post hoc analysis performed, it was concluded that right and left M1 segment diameter of group 1, group 2 and group 3 was found to be different from group 4 in both sexes (p<0.05). We believe that this study will both be a guide in radio-anatomic assessments to be performed and also increase microanatomic level of information in the surgical treatment of the artery by showing the morphometric changes that occur in M1 segment of MCA in T2DM diseases.

La arteria cerebral media (ACM), que otorga la mayor área de irrigación de las arterias que alimentan el cerebro, es un vaso importante cuya microanatomía debe ser bien conocida por su variación vascular. En patologías que afectan al sistema cerebrovascular, como la diabetes mellitus tipo 2 (DM2) y la hipertensión, las características morfométricas de la ACM cobran importancia. El objetivo de este estudio fue comparar las características morfométricas del segmento M1 de la ACM en pacientes con DM2 e hipertensos con las del grupo control sano mediante el uso de angiografía por tomografía computada (TC). El estudio fue realizado através de análisis morfométrico retrospectivo de imágenes de TC de 200 individuos entre 40 y 65 años de edad. Los individuos fueron divididos en cuatro grupos, como pacientes hipertensos (grupo 1), pacientes con DM2 (grupo 2), pacientes con hipertensión y DM2 (grupo 3) y grupo control sano (grupo 4). Las mediciones de longitud y diámetro del segmento M1 se realizaron y registraron utilizando imágenes 3D TC. Si bien se encontraron diferencias estadísticamente significativas entre los diámetros bilaterales de los segmentos M1 de mujeres y hombres (p0,05). Como resultado del análisis post hoc realizado, se concluyó que el diámetro de los segmentos M1 derecho e izquierdo del grupo 1, grupo 2 y grupo 3 fue diferente del grupo 4 en ambos sexos (p<0.05). Creemos que este estudio será una guía en las evaluaciones anátomo-radiológicas y también mejorará el nivel de información microanatómica en el tratamiento quirúrgico al mostrar los cambios morfométricos que ocurren en el segmento M1 de la ACM en las enfermedades con DM2.

Merosin-negative congenital muscular dystrophy: diffusion-weighted imaging findings of brain.

Merosin-negative congenital muscular dystrophy is a rare genetic disease of childhood involving the central and peripheral nervous system. There were high signal intensities throughout the centrum semiovale, periventricular, and sub-cortical white matters on T2-weighted images in a 4-year-old girl with merosin-negative congenital muscular dystrophy. An apparent diffusion coefficient map revealed increased signal intensity and apparent diffusion coefficient values in the periventricular and deep white matters. It may be attributable to increased water content in the white matter because of an abnormal blood-brain barrier rather than to decreased or abnormal myelination.

Torsion of a wandering spleen in an adolescent with Gaucher disease.

A wandering spleen is a rare condition characterized by the malposition of the spleen due to laxity or absence of its supporting ligaments. Although Gaucher disease generally presents with massive splenomegaly, which one of the predisposing causes of a wandering spleen, literature shows only one report of a wandering spleen in a child with Gaucher disease. In this case presentation, a 13-year-oldadolescent with Gaucher disease on enzyme replacement treatment was presented, who was detected having an abdominal mass on a routine visit and diagnosed with partial torsion of a wandering spleen associated with left lobe hypoplasia of the liver.