Intradiploic orbital roof meningioma with pneumosinus dilatans in a child: a case report and review of the literature

Intradiploic meningioma of the orbital roof is a type of intraosseous meningioma. It is very rare in children, and only 7 cases have been reported in the pediatric age group. We report a case of a 14-year-old female who presented with progressive exophthalmos, diplopia and pain involving the right eye. Computed tomography (CT) scan and magnetic resonance imaging (MRI) revealed an intradiploic orbital roof tumor with diploe expansion and pneumosinus dilatans. The tumor was completely removed surgically. The histopathological diagnosis was transitional meningioma. The clinical and radiological findings of intadiploic meningioma are discussed with a review of the relevant literature.     

Atypical meningioma and extensive calvarium defects in neurofibromatosis type 1

A 9-year-old girl with neurofibromatosis type 1 (NF1) presented with a massive atypical meningioma and calvarial defect. Skull radiographs and cranial CT showed an extensive lytic bone lesion at the vertex. MRI demonstrated a large mass invading the calvarium and sagittal sinus. The histopathological and immunohistochemical diagnosis of the resected mass was atypical meningioma. To our knowledge, this is the first case of NF1 associated with atypical meningioma and massive calvarial defect in a child.     

Geniculate ganglion meningioma

Primary ectopic meningiomas are rare, but may be seen in the head and neck region. The temporal bone and its neural foramen are rarely the site of a primary meningioma. This report describes the CT and MRI appearance of an ectopic meningioma arising at the anatomic location of the geniculate ganglion, and discusses the differential diagnosis as well as the possible origin of the tumor. Received: 24 February 1997 Accepted: 21 April 1997     

Progression of bone lesions in a child with primary hyperoxaluria type 1: evaluation by roentgenology and MRI

We report the radiological and MRI findings of bone oxalosis and their rapid and extensive progression in a child with primary hyperoxaluria (PH) type 1. We emphasize the spectrum of skeletal changes and their differences from renal osteodystrophy and the role played by imaging in the diagnosis and follow-up of oxalosis.

     

Cyst-like lesions following fracture in a child — sonographic appearances

Cyst-like lesions are a rare radiologic presentation following fractures in children. We report the case of a 3-year-old boy having three cyst-like lesions after a buckle fracture of his left radius that was diagnosed with digital radiography and CT scan. We also describe the lesions’ ultrasonographic appearance, which has not been addressed in other studies. The keys to diagnosis depend on the clinical context and the radiologic appearance. The cyst-like lesions occur several weeks after a non-displaced fracture in an asymptomatic child. The radiologic appearance shows small, well-defined lucent lesions located near the fracture site, in the subperiosteal space and bone callus. Ultrasonography can be useful to confirm the subperiosteal location and to exclude adjacent soft-tissue abnormalities. In cases requiring further clarity, MRI can be performed to identify the fatty content and exclude osteomyelitis.

     

Variability of T1-weighted signal intensity of pericallosal lipomas in the fetus

Background

Pericallosal lipomas are often associated with corpus callosum dysgenesis. The diagnosis of lipoma, suggested on ultrasonography, relies on the classic T1 hyperintensity on magnetic resonance imaging (MRI). However, this feature may be absent prenatally.

Objective

Our objective was to study the changes of T1 intensity in fetal lipomas with comparison to postnatal/postmortem data and to assess the factors influencing the signal variations of pericallosal lipomas on prenatal MRI.

Materials and methods

Patients with callosum dysgenesis and interhemispheric hyperechogenicity suggestive of a pericallosal lipoma with available postnatal or postmortem data were included. Gestational age, lipoma size and pattern, corpus callosum size and changes in fetal fat T1 intensity were recorded. Comparison with postmortem neuropathology was available for one fetus.

Results

Eleven patients with callosum dysgenesis and pericallosal lipomas (seven curvilinear and four tubulonodular) were included. All MRI scans were performed in the third trimester. Curvilinear lipomas were thinner and six cases were associated with prenatal T1 iso-intensity. Typical T1 hyperintensity appeared on postnatal MRI only. All tubulonodular lipomas were much larger and showed prenatal T1 hyperintensity. In two patients, the lipoma increased in size on postnatal MRI.

Conclusion

The type and size of a lipoma influence T1 prenatal intensity. Absence of T1 intensity was observed in curvilinear lipomas only. Curvilinear lipomas are much thinner. Changes in T1 intensity may also be related to fat maturation within the lipoma and, subsequently, to gestational age. In the case of callosum dysgenesis, absence of prenatal T1 pericallosal hyperintensity should not exclude the diagnosis of pericallosal lipoma.

     

Intra-articular venous malformation of the knee in children: magnetic resonance imaging findings and significance of synovial involvement

Background

Intra-articular venous malformations of the knee are an uncommon cause of unilateral knee pain in children. Timely diagnosis is important because lesions with intrasynovial involvement can lead to joint space hemorrhage and secondary cartilage damage.

Objective

To describe our tertiary center’s experience of diagnostics and typical magnetic resonance imaging (MRI) findings.

Materials and methods

A retrospective review of all patients ≤16 years of age managed for intra-articular venous malformations of the knee at our institution between 2002 and 2018.

Results

Of 14 patients (8 male), the mean age at presentation was 6 years (range: 0–14 years). The most common clinical findings were unilateral knee pain (93%), joint swelling (79%), quadriceps atrophy (50%) and a limited range of motion (29%). Cutaneous manifestations were present in four patients (29%). Contrast-enhanced MRI was available in all cases. After initial MRI, a vascular anomaly etiology had been identified in 11 cases (79%), and correctly reported as a venous malformation in 6 (55%). Three patients received entirely different diagnoses (arthritis, tumor or pigmented villonodular synovitis). Three of seven patients with intrasynovial lesions had established chondropathy at diagnosis. Two patients with lesions of the suprapatellar fat pad had intrasynovial involvement that was not visualised on MRI.

Conclusion

Although MRI usually permits the diagnosis, clinical awareness of these lesions is important for optimal imaging, accurate interpretation and timely diagnosis. Involvement of the intrasynovial cavity carries a risk of hemarthrosis and progressive chondropathy that may be underestimated by MRI.

     

Imaging diagnosis of retroperitoneal ganglioneuroma in childhood

Purpose. To demonstrate the typical appearance of retroperitoneal ganglioneuromas on CT and MRI.¶Materials and methods. Retrospective analysis of diagnostic imaging (five CT scans, three MRI scans) in five children aged 3–15 years with the histological diagnosis of ganglioneuroma.¶Results. The scans showed large (maximum 11 cm diameter), round or oval tumours with sharply defined margins. Intraspinal tumour involvement occurred in two cases. Comparing CT with MRI, MRI was more accurate in defining the intraspinal involvement. The ganglioneuromas were hypodense on unenhanced CT and showed moderate enhancement with administration of contrast medium. In three patients, CT demonstrated tumour calcification with a disseminated speckled pattern. On T1-weighted MRI the tumours were homogeneous and hypointense, showing marked enhancement after gadolinium administration. On T2-weighted scans the tumours were hyperintense.¶Conclusion. At the time of diagnosis, retroperitoneal ganglioneuromas are generally large tumours that can be shown well by CT and MRI. The appearance on CT more readily suggests the diagnosis, but MRI is superior for documenting local or intraspinal tumour extension and lacks radiation load.     

The DWI ‘reversal sign’ of white matter hypoxic ischaemic injury in older children: an unusual MRI pattern for age

We present two children beyond the neonatal and infant age who suffered global hypoxic events and showed an MRI appearance of reversal of the diffusion-weighted (DWI) and apparent diffusion coefficient (ADC) signal involving exclusively the white matter. This is an unusual distribution for this age group and may represent delayed postanoxic leukoencephalopathy. The appearance of this type of insult has been described as occurring in younger children more frequently than in adults. Awareness of this condition, the fact that it may occur earlier, and the peculiar and possibly deceptive DWI/ADC signal reversal pattern exclusively involving the white matter is critical for making a correct diagnosis and giving a prognosis.     

Congenital ganglioglioma: report of a case with an unusual imaging appearance

A newborn presented with a cerebral ganglioglioma. This is a rare diagnosis in a neonate. The imaging appearance (hyperdense on CT, hyperintense on T1-weighted and hypointense on T2-weighted MRI) is very unusual for this type of tumor. Received: 10 December 1996 Accepted: 24 March 1997     

Prenatal MRI diagnosis of fetal cerebral tuberous sclerosis

Tuberous sclerosis (TS) is an autosomal dominant phakomatosis. A high percentage of spontaneous mutations leads to the diagnosis of new cases in normal families. This diagnosis is suspected at antenatal ultrasound on the discovery of multiple cardiac tumors. Antenatal cerebral ultrasound shows a normal appearance in affected fetuses. Eight fetuses with multiple cardiac tumors were studied with antenatal MRI with, in five cases, an abnormal appearance showing hyperintense subependymal and cortical nodules on TI-weighted images. Among the three remaining patients MRI was non-contributive in one due to movement artefact, one had abnormal postnatal MRI consistent with TS and one had a normal postnatal and clinical examination. We conclude that MRI is a valuable tool in making the diagnosis of TS in fetuses with multiple cardiac tumors.     

Paediatric presentation of type 2 neurofibromatosis

BACKGROUND—Neurofibromatosis type 2 (NF2) is a highly penetrant autosomal dominant condition predisposing affected individuals to schwannomas and meningiomas. The proportion of children presenting with meningioma or schwannoma who have NF2 is not well described, and neither is the mode of presentation in most children with the inherited disease.
AIMS—To determine the frequency of childhood meningioma and schwannoma cases caused by NF2 and the mode of presentation.
METHODS—The records of the Manchester Children''s Tumour Registry from 1954 were searched for cases of meningioma and schwannoma. Paediatric presentation in a large UK series of NF2 was also studied.
RESULTS—18% (61/334) of patients with NF2 on the UK database presented in the paediatric age group (0-15 years), frequently with the symptoms of an isolated tumour. More than half had no family history to alert the clinician to their susceptibility. Three of 22 children presenting with a meningioma on the Manchester Children''s Tumour Registry have gone on to develop classic features of NF2.
CONCLUSIONS—Clinicians should suspect NF2 in children presenting with meningioma, schwannoma, and skin features, such as neurofibromas/schwannomas, but fewer than 6 café au lait patches, who thus fall short of a diagnosis of neurofibromatosis type 1.
     

Cystic metastatic cerebral neuroblastoma

Intracerebral neuroblastoma is an exceedingly rare manifestation of intracranial neuroblastoma and has been described as a solid lesion. Cystic metastatic intracerebral neuroblastoma has not been described. We report a case of histologically proven metastatic intracerebral neuroblastoma presenting in a cystic form 1 year after diagnosis and treatment of a solid intra-abdominal neuroblastoma. The imaging and clinical appearance of this case was indistinguishable from that of intracerebral cystic lesions caused by infection or inflammation. It has been suggested that, due to more aggressive treatment of neuroblastoma, patients are surviving longer and consequently intracranial involvement is becoming more frequent. It is important to be aware of this cystic manifestation of the disease.

     

Safety of repeated hyperpolarized helium 3 magnetic resonance imaging in pediatric asthma patients

Background

Hyperpolarized helium 3 magnetic resonance imaging (³He MRI) is useful for investigating pulmonary physiology of pediatric asthma, but a detailed assessment of the safety profile of this agent has not been performed in children.

Objective

To evaluate the safety of ³He MRI in children and adolescents with asthma.

Materials and methods

This was a retrospective observational study. ³He MRI was performed in 66 pediatric patients (mean age 12.9 years, range 8–18 years, 38 male, 28 female) between 2007 and 2017. Fifty-five patients received a single repeated examination and five received two repeated examinations. We assessed a total of 127 ³He MRI exams. Heart rate, respiratory rate and pulse oximetry measured oxygen saturation (SpO₂) were recorded before, during (2 min and 5 min after gas inhalation) and 1 h after MRI. Blood pressure was obtained before and after MRI. Any subjective symptoms were also noted. Changes in vital signs were tested for significance during the exam and divided into three subject age groups (8–12 years, 13–15 years, 16–18 years) using linear mixed-effects models.

Results

There were no serious adverse events, but three minor adverse events (2.3%; headache, dizziness and mild hypoxia) were reported. We found statistically significant increases in heart rate and SpO₂ after ³He MRI. The youngest age group (8–12 years) had an increased heart rate and a decreased respiratory rate at 2 min and 5 min after ³H inhalation, and an increased SpO₂ post MRI.

Conclusion

The use of ³He MRI is safe in children and adolescents with asthma.

     

Spine evaluation in children with anorectal malformations

The value of radiography, ultrasound and magnetic resonance imaging (MRI) in detecting skeletal and intraspinal pathology was assessed in infants with anorectal malformations, and the need for spinal MRI examination in this group of patients was evaluated. Twenty-one infants were examined with radiography, ultrasound and MRI of the lower spine. The detection of skeletal and intraspinal abnormalities was compared for the three imaging modalities. Fifteen patients were normal in all three examinations. Radiography showed bony skeletal abnormalities in six children, although sometimes very subtle. By ultrasound both bony and cartilaginous malformations were detected in all six patients and by MRI in five patients. Five of the six children with skeletal abnormalities had intraspinal pathology, detected in all cases by ultrasound and MRI. However, the abnormalities were more clearly demonstrated by MRI than by ultrasound. Spinal radiographs must be examined carefully for abnormalities, because they can indicate the presence or absence of intraspinal pathology. Normal radiographic and sonographic appearance of spinal anatomy in children with anorectal malformation makes MRI superfluous, but if radiographs or ultrasound are abnormal, MRI should be used to accurately depict possible intraspinal pathology.

     

Primary diffuse leptomeningeal glioneuronal tumors of the central nervous system: Report of three cases and review of literature

Abstract

Primary diffuse leptomeningeal glioneuronal tumors (DLGNT) are rare tumors, recently recognized as a unique entity based on their unique pathologic and clinical characteristics. We report three cases of DLGNT and compare their clinical characteristics and presentation with other reported cases, and with primary leptomeningeal gliomatosis. Because their prognosis is better than that of diffuse leptomeningeal gliomatosis, and pathologic diagnosis may be difficult, clinicians should consider this diagnosis in patients who present with new neurological symptoms, hydrocephalus and diffuse leptomeningeal enhancement on MRI. Further studies are required to better understand the unique biological characteristics of these tumors and to improve therapy.     

Mesenteric plexiform neurofibroma associated with Recklinghausen's disease

We present a rare case of plexiform neurofibroma involving the ileal mesentery of a 10-year-old boy with Recklinghausen's disease. MRI showed multiple ring-like structures in the masses on T2-weighted and contrast enhanced T1-weighted images. Probably these findings reflect enlarged peripheral nerves with myxoid degeneration. MRI is useful for the diagnosis of mesenteric plexiform neurofibroma because of this characteristic appearance. Received: 19 June 1995 Accepted: 8 March 1996     

Fetal bowel anomalies--US and MR assessment

The technical quality of prenatal US and fetal MRI has significantly improved during the last decade and allows an accurate diagnosis of bowel pathology prenatally. Accurate diagnosis of bowel pathology in utero is important for parental counseling and postnatal management. It is essential to recognize the US presentation of bowel pathology in the fetus in order to refer the patient for further evaluation or follow-up. Fetal MRI has been shown to offer some advantages over US for specific bowel abnormalities. In this paper, we review the normal appearance of the fetal bowel on US and MRI as well as the typical presentations of bowel pathologies. We discuss more specifically the importance of recognizing on fetal MRI the abnormalities of size and T1-weighted signal of the meconium-filled distal bowel.     

Magnetic resonance imaging of the hypothalamic-pituitary axis in the diagnosis of growth hormone deficiency

In order to investigate the relationship between pituitary appearance and the diagnosis of growth hormone deficiency (GHD), we have assessed magnetic resonance imaging (MRI) scans and GH status during provocation tests in 110 patients (78 males; median age 9.8, range 0.1-20 yr), evaluated for possible GH disorders. On the basis of pituitary function tests, patients were divided into GH deficient (GH peak < 15 mIU/l [5.8 ng/ml]) (n = 82) or GH sufficient (GH peak > 15 mIU/l) (n = 28). The former were further divided into those with multiple hormone deficits (MPHD) (n = 19) or isolated GHD - severe IGHD (peak GH < 8 mIU/l [3.1 ng/ml]) or partial IGHD (8-15 mIU/l). The appearance of the hypothalamic-pituitary (H-P) axis was classified as: (1) normal, (2) isolated hypoplastic stalk (HPS), (3) isolated hypoplastic anterior lobe (HPAL) (PHT SDS < -2.0), (4) HPS + HPAL or (5) ectopic posterior lobe (EPL). The last two were considered severe abnormalities. PHT SDS (mean +/- SD -2.0 +/- 2.2) was correlated to log peak GH levels in the whole group (r = 0.45; p < 0.0001) and in the GHD group (r = 0.39; p < 0.0001). Sixty-five out of 82 in the GHD group had a H-P axis abnormality (45 severe abnormalities), while 13 out of the 28 patients in the GH sufficient group also had an abnormality (3 severe, but none with an EPL). All patients with MPHD had a MRI abnormality, most commonly an EPL (79%). Thus the presence of any MRI abnormality as a marker for GHD would generate a sensitivity of 79%, but a specificity of only 54%, indicating that this could not be used to confirm GHD. However, the presence of either an EPL or HPS + HPAL on MRI is highly specific (100% and 89% respectively) and predictive of GHD (positive predictive value 100% and 79% respectively), indicating that these abnormalities provide confirmation of the diagnosis. We recommend that if clinical, auxological and biochemical data indicate a diagnosis of GHD, then a MRI scan should be undertaken to define the pituitary anatomy and to help confirm the diagnosis.