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Hypothalamic-Pituitary Function in Children With Optic Nerve Hypoplasia

Hypothalamic-Pituitary Function in Children With Optic Nerve Hypoplasia Abstract • We studied the clinical characteristics and hypothalamic-pituitary function in 23 patients with optic nerve hypoplasia (ONH), 6 months to 19 years old. All patients had decreased visual acuity and small optic discs; the septum pellucidum was absent in five of 19. Nine of 11 patients had minor EEG abnormalities, and two had microcephaly. The height, weight, and growth rate were normal in all patients 6 months to 3½ years old. Hypopituitarism was found in 15 patients. Fasting and stimulated prolactin levels and the area under the prolactin curve after thyrotropin releasing hormone were significantly greater than in controls and in patients with idiopathic hypopituitarism. These results associate ONH with a high incidence of hypopituitarism, hyperprolactinemia, and neurologic abnormalities. The normal growth in the absence of measurable growth hormone suggests that hyperprolactinemia may stimulate growth temporarily and that a normal height in childhood or the presence of the septum pellucidum do not exclude the possibility of hypopituitarism. (AJDC 1985;139:249-254) References 1. De Morsier G: Etudes sur les dysraphies cranio-encephaliques: III. Agenesie du septum lucidum avec malformation du tractus optique: La dysplasie septo-optique . Schweiz Arch Neurol Neurochir Psychiatr 1956;77:267-292. 2. Hoyt WF, Kaplan SL, Grumbach MM, et al: Septo-optic dysplasia and pituitary dwarfism . Lancet 1970;1:893-894.Crossref 3. Billson F, Hopkins IJ: Optic hypoplasia and hypopituitarism . Lancet 1972;1:905.Crossref 4. Harris RH, Haas L: Septo-optic dysplasia with growth hormone deficiency (De Morsier syndrome) . Arch Dis Child 1972;47:973-976.Crossref 5. Patel H, Tze WH, Chrichton JU, et al: Optic nerve hypoplasia with hypopituitarism . AJDC 1975;129:175-180. 6. Huseman CA, Kelch RP, Hopwood NJ, et al: Sexual precocity in association with septo-optic dysplasia and hypothalamic hypopituitarism . J Pediatr 1978;92:748-753.Crossref 7. Sheridan SJ, Robb RM: Optic nerve hypoplasia with diabetes insipidus . J Pediatr Ophthalmol Strabismus 1978;15:82-84. 8. Toublanc JE, Chaussain JL, Lejeune C, et al: Hypopituitarism avec hypoplasie des nerfs optiques . Arch Fr Pediatr 1976;33:67-75. 9. Costin G, Murphree L: Optic nerve hypoplasia with hypopituitarism, hyperprolactinemia and normal growth in infancy, abstract 72. Program and abstracts of the first joint meeting of the Lawson Wilkins Pediatric Society and the European Society for Pediatric Endocrinology, Geneva, Sept 9-11, 1981. 10. Brook CG, Sanders MD, Hoare RD: Septooptic dysplasia , Br Med J 1972;3:811-813.Crossref 11. Polomeno RC, Staudenmaier C, Guyda HJ, et al: Ocular defects and short stature . Can J Ophthalmol 1980;15:250-255. 12. Foley PT, Jacobs ST, Hoffman W, et al: Human prolactin and thyrotropin concentrations in the serum of normal and hypopituitary children before and after the administration of synthetic thyrotropin releasing hormone . J Clin Endocrinol 1972;51:2143-2150. 13. Granvill WA, Smith PF, Laugky WR: Elements of Differential and Integram Calculus . New York, Granville, 1957, pp 247-250. 14. Petersen RA, Walton DS: Optic nerve hypoplasia with good visual acuity and visual field defects: A study of children of diabetic mothers . Arch Ophthalmol 1977;95:254-258.Crossref 15. McKinna AJ: Quinidine induced hypoplasia of the optic nerve . Can J Ophthalmol 1966; 1:261-265. 16. Ellenberger C, Runyan TE: Holoprosencephaly with hypoplasia of the optic nerves, dwarfism, and agenesis of the septum pellucidum . Am J Ophthalmol 1970;70:960-967. 17. Wilson PT, Easley RB, Bolander FF, et al: Evidence of hypothalamic defect in septo-optic dysplasis . Arch Intern Med 1978;138:1276-1277.Crossref 18. Costin G, Kogut MD, Phillips LS, et al: Craniopharyngioma: The role of insulin in promoting postoperative growth . J Clin Endocrinol 1976;42:370-379.Crossref 19. Kenny FM, Guyda HJ, Wright JC, et al: Prolactin and somatomedin in hypopituitary patients with "catch up" growth following operations for craniopharyngioma . J Clin Endocrinol 1973;36:378-383.Crossref 20. Bucher H, Zapf J, Torresani T, et al: Insulin-like growth factors I and II, prolactin, and insulin in 19 growth hormone–deficient children with excessive, normal, or decreased longitudinal growth after operation for craniopharyngioma . N Engl J Med 1983;309:1142-1146.Crossref 21. McGarry EE, Beck JC: Some metabolic effects of ovine prolactin in man . Lancet 1962;4: 915-916.Crossref 22. Hurley TW, D'Ercole AJ, Handwerger S, et al: Ovine placental lactogen induces somatomedin: A possible role in fetal growth . Endocrinology 1977;101:1635-1639.Crossref 23. Bala RM, Bohnet HG, Carter JN, et al: Effect of ovine prolactin on serum somatomedin bioactivity in hyophysectomized female rats . Can J Physiol Pharmacol 1978;56:984-989.Crossref 24. Holder AT, Wallis M, Biggs P, et al: Effects of growth hormone, prolactin and thyroxine on body weight, somatomedin-like activity and in vivo sulphation of cartilage in hypopituitary dwarf mice . Endocrinology 1980;85:35-40.Crossref 25. Clemmons DR, Underwood LE, Ridgway EC, et al: Hyperprolactinemia is associated with increased immunoreactive somatomedin C in hypopituitarism . J Clin Endocrinol 1980;52: 731-735.Crossref 26. Bruyn GW: Handbook of Clinical Neurology . Amsterdam, North-Holland Publishing Co, 1977, vol 30, pp 299-336. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png American Journal of Diseases of Children American Medical Association

Hypothalamic-Pituitary Function in Children With Optic Nerve Hypoplasia

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References (26)

Publisher
American Medical Association
Copyright
Copyright © 1985 American Medical Association. All Rights Reserved.
ISSN
0002-922X
DOI
10.1001/archpedi.1985.02140050043019
Publisher site
See Article on Publisher Site

Abstract

Abstract • We studied the clinical characteristics and hypothalamic-pituitary function in 23 patients with optic nerve hypoplasia (ONH), 6 months to 19 years old. All patients had decreased visual acuity and small optic discs; the septum pellucidum was absent in five of 19. Nine of 11 patients had minor EEG abnormalities, and two had microcephaly. The height, weight, and growth rate were normal in all patients 6 months to 3½ years old. Hypopituitarism was found in 15 patients. Fasting and stimulated prolactin levels and the area under the prolactin curve after thyrotropin releasing hormone were significantly greater than in controls and in patients with idiopathic hypopituitarism. These results associate ONH with a high incidence of hypopituitarism, hyperprolactinemia, and neurologic abnormalities. The normal growth in the absence of measurable growth hormone suggests that hyperprolactinemia may stimulate growth temporarily and that a normal height in childhood or the presence of the septum pellucidum do not exclude the possibility of hypopituitarism. (AJDC 1985;139:249-254) References 1. De Morsier G: Etudes sur les dysraphies cranio-encephaliques: III. Agenesie du septum lucidum avec malformation du tractus optique: La dysplasie septo-optique . Schweiz Arch Neurol Neurochir Psychiatr 1956;77:267-292. 2. Hoyt WF, Kaplan SL, Grumbach MM, et al: Septo-optic dysplasia and pituitary dwarfism . Lancet 1970;1:893-894.Crossref 3. Billson F, Hopkins IJ: Optic hypoplasia and hypopituitarism . Lancet 1972;1:905.Crossref 4. Harris RH, Haas L: Septo-optic dysplasia with growth hormone deficiency (De Morsier syndrome) . Arch Dis Child 1972;47:973-976.Crossref 5. Patel H, Tze WH, Chrichton JU, et al: Optic nerve hypoplasia with hypopituitarism . AJDC 1975;129:175-180. 6. Huseman CA, Kelch RP, Hopwood NJ, et al: Sexual precocity in association with septo-optic dysplasia and hypothalamic hypopituitarism . J Pediatr 1978;92:748-753.Crossref 7. Sheridan SJ, Robb RM: Optic nerve hypoplasia with diabetes insipidus . J Pediatr Ophthalmol Strabismus 1978;15:82-84. 8. Toublanc JE, Chaussain JL, Lejeune C, et al: Hypopituitarism avec hypoplasie des nerfs optiques . Arch Fr Pediatr 1976;33:67-75. 9. Costin G, Murphree L: Optic nerve hypoplasia with hypopituitarism, hyperprolactinemia and normal growth in infancy, abstract 72. Program and abstracts of the first joint meeting of the Lawson Wilkins Pediatric Society and the European Society for Pediatric Endocrinology, Geneva, Sept 9-11, 1981. 10. Brook CG, Sanders MD, Hoare RD: Septooptic dysplasia , Br Med J 1972;3:811-813.Crossref 11. Polomeno RC, Staudenmaier C, Guyda HJ, et al: Ocular defects and short stature . Can J Ophthalmol 1980;15:250-255. 12. Foley PT, Jacobs ST, Hoffman W, et al: Human prolactin and thyrotropin concentrations in the serum of normal and hypopituitary children before and after the administration of synthetic thyrotropin releasing hormone . J Clin Endocrinol 1972;51:2143-2150. 13. Granvill WA, Smith PF, Laugky WR: Elements of Differential and Integram Calculus . New York, Granville, 1957, pp 247-250. 14. Petersen RA, Walton DS: Optic nerve hypoplasia with good visual acuity and visual field defects: A study of children of diabetic mothers . Arch Ophthalmol 1977;95:254-258.Crossref 15. McKinna AJ: Quinidine induced hypoplasia of the optic nerve . Can J Ophthalmol 1966; 1:261-265. 16. Ellenberger C, Runyan TE: Holoprosencephaly with hypoplasia of the optic nerves, dwarfism, and agenesis of the septum pellucidum . Am J Ophthalmol 1970;70:960-967. 17. Wilson PT, Easley RB, Bolander FF, et al: Evidence of hypothalamic defect in septo-optic dysplasis . Arch Intern Med 1978;138:1276-1277.Crossref 18. Costin G, Kogut MD, Phillips LS, et al: Craniopharyngioma: The role of insulin in promoting postoperative growth . J Clin Endocrinol 1976;42:370-379.Crossref 19. Kenny FM, Guyda HJ, Wright JC, et al: Prolactin and somatomedin in hypopituitary patients with "catch up" growth following operations for craniopharyngioma . J Clin Endocrinol 1973;36:378-383.Crossref 20. Bucher H, Zapf J, Torresani T, et al: Insulin-like growth factors I and II, prolactin, and insulin in 19 growth hormone–deficient children with excessive, normal, or decreased longitudinal growth after operation for craniopharyngioma . N Engl J Med 1983;309:1142-1146.Crossref 21. McGarry EE, Beck JC: Some metabolic effects of ovine prolactin in man . Lancet 1962;4: 915-916.Crossref 22. Hurley TW, D'Ercole AJ, Handwerger S, et al: Ovine placental lactogen induces somatomedin: A possible role in fetal growth . Endocrinology 1977;101:1635-1639.Crossref 23. Bala RM, Bohnet HG, Carter JN, et al: Effect of ovine prolactin on serum somatomedin bioactivity in hyophysectomized female rats . Can J Physiol Pharmacol 1978;56:984-989.Crossref 24. Holder AT, Wallis M, Biggs P, et al: Effects of growth hormone, prolactin and thyroxine on body weight, somatomedin-like activity and in vivo sulphation of cartilage in hypopituitary dwarf mice . Endocrinology 1980;85:35-40.Crossref 25. Clemmons DR, Underwood LE, Ridgway EC, et al: Hyperprolactinemia is associated with increased immunoreactive somatomedin C in hypopituitarism . J Clin Endocrinol 1980;52: 731-735.Crossref 26. Bruyn GW: Handbook of Clinical Neurology . Amsterdam, North-Holland Publishing Co, 1977, vol 30, pp 299-336.

Journal

American Journal of Diseases of ChildrenAmerican Medical Association

Published: Mar 1, 1985

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