The authors report 20 cases of giant craniopharyngioma in children operated on during the 6-year period from 1978 through 1984. The tumor size (maximal diameter) ranged from 5 to 11 cm. These patients presented with increased intracranial pressure, severe visual loss, and neurological and endocrinological deficits. Tumor growth had occurred in several directions, causing displacement of the circle of Willis, the optic apparatus, and the hypothalamus, as well as encroachment into the interpeduncular and prepontine cisterns and into the frontal, temporal, and posterior fossae. The surgical technique for total removal of these giant tumors in the last 10 cases is described. Emphasis is placed on the use of microsurgical technique during the first operation, close endocrinological follow-up, and early reevaluation by computed tomographic scanning.

A case is reported of a recently described neurocutaneous syndrome, manifested by a noncommunicating right lateral ventricular cyst, a left-sided lipoma of the scalp with alopecia, right-sided multiple truncal lipomas, right-sided ocular manifestations, left leg hypertrophy, and seizures. The findings in cases of encephalocraniocutaneous lipomatosis are described to demonstrate the spectrum of this syndrome.

Two infants are described with congenital cutis laxa. They represent two distinct disorders. In the first, congenital cutis laxa is associated with a generalized disorder of elastic tissue in which there may be diaphragmatic or other hernias, diverticula of the gastrointestinal or urinary tract and infantile emphysema. The disease is fatal often within the first year. In the second, congenital cutis laxa is associated with widely patent anterior fontanel, a variety of malformations, and retarded growth and development. Recognition of these distinct syndromes in the newborn period and their recessive inheritance permit realistic discussion of the prognosis which is very different from the benign dominant forms of cutis laxa.

Five female patients with primary hypothyroidism and radiological evidence of a pituitary enlargement were studied before and after a mean of 30 months (range 12–83 months) treatment with thyroxine (T₄). Before treatment, serum thyroid-stimulating hormone (TSH) levels were elevated in every patient (mean 392 mU/l, range 240–475) and prolactin levels in 4 (mean 79 µg/l, range 48–143 µg/l). CT scanning confirmed the presence of pituitary enlargement in the 4 patients studied, which was suprasellar in 3. The remaining patient had an enlarged fossa on a lateral skull radiograph. During treatment with T₄, TSH and prolactin levels were normal in all. Complete disappearance of the enlargement was seen on follow-up scans in all patients and 1 developed an empty sella. The induction of a pituitary enlargement by primary hypothyroidism results from reversible hyperplasia of both the TSH and prolactin-secreting cells in most instances. Occasionally, however, hyperplasia of the thyrotrophs can occur in isolation and an empty sella can occur after successful treatment with T₄. Thyroid function tests should be obtained in all hyperprolactinemic patients.

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