Abstract:BackgroundPituitary stalk thickening (PST) is a rare abnormality in the pediatric population. Its etiology is heterogeneous. The aim of the study was to identify important clinical, radiological and endocrinological manifestations of patients with PST and follow the course of the disease.Materials and MethodsIt is a study conducted in 23 patients (13 boys) with PST with/without central diabetes insipidus (CDI) diagnosed between 1990 and 2020 at Children’s Memorial Health Institute (CMHI) in Warsaw, Poland. We … Show more
“…Finally, pituitary stalk is a funnel-shaped structure that connects the hypothalamus to the pituitary gland. There are several etiologies that give rise to PST ( 30 , 31 ), which often manifests clinically with CDI ( 32 , 33 ): autoimmunity/inflammation (neuroinfundibulohypophysitis ( 34 )) – sometimes referred as idiopathic PST, infectious diseases (e.g. tuberculosis ( 35 )) or neoplastic lesions ( 33 , 36 ).…”
Section: Discussionmentioning
confidence: 99%
“…There are several etiologies that give rise to PST ( 30 , 31 ), which often manifests clinically with CDI ( 32 , 33 ): autoimmunity/inflammation (neuroinfundibulohypophysitis ( 34 )) – sometimes referred as idiopathic PST, infectious diseases (e.g. tuberculosis ( 35 )) or neoplastic lesions ( 33 , 36 ). Anti-pituitary, anti-hypothalamus autoantibodies or high IgG4 levels have been detected in some patients with hypophysitis.…”
IntroductionThe coronavirus disease 19 (COVID-19) pandemic has prompted the development of new vaccines to reduce the morbidity and mortality associated with this disease. Recognition and report of potential adverse effects of these novel vaccines (especially the urgent and life-threatening ones) is therefore essential.Case presentationA 16-year-old boy presented to the Paediatric Emergency Department with polyuria, polydipsia and weight loss over the last four months. His past medical history was unremarkable. Onset of symptoms was referred to be few days after first dose of anti-COVID-19 BNT162b2 Comirnaty vaccine and then worsened after the second dose. The physical exam was normal, without neurological abnormalities. Auxological parameters were within normal limits. Daily fluid balance monitoring confirmed polyuria and polydipsia. Biochemistry laboratory analysis and urine culture were normal. Serum osmolality was 297 mOsm/Kg H2O (285-305), whereas urine osmolality was 80 mOsm/Kg H2O (100-1100), suggesting diabetes insipidus. Anterior pituitary function was preserved. Since parents refused to give consent to water deprivation test, treatment with Desmopressin was administered and confirmed ex juvantibus diagnosis of AVP deficiency (or central diabetes insipidus). Brain MRI revealed pituitary stalk thickening (4 mm) with contrast enhancement, and loss of posterior pituitary bright spot on T1 weighted imaging. Those signs were consistent with neuroinfundibulohypophysitis. Immunoglobulin levels were normal. Low doses of oral Desmopressin were sufficient to control patient’s symptoms, normalizing serum and urinary osmolality values and daily fluid balance at discharge. Brain MRI after 2 months showed stable thicken pituitary stalk and still undetectable posterior pituitary. Due to persistence of polyuria and polydipsia, therapy with Desmopressin was adjusted by increasing dosage and number of daily administrations. Clinical and neuroradiological follow-up is still ongoing.ConclusionHypophysitis is a rare disorder characterized by lymphocytic, granulomatous, plasmacytic, or xanthomatous infiltration of the pituitary gland and stalk. Common manifestations are headache, hypopituitarism, and diabetes insipidus. To date, only time correlation between SARS-CoV-2 infection and development of hypophysitis and subsequent hypopituitarism has been reported. Further studies will be needed to deepen a possible causal link between anti-COVID-19 vaccine and AVP deficiency.
“…Finally, pituitary stalk is a funnel-shaped structure that connects the hypothalamus to the pituitary gland. There are several etiologies that give rise to PST ( 30 , 31 ), which often manifests clinically with CDI ( 32 , 33 ): autoimmunity/inflammation (neuroinfundibulohypophysitis ( 34 )) – sometimes referred as idiopathic PST, infectious diseases (e.g. tuberculosis ( 35 )) or neoplastic lesions ( 33 , 36 ).…”
Section: Discussionmentioning
confidence: 99%
“…There are several etiologies that give rise to PST ( 30 , 31 ), which often manifests clinically with CDI ( 32 , 33 ): autoimmunity/inflammation (neuroinfundibulohypophysitis ( 34 )) – sometimes referred as idiopathic PST, infectious diseases (e.g. tuberculosis ( 35 )) or neoplastic lesions ( 33 , 36 ). Anti-pituitary, anti-hypothalamus autoantibodies or high IgG4 levels have been detected in some patients with hypophysitis.…”
IntroductionThe coronavirus disease 19 (COVID-19) pandemic has prompted the development of new vaccines to reduce the morbidity and mortality associated with this disease. Recognition and report of potential adverse effects of these novel vaccines (especially the urgent and life-threatening ones) is therefore essential.Case presentationA 16-year-old boy presented to the Paediatric Emergency Department with polyuria, polydipsia and weight loss over the last four months. His past medical history was unremarkable. Onset of symptoms was referred to be few days after first dose of anti-COVID-19 BNT162b2 Comirnaty vaccine and then worsened after the second dose. The physical exam was normal, without neurological abnormalities. Auxological parameters were within normal limits. Daily fluid balance monitoring confirmed polyuria and polydipsia. Biochemistry laboratory analysis and urine culture were normal. Serum osmolality was 297 mOsm/Kg H2O (285-305), whereas urine osmolality was 80 mOsm/Kg H2O (100-1100), suggesting diabetes insipidus. Anterior pituitary function was preserved. Since parents refused to give consent to water deprivation test, treatment with Desmopressin was administered and confirmed ex juvantibus diagnosis of AVP deficiency (or central diabetes insipidus). Brain MRI revealed pituitary stalk thickening (4 mm) with contrast enhancement, and loss of posterior pituitary bright spot on T1 weighted imaging. Those signs were consistent with neuroinfundibulohypophysitis. Immunoglobulin levels were normal. Low doses of oral Desmopressin were sufficient to control patient’s symptoms, normalizing serum and urinary osmolality values and daily fluid balance at discharge. Brain MRI after 2 months showed stable thicken pituitary stalk and still undetectable posterior pituitary. Due to persistence of polyuria and polydipsia, therapy with Desmopressin was adjusted by increasing dosage and number of daily administrations. Clinical and neuroradiological follow-up is still ongoing.ConclusionHypophysitis is a rare disorder characterized by lymphocytic, granulomatous, plasmacytic, or xanthomatous infiltration of the pituitary gland and stalk. Common manifestations are headache, hypopituitarism, and diabetes insipidus. To date, only time correlation between SARS-CoV-2 infection and development of hypophysitis and subsequent hypopituitarism has been reported. Further studies will be needed to deepen a possible causal link between anti-COVID-19 vaccine and AVP deficiency.
“…The differential diagnosis on neuroimaging is not limited to other histiocytic disorders and also includes both tumoral entities, such as germ cell tumors, and autoimmune inflammatory conditions like lymphocytic hypophysitis ( 118 ).…”
Sellar/suprasellar tumors comprise about 10% of all pediatric Central Nervous System (CNS) tumors and include a wide variety of entities, with different cellular origins and distinctive histological and radiological findings, demanding customized neuroimaging protocols for appropriate diagnosis and management. The 5th edition of the World Health Organization (WHO) classification of CNS tumors unprecedently incorporated both histologic and molecular alterations into a common diagnostic framework, with a great impact in tumor classification and grading. Based on the current understanding of the clinical, molecular, and morphological features of CNS neoplasms, there have been additions of new tumor types and modifications of existing ones in the latest WHO tumor classification. In the specific case of sellar/suprasellar tumors, changes include for example separation of adamantinomatous and papillary craniopharyngiomas, now classified as distinct tumor types. Nevertheless, although the current molecular landscape is the fundamental driving force to the new WHO CNS tumor classification, the imaging profile of sellar/suprasellar tumors remains largely unexplored, particularly in the pediatric population. In this review, we aim to provide an essential pathological update to better understand the way sellar/suprasellar tumors are currently classified, with a focus on the pediatric population. Furthermore, we intend to present the neuroimaging features that may assist in the differential diagnosis, surgical planning, adjuvant/neoadjuvant therapy, and follow-up of this group of tumors in children.
“…Synchronous pineal and suprasellar tumors occur in 21% of cases [50][51]. GCT should be suspected in all children with PS thickening, especially with CDI [52]. Determining the underlying cause of CDI can be quite a challenge sometimes.…”
Section: Identi Cation Of the Underlying Cause Of CDImentioning
Background: Central diabetes insipidus (CDI) is a rare disorder in the pediatric population resulting from antidiuretic hormone (ADH) deficiency and caused by several underlying conditions. It is characterized by the excessive production of dilute urine and manifests with polyuria, nocturia, and polydipsia. An appropriate investigation requires a detailed medical history, physical examination, biochemical evaluation (including fluid deprivation test, potentially followed by desmopressin test), and neuroimaging. The diagnosis of CDI is often challenging, especially the underlying condition. The patients sometimes need long-term follow-up to establish the proper final diagnosis.
This paper highlights the diverse clinical presentation of children with CDI, diagnostic difficulties among patients presenting with polyuria and polydipsia, and the need for an individual approach in each case. The article also reviews the etiology, symptoms, diagnostic work-up, and management of CDI.
Case Presentation: We present four pediatric patients (aged 3-13.5 years) diagnosed with CDI of different etiology; one due to congenital anomaly - septo-optic-dysplasia (SOD) and three due to acquired processes such as Langerhans cell histiocytosis (LCH) and germ cell tumor (GCT) in two patients.
Conclusions: CDI was the first manifestation of a tumor or granuloma in all presented patients with acquired pathology. Due to the presented course of illness, it may be concluded, the coexistence of other pituitary hormone deficiencies at initially recognized idiopathic CDI should suggest a more complicated diagnostically problem. Case with a delayed diagnosis with SOD at the age of 13.5 years and case with GCT, where from the CDI diagnosis to the occurrence of pathological change in pituitary stalk 1.5 years have passed, highlight the need for a high clinical suspicion in patients with CDI.
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