Empty sella syndrome, panhypopituitarism, and diabetes insipidus.

We present an 18-month-old girl with short stature, obesity, panhypopituitarism, diabetes insipidus, and visual defects. Postmortem examination revealed brain atrophy due to a diffuse encephalopathy, numerous calcified neurons in cerebral cortex, deep telencephalic and diencephalic nuclei, diffuse neuronal necrosis in hypothalamic nuclei, moderate atrophy of optic nerves, very thin hypophyseal stalk, and empty sella with the hypophysis compressed to the dorsal aspect of the concavity. Our hypothesis is that the presence of an empty sella in a child with hypophyseal-hypothalamic abnormalities should alert physicians to the existence of hypothalamic lesions secondary to a perinatal insult. We discuss the possible pathogenesis of these findings as well as lines of evidence available in the literature.

Tratamento cirúrgico da Sela Vazia / Surgical treatment of Empty Sella

Os autores apresentam neste trabalho de revisão da literatura, várias séries dedicadas ao estudo da anatomia da sela túrcica, assim como à fisiopatologia, às principais formas de manifestações clínicas, bem como as modalidades diagnósticas empregadas e as principais indicações de intervenção neurocirúrgica no tratamento da sela vazia primária. Relatam as diversas modalidades terapêuticas empregadas pelos diversos autores, assim como os resultados alcançados pelos mesmos. Concluem que entre as várias proposições cirúrgicas, as mais importantes e correntemente usadas são as seguintes: a derivação ventrículo peritoneal, a craniotomia subfrontal e a abordagem transesfenoidal, que é a via mais comumente utilizada atualmente.

The authors present in this review several series aiming et in sellar anatomy, as well as physiopathology, the main clinical manifestations, and the diagnostic resources employed, and the most important indications for neurosurgical interventions in the treatment of primary empty sella. They report the different therapeutic modalities that have been used by the several authors, as well as their results. The authors conclude that among many surgical propositions the most important and used are: peritoneal shunt, subfrontal craniotomy and trans-sphenoidal approach - the latter being the mot used presently.

Análisis clínicos y de la función hipofisiaria en pacientes con el síndrome de la silla turca vacía primaria / Analysis of clinical and hypophyseal function in patients with primary empty sella turcica syndrome

Se informan los hallazgos clínicos, hormonales y radiológicos en 22 casos de silla turca vacía primaria (STVP), en pacientes que tenían un promedio de edad de 44,5 años y además predominó en mujeres. El cuadro clínico consistió en cefalea (90,9%), síndrome quiasmático (18%) y fístula del líquido cefalorraquideo en 13,6 por ciento. Todos los pacientes tenían obesidad y prácticamente la mitad (45.4%) padecián hipertensión arterial. El diagnóstico se confirmó mediante una tomografia axial computada de cráneo usando como medio de contraste a la metrizamida. En todos los casos se encontró que la función hipofisaria, evaluada por la concentración de las hormonas circulantes, era normal. La presencia de fístula de líquido cefalorraquideo o de un síndrome quiasmático fueron las indicaciones para realizar cirugía transesfenoidal. Siete pacientes que fueron sometidos a cirugía, seis evolucionaron satisfactoriamente

Hypothalamic-pituitary dysfunction in primary empty sella syndrome in childhood.

In a series of 37 consecutive CT scans performed in children referred to our pediatric endocrine unit, an empty (eight) or partially empty (one) sella turcica was found in nine (24%) patients with short stature or delay in sexual maturation, precocious puberty, or hypoparathyroidism. The size and contour of the sella were abnormal in only three patients. Five of the nine children had evidence of decreased growth hormone secretion as determined by subnormal GH secretory responses to provocative tests (peak GH concentration less than 7 ng/ml) or assessment of endogenous 24-hour GH secretion (mean 24-hour GH concentration less than 3 ng/ml). Two children had multiple pituitary hormone deficiencies. Although primary empty sella syndrome was often associated with hypothalamic-pituitary dysfunction in this series, the prevalence of an empty sella in normal children is unknown. Further identification and evaluation of children with empty sella may provide new information regarding the cause of pituitary dysfunction in childhood.