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CASE REPORT
Year : 2006  |  Volume : 1  |  Issue : 1  |  Page : 24-26
 

Suprasellar arachnoid cyst with bobble-head doll movements: A management option


Post Graduate Institute of Neurological surgery, Dr. Achanta Lakshmipathi Neurosurgical Centre, VHS Hospital, Chennai, India

Correspondence Address:
Vikram Muthusubramanian
Post Graduate Institute of Neurological Surgery, Dr. Achanta Lakshmipathi Neurosurgical Centre, VHS Hospital, Taramani, Chennai - 600 113
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/1817-1745.22944

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  Abstract 

The authors describe their experience with endoscopic ventriculo-cysto-cisternostomy and placement of cisterno-cysto-venticulo peritoneal shunt for suprasellar arachnoid cyst for a child with bobble head doll movements. The abnormal head movements improved and follow up imaging showed reduction in the cyst and ventricular size. The benefits of minimally invasive endoscopic ventriculocystocisternostomy with endoscopic guided placement of cisterno-cysto-ventriculo peritoneal shunt is been discussed.


Keywords: Suprasellar arachnoid cyst, bobble head doll movement, cisterno - cysto - venticulo - peritoneal shunt, endoscopic ventriculocystocisternostomy


How to cite this article:
Muthusubramanian V, Pande A, Vasudevan MC, Ravi R. Suprasellar arachnoid cyst with bobble-head doll movements: A management option. J Pediatr Neurosci 2006;1:24-6

How to cite this URL:
Muthusubramanian V, Pande A, Vasudevan MC, Ravi R. Suprasellar arachnoid cyst with bobble-head doll movements: A management option. J Pediatr Neurosci [serial online] 2006 [cited 2017 Jul 21];1:24-6. Available from: http://www.pediatricneurosciences.com/text.asp?2006/1/1/24/22944



  Introduction Top


Arachnoid cysts are intra arachnoid collections of cerebrospinal fluid and they are regarded as a developmental abnormality of the arachnoid, originating from a splitting or duplication of this membrane.[1],[2],[3] The surgical treatment is still controversial. The goal of surgical treatment is to normalize the cerebrospinal fluid flow and to establish a communication, permanently, between the cyst cavity and the intraventricular or/and subarachnoid space.[4],[5] Recently, a few authors have reported the successful management of arachnoid cysts using endoscopy.[6],[8],[15] An analysis of the 45[12],[13] reported cases of suprasellar arachnoid cyst suggested that direct removal of the cyst wall is better than a V-P shunt and cyst shunting is advisable for recurrence of the cyst. Endoscopic ventriculocystocisternostomy with endoscopic guided placement of a thin cisterno - cysto - ventriculo peritoneal shunt is being described as an option to attain the surgical goal.


  Case Report Top


A five year old boy presented with the complaints of intermittent head nodding of 2 yrs duration associated with tremors of the hands since 1 yrs. The symptoms were insidious in onset and gradually progressive with no other associated symptoms. The prenatal, natal and post natal periods were normal. Developmental milestones were normal. There was no neurological deficit. Head nodding was at the rate of 5 per minute at rest and 10 per minute with activity. CT and MRI scan of the brain revealed a suprasellar arachnoid cyst with obstructive hydrocephalus [Figure - 1].

Initially, stereotactic placement of the ventricular end of a Chabra venticulo peritoneal shunt tube into the cyst was attempted, but failed. The nodding movements persisted and post operative imaging showed that the ventricular end was in the ventricle and not in the cyst [Figure - 2] . Endoscopic ventriculocystocisternostomy was decided upon. Through a right prefrontal burrhole, the endoscope was introduced and a blue coloured thick walled cyst was seen. The foramen of Monro was found to be obstructed by the cyst. The wall of the cyst was cauterized using monopolar cautery and opened with scissors. CSF escaped through the opening into the ventricle. The cyst wall was thick and was found to have two layers. The opening was widened using scissors in a cruciate manner and the edges were cauterized using monopolar cautery. The foramen of Munro was free and on advancing the endoscope into the arachnoid cyst the basilar artery was visualized. The thin thecal end of a thecoperitoneal shunt was introduced into the cyst cavity through an instrument portal of the endoscope under vision. The distal end was connected to the reservoir of the previously positioned VP shunt tube after confirming the free flow of CSF. The thin tube was used as it could be introduced under vision through the instrument port of the endoscope instead of the thicker ventricular end of a ventriculoperitoneal shunt tube as this has to be done blindly through the endoscope port.

The nodding movements at rest came down drastically in the immediate post operative period with decreased frequency of movements during activity. On follow up, the nodding movements were decreasing progressively. Follow up imaging studies showed a significant decrease in the ventricular size and the size of the cyst [Figure - 3]. Histopathology confirmed an arachnoid cyst.


  Discussion Top


Approximately one per cent of all intracranial lesions are arachnoid cysts and nine per cent of these are suprasellar. The clinical manifestations of these cysts are obstructive hydrocephalus, visual impairment, endocrinal dysfunction, hypothalamic disturbances and gait ataxia. Bobble-head doll movements are rare, but have been reported by many following the first description by Benton, in 1966.[7] The bobble-head doll syndrome (BHDS) is characterized by a back-and-forth movement of the head with a frequency of 2 to 3 Hz, which increases during walking and excitement and decreases during concentration.[7]

The syndrome of bobble-head-doll movements is noticed more often in lesions, which cause third ventricle dilatation like suprasellar arachnoid cyst, colloid cyst, aqeductal stenosis, craniopharyngioma and rarely, choroids plexus papilloma of the third ventricle. Compression of the dorsomedial nucleus of the thalamus by the cyst, which stimulates the diencephalic extra-pyramidal pathways and results in bobbling movements of the head is the possible pathophysiology.[10],[11],[14]

Wiese and his colleagues[9] supported their theory of head bobbling as a "learned" behavior to ameliorate the headaches by serial metrizamide CT cisternography studies and demonstrated a decrease in the size of the suprasellar arachnoid cyst by bobbling movements. The establishment of a single CSF space, by surgically communicating the cyst with the ventricular system or the basal cisterns, appears to offer the best chance of success in the treatment of suprasellar arachnoid cysts. Sub-frontal fenestration of the cyst into the basal cisterns, a transcallosal fenestration of the cyst, cystoperitoneal shunt, percutaneous ventriculostomy and endoscopic marsupialization are various surgical options available to attain the goal of establishing a permanent communication. There is a significant incidence of recurrence irrespective of the initial procedure.[13],[15] In our case, to reduce the recurrence rate, an attempt was made to establish a permanent pathway between the cyst, basal cistern and the ventricular cavity by ventriculocystocisternostomy with placement of the shunt tube into the basal cistern through the cyst using the instruments port in the endoscope as a cisterno-cysto-ventriculo peritoneal shunt. The chances of spontaneous closure of the ventriculocystocisternostomy can also be reduced with maintenance of the CSF pathway between the cisterns, cysts and ventricle by this procedure.

To conclude the association of bobble-head-doll movements with suprasellar arachnoid cysts is extremely rare. Though open surgical drainage of the suprasellar cyst offers immediate regression of symptoms, maintenance of the communication by placement of a cisterno - cysto - ventriculo- peritoneal shunt under endoscopic guidance is an excellent alternative surgical method.

 
  References Top

1.Krawchenko J, Collins GH. Pathology of an arachnoid cyst. Case report. J Neurosurg 1979;50:224-8.   Back to cited text no. 1  [PUBMED]  
2.Robinson RG. Congenital cysts of the brain malformation. Prog Neurol Surg 1971;4:133-74.  Back to cited text no. 2    
3.Harsh NG, Edwards MS, Wilson CB. Intracranial arachnoid cysts in children. J Neurosurg 1986;64:835-42.  Back to cited text no. 3    
4.Pierre-Kahn A, Capelle L, Brauner R, Sainte-Rose C, Renier D, Rappaport R, et al. Presentation and management of suprasellar arachnoid cysts. Review of 20 cases. J Neurosurg 1990;73:355-9.  Back to cited text no. 4  [PUBMED]  
5.Hoffman HJ, Hendrick EB, Humphrey RP, Armstrong EA. Investigation and management of suprasellar arachnoid cysts. J Neurosurg 1982;57:597-602.  Back to cited text no. 5    
6.Gupta SK, Gupta VK, Khosla VK, Dash RJ, Bhansali A, Kak VK, et al. Suprasellar arachnoid cyst presenting with precocious puberty. Report of two cases. Neurol India 1999;47:148-51.  Back to cited text no. 6    
7.Benton JW, Nellhaus G, Huttenlocher PR. The bobble-head doll syndrome. Report of a unique truncal tremor associated with third ventricular cyst and hydrocephalus in children. Neurology 1966;16:725-9.  Back to cited text no. 7    
8.Desai KI, Nadkarni TD, Muzumdar D, Goel A Suprasellar arachnoid cyst presenting with bobble-head doll movements: a report of 3 cases. Neurol India 2003;51:407-9.  Back to cited text no. 8    
9.Wiese JA, Gentry LR, Menezes AH. Bobble-head doll syndrome. Review of the pathophysiology and CSF dynamics. Pediatr Neurol 1985;1:361-6.   Back to cited text no. 9  [PUBMED]  
10.Russo RH, Kindt GW. A neuroanatomical basis for the bobble-head doll syndrome. J Neurosurg 1974;41:720-3.  Back to cited text no. 10  [PUBMED]  
11.Parkinson D. Bobble-head doll syndrome. J Neurosurg 1996;84:538.  Back to cited text no. 11    
12.Takahashi T, Kawai S, Kaminoh T, Hiramatsu K, Maekawa M, Yuasa T, et al. Suprasellar arachnoid cyst-report of a case No Shinkei Geka 1982;10:435-41.  Back to cited text no. 12  [PUBMED]  
13.Rappaport ZH. Suprasellar arachnoid cysts: options in operative management, Acta Neurochir (Wien) 1993;122:71-5  Back to cited text no. 13  [PUBMED]  
14.Goikhman I, Zelnik N, Michowiz S. Bobble-head syndrome: a surgically treatable condition manifested movement disorder. Mov Disord 1998;13:192-4.  Back to cited text no. 14  [PUBMED]  
15.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;20:770-3.  Back to cited text no. 15  [PUBMED]  [FULLTEXT]


    Figures

[Figure - 1], [Figure - 2], [Figure - 3]


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   Abstract
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