Authors: Cabete S et al.
Cureus 17(11): e95877, November 2025. DOI: 10.7759/cureus.95877
This pediatric case report describes anesthetic management for a 10-year-old with Rothmund-Thomson syndrome (RTS) type 2 undergoing major orthopedic surgery (tibial osteosarcoma resection with fibular centralization)—the first such report for a major orthopedic procedure in RTS. The team anticipated multisystem issues: potentially difficult airway (micrognathia, retrognathia, high-arched palate, dental anomalies), challenging vascular access and arterial cannulation due to limb malformations and fragile skin, positioning constraints, and bleeding risk.
An inhalational induction facilitated IV placement, followed by videolaryngoscopic intubation after neuromuscular blockade. Management combined propofol TCI general anesthesia with lumbar epidural analgesia, invasive arterial pressure monitoring, processed EEG, cerebral oximetry, and multimodal analgesia (epidural ropivacaine/sufentanil, fentanyl, ketamine, acetaminophen, ketorolac). Blood-sparing measures included tranexamic acid and tourniquet use. Neuromuscular monitoring was not feasible because thumbs were absent. The course was hemodynamically stable with minimal blood loss; extubation was uneventful. Postoperatively, high-dependency monitoring and epidural analgesia enabled effective pain control; discharge occurred on postoperative day 8.
The discussion synthesizes RTS features relevant to anesthesia—skin fragility, skeletal defects, airway and vascular challenges, variable hematologic and endocrine issues, and elevated childhood osteosarcoma risk—and compares this case with two prior RTS anesthetic reports (endoscopy and cataract surgery). The authors emphasize meticulous preoperative planning, skin protection, positioning, readiness for difficult airway/vascular access, and individualized multimodal strategies. They advocate for disease-specific guidance (e.g., Orphananesthesia) given sparse evidence and heterogeneous phenotypes.
What You Should Know:
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RTS presents distinctive airway, vascular access, skin, and positioning challenges; anticipate these and plan equipment and expertise accordingly.
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Multimodal anesthesia with regional techniques (here, epidural) can provide stable intraoperative conditions and strong postoperative analgesia.
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Ultrasound guidance is valuable for both venous and arterial access in limb malformations; consider femoral sites if upper limbs are not feasible.
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Skin-safe taping, generous padding, and careful device placement are essential due to poikiloderma and fragility.
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Formal, anesthesia-focused RTS guidelines are needed; until then, thorough individualized assessment and planning are critical.
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