Changes of primary headache related white matter lesions in pediatric patients.

Bayram E, Yis U, Paketçi C, Okur D, Polat I, Çakmakci H, Hiz S, Anlar B. Changes of primary headache related white matter lesions in pediatric patients. Turk J Pediatr 2018; 60: 380-384. We aimed to describe the long-term prognosis of white matter lesions detected on magnetic resonance imaging in children with primary headache. Children who were admitted with the complaint of headache and had nonspecific white matter lesions on magnetic resonance imaging were included in the study. The clinical findings of the patients were reinvestigated using the same magnetic resonance imaging scanner and acquisition protocol after at least a two year period. Magnetic resonance imaging results of the patients were documented in detail. Findings of the baseline and follow-up studies were compared with each other by the same radiologist. Among the 11 patients, 8 ( 72.7%) were male and 3 (27.3%) were female. Mean age of patients at the time of second imaging was 12.9±2.3 years. Eight (72.7%) had migraine without aura, 1 (9.1%) had tension-type headache and 2 (18.2%) had migraine with aura. The mean clinical follow-up period of the patients was 4.31±1.31 years. All patients had low headache frequency on the last control visit when compared to the first clinical findings. The follow-up magnetic resonance imaging studies showed two newly developed white matter lesions in two patients who had migraine without aura and the white matter lesions disappeared in the patient who had tension-type headache, compared to the baseline neuroimaging. Findings of the baseline and long-term follow-up magnetic resonance imaging studies of the patients with primary headache showed no significant changes in terms of the location, size and laterality. Repeated neuro-imaging studies are not warranted in the absence of the progression in clinical findings.

The Effect of Dose-Volume Parameters on Central Nervous System Relapse in Pediatric Patients with Acute Leukemia Receiving Prophylactic Cranial Irradiation.

AIM OF THE STUDY: To investigate the effects of dosevolume parameters of brain parenchyma, optic nerves (ONs) and cribriform plate (CP), which were determined on central nervous system (CNS) control in pediatric leukemia patients who have undergone prophylactic cranial irradiation (PCI) at our department. PATIENTS AND METHODS: In the current study, the records of 14 patients were examined retrospectively. Along with the minimum and maximum doses for brain and CP, D95% (minimal dose received by the 95% volume of a structure) and V95% (percent volume of target receiving 95% of prescribed dose) could be obtained from the dose-volume histogram. Statistical analyses were conducted using the Mann Whitney test in SPSS-15. RESULTS: ALL/AML ratio was 9/5. CNS relapse was observed only in 2 patients. The minimum dose was 1249 (1100-1782) cGy, 1036 (547-1651) cGy, 856 (308-1460) cGy and 1234 (922-1727) cGy for brain parenchyma, right ON, left ON and CP, respectively. The value of D95%/D was 1.01 (1-1.06) and 0.99 (0.92- 1.06) for brain parenchyma and CP, respectively. V95% was 99.8 % (98.6%-100%) and 98.1% (80.5%-100%) for brain parenchyma and CP, respectively. The analyses revealed that none of the target tissue dose-volume parameters for PCI affected CNS relapse (p>0.05). CONCLUSION: In our study; it was found that the dosevolume parameters of the brain, CP and ONs did not have any effect on CNS relapse. Along with the other clinical factors, the scarce number of patients included in the study might have concealed the effects of parameters related to RT.

Posterior reversible leukoencephalopathy syndrome with spinal cord involvement in a 9-year-old girl.

We report the youngest pediatric case of posterior reversible leukoencephalopathy syndrome confined to brainstem and spinal cord. At presentation bicytopenia, renal derangement, visual disturbances, magnetic resonance imaging findings, increased protein content, IgG index and cell count in the cerebrospinal fluid led us to extensive search for myelitis. She received a short course of steroid treatment. The final diagnosis was hypertension due to reflux nephropathy. Severe hypertension that exceeds the range of autoregulation in anterior spinal territory may result in spinal posterior reversible leukoencephalopathy syndrome. Clinicians should be aware of spinal posterior reversible leukoencephalopathy syndrome when cases have extensive lesions in the brainstem and spinal cord with none or minimal clinical findings, so called "clinical radiologic dissociation".

Pleuropulmonary blastoma: a case report.

Pleuropulmonary blastoma is rare embryonal tumor of infancy and early childhood and it often arises from lung and more rarely from the parietal pleura. We present this entity which has no systematic data associated with its incidence in order to discuss clinical, histopathological, immunohistochemical features and the differential diagnosis. A three-year-old boy presented with fever showed signs of upper respiratory tract infection. Radiological examination revealed a solid mass filling the right hemithorax. The patient underwent core needle biopsy, wedge biopsy and lobectomy. Biopsy and surgical material were examined histopathologically. The tumor was composed of predominantly solid areas consisting blastemal cells with spindle, polygonal and round nuclei in the myxoid stroma. Immunohistochemical staining of the tumor cells were positive with vimentin and desmin. MIB-1 labeling index was above 90%. Histological diagnosis was pleuropulmonary blastoma type 3. The surgically sampled adjacent diafragma was also infiltrated with the tumor. The patient was treated with chemotherapy and showed no signs of recurrence in the follow-up of 9 months. Pleuropulmonary blastoma is a very rare childhood cancer that needs to be kept in mind in the pathological differential diagnosis of thoracic tumors in the children.

Expectoration of a foreign body at the late period: A case report.

Foreign body (FB) aspiration is a relatively common problem in children, particularly during the first 3 years of life. It is an emergency condition and the removal of the FB by bronchoscopy is the primary treatment. Children with undiagnosed retained foreign bodies may present with respiratory symptoms including recurrent or persistent wheezing, with or without respiratory failure. Spontaneous expectoration of a FB is a rare occurrence. Herein, we present a case that was diagnosed with FB aspiration during investigation for persistent wheezing and who expectorated part of a sunflower seed 2 months after aspiration.

Clinical and radiological course of simple renal cysts in children.

OBJECTIVE: To evaluate the clinical and, particularly, radiological course of simple renal cysts in children. MATERIALS AND METHODS: Children with simple renal cysts were retrospectively evaluated, especially for change in the cyst diameter during follow-up and complications. In addition, the rate of increase in cyst size per year was calculated, and those characteristics of the cyst were analyzed to predict aggressiveness. RESULTS: Simple renal cysts were detected in 45 (21 male) patients. Mean ages at diagnosis and follow-up period were 7.4 ± 4.9 and 2.9 ± 1.8 years, respectively. Forty-one of the 45 patients were followed up for longer than 1 year. Diameter of the cyst increased in 20 (49%), decreased in 4 (10%), unchanged in 13 (31%), and disappeared in 4 (10%) of patients. The average size increase and average rate of enlargement in simple cysts were 0.3 mm and 1.0% per year, respectively. Furthermore, in 19 (95%) patients, the cyst size increased in the first 2 years. Among baseline parameters, only initial cyst size was an independent predictor of annual growth rates (beta = 0.628; P <.001). The relationship between the initial cyst size and annual growth rates was determined as positive (r = 0.459, P = .003). Two patients with large cysts developed severe complication in the first 6 months. One of these underwent nephrectomy because of rapid increase in cyst diameter (170 mm), renal artery/vein compression, and massive hematuria. In the other patient with severe loin pain regarding simple cyst (73 mm), percutaneous aspiration was performed. CONCLUSION: Simple renal cysts in childhood tend to slowly increase in size. However, regular radiological follow-up might be important, especially in children with large size of cyst at diagnosis, because of more rapid increase in cyst size.

Severe neurologic involvement of Degos disease in a pediatric patient.

A 14-year-old male presented with paresthesias on the right upper and lower extremities, headache, and vomiting. In addition to worsening paresthesia and weakness on the right side of his body, blurred vision, fever, and skin lesions developed. He also had skin lesions characterized with 3-10 mm papules with a white atrophic center surrounded by pink rim mostly on the trunk and lower extremities. Brain magnetic resonance imaging showed chronic subdural effusion and encephalomalacia of the left cerebral hemisphere. Cerebrospinal fluid (CSF) examination revealed increased protein levels. Electromyography was consistent with diffuse polyradiculoneuropathy. Skin biopsy confirmed the diagnosis of a rare vasculopathy: Degos disease. A case presenting with chronic subdural effusion, encephalomalacia, elevated CSF protein, and polyradiculopathy should be carefully examined for skin lesions, which may suggest the diagnosis of Degos disease.

Place of ultrasonography in predicting vesicoureteral reflux in patients with mild renal scarring.

OBJECTIVE: To evaluate the role of renal ultrasonography (USG) in predicting vesicoureteral reflux (VUR) in children with mild renal scarring determined by dimercaptosuccinic acid scintigraphy performed after attack of urinary tract infections (UTI). METHODS: Dimercaptosuccinic acid scintigraphy, voiding cystourethrography (VCUG), and renal USG findings were evaluated retrospectively in children with UTI. Each kidney was evaluated as a separate renal unit (RU). RUs with severe scarring were excluded from the study. RUs having mild scarring with and without abnormal USG findings (group 1 and group 2, respectively) were compared in terms of the presence of VUR. RESULTS: There were a total of 228 patients (70 men, mean age 47.06 ± 44.14 months) and 456 RUs. Of the 185 RUs with mild scarring, 55 had abnormal USG findings (group 1), whereas 130 had normal USG findings (group 2). The rates of VUR and severe VUR (≥grade 4) were higher in group 1 compared with group 2 (69% vs 43%, P = .001 and 35% vs 7% respectively, P <.001). The sensitivity, specificity, positive predictive value, negative predictive value, and odds ratio of USG findings in predicting VUR in RU with mild scarring were 68%, 80%, 38%, 93%, and 8.2, respectively. CONCLUSION: Normal renal USG findings exclude a diagnosis of high-grade VUR to a large extend in children with UTI and mild renal scarring. Refraining from invasive VCUG might be a reasonable approach in these patients provided that no other predisposing factors for UTI and/or renal scarring present.

Importance of diazepam administration during electroencephalography in the differential diagnosis of cortical visual loss.

A 14-year-old boy presented with acute visual loss due to cortical blindness. Two weeks after the visual symptoms, the patient developed behavioral abnormalities. Brain magnetic resonance imaging (MRI) revealed hyperintense lesions at parieto-occipital lobes on T2-weighted and fluid attenuated inversion recovery images. Sleep and awake electroencephalography (EEG) were normal, but diazepam administration revealed bilateral periodic synchronous complexes occurring every 20 to 30 seconds. Elevated measles antibody titers in cerebrospinal fluid confirmed the diagnosis of subacute sclerosing panencephalitis. We conclude that visual loss due to cortical blindness is an important finding of subacute sclerosing panencephalitis. Diazepam administration during EEG should be a part of investigation in cases with unexplained cortical blindness.

Nasopharyngeal mature teratoma in the newborn.

Teratomas which originate from two or three germ layers are the most common congenital tumors of the childhood and are usually observed in the sacrococcygeal region. The nasopharynx is a considerably rare localization. Nazopharyngeal tumors may lead to significant findings including apnea, respiratory distress and stridor in newborns. In this study, a female newborn who developed respiratory distress minutes after cesarean delivery was presented. Examination following a difficult intubation and radiological examination revealed presence of a nasopharyngeal mass in the baby who was born at the 30(th) gestational age from a 30-year old primipar woman. The nasopharyngeal mass was excised and histopathological examination revealed mature teratoma. Although nasopharyngeal teratoma is a benign tumor, it may lead to urgency of airway management in the newborn. In this case presentation, the differential diagnosis and treatment of nasopharyngeal teratoma was discussed in accompaniment with the literature information.

Measurement of the apparent diffusion coefficient in paediatric mitochondrial encephalopathy cases and a comparison of parenchymal changes associated with the disease using follow-up diffusion coefficient measurements.

OBJECTIVES: To reveal the contribution of MRI and diffusion-weighted imaging (DWI) to the diagnosis of mitochondrial encephalopathy (ME) and to evaluate the parenchymal changes associated with this disease in the involved parenchymal areas using the apparent diffusion coefficient (ADC) parameter. METHODS: Ten patients who had undergone MRI and DWI analysis with a pre-diagnosis of neurometabolic disease, and who were subsequently diagnosed with ME in laboratory and/or genetic studies, were included in our study. ADC values were compared with a control group composed of 20 patients of similar age with normal brains. Evaluations involved measurements made in 20 different areas determined on the ADC map. The dominance or contribution of ADC coefficient measurements to the conventional sequences was compared with the controls. RESULTS: In the first examination, an increase in both diffusion and ADC values was detected in six cases and diffusion restriction and a decrease in ADC values in three patients. While an increase in both diffusion and ADC values was demonstrated in four cases, there was diffusion restriction and a decrease in ADC values in three cases in the control examinations. CONCLUSIONS: DWI provides information that complements conventional MRI sequences in the diagnosis of ME.

Pleuropulmonary Blastoma: A Case Report.

Pleuropulmonary blastoma is rare embryonal tumor of infancy and early childhood and it often arises from lung and more rarely from the parietal pleura. We present this entity which has no systematic data associated with its incidence in order to discuss clinical, histopathological, immunohistochemical features and the differential diagnosis. A three-year-old boy presented with fever showed signs of upper respiratory tract infection. Radiological examination revealed a solid mass filling the right hemithorax. The patient underwent core needle biopsy, wedge biopsy and lobectomy. Biopsy and surgical material were examined histopathologically. The tumor was composed of predominantly solid areas consisting blastemal cells with spindle, polygonal and round nuclei in the myxoid stroma. Immunohistochemical staining of the tumor cells were positive with vimentin and desmin. MIB-1 labeling index was above 90%. Histological diagnosis was pleuropulmonary blastoma type 3. The surgically sampled adjacent diafragma was also infiltrated with the tumor. The patient was treated with chemotherapy and showed no signs of recurrence in the follow-up of 9 months. Pleuropulmonary blastoma is a very rare childhood cancer that needs to be kept in mind in the pathological differential diagnosis of thoracic tumors in the children.

Assessment of atherosclerosis in obese adolescents: positive correlation of mean platelet volume and carotid intima media thickness.

AIMS: This study aims to assess the correlation of mean platelet volume (MPV) and common carotid artery (CCA) thickness in a population of obese adolescents. METHODS: Sixty-eight patients and 23 controls were enrolled. Anthropometric measurements, triglyceride, total cholesterol, high-density lipoprotein cholesterol, low-density lipoprotein cholesterol, alanine aminotransferase, aspartate aminotransferase, γ-glutamyl transpeptidase, hemoglobin, white blood cell count, platelet count, MPV and insulin resistance by homeostasis model of assessment of insulin resistance were investigated. Furthermore, CCA thickness was measured by high-resolution ultrasound. RESULTS: MPV and the left CCA thickness were significantly higher in obese adolescents than the healthy controls. The association between MPV and left CCA was checked by linear regression analysis. MPV explained 19% of the variation in left CCA (P < 0.001). At multiple regression analysis, MPV maintained a positive association with the left CCA thickness (P = 0.002) independently of fatty liver grade, relative weight, total cholesterol and homeostasis model of assessment of insulin resistance. CONCLUSION: MPV is significantly correlated with CCA thickness in obese adolescents.

Clinical course of children with nutcracker syndrome.

OBJECTIVE: To evaluate the clinical properties of patients with nutcracker syndrome (NCS). MATERIALS AND METHODS: The medical records of the patients with NCS were retrospectively investigated, and the patients' sex, age, presenting symptoms, urinalysis findings, associated diseases, and body mass indexes were recorded. RESULTS: A total of 23 nonconsanguineous patients (11 male and 12 female patients, mean age 141 ± 36 months). Two patients had posterior NCS. Seventeen patients presented with 1 or >1 symptom. Fatigue and lassitude (n = 1), flank pain (n = 8), abdominal pain (n = 7), varicocele (n = 2), and macroscopic hematuria (n = 9) were the presenting symptoms. Only 6 patients had received a diagnosis of NCS from the urinalysis findings, which revealed microscopic hematuria or non-nephrotic proteinuria. The urinalysis findings disclosed isolated proteinuria in 6, hematuria plus proteinuria in 8, and macroscopic hematuria in 9 patients. Orthostatic proteinuria was detected in 7 patients. In patients with regressed hematuria and proteinuria, the body mass index was markedly increased at the end of the follow-up period relative to at diagnosis (at diagnosis 16.9 ± 4.0 kg/m(2); at the end of the follow-up period 18.6 ± 4.6 kg/m(2); P = .028). CONCLUSION: The diagnosis of NCS should be considered in the presence of symptoms such as proteinuria and hematuria and should be absolutely ruled out before attempting renal biopsy. An apparent correlation between an increased body mass index and regression of symptoms was seen.

The relationships between renal compensatory hypertrophy etiologic factors and anthropometric development in the pediatric age group.

OBJECTIVE: To evaluate the factors associated with compensatory hypertrophy in the functional kidneys of children. METHODS: The medical files of patients with a solitary functional kidney were reviewed retrospectively. Data regarding anthropometric measurements, functional renal length, functional renal length of standard deviation score (SDS) of functional kidney at diagnosis, and end of follow-up were obtained. Patients were divided into 2 groups, those with a unilateral kidney function of <10% (hypoplasia, dysplasia, and atrophy, group 1) and those with a solitary kidney (agenesis, and multicystic dysplastic kidney, group 2). RESULTS: A total of 126 patients (70 boys) were evaluated. Both the sizes of the functional kidney and functional kidney SDS values at diagnosis were greater in group 1 relative to group 2. At the end of the follow-up period, anthropometric values including functional kidney size were higher in group 2. Functional kidney size of 2 SDS above the normal was mostly predictive at age 17.5 months (odds ratio [OR] 5.06) and at a body height of 82 cm (OR 5.57). CONCLUSION: The most determining factors for renal length SDS values were age and height. Solitary kidneys complete compensatory hypertrophy by 17.5 months of age, and after that their growth continues in parallel to normal growth.

Aicardi syndrome in two Turkish children.

Aicardi syndrome (AS) is an X-linked inherited disorder characterized by infantile spasms, chorioretinal lacunae, and agenesis or hypogenesis of the corpus callosum. The syndrome is more frequently seen in females but is observed in XXY male patients. Central nervous system, ocular, and costovertebral malformations may also seen in AS. Eye findings are of a considerable diagnostic importance; the chorioretinal lacunae are pathognomonic for AS and are generally bilateral. The outcome of the disease is generally severe, with a high mortality rate and poor developmental outcome. It is not clear which characteristics of the syndrome are related to a good prognosis in terms of psychomotor development, epileptic seizures, and survival. The purpose of this report was to demonstrate the spectrum of the clinical findings and the course of AS in two Turkish patients with different ocular and cranial MRI findings.

Molybdenum cofactor deficiency: review of 12 cases (MoCD and review).

Molybdenum cofactor deficiency is a rare inborn error of metabolism. The major clinical symptoms are intractable neonatal seizures, progressive encephalopathy, facial dysmorphic features and feeding difficulties. Most of the patients are misdiagnosed as hypoxic ischemic encephalopathy. The majority of patients have mutations in the MOCS1 and MOCS2 genes. Although the therapeutic treatment strategies have not been improved, genetic analysis is essential to elucidate the disease. Here, we report a review of 12 patients with Molybdenum cofactor deficiency reported from Turkey.

A case of rapid-onset obesity with hypothalamic dysfunction, hypoventilation, autonomic dysregulation, and neural crest tumor: ROHHADNET syndrome.

OBJECTIVE: Rapid-onset obesity with hypoventilation, hypothalamic dysfunction, and autonomic dysregulation (ROHHAD) is a rare disorder that mimics both common obesity and genetic obesity syndromes along with several endocrine disorders during early childhood. We aim to present the clinical features, laboratory and imaging results, and treatment outcomes of a patient with ROHHAD syndrome. METHODS: In this case report, we describe a 26-month-old boy who was admitted to our emergency department with dyspnea and cyanosis and was suspected to have ROHHAD syndrome due to his rapid-onset obesity and alveolar hypoventilation. RESULTS: A thoracal and abdominal magnetic resonance imaging was performed to demonstrate a possible accompanying neural crest tumor and it provided a yet asymptomatic retroperitoneal ganglioneuroblastoma. Based on these findings, the patient was diagnosed as ROHHADNET syndrome. CONCLUSION: Because of the high prevalence of cardiorespiratory arrest and probability of accompanying tumors, early recognition of ROHHAD syndrome is important. To prevent presumptive mortality and morbidity, ROHHAD syndrome should be considered in all cases of rapid and early-onset obesity associated with hypothalamic-pituitary endocrine dysfunctions.

Increased hippocampal volumes in lithium treated adolescents with bipolar disorders: a structural MRI study.

BACKGROUND: Structural neuroimaging studies in bipolar disorder (BD) have consistently identified several anatomical abnormalities in many brain areas related to mood regulation. Hippocampus is one of the key components of emotional regulatory networks in the brain. Evidence about hippocampal changes in BD is quite limited and inconsistent particularly for adolescent onset BD. It is aimed to compare hippocampus volumes of euthymic BD-I adolescents with healthy controls using structural MRI. METHODS: Hippocampal volumes of seventeen youths between 13 and 19 age period with DSM-IV BD (seven boys) and twelve healthy comparison subjects (five boys) were compared using structural MRI. Differences in hippocampal volumes between groups were tested. RESULTS: There was no significant difference between the right and left hippocampus volumes of patients with BD and the control group. However boys tended to have significantly larger right hippocampal volumes than girls both in BD and control group. Right hippocampal volumes were enlarged in lithium treated bipolar patients. This enlargement is not related to sex. LIMITATIONS: Future, longitudinal follow-up studies need large enough sample sizes of both sexes and a sex-matched healthy comparison group to sort out developmental, gender and medication influences on brain structures over time in BD. CONCLUSIONS: Lithium treatment in adolescent-onset BD has a significant effect on hippocampus volumes.

Vascular rings: presentation, imaging strategies, treatment, and outcome.

This study aimed to evaluate the presenting symptoms, the effectiveness of imaging methods, and the surgical treatment of vascular rings. Data for 44 patients (32 enrolled prospectively, 12 reviewed retrospectively) over a 10-year period in a tertiary referral center were analyzed. These patients comprised 25 patients with a left aortic arch and an aberrant right subclavian artery, 13 patients with a right aortic arch and a left subclavian artery originating from Kommerell's diverticulum, 1 patient with a right aortic arch and an aberrant left subclavian artery, 3 patients with a double aortic arch, and 2 patients with a pulmonary sling. Respiratory symptoms were found in 25 patients and dysphagia in 6 patients. Atypical symptoms such as reflex apnea, cyanosis, syncope episodes, and exercise-induced wheezing were noted in five patients. Associated congenital heart defects were detected in 41% of the patients. The diagnostic yield was 95.23% for barium esophagography, 54.54% for echocardiography, and 66.66% for computed tomography. The anatomy could be correctly identified by magnetic resonance imaging (MRI) in 97.43% and by angiography in 90.5% of the patients. Of the 30 patients who underwent surgery, 80% were completely relieved of symptoms during a mean follow-up period of 25 ± 33.5 months. Vascular rings should not be overlooked in infants with atypical symptoms. The authors' diagnostic procedure of choice is MRI because it is superior to angiography for delineating the relationship between abnormal vascular structures, trachea, and esophagus.