Table of Contents Author Guidelines Submit a Manuscript
Case Reports in Pediatrics
Volume 2016 (2016), Article ID 4328492, 5 pages
Case Report

Anterior Hypopituitarism and Treatment Response in Hunter Syndrome: A Comparison of Two Patients

1Department of Pediatrics, University of Saskatchewan, Royal University Hospital, 103 Hospital Drive, Saskatoon, SK, Canada
2Section of Pediatric Endocrinology, University of Calgary, Alberta Children’s Hospital, 2888 Shaganappi Trail NW, Calgary, AB, Canada
3Department of Diagnostic Imaging, University of Calgary, Alberta Children’s Hospital, Calgary, AB, Canada
4Departments of Medical Genetics and Pediatrics, University of Calgary, Alberta Children’s Hospital, 2888 Shaganappi Trail NW, Calgary, AB, Canada

Received 4 August 2016; Revised 14 October 2016; Accepted 24 October 2016

Academic Editor: Tarak Srivastava

Copyright © 2016 Munier A. Nour et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


Hypopituitarism is a clinically important diagnosis and has not previously been reported in Hunter syndrome. We contrast two cases with anatomic pituitary anomalies: one with anterior panhypopituitarism and the other with intact pituitary function. Patient 1, a 10-year-old boy with Hunter syndrome, was evaluated for poor growth and an ectopic posterior pituitary gland. Endocrine testing revealed growth hormone (GH) deficiency, secondary adrenal insufficiency, and tertiary hypothyroidism. An improvement in growth velocity with hormone replacement (GH, thyroxine, and corticosteroid) was seen; however, final adult height remained compromised. Patient 2, a 13-year-old male with Hunter syndrome, was evaluated for growth failure. He had a large empty sella turcica with posteriorly displaced pituitary. Functional endocrine testing was normal and a trial of GH-treatment yielded no significant effect. Panhypopituitarism associated with pituitary anomalies has not been previously reported in Hunter syndrome and was an incidental finding of significant clinical importance. In the setting of documented anterior hypopituitarism, while hormone replacement improved growth velocity, final height remained impaired. In patient 2 with equivocal GH-testing results, treatment had no effect on linear growth. These cases highlight the importance of careful clinical assessment in Hunter syndrome and that judicious hormone replacement may be indicated in individual cases.