Reliability of magnetic resonance imaging for the detection of hypopituitarism in children with optic nerve hypoplasia.

PURPOSE: It is essential to identify hypopituitarism in children with optic nerve hypoplasia (ONH) because they are at risk for developmental delay, seizures, or death. The purpose of this study is to determine the reliability of neurohypophyseal abnormalities on magnetic resonance imaging (MRI) for the detection of hypopituitarism in children with ONH. DESIGN: Cross-sectional study. PARTICIPANTS: One hundred one children with clinical ONH who underwent MRI of the brain and orbits and a detailed pediatric endocrinologic evaluation. METHODS: Magnetic resonance imaging studies were performed on 1.5-Tesla scanners. The imaging protocol included sagittal T1-weighted images, axial fast fluid-attenuated inversion-recovery/T2-weighted images, and diffusion-weighted images of the brain. Orbital imaging included fat-saturated axial and coronal images and high-resolution axial T2-weighted images. The MRI studies were reviewed by 2 pediatric neuroradiologists for optic nerve hypoplasia, absent or ectopic posterior pituitary, absent pituitary infundibulum, absent septum pellucidum, migration anomalies, and hemispheric injury. Medical records were reviewed for clinical examination findings and endocrinologic status. All patients underwent a clinical evaluation by a pediatric endocrinologist and a standardized panel of serologic testing that included serum insulin-like growth factor-1, insulin-like growth factor binding protein-3, prolactin, cortisol, adrenocorticotropic hormone, thyroid-stimulating hormone, and free thyroxine levels. Radiologists were masked to patients' endocrinologic status and funduscopic findings. MAIN OUTCOME MEASURES: Sensitivity and specificity of MRI findings for the detection of hypopituitarism. RESULTS: Neurohypophyseal abnormalities, including absent pituitary infundibulum, ectopic posterior pituitary bright spot, and absent posterior pituitary bright spot, occurred in 33 children. Magnetic resonance imaging disclosed neurohypophyseal abnormalities in 27 of the 28 children with hypopituitarism (sensitivity, 96%). A normal neurohypophysis occurred in 67 of 73 children with normal endocrinologic function (specificity, 92%). CONCLUSIONS: Neurohypophyseal abnormalities on MRI are sensitive and specific indicators of hypopituitarism in children with ONH.

Cavernous sinus syndrome from an internal carotid artery aneurysm in an infant with tuberous sclerosis.

Aneurysms are associated with tuberous sclerosis complex. We describe the first case of cavernous sinus syndrome from an intracavernous internal carotid artery aneurysm in a 9-month-old boy with tuberous sclerosis. The presence of an intracranial aneurysm should be considered in the differential diagnosis of children with tuberous sclerosis who develop cranial nerve deficits.

Long-term visual acuity and initial postoperative refractive error in pediatric pseudophakia.

OBJECTIVE: To examine the long-term best-corrected visual acuity (BCVA) in children who, during the amblyogenic period, underwent cataract extraction with intraocular lens (IOL) implantation and to see if initial postoperative refractive error following cataract extraction correlates with long-term BCVA. DESIGN: Retrospective chart review. PARTICIPANTS: Thirty-six eyes of 26 patients were included in the study. At least 2 years of follow-up, an age , 8.5 years at time of IOL implantation, an ability to participate in subjective visual acuity measurement, and an absence of congenital glaucoma and significant trauma were required for inclusion in the study. METHODS: Retrospective review of pseudophakic patients' charts at a tertiary care centre, with attention to initial and long-term pseudophakic refractive error, long-term BCVA, total myopic shift, length of follow-up, age at IOL implantation, and unilateral or bilateral cataract extraction with IOL implantation. RESULTS: Initial pseudophakic spherical equivalent (SE) showed a significant nonlinear relationship with most recentBCVAin unilateral cases and no relationship in bilateral cases (interaction p 0.001). Unilateral cases with initial pseudophakic SE between +1.75 D and +5.00 D showed better long-term BCVA than those with values below +1.75 D or above +5.00 D; this was irrespective of the refractive error of their fellow eye. CONCLUSIONS: In patients receiving lens implants in the first 8 years of life, we recommend aiming for low early postoperative hyperopia, taking into consideration each patient's situation and age.