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Pituitary dwarfism with small sella turcica

MedGen UID:
78777
•Concept ID:
C0271574
•
Disease or Syndrome
Synonyms: Empty Sella Syndrome, Primary; Primary Empty Sella Syndrome
SNOMED CT: Pituitary dwarfism with small sella turcica (91187007); Primary empty sella syndrome (91187007); Ferrier-Stone syndrome (91187007); Abnormal sella turcica syndrome (91187007); Familial panhypopituitarism with abnormal sella turcica (91187007); Familial absence of sella turcica (91187007)

Professional guidelines

PubMed

Neuroimaging in the diagnosis and treatment of intracranial pressure disorders.
Moreno ME, Del Carpio-O'Donovan R
Neurol Sci 2023 Mar;44(3):845-858. Epub 2022 Nov 5 doi: 10.1007/s10072-022-06478-x. PMID: 36333629
Etiology and management of amenorrhea in adolescent and young adult women.
Kerns J, Itriyeva K, Fisher M
Curr Probl Pediatr Adolesc Health Care 2022 May;52(5):101184. Epub 2022 May 4 doi: 10.1016/j.cppeds.2022.101184. PMID: 35525789
Idiopathic Intracranial Hypertension: Review of Clinical Syndrome, Imaging Findings, and Treatment.
Rehder D
Curr Probl Diagn Radiol 2020 May-Jun;49(3):205-214. Epub 2019 Mar 2 doi: 10.1067/j.cpradiol.2019.02.012. PMID: 31056359

Recent clinical studies

Etiology

A Comprehensive Review of Empty Sella and Empty Sella Syndrome.
Lundholm MD, Yogi-Morren D
Endocr Pract 2024 May;30(5):497-502. Epub 2024 Mar 12 doi: 10.1016/j.eprac.2024.03.004. PMID: 38484938
Etiology and management of amenorrhea in adolescent and young adult women.
Kerns J, Itriyeva K, Fisher M
Curr Probl Pediatr Adolesc Health Care 2022 May;52(5):101184. Epub 2022 May 4 doi: 10.1016/j.cppeds.2022.101184. PMID: 35525789
Insights into non-classic and emerging causes of hypopituitarism.
Prodam F, Caputo M, Mele C, Marzullo P, Aimaretti G
Nat Rev Endocrinol 2021 Feb;17(2):114-129. Epub 2020 Nov 27 doi: 10.1038/s41574-020-00437-2. PMID: 33247226
Primary empty sella.
De Marinis L, Bonadonna S, Bianchi A, Maira G, Giustina A
J Clin Endocrinol Metab 2005 Sep;90(9):5471-7. Epub 2005 Jun 21 doi: 10.1210/jc.2005-0288. PMID: 15972577
Pseudotumor cerebri.
Moffat FL
Can J Neurol Sci 1978 Nov;5(4):431-6. doi: 10.1017/s0317167100024227. PMID: 369670

Diagnosis

Empty sella syndrome: Multiple endocrine disorders.
Chiloiro S, Giampietro A, Bianchi A, De Marinis L
Handb Clin Neurol 2021;181:29-40. doi: 10.1016/B978-0-12-820683-6.00003-8. PMID: 34238465
MRI findings as markers of idiopathic intracranial hypertension.
Barkatullah AF, Leishangthem L, Moss HE
Curr Opin Neurol 2021 Feb 1;34(1):75-83. doi: 10.1097/WCO.0000000000000885. PMID: 33230036Free PMC Article
Neuroendocrine disorders: pituitary imaging.
Faje A, Tritos NA, Swearingen B, Klibanski A
Handb Clin Neurol 2016;136:873-85. doi: 10.1016/B978-0-444-53486-6.00044-2. PMID: 27430447
Septo-optic dysplasia.
Ferran Kd, Paiva IA, Gilban DL, Resende M, Souza MA, Beserra IC, Guimarães MM
Arq Neuropsiquiatr 2010 Jun;68(3):400-5. doi: 10.1590/s0004-282x2010000300014. PMID: 20602044
Pseudotumor cerebri.
Moffat FL
Can J Neurol Sci 1978 Nov;5(4):431-6. doi: 10.1017/s0317167100024227. PMID: 369670

Therapy

Insights into non-classic and emerging causes of hypopituitarism.
Prodam F, Caputo M, Mele C, Marzullo P, Aimaretti G
Nat Rev Endocrinol 2021 Feb;17(2):114-129. Epub 2020 Nov 27 doi: 10.1038/s41574-020-00437-2. PMID: 33247226
Macroprolactinemia and Empty Sella Syndrome.
Taieb A, Maha KN, El Abed YH, Beizig AM, Chadli MC, Ach K
Pan Afr Med J 2017;27:278. Epub 2017 Aug 14 doi: 10.11604/pamj.2017.27.278.11361. PMID: 29187947Free PMC Article
Growth hormone secreting pituitary microadenomas and empty sella - An under-recognized association?
Liu W, Zhou H, Neidert MC, Schmid C, Bernays RL, Ni M, Zhou D, Jia W, Jia G
Clin Neurol Neurosurg 2014 Nov;126:18-23. Epub 2014 Aug 17 doi: 10.1016/j.clineuro.2014.08.012. PMID: 25194306
Spontaneous CSF Leaks.
Wang EW, Vandergrift WA 3rd, Schlosser RJ
Otolaryngol Clin North Am 2011 Aug;44(4):845-56, vii. doi: 10.1016/j.otc.2011.06.018. PMID: 21819875
Pseudotumor cerebri.
Moffat FL
Can J Neurol Sci 1978 Nov;5(4):431-6. doi: 10.1017/s0317167100024227. PMID: 369670

Prognosis

Clinical course of primary empty sella in children: a singlecenter experience.
Besci Ö, Yaşar E, Erbaş İM, Acınıklı KY, Demir K, Böber E, Abacı A
Turk J Pediatr 2022;64(5):900-908. doi: 10.24953/turkjped.2021.4953. PMID: 36305440
Chiasmapexy for secondary empty sella syndrome: diagnostic and therapeutic considerations.
Graillon T, Passeri T, Boucekine M, Meyer M, Abritti R, Bernat AL, Labidi M, Dufour H, Froelich S
Pituitary 2021 Apr;24(2):292-301. Epub 2020 Nov 2 doi: 10.1007/s11102-020-01104-5. PMID: 33136230
Neuroendocrine disorders: pituitary imaging.
Faje A, Tritos NA, Swearingen B, Klibanski A
Handb Clin Neurol 2016;136:873-85. doi: 10.1016/B978-0-444-53486-6.00044-2. PMID: 27430447
Primary empty sella.
De Marinis L, Bonadonna S, Bianchi A, Maira G, Giustina A
J Clin Endocrinol Metab 2005 Sep;90(9):5471-7. Epub 2005 Jun 21 doi: 10.1210/jc.2005-0288. PMID: 15972577
Pseudotumor cerebri.
Moffat FL
Can J Neurol Sci 1978 Nov;5(4):431-6. doi: 10.1017/s0317167100024227. PMID: 369670

Clinical prediction guides

Different Cases of Short Stature.
Sathyanarayanan, Ramkumar, Dinesh S
J Assoc Physicians India 2022 Apr;70(4):11-12. PMID: 35443324
Primary empty sella: The risk factors and associations with the cerebral small vessel diseases-An observational study.
Chen T, Li G, Wu D, Xie B, Feng Y, Xiao S, Li J, Liu Y, Yang J, Li X
Clin Neurol Neurosurg 2021 Apr;203:106586. Epub 2021 Mar 5 doi: 10.1016/j.clineuro.2021.106586. PMID: 33730618
Macroprolactinemia and Empty Sella Syndrome.
Taieb A, Maha KN, El Abed YH, Beizig AM, Chadli MC, Ach K
Pan Afr Med J 2017;27:278. Epub 2017 Aug 14 doi: 10.11604/pamj.2017.27.278.11361. PMID: 29187947Free PMC Article
Primary empty sella and GH deficiency: prevalence and clinical implications.
Poggi M, Monti S, Lauri C, Pascucci C, Bisogni V, Toscano V
Ann Ist Super Sanita 2012;48(1):91-6. doi: 10.4415/ANN_12_01_15. PMID: 22456022
Primary empty sella.
De Marinis L, Bonadonna S, Bianchi A, Maira G, Giustina A
J Clin Endocrinol Metab 2005 Sep;90(9):5471-7. Epub 2005 Jun 21 doi: 10.1210/jc.2005-0288. PMID: 15972577

Recent systematic reviews

Trigeminal Neuralgia Secondary to Meckel's Cave Meningoencephaloceles: A Systematic Review and Illustrative Case.
Martínez JL, Domingo RA, Rowland NC, Vandergrift Iii WA
Neurol India 2022 May-Jun;70(3):857-863. doi: 10.4103/0028-3886.349629. PMID: 35864609
Ectopic GH-secreting pituitary adenoma of the clivus: systematic literature review of a challenging tumour.
Riccio L, Donofrio CA, Tomacelli G, De Blasi R, Melatini A
Pituitary 2020 Aug;23(4):457-466. doi: 10.1007/s11102-020-01057-9. PMID: 32504302
Primary Empty Sella Syndrome and the Prevalence of Hormonal Dysregulation.
Auer MK, Stieg MR, Crispin A, Sievers C, Stalla GK, Kopczak A
Dtsch Arztebl Int 2018 Feb 16;115(7):99-105. doi: 10.3238/arztebl.2018.0099. PMID: 29510819Free PMC Article
Treatment of empty sella associated with visual impairment: a systematic review of chiasmapexy techniques.
Barzaghi LR, Donofrio CA, Panni P, Losa M, Mortini P
Pituitary 2018 Feb;21(1):98-106. doi: 10.1007/s11102-017-0842-6. PMID: 29027644

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