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Anaesthesia for Proteus syndrome.

A Ceyhan1, Y Gülhan, T Cakan

  • 1Department of Anaesthesiology, Ministry of Health Ankara Training and Research Hospital, Ankara, Turkey.

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Summary
This summary is machine-generated.

This study reports a successful surgery for a 4-month-old boy with Proteus syndrome experiencing abdominal mass, hydroureter, and hydronephrosis. The successful general anesthesia management highlights safe anesthetic protocols for this rare condition.

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Area of Science:

  • Pediatric Surgery
  • Anesthesiology
  • Medical Genetics

Background:

  • Proteus syndrome is a rare congenital disorder characterized by segmental overgrowth.
  • Ureteral and renal anomalies, such as hydroureter and hydronephrosis, can occur in patients with Proteus syndrome.
  • Anesthetic management in patients with Proteus syndrome is not well-documented, with limited case reports.

Observation:

  • A 4-month-old male infant diagnosed with Proteus syndrome presented with a left abdominal mass.
  • The abdominal mass was attributed to left hydroureter and hydronephrosis secondary to left ureterovesical stenosis.
  • The patient underwent a surgical correction of the ureterovesical junction obstruction.

Findings:

  • The surgical procedure for the abdominal mass, hydroureter, and hydronephrosis was successfully completed in 4.5 hours.
  • General anesthesia was administered without any reported anesthetic complications.
  • This case represents one of the few documented instances of anesthetic management in an infant with Proteus syndrome.

Implications:

  • This successful anesthetic management provides valuable data for future surgical interventions in pediatric patients with Proteus syndrome.
  • The findings underscore the importance of careful preoperative assessment and tailored anesthetic plans for rare genetic syndromes.
  • Further research and case reporting are crucial to establish evidence-based anesthetic guidelines for Proteus syndrome.