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Central Diabetes Insipidus in Children and Adolescents: Twenty-Six Year Experience from a Single Centre
Hüseyin Anil Korkmaz1,2, Ritika R Kapoor1,3, Jennifer Kalitsi1,4
1Paediatric Endocrinology, Variety Club Children's Hospital, King's College Hospital NHS Foundation Trust, London, UK.
Insights
Central diabetes insipidus (CDI) in children is often caused by craniopharyngioma or acute CNS insults. Continued neuroimaging is crucial for idiopathic CDI cases, as some progress over time.
Area of Science:
- Pediatric Endocrinology
- Neuroendocrinology
- Pediatric Oncology
Background:
- Central diabetes insipidus (CDI) is a rare condition in children with varied causes.
- Understanding the specific etiologies and long-term outcomes is crucial for effective management.
Purpose of the Study:
- To determine the causes and long-term outcomes of central diabetes insipidus (CDI) in pediatric patients.
- To identify risk factors and prognostic indicators for CDI in children and adolescents.
Main Methods:
- A retrospective review of pediatric patients diagnosed with CDI between 1993 and 2019.
- Collection of clinical data including etiology, age at presentation, pituitary hormone deficiencies, and outcomes.
Main Results:
- Craniopharyngioma was the leading cause of CDI (n=44), frequently associated with panhypopituitarism.
- Acute central nervous system (CNS) insults led to high mortality or transient CDI.
- Idiopathic CDI was infrequent (n=5), with some cases progressing to germinoma after a median of 3.3 years.
Conclusions:
- Craniopharyngioma surgery is a primary cause of pediatric CDI with panhypopituitarism.
- Acute CNS insults in CDI patients indicate a poor prognosis.
- Extended neuroimaging follow-up (beyond 3 years) is recommended for idiopathic CDI to detect potential progression.
Introduction:
Paediatric cohorts of central diabetes insipidus (CDI) have shown varying prevalence for different causes of CDI. The objective of this study was to determine the causes of CDI and long-term outcome in children and adolescents from a Tertiary Paediatric Endocrinology unit.
Methods:
The clinic database was searched to identify patients with CDI managed between 1993 and 2019. Relevant clinical information was collected from patient records.
Results:
A total of 138 CDI patients, median age 6 years (range <1-18) at presentation, were identified. Principal CDI aetiologies were craniopharyngioma (n = 44), acute central nervous system (CNS) insult (n = 33), germinoma (n = 15), postneurosurgery (indication other than craniopharyngioma and germinoma, n = 20), midline CNS malformation (n = 14), Langerhans cell histiocytosis (n = 5), and familial (n = 2). Idiopathic CDI in this cohort was infrequent (n = 5). Patients with CNS malformations/infections presented with CDI at a younger age compared to patients with CNS tumours (p < 0.0001). Five patients, initially presenting as idiopathic CDI, were subsequently diagnosed with germinoma after a median interval of 3.3 years. All patients with CDI related to craniopharyngioma and nearly all (87%) patients with CDI related to germinoma had concomitant GH, ACTH, and TSH deficiency. The majority of patients who manifested CDI due to acute CNS insult either deceased (30%) or had transient CDI (33.3%).
Conclusion:
Surgery for craniopharyngioma was the most common underlying aetiology of CDI with ubiquitous occurrence of panhypopituitarism in these patients. Manifestation of CDI in patients with acute CNS insult carries poor prognosis. We affirm that neuroimaging assessment in idiopathic CDI should be continued beyond 3 years from diagnosis as a significant number of patients exhibited progression of infundibular thickening 3 years post-CDI diagnosis.
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