Craniosclerosis

Introduction to Cranial Narrow Cranial stenosis is a premature closure of one or more skull joints leading to head deformity, increased intracranial pressure, intelligent developmental disorders, and ocular symptoms. It is characterized by premature closure of single or multiple skull sutures. . basic knowledge The proportion of illness: 0.005% Susceptible people: no special people Mode of infection: non-infectious Complications: cleft lip, cleft palate, spina bifida, congenital heart disease, acute pulmonary edema, meningitis, osteomyelitis, epilepsy

Cause of cranial stenosis

(1) Causes of the disease

So far, the cause of this disease is unknown, and there is no satisfactory explanation. Some scholars have found that the disease is familial, so it is believed that the disease is related to heredity. The lesions are mostly concentrated in the coronal suture or multiple bone sutures. The reason for the unknown is that the cranial ossification that occurs at birth is called primary narrow cranial disease, and the early ossification of the cranial suture secondary to other diseases of the body is called secondary narrow cranial disease, such as excessive Early cranial ossification of patients with cretinism using thyroid hormone replacement therapy.

As early as 1975, Cohen and Converse et al. in 1976 elaborated on the etiology of the narrow cranial disease. The disease is considered to be a congenital malformation, but in general the cause is still unclear, possibly with the embryonic mid-embryo Developmental disorders may also be caused by the occurrence of heterotopic ossification centers in the osseous membrane tissue, and may also be related to the lack of certain matrix in the embryo. A few cases have genetic factors, and individual cases may be due to vitamin D deficiency and hyperthyroidism. To the point that Park and Power have suggested that the basic cause of the skull interstitial beam is incomplete, resulting in a reduction in the skull and premature ossification of the suture tissue.

The starting point and ossification of the cranial suture ossification, the corresponding cranio-sacral humerus, the effect of the skull base and the dura mater, etc. These problems are still not very clear, the development of the skull base is accompanied by the change of facial deformity The pathogenesis of cranial disease plays a leading role.

(two) pathogenesis

The skull consists of multiple skulls such as the frontal bone, the parietal bone, the tibia, and the sphenoid bone. Each skull is separated at the time of birth. There is a fiber commissure between the bone and the bone. It is called a suture. During normal development, the skull is in the process of normal development. The two must be fused together, and gradually grow. The harmonious balance between the two develops until the volume of the cranial cavity is basically fixed in adolescence, and then the ossification of the skull is combined. After the age of 30, the cranial suture is completely ossified, if some factors affect it. When the ossification of the cranial suture is carried out, the lesion can occur. The premature ossification of a cranial suture or multiple cranial sutures affects the development of the cranial cavity, while the brain tissue continues to grow and develop, resulting in a compensatory increase in the skull. Various skull deformities are formed.

In the first year of life, the brain grows at a high speed, which dominates the development of the frontal surface. The newborn grows fastest in the first year after birth, the brain volume increases by 1 time, the length increases by 4 cm, and the brain weight increases by 85% in the first half of life. In 1 year, the head circumference can complete 50% of the total increase, and in the first half of the month, the volume of the infant frontal leaves has reached 47%.

As the brain tissue grows and grows, the skull grows accordingly. The brain is wrapped by the dura mater attached to the cerebral palsy at the base of the skull. During the development process, the plastic skull of the baby plays a strong thrust from the inside to the outside, which dominates the skull. In the development of infants and young children, if one or several cranial sutures are closed prematurely, it will affect the growth and expansion of the skull, but the brain will continue to grow, and the compensatory expansion of the weak skull will be limited, that is, the skull will appear. The internal pressure is increased, which seriously affects the normal development of brain tissue and causes various brain dysfunction.

Cranial stenosis prevention

There is no effective preventive measure for this disease. Early detection and early diagnosis are the key to prevention.

Cranial stenosis Complications cleft lip and palate, spina bifida, congenital heart disease, acute pulmonary edema, meningitis, osteomyelitis, epilepsy

Narrow cranial disease often involves malformations in other parts of the body. The most common are symmetry and finger-to-toe deformity, as well as cleft lip, cleft palate, facial bone deformity, nasal bone collapse, spina bifida, sphenoid winglet overgrowth, posterior nostril atresia and nasal Pharyngeal obstruction, increased hard palate, congenital heart disease and abnormal genital tract.

Complications of surgical treatment of narrow cranial disease can be divided into two categories: intraoperative and postoperative complications:

Intraoperative complications

(1) sinus rupture bleeding: the incidence rate is 5.1%, often the superior sagittal sinus (1.9%), transverse sinus (1.3%) and skull sinus sinus (1.9%), the upper sagittal sinus rupture can cause a large number Loss of blood and circulatory system dysfunction, if it can be quickly recovered successfully, without any sequelae, sinus rupture often caused by tearing of the dura mater or separation of the bone flap, can be used for simple suture repair, if it is a skull barrier Venous hemorrhage can be stopped by bone wax.

(2) Dural injury: the majority of the dura mater is caused by the insertion of the dura mater into the skull. The incidence rate is 70%. It can be repaired by suture, and the dural laceration is rare. 4% need Periosteal patching.

(3) subdural hematoma: the incidence rate is 1.3%, which is caused by cortical dural venous rupture during intraoperative stripping of the dura mater. It is often a small hematoma of the frontal lobe, which can be excluded by dural opening.

(4) cerebral edema: caused by ventilatory disorders, often affecting the exposure of the skull base, the cerebral edema disappears after the ventilatory disorder is removed, and generally no postoperative sequelae.

2. Postoperative complications

(1) Epidural hematoma: The incidence rate is 1.9%, and its clinical manifestations are atypical. It is difficult to make a diagnosis. Therefore, if there are abnormal symptoms and signs in the early postoperative period, CT scan should be performed without hesitation.

(2) Failure of resuscitation: blood loss may continue during the whole operation, such as major bleeding during surgery, which is fatal to infants, often suffers from dyspnea, acute pulmonary edema and death, the incidence rate is 1.3%.

(3) Infection: postoperative infections include wound infection, meningitis and osteomyelitis. Incision infection is characterized by incision redness and swelling, no fever, and there is no major change in general. After local treatment and systemic medication, it can be generally controlled. In osteomyelitis, if the local drainage is ineffective, all infected bone flaps must be removed. Individual patients may develop meningitis, which is often life-threatening.

(4) cerebrospinal fluid rhinorrhea: the incidence rate is 1.9%, often occurs after craniocerebral stenosis, often complicated by meningitis, can be cured by lumbar subarachnoid drainage.

(5) scalp tension is too high: scalp tension is too high can cause incision split or scalp necrosis, this situation is rare, too high scalp tension can also shift the bone flap, extensive free scalp can reduce the tension of the suture.

(6) Bone resorption: bone flap absorption is rare, with an incidence of 0.7%, but this is one of the most worrying complications in cranioplasty. Once the bone flap is absorbed, it will inevitably lead to surgical failure.

(7) Postoperative epilepsy, visual acuity and oculomotor disorders: all rare.

Symptoms of cranial stenosis Common symptoms Oblique malformation Long head deformity Facial malformation Paranasal sinus dysplasia Short head malformation Eyeball prominent Cranial suture Premature closure Goldfish eye Repeated upper respiratory tract infection Triangular head deformity

Narrow cranial disease accounts for about 38% of head abnormalities. Its clinical manifestations mainly include skull deformities of various shapes. Because of the premature closure of the cranial suture, the growth of the skull is restricted, hindering the development of the brain, resulting in intracranial pressure. Increased, patients may have two eyes prominent, lower vision, eye movement disorders, optic disc edema or secondary atrophy, visual impairment or blindness, etc., some patients may have mental retardation, late can have headache, nausea, vomiting and other symptoms, some patients Seizures can occur due to atrophy of the cerebral cortex. The clinical manifestations of narrow cranial disease can be divided into two categories: head deformity and secondary symptoms.

1. The pointed deformity, also known as the tower-shaped skull, is more common, which is caused by the early closure of all cranial sutures. Because the growth of the skull is small except for the resistance of the anterior sacral sac, the other directions are restricted, so the head grows upwards in a tower shape. The bottom is under pressure, the eyelids become shallow, the eyeballs are prominent, and the paranasal sinus is poorly developed. As the brain tissue extends in the vertical direction, the upper and lower diameters of the skull increase, the anteroposterior diameter becomes shorter, and the anterior cranial fossa can be shortened to 1.5 cm. Small, short sacral fissure, marked increase in cerebral gyrus, enlarged saddle, delayed anterior condyle, and deformed frontal or posterior rotation of the frontal bone, causing the frontal and nasal ridges to form a line, the frontal nose disappears, typical The case is a cranial apex, and the posterior frontal spine is the main cause of head deformity. The middle part of the face can be normal. It is worth noting that the pointed deformity does not show obvious clinical manifestation before 2 to 3 years old. This is because of many cases. At 1 year old, the skull is normal, and at the age of 4, a typical pointed deformity occurs. True tip deformity with hand or foot and finger/toe deformity, called Saethre-Chotzen syndrome, fat cartilage hypoplasia For achondroplasia, optic atrophy Wide, flat head big nose, thick lips, are also deformed tip class common in infants, the sick child with arms and legs becomes shorter mental retardation, impaired vision, the cornea steatosis.

2. The scapular head deformity, also known as the long head deformity, is caused by the early closure of the sagittal suture. It is the most common cranial malformation in the craniosynostosis, accounting for 40% to 70%. The sagittal suture is closed prematurely. The lateral development is limited, that is, it expands forward and backward. As a result, the cranio-sacral canal is elongated before and after, and the left and right stenosis makes the skull a saddle-shaped deformity. The occipital and frontal poles are excessively bulged, and the frontal bone position can be high due to the narrow space between the ankles. The formation of pear-shaped forehead, sagittal sulcus caused by early closure of the sagittal suture, male majority, male to female ratio of 4:1, occasionally family history.

3. Triangular head deformity This type is rare, accounting for 5% to 10%, which is caused by the early closure of the frontal sulcus, but some of the frontal sulcus is still open, which is characterized by the front edge of the frontal scaly on the frontal joint. Protruding, showing an acute angle, the triangle is seen from above, the frontal bone is short and narrow, the anterior cranial fossa becomes smaller and shallower, the eyes are too close, and the forehead is thickened with osteophytes, often with other malformations.

4. Oblique head deformity, also known as partial head deformity, is a unilateral dysplasia of the frontal bone caused by unilateral coronal ossification, accounting for about 4%, bilateral growth of the skull is asymmetrical, and the frontal frontal bone is flat and retracted. The edge is elevated and retracted, and the lesion side affects the development of the brain tissue. The anterior iliac crest still exists, but it is biased to the healthy side. The premature closure of the epiphysis can be reached in the middle of the forehead. The asymmetry of the frontal bone affects the entire cranial sacral shape, and the sagittal suture The diseased side is biased, the frontal frontal bone and the parietal bone are over-expanded, and the ossification of the unilateral coronal suture can penetrate deep into the wing point and the skull base. Therefore, the oblique head deformity is almost always accompanied by facial asymmetry deformity, and with age. Increased and increased, the distance between the eyes becomes smaller, the forehead becomes narrower, the auricle and the external auditory canal can also be asymmetrical, but not obvious, the nasal deformity is more prominent, the oblique head deformity is more complicated with mental retardation, cleft palate, ocular fissure deformity, urinary System malformations and total forebrain deformities.

5. Short head deformity is caused by premature ossification of bilateral coronal sutures. The bilateral foreheads are flat and symmetrical after being closed. Therefore, it is also called flat head deformity or wide head deformity, accounting for 14.3%. Ossification of the sac, resulting in anterior and posterior diameter development of the skull and compensatory transverse diameter widening and cranial elevation, so the head is widened, the forehead is wide, the cranial fossa is enlarged, the eyelids are shallow, the sputum is poorly developed, the eyeball Obviously prominent, like the "goldfish eye."

The child may have obvious deformity within a few weeks after birth. The upper part of the frontal bone is tall and wide, the lower part is retracted, flat, sometimes concave. The upper part of the high and wide frontal bone often has a spherical protrusion in the surface structure. The lower part is retracted, the nasal bone is pulled backwards, the bridge of the nose is sunken, the nasopharyngeal cavity is small, and sometimes the skull base and hard palate are often deformed. The sick child often has repeated upper respiratory tract infection, and the ossified crown suture touches the rosary. Bone nodule.

6. Crouzon narrow cranial disease, also known as Crouzon skull bone dysplasia or Crouzon type craniofacial stenosis, was first reported by Crouzon in 1912, the main features of this disease are:

(1) The huge cranial cap and the cranial suture are closed early, and the coronal suture and herringbone suture are most common. The cranial apex is bulged due to the anterior tibial ossification.

(2) The normal mandible is relatively prominent compared with the small maxilla, and the facial nose and jaw are retracted, causing the occlusion to reverse, forming a false convex deformity to some extent.

(3) The nose is over-protrusive, and the sacral nose is pushed forward. The upper edge of the sac is pushed forward due to the short head deformity. The lower edge of the iliac crest is retracted due to the contraction of the jaw. The result is an extreme eyeball protrusion. The upper eyelid is widened to form the frog eye of Crouzon's disease, and the patient may be accompanied by ocular palsy.

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

(5) The disease may have increased intracranial pressure, loss of vision and mental retardation.

7.Apert craniofacial stenosis In 1906, Apert first reported that it is a hereditary disease characterized by a deformity of the pointed head and a deformity of the finger/toe. The facial deformity is more obvious, especially after the maxilla. The contraction is more obvious; the upper limit of the retraction is accompanied by a horizontal rotation on the top, so that the nasal root is deeply sunken under the eyebrow arch, causing the occlusion to open, the mouth opening and the middle of the upper lip seem to be pulled rearward. The eyeball is not prominent, and there are often extraocular strabismus. The facial deformity is obvious at birth.

Examination of cranial stenosis

1. The anterior and posterior slices of the X-ray skull of the pointed head can be seen obliquely in the lateral wall of the eyelid, the anterior cranial fossa becomes narrow, the bone density increases along the coronary sulcus, the calcium is calm, and there are often skull finger pressure cuts, lateral slices, frontal bone. After the rotation, the bones behind the frontal bone have no X-ray protrusion shadows, and the posterior cranial sac is normal.

2. The skull of the scaphoid skull can be seen as a boat-like deformity in the head. The bone density along the sagittal suture is increased, the calcium is calm, and the suture is not seen in severe cases. The coronary suture, herringbone seam, and scaly slit widening. Even separation, if there is an increase in intracranial pressure, it can be seen that the cerebral gyrus is increased.

3. The triangular head deformity X-ray shows that the frontal bone is short and highly convex. The orthotopic slice shows that the typical eyelid is too short and the inner wall of the iliac crest is vertical.

4. The X-ray of the oblique head deformity is a slanting head deformity, that is, the axial length of the eyelid is skewed upward and outward, and the skull is visible at the side of the coronal joint. The bone density is increased, the proximal wing point is higher, and the diseased side is in front of the skull. The fossa also became smaller, the anterior cranial fossa became steeper, the nose cone was skewed, and the sinus was biased toward the lesion side.

5. Short head deformity can be seen at both sides of the coronal joint at the increased bone density, the skull base changed to the anterior cranial fossa shortened and erected, the sphenoid winglets lifted up and back obliquely, the wing points raised and made As the nest deepens, the volume of the eyelids becomes smaller.

Diagnosis and identification of cranial stenosis

diagnosis

For patients with typical cranial malformation, the diagnosis is not difficult, but when the head is deformed after birth, it is often misdiagnosed as childbirth. If the head deformation does not disappear after a certain period of birth, the brain should be examined by X-ray film. The main manifestation is that the density of the skull joint is increased, the calcium is calm, and sometimes the cerebral gyrus is increased, and the post-bed decalcification and other signs of increased intracranial pressure are observed.

Differential diagnosis

The disease must be differentiated from small head disease. Microcephaly is a microcephaly caused by primary brain development disorder and the head does not increase. It is not the craniosynostosis that limits the development of brain tissue. The suture is also closed, for the secondary cranial closure, the patient often does not have increased intracranial pressure, mental and mental developmental disorders are more obvious, X-ray examination of the suture density can be normal or no brain back pressure traces and other signs of intracranial hypertension .

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