What is Diastematomyelia (congenital spinal deformity)?

From the Guidelines

Diastematomyelia is a type of split cord malformation that requires prompt surgical intervention to prevent further neurological deterioration, especially in symptomatic patients, as supported by the description of split cord malformations in the Pediatrics journal 1. This condition is characterized by a split in the spinal cord, which can be divided into two types: Type I, where the two hemicords are contained within separate dural sacs, and Type II, where the two hemicords are within a single dural sac 1. Some key points to consider in the management of diastematomyelia include:

• The presence of associated congenital malformations, such as vertebral malformations, lipomyelomeningocele, and neurenteric cysts 1
• The importance of early diagnosis and intervention to prevent progressive neurological deterioration
• The use of imaging studies, such as MRI and CT scans, to confirm the diagnosis and visualize the bony septum 1
• The role of surgical intervention in removing the septum and reuniting the spinal cord halves to prevent further neurological deterioration
• The need for careful monitoring in asymptomatic patients, with consideration for surgical intervention if symptoms develop In terms of treatment, surgical intervention is generally recommended for symptomatic patients to prevent further neurological deterioration, while asymptomatic patients may be monitored carefully 1. It is essential to consider the individual patient's symptoms, associated anomalies, and overall health when determining the best course of treatment for diastematomyelia.

From the Research

Definition and Classification of Diastematomyelia

• Diastematomyelia is a rare congenital abnormality of the spinal cord, where the spinal cord is split into two hemicords along the sagittal plane 2, 3, 4, 5, 6.
• It is classified into two types: type 1, where there are double dural tubes, and type 2, where there is a single dural tube 2, 5.

Clinical Characteristics and Symptoms

• Diastematomyelia often presents with distinct clinical characteristics, including cutaneous lesions, congenital scoliosis, and musculoskeletal anomalies 2, 3, 4, 5.
• Neurological manifestations, such as pain, numbness, and weakness, are common in patients with diastematomyelia 2, 3, 4, 5.
• Some patients may be asymptomatic, while others may experience progressive neurological deterioration 2, 4, 5.

Diagnosis and Treatment

• Diagnosis of diastematomyelia requires a thorough clinical evaluation, including spinal imaging, such as CT myelography and MRI 2, 3, 4, 5.
• Treatment of diastematomyelia depends on the type and severity of the condition, as well as the presence of neurological symptoms 2, 3, 4, 5.
• Surgical excision of the lesion is often necessary for patients with type 1 diastematomyelia, while conservative treatment may be recommended for asymptomatic patients or those with type 2 diastematomyelia 2, 3, 4, 5.

Prognosis and Outcome

• The prognosis for patients with diastematomyelia varies depending on the severity of the condition and the effectiveness of treatment 2, 3, 4, 5.
• Early recognition and diagnosis of diastematomyelia can help prevent further damage to the spinal cord and improve quality of life 6.
• Patients with diastematomyelia require long-term follow-up to monitor for any changes in their condition and to adjust treatment as needed 2, 3, 4, 5.