What Is Craniostenosis?

Cranial stenosis is the premature closure of one or more cranial sutures, which results in cranial deformities, increased intracranial pressure, mental retardation, and ocular symptoms. It is clinically characterized by premature closure of single or multiple skull sutures.

Basic Information

Visiting department

neurosurgery

Common locations

skull

Common causes

Unknown cause

Common symptoms

Mainly manifested as various deformed skull deformities and brain development disorders

Contagious

no

Causes of cranial stenosis

The cause is unknown.

Clinical manifestations of cranial stenosis

The clinical manifestations of cranial stenosis are mainly cranial deformities of various shapes. Due to the premature closure of the cranial suture, the growth of the skull is restricted, hindering the development of the brain, which results in increased intracranial pressure. Patients may have prominent two eyes, lower vision, eye movement disorders, disc edema or secondary atrophy, visual impairment or blindness. Some patients may have mental retardation, and symptoms such as headache, nausea, and vomiting may occur in the later stages. Some patients may experience seizures due to atrophy of the cerebral cortex. The clinical manifestations of cranial stenosis can be divided into two categories: head deformities and secondary symptoms.

Tip deformity

It is also called tower-shaped skull, which is more common. It is caused by the premature closure of all cranial sutures. Because the growth of the skull is restricted in all directions except for the resistance of the anterior cardia, the skull grows upward in a tower shape. The skull base is compressed and sunken, the eye sockets become shallow, the eyeballs are protruding, and the paranasal sinuses are not well developed. Because the brain tissue extends vertically, the upper and lower diameters of the skull increase, the anterior and posterior diameters become shorter, the anterior cranial fossa can be shortened to 1.33cm, the optic nerve foramen becomes smaller, the supraorbital fissure is shortened, the cerebral gyrus pressure is significantly increased, and the sphenoidal is enlarged. Delayed forehead closure. Pointed deformity of the frontal bone retracts or spins, so that the frontal bone and the nasal spine are in a line, the frontal nose angle disappears. The typical case is a cranial apical process. Frontal spinal rotation is the main cause of skull deformity. The middle of the face can be normal. It is worth pointing out that apical deformities do not show obvious clinical manifestations before the age of 2 to 3 years, because many cases have normal skulls at the age of 1 and typical apical deformities only appear at the age of 4 years. True acne deformity with hand or foot and finger / toe deformities is called Saethre-Chotzen syndrome. Fatty cartilage dysplasia manifests as cartilage dysplasia, optic nerve atrophy, wide and flat head and nose, and thick lips. It also belongs to the category of apical deformities. It is common in infants and young children with shortened arms and lower limbs accompanied by mental retardation and visual impairment. The cornea has lipid deposits.

2. Scaphoid deformity

Also known as long head deformity, caused by early closure of sagittal suture alone, it is the most common skull deformity in early closure of cranial suture. The sagittal suture is closed prematurely, and the head is laterally restricted, that is, it expands forward and backward. As a result, the cranial vault is elongated from front to back and narrowed from left to right, which causes the saddle deformity of the skull and excessive bulging of the pillow and forehead. The frontal position can be very high, forming a piriform forehead due to intertemporal fossa stenosis. The scaphoid deformity caused by early closure of the sagittal suture is mostly male, with a male to female ratio of 4: 1. Occasional family history.

3. Triangular head deformity

This type is rare and is caused by the early closure of the front seam, but some front seams are still open. It is characterized by the frontal sides of the frontal scales protruding forward at the frontal suture, with acute angles, and the head is triangular when viewed from above. The frontal bone is short and narrow, the anterior cranial fossa becomes smaller and shallower, and the eyes are too close. There is epiphyseal thickening at the forehead suture, often with other malformations.

4. Misalignment

Also known as migraine, is unilateral hypoplasia of the frontal bone caused by unilateral coronal suture ossification. The bilateral growth of the skull is asymmetric, the frontal bone of the affected side is flat and retracted, and the upper edge of the orbit is raised and retracted. The diseased side affects the development of brain tissue, the anterior condyle still exists, but it is biased towards the healthy side. Premature closure of the callus can be reached in the middle of the forehead. The asymmetry of the frontal bone affects the entire cranial vault shape, the sagittal suture is deviated to the disease side, and the frontal and parietal bones on the healthy side are overswelled. Ossification of unilateral coronal sutures can penetrate to the wing points and skull base. Therefore, almost all oblique head deformities are accompanied by facial asymmetry deformities, which increase with age. The distance between the eyes becomes smaller and the forehead becomes narrower. The auricle and external ear canal can also be asymmetric, but most of them are not obvious, and the orbital nose deformities are more prominent. Oblique head deformities are often associated with mental retardation, cleft palate, cleft lip deformities, urinary system deformities, and total forebrain deformities.

5. Short head deformity

Caused by premature ossification of the coronary sutures on both sides. The forehead is symmetrical and flat after the coronary suture is closed on both sides, so it is also called flat head deformity or wide head deformity. The ossification of the coronal sutures on both sides of the patient's skull caused dysplasia of the anterior and posterior diameter of the skull, compensatory transverse diameter widening, and elevation of the cranial crest, so the manifestation was widening of the skull, flat forehead, enlarged middle fossa, shallower orbit, orbit The tadpole is stunted and the eyeballs are prominent, like "goldfish eyes".

Patients can have obvious deformities within a few weeks after birth. The upper half of the frontal bone is high and wide, the lower half is retracted, flat, and sometimes sunken. The upper and upper frontal bones are often spherical protrusions in the surface structure. Upper; the lower half retracts, pulling the nasal bone to the rear, causing the bridge of the nose to sink. The nasopharyngeal cavity becomes smaller, sometimes the skull base and hard palate are often deformed, and children often have repeated upper respiratory tract infections. Ossified coronal sutures touch rosary bone nodules.

6. Crouzon stenosis

Also known as Crouzon craniofacial bone dysplasia or Crouzon type craniofacial stenosis, the main characteristics are:

(1) Huge skull cover and early closure of cranial sutures. Coronal sutures and herringbone sutures are the most common. Early anterior cardial ossification causes the cranial apex to bulge.

(2) The normal mandible is relatively prominent compared with the small maxilla, and the nasal and maxillofacial region of the face is retracted, causing the occlusion to reverse, and to a certain extent, a false convex jaw deformity is formed.

(3) Excessive protrusion of the nose is a hawk-nosed nose, the orbital wall is pushed forward, the upper orbital edge is retracted due to short head deformity, and the lower orbital edge is also retracted due to jaw retraction, resulting in extremely prominent eyeballs. The upper eye socket widens to form the frog eye of Crouzon's disease. Patients may be accompanied by eye movement paralysis.

(4) Most have hereditary and family history, also known as hereditary head and face bone development disorders.

(5) Cranial stenosis may include increased intracranial pressure, vision loss, and mental retardation.

7.Apert craniofacial stenosis

Presented as acrocephalic and a finger / toe malformation syndrome. The facial deformity is more obvious, especially the receding of the maxilla. The retracted forehead with horizontal rotation above it makes the root of the nose deeply recessed under the arch of the eyebrow, causing the occlusion to open and the mouth to open. The middle of the open and upper lip seemed to be pulled backwards. Eyeballs are not prominent, and there is often exotropia. Facial deformities are apparent at birth.

Cranial stenosis

Tip deformity

X-ray skull shows anterior orbital medial oblique, anterior cranial fossa narrowed, bone density increased along the coronal sutures, calcium deposits, and often cranial finger pressure notches. In the lateral radiograph, the frontal bone was rotated posteriorly, and the bone process behind the frontal bone showed no X-ray protrusion shadow, and the posterior cranial vault was normal.

2. Scaphoid deformity

Skull deformities can be seen on the plain radiograph of the skull. The bone density along the sagittal sutures is increased, and the calcium deposits are severe. In severe cases, the bone sutures cannot be seen, and the coronary sutures, herringbone sutures, and scaly sutures are widened or even separated. Increased intracranial pressure showed increased cerebral backpressure traces.

3. Triangular head deformity

The X-ray showed the frontal bone was short and highly protruding, and the orthophoto showed typical orbital shortness and vertical orbital wall.

4. Misalignment

The X-ray showed oblique head deformity, that is, the posterior orbital long axis was deviated outward. In the skull image, the bone density increased at the coronal suture on the side, and the near-wing point was higher. The anterior cranial fossa on the affected side also became smaller, and the anterior cranial fossa became steeper. The nose cone is skewed and the epistaxis is biased towards the side of the lesion.

5. Short head deformity

It can be seen that the bone density at the coronal sutures on both sides increased, the skull base changed to shorten the anterior cranial fossa and erect, the winglets of the sphenoid bone were raised high and deflected backwards, and the pterygoids were raised to deepen the temporal socket. The orbital volume becomes smaller.

Cranial stenosis diagnosis

For those with typical manifestations of head malformations, diagnosis is not difficult. However, when a skull deformity is found after birth, it is often misdiagnosed as a result of childbirth. If the skull deformity does not disappear in a certain period after birth, a craniocerebral plain radiograph should be performed. Mainly manifested as increased density of the skull suture, calcium deposits, and sometimes signs of increased intracranial pressure such as increased cerebral backpressure traces and decalcification of the posterior bed.

Cranial stenosis treatment

Surgical treatment is the only effective method. The main purpose of the surgery is to re-establish a new sulcus through bone suture reconstruction or craniotomy, so that the cranial cavity is enlarged to ensure the normal development of the brain.

The earlier the operation time, the better. Surgery should be performed within 7 months after birth, and the prognosis is good. The later the operation, the worse the effect. It is generally believed that if the child's body allows it, surgery should be performed as soon as possible after birth to release the narrowed cranial cavity as soon as possible to facilitate the development of brain tissue. If only 1 to 2 craniosynostosis is ossified, surgery can be performed 4 to 6 weeks after birth. If multiple craniosynthesis is ossified and intracranial pressure is increased, surgery should be performed at 1 week after birth to be successful.