Pediatric Traumatic Cervicothoracic Extradural Arachnoid Cyst

Abstract

Background Extradural spinal arachnoid cysts are an uncommon cause of myelopathy in pediatric populations, typically originating from congenital defects in the dura mater. However, there are no documented cases of multiple traumatic arachnoid cysts in children.

Case Description We report on a 3-year-old girl who presented with persistent neurologic deficits following a trauma, later diagnosed with multiple posttraumatic extradural arachnoid cysts. This is the first documented case of its kind in the literature.

Conclusion The definitive treatment for these cysts is surgical resection of the cyst wall and obliteration of the defect. This case underscores the importance of considering traumatic origins in the differential diagnosis of pediatric myelopathy.

Keywords

extradural arachnoid cyst - traumatic - multiple arachnoid cyst - pediatric

Introduction

Spinal extradural arachnoid cysts, rare entities comprising 1 to 3% of spinal tumors, can be incidentally diagnosed or present with spinal cord and/or nerve root compression.[1] [2] The etiology and pathogenesis are not fully understood, though they are often considered congenital, originating from a dural defect leading to arachnoid herniation and expansion. In pediatric patients, these cysts can also be acquired due to iatrogenic damage, hemorrhage, meningeal infection, inflammation, or trauma.[3] [4] This article reports on a 3-year-old girl with multiple posttraumatic spinal extradural arachnoid cysts, successfully treated with complete excision and obliteration of the dural defect.

Case Report

History and Physical Examination

A 3-year-old girl was referred to our hospital after being hit by a fast-moving car while crossing a road, presenting with right-sided body weakness. Upon examination, she had a scalp laceration and right-sided weakness with a motor power of 0/5 on the MRC scale. Brain and cervical CT scans were normal, but spine MRI revealed extensive cervical cord edema extending to the lower medulla. She developed respiratory failure and was placed on a mechanical ventilator. Over 6 weeks, her condition gradually improved, and she was discharged, showing improvement with physiotherapy.

Ten months posttrauma, the family observed worsening right leg weakness, causing a limp. She had no complaints of urinary difficulty or fecal incontinence. On physical examination, she was alert with hypertonic extremities, motor power of 5/5 in all extremities except the right lower extremity which was 4/5 on the MRC scale, and exaggerated deep tendon reflexes (3 + ) throughout, with preserved sensory modalities.

Imaging Findings

Initial spine MRI posttrauma showed cervicomedullary hyperintensity suggestive of cord edema with no extramedullary lesions. A recent MRI revealed a long-segment cystic lesion of CSF signal intensity with multiple band-like hypo intensities at the dorsal extradural space from C5 to T7, suggesting arachnoid cysts with multiple septations and diffuse cord compression ([Fig. 1A] and [B] ). The cervicothoracic spinal cord was displaced anteriorly and flattened.

Fig. 1 Preoperative sagittal T1W (A) Sagital T2W (B) and axial T2W (C) MR imaging sequences showing long segment multiple arachnoid cysts with CSF signal intensity at C5 to T7 level.

The managing team opted for surgical intervention. Using a posterior midline incision, C7 to T5 laminoplasty with T8–T9 laminectomy was performed ([Fig. 2]). The spinal canal was filled with multiple extradural cysts with septations and a 5 × 5 mm dural defect at the right dorsolateral aspect of the dura mater between the nerve root sleeves at C7 and C8. Complete resection and microsurgical repair of the dural defect were conducted using a prolene 7–0 suture. In the second week postop, she developed pseudomeningocele and signs of infection, necessitating a second operation. The primary defect site and multiple exposed dural sleeves were patched with a fat graft. A drain was left and removed on the third postop day, resulting in a complete resolution of pseudomeningocele. She was treated with antibiotics for 2 weeks, and her motor weakness improved significantly, leading to her discharge. Pathological examination confirmed the presence of arachnoid cysts. At her fourth-month follow-up, her weakness had resolved, although exaggerated reflexes persisted. Her control imaging ([Fig. 3]) also showed a complete resolution of the cyst. She has successfully returned to her daily routine, enjoying activities like playing with her friends.

Fig. 2 Intraoperative photograph showing the extradural arachinoid cyst with multiple septation (A) with underlying intact dura after removal of most of the cyst component. (B) Laminoplasty bone before (C, D) after replacement. (E) A nerve hook was inserted into the dural defect which was 5 mm × 5 mm sized at the right dorsolateral aspect of the dura mater around the C7 nerve root sleeve.

Fig. 3 Fourth month postoperative Sagittal T1W (A) Sagittal T2W (B) and axial T2W (C) MT imaging sequences at the same level as preop imaging showing complete resolution of arachinoid cyst.

Discussion

Spinal arachnoid cysts were first observed by Schlesinger in 1893 and reported by Spiller et al in 1903. Over the years, the classification has evolved, and Nabors et al categorized these cysts into three types: type I (extradural meningeal cysts without nerve root fibers), further divided into IA (extradural arachnoid cysts) and IB (sacral meningoceles); type II (extradural meningeal cysts with nerve root fibers); and type III (intradural meningeal or arachnoid cysts).[5] [6] [7] [8] Our patient presented with type IA cervicothoracic arachnoid cysts without nerve root fibers, aligning with this classification.

The etiology of arachnoid cysts is not fully understood, with congenital origins being the most common. They may arise from the pathological proliferation of arachnoid cells during embryonic development or congenital dural defects leading to arachnoid herniation. Cysts can expand due to mechanisms like the ball valve effect, free water entrance, and active CSF secretion.[5] [9] [10] [11] [12] [13] Congenital causes, with or without neural tube defects, predominate in pediatric cases. However, dural defects can also be acquired from spine surgery, trauma, chronic arachnoiditis, infections, or degenerative conditions.[2] [9] [14] [15] [16] Our patient's cysts were likely acquired following blunt spine trauma, evidenced by the absence of cysts on initial imaging.

Extradural arachnoid cysts are most commonly found in the thoracic spine (65%), especially in the middle to lower thoracic segments, followed by the thoracolumbar, lumbar, lumbosacral, sacral, and cervical regions.[17] Cervical cysts are particularly rare and usually short-segment, seldom extending into the thoracic region.[11] The extensive cervicothoracic cyst in our patient is rarely documented in the literature, making this case exceptional.

Clinical presentation varies with cyst location, size, and etiology, ranging from incidental findings to severe myelopathy. Symptoms can be progressive, intermittent, or sudden, often exacerbated by increased intraspinal pressure, such as during the Valsalva maneuver. Thoracic cysts typically cause shorter symptom duration due to the narrow canal, leading to pain, spasticity, numbness, and paraparesis, more common in adolescents. Lumbar cysts present with pain and radiculopathy, while sacral cysts often result in bowel or bladder dysfunction, typically in adults.[11] [17] [18] [19] Our patient presented with progressive right-sided weakness and hypertonia, emphasizing the varied symptomatology seen with these cysts.

MRI is the diagnostic modality of choice for spinal arachnoid cysts, providing detailed anatomical insights, compressive effects, and comparisons of CSF and cyst contents. Advanced techniques like Cine-MRI and 3D CISS help localize cyst communication sites, aiding minimally invasive surgical planning.[12] [20] [21] [22] CT myelography can also demonstrate the connection between the spinal subarachnoid space and the cyst cavity and bony erosion.[23] [24] [25] In our patient's case, detailed MRI findings were crucial in guiding diagnosis and surgical planning, revealing the cyst's extent and impact.

Conservative management may be appropriate for incidental or mild cases, but progressive neurologic symptoms necessitate surgical intervention. The goal is neural decompression and preventing cyst refilling, ideally through complete resection and closure of the communication with the subarachnoid space.[26] [27] [28] Partial resection may be required if cysts adhere to neural tissue or dura or if bleeding from the epidural venous plexus is profuse, with or without expansile duraplasty, to minimize recurrence risk.[29] [30] [31] In our patient's case, complete resection and dural defect repair were successfully performed to prevent recurrence.

For long-segment cysts, laminectomy, laminoplasty, or hemilaminectomy are described. Conventional laminectomy poses risks of postoperative instability, subluxation, or kyphotic deformity. Laminoplasty, offering adequate exposure, decompression, and maintaining spinal stability, is recommended, especially for multiple-segment cysts, to prevent postoperative kyphosis.[32] [33] [34] [35] Our patient underwent laminoplasty combined with laminectomy, ensuring adequate decompression and stability.

Neurological recovery depends on the patient's age, cyst size, and the duration and severity of spinal cord compression. Poor surgical outcomes are associated with long-standing spastic myelopathy, preoperative myelomalacia, and significant spinal cord atrophy.[35] Our patient's recovery was notable, with significant improvement in motor function despite persistent exaggerated reflexes.

Conclusion

Spinal arachnoid cysts are rare causes of myelopathy in the pediatric age group. The cause is thought to be congenital. However, multiple spinal extradural arachnoid cysts of traumatic origin have not been reported in a pediatric population. The surgical management should aim to decompress neural structures and obliterate the communication to avoid recurrence. New onset symptoms or failure to improve from the original traumatic neurologic status should raise suspicion for spinal arachnoid cysts.

Conflict of Interest

None declared.

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Publication History

Received: 06 November 2024

Accepted: 17 February 2025

Article published online:
24 March 2025