Hemimyelomeningocele: a rare and complex spinal dysraphism
L Jans1, P Vlummens, S Van Damme
1Department of Radiology and Medical Imaging, Ghent University Hospital, De Pintelaan 185, 9000 Gent, Belgium. lennartjans@hotmail.com
Summary
This study details a rare case of hemimyelomeningocele, a spinal dysraphism combining split cord malformation with myelomeningocele. Early diagnosis via MRI is crucial for effective neurosurgical treatment of this complex condition.
Area of Science:
- Neurology
- Developmental Biology
- Neurosurgery
Background:
- Spinal dysraphisms represent a spectrum of congenital anomalies affecting neural tube closure.
- Hemimyelomeningocele is an exceptionally rare variant, characterized by the coexistence of split cord malformation and myelomeningocele.
- Accurate diagnostic imaging is paramount for guiding appropriate clinical management.
Observation:
- A case of hemimyelomeningocele is presented, highlighting its unique anatomical features.
- Magnetic Resonance Imaging (MRI) revealed a divided spinal cord into two hemicords, separated by an osseous spur.
- The left hemicord was identified as forming the myelomeningocele component, defining the hemimyelomeningocele.
Findings:
- The MRI findings precisely delineated the complex anatomy of hemimyelomeningocele.
- This case underscores the specific presentation of split cord malformation associated with myelomeningocele.
- The bony spur's role in separating the hemicords was clearly visualized.
Implications:
- Early and precise diagnosis of spinal dysraphisms like hemimyelomeningocele is critical.
- Individualized neurosurgical strategies can be optimized based on accurate diagnostic information.
- Understanding rare spinal malformations advances the field of pediatric neurosurgery and developmental neurology.
Related Concept Videos
Herniated Intervertebral Disc l: Introduction
Intervertebral disc herniation refers to the displacement of the nucleus pulposus (the gel-like inner core of the disc) through a tear or weakened area in the annulus fibrosus (the outer fibrous ring). The displaced disc material extends beyond the normal boundaries of the disc space and may compress or irritate nearby spinal nerve roots or, less commonly, the spinal cord.Etiology and Risk FactorsHerniation commonly results from degeneration, in which aging reduces disc hydration and...
Neurulation
Neurulation is the embryological process which forms the precursors of the central nervous system and occurs after gastrulation has established the three primary cell layers of the embryo: ectoderm, mesoderm, and endoderm. In humans, the majority of this system is formed via primary neurulation, in which the central portion of the ectoderm—originally appearing as a flat sheet of cells—folds upwards and inwards, sealing off to form a hollow neural tube. As development proceeds, the anterior...
Hiatal Hernia
A hiatal hernia is the abnormal protrusion of the stomach or other abdominal organs through the esophageal hiatus of the diaphragm into the thoracic cavity.Normally, the gastroesophageal junction (GEJ) lies below the diaphragm and is supported by the phrenoesophageal membrane, the diaphragmatic crura, and connective tissues. Weakening of these structures—due to aging, congenital defects like a short esophagus, or increased intra-abdominal pressure from coughing, obesity, pregnancy, or heavy...
Cranial and Spinal Meninges
The cranial and spinal meninges are complex protective structures surrounding the central nervous system (CNS), consisting of the brain and spinal cord. These meninges consist of the dura mater, the arachnoid mater, and the pia mater. They protect the CNS, provide structural support, and aid in circulating cerebrospinal fluid (CSF).
Cranial Meninges
These meningeal layers cover the cranium. The dura mater is the outermost layer of cranial meninges. It is a thick and durable membrane of dense...
Cranial Meninges
These meningeal layers cover the cranium. The dura mater is the outermost layer of cranial meninges. It is a thick and durable membrane of dense...
Meiosis I
Meiosis is a carefully orchestrated set of cell divisions, the goal of which—in humans—is to produce haploid sperm or eggs, each containing half the number of chromosomes present in somatic cells elsewhere in the body. Meiosis I is the first such division, and involves several key steps, among them: condensation of replicated chromosomes in diploid cells; the pairing of homologous chromosomes and their exchange of information; and finally, the separation of homologous chromosomes by a...
Spinal Cord: Gross Anatomy
The spinal cord resides within the protective confines of the vertebral column. It is the main pathway for information traveling between the brain and the body. It plays a fundamental role in nearly all bodily functions, from simple reflexes to complex motor movements. The spinal cord begins at the medulla oblongata at the base of the brainstem and extends downward, terminating at the conus medullaris near the first and second lumbar vertebrae. The spinal cord's length in adults is...
