Plexiform neurofibroma treatment

Plexiform neurofibroma treatment

Since plexiform neurofibromas are a major cause of the burden of disease and may also progress to malignancy, many efforts have been undertaken to find a cure for these tumors. However, neither surgery nor medication has so far produced a breakthrough therapeutic success.

Plexiform neurofibromas with sizable intraspinal extensions and resultant spinal cord compromise pose challenging management problems, because these lesions may involve multiple nerves and engulf adjacent vascular and visceral structures 1).

Decisions about surgical treatment and frequency of follow-up must be made judiciously and individualized for each patient 2).

Plexiform neurofibromas arising in the orbito-temporal area pose a greater challenge due to its critical function and cosmetic importance of the face. Such plexiform neurofibromas, separately designated as orbito-temporal plexiform neurofibromas, show complex symptoms such as severe ptosis, ectropion, lacrimal gland dysfunction, and even vision loss 3).


A clinical phase I study reported significant shrinkage of plexiform neurofibromas following treatment with the MEK inhibitor selumetinib.

Vaassen et al., reported an 11-year-old NF1 patient with a large plexiform neurofibroma of the neck that had led to a sharp-angled kinking of the cervical spine and subsequent myelopathy. Although surgical stabilization of the cervical vertebral column was urgently recommended, the vertebral column was inaccessible due to extensive tumor growth. In this situation, treatment with the MEK inhibitor trametinib was initiated which resulted in a 22% reduction in tumor volume after 6 months of therapy and finally enabled surgery. These data show that MEK inhibitors may not lead to complete disappearance of NF1-associated plexiform neurofibromas but can be an essential step in a multimodal therapeutic approach for these tumors. The course of our patient suggests that MEK inhibitors are likely to play a significant role in providing a cure for one of the most devastating manifestations of NF1 4).

References

1)

Pollack IF, Colak A, Fitz C, Wiener E, Moreland M, Mulvihill JJ. Surgical management of spinal cord compression from plexiform neurofibromas in patients with neurofibromatosis 1. Neurosurgery. 1998 Aug;43(2):248-55; discussion 255-6. PubMed PMID: 9696077.
2)

Gutmann DH, Aylsworth A, Carey JC, Korf B, Marks J, Pyeritz RE, Rubenstein A, Viskochil D. The diagnostic evaluation and multidisciplinary management of neurofibromatosis 1 and neurofibromatosis 2. JAMA. 1997 Jul 2;278(1):51-7. Review. PubMed PMID: 9207339.
3)

Choi J, Choi HJ, Kang KJ, Kwon H, Shin J. Simultaneous Forehead Lift and Blepharoplasty Techniques in Management of Orbito-Temporal Plexiform Neurofibroma. J Craniofac Surg. 2019 Mar 14. doi: 10.1097/SCS.0000000000005448. [Epub ahead of print] PubMed PMID: 30889063.
4)

Vaassen P, Dürr N, Röhrig A, Willing R, Rosenbaum T. Trametinib Induces Neurofibroma Shrinkage and Enables Surgery. Neuropediatrics. 2019 May 29. doi: 10.1055/s-0039-1691830. [Epub ahead of print] PubMed PMID: 31141829.

Motocross accident

Motocross accident

Motocross is a form of off-road motorcycle racing held on enclosed off-road circuits. The sportevolved from motorcycle trials competitions held in the United Kingdom.

Motocross is a physically demanding sport held in all-weather conditions.

They have been gaining popularity among children and adolescents, raising concerns for increased risk of concussions in participating youth.


A 25-year-old man sustained a right-sided brachial plexus injury from a high-velocity motocross accident. Physical examination and electromyography were consistent with a pan-brachial plexopathy with no evidence of axonal continuity. The patient underwent a spinal accessory nerve to suprascapular nerve transfer and an intercostal nerve to musculocutaneous nerve transfer with interpositional sural nerve grafts. He recovered MRC 4/5 elbow flexion and MRC 2/5 shoulder abduction and external rotation. Twenty-two months post-injury the patient displayed a flicker of flexion of his flexor pollicis longus and flexor digitorum profundus to his index finger – he went on to recover a functional pinch. Thirty-six months post-injury the patient displayed a flicker of contraction in brachioradialis with motor unit potentials on electromyography. This case demonstrates that some patients may have capacity for functional recovery after prolonged denervation and highlights the potential impact of anatomical anomalies in the assessment and treatment of peripheral nerve injuries 1).


A 25-year-old man had a T11T12 fracture dislocation sustained in a motocross accident that resulted in a T11 American Spinal Injury Association Impairment Scale (ASIA) grade A traumatic spinal cord injury. He was treated with acute surgical decompression and spinal fixation with fusion, and enrolled in the spinal scaffold study. A 2 × 10 mm bioresorbable scaffold was placed in the spinal cord parenchyma at T12. The scaffold was implanted directly into the traumatic cavity within the spinal cord through a dorsal root entry zone myelotomy at the caudal extent of the contused area. By 3 months, his neurological examination improved to an L1 AIS grade C incomplete injury. At 6-month postoperative follow-up, there were no procedural complications or apparent safety issues related to the scaffold implantation.

Although longer-term follow-up and investigation are required, this case demonstrates that a polymer scaffold can be safely implanted into an acutely contused spinal cord. This is the first human surgical implantation, and future outcomes of other patients in this clinical trial will better elucidate the safety and possible efficacy profile of the scaffold 2).


Nearly half of all motocross competitors under the age of 18 reported concussion symptoms. Preventive measures are necessary to limit the negative impact from concussions. The risk of concussive injury can be decreased for pediatric motocross riders if they receive professional help with proper helmet fitting and through implementation of stricter guidelines regarding sponsorship 3).

Daniels et al. found a high occurrence of head injuries following pediatric off-road motorcycle riding or motocross accidents despite the use of helmets. Additionally, this study severely underestimates the rate of mild TBIs in this patient population. Our data indicate that motocross is a high-risk sport despite the use of protective gear. Riders and parents should be counseled accordingly about the risks prior to participation 4).

Increased degenerative changes in the cervical and thoracic spine were identified in adolescent motocross racers compared with age-matched controls. The long-term consequences of these changes are unknown; however, athletes and parents should be counseled accordingly about participation in motocross activities 5).

References

1)

Head LK, Wolff G, Boyd KU. Reinnervation of Extrinsic Finger Flexors and Brachioradialis 22 and 36 Months Following Traumatic Pan-Brachial Plexopathy: A Case Report. J Hand Surg Asian Pac Vol. 2019 Mar;24(1):118-122. doi: 10.1142/S2424835519720081. PubMed PMID: 30760136.
2)

Theodore N, Hlubek R, Danielson J, Neff K, Vaickus L, Ulich TR, Ropper AE. First Human Implantation of a Bioresorbable Polymer Scaffold for Acute Traumatic Spinal Cord Injury: A Clinical Pilot Study for Safety and Feasibility. Neurosurgery. 2016 Aug;79(2):E305-12. doi: 10.1227/NEU.0000000000001283. PubMed PMID: 27309344.
3)

Luo TD, Clarke MJ, Zimmerman AK, Quinn M, Daniels DJ, McIntosh AL. Concussion symptoms in youth motocross riders: a prospective, observational study. J Neurosurg Pediatr. 2015 Mar;15(3):255-60. doi: 10.3171/2014.11.PEDS14127. Epub 2015 Jan 2. PubMed PMID: 25555121.
4)

Daniels DJ, Clarke MJ, Puffer R, Luo TD, McIntosh AL, Wetjen NM. High occurrence of head and spine injuries in the pediatric population following motocross accidents. J Neurosurg Pediatr. 2015 Mar;15(3):261-5. doi: 10.3171/2014.9.PEDS14149. Epub 2015 Jan 2. PubMed PMID: 25555116.
5)

Daniels DJ, Luo TD, Puffer R, McIntosh AL, Larson AN, Wetjen NM, Clarke MJ. Degenerative changes in adolescent spines: a comparison of motocross racers and age-matched controls. J Neurosurg Pediatr. 2015 Mar;15(3):266-71. doi: 10.3171/2014.9.PEDS14153. Epub 2015 Jan 2. PubMed PMID: 25555120.

Meralgia Paresthetica Surgery

Meralgia Paresthetica Surgery

Options include:

NeurolysisNeurectomyTransposition.

Selective L2 nerve stimulation

In Neurectomy there are risks of denervation pain, and leaves an anesthetic area (usually a minor nuisance). May be best reserved for treatment failures.

There was insufficient evidence to recommended neurolysis or neurectomy treatment over the other. This highlights the importance of keeping a registry in order to compare outcomes between the two methods of treatment 1).

Approach

The operation is best performed under general anesthesia. A 4–6 cm oblique incision is centered 2 cm distal to the point of tenderness. Since the course of the nerve is variable, the operation is exploratory in nature, and generous exposure is required. If the nerve can’t be located, it is usually because the exposure is too superficial. If the nerve still cannot be found, a small abdominal muscle incision can be made and the nerve may be located in the retroperitoneal area. CAUTION: cases have occurred where the femoral nerve has erroneously been divided.

If neurectomy instead of neurolysis is elected, electrical stimulation should be performed prior to sectioning to rule out a motor component (which would disqualify the nerve as the LFCN). If the nerve is to be divided, it should be placed on stretch and then cut to allow the proximal end to retract back into the pelvis. Any segment of apparent pathology should be resected for microscopic analysis. Neurectomy results in anesthesia in the distribution of the LFCN that is rarely distressing and gradually reduces in size. A supra-inguinal ligament approach has also been described 2).

The considerable anatomic variability of this nerve may complicate surgical localization and thus prolong operative time. Ellis et al., report the use of preoperative high-resolution ultrasonography to map the LFCN in a patient with bilateral meralgia paresthetica. This simple, noninvasive imaging technique was seen to be effective at providing precise localization of the entrapped and, in this case, bilateral anatomically variant nerves. Preoperative high-resolution ultrasound mapping of the LCFN can be used to facilitate precise operative localization in the treatment of bilateral meralgia paresthetica. This is especially useful in the setting of suspected unusual nerve anatomy 3).


Malessy et al., presented a new technique using dynamic decompression and discussed the outcomes.

A retrospective cohort study was performed in a consecutive series of 19 cases. The goal of decompression was pain relief and recovery of sensation. The plane ventral to the LFCN was decompressed by cutting the fascia lata and the inferior aspect of the inguinal ligament. The plane dorsal to the LFCN was decompressed by cutting the fascia of the sartorius muscle. Subsequently, the thigh was brought in full range of flexion and extension/abduction. The authors identified and additionally cut fibers that tightened and caused compression at various locations of the LFCN during movement in all patients, referring to this technique as dynamic decompression. Postoperatively, an independent neurologist scored pain and sensation on a 4-point scale: completely resolved, improved, not changed, or worsened. Patients scored their remaining pain or sensory deficit as a percentage of the preoperative level. Statistical assessment was done using ANOVA to assess the association between outcome and duration of preoperative symptoms, BMI, and length of follow-up.

In 17 of the 19 cases (89%), the pain and/or paresthesia completely resolved. Patients in the remaining 2 cases (11%) experienced 70% and 80% reduction in pain. Sensation completely recovered in 13 of the 19 cases (69%). In 5 of the 19 cases (26%) sensation improved, but an area of hypesthesia remained. Four of these 5 patients indicated a sensory improvement of more than 75%, and the remaining patient had 50% improvement. Sensation remained unchanged in 1 case (5%) with persisting hypesthesia and mild hyperesthesia. There was no significant impact of preoperative symptom duration, BMI, and length of follow-up on postoperative outcome.

Dynamic decompression of the LFCN is an effective technique for the treatment of iMP. Most patients become completely pain free and sensation recovers considerably 4).

Transposition

Nineteen patients with meralgia paresthetica were treated in the Department of Neurological Surgery at University of Wisconsin between 2011 and 2016; 4 patients underwent simple decompression, 5 deep decompression, and 10 medial transposition. Data were collected prospectively and analyzed retrospectively. No randomization was performed. The groups were compared in terms of pain scores (based on a numeric rating scale) and reoperation rates.RESULTSThe numeric rating scale scores dropped significantly in the deep-decompression (p = 0.148) and transposition (p < 0.0001) groups at both the 3- and 12-month follow-up. The reoperation rates were significantly lower in the deep-decompression and transposition groups (p = 0.0454) than in the medial transposition group.CONCLUSIONSBoth deep decompression and transposition of the LFCN provide better results than simple decompression. Medial transposition confers the advantage of mobilizing the nerve away from the anterior superior iliac spine, giving it a straighter and more relaxed course in a softer muscle bed 5).

Neuromodulation

Philip et al., described the first reported use of pulsed radiofrequency neuromodulation to relieve the intractable pain associated with meralgia paresthetica.

A 33-year-old morbidly obese female with a history of lower back pain and previous spinal fusion presented with sensory dysesthesias and paresthesias in the right anterolateral thigh, consistent with meralgia paresthetica. Temporary relief occurred with multiple lateral femoral cutaneous nerve and fascia lata blocks at 2 different institutions. The patient expressed dissatisfaction with her previous treatments and requested “any” therapeutic intervention that might lead to long-lasting pain relief. At this time, we located the anterior superior iliac spine and reproduced concordant dysesthesia. Pulsed radiofrequency was then undertaken at 42 degrees C for 120 seconds followed by dexamethasone and bupivicaine. The patient reported exceptional and prolonged pain relief at 6-month follow-up.

Since this case report is not a prospective, randomized, controlled or blinded study, no conclusions may be drawn from the results attained on behalf of this single individual. Additional, larger group analyses studying this technique while eliminating bias from patient variables would be essential prior to assuming any validity to using pulsed radiofrequency techniques of neuromodulation for managing peripheral neuropathic pain processes.

The patient had experienced long-standing pain that was recalcitrant to conservative/pharmacologic therapy and multiple nerve blocks with local steroid instillations. A single treatment with pulsed radiofrequency resulted in complete and sustained cessation of pain. No side effects were evident. Pulsed radiofrequency of the LFCN may offer an effective, low risk treatment in patients with meralgia paresthetica who are refractory to conservative medical management or are unwilling or unfit to undergo surgery 6).

Case series

Patients whose symptoms did not improve after medical and conservative treatment for at least 3 months were included in this study. These patients underwent neurolysis and decompression surgery and had a mean postoperative follow-up of 38 months. Their pain levels were assessed by the VAS scoring system.

In 8 (61.5%) patients, the symptoms completely resolved within the first 3 months. In 5 (38.5%) patients, the complaints persisted partially and the recovery was observed after 12 months. In patients having a metabolic etiology, the duration of recovery was up to 12 months.

The long term results of surgery are good though only partial improvements in reported pain were seen in the early postoperative period, especially in patients with a metabolic etiology 7).


A total of 16 surgical decompressions could be identified. Retrospective analysis of prospectively collected patient data was performed, as well as systematic evaluation of the postoperative course, with regular follow-up examinations based on a standardized protocol. Pain was analyzed using an NRS (numeric rating scale). Several postsurgical parameters, including temperature hypersensitivity and numbness in the LFCN region, were compared with the presurgical data.Sixty-nine percent of patients had histories of trauma or surgery, which were designated as the onset of pain. Of these patients, 78% had hip prostheses, 2 had previous falls. Postoperatively, a significant reduction of 6.6 points in the mean NRS pain value was observed. All other evaluated parameters also improved postoperatively. Patient satisfaction was high, with 86% reporting complete satisfaction, and 14% reporting partial satisfaction.Previous studies favor either avulsion/neurectomy as the preferred procedure for MP treatment, or provide no recommendation. Our findings instead confirm the decompression/neurolysis approach as the primary surgical procedure of choice for the treatment of MP, if conservative treatment fails 8).


Six patients with intractable meralgia paresthetica with severe pain over antero-lateral thigh along the distribution of lateral cutaneous nerve of thigh which was further confirmed by nerve conduction study. These patients did not respond to the oral anti-neuropathic medications. The two successive diagnostic lateral femoral cutaneous nerve block not only had confirmed the diagnosis but also provided pain relief for a few days. Then the ultrasound-guided lateral femoral cutaneous nerve neurolysis was done using 50% alcohol. In all the patients, there were more than 50% decrease in pain intensity and improvement in quality of life after the procedure, and the relief and improvement were maintained for up to 12 weeks. This case series shows ultrasound-guided lateral femoral cutaneous nerve neurolysis is a safe and effective treatment for intractable meralgia paresthetica and also provides prolonged pain relief and is a good option in avoiding the surgery. Summary points The literature on neurolysis is rare, with only few case reports. This is the first case series on this topic, and it will greatly improve the evidence that ultrasound-guided neurolysis can also be used for intractable meralgia paresthetica patients who do not respond to conservative measures before proceeding to surgery 9).

References

1)

Payne R, Seaman S, Sieg E, Langan S, Harbaugh K, Rizk E. Evaluating the evidence: is neurolysis or neurectomy a better treatment for meralgia paresthetica? Acta Neurochir (Wien). 2017 May;159(5):931-936. doi: 10.1007/s00701-017-3136-x. Epub 2017 Mar 10. Review. PubMed PMID: 28283866.
2)

Aldrich EF, Van den Heever C. Suprainguinal Liga- ment Approach for Surgical Treatment of Meralgia Paresthetica. Technical Note. J Neurosurg. 1989; 70:492–494
3)

Ellis J, Schneider JR, Cloney M, Winfree CJ. Lateral Femoral Cutaneous Nerve Decompression Guided by Preoperative Ultrasound Mapping. Cureus. 2018 Nov 28;10(11):e3652. doi: 10.7759/cureus.3652. PubMed PMID: 30723651; PubMed Central PMCID: PMC6351113.
4)

Malessy MJA, Eekhof J, Pondaag W. Dynamic decompression of the lateral femoral cutaneous nerve to treat meralgia paresthetica: technique and results. J Neurosurg. 2018 Dec 1:1-9. doi: 10.3171/2018.9.JNS182004. [Epub ahead of print] PubMed PMID: 30544337.
5)

Hanna A. Transposition of the lateral femoral cutaneous nerve. J Neurosurg. 2018 Apr 1:1-6. doi: 10.3171/2017.8.JNS171120. [Epub ahead of print] PubMed PMID: 29652230.
6)

Philip CN, Candido KD, Joseph NJ, Crystal GJ. Successful treatment of meralgia paresthetica with pulsed radiofrequency of the lateral femoral cutaneous nerve. Pain Physician. 2009 Sep-Oct;12(5):881-5. PubMed PMID: 19787014.
7)

Ataizi ZS, Ertilav K, Ercan S. Surgical options for meralgia paresthetica: long-term outcomes in 13 cases. Br J Neurosurg. 2018 Nov 19:1-4. doi: 10.1080/02688697.2018.1538480. [Epub ahead of print] PubMed PMID: 30451004.
8)

Schwaiger K, Panzenbeck P, Purschke M, Russe E, Kaplan R, Heinrich K, Mandal P, Wechselberger G. Surgical decompression of the lateral femoral cutaneous nerve (LFCN) for Meralgia paresthetica treatment: Experimental or state of the art? A single-center outcome analysis. Medicine (Baltimore). 2018 Aug;97(33):e11914. doi: 10.1097/MD.0000000000011914. PubMed PMID: 30113491; PubMed Central PMCID: PMC6113044.
9)

Ahmed A, Arora D, Kochhar AK. Ultrasound-guided alcohol neurolysis of lateral femoral cutaneous nerve for intractable meralgia paresthetica: a case series. Br J Pain. 2016 Nov;10(4):232-237. Epub 2016 Sep 16. PubMed PMID: 27867513; PubMed Central PMCID: PMC5102099.
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