Pediatric Radiology

, Volume 26, Issue 1, pp 43–47 | Cite as

MR of the pituitary in patients with Prader-Willi syndrome: Size determination and imaging findings

  • L. Miller
  • M. Angulo
  • D. Price
  • S. Taneja
Article

Abstract

Prader-Willi syndrome (PWS) is an unusual genetic disorder characterized by short stature, obesity, hypogonadism, hypotonia, cognitive impairment, and dysmorphic facies. There is an interstitial deletion of the proximal long arm of chromosome 15 in about 70% of patients. Some of these clinical features suggest a central hypothalamic/pituitary dysfunction, and recent investigations have demonstrated a marked impairment in spontaneous growth hormone (GH) secretion. We studied 15 GH-deficient PWS patients by magnetic resonance imaging (MRI) to determine whether there was a diminution in the gross morphological size of the anterior pituitary gland, the site of GH synthesis. We also set out to catalog the pertinent imaging findings in this patient population. Our results indicate that this is the first report documenting pituitary size by MRI in PWS patients. No statistically significant difference was found in the height of the anterior pituitary gland in PWS patients compared with either normal children or children with isolated GH deficiency. An interesting imaging finding is that three of 15 patients (20%) demonstrated complete absence of the posterior pituitary bright spot (PPBS), and a fourth patient demonstrated a small PPBS. These observations reflect an objective physiologic disturbance in the hypothalamus. The clinical and radiologic implications of these findings are discussed.

Keywords

Growth Hormone Imaging Finding Hypogonadism Growth Hormone Deficiency Anterior Pituitary Gland 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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Copyright information

© Springer-Verlag 1996

Authors and Affiliations

  • L. Miller
    • 1
  • M. Angulo
    • 2
  • D. Price
    • 1
  • S. Taneja
    • 1
  1. 1.Department of RadiologyWinthrop University HospitalMineolaUSA
  2. 2.Department of Pediatrics, Division of Endocrinology and GeneticsWinthrop University HospitalMineolaUSA

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