What does resection of a calcified craniopharyngioma look like?

Craniopharyngioma (craniopharyngioma) originates from flat epithelial cells remaining in the pituitary gland during embryogenesis, and is a common congenital benign intracranial tumor, mostly located above the pterygoid saddle, and a few within the saddle. There are many synonyms for craniopharyngiomas related to the site of initiation and growth, such as suprasellar cysts, cranial buccal sac tumors, pituitary duct tumors, enamel cell tumors, epithelial cysts, and enamel tumors. Craniopharyngiomas start in children and adolescents. Its main clinical features include hypothalamic-pituitary dysfunction, increased intracranial pressure, visual acuity and visual field disorders, urolithiasis, and neurological and psychiatric symptoms.CT scan can make a clear diagnosis. Treatment is mainly surgical resection of the tumor. Most craniopharyngiomas originate from the residual squamous epithelial cells of the craniobuccal duct near the funnel, so the tumor is located on the saddle, forming the so-called “saddle-type” craniopharyngioma; a small number of tumors originate from the residual cells of the intermediate part, so the tumor is located in the saddle, forming the so-called “saddle-type” craniopharyngioma. A small number of tumors originate from residual cells in the middle part of the saddle, and the tumor is located inside the saddle, forming the so-called “intra-saddle type” craniopharyngioma. Some craniopharyngiomas are found both in the saddle and in the saddle, and the tumors are dumbbell-shaped. Its clinical manifestations include the following aspects: high cranial pressure caused by tumor occupying effect and obstruction of interventricular foramen; visual impairment caused by tumor compression of optic cross and optic nerve; hypothalamic-pituitary dysfunction caused by tumor compression of hypothalamus and pituitary gland; and neurological and psychiatric symptoms caused by tumor encroachment on other brain tissues. There are mainly the following 5 aspects: 1. Increased intracranial pressure, craniopharyngioma has a large volume, and as an intracranial occupying lesion, it can directly cause increased intracranial pressure through occupying effect. Craniopharyngioma can also compress the third ventricle, block the interventricular foramen and increase the intracranial pressure, which may be the most important cause of high intracranial pressure. Symptoms of increased intracranial pressure are common in children, and the most common manifestation is headache, which can be mild or severe, mostly occurring in the early morning, accompanied by vomiting, tinnitus, vertigo, photophobia, optic disc edema, and spreading nerve paralysis, etc. Autonomic dysfunction, such as fever, facial flushing, and sweating, may also be manifested. The headache is mostly located in the retro-orbital region, but can also be diffuse and radiate to the back of the neck and back. In children, before the bone suture is closed, it can be seen that the bone suture is separated, the head circumference is enlarged, and there is a broken can sound on percussion, and the scalp veins are enraged. Most of the cysts causing intracranial hypertension are large, and the tumor compresses the third ventricle and blocks the interventricular foramen, which may also cause obstructive hydrocephalus. Because the pressure inside the cyst can change by itself, sometimes the symptoms of intracranial hypertension appear to be relieved automatically. Occasionally, the rupture of intra-tumoral cyst and the overflow of cystic fluid into the subarachnoid space may cause chemical meningitis and arachnoiditis, which is manifested by the sudden onset of severe headache and vomiting, accompanied by meningeal irritation symptoms, such as cervical resistance, positive Kening’s sign, leukocytosis in the cerebrospinal fluid, and fever. In the late stage, the aggravation of intracranial hypertension may lead to coma. 2.Optic nerve compression manifests as visual acuity, visual field changes and fundus changes. Because the growth direction of suprasellar tumor is not regular, the compression site is different, which makes the visual field defects vary greatly, such as quadrant defects, partial blindness, dark spots, and so on. Tumor compression of the optic cross can cause visual field defects, commonly for the two temporal hemianopsia, such as bilateral temporal hemianopsia, suggesting that the compression is from the top downward, and the degree of damage on both sides may not be the same. If the tumor only compresses the optic tract on one side, homonymous hemianopsia is produced. If the tumor severely compresses the optic chiasm, primary optic atrophy may result; if the tumor invades the third ventricle, causing hydrocephalus and increased intracranial pressure, secondary optic atrophy may result. The oculomotor nerve may be involved, resulting in diplopia and other symptoms. Intra-saddle tumor compresses the optic cross from below and upwards, resulting in visual field defects as in pituitary tumor, and the vision loss is related to optic nerve atrophy. Sometimes, sudden blindness can be caused by hemorrhagic infarction at the optic crossroads and impaired blood circulation. In patients with primary optic nerve atrophy, optic disk edema rarely occurs again. Foster-Kennedy syndrome can occur when the tumor grows to one side. In children, early visual field defects are often unnoticeable and are not detected until there is a severe visual impairment. 3.Hypothalamic Symptoms: Craniopharyngioma compressing hypothalamus and pituitary gland can cause many kinds of endocrine metabolic disorders and hypothalamic dysfunction: tumor destroying supraoptic nucleus or pituitary gland can cause urolithiasis, and its incidence is about 20%; tumor invading hypothalamus thirst center can cause the patient’s annoying thirst and excessive drinking or losing the sense of thirst; tumor invading the satiety center can cause polyphagia or anorexia; tumor invading the thermoregulation center can cause fever; tumor invading the pituitary gland can cause the patient to have fever; tumor damaging the pituitary gland can cause the patient to have fever. Tumor damage to the pituitary portal system or direct invasion of the pituitary gland can cause hypopituitarism, tumor damage to the hypothalamic TRH, CRH, GnRH neurons can cause TSH, ACTH and gonadotropin deficiency; tumor damage to the hypothalamic inhibitory neurons can cause pituitary hyperfunction, with common manifestations such as precocious puberty, acromegaly, deepening of skin pigmentation, cortisolism, etc.; some patients have obesity and obesity. Some patients have obesity, drowsiness, mental disorder, vasodilatory dysfunction and other symptoms. (1) Obesity reproductive impotence syndrome: the nodal part of the hypothalamus manages sexual function and reproductive activity and accomplishes it through the gonadotropins of the anterior pituitary gland; the funnel part and the area around the gray nodule are related to the fat metabolism. The pressure and destruction of the above parts can produce obesity, underdevelopment of sexual organs in children, loss of libido in adults, menopause in women, lactation disorders, and loss of secondary sexual characteristics. (2) Thermoregulatory disorders: damage to the posterior hypothalamus clinically manifested as low body temperature (35 ℃ ~ 36 ℃), a few patients may have chills; hypothalamus anterior affected by central hyperthermia (39 ℃ ~ 40 ℃). (3) Uremia: It is manifested by increased urine output, which can reach thousands of milliliters or even more than 10,000 ml per day, thus drinking a lot of water, and children are prone to drowning in bed at night. The cause of dysuria is tumor damage to the supraoptic nucleus, paraventricular nucleus, hypothalamic-pituitary tract or neuropituitary gland causing decreased secretion of antidiuretic hormone (ADH) or lack of it, but polyuria is related to the normal secretion of ACTH, such as the anterior pituitary gland at the same time damage to the anterior pituitary gland, ACTH secretion is reduced, then it will not result in dysuria. Sometimes because of the hypothalamic thirst center at the same time damage, can produce uremia with reduced thirst syndrome, the patient has urinary avalanches, plasma hyperosmolar state, but no thirst. The urine osmolality does not rise or rises slightly during abstinence from drinking, and blood volume decreases with hypernatremia. The patient may produce headache, tachycardia, irritability, confusion, delirium or even coma, and sometimes episodic hypotension. (4) Drowsiness: seen in advanced cases, mild cases can still be awakened, while severe cases can sleep for the whole day. (5) Mental symptoms: such as forgetfulness, inattention, fictionalization, etc., related to the damage of hypothalamus-limbic system or hypothalamus-frontal lobe connection, which is more common in adults. (6) Bulimia or food refusal disorder: destruction of the satiety center in the medial ventral nucleus of the hypothalamus can have bulimia (patient is obese), destruction of the food-loving center in the lateral ventral nucleus can have anorexia or food refusal disorder (patient is thin). It is seldom seen clinically. (7) Hyperprolactinemia (PRL): in a few cases, the tumor affects the hypothalamus or pituitary stalk, resulting in a decrease in the secretion of prolactin inhibitory factor (PIF) and an increase in the secretion of PRL cells in the anterior pituitary gland, and the clinical syndrome of breast milk overflow and amenorrhea can be produced. (8) Loss of pituitary hormone secretion: the hypothalamus may be affected, leading to the loss of GHRH, TRH and CRH secretion, which may affect growth and thyroid and adrenal cortex dysfunction. 4.Pituitary dysfunction symptoms: Hypopituitarism is more common than hyperpituitarism, especially LH/FSH and GH deficiency. About 50% of pediatric patients have growth retardation, and about 10% of children have obvious dwarfism with hypogonadism. In adult patients, GH deficiency is not prominent, but hypogonadism is found in more than 30% of patients. Secondary hypothyroidism caused by TSH deficiency is seen in about one-fourth of patients, and secondary hypoadrenocorticism caused by ACTH deficiency is also not uncommon. Early manifestations of pituitary insufficiency in pediatric patients include delayed physical development, shortness, thinness, fatigue, reduced activity, smooth pale skin, yellowish color, and wrinkles, resembling old age. Teeth and bones stop developing, bones are not united or delayed united, sex organs are infantile, no secondary sexual characteristics, and there are also cases of anorchidism. A few may have chills, mild mucous edema, low blood pressure, or even Simmond’s disease. Adult females have menstrual disorders or menopause, infertility and premature aging. Men have decreased libido, hair loss, low blood pressure, low metabolism (up to 35%) and so on. 5. Neighboring symptoms: The tumor may grow around, such as growing to both sides and invading the temporal lobe, which may cause temporal lobe epilepsy. If the tumor expands downward and invades the cerebral peduncle, it may produce spastic hemiparesis, and even de-cerebralized ankylosis. Some patients may suffer from mental disorder, which may be manifested as memory loss or even loss of memory, emotional indifference, or in severe cases, blurred consciousness or dementia. If the tumor grows to the pars plana, it can produce cavernous sinus syndrome, causing Ⅲ, Ⅳ, Ⅵ pairs of cerebral nerve disorders; if it grows to the pterygoid sinus and sieve sinus, it can lead to rhinorrhea and leakage of cerebrospinal fluid, etc. If it grows to the anterior fossa of the cranium, it can produce psychiatric symptoms, such as memory loss, poor orientation, unable to take care of urine and faeces, as well as epilepsy, olfactory disorders, etc.; if it grows to the middle fossa of the cranium, it can produce temporal lobe epilepsy, phantom smells, phantom flavors, etc.; for a small number of patients, the tumor can grow backward and produce mental disorders. In a few patients, the tumor may grow backward and produce brainstem symptoms, or even grow to the posterior fossa of the skull and cause cerebellar symptoms. In a few patients, the olfactory nerve and facial nerve may also be involved, manifesting as loss of sense of smell and facial paralysis. The frequency of the above symptoms is slightly different in children and young patients from that in adults, with intracranial hypertension being the first symptom in the former and optic nerve compression in the latter. All patients may have endocrine changes, but they are detected earlier in adults. General laboratory tests are unremarkable. Endocrine function tests Most patients may show a low or delayed decline in the glucose tolerance curve, and a decline in various hormones such as blood T3, T4, FSH, LH, and GH. A small number of patients showed adenopituitary hyperfunction, and most of them showed varying degrees of adenopituitary and corresponding target gland hypoplasia. 1.Growth hormone (GH) measurement and GH excitation test craniopharyngioma children serum GH value is reduced, and insulin hypoglycemia, arginine, levodopa and other excitation test, there is no obvious elevation of the response, accounting for 66.7%. 2.Gonadotropin (GnH) urinary gonadotropin (FSH), luteinizing hormone (LH) measurement and GnH excitation test. Craniopharyngioma patients with reduced serum FSH, LH levels, and on the gonadotropin-releasing hormone (commonly used for 3, prolactin (PRL) determination of patients with elevated serum PRL levels, which may be due to the tumor blockade of prolactin-releasing inhibitory hormone (PIH) into the pituitary, so that the secretion and release of PRL increased, which can lead to breast milk overflow, amenorrhea. This may lead to breast spillage and amenorrhea, accounting for 50% of the cases. 4, Adenocorticotropic hormone ACTH, thyrotropic hormone TSH determination when the tumor severely compresses the pituitary tissue and atrophy, the patient’s serum ACTH, TSH are reduced. 5.Antidiuretic hormone (ADH) determination of craniopharyngioma patients serum ADH is often reduced. 6.Lumbar puncture with increased intracranial pressure may show elevated pressure by lumbar puncture, and cerebrospinal fluid assay has no obvious changes. Other auxiliary examination 1, cranial X-ray 80% ~ 90% of patients with abnormal changes in cranial radiographs. Children’s cranial radiographs have abnormalities Generally speaking, successful surgery can effectively alleviate the visual acuity and visual field changes caused by visual cross compression, as well as headaches and other symptoms caused by high cranial pressure, and can also restore adenopituitary function. However, many suprasellar craniopharyngiomas are closely connected with the surrounding brain tissues (especially the hypothalamus), which increases the difficulty of the surgery. Complete removal of the tumor is not mandatory for these patients, but partial resection can be taken, and the disadvantage of partial resection is the high rate of postoperative recurrence. Depending on the site of tumor growth, size, shape, degree of calcification, location of the cystic portion, as well as the relationship with the surrounding tissues and easy access to the cerebrospinal fluid pathway, etc., different approaches or modes of surgery need to be selected, and each has its own advantages and disadvantages. Frontal floor approach The main structures that can be exposed are the optic nerve, optic chiasm, internal carotid artery, anterior cerebral artery, and pituitary stalk. It is suitable for posterior optic chiasm, large tumors growing from the saddle to the saddle, or ventricular extracerebral tumors growing anterior to the optic chiasm on the saddle. This approach can be further divided into several different procedures: e.g., through the inferior optic cross, or if the optic cross is anterior, between the resection of the saddle node and the pterygoid plate to reach the optic nerve or the opening of the end plate, and between the internal carotid artery and the optic nerve or optic bundle to reach the tumor. Pterygoid point approach Similar to the temporal base approach, but with the shortest route to the suprasellar region. It allows exposure of the ipsilateral internal carotid artery, anterior cerebral artery, optic nerve and optic bundle, inferior and posterior aspect of the optic cross, pituitary stalk, floor of the third ventricle, interpeduncular fossa, and superior slope, and it is indicated for ventricular extracellular tumors that are growing in the saddle toward the suprasaddle or in the suprasaddle suboptic and posterior interpeduncular fossa. This approach is currently the most widely used and is the main method of surgical resection of craniopharyngiomas. Endplate approach The unilateral subfrontal approach, pterygoid approach, and bilateral frontal longitudinal fissure approach can be used to reach the posterior optic chiasm and open the endplates to expose tumors that extend beyond the third ventricle. Therefore, this approach is suitable for the anterior optic cross and the intra- and extra-ventricular tumors growing behind the anterior optic cross on the saddle. Transcallosal or lateral ventricular approach If the tumor grows into the third ventricle, it can be accessed via the corpus callosum (in case of insignificant enlargement of the lateral ventricle) or via the lateral ventricle (in case of interventricular foramen obstruction causing hydrocephalus). There are several ways to enter the third ventricle and expose the tumor: (1) isolation of the unilateral fornix; (2) isolation of a vein adjacent to the interventricular foramen; (3) subxiphoid entry; and (4) isolation of the internal cerebral vein. Transsphenoidal approach The transsphenoidal approach can be used for tumors that are completely located in the saddle or those that grow mildly from the saddle to the suprasellar region or to the pterygoid sinus. Postoperative care 1.Observation of hypothalamic damage: Because craniopharyngioma surgery has different degrees of damage to the hypothalamus, it is easy to cause uremia and water electrolyte disorders. Accurately record the change of urine volume per unit time, observe the color of urine, and measure the specific gravity of urine if necessary. Follow the doctor’s advice to take blood specimens at regular intervals for blood biochemistry examination. When the urine volume per hour is less than 250ml, it can be left untreated for the time being and continue to be observed. When the hourly urine volume is 350~450 m1 and the blood electrolytes are normal, use 2~6 U of posterior pituitary hormone according to the patient’s age and body weight.When the hourly urine volume is 450~550 ml, give rehydration fluids according to the blood electrolytes. If the blood sodium is more than 145 mmol/L, patients with clear consciousness can take oral rehydration with plain water to promote the discharge of blood sodium and prevent water loss; patients who cannot eat are injected with plain water through indwelling gastric tube. When the patient’s blood sodium is less than 135 mmol/L, oral rehydration salts or saline is given. 2.Assessment of consciousness: postoperative intracranial hematoma, electrolyte disorders caused by coma, low levels of hormones in the body is the main reason for the change of consciousness in craniopharyngioma. When the change of consciousness is sudden and accompanied by elevated blood pressure, rapid pulse and unequal pupil size, the change of intracranial pressure should be considered firstly, and the doctor should be reminded to perform CT examination. If the impaired consciousness is progressive and there are electrolyte changes, blood specimen should be taken immediately for urgent blood biochemistry examination. If the blood biochemistry is normal and the patient has clinical symptoms such as fatigue, it may be that the hormone supplementation is insufficient or the hormone is reduced too quickly, resulting in low hormone levels. In conclusion, when the patient has consciousness disorder, the nurse should evaluate the cause according to the data from various aspects, and report to the doctor in time, and actively cooperate with the resuscitation. 3, vision, visual field observation: the patient’s visual field has been recorded before the operation, after the operation, the visual field should be evaluated again to grasp the intracranial changes after the operation, usually in the patient’s postoperative mental status when the check, if the visual field has decreased compared with the preoperative, usually due to the damage of the operation; if there is a sudden change, consider the intracranial hemorrhage, notify the doctor in time to deal with the situation. 4. Observation of pupils and vital signs: changes in pupils often precede changes in vital signs, so it is necessary to strengthen the observation of bilateral pupil size, morphology, and response to light after surgery, and report any abnormality to the doctor in time. Continuous electrocardiographic monitoring should be given and recorded every 15-30 min until the condition stabilizes. The gradual increase of blood pressure and the formation of hypertension often suggests intracranial hypertension; slow and strong pulse suggests that the intracranial pressure has a tendency to increase, and fast and weak indicates that the effective blood volume is insufficient; irregular respiratory rate, shallow and shallow suggests that the respiratory center is impaired; elevated temperature suggests that there is central hyperthermia or infectious hyperthermia or dysfunction of the thermoregulatory center, such as hypothermia, cold limbs, suggesting that there is a possibility of shock. 5, position and drainage tube care: the patient is conscious, blood pressure is stable, take the head elevated 15-30 degrees slope position, in order to facilitate blood reflux, reduce intracranial pressure, keep the drainage tube unobstructed, the patient’s head to do appropriate restrictions, in the turning, treatment and other injuries operation, the action is gentle, slow, small angle, do not pull the drainage tube, to prevent the drainage tube pull off. Check whether the drainage tube is pressurized, twisted or angled at any time, and deal with the problem in time. Change the drainage bottle and surgical site dressing under aseptic operation every day, and pay attention to the amount, color and character of the drainage fluid.