Surgical Outcomes of Pediatric Craniopharyngiomas in Nepal: A Prospective Observational Study

Pineal Parenchymal Tumors of Intermediate Differentiation Treated With Ventricular Radiation and Temozolomide

Diabetes insipidus.

The Neuroendocrine Effects of Traumatic Brain Injury

Neuroendocrine dysfunction after traumatic brain injury (TBI) is under-diagnosed, under-treated, and may adversely affect the rate of recovery. Single or multiple pituitary-target hormone disruption occurs in up to two-thirds of persons with TBI, most commonly affecting the gonadal and growth hormone axes. The time course of decline in and recovery of pituitary function in relation to cognitive dysfunction and rehabilitation progress are not well described. This article reviews the clinical spectrum of neuroendocrine deficits after TBI and their underlying mechanisms. Future studies of the effects of hormonal replacement on recovery are recommended.

2010 Update: Consequences of Hypothalamic–Pituitary Dysfunction Following Traumatic Brain Injury in Children

Long-Term Outcomes and Complications in Patients With Craniopharyngioma: The British Columbia Cancer Agency Experience

Hypopituitarism may often occur in association with traumatic brain injury (TBI). Identification of reliable predictors of pituitary dysfunction is of importance in order to establish a rational testing approach. We searched the records of patients with TBI, who underwent neuroendocrine evaluation in our institution between 2007 and 2013. One hundred sixty-six adults (70% men) with TBI (median age: 41.6 years; range: 18-76) were evaluated at a median interval of 40.4 months (0.2-430.4).Of these, 31% had ≥1 pituitary deficiency, including 29% of patients with mild TBI and 35% with moderate/severe TBI. Growth hormone deficiency was the most common deficiency (21%); when body mass index (BMI)-dependent cutpoints were used, this was reduced to 15%. Central hypoadrenalism occurred in10%, who were more likely to have suffered a motor vehicle accident (MVA, p = 0.04), experienced post-traumatic seizures (p = 0.04), demonstrated any intracranial hemorrhage (p = 0.05), petechial brain hemorrhages (p = 0.017), or focal cortical parenchymal contusions (p = 0.02). Central hypothyroidism occurred in 8% and central hypogonadism in 12%; the latter subgroup had higher BMI (p = 0.03), were less likely to be working after TBI (p = 0.002), and had lower Global Assessment of Functioning (GAF) scores (p = 0.03). Central diabetes insipidus (DI) occurred in 6%, who were more likely to have experienced MVA (p < 0.001) or sustained moderate/severe TBI (p < 0.001). Patients with MVA and those with post-traumatic seizures, intracranial hemorrhage, petechial brain hemorrhages, and/or focal cortical contusions are at particular risk for serious pituitary dysfunction, including adrenal insufficiency and DI, and should be referred for neuroendocrine testing. However, a substantial proportion of patients without these risk factors also developed hypopituitarism.

Disclosure: A. Sehgal: None. D.M. Watashi: None. S.T. Sharma: None. INTRODUCTION: An ectopic posterior pituitary (EPP), predominantly at the median eminence, arising from abnormal embryonic development, is a rare entity with an incidence of 0.5 per 1,000,000 births. Although commonly associated with growth hormone (GH) deficiency, other pituitary hormone abnormalities can also be seen. We describe three cases with EPP and varying pituitary hormone dysfunction, emphasizing the need for close long-term follow-up of these patients. CASE DESCRIPTION: Case 1: 30-year-old male, born at full term, required NICU care for hypoglycemia. He was initially diagnosed with secondary adrenal insufficiency, central hypothyroidism, and hypogonadism and treated with hydrocortisone and levothyroxine. MRI Pituitary showed an EPP, absent pituitary stalk, and a small pituitary gland. He later developed GH deficiency and AVP deficiency and was placed on GH and desmopressin as well. Case 2: A 34-year-old male presented at age 1 year with failure to thrive. Diagnosed with GH deficiency and initiated on treatment. At age 2 years, he developed central hypothyroidism and was placed on levothyroxine. MRI Pituitary at age 6 years revealed an EPP and hypoplastic pituitary stalk. He later developed central adrenal insufficiency and central hypogonadism, prompting replacement therapy. Has not shown any evidence of AVP deficiency. Case 3: 42-year-old female diagnosed with GH deficiency at age 5 years. MRI showed an EPP and a hypoplastic infundibulum. Later developed secondary amenorrhea and was diagnosed with hypogonadotropic hypogonadism as well as central hypothyroidism. MRI Pituitary showed an EPP and hypoplastic pituitary stalk. She has not shown any signs of adrenal insufficiency or AVP deficiency. DISCUSSION: EPP results from defective neuronal migration and may occur in isolation or with stalk anomalies. While isolated GH deficiency is the most frequent presentation of EPP, it can be associated with panhypopituitarism or another individual pituitary hormone dysfunction. Posterior pituitary function frequently remains intact. Congenital hypopituitarism is classified as either hypothalamic or pituitary in origin, with associated gene defects like HESX1. It is often associated with hypoplasia of the anterior pituitary and the pituitary infundibulum, but the underlying mechanism is still not well understood. CONCLUSION: EPP is a rare condition that should be considered in patients diagnosed with hypopituitarism. MRI is the gold standard imaging modality to identify this condition. All three of our patients were diagnosed with EPP in childhood, either with isolated GH deficiency or with coexistent other pituitary hormone deficiencies. These patients require lifelong follow-up with an endocrinologist. Treatment consists of hormone replacement, with the main goals of alleviating symptoms and preventing complications. Presentation: Sunday, July 13, 2025

Studies investigating long-term health conditions in patients with craniopharyngioma are limited by short follow-up durations and generally do not compare long-term health effects according to initial craniopharyngioma treatment approach. In addition, studies comparing long-term health conditions between patients with childhood- and adult-onset craniopharyngioma report conflicting results. The objective of this study was to analyse a full spectrum of long-term health effects in patients with craniopharyngioma according to initial treatment approach and age group at craniopharyngioma presentation. Cross-sectional study based on retrospective data. We studied a single-centre cohort of 128 patients with craniopharyngioma treated from 1980 onwards (63 patients with childhood-onset disease). Median follow-up since craniopharyngioma presentation was 13 years (interquartile range: 5-23 years). Initial craniopharyngioma treatment approaches included gross total resection (n = 25), subtotal resection without radiotherapy (n = 44), subtotal resection with radiotherapy (n = 25), cyst aspiration without radiotherapy (n = 8), and 90Yttrium brachytherapy (n = 21). Pituitary hormone deficiencies (98%), visual disturbances (75%) and obesity (56%) were the most common long-term health conditions observed. Different initial craniopharyngioma treatment approaches resulted in similar long-term health effects. Patients with childhood-onset craniopharyngioma experienced significantly more growth hormone deficiency, diabetes insipidus, panhypopituitarism, morbid obesity, epilepsy and psychiatric conditions compared with patients with adult-onset disease. Recurrence-/progression-free survival was significantly lower after initial craniopharyngioma treatment with cyst aspiration compared with other therapeutic approaches. Survival was similar between patients with childhood- and adult-onset craniopharyngioma. Long-term health conditions were comparable after different initial craniopharyngioma treatment approaches and were generally more frequent in patients with childhood- compared with adult-onset disease.

Purpose : Idiopathic central diabetes insipidus (CDI) is defined in CDI patients without definite etiology. Some patients initially diagnosed as idiopathic CDI progressed to organic causes. We reviewed clinical, endocrinological, and radiological courses of 20 patients who was initially diagnosed as idiopathic CDI, to assess the predicting factors for progression to brain tumors. Methods : We reviewed the medical data and followed up their clinical courses in 20 CDI patients who had no definite organic etiology, such as malformation, tumor, at the time of diagnosis. Results : Our study included 15 males and 5 females. Mean age of CDI diagnosis was 7.8±3.6 (2.1- 14.7) years. Mean follow-up duration was 8.6±5.1 (1.5-18) years. Six (30%) patients were diagnosed as brain tumor during follow-up. Ten (50%) of 20 patients had growth hormone deficiency. Multiple pituitary hormone deficiencies were found more frequently in brain tumor patients than idiopathic patients (60% vs 7%, P=0.037). Pituitary stalk thickening (PST) and loss of posterior pituitary signal were observed in 9 patients (47%), respectively. The newly development of PST was observed in patients diagnosed as brain tumor. Conclusion : About 30% of idiopathic CDI patients progress to organic disease such as germ cell tumor or histiocytosis. If there are multiple anterior pituitary hormone deficiency or newly development of PST, more close and careful follow-up is needed.

Congenital Proprotein Convertase 1/3 Deficiency Causes Malabsorptive Diarrhea and Other Endocrinopathies in a Pediatric Cohort

Comparison of Minimally Invasive Total versus Subtotal Resection of Spinal Tumors: A Systematic Review and Meta-Analysis

Chapter 10 - Neuroendocrine manifestations of Erdheim–Chester disease

Short stature is defined as a height &lt;2 SD from the mean height for a child of the same sex, ethnicity and chronological age. We present a case series of proportionate short stature whose associated endocrinological deficits left us intrigued. A 9 ½-year-old boy presented with poor growth-velocity (GV) for 5 years and central diabetes insipidus (on the treatment for 1 year). His height was 118 cm (−2.6 SD) and his weight was 20 kg (−2.03 SD). MRI brain showed hypoplastic anterior pituitary with absent bright spot and growth hormone (GH) dynamics proved GH deficiency. A 10-year-4-month-old girl presented with poor GV. Her height was 106 cm (−4.44 SD) and her weight was 15 kg (−3.74 SD). Targeted investigations revealed multiple pituitary hormone deficiencies (central hypothyroidism, secondary adrenal insufficiency and GH deficiency). MRI brain showed reduced pituitary height with ectopic posterior pituitary. GH therapy commenced only after coverage with hydrocortisone. A 1-year-old boy was admitted with failure to gain weight and height for 4 months of age. His length was 57 cm (−7.89 SD) and weight: was 4.6 kg (−5.86 SD) with immature facies, frontal bossing and midfacial hypoplasia. Low GH values at the time of critical sample (blood glucose = 36 mg/dl) revealed GH deficiency. MRI brain demonstrated a hypoplastic pituitary gland. All proportionate short-statured children without obvious dysmorphism need detailed evaluation. GH deficiency can present as a spectrum from isolated deficiency to multiple pituitary (anterior and posterior) deficiencies and so the order of correction of the deficiencies is equally important.

Summary&#x0D; A 36-year-old female patient was diagnosed with diabetes insipidus after sublabial transsphenoid hypophysectomy (SLTH) surgery. The patient had pituitary adenoma. The patient undergoes 14 days of care in the ICU with titrated intravenous vasopressin dose(0,01-0,3unit/hour) and later subcutaneous dose(6-13unit/8 hours). Subcutaneous vasopressin started on day 3 while intravenous was tapering down; at the early transition from the intravenous vasopressin route to the subcutaneous vasopressin route on day 7, there is a sharp surge of urine production as well at plasma sodium level. The intravenous vasopressin started again, along with the elevated dose of subcutaneous vasopressin. The patient shows a response to therapy after a watchfully titrated dose.&#x0D; &#x0D; Background&#x0D; Diabetes insipidus is a combination of signs and symptoms generating a plentiful volume of urine and causing elevated serum osmolality. There are two types of diabetes insipidus: central diabetes insipidus and nephrogenic diabetes insipidus. Central neurogenic diabetes insipidus occurs when the production of the hormone Arginine Vasopressin (AVP) is low. In contrast, nephrogenic diabetes insipidus occurs when the kidneys cannot respond to high levels of the hormone AVP. Postsurgical central insipidus can be categorized into transient, permanent, and triphasic. Transient courses of diabetes insipidus following surgery represent most of the cases. Temporary diabetes insipidus is thought to be caused by temporary dysfunction of AVP-producing neurons as a result of direct surgical trauma or indirect after-surgical edema. The incidence of diabetes insipidus in patients who underwent pituitary surgery is 5%, and 4.6% of these patients will have only transient diabetes insipidus, and only 0.4% became permanent. &#x0D; Transphenoidal surgery is considered a minimally invasive and effective procedure for pituitary adenomas. Diabetes Insipidus after this surgery is not an uncommon complication, even though the reported rate of postsurgical central diabetes insipidus varies widely from 1 to 67%. Postoperative temporary diabetes insipidus gradually resolves up to 6 months.&#x0D; &#x0D; Case Presentation&#x0D; A 36-year-old female patient presented chief complaints of headache and blurred vision, which gradually worsened one year ago. After undergoing several examinations, the patient was diagnosed with pituitary adenoma. The patient underwent a sublabial transsphenoidal hypophysectomy. The duration was three long hours and uneventful.&#x0D; On Day 0, the patient arrived at the intensive care unit (ICU) intubated, hemodynamically stable, and sedated. The patient is then monitored and weaned; a brain protection strategy and strict fluid balance urine collection and pain management are applied. On day 1, the patient was then extubated. The patient was examined for several parameters, such as electrolytes, kidney function, and blood glucose level. The patient began to significantly increase urine output (&gt;5 milliliters/kilogram body weight/hour). Increased urine production is accompanied by a simultaneous decrease in urine-specific gravity (&lt;1.005) and an increase in serum sodium level up to 151 mmol/liter. The patient was diagnosed with postsurgical diabetes insipidus. The patient started receiving intravenous vasopressin at a dose of 0.3 units/hour and titrated according to urine production until the target urine output was reached after the third day of care. After urine is reached, the dose of vasopressin slowly decreases, and the administration begins to transition to the subcutaneous route. On day 7 of treatment, when the intravenous vasopressin dose had been discontinued and the vasopressin dose at the 8u/h point, there was a significant urine production spike and an increase in the plasma sodium level to 156 mmol/liter. On the eighth day of treatment, the administration of vasopressin was again given intravenously and subcutaneously until a decrease in urine production towards the target was achieved. Finally, on days 11 to 14, vasopressin is administered only subcutaneously until the patient is discharged from the ICU. The patient was successfully discharged to the ward with a tapering-off subtotal dose.&#x0D; &#x0D; &#x0D; Discussion&#x0D; The patient developed polyuria within the initial hours of treatment. Polyuria is a hallmark sign of diabetes insipidus. The clinician should be aware of other polyuria causes, such as postoperative hypervolemia, hyperglycemia, and the use of diuresis drugs. This differential diagnosis must be excluded. In this case, the differential diagnosis was excluded through proper fluid balance calculations, monitoring serum electrolytes and glucose levels, and ensuring the absence of diuretic use. Confirmation of the postoperative central diabetes insipidus is made based on findings of high urine output (5 ml/kg BW/hour), urine specific gravity (&lt;1.005), response to vasopressin, average blood glucose level, and absence of diuretic use.&#x0D; Diabetes Insipidus is the body's inability condition to concentrate urine due to defective production of the antidiuretic hormone (central diabetes insipidus) or nephrogenic diabetes insipidus (NDI), which corresponds to the insensitivity of the kidney to the antidiuretic effects of vasopressin. Diabetes insipidus (DI) is a syndrome characterized by polyuria (&gt;30ml/kg/24H) of hypotonic urine, equivalent polydipsia, and hypernatremia. The patient shows elevated urine volume (108 cc/kg/24H) and blood sodium levels (144-151mmol/L). The primary therapy was the titrated vasopressin dose, in conjunction with electrolytes and fluid management. Vasopressin titration is based on patient clinical condition, urin output, fluid management, oral intake, and laboratory measures (natrium blood level and urin osmolarity).&#x0D; Transient Diabetes Insipidus must be closely monitored after neurosurgical operations, especially in regions adjacent to the pituitary DI. Transient Management with good monitoring is the key. The risk of morbidity comes from the risk of untreated dehydration, electrolyte imbalance&#x0D; Intravenous vasopressin provides a rapid effect with lower doses. At the same time, subcutaneous administration requires caution in critically ill patients because absorption is slow, resulting in a slow effect and the need for higher doses. The conversion of the administration route needs to consider the patient's pharmacology, route, and hemodynamics.&#x0D; &#x0D; References&#x0D; Leroy, C., Karrouz, W., Douillard, C., Do Cao, C., Cortet, C., Wémeau, J.-L., Vantyghem, M.-C., 2013. Diabetes insipidus. Ann. Endocrinol. 74, 496–507. https://doi.org/10.1016/j.ando.2013.10.002&#x0D; Priya, G., Kalra, S., Dasgupta, A., Grewal, E., 2021. Diabetes Insipidus: A Pragmatic Approach to Management. Cureus. https://doi.org/10.7759/cureus.12498&#x0D; Schreckinger, M., Szerlip, N., Mittal, S., 2013. Diabetes insipidus following resection of pituitary tumors. Clin. Neurol. Neurosurg. 115, 121–126. https://doi.org/10.1016/j.clineuro.2012.08.009&#x0D; Sharman, A., Low, J., 2008. Vasopressin and its role in critical care. Contin. Educ. Anaesth. Crit. Care Pain 8, 134–137. https://doi.org/10.1093/bjaceaccp/mkn021&#x0D; &#x0D; &#x0D;