Pituitary Function Tests Research Articles

Genetic Analysis of Multiple Endocrine Neoplasia Type 1 (MEN1) Leads to Misdiagnosis of an Extremely Rare Presentation of Intrasellar Cavernous Hemangioma as MEN1.

BackgroundMultiple endocrine neoplasia type 1 (MEN1) is a rare inherited disorder characterized by the simultaneous occurrence of endocrine tumors in target tissues (mainly the pituitary, endocrine pancreas, and parathyroid glands). MEN1 is caused by mutations in the MEN1 gene, which functions as a tumor suppressor and consists of one untranslated exon and nine exons encoding the menin protein. This condition is usually suspected when we encounter patients diagnosed with tumors in multiple endocrine organs, as mentioned above.MethodsA 65-year-old woman who underwent surgery for a pancreatic tumor (serous cystadenoma) 5 years previously was referred to our hospital due to neurologic symptoms of diplopia and left ptosis. Brain magnetic resonance imaging revealed a 3.4-cm lesion originating from the cavernous sinus wall and extending into the sellar region. It was thought to be a nonfunctioning tumor from the results of the combined pituitary function test. Incidentally, we found that she also had a pancreatic tumor, indicating the necessity of genetic analysis for MEN1.ResultsGenomic analysis using peripheral leukocytes revealed a heterozygous c.1621G>A mutation in the MEN1 gene that was previously reported to be either a pathogenic mutation or a simple polymorphism. We pursued a stereotactic approach to the pituitary lesion, and microscopic findings of the tumor revealed it to be an intrasellar cavernous hemangioma, a rare finding in the sellar region and even rarer in relation to oculomotor palsy. The patient recovered well from surgery, but refused further evaluation for the pancreatic lesion.ConclusionThere is great emphasis placed on genetic testing in the diagnosis of MEN1, but herein we report a case where it did not assist in diagnosis, hence, further discussion on the role of genetic testing in this disease is needed. Also, in cases of pituitary tumor with cranial nerve palsy, despite its low prevalence, intrasellar cavernous hemangioma could be suspected.

Prevalence of hypopituitarism after intracranial operations not directly associated with the pituitary gland

BackgroundOver the last few years, awareness and detection rates of hypopituitarism following traumatic brain injury (TBI) and subarachnoid hemorrhage (SAH) has steadily increased. Moreover, recent studies have found that a clinically relevant number of patients develop pituitary insufficiency after intracranial operations and radiation treatment for non-pituitary tumors. But, in a substantial portion of more than 40%, the hypopituitarism already exists before surgery. We sought to determine the frequency, pattern, and severity of endocrine disturbances using basal and advanced dynamic pituitary testing following non-pituitary intracranial procedures.Methods51 patients (29 women, 22 men) with a mean age of 55 years (range of 20 to 75 years) underwent prospective evaluation of basal parameters and pituitary function testing (combined growth hormone releasing hormone (GHRH)/arginine test, insulin tolerance test (ITT), low dose adrenocorticotropic hormone (ACTH) test), performed 5 to 168 months (median 47.2 months) after intracranial operation (4 patients had additional radiation and 2 patients received additional radiation combined with chemotherapy).ResultsWe discovered an overall rate of hypopituitarism with distinct magnitude in 64.7% (solitary in 45.1%, multiple in 19.6%, complete in 0%). Adrenocorticotropic hormone insufficiency was found in 51.0% (partial in 41.2%, complete in 9.8%) and growth hormone deficiency (GHD) occurred in 31.4% (partial in 25.5%, severe in 5.9%). Thyrotropic hormone deficiency was not identified. The frequency of hypogonadism was 9.1% in men. Pituitary deficits were associated with operations both in close proximity to the sella turcica and more distant regions (p = 0.91). Age (p = 0.76) and gender (p = 0.24) did not significantly differ across patients with versus those without hormonal deficiencies. Groups did not significantly differ across pathology and operation type (p = 0.07).ConclusionHypopituitarism occurs more frequently than expected in patients who have undergone neurosurgical intracranial procedures for conditions other then pituitary tumors or may already exists in a neurosurgical population before surgery. Pituitary function testing and adequate substitution may be warranted for neurosurgical patients with intracranial pathologies at least if unexplained symptoms like fatigue, weakness, altered mental activity, and decreased exercise tolerance are present.

A novel hook-shaped enhancement on contrast-enhanced sagittal magnetic resonance image in acute Sheehan’s syndrome: a case report

We report characteristic magnetic resonance (MR) image findings in a case of Sheehan's syndrome. A 37-year-old woman experienced complications of retained placenta and massive bleeding (3600 g) during delivery of a full-term baby. A pituitary function test demonstrated panhypopituitarism. MR image of the pituitary gland on postpartum day 10 revealed swelling of the anterior lobe. A hook-shaped enhancement was demonstrated on a sagittal image. The pituitary stalk, majority of the marginal zone of the anterior lobe, the anterior lobe just in front of the posterior lobe, and posterior lobe were well enhanced. In contrast, the central portion and the superior margin, just in front of the stalk insertion of the anterior lobe, were not enhanced. Anatomically, blood supply to these unenhanced portions of the anterior lobe was via the hypophyseal long portal vein and trabecular artery, which are tributaries of the superior hypophyseal artery that originate far from the internal carotid artery. Based on clinical history and MR image findings, the patient was diagnosed with Sheehan's syndrome and treated with hydrocortisone and levothyroxine. Follow-up MR image revealed marked atrophy of the anterior lobe. The characteristic hook-shaped enhancement in Sheehan's syndrome well reflected the vulnerability to massive bleeding based on the complex pituitary vasculature, which has not been reported previously. MR image with contrast enhancement is useful in the diagnosis of the acute phase of Sheehan's syndrome and in evaluating infarction of the anterior lobe.

Significance of pseudocapsule in the excision of pituitary adenomas in transsphenoidal surgery

To explore the significance of pseudocapsule in the excision of pituitary adenomas in transsphenoidal surgery. For 22 patients with pituitary adenomas over a period of 2 years at Peking Union Medical College Hospital, resection of pseudocapsule was applied for complete tumor removal. Pituitary function test and radiological imaging were performed at pre-operation, 3 months post-operation and at subsequent 6-12 months intervals postoperatively. All pituitary adenomas were totally removed under microscope. The symptoms of headache, disorder of sight and visual field disappeared postoperatively in nonfunctional pituitary adenomas. The GH levels of 2/5 growth hormone secreting adenoma patients were 4.2 and 7.7 µg/L while it was under 1 µg/L for another 3. The postoperative level of prolactin was 4.3 µg/L in prolactin secreting adenoma. The level of adrenocorticotropic hormone decreased under 5 ng/L except one was 15.7 ng/L. Leakage of cerebrospinal fluid occurred intraoperatively in 3 patients and postoperatively in 1. No leakage was found after repair. Diabetes insipidus occurred in one patient and was controlled with Minirin. Pseudocapsule was confirmed by pathological examination. Special staining revealed reticulum fibers in pseudocapsule. Resection of pseudocapsule may achieve a higher remission rate without deteriorating pituitary function.

Central Diabetes Insipidus: A Previously Unreported Side Effect of Temozolomide

Temozolomide (TMZ) is an alkylating agent primarily used to treat tumors of the central nervous system. We describe 2 patients with apparent TMZ-induced central diabetes insipidus. Using our institution's Research Patient Database Registry, we identified 3 additional potential cases of TMZ-induced diabetes insipidus among a group of 1545 patients treated with TMZ. A 53-year-old male with an oligoastrocytoma and a 38-year-old male with an oligodendroglioma each developed symptoms of polydipsia and polyuria approximately 2 months after the initiation of TMZ. Laboratory analyses demonstrated hypernatremia and urinary concentrating defects, consistent with the presence of diabetes insipidus, and the patients were successfully treated with desmopressin acetate. Desmopressin acetate was withdrawn after the discontinuation of TMZ, and diabetes insipidus did not recur. Magnetic resonance imaging of the pituitary and hypothalamus was unremarkable apart from the absence of a posterior pituitary bright spot in both of the cases. Anterior pituitary function tests were normal in both cases. Using the Research Patient Database Registry database, we identified the 2 index cases and 3 additional potential cases of diabetes insipidus for an estimated prevalence of 0.3% (5 cases of diabetes insipidus per 1545 patients prescribed TMZ). Central diabetes insipidus is a rare but reversible side effect of treatment with TMZ.

Hypopituitarism in patients with vasculotoxic snake bite envenomation related acute kidney injury: a prospective study on the prevalence and outcomes of this complication

Acute kidney injury (AKI) is common in patients with vasculotoxic snake bite (SB) envenomation but hypopituitarism (HP) is an uncommonly reported complication. We conducted a prospective observational study on survivors of SB-AKI who were evaluated and followed up from September 2010 till September 2012. Pituitary function tests were done if they developed any symptoms of HP. MRI of the hypothalamo-pituitary axis was done in those with documented HP. Response to therapy in the form of improvement in the quality of life (QoL) was evaluated by asking patients to mark on a visual analogue scale marked over 0-100 mm which was reported as percentage improvement. 126 patients were included for this study (30 were lost to follow up and were excluded). 25 cases were clinically suspected to have pituitary dysfunction and underwent evaluation with 9 (9.37%, n = 96) found to have evidence of HP. One child had partial empty sella on MRI with anterior as well as posterior pituitary abnormality and stunting; imaging was normal in others. Higher number of patients with HP had hypotension (p = 0.005, n = 7), coagulation abnormalities (p = 0.005, n = 9), severe clinical snake bite envenomation (p = 0.024, n = 9) and progression to chronic kidney disease (CKD) (p = 0.001, n = 5) as compared to those who did not. Dialysis dependence at presentation was not significantly different (p = 0.348, n = 9). Only development of CKD on follow up predicted the development of HP. Patients had an improvement in the QoL after treatment with mean score on the visual analogue scale of 66.67 ± 14.14%. HP is not very uncommon in patients with severe vasculotoxic SB-AKI. Threshold of clinical suspicion and evaluation should be low as it causes significant morbidity.

Endocrinopathies in transfusion‐associated iron overload

Transfusional iron overload primarily results in reticuloendothelial iron accumulation, which is considered to be less harmful than parenchymal iron accumulation. However, systematic and comprehensive data on endocrine function in transfusion-associated haemochromatosis are limited. We examined 25 aplastic anaemia patients (11 men and 14 women) diagnosed with transfusion-associated haemochromatosis at a single institution. Pituitary function was determined with a combined pituitary function test. On a different day, a 75-g oral glucose tolerance test was performed. The bone mineral density (BMD) of the lumbar spine and total hip was assessed with dual-energy X-ray absorptiometry. Twenty-two (88%) of these 25 patients had at least one endocrine abnormality, and 12 had more than one abnormality. The most common pituitary hormonal deficiency involved the pituitary-gonadal axis; 54% of the total subjects had hypogonadotropic hypogonadism. Two patients had an insufficient cortisol response to corticotrophin-releasing hormone stimulation. No patient had a deficiency of growth hormone or thyroid-stimulating hormone. Twelve (48%) had diabetes mellitus, and these patients tended to have higher concentrations of ferritin, alanine aminotransferase and γ-glutamyl transferase. Osteoporosis (T-score <-2·5 SD) was observed in 48% of patients. The reduction in BMD was more pronounced in the lumbar spine than in the total hip. The patients with osteoporosis were accompanied by hypogonadism, which predominantly affected the trabecular bone. Our observations suggest that endocrinopathies are common in transfusion-associated haemochromatosis.

PROP-1 gene mutations in a 63-year-old woman presenting with osteoporosis and hyperlipidaemia

PROP-1 gene mutations have been reported as a cause of combined pituitary hormone deficiency. Physical and hormonal phenotypes of affected individuals are variable. We report a 63-year-old female who presented with osteoporosis. She was short, did not enter puberty spontaneously and had primary amenorrhea. Biochemical evaluation revealed secondary hypothyroidism and mixed hyperlipidaemia, while dynamic testing of pituitary function was diagnostic of hypopituitarism. Bone density in the lumbar spine disclosed osteoporosis. DNA analysis showed that the patient was homozygote for the R73H mutation of the PROP-1 gene. The unfavourable long-term course of an untreated patient with PROP-1 gene mutation emphasizes the need for early aetiologic classification and proper management and follow-up of patients with short stature and/or disturbances of pubertal development.

Hypothalamic Hypopituitarism Caused by Pituitary Stalk Dysgenesis

본 증례는 9세경 ì €ì‹ ìž¥ìœ¼ë¡œ 성장호르몬 및 ê°‘ìƒì„ í˜¸ë¥´ëª¬ 이상을 ì§„ë‹¨ë°›ê³ ë¶ˆê·œì¹™í•˜ê²Œ 치료받았던 ê³¼ê±°ë ¥ì´ 있는 28세 남자 환자가 성기능 ìž¥ì• ì— 대한 불안으로 내원하여 뇌하수체 줄기 ë‹¨ì ˆì— 의한 시상하부성 뇌하수체 호르몬 이상을 ì§„ë‹¨ë°›ê³ ì‚¬ì¶˜ê¸° 발현과 생식기능의 ìœ ì§€ë¥¼ 위한 치료를 시행하게 된 1예를 경험하였기에 ë¬¸í—Œê³ ì°°ê³¼ 함께 ë³´ê³ í•˜ëŠ” 바이다. 중심 단어: 시상하부성 뇌하수체 ê¸°ëŠ¥ì €í•˜ì¦; 뇌하수체줄기 í˜•ì„±ë¶€ì „; 사춘기 지연

Surgical and Endocrinological Outcomes in the Treatment of Growth Hormone-Secreting Pituitary Adenomas According to the Shift of Surgical Paradigm

Extensive data exist regarding the success rates and long-term outcomes of transsphenoidal adenomectomy (TSA) of growth hormone (GH)-secreting pituitary tumors; however, few data exist regarding the extent of adenomectomy. To evaluate surgical outcomes for the treatment of GH-secreting pituitary adenomas with regard to the extent of adenomectomy. A retrospective study of 282 patients with GH-secreting pituitary tumors who underwent TSA. Three surgical paradigms (1, 2, and 3) were applied, all of which differed in extent of adenomectomy. All participants were evaluated with oral glucose tolerance tests (OGTTs) at 6-month intervals for 1.5 years and combined pituitary function tests at 1.5-year intervals after TSA. All surgeries were conducted by a single neurosurgeon at a single medical center. Biochemical remission was defined with insulinlike growth factor 1 and OGTT results. The overall surgical remission rates were 89%, 87%, 64%, 70%, and 50% (nadir GH <1 ng/mL on OGTTs: 96%, 95%, 73%, 84%, and 56%) for modified Hardy classifications I, II, IIIA, IIIB, and IV, respectively. The remission rates for modified Hardy classification I-IIIB improved to 42%, 68%, and 84% after application of surgical paradigms 1, 2, and 3, respectively (P = .002). Aggressive surgical resection did not worsen hypopituitarism. Among the 42 patients with modified Hardy classification IV, 24 (57%) achieved remission without recurrence after applying the aggressive paradigm 3 surgery. An aggressive surgical approach may be critical to managing GH-secreting pituitary adenomas and does not increase the risk of postoperative hypopituitarism. ACTH, corticotropinCPFT, combined pituitary function testCV, coefficient of variationGH, growth hormoneOGTT, oral glucose tolerance testPRL, prolactinTSA, transsphenoidal adenomectomyTSH, thyroid-stimulating hormone.

Sphenoid mucocele: an uncommon complication of a rare condition

A 58-year-old white woman presented with sudden onset of diplopia, headache and vomiting with a history of tiredness and lethargy over the past four to six months. She had smooth, pale, hairless skin and on examination she was found to have left-sided third and sixth nerve palsy. Laboratory tests confirmed pan-anterior hypopituitarism. Computerized tomography scan of head and magnetic resonance imaging appearances were consistent with those of a sphenoid sinus mucocoele. Following adequate replacement with hydrocortisone and thyroxine she underwent sphenoid mucocoele drainage and endoscopic left sphenoethmoidectomy. Her symptoms were relieved over the next few days and she had a near-total recovery of ophthalmoplegia over the following three months. Pituitary function tests showed partial resolution of hypopituitarism with recovery of hypothalamic-pituitary-adrenal axis and hydrocortisone therapy was withdrawn, but she continued to require thyroxine.

Post-traumatic hypopituitarism (PTHP): an under-diagnosed complication

One of the main objectives of this article is to advance understanding of a substantially under-diagnosed complication of brain injury. Damage to the hypothalamus or pituitary gland can affect the production and secretion of several hormones, specifically growth hormone, luteinising hormone, follicle stimulating hormone, prolactin, cortisol, adrenocorticotropic hormone, thyroid stimulating hormone, testosterone and oestradiol. Low levels of these hormones can lead to a variety of clinical manifestations and can have serious physical and/or psychological effects including on a person's quality of life. Even small disruptions in hormone levels can affect growth and development, concentration, and sexual function, causing depression, difficulty with personal intimacy and difficulty sustaining personal relationships. Collaboration between neurosurgeons, neuropsychologists and endocrinologists is required to review which effects may be related to pituitary levels and which may be physiological results of the brain injury. However, for patients presenting with overt signs of hypopituitarism it is not unreasonable to advocate baseline pituitary function tests to diagnose biochemical deficiencies.

A case of phace syndrome and acquired hypopituitarism?

BackgroundPHACE is a neurocutaneous syndrome associated with: Posterior fossa brain malformations, large “segmental” facial hemangiomas, arterial cerebrovascular-, cardiovascular-, and eye anomalies.Case vignetteWe are reporting a girl with PHACE syndrome. The patient had a congenital right-sided facial hemangioma with plaque-morphology. At age 11 years and 2 months she presented with short stature, markedly decreased growth velocity and signs and symptoms suggestive of hypothyroidism. Magnetic Resonance Imaging (MRI) of the brain revealed complex structural and cerebrovascular arterial anomalies, including an empty sella. Testing of pituitary function revealed multiple pituitary dysfunctions, including absolute growth hormone deficiency, hypogonadotropic hypogonadism, central hypothyroidism, and secondary adrenal insufficiency.ConclusionsThis case suggests the necessity to screen all patients with PHACE syndrome and intracranial malformations for pituitary dysfunction at regular intervals.

Indicaciones y secuencia de empleo de las pruebas funcionales hipofisarias. Criterios de valoración

Pituitary function tests are useful in the diagnosis of hypothalamic-pituitary pathology. The clinical history, physical examination and basal hormonal determinations will guide the application of these tests. When hormonal hypofunction is suspected we will use stimulation tests are used, but in cases of hormone hypersecretion suppression tests will be used. They are not necessary in the evaluation of all hypothalamus-pituitary axes.

Testing the hypothalamo-pituitary-adrenal axis

Aims There remains considerable debate about the best way to assess adrenal function. In our unit, adrenal function in children with suspected pituitary disease is initially tested using dynamic testing of the HPA axis (insulin tolerance test (ITT) or glucagon stimulation test (GST)), with failures (peak cortisol Methods This study took place in a single paediatric endocrinology unit. Endocrine records from November 1999– March 2010 identified patients having anterior pituitary function testing with ITT or GST, and also subsequent SST. Demographic, clinical and endocrine data were subsequently extracted from clinical and laboratory records. Results Over the 11 year collection period 509 ITTs and 164 GSTs were performed. Subsequent SSTs for failed tests were performed in 11 (2.2%) ITTs and 18 (11.0%) GSTs. Overall 42 children with paired tests were identified; 19 underwent ITT (median age 15.2 years, 12 males) and 23 GST (median age 8.8 years, 12 males), followed by SST within one year. The correlation coefficient between baseline (9 am) cortisols on the 2 consecutive tests was 0.36 (p=0.02); using a cutoff of ≥200 nmol/L there was concordance in 29/42 (69%). For ITTs there was a correlation coefficient of 0.34 (p=0.15) between the peak ITT cortisol compared to the 30 minute SST value. Using cutoffs of a peak ITT/GST cortisol level of ≥500 nmol/l and ≥550 nmol/l for the 30 minute SST cortisol level there was concordance in 14/19 (73.7%) ITTs. For GST the correlation coefficient was 0.56 (p=0.0057), and concordance in 11/23 (47.8%). Conclusion There is considerable variation in baseline cortisol levels, making its use and also that of incremental cortisol levels unhelpful in the diagnosis of adrenal insufficiency. Whilst there is a better correlation between the SST cortisol levels at 30 minutes and peak cortisol levels during the GST compared to the ITT concordance between tests is better in the latter. It would appear that the ITT is a good diagnostic test for the HPA axis; the GST less so. For patients having a subnormal cortisol response during the GST we would recommend SST testing.

Unusual cause of primary amenorrhea due to kissing internal carotid arteries

Disorders of anterior pituitary like tumors may cause amenorrhea. Non neoplastic sella lesions like infiltrative or infectious diseases may compress pituitary and may lead to secondary amenorrhea by hypogonadotropic hypogonadism. Congenital isolated gonadotropin deficiency such as Kallman’s syndrome may cause primary amenorrhea [1]. Uncommon vascular origin situations may also effect pituitary. Around sella aneurysm like internal carotid artery may lead to amenorrhea. Aneurysms of carotid arteries rarely simulate pituitary adenoma by expanding mass within sella. Carotid aneurysms may compress pituitary. These aneurysms may cause hypopituitarism. Generally these aneurysm cause more than one pituitary hormonal deficiencies [2–4]. The most common initial deficiencies are the gonadotropins [2]. Several congenital anomalies of internal carotid artery especially agenesis and congential hypopituitarism have been reported [5, 6]. A 23-year-old lady presented with primary amenorrhea to our department. She stated that when she was 16 years old, she was admitted to another hospital for primary amenorrhea. At that time, telarche did not occurred and she had little pubic hair. Her bone age was 15 and chromosomal analysis had been revealed normal XX karyotype. Her pelvic ultrasonography had revealed hypoplasia of her genital organs. She had been started oral conraceptives. She had been told that she had delayed puberty. In 2004 thyroxine replacement had been started for hypothyroidism due to hashimoto thyroiditis in public hospital. In physical examination axillary and pubic hairs and external genitalia were normal. Secondary sexual characters were normal. Her weight was 178 cm, weight was 89.2 kg and BMI was 28.2. Pituitary function testing demonstrated isolated hypogonadotropic hypogonadism. Prolactin and other pituitary hormone levels were in normal range. Magnetic resonance Imaging (MRI) was performed and uncover bilateral close dilated internal carotid arteries (ICAs) approaching to the midline where causing pituitary gland compression. Distance between ICAs was shortened in MRI angiography (Fig. 1). Vascular causes are very rare cause of pituitary hormonal deficiencies. These are generally because of ICA aneurysm or thrombosis/occlusion [1]. Isolated hormonal deficiency due to or conjunction with vascular anomaly did not reported before. Here we report probable congenital hypogonodotropic hypogonadism that cause primary amenorrhea due to ICAs position anomaly (medial proximity). MRI angiography may be helpful to identify vascular etiologies. In the future, development in imaging techniques could identify some of the rare etiologies of pituitary hormonal deficiencies. M. Sahin B. Karbek I. O. Unsal A. Gungunes N. Colak B. Ucan E. Cakal M. Ozbek T. Delibasi Department of Endocrinology and Metabolic Diseases, Diskapi Yildirim Beyazit Training and Research Hospital, Ankara, Turkey

Cavernous Carotid Artery Aneurysm, a Rare Cause of Intrasellar Mass and Hyperprolactinemia

A 37-yr-old gravida 1 para 1 woman was evaluated for increasing headaches. She had noted “spots” in her peripheral visual fields and reported mild galactorrhea for 4 yr. Magnetic resonance imaging (MRI) revealed a 2.5 1.6 1.6 cm sellar mass contiguous with the right midcavernous carotid artery (Fig. 1A). On T1-weighted images, the mass revealed partial flow void and a heterogeneous hypoto isointense central area. The pituitary was displaced superiorly. The stalk was slightly deviated to the right. The optic chiasm was unaffected. Computed tomography angiogram (CTA) confirmed the presence of a large intrasellar aneurysm (Fig. 1, B and C). The patient underwent selective coil embolization (Fig. 2, A and B), and her headaches resolved. Pituitary function tests performed before embolization and 2 months after the procedure were normal except for mild persistent hyperprolactinemia [prolactin, 37 ng/ml (1608 pmol/liter); normal, less than 20 ng/ml]. Repeat angiography at 6 months showed a small neck residual. Sellar aneurysms are an uncommon subtype of intracranial aneurysm and rarely cause hypopituitarism (1). They may occur concomitantly with a pituitary adenoma, although there may be no association between the two conditions (2). Preoperative diagnosis of a sellar aneurysm is imperative to avoid a potential surgical catastrophe. Typical characteristics on MRI include a flow void sign on T1or T2-weighted images and an “onion-skin” appearance on T1 (if clot is present). These findings appear to have good sensitivity (3), although some cystic lesions may appear equally hypointense. Small aneurysms may be better visualized by digital subtraction angiography or CTA (4, 5).

Permanent Hypopituitarism Is Rare after Structural Traumatic Brain Injury in Early Childhood

We sought to determine the incidence of permanent hypopituitarism in a potentially high-risk group: young children after structural traumatic brain injury (TBI). We conducted a cross-sectional study with longitudinal follow-up. Dynamic tests of pituitary function (GH and ACTH) were performed in all subjects and potential abnormalities critically evaluated. Puberty was clinically staged; baseline thyroid function, prolactin, IGF-I, serum sodium, and osmolality were compared with age-matched data. Diagnosis of GH deficiency was based on an integrated assessment of stimulated GH peak (<5 μg/liter suggestive of deficiency), IGF-I, and growth pattern. ACTH deficiency was diagnosed based on a subnormal response to two serial Synacthen tests (peak cortisol <500 nmol/liter) and a metyrapone test. We studied 198 survivors of structural TBI sustained in early childhood (112 male, age at injury 1.7 ± 1.5 yr) 6.5 ± 3.2 yr after injury. Sixty-four of the injuries (33%) were inflicted and 134 (68%) accidental. Two participants had developed precocious puberty, which is within the expected background population rate. Peak stimulated GH was subnormal in 16 participants (8%), in the context of normal IGF-I and normal growth. Stimulated peak cortisol was low in 17 (8%), but all had normal ACTH function on follow-up. One participant had a transient low serum T(4). Therefore, no cases of hypopituitarism were recorded. Permanent hypopituitarism is rare after both inflicted and accidental structural TBI in early childhood. Precocious puberty was the only pituitary hormone abnormality found, but the prevalence did not exceed that of the normal population.

Optimal Surgical Approaches for Rathke Cleft Cyst With Consideration of Endocrine Function

Surgical indications for Rathke cleft cyst are not clear. To evaluate postoperative outcomes in terms of endocrine function. The study analyzed a total 73 patients who underwent transsphenoidal surgery. All patients underwent a visual field test, combined pituitary function test, and magnetic resonance imaging before and after surgery. A follow-up combined pituitary function test was performed at 1.5-year intervals. The mean age at the time of surgery was 35 ± 14 years, and the male/female ratio was 1:1.25 (33/40). The mean follow-up duration after surgery was 59 ± 39 months. The most common symptoms were headache (84%), visual disturbance (48%), and polyuria (38%). After transsphenoidal surgery, 75% of polyuria and 96% of visual field defects were resolved, and pituitary function improved in 42% of patients. The mean age of patients who exhibited worsened hypopituitarism was significantly higher than that of patients who exhibited unchanged or improved hypopituitarism (44 ± 15.7 vs 33 ± 13.5 years; P = .02). Twelve patients (16%) experienced recollection of cyst, but none required reoperation. Five of the recollected cysts presented with characteristics that were different from those of the initial lesions, and 2 recollected cysts underwent spontaneous regression. Minimal incision with radical removal of cyst content is reasonable to prevent the development of endocrine disturbances and other complications. Individualized risks and benefits must be assessed before a decision is reached regarding surgery and surgical method. Patients with recurrent Rathke cleft cyst require careful follow-up with special attention rather than a hasty operation.

The diagnosis was in her birthday party!

An 89-year-old lady was admitted to our hospital with a history of recurrent falls and episodic nocturnal confusion. Routine testing revealed capillary blood glucose levels fluctuating between 1.6 and 3.2 mmol/l (28.8–57.6 mg/dl). There was no history of diabetes mellitus and she was not on medications that could cause hypoglycemia. A detailed history and observation discounted the possibility of accidental or malicious hypoglycemia. Her confusion improved with glucogel (glucose 10 g/25-g tube) and taking frequent carbohydrate meals, thereby demonstrating Whipple's triad (simultaneous symptoms and laboratory demonstration of hypoglycaemia with resolution of symptoms on achieving normoglycemia). A biochemistry screen showed normal liver, renal, pituitary and adrenal function tests. A review of her case notes revealed a diagnosis of ‘seizure disorder’ by a …