Pituitary Hypofunction Research Articles

Sheehan’s syndrome presenting with panhypopituitarism and central diabetes insipidus: a case report

BackgroundSheehan’s syndrome is a rare condition, which is classically characterized by anterior pituitary hypofunction following postpartum shock or hemorrhage. While diabetes insipidus (DI) is not commonly associated with Sheehan’s syndrome, we present a rare case of a multiparous female developing rapid-onset panhypopituitarism and DI following severe postpartum hemorrhage.Case presentationA previously healthy 39-year-old woman, gravida 5, para 4, presented with hypovolemic shock after vaginal delivery, attributed to severe postpartum hemorrhage, necessitating emergent hysterectomy. Although her shock episodes resolved during hospitalization, she developed intermittent fever, later diagnosed as adrenal insufficiency. Administration of hydrocortisone effectively resolved the fever. However, she subsequently developed diabetes insipidus. Diagnosis of Sheehan’s syndrome with central diabetes insipidus was confirmed through functional hormonal tests and MRI findings. Treatment consisted of hormone replacement therapy, with persistent panhypopituitarism noted during a ten-year follow-up period.ConclusionsSheehan’s syndrome is a rare complication of postpartum hemorrhage. Central diabetes insipidus should be suspected, although not commonly, while the patient presented polyuria and polydipsia. Besides, the potential necessity for long-term hormonal replacement therapy should be considered.

Partial hypopituitarism with ACTH deficiency as the main manifestation as a complication of hemorrhagic fever with renal syndrome

Hypopituitarism is a relatively rare complication of hemorrhagic fever with renal syndrome. However, almost all available reported cases were total anterior pituitary hypofunction, isolated growth-hormone deficiency, or isolated gonadotropin deficiency. Here, we firstly describe a patient with partial hypopituitarism with ACTH deficiency as the main manifestation as a complication of hemorrhagic fever with renal syndrome.

Clinical characteristics of programmed cell death-1 inhibitor-associated hypophysitis

Objective: To explore the clinical features of programmed cell death-1 (PD-1) inhibitor-associated hypophysitis and improve the understanding of the disease. Methods: For the present retrospective case series study, the clinical data of patients with PD-1 inhibitor-associated hypophysitis who were treated at the Affiliated Hospital of Hebei University and the 3rd Hospital of Hebei Medical University from January 2020 to May 2023 were collected for analysis of clinical manifestations and prognosis. Results: Fifteen cases of PD-1 inhibitor-induced hypophysitis were included, with 13 males and 2 females. The mean age of onset was (62.1±7.5) years, and the median time of onset was 6.5 (4.7, 11.6) cycles of PD-1 inhibitor. At diagnosis, 14 patients complained of gastrointestinal symptoms, and 12 patients complained of fatigue. There were 12, 1, 1, 5, and 1 cases of hyponatremia, hypokalemia, hypoglycemia, hypotension, and fever, respectively. Secondary adrenocortical insufficiency occurred in all cases. Moreover, four patients had secondary hypothyroidism, and two patients had secondary hypogonadism. Posterior pituitary hypofunction was not found. Pituitary MRI showed one case each of vacuolar sella turcica, pituitary cystic lesion, pituitary stalk slightly shifted to the left, high metabolism in the sella turcica, and pituitary abnormal signal, while no abnormalities were found in 11 cases. The follow-up time was (47.66±11.93) weeks. At the last follow-up, one patient's serum levels of adrenocorticotropic hormone and cortisol returned to normal. Conclusions: Hypophysitis associated with PD-1 inhibitors occurs later, and gastrointestinal symptoms and fatigue are the most common clinical manifestations. PD-1 inhibitor-associated hypophysitis mainly manifests as adrenocortical hypofunction, and some cases manifest as hypothyroidism and hypogonadism. In addition, patients with PD-1 inhibitor-associated hypophysitis show no obvious imaging changes in the pituitary gland.

Pituitary-Related Adverse Events and Onset Patterns Caused by Immune Checkpoint Inhibitors: Analysis Using the Japanese Adverse Drug Event Report Database.

Background and Objectives: One type of immune-related adverse event caused by immune checkpoint inhibitors (ICIs) is pituitary-related adverse events. The management of pituitary-related adverse events is important because they can be fatal if not treated promptly. Therefore, this study was conducted to investigate the onset of pituitary-related adverse events using the Japanese Adverse Drug Report (JADER) database. Materials and Methods: Cases registered in the JADER database from 2004 to 2019 were used. The target drugs were ipilimumab, nivolumab, pembrolizumab, avelumab, atezolizumab, and durvalumab, and the target adverse events were the high-level terms "Anterior pituitary hypofunction," "Anterior pituitary hyperfunction," "Posterior pituitary disorder," and "Pituitary neoplasm" in the Medical Dictionary for Regulatory Activities, Japanese version (MedDRA/J). The information component (IC) was used for signal detection and IC delta (ICΔ) was used for women-related signals. Onset timing and patterns were analyzed using the Weibull distribution. Results: Signals were detected with ipilimumab, nivolumab, pembrolizumab, and atezolizumab in "Anterior pituitary hypofunction," with ICs and 95% credible intervals (95%CrI) of 5.53 (5.30-5.69), 4.96 (4.79-5.08), 4.04 (3.76-4.25), and 2.40 (1.53-3.00). Significant signals were detected in women, except for atezolizumab. Additionally, the time of onset was classified as the wear-out failure type. Inverse signals were detected with ipilimumab and nivolumab in "Posterior pituitary disorder," with ICs (95%CrI) of -1.24 (-2.80--0.26), and -0.89 (-1.64--0.37). Conclusions: Anterior pituitary hypofunction is likely to occur with the long-term administration of ipilimumab, nivolumab, and pembrolizumab. Further investigation is needed to determine the differences in the tendencies to detect signals in the anterior and posterior pituitaries between ipilimumab and nivolumab.

Endoscopic endonasal marsupialization of rathke cleft cysts: clinical outcomes and risk factors analysis of visual impairment, pituitary dysfunction, and CSF leak.

This paper assesses the clinical and imaging characteristics, histopathological findings, and treatment outcomes of patients with Rathke's cleft cyst (RCC), as well as identifies potential risk factors for preoperative visual and pituitary dysfunction, intraoperative cerebrospinal fluid (CSF) leak, and recurrence. Through analyzing these factors, the study aims to contribute to the current understanding of the management of RCCs and identify opportunities for improving patient outcomes. We performed a retrospective analysis of 45 RCC patients between ages 18-80 treated by Endoscopic Endonasal Approach (EEA) and cyst marsupialization between 2010 and 2022 at a single institution. The median patient age was 34, and 73% were female. The mean follow-up was 70 ± 43 months. Preoperative visual impairment correlated with cyst diameter (OR = 1.41, 95% CI = 1.07 to 1.85, p-value = 0.01) and older age (OR = 1.06, 95% CI = 1.01 to 1.11, p-value = 0.02). Intraoperative CSF leaks were 11 times more likely for cysts ≥ 2cm (OR = 11.3, 95% CI = 1.25 to 97.37, p-value = 0.03), with the odds of leakage doubling for every 0.1cm increase in cyst size (OR = 1.41, 95% CI = 1.08 to 1.84, p-value = 0.01). Preoperative RCC appearing hypointense on T1 images demonstrated significantly higher CSF leak rates than hyperintense lesions (OR = 122.88, 95% CI = 1.5 to 10077.54, p-value = 0.03). Preoperative pituitary hypofunction was significantly more likely in patients with the presence of inflammation on histopathology (OR = 20.53, 95% CI = 2.20 to 191.45, p-value = 0.008 ) and T2 hyperintensity on magnetic resonance imaging (MRI) sequences (OR = 23.2, 95% CI = 2.56 to 211.02, p-value = 0.005). Notably, except for the hyperprolactinemia, no postoperative improvement was observed in pituitary function. Carefully considering risk factors, surgeons can appropriately counsel patients and deliver expectations for complications and long-term results. In contrast to preoperative visual impairment, preoperative pituitary dysfunction was found to have the least improvement post-surgery. It was the most significant permanent complication, with our data indicating the link to the cyst signal intensity on T2 MR and inflammation on histopathology. Earlier surgical intervention might improve the preservation of pituitary function.

Hydrocortisone-induced blood pressure reduction in a patient with anterior pituitary hypofunction: a case report

Hydrocortisone is widely used for the anti-inflammatory and immunosuppressive effects and physiological substitute of endogenous glucocorticoid. Allergic reaction to hydrocortisone is infrequent, but once it occurs, it can affect the...

RF13 | PMON83 Culture Negative Pituitary Abscesses: A Case Series

Abstract Introduction Pituitary abscesses are rare, representing less than 1% of pituitary lesions. Patients may present with pituitary hypofunction, headache, and/or vision changes, often without systemic or biochemical signs of infection, with diagnosis only made intraoperatively. Cases A 74-year-old man with history of diabetes and coronary artery disease presented with headache and left-eye ptosis. MRI revealed a 1.8 cm hypoenhancing cystic, hemorrhagic sellar mass extending into the left cavernous sinus. Pre-operative labs showed AM cortisol 1.1 ug/dL, testosterone 43 ng/dL (reference range 250-1100), FSH 15.3 mIU/mL, LH 4.5 mIU/mL consistent with adrenal insufficiency and hypogonadism. He underwent transsphenoidal pituitary resection (TSR) during which frank pus was demonstrated after incision into the dura overlying the pituitary. Surgical pathology showed necrosis and granulation-type reaction with acute inflammatory cell infiltrates. Gram stain and methenamine silver stains did not reveal definite micro-organisms while tissue bacterial cultures showed normal skin flora. He remained hemodynamically stable without fevers throughout the hospitalization and was discharged on empiric antibiotics and hydrocortisone. A 74-year-old female with history of a pituitary macroadenoma was admitted for worsening headaches. Labs were consistent with her known history of secondary adrenal insufficiency and hypogonadotropic hypogonadism. Repeat MRI showed characteristics suggestive of hemorrhage within a 1.8 cm intrasellar mass with inhomogeneous and peripheral enhancement after contrast. She underwent TSR and intraoperatively frank pus was encountered. Surgical pathology showed sinonasal mucosa with chronic inflammation. There was no growth in aerobic, fungal, or AFB cultures; anaerobic cultures grew rare commensal bacteria. She was discharged with a course of antibiotics and remains on hydrocortisone. A 42-year-old male presented with headache and was found to have a 2.5 cm sellar mass with rim enhancement elevating and compressing the optic chiasm on MRI. Labs were significant for AM cortisol 4.9 ug/dL and testosterone 8 ng/dL, FSH 0.4 mIU/mL, LH <0.2 mIU/mL consistent with adrenal insufficiency and hypogonadism. He underwent TSR and was found to have purulent debris in the sella. Pathology showed epithelial tissue fragments insufficient for diagnosis, and cultures grew few skin and oropharyngeal contaminants. He was discharged on hydrocortisone with a course of antibiotics. Conclusion We describe three patient cases presenting with headache and varying degrees of hypopituitarism, found to have purulent material emanating from the sella during transsphenoidal resection. None presented with clinical or biochemical systemic signs of infection. MRI imaging was variable, ranging from cystic, hemorrhagic features to peripheral enhancement. Surgical pathology and cultures were unrevealing for a definitive infectious source or other cystic lesion such as Rathke's cleft cyst. This case series demonstrates the potential nonspecific presentation of pituitary abscesses including negative cultures and pathology. It is important to keep this rare diagnosis in the differential for patients presenting with pituitary disease. Presentation: Sunday, June 12, 2022 12:30 p.m. - 12:35 p.m., Monday, June 13, 2022 12:30 p.m. - 2:30 p.m.

ODP279 A Case of Nephrogenic Diabetes Insipidus Diagnosed at an Advanced Age in a Female Patient with an AVPR2 Gene Mutation and Skewed×Inactivation

Abstract Background Arginine vasopressin receptor 2 (AVPR2) gene mutationsare the most common cause of congenital nephrogenic diabetes insipidus (CNDI), and they are inherited in an X-linked recessive manner. Women are generally asymptomatic or mildly affected, but atypically severe cases have been reported. Skewed X inactivation, in which the X chromosome is unevenly active, is a cause of this atypical severe case.×chromosome inactivation is a developmental compensation mechanism that results in equal doses of X-linked genes between XX females and XY males and has been reported to be accentuated by aging. Clinical Case A 69-year-old woman had no family history of diabetes insipidus. Immediately after undergoing a craniotomy for subarachnoid hemorrhage in July, she showed marked polyuria of approximately 8000 mL/day and hypernatremia of 161 mEq/L. The use of desmopressin (15mcg/kg/day) did not improve herpolyuria. She was referred to our department in September for further investigation and treatment. Her urine osmolality remained hypotonic at approximately 60–80 mOsm/kg/H 2 O (50–1300 mOsm/kg/H 2 O) according to a water restriction test. Her urine osmolality increased slightly to 106 mOsm/kg/H 2 O in a desmopressin test. These results indicated that desmopressin was ineffective, and the patient was diagnosed with nephrogenic diabetes. Her urine output decreased to approximately 2500 mL/day with trichlormethiazide treatment. There was no significant anterior pituitary hypofunction. Genetic examination revealed a heterozygous mutation of the AVPR2 gene (c.656T>G, p. Leu219Arg) in her X chromosome, and her X chromosome activity ratio was 79: 21 in a human androgen receptor assay, indicating skewed X inactivation. Conclusion We observed a case of CNDI in an elderly woman with no family history of the disease. In this case, a combination of the skewed X inactivation, presumably accentuated by aging, and the onset of subarachnoid hemorrhage, reduced her drinking water and led to the diagnosis of CNDI.

Rapidly Progressive Pituitary Apoplexy in a Patient with COVID-19 Disease Treated with Endoscopic Endonasal Surgery.

This report describes a case of pituitary apoplexy with rapidly evolving hemorrhage in a 74-year-old female with coronavirus disease 2019 (COVID-19) disease. The patient presented with severe headache and mild respiratory symptoms, with laboratories concerning for pituitary hypofunction. Brain imaging demonstrated a sellar mass concerning for a pituitary adenoma with ischemic apoplexy. She subsequently developed visual deficits within 24 hours of presentation, and repeat imaging demonstrated evolving hemorrhage and new mass effect on the optic chiasm. She was successfully managed with urgent endoscopic endonasal surgery despite her COVID-19 positive status by taking special intraoperative precautions to mitigate SARS-CoV2 transmission risk. Only a handful of cases of pituitary apoplexy have been reported in association with COVID-19 disease, and even fewer reports exist of endonasal procedures in such cases. We discuss the potential implication of COVID-19 in the occurrence of pituitary apoplexy, in addition to the safety and success of endonasal surgery in this population.

Nonfunctioning Pituitary Microadenomas: Should Imaging Interval be Extended? A Large Single-center Cohort Study.

Characterization of the clinical features and natural history of nonfunctioning pituitary microadenomas (NFPmAs) is limited by heterogeneous and small-scale studies. To characterize the clinical presentation and natural history of NFPmAs and evaluate if imaging follow-up interval can be extended. Retrospective single-center cohort study (years 2006-2021) of conservatively managed patients with NFPmAs. Initial symptoms, pituitary function, and tumor size were assessed. A change in NFPmA size ≥2 mm, as determined by pituitary or brain magnetic resonance imaging (MRI), was considered significant. There were 347 patients in the study cohort. Headache (78.4%) and fatigue (70.0%) were commonly reported despite no evidence of mass effect or significant pituitary hypofunction. Pituitary deficiencies at baseline were rare, with hypogonadism being most common (5.1%). During a median imaging follow-up period of 29 months (range 3-154), 8.1% of NFPmAs grew. Growth incidence was 2.1 per 100 person-years with a mean and median time to growth of 38.1 (SD ± 36.4) and 24.5 (interquartile range 12.0-70.8) months, respectively. Tumor growth was mild and not associated with new pituitary deficiencies or visual deficits. These data indicate that the natural history of NFPmAs is overall benign. Consequently, we propose that the initial MRI follow-up timeline for NFPmAs can be extended up to 3 years unless a lesion is close to the optic chiasm, there are worrisome mass effect symptoms, or new pituitary deficiencies.

Experience in the diagnosis and treatment of the postoperative complications of craniopharyngiomas through expanded endoscopic endonasal transsphenoidal approach

Objective:To summarize the clinical characteristics of the postoperative complications of surgical resection of craniopharyngiomas through expanded endoscopic endonasal transsphenoidal approach （EEETA）. Strategies for prevention and management were also discussed. Methods:The clinical data of the patients who were treated through EEETA were retrospectively reviewed. The occurrence of post-operative complications were recorded. Results:Partial removal of the tumors were accomplished in 11 cases and subtotal removal in 4 cases. The major postoperative complications were anterior pituitary hypofunction（11/15）, diabetes insipidus（8/15）, epistaxis（3/15）, cerebrospinal fluid rhinorrhea（1/15）. The cases were treated symptomatically or by re-operation. Of all the cases,10 patients were improved,1 patient had drowsiness,3 suffered from multiple organ failure，and 1 patient died. Conclusion:To prevent and reduce the postoperative complications of EEETA, first of all, it is essential to evaluate the need for surgical intervention and perform a comprehensive preoperative assessment. Critical nerves and vessels should be preserved carefully during operation for the sake of avoiding injuries normal pituitary and hypothalamus. Furthermore, reconstruction of the skull base is critical. The standard procedure of nasal endoscopy and the experience of the surgeons are quite significant, while the operation needs multidisciplinary collaborations.

Endocrine Dysfunction in Children with Zika-Related Microcephaly Who Were Born during the 2015 Epidemic in the State of Pernambuco, Brazil.

Congenital viral infections and the occurrence of septo-optic dysplasia, which is a combination of optic nerve hypoplasia, abnormal formation of structures along the midline of the brain, and pituitary hypofunction, support the biological plausibility of endocrine dysfunction in Zika-related microcephaly. In this case series we ascertained the presence and describe endocrine dysfunction in 30 children with severe Zika-related microcephaly from the MERG Pediatric Cohort, referred for endocrinological evaluation between February and August 2019. Of the 30 children, 97% had severe microcephaly. The average age at the endocrinological consultation was 41 months and 53% were female. The most frequently observed endocrine dysfunctions comprised short stature, hypothyroidism, obesity and variants early puberty. These dysfunctions occurred alone 57% or in combination 43%. We found optic nerve hypoplasia (6/21) and corpus callosum hypoplasia (20/21). Seizure crises were reported in 86% of the children. The most common—and clinically important—endocrine dysfunctions were pubertal dysfunctions, thyroid disease, growth impairment, and obesity. These dysfunctions require careful monitoring and signal the need for endocrinological evaluation in children with Zika-related microcephaly, in order to make early diagnoses and implement appropriate treatment when necessary.

Craniopharyngioma in the Elderly: A Multicenter and Nationwide Study in Spain

Background: Craniopharyngioma (CP) is a rare tumor in the elderly whose clinical features and prognosis are not well known in this population. Aim: To evaluate the clinicopathological features and therapeutic outcomes of CP diagnosed in the elderly. Patients and Methods: This was a retrospective, multicenter, national study of CP patients diagnosed over the age of 65 years and surgically treated. Results: From a total of 384 adult CP patients, we selected 53 (13.8%) patients (27 women [50.9%], mean age 72.3 ± 5.1 years [range 65–83 years]) diagnosed after the age of 65 years. The most common clinical symptoms were visual field defects (71.2%) followed by headache (45.3%). The maximum tumor diameter was 2.9 ± 1.1 cm. In most patients, the tumor was suprasellar (96.2%) and mixed (solid-cystic) (58.5%). The surgical approach most commonly used was transcranial surgery (52.8%), and more than half of the patients (54.7%) underwent subtotal resection (STR). Adamantinomatous CP and papillary CP were present in 51 and 45.1%, respectively, with mixed forms in the remaining. Surgery was accompanied by an improvement in visual field defects and in headaches; however, pituitary hormonal hypofunction increased, mainly at the expense of an increase in the prevalence of diabetes insipidus (DI) (from 3.9 to 69.2%). Near-total resection (NTR) was associated with a higher prevalence of DI compared with subtotal resection (87.5 vs. 53.6%, p = 0.008). Patients were followed for 46.7 ± 40.8 months. The mortality rate was 39.6% with a median survival time of 88 (95% CI: 57–118) months. DI at last visit was associated with a lower survival. Conclusion: CP diagnosed in the elderly shows a similar distribution by sex and histologic forms than that diagnosed at younger ages. At presentation, visual field alterations and headaches are the main clinical symptoms which improve substantially with surgery. However, surgery, mainly NTR, is accompanied by worsening of pituitary function, especially DI, which seems to be a predictor of mortality in this population.

MON-289 Pituitary Metastasis: A Systematic Review

Metastatic lesions to the pituitary are uncommon as well as concerning as they do not always have a characteristic radiographic appearance and can be easily missed. We conducted a systematic review of all the published cases and case series of pituitary metastasis to further understand the unique characteristics of these lesions. Using Pubmed and Embase as the primary search engines, we reviewed all cases published from January 1980 to July 2019. A total of 175 unique cases were included in the study. Over 400 cases were collected amongst which a total of 278 cases were included in the study. As part of a challenge with any retrospective study some data points were missing from cases reviewed. Only English language publications were included in the study. The study revealed a predominance of females with 121 cases. Median age was noted to be 61 years. Only 40 patients had previously known metastatic disease. 70 patients were noted have primary cancer arising in the breast which was noted to be the most common primary cancer followed by 64 cases of primary cancer arising from the lungs. Majority of the patients (162 cases) had pituitary hypofunction with deficiency of one or more pituitary hormones. 97 cases were reported to have diabetes insipidus on presentation. Only 40 patients had no visual field deficits whereas 77 patients were reported to have abnormal eye movements. Displacement of the gland superiorly towards optic chiasm was the most common radiographic features in 137 reported cases. Although not commonly reported in most of the published literature, 45 cases were noted to have bony erosion due to expansion of the gland. Only 5 cases were reported to have no contrast enhancement, although many case reports did not specify contrast enhancement of the pituitary. 22 cases were noted to have an aggressive or rapid growth pattern of the pituitary. 13 cases were noted to have edema around the optic chiasm. The pituitary should not be overlooked as a site of metastasis. Many cases can present asymptomatically without biochemical or radiographic characteristics of metastatic lesion. Any biochemical or clinical sign of pituitary pathology in a patient with known cancer should raise suspicion for sellar metastasis. Unique radiographic characteristics should alert the clinicians to consider this possibility. Our study revealed many unique characteristics of metastatic lesions of the pituitary. This should allow clinicians to become aware of the more common findings in these patients allowing prompt diagnosis.

A Case Series of Patients with Isolated IgG4-related Hypophysitis Treated with Rituximab

ContextThe acute presentation of immunoglobulin G4 (IgG4)-related hypophysitis can be indistinguishable from other forms of acute hypophysitis, and histology remains the diagnostic gold standard. The high recurrence rate necessitates long-term immunosuppressive therapy. Rituximab (RTX) has been shown to be effective in systemic IgG4-related disease (IgG4-RD), but experience with isolated pituitary involvement remains limited.Case DescriptionWe report 3 female patients with MRI findings suggestive of hypophysitis. All patients underwent transsphenoidal biopsy and fulfilled diagnostic criteria for IgG4-related hypophysitis. Treatment with glucocorticoids (GCs) resulted in good therapeutic response in Patients 1 and 2, but the disease recurred on tapering doses of GCs. GC treatment led to emotional lability in Patient 3, necessitating a dose reduction. All 3 patients received RTX and Patients 2 and 3 received further courses of treatment when symptoms returned and B-cells repopulated. Patient 3 did not receive RTX until 12 months from the onset of symptoms. Patient 1 was not able to have further RTX treatments due to an allergic reaction when receiving the second dose. Rituximab treatment resulted in sustained remission and full recovery of anterior pituitary function in Patients 1 and 2, with complete resolution of pituitary enlargement. By contrast, Patient 3 only showed a symptomatic response following RTX treatment, but pituitary enlargement and hypofunction persisted.ConclusionRituximab treatment for IgG4-related hypophysitis resulted in sustained remission in 2 patients treated early in the disease process but only achieved partial response in a patient with chronic disease, suggesting that early therapeutic intervention may be crucial in order to avoid irreversible changes.

The effects of experimentally irradiated pituitary gland on the growth of rat’s tibia, skull, maxilla and mandible

Background/Aim. The reaction of an organism to radiation depends on the level of irradiation and the sensitivity of the affected tissue cells. The biological effects on the cells and tissues are proportional to the absorbed radiation energy. The aim of our study was to examine the effects of hypofunction of the pituitary gland, previously irradiated with x-rays, on the growth of rat?s craniofacial and stomatognathic system as well as rat?s tibia. Methods. A total dose of x-rays of 27.92 Gy was applied in eight sessions in the period from 8 to 63 days of animal age on their heads and the effects of irradiation-induced hypofunction of the pituitary gland on the growth of rat?s craniofacial and stomatognathic system and tibia were examined. In order to differentiate the effects of the irradiated pituitary gland from the direct effects of x-rays on the head, the experiment was set as a double study. One group of animals had the pituitary gland protected with a lead plate set beneath the projection of the gland, while the second group was irradiated with the same dose, but without the protection. The control group of animals were non-irradiated. Results. Growth measurements of numerous parameters of the craniofacial and stomatognathic system as well as tibia in rats with the entire locally irradiated head showed a statistically significant delay, compared to both the non-irradiated control group and the group with the protected pituitary gland. Conclusion. The damage of the stomatognathic and craniofacial system was greater in animals with irradiated head without pituitary gland protection compared to those with the gland protection whereby the growth of the craniofacial system was most affected. Irraduition-induced pituitary gland hypofunction and irradiation itself showed a stronger effect on the lower jaw growth rate impairment compared to the upper jaw. Similarly, the delay in tibia growth was more pronounced in animals without pituitary gland protection during irradiation of their heads compared to non-protected ones.

Evidence of Pituitary Hypoplasia Associated with Partial Central Diabetes Insipidus in a Young Persian Cat

Background: Congenital anomalies are an uncommon pituitary hypofunction cause associated to multiple hormone deficiencies. Congenital hyposomatotropism is often related to an inherited anomaly, characterized mainly by delayed growth. It is not uncommon to find associated thyroid-stimulating hormone and gonadotropin deficiencies. Pituitary malformation may be associated to progressive cystic lesion expansion. Central diabetes insipidus (CDI) is another rare disease associated to polyuria (PU) and polydipsia (PD) secondary to antidiuretic hormone (ADH) deficient secretion. The aim of this report is to describe a likely case of pituitary hypoplasia, associated with partial CDI in a cat.Case: A 9-month-old unneutered male Persian cat weighing 2 kg was presented due to severe polyuria and polydipsia associated with growth deficit when compared with its sibling. After clinical and laboratory evaluations during the months in which the patient was monitored, a reduced serum concentration of insulin-like growth factor-1 (IGF-1), thyroid-stimulating hormone (TSH), thyroid hormones, and testosterone was documented, confirming the diagnosis of hyposomatotropism, hypogonadism, and secondary hypothyroidism. Furthermore, therapeutic diagnosis with desmopressin revealed partial central diabetes insipidus (CDI). As the sibling showed normal development aging 13-months, a radiographic examination of the forelimb (carpus) was performed on both cats. There was lack of growth plate fusion in the patient, without any other evidence of dysgenesis, whereas complete epiphyseal closure was observed in the sibling. Despite desmopressin and levothyroxine therapeutic prescription the owners refuse further follow-up to the case.Discussion: Notwhistanding neuroimaging was not available for investigation of pituitary aspect in this particular case, the clinical symptoms added to the results of the complementary tests were consistent with pituitary hypoplasia, associated with hyposomatotropism, secondary hypothyroidism, hypogonadism, and partial CDI. Hyposomatotropism was presumably diagnosed based on the patient’s clinical characteristics, which included proportional growth delay, delayed tooth eruption, delayed growth plate fusion, associated with serum reduced IGF-1 results in comparison with its sibling. The report of low free T4 by equilibrium dialysis and of low total T4 levels, associated with low TSH levels, was considered compatible with secondary hypothyroidism. TRH stimulation test is considered the gold standard for secondary hypothyroidism diagnosis since low TSH could be secondary to assay´s low sensibility. However, normal TSH and thyroid hormone results in the sibling results ruled out this possible dismissed diagnose. The patient’s lack of sexual interest, associated with hypotestosteronemia and underdeveloped genitals (absence of penile spines and testicular hypoplasia), indicates hypogonadism. Finally, partial CDI diagnosis was demonstrated by cat´s partial ability to increase urinary specific gravity under water deprivation often made by the owners, as well as the response pattern to desmopressin therapy. Owing the lack of neurological signs expected to be associated with neoplastic or traumatic hypopituitarism etiology, hypoplasia hypothesis was raised. Quite often, patients with pituitary hypoplasia develop Rathke cleft cysts that might expand over time. In the present case, partial CDI is likely to be caused by the compression of the neurohypophysis by cyst formation secondary to adenohypophyseal hypoplasia since this kind of pituitary congenital anomaly does not justify per se neurohypophysis implications.

Endoscopic Assisted Microscopic Surgical Removal of Craniopharyngioma

Background: Craniopharyngioma is a benign tumor which represents 2% - 3% of all intracranial tumors, there are two types: childhood type which affects children between 5 and 10 years and adulthood type which affects patients 50 - 60 years old. The presenting symptoms develop over years and include visual, endocrine, hypothalamic, neurological and neurophysiological manifestations. Surgery is the treatment of choice. Postoperative radiotherapy, gamma-knife and intra tumoral injection of chemotherapeutic drugs have been used as an adjuvant therapy in some cases. Objectives: In this study, we evaluated the role of endoscopy in assisting microscopic surgical removal of craniopharyngioma. Methods: Eleven patients were operated upon in Cairo University Hospitals, Egypt. All operations were done using microscope through the subfrontal approach. At the end of surgery, the endoscope was used to detect any residual tumor in the subchiasmatic and retrochiasmatic areas and to visualize the posterior part of the tumor which couldn’t be seen by the microscope to check if it was adherent to the hypothalamus and to evaluate whether to be removed or not. Results: The study included eleven cases, four of which were childhood type and seven adult type craniopharyngiomas. Total removal was achieved in six cases (five cases of adulthood type). Oumaya reservoir was inserted in five cases; ventriculoperitoneal shunt was needed in five cases. All cases suffered from temporary diabetes insipidus postoperatively, while only two cases developed permanent diabetes insipidus. Three cases presented preoperatively with pituitary hypofunction and two cases developed postoperative pituitary hypofunction, which necessitated hormone replacement therapy. Conclusion: Craniopharyngioma is one of the most difficult and challenging tumors for neurosurgeons due to its relation to optic nerve, hypothalamus and vascular system formed by Willis circle and its perforating branches. Endoscopy has a role in decision making after microscopic removal of craniopharyngioma.

SURG-02. STAGED SURGERY FOR SELECTED GIANT ADULT PITUITARY ADENOMA

Abstract PURPOSE Combined surgical strategy with both trans-cranial surgery and trans-sphenoid surgery is necessary for selected giant pituitary adenoma with both intra-cranial and intra-sphenoidal invasion. Cases of staged surgery were reviewed retrospectively to deeply investigate this treatment strategy. METHODS Adult cases received staged surgery were reviewed. Data regarding clinical presentation, laboratory tests, image examination, surgery details, and outcome were analyzed. The size and invasion was evaluated by pre-op MR and intra-op observation. Diagnosis was confirmed by histology examination. Literatures were reviewed. RESULTS 8 NFPAs were included. 4 received trans-cranial resection first while the other 4 received trans-sphenoidal resection first. Time interval between two operations was 1–2 months. After the first surgery, the rate of visual defects and of pituitary hypofunction improvement were higher in trans-cranial first group. 1 apoplexy and 1 CSF rhinorrhea were observed in trans-sphenoidal first group. After the second surgery, the extend of resection and the rate of complete visual symptoms improvement were higher in trans-cranial first group. All pituitary hypofunction resolved completely in long-term follow-up. 11 literatures were reviewed. CONCLUSIONS An appropriate surgical strategy is essential for selective pituitary adenoma requiring combined resection. For staged surgery cases, taking trans-cranial approach for first stage with trans-sphenoidal subsequently, comparing to the inverse resection order, would offer higher extent of resection with less recurrence, earlier and better symptom improvement, and less post-op complications after first stage. Further investigation is necessary.

The functional evaluation of pituitary in patients with a surgical resection of sellar tumours.

IntroductionThe aim of this study was to analyse the incidences of hypopituitarism before and after surgical resection of sellar tumours and to find the factors related to the incidences.Material and methodsFrom January 2009 to December 2011, 191 patients in the Department of Neurosurgery in Xiangya Hospital, who underwent the surgical resection of sellar tumours, were included in this retrospective analysis. Pre- and postoperative pituitary function assessments were performed by the detection of hormone levels. Tumour size and location were analysed by magnetic resonance imaging (MRI).ResultsIn total 152 (79.6%) patients had anterior pituitary hypofunction preoperatively, and 176 (92.1%) patients had anterior pituitary hypofunction postoperatively. The pre- and postoperative adrenal cortex hypofunction incidences were 83 (43.5%) and 103 (53.9%), respectively. Ninety-three (48.7%) patients had thyroid hypofunction preoperatively, and 101 (52.9%) patients had anterior pituitary hypofunction postoperatively. The pre- and postoperative hypogonadism incidences were 131 (68.6%) and 160 (83.8%), respectively. The postoperative incidences of anterior pituitary hypofunction and hypogonadism in patients with craniopharyngioma or pituitary tumours were both significantly higher than the preoperative incidences. Surgery resection methods and tumour sizes were found to be related to the incidence of postoperative hypogonadism.ConclusionsTo sum up, we found that the postoperative incidences of hypopituitarism were higher than the preoperative incidences. Tumour type, surgery resection methods, and tumour sizes were important contributing factors to the incidence of postoperative hypogonadism.