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.

Persistent craniopharyngeal canal, bilateral microphthalmia with colobomatous cysts, ectopic adenohypophysis with Rathke cleft cyst, and ectopic neurohypophysis: case report and review of the literature.

INTRODUCTION: Persistent craniopharyngeal canal (PCC) is a rare anomaly of the base of the skull. PCC is defined as a well-corticated osseous canal, extending from the roof of the nasopharynx to the base of the sella over the course of the sphenoid corpus. We reported a PCC case with unique associations. The magnetic resonance imaging findings are discussed. CASE PRESENTATION: We report a case of a 2-year-old boy with persistent craniopharyngeal canal, bilateral microphtalmia with large colobomatous cyst, and ectopic adenohypophysis with Rathke cleft cyst. He also has ectopic neurohypophysis and optic atrophy. CONCLUSION: The presence of orbital and optic tract malformations, craniofacial and intracranial anomalies, and tumors can accompany craniopharyngeal canals (CCs). MRI is helpful in the evaluation of PCC and associated anomalies in these patients.

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.

A Newborn with an Alternative Porto-Caval Shunt.

BACKGROUND: Absent ductus venosus (ADV) is a rare condition, but it should be known that this embryonic anomaly may be detected by fetal echocardiographic or newborn ultrasound examinations. CASE REPORT: We present a baby with an ADV and an accompanying alternative porto-caval shunt between the right portal vein and inferior vena cava detected on postnatal ultrasound examination. CONCLUSIONS: Variations in the fetal umbilical or porto-systemic circulations should be detected by fetal or newborn ultrasound examinations and kept in mind before common interventions such as UV catheterizations.

Association between deep neck space abscesses and internal carotid artery narrowing in pediatric patients

Background/aim: Our aim was to interpret the effects of deep neck space abscesses on the adjacent carotid artery according to abscess location, as well as to determine narrowing by calculating the mean stenosis ratios.Materials and methods: Neck computed tomography scans and clinical data of 45 children with neck abscesses were evaluated retrospectively for abscess location and internal carotid artery narrowing. The lumen areas of the carotid arteries were measured from standard levels, and stenosis ratios were calculated with two different techniques. The mean stenosis ratios of each group according to abscess location were then compared with the control group.Results: Among the 45 abscesses included in the study, 51.1% (n = 23/45) were located in the peritonsillar region, 37.8% (n = 17/45) were located in the parapharyngeal-lateral retropharyngeal space, and 11.1% (n = 5/45) were in the midline retropharyngeal space. We found a statistically significant difference between the mean stenosis ratios of the ipsilateral side of the parapharyngeal-lateral retropharyngeal abscesses and the control group (P < 0.01).Conclusion: The children with parapharyngeal-lateral retropharyngeal abscesses all had narrowing in the adjacent carotid lumen to some degree. Although most of the patients had no clinical symptoms, radiologists have to be aware of this arterial complication to prevent further progress and fatal complications.

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.

Imaging features of Burkitt lymphoma in pediatric patients.

Burkitt lymphoma is an aggressive and rapidly growing tumor that is curable and highly sensitive to chemotherapy. It can affect almost every tissue in the body, producing various clinical presentations and imaging appearances, according to the predilection of the different subtypes for certain sites. Awareness of its diagnostically specific imaging appearances plays an important role in rapid detection and treatment. In this pictorial review, we aimed to identify the most common imaging features of Burkitt lymphoma in pediatric patients.

Juvenile Nasopharyngeal Angiofibroma: Magnetic Resonance Imaging Findings.

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.

Diagnostic performance of heavily T2-weighted techniques in obstructive hydrocephalus: comparison study of two different 3D heavily T2-weighted and conventional T2-weighted sequences.

PURPOSE: To evaluate efficacy of three-dimensional (3D) heavily T2-weighted (W) MRI sequences in assessment of cerebrospinal fluid (CSF) pathways and to compare two different types of 3D heavily T2W MRI sequences (CISS and SPACE) with two-dimensional (2D) T2W turbo spin echo (TSE) sequences for hydrocephalus with intraventricular obstruction. MATERIALS AND METHODS: Sixty-two patients who were diagnosed with intraventricular obstructive hydrocephalus, according to clinical and radiological findings, were included in this retrospective study. 2D-TSE-T2, 3D-CISS, and 3D-SPACE, which are part of the protocol, were analyzed quantitatively by measuring ventricle-to-parenchyma contrast-to-noise ratio (CNR), and qualitatively by evaluating the capabilities of visualization of the obstructive pathology, overall image quality, severity of artifacts, and delineation of the CSF pathways. One-way ANOVA and Friedman's test were used for statistical analysis. RESULTS: CNR between CSF and brain parenchyma was significantly higher using 3D-SPACE sequences compared with 3D-CISS and 2D-TSE-T2 sequences. The qualitative findings showed that 3D heavily T2W sequences were superior to 2D-TSE-T2 sequences. 3D-SPACE sequences showed fewer artifacts than 3D-CISS or 2D-TSE-T2 sequences. CONCLUSION: 3D heavily T2W sequences are necessary tools for assessment of CSF pathways in patients with intraventricular obstructive hydrocephalus. 3D-SPACE sequences allowed heavy T2W, which is necessary for CSF flow imaging and provided significantly fewer image artifacts and improved CNR in comparison with 3D-CISS sequences.

Evaluation of ovarian reserve based on hormonal parameters, ovarian volume, and antral follicle count in women with type 2 diabetes mellitus.

OBJECTIVE: The aim of this study was to evaluate ovarian reserve of women with type 2 diabetes mellitus (T2DM). METHODOLOGY: Eighty-nine women with T2DM and 73 healthy controls were enrolled and divided into three age groups [group 1 (20-29 yr), seven diabetics and 18 healthy controls; group 2 (30-39 yr): 35 diabetics and 35 healthy controls; and group 3 (40-49 yr): 47 diabetics and 20 healthy controls]. All participants were subjected to transvaginal ultrasonographic examination on the third day of their menstrual periods for the determination of ovarian volume and total antral follicle count (AFC). RESULTS: A significant difference in mean FSH levels (international units per liter) was observed between women with diabetes and healthy controls in all age groups (group 1, 7.8 ± 0.9 vs. 5.0 ± 1.0; group 2, 8.2 ± 1.1 vs. 7.2 ± 1.8; group 3, 9.5 ± 3.2 vs. 6.4 ± 2.4; P < 0.001 for all). Similarly, mean AFC was significantly lower in patients with T2DM than in healthy controls in all age groups (group 1, 21.1 ± 4.8 vs. 25.0 ± 9.1; group 2, 10.4 ± 5.2 vs. 23.0 ± 9.5; group 3, 6.0 ± 3.5 vs. 21.7 ± 2.1; P < 0.001 for all). A statistically significant difference in total ovarian volume was only observed in group 1 (9.7 ± 3.0 in T2DM patients vs. 6.8 ± 2.7 in healthy controls; P = 0.002). AFC was found to be negatively correlated with FSH (r = -0.406, P < 0.001), age (r = -0.618, P < 0.001), glycolized hemoglobin (r = -0.505, P < 0.001), and fasting blood glucose (r = -0.687, P < 0.001). CONCLUSION: In this pioneer study, the first to evaluate ovarian reserve in T2DM patients, we managed to demonstrate lower ovarian reserves in women with diabetes compared with healthy controls.