pituitrin and Craniopharyngioma

Pre-operative central diabetes insipidus has been reported in 8-35% of patients affected with craniopharyngioma, and in 70-90% after surgery. The management of postoperative polyuria and polydipsia can be challenging and fluid balance needs to be closely monitored. The classical triphasic pattern of endogenous vasopressin secretion--an initial phase of symptomatic diabetes insipidus occurring 24 hours after surgery; a second phase of inappropriate vasopressin secretion potentially causing hyponatraemia; and a third phase with a return to diabetes insipidus occurring up to 2 weeks later--is often complicated by cerebral salt wasting and thirst disorders. Inadequate adrenal replacement therapy and anticonvulsant agent treatment may increase the risk of life-threatening hyponatraemia in the course of desmopressin (DDAVP) treatment. Appropriate management, in order to avoid life-threatening or disabling electrolyte disturbances, requires a good grasp of the relevant pathophysiology. We review here the pathophysiology and management of the multiple fluid disorders encountered following surgery for craniopharyngiomas.

Topics: Child; Craniopharyngioma; Diabetes Insipidus; Humans; Hyponatremia; Neurosurgical Procedures; Pituitary Neoplasms; Postoperative Complications; Thirst; Vasopressins; Water-Electrolyte Imbalance

Due to the proximity of craniopharyngiomas to the hypothalamus and pituitary gland, most children and adolescents presenting with these tumors will exhibit significant endocrine dysfunction. After treatment, these impairments can become a major cause of morbidity and mortality.. The postoperative course of children undergoing surgery for craniopharyngioma is reviewed.. Even if hormone levels seem to be adequate in the short term after treatment, deficiencies may develop over years and need to be monitored closely.

Topics: Adrenocorticotropic Hormone; Body Weight; Child; Child, Preschool; Craniopharyngioma; Endocrine System; Endocrine System Diseases; Follicle Stimulating Hormone; Humans; Luteinizing Hormone; Neurophysins; Pituitary Neoplasms; Postoperative Complications; Protein Precursors; Thyrotropin; Vasopressins

Topics: Craniopharyngioma; Endocrine Glands; Feedback; Female; Follicle Stimulating Hormone; Gonadotropin-Releasing Hormone; Humans; Hypogonadism; Hypothalamus; Luteinizing Hormone; Male; Neurosecretion; Pituitary Gland; Pituitary Hormone-Releasing Hormones; Pituitary Hormones, Anterior; Prolactin; Stimulation, Chemical; Thyroid Diseases; Thyrotropin; Thyrotropin-Releasing Hormone; Vasopressins; Water-Electrolyte Balance

A 3-year-old girl presented with osmotic demyelination syndrome after undergoing uneventful neuroendoscopic cystostomy for a growing cystic suprasellar craniopharyngioma following microscopic subtotal resection 1 year previously. Endocrinopathy had well been controlled by hormone replacement therapy and administration of 1-amino-8-d-arginine-vasopressin with serum sodium concentration within the normal range. She presented generalized seizure and fever on postoperative day 7, with hyponatremia beginning on postoperative day 4 and deteriorating despite frequent correction. The serum sodium concentration began to fluctuate on the same day, in the range 111-164 mEq/l, which lasted for 2 weeks, refractory for intense management. Her body temperature also fluctuated between hypo- and hyperthermia not correlated with serum inflammatory markers. Her conscious disturbance progressively deteriorated with spastic paraparesis. T(2)-weighted magnetic resonance (MR) imaging taken on postoperative day 19 revealed hyperintense areas in the pons, external capsule, bilateral thalami, and basal nuclei, which had not been recognized before, suggesting osmotic demyelination syndrome causing central pontine and extrapontine myelinolysis. MR imaging taken on postoperative days 230 and 360 showed some diminished lesions but others persisted and resulted in a cavity. The patient's depressed conscious level did not improve. Suprasellar craniopharyngioma with long-standing hypothalamic dysfunction may be associated with severe osmotic demyelination syndrome even after less invasive surgery, so serum sodium derangement after surgery should be promptly corrected even if only subtle signs are present.

Topics: Brain; Child, Preschool; Consciousness Disorders; Craniopharyngioma; Disease Progression; Female; Fever; Hormone Replacement Therapy; Humans; Hyponatremia; Hypothalamus; Magnetic Resonance Imaging; Myelinolysis, Central Pontine; Nerve Fibers, Myelinated; Neurosurgical Procedures; Pons; Postoperative Complications; Vasopressins; Water-Electrolyte Balance

In this study we aimed to establish the frequency of postoperative diabetes insipidus and the incidence and characteristics of abnormalities of thirst in a cohort of patients with craniopharyngioma, in whom neurosurgery had been performed.. Diabetes insipidus was determined by either standard criteria for diagnosis in the immediate postoperative period, or by water deprivation test, in all craniopharyngioma and pituitary tumour patients who underwent surgery in Beaumont Hospital between the years 1986 and 1998. Osmoregulated thirst and vasopressin release were studied during a 2-h infusion of hypertonic (5%) saline followed by a 30-min period of free access to water.. Data on the incidence of postoperative diabetes insipidus was collected in 26 patients with craniopharyngioma and 154 patients with pituitary adenomata. We recruited 16 healthy control patients, 16 patients with cranial diabetes insipidus following pituitary tumour surgery and 16 patients with cranial diabetes insipidus following craniopharyngioma resection for the hypertonic saline infusion study.. Twenty-five patients out of 26 (96%) patients developed diabetes insipidus after surgery for craniopharyngioma, a much higher incidence than after surgery for suprasellar (26/88, 30%, P < 0.001) or intrasellar pituitary tumours (9/66, 14%, P < 0.001). Hypertonic saline infusion identified abnormal thirst responses in five of the 16 craniopharygioma patients studied; all of the pituitary tumour patients had a normal thirst response. Three of the craniopharyngioma patients had adipsic diabetes insipidus whilst two had polydipsic diabetes insipidus.. This study demonstrates following surgery for craniopharyngioma there is a high incidence of cranial diabetes insipidus and a significant incidence of abnormal thirst responses to osmotic stimuli.

Topics: Adult; Blood Pressure; Cohort Studies; Craniopharyngioma; Diabetes Insipidus; Drinking; Female; Humans; Male; Middle Aged; Neurosurgical Procedures; Osmolar Concentration; Pituitary Neoplasms; Postoperative Complications; Retrospective Studies; Saline Solution, Hypertonic; Thirst; Vasopressins

Topics: Child; Craniopharyngioma; Craniotomy; Dehydration; Diagnosis, Differential; Female; Humans; Hypernatremia; Hypothalamic Diseases; Pituitary Neoplasms; Syndrome; Vasopressins

Topics: Adolescent; Chlorides; Craniopharyngioma; Humans; Hyponatremia; Hypopituitarism; Male; Neoplasm Recurrence, Local; Pituitary Neoplasms; Postoperative Complications; Sodium; Syndrome; Vasopressins

A case of pulmonary embolism associated with diabetes insipidus is reported in an 18-year-old male. The patient, who had been treated with DDAVP for diabetes insipidus and hydrocortisone for hypocorticism for two years after first operation for the removal of craniopharyngioma, was admitted with recurrence of that tumor. Diabetes insipidus immediately after second operation was controlled with intermittent drip infusion of a small amount of aqueous pitressin under monitorings of body weight hourly using a patient weighing system to keep the weight changes within +/- one kilogram. Serum and urine electrolytes levels, osmolarity, and free water clearance were also monitored every three hours to maintain water-electrolytes balances appropriately. Postoperative course had been uneventful except that CSF rhinorrhea occurred 7 days after operation. The patient was, then, kept in bed with horizontal plane to avoid further leakage of CSF. Two days later, he developed chest pain suddenly with tachypnea, tachycardia, and general cyanosis. The arterial-BGA showed PaO2 of 53.5mmHg and PaCO2 of 35.3mmHg in room air. The definite diagnosis of pulmonary embolism was made by technetium microaggregate lung perfusion scans and by pulmonary angiograms. The patient was treated with heparin, 15000IU/day, and urokinase, 720000IU/day. The symptoms due to pulmonary embolism had improved gradually within a couple of weeks. Recent articles have shown an unexpected high incidence of deep vein thrombosis and pulmonary embolism in neurosurgical patients associated with the elevation of blood coagulability. Brain tumors, especially suprasellar mass with hypothalamic dysfunction have been suggested to cause thromboembolic disorders frequently. The clinical course was described and factors causing pulmonary embolism on this patient was discussed.

Topics: Adolescent; Craniopharyngioma; Diabetes Insipidus; Heparin; Humans; Lung; Male; Neoplasm Recurrence, Local; Pituitary Neoplasms; Postoperative Complications; Pulmonary Embolism; Radionuclide Imaging; Tomography, X-Ray Computed; Urokinase-Type Plasminogen Activator; Vasopressins

Topics: Atrial Natriuretic Factor; Clofibrate; Craniopharyngioma; Female; Humans; Hydrocortisone; Hypernatremia; Middle Aged; Pituitary Neoplasms; Postoperative Complications; Vasopressins

Topics: Adolescent; Adult; Child; Child, Preschool; Craniopharyngioma; Diabetes Insipidus; Female; Follow-Up Studies; Humans; Hydrocortisone; Male; Pituitary Neoplasms; Postoperative Complications; Vasopressins

Topics: Adolescent; Adult; Craniopharyngioma; Diabetes Mellitus; Humans; Pituitary Neoplasms; Postoperative Complications; Vasopressins

Topics: Blood Glucose; Child; Child, Preschool; Craniopharyngioma; Diabetes Insipidus; Diabetic Coma; Female; Humans; Hyperglycemic Hyperosmolar Nonketotic Coma; Pituitary Neoplasms; Postoperative Complications; Vasopressins

Topics: Clofibrate; Craniopharyngioma; Diabetes Insipidus; Drinking; Humans; Neoplasm Recurrence, Local; Pituitary Neoplasms; Postoperative Complications; Sodium; Vasopressins

A 49-year-old man was submitted to neurosurgery for a cranio-pharyngioma. The lesion, which appeared to involve the antero-inferior wall of the third ventricle, caused lack of appropriate antidiuretic hormone (ADH) release in response to hypernatraemia and plasma hyperosmolality. The probable mechanism of this hypothalamic syndrome is suggested.

Topics: Craniopharyngioma; Humans; Hypernatremia; Hypothalamus; Male; Middle Aged; Osmolar Concentration; Pituitary Neoplasms; Postoperative Complications; Potassium; Sodium; Vasopressins

Topics: Child; Craniopharyngioma; Female; Humans; Hyponatremia; Inappropriate ADH Syndrome; Infant; Male; Pituitary Neoplasms; Postoperative Care; Postoperative Complications; Sodium; Time Factors; Vasopressins; Water-Electrolyte Imbalance

For the assessment of hypothalamo-pituitary-adrenal function in the presence of pituitary adenomas and craniopharyngiomas, insulin tests, lysine-vasopressin tests, and rapid ACTH tests were performed and plasma cortisol was assayed. Rapid ACTH test and lysine-vasopressin test, which examine adrenal and mainly pituitary function respectively, showed normal function in ten among 14 cases. But insulin test, which examines the whole hypothalamo-pituitary-adrenal function, showed various levels of abnormality in eight among 14 cases. Frequent association of functional disturbances of this axis in these diseases was stressed.

Topics: Adenoma, Chromophobe; Adolescent; Adrenocorticotropic Hormone; Adult; Aged; Child; Craniopharyngioma; Female; Humans; Hydrocortisone; Hypothalamo-Hypophyseal System; Insulin; Lypressin; Male; Middle Aged; Pituitary Neoplasms; Pituitary-Adrenal System; Vasopressins

The Medical Research Council of Canada has initiated human growth hormone (hGH) therapy in 151 patients with documented complete hGH deficiency that was idiopathic in 76% of cases, secondary to craniopharyngioma (organic) in 17% and of varied cause in 7%. Approximately 50% of the patients with idiopathic disease had isolated hGH deficiency; during therapy thyroid deficiency developed in five patients and cortisol deficiency in three. A similar increase in mean height velocity occurred in the first treatment phase for patients less than 12 years old (0.93 plus or minus 0.30 cm/mo) and those 12 years and older (0.86 plus or minus 0.29 cm/mo). Although subsequent courses of hGH therapy yielded significantly diminished response in both age groups, this diminution was not progressive: the height velocity of the younger patients returned to 0.82 plus or minus 0.26 cm/ml in the fifth therapy phase. The mean height velocity attained at the optimal dosage (0.20 to 0.29 units/kg three times per week) for each age group did not differ significantly. Despite therapy being carried out for only 6 months of the year, normal increment ratios for height age and bone age against chronologic age were observed in the patients with idiopathic disease. In only four patients did treatment failure occur, and three of these were more than 20 years old. The addition of fluoxymesterone (10 mg/d) to the hGH therapeutic regimen (15 units/wk), when diminished response to hGH alone became evident, promoted an enhanced growth response in 9 of 11 older patients. These data indicate that age of the patient and dosage of hGH, but not diagnostic category, were important influences on the response to therapy. Younger patients responded best and maintained a higher mean growth velocity than older patients during intermittent hGH therapy

Topics: Adolescent; Age Determination by Skeleton; Antibodies; Body Height; Brain Neoplasms; Canada; Child; Cortisone; Craniopharyngioma; Female; Fluoxymesterone; Growth Disorders; Growth Hormone; Humans; Hypopituitarism; Male; Pituitary Hormones; Prolactin; Puberty; Thyroxine; Vasopressins

Six children with human growth hormone (hGH) deficiency became hypothyroid during the course of their therapy with hGH. This was accompanied by a decreasing growth rate, clinical symptoms of hypothyroidism and decreased serum T4 concentrations. Three of the 6 patients returned to the euthyroid state, both clinically and biochemically, with cessation of hGH therapy, and reinstitution of hGH precipitated hypothyroidism again in 2 of the three. The patients who remained hypothyroid have evidence of multiple pituitary trophic hormone deficiencies while those who reverted to euthyroidism appear to have isolated hGH deficiency. Evaluation of thyroid function while on hGH showed low T4, free T4 and T3 concentrations. The serum thyrotropin (TSH) response to thyrotropin-releasing hormone (TRH) was absent or markedly blunted in 4 of 6 patients while receiving long-term hGH therapy but was normal or exaggerated in all patients when tested before or after only 5 days of hGH therapy. These data indicate that exogenous hGH results in an inhibition of the TSH response to TRH. The mechanism of this inhibition is unclear, but we postulate that it may be mediated by somatostatin secretion in response to pulse doses of hGH.

Topics: Age Determination by Skeleton; Body Height; Craniopharyngioma; Diabetes Insipidus; Dwarfism, Pituitary; Female; Growth; Growth Hormone; Humans; Hypopituitarism; Hypothyroidism; Male; Thyroid Gland; Thyroxine; Vasopressins

Topics: Brain Neoplasms; Craniopharyngioma; Drug Therapy, Combination; Female; Glioma; Humans; Male; Methylprednisolone; Vasopressins

A 29-year-old woman with evidence of a craniopharyngioma and documented panhypopituitarism is described. Clinical and laboratory evaluation revealed deficiencies of follicle-stimulating hormone, luteinizing hormone, thyroid-stimulating hormone, growth hormone, prolactin, adrenocorticotropic hormone and antidiuretic hormone. Prompt release of several pituitary hormones was noticed after administration of the hypothalamic releasing hormones FSH/LH-RF and thyrotropin-releasing hormone, whereas insulin-induced hypoglycemia, levodopa, chlorpromazine and clomiphene citrate, all of which act at the level of the hypothalamus, did not alter basal pituitary secretion. The patient's height of 60 inches, despite panhypopituitarism, and the interpretation of the above data are discussed in the light of current concepts regarding the dynamics of the hypothalamic-hypophyseal system.

Topics: Adolescent; Adrenocorticotropic Hormone; Adult; Child; Chlorpromazine; Clomiphene; Craniopharyngioma; Dwarfism, Pituitary; Female; Follicle Stimulating Hormone; Gonadotropin-Releasing Hormone; Growth Hormone; Humans; Hypopituitarism; Hypothalamo-Hypophyseal System; Insulin; Levodopa; Luteinizing Hormone; Pituitary Function Tests; Pituitary Gland; Pituitary Hormones; Prolactin; Thyrotropin; Thyrotropin-Releasing Hormone; Vasopressins

Topics: Acute Disease; Anuria; Child; Craniopharyngioma; Depression, Chemical; Diabetes Insipidus; Female; Humans; Injections, Intravenous; Phenytoin; Pituitary Neoplasms; Postoperative Care; Postoperative Complications; Secretory Rate; Sodium; Stimulation, Chemical; Time Factors; Vasopressins

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adenoma, Chromophobe; Adrenocorticotropic Hormone; Adult; Arginine; Blood Glucose; Craniopharyngioma; Diagnosis, Differential; Female; Glucose; Glucose Tolerance Test; Gonadotropins, Pituitary; Growth Hormone; Humans; Hypothalamo-Hypophyseal System; Insulin; Male; Metyrapone; Middle Aged; Pituitary Gland; Pituitary Hormones, Anterior; Pituitary Neoplasms; Pituitary-Adrenal System; Sella Turcica; Thyroid Gland; Vasopressins

Topics: Acromegaly; Adenoma, Acidophil; Adenoma, Chromophobe; Adult; Brain Neoplasms; Craniopharyngioma; Diabetes Insipidus; Female; Humans; Luteinizing Hormone; Male; Meningioma; Metyrapone; Pinealoma; Pituitary Hormone-Releasing Hormones; Pituitary Neoplasms; Radioimmunoassay; Thyrotropin; Thyrotropin-Releasing Hormone; Vasopressins

Topics: Adenoma, Chromophobe; Adrenocorticotropic Hormone; Adult; Aged; Child; Craniopharyngioma; Female; Follicle Stimulating Hormone; Growth Hormone; Humans; Hypopituitarism; Hypothalamus; Hypothyroidism; Luteinizing Hormone; Male; Meningioma; Middle Aged; Pituitary Neoplasms; Radioimmunoassay; Thyrotropin; Thyrotropin-Releasing Hormone; Time Factors; Vasopressins

Topics: 17-Hydroxycorticosteroids; Acromegaly; Adenoma, Chromophobe; Adolescent; Adrenal Glands; Adrenocorticotropic Hormone; Adult; Child; Craniopharyngioma; Dexamethasone; Humans; Hypoglycemia; Hypothalamus; Insulin; Lysine; Metyrapone; Middle Aged; Pituitary Gland; Pituitary Neoplasms; Pituitary-Adrenal Function Tests; Pyrogens; Sella Turcica; Vasopressins

Topics: Adrenocorticotropic Hormone; Adult; Aged; Astrocytoma; Brain Neoplasms; Cerebral Ventriculography; Craniopharyngioma; Echoencephalography; Electroencephalography; Female; Glucocorticoids; Humans; Male; Meningioma; Methylprednisolone; Middle Aged; Neurofibromatosis 1; Vasopressins; Visual Field Tests

Topics: Adolescent; Adult; Blood Urea Nitrogen; Brain Neoplasms; Chlorides; Craniopharyngioma; Diabetes Insipidus; Drinking Behavior; Dysgerminoma; Female; Humans; Hydrocortisone; Hypernatremia; Hypothalamus; Male; Middle Aged; Osmolar Concentration; Potassium; Sella Turcica; Sodium; Thirst; Thyroid Function Tests; Triiodothyronine; Vasopressins; Water-Electrolyte Balance

Topics: Adenoma; Adolescent; Adult; Craniopharyngioma; Diabetes Insipidus; Diuresis; Female; Humans; Hypophysectomy; Kidney Concentrating Ability; Male; Middle Aged; Osmolar Concentration; Osmotic Pressure; Pituitary Neoplasms; Thirst; Vasopressins

Topics: Adult; Chorionic Gonadotropin; Cortisone; Craniopharyngioma; Desoxycorticosterone; Estrogens; Female; Gestational Age; Gonadotropins, Pituitary; Humans; Hypophysectomy; Hypopituitarism; Infertility, Female; Menopause; Ovulation; Pituitary Neoplasms; Pregnancy; Pregnanediol; Vasopressins

Topics: Adolescent; Adult; Brain Neoplasms; Craniopharyngioma; Diabetes Insipidus; Diuresis; Humans; Hydrocortisone; Hypopituitarism; Pituitary-Adrenal Function Tests; Vasopressins; Water-Electrolyte Balance

Topics: Brain Neoplasms; Child; Chlorpropamide; Craniopharyngioma; Cysts; Dehydration; Diabetes Insipidus; Drinking Behavior; Female; Humans; Kidney Concentrating Ability; Male; Obesity; Osmolar Concentration; Sodium; Thirst; Vasopressins; Water-Electrolyte Balance

Topics: Acromegaly; Adenoma, Chromophobe; Craniopharyngioma; Female; Headache; Humans; Hypogonadism; Insulin; Iodine Radioisotopes; Male; Metyrapone; Optic Atrophy; Osteoporosis; Pituitary Neoplasms; Pituitary-Adrenal Function Tests; Skull Neoplasms; Thyroid Function Tests; Vasopressins; Vision Disorders

Topics: Acetates; Acute Disease; Adult; Chlorides; Coma; Cortisone; Craniopharyngioma; Diabetes Insipidus; Diet Therapy; Electrocardiography; Humans; Hypernatremia; Hypokalemia; Male; Natriuresis; Osmolar Concentration; Paralysis; Pituitary Neoplasms; Postoperative Complications; Potassium; Sodium; Thirst; Thyroxine; Vasopressins

Topics: Adolescent; Adrenocorticotropic Hormone; Blood Glucose; Body Height; Body Weight; Brain Neoplasms; Child; Craniopharyngioma; Exercise Test; Feeding and Eating Disorders; Female; Glucose Tolerance Test; Gonadotropins; Growth; Growth Hormone; Humans; Hypothalamus; Insulin; Male; Obesity; Pituitary Neoplasms; Postoperative Complications; Radioimmunoassay; Thirst; Thyrotropin; Vasopressins; Vision Disorders

Topics: Adolescent; Adult; Child; Chorionic Gonadotropin; Craniopharyngioma; Female; Follicle Stimulating Hormone; Growth Hormone; Humans; Hypopituitarism; Infertility, Female; Male; Ovulation; Pituitary Function Tests; Pregnancy; Vasopressins

Topics: Adenoma, Chromophobe; Adolescent; Adrenocorticotropic Hormone; Adult; Aged; Craniopharyngioma; Female; Glucose Tolerance Test; Gonadotropins; Growth Hormone; Humans; Hydrocortisone; Hypopituitarism; Iodine; Male; Melanocyte-Stimulating Hormones; Middle Aged; Pituitary Neoplasms; Pituitary-Adrenal Function Tests; Thyroid Function Tests; Thyrotropin; Vasopressins; Water-Electrolyte Balance

Topics: Blood Cell Count; Blood Chemical Analysis; Child; Cortisone; Craniopharyngioma; Diabetes Insipidus; Electroencephalography; Humans; Hyponatremia; Hypopituitarism; Male; Radiography; Sella Turcica; Vasopressins; Water-Electrolyte Balance

Topics: Craniopharyngioma; Diabetes Insipidus; Estrogens; Humans; Hypogonadism; Medroxyprogesterone; North Carolina; Pituitary Neoplasms; Testosterone; Vasopressins