Anaesthetic Management of A Patient With Neurofibromatosis Undergoing Surgery For Ectopic Pregnancy

Abstract

Background: Neurofibromatosis type 1 (NF1) is a multisystem genetic disorder characterized by multiple cutaneous neurofibromas and café-au-lait macules, with variable neurological, cardiovascular, respiratory and musculoskeletal involvement. Anaesthetic management may be challenging because of potential airway involvement, cardiovascular abnormalities, spinal pathology and associated pheochromocytoma. Pregnancy and laparoscopic surgery require additional perioperative considerations.

Case Presentation: A 28-year-old woman, G4P2L2A1, at 6 weeks and 5 days of gestation, presented for termination of an unwanted pregnancy with planned laparoscopic sterilization. She was a known case of NF1 for approximately 4 years and was not receiving regular medication for the condition. Examination revealed multiple café-au-lait macules and numerous cutaneous neurofibromas over the trunk and back. She was haemodynamically stable, with a pulse rate of 78 beats/min, blood pressure of 110/70 mmHg, and oxygen saturation of 99% on room air. Her height was 148 cm, weight 68 kg, and body mass index 31 kg/m². Airway assessment showed approximately three-finger-breadth mouth opening with adequate neck movements and no clinically evident airway obstruction. Laboratory investigations revealed haemoglobin 11.9 g/dL, platelet count 3.6 lakh/mm³, serum creatinine 0.44 mg/dL, sodium 141 mmol/L, and potassium 3.8 mmol/L. Electrocardiography showed normal sinus rhythm, while echocardiography demonstrated preserved left ventricular systolic function with an ejection fraction of 68%, without regional wall-motion abnormality. Neurological evaluation did not reveal significant abnormality. Ultrasonography demonstrated an early intrauterine pregnancy with a crown-rump length of 4.7 mm and a 30 × 5 mm subchorionic haemorrhage. The patient was classified as ASA II and underwent the planned procedure under general anaesthesia with appropriate preparation for potential difficult airway management and vigilant perioperative monitoring. The perioperative course was uneventful.

Conclusion: NF1 requires careful multisystem evaluation before anaesthesia, particularly during pregnancy. Assessment of the airway, cardiovascular and neurological systems, consideration of associated spinal pathology and preparation for potential airway difficulty are essential. Individualized anaesthetic planning and vigilant perioperative monitoring can facilitate safe management of patients with NF1 undergoing laparoscopic procedures during early pregnancy.