Pituitary stalk interruption syndrome

Case contributed by Dr Naim Qaqish

Presentation

Delayed puberty.

Patient Data

Age: 10 years
Gender: Male

Normal anterior lobe signal and enhancement, however, it appears at the lower limits of normal measuring around 2.5 mm.

The posterior lobe signal is not visualized with around 4 mm hyperintense signal in the hypothalamic region suggesting ectopic posterior lobe.

The distal part of the pituitary stalk is not visualized indicating agenesis.

Findings are indicating pituitary stalk interruption syndrome.

Case Discussion

This is a 10-year-old child known to have growth retardation, and delayed puberty. Upon evaluation of hormone levels growth hormone deficiency was reported. MRI of the pituitary region was ordered by his referring physician to rule out pituitary pathology. MRI findings were compatible with PSIS (pituitary stalk interruption syndrome A.K.A pituitary stalk transection syndrome) which is a congenital disorder characterized by a triad of, 

Initially can present as isolated growth hormone deficiency, and may progress to multiple pituitary hormones deficiencies with the eventual development of panhypopituitarism with preservation of posterior pituitary function.

The diagnosis of pituitary stalk interruption syndrome (PSIS) is confirmed by MRI as in this case.

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Case information

rID: 73108
Published: 28th Dec 2019
Last edited: 29th Dec 2019
Inclusion in quiz mode: Included

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