Very rare, non-malignant syndrome characterized by
abnormal morphogenesis affecting both kidneys and lungs.
Cystic Hamartoma of Lung and Kidney.
Three cases have been reported
worldwide. Inheritance unknown.
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).
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