Pituitary Morphology and Function in 43 Children with Central Diabetes Insipidus

Abstract

Objective. In pediatric central diabetes insipidus (CDI), etiology diagnosis and pituitary function monitoring are usually delayed. This study aimed to illustrate the importance of regular follow-up and pituitary function monitoring in pediatric CDI. Methods. The clinical, hormonal, and neuroradiological characteristics of children with CDI at diagnosis and during 1.5–2-year follow-up were collected and analyzed. Results. The study included 43 CDI patients. The mean interval between initial manifestation and diagnosis was 22.29 ± 3.67 months (range: 2–108 months). The most common complaint was polyuria/polydipsia. Causes included Langerhans cell histiocytosis, germinoma, and craniopharyngioma in 2, 5, and 4 patients; the remaining were idiopathic. No significant changes were found during the 1.5–2 years after CDI diagnosis. Twenty-three of the 43 cases (53.5%) had ≥1 anterior pituitary hormone deficiency. Isolated growth hormone deficiency was the most frequent abnormality (37.5%) and was not associated with pituitary stalk diameter. Multiple pituitary hormone deficiencies were found in 8 cases with pituitary stalk diameter > 4.5 mm. Conclusion. Diagnosis of CDI is usually delayed. CDI with a pituitary stalk diameter > 4.5 mm carries a higher risk of multiple pituitary hormone deficiencies. Long-term MRI and pituitary function follow-ups are necessary for children with idiopathic CDI.

Evolving pituitary hormone deficits in primarily isolated GHD: a review and experts’ consensus

“…Therefore, the occurrence of diabetes insipidus in children with isolated GHD should always arouse suspicion of the presence of histiocytosis, germinoma, or infundibulitis, which all can cause GHD. In such children, a lesion of the pituitary stalk and the pituitary gland should be excluded by cranial magnetic resonance imaging (cMRI) with gadolinium contrast [ 61 ].…”

Central diabetes insipidus and pituitary stalk thickening in adults: distinction of neoplastic from non-neoplastic lesions

“…Adult patients with pituitary stalk lesions commonly present with varying degrees of central diabetes insipidus (CDI), hypopituitarism, and hyperprolactinemia. In particular, the association of CDI and pituitary stalk thickening (PST) may be caused by several diseases, including neoplastic, infectious, and inflammatory conditions (1,2,3,4,5,6,7,8,9,10,11), and differential diagnosis is mandatory in order to propose an appropriate treatment and follow-up. This diagnosis remains challenging, principally for two reasons: (i) there are few specific signs on MRI, which may help to identify the underlying disease (3) and (ii) biopsies of lesions in this area are not often performed as they may be associated with significant morbidity such as cerebrospinal fluid (CSF) leak, meningitis or permanent panhypopituitarism (12).…”

Clinicopathological features and BRAFV600E mutations in patients with isolated hypothalamic-pituitary Langerhans cell histiocytosis

“…Recently, the use of neuroendoscopic biopsy for suprasellar masses has increased, and with this technique, it is possible to obtain an accurate pathological diagnosis [ 24 ], although sometimes the pathological diagnosis is difficult due to small specimens with prominent sampling artefacts and haemorrhage [ 25 ]. Distinguishing this condition from other diseases that can cause CDI, such as craniopharyngioma, germinoma, sarcoidosis, hypophysitis, and tuberculosis, is essential [ 2 , 10 , 26 ], and the pathological signature with both CD1a-positive, Langerin-positive, and S-100-positive infiltrating histiocytes and increased presentation of eosinophils has made the differential diagnosis relatively easy. In addition, differentiation from other non-Langerhans cell histiocytoses, such as Erdheim-Chester disease or Rosai-Dorfman disease, is also important.…”