Suprasellar arachnoid cyst clinical features

Suprasellar arachnoid cysts can be diagnosed as incidental in asymptomatic patients 1).

There are three reported cases of spontaneous disappearance of a suprasellar arachnoid cyst 2).

Obstructive hydrocephalus is the most common cause of initial symptoms and occurs in almost 90% of the patients with suprasellar arachnoid cyst 3) , and can have varied presentations with signs and symptoms of obstructive hydrocephalus by compressing the third ventricle that require urgent decompression. These patients may present with nonfocal symptoms that can quickly lead to a life-threatening condition if not accurately diagnosed and treated.

Urgent endoscopic third ventriculostomy results in normalization of intracranial pressure, return of normal CSF flow, and relief of symptoms 4).

Other clinical features

Visual disturbance

Endocrine disease.

Distortion of the pituitary infundibulum can also result in endocrine dysfunction

Gait ataxia.

Rarely bobble head doll movement 5) 6) 7) 8) 9).

Ramesh et al. present three cases with bobble-head doll syndrome associated with a large suprasellar arachnoid cyst and obstructive hydrocephalus 10).

Precocious puberty

Precocious puberty is rarely the presenting sign. It is characterized by early onset, patent symptoms and frequent association with growth hormone deficiency. The latter represents a further risk of short stature. Evolution of precocious puberty varied from one case to another, without any relation with the quality of control of the arachnoid cyst and associated hydrocephalus. Half-yearly follow-up of height and bone age allowed for deciding a suppressive treatment of precocious puberty and a substitutive growth hormone therapy when needed 11).

Various forms of symptomatic and idiopathic epilepsy and other psychoneurological disorders (disorders of behavior and emotions, obsession-compulsion syndromes, stereotypias, aggression, compulsive ideas and movements, anorexia or hypothalamic obesity) coincident with one or more endocrine disorders such as precocious or delayed puberty, multihormonal pituitary deficiency, panhypopituitarism and secondary hypothyroidism were detected 12).

Presenting with signs of normal pressure hydrocephalus. 13).


1)
Monsalve G, Luque JC. Giant suprasellar arachnoid cyst. A case with severe brain stem compression: an incidental finding in a head trauma. Pediatr Neurosurg. 2007;43(2):174-6. PubMed PMID: 17337937.
2)
Moon KS, Lee JK, Kim JH, Kim SH. Spontaneous disappearance of a suprasellar arachnoid cyst: case report and review of the literature. Childs Nerv Syst. 2007 Jan;23(1):99-104. Epub 2006 Aug 30. PubMed PMID: 16944178.
3)
Al-Holou WN, Terman S, Kilburg C, Garton HJ, Muraszko KM, Maher CO. Prevalence and natural history of arachnoid cysts in adults. J Neurosurg. 2013 Feb;118(2):222-31. doi: 10.3171/2012.10.JNS12548. Epub 2012 Nov 9. PubMed PMID: 23140149.
4)
Mattox A, Choi JD, Leith-Gray L, Grant GA, Adamson DC. Guidelines for the management of obstructive hydrocephalus from suprasellar-prepontine arachnoid cysts using endoscopic third ventriculocystocisternostomy. Surg Innov. 2010 Sep;17(3):206-16. doi: 10.1177/1553350610377212. Epub 2010 Aug 5. Review. PubMed PMID: 20688773.
5)
Wiese JA, Gentry LR, Menezes AH. Bobble-head doll syndrome: review of the pathophysiology and CSF dynamics. Pediatr Neurol. 1985 Nov-Dec;1(6):361-6. Review. PubMed PMID: 3916908.
6)
Fioravanti A, Godano U, Consales A, Mascari C, Calbucci F. Bobble-head doll syndrome due to a suprasellar arachnoid cyst: endoscopic treatment in two cases. Childs Nerv Syst. 2004 Oct;20(10):770-3. PubMed PMID: 15057560.
7)
Van Beijnum J, Hanlo PW, Han KS, Ludo Van der Pol W, Verdaasdonk RM, Van Nieuwenhuizen O. Navigated laser-assisted endoscopic fenestration of a suprasellar arachnoid cyst in a 2-year-old child with bobble-head doll syndrome. Case report. J Neurosurg. 2006 May;104(5 Suppl):348-51. PubMed PMID: 16848093.
8)
Alexiou GA, Sfakianos G, Prodromou N. Giant suprasellar arachnoid cyst with head bobbing. Mov Disord. 2013 Aug;28(9):1216. doi: 10.1002/mds.25511. Epub 2013 Aug 2. PubMed PMID: 23913743.
9)
Hagebeuk EE, Kloet A, Grotenhuis JA, Peeters EA. Bobble-head doll syndrome successfully treated with an endoscopic ventriculocystocisternostomy. Case report and review of the literature. J Neurosurg. 2005 Sep;103(3 Suppl):253-9. PubMed PMID: 16238079.
10)
Ramesh S, Raju S. Suprasellar arachnoid cyst presenting with bobble-head doll syndrome: Report of three cases. J Pediatr Neurosci. 2015 Jan-Mar;10(1):18-21.doi: 10.4103/1817-1745.154321. PubMed PMID: 25878736; PubMed Central PMCID:PMC4395937.
11)
Brauner R, Pierre-Kahn A, Nemedy-Sandor E, Rappaport R, Hirsch JF. [Precocious puberty caused by a suprasellar arachnoid cyst. Analysis of 6 cases]. Arch Fr Pediatr. 1987 Aug-Sep;44(7):489-93. French. PubMed PMID: 3426371.
12)
Starzyk J, Kwiatkowski S, Kaciński M, Kroczka S, Wójcik M. [Structural CNS abnormalities responsible for coincidental occurrence of endocrine disorders, epilepsy and psychoneurologic disorders in children and adolescents]. Przegl Lek. 2010;67(11):1120-6. Polish. PubMed PMID: 21442961.
13)
De Bonis P, Novegno F, Tamburrini G, Anile C, Pompucci A, Mangiola A. Teaching NeuroImage: Prepontine-suprasellar arachnoid cyst presenting with signs of normal pressure hydrocephalus. Neurology. 2008 Feb 5;70(6):e26. doi: 10.1212/01.wnl.0000296823.01603.44. PubMed PMID: 18250284.
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