Abstract

We present the unique case of an 8 month old infant who required extracorporeal membrane oxygenation (ECMO) after neonatal repair of tetralogy of Fallot. While on ECMO, he developed grade 3 intraventricular hemorrhage resulting in hydrocephalus requiring ventriculoperitoneal (VP) shunt placement at 5 months of life. He presented to cardiology clinic with a 2-month history of poor weight gain, tachypnea, and grunting and was found to have a large right sided pleural effusion. This was proven to be cerebrospinal fluid (CSF) accumulation secondary to poor peritoneal absorption with subsequent extravasation of CSF into the thoracic cavity via a diaphragmatic defect. After diaphragm repair, worsening ascites from peritoneal malabsorption led to shunt externalization and ultimate conversion to a ventriculoatrial (VA) shunt. This is the second reported case of VA shunt placement in a child with congenital heart disease and highlights the need to consider CSF extravasation as the cause of pleural effusions in children with VP shunts.

Highlights

  • We present the unique case of an 8 month old infant who required extracorporeal membrane oxygenation (ECMO) after neonatal repair of tetralogy of Fallot

  • Case report We present the case of an 8 months old diagnosed postnatally with tetralogy of Fallot (TOF)

  • We present the case of an infant with TOF requiring neonatal repair and postoperative ECMO support

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Summary

Discussion

We present the case of an infant with TOF requiring neonatal repair and postoperative ECMO support. IVH while on ECMO led to hydrocephalus requiring VP shunt placement This shunt had to be converted to a VA shunt due to poor peritoneal absorption of CSF, a phenomenon recognized by a persistent right sided pleural effusion secondary to a diaphragmatic hernia. This is the second reported case of VA shunt placement in a child with congenital heart disease (CHD), the first in a patient with TOF. Patients with right-sided CHD, such as our patient, may have a compounded risk of pulmonary hypertension and subsequent right ventricular dysfunction This is merely speculation as this is only the second reported case of a VA shunt in a child with CHD (Niggemann et al 1990).

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