A rare MRI finding of NF-1: perineural arachnoidal gliomatosis.

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.

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

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

Pleural Thickening after Pleural Effusion: How can we Follow-Up in Children?

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.

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

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

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

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

Pterygopalatine Fossa: Not a Mystery!

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.

Unknown vascular compression of the airway in patients with congenitalheart disease and persistent lower respiratory symptoms

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.

Neurocutaneous melanocytosis, hemimegalencephaly and large ovarian cyst in a newborn.

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.

Diagnostic dilemma: osteopetrosis with superimposed rickets causing neonatal hypocalcemia.

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.

Successful multimodal treatment for aggressive metastatic and recurrent fibrolamellar hepatocellular carcinoma in a child.

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.

Radiologic findings of Patterson-Lowry rhizomelic dysplasia in two sisters.

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) inheritance. The present patients help us to understand the genetic relationships and skeletal variabilities of this rare entity.

An unusual cause of renal vein thrombosis in a newborn: COVID-19.

There is no information on renal vein thrombosis induced by COVID-19 infection in a neonate. Few cases of renal vein thrombosis caused by COVID-19 infection have been reported in predominantly adult patients. On day 25 after birth, a newborn whose mother was infected with COVID-19 had renal vein thrombosis. We believed that our patient's renal vein thrombosis was caused by postnatal transmission of the COVID-19 infection that the mother had acquired during birth. The clinical and radiologic findings of these unusual renal complications in a neonate, as well as treatment options, are presented.

Three Different Faces of Schwannoma in Pediatric Patients.

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.

Optic Canal Diameters According to Age in the Pediatric Population.

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

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.

Pigeon breeder`s disease as a cause of hypersensitivity pneumonia in children.

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.

Obstructive jaundice and severe pancreatitis due to the foramen of Winslow hernia with multiple anomalies.

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.

An adolescent case of extensive Behçet`s disease successfully treated with Infliximab.

Isiyel E, Bakkaloglu S, Oguz D, Yenicesu I, Boyunaga Ö, Özdemir Y, Damar Ç, Kandur Y, Akçaboy M, Aslan AT, Sismanlar T, Hasanoglan E, Buyan N. An adolescent case of extensive Behçet`s disease successfully treated with Infliximab. Turk J Pediatr 2019; 61: 585-588. Cardiac involvement is an uncommon and life-threatening complication of Behçet`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 Behçet`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 Behçet`s Disease resistant to conventional treatment.

Management of plexiform neurofibroma with interferon alpha.

Plexiform neurofibroma is a relatively common but potentially devastating manifestation of neurofibromatosis type 1 (NF 1). A substantial number of plexiform neurofibroma causes morbidity. Various treatment modalities are considered to decrease pain. In this paper a case with plexiform neurofibroma causing severe pain and in whom alpha-interferon was used is presented.