In a retrospective study, the intra- and early postoperative data of 39 children with 46 operations for craniopharyngioma were analyzed. Diabetes insipidus (DI) occurred in 30 out of 32 cases without preoperative evidence of DI. We observed that all children who did not have a pituitary stalk preserved and 5 out of 7 patients with preserved pituitary stalk developed DI within 18 h of surgery. Short-term inappropriate secretion of antidiuretic hormone (SIADH) occurred in 2 children, but was quickly followed by DI. The time of onset of DI and SIADH did not correlate with sex, age, body weight, location of tumor, or duration or extent of surgery. Parenteral desmopressin was an effective treatment for intra- and postoperative DI. The duration of the clinical effect of desmopressin administration varied in different patients between 4 and 23 h. An approach to the immediate intra- and postoperative management of children with craniopharyngioma is presented.

Ultrasonography revealed a suprasellar tumor in a fetus at 28 weeks of gestation. The male newborn, delivered 10 weeks later, was operated at the age of 17 days, and a craniopharyngioma was completely removed. Intraoperatively, inappropriate secretion of antidiuretic hormone occurred and was followed by diabetes insipidus causing imbalance of fluid and electrolytes. The tumor recurred and was totally removed 1 year later. Further development was uneventful and, at the age of 8 years, the boy is in generally good mental and physical condition except for a left-sided hemiparesis. In contrast to the poor outcome of neonatal craniopharyngioma reviewed in the literature, this case may encourage radical surgery even in the very young.

Cardiac involvement is common in adult patients with the presence of HLA B27 with or without the HLA B27-associated spondyloarthropathy ankylosing spondylitis. Most patients with juvenile spondyloarthropathy, which begins at age 16 or younger, do not have spinal involvement and there are only few reports of cardiac involvement. This study sought to assess the prevalence of carditis in patients with HLA B27-associated juvenile arthritis (B27-JA). In a controlled study, 40 patients with B27-JA, among them only 1 with ankylosing spondylitis, were examined by electrocardiogram and echocardiography with pulsed and color-flow Doppler at rest and at the termination of a bicycle exercise and compared to an age- and sex-matched control group negative for HLA B27. Four patients with B27-JA, and none in the control group, had inflammatory aortic regurgitation. Late diastolic flow velocity was significantly increased in patients with B27-JA at the termination of exercise. HLA B27 is a risk factor for endo-/myocardial damage in patients with B27-JA, even in the presence of only short and mild articular disease. Patients with B27-JA should be screened for the presence of aortic regurgitation.

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