Lowe's syndrome with Fanconi syndrome for ocular surgery: perioperative anesthetic considerations

Ravindra Pandey1, Rakesh Garg, Chandrashish Chakravarty

  • 1Department of Anaesthesiology and Intensive Care, All India Institute ofMedical Sciences, Ansari Nagar, NewDelhi 110029, India. trips22003@yahoo.co.in

Insights

Lowe's syndrome, a rare inherited disorder, requires careful management of electrolyte imbalances before surgery. Successful anesthesia and perioperative care are crucial for patients with this condition.

Area of Science:

  • Biochemistry
  • Pediatrics
  • Genetics

Background:

  • Lowe's syndrome is a rare X-linked inherited metabolic disorder.
  • It is characterized by congenital cataracts, intellectual disability, and renal tubulopathy.
  • Fanconi's syndrome is a generalized proximal renal tubule dysfunction.

Observation:

  • A case of a 4-month-old infant with Lowe's syndrome and Fanconi's syndrome is presented.
  • The infant required bilateral congenital cataract surgery.
  • Preoperative electrolyte imbalances were noted.

Findings:

  • Electrolyte imbalances were corrected with potassium, calcium, magnesium, phosphate, and bicarbonate supplementation.
  • Anesthesia was administered successfully using appropriate agents and monitoring.
  • The perioperative period was managed with careful attention to fluid and electrolyte balance.

Implications:

  • This case highlights the importance of thorough preoperative evaluation and optimization in patients with Lowe's syndrome.
  • Careful anesthetic management and meticulous fluid and electrolyte monitoring are essential for successful surgical outcomes.
  • Early diagnosis and management can improve the quality of life for affected individuals.

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