Email id: info@ijclinmedcasereports.com
Pituitary Stalk Interruption Syndrome: A Diagnosis Not to be Missed
Essetti S*, Faraj C, Chait F, Sfar K, Allali N, El Haddad S and Chat L
Department of Pediatric and Gynecology Radiology, Children's hospital, University Mohammed V, Rabat, Morocco
Received Date: 08/06/2023; Published Date: 19/10/2023
*Corresponding author: Essetti Sara, Department of Pediatric and Gynecology Radiology, Children's hospital, University Mohammed V, Rabat, Morocco
Pituitary stalk interruption syndrome (PSIS) is a rare congenital abnormality of the pituitary responsible for anterior pituitary deficiency. It is characterized by a triad of thin or interrupted pituitary stalk, small or absent pituitary gland, and ectopic posterior pituitary location [1]
The cause of PSIS is still unknown and many theories are proposed like mutations in the genes involved in pituitary embryogenesis or perinatal asphyxia [2]
Most cases of stalk transection syndrome present with growth retardation in childhood in association with GH deficiency. Posterior pituitary function is intact.
MRI is diagnostic showing the characteristic triad (Figure 1):
- Ectopic posterior pituitary (A). The ectopic neurohypophysis is most commonly seen in the infundibular recess or the hypothalamus.
- Thin or absent pituitary stalk (B): evaluated in postcontrast images
- Anterior pituitary hypoplasia (C)
Treatment consists of hormonal replacement.
Figure 1: Hypothalamic-pituitary MRI of a 5 years old girl with short stature and growth hormone deficiency:
A: Sagittal T1 weighted image showing ectopic posterior pituitary bright spot (green arrow).
B: Postcontrast coronal T1-weighted image showing non-visualization of the pituitary stalk.
C: Postcontrast Sagittal T1 weighted image showing anterior pituitary measuring 2 mm in height (blue arrow).
References
- Kulkarni C, Moorthy S, Pullara S, Rajeshkannan R, Unnikrishnan A. Pituitary stalk transection syndrome: Comparison of clinico-radiological features in adults and children with review of literature. Indian Journal of Radiology and Imaging, 2012; 22(3): 182.
- Loachimescu AG, Hamrahian AH, Stevens M, et-al. The pituitary stalk transection syndrome: multifaceted presentation in adulthood. Pituitary, 2013; 15(3): 405-411.
