Paranasal sinus dysplasia
Introduction
Introduction Cranial stenosis showed depression of the skull base, shallow eyelids, prominent eyeballs, and poor development of the paranasal sinus. Cranial stenosis is the premature closure of one or more skull joints leading to head deformity, increased intracranial pressure, intelligent developmental disorders, and ocular symptoms. Clinically characterized by premature closure of single or multiple skull sutures. 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. Most of the lesions are concentrated in the coronal suture or multiple sutures. Some scholars have unknown reasons. The cranial suture 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. Early cranial ossification of patients with cretinism who overuse thyroid hormone replacement therapy.
Cause
Cause
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. Most of the lesions are concentrated in the coronal suture or multiple sutures. Some scholars have unknown reasons. The cranial suture 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. Early cranial ossification of patients with cretinism who overuse thyroid hormone replacement therapy.
As early as 1975, Cohen and Converse et al. in 1976 elaborated on the cause of the narrow cranial disease. It is believed that this disease is a congenital developmental malformation, but in general its cause is still unclear, which may be related to the development of embryonic mesodermal developmental disorders, or it may be due to the occurrence of heterotopic ossification center in the bone-seal tissue. May be related to the lack of certain matrices in the embryo. A few cases have genetic factors. Individual cases can be caused by vitamin D deficiency and hyperthyroidism. The basic reason that Park and Power have suggested is that the skull interstitial bundle is incompletely grown, resulting in a reduction in the skull and premature ossification of the suture tissue.
Examine
an examination
Related inspection
Otolaryngology CT examination of nasopharyngeal MRI
The starting point of ossification of the cranial suture and how the ossification spreads, the corresponding cranio-sacral humerus, the skull base and the role of the dura mater are not well understood. The development of the skull base plays a leading role in the pathogenesis of the narrow cranial disease with facial deformity changes.
Narrow cranial disease accounts for about 38% of head abnormalities, and its clinical manifestations are mainly manifested in various shapes of skull deformities. Because the cranial suture is closed prematurely, the growth of the skull is restricted, hindering the development of the brain, resulting in an increase in intracranial pressure. Patients may have two eyes protruding, lower vision, ocular dyskinesia, optic 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 advanced stage. Some patients may have seizures due to atrophy of the cerebral cortex. The clinical manifestations of narrow cranial disease can be divided into two major categories: head deformity and secondary symptoms.
1. Apical deformity: Also known as tower-shaped skull, it is more common, which is caused by premature closure of all cranial sutures. Because the growth of skull is small except for the resistance of the frontal sacral, all other directions are restricted, so the head grows upwards in a tower shape. The base of the skull is depressed, 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, the anterior cranial fossa can be shortened to 1.5 cm, the optic nerve pores become smaller, the supraorbital fissure is shorter, the cerebral gyrus is significantly increased, and the sella is enlarged. The front squat closing delay. The orthodontic frontal or posterior rotation of the frontal bone causes the frontal bone and the nasal ridge to form a line, and the frontal nose disappears. A typical case is the cranial apex. The posterior frontal rotation is the main cause of head deformity. The middle of the face is normal. It is worth noting that the deafness does not show significant clinical manifestations before the age of 2 to 3 years. This is because many cases have normal skulls at 1 year of age, and typical teratomas occur at 4 years of age. True pointed deformity with hand or foot and finger toe deformity, known as Saethre-Chotzen syndrome. Fatty cartilage hypoplasia manifests as achondroplasia, optic atrophy, wide head and flat nose, thick lips, also belong to the type of pointed deformity, common in infants and young children, short arms and lower limbs with mental retardation, visual impairment The cornea has lipid deposits.
2. Scapular head deformity: Also known as long head deformity, caused by early closure of sagittal suture alone, it is the most common cranial malformation in craniosynostosis, accounting for 40% to 70%. The sagittal suture is closed prematurely, and the head is laterally restricted, that is, it expands forward and backward. As a result, the cranio-sacral stenosis is elongated before and after, and the left and right stenosis makes the skull a saddle-shaped deformity, and the occipital and frontal poles are excessively bulged. The frontal bone position can be very high, resulting in a pear-shaped forehead due to the narrow gap between the armpits. Sagittal head deformity 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 forehead is still open. The feature is that the sides of the frontal scaly portion of the frontal sulcus protrude forwardly at an acute angle, and the head is triangular in shape, the frontal bone is short and narrow, and the anterior cranial fossa becomes smaller and shallower, and the eyes are too close. There is osteophyte thickening at the forehead, often with other deformities.
4. Oblique head deformity: also known as partial head deformity, which is a unilateral dysplasia of the frontal bone caused by unilateral coronal ossification, accounting for about 4%. The bilateral growth of the skull was asymmetrical, and the frontal frontal bone was flattened and retracted. The lesion side affects the development of brain tissue, and the anterior iliac crest still exists, but it is biased to the healthy side. 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, the sagittal suture is biased to the disease side, and the frontal frontal bone and the parietal bone are excessively bulged. 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 it is aggravated with age. The distance between the eyes becomes smaller and the forehead becomes narrower. The auricle and the external auditory canal can also be asymmetrical, but not obvious, and the deformity of the ankle is more pronounced. Oblique head deformity combined with mental retardation, cleft palate, ocular fissure malformation, urinary system malformation and total forebrain malformation.
5. Short head deformity: It is caused by premature ossification of bilateral coronal sutures. After the bilateral coronal sutures are closed, the forehead is flat and symmetrical, so it is also called flat head deformity or wide head deformity, accounting for about 14.3%. The coronal ossification of the skull on both sides of the patient's head causes the anterior and posterior diameter development of the skull and the compensatory transverse diameter widening and cranial elevation. Therefore, the head is widened, the forehead is wide, the cranial fossa is enlarged, and the eyelid is shallow. dysplasia, the eyeball is 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. Upper; the lower half retracts, pulling the nasal bone to the rear and causing the bridge of the nose to sink. Nasopharyngeal cavity becomes smaller, sometimes the skull base and hard palate often have deformities, and sick children often have repeated upper respiratory tract infections. The ossified coronal suture touches the beaded bone nodule.
6. Crouzon narrow cranial disease: Also known as Crouzon skull bone dysplasia or Crouzon type craniofacial stenosis. It 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 widens to form the frog eye of Crouzon's disease. 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: First reported by Apert in 1906, it is a hereditary disease characterized by a teratoma and a deformity syndrome of the fingers. The facial deformity is more obvious, especially the retraction of the maxilla is more obvious; the upper digit of the retraction is accompanied by a horizontal rotation on the top, so that the nasal root is deeply depressed under the eyebrow arch, causing the occlusion to open and the mouth The middle of the open and upper lip seems to be pulled to the back. The eyeball is not prominent, and there is often extraocular strabismus. Facial deformities are evident at birth.
For patients with typical cranial malformations, the diagnosis is not difficult. However, when the head is deformed after birth, it is often misdiagnosed as a result of childbirth. If the head deformation does not disappear after a certain period of time after birth, a cranial X-ray examination should be performed. Mainly manifested as increased density of the skull suture, calcium deposition, and sometimes increased signs of increased cerebral gyrus, post-bed decalcification and other signs of increased intracranial pressure.
Diagnosis
Differential diagnosis
The starting point of ossification of the cranial suture and how the ossification spreads, the corresponding cranio-sacral humerus, the skull base and the role of the dura mater are not well understood. The development of the skull base plays a leading role in the pathogenesis of the narrow cranial disease with facial deformity changes.
Narrow cranial disease accounts for about 38% of head abnormalities, and its clinical manifestations are mainly manifested in various shapes of skull deformities. Because the cranial suture is closed prematurely, the growth of the skull is restricted, hindering the development of the brain, resulting in an increase in intracranial pressure. Patients may have two eyes protruding, lower vision, ocular dyskinesia, optic 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 advanced stage. Some patients may have seizures due to atrophy of the cerebral cortex. The clinical manifestations of narrow cranial disease can be divided into two major categories: head deformity and secondary symptoms.
1. Apical deformity: Also known as tower-shaped skull, it is more common, which is caused by premature closure of all cranial sutures. Because the growth of skull is small except for the resistance of the frontal sacral, all other directions are restricted, so the head grows upwards in a tower shape. The base of the skull is depressed, 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, the anterior cranial fossa can be shortened to 1.5 cm, the optic nerve pores become smaller, the supraorbital fissure is shorter, the cerebral gyrus is significantly increased, and the sella is enlarged. The front squat closing delay. The orthodontic frontal or posterior rotation of the frontal bone causes the frontal bone and the nasal ridge to form a line, and the frontal nose disappears. A typical case is the cranial apex. The posterior frontal rotation is the main cause of head deformity. The middle of the face is normal. It is worth noting that the deafness does not show significant clinical manifestations before the age of 2 to 3 years. This is because many cases have normal skulls at 1 year of age, and typical teratomas occur at 4 years of age. True pointed deformity with hand or foot and finger toe deformity, known as Saethre-Chotzen syndrome. Fatty cartilage hypoplasia manifests as achondroplasia, optic atrophy, wide head and flat nose, thick lips, also belong to the type of pointed deformity, common in infants and young children, short arms and lower limbs with mental retardation, visual impairment The cornea has lipid deposits.
2. Scapular head deformity: Also known as long head deformity, caused by early closure of sagittal suture alone, it is the most common cranial malformation in craniosynostosis, accounting for 40% to 70%. The sagittal suture is closed prematurely, and the head is laterally restricted, that is, it expands forward and backward. As a result, the cranio-sacral stenosis is elongated before and after, and the left and right stenosis makes the skull a saddle-shaped deformity, and the occipital and frontal poles are excessively bulged. The frontal bone position can be very high, resulting in a pear-shaped forehead due to the narrow gap between the armpits. Sagittal head deformity 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 forehead is still open. The feature is that the sides of the frontal scaly portion of the frontal sulcus protrude forwardly at an acute angle, and the head is triangular in shape, the frontal bone is short and narrow, and the anterior cranial fossa becomes smaller and shallower, and the eyes are too close. There is osteophyte thickening at the forehead, often with other deformities.
4. Oblique head deformity: also known as partial head deformity, which is a unilateral dysplasia of the frontal bone caused by unilateral coronal ossification, accounting for about 4%. The bilateral growth of the skull was asymmetrical, and the frontal frontal bone was flattened and retracted. The lesion side affects the development of brain tissue, and the anterior iliac crest still exists, but it is biased to the healthy side. 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, the sagittal suture is biased to the disease side, and the frontal frontal bone and the parietal bone are excessively bulged. 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 it is aggravated with age. The distance between the eyes becomes smaller and the forehead becomes narrower. The auricle and the external auditory canal can also be asymmetrical, but not obvious, and the deformity of the ankle is more pronounced. Oblique head deformity combined with mental retardation, cleft palate, ocular fissure malformation, urinary system malformation and total forebrain malformation.
5. Short head deformity: It is caused by premature ossification of bilateral coronal sutures. After the bilateral coronal sutures are closed, the forehead is flat and symmetrical, so it is also called flat head deformity or wide head deformity, accounting for about 14.3%. The coronal ossification of the skull on both sides of the patient's head causes the anterior and posterior diameter development of the skull and the compensatory transverse diameter widening and cranial elevation. Therefore, the head is widened, the forehead is wide, the cranial fossa is enlarged, and the eyelid is shallow. dysplasia, the eyeball is 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. Upper; the lower half retracts, pulling the nasal bone to the rear and causing the bridge of the nose to sink. Nasopharyngeal cavity becomes smaller, sometimes the skull base and hard palate often have deformities, and sick children often have repeated upper respiratory tract infections. The ossified coronal suture touches the beaded bone nodule.
6. Crouzon narrow cranial disease: Also known as Crouzon skull bone dysplasia or Crouzon type craniofacial stenosis. It 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 widens to form the frog eye of Crouzon's disease. 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: First reported by Apert in 1906, it is a hereditary disease characterized by a teratoma and a deformity syndrome of the fingers. The facial deformity is more obvious, especially the retraction of the maxilla is more obvious; the upper digit of the retraction is accompanied by a horizontal rotation on the top, so that the nasal root is deeply depressed under the eyebrow arch, causing the occlusion to open and the mouth The middle of the open and upper lip seems to be pulled to the back. The eyeball is not prominent, and there is often extraocular strabismus. Facial deformities are evident at birth.
For patients with typical cranial malformations, the diagnosis is not difficult. However, when the head is deformed after birth, it is often misdiagnosed as a result of childbirth. If the head deformation does not disappear after a certain period of time after birth, a cranial X-ray examination should be performed. Mainly manifested as increased density of the skull suture, calcium deposition, and sometimes increased signs of increased cerebral gyrus, post-bed decalcification and other signs of increased intracranial pressure.
