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 International Journal of Medical Sciences and Pharma Research 

Open Access to Medical Science and Pharma Research

Copyright  © 2026 The   Author(s): This is an open-access article distributed under the terms of the CC BY-NC 4.0 which permits unrestricted use, distribution, and reproduction in any medium for non-commercial use provided the original author and source are credited

 

Anaesthetic Management of a Patient with Neurofibromatosis and a Large Orbital Tumor: A Case Report

Oshunpidan Adekunbi *, Adeoye Ebanehita , Oshunpidan Festus , Oshola Hammed , Sule Samuel 

Dukes Neurosurgery and Specialist Hospital, Lagos, Nigeria.

Lagos State University Teaching Hospital, Ikeja, Lagos, Nigeria.

 

 

INTRODUCTION

Neurofibromatoses are a group of hereditary diseases transmitted in an autosomal dominant fashion. They usually form tumors of ectoderm and mesoderm tissues, said to be the most common type of neurocutaneous syndrome.2 There are two main types based on clinical and genetic grounds, which include Type 1(NF1, Von Recklinghausen’s, Peripheral Neurofibromatosis) and Type 2 (NF2, Bilateral Acoustic Neurofibromatosis, Central Neurofibromatosis). Neurofibromas are characteristic of the condition, found along all neurons paths, oropharynx, and larynx, which can cause difficulties with laryngoscopy. 3,4,5 Neurofibromatosis type 1 is caused by a mutation of the NF1 gene that results in a nonfunctional neurofibromin protein. Neurofibromatosis-1 affects most organ systems and present with complications of the central nervous, respiratory, cardiovascular, musculoskeletal, gastrointestinal and genitourinary systems. These systems all present with different considerations that pose challenges for anaesthesiologists.

 

 

CASE REPORT 

A 36-year-old woman presented with increasing proptosis, craniofacial swelling, and difficulty breathing. Craniofacial swelling had been gradually increasing in size over 5 years, associated with an increase in the size of the eyes, more on the left than the right. She also had a recurrent history of blocked nostrils, heavy snoring, and difficulty in breathing when lying flat. She had no comorbidities, had a previous craniotomy 5years prior to presentation for brain tumor excision. There were no other significant findings in her history. 

Examination revealed a young woman with facial asymmetry, bilateral proptosis which was greater on the left side. No neurological deficits, multiple nodules on the face, neck, upper half of the body, macroglossia with Mallampati of 3. The respiratory and cardiovascular systems were essentially normal. Pre- operative investigations were normal except for a low cortisol level, for which the patient was managed with prednisolone, and the cortisol level was repeated after a week, which was normal. High-risk anaesthesia consent was taken from the patient. Preparation for difficult intubation was made (MAC 5, Mc Coy and Videolaryngoscope were all available, bougie, and FONA equipment). Venous access was with a 3-way femoral central line because of the multiple nodules in the upper part of the body.

Premedication given were Lidocaine(80mg), Dexamethasone(8mg), Tranexamic Acid(1g). Standard monitors were attached BP, Pulse oximeter, ECG, Temperature probe. She was pre-oxygenated for five minutes. Induced with Thiopentone (300mg), paralyzed with Suxamethonium (100mg), laryngoscopy was done using a MAC 5 aided with a bougie, a 7mm armoured ETT tube was railroaded into the trachea, and the placement was confirmed by auscultation and capnography. 

Maintenance was TIVA using propofol (0.1 – 0.2 mg/kg/min) and oxygen. The analgesia used was Fentanyl and PCM. Paralysis was with Pancuronium (0.1mg/kg as an initial dose, then 1/3rd of the it for maintenance), 0.9% normal saline was used intra-operatively, and 2 pints of blood was given during surgery. There were two episodes of critical incidents intra-operatively: the first was bradycardia, the surgeons were notified, and this resolved spontaneously; the second was hypotension, which was managed with 10mg of Ephedrine. The surgery done was frontoorbitozygomatic craniotomy with tumor excision and maxillectomy. 

The duration of surgery was 5hours 27minutes, while anaesthesia lasted 7hours 2minutes. She was extubated awake after surgery, transferred to HDU, moved to the open ward 2nd day post op, and was discharged home 6days after surgery. Patient re- presented 6weeks later for parotidectomy. There was no critical incident, and she was discharged 3 days after surgery. 

DISCUSSION 

Neurofibromatosis type 1 (NF-1) is a complex multisystemic genetic disorder associated with the mutation of a gene on chromosome 17. The changes in the neurofibromin protein produce multiple effects on ectodermal and mesodermal tissue, with the formation of tumors primarily in the nervous system and the skin. 

Clinical manifestations are extremely variable, even among patients with the same genotype.3,4,5Therefore, a thorough evaluation of anticipated complications must be done when planning anaesthesia. The examination must include airway assessment, respiratory, cardiovascular, and neurological system examination and rule out vertebral anomalies. This case highlights the importance of careful assessment and pre-emptive management of neurofibromatosis, as we can encounter them at a point in time in our anaesthesia practice. In neurofibromatosis, macroglossia, abnormal formations in the tongue, presence of plexiform fibromas in the pharynx, larynx, and supraglottic region can prevent endotracheal intubation and cause upper airway obstruction during anesthetic induction. presence of macroglossia, macrocephaly, mandibular abnormalities and cervical spine involvement may contribute to difficulties in airway management.2 For these presentations, history of dysphagia, dysarthria, presence of stridor, and voice changes should be questioned in the patients prior to anaesthesia. 6,7 The patient had an history of stridor however, she had no history of dyspnea, macroglossia was also noted. A difficult airway was anticipated in this patient, therefore a MAC 5 laryngoscope was used, and the endotracheal tube railroaded with a bougie into the trachea. The majority of the patients with neurofibromatosis present with cranial or spinal tumors, this accounts for a major portion of the morbidity and mortality in them.1,2 Hypertension due to renal artery stenosis or occult phaeochromocytoma may be found in patients with neurofibromatosis, though this patient was not a known hypertensive. 4,7 During the anaesthesia rather than hypertension, we had hypotensive episodes due to the proximity of the tumor to the orbit which triggered occulocardiac reflex during the intra-cranial dissection by the surgeons. This was abated by immediately alerting the surgeons of the haemodynamic changes observed, which further emphasises the importance of vigilance in the monitoring of patients. The anaesthesia management of patients with neurofibromatosis is rare in our practice, however proper peri-operative evaluation, preparation, and vigilance to details will give the optimum outcome in these patients. 

CONCLUSION 

Anaesthesia in the presence of neurofibromatosis may be hazardous due to associated conditions and widespread organ involvement. Their diverse presentations present as a challenge for both anaesthetists and surgeons. The management of patients with neurofibromatosis in a resource-challenged environment like ours may pose difficulties for anaesthetists; however, adequate preoperative assessment, preparation, and vigilance during surgery will ensure a good perioperative outcome for the patient. 

Conflict of Interest: The authors declare no potential conflict of interest concerning the contents, authorship, and/or publication of this article.

Author Contributions: All authors have equal contributions in the preparation of the manuscript and compilation.

Source of Support: Nil

Funding: The authors declared that this study has received no financial support.

Informed Consent Statement: Informed consent was obtained from the subject involved in the study. 

Data Availability Statement: The data presented in this study are available on request from the corresponding author. 

Ethical approval: Not applicable.

REFERENCES

1. Kishan RB, Durga SV, Mohan K, Swapna T, Maneendra S, Murthy SGK. Anaesthetic Considerations in a Patient with Von Recklinghausen Neurofibromatosis. J Anaesthesiol Clin Pharmacol. 2010 Oct-Dec; 26(4):553-554. https://doi.org/10.4103/0970-9185.74613 PMid:21547193 PMCid:PMC3087283

2. Hirsch NP, Murphy A, Radcliffe JJ. Neurofibromatosis: clinical presentations and anaesthetic implications. BJA 2001;86(4):55-64. https://doi.org/10.1093/bja/86.4.555 PMid:11573632

3. Polyxeni TA, Constantinos NB, Anteia P. Anesthetic management of a patient with type 1 neurofibromatosis and an occult phaeochromocytoma: a case report. Brazilian Journal of Anesthesiology 2023;73(5):695-698. https://doi.org/10.1016/j.bjane.2021.02.045 PMid:33819497 PMCid:PMC10533959

4. Charles JF, Samir T, Aaron JK, Stephanie R, Jacqueline VA, Alan DK. Perioperative Management of Neurofibromatosis Type 1. Oshsner Journal June 2012;12(2):111-121.

5. Fisher M. Anaesthesia difficulties in Neurofibromatosis. Anaesthesia, 1975;30(5):648-650. https://doi.org/10.1111/j.1365-2044.1975.tb00925.x PMid:811128

6. Priyanka S. Neurofibromatosis: Challenge for Anaesthetist. Jour Col Phy and Surg Pakistan. 2015;(25):73-75.

7. Filiz BCE, İrem GO. Anesthesia management in a pregnant patient with neurofibromatosis. J Surg Med. 2022;6(5):591-593. https://doi.org/10.28982/josam.907090