Persistent Diabetes Insipidus Research Articles

THE IMPORTANCE OF EARLY DETECTION OF DIABETES INSIPIDUS IN CHILDHOOD

<p class="p1"><strong>Introduction:</strong></p> <p class="p1">Diabetes insipidus (DI) is a disease that occurs due to inappropriate secretion of anti-diuretic hormone from the pituitary, or as a result of disorder in which the level of the kidneys cannot adequately respond to the secretion of this hormone. Also, it is known as central diabetes insipidus. The most common causes are head traumas, tumors of the hypothalamus and pituitary glands, inflammatory processes, histiocytosis, anomalies in the development of brain. It can appear in the form of familial diabetes insipidus or in certain syndromes (Wolfram syndrome). It is characterized by hypotonic polyuria higher than 3l/24h (which persists if even taking liquids stops), then by nocturia and compensatory polydipsia. Enuresis often occurs among children.</p> <p class="p1"><strong>Case report:</strong></p> <p class="p1">A boy, aged 11, lives with his mother and brothers. Mother noticed that the boy was urinating frequently in last few months (diuresis 4.6 l/24h, and 3.25 l/24h). After two months, the boy developed double images and severe headaches, vomiting, inability to see, squinting in the right eye and headache in the back of the head. MNR of the endocranium indicates the presence of a tumor formation. The tumor was surgically removed, and the boy started with chemotherapy and radiotherapy. Due to persistent diabetes insipidus, the boy started using desmopressin-acetate - in tablet form. Active substance desmopressin - acts in the same way as the natural hormone vasopressin and regulates the kidney's ability to concentrate urine. The positive effect of taking the drug appeared after three weeks from the start of taking the therapy.</p> <p class="p1"><strong>Conclusion</strong>:</p> <p class="p1">Central (neurogenic) DI occurs as a result of a relative or absolute deficiency of antidiuretic hormone, which is responsible for the osmolality of body fluids. Based on this case, we want to show the importance of early diagnosis of the disease in order to improve the prognosis and the necessity of careful monitoring of these patients.</p>

8142 Endocrine Considerations Prior to Pituitary Tumor Resection

Abstract Disclosure: P. Einafshar: None. T. Delibasi: None. Endocrine Considerations in Pituitary Tumor Resection Introduction: Diabetes Insipidus (DI) is an endocrine condition involving a disturbance to the action of Anti-Diuretic Hormone (ADH) resulting in polyuria, and polydipsia with complications such as severe water and electrolyte disturbances. It can be classified into Central DI which affects the secretion of ADH or Nephrogenic DI which affects peripheral ADH receptors in Distal Convoluted Tubules (DCTs) in the renal system. Both subtypes however present similarly and are usually known to be transient. It is important to consider the most common etiologies contributing to both central and nephrogenic DI as severe complications may arise if not diagnosed and treated promptly. The most important step is to monitor and manage water and electrolytes and avoid excessive water loss which can lead to irreversible complications. This can be accomplished by introducing synthetic ADH called DDAVP or Desmopressin. Case Description: A 57-year-old female was admitted in preparation for a supra-orbital resection of a suprasellar mass. Two hours post-procedure, she was found to have an increase in urine output with her labs notable for a significant acute increase in serum sodium and a decrease in urine osmolality. Upon emergent Endocrinology evaluation, due to concerns of Acute Diabetes Insipidus in the setting of insult to the pituitary stalk, she was started on emergent treatment with IV DDAVP. Her course was complicated with persistent DI resulting in a case of permanent DI requiring out-patient daily treatments with DDAVP. Discussion: Injury to the pituitary stalk resulting in various endocrine abnormalities has been studied involving a trans-sphenoidal resection of pituitary tumors, but there is a paucity of cases reported when patients undergo a supra-orbital surgical approach. It is vital for Endocrinology and Neurosurgical services to be aware of this phenomenon, and a multi-disciplinary approach to patient management should be considered. Furthermore, close follow-up of pituitary hormones in the post-operative setting is imperative in order to avoid severe complications, such as brain damage or poor mental status. We hope to educate residents and fellows about this phenomenon that is rarely reported in the literature but can have profound effects on patient’s quality of life if not considered by the consulting Endocrinology team. References Christ-Crain, M., Winzeler, B., & Refardt, J. (2021). Diagnosis and management of diabetes insipidus for the internist: an update. Journal of internal medicine, 290(1), 73-87. Mutter, C. M., Smith, T., Menze, O., Zakharia, M., & Nguyen, H. (2021). Diabetes insipidus: pathogenesis, diagnosis, and clinical management. Cureus, 13(2). Presentation: 6/1/2024

Hypophisitis in pregnant women with persistent diabetes insipidus in the outcome

Autoimmune/lymphocytic hypophysitis is one of the rare causes of central diabetes insipidus in adults and is most common among women in the second or third trimester of pregnancy. Numerous studies have shown that lymphocytic hypophysitis is characterized by a very variable clinical signs with the development of neurological symptoms, visual disturbances and hypopituitarism with partial or complete loss of pituitary function, as well as a number of features in magnetic resonance imaging (MRI). Isolated lymphocytic indibuloneurohypophysitis occurs in fewer cases and involves the posterior lobe and stalk of the pituitary gland with a clinical presentation of diabetes insipidus. The above clinical case describes the development of hypophysitis in a pregnant woman with a predominant lesion of the posterior pituitary gland and an outcome in diabetes insipidus, which persists 6 years after pregnancy and childbirth. In the article some aspects of the differential diagnosis of diabetes insipidus in pregnant women, as well as instrumental diagnosis and treatment approaches of hypophysitis are discussed.

Prevalence and Clinical Course of Water and Electrolyte Disturbances Following Transsphenoidal Pituitary Adenoma Surgery in Immediate and Early Postoperative Period: A Prospective Observational Study.

Transsphenoidal pituitary adenoma surgery (TSS) was commonly associated with water and electrolyte disturbances (WEDs) in the postoperative period, which could lead to prolonged hospital stay, readmission and is rarely life threatening. The present study aimed to investigate the prevalence and predictive factors of WEDs following TSS. Fifty-eight patients with pituitary adenoma were prospectively studied for the occurrence of WEDs. Patients were checked at 6 weeks postoperatively for persistence of diabetes insipidus and new-onset hormone deficiencies or recovery. Multivariate regression was applied to determine predictive factors for the occurrence of WEDs. A total of 58 patients underwent TSS (median age: 43 years, 66% male). In the immediate postoperative period, 16 (27.6%) had transient diabetes insipidus (DI), two (3%) had transient DI followed by syndrome of inappropriate antidiuretic hormone (SIADH), five (8.6%) had isolated SIADH, five (8.6%) had persistent DI and only one patient had a triple-phase response. At 6 weeks, five (11%) patients continued to have persistent DI. In multivariate analysis, apoplexy and duration of surgery were predictive of DI occurrence. Recovery rate at 6 weeks was 11.1%, 13% and 9.3% for cortisol, thyroid and gonad axis, respectively. New-onset hormone deficiencies at 6 weeks were 5.6%, 5.6% and 7.4% for cortisol, thyroid and gonad axis, respectively. WEDs remain an important concern post-TSS. Timely follow-up should always be integral part of postoperative care for early diagnosis of new hormone deficiencies and avoiding unnecessary treatment in those with recovered axis.

Evaluation of postoperative fluctuations in plasma sodium concentration and triphasic response after pediatric craniopharyngioma resection: A French cohort study.

Disturbances in plasma sodium levels are a major complication following recent resections of craniopharyngiomas in children. They must be properly managed to avoid neurological sequelae. We aimed to describe the variations and characteristics of postoperative natremia in children who had undergone a first craniopharyngioma resection with a particular focus on the frequency of triphasic syndrome in these patients. Paediatric patients with craniopharyngiomas who underwent a first surgical resection in the neurosurgery department of the Hôpital Femme Mère Enfant (Lyon, France) between January 2010 and September 2021 were included in the present study and the medical records were analysed retrospectively. A total of 26 patients were included. Of these, 17 (65.4%) had a postoperative course characterised by the occurrence of both initial diabetes insipidus (DI) and hyponatremia a few days later. Eight patients (30.8%) presented then with isolated and persistent DI. Patients with the triphasic syndrome had a significantly higher grade of Puget classification on MRI (1 and 2), compared to the other patients. Dysnatremia is common after craniopharyngioma resections in children. This immediate postoperative complication is particularly difficult to manage and requires rapid diagnosis and prompt initiation of medical treatment to minimize fluctuations in sodium levels and avoid neurological sequelae.

Triphasic response after endoscopic craniopharyngioma resection and its dependency on infundibular preservation or sacrifice.

Surgery is the primary treatment for craniopharyngioma with the preservation of hypothalamic function of paramount importance. Infundibular preservation is debated, as maximal resection decreases recurrence rates but causes hypopituitarism. A triphasic response of diabetes insipidus (DI), syndrome of inappropriate antidiuretic hormone secretion (SIADH), and recurrent DI has been described after pituitary surgery, but the impact of infundibular preservation on the triphasic response following craniopharyngioma resection has not been well established. The authors' objective was to assess postoperative fluid and sodium balance and differences in ADH imbalance management following endonasal craniopharyngioma resection based on infundibular transection status. This is a retrospective cohort study of 19 patients with craniopharyngioma treated with endoscopic endonasal resection between 2014 and 2021. Resection was dichotomized into infundibular transection or preservation. Postoperative triphasic response, time to DI, and time to ADH replacement were compared using Fisher's exact test and Kaplan-Meier analysis. Based on surgeon impression, 10 patients had infundibular transection and 9 had infundibular preservation. Overall, 16 patients experienced DI, 12 experienced persistent DI, and 6 experienced SIADH. A postoperative triphasic response occurred in 40% (n = 4) of transection patients without preoperative DI and 11% (n = 1) of preservation patients without preoperative DI. The median time to postoperative DI (0.5 vs 18.0 hours, p = 0.022) and median time to ADH replacement therapy (4.5 vs 24 hours, p = 0.0004) were significantly shorter in the transection group than in the preservation group. Following endonasal craniopharyngioma resection, the triphasic response occurs in nearly half of infundibular transection cases. DI begins earlier with infundibular transection. On the basis of the study findings in which no patients met the criteria for SIADH or were endocrinologically unstable after postoperative day 6, it is reasonable to suggest that otherwise stable patients can be discharged at or before postoperative day 6 when ADH fluctuations have normalized and endocrinopathy is appropriately managed with oral desmopressin. Infundibular transection status may impact postoperative hormonal replacement strategies, but additional studies should evaluate their efficacies.

Endocrine Disorders in Children with Brain Tumors: At Diagnosis, after Surgery, Radiotherapy and Chemotherapy.

Brain tumors are the second most frequent type of all pediatric malignancies. Depending on their localization, patients with brain tumors may present neurological or ophthalmological symptoms, but also weight anomalies and endocrine disorders ranging from growth hormone deficiency, anomalies of puberty, diabetes insipidus to panhypopituitarism. Immediately at diagnosis, all patients with brain tumors require a complete assessment of the hypothalamic-pituitary function in order to address eventual endocrine disorders. Moreover, children and adolescents undergoing brain surgery must receive peri- and postoperative hydrocortisone stress therapy. Post-operative disorders of water homeostasis are frequent, ranging from transient diabetes insipidus, as well as syndrome of inappropriate antidiuretic hormone secretion to persistent diabetes insipidus. Late endocrine disorders may result from surgery near or within the hypothalamic-pituitary region. Pituitary deficits are frequent after radiotherapy, especially growth hormone deficiency. Thyroid nodules or secondary thyroid cancers may arise years after radiotherapy. Gonadal dysfunction is frequent after chemotherapy especially with alkylating agents. Early detection and treatment of specific endocrine disorders at diagnosis, perioperatively, and during long-term follow-up result in improved general and metabolic health and quality of life.

Abstract #1178145: A Rare case of Pituitary Apoplexy associated with COVID infection presenting with Central Diabetes Insipidus

Pituitary apoplexy (PA) is rare and occurs in up to 20% of patients with a non-functioning pituitary adenoma. It can occur de novo or can be precipitated by surgery, head trauma, anticoagulant therapy, and pregnancy among others. Incidence of persistent Diabetes Insipidus (DI) is only about 2% in such patients. Limited literature exists on the association of PA and COVID infection. We present a case of PA and subsequent central DI in a patient with a pituitary adenoma and severe COVID-19 pneumonia. A 64-year-old male with a history of a non-functioning pituitary macroadenoma (2 cm) of 5 months duration, primary hypothyroidism, and acute myeloid leukemia on chemotherapy presented to the emergency department with one day of headache, fever, and cough. He was diagnosed with COVID-19 pneumonia and started on dexamethasone, broad spectrum antibiotics and supplemental oxygen. Sudden loss of peripheral vision in his left eye with a persistent headache and altered mental status led to plain computed tomography that revealed a hyperattenuating suprasellar mass compressing the optic chiasm. MRI brain showed a T2 hypointense acutely hemorrhagic suprasellar mass compressing the infundibulum and the optic chiasm indicative of PA. Labs showed thrombocytopenia with platelet count 55,000/mcL (130-400), low AM cortisol 3 mcg/dL, normal prolactin 10 ng/mL, normal thyroid function and preserved somatotrophic axis. In addition, the patient developed polyuria with a urine output of up to 8 liters in a 24-hour period, acute hypernatremia (sodium 172 mmol/L), urine specific gravity < 1.005 and serum osmolality 360 mOsm/kg (275-295 mOsm/kg). Conservative management with stress dose hydrocortisone and desmopressin was the first line of therapy. Levothyroxine was continued at home dose. After resolution of COVID-19 pneumonia and improvement in electrolyte disarray, the patient underwent pituitary decompression surgery in 3 weeks with subsequent resolution of DI post-operatively. He was discharged home on hydrocortisone (20 mg total daily dose) and levothyroxine with close endocrinology follow up. Association of COVID infection and PA has been reported in 10 patients in the literature so far. Although the exact mechanism is unknown, SARS-CoV-2 neural invasion via cerebral ACE-2 expression can lead to oxidative stress and thrombogenesis, ultimately predisposing to neural hemorrhage. Thrombocytopenia secondary to chemotherapy can be a contributing factor in our patient. DI is rare and usually resolves after decompression surgery. Further research focusing on the association and pathophysiology of COVID-19 with PA in those without precipitating factors are needed.

Abstract 72: Prevalence, course, and predictive factors of water and electrolyte disturbances (WEDs) following transsphenoidal pituitary surgery (TSS) in patients with pituitary adenoma

Background: TSS is commonly associated with various WEDs in postoperative period, which can lead to prolonged hospital stay, readmission and rarely be life threatening. There is scarcity of prospective studies regarding various factors which could predict occurrence of these disturbances.Aims and Objectives: To prospectively study the prevalence, presentation and clinical course of WEDs following TSS in patients with pituitary adenoma and assess potential risk factors for these disturbances.Results: Forty patients (median age 45.5 years, 60% male) harbored 25 clinically hormone inactive and 15 hormone secreting pituitary adenoma. Postoperative WEDs occurred in 45 % of the patients; in 22.5 % as transient diabetes insipidus (DI) %, in 5 % as biphasic response, in 5% as isolated SIADH, in 12.5% as persistent DI. Median day of onset of DI was day 2 and remission was day 4. Median day of onset of SIADH was day 5 and remission was day 9. Younger age and greater adenoma volume were associated with increased risk of DI.Conclusion: WEDs are common following TSS. Diabetes insipidus was more common than SIADH.

Plasma sodium and potassium concentrations after hypophysectomy in dogs with corticotroph adenomas.

BackgroundElectrolyte abnormalities, especially hypernatremia, are frequent complications after transsphenoidal hypophysectomy in dogs with pituitary‐dependent hypercortisolism.ObjectivesTo describe electrolyte abnormalities after transsphenoidal hypophysectomy and to investigate possible associations between postoperative hypernatremia and clinical and surgical variables as well as with postoperative outcome.AnimalsOne hundred and twenty‐seven client‐owned dogs.MethodsDogs with pituitary corticotroph adenomas that underwent transsphenoidal hypophysectomy were retrospectively included. Plasma sodium and potassium concentrations were measured −2, +2, +8, +24, and +48 hours from hypophysectomy. Clinical (breed, age, body weight, skull type, urinary cortisol/creatinine ratio, percentage of suppression to dexamethasone) and surgical variables (duration of anesthesia and surgery, pituitary dimensions) were compared to the development of hypernatremia.ResultsPostoperative hypernatremia developed in 46.5% (57/127) of dogs and hyponatremia in 6.3% (8/127). Plasma sodium concentration increased after surgery and peaked at 8 hours after surgery, normalizing after 24 to 48 hours. Plasma potassium concentration increased without exceeding the reference limit.No significant associations were found between clinical and surgical variables and hypernatremia, or between hypernatremia and postoperative death, long‐term survival or recurrence.Surgery time was significantly longer in dogs that developed persistent diabetes insipidus (P = .02) and persistent diabetes insipidus occurred more frequently in dogs with enlarged pituitary glands (P = .01).Conclusion and Clinical ImportanceHypernatremia remains a frequent postoperative complication after transsphenoidal hypophysectomy but did not appear to have an impact on postoperative outcome. No predisposing factor to postoperative hypernatremia was identified. Variations in plasma potassium concentrations do not seem to influence postoperative outcome.

Primary Amenorrhea and Hyperandrogenism: Presenting Features of a Growth Hormone Producing Pituitary Adenoma in a Female Adolescent

Background: The combination of obesity, metabolic syndrome, hyperandrogenism and amenorrhea can be a common presentation of conditions such as polycystic ovarian syndrome. Growth hormone (GH) secreting pituitary adenomas are rare in children, but can present with a similar picture. Clinical Case: A 15-year 4-month old female was evaluated for primary amenorrhea and metabolic syndrome. Her weight was +2.96 SD, height +3.0 SD (mid-parental height 44th percentile, -0.1 SD) and BMI +2.53 SD, all >99th percentile. Weight and BMI were >99th percentile (weight +3.0 SD, BMI +2.4 SD) since 11 years of age and height was >99th percentile (+ 2.7 SD) since 8 years of age. Physical exam was remarkable for acanthosis, deepened voice, no hirsutism, Tanner III breasts and Tanner IV pubic hair. Laboratory evaluation was notable for prolactin (PRL) 147.8 ng/dL (2.6-18.0), LH <0.02 mIU/mL, FSH <0.09 mIU/mL, estradiol 23 pg/mL, total testosterone 81 ng/dL (<41), androstenedione 673 ng/mL (43-180), DHEA-S 627 mcg/dL (42-162), dyslipidemia and normal 17 hydroxy-progesterone and thyroid function tests. Bone age was 16 years. Brain MRI showed a 15x7mm pituitary macroadenoma without suprasellar invasion. Pituitary evaluation revealed IGF-1 619 ng/mL (192-568), IGF-BP3 5.15 mg/L (2.64-6.43) and adrenal insufficiency. Treatment with cabergoline 0.5 mg/week and maintenance hydrocortisone were initiated. An OGTT demonstrated baseline GH 4.9 ng/mL (0-6) and nadir 3.62 ng/mL (normal <1) consistent with GH excess. Surgery for the pituitary adenoma was recommended but unfortunately postponed. Four months later, her BP was elevated, she had clinical signs of diabetes insipidus (DI), PRL was 43.2 ng/dL and she failed a repeat GH suppression test. Follow-up MRI showed pituitary mass enlargement (17x9mm). Patient underwent total trans-sphenoidal tumor resection. Pathology confirmed a sparsely granulated (SG) GH adenoma immunoreactive for GH, PRL and estrogen receptor. Post-operative OGTT showed adequate GH suppression, normal prolactin levels, persistent testosterone elevation and DI. Conclusions: Hyperprolactinemia, hyperandrogenism and metabolic syndrome could be the presenting features of pituitary GH adenomas even in the absence of acromegalic features. Specifically, SG pituitary adenomas are frequently seen in young adults, are larger, more invasive and less likely to respond to medical treatment. Timely diagnosis and surgical treatment of GH adenomas is essential to prevent the high morbidity and mortality.

Diabetes insipidus and syndrome of inappropriate antidiuresis (SIADH) after pituitary surgery: incidence and risk factors.

Electrolyte disorders are relatively frequent and potentially serious complications after pituitary surgery. Both DI (diabetes insipidus) and SIADH (syndrome of inappropriate antidiuresis) can complicate and prolong hospital and intensive care unit stay, and the latter may even be preventable. We aim to assess the incidence of both electrolyte disorders and their risk factors. From a prospective registry of patients who underwent endoscopic transnasal transsphenoidal surgery (TSS) for pituitary adenoma, patients with postoperative DI and SIADH were identified. Univariable and multivariable statistics were carried out to identify factors independently associated with the occurrence of either DI or SIADH. A total of 174 patients were included, of which 73 (42%) were female. Mean age was 54years (range 20-88). During postoperative hospital stay, 13 (7.5%) patients presenting with DI and 11 (6.3%) with SIADH were identified. Patients who developed DI after surgery had significantly longer hospital stays (p = 0.022), as did those who developed SIADH (p = 0.002). Four (2.3%) patients were discharged with a diagnosis of persistent DI, and 2 (1.1%) with the diagnosis of SIADH. At the last follow-up, 5 (2.9%) patients presented with persistent DI, while none of the patients suffered from SIADH. Younger age (odds ratio (OR) 0.97, 95% confidence interval (CI) 0.94-1.01, p = 0.166) and pituitary apoplexy (OR 2.69, 95% CI 0.53-10.65, p = 0.184) were weakly associated with the occurrence of DI. We identified younger age (OR 0.96, 95% CI 0.92-0.99, p = 0.045) and lower preoperative serum sodium (OR 0.83, 95% CI 0.71-0.95, p = 0.008) as independent risk factors for SIADH. Although we found a weak association among age, pituitary apoplexy, and the occurrence of DI, no independent predictor was identified for DI. For postoperative SIADH however, lower age and preoperative serum sodium were identified as significant predictors. None of these findings were sufficiently supported by preexisting literature. Both electrolyte disorders are exquisitely hard to predict preoperatively, and further research into their early detection and prevention is warranted.

Postoperative Hormonal Outcomes in Patients with Large and Giant Non-functioning Pituitary Adenomas.

To study hormonal axis (HA) dysfunction pre-operatively and at three months after surgery in patients with large (>3 cms) (Hardy's grade C) and giant (>4 cms) nonfunctioning pituitary adenomas (NFPA). One hundred thirty nine patients operated between 2006 and 2017, with 3 months post-operative hormonal evaluation, were included in this retrospective study. HA damage was categorized as 0 to 3 based on number of axes (thyrotroph, corticotroph and gonadotroph) that were affected. Risk factors studied for HA dysfunction before and after surgery included duration of symptoms, size of tumor, diabetes mellitus, hypertension and extent of resection. Preoperatively 45 (32.3%) had no axis involvement, 34 (24.4%), 36 (25.8%) and 24 (17.2%) had one, two and three axes involvement respectively. Thyrotroph axis was affected in most patients. Tumor volume had significant correlation with preoperative pituitary dysfunction (P < 0.000). Post-operatively HA function remained same in 100 (72%), improved in only 7 (5%) and worsened in 32 (23%) of the patients. Of the 3 HA, corticotroph function worsened in most patients. None of the patients who had dysfunction in all three axes had improvement after surgery. No significant risk factors were associated with post-operative pituitary function outcomes. Persistent diabetes insipidus was noted in six (4.3%) patients. Pre-operatively anterior pituitary dysfunction is noted in nearly two-thirds of patients with large and giant NFA. Tumor volume >15 cc had significant correlation with pre-operative panhypopituitarism. Post-operatively, pituitary function remains the same in nearly three quarters of the patients. No significant risk factors were found for post-operative hormonal outcomes.

Endoscopic Endo-nasal Trans-Sphenoidal Approach for Pituitary Adenomas: A Prospective Study.

The surgical approach for hypophysectomy has undergone sweeping revolution in the past three decades. With the advent of endoscopes, better instrumentation, better illumination and viewing cameras, endoscopic endo-nasal trans-sphenoidal approach to sella has now largely become the norm. The aim of this study is to present our experience, analysing the surgical outcomes of this approach in patients with pituitary adenoma, pertaining to entirety of tumor removal, alleviation of symptoms and rate of complications. This prospective studywas conducted at our tertiary health care centre from June 2012 to June 2015. A total of 14 patients, meeting the inclusion criteria, underwent endoscopic trans-sphenoidal hypophysectomy for pituitary adenoma. Age of presentation ranged from 19 to 73years (mean 43.6years). 9 patients were female and 5 were male. The most common presenting symptom was headache, followed by visual disturbances. Amongst those with hormonal imbalance, most common were prolactinomas and growth hormone secreting adenomas. Preoperative MRI brain showed macroadenomas in all 14 patients. 7 (50%) patients had suprasellar extension, while 5 (36%) patients had intracavernous extension too. CT paranasal sinuses provided the roadmap for surgery by identifying anatomical variations. Alleviation of headache occurred in all cases. Normalization of altered hormonal profile was seen in all cases. 83.3% of our patients with visual field defects on perimetry showed improvement post-surgery. Recidivism was directly related to the size and extent of adenoma. Transient diabetes insipidus was seen in 4 (28%) cases. Persistent diabetes insipidus occurred in 1 (7%) patient. CSF leak was seen in 2 (14%) patients, 1 (7%) patient developed postoperative meningitis. Most common nasal complication was excessive crusting. There was no incidence of any vascular complications, focal neurological deficit or hypopituitarism in our study. The pure endoscopic approach is a safe, efficacious, and minimally invasive technique for the removal of pituitary adenomas. The results have been encouraging in our prospective study. However, the importance of learning curve in endoscopic skull base surgery and use of a multi-disciplinary collaboration cannot be overemphasized.

Long-term follow-up of female prolactinoma patients at child-bearing age after transsphenoidal surgery

Prolactinoma is the most common subtype of functional pituitary adenoma. Effective therapy is required for women of child-bearing age to achieve remission at serum prolactin level and regain reproductive function. To evaluate long-term outcomes, including menstrual recovery, after transsphenoidal surgery on female prolactinoma patients by experienced neurosurgeons. Observational study. Consecutive female prolactinoma patients aged from 16 to 45 years were included. Histological analysis after surgery revealed adenoma with positive staining for prolactin. Plurihormonal cell adenomas were excluded. Clinical manifestations, surgical indications, serum prolactin level before and after surgery, surgical complications, pituitary fuctions, drug maintenance, the status of menstruation, and pregnancy. Sixty-three consecutive female patients with a mean age of 29.5 ± 1.1 years were included. Based on magnetic resonance imaging findings before surgery, 31 (49.2%) patients had microadenoma, and the remaining 32 (50.8%) had macroadenoma. The median follow-up after transsphenoidal surgery was 53 (33-74) months, and long-term surgical remission was achieved in 50 (79.37%) patients with 28 (90.32%) microadenomas and 22 (68.75%) macroadenomas. No meningitis or persistent cerebrospinal fluid leaks occurred. Only one case suffered from persistent diabetes insipidus at follow-up. No severe pituitary dysfunction was observed in microprolactinoma patients. Of patients with menstrual disorders, 85% regained regular menstrual cycles after surgery. Nineteen patients in this cohort desired pregnancy and 15 of them successfully gave birth after surgery. All 17 microadenoma patients with modern surgical indications achieved normal prolactin levels and regular menstrual cycles with only one patient on drug therapy at follow-up. Long-term follow-up showed a high remission rate in female prolactinoma patients, especially in microadenoma patients, after surgery. Transsphenoidal surgery performed by experienced neurosurgeons may offer a valuable approach to treat female microprolactinoma patients of child-bearing age with modern indications for surgery.

Gross Total Resection of Chordoid Glioma of the Third Ventricle via Anterior Interhemispheric Transcallosal Transforaminal Approach at Two Stages

Suprasellar tumors in particular tumors located in the retrochiasmatic area and anterior third ventricle are challenging cases in terms of optimal surgical exposure. Several approaches have been described including transsylvian, translamina terminalis, endoscopic endonasal, and anterior interhemispheric. Each approach has advantages and disadvantages. In this video, we present a case of retrochiasmatic anterior third ventricular tumor that was operated via anterior interhemispheric transcallosal transforaminal approach. The patient is a 42-year-old female who presented with sudden onset of severe headache and depressed level of consciousness. Computed tomography (CT) scan of the head showed a hemorrhage in the third ventricle and suprasellar cisterns. CT angiogram and magnetic resonance imaging (MRI) confirmed diagnosis of hemorrhagic mass lesion in the third ventricle. Upon further questioning of her family, we found out that she was having excessive urination and short-term memory problems for last 2 weeks. First, ventriculostomy was placed for obstructive hydrocephalus. She then underwent surgical resection via anterior interhemispheric transcallosal transforaminal approach. Foramen of Monro was enlarged by performing transchoroidal dissection. Using transforaminal route, tumor was resected. Due to the narrow surgical corridor and high vascularity of the tumor, decision was made to come back at a second stage. Using same surgical approach, in the second stage, gross total resection was performed. Postoperative MRI confirmed gross total resection. Histopathology was chordoid glioma of the third ventricle. She made excellent recovery with persistent diabetes insipidus. Currently, she is completing radiation therapy. In this video, we demonstrate techniques and pitfalls of anterior interhemispheric transcallosal approach to anterior third ventricular tumor. The link to the video can be found at: https://youtu.be/CI5c6Zup8sY .

Endoscopic endonasal approach for craniopharyngioma: the importance of the relationship between pituitary stalk and tumor.

OBJECTIVE The endoscopic endonasal approach (EEA) is commonly used for the treatment of craniopharyngioma; therefore, it is essential to analyze outcomes in order to understand the benefits and drawbacks. The goal of this paper was to evaluate the clinical features and outcomes associated with this treatment approach. METHODS From July 2010 to March 2016, 82 adult craniopharyngioma patients underwent an EEA at the authors' institution. Of these cases, intraoperative records and immediate postoperative MR images were available for 68 patients. The patients underwent systemized endocrinological evaluation. Eighteen of 68 patients who underwent EEA for recurrence or regrowth of residual lesions after previous surgical management were excluded in the analysis of the anatomical tumor classification. The authors retrospectively analyzed preoperative clinical features and previous anatomical classifications, focusing on the relationship of the pituitary stalk and tumor, to determine predictive factors for the clinical outcome, such as the extent of resection, visual function, endocrinological function, recurrence rate, and complications. RESULTS The mean tumor size was 2.5 cm (3.1 cm for primary tumors and 1.9 cm for recurrent lesions). Gross-total resection (GTR) was achieved in 62 (91.1%) patients (48 [96.0%] patients with primary tumors and 14 [77.8%] patients with recurrent tumors). The rate of GTR was higher in the primary group than in the group with recurrence (p = 0.038). The overall pre- and postoperative visual impairment scale (VIS) scores were 40.8 and 22.1, respectively (50.9 and 14.3 in the primary group and 30.7 and 29.9 in patients with recurrence, respectively). The improvement rate in VIS score was higher in the primary group than in the recurrent group (p = 0.001). Endocrinological function was improved in 4 patients (5.9%) and deteriorated in 32 of 68 patients (47.1%). Tumor invasion into the center of the pituitary stalk affected the postoperative outcomes most significantly. Cognitive dysfunction was observed in 22 patients before surgery and improved in 20 patients (90.9%) after surgery. Hydrocephalus was found in 7 patients and resolved after surgery in all cases. CSF leakage occurred in 2 (2.9%) of 68 patients and was repaired by revision surgery in both patients. Ten patients without CSF leakage also received antibiotics for the treatment of meningitis. The infection rate was higher in the recurrent group. Postoperative endocrinological evaluation showed no deficits in 12 patients and panhypopituitarism in 55 patients. The remaining patient had growth hormone deficiency. Forty-three patients had new-onset diabetes insipidus, and 1 patient had persistent diabetes insipidus after surgery. There were 2 (2.9%) cases of recurrence during the mean 30.7-month follow-up period; one patient underwent radiosurgery and the other underwent reoperation. CONCLUSIONS The EEA resulted in excellent surgical outcomes and acceptable morbidity rates, regardless of the anatomical location of the tumor. Invasion of the craniopharyngioma into the center of the pituitary stalk has strong predictive power for postoperative endocrinological outcome.

Effect of Preserving the Pituitary Stalk During Resection of Craniopharyngioma in Children on the Diabetes Insipidus and Relapse Rates and Long-Term Outcomes.

The objective of this study was to investigate the effect of preserving an infiltrated pituitary stalk during the resection of craniopharyngioma of pituitary stalk origin on postoperative outcomes and thus provide a theoretical basis for microsurgical treatment and prognosis. We screened the clinical data of all 103 pediatric patients with craniopharyngioma undergoing surgical treatment at our department between January 2006 and January 2013 and conducted a retrospective analysis of 82 patients with craniopharyngioma originating in the pituitary stalk. The patients were followed up from 12 months to 8 years. We analyzed the effect of preserving the pituitary stalk on the early and persistent diabetes insipidus rates, postoperative relapse rate, and mortality. In the total resection group (n = 67), the early and persistent diabetes insipidus rates were significantly lower in the 46 patients (68.7%) with a pituitary stalk than in those whose pituitary stalk was removed (P < 0.05); no significant difference was observed in the relapse rate between the 2 subgroups (P > 0.05). In the subtotal resection group (n = 15), a significant difference was observed in the early and persistent diabetes insipidus rates (P < 0.05), but no significant difference was observed in the relapse rate between the patients with a pituitary stalk and those whose pituitary stalk was removed (P > 0.05). For children with craniopharyngioma of pituitary stalk origin, preserving the pituitary stalk has a significant effect on the early and persistent diabetes insipidus rates. When intraoperative exploration showed excessive adhesion between the tumor and pituitary stalk, we opted to preserve the pituitary stalk, which significantly reduced the early and persistent postoperative diabetes insipidus rates, without significantly increasing the relapse or mortality rate.

Microscopic Transnasal Transsphenoidal Surgery for Pediatric Pituitary Adenomas

Microscopic transnasal transsphenoidal surgery (MTTS) has been a time-confirmed effective treatment for pituitary adenomas; however, the data supporting its use in pediatric patients is limited. In this study, the authors summarize the authors' 5-year experience of pituitary adenomectomy via MTTS in a pediatric population. The authors retrospectively reviewed the medical records of 35 patients aged ≤18 years who underwent microscopic transnasal transsphenoidal approach for pituitary adenomas between January 2011 and December 2015. Age, sex, symptoms, tumor size, extent of tumor resection, surgical outcomes, and complications were reviewed. All patients underwent MTTS 39 times. Total resection was obtained in 31 (88.6%) patients, while subtotal resection was achieved in 4 (11.4%) and no patient had partial resection. For the 21 patients with preoperative visual impairment, 18 (85.7%) resolved and 3 (14.3%) obtained subjective improvement. Of the 33 patients with preoperative hyperhormonal levels, endocrine function reduced to normal reference range in 28 (84.8%), decreased in 3 (9.1%) and 2 (6.1%) patients had no change. Diabetes insipidus occurred in 4 (11.4%) patients, of whom 1 patient (2.9%) developed persistent diabetes insipidus that was treated with Minirin. One patient incurred postoperative cerebrospinal fluid leakage that was resolved by lumbar drainage. Hypopituitarism occurred in 3 patients, of whom 1 patient developed permanent hypopituitarism that required hormone replacement therapy. There were no patients of death, intracranial hematoma, or meningitis. In conclusion, MTTS is a safe and effective surgical option for pediatric pituitary adenomas with minimal morbidity and excellent outcomes.

Achieving a cure for hypothalamic hamartomas: aSisyphean quest?

Achieving a cure for hypothalamic hamartomas: aSisyphean quest?