Kim Hayoun,  Yu In Kyu

Final Pr. ID: Poster #: SCI-034

It has been known that the pituitary gland volume (PV) in idiopathic central precocious puberty (IPP) is significantly higher than in healthy children. However, most PV measurements rely on manual quantitative methods, which are time-consuming and labor-intensive. This study aims to automatically measure the PV of patients with IPP using artificial intelligence to accurately quantify the correlation between IPP and PV, and to improve the efficiency of diagnosing IPP. Read More

Authors:  Kim Hayoun , Yu In Kyu

Keywords:  Pituitary Abnormalities, Puberty, Artificial Intelligence

Chen Danling,  Kuehne Alexander,  Hwang James,  Benyakoub Amine,  Ehrlich Lauren,  Lisse Sean

Final Pr. ID: Poster #: CR-047

Pallister-Hall syndrome is an exceedingly rare genetic disorder characterized by multiorgan anomalies encompassing hypothalamic hamartoma, postaxial polydactyly, bifid epiglottis, renal malformations and imperforate anus. The syndrome arises from a mutation in the GLI3 gene, which can be inherited in autosomal dominant fashion or occur de novo. A significant manifestation of Pallister-Hall syndrome is the presence of a hypothalamic hamartoma, a benign tumor of the hypothalamus. Although benign, these lesions can cause severe endocrine dysfunction by interfering with the hypothalamic-pituitary axis. Abnormal neural or glial cell migration in the tuber cinereum of the hypothalamus leads to abnormal expansion of the hypothalamus, manifesting in a spectrum of pituitary dysfunction ranging from pituitary displacement to severe pan-hypopituitarism. A well-established feature of the hypothalamic hamartomas associated with Pallister-Hall syndrome is the incidence of gelastic seizures (so-called “laughing” seizures), characterized by episodes of involuntary laughing.

A male neonate was born with polydactyly, bifid epiglottis, cryptorchidism, and Hirschsprung’s disease. The patient was additionally found to have right sided vesicouteral reflux requiring extravesical ureteral reimplantation. Genetic analysis revealed a mutation in the GLI3 gene, compatible with Pallister-Hall syndrome. Once diagnosed, the patient underwent contrast-enhanced MR imaging of the brain which demonstrated a large lobulated 2.6 x 2.0 cm suprasellar mass with encroachment posteriorly upon the midbrain and associated superior displacement of the third ventricle. The lesion showed low-grade enhancement following gadolinium contrast administration, displayed no significant abnormality on diffusion-weighted or susceptibility weighted imaging, and was isodense to brain tissue on all sequences. Serial follow-up MRIs demonstrated minimal increase in size of the non-enhancing suprasellar mass. Findings were suggestive of hypothalamic hamartoma, in keeping with the classical presentation of Pallister-Hall syndrome. Read More

Authors:  Chen Danling , Kuehne Alexander , Hwang James , Benyakoub Amine , Ehrlich Lauren , Lisse Sean

Keywords:  Pituitary Abnormalities, Genetics, Genitourinary

Shatadal Alankrit,  Chapman Teresa

Final Pr. ID: Poster #: EDU-013

Pediatric endocrine disorders often require diagnostic imaging, either as part of the diagnostic evaluation or for follow-up of known entities. Endocrine disorders may have central causes arising from pathological processes involving the hypothalamus and pituitary gland, or they may relate to a peripheral overproduction or underproduction of hormones in the body. Depending on the endocrinopathy, imaging with radiography, ultrasound, MRI, CT, or nuclear scintigraphy may be appropriate. This educational exhibit will provide the learner with a broad example of various pediatric endocrine disorders and discuss the demographics, clinical presentations, laboratory evaluation, diagnostic considerations, and appropriate imaging for these conditions. Cases will cover the following entities as they relate to pediatric imaging:
1) Intracranial anomalies and acquired diseases associated with endocrine disorders, including congenital anomalies (agenesis of corpus callosum, absence of septum pellucidum, ectopic posterior pituitary and disruption of pituitary stalk, Kallman syndrome) as well as acquired disorders (midline pituitary microadenoma / prolactinoma, central diabetes insipidus, intrasellar tumors, hypothalamic hamartoma, diabetic ketoacidosis);
2) Thyroid diseases, including thyroiditis, thyroid papillary carcinoma and therapy;
3) Adrenal diseases, including congenital adrenal hyperplasia, adrenal neurogenic tumors, adrenal hemorrhage, and adrenal infarction;
4) Gonadal disorders, including testicular adrenal rest tumors, stromal tumors with hormone overproduction, Mayer-Rokitansky-Kuster-Hauser syndrome;
5) Skeletal disorders, such as osteomalacia. Read More

Authors:  Shatadal Alankrit , Chapman Teresa

Keywords:  Adrenal, Pituitary Abnormalities, Congenital

Lake Long Sarah,  Vaughan Bridget,  Ward Kenneth,  Bisset George,  Wasilewska Ewa,  Karcher Gerald,  Urbine Jacqueline

Final Pr. ID: Poster #: EDU-073

Objectives:

Causes of both hypo- and hyper-secretion of pituitary hormones are important developmental pediatric pathologies for which imaging is critical for diagnostic evaluation. While various etiologies cause disruption of the hypothalamic-pituitary axis, brain magnetic resonance imaging (MRI) with dedicated pituitary gland sequences are critical to the diagnosis of structural etiologies. Congenital anomalies such as ectopic posterior pituitary which is related to short statue warrants assessment for associated midline anomalies such as optic nerve hypoplasia and corpus callosal dysgenesis. Masses yielding common symtomatic causes of pituitary dysfunction such as growth hormone deficiency secondary to rathke cleft cysts or pitutary adenomas necessitate surgical evaluation. We aim to comprehensively review overall causes related to these pathologies and relevant imaging findings which are fundamental to guide further management.

Learning Points:
This educational exhibit will discuss:
1. Embryology and development of the pituitary gland
2. Pathophysiologic review of the hypothalamic-pituitary-adrenal axis
3. Imaging protocols for evaluation of central structural causes
4. Sella/pituitary normal variants
5. Overview of positive findings on brain/pituitary magnetic resonance imaging (MRI) and case examples related to pituitary hormone hyper-secretion, such as central precocious puberty
6. Overview of positive findings on brain/pituitary MRI and case examples related to hypopituitarism, such as growth hormone deficiency

Discussion/Take-Home Points:

Proper identification of various structural causes of pituitary dysfunction on brain MRI is critical to further diagnostic, medical, or surgical management in the pediatric population. Read More

Authors:  Lake Long Sarah , Vaughan Bridget , Ward Kenneth , Bisset George , Wasilewska Ewa , Karcher Gerald , Urbine Jacqueline

Keywords:  Pituitary Abnormalities, Pediatric Radiology, MRI

Rai Aayushi,  Gupta Rachita,  Setty Bindu

Final Pr. ID: Poster #: EDU-077

The pituitary gland, the epicenter of various regulatory hormones, plays an unquestionably important role in determining timely growth and sexual maturation. Indeed, multiple studies have examined the role of Growth Hormone (GH) and Gonadotrophic Releasing Hormone (GnRH) in treating delayed and precocious puberty respectively.[1]

Research has shown the importance of imaging the pituitary gland in patients with growth disturbances and/or documented endocrine abnormalities, in differentiating and classifying disorders based on etiology, prognosis and management.[2] Currently, data are limited in identifying a correlation between bone age abnormalities and pituitary findings via MRI, in children diagnosed with deviations in pubertal development. We present a comprehensive review of common structural abnormalities affecting the pituitary as seen on MRI – including developmental (dysgenesis/hypoplasia), traumatic, and neoplastic processes (both benign and malignant) – and how those pituitary lesions correlate with bone age and endocrine function in patients with growth failure and precocious puberty. Establishing a correlation between a patient’s endocrine profile, bone age and pituitary morphology on MRI imaging can be extremely useful in the judicious management of patients, in terms of patient selection, early diagnosis and treatment. Moreover, our review aims to highlight the importance of imaging in the workup of patients with known or suspected growth disturbances, illustrated via concept maps. The concordance of endocrine abnormalities and clinical information (including age, sex and ethnicity demographics) with imaging data will also be reviewed to demonstrate various patterns of disease presentation and diagnosis.

References:
[1] Du X.F., Yang X.H., Li J., Hao M., Guo Y.H. Growth hormone co-treatment within a gnrh agonist long protocol improves implantation and pregnancy rates in patients undergoing IVF-ET. Arch. Gynecol. Obstet. 2016;294:877–883. doi: 10.1007/s00404-016-4163-1.


[2] Di Iorgi N, Iorgi ND, Allegri AEM et al (2012) The use of neuroimaging for assessing disorders of pituitary development. Clin Endocrinol 76:161–176
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Authors:  Rai Aayushi , Gupta Rachita , Setty Bindu

Keywords:  Puberty, Pituitary abnormalities