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Background: Schwannomas arise from nerve sheaths of cranial, peripheral, and spinal nerve or nerve roots. Most intracranial schwannomas arise from the cranial nerves, predominantly the vestibulocochlear nerve. In addition to cranial nerve schwannomas, intraparenchymal schwannomas of the brain and intramedullary schwannomas of the spinal cord are extremely rare. Case Report: In this case report, we describe the imaging findings of three diverse cases of schwannoma at different locations and unique presentations with acute neurological symptoms in the pediatric age group. Conclusion: Schwannomas should be considered and included in the differential diagnosis of intracranial or intraspinal intramedullary space-occupying lesions in pediatric patients.

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.

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.]

Optic pathway gliomas are the most common central nervous system neoplasms in patients with neurofibromatosis type 1. Perineural arachnoidal gliomatosis is a rare and distinctive growth pattern of optic nerve glioma, in which the tumor infiltrates through the pia mater and pre-dominantly involves the subarachnoid space around the optic nerve. Here, we report an 8-year-old girl with perineural arachnoidal gliomatosis associated with neurofibromatosis type 1.

Introduction: Pulmonary nodules detected by thoracic imaging in children can be seen as a systemic disease finding or incidentally. Aims: To evaluate children referred to pediatric pulmonology department with pulmonary nodules Methods: Gender, age at admission, symptom, pulmonary function tests, radiologic results, diameters and locations of nodules of all children with pulmonary nodules were analyzed. Results: The mean age of all 45 patients was 12.3±3.8 years and 62.2% were female. The mean follow-up duration was 12.9±19.9 months. 26 (57.8%) patients had symptoms at admission and 46.2% of symptoms were cough. 24 (53.3%) patients had comorbid diseases, 7 (29.2%) had rheumatological, and others had gastroenterological, nephrological, dermatological and cardiological diseases. Chest radiographs were mostly normal (57.1%), while the most common finding on thoracic CT was nodule with fibrotic thickening (87.5%). The mean diameter of nodule was 3.2±2.6 mm. Solitary nodule was found in 31 (68.9%) patients and 21 of them had symptoms at admission. Presence of symptoms was more common in patients with solitary nodules than those without (p=0.045). Nodules were most common in the lower lobes of the lungs (77.1%) and 50.0% of the nodules were only in the right lung. 25 (55.6%) patients had pulmonary function tests and mean FEV1, FVC, FEV1/FVC, and MEF25–75 values were 100.4±12.1%, 96.8±13.2%, 103.8±9.6, and 98.9±28.5%, respectively. There was no significant difference between diameter and location of nodules and gender (p>0.05). The diameter and location of nodules were not correlated with age at admission and symptoms. Conclusions: Nonspecific pulmonary nodules in children can be seen and may have no effect on pulmonary function test of children.

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.

Introduction: Bronchiolitis obliterans (BO) is as a rare pulmonary complication of Stevens Johnson Syndrome (SJS). We present a patient who was thought to have BO after SJS but there was no radiological finding compatible with BO on the first thorax CT. However one month later thorax CT findings became consistent with BO. Case Report: A 12-year-old female patient was diagnosed with SJS after using oseltamivir, ibuprofen and trimethoprim sulfometaxazole and was treated with IVIG and steroids. One month later, she had cough, shortness of breath. Physical examination revealed bilateral decreased breath sounds, common rhonchi and crackles on both lungs. Oxygen saturation was 95% and respiratory rate was 20/min. Obstructive and restrictive patterns were detected in pulmonary function test (PFT). There was no findings compatible with BO on thorax CT in the expiratory phase. Steroid, azithromycin and chest physiotherapy were started due to the clinical findings and PFT results suggesting BO. In the second month control, bilateral patchy air trapping and peribronchial thickening were detected in thorax CT in the expiratory phase, which are thought to be compatible with BO. Steroid therapy was tapered and stopped due to significant improvement in the patient9s clinical findings. Method: Bronchiolitis obliterans secondary to SJS is generally progressive and has poor prognosis. Close monitoring of patients with SJS is important for timely detection of BO. BO is mainly diagnosed by thorax CT however radiological findings may occur in the late period.

BACKGROUND AND OBJECTIVES Hypersensitivity pneumonia is a complex condition due to exposure time, intensity, different clinical presentation, and treatment practices. We aimed to evaluate the patients that were diagnosed with hypersensitivity pneumonia (HSP) due to exposure to pigeons and a review of the literature for diagnosis and treatment of Pigeon Breeder`s Disease (PBD) in children. METHOD Between the years of 2009-2018, patients who were diagnosed with HSP due to PBD were included in the study in a pediatric pulmonology department. Findings of our patients, treatments, and prognoses were compared with 17 articles in the literature about PBD in children. RESULTS In a 9 year-period, 6 patients were diagnosed as HSP due to PBD. The mean age of the patients was 8.8 ± 5.4 years and the average duration of pigeon exposure was 60.1 ± 6.5 days. Precipitating antibodies were positive in 3 patients. In four cases, symptoms were resolved with only prevention of pigeon exposure. Two patients who had close contact with pigeons needed oxygen supplementation and steroid therapy. CONCLUSION Hypersensitivity pneumonia should be considered for the differential diagnosis of patients that present with respiratory distress, cough, fever, and weight loss. Prolonged exposure and close contact may worsen the clinical symptoms. In most cases, only exposure prevention is enough, while steroid therapy, oxygen support, and intensive care monitoring may be required in severe cases.

Introduction No clear information exists about the factors affecting pleural thickening following parapneumonic effusion in children. We aimed to investigate factors that affect the resolving time of pleural thickening after parapneumonic effusion. Methods Between the years of 2007-18, 91 patients, which were followed due to diagnosis of pleural thickening after parapneumonic effusion, were assessed. Ages, complaints, physical examination findings, laboratory results, chest x-ray and ultrasonography findings, treatments, duration of treatment and recovery time of the patients were examined terms in of pleural thickening resolving time. Results The mean age of patients was 7.5 ± 5.0 years. Pleural thickening resolving time was 151 ± 6.8 days. The resolving time for pleural thickening was delayed with older ages, longer duration of complaints, fever before hospital admission and treatment, lower oxygen saturation at the time of admission, crackles in the physical examination, higher white blood cell count and pleural fluid density (p = 0.018, p = 0.001, p = 0.021, p = 0.020, p = 0.024, p = 0.025, p = 0.021, p = 0.019). In addition, the amount of effusion measured by thorax ultrasonography, fibrinolytic usage, and complications had a role in the delayed resolving time (p = 0.034, p = 0.001, p = 0.034). Pleural thickening resolved in 80% of the patients. Conclusion In this report, 80% of pleural thickening, following parapneumonic effusion resolved within 5 months. Patients who do not have a complication during follow-up are not required to monitor with frequent chest x-ray. Patients with a higher amount of pleural effusion, complications and need for fibrinolytic treatment should be followed more carefully.

Internal hernia through the foramen of Winslow is a very rare condition, especially in children. Here we report a 16-month-old girl who presented with obstructive jaundice and elevation of pancreatic enzymes and was ultimately diagnosed with internal hernia and malrotation by radiologic investigation and open approach surgery. To the best of our knowledge, obstructive jaundice with pancreatitis and other congenital abnormalities in children with the foramen of Winslow hernia have not been reported previously in the literature.

Arterial ischaemic stroke in the paediatric population is considered a rare disease, and its diagnosis is often delayed due to the subtlety and variability of clinical symptoms, especially in younger patients. The clinical presentation and imaging features of ischaemic stroke in the paediatric population are variable depending on the underlying cause, affected artery and patient’s age. Literally, acute occlusion of the middle cerebral artery shows significant clinical signs and symptoms, and riotous imaging findings due to the size of the territory. Here, we present a case of a 15-year-old boy who unusually had subtle and intermittent clinical symptoms in spite of a complete acute occlusion in his right middle cerebral artery.

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.

Background/aim: Airway compression (AC) by vascular structures is an important complication of congenital heart disease (CHD) that often goes unrecognized. It is not easy to identify whether CHD patients require additional invasive examinations or not. Therefore, the present study aims to develop an AC diagnostic algorithm for CHD patients. Materials and methods: CHD patients with persistent respiratory symptoms that were treated between January 2007 and December 2015 were retrospectively reviewed. The following data were recorded for all CHD patients with AC: age, cardiac anomalies, the compressed structure, the airway diameter ratio (ADR), the compressing structure(s), treatment, and follow-up.Results: During the 8-year study period, 62 of 253 CHD patients had persistent respiratory symptoms, of which 11 cases were diagnosed as AC via bronchoscopy and/or thoracic computed tomography angiography. The most frequently affected structures were the left main bronchus and trachea, and the most common compressing structure was the right pulmonary artery. The ADR was near total compression in 3 patients and >0.50 in 3 patients. During follow-up, 5 of the 11 patients with AC underwent surgery, 2 died, and 4 were followed clinically. Patients with ADR of >0.50 did not require surgery and were followed clinically. Conclusion: CHD patients with persistent respiratory symptoms associated with lower respiratory airway obstruction should be evaluated via invasive examination. An AC diagnostic algorithm for pediatric CHD patients was developed.

Isolated intracranial Rosai–Dorfman disease (RDD) is extremely rare in pediatric patients. We present the case of a 22-month-old boy whom had isolated intracranial RDD involvement. To our knowledge, a parieto-occipital regional involvement without a dural tail sign has not been previously documented. Also, the mass contained hyperintense central T1 foci, and hypointense T2 and gradient echo foci; which are helpful in the differential diagnosis from meningioma. The magnetic resonance and computed tomography imaging findings are discussed and the follow-up course is presented in this paper.

Systemic lupus erythematosus (SLE) is a systemic autoimmune disease which has broad pleuropulmonary manifestations. One of the rare and mortal complications is acute lupus pneumonitis, which is reported very rarely, especially in childhood. Herein, we report an 8-year-old girl with isolated acute lupus pneumonitis as the initial presentation that required a lung biopsy for diagnosis. Although she had improvement with the administration of steroids, steroid treatment was reduced due to the drug’s side effects resulting in the addition of azathioprine and mycophenolate mofetil to the treatment regimen. After the new regimen failed to result in clinical improvement, hydroxychloroquine treatment was started and a significant improvement was observed. Acute lupus pneumonitis is an uncommon manifestation of SLE and diagnosis may be difficult in patients without other organ involvement.

Objective: Inhalation of pigeon avian protein antigens is the most common cause of hypersensitivity pneumonia (HSP). In this study, we aimed to evaluate the patients who were diagnosed as HSP due to pigeon antigens in a pediatric pulmonology department. Method: Clinical, laboratory and radiological data of patients diagnosed as Pigeon Breeder’s Disease was reviewed in a pediatric pulmonology department. Results: In an 8 year-period 6 patients were diagnosed as HSP due to pigeon antigens. All patients’ fathers were feeding pigeons for hobby or economic reasons. All patients had cough, shortness of breath and high fever. The mean age of the patients was 7.6 years and the average duration of pigeon exposure was 2 months. Precipitin antibody against pigeon avian protein antigen was positive in 3 patients. Bilateral symmetric ground-glass opacities and multiple centrilobular nodules were detected in thorax CT consistent with HSP in all patients. In four cases symptoms were resolved with only prevention of pigeon exposure. Two patients who had close contact with the pigeon needed oxygen supplementation and steroid therapy. Conclusion: HSP should be considered in the differential diagnosis of patients who present with respiratory distress, cough, fever and weight loss. Detailed contact history should be asked. Precipitant antibody positivity may not be present in every patient. Radiological diagnosis may be helpful. Not only prolonged exposure but also close contact may worsen clinical symptoms. Only exposure prevention is enough in most cases while steroid therapy, oxygen support and intensive care monitoring may be required in severe cases.

The pterygopalatine fossa is an important anatomic crossroads that is connected with numerous intra- and extracranial spaces via foramina and fissures. Although this fossa is small, its central location in the skull base and its communications provide clinical, radiological, and anatomical significance. In this pictorial review, we aimed to describe the radiologic anatomy of the pterygopalatine fossa, as well as to give some pathologic examples to better understand this major conduit.

Isiyel E, Bakkaloglu S, Oguz D, Yenicesu I, Boyunaga O, Ozdemir Y, Damar C, Kandur Y, Akcaboy M, Aslan AT, Sismanlar T, Hasanoglan E, Buyan N. An adolescent case of extensive Behcet`s disease successfully treated with Infliximab. Turk J Pediatr 2019; 61: 585-588. Cardiac involvement is an uncommon and life-threatening complication of Behcet`s Disease. We present a 14-year-old boy, admitted to our hospital for recurrent hemoptysis. In his radiologic evaluation, a right ventricular thrombus and pulmonary arterial aneurysm were identified. He was diagnosed with Behcet`s Disease, and then he received prednisone and cyclophosphamide. However, his cardiac thrombus enlargened. After his treatment was replaced with infliximab, the pulmonary aneurysms regressed, and the cardiac thrombus disappeared. In conclusion, infliximab should be considered as a reliable option for vascular Behcet`s Disease resistant to conventional treatment.

© 2016 The Author(s).Purpose: Juvenile nasopharyngeal angiofibroma (JNA) is a rare tumor that exhibits a predictable spreading pattern. Radiologist's prior knowledge on the tumor's characteristics aids in establishing a diagnosis. We aimed to report the characteristic Magnetic Resonance Imaging (MRI) findings and the spread patterns of JNA. Materials and methods: We retrospectively evaluated the MRI findings and extension pathways of 6 cases of JNA. Results: The patients' age ranged from 8 to 16 years and all patients were male. The tumors were classified according to the Onerci system. Tumors were largely isointense to muscle on T1-weighted images and hyperintense on T2-weighted images. All lesions had internal signal-void regions and all exhibited intense enhancement after IV contrast injection. Diffusion restriction was not an associated feature. ADC values for these tumors were high. The evaluation of the available MR angiography studies of three patients showed the blood supply to the tumor to be mainly from the internal maxillary branch of the external carotid artery. In all patients, the diagnosis was based on MR images and a surgical excision was planned. Conclusion: The diagnosis can be established based on the characteristic imaging findings and the clinical history without performing a biopsy.

Central tegmental tract is an extrapyramidal tract between red nucleus and inferior olivary nucleus which is located in the tegmentum pontis bilaterally and symmetrically. The etiology of the presence of central tegmental tract hyperintensity on MRI is unclear.In this study our aim is to evaluate the frequency of central tegmental tract lesions in patients with cerebral palsy and control group, as well as to determine whether there is an association between central tegmental tract lesions and cerebral palsy types.Clinical and MRI data of 200 patients with cerebral palsy in study group (87 female, 113 male; mean age, 5.81years; range, 0-16years) and 258 patients in control group (114 female, 144 male; mean age, 6.28years; range, 0-16years) were independently evaluated by two reader for presence of central tegmental tract hyperintensity and other associated abnormalities.Central tegmental tract hyperintensities on T2WI were detected in 19% of the study group (38/200) and 3.5% of the control group (9/258) (p0.0001). Among the total of 38 central tegmental tract lesions in study group, the frequency of central tegmental tract hyperintensity was 16% (24/150) in spastic cerebral palsy and 35% (14/40) in dyskinetic cerebral palsy (p=0.0131).The prevalence of central tegmental tract hyperintensity is higher in patients with cerebral palsy particularly in dyskinetic type. We suggest that there is an increased association of the tegmental lesions with dyskinetic CP. Patients with cerebral palsy and ischemic changes were more likely to have central tegmental tract lesions. According to our results we advocate that an ischemic process may have a role in the etiopathogenesis.

Nodular regenerative hyperplasia (NRH) and focal nodular hyperplasia (FNH) of the liver rarely occur in children after completion of tumor therapy. These lesions mimic hepatic metastasis and they must be distinguished from metastatic lesions. The authors present 2 children, one with NRH and one with FNH, after undergoing antineoplastic therapy for non-hepatic childhood solid tumors and discuss their patients in the context of the literature.

Introduction: Congenital heart diseases may cause respiratory symptoms due to increased or decreased pulmonary flow. Method: Congenital heart disease patients with persistent respiratory symptoms were reviewed. Results: 251 patients with congenital heart disease were followed for respiratory symptoms. Six patients (2 %) had evaluated detailed because of persistent and recurrent respiratory symptoms. The mean age was 3,7±2,9 years.They had complex cardiac anomalies such as transposition of great arteries, interrupted arcus aorta, ventricular septal defect, atrial septal defect, pulmonary artery atresia, truncus arteriosus type 4. Two of them were operated. Prolonged wheezing and recurrent pneumonia in the same lobe were the most common finding. Four of them had dilated pulmonary artery, one of them had dilated ventricule and the other one had dilated descending aorta which compress to the bronchi or trachea.Although all of them had being followed since birth for congenital heart diseases, the vascular compression was diagnosed average 3,7 years later. Echocardiography and catheter angiography were perfomed before, but vascular compression was detected with CT- angiography. One of them was re-operated, one of them had operation for stent replacement then lobectomy was performed, one of them was died and the others were being followed.Conclusion: Due to flow or pressure changes, cardiac or vascular enlargement may cause compression in thoracic structures. Patients with congenital heart diseases with persistent pulmonary symptoms, should be evaluated with CT angiography in terms of contiguity and compression of the vascular or cardiac structures to pulmonary components.

Desmoplastic infantile ganglioglioma is a paediatric brain tumor that is commonly seen in the infantile age group. Literature on the non-infantile variant of this low-grade supratentorial neoplasm is very scarce, except for a few case reports. Herein, we report a case of desmoplastic non-infantile ganglioglioma occurring at the age of 6 years and describe its conventional and advanced magnetic resonance imaging characteristics.

Osteopetrosis is a rare genetic condition of reduced osteoclastic bone resorption which causes defective bone remodeling and skeletal sclerosis during growth, having effects on many organs and tissues. Mutation of T-cell immune regulator 1 (TCRG1) gene is the most common genetic defect leading to osteopetrosis, with poor prognosis. The autosomal recessive form presents in the infantile period (also known as malignant infantile osteopetrosis - MIOP), and is characterized by fractures, short stature, hepatosplenomegaly, compressive neuropathies, hypocalcemia and pancytopenia. Being a rare disease with non-specific clinical manifestations, the diagnosis is difficult and usually delayed. Rickets is a characteristic feature of MIOP which results from the defect in osteoclasts to provide a normal Ca/P balance resulting in the poor mineralization of the osteoid. Various treatment options have been suggested for osteopetrosis, but hematopoietic stem cell transplantation still remains the only curative treatment option presently. The authors report the case of a 46-day-old girl with late-onset neonatal hypocalcemia and rickets that was later diagnosed as osteopetrosis. This case report emphasizes that infantile osteopetrosis is an important cause of neonatal hypocalcemia. As irreversible complications develop within the first months of life, immediate diagnosis and early intervention are crucial and may be life-saving.

Anterior mediastinal and adenoid masses in children after cessation of chemotherapy for malignant disease open cause a diagnostic problem. Differential diagnosis of thymic enlargement and adenoid hyperplasia from recurrence frequently poses a challenge both for the radiologist and the physician. In this study the authors evaluated, 491 patients with different malignant tumors for thymic and adenoid hyperplasia. Thymic hyperplasia was seen in 18 patients (5 Hodgkin disease (HD), 5 non-Hodgkin lymphoma (NHL), 4 Wilms tumor, 2 germ cell tumor, 1 Ewing sarcoma, and 1 neuroblastoma), only adenotonsillar hyperplasia was seen in 6 patients, all with NHL, and both thymic and adenotonsillar hyperplasia were seen in 3 patients (1 HD, 2 NHL). In 5 patients, adenoid hyperplasia was proven by biopsy; 1 patient. underwent to adenoidectomy. Their histopathologic investigation showed polyclonal follicular hyperplasia. The authors recommend that patients with thymic and/or adenotonsillar enlargement after successful treatment of their primary malignancy should be evaluated cautiously before an invasive procedure is planned.

Background: Screening for developmental dysplasia of the hip (DDH) is widely recommended for all infants to prevent disability from late diagnosis of dislocation of the hip. The present study evaluates the results of screening for developmental dislocation of hip in a clinic in Turkey over the course of 7 years. Methods: Hospital records of 5798 infants who were examined regularly until walking age at Gazi University well child clinics between January 1995 and December 2001 were reviewed. Infants with known risk factors for DDH such as breech presentation, family history of DDH or swaddling, and of infants with physical examination findings suggestive of DDH, were referred to orthopedic surgeons for diagnosis. Based on this final diagnosis, sensitivity, specificity, positive and negative predictive values of risk factors and physical examination findings were calculated. Results: Of the 5798 infants, risk factors were detected in the medical history of 111 infants, and in 14 infants a musculoskeletal deformity was detected. In 606 infants the physical examination findings were suggestive of DDH. Ten patients were subsequently diagnosed with DDH. The sensitivity, specificity, positive predictive value and negative predictive values of having a risk factor for DDH in history were 10.0%, 98.1%, 0.9%, 99.8%, and having abnormal hip examination findings were 100.0%, 88.9%, 1.6% and 100.0%, respectively. Conclusion: A careful history and physical examination is the cornerstone of DDH screening. Serial hip examinations performed during health examination visits provide an opportunity to identify DDH cases. The sensitivity of risk factors in history and physical examination findings together is high enough to be accepted as a screening tool.

A13-year-old girl with complaints of difficulty in walking, left leg pain, and lower back pain of 1 month duration was admitted to our pediatric neurology department. Having the symptoms of lumbosacral radiculopathy, a magnetic resonance imaging (MRI) was ordered to exclude any herniated disc, spinal stenosis, or arthritic and degenerative changes of the facet joints. Lumbosacral MRI demonstrated a 4-cm×3.5cm×3.5-cm well-defined, expansile lesion of the left side of the sacral bone at the level of the S1–S2 vertebrae extending to the left sacroiliac joint (Figs. 1 and 2). The lesion was hypointense on T1WI and hyperintense on T2WI containing cysts with internal septations and characteristic fluidfluid levels representing blood of variable ages that was compatible with aneurysmal bone cyst (Fig. 2a and 2b). At the level of S1 and S2 the nerve roots at the left side were compressed by this expansile lesion. After contrast administration, we did not observe any enhancement or solid component distinctively. The left piriformis muscle had denervation-related muscle atrophy because of entrapment neuropathy of the piriformis nerve, originating in the sacral plexus and arising from the posterior division of the ventral rami of the first and second sacral nerve (Fig. 2c).An open bone biopsy confirmed the diagnosis of aneurysmal bone cyst which

Şişmanlar T, Aslan AT, Öztunalı Ç, Boyunağa Ö. Left upper lobe atelectasis due to plastic bronchitis. Turk J Pediatr 2017; 59: 207-209. Plastic bronchitis is a rare condition in children, characterized by expectoration of branching bronchial casts. It can cause atelectasis in the lung. Herein we reported a 4.5-year-old boy with left upper lobe atelectasis due to plastic bronchitis. Although his chest X-ray is specific for left upper left atelectasis, thoracic computerized tomography had been performed and was compatible with obliterated left upper lobe bronchus. Typical radiological appearance of the left upper lobe atelectasis is not well known by clinicians which results unnecessary further examinations such as computerized tomography which exposes high dose radiation. We want to emphasize the long-term side effects of radiation and avoid unnecessary examinations in children.

We report a case of a newborn girl with neurocutaneous melanocytosis, hemimegalencephaly and a large ovarian cyst. She also had melanocyte deposition in the filum terminale. The ultrasound and the magnetic resonance imaging findings are discussed.

We report two sisters who have a rare skeletal abnormality termed Patterson-Lowry rhizomelic dysplasia. The typical findings of these cases on bone survey are isolated shortening and proximal metaphyseal enlargement and cupping of the bilateral humeri. The elder sister also has coxa vara deformity and dysplastic proximal femoral epiphyses on both sides. The younger sister has normal hip joint bones bilaterally, but her proximal femoral epiphyses are smaller than normal. All other bones of the sisters are of normal size and configuration. Our patients are two siblings, and their parents are first degree relatives, suggesting autosomal-recessive (AR) inheritence. The present patients help us to understand the genetic relationships and skeletal variabilities of this rare entity.

Fibrolamellar variant of hepatocellular carcinoma (FLHCC) does not have a favorable prognosis than conventional HCC, and there is no difference regarding the response to chemotherapy and the degree of surgical resectability. FLHCC commonly recurs after complete surgical resection, and there is a high rate of lymph node metastases. Herein, we report a 12-year-old girl with metastatic FLHCC with multiple recurrences aggressively treated with surgery, chemotherapy, and antiangiogenic agents. She is in complete remission after 4 years and 2 months after the diagnosis of metastatic FLHCC. The standard treatment of FLHCC is excision of the primary tumor and its metastases. Chemotherapy for FLHCC is controversial, and it has been suggested that cytoreductive chemotherapy was ineffective and adjuvant chemotherapy did not improve survival. Our patient with multiple recurrences was successfully treated with surgery, first-line chemotherapy with cisplatin and doxorubicin, second-line chemotherapy with 5-fluorouracil/interferon-α combination, and adjuvant antiangiogenic agents like cyclophosphamide and thalidomide. As FLHCC patients have no underlying liver disease, they can tolerate higher doses of chemotherapy compared with conventional HCC patients. We support the use of repeated aggressive surgery with adjuvant chemotherapy and antiangiogenic therapy, which provided complete remission in our patient with metastatic and recurrent FLHCC.

Intussusception is a rare entity in neonates. It may present with non-specific signs including abdominal distension, feeding intolerance, vomiting and bloody stools. Symptomatology is similar to Necrotizing Entero-Colitis (NEC). Ultrasound can help to establish early diagnosis in neonate. A 27-week preterm newborn was initially suspected as NEC based on abdominal distention, bilious vomiting, worsening clinical condition and dilated loops of bowel on X-ray, which turned out to be ileo-ileal intussusception. Diagnosis was made by ultrasound obtained for a palpable mass to rule out intra abdominal abscess and lack of improvement in clinical condition despite 5 days of conservative treatment. Surgery was performed consisting of removal of the necrotic intussusception area and end-to-end anastomosis and patient was discharged from hospital on day 60 of life. As a conclusion, pathological abdominal findings in preterm newborns can also be due to conditions other than NEC and ultrasound may be a useful tool for timely and accurate diagnosis.

Knowledge of the normal anatomy and development of vertebrae, as well as of the changes in the vertebral bone marrow and spinal cord according to age are mandatory to interpret radiological images of these regions accurately. Imaging of the spine can be performed by conventional radiography, ultrasonography (US), computerized tomography (CT), digital subtraction angiography (DSA) or magnetic resonance imaging (MRI). With conventional radiography, anteroposterior (AP), lateral, and oblique projections of the vertebral column, as well as specific structural imaging, should be obtained (e.g. AP open mouth for the odontoid process). Conventional radiographs provide valuable information regarding the bony structures of the spinal column, facet joints, disc spaces, and foramina, while only limited information regarding the paraspinal soft tissues can be obtained. The spinal cord is well seen with US in the first few months of life, but at a later age visualization of the cord is not satisfactory.

We report a 2-year-old boy who had a family history of neurofibromatosis, multiple cafe-au-lait spots oil the trunk, tibial pseudarthrosis, and was diagnosed with a malignant triton tumor of the pelvis. To our knowledge our case is one of the youngest patients reported with a malignant triton tumor and the second pediatric case with a pelvic malignant triton tumor.

In this report we describe an unusual case of neuroblastoma that presented with an initial manifestation of protein losing enteropathy of secondary to intestinal lymphangiectasia.

The authors describe a 6-year-old boy diagnosed with mediastinal Burkitt lymphoma with tumor invasion into bone marrow and both kidneys. After receiving chemotherapy according to NHL BFM-95 protocol for the high-risk disseminated lymphoma, the patient reached complete remission. He relapsed in the mediastinum at 5 months from the diagnosis. He underwent thoracotomy and tumor mass was removed by inferior lobectomy of right lung. Residual tumor progressed rapidly. Autologous stem cell transplantation could not be performed because of unresponsiveness to cytoreductive chemotherapy. Twenty-three days after the last chemotherapy course, he received rituximab at a dose of 375 mg/m(2) by intravenous infusion weekly, for a total of 8 dose. However, multiple intra-abdominal metastatic lesions were detected at the end of the therapy. Palliative radiotherapy was applied to these sites. He died because of disease progression, 11 months after the diagnosis.

Introduction Renal vein thrombosis (RVT) in neonates is a rare condition that carries low mortality but high morbidity. Aetiology isn’t fully understood; predisposing factors are dehydratation, sepsis, asphyxia, polycythemia, maternal diabetes, traumatic delivery, congenital renal vein defects, umblical catheterisation, prothrombotic conditions. Case presentation 36 week baby was born by C/S to 32 year-old, gravida 2 mother. The only prenatal risk was gestational diabetes. She was born early because of fetal distress. Evaluation of the infant for jaundice revealed left flank mass and edematous left leg in the second day of life. No effusion was detected in the joints of hip and knee. Lower extremity doppler USG was normal. Abdominal USG showed enlarged left kidney. Doppler USG showed thrombus in the inferior venae cavae, extending to left renal vein, main iliac veins, right external iliac vein. Right renal vein drained to retroperitoneal collaterals. Abdominal tomography confirmed USG. Retrospectively antenatal history revealed enlarged left kidney determined by USG performed right before birth. Based on retroperitoneal collaterals and prenatal USG we think RVT probably began in utero. There was no evidence of sagittal sinus thrombosis. The neonate was treated with LMWH. The results of clotting studies of mother were normal; heterozygot mutation of factor V Leiden and MTHFR gene were found in the baby. Follow-up renal scan at 3 months documented a non-functioning left kidney. Conclusion In neonatal period; when renal vein thrombosis and disseminated thrombosis is detected in the absence of other risk factors, prothrombotic conditions should be searched.

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 Maroteaux–Lamy, 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.