Charles M. Glasier

Intracranial aneurysms in infants and children

Background. The diagnosis and imaging of pediatric aneurysms has changed since the advent of MR and MRA. Objective. To update the literature on pediatric aneurysms and better define the appropriate work-up of childhood aneurysms in 1997. Materials and methods. Retrospective review of 21 children (12 boys, 9 girls) with 25 aneurysms from three institutions over a 20-year period was performed. Imaging studies were mixed and included CT (19 patients), MR (11 patients), MRA (6 patients) and angiography (18 patients). Results. Eighteen of 25 aneurysms were congenital saccular, 6 were mycotic, and 1 was post-traumatic. Of these, 44 % were in the posterior circulation. Nine aneurysms arose from distal arterial branches. Forty percent were large (between 1–2.5 cm) and 16 % were giant (> 2.5 cm). CT and MR showed hemorrhage, and frequently revealed the aneurysms as a focal mass with or without enhancement and flow void. Six children had MRA which revealed aneurysms in four patients. All patients with MRA had corresponding conventional angiography. Conclusion. Characteristics of pediatric aneurysms include diversity of type, increased incidence in the posterior fossa, peripheral location, and large size. CT, MR and MRA are useful in the diagnosis with conventional angiography essential for preoperative planning.

Usefulness of scanning procedures for diagnosis of fever of unknown origin in children

During a 5-year study period, 109 patients were referred to a large childrens hospital for evaluation of prolonged fever of unknown origin, defined as temperature greater than or equal to 38 degrees C (100.4 degrees F) for 3 weeks or longer and negative findings on initial examination. A two-phase protocol of outpatient followed by inpatient diagnostic studies was instituted for most patients. Confirmed diagnoses were achieved in just 36 of these children (33%) in the following disease categories: infectious, 24 (22%); autoimmune, 7 (6%); and neoplastic, 2 (2%). Scanning or special procedures and the number with positive results (in parentheses) were as follows: abdominal ultrasonography, 43 (8); abdominal computed tomography, 14 (3); indium scan 11 (5); gallium scanning, 4 (1), upper gastrointestinal tract series, 13 (2); technetium bone scanning 15 (2); bone marrow examination, 16 (1); and cranial computed tomography, 7 (0). These studies rarely led to an unsuspected diagnosis. It appears most appropriate in evaluating fever of unknown origin in children to obtain only basic laboratory studies such as a complete blood cell count, urinalysis and culture, chest radiograph, tuberculin skin test, and, in the older child, an antinuclear antibody titer. When these test results are negative, almost all children can be observed clinically for progression of illness or a focus that might then direct specific diagnostic procedures.

Transcranial Doppler, MRA, and MRI as a screening examination for cerebrovascular disease in patients with sickle cell anemia: an 8-year study

Objective. The authors previously reported five transcranial Doppler ultrasonography (TCD) findings as significant in detecting clinical cerebrovascular disease in a 4-year study in patients with sickle cell disease. This is a follow-up to evaluate the validity of the original findings over another 4-year period during which the study population doubled. A clinical follow-up of the original asymptomatic sickle cell patients with positive TCD, MRA, and MRI was also made. Materials and methods. Over an 8-year period TCD, MRI, and MRA were prospectively performed in 90 sickle cell patients who were clinically asymptomatic for stroke and in 27 sickle cell patients with clinical stroke. Results. Of the 4 out of original 46 control patients in 1992 who had positive MRA and TCD, 3 have subsequently had clinical stroke. None of the 9 original patients with positive TCD and positive MRI but negative MRA have developed stroke. All five original TCD indicators of disease were still significant (P < 0.05) for detecting clinical disease: maximum velocity in ophthalmic artery (OA) > 35 cm/s, mean velocity in middle cerebral artery (MCA) > 170 cm/s, resistive index (RI) in OA < 50, velocity in OA greater than in MCA, and velocity in posterior cerebral (PCA), vertebral, or basilar arteries greater than in MCA. Four additional factors were also significant: turbulence, PCA or ACA without MCA, RI < 30, and maximum velocity in MCA > 200 cm/s. Conclusion. Positive MRA with a positive TCD in an asymptomatic patient in long-term follow-up suggests a trend for developing clinical stroke. A 4- to 8-year follow-up of nine patients with positive TCD, positive MRI, but not positive MRA did not show development of clinical stroke. Nine Doppler findings are significant in screening for clinically symptomatic vascular disease in sickle cell patients. It is recommended that children with sickle cell disease be screened for cerebrovascular disease with TCD. If one or two indicators of abnormality are present, MRA is recommended. If the MRA is positive, the patient may be considered for transfusion therapy or other treatment for prevention of stroke.

Significance of opacification of the maxillary and ethmoid sinuses in infants

To evaluate the incidence and significance of radiographic sinus opacification in infants, we performed computed tomography (CT) of the maxillary and ethmoid sinuses in conjunction with routine cranial CT in 100 infants from birth to 12 months of age. CT was performed for indications other than sinusitis. Prospective concurrent clinical history was obtained and physical examination of the upper respiratory tract was performed. Of 100 infants, 16 had hypoplasia of the maxillary sinuses; 81% (13/16) of these were less than 2 months of age. The antra showed progressive increase in size during the first year of life. Of the 100 infants, 70 had CT sinus opacification, including 67% of those without historical or physical evidence of upper respiratory tract infection. There was a positive correlation of CT findings between the maxillary and ethmoid sinuses in 80% of the infants older than 2 months of age but in only 49% of the younger infants. Radiographic sinus opacification in infants is of uncertain significance and is not diagnostic of upper respiratory tract infection, much less of sinusitis.

Perinatal cortical and subcortical visual loss: Mechanisms of injury and associated ophthalmologic signs

PURPOSE To determine whether term and preterm injuries to the retrogeniculate visual system are associated with recognizable patterns of ophthalmologic abnormalities and whether these patterns can be attributed to cortical (gray matter) or subcortical (white matter) injury. DESIGN A retrospective case series. PARTICIPANTS One hundred children with clinical and neuroimaging signs of perinatal posterior visual pathway injury who were examined at Arkansas Childrens Hospital Eye Clinic between 1989 and 1999. METHODS We reviewed magnetic resonance images or computed tomographic scans from 50 children with cortical (predominantly or exclusively involving cortical gray matter) and 50 children with subcortical (predominantly or exclusively involving subcortical white matter) perinatal injury to the retrogeniculate visual system. Ophthalmologic abnormalities were analyzed retrospectively in each group. MAIN OUTCOME MEASURES Conjugate gaze deviation, type of strabismus, abnormal eye movements, and optic disc morphology. RESULTS Horizontal conjugate gaze deviation, exotropia, and a normal optic disc appearance were significantly more common in cortical than in subcortical visual loss. Tonic downgaze, esotropia, and optic nerve hypoplasia (with or without coexisting pallor) were significantly more common in subcortical than in cortical visual loss. CONCLUSIONS Perinatal cortical and subcortical visual loss produce differing profiles of ophthalmologic dysfunction. A reclassification of periventricular leukomalacia and other forms of retrogeniculate white matter injury as subcortical visual loss would increase diagnostic specificity.

SEPTO-OPTIC DYSPLASIA: THE CLINICAL INSIGNIFICANCE OF AN ABSENT SEPTUM PELLUCIDUM

Neurodevelopmental assessments were performed on seven patients with optic nerve hypoplasia and absence of the septum pellucidum on MRI. The evaluation included neurological status, language development, neuropsychological functioning, and behavioral and emotional adjustment. Six of the seven were found to have normal cognitive development, intact neurological status, normal language development and age‐appropriate behavior. Abnormal findings included early poor motor coordination, which was felt to be closely related to decreased visual acuity, as well as subtle visual attentional problems which occurred even in patients who had normal vision in one eye. Congenital absence of the septum pellucidum was not associated with significant intellectual, behavioral or neurological deficits in the majority of these patients.

Aicardi syndrome: More than meets the eye

An eight-month-old girl with infantile spasms and apparent blindness had electroencephalographic findings compatible with Aicardi syndrome. In addition to optic nerve hypoplasia, there were multiple congenital retinal malformations in the right eye, including chorioretinal lacunae, anomalous retinal vessels, posterior scleral ectasia, and a peripheral fibrous ridge. Magnetic resonance imaging demonstrated agenesis of the corpus callosum, absence of the septum pellucidum, optic nerve and chiasmal hypoplasia, pachygyria, cortical heterotopias, colpocephaly, and hypoplasia of the cerebellar vermis. This patient illustrates the broad spectrum of cerebroretinal malformations now known to characterize Aicardi syndrome.

Efficiency of the bone scan for occult limping toddlers.

Fifty consecutive occult limping toddlers were prospectively evaluated by acute triphase 99mTc MDP scintigraphy (TTS) at Arkansas Childrens Hospital from 1984 through 1989. Only patients with a limp that could not be diagnosed by an orthopaedist were included. TTS proved essential in localizing the lesion in 27 patients (54%). With only two false negatives and one false positive, this test was shown to be highly sensitive, specific, efficient, and predictive, especially as compared with temperature, white blood cell (WBC) count, erythrocyte sedimentation rate (ESR), and plain radiography. Because no infections were missed by TTS, patients with a normal TTS could be safely observed as outpatients, saving thousands of health care dollars in this small series.

Nerve Root Enhancement on Spinal MRI in Pediatric Guillain-Barré Syndrome

Guillain-Barré syndrome diagnosis is based on clinical presentation and supportive diagnostic testing. In its early stage, no single, reliable diagnostic test is available. However, a finding of nerve root enhancement on spinal magnetic resonance imaging may be useful. We evaluated the frequency of nerve root enhancement on spinal magnetic resonance imaging in children with Guillain-Barré syndrome. At a single tertiary pediatric center, we conducted a retrospective chart review of children with Guillain-Barré syndrome who had complete spinal or lumbosacral spinal magnetic resonance imaging with gadolinium administration from January 2002-January 2009. Twenty-four consecutive patients were identified. Spinal nerve root enhancement with gadolinium was present in 92% (22/24) of children with Guillain-Barré syndrome on initial spinal magnetic resonance imaging (95% confidence interval, 0.745-0.978). This finding increased to 100% of patients, after two patients underwent repeat spinal magnetic resonance imaging that did reveal nerve root enhancement. Patterns of enhancement were variable, but involved the thoracolumbar nerve roots in all patients. Enhancement of nerve roots with gadolinium on initial spinal magnetic resonance imaging was frequently present in these children with Guillain-Barré syndrome. Spinal magnetic resonance imaging is a sensitive diagnostic test and should be considered an additional diagnostic tool in select cases.

High resolution ultrasound with Doppler: A diagnostic adjunct in orbital and ocular lesions in children

Twenty-six infants and children with orbital and ocular pathology were examined with ultrasound (US) utilizing real-time imaging and Duplex Pulsed Doppler evaluation. Twenty-two of these patients underwent concurrent orbital computed tomography (CT) and two had magnetic resonance imaging (MRI). Orbital and periorbital lesion included hemangioma, dermoid, lymphangioma, rhabdomyosarcoma, encephalocoele and abscess. Ocular lesions included infection, trauma, retinal detachment, retinoblastoma, Coats disease, and persistent hyperplastic primary vitreous. High resolution US with Doppler provided unique diagnostic information in patients with penetrating ocular trauma, orbital and periorbital masses, and intraocular structural abnormalities. High resolution US examination of the eye and periorbital tissues is readily performed using widely available equipment and often delineates subtle structural abnormalities not shown by CT or MRI.