Een tiener met Prader-Willi-syndroom zonder eetlust

Background

Prader-Willi syndrome is characterised by hyperphagia and binge eating, without regurgitation

Case description

We present a 16-year-old girl with Prader-Willi syndrome exhibiting loss of appetite, stomach ache and regurgitation. Gastro-enteritis was suspected. However, she rapidly developed severe septic shock. During emergency surgery, a fully necrotic and ruptured stomach was seen. Despite respiratory, haemodynamic and surgical efforts, the patient died of necrotic intestinal bleeding.

Conclusion

Binge eating or deviant gastric homeostasis could account for the relatively high incidence of gastric necrosis in patients with Prader-Willi syndrome. Loss of appetite and regurgitation in patients with this syndrome should be considered as warning signs of a possible life-threatening disorder.

Conflict of interest and financial support: none declared. Due to the untimely death of Dr. Hack his ICMJE form is missing.