Abstract
Certain pituitary imaging abnormalities are a specific indicator of hypopituitarism. The objective of this study is to compare phenotypical features with radiological findings in patients with congenital growth hormone deficiency (GHD). Magnetic Resonance imaging (MRI) was performed in 103 patients [72 with Isolated GHD (IGHD) and 31 with Combined Pituitary Hormone Deficiency (CPHD)]. Images were assessed for the following abnormalities: (1) small/absent anterior pituitary, (2) thin or interrupted pituitary stalk (PSA), and (3) Ectopic posterior pituitary (EPP), and (4) others. Radiological findings were correlated with the clinical and biochemical parameters. MRI abnormalities were observed in 48.6% patients with IGHD, 93.5% with CPHD. Jaundice, hypoxia, hypoglycemia and breech deliveries were more common in EPP/PSA group. EPP/PSA was observed in 87.1% patients with severe GHD (peak GH < 3 μg/L) as compared to 12.9% with mild to moderate GHD (peak GH: 3–10 μg/L). Amongst CPHD, EPP/PSA was present in 80% of subjects with associated hypocortisolism ± hypothyroidism as compared to 18.2% of subjects with hypogonadism. Over a mean follow up period of 4.5 years, 5.4% of subjects with IGHD and abnormal MRI progressed to CPHD while none of those with normal MRI progressed. This study emphasizes a significant clinico-radiological correlation in Asian Indian GHD patients. MRI abnormalities in the hypothalamic pituitary area, especially EPP/PSA are more common in patients with CPHD and severe GHD. Among CPHD, EPP/PSA predicts association with hypothyroidism or hypocortisolism. IGHD with MRI abnormality may evolve into CPHD.
Similar content being viewed by others
References
Scotti G, Triulzi F, Chiumello G, Di Natale B et al (1989) New imaging techniques in endocrinology: magnetic resonance of the pituitary gland and sella turcica. Acta Paediatr Scand 356:5–14
Hamilton J, Blaser S, Daneman D et al (1998) MR imaging in idiopathic growth hormone deficiency. Am J Neuroradiol 19:1609–1615
Tillmann V, Tang VW, Price DA et al (2000) Magnetic resonance imaging of the hypothalamic pituitary axis in the diagnosis of growth hormone deficiency. J Pediatr Endocrinol Metab 13:1577–1583
Bozzola M, Mengarda F, Sartirana P et al (2000) Long-term follow-up evaluation of magnetic resonance imaging in the prognosis of permanent GH deficiency. Eur J Endocrinol 143:493–496
Maghnie M, Strigazzi C, Tinelli C, Autelli M et al (1999) Growth hormone (GH) deficiency (GHD) of childhood onset: reassessment of GH status and evaluation of the predictive criteria for permanent GHD in young adults. J Clin Endocrinol Metab 84:1324–1328
Kikuchi K, Fujisawa I, Momoi T et al (1988) Hypothalamic-pituitary function in growth hormone deficient patients with pituitary stalk transection. J Clin Endocrinol Metab 67:817–823
di Natale B, Pellini C, Ackermann S et al (1994) Persisting functional connection in growth-hormone-deficient patients with a transected stalk. Horm Res 41:193–196
Sloop KW, Walvoord EC, Showalter AD et al (2000) Molecular analysis of LHX3 and PROP-1 in pituitary hormone deficiency patients with posterior pituitary ectopia. J Clin Endocrinol Metab 85:2701–2708
Caravalho LR, Woods KS, Mendonca BB et al (2003) A homozygous mutation in HESX1 is associated with evolving hypopituitarism due to impaired repressor–corepressor interaction. J Clin Invest 112:1192–1201
Machinis K (2001) Syndromic short stature in patients with a germline mutation in the LIM homeobox LHX4. Am J Hum Genet 69:961–968
Woods KS, Cundall M, Turton J et al (2005) Over- and underdosage of SOX3 is associated with infundibular hypoplasia and hypopituitarism. Am J Hum Genet 76:833–849
Argyropoulou M, Perignon F, Brauner R et al (1992) Magnetic resonance imaging in the diagnosis of growth hormone deficiency. J Pediatr 120:886–891
Bozzola M, Adamsbaum C, Biscaldi I et al (1996) Role of magnetic resonance imaging in the diagnosis and prognosis of growth hormone deficiency. Clin Endocrinol 45:21–26
Dutta P, Bhansali A, Singh P et al (2009) Congenital hypopituitarism: clinico-radiological correlation. J Pediatr Endocrinol Metab 22(10):921–928
Agarwal DK, Agarwal KN, Upadhyay SK et al (1992) Physical and sexual growth pattern of affluent Indian children from 6 to 18 years of age. Indian Pediatr 29:1082–1203
Greulich WN, Pyle SI (1959) Radiographic atlas of skeletal development of the hand and wrist, 2nd edn. Stanford University Press, Stanford
Marshall WA, Tanner JM (1969) Variation in the pattern of pubertal changes in girls. Arch Dis Child 44:291–303
Marshall WA, Tanner JM (1970) Variation in the pattern of pubertal changes in boys. Arch Dis Child 45:13–23
Lee PA, Mazur T, Danish R et al (1980) Micropenis I: criteria, etiologies and classification. Johns Hopkins Med J 146:156–163
LaFranchi S (2008) Disorders of the thyroid gland. In: Kliegman RM (ed) Nelson textbook of pediatrics. W.B.Saunders, Philadelphia, pp 2316–2340
Argyropoulou M, Perignon F, Brunelle F et al (1991) Height of normal pituitary gland as a function of age evaluated by magnetic resonance imaging in children. Pediatr Radiol 21:247–249
Simmons GE, Suchnicki JE, Rak KM, Damiano TR (1992) MR imaging of the pituitary stalk: size, shape, and enhancement pattern. Am J Roentgenol 159:375–377
Acharya SV, Gopal RA, Lila AR et al (2011) Phenotype and radiological correlation in patients with growth hormone deficiency. Indian J Pediatr 78:49–54
Dutta DC (2006) Malpresentation and malposition. In: Textbook of obstetrics, 6th edn. New Central Book Agency, Kolkata, pp 375–381
Murray PG, Hague C, Fafoula O et al (2008) Associations with multiple pituitary hormone deficiency in patients with an ectopic posterior pituitary gland. Clin Endocrinol 69:597–602
Arrigo T, De Luca F, Maghnie M et al (1998) Relationships between neuroradiological and clinical features in apparently idiopathic hypopituitarism. Eur J Endocrinol 139:84–88
Bordallo MA, Tellerman LD, Bosignoli R et al (2004) Neuroradiological investigation in patients with idiopathic growth hormone deficiency. Jornal de Pediatria 80(3):223–228
Nagel BHP, Palmbach M, Petersen D et al (1997) Magnetic resonance images of 91 children with different causes of short stature: pituitary size reflects growth hormone secretion. Eur J Pediatr 156:758–763
Kornreich L, Horev G, Lazar L et al (1997) MR findings in hereditary isolated growth hormone deficiency. Am J Neuroradiol 18:1743–1747
Marcu M, Trivin C, Souberbielle JC et al (2008) Factors influencing the growth hormone peak and plasma insulin-like growth factor I in young adults with pituitary stalk interruption syndrome. BMC Endocrine Disorders 8:7
Tauber M, Chevrel J, Diene G et al (2005) Long-term evolution of endocrine disorders and effect of GH therapy in 35 patients with pituitary stalk interruption syndrome. Horm Res 64:266–273
Pinto G, Netchine I, Sobrier M et al (1997) Pituitary stalk interruption syndrome: a clinical- biological-genetic assessment of its pathogenesis. J Clin Endocrinol Metab 82:3450–3454
Chen S, Léger J, Garel C et al (1999) Growth hormone deficiency with ectopic neurohypophysis: anatomical variations and relationship between the visibility of the pituitary stalk asserted by magnetic resonance imaging and anterior pituitary function. J Clin Endocrinol Metab 84:2408–2413
Le′ger J, Danner S, Simon D et al (2005) Do all patients with childhood-onset growth hormone deficiency (GHD) and ectopic neurohypophysis have persistent GHD in adulthood? J Clin Endocrinol Metab 90:650–656
Louvel M, Marcu M, Trivin C et al (2009) Diagnosis of growth hormone (GH) deficiency: comparison of pituitary stalk interruption syndrome and transient GH deficiency. BMC Pediatr 9:29
Rottembourg D, Linglart A, Adamsbaum C et al (2008) Gonadotrophic status in adolescents with pituitary stalk interruption syndrome. Clin Endocrinol 69:105–111
Cacciari E, Zucchini S, Carla G et al (1990) Endocrine function and morphological findings in patients with disorders of hypothalamo-pituitary area: a study with magnetic resonance. Arch Dis Child 65:1199–1202
Conflicting interests and financial disclosure
None.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Jagtap, V.S., Acharya, S.V., Sarathi, V. et al. Ectopic posterior pituitary and stalk abnormality predicts severity and coexisting hormone deficiencies in patients with congenital growth hormone deficiency. Pituitary 15, 243–250 (2012). https://doi.org/10.1007/s11102-011-0321-4
Published:
Issue Date:
DOI: https://doi.org/10.1007/s11102-011-0321-4