Seven distinct coexistent cranial and spinal anomalies
Hakan Emmez1, Nil Tokgöz, Fikret Dogulu
1Department of Neurosurgery, Gazi University School of Medicine, Ankara, Turkey. hakanemmez@hotmail.com
Pediatric Neurosurgery
|August 12, 2006
Summary
This case report details an extremely rare instance of seven severe craniospinal anomalies in a one-year-old with spina bifida. Early diagnosis and intervention are crucial for managing this life-threatening condition.
Area of Science:
- Pediatric Neurology
- Developmental Biology
- Congenital Malformations
Background:
- Spina bifida is a complex birth defect involving the spine and spinal cord.
- Multiple cranial and spinal anomalies are often associated with spina bifida, but the extent can vary significantly.
Observation:
- A one-year-old girl presented with hydrocephalus, Chiari malformation, syringohydromyelia, split cord malformation, dermal sinus tract, lumbosacral myelomeningocele, and tethered cord.
- The patient exhibited an enlarged head and an infected myelomeningocele, leading to a comatose state upon admission.
Findings:
- The coexistence of seven distinct severe craniospinal anomalies in a single patient is exceptionally rare.
- The patient's presentation was complicated by meningitis and sepsis, ultimately leading to mortality.
Implications:
- Early and comprehensive diagnostic imaging, such as magnetic resonance imaging, is vital for identifying associated anomalies in spina bifida.
- Timely surgical interventions, including shunt placement and myelomeningocele repair, are critical for improving outcomes and preventing fatal complications.
Related Concept Videos
01:19Cranial 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...
01:09Overview of the Axial Skeleton
The skeleton is subdivided into two major divisions—the axial skeleton and the appendicular skeleton. The axial skeleton forms the vertical, central axis of the body. It includes all of the bones of the head, neck, chest, and back. It protects the brain, spinal cord, heart, and lungs. It also serves as the attachment site for muscles that move the head, neck, and back and for muscles that act across the shoulder and hip joints to move their corresponding limbs.
The axial skeleton of the adult...
The axial skeleton of the adult...
01:14Cranial Bones: Superior and Posterior View
The superior view of the cranium shows the frontal and paired parietal bones.
The frontal bone is the single bone that forms the forehead. At its anterior midline, between the eyebrows, there is a slight depression called the glabella. The frontal bone also forms the supraorbital margin of the orbit. Near the middle of this margin is the supraorbital foramen, the opening that provides passage for a sensory nerve to the forehead. The frontal bone is thickened just above each supraorbital margin,...
The frontal bone is the single bone that forms the forehead. At its anterior midline, between the eyebrows, there is a slight depression called the glabella. The frontal bone also forms the supraorbital margin of the orbit. Near the middle of this margin is the supraorbital foramen, the opening that provides passage for a sensory nerve to the forehead. The frontal bone is thickened just above each supraorbital margin,...
01:22Sutures of the Skull
The human skull is composed of several bones that come together to protect the brain and support the structures of the face. The junctions where these bones meet are called sutures.
Sutures are immobile joints between adjacent bones of the skull. The narrow gap between the bones is filled with dense, fibrous connective tissue that unites the bones. The long sutures located between the skull bones are not straight but instead follow irregular, tightly twisting paths. These twisting lines tightly...
Sutures are immobile joints between adjacent bones of the skull. The narrow gap between the bones is filled with dense, fibrous connective tissue that unites the bones. The long sutures located between the skull bones are not straight but instead follow irregular, tightly twisting paths. These twisting lines tightly...
01:54The Spinal Cord
The spinal cord is the body’s major nerve tract of the central nervous system, communicating afferent sensory information from the periphery to the brain and efferent motor information from the brain to the body. The human spinal cord extends from the hole at the base of the skull, or foramen magnum, to the level of the first or second lumbar vertebra.
01:23Spinal Nerves: Anatomy
Spinal nerves are pivotal conduits in the nervous system, bridging the central nervous system (CNS) with the peripheral nervous system (PNS). These nerves enable a complex communication network between the brain, spinal cord, and the rest of the body, facilitating sensory input, motor output, and autonomic functions.
There are 31 bilateral pairs of spinal nerves, each emerging from the spinal cord through the intervertebral foramina—openings between adjacent vertebrae. These nerves are...
There are 31 bilateral pairs of spinal nerves, each emerging from the spinal cord through the intervertebral foramina—openings between adjacent vertebrae. These nerves are...