Anesthetic management of a child with nephrotic syndrome undergoing open heart surgery: Report of a rare case

Abstract

The congenital nephrotic syndrome (NS) in infancy and childhood is an important entity but combination with acyanotic congenital heart disease is uncommon. Anesthesia in such cases is challenging because of associated problems like hypo-protienemia, anti-thrombin III deficiency, edema, hyperlipidemia, coagulopathy, cardiomyopathy, immunodeficiency, increased lung water etc. We describe anesthetic management of a patient with childhood NS and sinus venosus atrial septal defect (ASD) undergoing open heart surgery. We also suggest guidelines for safe conduct of anesthesia and CPB in such patients.

Anesthetic Implications of Nephrotic Syndrome in Neurosurgical Practice

“…Effect of benzodiazepines and thiopentone is prolonged due to their increased free fraction secondary to hypoalbuminemia. 3 Edema and steroid treatment-induced difficult intravenous access, acetazolamide-related metabolic acidosis, steroid-related osteoporosis are few other concerns. Diuretics, alternating normal saline with RL, can prevent further sodium overload and for osmotherapy mannitol is used.…”

“…5 Intraoperative antifibrinolytics commonly used in neurosurgery to minimize blood loss are not an option here, as they can induce tubular proteinuria. 3 Central venous catheters can be thrombogenic in already existing prothrombotic state and hence used only for specific indication and duration. 5 Rapid sodium fluctuations can contribute to seizures.…”

“…Systemic manifestations include hypertension, hyperlipidemia, restricted cardiomyopathy, pleural effusion, chronic kidney disease, seizures, cerebral venous thrombosis, IIH, posterior reversible encephalopathy syndrome. 3 5 Also paraneoplastic NS is described in a patient with meningioma who achieved remission following surgery. 6 IIH (headache, papilledema, raised CSF pressure) can occur in NS associated renal failure, corticosteroids, or cyclosporine therapy more common in fertile obese females, 5 7 8 similar to our patient.…”

“…1 Hence, avoiding elective neurosurgeries during NS relapse phase would prevent perioperative thromboembolic complications, considering the possible postoperative initiation delay of pharmacoprophylaxis in them. Chronic steroids can cause hypernatremia, hypertension, hypokalemia, thromboembolism, and infections 3 Galloway-Mowat syndrome with facial dysmorphism, hypotonia, seizure is a rare association of NS. 9…”

“…1,2 NS can involve multiple systems with cortical venous thrombosis and idiopathic intracranial hypertension (IIH) as neurological manifestations. 3,4 However, to the best of our knowledge, anesthetic implications of NS patients for neurosurgery are not previously described. We report anesthetic concerns among these subsets in an adult patient for the surgical management of benign intracranial hypertension.…”