Gastrostomy Tube Insertion in Pediatric Patients With Autosomal Recessive Polycystic Kidney Disease (ARPKD): Current Practice

Abstract

Introduction: Autosomal recessive polycystic kidney disease (ARPKD) is a severe hepatorenal disorder of childhood. Early renal disease in ARPKD may require renal replacement therapy and is associated with failure to thrive resulting in a need for nasogastric tube feeding or gastrostomy. In ARPKD patients, the benefit of a gastrostomy in nutrition and growth needs to be weighed against the potential risk of complications of congenital hepatic fibrosis (CHF) and portal hypertension like variceal bleeding. CHF in ARPKD has thus been considered as a relative contraindication for gastrostomy insertion. Yet, data on gastrostomies in pediatric patients with ARPKD is lacking.Methods: We conducted a web-based survey study among pediatric nephrologists, pediatric hepatologists and pediatric gastroenterologists on their opinions on and experiences with gastrostomy insertion in ARPKD patients.Results: 196 participants from 39 countries shared their opinion. 45% of participants support gastrostomy insertion in all ARPKD patients, but portal hypertension is considered to be a contraindication by a subgroup of participants. Patient-specific data was provided for 38 patients indicating complications of gastrostomy that were in principal comparable to non-ARPKD patients. Bleeding episodes were reported in 3/38 patients (7.9%). Two patients developed additional severe complications. Gastrostomy was retrospectively considered as the right decision for the patient in 35/38 (92.1%) of the cases.Conclusions: This report on the results of an online survey gives first insights into the clinical practice of gastrostomy insertion in ARPKD patients. For the majority of participating physicians benefits of gastrostomy insertion retrospectively outweigh complications and risks. More data will be required to lay the foundation for clinical recommendations.

Molecular Pathophysiology of Autosomal Recessive Polycystic Kidney Disease

“…Note that prenatal diagnosis and termination of pregnancy are factors to consider in the epidemiology of the disease [ 15 , 17 ]. Another problem derived from kidney enlargement and pulmonary hypoplasia, in addition to early uremia and pulmonary immaturity, includes the difficulty of enteral feeding that could complicate nutrition, requiring persistent nasogastric feeding [ 11 , 18 ].…”

Malnutrition in Pediatric Chronic Cholestatic Disease: An Up-to-Date Overview

“…When nasogastric tube feeding is not feasible or inadequate for meeting caloric needs, jejunal tube feeding [ 85 ], or even an endoscopic gastrostomy if portal hypertension is mild [ 86 , 87 , 88 ] may be proposed if a multidisciplinary team can provide active follow-up and care for the child and the device is available. Gastrostomy feeding, however, should be avoided in children with CCLD because there is a risk of peritoneal infection and variceal bleeding (if portal hypertension is present) [ 36 , 89 ].…”

Early clinical management of autosomal recessive polycystic kidney disease

“…Furthermore, the presence of stomal varices due to portal hypertension could be a concern. Such considerations resulted in the classification of ARPKD as a relative contraindication for gastrostomy insertion [ 66 , 67 ].…”