ESPR EUROPEAN SOCIETY FOR PEDIATRIC RESEARCH Siena, Italy August 31, 2005 – September 3, 2005

330 Neonatal Panhypopituitarism, Agenesis of the Carotid Artery and Midline CNS Abnormalities


Background: Panhypopituitarism is a condition characterized by inadequacy or absence of all anterior pituitary hormones due to a variety of disorders, resulting in the destruction of substantially all the anterior pituitary gland. The lack of pituitary hormones causes a reduction in the normal functioning of the endocrine glands.

Case report: Preterm baby, born at 37 weeks of gestation after emergency caesarean section for acute fetal distress. Apgar at 1 min= 4, at 5 min= 6; intubated and admitted to NICU, assisted ventilation for RDS. Clinical signs at birth: systemic hypotension, hyponatremia, craniofacial dysmorphism (right microphtalmia, right preauricolar tags, right face hypoplasia, cleft palate and bifid uvula). Additional features: patent ductus arteriosus, pneumothorax, pulmonary hypertension, persistent jaundice. Low levels of T4 and TSH were detected at neonatal screening. Substitutive therapy with oral L-tiroxina, hydrocortisone and subcutaneous GH was started and normalization of hormonal profile was followed.

Clinical and laboratory investigations: Low levels of FT4 (0,38 ng/ml), TSH (0,13 mUI/l) and basal cortisol (0,35 ng/ml) were detected. Levels of ACTH, GH and PRL were undetectable. Abdominal ultrasonography, eye fundus examination, auditory screening were normal. Brain RMI and angio-RMI showed adenohypophysial agenesis with ectopical neurohypophysis and agenesis of internal right carotid artery.

Discussion: We describe a newborn with panhypopituitarism, agenesis of the carotid artery and midline CNS abnormalities. This report supports the theory of an embryologic defect as the cause of the pituitary insufficiency. Indeed during embryogenesis the internal carotid artery and the pituitary gland develop in the 4th embryonic week. A possible relationship exists between internal carotid artery agenesis, pituitary aplasia or hypoplasia and midline CNS abnormalities.

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Scarinci, R., De Marco, L., Perrone, S. et al. 330 Neonatal Panhypopituitarism, Agenesis of the Carotid Artery and Midline CNS Abnormalities. Pediatr Res 58, 411 (2005).

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