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Idiopathic Central Diabetes Insipidus Is Associated with Abnormal Blood Supply to the Posterior Pituitary Gland Caused by Vascular Impairment of the Inferior Hypophyseal Artery System

Departments of Pediatrics (M.M., M.A., N.d.I.) and Radiology (G.M.), Istituto di Ricovero e Cura a Carattere Scientifico Policlinico S. Matteo, University of Pavia, 27100 Pavia; Department of Radiology (E.G.), Università dell’Insubria, 21100 Varese; Department of Pediatrics (M.L.M.-B.), Rome "Tor Vergata", 00144 Rome; Ospedale S. Paolo (A.C.), 17100 Savona; and Department of Pediatrics (S.B.), University of Parma, 43100 Parma, Italy

Address all correspondence and requests for reprints to: Mohamad Maghnie, M.D., Ph.D., Department of Pediatrics, Istituto di Ricovero e Cura a Carattere Scientifico Policlinico S. Matteo, Viale Golgi 2, 27100 Pavia, Italy. E-mail: maghnie{at}smatteo.pv.it.

Central diabetes insipidus (CDI) has been linked to vascular central nervous system damage, although the pathophysiology of the mechanism has never been perfectly understood. Indeed, the vascular system of human pituitary gland has rarely been the subject of rigorous investigation except at postmortem. Recently, studies of pituitary gland blood supply have been carried out by means of a time evaluation of pituitary gland enhancement with noninvasive dynamic magnetic resonance (MR) imaging after contrast medium injection. In the present study, we decided to investigate the status of posterior pituitary blood supply by evaluating vascular pituitary patterns in a group of 19 patients with idiopathic CDI in whom previous standard MR imaging had failed to identify causal specific lesions. The control group was composed of 55 subjects with a median age of 12 yr (range, 4.2–17 yr) who had idiopathic isolated GH deficiency and normal pituitary morphology and 15 young adults (18–25 yr) who had normal pituitary gland and no endocrine dysfunction.

Nineteen patients (12 females and seven males), ranging in age at the time of diagnosis of CDI from 0.5–14.9 yr (median, 5 yr), were examined with dynamic MR imaging between 1990 and 1997 at a median age of 14.1 yr (range, 5.0–26.3 yr). CDI was diagnosed according to clinical findings of polyuria and polydipsia, water deprivation test, and desmopressin acetate therapeutic trial. All of the patients had permanent CDI and were being treated with satisfactory results with desmopressin, two to three times daily, either intranasally or orally. The previous MR imaging findings of the 19 CDI patients had shown the absence of posterior pituitary hyperintensity, normal pituitary stalk, and normal anterior pituitary size.

Enhancement of the straight sinus, representing a temporal reference point and occurring in normal subjects simultaneously to that of the posterior pituitary gland, was observed in all subjects after iv gadopentetate dimeglumine administration, with no substantial differences between patients and controls. However, the enhancement of the posterior pituitary lobe occurred simultaneously with the enhancement of the straight sinus in all of the controls but in only 14 of the 19 patients with CDI. In the remaining five patients, the enhancement of the straight sinus was not associated with the expected contrast enhancement of the posterior pituitary gland, suggesting abnormal blood supply to the posterior pituitary lobe. This is in keeping with vascular impairment of the inferior hypophyseal artery system and suggests that abnormal blood supply to the posterior pituitary gland is associated with what, until now, has been considered idiopathic CDI.

Abbreviations: CDI, Central diabetes insipidus; GD-DTPA, gadopentetate dimeglumine-diethylenetriamine pentaacetic acid; MR, magnetic resonance.