Pituitary Stem Interruption Syndrome (SITP) diagnosed in childhood in a case at the Center Hospitalier Universitaire de Libreville (CHUL)
DOI:
https://doi.org/10.55677/IJCSMR/V2I12-01/2022Keywords:
Growth retardation, Congenital hypogonadotropic hypogonadism, pituitary stalk interruption syndromeAbstract
Interrupted pituitary stalk syndrome (PITS) is a hypothalamic-pituitary morphological abnormality characterized by a slender or absent pituitary stalk, anterior pituitary hypoplasia, and ectopic posterior pituitary. Hormonal assessment and imaging are crucial for the diagnosis, early management can improve the child's stature prognosis.
We report the case of an 8-year-old child seen in endocrinology consultation for growth retardation. His medical history revealed acute fetal distress at birth, neonatal jaundice, and numerous consultations in early childhood for this anomaly. The clinical examination found a statural delay of -3DS, bilateral cryptorchidism, with small testicles. The hormonal balance found multiple pituitary deficits and Magnetic Resonance Imaging showed an absence of the pituitary stalk, anterior pituitary hypoplasia, and ectopia of the posterior pituitary. Deficient hormone supplementation was instituted. This case relates the typical picture of hypopituitarism by the absence of the pituitary stalk in a context where the disease is probably underdiagnosed due to lack of technical support in sub-Saharan Africa and little known. This explains the diagnostic error in our case, moreover, it allows us to pay particular attention to the meticulous monitoring of the growth of children who have presented perinatal anomalies, and the multidisciplinary exploration in the event of growth disorders.
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