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Objective: Spontaneous cerebrospinal fluid (CSF) leaks represent a significant challenge due to frequent association with elevated intracranial pressure (ICP) and higher risk of surgical failure. The study objective was to review management strategy and identify factors associated with need for acetazolamide and/or ventriculoperitoneal shunt (VPS) placement. Study Design: Retrospective data analysis. Methods: Chart review performed from 1999 to 2009 at a tertiary-care medical center. Results: A total of 105 patients underwent CSF leak repair; 39 patients (37.1%) were treated for spontaneous CSF leaks. Mean age was 57.7 years and 33 were female (85%). Average body mass index (BMI) was 38.5 kg/m2. The most common sites were cribriform plate (51%), sphenoid lateral pterygoid recess (31%), and ethmoid roof (8%). All patients underwent endoscopic repair utilizing image guidance with multilayered closure in most cases. Five patients (12.8%) developed recurrent CSF leak with mean ICP of 27.0 cm H2O, compared to 25.0 cm H2O for those without recurrence (P = .33). All had successful rerepair at mean follow-up of 2.8 years. Acetazolamide was used in nine patients, whereas six patients underwent VPS placement for elevated ICP management. Diagnosis of benign intracranial hypertension (BIH) was statistically associated with need for acetazolamide or VPS (P < .001), whereas elevated ICP reached borderline significance (P = .049). Conclusions: Management of spontaneous CSF leaks requires a comprehensive strategy after endoscopic repair. Diagnosis of BIH may be associated with requirement of further ICP treatment. Close ICP monitoring, coupled with selective use of acetazolamide and VPS, may decrease risk of failure. Laryngoscope, 2010

The pituitary function in 20 patients with primary empty sella syndrome has been evaluated. The only endocrine symptom was secondary amenorrhoea in four patients. In half of the patients, pituitary function tests showed minor abnormalities, the most common being impaired growth hormone response to glucagon. In addition to the empty sella, other intracranial abnormalities such as pituitary tumour and hydrocephalus were found in four patients. Although commonly a benign condition, the empty sella may occasionally be associated with clinically important endocrine dysfunction, pituitary tumours and other intracranial abnormalities.

In humans, the empty sella is defined as herniation of the subarachnoidal space into the sella turcica with invisible (total empty sella) or reduced (partial empty sella) hypophyseal size. An empty sella can be associated with endocrine disturbances. The purpose of this study was to determine if the empty sella exists in dogs or cats and whether it is associated with endocrinopathy or hydrocephalus. Archived magnetic resonance images of the head of 370 dogs and 77 cats were re-evaluated specifically for the presence or absence of the pituitary gland. Hypophyseal tissue filling more than 50% of the sella was considered normal, between 30% and 50% was defined as partial empty sella and less than 30% was defined as total empty sella. In patients with an empty sella, and in all other imaged dogs of the affected breeds, the ventricle to brain ratio was measured. In patients with an empty sella, the medical record was searched for evidence of endocrinopathy. No cat had an empty sella but 11 dogs (3%) had a small or missing hypophysis. Seven of these dogs had a ventricle to brain ratio greater than 15%. Only small dogs (mean weight of 6.7kg) were affected. One dog with an empty sella had signs of central hyperadrenocorticism while the other 10 had no sign of endocrinopathy. We conclude that the empty sella exists in dogs but is not generally associated with endocrinopathy.

A 63-year-old female with known empty sella syndrome underwent coronary artery bypass grafting surgery. She became hypotensive immediately postoperatively and this did not respond to fluid resuscitation and inotropic therapy. Surgical re-exploration was undertaken and did not reveal any surgical cause. Pulmonary artery catheterisation confirmed a profound vasodilatory component to her shock. We believe this was due to unmasking of posterior pituitary hypofunction, in particular vasopressin insufficiency, due to metabolic stress. This rapidly corrected with an exogenous vasopressin infusion.

Hypogonadotropic hypogonadism is one of the causes of infertility. In women with hypogonadotropic hypogonadism both follicle-stimulating hormone and luteinizing hormone are required to induce optimal follicular growth and steroidogenesis. We described a case of singleton pregnancy in a 38-year-old patient, presenting with primary hypogonadotropic amenorrhea and empty sella syndrome, treated with human menopausal gonadotropins and performing intrauterine insemination in first attempt. This therapy led to maturation of two follicles and one of them was fertilized. A singleton pregnancy ensued and a normal infant was delivered by cesarean section.

2 patients with diabetes insipidus established in the preleukaemic phase of acute myeloid leukaemia are described. Both patients had probably coincidentally an empty sella turcica showed by a computerized tomography. In one of them, leukaemia developed secondary to a cytostatic therapy for a vesical cancer. In this patient, blast cells appeared in cerebrospinal fluid after the patients had become polyuric and later on autopsy findings showed a peripituitary leukaemic infiltrate. In the other patient, diabetes insipidus and the acute phase of leukaemia developed after a long period with a dysmyelopoietic syndrome. Hormonal tests suggested a hypothalamic injury in both patients due to a leukaemic infiltrate at least in the former of them. It is concluded that diabetes insipidus as a consequence of hypothalamic lesion may develop in the preleukaemic phase of acute myeloid leukaemia and become evident even before any other symptoms of leukaemia have manifested.

A patient with 18p monosomy and GH deficiency due to pituitary hypoplasia, who showed an excellent response to GH-treatment, is described. Patients with this syndrome should be considered for endocrine evaluation, as they can benefit from hormonal substitution.

Summary OBJECTIVE We evaluated the presence of anti-pituitary hormone autoantibodies (APHA) in patients with primary empty sella syndrome and pituitary tumours and examined the correlation of positive antibodies with the hormonal deficiencies. DESIGN Case-control, retrospective study. PATIENTS Eleven patients were identified with primary empty sella syndrome or a pituitary tumour by magnetic resonance imaging or computed tomography scanning. Six healthy, normal subjects without evidence of a pituitary problem served as the control group. MEASUREMENTS Anti-pituitary hormone autoantibodies against purified pituitary hormones were measured in ail subjects utilizing an immunoblotting technique. All patients with pituitary disease had their medical records reviewed for any hormonal evaluation. RESULTS All of the normal subjects were negative for antipituitary hormone antibodies. Forty-five per cent of patients with pituitary disease (pituitary tumours or primary empty sella syndrome) had positive antipituitary hormone antibodies. Of the five patients with positive antipituitary hormone antibodies, anti-ACTH antibodies were the most common (5/5) followed by anti-TSH and anti-GH antibodies (2/5 for each). The hormonal deficiencies failed to correspond with the antipituitary hormone antibodies. Anti-ACTH antibody had a sensitivity of 50% with a specificity of 56%. The anti-TSH antibody yielded a sensitivity of 67% with a specificity of 100%. The anti-FSH/ LH antibody reported a 0% sensitivity. CONCLUSIONS Detection of antipituitary hormone antibody was unable to discriminate between empty sella syndrome and pituitary tumours. The presence of these antipituitary hormone antibodies were neither specific for, nor predictive of, the endocrine deficiencies.

In normal subjects, the glomerular filtration rate (GFR) and effective renal plasma flow (ERPF) acutely increase in response to infusion of amino acids and to low doses of dopamine. It is uncertain whether circulatory growth hormone (GH) is a permissive factor for these stimulatory effects. GFR and ERPF (constant infusion technique using I-125-iothalamate and I-131-hippuran, respectively) were measured before and during the infusion of dopamine and amino acids in 8 GH deficient subjects. The clearance study was repeated during concomitant administration of octreotide to investigate whether this somatostatin analogue would modify the amino acid and dopamine-induced renal haemodynamic changes. Dopamine increased baseline GFR from 89 +/- 3 (mean +/- SEM, n = 8) to 102 +/- 4 ml min-1 1.73 m-2 and ERPF from 352 +/- 19 to 476 +/- 26 ml min-1 1.73 m-2, p

Haemorrhagic fever with renal syndrome (HFRS) in Scandinavia is called nephropathia epidemica (NE), and is caused by the Puumala-virus, which belongs to the Hanta-virus genus. The clinical course of NE is mostly benign, complications are uncommon, and deaths are rarely observed. We report the cases of three patients who developed serious complications in the course of NE caused by the Puumala-virus. One patient died within 24 h after admission, another developed progressive neuromuscular dysfunction (Guillain-Barré syndrome) which required auxiliary ventilation for several weeks before a slow recovery, and a third patient developed empty sella syndrome with pituitary gland insufficiency. In the first two cases the diagnosis of NE was confirmed by a rapid avidity assay for IgG antibody against Puumala-virus. In the third case the clinical course, and demonstration of NE immunity 16 years later, suggested that NE might have caused the hypopituitarism. Some patients with NE caused by the Puumala-virus may require intensive-care treatment, and the development of late complications such as empty sella syndrome and hypopituitarism should be taken into consideration.

Rearrangements of the proximal long arm of chromosome 15 have been found in most patients with the Prader-Willi syndrome (PWS) and in some with Angelman syndrome. We present an individual with syndromic obesity and her normal mother, who both have an abnormal chromosome 15. The proposita is a 26-year-old woman with marked obesity, acanthosis nigricans, short fingers, and severe cone degeneration of the retina. She has high plasma insulin levels, hypothyroidism, and an empty sella on CT scan. High-resolution chromosome banding demonstrated an increase in band 15q12. Further analysis showed the same abnormal 15 in her normal mother but not in her normal sister. This case and recent reports in the literature indicate that duplications of chromosome 15q in the PWS region may be associated with a syndrome of obesity, acanthosis nigricans, empty sella, and rodcore dystrophy as well as with a normal phenotype. Whether normal individuals with such a duplication carry increased risk of having offspring with an obesity syndrome is yet to be determined.

A case of ectopic pituitary adenoma in the sphenoid sinus associated with an empty sella is reported. The coexistence of an ectopic pituitary adenoma and an empty sella is quite rare. The diagnosis was made with an intraoperative finding of the intact dura mater of the sellar floor. In the present case, the hypointense line that coated the pituitary gland was clearly demonstrated on 3-tesla T2-weighted magnetic resonance imaging. The hypointense line is considered to be the pituitary capsule and was critical in diagnosing this rare entity.

The empty-sella syndrome is uncommon in pediatric patients. Hypothalamic-pituitary dysfunction is common in these patients but involvement of the posterior pituitary gland is very rare. We report a seven-year-old girl with empty-sella syndrome who first developed arginine-vasopressin deficient diabetes insipidus and then anterior pituitary gland deficiency. The empty-sella syndrome should be included among the causes of arginine-vasopressin deficient diabetes insipidus in pediatric patients.

The diaphragma sellae, the pituitary foramen, and the perisellar arachnoid were studied in cadaver sphenoid blocks. Special attention was paid to the dissection of the perforated arachnoid membrane (of Lilliequist). Results were compared with observations made during surgery and with data reported in the literature. The pathophysiology of the intrasellar arachnoid diverticulum, the empty sella syndrome, and the intrasellar arachnoid cyst are discussed. Precise knowledge of the microsurgical anatomy and anatomic variations of the dura mater and arachnoid is imperative for a safe trans-sphenoidal approach to the hypophysis cerebri (pituitary gland) and transcranial approach to the sellar region.

SUMMARY We report an 8-year follow-up of three sisters born of a consanguineous marriage and who originally presented with short stature and enlarged pituitary fossa. All have shown progressive failure of anterior pituitary hormone function. In addition, the two eldest sisters were eventually found to have an empty sella while the youngest, who initially showed an enhancing intrasellar mass consistent with a tumour, later showed an empty sella. A familial pituitary tumour developing early in childhood but subsequently undergoing involution could account for these findings.

SUMMARY Forty-four untreated patients with an enlarged sella were studied (excluding patients with acromegaly, Cushing's disease, and those with radiological evidence of suprasellar extension). In 20 patients CT revealed a completely or partially empty sella. Based on recent studies we take this finding to signify the previous presence of a pituitary adenoma which has undergone complete or partial necrosis. Ten of the 20 patients had in fact experienced symptoms typical of a pituitary apoplexy compared with only one out of the other 24 patients. Adrenal, thyroid, and growth-hormone insufficiency occurred as often in patients with an empty sella as in those with a solid pituitary tumour. In contrast, plasma prolactin levels were much lower in patients with an empty sella than in patients with a solid tumour (11 vs 166 ng/ml). It is assumed that this discrepancy reflects previous necrosis occurring in an adenoma hyper-secreting prolactin. These results emphasize the importance of taking the spontaneous course of pituitary adenomas into account when assessing the effect of various treatment protocols.

Over recent years empty sella turcica has become more frequently diagnosed with high resolution coniputerized tomography and the associated clinical abnornialities have been better described. In this article the spectrum of clinical presentation is based on a review of 26 cases with six illustrative case reports. Reconimendations for management and further assessment are presented. It is important for clinicians to be aware of the varying presentations of this syndrome, since it should not be considered simply as an incidental finding. A patient diagnosed with empty sella syndrome requires clinical and endocrine evaluation, and appropriate follow-up as determined by initial test results.

In 63 consecutive patients with primary hyperparathyroidism (HPT) a prospective screening study was undertaken for coexistent multiple endocrine neoplasma-(MEN)-syndrome type 1. The screening consisted of a clinical examination, a radiological examination of the sella turcica with skeletal tomography (and in equivocal cases computed tomography), visual field examination by perimetry and a hormonal evaluation including measurements of the serum levels of prolactin, gastrin, pancreatic polypeptide (PP) and subunits of human chorionic gonadotrophin (HCG-alpha and -beta). Clinical examination did not reveal any signs of endocrine disease suggestive of a MEN-1 syndrome. In only one case there was a radiological abnormality of the sella turcica; this patient had an empty sella syndrome and a raised serum prolactin value. All other prolactin values were within the normal range. In 41% of the patients raised serum gastrin levels were found; these tended to normalize after parathyroidectomy. As a group, patients with raised gastrin values were older than the others and generally they had hypo- or achlorhydria. The serum PP levels were raised in 28% of the patients but there was no clinical evidence of a pancreatic tumour in any of these cases, and the serum HCG-alpha and -beta levels were within the normal range in all patients but two. We conclude that the incidence of MEN-1 syndrome in unselected patients with primary HPT must be low, and that investigations for this syndrome are justified only in HPT patients with specific symptoms or with a positive family history.

A case of isolated ACTH deficiency is described. The patient had an empty sella and longstanding radio-iodine induced hypothyroidism. The association between isolated ACTH deficiency, empty sella and hypothyroidism is reviewed. It has not previously been recognized that the commonest endocrine association of isolated ACTH deficiency is primary thyroid failure. ACTH secretion does not recover following thyroxine replacement treatment.

A total of 10,550 'normal' adults (8450 men and 2100 women) in the general population were screened by a paired assay method for serum PRL and 40 subjects with hyperprolactinaemia (greater than 75 micrograms/l) were detected. Of these, five patients (three men and two women) with pituitary prolactinoma, one man with empty sella syndrome, 10 cases of 'big' prolactinaemia, seven pregnant women and 13 subjects with drug-induced hyperprolactinaemia were found. The patients with prolactinoma had few if any complaints (asymptomatic prolactinoma).

Three related disorders in the same patient, namely bilateral primary intracranial cholesteatomas, an “empty sella” syndrome, and a cerebrospinal rhinorrhea are reported. No previous report of bilateral symmetrical cholesteatomas has been made, though single intracranial cholesteatomas have frequently been recorded in medical literature. The “empty sella” syndrome is generally considered to be from a herniation of the subarachnoid into the sella through a deficient diaphragma sella, and was first defined by Ommaya in 1968. Non-traumatic (spontaneous) rhinorrhea remains an uncommon disease initially described in 1826. Experience of the individual otolaryngologist is limited in this region because of infrequent occurrence and because definitive treatment is directed to other specialties. On the other hand, the ear, nose and throat physician may be the first to interpret a drainage from the nose. With this in mind, the etiologic, clinical and management factors in the present case are discussed.