Perioperative anesthetic management of Wilms tumor in a child with Fanconi anemia and horseshoe kidney

The case report, “Perioperative Anesthetic Management of Wilms Tumor in a Child with Fanconi Anemia and Horseshoe Kidney: A Case Report,” was carried out by Shreyasi Mallick, Sangeeta K. S., and Tharun Kumar K. B. in The Open Anesthesia Journal.

Doctors in India have reported how a carefully coordinated, multidisciplinary anaesthetic strategy enabled the safe removal of a kidney cancer in a four-year-old boy whose combination of underlying conditions, a rare inherited bone marrow failure syndrome, a malformed kidney, a heart defect, and a spinal anomaly made every aspect of surgical planning and intraoperative management exceptionally demanding.

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A Child Facing Multiple Simultaneous Challenges

Fanconi anaemia (FA) is a rare hereditary disorder in which the body cannot properly repair damaged DNA. It causes the bone marrow to gradually fail, leading to dangerously low counts of red blood cells, white blood cells, and platelets a state known as pancytopenia. Children with Fanconi anaemia are also born with a wide range of structural abnormalities, and they carry a significantly elevated risk of developing cancers, particularly at a young age. The patient in this case was a four-year-old boy weighing just 12 kilograms, carrying a confirmed diagnosis linked to a mutation in the FANCD1/BRCA2 gene. He had also developed Wilms tumour a kidney cancer that is the most common renal malignancy in children measuring 7 by 5 centimetres and graded as Stage III, meaning it had spread locally beyond the kidney. Compounding matters further, the tumour arose in a horseshoe kidney, a congenital anatomical variant in which the two kidneys are fused at their lower poles, making surgical access and the preservation of remaining kidney function considerably more complex than in a standard nephrectomy. The child additionally had a Type IV membranous ventricular septal defect, a hole between the two lower chambers of the heart and spina bifida occulta, a mild but relevant spinal anomaly. Managing anaesthesia for any one of these conditions in a child would be demanding; managing all of them together in a single operative session, for a procedure involving extensive abdominal dissection and significant bleeding risk, required a level of preparation that went well beyond routine oncological surgery.

How the Team Managed Each Layer of Risk

Before surgery could proceed, the child received six cycles of neoadjuvant chemotherapy with vincristine and actinomycin-D to reduce the tumour size, followed by careful haematological optimisation: transfusions of packed red blood cells and platelets brought his blood counts to levels that were safer for a major operation, though still far below normal. The anaesthetic team addressed the airway problem first. The child had micrognathia an unusually small jaw which makes conventional laryngoscope-guided intubation difficult and potentially dangerous. Videolaryngoscopy, which provides an enhanced magnified view of the airway on a screen, was used to place the breathing tube safely. Throughout the operation, the team employed invasive haemodynamic monitoring to track blood pressure and cardiac function in real time, and administered tranexamic acid a drug that helps the blood clot as a continuous infusion to reduce the risk of uncontrolled bleeding, which was the single greatest threat given the child’s severely impaired clotting capacity from his FA-related pancytopenia. Estimated blood loss during the operation reached 400 millilitres, representing approximately 30% of the child’s total blood volume a substantial loss for a 12-kilogram patient. This was managed with 200 millilitres of packed red blood cells and one unit of platelets. Pain management after surgery required particular care. Non-steroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen, which are commonly used for post-operative pain in children, were avoided because of the risk of further kidney damage in a child who had already received nephrotoxic chemotherapy and was now left with reduced renal tissue following tumour resection and partial kidney removal. Instead, the team performed ultrasound-guided bilateral posterior quadratus lumborum nerve blocks regional anaesthetic injections placed under image guidance into a fascial plane of the abdominal wall on both sides using 0.3 millilitres per kilogram of 0.25% ropivacaine per side. This technique delivered effective, opioid-sparing pain control, with the child’s pain scores remaining at or below 3 on the FLACC scale for 18 to 20 hours after surgery, avoiding both the risks of heavy opioid use and the renal risks of NSAIDs.

The Outcome and What This Case Adds

The child was extubated his breathing tube removed while awake in the operating theatre at the end of the procedure, and was discharged on postoperative day 10 with intact renal function. The authors note that the published literature on anaesthetic management specifically for FA patients undergoing major oncological surgery is very limited, and that this case demonstrates how the combination of preoperative multidisciplinary planning, video-assisted airway management, goal-directed blood component therapy with antifibrinolytic support, and ultrasound-guided regional analgesia can together achieve a safe outcome in what would otherwise be a potentially fatal surgical scenario. The case reinforces the value of regional anaesthesia techniques particularly fascial plane blocks such as the quadratus lumborum block as an effective and organ-safe alternative to systemic analgesics in children with compromised physiology. The case was led by corresponding author Dr. Shreyasi Mallick of the Department of Anesthesiology, Kalinga Institute of Medical Sciences, Bhubaneswar, India, with co-authors Dr. Sangeeta K. S. of the Department of Anesthesiology, Yashoda Hospital, Secunderabad, India, and Dr. Tharun Kumar K. B. of the Department of Anesthesiology, Kalinga Institute of Medical Sciences, Bhubaneswar, India.

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Article title: Perioperative Anesthetic Management of Wilms Tumor in a Child with Fanconi Anemia and Horseshoe Kidney: A Case Report

DOI: 10.2174/01258964584904260909111216
http://dx.doi.org/10.2174/01258964584904260909111216