Robert G. Wells

Early Stroke and Cerebral Vasculopathy in Children With Facial Hemangiomas and PHACE Association

PHACE association is a rare neurocutaneous syndrome that may include posterior fossa malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, eye abnormalities, and sternal defects. The arterial abnormalities may be developmental or acquired and usually involve the cervical and cerebral vasculature. We believe that infants with PHACE association are at increased risk of arterial ischemic stroke and describe 5 infants with facial hemangiomas and structural and acquired arterial anomalies, all of whom suffered an arterial ischemic stroke during infancy.

MR features of fleeting CNS lesions associated with Epstein-Barr virus infection

The magnetic resonance and CT findings in a 10-year-old patient with neurologic abnormalities associated with Epstein-Barr virus infection are reported. Multiple focal lesions of various sizes were demonstrated with involvement at one time or another of both cerebral hemispheres, the right thalamus, the right cerebellar hemisphere, and the right optic nerve. A unique finding was the fleeting nature of the lesions with new lesions appearing as others resolved. The clinical presentation, laboratory studies, and radiologic patterns suggest the diagnosis of immune-related acute disseminated encephalomyelitis associated with Epstein-Barr virus infection.

Osteoblastoma response to chemotherapy

An 8‐year‐old boy had an osteoblastoma of the body of C2. After attempted excision, the tumor recurred rapidly and massively. The tumor shrank progressively after treatment with high‐dose methotrexate, doxorubicin, and cisplatin. The patient has stopped treatment and been stable for 33 months. Surgical excision remains the treatment of choice for osteoblastoma. Chemotherapy may be useful in selected patients with a recurrent, aggressive tumor or in patients with surgically inaccessible disease.

Pulmonary aspergillosis appearing as chronic nodular disease in chronic granulomatous disease.

Chronic nodular pneumonia is unusual in children. Three children are described who presented with diffuse nodular pulmonary disease and in whom lung biopsy demonstratedAspergillus infection. One child was known to have chronic granulomatous disease of childhood (CGD) and further investigation demonstrated CGD in the other two patients as well. These cases indicate thatAspergillus infection and CGD should be considered in the differential diagnosis in children who present with chronic diffuse nodular pneumonia.

Technetium 99m pertechnetate thyroid scintigraphy: congenital hypothyroid screening

Technetium 99m pertechnetate thyroid scans were performed on 57 infants referred for evaluation of suspected congenital hypothyroidism. Thyroid anatomy may be characterized by four general types, based on the scintigraphic findings: (1) normal size and location; (2) ectopic location; (3) no detectable thyroid activity; (4) normal location with increased size or uptake. There are diverse etiologies of congenital hypothyroidism. Correlation of thyroid scintigraphy with blood T4 and TSH levels allows specific etiological diagnosis in the majority of cases of congenital hypothyroidism.

Hemimegalencephaly: Clinical, EEG, neuroimaging, and IMP-SPECT correlation

Iofetamine-single photon emission computed tomography (IMP-SPECT) was performed on 2 girls (5 1/2 and 6 years of age) with histories of intractable seizures, developmental delay, and unilateral hemiparesis secondary to hemimegalencephaly. Electroencephalography (EEG) revealed frequent focal discharges in 1 patient, while a nearly continuous burst suppression pattern over the malformed hemisphere was recorded in the other. IMP-SPECT demonstrated a good correlation with neuroimaging studies. In spite of the different EEG patterns, which had been proposed to predict contrasting clinical outcomes, both IMP-SPECT scans disclosed a similar decrease in tracer uptake in the malformed hemisphere. These results are consistent with the pattern of decreased tracer uptake found in other interictal studies of focal seizures without cerebral malformations. In view of recent recommendations for hemispherectomy in these patients, we suggest that the IMP-SPECT scan be used to compliment EEG as a method to define the extent of abnormality which may be more relevant to long-term prognosis than EEG alone.

The child with acute leg pain

Diagnostic imaging is an essential component in the evaluation of a child with acute lower extremity pain or limp. Most, but not all, of the etiologies are traumatic. Frequently, the history and clinical examination are not specific. Radionuclide bone imaging is extremely rewarding in these circumstances. It represents the most useful examination used in evaluating the pathophysiology and full extent of the disorder. This article discusses the clinical presentation, pathophysiology, and characteristic imaging observations in the more common problems associated with hip and/or leg pain.

Inflammatory fibroid polyp of the stomach in a child

A large inflammatory fibroid polyp of the stomach with endo-exophylic growth is described in a 5-year-old. CT and UGI demonstrate the lesion.

Left ventricular diverticulum: MRI demonstration (congenital broad-based left ventricular aneurysm)

The case presented is that of a 3-year-old boy with an asymptomatic left ventricular diverticulum. The value of MRI in the detection and classification of this entity is illustrated.

Normal High-Resolution Cerebral 99mTc-HMPAO SPECT Scans in Symptomatic Neonates Exposed to Cocaine

Vascular constriction is said to account for a variety of clinical effects of cocaine. High-resolution 99mTc-hexamethyl-propylene amine oxime single photon emission computed tomographic (SPECT) scans, which measure cerebral blood flow, were used to determine whether neonatal brain perfusion deficits are present in newborns with confirmed cocaine exposure. Normal, age-appropriate SPECT scans were found in 21 babies. Conventional neuroimaging was also performed when possible. All but one of the 14 magnetic resonance imaging (MRI) scans and one computed tomographic scan were normal. One MRI showed a mild delay in myelination. All but four neonates had behavioral or electroencephalographic abnormalities, and microcephaly was found in five of 21. The normal neonatal SPECT scans contrast with findings in adult cocaine users, which typically report abnormal findings of cerebral hypoperfusion. This study identifies a unique lack of corresponding cerebral vascular pathology in symptomatic neonates. It raises the possibility that many of these children can escape significant ischemic injury. (J Child Neurol 1994;9:278-280).