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Biomedical subjects

T Y Poussaint

Publications and source records attributed to T Y Poussaint.

29 records · Page 2Linked to original sources

Diencephalic syndrome: clinical features and imaging findings.

PURPOSE: To emphasize the importance of imaging in children with diencephalic syndrome due to hypothalamic/chiasmatic astrocytomas. METHODS: Findings in nine patients (mean age, 26 months) with diencephalic syndrome and hypothalamic/chiasmatic astrocytomas were analyzed retrospectively, including reviewing clinical records, imaging examinations, and follow-up studies. RESULTS: Symptoms and signs included failure to thrive (n = 9), nystagmus (n = 3), visual field defects (n = 1), optic pallor (n = 1), emesis (n = 2), and headache (n = 1). All patients had hypothalamic/chiasmatic masses. Five patients underwent biopsy, and, in all cases, specimens showed low-grade astrocytoma. Imaging studies were available in eight patients. All tumors were large (median maximum diameter, 3.5 cm), involved the chiasm and hypothalamus, and showed homogeneous enhancement. Three patients had hydrocephalus and two had metastases. At follow-up, five patients had recurrent disease and two had died. CONCLUSION: Diencephalic syndrome is a rare cause of failure to thrive in childhood, and diagnosis of a hypothalamic/ chiasmatic astrocytoma might therefore be delayed. The astrocytomas associated with this syndrome are larger, occur at a younger age, and are often more aggressive than other astrocytomas arising in this region.

Astrocytoma↗

Maternal phenylketonuria: magnetic resonance imaging of the brain in offspring.

Phenylketonuria (PKU) produces white matter changes identifiable by magnetic resonance imaging. These changes occur postnatally. Offspring of untreated mothers with PKU also have a brain effect, expressed as microcephaly and mental retardation. This effect occurs prenatally. To determine whether the white matter changes seen in PKU are also present in maternal PKU offspring, despite the different developmental stages of exposure to PKU, we performed brain magnetic resonance imaging studies in seven maternal PKU offspring, five from essentially untreated pregnancies and two from treated pregnancies. None had white matter changes, although the one offspring with PKU had delayed myelination. However, hypoplasia of the corpus callosum was present in three of the four offspring from untreated pregnancies and in the offspring from a maternal PKU pregnancy not treated until the third trimester. Unlike PKU, white matter changes are not a feature of the brain effect in maternal PKU. However, hypoplasia of the corpus callosum is a feature of maternal PKU and is probably a result of inhibition of corpus callosum development at 8 to 20 weeks of gestation. The hypoplastic corpus callosum could be a marker for brain effect in maternal PKU and may have implications for the cognitive deficits in these offspring.

Adolescent↗

Structural abnormalities in brain magnetic resonance images of depressed children.

OBJECTIVE: Brain magnetic resonance images (MRIs) of 65 children and adolescents who were hospitalized with depressive disorders (DD) were compared with the brain MRIs of 18 hospitalized psychiatric controls (PC) without a depressive disorder. METHOD: Volumetric analyses were used to measure frontal lobe volumes (FLV), lateral ventricular volumes (VV), and total cerebral volumes (CV) for all subjects. To correct for differences in absolute cerebral volume associated with different body and head size, the ratios of FLV/CV and VV/CV were used to compare differences between the two groups. A multivariate analysis was used to control for the effects of several independent variables (age, sex, diagnosis). RESULTS: Significant differences were seen in the FLV/CV ratio and the VV/CV ratio when the results were compared between the two groups (DD versus PC). The DD group had a significantly smaller FLV/CV ratio (t = 2.148, df = 79, p = .035) and a significantly larger VV/CV ratio (t = -2.093, df = 79, p = .040). CONCLUSION: The findings are consistent with previous reports in depressed adults and may implicate the frontal lobes in the pathogenesis of early-onset depressive disorders.

Adolescent↗

Pediatric orbital and visual pathway lesions.

This article presents the clinical and imaging aspects of some common and important orbital and optic pathway lesions occurring in childhood. Lesions that arise outside of and encroach on the orbit and visual pathways are included. Abnormalities intrinsic to the globe are covered elsewhere in this issue.

Adolescent↗

Hemorrhagic vasculopathy after treatment of central nervous system neoplasia in childhood: diagnosis and follow-up.

PURPOSE: To review the clinical data, imaging findings, and intermediate outcomes of a series of children with hemorrhagic vasculopathy after treatment for intracranial neoplasia. METHODS: We retrospectively analyzed the medical records and imaging examinations of 20 pediatric patients (ages 1 to 15 years) with intracranial neoplasia in whom delayed intracranial hemorrhage developed after cranial irradiation or radiation combined with systemic or intrathecal chemotherapy. Patients with intracranial hemorrhage from other identifiable causes were excluded. Histopathologic analysis was available in four patients. RESULTS: Twenty patients with delayed intracranial hemorrhage received cranial irradiation alone (n = 9) or combined radiation and chemotherapy (n = 11) for primary brain tumors (n = 13), leukemia (n = 6), or lymphoma (n = 1). Imaging findings were consistent with hemorrhages of varying ages. The hemorrhages were not associated with tumor recurrence nor second tumors. Except for location of the hemorrhage, no significant relationship was established between outcome and original diagnosis, radiation dose (range, 1800 to 6000 centigray), chemotherapeutic agent or dosage, age at treatment, or interval between therapy and hemorrhage (mean, 8.1 years). Only brain stem hemorrhage was associated with a poor outcome. CONCLUSION: In children with central nervous system neoplasia who have undergone cranial irradiation, or radiation combined with chemotherapy, delayed intracranial hemorrhage may develop.

Adolescent↗

Dysraphic myelodysplasias associated with urogenital and anorectal anomalies: prevalence and types seen with MR imaging.

OBJECTIVE: The purpose of this study was to determine by MR imaging the prevalence and types of dysraphic abnormalities of the spinal cord (i.e., myelodysplasias) associated with urogenital and anorectal malformations of childhood. MATERIALS AND METHODS: Since 1987, 92 patients with imperforate anus complex, cloacal malformation, and cloacal exstrophy have had MR imaging as a screening examination for occult dysraphic myelodysplasia. The prevalence and types of myelodysplasia were determined for each group. RESULTS: The prevalence of dysraphic myelodysplasia in each group of children was 17% (1/6) for low imperforate anus (ectopic anus), 34% (11/32) for high imperforate anus (with fistulization), 46% (19/41) for cloacal malformation, and 100% (13/13) for cloacal exstrophy. The most common abnormalities in each group were tethered cord with intradural or filar lipoma in imperforate anus; low-placed or dysplastic conus medullaris and tethered cord with lipoma or myelolipoma in cloacal malformation; and lipomyelocele, lipomyelomeningocele, or lipomyelocystocele in cloacal exstrophy. CONCLUSION: Our results show that the prevalence of myelodysplasia as seen on MR imaging is high in patients with urogenital and anorectal anomalies.

Anus, Imperforate↗

Cystic retrocerebellar malformations: unification of the Dandy-Walker complex and the Blake's pouch cyst.

Twenty-six cases of developmental retrocerebellar cyst (RCC) formation are studied with respect to determining the usefulness and anatomic relevance of separate terms currently in use, including Dandy-Walker complex, Dandy-Walker malformation, Dandy-Walker variant, mega-cisterna magna (MCM), and Blake's pouch cyst. An anatomic and embryological continuum between Dandy-Walker complex and Blake's pouch cyst is proposed. A method for the useful assessment of RCC is outlined. The patency or closure of the aqueduct is crucial to the evaluation and management of hydrocephalus associated with RCC formation.

Arachnoid Cysts↗

Structural anomalies revealed by neuroimaging studies in the brains of patients with neurofibromatosis type 1 and large deletions.

PURPOSE: The basis for cognitive problems in patients with neurofibromatosis type 1 (NF1) is unknown. A subset of NF1 patients with deletion of the entire NF1 gene has severe learning problems or mental retardation. We have reviewed neuroimaging studies (CT and MRI) in five such patients to determine whether structural anomalies in the brain are present and might explain the impaired cognitive function. METHODS: Five patients with NF1 and deletion of the entire gene were identified by FISH studies. A retrospective review was conducted of CT and MRI images, as well as of data from developmental assessments. RESULTS: All five patients had severe developmental impairment. None had been exposed to chemotherapy or radiation therapy. All had multiple regions of bright T2 signal intensity. Structural anomalies were seen in three of the five patients and included callosal dysgenesis in one, septum cavum vergae and pellucidum in two, mega cisterna magna in one, and Chiari I malformation with severe hydrocephalus in one patient. CONCLUSION: Individuals with NF1 and large gene deletions have an increased frequency of structural anomalies of the brain not usually seen in NF1 patients. This suggests that the mental retardation in these individuals is due, at least in part, to abnormal brain development rather than a defect in brain function due to haplosufficiency of the NF1 gene product.

Adolescent↗

Hemorrhagic pituitary adenomas of adolescence.

PURPOSE: To review the clinical and MR imaging findings in adolescents with hemorrhagic pituitary adenomas and to compare those findings with pathologic results and outcome. METHODS: We reviewed the clinical records, imaging examinations, surgical and pathologic findings, and follow-up studies in 11 girls and six boys (12 to 20 years old; mean age, 16 years) with pituitary adenomas who were treated at our institution between August 1986 and June 1995. RESULTS: Of the 17 adenomas, eight were macroadenomas (> 1 cm) in patients 14 to 18 years old (three girls, five boys). Six of the macroadenomas were grossly hemorrhagic, and appeared as high-intensity intrasellar/suprasellar masses on all MR sequences obtained before definitive diagnosis and treatment. Clinical presentation in the patients with the hemorrhagic macroadenomas included headache (five), visual field deficits (three), and neuroendocrine symptoms (three). One patient was asymptomatic. The preliminary clinical and imaging diagnoses were craniopharyngioma or Rathke's cyst in five of the six cases. Pathologic diagnoses were prolactinoma in four patients, plurihormonal (prolactin/follicle-stimulating hormone) tumor in one patient, and nonfunctioning adenoma in one patient. Surgical resection was performed in all six hemorrhagic tumors and radiation therapy was required in three cases. CONCLUSION: Pituitary adenomas uncommonly occur in childhood and are usually seen in adolescence. The majority of the macroadenomas are hemorrhagic and often occur in male subjects. The clinical and MR imaging features may mimic craniopharyngioma or Rathke's cyst. These tumors often require surgery and/or radiation therapy.

Adenoma↗