Variation in the MRI appearance of the canine pituitary gland.

A recent publication described pathological findings in the pituitary gland incidentally discovered during routine necropsies of the brain of dogs and cats; however, imaging characteristics of these lesions were not reported. Aims of this retrospective, observational study were to characterize MRI variants and incidental lesions in pituitary glands of dogs with no clinical signs of pituitary disease. Cranial MRIs from dogs with no suspicion of pituitary disease, based on history and presenting clinical signs, were retrieved from a veterinary teleradiology database during the period of January 2014 to January 2016. Images were reinterpreted by two observers and pituitary lesions were described based on consensus. A total of 580 scans were evaluated and pituitary lesions were detected in 78 dogs (13.44%). Pituitary cystic lesions were the most common finding and occurred in 31 dogs (5.34%). Of these 31 dogs, the majority (74%) were of toy or brachycephalic breed. Partial or total empty sella lesions were detected in 14 dogs (2.41%), and all of these were small or toy breeds. A significantly increased incidence of the partial empty sella lesion was found in male dogs (P = .034). Pituitary lesions greater than 1 cm occurred rarely (0.69%). There was a significant association between low-field (LF) MRI strength and detection of a partial or total empty sella lesion (P = .0112), and detection of a pituitary lesion greater than 1 cm (P = .0125). A significant difference was present between the MRI field strength (FS) that identified pituitary cysts and the FS that detected an empty sella (P = .0068), with the former being a high FS and the latter a LF strength. The findings from this study indicated that up to 13% of dogs with no presenting clinical signs of pituitary disease may have MRI pituitary lesions.

Chiasmapexy for secondary empty sella syndrome: diagnostic and therapeutic considerations.

PURPOSE: Secondary empty sella syndrome (SESS) following pituitary surgery remains a diagnostic and therapeutic challenge. The aim of this study was to specify the diagnostic criteria, surgical indications and results of chiasmapexy in the SESS. METHODS: Three cases from two experienced neurosurgical centers were collected and the available literature was reviewed. RESULTS: The 3 patients were operated for a giant non-functioning pituitary adenoma, a cystic macroprolactinoma, and an arachnoid cyst respectively. Postoperative visual outcome was initially improved, and then worsened progressively. At the time of SESS diagnosis, visual field defect was severe in all cases with optic nerve (ON) atrophy in 2 cases. Patients were operated via an endoscopic endonasal extradural approach. One patient was re-operated because of early fat reabsorption. Visual outcome improved in 1 case and stabilized in 2 cases. Statistical analyses performed on 24 cases from the literature review highlighted that patient age and severity of the preoperative visual defect were respectively significant and nearly significant prognostic factors for visual outcome, unlike the surgical technique. CONCLUSION: T2-weighted or CISS/FIESTA sequence MRI is mandatory to visualize adhesions, ON kinking and neurovascular conflict. TS approach is the most commonly used approach. The literature review could not conclude on the need for an intra or extradural approach suggesting case by case adapted strategy. Intrasellar packing with non-absorbable material such as bone should be considered. Severity of the visual loss clearly decreases the visual outcome suggesting early chiasmapexy. In case of severe and long standing symptoms before surgery, benefits and surgical risks should be carefully balanced.

Clinical presentation, evaluation and case management of primary empty sella syndrome: a retrospective analysis of 10-year single-center patient data.

BACKGROUND: Primary Empty Sella (PES) syndrome is an increasingly common disorder, mostly diagnosed as an incidental finding during brain imaging scans. We intended to review the clinical management and hormonal profile of patients with PES. METHODS: The study included ten-year retrospective analysis of registry containing PES cases in the period 2007 to 2017, from a single tertiary care center. The keyword 'primary empty sella' was used to retrieve patient details from the radiology unit. The clinical and biochemical profile of PES patients was analyzed. Case management of PES patients and their rate of referral to endocrinologists was explored. RESULTS: The registry had 765 cases with a male: female ratio of 1:3.8 suggesting female predominance by almost four times. Although not significant, the onset of disease was earlier for males [Mean ± standard deviation (SD) (46.7 years ±17.3 vs 48.8 years±14.1), p = 0.110]. Almost 79% of the cases were found as an incidental finding during Magnetic Resonance Imaging. Of the total PES cases, only 20% were referred to the endocrinologists and the rest were handled by general physicians. Only 1-2.5% of the cases were evaluated for gonadal, growth and adrenal hormones by the general physicians. The hormonal evaluation by the endocrinologists was also found to be sub-optimal. Headache and visual disturbances were the most common presenting complaints followed by menstrual abnormalities. Endocrine abnormalities like thyroid dysfunction, hyperprolactinemia, hypogonadism and hypocortisolism were highly prevalent among those assessed. CONCLUSION: There is a gross under-evaluation of hormonal assessment and minimal case-referral to Endocrinologists. PES is associated with varying degrees of hormonal dysfunction, and hence early assessment and management is needed. Establishing a standard protocol for diagnosis and case management is essential with the involvement of a multidisciplinary team consisting of endocrinologists, neurologists, primary care phys icians and ophthalmologists.

Retinal nerve fibre and ganglion cell inner plexiform layer analysis by optical coherence tomography in asymptomatic empty sella patients.

Purpose: To investigate the clinical importance of the thicknesses of the retinal nerve fibre (RNFL) and ganglion cell and inner plexiform layer (GCL+) by spectral domain optic coherence tomography (SD-OCT) in asymptomatic empty sella (ES) patients.Materials and methods: In this cross-sectional, non-randomized prospective study, 44 ES patients and 74 age- and sex-matched healthy individuals were evaluated. All the patients and controls competed an automated 30-2 visual field (VF) test. The mean deviation (MD), pattern standard deviation (PSD), RNFL, and GCL + thickness values obtained with SD-OCT were compared statistically between the two groups.Results: No marked VF defects were found in either group, and there was no statistically significant between-group difference in MD or PSD values. In terms of RNFL thickness, the average and superior quadrant RNFL values of the ES patients were thinner than those of the controls, with statistical significance (p = 0.013 and p = 0.043, respectively). Although other measured RNFL quadrant thicknesses and foveal thickness (FT), macular volume (MV), and average macular thickness (AMT) values were reduced in the ES group, these differences were not statistically significant. The average GCL + value and GCL + values in six sectors in the patient group were significantly lower than those in the control group.Conclusions: Asymptomatic ES patients have a risk of primary ES syndrome and should be followed up using a multidisciplinary approach. Objective and quantitative RNFL and GCL + thickness measurements obtained with OCT can provide valuable data for monitoring these patients.

Empty Sella in the Making.

BACKGROUND: Pituitary apoplexy may occur when a large tumor compresses or outgrows its nutrient supply, resulting in ischemic necrosis and hemorrhage. Although once deemed a neurosurgical emergency, increasing evidence suggests that conservative management of pituitary apoplexy leads to favorable neuro-ophthalmologic and endocrinologic outcomes as well. Spontaneous remission after pituitary apoplexy has been described in functioning pituitary adenomas, but it is a rare occurrence in nonfunctioning tumors. CASE DESCRIPTION: We report a man that presented with pituitary apoplexy of a nonfunctioning pituitary macroadenoma that was managed conservatively and treated hormonally for hypopituitarism during a 2-year follow-up period, with serial neuroimaging demonstrating significant tumor volume reduction with almost complete resolution resulting in partial empty sella. In addition, a short literature review was performed pertaining to the management of pituitary apoplexy with emphasis on a more conservative approach. CONCLUSIONS: A subset of patients with pituitary apoplexy without altered consciousness and nonprogressive or mild ophthalmologic deficits may be managed conservatively; however, lifelong periodic assessment, preferably by a specialized multidisciplinary pituitary team, is essential until clinical outcomes become clear.

Empty sella associated with growth hormone deficiency and polydactyly.

Empty sella means the absence of the pituitary gland on cranial computed tomography or magnetic resonance imaging. Empty sella syndrome is the pathological variant of the imaging-described empty sella. We present the case of a male Caucasian child, aged four years and two months, for short stature and diagnosed by imaging procedures as empty sella. The cause of short stature was isolated growth hormone (GH) deficiency. Associated he presented left hand postaxial polydactyly. In connection with this particular case, we propose a review of current knowledge in empty sella syndrome. The particularity of reported case consists of association empty sella with GH deficiency and polydactyly. The association of empty sella with polydactyly is not reported yet in the medical literature and is probably coincidental.

Pituitary morphovolumetric changes in Alström syndrome.

PURPOSE: Alström syndrome (AS) is a rare monogenic ciliopathy characterized by cone-code dystrophy, leading to early blindness, and obesity. Early endocrinological dysfunctions, especially growth hormone deficiency and hypogonadism, are detected in about half of AS patients. This MRI study investigates the presence of pituitary gland abnormalities in a large cohort of AS patients. METHODS: Pituitary morphological changes (gland flattening with partial or total empty sella) were evaluated on midsagittal high-resolution T1-weighted images of 32 AS patients (mean-age 23.2±9.4 years; range: 6-45, 15 females) and 21 unrelated healthy subjects (mean age 23.2±11.2 years; range: 6-43; 10 females). RESULTS: Among AS patients, 11/32 (34%) had total empty sella and 6/32 (19%) partial empty sella, while 3/21 (14%) of controls had partial empty sella and none presented with total empty sella (P<0.005). AS patients harboring a total or partial empty sella did not differ from those with normal pituitary gland for gender (P=0.98), BMI (P=0.10) or visual impairment (P=0.21), while the presence of empty sella was associated with an older age (P=0.007) being especially frequent above the age of 30. CONCLUSIONS: Total or partial empty sella appears commonly during the course of AS. Pituitary gland flattening might represent the morphological underpinning of subtle endocrinologic dysfunctions and raises the need to further investigate the pituitary function in this rare ciliopathy.

[Analysis of the joint and a posteriori probability between primary empty sella, its comorbidities and audiovestibular pathology]. / Análisis de la probabilidad conjunta y a posteriori entre aracnoidocele selar primario, sus comorbilidades y enfermedad audiovestibular.

BACKGROUND: Primary empty sella is a herniation of the sellar diaphragm into the pituitary space. It is an incidental finding and patients may manifest neurological, ophthalmological and/or endocrine disorders. Episodes of vertigo, dizziness, and hearing loss, have been reported. OBJECTIVE: To determine the conditional probability, as well as the statistical dependency, through the Bayesian analysis in patients with primary empty sella and audiovestibular disorders. PATIENTS: Individuals who attended the National Rehabilitation Institute from January 2010 to December 2011, diagnosed with primary empty sella and audiovestibular disorders. MATERIAL AND METHODS: An analysis was performed on a sample of 18 patients with a diagnosis of primary empty sella confirmed with magnetic resonance studies and who had signs of vertigo, hearing loss and dizziness. RESULTS: Of the 18 patients studied, 3 (16.66%) had primary empty sella as the only clinical evidence. In 9 patients (50%) empty sella was associated with vertigo, and 16 patients (88.88%) were diagnosed with hearing loss, with sensorineural hearing loss being the most frequent (77.77%). The intersection between the proportions of primary empty sella with the presence and type of hearing loss was calculated. Thus for sensorineural hearing loss, the calculated ratio was P(AB)=0.6912, and for conductive and mixed hearing loss the value of P(AB)=0.0493 in both cases. CONCLUSIONS: Bayesian analysis and conditional probability enables the dependence between two or more variables to be calculated. In this study both mathematical models were used to analyse comorbidities and audiovestibular disorders in patients diagnosed with primary empty sella.

The association between petrous apex cephalocele and empty sella.

PURPOSE: Petrous apex cephalocele (PAC) is a rare lesion that has been linked to empty sella in several case reports. The aim of this study is to document the incidence of empty sella and PAC in consecutive brain MRI studies and study the association between these two lesions to better understand the underlying etiology of PAC. METHODS: A total of 2410 brain MRI studies were performed in our institution in the period from January 2011 to December 2011. After eliminating duplicated studies, a total of 2093 studies met our inclusion criteria. Retrospective analysis of patients' head MRI was performed by two radiologists independently to identify the presence of empty sella and/or PAC. RESULTS: Empty sella was found in 322 (15.4 %) patients. PAC was found in 111 (5.3 %) patients (age range 6-81 years) of which 87 were females and 24 were males. Of all the patients with PAC, 77 (69.4 %) patients had associated empty sella. Bilateral PAC was more common and seen in 77 patients. CONCLUSION: PAC is associated with empty sella, and both lesions are probably related to the same cause.

Pituitary imaging findings in male patients with hypogonadotrophic hypogonadism.

CONTEXT: Data on pituitary imaging in adult male patients presenting with hypogonadotrophic hypogonadism (HH) and no known pituitary disease are scarce. OBJECTIVE: To assess the usefulness of pituitary imaging in the evaluation of men presenting with HH after excluding known pituitary disorders and hyperprolactinemia. DESIGN: A historical prospective cohort of males with HH. PATIENTS: Men who presented for endocrine evaluation from 2011 to 2014 with testosterone levels <10.4 nmol/L (300 ng/mL), normal LH and FSH levels and no known pituitary disease. RESULTS: Seventy-five men were included in the analysis. Their mean age and BMI were 53.4 ± 14.8 years and 30.7 ± 5.2 kg/m2, respectively. Mean total testosterone, LH, and FSH were 6.2 ± 1.7 nmol/L, 3.4 ± 2 and 4.7 ± 3.1 mIU/L, respectively. Prolactin level within the normal range was obtained in all men (mean 161 ± 61, range 41-347 mIU/L). Sixty-two men had pituitary MRI and 13 performed CT. In 61 (81.3%) men pituitary imaging was normal. Microadenoma was found in 8 (10.7%), empty sella and thickened pituitary stalk in one patient (1.3%) each. In other four patients (5.3%) a small or mildly asymmetric pituitary gland was noted. No correlation was found between testosterone level and the presence of pituitary anomalies. CONCLUSIONS: This study suggests that the use of routine hypothalamic-pituitary imaging in the evaluation of IHH, in the absence of clinical characteristics of other hormonal loss or sellar compression symptoms, will not increase the diagnostic yield of sellar structural abnormalities over that reported in the general population.

[Related factors analysis of spontaneous cerebrospinal fluid leak recurrence after endoscope operation].

OBJECTIVE: To Summarize and analyze the clinical characteristics and treatment of patients with spontaneous cerebrospinal fluid rhinorrhea, and to explore the related factors of recurrence. METHOD: Retrospective- ly analyze the clinical data of 58 patients of spontaneous cerebrospinal fluid rhinorrhea from July 2002 to July 2012, combined with its clinical characteristics, and statistically analyze the related recurrence factors. RESULT: Fifty-eight cases were accepted the nasal endoscopic repairment of cerebrospinal fluid rhinorrheak, follow-up 3 years, 20 cases (34.5%) recurred, 1 case recurred in half a year after operation. In the first year, there were 10 cases recurred. In the second year there were 9 cases recurred, and 1 case recurred in the third year. Through multiariable analysis it was found that higher BMI, empty sella and skull base bone defect were the independent risk factors influencing the recurrence of spontaneous cerebrospinal fluid rhinorrhea (P < 0.05). CONCLUSION: The recurrence rate of spontaneous cerebrospinal fluid rhinorrhea is high, needing long-term follow-up. Higher BMI, empty sella, skull base bone defect are independent risk factors of recurrence of the disease, and the treatment should be individualized.

Sheehan's syndrome with cardiac arrest: a case report and review of the literature.

A 62-year-old woman was admitted to our hospital because of unconsciousness and hypoglycaemia. She had a history of weakness and fatigue after postpartum haemorrhage in 1983. Unfortunately, she was not diagnosed with Sheehan's syndrome and did not receive sufficient professional treatment due to the limited medical resources in her hometown. A laboratory examination at the local hospital revealed severe hypoglycaemia (1.8 mmol/L) with relatively low serum potassium (2.9 mmol/L). The woman appeared twice in the hospital with cardiac arrest, and her medical history, and the later laboratory investigations were consistent with Sheehan's syndrome. Empty sella was also found by magnetic resonance imaging (MRI). We conclude that Sheehan's syndrome may lead to cardiac arrest with the complication of hypokalaemia and deserves the vigilance of clinicians.

A rare case of pituitary infarction leading to spontaneous tumour resolution and CSF-sella syndrome in an 11-year-old girl and a review of the paediatric literature.

Pituitary infarction or apoplexy with spontaneous cure of the underlying pituitary adenoma is rare. In the paediatric population, we found only a few reported cases. We report a rare case of pituitary infarction progressing to CSF-sella syndrome (or empty sella) in an 11-year-old girl. She presented with sudden onset vomiting, moderate headaches, lethargy, weight loss, and tall stature above her mid-parental height. She did not have any severe symptoms of apoplexy. Her clinical and radiological findings suggested infarction of a pituitary lesion, such as a pituitary adenoma or infarction of a cystic lesion, such as a Rathke's cleft cyst. In this report, we discuss her case of probable infarction of a growth hormone secreting adenoma with a phase of accelerated growth ending up with total anterior pituitary insufficiency. The differential diagnosis and review of the rare cases of paediatric pituitary infarction in the literature will be discussed.

Effect of primary empty sella syndrome on pituitary surgery for Cushing's disease.

OBJECT: Primary empty sella syndrome (ESS) results from herniation of arachnoid mater into the pituitary fossa. It has been suggested to have a negative effect on pituitary surgery; however, outcomes in this cohort have not been defined. This study was performed to determine the effect of ESS on immediate and long-term biochemical outcome after pituitary surgery for Cushing's disease (CD). METHODS: Using a matched cohort study design, the authors followed patients treated with pituitary surgery for CD with and without ESS. Complete ESS was defined as pituitary gland height ≤ 2 mm, whereas partial ESS was defined as pituitary gland height > 2 mm but less than three-quarters of the total sellar depth. The primary end points were immediate and long-term biochemical outcome. Cerebrospinal fluid leaks were recorded as a secondary end point. RESULTS: Seventy-eight patients with CD and primary ESS were identified and matched with 78 patients with CD without ESS. After surgical management, immediate biochemical remission was achieved in 69 patients (88%) with ESS and 75 controls (96%, p = 0.10). Long-term remission was achieved in most patients in both groups (5-year cure: 85% vs 92%, p = 0.10). Among patients with ESS, the presence of complete ESS predicted a worse long-term outcome (p = 0.04). Intraoperative CSF leaks were significantly more frequent with ESS (54% vs 24%, p < 0.001), and despite sellar floor repair, the rate of postoperative CSF leaks was also increased (6% vs 3%, p = 0.27). CONCLUSIONS: Biochemical outcome after pituitary surgery for CD was worse in patients with complete ESS, and the risk of a CSF leak was increased with both partial and complete ESS. However, as outcome remains superior to those following alternative therapies and the biology of these tumors is unchanged in the setting of ESS, pituitary surgery should remain the initial treatment of choice.

Ectopic pituitary adenoma associated with an empty sella presenting with hearing loss: case report with literature review.

Ectopic pituitary adenomas are uncommon entities that may pose substantial diagnostic challenges. In the majority of these cases, patients present with endocrine and/or nasal obstruction symptoms. We report the case of an ectopic pituitary adenoma in a 76-year-old man with an empty sella who initially presented with right-sided hearing loss progressing to bilateral hearing loss over the next 4 years. Neuroimaging studies revealed a large, expansile central skull base mass replacing the clivus and sphenoid sinus, and invading the internal auditory canals and inner ear bilaterally. The tumor also involved the floor of the middle cranial fossae and bilateral medial temporal and occipital bones. Histopathologic examination, including immunohistochemical studies, revealed a sparsely granulated lactotroph adenoma. Hearing loss in a patient with ectopic pituitary adenoma constitutes an extremely unusual presentation. This case was further complicated by the presence of an empty sella and the absence of symptoms related to hyperprolactinemia.

Spontaneous lateral sphenoid cephaloceles: anatomic factors contributing to pathogenesis and proposed classification.

SUMMARY: Spontaneous lateral sphenoid cephaloceles arise from bony defects in the lateral sphenoid, in the absence of predisposing factors such as trauma, surgery, mass, or congenital skull base malformation. We reviewed CT and MR imaging findings and clinical data of 26 patients with spontaneous lateral sphenoid cephaloceles to better understand anatomic contributions to pathogenesis, varying clinical and imaging manifestations, and descriptive terminology. Two types of spontaneous lateral sphenoid cephaloceles were identified. In 15 of 26 patients, a type 1 spontaneous lateral sphenoid cephalocele was noted, herniating into a pneumatized lateral recess of the sphenoid sinus, and typically presenting with CSF leak and/or headache. In 11 of 26 patients, a type 2 spontaneous lateral sphenoid cephalocele was noted, isolated to the greater sphenoid wing without extension into the sphenoid sinus, presenting with seizures, headaches, meningitis, cranial neuropathy, or detected incidentally. All patients had sphenoid arachnoid pits, and 61% of patients had an empty or partially empty sella, suggesting that altered CSF dynamics may play a role in their genesis.

Osteoporotic fractures and persistent non-fusion of the hand epiphyses caused by empty sella syndrome in an adult: a case report.

We report a case of primary empty sella syndrome (ESS) resulting in osteoporotic fractures and persistent non-fusion of the hand epiphyses, and discuss the potential pathogenesis of this disease. A 41-year-old man presented with pain in the right hand and back after a fall. X-radiographs revealed persistent epiphyses and severe osteoporosis. Serum phosphorus and prolactin levels were above normal levels, and free triiodothyronine, free thyroxine and testosterone levels were below normal limits. Magnetic resonance imaging of the head revealed empty sella. A lumbar bone mineral density examination indicated severe osteoporosis. ESS caused a systemic hormone disorder in this patient, resulting in osteoporotic fractures and persistent non-fusion of the hand epiphyses. Possible causes of this anomaly are chronic or congenital abnormities of the pituitary gland.

Autoimmune hypophysitis may eventually become empty sella.

Autoimmune hypophysitis (AH) is commonly believed to be a rare chronic inflammatory condition of the pituitary gland. In clinical practice, however, the disease is often seen indeed. It typically presents with hypopituitarism and pituitary mass found by MRI. We report here unusual presentations of two females with AH followed by empty sella syndrome. The two females, aged at 64 and 57-years-old, presented with anterior pituitary dysfunction, diplopia and diabetes insipidus. By MRI the two patients shared the common characteristics with diffuse homogenous contrast enhancement of the gland and increased stalk thickness. After a long period treatment with glucocorticoids, empty sella was eventually detected by MRI.