Sialidosis is a rare autosomal-recessive lysosomal storage disease due to mutations in the NEU1 gene leading to a deficit of alpha-n-acetyl neuraminidase and causing aberrant accumulation of sialylated glycoproteins/peptides and oligosaccharides in the lysosomes of various organs and tissues. Type II sialidosis (dysmorphic form) is classified into three subgroups based on the age of onset and the clinical severity: Congenital or neonatal, infantile (onset 0-12 months) and juvenile form (onset 13 months-20 years). We report the case of a 3-year-old boy with sialidosis type II infantile form, who developed a voluminous ascites. To the best of our knowledge, ascites is not described in the infantile form but in the congenital form of the disease. Ascites seems to be of a multifactorial origin regarding our investigations: on the one hand, portal hypertension and on the other hypoalbuminemia maintained by proteinuria secondary to nephrosialidosis. Loss of plasma proteins in the gastrointestinal tract (protein-losing enteropathy) should also be considered in the case of portal hypertension and damages of the reticuloendothelial system.

Objectives: Gastrostomy placement is a standard procedure for children requiring enteral feeding for more than 3–6 weeks. Various techniques have been described (percutaneous endoscopic, laparoscopy, and laparotomy), and many complications have been reported. In our center, gastrostomy placement is performed either percutaneously by pediatric gastroenterologists, by laparoscopy/laparotomy by the visceral surgery team, or jointly, that is laparoscopic-assisted percutaneous endoscopic gastrostomy. This study aims to report all complications and identify risk factors and ways to prevent them. Methods: This is a monocentric retrospective study including children younger than 18 years who underwent gastrostomy placement (percutaneous or surgical) between January 2012 and December 2020. Complications that occurred up to 1 year after placement were collected and classified according to their time of onset, degree of severity, and management. A univariate analysis was conducted to compare the groups and the occurrence of complications. Results: We established a cohort of 124 children. Sixty-three (50.8%) presented a concomitant neurological disease. Fifty-nine patients (47.6%) underwent endoscopic placement, 59 (47.6%) surgical placement, and 6 (4.8%) laparoscopic-assisted percutaneous endoscopic gastrostomy. Two hundred and two complications were described, including 29 (14.4%) major and 173 (85.6%) minor. Abdominal wall abscess and cellulitis were reported 13 times. Patients who underwent surgical placement presented more complications (major and minor combined) with a statistically significant difference compared with the endoscopic technique. Patients with a concomitant neurological disease had significantly more early complications in the percutaneous group. Patients with malnutrition had significantly more major complications requiring endoscopic or surgical management. Conclusion: This study highlights a significant number of major complications or complications requiring additional management under general anesthesia. Children with a concomitant neurological disease or malnutrition are at greater risk of severe and early complications. Infections remain a frequent complication, and prevention strategies should be reviewed.