IS Case 618: Congenital absence of anterior pituitary gland
Imaging Sciences URMC 2011
Publication Date: 2011-12-12
History
Patient is a 24-year-old female with history of panhypopituitarism of unknown cause since infancy.
Findings
MRI with and without contrast demonstrated absence of anterior pituitary gland, with an ectopically high posterior pituitary connected to a short infundibular stalk. In addition, the sella is flat, which likely indicates that the anterior pituitary was not formed.
Diagnosis
Discussion
Congenital absence of the anterior pituitary gland is a rare disorder, with the majority of cases ending with neonatal death. One of the more recent cases was presented in the December 2006 issue of Radiology, in which the newborn patient presented with hypoglycemia and other abnormalities.
In the setting of panhypopituitarism, absence of an identifiable anterior pituitary gland together with a flat sella strongly suggests the diagnosis of congenital absence (aplasia) of the anterior pituitary. The flatten sella is a result of the lack of normal modeling of the sella by the mass effect of the anterior pituitary.
In addition to the absent anterior pituitary, this case demonstrates an ectopically high posterior pituitary connected to a shortened infundibular stalk. Although possible, a hypoplastic ectopic anterior pituitary connected to the shortened infundibular stalk or elsewhere is much less likely.
References
- Cervantes LF, Altman NR, Medina LS. Case 102: pituitary aplasia. Radiology 2006 Dec;241(3):936-8. PMID: 17114634
- Zucchini S, Ambrosetto P, Carlà G, Tani G, Franzoni E, Cacciari E. Primary empty sella: differences and similarities between children and adults. Acta Paediatr 1995 Dec;84(12):1382-5. PMID: 8645956
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