H. Cortina

Neuroimaging in tuberous sclerosis: A clinicoradiological evaluation in pediatric patients

CT, MRI and neurological features of 27 children with tuberous sclerosis were prospectively compared. Imaging studies were positive in 92.5% of cases. CT was more useful in detecting subependymal nodules, while MRI showed the number and location of cerebral cortical and subcortical lesions more accurately. Cortical lesions in the cerebellar hemispheres were present in 26% of patients. Gadolinium-DTPA used in 10 patients showed slight enhancement of the subependymal lesions in 80% of cases, probably representing active lesions with alteration of the blood-brain barrier. None of the cortical and subcortical lesions enhanced. Giant-cell astrocytomas were detected in 5 patients, the postcontrast CT and MRI studies improving their assessment. Unlike subependymal nodules, all tumors showed marked enhancement regardless of their size. Seizures were present in 96% of patients, mostly beginning before 1 year of age. There was no clear relationship between the radiological features and the neurological evolution of these patients. There-fore, it is not possible to establish a clinical prognosis based on the radiological findings. MRI is the procedure of choice in the diagnosis of tuberous sclerosis. When pre and postcontrast MRI are negative, CT is used to exclude small calcified subependymal nodules. MRI followup is required only when tumoral development is clinically suspected.

Neurofibromatosis type 1 in children: MR imaging and follow-up studies of central nervous system findings

PURPOSE To determine the frequency, evolution and diagnostic impact of characteristic central nervous system MR imaging lesions in children with neurofibromatosis type 1 (NF1). SUBJECTS We reviewed 89 children with established or clinically suspected disease. A final diagnosis of NF1 was made in 72 (age range, 10 months to 14 years). RESULTS Hyperintense lesions on long TR images were detected in 78% of patients, principally involving the basal ganglia, cerebellum and brain stem. In 30% of the globus pallidus lesions, hyperintensity was seen on short TR images, being usually isointense on IR T1 weighted images. Globus pallidus lesions did not enhance. Eight patients presented atypical unenhanced lesions showing either edema, mass effect or hypointensity on short TR images; 2 of them were considered symptomatic brain stem gliomas. Six other children showed one or more growing enhanced cerebral lesions classified as tumors. Other child developed a growing enhanced lesion that markedly decreased in the follow-up studies. Twenty patients (28%) had optic gliomas. In two children, under 6 years old, this tumor appeared de novo. Forty-five children had several follow-up MR imaging studies (mean interval, 3 years). Regression of the basal ganglia lesions, both in size and/or intensity was noticed in 42% of cases, enlargement or new appearance of lesions in 24.5%, mixed increased/decreased in 7%, and stability in 26.5%. White matter lesions of the cerebellum and brain stem decreased in size in 40%, grew in 15.5%, showed a mixed increased/decreased pattern in 11%, and remained unchanged in 33.5% of cases. An involutional tendency of these lesions occurred in children older than 10 years, while progression was more frequent in younger children (P<0.05). CONCLUSIONS Hyperintense lesions are highly prevalent and characteristic in patients with NF1. MR imaging contributed to a definitive diagnosis of NF1 in 53% of suspected cases. Follow-up studies are necessary in the evaluation of suspected NF1, even if the first examination is negative.

Imaging considerations of central nervous system manifestations in pediatric patients with neurofibromatosis type 1

CT and MRI were used in a prospective study of the central nervous system (CNS) manifestations in 41 consecutive children with neurofibromatosis type 1 (NF-1). Gadolinium-DTPA was used in 15 patients. MRI was more effective than CT in delimiting the extension of the optic pathway glioma and in evaluating associated cerebral malformations. MRI visualized lesions generally undetected by CT, in the form of iso- or hyperintense foci with respect to the cerebral cortex in T2-weighted sequences. Well-delimited lesions of high signal intensity were observed in the globus pallidus (22 cases), the internal capsule (6 cases), corpus callosum (2 cases), anterior commissure (1 case) and semioval center (2 cases). Poorly defined hyper- or isointense areas were also observed affecting the cerebellar white matter (21 cases) and brain stem (17 cases). None of these lesions showed Gadolinium-DTPA enhancement, and were of no clinical significance. MRI has displaced CT in the initial diagnosis of patients with NF-1. Periodic annual MRI controls are only justified in patients with MRI changes to evaluate the progression or stabilization of the lesions.

Hallazgos radiológicos del síndrome de Gorham-Stout

El sindrome de Gorham-Stout, o sindrome del hueso fantasma o evanescente, es una rara enfermedad de etiologia y patogenia desconocida. Se caracteriza por una osteolisis masiva localizada, rapidamente progresiva, asociada con una proliferacion de estructuras vasculares de etiologia benigna, donde es representativa la ausencia de nueva formacion osea. Presentamos dos casos de esta enfermedad con los hallazgos clinicos, histologicos y radiologicos caracteristicos. El primero en un nino de 6 anos con afectacion de cubito derecho, y el segundo en una paciente de 15 anos con afectacion costal derecha asociada a quilotorax.

Eikenella corrodens skull infection: A case report with review of the literature

BACKGROUND Subgaleal abscesses and skull osteomyelitis are very uncommon since the introduction of antibiotics. Eikenella corrodens infection is extremely rare in childhood and has never been reported in calvarial osteomyelitis. METHODS We present a previously healthy 9-year-old boy, with a history of frontal contusion without injury, who developed E corrodens osteomyelitis of the skull. The radiographic findings are reviewed, including skull films and computed tomographic scans. The patient underwent surgical debridement of the lesion, as well as systemic medical therapy with amoxicillin. We review the medical and surgical therapy for such lesions. The differential diagnosis of a posttraumatic scalp swelling is also reviewed. RESULTS Good resolution after debridement and antibiotic therapy is reported. CONCLUSIONS Surgical intervention is emphasized for the removal of bony sequestrum and nonviable bone while maintaining an intact dura.

Sonographic findings in aneurysmal bone cyst in children: Correlation with computed tomography findings

This study compares the value of sonography and computed tomography (CT) in assessing fluid‐fluid levels (FFLs) in aneurysmal bone cysts (ABCs) in children.

Effectiveness of pneumatic reduction of ileocolic intussusception in children

A total of 158 consecutive therapeutic air enemas were performed upon 133 patients during a 2-year period. Perforation and/or an impairment of the general condition of the children were the only main contraindications for the reduction attempt (five patients). Successful reductions were achieved in 89% of cases with no complications. Operation was performed in all 17 cases of unsuccessful reduction: resection in four cases, easy manual reduction in nine, difficult manual reduction in two, and spontaneous reduction in two. There were 16 (12%) patients with one or more recurrent episodes. In 23 (16%) cases, following pneumatic reduction, a swollen ileocecal valve showing a multiple appearance was observed. This fact sometimes results in a differential diagnosis with incomplete reduction; in all these cases, small bowel aeration was always a sign of complete reduction. No significant difference was noted between a swollen ileocecal valve and patient age, duration of symptoms, presence of small bowel obstruction, and further recurrences. Air enema has improved our previous success obtained with hydrostatic reduction (79%), as well as being a simpler, cleaner, and faster technique for intussusception reduction in children.

Osteochondroma post osteomyelitis

We report a case of radial metaphyseal osteochondroma secondary to osteomyelitis in a premature baby of 28 weeks. This is the second case with these characteristics that has been described in the literature. A possible pathogenesis is related to trauma during drainage of the abscess or following the associated inflammatory process.