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Spinal cystic dysraphisms are typically cerebrospinal fluid cystic masses with or without neural tissues, connected to the spinal cord with pedunculated or sessile stalk.
Based on the contents of the cyst, they are classified into three types; myelomeningocele, myelocystocele, and meningocele.1–10
In myelomeningocele the corresponding cyst that contains neural elements protrudes posteriorly in cervical region The dome of a myelomeningocele is made of squamous or cicatricial epithelium, but the base of the dome is usually covered with full-thickness skin.
Myelocystocele is a rare entity characterized by a cyst, in which distended central canal herniates posteriorly into a meningocele (so-called cyst within a cyst).1,5 This means that the outer cyst is in continuity with the subarachnoid space (meningocele) and the inner cyst is in continuity with a distended central canal. The coverage of the dome in myelocystocele is intact skin.
The third types are meningoceles, which are cavities without neural elements connecting to the spinal cord with a stalk.6,7,9 In meningoceles the coverage of the dome is full-thickness skin.7–9 The meningoceles can be classified into cystic or true meningoceles and solid or rudimentary meningoceles. A true meningocele is a cerebrospinal fluid-containing soft mass connecting to the subarachnoid space via a patent stalk where rudimentary meningocele is a solid mass attached to the spinal cord via a fibrous stalk.6,7,9–12 Rudimentary meningocele is composed of meningocytes and psammoma bodies easily misdiagnosed as skin meningiomas in pre-magnetic resonance imaging (MRI) era.9–12 Both true and rudimentary cervical meningoceles are rare in adults with a few published cases.
The aim of surgery in an asymptomatic adult with such complicated cervical spinal dysraphism is to prevent late onset tethered cord syndrome. In the remaining subjects, cosmetic reasons is the aim of surgical intervention. In symptomatic patients detethering is indicated, which includes removal of the sac, following the stalk to the dura after limited laminectomy, dural opening, intradural exploration, and detethering.6,7,9
Herein, we present an asymptomatic adult with cervical rudimentary meningocele who was surgically treated.
Illustrative Case
A 34-year-old male was admitted for surgical excision of a congenital skin-covered solid mass lesion located in the posterior midline aspect of the neck for cosmetic reasons (Fig. 1). Although the full-thickness skin covered mass was noted at birth, his parents had declined imaging survey and surgical intervention.
Appearance of the large full skin-covered mass at the posterior aspect of the neck.
His neurological evaluation was normal. Lateral cervical spine radiograph showed elongated C4 and C5 spinal processes.
Sagittal T1- and T2-weighted MRI of the cervical spine showed a solid mass at the midline of the posterior aspect of neck with a fibrous stalk extending from the bottom of the sac to the dorsal aspect of the cervical cord through a bifid C4 and C5 laminas. Limited split of the spinal cord and a focal bulge of its posterior half at the site of stalk attachment were demonstrated (Fig. 2).
Preoperative cervical two-dimensional computed tomography (upper) sagittal images show an eye shape hole through which the stalk traverse (white arrow); coronal images show the same (lower).
In a computed tomography scan, the solid mass, spina bifida at the C4–5 levels, and a star-shaped hole for passage of the stalk were demonstrated (Fig. 3). In further imaging survey, there was no evidence of hydrocephalus, craniovertebral junction anomalies or dysraphism in other parts of the spine.
Note the remaining tiny part of the stalk attached to the spinal cord.
With diagnosis of rudimentary meningocele, the patient was taken up for surgery. Under general anesthesia and with the patient in the prone position, the mass was defined, isolated via an elliptical incision, and subsequently excised. Then, paravertebral muscles were stripped off the laminas proximal and distal to spina bifida, followed by C4 and C5 laminectomy. Then, the fibrous core was identified and followed to the center of dura. Subsequently, the dura was incised in small elliptical fashion around the fibrous core. Cutting the stalk close to the spinal cord (detethering), dural, and wound closures were the final steps of surgery (Fig. 3). The postoperative course was uneventful and the patient was discharged a few days later. The histopathologic examination of the mass showed clusters of meningothelial cells and psammoma bodies, confirming its rudimentary nature (Fig. 4).
Pathology of the rudimentary meningocele: (A) Hematoxylin and eosin stain ×100 to higher magnifications: proliferation of meningothelial cells in close proximity of psammoma bodies. (B) Psammoma bodies in higher magnification, white arrows. (C) Immunostaining for epithelial membrane antigen: positive immunostaining reaction of proliferated meningothelial cells shown as dark brown (immunohistochemistry [IHC] stain ×100). (D) Positive immunostaining reaction of proliferated meningothelial cells with progesterone receptor marker (IHC stain ×200).
Discussion
Observations
Cervical meningoceles are mostly discovered as a cystic mass in children,6,7,9 and their presentation as a solid mass with normal skin/labeled as rudimentary is rare, in particular in adults.5–7,9–12
Unlike adults with cervical myelomeningocele who shows variable degree of neurological impact, adults with meningoceles of the same region despite cord tethering may remain asymptomatic throughout the life.1–9 Less frequently the neurological symptoms due to the tethering of the cord may be appear in the third or fourth decade of life, usually mild in comparison to the similar lesions in the lower levels.5–7,9 This discrepancy can be explained by the fact that developmental ascent of the spinal cord relative to the bony spinal column occurs in less intensity in the cervical region than the thoracolumbar and lumbosacral regions.13 As a result, the neurological symptoms are expected to be less pronounced in the cervical spine or even tending to remain occult in adult life.13
A couple of anomalies might be presented in different types of cervical cystic dysraphism including diastematomyelia, Chiai malformation type II, hydromyelia, Klippel Feil syndrome., hydrocephalus, and thin corpus callosum.5–7,9–12
Although surgical intervention is indicated in symptomatic adults,5–7,9 it is controversial in asymptomatic ones. Most authors are in favor of surgery rather than periodic neurological evaluation of the affected patients or for cosmetic reasons.5–7,9
Surgical strategy aims at removal of the mass, excision of the stalk, intradural exploration, and complete detethering for optimal outcome.
Simple surgical excision of the sac and ligation of the stalk without intradural release of the tethering structures are not sufficient in cervical meningoceles.14–18 In such case, late onset neurological symptoms due to the tethering of the spinal cord may be appear in fourth and fifth decade of life.17,18
Undoubtedly, true and rudimentary meningoceles of the cervical regions have a more favorable outcome then myelomeningoceles and meningocystoceles, with respect to the structural and clinical differences exist between these entities.1–4,8
Rarely, an untreated meningocele may become the seat for malignant changes, in particular untreated lumbosacral ones.19,20 Malignant transformation in neglected cervical meningoceles is extremely rare and to the best of our knowledge, only 2 cases have been reported in the English literature to date.19,20
Lessons
Rudimentary cervical meningocele is a rare neural-type defect with abnormal attachment of the neural tube to the skin. Although close follow-up of asymptomatic adults with cervical meningocele may be reasonable, surgical resection of the mass along with spinal cord detethering is highly indicated in symptomatic patients.
Disclosures
The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.
Author Contributions
Conception and design: A Rahimizaideh, Mohammadi Moghaddam, S Rahimizadeh. Acquisition of data: A Rahimizaideh, Ahmadi, S Rahimizadeh, Williamson, Hajialiloo Sami. Analysis and interpretation of data: A Rahimizaideh, S Rahimizadeh, Williamson, Amirzadeh. Drafting the article: A Rahimizaideh, Ahmadi, S Rahimizadeh, Williamson. Critically revising the article: A Rahimizaideh, S Rahimizadeh, Williamson. Reviewed submitted version of manuscript: A Rahimizaideh, S Rahimizadeh, Williamson, Hajialiloo Sami. Approved the final version of the manuscript on behalf of all authors: A Rahimizaideh. Administrative/technical/material support: Ahmadi. Photomicrograph preparation: Ahmadi.
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