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Patients with pituitary stalk interruption syndrome (PSIS) are initially referred for hypoglycemia during the neonatal period or growth retardation during childhood. PSIS is either isolated (nonsyndromic) or associated with extra-pituitary malformations (syndromic). To compare baseline characteristics and long-term evolution in patients with PSIS according to the initial presentation. Sixty-seven patients with PSIS were included. Data from subgroups were compared: neonates (n = 10) versus growth retardation patients (n = 47), and syndromic (n = 32) versus nonsyndromic patients (n = 35). Neonates displayed a more severe hormonal and radiological phenotype than children referred for growth retardation, with a higher incidence of multiple hormonal deficiencies (100% versus 34%; P = 0.0005) and a nonvisible anterior pituitary lobe (33% versus 2%; P = 0.0017). Regular follow-up of growth might have allowed earlier diagnosis in the children with growth retardation, as decreased growth velocity and growth retardation were present respectively 3 and 2 years before referral. We documented a progressive worsening of endocrine impairment throughout childhood in these patients. Presence of extra-pituitary malformations (found in 48%) was not associated with more severe hormonal and radiological characteristics. Growth under GH treatment was similar in the patient groups and did not vary according to the pituitary MRI findings. PSIS diagnosed in the neonatal period has a particularly severe hormonal and radiological phenotype. The progressive worsening of endocrine impairment throughout childhood justifies periodic follow-up to check for additional hormonal deficiencies.

Abstract Context Inferior petrosal sinus sampling (IPSS) is used to diagnose Cushing’s disease (CD) when dexamethasone-suppression and CRH tests, and pituitary magnetic resonance imaging (MRI), are negative or give discordant results. However, IPSS is an invasive procedure and its availability is limited. Objective To test a noninvasive diagnostic strategy associated with 100% positive predictive value (PPV) for CD. Design Retrospective study. Setting Two university hospitals. Patients A total of 167 patients with CD and 27 patients with ectopic ACTH-syndrome investigated between 2001 and 2016. Main Outcome Measure(s) Performance of a strategy involving the CRH and desmopressin tests with pituitary MRI followed by thin-slice whole-body computed tomography (CT) scan in patients with inconclusive results. Results Using thresholds of a cortisol increase > 17% with an ACTH increase > 37% during the CRH test and a cortisol increase > 18% with an ACTH increase > 33% during the desmopressin test, the combination of both tests gave 73% sensitivity and 98% PPV of CD. The sensitivity and PPV for pituitary MRI were 71% and 99%, respectively. CT scan identified 67% EAS at presentation with no false-positives. The PPV for CD was 100% in patients with positive responses to both tests, with negative pituitary MRI and CT scan. The Negative Predictive Value was 100% in patients with negative responses to both tests, with negative pituitary MRI and positive CT scan. Using this strategy, IPPS could have been avoided in 47% of patients in whom it is currently recommended. Conclusions In conjunction with expert radiologic interpretation, the non-invasive algorithm studied significantly reduces the need for IPSS in the investigation of ACTH-dependent Cushing’s syndrome.

PurposeTo make individualised preoperative prediction of non-functioning pituitary adenoma (NFPAs) subtypes between null cell adenomas (NCAs) and other subtypes using a radiomics approach.MethodsWe enrolled 112 patients (training set: n = 75; test set: n = 37) with complete T1-weighted magnetic resonance imaging (MRI) and contrast-enhanced T1-weighted MRI (CE-T1). A total of 1482 quantitative imaging features were extracted from T1 and CE-T1 images. Support vector machine trained a predictive model that was validated using a receiver operating characteristics (ROC) analysis on an independent test set. Moreover, a nomogram was constructed incorporating clinical characteristics and the radiomics signature for individual prediction.ResultsT1 image features yielded area under the curve (AUC) values of 0.8314 and 0.8042 for the training and test sets, respectively, while CE-T1 image features provided no additional contribution to the predictive model. The nomogram incorporating sex and the T1 radiomics signature yielded good calibration in the training and test sets (concordance index (CI) = 0.854 and 0.857, respectively).ConclusionThis study focused on the preoperative prediction of NFPA subtypes between NCAs and others using a radiomics approach. The developed model yielded good performance, indicating that radiomics had good potential for the preoperative diagnosis of NFPAs.Key points• MRI may help in the pre-operative diagnosis of NFPAs subtypes• Retrospective study showed T1-weighted MRI more useful than CE-T1 in NCAs diagnosis• Treatment decision making becomes more individualised• Radiomics approach had potential for classification of NFPAs

A major concern associated with ZIKV infection is the increased incidence of microcephaly with frequent calcifications in infants born from infected mothers. To date, postmortem analysis of the central nervous system (CNS) in congenital infection is limited to individual reports or small series. We report a comprehensive neuropathological study in ten newborn babies infected with ZIKV during pregnancy, including the spinal cords and dorsal root ganglia (DRG), and also muscle, pituitaries, eye, systemic organs, and placentas. Using in situ hybridization (ISH) and electron microscopy, we investigated the role of direct viral infection in the pathogenesis of the lesions. Nine women had Zika symptoms between the 4th and 18th and one in the 28th gestational week. Two babies were born at 32, one at 34 and 36 weeks each and six at term. The cephalic perimeter was reduced in four, and normal or enlarged in six patients, although the brain weights were lower than expected. All had arthrogryposis, except the patient infected at 28 weeks gestation. We defined three patterns of CNS lesions, with different patterns of destructive, calcification, hypoplasia, and migration disturbances. Ventriculomegaly was severe in the first pattern due to midbrain damage with aqueduct stenosis/distortion. The second pattern had small brains and mild/moderate (ex-vacuo) ventriculomegaly. The third pattern, a well-formed brain with mild calcification, coincided with late infection. The absence of descending fibres resulted in hypoplastic basis pontis, pyramids, and cortico-spinal tracts. Spinal motor cell loss explained the intrauterine akinesia, arthrogryposis, and neurogenic muscle atrophy. DRG, dorsal nerve roots, and columns were normal. Lympho-histiocytic inflammation was mild. ISH showed meningeal, germinal matrix, and neocortical infection, consistent with neural progenitors death leading to proliferation and migration disorders. A secondary ischemic process may explain the destructive lesions. In conclusion, we characterized the destructive and malformative consequences of ZIKV in the nervous system, as reflected in the topography and severity of lesions, anatomic localization of the virus, and timing of infection during gestation. Our findings indicate a developmental vulnerability of the immature CNS, and shed light on possible mechanisms of brain injury of this newly recognized public health threat.

ABSTRACT The pituitary and pineal glands are two small yet critical brain structures that help to modulate the human endocrine system. Unfortunately, very little research has been devoted to segmenting the pineal gland, and existing methods for pituitary segmentation focus only on the entire gland without distinguishing between its two lobes. To fill this gap, this work presents the first deep‐learning‐based tool for segmentation of both the pineal and pituitary glands in T1‐weighted MRI. A five‐fold cross‐validation study was conducted on a manually labeled training dataset and produced segmentations with accuracy comparable to similar methods for segmenting other small brain structures. Model performance was then tested in three publicly available datasets using a total of n = 816 subjects, the results of which were both highly reproducible and robust to differences in MRI scanners and acquisition protocols. Finally, an analysis was performed to identify group differences related to sex and the diagnosis of schizophrenia and showed that volumes measured from the output segmentations were effective at discerning sex‐ and disease case‐related differences in the pituitary and pineal glands. This work presents a deep‐learning‐based tool for automatic segmentation of the pituitary and pineal glands. It has two novel aspects: it considers the anterior and posterior lobes as separate labels rather than a single, combined structure, and it is also the first deep‐learning protocol for pineal gland segmentation.

Background and Purpose The infundibular recess (IR), commonly illustrated as a V -shaped hollow in the sagittal view, is recognized as a small extension of the third ventricle into the pituitary stalk. The precise morphology of the human IR is unknown. The present study sought to delineate the morphology of the IR using magnetic resonance imaging. Materials and Methods Subjects included 100 patients without acute cerebral infarcts, intracranial hemorrhage, intrasellar or suprasellar cysts, hydrocephalus, inflammatory disease, or brain tumors. Patients with symptoms of increased intracranial pressure, intracranial hypotension, or pituitary dysfunction were excluded. Thin-sliced, seamless T2-weighted sequences involving the optic chiasm, entire pituitary stalk, and pituitary gland were performed in axial and sagittal planes for each patient. The numbers of slices delineating the pituitary stalk and IR were recorded from the axial images and quantified as ratios. Results The pituitary stalk consistently appeared as a styloid- or cone-shaped structure with variable inclinations toward the third ventricle floor. The IR was delineated as a smoothly tapering, tubular extension of the third ventricle located in the central portion of the pituitary stalk. In 81 % of patients, the IR passed through the entire length of the pituitary stalk and reached the upper surface of the pituitary gland, which was identified in 40 % of the midsagittal images. Conclusions The IR is a cerebrospinal fluid-filled canal passing through the center of the pituitary stalk and connects the third ventricle to the pituitary gland. It may function in conjunction with the pituitary gland.

Abstract Context Silent pituitary adenomas are anterior pituitary tumors with hormone synthesis but without signs or symptoms of hormone hypersecretion. They have been increasingly recognized and represent challenging diagnostic issues. Evidence Acquisition A comprehensive literature search was performed using MEDLINE and EMBASE databases from January 2000 to March 2018 with the following key words: (i) pituitary adenoma/tumor and nonfunctioning; or (ii) pituitary adenoma/tumor and silent. All titles and abstracts of the retrieved articles were reviewed, and recent advances in the field of silent pituitary adenomas were summarized. Evidence Synthesis The clinical and biochemical picture of pituitary adenomas reflects a continuum between functional and silent adenomas. Although some adenomas are truly silent, others will show some evidence of biochemical hypersecretion or could have subtle clinical signs and, therefore, can be referred to as clinically silent or “whispering” adenomas. Silent tumors seem to be more aggressive than their secreting counterparts, with a greater recurrence rate. Transcription factors for pituitary cell lineages have been introduced into the 2017 World Health Organization guidelines: steroidogenic factor 1 staining for gonadotroph lineage; PIT1 (pituitary-specific positive transcription factor 1) for growth hormone, prolactin, and TSH lineage, and TPIT for the corticotroph lineage. Prospective studies applying these criteria will establish the value of the new classification. Conclusions A concise review of the clinical and pathological aspects of silent pituitary adenomas was conducted in view of the new World Health Organization classification of pituitary adenomas. New classifications, novel prognostics markers, and emerging imaging and therapeutic approaches need to be evaluated to better serve this unique group of patients. We present a concise review of the clinical and pathological aspects of silent pituitary adenomas in view of the new World Health Organization classification of pituitary adenomas.

The purpose of the study is to evaluate the positive predictive value of the combined pituitary dynamic enhanced MRI (dMRI) and high-dose dexamethasone suppression tests (HDDST) in the diagnosis of Cushing’s disease (CD) bypassing bilateral inferior petrosal sinus sampling (BIPSS). A total of 118 patients with Cushing’s syndrome (CS), who underwent pituitary dMRI, HDDST and BIPSS were included. Positive predictive value of pituitary dMRI, HDDST, BIPSS and the combined test were calculated and tumor lateralization accuracy was further analyzed. The positive predictive value of the combined pituitary dMRI and HDDST was 98.6%, higher than that of BIPSS. There were 96.8% of patients, who had either negative findings in pituitary dMRI or HDDST, showing centralizing BIPSS results. For tumor lateralization, the accuracy by pituitary dMRI was 88.6%, whereas BIPSS was 57.5%. Therefore, CS patients with both positive findings in pituitary dMRI and HDDST need no further invasive evaluation to establish the definite diagnosis of CD. BIPSS will improve the diagnostic accuracy when negative findings were found in either pituitary dMRI or HDDST.

The definitive treatment for pituitary adenoma is transsphenoidal surgical resection. Conventional white light imaging shows limited contrast between the adenoma and the pituitary gland, and only the tissue surface is visualized, leaving a pressing unmet need for improved intraoperative adenoma delineation to preserve pituitary function during surgery. To evaluate the potential of multispectral imaging to enhance visualization of adenoma during transsphenoidal resection. A multispectral camera based on a spectrally resolved detector array was coupled to a standard 4-mm rigid endoscope for imaging, such that the camera head could easily be switched with the standard of care camera head during surgery. The multispectral imaging (MSI) endoscope was deployed during transsphenoidal surgery, and usable data were obtained from 12 patients. MSI was able to distinguish between an adenoma and a healthy pituitary based on the spectral angle with the reference spectrum of blood. The MSI endoscope holds the potential to differentiate adenoma tissue and healthy pituitary. With further development, MSI endoscopy could enable real-time label-free delineation of tumors during surgery, based on quantitative thresholds, which should contribute to improving the completeness of resection, while helping to preserve the pituitary gland, preventing serious life-changing complications.