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[Clinically undiagnosed glycogen storage disease type I as cause of postoperative death (author's transl)]
Insights
A patient experienced sudden cardiac arrest post-cholecystectomy due to undiagnosed Glycogen Storage Disease Type I. This rare metabolic disorder caused fatal hypokalaemia via hypoglycemia and acidosis.
Area of Science:
- Metabolic Disorders
- Cardiology
- Genetics
Background:
- Cholecystectomy is a common surgical procedure.
- Glycogen Storage Disease Type I (GSD I) is a rare inherited metabolic disorder affecting glycogen metabolism.
- Undiagnosed metabolic disorders can present with unexpected complications.
Observation:
- A 30-year-old patient underwent cholecystectomy for cystic duct obstruction.
- Postoperatively, the patient experienced sudden cardiac arrest three hours after anesthesia recovery.
- Necropsy revealed Glycogen Storage Disease Type I as the underlying condition.
Findings:
- The patient presented with normal preoperative clinical and chemical findings.
- Hypoglycemia and metabolic acidosis, characteristic of GSD I, were likely present.
- These metabolic derangements probably led to hypokalaemia, causing cardiac arrest.
Implications:
- Highlights the importance of considering rare metabolic disorders in unexplained perioperative complications.
- Undiagnosed GSD I can lead to severe, life-threatening metabolic disturbances.
- Emphasizes the need for thorough metabolic screening in patients with atypical presentations.
Abstract:
A 30-year-old patient was admitted to hospital for cholecystectomy with a diagnosis of complete obstruction of the cystic duct. The preoperative clinical and chemical findings were normal. Following cholecystectomy the patient recovered normally from the anaesthetic. Three hours later sudden cardiac arrest occurred. Necropsy revealed glycogen storage disease type I. Hypoglycaemia and metabolic acidosis had probably led to hypokalaemia which is considered as the cause of cardiac failure.