Chronic subdural hematoma associated with the

Chronic subdural hematoma associated with the middle fossa arachnoid cyst: pathogenesis and review of its management Young Seok Kwak, Sung Kyoo Hwang, Seong Hyun Park & Ji Young Park

Child's Nervous System ISSN 0256-7040 Childs Nerv Syst DOI 10.1007/s00381-012-1896-4

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Author's personal copy Childs Nerv Syst DOI 10.1007/s00381-012-1896-4

ORIGINAL PAPER

Chronic subdural hematoma associated with the middle fossa arachnoid cyst: pathogenesis and review of its management Young Seok Kwak & Sung Kyoo Hwang & Seong Hyun Park & Ji Young Park

Received: 13 December 2011 / Accepted: 9 August 2012 # Springer-Verlag 2012

Abstract Background The pathogenesis of chronic subdural hematoma (CSDH) associated with an arachnoid cyst (AC) is still not clear. We propose an origin of initial bleeding of CSDH in patients with AC based on our experience and discuss the management of this disease. Materials and methods The total number of operations included in this study was 23. Eleven cases were indicated because of associated CSDH (group 1), and the other cases were due to different reasons (group 2). The relationship of the AC and dura was evaluated in patients who did not have CSDH (group 2) because patients with CSDH would have had structural changes of AC due to hematoma. Results In group 1, the age distribution was from 8 to 61 years (mean 23.0). The development of CSDH was not related with Galassi types. The hematomas were located outside of the outer cyst membrane in six patients and both inside and outside in three patients. In two patients, the location could not be reviewed. After suspicion of the outer cyst membrane as the origin of the bleeding site, we found small bridging vessels between the dura and outer cyst membrane in three of five consecutive patients in group 2. Conclusions Based on our surgical experience of AC cyst, we found small bridging vessels between the dura and outer membrane of the AC. We suggested that these small vessels are the source of initial bleeding leading to CSDH in AC. Y. S. Kwak : S. K. Hwang (*) : S. H. Park Department of Neurosurgery, Kyungpook National University Hospital, 50 Samdeokdong Jung-gu, Daegu 700-721, South Korea e-mail: [email protected] J. Y. Park Department of Pathology, Kyungpook National University Hospital, Daegu, South Korea

Keywords Arachnoid cyst . Middle fossa . Chronic subdural hematoma . Pathogenesis . Management

Introduction Intracranial arachnoid cyst (AC) is a congenital extra-axial partially transparent cystic lesion containing cerebrospinal fluid (CSF) or CSF-like clear fluid, and the middle fossa is the most common location of this lesion [17, 24]. The natural history of the middle fossa AC is not exactly known because many children who have the lesion are asymptomatic and recognized incidentally by CT or MRI scan taken due to other conditions such as head injury. However, middle fossa AC is sometimes symptomatic through headache, epilepsy, or intracranial hematoma [17, 24]. One of the complications of AC is the development of chronic subdural hematoma (CSDH) [1, 4, 13, 23]. The traumatic CSDH, even though the trauma may be trivial or unrecognized, usually develops in the elderly and infants and is uncommon in older children or young adults [11]. When we find cases of children or young adults presenting with CSDH, we have to consider the possibility of unrecognized middle fossa AC as the cause of CSDH. Pathogenesis of CSDH associated with the AC is still not exactly known. It is considered to emerge from the vessels surrounding the cyst wall or floating inside the cyst [13, 23, 25]. Several authors have emphasized the structures between the dura and arachnoid membrane; however, it is nearly impossible to find the bleeding point during operation of CSDH associated with AC because the original anatomical structures are already distorted by the hematoma [10, 11]. In this study, we propose an origin of initial bleeding of CSDH according to our experience of the surgery and microscopic findings of AC without CSDH and discuss the management of CSDH associated with AC.

Author's personal copy Childs Nerv Syst

Materials and methods

Table 1 Demographic data of the arachnoid cyst presented with chronic subdural hematoma

We retrospectively reviewed operation records and radiological findings of all operated AC patients with or without CSDH between 1996 and 2009. All included patients were operated by the senior author (SKH). The total number of operated patients was 23. Eleven of them were operated on due to the associated CSDH (group 1). All of the patients were operated with craniotomy, partial or subtotal removal of the membranes of CSDH and outer membrane of the cyst, and fenestration to the basal cistern. Operative indications of group 2 patients varied including Galassi type 3, development of subdural hygroma, and symptomatic AC such as seizure, headache, or signs of increased intracranial pressure. Operation methods were similar to group 1 except the absence of the removal of the hematoma membrane. Incidentally found Galassi type 1 or 2 ACs were usually not operated on. Galassi type 1 was defined as a small semicircular cyst confined to the anterior part of the temporal fossa, type 2 as a quadrangular medium-sized temporal cyst, and type 3 as a large oval fluid collection with midline shift [7]. The hypothesis of the bleeding site came from the patients who did not have CSDH (group 2) because patients with CSDH would have had anatomical changes of AC due to the hematoma.

ID

Sex/age

Trauma history

Site of hematomaa

1 2 3 4 5 6 7 8 9 10 11c

M/45 M/8 M/61 M/46 M/19 M/19 F/10 M/11 M/12 M/11 M/11

1 month ago No Nob 4 months ago No 3 months ago No 2 weeks ago No No No

I&O I&O O O I&O O O O

Results The total number of patients with the middle fossa AC included in this study was 23. Of these, 11 patients had CSDH (group 1), whereas 12 did not have CSDH (group 2). In group 1, the numbers of patients with Galassi types 1, 2, and 3 were 5, 6, and 0, respectively, showing that the development of CSDH was not related with the Galassi types. In group 2, the numbers were 2, 5, and 5. Age distribution of group 1 patients was from 8 to 61 years (mean 23.0). Six of them were below the age of 15 years. The hematomas were located outside of the cyst membrane in six patients, whereas three patients had the hematoma both inside and outside of the cyst membrane. The location of the hematoma was not described in the operation records of two patients. Four patients presented with history of trauma history. In group 2, ten patients were male and two were female. Age distribution was from 2 to 15 years old (mean 8.3). Among them, operation was performed on two patients due to associated subdural hygroma. Also, two patients with Galassi type 3 AC were operated with cystoperitoneal shunt because of the enlarging cyst with increased intracranial pressure after craniotomy and fenestration. Patients’ demographic data are shown in Tables 1 and 2. During the operation, we found small connecting vessels between the outer arachnoid membrane and dura and

O

a Hematoma accumulated inside of the outer cystic membrane (I), or outside (O), or both (I & O). Blank cells had no description on the record b

This patient was under peritoneal dialysis due to chronic renal failure

c

This patient was re-operated using craniotomy and fenestration due to recurrence 2 months after burr hole drainage by another medical center

suspected these vessels as the primary site of initial bleeding. After our first suspicion, we found the same vessels in two more patients among the four consecutive patients in group 2. Table 2 Demographic data of the arachnoid cyst that did not show chronic subdural hematoma ID

Sex/age

Galassi type

Operative indications

1

M/9

2

Headache

2

M/14

1

Seizure

3

M/5

3

4

M/6

2

Headache

5

M/15

3

Acute epidural hematoma

6

F/11

1

Seizure

7

M/9

2

Exophthalmus

8

M/5

2

Subdural hygroma

9

M/9

3

10

M/9

2

Subdural hygroma Headache

11

F/2

3

12

M/5

3

Remarks

Bridging vein contained in the outer membrane Two small veins on the outer cyst wall Craniotomy and fenestration followed by cystoperitoneal shunt

Hygroma followed by cystoperitoneal shunt

Minute vessel on the outer cyst membrane and connection to dura Thick whitish outer cyst membrane, associated with hydrocephalus

Increased ICP

Small vascular connection to the dura


Case presentations Case 1. This 9-year-old-male patient presented with headache for several months. Neurological examination was normal. MRI revealed Galassi type 2 AC on the right fronto-temporo-parietal. A small amount of subdural hygroma was also noted. Right frontotemporo-parietal craniotomy was conducted followed by partial removal of the outer membrane and fenestration with the basal cistern. The CSF pressure was slightly high. During the operation, the outer membrane had small vessels on the cyst wall and some were connected with the dura (Fig. 1). Histopathologic examination revealed small vessels between the dura and arachnoid membrane (Fig. 2).

Fig. 2 Microscopic view of case 1 revealed small vessels (arrows) between the arachnoid membrane and overlying dura tissue. Hematoxylin eosin (a), EMA (b), and CD34 (c) staining (×100)

Fig. 1 Magnetic resonance imaging of case 1 revealed a Galassi type 2 arachnoid cyst on the right middle fossa (a). Operative finding showed a connecting small vessel between the dura and arachnoid cyst (arrow) (b)

Case 2. This 2-year-old female presented with a large head but otherwise normal development. She was born by uneventful normal vaginal delivery. MRI


revealed a large left fronto-temporo-parietal AC with midline shift. Craniotomy revealed moderately tense dura. Upon the opening of the dura, the arachnoid membrane was relatively tightly adhered to the dura, and small vessels were found on the outer membrane connecting with the dura. Separation of the arachnoid membrane from the dura caused oozing from the dura and tearing of small vessels (Fig. 3). Case 3. This 5-year-old female presented with headache for several months. She had no neurological focal deficit and showed normal development. MRI revealed a large fronto-temporo-parietal AC extending to the ambient and quadrigeminal cisterns with midline shift. Craniotomy was performed and the opening of the

Fig. 3 MRI scan in case 2 showed a Galassi type 3 arachnoid cyst on the left middle fossa (a) and operative view showing adhesion of the arachnoid membrane and dura with connection by small vessels (arrow) (b)

dura revealed small vessels running through the outer arachnoid membrane and some vessels connecting with the dura. Separation of the vessels resulted in small bleeding from the AC and dura (Fig. 4).

Discussion AC is an extra-axial cystic lesion with the wall composed of arachnoid membrane and filled with CSF or CSF-like fluid. Its pathogenesis is not clear; however, the most accepted theory is that the cyst is a result of an aberrant development of the subarachnoid space during splitting of the primitive perimedullary mesh by CSF flow [18, 19]. AC can develop in any part of the intracranial space, and the symptoms and signs are different depending on the location [17, 24]. The most common predilection site is

Fig. 4 MRI shows a large cystic lesion on the right middle fossa extending to the ambient cistern (a). The operative view showing the vessels connecting the arachnoid cyst and overlying dura (arrow) (b)


the middle fossa. Many of them are identified incidentally by CT or MRI scans taken due to other conditions, most commonly head injury. In symptomatic patients, headache, seizure, or signs of increased intracranial pressure are the main manifestations of the lesion. One of the common complications of AC is the development of CSDH or subdural hygroma [13]. The incidence of subdural fluid collection as a complication of the middle fossa AC is reported to be from 6.6 to 17.5 % [6–8, 21, 23]. However, these data are questionable because many patients with AC are not diagnosed before taking a CT or MRI scan due to various reasons. Parsch et al. [15] reported that among their 658 cases of subdural hematoma or hydroma, 16 (2.4 %) were associated with AC. Several authors have reported about the origin of CSDH in AC [4, 13, 23, 25]. Page et al. [13] proposed two theories to explain this. The first theory involved the rupture of bridging veins or vessels in the cyst wall due to the easy transfer of pressure through the cyst. The second theory indicated that AC is less compliant than the normal brain resulting in reduced intracalvarial cushioning following trauma. The underlying cyst cavity provides fluctuating movement of the cystic fluid, transferring the shearing force to the outer membrane thus tearing the small vessels between the outer arachnoid membrane and dura resulting in small bleeding which progresses the condition to the symptomatic chronic stage. According to Haines et al. [10], the subdural space is not a potential space contrary to pleural cavity, but the dura is continuous and tenuously attached to the arachnoid by a few cell junctions. There is a continuity of cellular layers and no intervening space. Collection of hematoma or fluid is usually not subdural in location but is rather created by a shearing open of tissue damage. Our microscopic findings of the outer arachnoid membrane contained some of the dural collagen tissue which supports the findings by Haines et al. [10]. Their anatomical findings also provide the rationale of the development of CSDH in AC other than the middle fossa, such as cerebral convexity whose cyst wall is in contact with the dura matter [4]. We experienced cases of CSDH associated with the small cerebral convexity AC (not reported). Go et al. [9] postulated that the cysts situated at the convexity of the frontal and parietal lobes may be related to those of the Sylvian area, while basal and suprasellar cysts may not be true ACs but leptomeningeal fluid collections that occur secondary to trauma or meningitis. Most traumas are considered minor or trivial in patients with CSDH. In our series, 7 of 11 patients with CSDH had no recognizable history of trauma. However, if we consider the high level of physical activeness in young patients, minor traumas could be overlooked by patients. Popular sports activities such as soccer or basketball were reported as causes of CSDH associated with AC in young adolescents [16, 22]. Chronicity of the

hematoma in most patients associated with AC with absent or trivial history of trauma shows that bleeding should be slow from the small vessels. It is conceivable that floating or attached veins around the AC are too large to provoke slow developing CSDH. Wester and Helland [23] proposed a pathophysiological mechanism as the loose attachment between the arachnoid and the innermost layers of the dura. They observed the easy oozing of blood from multiple, small vascular openings in the dural surface, and a light manipulation of the parietal membrane easily induced the leakage of blood in their operative fields. Kushida et al. [11] reported in their CSDH cases that unusual small veins were observed which run on the surface of the membranous capsule of the AC and the infrequent bridging to the Sylvian fissure at operation. However, it is very difficult to find the point of bleeding during the operation of CSDH associated with AC, and this is the reason why we investigated in patients without CSDH. Our series also did not show the bleeding point in CSDH; however, we added gross and microscopic structures of AC in patients without CSDH. More intense studies should be accumulated to elucidate the mechanism of the development of CSDH in AC to provide the most appropriate management method for CSDH associated with AC. In our series, the age difference between the two groups is not negligible and it presents as a limitation of our report. However, we do not know of any morphological changes of the arachnoid membrane according to age and its significance on our explanation of the pathogenesis. It is not fully understood how the cystoperitoneal shunt, which is another option to manage AC, prevents the development of CSDH even though the outer membrane remains intact. In our opinion, it seems likely that the shunt can compensate for the intracystic flow movement to prevent the tearing of the vessels connecting the AC and dura by reducing the shearing movement of the cyst wall. However, we can imagine that the shunt catheter may prevent movement of these vessels which may contradict our hypothesis. As we mentioned above, we conducted this study in AC patients without CSDH. We found connecting small vessels between the outer arachnoid membrane and dura, and tearing of those vessels during the operation resulted in small bleeding from the dura and arachnoid membrane. We did not try to find these in patients with CSDH because the anatomy would have been displaced due to the hematoma formation. Our findings also provide the rationale that accumulation of most CSDHs is outside of the cyst and the development of CSDH was not related to the cyst size. The development of hematoma can happen both inside and outside of the cyst. Wester and Helland [23] reported that among their 11 cases, six cases were both intracystic and subdural while five were subdural. Reports of tearing of the outer membrane in AC cases associated with subdural


hygroma [5, 21] provide the possibility that combined intracystic and extracystic hematoma is due to the entering of the hematoma through the torn outer arachnoid membrane during trauma. Regarding the treatment of AC, there are several options including craniotomy and fenestration, endoscopic fenestration, and cystoperitoneal shunt. Optimal management of the AC associated with subdural fluid collection also remains controversial [21]. Sprung et al. [21] reported similar results of rebleeding in both burr hole trephination without opening of the cyst and craniotomy groups. Successful treatment by simple drainage of CSDH with the AC intact was also reported [1, 2, 4]. In our earlier experiences, simple drainage of CSDH was not efficient because of the recurrence of hematoma. In the recurred cases, the hematoma did not recur after performing craniotomy with partial or subtotal removal of the membranes of hematoma and AC. We think the surgery of the AC is mandatory either by cyst membrane removal or cystoperitoneal shunt. However, it is still a very controversial choice between the removal of the membrane and simple burr hole trephination [1, 12, 14, 15]. Endoscopic removal and fenestration could be an option of management [3, 20]. There should be special attention given to the possibility of association of AC, especially the middle fossa or convexity, when young patients presented with CSDH [13, 21].

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