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Intramedullary lesions of the conus medullaris: differential diagnosis and surgical management

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Abstract

The medullary conus represents a distinct entity of the spinal cord regarding its anatomical, clinical and microsurgical features. An overview of the pathologic processes of this region is provided. Epidemiological, clinical and neuroradiological characteristics of neoplastic (glial tumors, non-glial tumors, metastasis, primary melanomas) and non-neoplastic lesions (granulomatous lesions, abscess, parasitic infections, vascular, demyelinating and dysembryogenetic lesions) are discussed. Main MR imaging characteristics used to differentiate neoplastic from non-neoplastic lesions consist in pathological spinal cord expansion, gadolinium-enhancement and tumoural cyst formation. Management strategies differ substantially, depending on the kind of lesion. According to the suspected pathological entity radical resection, biopsy or conservative treatments are reasonable options. Intraoperative electrophysiological monitoring is a fundamental part of the surgical setting.

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Authors and Affiliations

  1. Department of Neurosurgery, Eberhard-Karls-University, Tübingen, Germany

    Florian H. Ebner, Florian Roser, Marcus A. Acioly & Marcos Tatagiba

  2. Department of Neuroradiology, Eberhard-Karls-University, Tübingen, Germany

    Wolfgang Schoeber

  3. Department of Neurosurgery, University Hospital Tuebingen, Hoppe-Seyler-Straße, 72076, Tübingen, Germany

    Florian H. Ebner

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  1. Florian H. Ebner
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  2. Florian Roser
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Correspondence to Florian H. Ebner.

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Comments

Dattatraya Muzumdar, Mumbai, India

Ebner et al. have written a concise review of the anatomical, pathological, and surgical aspects of intramedullary tumors of the conus medullaris. They have discussed the radiological features differentiating various types of common and uncommon intramedullary lesions encountered in the conus medullaris. They also discuss the surgical strategy and nuances in a lucid manner.

Conus medullaris is the terminal part of the spinal cord which comprises the lumbosacral segments concerned with the muscle power and sensations in lower limbs and control of sphincteric functions. Although the cross section of the conus medullaris is comparatively small, it is responsible for crucial functions of the human body.

The commonest intramedullary lesions are ependymomas, astrocytomas, and epidermoid or dermoid cysts. The clinical presentation of intramedullary conus lesions is innocuous in the early phase of the disease and can become widespread later. The radiological diagnosis of conus lesions in differentiating benign versus malignant pathology has become more precise with the availability of advanced MR imaging sequences and facilitates early treatment decision-making. Tuberculomas and cysticercosis are seen fairly common in endemic regions and form an important differential diagnosis. In certain selected cases viz. cysticercosis and tuberculomas, the diagnosis is established with reasonable certainty on MR imaging. In such situations, surgery can be completely avoided and resolution of the disease can be attained on appropriate medical treatment.

Complete excision of a myxopapillary ependymoma should be attempted for a possible cure while only a biopsy or conservative resection may be possible in an astrocytoma. Radiology is helpful to determine the extent of surgical resection to be performed. Perhaps the most important aspect of conus lesions is to weigh the benefits of surgical treatment versus the natural history of the disease. Surgery is necessary for establishing the diagnosis and is gratifying if there is a good plane of cleavage found between the lesion and the vital cord tissue allowing complete extirpation of the lesion. Any additional neurological deficit should not be inflicted since the damage to vital sphincter centers could be irreversible. The intraoperative judgment on the part of the neurosurgeon is crucial for optimal surgical outcome. In certain cases, only a biopsy or conservative resection is possible and should be performed. Overzealous attitude has the potential to do more harm than good. Intraoperative monitoring techniques viz. MEP/SSEP, urodynamics is a useful guide during surgery and helps to minimize surgery-related morbidity and preserve neurological function. The preoperative status of the patient is an important determinant of the long-term outcome and prognostication.

Niklaus, Krayenbühl, Zurich, Switzerland

Ebner and colleagues review the literature of intramedullary lesions of the conus medullaris. They briefly summarize the anatomical and clinical aspects of this region and then present the radiological and clinical findings as well as therapeutic options of the whole variety of pathologies occurring in this area. This comprises neoplastic lesions of glial or non-glial origin, metastatic tumors, non-neoplastic lesions like granulomatous diseases, bacterial and parasitic infections as well as vascular, demyelinating and dysembryogenetic lesions. The authors also summarize the current surgical technique, stressing the importance of intraoperative monitoring.

This is a very valuable review, especially for young neurosurgeons, that offers a good overview over the different pathologies of the conus medullaris including their surgical treatment.

Jörg Klekamp, Quakenbrück, Germany

Ebner et al. provide an overview on the differential diagnosis of intramedullary lesions of the conus medullaris, their clinical presentation and imaging features. The intraoperative technique of dissection is described and the importance of intraoperative monitoring of neurological functions is stressed. A few points, however, I would like to add:

Basically, any intramedullary lesion described in this article may be encountered anywhere else in the spinal cord. Therefore, similar criteria for the differential diagnosis or the surgical technique apply for lesions in the conus as for other parts of the spinal cord.

From my experience, however, there is a big difference in terms of surgical morbidity. Surgical morbidity for conus tumors is considerably higher compared to lesions of the cervical cord, for instance, even with intraoperative monitoring. Especially for sphincter and sexual functions, this has to be born in mind. Unlike for postoperative problems of motor power or limb coordination, which carry a good prognosis for recovery with appropriate rehabilitation, sphincter disturbances and sexual dysfunctions hardly ever recover. Whenever the lesion to be operated does not reach the pial surface of the conus, surgery carries considerable risks in this respect.

The authors have mentioned a couple of entities that may look like neoplasms but resemble inflammatory or demyelinating lesions. Given the risks of permanent morbidity with surgical removal one should obvioulsy avoid operations for such lesions. As the authors mention, lack of a space occupying effect should make any neurosurgeon sceptical as to the necessity of an operation for a conus lesion. A second point, however, should be born in mind. Intramedullary neoplasms typically cause a slowly, steadily progressive clinical course over months or even years. The only exceptions are malignant tumors or those associated with intramedullary hermorrhages – both easily differentiated from a demyelinating or inflammatory lesion. The latter typically cause rapid neurological deteriorations with periods of intermittent recovery – something never to be seen with an intramedullary neoplasm. In cases of doubt, it is a wise decision to wait for a few weeks and perform a second MRI scan. If the lesion demonstrates an altered signals pattern, a neoplasm can be considered excluded.

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Ebner, F.H., Roser, F., Acioly, M.A. et al. Intramedullary lesions of the conus medullaris: differential diagnosis and surgical management. Neurosurg Rev 32, 287–301 (2009). https://doi.org/10.1007/s10143-008-0173-1

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