Abstract
Pituitary imaging abnormality is a specific indicator of hypopituitarism. This study involved a retrospective review of 59 children diagnosed with pituitary stalk interruption syndrome (PSIS). Of the 59 eligible patients, 54 were born by breech delivery, and there was a significant difference between numbers of patients with breech and head-presenting birth. In order to discuss the relationship between pituitary functions and delineation of pituitary structure in magnetic resonance imaging (MRI), a control analysis was carried out in children with PSIS. Fifty-nine children were subdivided into two groups: group I (partial PSIS, 20 cases) and group II (complete PSIS, 39 cases). There was a significantly small anterior pituitary in both groups of PSIS compared with controls (P < 0.001). The incidence of ectopic posterior pituitary (EPP) was significantly higher in group II (P < 0.001). Before and after hormone replacement therapy, pituitary functions were measured and compared with controls. The levels of growth hormone (GH), free thyroxine (FT4), thyroid-stimulating hormone (TSH), adrenocorticotropic hormone (ACTH), and cortisol (COR) were significantly lower in group II (P < 0.05). The dosage of levothyroxine sodium in group II was significantly higher than in group I (P < 0.01). Conclusion. On the basis of birth history, breech presentation may a forewarning for subsequent pituitary hormone deficiencies. Grades of MRI can predict occurrence and severity of PSIS, which are also correlated with the levels of the pituitary target hormone deficiencies. Interruption of pituitary stalk and ectopic posterior pituitary both represent important markers of pituitary structure and function.
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Acharya SV, Gopal RA, Lila A, Sanghvi DS, Menon PS, Bandgar TR, Shah NS (2011) Phenotype and radiological correlation in patients with growth hormone deficiency. Indian J Pediatr 78(1):49–54
Arslanoğlu I, Kutlu H, Işgüven P, Tokuş F, Işik K (2001) Diagnostic value of pituitary MRI in differentiation of children with normal growth hormone secretion, isolated growth hormone deficiency and multiple pituitary hormone deficiency. J Pediatr Endocrinol Metab 14(5):517–523
Davis SW, Castinetti F, Carvalho LR, Ellsworth BS, Potok MA, Lyons RH, Brinkmeier ML, Raetzman LT, Carninci P, Mortensen AH, Hayashizaki Y, Arnhold IJ, Mendonça BB, Brue T, Camper SA (2010) Molecular mechanisms of pituitary organogenesis: in search of novel regulatory genes. Mol Cell Endocrinol 323(1):4–19
Di Iorgi N, Allegri AE, Napoli F, Bertelli E, Olivieri I, Rossi A, Maghnie M (2012) The use of neuroimaging for assessing disorders of pituitary development. Clin Endocrinol (Oxf) 76(2):161–176
El Chehadeh S, Bensignor C, de Monléon JV, Méjean N, Huet F (2010) The pituitary stalk interruption syndrome: endocrine features and benefits of growth hormone therapy. Ann Endocrinol (Paris) 71(2):102–110
Ikram MF, Sajjad Z, Shokh I, Omair A (2008) Pituitary height on magnetic resonance imaging observation of age and sex related changes. J Pak Med Assoc 58(5):261–265
Jagtap VS, Acharya SV, Sarathi V, Lila AR, Budyal SR, Kasaliwal R, Sankhe SS, Bandgar TR, Menon PS, Shah NS (2012) Ectopic posterior pituitary and stalk abnormality predicts severity and coexisting hormone deficiencies in patients with congenital growth hormone deficiency. Pituitary 15(2):243–250
Kalina MA, Kalina-Faska B, Gruszczyńska K, Baron J, Małecka-Tendera E (2012) Usefulness of magnetic resonance findings of the hypothalamic-pituitary region in the management of short children with growth hormone deficiency: evidence from a longitudinal study. Childs Nerv Syst 28(1):121–127
Maghnie M, Genovese E, Villa A, Spagnolo L, Campan R, Severi F (1996) Dynamic MRI in the congenital agenesis of the neural pituitary stalk syndrome: the role of the vascular pituitary stalk in predicting residual anterior pituitary function. Clin Endocrinol (Oxf) 45(3):281–290
Maghnie M, Salati B, Bianchi S, Rallo M, Tinelli C, Autelli M, Aimaretti G, Ghigo E (2001) Relationship between the morphological evaluation of the pituitary and the growth hormone (GH) response to GH-releasing hormone Plus arginine in children and adults with congenital hypopituitarism. J Clin Endocrinol Metab 86(4):1574–1579
Qi Wei P, Hui Juan Z, Feng Ying G, Nai Shi L, Tao Z, Gang B, Hui P, Xian-wei Z (2011) Magnetic resonance image of sellar region in pituitary stalk interruption syndrome in children and adolescents. Zhongguo Yi Xue Ke Xue Yuan Xue Bao 33(1):9–13
Reynaud R, Albarel F, Saveanu A, Kaffel N, Castinetti F, Lecomte P, Brauner R, Simonin G, Gaudart J, Carmona E, Enjalbert A, Barlier A, Brue T (2011) Pituitary stalk interruption syndrome in 83 patients: novel HESX1 mutation and severe hormonal prognosis in malformative forms. Eur J Endocrinol 164(4):457–465
Rottembourg D, Linglart A, Adamsbaum C, Lahlou N, Teinturier C, Bougnères P, Carel JC (2008) Gonadotrophic status in adolescents with pituitary stalk interruption syndrome. Clin Endocrinol (Oxf) 69(1):105–111
Seminara S, Stagi S, Candura L, Scrivano M, Lenzi L, Nanni L, Pagliai F, Chiarelli F (2005) Changes of thyroid function during long-term hGH therapy in GHD children. A possible relationship with catch-up growth? Horm Metab Res 37(12):751–756
Simmons GE, Suchnicki JE, Rak KM, Damiano TR (1992) MR imaging of the pituitary stalk: size, shape, and enhancement pattern. AJR Am J Roentgenol 159(2):375–377
Tauber M, Chevrel J, Diene G, Moulin P, Jouret B, Oliver I, Pienkowski C, Sevely A (2005) Long-term evalution of endocrine disorders and effect of GH therapy in 35 patients with pituitary stalk interruption syndrome. Horm Res 64(6):266–273
Triulzi F, Scotti G, di Natale B, Pellini C, Lukezic M, Scognamiglio M, Chiumello G (1994) Evidence of a congenital midline brain anomaly in pituitary dwarfs: a magnetic resonance imaging study in 101 patients. Pediatrics 93(3):409–416
Tsai SL, Laffan E, Lawrence S (2012) A retrospective review of pituitary MRI findings in children on growth hormone therapy. Pediatr Radiol 42(7):799–804
Zimmermann A, Schenk JP, Grigorescu Sido P, Pfaffle R, Lazea C, Zimmermann T, Heinrich U, Weber MM, Bettendorf M (2007) MRI findings and genotype analysis in patients with childhood onset growth hormone deficiency–correlation with severity of hypopituitarism. J Pediatr Endocrinol Metab 20(5):587–596
Acknowledgments
This study was supported by a grant from Shandong Science and Technology Commission (No. 2013GSF11817), China. The sponsors had no role in the design and conduct of the study; collection, management, analysis, and interpretation of the data; or preparation of the manuscript. The authors have disclosed no relevant financial relationship. We are grateful to all the patients involved in this study. We would like to thank editorial assistance in the preparation of this manuscript.
Conflict of interest
Dr. Li is an employee of Provincial Hospital affiliated to Shandong University and has applied for funding of the above research. Qian Wang, Yanyan Hu, and Xiaojun Sun are all graduate students of Provincial Hospital affiliated to Shandong University. Dr. Li took charge of this study. All authors declare that they have no conflict of interest.
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Wang, Q., Hu, Y., Li, G. et al. Pituitary stalk interruption syndrome in 59 children: the value of MRI in assessment of pituitary functions. Eur J Pediatr 173, 589–595 (2014). https://doi.org/10.1007/s00431-013-2214-1
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DOI: https://doi.org/10.1007/s00431-013-2214-1