|Year : 2015 | Volume
| Issue : 3 | Page : 165-167
Maxillectomy for malignant nasopharyngeal neurofibroma: Anesthetic considerations
Shagun Bhatia Shah, Anita Kulkarni
Department of Anaesthesiology, Rajiv Gandhi Cancer Institute and Research Centre, New Delhi, India
|Date of Web Publication||16-Jul-2015|
Shagun Bhatia Shah
H. No: 174 - 175, Ground Floor, Pocket-17, Sector-24, Rohini, New Delhi - 110 085
Source of Support: None, Conflict of Interest: None
Neurofibromatosis-1 (NF-1) or von Recklinghausen's disease is the most common disease inherited by a single gene and is multisystemic in nature. Anesthesia is required for excision of neurofibromas, which may cause cosmetic disfigurement, pressure effects (obstructive uropathy, hydronephrosis, kyphoscoliosis, hydrocephalus, proptosis, and superior vena cava syndrome) and may undergo malignant change. We report here the anesthetic management of an NF-1 patient with a malignant change in a nasopharyngeal neurofibroma requiring left maxillectomy and split skin grafting.
Keywords: Difficult Intubation, malignancy, nasopharyngeal neurofibroma
|How to cite this article:|
Shah SB, Kulkarni A. Maxillectomy for malignant nasopharyngeal neurofibroma: Anesthetic considerations. Int J Health Allied Sci 2015;4:165-7
|How to cite this URL:|
Shah SB, Kulkarni A. Maxillectomy for malignant nasopharyngeal neurofibroma: Anesthetic considerations. Int J Health Allied Sci [serial online] 2015 [cited 2020 May 27];4:165-7. Available from: http://www.ijhas.in/text.asp?2015/4/3/165/160891
| Introduction|| |
Facial neurofibromas are notorious for soft tissue infiltration and osseous dysplasia. Cancer risks and the disease's completely unpredictable evolution urge a regular and multidisciplinary patient follow-up. Potential anesthetic challenges include difficult airway, , abnormal spinal anatomy, peripheral neurofibromas and systemic lesions causing multisystem derangements. Neurofibromatosis type 1 (NF-1) represents a major risk factor for development of malignancy, particularly malignant peripheral nerve sheath tumors (MPNST), optic gliomas, and leukemia. ,,,
| Case report|| |
We present here a case of a 64-year-old 42 kg woman suffering from NF-1 or von Recklinghausens disease for 45 years [Figure 1]. She presented with left nasal obstruction, discharge, cough and voice change spanning 2 months. Her skin displayed neurofibromatous nodules of all sizes one which had undergone malignant transformation contrast-enhanced computed tomography revealed a mass in the left nasal cavity, extending into the nasopharynx diagnosed as MPNST on biopsy. She was posted for left maxillectomy and split skin grafting (SSG).
Preoperative investigations included a complete blood count, hepatic, renal and pulmonary function test (PFT), coagulation profile, serum electrolytes, electrocardiogram, echocardiogram, chest radiograph, and arterial blood gas sample on room air, which were all within normal limits. She had adequate mouth opening, temporomandibular joint, and neck movements and was of Mallampati class-II. On direct laryngoscopy, both vocal cord movements were normal. Oral premedication with ranitidine 150 mg, granisetron 2 mg and alprazolam 0.5 mg the night before surgery was given.
In the operation theater midazolam, 1 mg was injected which decreased her blood pressure from 164/92 mmHg to 123/63 mmHg. All monitors including the peripheral nerve stimulator (PNS) were attached. The nature and extent of surgery required a nasotrachealintubation. The right nostril was prepared with oxymetazoline drops and lignocaine soaked nasal pack. Fentanyl 90 μg was given. Inhalational induction was performed with sevoflurane increased in increments of 0.5% uptil 4%. A 6.5 mm ID cuffed portex endotracheal tube (ETT) made pliable by immersion in hot water using a flexible fiberoptic bronchoscope inserted via the right nostril into the nasopharynx after generous lubrication with water soluble jelly. Clinical and capnographic confirmation of ETT placement was done. Nasal bleeding occurred while sliding the ETT over the fiberoptic cable and 30 ml of blood was suctioned out of the oral cavity. The nostril was manually compressed over the tube for a few minutes. This stopped the bleeding. Anesthesia was maintained with oxygen and nitrous oxide (40-60%), sevoflurane and intermittent fentanyl boluses of 20 μg whenever the systolic blood pressure rose more than 20% above baseline. The neuromuscular relaxant used was atracurium 30 mg bolus, followed by 5 mg top up doses as guided by the PNS. Dexamethasone 8 mg, ranitidine 50 mg, ondansetron 8 mg and paracetamol 1 g were the other drugs injected intravenously.
The surgery lasted 3 h. Blood loss was replaced with 1 L of crystalloids, 500 ml of colloid and two units of packed red blood cells were transfused. Neuromuscular blockade was reversed with neostigmine and glycopyrrolate. Emergence was smooth and complete and nasal ETT electively left in situ due to the nature and extent of surgery and because of an anticipated difficult re-intubation. The patient was kept on a T-piece overnight on spontaneous breathing and extubated successfully next morning. Intravenous morphine 1.5 mg 6 hourly provided postoperative analgesia.
| Discussion|| |
The NF are autosomal dominant diseases with a prevalence of 1 in 5000. NF-1 gene has been localized to tumor suppressor chromosome 17q 11.2, any mutation predisposing to malignant change. Six or more cafe-au-lait spots (hyperpigmented macules) and two or more Lisch nodules (benign melanotic iris hamartomas) are pathognomonic. Bony lesions include dysplasia of sphenoid wing and thinning of long bones. ,, Malignant transformation of neurofibromas at multiple sites including the thyroid gland, has been documented. 
Key emphasis areas include a detailed airway, cardiorespiratory (hypertrophic cardiomyopathy and outflow, obstruction, atrial septal defect, pulmonary stenosis, coarctation of aorta, mitral valve regurgitation and prolapse),  hepatorenal (obstructive uropathy),  gastrointestinal (bleeds and duodenal carcinoids), central (epilepsy, cognitive and learning deficits, central sleep-apnea syndrome) and peripheral nervous system workup to avoid misadventure. ,,,
Anticipating nasal and nasopharyngeal lesion cautious nasotracheal intubation and nasogastric tube placement was done to prevent bleeding complication. When the larynx is involved, it is often the aryepiglottic fold or arytenoids, reflecting areas richest in terminal nerve plexuses. 
One must anticipate a difficult intubation in NF-1 patients. Use of Rendall Baker Soucek facemask and left molar laryngoscopy for ventilation and intubation have been described.  Macroglossia and lesions of laryngopharynx are documented causes of sudden airway obstruction on induction of anesthesia.  Loss of nasal intonation of voice was the symptom which prompted us to opt for direct laryngoscopy to rule out vocal cord lesions. We opted for inhalational induction of anesthesia and preservation of spontaneous respiration and were prepared with a difficult intubation cart that included provision for cricothyrotomy and emergency tracheostomy (surgical airway). We used fentanyl to prevent the laryngoscopy and intubation response as NF-1 patients are prone to hypertension due to documented association with multiple endocrine neoplasia-II, renal artery dysplasia, pheochromocytoma and hypertrophic cardiomyopathy. Mediastinal neurofibromas may result in superior vena caval (SVC) obstruction. ,,,
Arterial blood gas sampling and PFT were obtained before surgery due to a documented association of NF-1 with pulmonary fibrosis, cystic lung disease, central alveolar hypoventilation, kyphoscoliosis, right ventricular failure, tracheobronchial and intrapulmonary neurofibroma and pulmonary fibrosis. ,,,
Resistance to succinylcholine and a paradoxical sensitivity to nondepolarizing neuromuscular blocking agents require a PNS as a guide, especially with associated renal dysfunction. We intubated the trachea after single second twitches disappeared, maintained muscle relaxation with small boluses maintaining a low intensity first twitch of train of four (TOF) at all times and waited for the TOF-ratio to reach 0.9 and deep brain stimulation to show equal intensity twitches before giving the reversal agents. 
SSG, if required, as in our case, can be harvested from the nerofibromatous skin but the blade tends to go deeper at points of individual skin lesions and pockets of skin may have to be sutured at the donor site.
Silent neurofibromas involve spinal cord and nerve roots in up to 40% of patients. Gliomas, meningiomas, hydrocephalus and spina bifida have been documented. Neurofibromas at the site of the block can also pose difficulties. Peripheral nerves affected with intraneural neurofibromas can also be successfully blocked with ultrasound-guided local anesthetic blocks avoiding intralesional injections. Potential issues unique to NF patients include alterations in nerve conduction, the spread of local anesthetic, and onset and duration of blockade. 
Despite adequate preparation with nasal drops, nasal packing with lignocaine soaked ribbon gauze, lubricating jelly and gentle handling, nasal bleeding may still complicate an already difficult airway, especially if fiberoptic bronchoscopic tracheal intubation is planned. However, forewarned is forearmed. Had we not anticipated a difficult airway and nasal bleed, any lesser degree of preparation of the airway may have resulted in catastrophic bleeding or a ventilatory misadventure. Additional measures are using adrenalin along with lignocaine (in noncardiac patients) for the nasal pack, avoiding nasal instrumentation, PNS usage, fentanyl to counter hemodynamic response to intubation and elective postoperative retention of the ETT with patient's consent. These measures may not be a fool proof solution to the highlighted problems, but definitely go a long way in mitigating them.
| Conclusion|| |
NF-1 may affect the airway, cardiorespiratory, peripheral and central nervous, musculoskeletal, gastrointestinal, and the urogenital systems. A detailed and systematic preoperative assessment is mandatory keeping in mind the diverse clinical manifestations. Anesthesia is often required for excision of neurofibromas, which may cause cosmetic disfigurement, pressure effects (obstructive uropathy, hydronephrosis, kyphoscoliosis, hydrocephalus, proptosis, and SVC syndrome) and malignant change. For a rational peri-operative management, anesthesiologists must tailor their technique to suit individual patients.
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Conflicts of interest
There are no conflicts of interest.
| References|| |
Fox CJ, Tomajian S, Kaye AJ, Russo S, Abadie JV, Kaye AD. Perioperative management of neurofibromatosis type 1. Ochsner J 2012;12:111-21.
Saini S, Bansal T. Anesthetic management of difficult airway in a patient with massive neurofibroma of face: Utility of Rendell Baker Soucek mask and left molar approach for ventilation and intubation. J Anaesthesiol Clin Pharmacol 2013;29:271-2.
Korf BR. Malignancy in neurofibromatosis type 1. Oncologist 2000;5:477-85.
Hirsch NP, Murphy A, Radcliffe JJ. Neurofibromatosis: Clinical presentations and anaesthetic implications. Br J Anaesth 2001;86:555-64.
Savar A, Cestari DM. Neurofibromatosis type I: Genetics and clinical manifestations. Semin Ophthalmol 2008;23:45-51.
Leslie MD, Cheung KY. Malignant transformation of neurofibromas at multiple sites in a case of neurofibromatosis. Postgrad Med J 1987;63:131-3.
Shah S, Murthy PV, Gopalkrishnan G, Pandey AP. Neurofibromatosis of the bladder and urethra presenting as obstructive uropathy. Br J Urol 1988;61:364-5.
Rahbar R, Litrovnik BG, Vargas SO, Robson CD, Nuss RC, Irons MB, et al.
The biology and management of laryngeal neurofibroma. Arch Otolaryngol Head Neck Surg 2004;130:1400-6.
Crozier WC. Upper airway obstruction in neurofibromatosis. Anaesthesia 1987;42:1209-11.
Rossi SE, Erasmus JJ, McAdams HP, Donnelly LF. Thoracic manifestations of neurofibromatosis-I. AJR Am J Roentgenol 1999;173:1631-8.
Richardson MG, Setty GK, Rawoof SA. Responses to nondepolarizing neuromuscular blockers and succinylcholine in von Recklinghausen neurofibromatosis. Anesth Analg 1996;82:382-5.
Kara M, Yilmaz A, Ozel S, Ozçakar L. Sonographic imaging of the peripheral nerves in a patient with neurofibromatosis type 1. Muscle Nerve 2010;41:887-8.