What is myeloptosis (Myeloptosis, also known as Myeloschisis)?

From the Guidelines

Myeloptosis, also known as Myeloschisis, is a congenital defect of the spinal cord where the neural tube fails to close completely, resulting in a cleft or split in the spinal cord. This condition can lead to a range of neurological symptoms and complications, including motor and sensory deficits, bladder and bowel dysfunction, and increased risk of infection. The provided evidence does not directly address myeloptosis, but it discusses myelopathy, which is a clinical diagnosis based on signs and symptoms of spinal cord dysfunction 1. Myelopathy can be due to primary intrinsic disorders of the spinal cord, including neoplastic, infectious, inflammatory, neurodegenerative, vascular, nutritional, and idiopathic conditions. Imaging plays a crucial role in refining the differential diagnosis, with MRI being the mainstay in the evaluation of myelopathy due to its superb contrast resolution of the spinal cord 1. Key points to consider in the diagnosis and management of myeloptosis include:

• Congenital defect of the spinal cord
• Neural tube defect
• Cleft or split in the spinal cord
• Neurological symptoms and complications
• Importance of imaging, particularly MRI, in diagnosis and management It is essential to note that the provided evidence does not directly address myeloptosis, and the information provided is based on the available data on myelopathy. However, the most critical aspect of managing myeloptosis is early detection and treatment to prevent or minimize complications and improve quality of life. In clinical practice, a multidisciplinary approach, including neurosurgery, physical medicine, and rehabilitation, is essential for the management of myeloptosis 1.

From the Research

Definition of Myeloptosis (Myeloschisis)

• Myeloschisis, also known as myeloptosis, is a severe form of spina bifida characterized by a protrusion of the meninges and spinal cord through a defect in the vertebral arches 2.
• It is often associated with hydrocephalus, which requires early permanent CSF diversion and revision surgeries 3.
• Myeloschisis is a type of open neural tube defect (oNTD) that can be covered with a thin pseudomembrane, putting patients at significant risk of infections 4.

Characteristics and Treatment

• The management and prevention of myeloschisis-associated hydrocephalus has evolved over time, with treatment modalities including ventriculoperitoneal shunt (VPS) insertion and endoscopic third ventriculostomy (ETV) 2.
• Surgical intervention, such as shunt insertion and closure of the oNTD, is often necessary to manage myeloschisis and prevent further complications 4.
• Early recognition and treatment of myeloschisis are essential for optimal outcomes, and prenatal diagnosis and early corrective surgical intervention are recommended to prevent deterioration in neurological function 5.

Complications and Outcomes

• Shunt infections and malfunctions are common complications in patients with myeloschisis, with a shunt revision rate of 21.1% per person-year and a shunt infection rate of 2.1% per person-year over a mean follow-up of 10.8 years 3.
• Patients with myeloschisis are at risk of developing motor deficits, with a reported incidence of 33.3% in one study 5.
• VACTERL association is a common condition in patients with myeloschisis and should be investigated 5.