Figure 1
MRI of pituiatary transection syndrome
MRI of the pituitary gland sagittal post contrast images showing ectopic posterior pituitary gland, small anterior pituitary gland and absent pituitary stalk.
Pituitary transection syndrome
SectionNeuroradiology
Case TypeClinical Cases
AuthorsMohamed Khaled Metkees MD1, 2, Jayapalli Rajiv Bapuraj MD1, Ashok Srinivasan MD1
Connected authors
14 years, female
Clinical History
A 14-year-old female patient on thyroid hormone replacement therapy presented for delayed puberty, retarded milestones of development and below-average learning success. Bone age examinations revealed that skeletal maturation was approximately 45-47 months. Laboratory examinations showed low FSH, LH, cortisol, IGF-1, IGFBP-3, and free T4. Chromosome analysis revealed normal female karyotype.
Imaging Findings
MRI of the pituitary gland revealed that the posterior pituitary gland appeared ectopic, demonstrating high T1 signal intensity in the midline at the median eminence. Additionally, the anterior pituitary gland appeared small with an absent pituitary stalk. The pituitary stalk was not visible on pre- and postcontrast imaging. Conglomeration of findings consisting of the ectopic posterior pituitary gland, small anterior pituitary gland and absent pituitary stalk can be been seen in the setting of pituitary stalk transection syndrome.
Discussion
The pituitary stalk transection was described after introduction of MRI with the following characteristics: small anterior pituitary gland, thin or absent infundibulum after gadolinium administration and ectopic posterior pituitary location [1].
Imaging predictors of hormonal phenotype in patients with stalk transection syndrome (MPHD versus isolated GH deficiency) have been proposed. In a study of 14 patients with hypopituitarism aged 10–25 years, a thin visible infundibulum was identified in 8 after gadolinium administration; among them, 6 had isolated GH deficiency and 2 MPHD. In the same study, 5 of the 6 children in whom the infundibulum was not identified had MPHD [2]. A third study in 25 children with hypopituitarism aged 0.9–17.4 years, 12 of 14 with a visible infundibulum had isolated GHD. In the same study, 10 of 11 children without a visible infundibulum had MPHD. This study also suggested that ectopic posterior pituitary gland location at the level of the median eminence was more likely to be associated with MPHD (11/11 patients), while its location at lower stalk levels was associated with isolated GHD (12/14 patients) [3]. The most recent study of 26 adult patients with hypopituitarism did not support an association between the degree of hypopituitarism and pituitary stalk visibility or location of the ectopic posterior gland [4]. These discordant findings have probably a two-fold explanation: different timing of hormonal evaluation across studies and insufficient statistical power due to the small numbers of patients [5].
The pituitary stalk transection (dysgenesis) syndrome should be considered in adults with hypopituitarism. Adult endocrinologists diagnose this entity in patients who were previously thought to have idiopathic GH deficiency or MPHD. The presence of MRI characteristics compatible with the pituitary stalk transection syndrome should prompt a full pituitary hormonal evaluation [5].
Imaging predictors of hormonal phenotype in patients with stalk transection syndrome (MPHD versus isolated GH deficiency) have been proposed. In a study of 14 patients with hypopituitarism aged 10–25 years, a thin visible infundibulum was identified in 8 after gadolinium administration; among them, 6 had isolated GH deficiency and 2 MPHD. In the same study, 5 of the 6 children in whom the infundibulum was not identified had MPHD [2]. A third study in 25 children with hypopituitarism aged 0.9–17.4 years, 12 of 14 with a visible infundibulum had isolated GHD. In the same study, 10 of 11 children without a visible infundibulum had MPHD. This study also suggested that ectopic posterior pituitary gland location at the level of the median eminence was more likely to be associated with MPHD (11/11 patients), while its location at lower stalk levels was associated with isolated GHD (12/14 patients) [3]. The most recent study of 26 adult patients with hypopituitarism did not support an association between the degree of hypopituitarism and pituitary stalk visibility or location of the ectopic posterior gland [4]. These discordant findings have probably a two-fold explanation: different timing of hormonal evaluation across studies and insufficient statistical power due to the small numbers of patients [5].
The pituitary stalk transection (dysgenesis) syndrome should be considered in adults with hypopituitarism. Adult endocrinologists diagnose this entity in patients who were previously thought to have idiopathic GH deficiency or MPHD. The presence of MRI characteristics compatible with the pituitary stalk transection syndrome should prompt a full pituitary hormonal evaluation [5].
Differential Diagnosis List
Pituitary stalk transection syndrome
no
no
Final Diagnosis
Pituitary stalk transection syndrome
References
[1] Fujisawa I, Kikuchi K, Nishimura K, Togashi K, Itoh K, Noma S, Minami S, Sagoh T, Hiraoka T, Momoi T et al (1987) Transection of the pituitary stalk: development of an ectopic posterior lobe assessed with MR imaging. Radiology 165(2):487–489 (PMID: 3659371)
[2] Genovese E, Maghnie M, Beluffi G, Villa A, Sammarchi L, Severi F, Campani R (1997) Hypothalamic-pituitary vascularization in pituitary stalk transection syndrome: is the pituitary stalk really transected? The role of gadolinium-DTPA with spinecho T1 imaging and turbo-FLASH technique. . Pediatr Radiol 27(1):48–53 (PMID: 8995168)
[3] Chen S, Leger J, Garel C, Hassan M, Czernichow P (1999) Growth hormone deficiency with ectopic neurohypophysis: anatomical variations and relationship between the visibility of the pituitary stalk asserted by magnetic resonance imaging and anterior pituitary function. J Clin Endocrinol Metab 84(7): 2408–2413 (PMID: 10404812)
[4] Fernandez-Rodriguez E, Quinteiro C, Barreiro J, Marazuela M, Pereiro I, Peino R, Cabezas-Agricola JM, Dominguez F, Casanueva FF, Bernabeu I (2011) Pituitary stalk dysgenesis-induced hypopituitarism in adult patients: prevalence, evolution of hormone dysfunction and genetic analysis. Neuroendocrinology 93(3):181–188 (PMID: 21304225)
[5] Ioachimescu AG, Hamrahian AH, Stevens M, Zimmerman RS (2012) The pituitary stalk transection syndrome: multifaceted presentation in adulthood. Pituitary 2012 Sep; 15(3):405-11 (PMID: 21826468)
Case information
| URL: | https://www.eurorad.org/case/12367 |
| DOI: | 10.1594/EURORAD/CASE.12367 |
| ISSN: | 1563-4086 |
Figure 2
MRI of pituiatary transection syndrome
MRI of the pituitary gland coronal post contrast images showing ectopic posterior pituitary gland, small anterior pituitary gland and absent pituitary stalk.
Figure 3
MRI of pituiatary transection syndrome
MRI of the pituitary gland sagittal precontrast image showing ectopic posterior pituitary gland, small anterior pituitary gland and absent pituitary stalk.
MRI of pituiatary transection syndrome
MRI of the pituitary gland sagittal post contrast images showing ectopic posterior pituitary gland, small anterior pituitary gland and absent pituitary stalk.
Department of Radiology, University of Michigan, Ann Arbor, USA
Department of Radiology, University of Michigan, Ann Arbor, USA
MRI of pituiatary transection syndrome
MRI of the pituitary gland coronal post contrast images showing ectopic posterior pituitary gland, small anterior pituitary gland and absent pituitary stalk.
Department of Radiology, University of Michigan, Ann Arbor, USA
Department of Radiology, University of Michigan, Ann Arbor, USA
MRI of pituiatary transection syndrome
MRI of the pituitary gland sagittal precontrast image showing ectopic posterior pituitary gland, small anterior pituitary gland and absent pituitary stalk.
Department of Radiology, University of Michigan, Ann Arbor, USA
Department of Radiology, University of Michigan, Ann Arbor, USA