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Empty sella syndrome

MedGen UID:
41766
•Concept ID:
C0014008
•
Disease or Syndrome
Synonym: Empty sella turcica
SNOMED CT: Empty sella syndrome (237722004)
 
HPO: HP:6000483
Monarch Initiative: MONDO:0006740

Definition

Empty sella or arachnoidocele has been defined as the herniation of the subarachnoid space within the sella turcica, associated with elongated pituitary stalk and flattening of the pituitary gland. [from HPO]

Professional guidelines

PubMed

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
Usefulness of magnetic resonance findings of the hypothalamic-pituitary region in the management of short children with growth hormone deficiency: evidence from a longitudinal study.
Kalina MA, Kalina-Faska B, Gruszczyńska K, Baron J, Małecka-Tendera E
Childs Nerv Syst 2012 Jan;28(1):121-7. Epub 2011 Sep 21 doi: 10.1007/s00381-011-1594-7. PMID: 21935593Free PMC Article
Spontaneous osteodural defects of the temporal bone: diagnosis and management of 12 cases.
Markou K, Goudakos J, Franco-Vidal V, Vergnolles V, Vignes JR, Darrouzet V
Am J Otolaryngol 2011 Mar-Apr;32(2):135-40. Epub 2010 Apr 13 doi: 10.1016/j.amjoto.2009.12.003. PMID: 20392531

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
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
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
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

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
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
Primary Empty Sella.
Mehla S, Chua AL, Grosberg B, Evans RW
Headache 2020 Nov;60(10):2522-2525. Epub 2020 Oct 10 doi: 10.1111/head.13987. PMID: 33038281
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
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

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
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
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
Hyperprolactinaemia and the empty sella.
Thwin M, Brophy BP
J Clin Neurosci 2012 Apr;19(4):605-6. Epub 2012 Jan 14 doi: 10.1016/j.jocn.2011.06.025. PMID: 22245276
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

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
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
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
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
The empty sella. A reappraisal of etiology and pathogenesis.
Bjerre P
Acta Neurol Scand Suppl 1990;130:1-25. PMID: 2220326

Clinical prediction guides

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
Extradural Remodeling in Empty Sella Syndrome: 3-Dimensional Operative Video.
Campero A, Baldoncini M, Martinez-Font A, Villalonga JF
World Neurosurg 2021 Mar;147:66. Epub 2021 Jan 6 doi: 10.1016/j.wneu.2020.12.065. PMID: 33359078
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 (PES): a review of 175 cases.
Guitelman M, Garcia Basavilbaso N, Vitale M, Chervin A, Katz D, Miragaya K, Herrera J, Cornalo D, Servidio M, Boero L, Manavela M, Danilowicz K, Alfieri A, Stalldecker G, Glerean M, Fainstein Day P, Ballarino C, Mallea Gil MS, Rogozinski A
Pituitary 2013 Jun;16(2):270-4. doi: 10.1007/s11102-012-0416-6. PMID: 22875743
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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