Gadde Judith,  Rebsamen Susan,  Kennedy Tabassum

Final Pr. ID: Poster #: EDU-040

1. To briefly review the embryology and anatomy of the pituitary gland.
2. To illustrate the imaging spectrum of duplication of the pituitary gland through an example, to include often associated anomalies such as thickening of the hypothalamus and a palatal teratoma.
3. To review the theories of pathogenesis leading to duplication of the pituitary gland. Read More

Authors:  Gadde Judith , Rebsamen Susan , Kennedy Tabassum

Keywords:  Duplication, pituitary, Embryology, Teratoma

Sarma Asha,  Knake Lindsey,  Whigham Amy,  Hernanz-schulman Marta,  Anani Uchenna,  Pruthi Sumit

Final Pr. ID: Poster #: CR-023

Duplication of the pituitary gland (DPG) is an extremely rare malformation, with only 40 reported cases in the medical literature. Phenotypes range from isolated DPG with normal development to cases with additional anomalies leading to morbidity and mortality (“DPG-plus” syndrome). We describe a patient with comprehensive fetal and postnatal imaging detailing known findings of DPG-plus syndrome and previously undescribed brain and body anomalies.

An infant male was diagnosed with an obstructive oral cavity mass by fetal MRI. Postnatal neuroimaging showed a fatty oral cavity mass containing dysmorphic mandibular elements. Upon biopsy, pathological assessment showed duplication of the maxillary complex versus mesenchymal hamartoma. Other anomalies included duplicated nasal cavity, absent olfactory bulbs, duplicated sella with sellar spine, optic chiasm and tubomamillary fusion, duplicated basilar artery, persistent falcine sinus, cleft palate, and bifid tongue. In addition, there was an unusual complex brainstem and cerebellar anomaly and extensive anterior cervical vertebral clefting with anterior cervicomedullary junction myelomeningocele. Body imaging showed horseshoe pulmonary sequestration, hiatal hernia, multiple intrathoracic and intraabdominal spleens in a manner inconsistent with heterotaxy, midline liver, duplicated IVC, and vertebral anomalies. Echocardiogram showed double outlet right ventricle. Genetics evaluation revealed variants of unknown significance in CCDC39, TBX5, and ZMYND10 that were considered unlikely to be related to the observed anomalies. Due to poor neurologic prognosis, the patient was transitioned to comfort care and passed away at 4 weeks of age. Read More

Authors:  Sarma Asha , Knake Lindsey , Whigham Amy , Hernanz-schulman Marta , Anani Uchenna , Pruthi Sumit

Keywords:  Duplicated pituitary gland plus, Syndrome

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