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Very rare congenital genetic disorder with hepato-reno-pancreatic involvement combined with Dandy-Walker malformation.

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Renal-Hepatic-Pancreatic Dysplasia with Dandy-Walker Cyst.

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Very rare syndrome (<10 cases worldwide) with probably autosomal recessive inheritance.

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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.

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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.

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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.

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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 106:484, 1963.  [PubMed: 14077166]
Gulcan H, Duman N, Kumral A, et al: Goldston syndrome: Report of a case. Genet Couns 12:263, 2001.  [PubMed: 11693790]
Moerman P, Pauwels P, Vandenberghe K, et al: Goldston syndrome reconsidered. Genet Couns 4:97, 1993.  [PubMed: 8357569]

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