Congenital Diaphragmatic Hernia and Congenital Nephrotic Syndrome in a Low-Birth-Weight Infant: A Case Report

TL;DR

A low-birth-weight infant with congenital diaphragmatic hernia and nephrotic syndrome required complex dialysis and surgical management, but ultimately succumbed to complications.

Contribution

This case report highlights the challenges of managing overlapping congenital conditions in low-birth-weight infants requiring dialysis and surgical intervention.

Findings

The infant required continuous hemodialysis due to peritonitis and surgical site complications.

Vascular occlusion and superior vena cava syndrome prevented continuation of dialysis.

Multidisciplinary management is crucial for infants with CDH and CNS.

Abstract

We encountered a case of congenital diaphragmatic hernia (CDH) and congenital nephrotic syndrome (CNS) in a low-birth-weight infant weighing < 2 kg. Dialysis was required due to progressive acute kidney injury in the early postnatal period, and a peritoneal dialysis (PD) catheter was placed during CDH repair surgery. During the postoperative acute phase, continuous hemodialysis (CHD) was performed to minimize stress on the surgical site; however, owing to PD-related peritonitis and sutural insufficiency of the diaphragm, the transition to PD was not feasible, necessitating prolonged CHD. During the course of the illness, the patient developed vascular occlusion in the vessels suitable for blood access and superior vena cava syndrome, ultimately rendering continued CHD and PD impossible, leading to death at 74 days of age. Respiratory and circulatory management are required during the perioperative period of CDH repair, necessitating multiple supportive therapies and careful nutritional management. This leads to a complex vicious cycle of complications, including protein loss due to CNS, coagulation disorders, circulatory failure, delayed wound healing because of malnutrition, increased susceptibility to infection, thrombosis, and infections associated with CHD and PD. Optimization of supportive therapies, including systemic anticoagulation management, infection control, and early establishment of nutrition, is considered crucial for the safe implementation of CHD or PD in such diseases.

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