Delayed Onset Huge Refractory Multiloculated Cervical Pseudomeningocele after Retromastoid Craniectomy: Case Report and Review of Literature

Vinu V Gopal, Mahadevan K

Department of Neurosurgery, Government Medical College, Kottayam, Kerala, India*

Corresponding Author: Dr. Vinu V Gopal, Department of Neurosurgery, Government Medical College, Kottayam, Kerala, India. Phone: +91-9656769340, Email: vinoogopa@gmail.com

Abstract

Pseudomeningoceles after posterior fossa surgery pose a technical challenge to operating neurosurgeons. Cervical pseudomeningocele is unique in that herniation of neural tissue into pseudomeningocele sac produces catastrophic complications. Patient usually presents with a postural headache. Treatment includes extrinsic compression, cerebrospinal fluid diversion followed by primary operative dural repair if refractory. In our case report, we present a rare complication of delayed onset huge refractory multiloculated cervical pseudomeningocele after retromastoid craniectomy, which was surgically repaired. Detailed review of the literature regarding its pathogenesis and a technical note of surgical repair is included.

Key words: Delayed, Cervical, Pseudomeningocele

 

Introduction

Pseudomeningocele is an abnormal collection of cerebrospinal fluid (CSF) in a sac lined by a fibrous membrane that communicates with the subarachnoid space. This was first reported by Hyndman and Gerber in 1946.1 The terms “meningocele spurious,” “pseudocyst” or “false cyst,” have also been used to describe the condition.2 Pseudomeningoceles can be congenital, idiopathic, post-traumatic or post-surgical. The majority are often asymptomatic. Headache is the most common presenting symptom.

CSF collects in surrounding tissue when CSF pulse pressure exceeds local tissue interstitial pressure.3 The fistula track usually contain a flap of tissue, which act as a valve allowing CSF to flow in one direction only leading to a gradual increase in size during each cardiac cycle.3 The cavity is lined by fibrous membrane instead of leptomeningeal lining hence called pseudomeningocele.3 Cervical pseudomeningocele is unique in that herniation of neural tissue produces catastrophic complications.4

Treatment modalities include extrinsic compression, CSF diversion followed by primary operative dural repair if refractory.

 

Case Report

Our patient was a 35-year-old female who presented with left cerebellopontine (CP) angle tumor of size 4 cm × 4 cm × 3.3 cm associated with severe left sensorineural deafness. Computerized tomography (CT) showed brillianty enhancing tumor in left CP angle region (Figure 1).

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Figure 1. Pre-operative contrast magnetic resonance imaging showing 4 cm × 4 cm cerebellopontine angle lesion

She was taken up for retromastoid retrosigmoid craniectomy in lateral position under general anesthesia. Complete excision of tumor was done. Post-operative period was uneventful except for Grade 2 House Brackmann facial palsy. Patient was discharged on day 10 after suture removal without any evidence of CSF leak. Post-operative CT scan showed complete tumor removal with no evidence of pseudomeningocele (Figure 2). Histopathology report came as vestibular schwannoma.

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Figure 2. Immediate post-operative computerized tomography scan showing complete tumour removal with no evidence of pseudomenigocele

Two months later, she was readmitted with huge 10 cm × 8 cm tense swelling beneath the surgical scar along with neck pain and postural headache. From the history, we found that the swelling was gradually increasing in size over the past 1-month. She was afebrile with no evidence of CSF leak outside.

On examination, a swelling of size 10 cm × 8 cm which was fluctuant and trans illuminant was found beneath the scar (Figure 3).

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Figure 3. Pre-operative picture showing pseudomeningocele with healed intact wound

No fresh neurological deficit was found. There was no clinical evidence of hind brain herniation like pyramidal signs or lower cranial nerve palsy. CT scan showed huge pseudomeningocele (Figure 4).

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Figure 4. Computerized tomography scan showing huge refractory pseudomeningocele thick white arrow showing lesion

After getting informed written consent lumbar drain was put under strict aseptic condition. Since the swelling did not decrease in size considerably following continuous lumbar drainage, the patient was planned for open repair of the dural fistula after getting informed written consent.

Operative Findings

The incision was put over the same surgical scar. The pseudomeningocele sac was dissected out and opened to find a multiloculated cavity with glossy inner membrane (Figure 5).

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Figure 5. Craniectomy site showing multiloculated pseudomeningocele (long white arrow) with the small defect (small white arrow)

A small opening of size 4 mm × 4 mm was seen at the lower end of the dural incision (Figure 6). Pseudomeningocele sac was excised and send for histopathology, which showed a fibrotic wall with no epithelialization.

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Figure 6. Craniectomy site after excision of pseudomeningocele sac. Trying to suture the small defect primarily

The margins of the fistula were found fibrosed. We tried a primary repair of the defect but was failed (Figure 6). We excised the fibrotic tissue and enlarged the dural opening (Figure 7).

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Figure 7. Small defect is enlarged after excising the fibrotic periphery of small defect

The enlarged fistula was then closed by patch repair using occipital fascia along with fat and muscle (Figure 8).

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Figure 8. The enlarged defect is closed using autologous dural substitute obtained from occipital fascia

This was reinforced with pericranial onlay patch. Intraoperative valsalva maneuver showed no CSF leak. Wound was closed in layers with prophylactic continuous lumbar drainage. Post-operative CT scan showed complete resolution of pseudomeningocele (Figure 9).

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Figure 9. Post-operative computerized tomography scan after repair of dural leak and excision of pseudomeningocele sac

Lumbar drain was removed on 3rd day. Patient was discharged of day 10 with no evidence of pseudomeningocele (Figure 10).

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Figure 10. Post-operative picture showing healed wound with no pseudomeningocele

 

Discussion

Pseudomeningocele is an abnormal collection of CSF sac lined by fibrous membrane that communicates with subarachnoid space.

Pseudomeningocele was first reported by Hyndman and Gerber in 1946.1 The terms “meningocele spurious,” “pseudocyst” or “false cyst,” have also been used to describe pseudomeningoceles.2 Pseudomeningoceles can be congenital, idiopathic, post-traumatic or post-surgical. Congenital pseudomeningoceles are usually associated with neurofibromatosis and marfan syndrome.1 Traumatic cervical pseudomeningocele can occur in young patients following brachial plexus injury due to direct injury to duramater causing CSF fistula.5 Muscular weakness from nerve injury can promote growth of the pseudomeningocele sac. Post-operative pseudomeningoceles may result from a tear in the duramater and pia-arachnoid that is unnoticed and is left open during surgery.6 CSF extravasates into the paraspinal soft-tissue space. The CSF may be absorbed initially but, after progressive fibrosis of the surrounding pseudocapsule, CSF is absorbed less readily, resulting in pseudomeningocele formation.6

Presentation

Patients with pseudomeningoceles present most often with postural headache as a result of reduced intracranial pressure following the reduction of CSF volume.7 Some pseudomeningoceles present as fluctuant transilluminant mass that enlarges with coughing and sneezing.8

Pathogenesis

Dural defects cause CSF to collect in the surrounding tissue when CSF pulse pressure exceeds the interstitial pressure of surrounding tissue.3 The fistula track may contain a flap of tissue which acts as a valve allowing CSF to flow in one direction only leading to a gradual increase in size during each cardiac cycle.3 The cavity is lined by fibrous membrane instead of leptomeningeal lining hence called pseudomeningocele. Rare reports of serious complication of herniation of neural tissue are reported.4

Management

The management of pseudomeningocele is controversial particularly in asymptomatic patients. Optimal management is dependent on many factors, including sac size, location, and symptoms.5 Small pseudomeningoceles with minimal symptoms require no treatment. The conservative management in asymptomatic patients was accepted because even large pseudomeningoceles “scar down” and resolves over time.

Treatment includes extrinsic compression, CSF diversion and primary operative dural repair. Extrinsic compression promote dural scarring by increasing surrounding tissue interstitial pressure.9 Symptomatic pseudomeningoceles can be effectively managed with continous lumbar drainage.

Weng et al10 described open excision of pseudomeningocele to promote CSF absorption into soft tissue along with primary dural closure. Primary dural closure is usually practically impossible because of dural desiccation often requiring patch closure. Large dural defects can be closed with patch techniques using an autologous tissue, dural allografts, or fibrin glue along the suture line. Removal of pseudomeningoceles sac and the detethering of neural tissue is required if they adhere to duramater.

There are few reports of giant pseudomeningoceles (>8 cm).11,12 Open surgery with excision of pseudomeningocele sac, repair of a dural fistula, and prophylactic lumbar drainage is recommended. The combination method of treatment was found safe and effective.11,12

Cervical pseudomeningocele present a challenge since herniation of neural tissue produces catastrophic complications. So, early surgical intervention is indicated.

Delayed infection of pseudomeningocele was also reported by Koo et al.13

 

Conclusion

Pseudomeningoceles after posterior fossa surgery pose a technical challenge to operating neurosurgeons. We present a rare case of delayed onset huge refractory multiloculated cervical psedomeningocele after retromastoid craniectomy, which was successfully repaired. Detailed review of literature regarding its mechanism of progression and a technical note of surgical repair is included.

 

End Note

Author Information

  1. Dr. Vinu V Gopal, Assistant Professor, Department of Neurosurgery, Government Medical College, Kottayam, Kerala, India; A9 Quarters, Medical College, Gandhinagar (post), Kottayam – 686 008, Kerala, India. Phone: +91-9656769340, Email: vinoogopa@gmail.com

  2. Mahadevan K, Professor and Head, Department of Neurosurgery, Medical College, Kottayam, Kerala, India; A2 Quarters, Medical College, Gandhinagar (post), Kottayam – 686 008, Kerala, India. Phone: +91-9847292438, Email: drkmnsk@gmail.com

Acknowledgement

To all staff members and postgraduates of Department of Neurosurgery, Government Medical College, Kottayam, Kerala, India.

Conflict of Interest

This study has not received any financial support. Authors alone are responsible for the content of the paper. There are no conflicts of interest.

List of Abbreviations

  • CSF: Cerebrospinal fluid

  • CP angle: Cerebelopontine angle

  • CT: Computerized tomography

  • HPR: Histopathology report

References

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2. Miller PR, Elder FW, JrMeningeal pseudocysts (meningocele spurius) following laminectomy. Report of ten casesJ Bone Joint Surg Am 1968; 50: 268-76.[PubMed]

3. Hawk MW, Kim KD, Review of spinal pseudomeningoceles and cerebrospinal fluid fistulasNeurosurg Focus 2000; 9: e5.[CrossRef] [PubMed]

4. Akhaddar A, Boulahroud O, Boucetta M, Nerve root herniation into a calcified pseudomeningocele after lumbar laminectomySpine J 2012; 12: 273.[CrossRef] [PubMed]

5. Nairus JG, Richman JD, Douglas RA, Retroperitoneal pseudom eningocele complicated by meningitis following a lumbar burst fracture. A case reportSpine (Phila Pa 1976) 1996; 21: 1090-3.[CrossRef]

6. Rinaldi I, Hodges TO, Iatrogenic lumbar meningocoele: Report of three casesJ Neurol Neurosurg Psychiatry 1970; 33: 484-92.[CrossRef] [PubMed] [PMC Free Article]

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8. Rocca A, Turtas S, Pirisi A, Agnetti V, Iatrogenic lumbar pseudomeningoceleZentralbl Neurochir 1986; 47: 311-5.[PubMed]

9. Mehendale NH, Samy RN, Roland PS, Management of pseudomeningocele following neurotologic proceduresOtolaryngol Head Neck Surg 2004; 131: 253-62.[CrossRef] [PubMed]

10. Weng YJ, Cheng CC, Li YY, Huang TJ, Hsu RW, Management of giant pseudomeningoceles after spinal surgeryBMC Musculoskelet Disord 2010; 11: 53.[CrossRef] [PubMed] [PMC Free Article]

11. Hader WJ, Fairholm D, Giant intraspinal pseudomeningoceles cause delayed neurological dysfunction after brachial plexus injury: Report of three casesNeurosurgery 2000; 46: 1245-9.[CrossRef]

12. Singh M, Kasliwal MK, Mahapatra AK, Giant cervical pseudomeningocoele following brachial plexus traumaJ Clin Neurosci 2008; 15: 310-3.[CrossRef] [PubMed]

13. Koo J, Adamson R, Wagner FC, JrHrdy DB, A new cause of chronic meningitis: Infected lumbar pseudomeningoceleAm J Med 1989; 86: 103-4.[CrossRef]

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