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diabetes insipidus [keywords]
- Central diabetes insipidus: alert for dehydration in very low birth weight infants during the neonatal period. A case report. [JOURNAL ARTICLE]
- Sao Paulo Med J 2014 Sep 26.:0.
Central diabetes insipidus (CDI) is a rare cause of hypernatremia during the neonatal period. The diagnosis is particularly difficult in very low birth weight (VLBW) newborns.We report on a preterm newborn who presented CDI soon after birth. On the third day of life, signs of dehydration were present despite normal fluid supply. The diuresis rate was 4.4 ml/kg/h. Although the fluid supply was then increased, the dehydration continued, with hypernatremia, normal glycemia, diuresis of 7.4 ml/kg/h and urine density of 1005 mOsmol/l. Thus, a diagnostic hypothesis of diabetes insipidus was raised. A test with a nasal vasopressin analogue (dDAVP) was performed and CDI was confirmed. Reduction of the fluid supply became possible through appropriate treatment.The diagnosis of CDI is rarely made during the neonatal period, especially in VLBW newborns, because of the difficulty in detecting elevated diuresis. Persistent hypernatremia, usually accompanied by hyperthermia despite abundant fluid supply, weight loss and low urine osmolality are important signs of alert.
- Endoscopic endonasal transsphenoidal surgery of 1,166 pituitary adenomas. [JOURNAL ARTICLE]
- Surg Endosc 2014 Oct 1.
To report the results of a series of patients undergoing pure endoscopic endonasal pituitary surgery and to evaluate the efficacy and safety of this procedure.The data of 1,166 patients that underwent endoscopic endonasal transsphenoidal adenoma removal between December 2006 and June 2013 were retrospectively reviewed. Pre- and postoperative hormonal status (3 months after surgery) were analyzed and compared with the clinical parameters originally presented by the patients. The incidences of tumor removal, hormonal control, and tumor removal complications were retrospectively analyzed.Out of 577 nonfunctioning adenomas, 180 were growth hormone (GH) secreting, 308 prolactin (PRL) secreting, 26 mixed GH/PRL adenomas, 68 adrenocorticotropin secreting, and 7 thyroid-stimulating hormone-secreting adenomas. The gross total removal of pituitary adenomas was achieved in 98 % of microadenomas, 92 % of macroadenomas, and 76 % of giant adenomas. Hormonal control was achieved in 47 (69 %) cases of ACTH adenomas, 119 (66 %) GH adenomas, 262 (85 %) PRL adenomas, and 6 (86 %) TSH adenomas. Postoperative complications were observed in 168 (14.4 %) patients. The most frequent complications were diabetes insipidus (7 %), epistaxis (1.7 %), hyposmia (1.5 %), anterior lobe insufficiency (1.3 %) ,and CSF leaks (0.6 %).The pure endoscopic approach is a safe, efficacious, and minimally invasive technique for the removal of pituitary adenomas. A higher gross total resection rate is vital for non-functional and functional adenomas. For patients with functional adenomas, while hormonal remission is unlikely to be achieved by surgery, the use of adjuvant therapy is advocated to obtain long-term hormonal control.
- Expanding indications for the extended endoscopic endonasal approach to hypothalamic gliomas: preliminary report. [Journal Article]
- Neurosurg Focus 2014 Oct; 37(4):E11.
Object In the last decade the indications for the endoscopic endonasal approach have been progressively expanded to include lesions that have not been traditionally considered amenable to resection through the transsphenoidal route. In this study, the authors analyze their experience with hypothalamic gliomas treated via the endoscopic endonasal approach. Methods Consecutive cases of hypothalamic gliomas treated since 2007 via an endoscopic endonasal approach were reviewed. Preoperative and postoperative neuroimaging as well as endocrinological, neurological, and visual symptoms were analyzed to assess the surgical outcome. Signs and symptoms of hypothalamic dysfunction including body mass index (BMI), memory, sleep-wake rhythm, and polyphagia were prospectively collected pre- and postoperatively to assess hypothalamic function. Quality of life was evaluated using the Katz scale. Results In the initial phase the endoscopic endonasal approach was adopted in 3 cases with a palliative intent, to obtain a biopsy sample or for debulking of the mass followed by radio- or chemotherapy. In 2 later cases it was successfully adopted to achieve gross-total tumor resection. Complications consisted of 2 postoperative CSF leaks, which required an endoscopic endonasal reintervention. Visual deficit improved in 3 cases and normalized in the other 2. Four patients developed diabetes insipidus, and 3 an anterior panhypopituitarism. All patients had a moderate increase in BMI. No patients presented with any other signs of hypothalamic damage, and their quality of life at follow-up is normal. Conclusions Despite the limitations of a short follow-up and small sample, the authors' early experience with the endoscopic endonasal approach has revealed it to be a direct, straightforward, and safe approach to third ventricle astrocytomas. It allowed the authors to perform tumor resection with the same microsurgical technique: dissecting the tumor with 2 hands, performing a central debulking, and controlling the bleeding with bipolar coagulation. The main limitations were represented by some anatomical conditions, such as the position of the chiasm and the anterior communicating artery complex and, finally, by the challenge of watertight plastic repair. To definitively evaluate the role of this approach in hypothalamic gliomas, a comparison with transcranial series would be necessary, but due to the rarity of these cases such a study is still lacking. The authors observed that more aggressive surgery is associated with a worse endocrinological outcome; thus they consider it to be an open question (in particular in prepubertal patients) whether radical removal is an advisable goal for hypothalamic gliomas.
- Endoscopic endonasal transsphenoidal surgery: implementation of an operative and perioperative checklist. [Journal Article]
- Neurosurg Focus 2014 Oct; 37(4):E1.
Endoscopic endonasal surgery relies heavily on specialized operative instrumentation and optimization of endocrinological and other critical adjunctive intraoperative factors. Several studies and worldwide initiatives have previously established that intraoperative and perioperative surgical checklists can minimize the incidence of and prevent adverse events. The aim of this article was to outline some of the most common considerations in the perioperative and intraoperative preparation for endoscopic endonasal transsphenoidal surgery. The authors implemented and prospectively evaluated a customized checklist at their institution in 25 endoscopic endonasal operations for a variety of sellar and skull base pathological entities. Although no major errors were detected, near misses pertaining primarily to missing components of surgical equipment or instruments were identified in 9 cases (36%). The considerations in the checklist provided in this article can serve as a basic template for further customization by centers performing endoscopic endonasal surgery, where their application may reduce the incidence of adverse or preventable errors associated with surgical treatment of sellar and skull base lesions.
- Lymphocytic hypophysitis associated with pediatric multiple sclerosis. [Journal Article]
- Pediatr Neurol 2014 Oct; 51(4):580-2.
Lymphocytic hypophysitis (LH) is a rare inflammatory disorder of the pituitary gland and infundibulum most often observed in the setting of autoimmune disease with a variety of clinical and endocrine presentations. The association between lymphocytic hypophysitis and multiple sclerosis has not been reported.We describe an adolescent boy with polyfocal neurological signs including optic neuritis as well as hypopituitarism and diabetes insipidus related to lymphocytic hypophysitis. His imaging met 2010 McDonald criteria for multiple sclerosis. This diagnosis was further supported by cerebrospinal fluid analysis and a negative evaluation for an alternate diagnosis. Imaging features of lymphocytic hypophysitis include an absent posterior pituitary bright spot with an enlarged cystic pituitary gland, stalk thickening, and improvement with corticosteroid therapy.Lymphocytic hypophysitis and multiple sclerosis share a common autoimmune pathogenesis, perhaps explaining the co-occurrence of the diseases. The presentation of endocrinologic disturbances such as diabetes insipidus with typical features of a multiple sclerosis attack should prompt further investigation of possible comorbid inflammatory disease involving the hypothalamic-pituitary axis.
- [Extradural temporopolar approach for surgical management of paraclinoid lesions]. [English Abstract, Journal Article]
- No Shinkei Geka 2014 Oct; 42(10):907-16.
<i>Objective</i>:Surgical treatment of paraclinoid tumors adjacent to important anatomical structures, such as the optic nerve, optic chiasm, pituitary stalk, and internal carotid artery, should emphasize maximum resection and preservation of visual function. Thus, early localization and exposure of the optic nerve, and complete mobilization and decompression of the optic nerve and internal carotid artery, are necessary in order to prevent intraoperative neurovascular injuries. However, this technique requires wide exposure of the optic-carotid space through the interpeduncular cistern. We have developed an extradural temporopolar approach for resection of paraclinoid tumors, which can also allow early devascularization of arteries that feed the tumors. We evaluated the surgical outcomes of this approach, paying special attention to clinical and visual outcomes.<br/> <i>Patients and Methods</i>:Thirteen patients(4 men, 9 women;24-78 years, mean age 54 years), underwent tumor removal using an extradural temporopolar approach between March 2000 and April 2013. We retrospectively reviewed medical charts, radiological findings, intensive care unit flow sheets, and surgical records.<br/> <i>Results</i>:Histological diagnoses included craniopharyngioma(4 cases), pituitary adenoma(3 cases), medial sphenoid ridge meningioma(2 cases), tuberculum sellae meningioma(2 cases), trigeminal neurinoma(1 case), and malignant epidermoid(1 case). Tumors were 1.5-6.0cm in length(mean 3.2cm). Tumors were cystic in all 4 craniopharyngiomas and solid in the other 9 cases. Preoperative neurological deficits included visual disturbance in 10 patients, double vision caused by oculomotor nerve palsy in 2 patients, and vertigo in 1 patient. Additional orbitozygomatic craniotomies were performed in 8 patients. Total tumor removal was performed in 7 patients and subtotal removal performed in 6. The pituitary stalk was microscopically preserved in all patients. Postoperative complications included transient diabetes insipidus in 2 patients, chronic subdural hematoma in 1 patient, and abducens palsy in 1 patient. All complications were completely resolved. Surgical outcomes on discharge were recorded as follows:good recovery(11 patients), moderate disability(1 patient), and severe disability(1 patient, who suffered preoperative consciousness disturbance). Visual outcomes were improved in 7 patients, unchanged in 5, and worsened in 1, with recurrence.<br/> <i>Conclusions</i>:The present extradural temporopolar approach, which allows early decompression of the optic nerve, and early devascularization and detachment of the tumor, may lead to greater tumor resection and improved visual outcomes in patients with paraclinoid tumors.
- Diabetes insipidus in pediatric germinomas of the suprasellar region: characteristic features and significance of the pituitary bright spot. [JOURNAL ARTICLE]
- J Neurooncol 2014 Sep 30.
The pituitary bright spot is acknowledged to indicate functional integrity of the posterior pituitary gland, whilst its absence supports a diagnosis of central diabetes insipidus (DI). This feature was evaluated, together with the incidence and clinical characteristics of DI in children with suprasellar/neurohypophyseal germinomas. We performed a review of all suprasellar (SS) or bifocal (BF) germinoma pediatric patients treated in Toronto since 2000. Demographics, symptomatology, treatment outcome and imaging were evaluated. Nineteen patients fulfilled inclusion criteria (10 SS, 9 BF; median age 12.5 years (6.2-16.8 years)). All remained alive at 6.4 years median follow-up (1.2-13.7 years) after receiving chemotherapy and radiotherapy (13 focal/ventricular, four whole brain, two neuraxis), with only one progression. All had symptoms of DI at presentation with a symptom interval above one year in eight cases (42 %). Desmopressin was commenced and maintained in 16 patients (84 %). The pituitary bright spot was lost in most diagnostic interpretable cases, but was appreciated in three patients (18 %) who had normal serum sodium values compared to 'absent' cases (p = 0.013). For two such cases, spots remained visible until last follow-up (range 0.4-3.3 years), with one still receiving desmopressin. No case of bright spot recovery was observed following therapy. Protracted symptom intervals for germinoma-induced central DI may reflect poor clinical awareness. Explanations for persistence of the pituitary bright spot in symptomatic patients remain elusive. Desmopressin seldom reverses the clinical features of germinoma-induced DI to allow discontinuation, nor does treatment cause bright spot recovery.
- The Case | Cystic renal disease, nephrogenic diabetes insipidus, and polycytemia. [Journal Article]
- Kidney Int 2014 Oct; 86(4):863-4.
- Complications following primary and revision transsphenoidal surgeries for pituitary tumors. [JOURNAL ARTICLE]
- Laryngoscope 2014 Sep 27.
This study aimed to determine the incidence of major complications following both primary and revision transsphenoidal pituitary surgery. Major complications included endocrinopathic, skull base, orbital, hemorrhagic and thromboembolic complications, respiratory failure, and death. Secondarily, this study aimed to examine factors associated with the occurrence of complications.Retrospective cohort analysis of California and Florida all-payer databases from 2005 to 2008.The major complication rate following both primary and revision transsphenoidal pituitary surgery was calculated. Bivariate analyses were performed to investigate the relationship of patient characteristics with complication occurrence, and a multivariate model was constructed to determine risk factors associated with these complications.There were 5,277 primary cases and 192 revision cases that met inclusion criteria. There was a nonsignificant absolute difference of 3.09% (95% confidence interval [CI]: -11.00 to 16.14) between the rate of complications following primary (n = 443, 8.39%) and revision (n = 22, 11.46%) surgeries. Multivariate analyses showed that patients with Medicare (odds ratio [OR]:1.74, 95% CI: 1.17 to 2.61), Medicaid (OR: 2.13, 95% CI: 1.59 to 2.86), or a malignant neoplasm (OR: 3.10, 95% CI: 1.62 to 5.93) were more likely to have complications.The rate of major complications following transsphenoidal pituitary surgery is lower than earlier retrospective reports. The overall complication rate following revision surgery was not significantly different from primary surgery. Insurance status and a diagnosis of a malignant neoplasm were associated with a higher rate of complications.2C Laryngoscope, 2014.
- Congenital central diabetes insipidus and optic atrophy in a Wolfram newborn: is there a role for WFS1 gene in neurodevelopment? [JOURNAL ARTICLE]
- Ital J Pediatr 2014 Sep 26; 40(1):76.
BackgroundWolfram syndrome (WS) is an autosomal recessive neurodegenerative disorder characterized by diabetes mellitus (DM), optic atrophy (OA), central diabetes insipidus (CDI) and deafness (D).The phenotype of the disease has been associated with several mutations in the WFS1 gene, a nuclear gene localized on chromosome 4. Since the discovery of the association between WFS1 gene and Wolfram syndrome, more than 150 mutations have been identified in WS patients.We previously described the first case of perinatal onset of Wolfram syndrome newborn carrying a segmental uniparental heterodysomy affecting the short arm of chromosome 4 responsible for a significant reduction in wolframin expression.Here we review and discuss the pathophysiological mechanisms that we believe responsible for the perinatal onset of Wolfram syndrome as these data strongly suggest a role for WFS1 gene in foetal and neonatal neurodevelopment.Case presentationWe described a male patient of 30 weeks¿ gestation with intrauterine growth restriction and poly-hydramnios.During the first days of life, the patient showed a 19% weight loss associated with polyuria and hypernatremia. The presence of persistent hypernatremia (serum sodium 150 mEq/L), high plasma osmolarity (322 mOsm/L) and low urine osmolarity (190 mOsm/l) with a Uosm/Posm ratio < 1 were consistent with CDI. The diagnosis of CDI was confirmed by the desmopressin test and the brain magnetic resonance imaging (MRI) at 34 weeks of age, that showed the lack of posterior pituitary hyperintense signal. In addition, a bilateral asymmetrical optic nerve hypoplasia associated with right orbital bone hypoplasia was observed, suggesting the diagnosis of WF.During the five years follow-up the patient did not developed glucose intolerance or diabetes mellitus. By the end of the second year of life, primary non-autoimmune central hypothyroidism and mild neurodevelopment retardation were diagnosed.ConclusionsThe analysis of our case, in the light of the most recent literature, suggests a possible role for WFS1 gene in the development of certain brain structures during the fetal period.Wolfram syndrome should be considered in the differential diagnosis of the rare cases of congenital central diabetes insipidus developed in the neonatal period.