Endocrine Abstracts

JOINT3486

Introduction: Craniopharyngioma is a rare benign epithelial tumor in children, typically located near the pituitary stalk. Although benign and extracerebral, this tumor remains serious due to frequent visual, neuro-intellectual, and endocrine sequelae, including pituitary insufficiency, growth retardation, pubertal delay, and diabetes insipidus. However, significant progress in surgery, radiotherapy, and medical management has markedly improved the prognosis of this disease.

Case report: This is a 14-year-old patient who, in 2019, developed a polyuric-polydipsic syndrome (PUPD) that significantly impaired his daily life. This led his family to consult a pediatrician, where a screening assessment for diabetes was conducted, which returned normal results, leading to a diagnosis of psychogenic PUPD. Three years later, the condition worsened, with the onset of a visual disturbance (bilateral diplopia), prompting an ophthalmologic examination followed by a brain CT scan, which suggested the presence of a craniopharyngioma, leading to his transfer to our facility for specialized care.

Discussion: Diabetes insipidus (DI) is a polyuric-polydipsic syndrome (PUPD) characterized by the excretion of large volumes of diluted, hypotonic urine due to a deficiency of antidiuretic hormone (ADH), leading to thirst and polydipsia. This condition is caused by damage to the posterior pituitary gland, often due to tumors such as craniopharyngiomas, infiltrative, traumatic, or vascular lesions, resulting in a deficiency in the production of antidiuretic hormone (arginine vasopressin). DI has been shown to occur in 46.8% of pediatric craniopharyngiomas. This central form of diabetes insipidus must be distinguished from other causes of polyuria and polydipsia, particularly psychogenic and nephrogenic causes, which are more common Treatment is primarily based on hormonal replacement, with desmopressin (Minirin) being the preferred synthetic analogue of ADH, offering a potent antidiuretic effect. In addition, proper hydration and treatment of the underlying etiology must be ensured, along with regular clinical monitoring. Nowadays, optimal management of cranopharungioma is based on a multimodal strategy involving a combination of surgery and, in some cases, radiotherapy, with the aim of controlling the disease and limiting morbidity.

Conclusion: DI is a rare condition that requires a thorough etiological investigation, with MRI playing a crucial role in diagnosis From our observation, we note that diabetes insipidus can be the first clinical sign of a craniopharyngioma, which must be differentiated from psychogenic causes. Early recognition is essential for effective treatment. Recognizing this association is crucial for early diagnosis and effective treatment, which can lead to improved long-term outcomes.