Pituitary stalk transection syndrome: Comparison of clinico-radiological features in adults and children with review of literature

Kulkarni, Chinmay; Moorthy, Srikanth; Pullara, Sreekumar Karumathil; Rajeshkannan, Ramiah; Unnikrishnan, Ambika Gopalakrishnan

doi:10.4103/0971-3026.107179

Cited by 24 publications

(27 citation statements)

References 18 publications

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“…A possible explanation is that pituitary insufficiency may be caused by transient anoxia of the hypothalamus at the time of delivery (38,39). Another hypothesis is that anterior lobe hypoplasia and dysfunction result in decreased hormone secretion, which in turn causes increased incidence of breech presentation (20). Dystocia including caesarean or forceps is also present in PSIS patients.…”

Section: Perinatal Eventsmentioning

confidence: 99%

Pituitary Stalk Interruption Syndrome: From Clinical Findings to Pathogenesis

Wang

Guo

Han

et al. 2017

J Neuroendocrinology

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Pituitary stalk interruption syndrome (PSIS) is a rare congenital defect manifesting with varying degrees of pituitary hormone deficiency. The signs and symptoms of PSIS during the neonatal period and infancy are often overlooked and therefore diagnosis is delayed. The typical manifestations of PSIS can be detected by magnetic resonance imaging. Several genes in the Wnt, Notch and Shh signalling pathways related to hypothalamic-pituitary development, such as PIT1, PROP1, LHX3/LHX4, PROKR2, OTX2, TGIF and HESX1, have been found to be associated with PSIS. Nevertheless, the aetiology in the majority of cases still remains unknown. In the present review, we provide an overview of clinical features of PSIS and summarise our current understanding of the underlying pathogenic mechanisms for this rare syndrome. Furthermore, we propose future research directions that may help our understanding of the aetiology of PSIS.

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Section: Perinatal Eventsmentioning

confidence: 99%

Pituitary Stalk Interruption Syndrome: From Clinical Findings to Pathogenesis

Wang

Guo

Han

et al. 2017

J Neuroendocrinology

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“…Furthermore, CISS obtained at submillimetric thickness can provide more detailed visualization of the suprasellar compartment due to good contrast between CSF and the surrounding structures. 26 While CISS imaging has been shown to be effective at predicting pituitary macroadenoma consistently, its ability to detect pituitary adenoma in CD patients has never been reported. 10,27 In our patient cohort, we found that CISS sequence had a similar, albeit slightly lower, sensitivity to CE-VIBE at detecting pituitary adenoma in CD patients (►Fig.…”

Section: Discussionmentioning

confidence: 99%

Comparison of Constructive Interference in Steady-State and T1-Weighted MRI Sequence at Detecting Pituitary Adenomas in Cushing's Disease Patients

et al. 2018

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Background T1-weighted magnetic resonance imaging (MRI) remains the standard pituitary imaging sequence. However, up to 50% of pituitary adenomas are missed with standard imaging in Cushing's disease (CD) patients. Utility of other imaging sequences needs be explored. Objective To compare a T2-gradient echo sequence, constructive interference in steady state (CISS), with a contrast-enhanced (CE) T1-weighted sequence, volume-interpolated breath-hold examination (VIBE), in the detection of pituitary adenomas in CD patients. Design Retrospective analysis of CD patients who underwent endoscopic transsphenoidal pituitary adenomectomy at our institution. Patients and Measurement Twelve patients were included in the study. Two neuroradiologists, who were blinded to the patients' clinical and surgical findings, independently reviewed the CE-VIBE and CISS images. Localization of pituitary adenoma from imaging was compared with intraoperative localization. Results The averaged sensitivity of detecting pituitary adenoma in CD patients was not significantly different between CE-VIBE sequence (63%) and CISS sequence (54%). The positive predictive value was 75% (Observer A) and 100% (Observer B) with CE-VIBE sequence, and 64% (Observer A) and 100% (Observer B) with CISS sequence. In two patients, pituitary adenoma was easily localized with CISS sequence but hard to detect with CE-VIBE sequence. In two other patients, the adenoma was much better delineated on CE-VIBE sequence. Conclusion In our series, the addition of CISS sequence to T1-weighted MRI imaging protocols improved the detection of pituitary adenomas in CD patients. CISS sequence may be a useful adjunct to T1-weighted pituitary MRI protocols and an appropriate alternative for patients with gadolinium contraindications.

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“…Была сформулирована теория механического разрыва стебля гипофиза в момент родов за счет натяжения стебля и травмы в узком месте диафрагмы турецкого седла или возникающей ишемии данной области. По мере накопления клинических данных были описаны пациенты, рожденные в головном предлежании без сопутствующей гипоксии или рожденные путем кесарева сечения [6]. В настоящий момент высокий процент ягодичного предлежания среди пациентов с гипопитуитаризмом объ- ясняют врожденным дефицитом гормонов аденогипофиза или дефицитом других факторов, приводящих к снижению двигательной активности плода [6].…”

Section: Discussionunclassified

“…По мере накопления клинических данных были описаны пациенты, рожденные в головном предлежании без сопутствующей гипоксии или рожденные путем кесарева сечения [6]. В настоящий момент высокий процент ягодичного предлежания среди пациентов с гипопитуитаризмом объ- ясняют врожденным дефицитом гормонов аденогипофиза или дефицитом других факторов, приводящих к снижению двигательной активности плода [6]. При проведении генетических исследований среди пациентов с синдромом «разрыва» воронки гипофиза мутации в генах, участвующих в эмбриональном развитии гипофиза, выявляются крайне редко.…”

Section: Discussionunclassified

Hypoglycemia as a manifestation of congenital multiple pituitary hormone deficiency in patients without growth retardation: a clinical series

Kareva

Андреевна²,

Orlova

et al. 2017

Probl Endokrinol (Mosk)

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Congenital hypopituitarism is usually diagnosed in children with growth retardation. Severe life-threatening hypoglycemia and cholestasis can be early manifestations of hypopituitarism in neonates. The pituitary stalk interruption syndrome revealed by MRI confirms the diagnosis of congenital hypopituitarism. We report six cases of children admitted with recurrent ketotic hypoglycemia since early age. The median age of the first clinical presentation of hypoglycemia was 16 months. The median age at primary endocrinological examination was 45 months. At the first examination none of the patients had growth failure. Neonatal jaundice was noticed in four patients. Free T4 levels were decreased in all the patients (median level, 8.6 pmol/l; the lower limit of normal being 10 pmol/l), while the TSH level was normal or moderately increased, suggesting secondary hypothyroidism. Cortisol levels were low (median 92 nmol/L; range, 37—130 nmol/l). IGF-1 level was below the limit of detection (<25 ng/ml) in all patients and reached the normal values in none of patients. All children had elevated prolactin levels: 540—1778 mU/l (normal level, 90—540 mU/l). MRI of the brain revealed similar abnormalities in the chiasmal sellar region in all the patients: anterior pituitary hypoplasia, thin or interrupted pituitary stalk, ectopic neurohypophysis into the chiasm and the hypothalamic structures. Ketotic hypoglycemia can be the first manifestation of congenital hypopituitarism before the growth failure. Hormonal results showing secondary hypothyroidism, secondary adrenal failure, low IGF-1 and pituitary stalk interruption syndrome detected by MRI are sufficient for making the diagnosis of congenital combined pituitary deficiency in children with hypoglycemia; GH-stimulation tests could be avoided in these cases.

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Pituitary stalk transection syndrome: Comparison of clinico-radiological features in adults and children with review of literature

Cited by 24 publications

References 18 publications

Pituitary Stalk Interruption Syndrome: From Clinical Findings to Pathogenesis

Pituitary Stalk Interruption Syndrome: From Clinical Findings to Pathogenesis

Comparison of Constructive Interference in Steady-State and T1-Weighted MRI Sequence at Detecting Pituitary Adenomas in Cushing's Disease Patients

Hypoglycemia as a manifestation of congenital multiple pituitary hormone deficiency in patients without growth retardation: a clinical series

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