Incidental finding of an aberrant left pulmonary artery, an aberrant right subclavian artery, and a tracheal bronchus in an adult woman with a meningioma and a history of medulloblastoma.

The aberrant left pulmonary artery and the aberrant right subclavian artery are rare congenital vascular anomalies, and the tracheal bronchus is a rare congenital respiratory anomaly. A 33-year-old female patient, with a history of desmoplastic medulloblastoma, was surgically treated at our hospital for a meningioma. On the second postoperative day, the patient complained of shortness of breath and chest pain. Contrast-enhanced multislice computed tomography was negative for pulmonary embolism, but incidentally revealed all three congenital anomalies. In our report, we detail this exceedingly rare case.

Recurrent meningitis in children: etiologies, outcome, and lessons to learn.

PURPOSE: Recurrent meningitis in children is a rare condition. However, its early recognition is important in order to prevent serious complications. This study aims to review cases of recurrent meningitis in children. METHODS: This is a retrospective study that included children diagnosed with recurrent meningitis and who were followed at child neurology clinic at the Jordan University Hospital from January 2001 to June 2017. RESULTS: Thirteen patients were included (nine males and four females). Age of first episode of meningitis ranged from 2 months to 9.5 years. The delay in diagnosis of the underlying cause after the first episode ranged from 6 months to 2.5 years. Underlying causes included inner ear malformation in one patient, skull fractures in two, and dermal sinuses (thoracic spinal and occipital dermal sinus) in two patients. No identifiable cause was found in eight patients. Streptococcus pneumoniae was identified in four (31%) patients, Staphylococcus aureus in two (15%), and no organism was isolated in seven (54%). Three patients (23.1%) developed neurological sequel including developmental delay, limb spasticity, and epilepsy. Two patients had sensorineural hearing loss related to meningitis, and two patients had sensorineural hearing loss mostly related to their original disease. CONCLUSION: A detailed history, examination, and thorough investigations are necessary to determine the underlying cause of recurrent meningitis. In addition, in patients with positive CSF bacterial culture, finding the underlying etiology is very likely.

Median pericallosal artery with double proximal origin: case report and clinical consideration.

A 76-year-old male patient underwent magnetic resonance angiography of the head and neck vessels due to a recent incident of transitory ischemic attack. Cerebral angiogram uncovered the double proximal origin of the median unpaired pericallosal artery from the duplicated anterior communicating complex. The vessel curved around the corpus callosum and irrigated the paracentral lobule and the medial parietal cortical areas. The main trunks of the anterior cerebral arteries showed a branching pattern of the marginal callosal arteries, supplying orbital and frontal territories. The pre-communicating segment of the left anterior cerebral artery was identified as hypoplastic. The co-existence of the duplicated anterior communicating artery, with the medial pericallosal artery ascending from it, represents a potential danger for both open and endovascular surgery on the anterior circle of Willis as the deep half of this complex is obscured from the surgeon's eyes. Thorough interpretation of preoperative radiographic images and understanding of the developmental mechanisms of such variability are vital. The described branching arrangement of the anterior communicative region and possible mechanisms of migration with following fusion of the pericallosal arteries are discussed in this paper.

Factors Affecting Attenuation of Dural Sinuses on Noncontrasted Computed Tomography Scan.

BACKGROUND AND PURPOSE: Noncontrasted computed tomography (NCCT) is used as the initial neuroimaging test of choice for patients who present with new-onset neurological symptoms. An apparently hyperattenuated venous sinus may lead to the suspicion of cerebral venous sinus thrombosis (CVST). Improved understanding of all factors that can affect attenuation of dural sinuses can guide triage of patients to or from further investigations of suspected CVST. The purpose of this retrospective study was to assess the effect of different factors including hematocrit (HCT), hemoglobin (Hb), age, BUN/Cr ratio (blood urea nitrogen-to-creatinine ratio), and gender on the attenuation of dural sinuses on brain NCCT. METHODS: A total of 1293 patients with neurological symptoms who presented to the emergency department were included in this study. For each patient, clinical assessment, laboratory investigations, and brain NCCT were reviewed. For each brain NCCT, the average attenuation of superior sagittal sinus and both right and left sigmoid sinuses was measured. RESULTS: Positive significant correlations were found between average attenuation of dural sinuses on one hand and each of age, Hb, and HCT on the other hand. No significant correlation was found between average attenuation and BUN/Cr ratio. Gender discrepancy was also significant as higher attenuation was found in men. CONCLUSION: Age, gender, and Hb levels are the main factors that should be taken into account upon the assessment of dural sinuses on brain NCCT. The highest normal attenuation is predicted in an elderly polycythemic man and the lowest is predicted in a young anemic woman.

Spinal cord infarction in giant cell arteritis associated with scalp necrosis.

Spinal cord infarction is extremely rare in patients with giant cell arteritis (GCA). There are only four case reports in the literature. We describe a 65-year-old man who presented with sudden paraplegia and back pain of 4-days duration with sensory loss below the umbilicus and bilateral scalp necrosis. Magnetic resonance imaging finding was consistent with dorsal spinal cord infarction. Biopsy of the temporal artery confirmed the diagnosis of GCA. The patient was treated with high dose of corticosteroids, which resulted in healing of the scalp ulcerations in 3 weeks, but the paraplegia was irreversible. To our knowledge, this is the first report of spinal cord infarction and simultaneous occurrence of bilateral scalp necrosis in a histopathologically proven GCA. Although literature about spinal cord involvement in GCA is very limited, cord infarction is associated with high mortality and therapeutic challenges since little is understood regarding the pathogenesis that leads to infarction.

Enhancing Diagnostic Accuracy Through Neuroimaging Revisions in Pediatric Pseudotumor Cerebri Syndrome: A Cross-Sectional Study.

BACKGROUND: This cross-sectional study aimed to report all neuroimaging findings suggestive of raised intracranial pressure in children with pseudotumor cerebri syndrome (PTCS), before and after re-review by two neuroradiologists. METHODS: We included 48 children aged <18 years diagnosed with PTCS between 2016 and 2021. Clinical and radiological data were obtained from their medical files. Two neuroradiologists independently re-reviewed all neuroimages, and the average of their assessments was compared with the initial neuroimaging reports; an additional review was done to analyze inter- and intraclass correlation. RESULTS: The initial neuroimaging reports showed under-reporting of findings, with only 26 of 48 (54.1%) patients identified with abnormal reports. After revision, the proportion of the reported findings increased to 44 of 48 (91.6%). Distention of the perioptic space was the most commonly reported finding after revision (36.5 of 48; 76%). Flattening of the posterior globe and empty sella were initially under-reported but improved after revision. Moreover, several findings suggestive of increased intracranial pressure not mandated by Friedman criteria were identified, such as narrowing of the Meckel cave, posterior displacement of the pituitary stalk, and narrowing of the cavernous sinus. Analysis of associations between neuroimaging findings and demographic and clinical characteristics yielded no statistically significant results. The inter- and intraclass correlation results demonstrated a significant agreement between raters and within each rater's assessment (P < 0.05). CONCLUSIONS: This study highlights the impact of image revision in enhancing PTCS diagnosis. Intra- and interclass correlations underscore the reliability of the review process, emphasizing the importance of meticulous image analysis in clinical practice.

Loss of CHSY1, a secreted FRINGE enzyme, causes syndromic brachydactyly in humans via increased NOTCH signaling.

We delineated a syndromic recessive preaxial brachydactyly with partial duplication of proximal phalanges to 16.8 Mb over 4 chromosomes. High-throughput sequencing of all 177 candidate genes detected a truncating frameshift mutation in the gene CHSY1 encoding a chondroitin synthase with a Fringe domain. CHSY1 was secreted from patients' fibroblasts and was required for synthesis of chondroitin sulfate moieties. Noticeably, its absence triggered massive production of JAG1 and subsequent NOTCH activation, which could only be reversed with a wild-type but not a Fringe catalytically dead CHSY1 construct. In vitro, depletion of CHSY1 by RNAi knockdown resulted in enhanced osteogenesis in fetal osteoblasts and remarkable upregulation of JAG2 in glioblastoma cells. In vivo, chsy1 knockdown in zebrafish embryos partially phenocopied the human disorder; it increased NOTCH output and impaired skeletal, pectoral-fin, and retinal development. We conclude that CHSY1 is a secreted FRINGE enzyme required for adjustment of NOTCH signaling throughout human and fish embryogenesis and particularly during limb patterning.

Phenotypical Variability of the Internal Acoustic Canal in the Middle Cranial Fossa Surgery.

Introduction The wide range of anatomical variability of the structures of the middle cranial fossa (MCF) and the lack of reliable surgical landmarks contribute to a high level of complications in the surgical treatment of vestibular schwannomas. We hypothesized that the cranial phenotype influences the shape of the MCF, the orientation of the pyramid of the temporal bone, and the relative topography of the internal acoustic canal (IAC). Methods The skull base structures were studied on 54 embalmed cadavers and 60 magnetic resonance images of the head and neck by photo modeling, dissection, and three-dimensional analysis techniques. By the value of the cranial index, all specimens were subdivided into dolichocephalic, mesocephalic, and brachycephalic groups for comparison of variables. Results The length of the superior border of the temporal pyramid (SB), the apex to squama distance, and the width of the MCF all peaked in the brachycephalic group. The value of the angle between the SB and the axis of the acoustic canal varied from 33 to 58 degrees; it peaked in the dolichocephalic group and showed its smaller value in the brachycephalic one. The pyramid to squama angle had reversed distribution and dominated in the brachycephalic group. Conclusion The cranial phenotype influences the shape of the MCF, temporal pyramid, and IAC. Presented in this article data help specialists operating on the vestibular schwannoma to localize the IAC based on the individual shape of a skull.

Presentation of a large pseudoaneurysm of the brachial artery: A case report.

Brachial artery pseudoaneurysms (PSA) are considered uncommon, but they can be limb and life threatening. Most etiologies are related to iatrogenic injury or following a fracture. Here we present a 37-year-old male complaining of a large swelling in his right upper arm that was gradually increasing in size for the last 7 months. It started with a sudden onset of pain while lifting heavy boxes at his daily job. Ultrasound and MRA showed a pseudoaneurysm of the right brachial artery. Resection of the PSA was performed with a vein interposition graft. This case illustrates the significance of considering PSA as a differential diagnosis in patients presenting with upper arm swelling without history of obvious trauma.

Pituitary abscess presenting two years after the diagnosis of a pituitary lesion in a patient with panhypopituitarism.

Pituitary abscess is a rare condition. Here, we present the case of a young male patient who was initially found to have a pituitary lesion following the diagnosis of panhypopituitarism. Two years later, he presented with severe headache and was subsequently diagnosed intraoperatively with pituitary abscess. At a follow-up of 6 years after surgery, the patient was continuing to do very well. We discuss the differential diagnosis and demonstrate the evolution of the pituitary lesion on magnetic resonance imaging at four different time points: at the time of the detection of the initial lesion; two years later at the time of the diagnosis of the pituitary abscess; at 7 weeks post operatively; and finally after six years from the pituitary surgery.

Pseudotumor cerebri syndrome in children: Clinical characteristic and re-classification.

AIM: To describe the clinical characteristics of children with pseudotumor cerebri syndrome (PTCS) who were diagnosed according to the modified Dandy criteria and to reclassify them according to the newly proposed diagnostic criteria by Freidman. METHODOLOGY: This retrospective study included the period from January 2016-to July 2021. RESULTS: 50 patients were included; 34 males and 16 females with a male to female ratio of 2.1:1. The average age at onset of symptoms was 8 years. Obesity was noticed in 6 (12%) patients; 34 (68%) had symptoms upon presentation. The most common presenting symptom was headache (28 patients; 56%), papilledema was present in 33 (66%) patients. Most patients (37; 74%) had an initial cerebrospinal fluid (CSF) pressure ≥280 mmH2O. At last follow-up, papilledema resolved in 11/32 (34.3%) patients, and headache resolved in 17/23 (74%) patients. 22/50 (44%) patients fulfilled the definite criteria proposed by Freidman, 11/50 (22%) fulfilled the probable, 10/50 (20%) were categorized as possible, and 7 (14%) patients were categorized as unmet. CONCLUSION: PTCS is a chronic condition. Managing patients who do not have papilledema or who do not meet the newly proposed higher CSF pressure is challenging. Although, applying the newly proposed criteria captured most of our patients, however, around one quarter were managed based on clinical experience. This study indicates a strong need for future guidelines tailored specifically for children, taking into consideration that the cut-off point of CSF pressure might not be similar for all populations.

Is autosomal recessive Silver-Russel syndrome a separate entity or is it part of the 3-M syndrome spectrum?

Intrauterine growth retardation (IUGR) is a nonspecific finding that occurs in approximately 0.17% of all live-births. However, IUGR can also be a significant feature of many recognized genetic syndromes including Silver-Russel syndrome (SRS), Three M syndrome (3-M), Dubowitz syndrome, and Mulibrey nanism. Differentiation of 3-M syndrome from autosomal recessive SRS has been difficult because of the phenotypic variability of the latter. Limb length asymmetry is seen in over half of those with autosomal recessive SRS, but not in individuals with 3-M syndrome. Characteristic radiologic findings of 3-M syndrome are not present in SRS. We used single nucleotide polymorphism (SNP) microarrays to investigate the cause of phenotypic features of SRS that shows autosomal recessive inheritance in three consanguineous families, two from United Arab Emirates (UAE), and one from Jordan. The mapped regions contained CUL7 and OBSL1, the genes that have recently been shown to cause 3-M syndrome. Subsequently, direct DNA sequencing of CUL7 and OBSL1 genes revealed novel mutations in both genes including two mutations in OBSL1 [c.1119G>C (p.W373C) and c.681_682delinsTT (p.Q228X)], and a nonsense mutation in CUL7 [c.203G>A (p.W68X)]. In addition, a six nucleotide deletion in CUL7 [c.649_654delAGCCGC (p.217_218delSR)] was found in a consanguineous family from UAE that had the typical features of 3-M. As a result of these findings, we question the identity of the autosomal recessive SRS and suggest that all apparently recessive SRS families should be tested for mutations in CUL7 and OBSL1.

Bilateral very large calcium oxalate stones in the seminal vesicles: case report and literature review.

A 29-year-old man with hypospadias and bilateral undescended testicles presented with recurrent attacks of lower urinary tract infections and painful ejaculation. He was diagnosed to have very large bilateral seminal vesicle stones. The pelvis X-ray showed two radio-densities located in the pelvis with symmetrical appearance while Ultrasound showed them as echogenic structures with posterior acoustic shadowing. In magnetic resonance imaging (MRI) of the pelvis they appeared hypointense in both T1-weighted and T2-weighted images while pelvic computed tomography scan (CT scan) showed bilateral huge stones in the seminal vesicles. The stones were extracted by open surgery through the bladder after transurethral excision of the ejaculatory ducts. Here, we report the first case of bilateral, large, heart-shaped, calcium oxalate monohydrate of seminal vesicle caliculi with brief literature review.

Clinical and radiological features of Takayasu's arteritis patients in Jordan.

The prevalence of Takayasu's arteritis (TA) varies greatly among world populations, and little is known about this disease in Eastern Mediterranean Arab populations. We conducted a retrospective chart review of patients diagnosed with TA from 1996 to 2008 at a single large referral center in Jordan. Eight patients (seven females, one male) with angiographically diagnosed TA were seen at the Jordan University Hospital between 1996 and 2008. All patients were of Arabic ethnicity. The age at presentation ranged from 14 to 50 years, and delay in diagnosis ranged from 1 to 10 years. Extra-vascular manifestations included nodular episcleritis, elevated liver enzymes, erythema nodosum, inflammatory-bowel-disease-like illness, Raynaud's phenomena, and constitutional symptoms. Vascular symptoms included postural dizziness, central nervous system deficits, amauroses fugax, and transient ischemic attacks. Aortic arch vessels were involved in all patients, the abdominal aorta was involved in five patients, and the renal arteries in four patients. Major clinical events including severe stroke and cardiac failure were associated with mortality in two patients. Treatment with corticosteroids and immunosuppressive agents resulted in improvement in five patients with follow-up ranging from 3 to 12 years. In conclusion, TA is seen in Arabs, and the clinical spectrum of TA in Arabs in Jordan is similar to that reported in other countries. Increased awareness of the disease may shorten the time to diagnosis and result in a more reliable estimate of disease prevalence.

Polyarthritis with chondronecrosis associated with osteonecrosis, panniculitis and pancreatitis.

Arthritis associated with panniculitis complicating pancreatitis is well described in the literature, usually associated with osteonecrosis. Chondronecrosis has not been reported before in association with pancreatitis. We report a patient with chronic pancreatitis who presented with polyarthritis, panniculitis, osteonecrosis, but in addition had clear evidence of chondronecrosis. We suggest that direct extension of noxious materials from nearby subchondral osteonecrotic bone lesion could be the cause of the osteonecrosis and one of the pathological mechanisms leading to arthritis in patients with pancreatitis.

Aneurysmal bone cyst concomitant with fibrous dysplasia in the frontal bone.

Secondary aneurysmal bone cyst in fibrous dysplasia is exceedingly rare, especially in the skull and particularly in the frontal bone. We present a case of aneurysmal bone cyst concomitant with fibrous dysplasia in the frontal bone in a 15-year-old male patient presenting with headache and euphoria with an uncharacteristic imaging appearance and treated successfully by total resection.

Descriptive epidemiological analysis, MRI signals intensity and histopathological correlations of meningiomas.

OBJECTIVES: To present our experience in operated meningioma cases regarding their prevalence, anatomical location, multiplicity, presenting signs and symptoms, and the possible correlation between MRI signal intensity and histological grades to set criteria for radio-pathological diagnosis. METHODS: In this retrospective study, operated meningioma cases in the Department of Neurosurgery, Jordan University Hospital (JUH), Amman, Jordan between January 1997 and January 2007 were reviewed. Our study included 90 cases, and their medical records, histopathological reports, and neuroimages were analyzed thoroughly. RESULTS: Meningioma was more common in females than males with a ratio of 2.2:1. Para-sagittal meningiomas were the most common (23.3%). Multiple intracranial meningiomas were found in 4.4% of the cases. Most cases were of benign histopathology and exhibited iso-intense signals on T1 and T2, and appeared with hyper-intense signals on FLAIR with vivid enhancement. CONCLUSION: The prevalence of meningioma among genders and its anatomical location at JUH corresponds to the published medical literature worldwide. There was no correlation between signal intensities (as seen on T1WI, T2WI, and FLAIR sequences), enhancement pattern on one side, and histological grades on the other side.

Pituitary magnetic resonance imaging experience in Jordan.

OBJECTIVE: To assess the pituitary findings as demonstrated on MRI and to compare the results with the data published in the literature. METHODS: One thousand, one hundred and thirty-eight pituitary MRI`s with and without intravenous contrast media (gadolinium) were performed over 6 years from 2001 to 2007 in the Department of Diagnostic Radiology, Jordan University Hospital, Amman, Jordan. The patients were referred from various departments and were evaluated for pituitary, other sellar, and juxtasellar abnormalities. The results were compared with those in the published literature. RESULTS: Four hundred and eight-three normal scans were excluded from the study. The remaining 655 were abnormal, pituitary adenoma was detected in 327 (49.9%), microadenoma was present in 213 (32.5%), and macroadenoma in 114 (17.4%). Partial empty sella was seen in 157 (24%), diffuse pituitary gland enlargement in 98 (14.9%), ectopic pituitary posterior lobe in 13 (2%), and other findings in 31 (4.7%). CONCLUSION: The incidence of pituitary adenoma was equal in both genders; however, microadenoma was more common, affected a younger age group, and was predominately seen in females. The other parameters showed agreement with the published literature.

Pseudoaneurysm of the breast after blunt trauma: successful treatment with ultrasound-guided compression.

We present a case of breast pseudoaneurysm following a blunt trauma in a 58-year-old woman. Few cases of breast pseudoaneurysm have been reported in the literature, and most of these are related to previous interventional procedures. Pseudoaneurysm was suspected on real-time sonography and confirmed with color Doppler and spectral wave analysis, which revealed a characteristic to-and-fro pattern. Unlike previously reported cases, treatmentwith ultrasound-guided compression was successful.