Anaesthetic challenges in central hepatectomy for hepatoblastoma in an infant with Simpson–Golabi–Behmel syndrome

Dear Editor,

Simpson–Golabi–Behmel syndrome (SGBS) is a rare X-linked overgrowth disorder characterised by craniofacial dysmorphism, congenital anomalies, and an increased risk of embryonal tumours such as hepatoblastoma.[1,2] Anaesthetic management is particularly challenging in infancy, and reports describing central hepatectomy in infants with SGBS are scarce.[3] We describe the perioperative management of a 3-month-old infant with genetically confirmed SGBS undergoing central hepatectomy.

The infant had macroglossia, macrocephaly, hypertelorism, macrostomia, and accessory nipples. Echocardiography showed a 2-mm atrial septal defect, 3-mm perimembranous ventricular septal defect with left-to-right shunt, mild right ventricular hypertrophy, bilateral peripheral pulmonary stenosis, mild left pulmonary artery hypoplasia, and an ejection fraction of 76%, without pulmonary hypertension. Following neoadjuvant cisplatin chemotherapy, resection was planned at 3 months of age (weight 8 kg) with normal baseline investigations.

Major concerns included difficult airway, difficult vascular access, congenital cardiac lesions, prolonged surgery, major blood loss, coagulopathy, hypothermia, electrolyte disturbances, and possible postoperative intensive care. Breastfeeding was continued until 4 hours before surgery. A difficult airway cart containing a paediatric video laryngoscope, flexible bronchoscope, supraglottic airway devices, bougie, stylet, and appropriate endotracheal tubes was prepared.

As no intravenous access was available, anaesthesia was induced with inhaled sevoflurane. After intravenous access was secured and adequate mask ventilation confirmed, neuromuscular blockade was administered and tracheal intubation was successfully achieved using videolaryngoscope. Ultrasound-guided peripheral, arterial, and central venous access facilitated vascular cannulation.[4]

Anaesthesia was maintained with sevoflurane in an oxygen–air mixture and multimodal analgesia. Tranexamic acid (80 mg; ~10 mg/kg) was administered. A relatively low central venous pressure (5–8 cm H₂O) was maintained to minimise hepatic venous bleeding while preserving systemic perfusion.[5]

The procedure lasted 6 hours with an estimated blood loss of 350 mL. Haemodynamic management was guided by invasive arterial pressure, urine output, and serial blood gas analyses. The patient received 160 mL packed red blood cells and 80 mL fresh frozen plasma. Hypotension was treated with intravenous phenylephrine boluses (10 µg) followed by noradrenaline infusion to maintain a mean arterial pressure of 45–50 mmHg.

Serial blood gas analyses demonstrated progressive metabolic acidosis with hypocalcaemia and hypokalaemia during hepatic resection. Acidosis was associated with haemodynamic instability requiring escalation of vasoactive support and likely reflected reduced hepatic metabolic function, blood loss, and transfusion-related effects. Frequent monitoring enabled timely correction of acid–base and electrolyte disturbances. Normothermia was maintained throughout surgery.

The infant remained intubated and was transferred electively to the paediatric intensive care unit because of metabolic acidosis and circulatory instability. Ventilatory and inotropic support were required initially, and the trachea was successfully extubated on postoperative day 1 with an uneventful recovery.

This case highlights the importance of anticipating difficult airway, preparing advanced airway devices, and using ultrasound-guided vascular access in syndromic infants.[4] Major paediatric hepatectomy is associated with significant blood loss, metabolic acidosis, and electrolyte disturbances requiring vigilant monitoring.[5] Congenital cardiac lesions further complicate perioperative haemodynamic management. Previous reports have focused mainly on phenotypic features and tumour associations, with limited emphasis on perioperative anaesthetic management.[1,2,3,5]

Successful management in this case depended on meticulous planning, anticipation of airway difficulty, early ultrasound-guided vascular access, and prompt correction of metabolic and haemodynamic disturbances.

Author Declaration

This patient has previously been reported in BMJ Case Reports from the paediatric haemato-oncology perspective.[3] The present report is intended for an anaesthesiology readership and focuses exclusively on perioperative anaesthetic management. The content is distinct from the previous publication.

Declaration of patient consent

The authors certify that they obtained appropriate patient consent from the patient’s parents for publication of the clinical information. Consent for publication of photographs was not obtained; therefore, no patient images are included.

Disclosure of use of artificial intelligence (AI)-assistive or generative tools

The authors utilised ChatGPT (OpenAI, San Francisco, CA, USA) to assist with language editing and improvement of manuscript readability. The authors reviewed and edited all generated content and take full responsibility for the final manuscript.

Author contibutions

NSMJ: Conceptualisation, anaesthetic management, manuscript drafting, critical revision. RD: Perioperative data collection, manuscript drafting, revision. AC: Literature review, data curation, manuscript drafting. All authors reviewed the manuscript

Conflicts of interest

There are no conflicts of interest.

Funding Statement

Nil.