Very rare congenital genetic disorder with
hepato-reno-pancreatic involvement combined with Dandy-Walker malformation.
Renal-Hepatic-Pancreatic Dysplasia with Dandy-Walker Cyst.
Very rare syndrome (<10 cases
worldwide) with probably autosomal recessive inheritance.
Main features are dysplastic kidneys, pancreas
and liver anomalies, and presence of Dandy-Walker malformation. Other
features can include oligoamnios, polyhydramnios, hypoplastic lungs, and
duplication of the gut.
Assess liver, renal and pancreatic function.
Obtain a complete blood cell count (renal anemia) and check blood
glucose. Check for history of apnea (Dandy-Walker malformation) and signs of increased
intracranial pressure. Assess for signs of difficult airway management.
High rate of anesthetic implications.
Hepatic, renal, neurologic, respiratory, and liver function should be assessed
and their influence on anesthetic drug metabolism and perioperative fluid regimen
should be considered. Avoid hypotension, hypercapnia and increased body temperature
in the presence of increased intracranial pressure.
In the presence of hepatic and/or renal
anomalies, cis-atracurium is the neuromuscular blocker of choice, and isoflurane
and desflurane are the inhalational anesthetics of choice, although sevoflurane is expected
not to cause any harm if used for a short period of time (e.g., induction of anesthesia).
Goldston AS, Burke EC, D'Agostino A, et al: Neonatal polycystic kidney
with brain defect. Am J Dis Child
Gulcan H, Duman N, Kumral A, et al: Goldston syndrome: Report of a case.
Moerman P, Pauwels P, Vandenberghe K, et al: Goldston syndrome reconsidered.