CASE-BASED UPDATE

Cavum septum pellucidum cyst in children:a case-based update

Alin Borha & Keven F. Ponte & Evelyne Emery

Received: 6 February 2012 /Accepted: 5 April 2012 /Published online: 29 April 2012# Springer-Verlag 2012

AbstractBackground Cavum septum pellucidum (CSP) cysts arerare lesions which are frequently asymptomatic. Someclinical findings may be associated with CSP cysts, suchas headache and other symptoms of increased intracranialpressure, neurological deficit, and mental status changes.There is still controversy in the management of symp-tomatic cases, especially in children. The main difficultyis to establish a correlation between symptoms and thecyst. When indicated, the treatment is essentially surgi-cal, and the ideal operative technique is also a matter ofdebate.Case report We present a case of a 14-year-old boy witha symptomatic CSP cyst who was successfully treatedby neuronavigation-assisted neuroendoscopy with a bi-lateral fenestration. A literature review is provided withregard to clinical presentation, treatment, and outcome inchildren.Conclusion The treatment is considered whenever there isan association of a CSP cyst on imaging studies and symp-toms attributable to the obstruction of the cerebrospinal fluidflow or direct compression of surrounding structures by thecyst. Endoscopic fenestration is a less invasive and highlyeffective technique, and is currently the treatment of choicefor such lesions in children.

Keywords Cavum septum pellucidum . Cyst .

Neuroendoscopy . Fenestration . Pediatric

Introduction

Cavum septum pellucidum (CSP) is the persistence of a cavitybetween the leaves of the septum pellucidum and is seen in upto 82 % of full-term neonates [25]. However, cysts of CSP aremore rare with an incidence of 0.04 % found by Wang et al.[29] in radiological studies. Moreover, symptomatic CSPcysts are very rare lesions, and only some cases have beendescribed in the literature [1–10, 12, 14, 16–21, 27, 30–32].These cases are classically treated by surgical approaches,including open surgical procedures, conventional shunting,and stereotactic fenestration. Since 1995 when first endoscop-ical approach was described by Jacowski et al. [13], a vastmajority of authors have recommended neuroendoscopy asthe procedure of choice in treating such lesions [3, 7, 9, 10,18–20, 27, 32]. However, such a technique is the purpose oftechnical refinement and controversies. We present a case of a14-year-old boy with a symptomatic CSP cyst successfullytreated by neuronavigation-guided neuroendoscopic fenestra-tion and provide a literature review considering the clinicalpresentation, management, and outcome of such lesions inpediatric population.

Case report

A 14-year-old boy without previous medical history presentedtwo episodes of severe acute headaches and vomiting beforeadmission. There was also a brief loss of consciousness duringthe second episode. It resolved spontaneously after severalhours and bed rest. In addition, he complained of decreasedconcentration and attention, with progressive decline in hisschool performance. Clinical examination revealed no focaldeficit, and there was no abnormality in fundi examination.Computed tomography (CT) scan and magnetic resonance

A. Borha (*) :K. F. Ponte : E. EmeryDepartment of Neurosurgery, University Hospital of Caen,University of Lower Normandy,Avenue Cote de Nacre,Caen, Francee-mail: alin_borha@hotmail.com

Childs Nerv Syst (2012) 28:813–819DOI 10.1007/s00381-012-1760-6

imaging (MRI) revealed an enlarged cyst of cavum septumpellucidum (Fig. 1). Considering that symptoms were severeand related to the CSP cyst, it was decided to treat the cystwith an endoscopic fenestration into the lateral ventricles.

Endoscopic surgery was performed under general anaes-thesia and neuronavigation guidance. The patient wasplaced in supine position, with the head fixed in a Mayfieldhead holder and the neck flexed 15°. Under the guidance ofa neuronavigator system (Vectorvision Classic; Brain Lab,Heimstetten, Germany), we designated the best trajectory tothe right lateral ventricle in close relation with the cyst. Theanterior horn of the right lateral ventricle was initially cho-sen as a target. A small straight cranial incision and a 25-mmcraniotomy were made over the right coronal suture and5 cm lateral to the midline. This slightly larger craniotomyallows a free inclination of the endoscope to reach thecontralateral ventricle. After opening of the dura, a bluntintroducer with sheath was passed through the brain, andcerebrospinal fluid was encountered. The blunt introducerwas removed, and a 30° angle rigid neuroendoscope (KarlStorz GmbH & Co, Tuttlingen, Germany) was insertedunder navigation control. Inside the small anterior horn ofthe right lateral ventricle, the right lateral wall of the cystunder tension was easily observed. The normal ventricularanatomy was identified. The bowing wall of the cyst par-tially obstructed the opening of the foramen of Monro. Afenestration was performed in the right wall using the for-ceps under direct endoscopic visualization. The cystic wallwas perforated by advancing the penetrator of the workingtip carefully against the cystic pressure. With further ad-vance of the working tip after cyst perforation, the graspingbasket controlled in the distal end was opened slowly todilate the penetrated hole. We realized two holes in anavascular region. The endoscope was then passed into thecavum itself. Once the relationship between the left ventricleand the left lateral wall of the cyst was determined, a second

fenestration was realized in the left cyst wall. When theworking tip was withdrawn, the patency of dilated holewas checked and confirmed. The cyst shrank after its con-tents were expelled. The right foramina of Monro could beinspected and was found out to be unblocked. There was noadhesion. The patient remained stable after surgery. Postop-erative CT scan and MRI confirmed a reduction of the CSPcyst, which resembled a simple cavum, and normal ventric-ular size (Fig. 2). The patient presented no recurrence ofsymptoms at 18 months follow-up.

Discussion and literature review

Definition and epidemiology

Embryologically, the septum pellucidum is formed by twoclosely opposed leaves that enclose a cavity—the cavumseptum pellucidum (CSP). At approximately 6 months ofintrauterine life, the leaves of the septum begin to fuse in acaudal-to-rostral direction, and the cavity usually disappearswithin 3 months postnatal [17, 22, 28]. However, the CSPmay persist and can be seen even in adults.

The CSP often communicates with a posterior cavitynamed cavum vergae (CV). They are essentially the samestructure, separated by an arbitrary vertical plane formed bythe columns of the fornix [17, 22, 25, 28]. Historically, theCSP and the CV were considered fifth and sixth ventricles,respectively [25]. This argument was refuted since they donot contain choroid plexus [28].

The presence of a CSP represents a normal anatomicalvariant. Its age-dependent prevalence attests its normal de-velopment with ultimate regression. CSP is estimated tooccur in all premature infants, in 85 % of full-term neonates,and in 12 % of children between 6 months and 16 years old[25]. In adults, the prevalence varies significantly depending

Fig. 1 Axial (a) and coronal (b) T2-weighted magnetic resonance imagesshowing a cyst of the cavum septum pellucidum under tension (withbowling of the walls). The ventricular volume is normal. c Intraoperative

screenshot of neuronavigation guidance before contralateral fenestration(right–left reversed). The lateral cyst walls are immediately adjacent to thecaudate nucleus

814 Childs Nerv Syst (2012) 28:813–819

on detection methods and anatomical definition, and rangesfrom 4 to 74 % [25, 26].

The CSP cavity may also expand forming a CSP cyst,probably by an ability to secrete fluid internally [17]. Thewell-accepted definition of a CSP cyst is a cystic structurehaving a width of 10 mm or more in the region of theseptum pellucidum, the walls of which exhibiting lateralbowing [23]. Some authors have defined this condition asexpanding septum pellucidum cyst or true septum pelluci-dum cyst [17]. The incidence of CSP cysts is very low in theliterature. In a database of 54,000 patients who underwent aCT or MRI, Wang et al. found 22 cases (0.04 %) with adilated CSP cyst [29].

CSP cyst is classically classified as communicating andnoncommunicating cyst, depending on whether it communi-cates with the ventricles or not [17, 25]. Noncommunicatingcyst may become communicating by spontaneous ruptureor during head trauma, diagnostic procedures (ventricu-lography and pneumoencephalography), or surgery [17].The reverse is also possible if fibrosis occurs at the site ofcommunication.

Clinical presentation and pathophysiology

Usually CSP cysts are asymptomatic and are incidentallydiscovered after radiological imaging for other reasons suchas trauma. Symptomatic patients are very rare and usuallypresent with symptoms of increased intracranial pressure,such as acute or chronic headaches, papilledema, emesis,and syncope. They can also be associated with cognitiveimpairment, emotional and behavioral disturbances, andvisual and sensorimotor findings.

There are very few articles in the literature addressingthis pathology [1–10, 12, 14, 16–21, 27, 30–32]. They areusually case reports or small series generally includingadults and children. To our knowledge, this is the first articlethat reviews the subject only in pediatric patients and in theera of endoscopic approaches.

Including the current case, we found only 31 cases ofchildren with symptomatic CSP cysts under the age of18 years in English literature with radiological confirmation(Table 1). The mean age was 7.3 years (2 months–14 years),and the vast majority was males (83.9 %). For this review,cases were not considered without data on clinical andradiological findings, follow-up, and outcome.

The most frequent symptom was headache in 19 children(61.3 %). The headaches are often intermittent, but can alsobe abrupt. In 57.9 % of cases, they were associated withnausea (two), vomiting (four), papilledema (two), optic pal-lor (one), or syncope (two). Headache was not associatedwith any objective clinical sign in eight cases (42.1 %), andmay be associated with nausea only, dizziness, or behavioralchanges. It was the only symptom in three cases. In only twocases was it present in children under 5 years, and in bothcases, it was associated with vomiting.

In total, focal neurological deficit was present in sevenchildren (22.6 %), who presented with paraparesis (two),hemiparesis (two), and other pyramidal signs (three). Seiz-ures were described in three patients (9.7 %). Visual impair-ment was detected in two patients, one with bitemporalhemianopsia. Mental status changes were present in 13patients (41.9 %) and were associated with developmentaldelay in five.

Hydrocephalus was present in ten children (32.3 %). In70 % of cases, it occurred under the age of 5 years. In fivecases, hydrocephalus was associated with clinical signs ofincreased intracranial pression (ICP), and in three cases, itwas associated with no other clinical finding. Macrocraniawas present in two children with a giant CSP cyst [3, 7], andin another with hydrocephalus [31].

Three physiopathological mechanisms of CSP cystssymptoms have been described [17]:

1. An increased intracranial pressure—considered to re-sult from the obstruction of interventricular foramina bythe cyst (ball valve phenomenon);

Fig. 2 Axial fluid-attenuatedinversion recovery (a) andcoronal T2-weighted (b) mag-netic resonance images obtained18 months after endoscopicfenestration, demonstrating aremarkable reduction of the cyst.The arrow shows one of thefenestrations on the cyst wall

Childs Nerv Syst (2012) 28:813–819 815

Table 1 Summary of the cases in the literature of symptomatic CSP cysts in children confirmed by imaging studies

Author, year Age, sex Clinical features Treatment Follow-up(months)

Complications Results

Dandy 1931 [6] 4.5 years old, M Vomiting, seizures, hyperreflexia,hyperthermia, mental statuschanges, dev. delay

Transcallosalfenestration

8 None Asymptomatic

Echternacht andCampbell 1946 [8]

2 months, M Seizures, mentalstatus changes

None 3 Asymptomatic

Heiskanen 1973 [12] 22 months, F Papilledema, paraparesis,mental status changes,dev. delay, hydrocephalus

Transcorticalfenestration

12 None Asymptomatic

Kansu and Bertan1980 [14]

8 years old, M Headache, diminishedvisual acuity, bitemporalhemianopsia, optic atrophy

Transcorticalfenestration

2 None Improved

Amin 1986 [1] 14 years old, M Headache, papilledema Transcorticalfenestration

17 None Asymptomatic

Aoki 1986 [2] 11 years old, M Hemiparesis, dev. delay Transcallosalfenestration

12 None Improved

Wester et al. 1990 [30] 8 years old, M Impaired vision, ataxia,hydrocephalus

Stereotactic CVS 3 None Asymptomatic

Krauss et al. 1991 [16] 2.9 years old, F Dev. delay, mental statuschanges, gait ataxia,paraparesis, hydrocephalus

Stereotactic CVS 12 None Improved

Behrens and Ostertag1993 [4]

11 years old, F Headache Stereotactic CVS 12 None Asymptomatic

11 years old, F Headache Stereotactic CVS 12 None Asymptomatic

Wester et al. 1995 [32] 2 years old, M Hydrocephalus, macrocrania Stereotactic CVS 72 None Not improved

4 years old, M Headache, vomiting,hydrocephalus

Stereotactic CVS,then CPS

12 None Not improved(asymptomaticafter CPS)

6 months, M Hydrocephalus VPS 23 None Asymptomatic

Miyamori et al. 1995 [21] 6 years old, M Beh. dist., “unstable gait,”dev. delay

Stereotactic CPS ? None Improved

Bayar et al. 1996 [3] 18 months, M Mental status changes, dev. delay,hyperreflexia, unconsciousness,impaired respiratory function,hydrocephalus, macrocrania

CPS – None Not improved (diedof respiratory failureon the 3rd postoperativeday)

Lancon et al. 1996 [17] 8 years old, M Headache, syncope, beh. dist. CPS 12 None Asymptomatic

Lancon et al. 1999 [18] 6 years old, M Headache, syncope;neuropsychological deficits

Endoscopy 18 None Asymptomatic

Gangemi et al. 1999 [9] 13 years old, M Headache, seizures Endoscopy ? None Improved

Gangemi et al. 2002 [10] 8 years old, M Headache, restlessness, mentalstatus changes

Endoscopy 15 None Asymptomatic

7 years old, M Headache, vomiting, hemiparesis,mental status changes,hydrocephalus

Endoscopy 6 None Asymptomatic

Donati et al. 2003 [7] 17 months, M Macrocrania, papilledema,hyperreflexia, dev. delay

VPS, then Endoscopy 36 None Not improved(asymptomatic afterEndoscopy)

Chiu et al. 2005 [5] 14 years old, M Headache, dizziness Endoscopy 54 None Asymptomatic

Miki et al. 2005 [20] 9 years old, F Headache, nausea Endoscopy 64 None Asymptomatic

14 years old, M Headache, nausea Endoscopy 33 None Asymptomatic

Weyerbrock et al.2006 [32]

11 years old, M Headache, papilledema,hydrocephalus

Endoscopy 18 None Asymptomatic

Meng et al. 2006 [19] 13 years old, M Headache, dizziness, beh. dist. Endoscopy 60 Bleeding Asymptomatic

3 years old, M Headache, vomiting Endoscopy 18 None Asymptomatic

8 years old, M Headache, beh. dist. Endoscopy 12 None Asymptomatic

3 years old, M Hydrocephalus Endoscopy 12 None Asymptomatic

Tamburrini et al.2007 [27]

8 years old, M Headache Endoscopy 36 None Asymptomatic

Present case 14 years old, M Headache, vomiting, syncope,decreased concentration andattention

Endoscopy 18 None Asymptomatic

Dev. delay developmental delay, beh. dist. behavioral disturbances, CVS cystoventricular shunt, CPS cystoperitoneal shunt, VPS ventriculoper-itoneal shunt, Endoscopy endoscopic fenestration

816 Childs Nerv Syst (2012) 28:813–819

2. A compression of the hypothalamoseptal triangle whichincludes the specific septal, periseptal nuclei, and associ-ated projection pathways—related to neuropsychiatricsymptoms and compression of optic chiasm and pathways;

3. A chronic deep venous impairment—displacement andstretching of the internal cerebral and subependymalveins that may be responsible for progressive focaldeficits [2, 7, 20].

In general, a child with an expansive cyst presents atdiagnosis with signs of increased ICP or other objectivesigns (seizures, hydrocephalus, or focal deficit). However,the psychological symptoms are often already existent, butthey are neglected. In fact, mental status changes were rarelythe only clinical presentation of the patient. Regularly, men-tal status changes were associated to symptoms or signs ofincreased ICP, except in three cases. In the first, the childpresented with convulsions [8], in the second case, therewas gait ataxia, paraparesis, and hydrocephalus, and in thelast one, there was also an “unstable gait” [21].

In our case, the child presented with acute headaches,vomiting, and a syncopal episode, probably by a mechanismof ball valve phenomenon with intermittent acute hydro-cephalus. In addition, the patient had a history of decreasedconcentration and attention, with progressive decline in hisschool performance, which may indicate a mass effect of thecyst on the adjacent limbic structures.

Diagnosis

Usually, radiological diagnosis of CSP cysts is establishedon computed tomography (CT) scan and magnetic reso-nance imaging (MRI). These studies reveal a cavity betweenthe lateral ventricles with different degrees of bowing of thecyst walls and obstructive hydrocephalus. However, it is notso easy to establish a correlation between the cyst revealedon imaging studies and symptoms.

The symptoms are frequently nonspecific. Furthermore,some expanding cysts remain asymptomatic despite radio-logical evidence of mass effect [17, 19, 31]. The diagnosisof a symptomatic CSP cyst is even more difficult if the cystis not an isolated lesion, particularly in pediatric patients.Careful consideration of the history and neurological exam-ination is therefore essential [17].

Headaches may result from intermittent obstruction ofthe interventricular foramina caused by the cyst [1, 17],but hydrocephalus is not always documented radiologi-cally. In the pediatric population review, hydrocephaluswas associated with headache in only three patients(30 % of children with hydrocephalus). The hypothesisis that headaches are caused by transient obstructivehydrocephalus or directly by the mass effect of the cyston surrounding structures.

Wester et al. [31] suggest that the relationship betweenCSP cysts and hydrocephalus is complex, and concomitanthydrocephalus may not be a consequence of the cyst. Com-municating the cyst to the lateral ventricles by a fenestrationwill make the cyst collapse, but it will not necessarilyalleviate the symptoms caused by hydrocephalus. Theyreported two cases of CSP cysts and concomitant hydro-cephalus that were not improved following stereotactic cys-toventricular shunt. Lancon et al. [18] proposed thatadhesions at the foramen of Monro may be present andcontribute to the persistence of hydrocephalus followingcyst fenestration.

It seems that a basic factor of symptomatic CSP cyst isthat it is noncommunicating [17, 21, 25]. However, theproof of communication with the ventricular system isdifficult in routine neuroimaging studies [10]. Maybe theaddition of modern neuroimaging in the preoperative eval-uation, such as MRI with study of cerebrospinal fluidflow dynamics, may help to establish the real status ofthe cyst. Miki et al. [20] proposed to add other investiga-tions to monitoring cerebral blood flow such as perfusionMRI to corroborate the diagnosis before a treatment has tobe decided, offering an objective evaluation of peripheraltissue damage due to direct compression of the cyst. Theydemonstrated an alteration of perfusion in the anterior partof corpus callosum on perfusion MRI, which improvedafter treatment.

Management and outcome

The origin of the symptoms is not always clear and,additionally, the natural history of this rare condition isnot well known. Thus, the indications for treatment remaincontroversial.

Cases with spontaneous resolution of the cyst were de-scribed in the literature [15, 24]. Dramatic evolution hasbeen also described in a few patients. The case reported byBayar et al. [3] illustrates a giant cyst extending into theposterior fossa. The patient died of a respiratory failure,probably secondary to brainstem compression, on the thirdpostoperative day of a cystoperitoneal shunt.

Finally, several authors agree that CSP cyst may yieldincapacitating symptoms or important neurological dysfunc-tion, and symptomatic cases must be treated. The symptomsare relieved in almost all cases when the cyst is decom-pressed. In this review, only one child was reported ashaving a symptomatic CSP cyst demonstrated in radiologi-cal studies and was not treated [8]. The symptoms resolvedspontaneously, probably due to rupture of the cyst. Thiswould explain the findings of a communicating cyst in thispatient.

Currently, the treatment is considered whenever there isan association of a CSP cyst on imaging studies and clinical

Childs Nerv Syst (2012) 28:813–819 817

signs and symptoms attributable to the obstruction of thecerebrospinal fluid flow in the foramen of Monro or directcompression of surrounding tissues by the cyst [17, 20]. It isimportant to consider that not all patients with evidence ofincreased ICP present with hydrocephalus, and also theabsence of clinical increased ICP or hydrocephalus is not acontraindication to surgery in a patient with mental statuschanges or focal neurological deficits attributable to the CSPcyst [17].

The primary goal of treatment is to relieve the mass effectcaused by the cyst, and it is exclusively surgical. Proposedtechniques include open surgical procedures—craniotomyand cyst fenestration (via a trancallosal or transcortical ap-proach)—ventriculoperitoneal or cystoperitoneal shunting[17], stereotactic fenestration, and cystoventriculostomy us-ing neuroendoscopic techniques with or without imageguidance.

First treated CSP cyst was published by Dandy in 1931[6], who treated a 4.5-year-old boy by a transcallosal fenes-tration. Then after, 29 cases of treatment in children werepublished (Table 1). In total, 15 children were treated bynonendoscopic techniques, and since 1999, 15 childrenwere all treated by endoscopy. Of the first ones, 66.7 %(10/15) were asymptomatic, and 26.7 % improved theirsymptoms in mean follow-up of 16.1 months. Wester et al.[32] described a case of a patient with a CSP cyst andconcomitant hydrocephalus, not corrected by a cystoven-tricular shunt, and a second patient who was reoperated onafter a cystoventricular shunt and remained asymptomaticfollowing a conversion to a cystoventriculoperitoneal shunt.Donati et al. [7] presented a case of a child unsuccessfullytreated by a ventriculoperitoneal shunt, requiring reopera-tion with endoscopy. There was one case report of death bya giant cyst despite a cystoperitoneal shunt [3]. Follow-upwas not specified in two cases, and only four cases (12.9 %)have been followed for more than 4 years.

In the review by Lancon et al. [17], including 18 adultand pediatric patients with symptomatic CSP cysts, fourpatients were not treated, and symptoms resolved after rup-ture of the cyst, probably during exams of ventriculography/pneumoencephalography. These tests are no longer current-ly practiced. The other 14 patients were treated by non-endoscopic techniques, and most of them (83 %) wereasymptomatic at follow-up. The three patients (17 %) whodid not fully recover following treatment were boys aged 6,8, and 11 years [2, 14, 21].

Since Jackowski et al. [13] reported in 1995 the firstendoscopic fenestration of a large midline cyst in a 60-year-old man, the endoscopic technique has become theprocedure of choice to treat these rare lesions. Lancon etal. [18] have called into question the diagnosis of a true CSPcyst of Jackowski’s patient and published the first case ofCSP cyst in a child treated by endoscopy.

Three endoscopic approaches were described:

1. A frontal approach on the coronal suture 3 cm from themidline targeting the frontal horn of the lateral ventricle(used by the majority of authors);

2. The same cortical frontal approach but targeting directlythe cyst that is punctured and then a fenestration of thetwo walls is performed [20]; or

3. An occipital burr hole to optimize the trajectory into theatrium of the lateral ventricle, which would allow toapproach both leaflets of the cyst perpendicularly [10, 11].

The endoscopic fenestration offers a less invasive ap-proach, direct visualization, and good effectiveness. Directvisualization of neural and vascular structures prevents in-advertent injury. It is also important to inspect the foraminaof Monro to search potential adhesions, which may play arole in the hydrocephalus to persist after apparently success-ful drainage of the cyst [18]. In addition, this techniqueavoids the need to place a shunt and allows to perform abiopsy of the cyst walls.

In the literature, the children treated by endoscopy did allfully recover or improved following treatment with a meanfollow-up of 28.6 months. Complications following endos-copy in children were rare, with only one case with ableeding during surgery [19].

The main issue is loss of precise target to establish a safecommunication between the cyst and ventricles. Avoidingthese pitfalls in preoperative planning can include echogra-phy [32] or neuronavigation guidance [5].

Our patient was treated by an endoscopic bilateral fenes-tration assisted by neuronavigation, with an excellent resultas most of the cases described in the literature. PostoperativeMRI confirms the reduction in cyst size and no sign oftension inside. The child is symptom free 18 months afterthe treatment.

In our opinion, assisted neuronavigation endoscopy isuseful to select the best operative trajectory and also toperfectly find out the ventricular horn due to fact that itcan be very small because of cyst walls bowing. Once in theventricle, ideally both walls of the cyst should be opened.However, cases with only one wall opened have been de-scribed with good results [5, 9, 10]. Usually, the perforationof the first wall does not cause any particular problem. Theperforation of the contralateral wall of the cyst can be moredifficult due to the proximity of contralateral neural struc-tures and also due to the lack of tension in the wall once thecyst is opened. In such a perspective, neuronavigation canbe useful even though possible shift of the cyst walls can beseen. Contralateral ventricular wall usually does not have ashift. Although bilateral fenestration may be technically reas-suring, it is likely that a unilateral fenestration, if of adequatedimension, would decompress the cyst and eliminate theobstruction of both foramina of Monro. Postoperative success

818 Childs Nerv Syst (2012) 28:813–819

can be judged by decompression of the cyst and resultantalleviation of the symptoms.

There are very few articles on the subject of symptomaticCSP cysts and no clinical trial on the treatment of theselesions. To date, there is no sufficient evidence to establishconsistent recommendations for the management of CSPcysts. However, it is very difficult to conduct prospectivestudies on this rare condition, and treatment recommenda-tions can only be established based on some case reports andexperience of experts.

Conclusion

Cavum septum pellucidum cysts are a rare condition in chil-dren, and the most critical difficulty in the management ofthese lesions is to determine the correlation between symp-toms and the cyst. A rigorous anamnesis, neurological andradiological examinations are necessary before treatment. As-sociation of a CSP cyst and symptoms of mass effect andincreased intracranial pressure such as important headachescan represent an indication for surgical treatment. Neuroendo-scopic fenestration is a well-established therapeutic option andcurrently the treatment of choice for symptomatic CSP cysts.

References

1. Amin BH (1986) Symptomatic cyst of the septum pellucidum.Childs Nerv Syst 2(6):320–322

2. Aoki N (1986) Cyst of the septum pellucidum presenting as hemi-paresis. Childs Nerv Syst 2(6):326–328

3. Bayar MA, Gökçek C, Gökçek A, Edebali N, Buharali Z (1996)Giant cyst of the cavum septi pellucidi and cavum Vergae withposterior cranial fossa extension: case report. Neuroradiology 38(Suppl 1):S187–S189

4. Behrens P, Ostertag CB (1993) Stereotactic management of con-genital midline cysts. Acta Neurochir (Wien) 123(3–4):141–146

5. Chiu CD, Huang WC, Huang MC, Wang SJ, Shih YH, Lee LS(2005) Navigator system-assisted endoscopic fenestration of asymptomatic cyst in the septum pellucidum—technique and casesreport. Clin Neurol Neurosurg 107(4):337–341

6. Dandy WE (1931) Congenital cerebral cysts of the cavum septipellucidi (4 fth ventricle) and cavum vergae (sixth ventricle).Diagnosis and treatment. Arch Neurol Psychiatry 25:44–66

7. Donati P, Sardo L, Sanzo M (2003) Giant cyst of the cavum septipellucidi, cavum Vergae and veli interpositi. Minim Invasive Neu-rosurg 46(3):177–181

8. Echternacht AP, Campbell JA (1946) Mid-line anomalies of the brain;their diagnosis by pneumoencephalography. Radiology 46(2):119–131

9. Gangemi M, Maiuri F, Colella G, Sardo L (1999) Endoscopicsurgery for intracranial cerebrospinal fluid cyst malformations.Neurosurg Focus 6(4):E6

10. Gangemi M, Maiuri F, Cappabianca P, Alafaci C, de Divitiis O,Tomasello F, de Divitiis E (2002) Endoscopic fenestration ofsymptomatic septum pellucidum cysts: three case reports withdiscussion on the approaches and technique. Minim Invasive Neu-rosurg 45(2):105–108

11. Greenfield JP, Souweidane MM (2005) Endoscopic managementof intracranial cysts. Neurosurg Focus 19(6):E7

12. Heiskanen O (1973) Cyst of the septum pellucidum causing in-creased intracranial pressure and hydrocephalus. Case report. JNeurosurg 38(6):771–773

13. Jackowski A, Kulshresta M, Sgouros S (1995) Laser-assistedflexible endoscopic fenestration of giant cyst of the septum pellu-cidum. Br J Neurosurg 9(4):527–531

14. Kansu T, Bertan V (1980) Fifth ventricle with bitemporal hemi-anopsia. Case report. J Neurosurg 52(2):276–278

15. Koçer N, Kantarci F, Mihmalli I, Işlak C, Cokyüksel O (2000)Spontaneous regression of a cyst of the cavum septi pellucidi.Neuroradiology 42(5):360–362

16. Krauss JK, Mohadjer M, Milios E, Scheremet R, Mundinger F(1991) Image-directed stereotactic drainage of the symptomat-ic cavum septi pellucidi et vergae. Neurochirurgia (Stuttg) 34(2):57–61

17. Lancon JA, Haines DE, Raila FA, Parent AD, Vedanarayanan VV(1996) Expanding cyst of the septum pellucidum. J Neurosurg85:1127–1134

18. Lancon JA, Haines DE, Lewis AI, Parent AD (1999) Endo-scopic treatment of symptomatic septum pellucidum cysts: withsome preliminary observations on the ultrastructure of the cystwall: two technical case reports. Neurosurgery 45(5):1251–1257

19. Meng H, Feng H, Le F, Lu JY (2006) Neuroendoscopic manage-ment of symptomatic septum pellucidum cysts. Neurosurgery 59(2):278–283

20. Miki T, Wada J, Nakajima N, Inaji T, Akimoto J, Haraoka J (2005)Operative indications and neuroendoscopic management of symp-tomatic cysts of the septum pellucidum. Childs Nerv Syst 21(5):372–381

21. Miyamori T, Miyamori K, Hasegawa T, Tokuda K, Yamamoto Y(1995) Expanded cavum septi pellucidi and cavum vergae associ-ated with behavioral symptoms relieved by a stereotactic proce-dure: case report. Surg Neurol 44(5):471–475

22. Pearce JM (2008) Some observations on the septum pellucidum.Eur Neurol 59(6):332–334

23. Sarwar M (1989) The septum pellucidum: normal and abnormal.AJNR Am J Neuroradiol 10(5):989–1005

24. Sayama CM (2006) Spontaneous regression of a cystic cavumseptum pellucidum. Acta Neurochir (Wien) 148:1209–1211

25. Shaw CM, Alvord EC (1969) Cava septi pellucidum et vergae:their normal and pathological states. Brain 92:213–224

26. Souweidane MM, Hoffman CE, Schwartz TH (2008) Transcavuminterforniceal endoscopic surgery of the third ventricle. J Neuro-surg Pediatr 2(4):231–236

27. Tamburrini G, D’Angelo L, Paternoster G, Massimi L, CaldarelliM, Di Rocco C (2007) Endoscopic management of intra andparaventricular CSF cysts. Childs Nerv Syst 23(6):645–651

28. Tubbs RS, Krishnamurthy S, Verma K, Shoja MM, Loukas M,Mortazavi MM, Cohen-Gadol AA (2011) Cavum velum interpo-situm, cavum septum pellucidum, and cavum vergae: a review.Childs Nerv Syst 27(11):1927–1930

29. Wang KC, Fuh JL, Lirng JF, Huang WC, Wang SJ (2004) Head-ache profiles in patients with a dilatated cyst of the cavum septipellucidi. Cephalalgia 24(10):867–874

30. Wester K, Pedersen PH, Larsen JL, Waaler PE (1990) Dynamicaspects of expanding cava septi pellucidi et Vergae. Acta Neuro-chir (Wien) 104(3–4):147–150

31. Wester K, Krakenes J, Moen G (1995) Expanding cava septipellucidi and cava vergae in children: report of three cases. Neu-rosurgery 37(1):134–137

32. Weyerbrock A, Mainprize T, Rutka JT (2006) Endoscopic fenes-tration of a symptomatic cavum septum pellucidum: technical casereport. Neurosurgery 59(4 Suppl 2):ONSE491

Childs Nerv Syst (2012) 28:813–819 819