Visual Diagnosis Pituitary Stalk Interruption Syndrome Presenting With Growth Retardation Güliz Yılmaz MD * Near East University, Radiology, Nicosia, Turkey Patient Description This four-year-old boy presenting with short stature was examined using hypophysis magnetic resonance imaging (MRI). The results of the MRI scan revealed a normal anterior pituitary gland at the sella turcica, while the infundibulum was not detected on noncontrast or intravenous contrast-enhanced images. An ectopic neurohypophysis appeared as a hyperintense focus at the location of the pituitary stalk origin on T1-weighted images (Figure). Discussion Pituitary stalk interruption syndrome (PSIS) is a rare congenital anomaly of the hypophysis that is characterized by the triad of interrupted pituitary stalk, aplasia or hypo- plasia of the anterior pituitary, and an absent or ectopic posterior pituitary. Although the cause of PSIS has not fully understood, perinatal injury resulting in defective organogenesis has been suggested. 1 The clinical presentation of PSIS varies with the age at the time of diagnosis; manifestations may include neonatal hypoglycemia, prolonged neonatal jaundice, or cryptorchidism and micropenis in neonates or short stature in older children. 2,3 The clinical findings have also been related to isolated growth hormone deficiency or combined multiple pituitary hormone deficiency. 4 In addition to the clinical features of pituitary hormonal deficiency, the diagnosis is confirmed by demonstrating the abnormalities of the pituitary gland and hypothalamus FIGURE. (A) Sagittal T1-weighted magnetic resonance image showing a hyperintense ectopic neurohypophysis (thin arrow) and a small anterior pituitary gland (thick arrow) at the normal location. (B) The hypophysis infundibulum is not demonstrated on a coronal contrast-enhanced T1-weighted image with fat suppression (thick arrow). The hyperintense ectopic neurohypophysis (thin arrow) and the optic chiasm (curved arrow) on the either side of ectopic neurohypophysis are observed. * Communications should be addressed to: Dr. Yılmaz; Near East University; Radiology; 99100, Nicosia, Turkey. E-mail address: glz.yilmaz@hotmail.com Contents lists available at ScienceDirect Pediatric Neurology journal homepage: www.elsevier.com/locate/pnu 0887-8994/$ e see front matter Ó 2016 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.pediatrneurol.2016.05.002 Pediatric Neurology xxx (2016) 1e2