Martell GMR, Cuevas BMA

Language: Spanish
References: 9
Page: 32-36
PDF size: 222.62 Kb.

ABSTRACT

Pituitary stalk disruption syndrome is a congenital anomaly characterized by neuroradiologic demonstration of an absent, interrupted, or hypoplastic pituitary stalk, aplastic/ hypoplastic adenohypophysis, and ectopic neurohypophysis. This syndrome has been related to severe forms of congenital hypopituitarism, associated with multiple deficiencies of pituitary hormones. Perinatal signs and symptoms presented by patients include hypoglycemia in up to 61%, jaundice in 38%, micropenis in 77% and cholestasis in 19%, neonatal seizures occurred in 75% of children. During childhood, they tend to have short stature and a decrease in growth speed, as well as a delay in the expression of secondary sexual characteristics.
In our clinical case, an adolescent patient was presented whose main clinical manifestations were delayed secondary sexual characteristics, the main findings were found in nuclear magnetic resonance, including absence of the pituitary stalk, ectopic neurohypophysis, located adjacent to the tuber cinereum and hypoplastic adenohypophys.

REFERENCES

1. Castro LC, Martín SE, Bulacio S, Dichko D, Silvano L, Sobrero G, Miras MB. Síndrome de interrupción del tallo pituitario. Relaciones clínicas, bioquímicas y neurorradiológicas. Revista argentina de endocrinología y metabolismo. 2017;54(4):151-159. https://doi.org/10.1016/j. raem.2017.08.001

2. Arslan H, Saylık M, Akdeniz H. MRI findings of coexistence of ectopic neurohypophysis, corpus callosum dysgenesis, and periventricular neuronal heterotopia. J Clin Imaging Sci. 2014;4:22.

3. Sandoval-Pacheco R, Ramos-Avellaneda L, Vázquez-Galeana JA, Bravo-Valle J. Disgenesia del tallo hipofisario en derechohabientes menores de 15 años de edad del Sector Salud Militar: descripción de 3 casos. Rev Sanid Milit Mex. 2017;69:579-84. Disponible en: https://www.medigraphic. com/pdfs/sanmil/sm-2015/sm156j.pdf

4. Van der Linden AS, van Es. HW Case 112: pituitary stalk transection syndrome with ectopic posterior pituitary gland. Radiology. 2007;243(2):594-59.

5. Lahiri AK, Sundareyan R, Jenkins D, Nilak A. MRI of ectopic posterior pituitary gland with dysgenesis of pituitary stalk in a patient with hypogonadotropic hypogonadism. Radiol Case Rep. 2018;13(4):764-76.

6. Léger J, Danner S, Simon D, Garel C, Czernichow P. Do all patients with childhood-onset growth hormone deficiency (GHD) and ectopic neurohypophysis have persistent GHD in adulthood? J Clin Endocrinol Metab. 2005; 90(2):650-65.

7. Franulic Guggiana M, Vásquez olmos C, Carrillo García M, Núñez Peynado EM, Veas López AB; Murcia/ES. Hipófisis. Anatomía y principal patología tumoral. Congress SERAM 2014. Póster: S-1009. https://dx.doi.org/10.1594/ seram2014/S-1009

8. Shields R, Mangla R, Almast J, et al. Magnetic resonance imaging of sellar and juxtasellar abnormalities in the paediatric population: an imaging review. Insights Imaging. 2015:6:241-60. https://doi.org/10.1007/s13244-015- 0401-5

9. Voutetakis A, Sertedaki A, Dacou-Voutetakis C. Pituitary stalk interruption syndrome: Cause, clinical manifestations, diagnosis, and management. Curr Opin Pediatr. 2016;28:545-50.