Manoj Phalak and Ashok K. Mahapatra

Introduction

Split cord malformation (SCM) is a type of neural tube defect in which the spinal cord is divided into two segments longitudinally. In 1992, Pang et al2^,2 proposed an embryogenic mechanism for this disorder and classified SCM into two types, which were earlier considered as two separate diseases.

Since then, there have been several classifications schemes for SCM.² The importance of these classification schemes comes from the fact that these may be related to the surgical plan as well as the immediate postoperative complications and long-term outcomes.

Surgery remains the mainstay of the treatment for all types of SCM amid some doubts regarding the need for surgery in asymptomatic type II SCM patients. However, with increasing age, the risk of developing neurological deficits increases in previously asymptomatic children.² Thus, surgery must be preferred for asymptomatic children as well. It must be ensured that surgery must not only take care of the fibrous and bony spur but also the associated pathologies like lipoma, teratoma, etc., which may be responsible for tethering.

In fact, on detailed evaluation, many such apparently asymptomatic children demonstrated signs of spinal cord dysfunction on objective testing like urodynamic studies. Unlike other etiologies of spinal cord tethering, there is paucity of literature regarding the long-term outcomes of SCM after surgical correction.

If all the tethering elements are surgically released and associated pathologies are taken care of, the outcomes of surgery for SCM are generally considered to be good. However, Pang et al² observed significant incidence of associated ventral tethering, which must be removed to avoid the need for repeat surgery. MRI is not a sensitive investigation for detecting ventral tethering, and CT myelography is considered to be a better method for the same.²

The long-term outcomes of SCM can be discussed in terms of progression of the symptoms of spinal cord dysfunction and the need for repeat surgery due to retethering or reformation of spur, resulting in the recurrence of the symptoms or deterioration in previously stable neurological deficit.

In most of the studies, improvement or stabilization of symptoms has been reported in >90% of patients.^2,2,2,2

Axial Back Pain

Axial back pain improves or stabilizes in the majority of the patients.^2,2 This improvement may sometimes be confounded by factors like complexity of SCM, which is associated with greater incidence of retethering and progressive orthopedic deformity after surgery.²

Neurological Deficits

More than 90% of those who present with neurological deficits preoperatively either experience complete or partial resolution of symptoms or the symptoms remain stable.2 In the remaining 10% of patients, there is risk of further deterioration. However, no patient who was initially asymptomatic has been reported to suffer neurological deterioration after an uneventful surgery.

Related posts:

Introduction to Split Cord Malformations Split Cord Malformations Type I Anesthesia for Neural Tube Defects Prophylactic Surgery in Split Cord Malformations Protocol for Management of Split Cord Malformations Composite Split Cord Malformations or Multisite Split Cord Malformations

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