pituitary stalk interruption syndrome: magnetic resonance findings

School ager

with growth hormone deficiency. Sagittal (above) and coronal (below) T1 MRI without contrast of the sella shows absence of the pituitary stalk between the sella and the hypothlamus. In addition, the high signal intensity posterior pituitary bright spot, which should be located posteriorly in the sella turcica, is instead located in the inferior aspect of the hypothalamus.The diagnosis was pituitary interrupted stalk syndrome and ectopic posterior pituitary.

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Pituitary

dwarf with ectopic neurohypophysis. Posterior pituitary bright spot is seen at the level of median eminence. This is suggestive of ectopic posterior pituitary. Pituitary stalk is thin or absent and adenohypophysis is small in size.

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Pituitary

stalk transection syndrome in a 16 years old girl presenting with short stature and delayed puberty – A case report. Barely perceptible midline pituitary stalk which could hardly be figured out on Coronal MR image

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Pituitary

stalk transection syndrome in a 16 years old girl presenting with short stature and delayed puberty – A case report. Non enhancing imperceptible pituitary stalk with ectopic posterior pituitary

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Pituitary

stalk transection syndrome in a 16 years old girl presenting with short stature and delayed puberty – A case report. Small sized pituitary gland with absent posterior pituitary bright spot and ectopic posterior pituitary bright spot (arrow)

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Ectopic

neurohypophysis in a child with anterior hypopituitarism. MRI of the pituitary gland T1 weighted sagittal image showing ectopic neurohypophysis, hypoplastic anterior pituitary gland and absent pituitary stalk.

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Ectopic

neurohypophysis in a child with anterior hypopituitarism. T1 fat saturation image showing hyperintense signal of ectopic neurohypophysis differentiating from lipoma.

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Pituitary stalk interruption syndrome, also known as pituitary stalk transection syndrome, is a syndrome characterized by an absent or hypoplastic anterior pituitary gland, thin or absent infundibulum, and ectopic posterior pituitary location.

Epidemiology

Pituitary stalk interruption syndrome is very rare with an estimated incidence of 0.5 in 1 million births .

Clinical presentation

Patients with pituitary stalk interruption usually present in the first decade of life with deficiency of growth hormones resulting in growth retardation . What can initially present as isolated growth hormone deficiency may progress to multiple pituitary hormones deficiencies with the eventual development of panhypopituitarism with preservation of posterior pituitary function .

The combination of hyperprolactinemia and hypothyroidism is known as Pickardt syndrome .

In addition to endocrinological manifestations, there appears to be an increased incidence of breech presentation and difficult delivery . A number of additional features may be encountered later in childhood including learning difficulties and seizures .

Pathology

Various hypotheses have been proposed to explain the syndrome such as defective migration of the pituitary gland during intrauterine life or ischemia with subsequent reorganization of the infundibular axons and development of an ectopic posterior pituitary .