Ann Pediatr Endocrinol Metab.  2017 Sep;22(3):208-212. 10.6065/apem.2017.22.3.208.

Delayed diagnosis of pituitary stalk interruption syndrome with severe recurrent hyponatremia caused by adrenal insufficiency

• Jang KM
• Ko CW

• KMID: 2396142
• DOI: http://doi.org/10.6065/apem.2017.22.3.208

Abstract

Pituitary stalk interruption syndrome (PSIS) involves the occurrence of a thin or absent pituitary stalk, hypoplasia of the adenohypophysis, and ectopic neurohypophysis. Diagnosis is confirmed using magnetic resonance imaging. Patients with PSIS have a variable degree of pituitary hormone deficiency and a wide spectrum of clinical manifestations. The clinical course of the disease in our patient is similar to that of a syndrome of inappropriate antidiuretic hormone secretion. This is thought to be caused by failure in the suppression of vasopressin secretion due to hypocortisolism. To the best of our knowledge, there is no case report of a patient with PSIS presenting with hyponatremia as the first symptom in Korean children. Herein, we report a patient with PSIS presenting severe recurrent hyponatremia as the first symptom, during adolescence and explain the pathophysiology of hyponatremia with secondary adrenal insufficiency.

Keyword

Pituitary stalk interruption syndrome; Hyponatremia; Hypopituitarism; Inappropriate ADH syndrome; Adrenal insufficiency

MeSH Terms

Adolescent
Adrenal Insufficiency*
Child
Delayed Diagnosis*
Diagnosis
Humans
Hyponatremia*
Hypopituitarism
Inappropriate ADH Syndrome
Magnetic Resonance Imaging
Pituitary Gland*
Pituitary Gland, Anterior
Pituitary Gland, Posterior
Vasopressins
Vasopressins

Figure

• 

  Fig. 1. The patient's height from 6 to 13 years of age was based on school physical records and was not measured in the last 8 years. The growth curve showed markedly short stature and growth rates less than 5 cm/yr during childhood.

• 

  Fig. 2. The left-hand anteroposterior view represents between 14 and 15 years of bone age using the standards of the Greulich-Pyle method, which is delayed compared to chronological age of 24 year-old.

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  Fig. 3. The arrows indicate absent pituitary stalk, hypoplasia of the adenohypophysis, and absent posterior pituitary gland on sella magnetic resonance imaging.

Reference

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