Guido RA, Calderón VCA, Hernández OP, Rocha AJM

Language: Spanish
References: 8
Page: 35-39
PDF size: 152.16 Kb.

ABSTRACT

Interrupted pituitary stalk syndrome is a rare congenital disorder, clinically characterized by deficiency of growth hormone secretion, either isolated or associated with deficiencies of other anterior pituitary hormones. Radiologically, it is identified by absence or interruption of the pituitary stalk, ectopia of the posterior pituitary, and hypoplasia or absence of the anterior pituitary.
We report the case of a female patient admitted due to recurrent episodes of upper gastrointestinal bleeding of variceal origin, who underwent multiple esophageal variceal ligations and received multidisciplinary management by the gastroenterology service. She presented with generalized colitis, chronic liver disease, portal hypertension, portosystemic collateral circulation, hepatosplenomegaly, and ascites, as well as a gynecological history of absent development of secondary sexual characteristics and uterine hypoplasia. The syndromic phenotype prompted imaging studies, including brain magnetic resonance imaging, which confirmed findings consistent with pituitary stalk interruption syndrome.
In patients with hypopituitarism, progression from non-alcoholic fatty liver disease (NASH) to decompensated cirrhosis may occur within an average of 6.9 years, demonstrating that pituitary hormone deficiency accelerates liver damage and its clinical progression. Therefore, in clinical practice, pituitary hormone insufficiency should be considered a possible etiology in cases of unexplained liver disease.
Due to its low prevalence and adverse outcome in the absence of early diagnosis, pituitary stalk interruption syndrome requires special attention. Delays in initiating treatment may lead to severe complications, including death during the first decades of life. Early identification through imaging studies, particularly brain magnetic resonance imaging, is essential for appropriate therapeutic management and follow-up of these patients.

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