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Atlanto-occipital dislocation, orthopedic decapitation, or internal decapitation describes ligamentous separation of the spinal column from the skull base. It is possible for a human to survive such an injury; however, 70% of cases result in immediate
death Death is the end of life; the irreversible cessation of all biological functions that sustain a living organism. Death eventually and inevitably occurs in all organisms. The remains of a former organism normally begin to decompose sh ...
. It should not be confused with atlanto-axial dislocation, which describes ligamentous separation between the first and second cervical
vertebra Each vertebra (: vertebrae) is an irregular bone with a complex structure composed of bone and some hyaline cartilage, that make up the vertebral column or spine, of vertebrates. The proportions of the vertebrae differ according to their spina ...
.


Mechanism

The injury is a result of disruption of the stabilizing ligaments between the
occiput The occipital bone () is a cranial dermal bone and the main bone of the occiput (back and lower part of the skull). It is trapezoidal in shape and curved on itself like a shallow dish. The occipital bone lies over the occipital lobes of the ...
, or posterior skull base, and the C1 vertebral body, otherwise known as the
atlas An atlas is a collection of maps; it is typically a bundle of world map, maps of Earth or of a continent or region of Earth. Advances in astronomy have also resulted in atlases of the celestial sphere or of other planets. Atlases have traditio ...
. The diagnosis is usually suspected by history and physical exam, but confirmed by imaging, typically by CT due to its faster speed in the acute trauma setting, although
MRI Magnetic resonance imaging (MRI) is a medical imaging technique used in radiology to generate pictures of the anatomy and the physiological processes inside the body. MRI scanners use strong magnetic fields, magnetic field gradients, and rad ...
can also help with assessment in equivocal cases. The treatment is initial stabilization with a cervical spine collar, and then surgical intervention in cases in which reversal of paralysis is possible. The most common mechanism of injury is high-speed motor vehicle accidents. The injury is more likely in children due to the large size of their heads relative to their bodies, and more horizontal orientation of the occipital condyles. It represents <1% of all cervical spine injuries. Several subtypes of atlanto-occipital dislocation are known. One suggested categorization scheme includes anterior, vertical and posterior of the head relative to the spine. Other variants with lateral or rotatory displacement have been described, as well as mixed types. Common etiology for such injuries is sudden and severe deceleration leading to a whiplash-like mechanism.


Diagnosis

The distances between the dens and surrounding structures are also key features that can suggest the diagnosis, with the normal distance between the dens and basion (i.e., dens–basion interval; BDI) measuring less than 9 mm on CT, and the distance between the atlas and dens (i.e., atlas–dens interval; ADI) measuring less than 3 mm on CT, although this can be increased in cases of
rheumatoid arthritis Rheumatoid arthritis (RA) is a long-term autoimmune disorder that primarily affects synovial joint, joints. It typically results in warm, swollen, and painful joints. Pain and stiffness often worsen following rest. Most commonly, the wrist and h ...
due to
pannus Pannus is an abnormal layer of fibrovascular tissue or granulation tissue. Common sites for pannus formation include over the cornea, over a joint surface (as seen in rheumatoid arthritis), or on a prosthetic heart valve. Pannus may grow in a t ...
formation. Several indirect measurements on CT can be used to assess ligamentous integrity at the craniocervical junction. The Wackenheim line, a straight line extending along the posterior margin of the clivus through the dens, should not intersect the dens on plain film, with violation of this relationship raising concern for basilar invagination. The
basion The foramen magnum () is a large, oval-shaped opening in the occipital bone of the skull. It is one of the several oval or circular openings (foramina) in the base of the skull. The spinal cord, an extension of the medulla oblongata, passes thro ...
to
axion An axion () is a hypothetical elementary particle originally theorized in 1978 independently by Frank Wilczek and Steven Weinberg as the Goldstone boson of Peccei–Quinn theory, which had been proposed in 1977 to solve the strong CP problem ...
interval, or BAI, is also used, which is determined by measuring the distance between an imaginary vertical line at the anterior skull base, or basion, at the
foramen magnum The foramen magnum () is a large, oval-shaped opening in the occipital bone of the skull. It is one of the several oval or circular openings (foramina) in the base of the skull. The spinal cord, an extension of the medulla oblongata, passes thro ...
, and the axis of the cervical spine along its posterior margin, which should measure 12 mm, an assessment more reliable on
radiograph Radiography is an imaging technique using X-rays, gamma rays, or similar ionizing radiation and non-ionizing radiation to view the internal form of an object. Applications of radiography include medical ("diagnostic" radiography and "therapeu ...
than CT. The distance between the atlas and the occipital condyles, the atlanto-occipital interval (AOI), should measure less than 4 mm, and is better assessed on coronal images. The Powers ratio was formerly used, which was the tip of the basion to the spinolaminar line, divided by the distance from the tip of the opisthion to the midpoint of the posterior aspect of the anterior arch of C1. It is no longer recommended due to low sensitivity and difficulty identifying landmarks. It also will miss vertical or posterior displacement of the cervical spine. Other measurements include occiput-atlas distance, angle between anterior arch of atlas and axis, vertical distance between posterior arch of atlas and spinous process of axis, vertical atlanto-dens interval, and joint space between C1 and C2.


Treatment

Treatment involves fixation of the cervical spine to the skull base, or occipitocervical fusion, using paramedian rods and transpedicular screws with cross-links for stabilization. The patient is subsequently unable to rotate their head in the horizontal plane. If there is obstructive
hydrocephalus Hydrocephalus is a condition in which cerebrospinal fluid (CSF) builds up within the brain, which can cause pressure to increase in the skull. Symptoms may vary according to age. Headaches and double vision are common. Elderly adults with n ...
, a pseudomeningocele can form, which is decompressed at the time of surgery.


Prognosis

The injury is immediately fatal in 70% of cases, with an additional 15% surviving to the emergency room but dying during the subsequent hospital stay. A
basion The foramen magnum () is a large, oval-shaped opening in the occipital bone of the skull. It is one of the several oval or circular openings (foramina) in the base of the skull. The spinal cord, an extension of the medulla oblongata, passes thro ...
- dental interval (BSI) of 16 mm or greater is associated with mortality. In those with neurologic deficits, survival is unlikely. Most deaths result from mechanical damage to the
spinal cord The spinal cord is a long, thin, tubular structure made up of nervous tissue that extends from the medulla oblongata in the lower brainstem to the lumbar region of the vertebral column (backbone) of vertebrate animals. The center of the spinal c ...
and lower
brainstem The brainstem (or brain stem) is the posterior stalk-like part of the brain that connects the cerebrum with the spinal cord. In the human brain the brainstem is composed of the midbrain, the pons, and the medulla oblongata. The midbrain is conti ...
, ranging from localized
contusion A bruise, also known as a contusion, is a type of hematoma of tissue, the most common cause being capillaries damaged by trauma, causing localized bleeding that extravasates into the surrounding interstitial tissues. Most bruises occur clo ...
to
diffuse axonal injury Diffuse axonal injury (DAI) is a brain injury in which scattered lesions occur over a widespread area in white matter tracts as well as grey matter. DAI is one of the most common and devastating types of traumatic brain injury and is a major ca ...
to complete transection. Vascular complications are also frequent and may contribute significantly to delayed mortality. Combined dissections of the
vertebral Each vertebra (: vertebrae) is an irregular bone with a complex structure composed of bone and some hyaline cartilage, that make up the vertebral column or spine, of vertebrates. The proportions of the vertebrae differ according to their spina ...
and
carotid arteries In anatomy, the left and right common carotid arteries (carotids) () are arteries that supply the head and neck with oxygenated blood; they divide in the neck to form the external and internal carotid arteries. Structure The common carotid ...
can lead to severe
cerebral ischemia Brain ischemia is a condition in which there is insufficient bloodflow to the brain to meet metabolic demand. This leads to poor oxygen supply in the brain and may be temporary such as in transient ischemic attack or permanent in which there is ...
, whereas rupture of the vertebral artery– PICA junction results in
subarachnoid hemorrhage Subarachnoid hemorrhage (SAH) is bleeding into the subarachnoid space—the area between the arachnoid (brain), arachnoid membrane and the pia mater surrounding the human brain, brain. Symptoms may include a thunderclap headache, severe heada ...
, compressing the brainstem. Hydrocephalus may also develop and cause a dangerous increase of
intracranial pressure Intracranial pressure (ICP) is the pressure exerted by fluids such as cerebrospinal fluid (CSF) inside the skull and on the brain tissue. ICP is measured in millimeters of mercury ( mmHg) and at rest, is normally 7–15 mmHg for a supine adu ...
. Further possible complications include damage to prevertebral structures (
pharynx The pharynx (: pharynges) is the part of the throat behind the human mouth, mouth and nasal cavity, and above the esophagus and trachea (the tubes going down to the stomach and the lungs respectively). It is found in vertebrates and invertebrates ...
, lower cranial nerves) and the
cerebellum The cerebellum (: cerebella or cerebellums; Latin for 'little brain') is a major feature of the hindbrain of all vertebrates. Although usually smaller than the cerebrum, in some animals such as the mormyrid fishes it may be as large as it or eve ...
. Therefore, initial survivors of atlantooccipital dislocation may show severe and variable neurologic deficits, including reversible or irreversible
tetraplegia Tetraplegia, also known as quadriplegia, is defined as the dysfunction or loss of motor and/or sensory function in the cervical area of the spinal cord. A loss of motor function can present as either weakness or paralysis leading to partial ...
, multiple
cranial nerve Cranial nerves are the nerves that emerge directly from the brain (including the brainstem), of which there are conventionally considered twelve pairs. Cranial nerves relay information between the brain and parts of the body, primarily to and f ...
deficits, loss of consciousness, and recurrent
respiratory The respiratory system (also respiratory apparatus, ventilatory system) is a biological system consisting of specific organs and structures used for gas exchange in animals and plants. The anatomy and physiology that make this happen varies gr ...
and/or
cardiac arrest Cardiac arrest (also known as sudden cardiac arrest CA is when the heart suddenly and unexpectedly stops beating. When the heart stops beating, blood cannot properly Circulatory system, circulate around the body and the blood flow to the ...
s. Children are more likely to survive with neurologic compromise than adults. Isolated cases of near-complete recovery after life-threatening symptoms are known. In case of posterior displacement of the head, a concomitant fracture of the
atlas An atlas is a collection of maps; it is typically a bundle of world map, maps of Earth or of a continent or region of Earth. Advances in astronomy have also resulted in atlases of the celestial sphere or of other planets. Atlases have traditio ...
with backward migration of the
posterior arch In anatomy, the atlas (C1) is the most superior (first) cervical vertebra of the spine and is located in the neck. The bone is named for Atlas of Greek mythology, just as Atlas bore the weight of the heavens, the first cervical vertebra suppor ...
is associated with improved chance of survival, as this allows the spinal cord and
medulla Medulla (Latin for "marrow") or medullary may refer to: Science * Medulla oblongata, a part of the brain stem * Renal medulla, a part of the kidney * Adrenal medulla, a part of the adrenal gland * Medulla of ovary, a stroma in the center of the ...
to migrate backward without getting crushed.


Proximal cervical fractures associated with injury

The Jefferson fracture can be associated with this injury, with the C1 ring, or atlas, being fractured in several places, allowing the spine to shift forward relative to the skull base. The
Hangman's fracture Hangman's fracture is the colloquial name given to a fracture of both Pedicle of vertebral arch, pedicles, or ''pars interarticularis, partes interarticulares'', of the ''axis vertebra'' (C2 vertebra, C2). Causes The injury mainly occurs from fal ...
which is a fracture of the C2 vertebral body or dens of the cervical spine upon which the skull base sits to allow the head to rotate, can also be associated with atlanto-occipital dislocation. Despite its eponym, the fracture is not usually associated with a hanging mechanism of injury.


References

{{DEFAULTSORT:Internal Decapitation Medical emergencies Injuries of neck