Abstract
Congenital diabetes insipidus has not previously been reported in association with gastroschisis and its' associated anomalies. This is an unusual case of an in utero cerebral vascular accident causing diabetes insipidus, optic atrophy and left cerebral hemisphere atrophy. A 36 week gestation infant with a birth-weight of 2.7 Kg. was noted to have herniation of the abdominal wall exposing intestinal contents at birth requiring surgery in the first hours of life. On the 10th post-operative day diabetes insipidus was noted. Serum sodium was 160 mEq/L, serum osmolality was 312, urine osmolality = 83. Aqueous pitressin was administered I.M. resulting in brisk response. The management of a diabetes insipidus in this infant was easier to control with dilute intra-nasal desmopressin acetate (DDAVP) as compared to pitressin tannate in oil injections. TSH, growth hormone and metyrapone-pituitary adrenal axis studies were normal. CAT-scan of the head revealed left cerebral atrophy resulting from a vascular accident. In conclusion, this case represents an in utero cerebral vascular accident causing diabetes insipidus, optic atrophy and left cerebral atrophy associated with gastroschisis. Recent evidence shows gastroschisis to represent an in utero vascular accident to the abdominal wall musculature.
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Linarelli, L. Neonatal Diabetes Insipidus Associated with Optic Atrophy, Cerebral Atrophy and Gastroschisis. Pediatr Res 15, 1562 (1981). https://doi.org/10.1203/00006450-198112000-00162
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DOI: https://doi.org/10.1203/00006450-198112000-00162