Pediatric empty saddle syndrome
Introduction
Introduction to pediatric empty saddle syndrome Empty saddle syndrome (emptysellasy syndrome) is also known as Busch-Colby syndrome, saddle septal defect, arachnoid cyst, saddle arachnoid diverticulum, saddle arachnoid cyst. This symptom refers to a series of clinical symptoms caused by the compression of the pituitary gland into the saddle. basic knowledge Sickness ratio: 0.05% Susceptible people: children Mode of infection: non-infectious Complications: cerebrospinal fluid rhinorrhea
Cause
Causes of pediatric empty saddle syndrome
Transmission of cerebrospinal fluid pressure (25%):
When the saddle septum is defective, the subarachnoid space on the saddle is inserted into the sella through an incomplete saddle barrier. When there is increased intracranial pressure, such as hypertension, obesity, heart failure, etc., it is more likely to cause empty saddle sign, cerebrospinal fluid pressure. Increased, the amount of cerebrospinal fluid in the subarachnoid space is also increased, which may be one of the causes of this disease.
Endocrine factors (25%):
In thyroid, adrenal or hypogonadal dysfunction, the pituitary gland is increased and the saddle septum is enlarged. The above-mentioned dysfunction is given to the replacement therapy, the enlarged pituitary is gradually restored, and the enlarged saddle septum can cause empty Saddle sign.
Pituitary cyst rupture (25%):
In the saddle or parasagittal cyst, small cysts in the middle of the pituitary can confluence to increase the compression of the pituitary or rupture into the subarachnoid space, can produce symptoms, saddle cysts are not uncommon in children.
Arachnoid adhesion in the sellar region (5%):
Adhesion of the arachnoid, resulting in poor drainage of the cerebrospinal fluid, increased pressure, and caused the disease.
Pituitary lesions (5%):
Pituitary ischemia, atrophy and infarction are also one of the causes of this disease.
Prevention
Pediatric empty saddle syndrome prevention
At present, there is no systematic preventive measures, there is no need to pay attention to, and timely detection of timely treatment.
Complication
Complications of pediatric empty saddle syndrome Complications Cerebrospinal fluid rhinorrhea
May be associated with thyroid, adrenal or hypogonadal disease, can occur severe vision loss, cerebrospinal fluid rhinorrhea, endocrine changes, skeletal deformities and so on.
Symptom
Symptoms of pediatric saddle syndrome Syndrome Common symptoms Chondroital dysplasia and... Vision vision changes pituitary dysfunction Cerebrospinal fluid rhinorrhea field of view shrinks
1. Organ dysfunction Wilkinson reported 16 children with this disease, 7 had visual organ disorders, 3 of which had abnormal crystal and iris, 3 had severe visual loss, and 2 had optic atrophy or reduced visual field.
2. The incidence of cerebrospinal fluid rhinorrhea is about 7% to 19.2%, and there are many headaches.
3. Endocrine changes Many adult patients have no symptoms or signs of endocrine changes, some may have hypofunction of the anterior pituitary and posterior lobes, and children may have hypothalamic-pituitary axis function abnormalities.
4. Children with skeletal malformations may be associated with skeletal dysplasia syndrome.
Examine
Examination of pediatric empty saddle syndrome
Secondary infection may have a peripheral blood leukocyte count and a significant increase in neutrophils; cerebrospinal fluid examination pressure increased, cerebrospinal fluid white blood cell count can be significantly increased during intracranial infection; endocrine changes, hypothalamic-pituitary function test results can be found abnormally .
Endocrine function test: The blood concentration of pituitary hormone in patients with primary empty saddle is mostly normal. Even for the stimulation test of pituitary hormone, most patients are basically normal, but recently Bianconcini et al have made comprehensive and 71 cases of primary empty saddle syndrome. Detailed pituitary hormone assays, including excitatory and inhibitory tests of pituitary reserve function, showed that 50.7% of patients had one or more endocrine abnormalities, of which prolactin increased by 14%, pituitary hypofunction accounted for 10.4%, and gonadal function Decreased 7%, diabetes insipidus 2.8%, ACTH increased by 1.4%, growth hormone decreased by 15.4%, pituitary adenoma 8.4%, the author had 10 pituitary-target gland dynamics test for severe primary empty saddle, including pituitary gland The function of the leaves decreased, the prolactin increased (the CT scan showed no signs of microadenomas), the growth hormone deficiency, the decrease of gonadotropin and the decrease of ACTH were in 1 case, and 5 cases showed normal function of pituitary storage. One of the gonads of postmenopausal women Hormone is still high; 1 case of insulin-hypoglycemia excitatory test shows that growth hormone is weakly reactive, but this case is obviously obese, while normal human obesity growth hormone can also be weakly reactive. Therefore, the empty saddle syndrome should still be followed regularly for endocrine function.
X-ray examination of the head flat film, mostly manifested as increased saddle, gas cerebral angiography and CT brain scan for the diagnosis of this disease has a definite help, can be found that gas freely into the saddle and visible liquid level which is the main diagnostic basis of this disease, X-ray bone examination in children can detect deformities.
1. X-ray examination of the saddle flat and body imaging: typical morphological changes accounted for 75%, such as:
(1) Deformation: The lateral position shows that the sella is enlarged, and when the sphenoid sinus is dysplastic, the saddle depth is enlarged and oval, and when the sphenoid sinus is well developed, the saddle is enlarged in a rectangular shape, and the anterior slice shows a uniform symmetrical depression at the bottom of the saddle. It is concave.
(2) Bone quality changes in the saddle floor: On the lateral position, the saddle bottom bone uniformity is thickened in the empty saddle, and the saddle bottom bone is thinner in the pituitary adenoma.
2. Gas cerebral angiography (PEG) shows that there is gas filling in the saddle, and occasionally the pituitary is crescent-shaped and pushed into the lower part of the saddle.
3. Carotid angiography failed to show the expansion of the existing saddle tumor to the saddle, which helps to negate the existence of pituitary adenoma.
4. Isotope cerebral angiography Only those who have cerebrospinal fluid rhinorrhea need to perform this test.
5. CT scan and magnetic resonance imaging High-resolution CT coronal or magnetic resonance imaging can make a rapid and non-invasive diagnosis. Typical images show saddle-swelling, pituitary atrophy, and being filled with the same low density as the saddle-upper pool. Cerebrospinal fluid replacement, if the contrast-enhanced direct coronal CT scan, no enhancement, severely depressed pituitary, was pushed to the lower flank, the pituitary stalk can reach the saddle bottom, forming a characteristic "funnel"" (infundibulum).
Diagnosis
Diagnosis and diagnosis of pediatric empty saddle syndrome
According to clinical features and auxiliary examination can be diagnosed.
Different from intracranial tumors, brain CT scan, gas cerebral angiography can help identify.
