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Postoperative encephalopathy with choreoathetosis is a serious neurologic complication of childhood cardiac surgery. An inadequate understanding of the long-term outcome has limited family counseling and the institution of intervention strategies. Our objective was to define the long-term neurologic, cognitive, and behavioral outcome in this group of patients. By retrospective review we identified all cases of choreoathetosis in our cardiac intensive care unit from 1986 through 1995. During this decade we described a sudden transient increase in postoperative choreoathetosis that disappeared as we modified treatment strategies in perioperative care. These children underwent a comprehensive battery of neurologic, cognitive, and behavioral tests to determine in detail their long-term outcome. Of the 36 cases identified of which three were deceased, four were abroad, and eight were lost to follow-up, 21 families were approached, and 15 of the 21 (71%) consented to testing. We found pervasive deficits in memory, attention, and language, with a median full-scale IQ of 67 (range = 40-122), as well as in motor function, including persistent dyskinesia in seven of 15 (47%). The pervasive and enduring cognitive and motor deficits in survivors of postoperative choreoathetosis caution for guarded predictions of outcome and highlight the need for careful neurologic and cognitive evaluation in all children with choreoathetosis after open heart surgery. [Copyright &y& Elsevier]

Objective: Perioperative stroke and periventricular leukomalacia have been reported to occur commonly in infants with congenital heart disease. We aimed to determine the incidence and type of brain injury in infants undergoing 2-ventricle repair in infancy and to determine risk factors associated with such injury. Methods: Forty-eight infants enrolled in a trial comparing 2 different hematocrits during surgical repair of congenital heart disease underwent brain magnetic resonance imaging scans and neurodevelopmental testing at 1 year of age. Results: Eighteen (38%) of our subjects had tiny foci of hemosiderin by susceptibility imaging, without evidence of abnormalities in corresponding regions on conventional magnetic resonance imaging sequences. Subjects with foci of hemosiderin had a significantly lower Psychomotor Developmental Index at 1 year of age (79.6 ± 16.5, mean ± standard deviation) compared with subjects without these foci (89.5 ± 15.3; P = .04). Older age at surgery and diagnostic group were significantly associated with the presence of hemosiderin foci. Only 1 subject had a small stroke (2%), and 2 subjects had periventricular leukomalacia (4%). Conclusion: Foci of hemosiderin without radiologic evidence of ischemic brain injury are an abnormality associated with adverse neurodevelopmental outcome not previously described in magnetic resonance imaging studies of children with surgically repaired congenital heart disease. The association of hemosiderin foci with older age at surgery and cardiac diagnosis, and not with risk factors associated with brain injury, in previous studies suggests that the cause and pathogenesis of this abnormality are different from ischemic brain lesions reported previously. [Copyright &y& Elsevier]

Objective: Advances in fetal magnetic resonance imaging allow the detection of subtle anatomic anomalies of unclear long-term clinical significance. The purpose of this study was to examine the accuracy of fetal magnetic resonance imaging in the diagnosis of isolated inferior vermian hypoplasia and to describe the neurodevelopmental outcome. Study design: We reviewed all cases with fetal and postnatal magnetic resonance imaging studies between 1999 and 2003 and identified 19 cases with a diagnosis of isolated inferior vermian hypoplasia. We compared prenatal and postnatal magnetic resonance imaging studies and evaluated subjects using developmental scales. Results: Isolated inferior vermian hypoplasia was confirmed by postnatal magnetic resonance imaging in 68% of the patients (13/19); the remaining 6 patients had normal postnatal magnetic resonance imaging results. On developmental testing at mean age 19.8 ± 4.9 months, 3 infants (23%) with confirmed postnatal diagnosis demonstrated motor and language delays and functional difficulties, and 2 infants (15%) had behavioral problems; none of the infants with normal postnatal magnetic resonance imaging studies were delayed. Conclusion: Isolated inferior vermian hypoplasia in the second trimester may be over-diagnosed by fetal magnetic resonance imaging and therefore warrants postnatal magnetic resonance imaging confirmation. [ABSTRACT FROM AUTHOR]

Background. Previously, neurologic dysfunction was estimated to complicate 25% or less of pediatric open-heart operations. We sought to determine the current incidence and spectrum of early postoperative neurologic complications.Methods. We undertook a retrospective review of all patients undergoing open heart operations in 1 year at our institution with the goal to identify all neurologic complications occurring in the early postoperative period.Results. Open-heart operations were performed in 706 children. Sixteen children (2.3%) had neurologic complications develop, including 9 (1.3%) with definite clinical seizures, 1 with suspected seizures and bilateral subdural hemorrhage, 2 with coma after cardiac arrest, 2 with transient mild choreoathetosis, 1 with facial palsy, and 1 with persistent irritability. Causes of seizure were cyclosporin A toxicity posttransplant (4), cerebral ischemia post cardiac arrest (3), and unknown (2). In infants less than 1 year of age, the incidence of seizures was 1.2%.Conclusions. This review suggests a decrease in acute neurologic morbidity after pediatric open heart operation. Clinical seizures remain the most common complication. Posttransplant, cyclosporin-associated seizures have emerged as an important etiologic category, coincident with an increase in cardiac transplantation in children. [Copyright &y& Elsevier]

Although cerebellar injury in the premature infant is an increasingly recognized form of neonatal brain injury, its structural and functional outcomes remain poorly defined in the term infant. The objective of this cross-sectional study was to characterize the clinical and magnetic resonance imaging features and neurodevelopmental outcome in children, born at term, with cerebellar injury. Over a 5-year period, 20 infants were identified with ischemic (n = 3) or hemorrhagic (n = 17) cerebellar injury. Lesions were small (<1 cm) in 12 cases, and large in 8 cases. Prenatal and intrapartum factors frequently documented in term infants with cerebellar injury included primiparity (55%), advanced maternal age (30%), group B streptococcus-positive mothers (35%), abnormal fetal heart rate (35%), instrumented delivery (30%), and cesarean section (25%). At follow-up of 18 cases (median age, 32 months), 39% had neurologic abnormalities. Gross motor delays, expressive language deficits, and externalizing behavioral problems were the most common (44%). Cognitive deficits were present in one third of cases. Larger cerebellar lesions were associated with significantly lower cognitive, gross motor, expressive language, and social-behavioral scores. Cerebellar injury in the term infant is associated with a broad spectrum of neurodevelopmental disabilities, particularly in infants with large cerebellar lesions. [Copyright &y& Elsevier]