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Very rare, non-malignant syndrome characterized by abnormal morphogenesis affecting both kidneys and lungs.

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Cystic Hamartoma of Lung and Kidney.

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Three cases have been reported worldwide. Inheritance unknown.

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Clinical features involve the lungs (hamartomatous pulmonary cysts, lung fibrosis respiratory distress, chronic inflammatory lung disease) and the genitourinary system (bilateral multilocular cysts of the kidney, abdominal masses, renovascular hypertension, cellular mesoblastic nephroma, and possible markedly hyperplastic renomegaly with medullary dysplasia).

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No literature available. Evaluate renal function (clinical, ultrasound, laboratory) and adapt intraoperative fluid regimen and drug choice (avoid nephrotoxic drugs and prefer those with nonrenal elimination). Evaluate respiratory function (clinical, chest radiographs, CT, pulmonary function tests, arterial blood gas analysis). Postoperative ventilatory support might be necessary. Perioperative chest physiotherapy can be useful. Avoid excessive airway pressures that could lead to rupture of pulmonary malformations. Intraoperative invasive blood pressure monitoring may be justified.

Graham JM Jr, Boyle W, Troxell J, et al: Cystic hamartomata of lung and kidney: A spectrum of developmental abnormalities. Am J Med Genet 27:45, 1987.  [PubMed: 3605206]

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