Anesthetic management of neuroblastoma excision in child with Kinsbourne syndrome

Dear Editor,

Neuroblastoma, linked with opsoclonus-myoclonus-ataxia syndrome (OMAS), also termed Kinsbourne disease, manifests as ataxia, erratic eye movements, and myoclonus.[1] This paraneoplastic syndrome is thought to be the result of autoimmune responses in the nervous system.[1]

A 2-year-old boy, weighing 14 kg, presented with history of frequent falls and body tremors for the past 2 weeks. Child’s inability to maintain balance without assistance, coupled with involuntary jerky eye movements upon attempting to focus, prompted further evaluation. Clinical examination revealed blood pressure of 110/80 mmHg along with myoclonus, opsoclonus, dysmetria, and truncal ataxia. Laboratory tests were within normal parameters. Magnetic resonance imaging delineated right adrenal neuroblastoma with dimensions of 8.6 × 7.2 × 8.2 cm impinging upon the right hepatic lobe and the upper renal pole [Figure 1]. DOTATEC-positron emitted tomography scan showed somatostatin receptor expressing soft tissue mass in the right suprarenal region.

Figure 1.

CEMRI depicting right adrenal neuroblastoma (red arrow) superiorly reaching the right lobe of the liver and inferiorly compressing the upper pole of the kidney. CEMRI = Contrast-enhanced magnetic resonance imaging

Upon admission, child’s Mitchell and Pike OMAS score[1] was 9. Treatment commenced with intravenous immunoglobulin and adrenocorticotrophic hormone along with amlodipine, which resulted in normalized blood pressure readings. The following month of therapy, with partial resolution of neurologic symptoms, the child was posted for neuroblastoma excision.

On the day of surgery, the patient received premedication with 0.5 mg intravenous midazolam, followed by induction using 20 μg fentanyl, 20 mg propofol, and 7.5 mg atracurium. Intubation achieved with 4.5 mm sized micro-cuff endotracheal tube and invasive monitoring was established via left radial artery cannulation. Epidural catheter was placed between T10 and T11 vertebrae. Anesthesia was maintained with isoflurane and intermittent atracurium boluses. Blood loss was estimated at 120 ml and compensated with a transfusion of 8 ml/kg. Intraoperative hypotension was managed with dopamine infusion at a rate of 5 μg/kg/min. Continuous epidural infusion of 0.1% ropivacaine at 4 ml/h was continued both intraoperatively and postoperatively, along with intravenous paracetamol 300 mg administered toward the end. The child’s recovery was smooth and was discharged 5 days postsurgery. Follow-up assessments indicated improvement in neurologic symptoms, albeit they were not entirely resolved.

Anesthetic agents have variable effects in triggering OMAS symptoms in such patients. While opioids, inhalational agents, non-depolarizing muscle relaxants (NDMRs), neostigmine, and ketorolac do not exacerbate OMAS symptoms, ketamine and etomidate lead to worsening.[2,3] Due to rarity of this condition, only four case reports have been described till date.[2,3,4,5] Out of these four, in three cases, only general anesthesia was used for case management. Fourth case practiced general anaesthesia alongwith caudal block for perioperative analgesia.[5] There is no case report describing the utility of thoracic epidural analgesia in reducing anesthetic requirement intraoperatively and also in the management of postoperative analgesia. In our case, adjunctive use of regional anesthesia along with general anesthesia effectively decreased intraoperative requirements for intravenous and volatile agents, thereby providing rapid awakening, effective blunting of surgical stress response, and limiting the need for parenteral opioids and supplemental anesthesia.

This case highlights the possible safe approach in patients with Kinsbourne syndrome.

Conflicts of interest

There are no conflicts of interest.

Funding Statement

Nil.