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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.
Abstract:
Lowe's syndrome is a rare inherited metabolic disorder characterized by mental retardation, kidney malfunction, and abnormalities of the eyes and bones. A 4 month-old child with Lowe's and Fanconi's syndrome, undergoing bilateral congenital cataract surgery, is presented. Preoperative electrolyte imbalance was corrected by potassium, calcium, magnesium, phosphate, and bicarbonate supplementation. Anesthesia was administered uneventfully using appropriate anesthetic agents and monitoring. Adequate preoperative evaluation and optimization, along with selection of anesthetic agents and fluid and electrolyte management with appropriate perioperative monitoring, is key to a successful outcome.
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