Gastric dilatation and abdominal compartment syndrome in a child with Prader-Willi syndrome

Clara Blat, Elisenda Busquets, Teresa Gili, Assumpta Caixàs, Elisabeth Gabau, Raquel Corripio

Research output: Contribution to journalArticleResearchpeer-review

1 Citation (Scopus)

Abstract

Objective: Rare disease Background: Prader-Willi syndrome (PWS) is a genetic disorder characterized by initial muscular hypotonia and feeding difficulties, and later an insatiable appetite, hyperphagia and obesity along with mild to moderate intellectual impairment. Affected individuals’ food-seeking behavior and suspected delayed gastric emptying can lead to gastric dilatation with subsequent necrosis and perforation. Case Report: We present the case of a 5-year-old boy diagnosed with Prader-Willi syndrome at neonatal age due to muscular hypotonia, who started growth hormone therapy at 20 months. He presented with two episodes of a rapidly progressing gastric dilatation that led to abdominal hypertension and secondary shock at the age of 2 and 5. No large amount of food was eaten before any of the episodes, and he had abdominal pain and vomiting on both occasions. On arrival at the emergency room, a nasogastric tube was placed and aspiration of food material was performed. Abdominal X-ray and CT scan revealed massive gastric dilatation. He was admitted at the Pediatric Intensive Care Unit and after a variable period of fasting, tolerated oral intake and could be discharged. Conclusions: Gastric dilatation due to gastroparesis in PWS is a rare complication. However, it is a life-threatening situation and physicians should therefore maintain a high level of suspicion for gastric dilatation when patients present with warning symptoms such as abdominal pain or discomfort and vomiting.

Original languageEnglish
Pages (from-to)637-640
Number of pages4
JournalAmerican Journal of Case Reports
Volume18
DOIs
Publication statusPublished - 7 Jun 2017

Keywords

  • Gastric dilatation
  • Gastroparesis
  • Intra-Abdominal hypertension
  • Prader-Willi syndrome

Fingerprint

Dive into the research topics of 'Gastric dilatation and abdominal compartment syndrome in a child with Prader-Willi syndrome'. Together they form a unique fingerprint.

Cite this