Importance of Sonography for Guiding Central Venous Cannulation in Patients with Neurofibromatosis/Norofibromatoz Hastalarinda Santral Venoz Kanulasyona Rehberlikte Sonografinin Onemi.
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A 15-year-old boy with neurofibromatosis type 1 (NF1) was referred to us for central venous catheter insertion, and on ultrasound of the neck, he was found to have extensive involvement of the brachial plexus due to the nerve sheath tumour. Multiple hypoechogenic lesions resembling the internal jugular vein and internal carotid artery were visualised and could be differentiated from the vessels by Doppler ultrasound. The importance of analyzing sonographic images of nerve sheath tumours, which can mimic blood vessels, and the importance of Doppler ultrasound for guiding central venous catheters in such patients to avoid nerve injury are discussed in this case report.
Keywords: Neurofibromatosis, ultrasound, central venous cannulation
Norofibromatoz tip 1 olan 15 yasinda bir erkek hasta santral venoz kateter takilmasi icin bize yonlendirildi. Hastanin boyun ultrasonunda sinir kilifi tumorunden dolayi yaygin brakiyal pleksus tutulumu izlendi. Internal juguler ven ve internal karotis arteri animsatan coklu hipoekojen lezyonlar goruldu ve Doppler ultrason ile damarlardan ayirt edilebildi. Bu vaka sunumunda, kan damarlarmi taklit edebilen sinir kilifi tumorlerinin sonografik goruntulerinin onemi ve bu hastalarda sinir hasarlarindan kacinmak icin santral venoz kateterlerine rehberlik etmesi acisindan Doppler ultrasonun onemi tartisilmaktadir.
Anahtar Sozcukler: Norofibromatoz, ultrason, santral venoz kanulasyon
Neurofibromatosis type 1 (NF1) involves the peripheral nerves with an incidence of 1:2500-3300; however, the involvement of the brachial plexus (BP) is rare (1). It can cause wide distortion of the underlying BP anatomy, and central venous cannulation (CVC) can be challenging because of the risk involved in causing permanent nerve injury. In many developing countries, ultrasound (US) is not freely available and central venous catheter insertion is commonly performed using the traditional landmark technique.
We report a case of a patient who required CVC and had extensive involvement of BP caused by the nerve sheath tumour, which resembled the internal jugular vein and external carotid artery, as observed on US of the neck. We discuss the importance and role of sonography for guiding CVC in patients with NF to avoid nerve injury.
A 15-year-old boy with NF1 had neuropathic pain along the supply of the right ulnar nerve for the past 3 years and was referred to us for CVC because of difficult peripheral venous access. He was on oral non-steroidal anti-inflamatory drugs (NSAIDS) and had received 1 session of pulse radio frequency ablation a month previously for the neuropathic pain. After explaining the procedure, written consent was obtained and the patient was positioned for right internal jugular vein cannulation. Standard monitors for American Society of Anesthesiology grade 1 were attached and supplementary oxygen was given via a nasal prong with a flow of 3 L [min.sup. -1]. Intravenous fentanyl bolus of 0.5 [micro]g [kg.sup. -1] was given for sedation. An initial US scan was performed with the middle of the probe along the apex of the triangle formed by the 2 heads of sternocleidomastoid muscles and clavicle using SonoSite 180 plus and a linear array 6-13 MHz probe (SonoSite Drive SE Bothell, WA, USA). Multiple vascular-like structures having the same diameter of the internal jugular vein and external carotid artery were visualised (Figures 1 and 2). Initially, they were thought to be some anatomical variants and branches of the internal jugular vein, but on applying pressure, they were not compressible. The internal carotid artery could not be differentiated from these structures because the usual arterial pulsation was not evident in this case. On tracing the structures cranially and caudally, similar vascular-like structures were seen, and only on colour Doppler US, the internal jugular vein and external carotid artery could be delineated from the neural tissue, which has extensively involved the BP.
The skin and subcutaneous tissues were anaesthetised with 2% lidocaine using a 25-gauge needle. After skin anaesthesia, a 16-gauge, 10-cm-long needle was introduced in an out-of-plane technique. The tip of the needle was advanced under direct visualisation without piercing the nerve until it punctured the internal jugular vein and negative aspiration confirmed free flow of blood. A 7-Fr triple lumen catheter (Certrofix, B.Braun Melsungen AG, Germany) was successfully placed using the Seldinger technique. All ports were aspirated and flushed with heparinised saline, and the triple lumen catheter was secured.
During the procedure, the patient had no discomfort, pain or any sign of the needle piercing the neural tissues. The right internal jugular vein was successfully cannulated under US guidance without nerve injury. An US scan on the other side of neck showed a similar sonographic image of BP involvement. An US scan of the bilateral groin area showed a normal anatomical relation between the vascular structures and femoral nerve.
Neurofibromatosis type 1 is an autosomal dominant disease, with NF1 being the most common disease that affects a wide range of physiological systems (2). NF1 presents with neuro-cutaneous symptoms such as cafe au lait spots (95%) and axillary freckles (75%) (1), which were evident in our case. Reynolds et al. (3) have described the sonographic appearance of nerve sheath tumours as a hypoechoeic mass with an echogenic ring and posterior acoustic enhancement (4), and Beggs et al. (5) have described the nerve running into the tumour as a pathognomonic feature of nerve sheath tumours. These sonographic features of nerve sheath tumours can resemble those of vascular structures, and these tumours can therefore be mistaken for vessels during CVC. In our case, the carotid artery also appeared hypoechoeic with a hyperechoeic ring and it was non-compressible. US alone could not differentiate it from neural tissues, and it was clearly differentiated only on colour Doppler US. This shows that high-cost scanners with colour Doppler US facility should be used in patients with NF because differentiating between vascular structures and nerve tissues can be difficult. Other methods that can be used to differentiate between these are dynamic compression of the probe over the skin surface that can easily compress the vein and the arterial pulsation (although it was not evident in our case) can help differentiate vascular structures from neural tissues.
Although the internal jugular vein was successfully cannulated under US guidance in our case, the sonographic image showed that there is a high risk of nerve injury during CVC in NF patients involving BP. The large needle size used can cause permanent injury to the nerve; therefore, Doppler US is highly recommended in these patients for CVC.
We emphasise the importance of central venous catheter insertion in patients with NF under sonographic guidance to avoid nerve injury. In centres where US is routinely not available, femoral vein cannulation can be considered. Cutaneous markers such as cafe au lait spots and axillary freckles can be used as clinical markers for detecting possible underlying nerve sheath tumours, which can involve BP, when considering CVC.
Informed Consent: Written informed consent was obtained from patient who participated in this case.
Peer-review: Externally peer-reviewed.
Author Contributions: Concept - N.N.; Design - N.N., V.K.M.; Supervision - N.N., V.K.M.; Data Collection and/or Processing - N.N., V.K.M.; Analysis and/or Interpretation - N.N., V.K.M.; Literature Search - N.N., V.K.M.; Writing Manuscript - N.N.; Critical Review - N.N., V.K.M.
Conflict of Interest: No conflict of interest was declared by the authors.
Financial Disclosure: The authors declared that this study has received no financial support.
Hasta Onami: Yazili hasta onami bu olguya katilan hastadan alinmistir.
Hakem Degerlendirmesi: Dis bagimsiz.
Yazar Katkilari: Fikir - N.N.; Tasarim - N.N., V.K.M.; Denetleme - N.N., V.K.M.; Veri Toplanmasi ve/veya Islemesi - N.N., V.K.M.; Analiz ve/veya Yorum - N.N., V.K.M.; Literatur Taramasi - N.N., V.K.M.; Yaziyi Yazan - N.N.; Elestirel Inceleme - N.N., V.K.M.
Cikar Catismasi: Yazarlar cikar catismasi bildirmemislerdir.
Finansal Destek: Yazarlar bu calisma icin finansal destek almadiklarini beyan etmislerdir.
(1.) Binder DK, Smith JS, Barbaro NM. Primary brachial plexus tumors: Imaging, surgical, and pathological findings in 25 patients. Neurosurg Focus 2004; 16: E11.
(2.) Hirsch NP, Murphy A, Radcliffe JJ. Neurofibromatosis: clinical presentations and anaesthetic implications. Br J Anaesth 2001; 86: 555-64. [CrossRef]
(3.) Reynolds DL, Jacobson JA, Inampudi P, Jamadar DA, Ebrahim FS, Hayes CW Sonographic characteristics of peripheral nerve sheath tumors. AJR Am J Roentgenol 2004; 182: 741-4. [CrossRef]
(4.) Kara M, Yilmaz A, Ozel S. Sonographic imaging of the peripheral nerves in a patient with neurofibromatosis type 1. Muscle Nerve 2010; 41: 887-8. [CrossRef]
(5.) Beggs I. Sonographic appearances of nerve tumors. J Clin Ultrasound 1999; 27: 363-8. [CrossRef]
V.K. Mohan, Neisevilie Nisa
All India Institute of Medical Sciences, New Delhi, India
Address for Correspondence/Yazisma Adresi: Neisevilie Nisa E-mail: email@example.com
[c] Copyright 2017 by Turkish Anaesthesiology and Intensive Care Society - Available online at www.jtaics.org
[c] Telif Hakki 2017 Turk Anesteziyoloji ve Reanimasyon Dernegi - Makale metnine www.jtaics.org web sayfasindan ulasilabilir.
Received / Gelif Tarihi : 26.11.2016
Accepted / Kabul Tarihi: 20.01.2017
Available Online Date /Cevrimici Yayin Tarihi : 27.04.2017
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|Title Annotation:||Case Report / Olgu Sunumu|
|Author:||Mohan, V.K.; Nisa, Neisevilie|
|Publication:||Turkish Journal of Anaesthesiology and Reanimation|
|Article Type:||Case study|
|Date:||Jun 1, 2017|
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