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Year : 2023  |  Volume : 2  |  Issue : 1  |  Page : 44-46

Regional anesthesia for a patient with extensive von Recklinghausen's disease

1 Department of Anaesthesia and Intensive Care, Maharishi Markandeshwar Institute of Medical Sciences and Research, Mullana, Haryana, India
2 Department of Emergency Medicine, Dr. B. R. Ambedkar State Institute of Medical Sciences, Mohali, Punjab, India

Date of Submission12-Nov-2022
Date of Decision04-Jan-2023
Date of Acceptance05-Jan-2023
Date of Web Publication25-May-2023

Correspondence Address:
Dr. Sahil Garg
Department of Anaesthesia and Intensive Care, Maharishi Markandeshwar Institute of Medical Sciences and Research, Mullana - 133 203, Haryana
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jica.jica_37_22

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We report the case of a 60-year-old male posted for surgery on the left inner thigh mass with extensive neurofibromas covering the whole body. The patient was administered a subarachnoid block at L3-L4 level for excision of the mass on the left inner thigh. There were no postoperative anesthetic complications. This case report aims to highlight the importance of regional anesthesia in patients with neurofibromatosis and its anesthetic implications.

Keywords: Neurofibromatosis, regional anesthesia, von Recklinghausen's disease

How to cite this article:
Kaur H, Garg S, Gupta R, Yadav T, Prasad S. Regional anesthesia for a patient with extensive von Recklinghausen's disease. J Ind Coll Anesth 2023;2:44-6

How to cite this URL:
Kaur H, Garg S, Gupta R, Yadav T, Prasad S. Regional anesthesia for a patient with extensive von Recklinghausen's disease. J Ind Coll Anesth [serial online] 2023 [cited 2023 Oct 1];2:44-6. Available from: https://www.jicajournal.in//text.asp?2023/2/1/44/377597

  Introduction Top

Neurofibromatosis is an autosomal dominant neurocutaneous single-gene disorder with an incidence of 1 in 3500 individuals at birth.[1],[2] Neurofibromatosis is classified into NF1 and NF2. NF1 was initially described in 1882 by Dr. Friedrich Daniel von Recklinghausen and accordingly called as von Recklinghausen's disease.[1] NF1 is characterized by multiple cafe-au-lait spots on the skin, peripheral tumors, and a variety of dysplastic abnormalities of the skin, nervous system, bones, endocrine organs, and blood vessels.[3] Hypertension is the most common finding in NF1 and can be multifactorial being either essential hypertension or resulting from renovascular disease, aortic coarctation, or pheochromocytoma. NF2 is characterized by bilateral vestibular schwannomas (tumors arising from the vestibular branch of the VIII cranial nerve).[4] Both NF1 and NF2 can present as a nongeneralized form in which only one part of the body is affected. This requires an astute decision by an anesthesiologist confronted with such a case posted for surgery as unique obstacles must be overcome for success.

  Case Report Top

A 60-year-old, 55 kg, 170 cm male patient with a known case of von Recklinghausen's disease was scheduled for excision of the left inner thigh mass. The patient had extensive neurofibromatous lesions on the back, as shown in [Figure 1]. The patient had a previous history of pulmonary tuberculosis 10 years back treated by antitubercular treatment for 8 months. He was a reformed smoker for 9 years. General physical and systemic examinations were normal. Routine laboratory investigations were within normal limits (WNL). Noncontrast computed tomography (NCCT) head and magnetic resonance imaging (MRI) brain with the whole spine were done to evaluate any neurofibromatous lesions in the brain and spinal cord, which were found to be normal. Airway examination was WNL with MP grade 1. Regional anesthesia was planned for surgery. Anesthetic procedure was explained to the patient, and written informed consent was taken. In the preoperative room, an 18G cannula was secured. After shifting to the operation theater, multipara monitor was attached (heart rate, noninvasive blood pressure, and SpO2). Intravenous fluid was started. After cleaning and draping in sitting position, the patient was administered a subarachnoid block (SAB) at L3-L4 intervertebral space with 25G Quincke's spinal needle and 3 ml of 0.5% heavy bupivacaine given in the subarachnoid space. The patient was put in supine position immediately. T10 level of block was achieved after 5 min. Surgery started under adequate analgesia and lasted for 90 min with minimal blood loss. 1200 ml of IV crystalloids were given. The patient was shifted to the postoperative room after 30 min and then shifted to the ward with normal vitals. Follow-up of the patient was done in the ward from postoperative day 1 to day 3 and was uneventful. The patient was discharged on postoperative day 3 uneventfully.
Figure 1: Image showing extensive neurofibromatous lesions on the back of the patient

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  Discussion Top

Neurofibromatosis is a group of hereditary autosomal dominant single-gene disorders divided into type 1 and type 2 that have widespread effects on ectodermal and mesodermal tissues.[5] NF1 is more prevalent than NF2. NF1 is characterized by cafe-au-lait spots (99%), cutaneous neurofibroma (95%), Lisch nodules (95%), plexiform neurofibromas (30%), and sometimes pheochromocytomas. NF2 is characterized by bilateral vestibular schwannomas. Tumors may also involve the tongue, larynx, pharynx, and chest wall causing difficult airway. Tumors may involve the central nervous system manifesting as cognitive disabilities, behavioral issues, and motor delays.[6] Hence, anesthetic assessment should be done via MRI and CT scan of the head and spine.[5] The choice between general and regional anesthesia in patients with neurofibromatosis requires careful evaluation.

Traditionally, general anesthesia has been the usual approach as coexisting cranial and spinal neuromas might worsen neurological outcomes in patients who have been taken for regional anesthesia. Decision between regional and general anesthesia should be meticulously weighed. The identification of spinous process at the back is quite difficult, but not impossible by an experienced anesthesiologist. There are many case reports suggestive of increased sensitivity of patients with NF1 to nondepolarizing muscle relaxants.[7],[8]

The patient was reluctant to undergo general anesthesia. So, extent of lesions was assessed on the back of patient and it was found to be away from the midline. NCCT head and MRI brain with whole spine were also normal. Hence, Regional anaesthesia was planned.

Similar to our case, Singh et al. administered SAB in a preeclamptic parturient with von Recklinghausen's neurofibromatosis.[9] Similarly, Demir et al. gave SAB for cesarean section in a neurofibromatosis patient with severe kyphoscoliosis.[10]

  Conclusion Top

Regional anesthesia may be considered a good choice in patients with neurofibromatosis to avoid complications associated with general anesthesia. Hence, an anesthesiologist must perform a thorough preoperative evaluation of every patient presenting with neurofibromatous lesions and should make appropriate decision regarding anesthetic plan.

Informed consent

Written informed consent for publication was taken from the patient.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that his name and initials will not be published and due efforts will be made to conceal his identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Fox CJ, Tomajian S, Kaye AJ, Russo S, Abadie JV, Kaye AD. Perioperative management of neurofibromatosis type 1. Ochsner J 2012;12:111-21.  Back to cited text no. 1
Mulvihill JJ, Parry DM, Sherman JL, Pikus A, Kaiser-Kupfer MI, Eldridge R. NIH conference. Neurofibromatosis 1 (Recklinghausen disease) and neurofibromatosis 2 (bilateral acoustic neurofibromatosis). An update. Ann Intern Med 1990;113:39-52.  Back to cited text no. 2
Hirata D, Nara H, Inaba T, Muroi R, Kanegane H, Miyawaki T, et al. Von Recklinghausen disease in a patient with X-linked agammaglobulinemia. Intern Med 2002;41:1039-43.  Back to cited text no. 3
Mautner VF, Asuagbor FA, Dombi E, Fünsterer C, Kluwe L, Wenzel R, et al. Assessment of benign tumor burden by whole-body MRI in patients with neurofibromatosis 1. Neuro Oncol 2008;10:593-8.  Back to cited text no. 4
Hirsch NP, Murphy A, Radcliffe JJ. Neurofibromatosis: Clinical presentations and anaesthetic implications. Br J Anaesth 2001;86:555-64.  Back to cited text no. 5
Gutmann DH, Parada LF, Silva AJ, Ratner N. Neurofibromatosis type 1: Modeling CNS dysfunction. J Neurosci 2012;32:14087-93.  Back to cited text no. 6
Magbagbeola JA. Abnormal responses to muscle relaxants in a patient with Von Recklinghausen's disease (multiple neurofibromatosis). Br J Anaesth 1970;42:710.  Back to cited text no. 7
Manser J. Abnormal responses in Von Recklinghausen's disease. Br J Anaesth 1970;42:183-4.  Back to cited text no. 8
Singh T, Hooda S, Anand A, Kaur K, Bala R. Anesthetic consideration in a preeclamptic parturient with Von Recklinghausen's neurofibromatosis. J Obstet Anaesth Crit Care 2014;4:38-40.  Back to cited text no. 9
  [Full text]  
Demir I, Kılıç ET, Akdemir MS. Subarachnoid block a safe choice for cesarean section in neurofibromatosis patient with severe kyphoscoliosis. Anesth Essays Res 2018;12:273-5.  Back to cited text no. 10
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