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

Fanconi anemia (FA) is a rare inherited bone marrow failure syndrome with multisystem congenital anomalies and a markedly elevated risk of malignancy. Children with FA undergoing major oncological surgery present significant anesthetic challenges, including severe pancytopenia, structural airway anomalies, congenital cardiac defects, and amplified hemorrhagic diathesis.

We report the perioperative management of a 4-year-old, 12 kg boy with FA (FANCD1/BRCA2 biallelic mutation), a 7×5 cm Wilms tumor (Stage III) in the right moiety and isthmus of a horseshoe kidney, a Type IV membranous ventricular septal defect (VSD), and spina bifida occulta. After six cycles of neoadjuvant chemotherapy (vincristine-actinomycin-D), the child underwent radical tumor resection with isthmectomy under general anesthesia. Preoperative hematological optimization included packed red blood cell (PRBC) and platelet transfusion. The patient underwent videolaryngoscopy-facilitated intubation due to micrognathia. Invasive hemodynamic monitoring, Tranexamic acid infusion, and blood component therapy were employed to mitigate hemorrhagic risk. Ultrasound-guided bilateral posterior quadratus lumborum (QL) blocks (0.3 mL/kg of 0.25% ropivacaine per side) provided effective opioid-sparing analgesia, with Face, Legs, Activity, Cry, Consolability (FLACC) scores ≤3 for 18–20 hours, avoiding non-steroidal anti-inflammatory drugs (NSAIDs) given the potential chemotherapy-related nephrotoxicity risk. Estimated blood loss was 400 mL (~30% blood volume), managed with 200 mL PRBC and one platelet unit. The child was extubated awake in the operating theatre and discharged on postoperative day 10 with intact renal function.

Meticulous multidisciplinary preoperative planning, video-assisted airway management, goal-directed hemostatic therapy, and ultrasound-guided fascial plane analgesia were integral to safe perioperative management in pediatric FA undergoing high-risk oncological surgery.