Cardiovasc Intervent Radiol DOI 10.1007/s00270-013-0821-x

CASE REPORT

Embolization of the Neck Lymphatic Varix, Causing Periodic Neck Swelling Anthony K. Guzman • Brian A. Pukenas • Yan Yan • Jason G. Newman • Maxim Itkin

Received: 12 September 2013 / Accepted: 10 November 2013 Ó Springer Science+Business Media, LLC (outside the USA) 2013

Abstract We report a case of a 44-year-old female patient, presented to us after years of recurrent intermittent episodes of unilateral left neck swelling. An MR lymphangiogram demonstrated a lymphatic varix at the confluence of the left upper extremity lymphatic ducts, confirmed by intranodal axillary lymphangiography. After successful catheterization of the feeding lymphatic vessels, the varix was successfully embolized with detachable microcoils and an autologous blood patch. The patient has been free from symptoms on subsequent outpatient follow-up. Keywords Embolization  Neck swelling  Lymphatic malformation

A. K. Guzman  Y. Yan Perelman School of Medicine, 600 Market Street, Philadelphia, PA 19104, USA e-mail: [email protected] Y. Yan e-mail: [email protected] B. A. Pukenas  M. Itkin (&) Department of Radiology, Penn Medicine, Hospital of University of Pennsylvania, 3400 Spruce Street, Philadelphia, PA 19104, USA e-mail: [email protected] B. A. Pukenas e-mail: [email protected] J. G. Newman Department of Otolaryngology, Penn Medicine, Pennsylvania Hospital, 811 Spruce Street, Philadelphia, PA 19107, USA e-mail: [email protected]

Introduction Cystic dilation of the distal thoracic duct is a rare cause of neck swelling [1]. The described treatment for this type of lesion has typically been surgical resection [2]. However, nonsurgical approaches such as sclerotherapy are increasingly employed in the treatment of lymphatic malformations [3]. Similarly, thoracic duct embolization is a minimally invasive technique that was developed to treat chylous leaks [4]. In this case report, we describe an adult female patient presenting with recurrent episodes of severe unilateral neck swelling requiring multiple hospitalizations and ICU observation for airway protection. MR lymphangiogram demonstrated a cystic dilatation of the lymphatic vessels draining the arm and left chest wall. Axillary intranodal lymphangiogram was performed to confirm the finding, and the lesion was then successfully treated by embolization with microcoils and autologous blood.

Case Report A 44-year-old female with past medical history significant for allergic rhinitis and mild intermittent asthma originally presented to an outpatient allergist office for evaluation of recurrent paroxysmal unilateral soft tissue edema confined to the left side of the neck. She described four distinct episodes of symptoms. The first episode of swelling occurred 4 years before current presentation. At that time, she was admitted and underwent a CT scan, which was consistent with cellulitis in her left neck and left superior mediastinitis. She was treated with intravenous antibiotics and was discharged on oral clindamycin. She experienced gradual, full resolution of her symptoms within days of discharge.

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One month later, the left neck swelling recurred more severely, prompting presentation at the emergency department, where the patient reported associated stiffness and moderate pain. She underwent a CT scan, which revealed inflammation and edema within the left neck. Notably, the patient was nontoxic-appearing and afebrile but did have a white cell count of 14,000/lL. She was admitted for further evaluation and was started on empiric vancomycin and piperacillin/tazobactam. Otolaryngology evaluation was normal. Ultrasound of the left neck did not reveal any venous thrombus, and chest X-ray was unremarkable. Due to concern for atypical hereditary angioedema, C1q and ACE inhibitor levels were ordered, which were normal. Her symptoms improved within 48 h, and she was discharged home with a course of oral clindamycin, because it was thought that she had experienced a pharyngeal bacterial infection. Her symptoms completely resolved within a few days postdischarge. The patient was symptom-free for 4 years, until a third episode of unilateral neck edema that developed overnight, this time spanning her left chest wall, axilla, and flank. It was associated with generalized stiffness, odynophagia, globus pharyngis, and moderate pain on the left side of her neck. In the emergency department, she was found to be afebrile with a normal white cell count. A CT scan was performed, followed by MRI, which were consistent with inflammatory changes in the left side of her neck and left superior mediastinum, felt to be secondary to cellulitis; there was no evidence of focal fluid collection. Otolaryngology was consulted, and an endoscopy revealed no airway edema. Nonetheless, the patient was admitted to the intensive care unit for close monitoring, in the event that the swelling worsened to compromise the airway. She was administered empiric vancomycin and piperacillin/tazobactam, as well as intravenous steroids. The patient was discharged to home 72 h after admission, without a definitive diagnosis. The symptoms improved again within several days. Few weeks later, the most severe episode of neck swelling occurred, resulting in admission to the intensive care unit due to suspected airway compromise. The patient again received steroids and antibiotics, but the latter was discontinued after infectious etiology was deemed unlikely. Given the importance of ruling out a malignant process, it was recommended that the patient undergo an open surgical lymph node biopsy. In addition to sampling nodes from the patient’s left neck, the surgeons from the department of otolaryngology also performed an exploratory neck dissection to attempt to identify the source of the swelling. The procedure revealed a large amount of lymphatic fluid infiltrating the soft tissue, but no confirmation of a specific source. In addition, general fullness of the lymphatic vessels was noted. Surgical pathology of a

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left supraclavicular lymph node revealed no evidence of vascular malformation, infection or neoplastic process. The patient was subsequently referred to interventional radiology for lymphangiography and possible thoracic duct embolization. Of note, the patient had no history of prior catheter placement or neck surgery that could suggest a postprocedural lymphocele. Upon presentation to interventional radiology clinic, the patient reported feeling well overall, but endorsed ongoing ‘‘fullness’’ in her neck. In addition, she reported intermittent shortness of breath, left lower back and neck pain, as well as a ‘‘pressure-like’’ headache exclusively during episodes of swelling. Physical examination was only notable for a unilateral, left-sided cervical soft-tissue edema with scattered, nondistinct areas of tenderness. There was no erythema, tracheal deviation, or cervical or supraclavicular lymphadenopathy. The patient underwent an ultrasound-guided left neck aspiration, in which one tube of hazy fluid was aspirated. The fluid analysis demonstrated 35,985 WBC/lL with a 98 % lymphocytic predominance, 15,378 RBC/lL, 26 mg/dL triglycerides, and absent chylomicrons. These laboratory findings confirmed lymphatic, nonchylous origin of the fluid. Given the fullness of the lymphatic vasculature visualized in the surgical biopsy and the lymphatic origin of the aspirated fluid, it was hypothesized that a lymphatic lesion, such as a lymphatic malformation or lymphangiectasis could be the cause of the patient’s symptoms. To further define the lymphatic anatomy, the patient underwent MR lymphangiography (heavy weighted T2 sequence) [5] of

Fig. 1 Heavy T2-weighted image of the neck, demonstrating distal part of the thoracic duct (arrow) adjacent to the lymphatic varix (arrowhead)

A. K. Guzman et al.: Neck Lymphatic Varix Embolization

Fig. 3 Injection of the contrast through the microcatheter (white arrow) of the lymphatic varix (white arrow head). There is brisk flow of the contrast in the subclavian vein (black arrowhead)

Fig. 2 Intranodal lymphangiogram of the left axillary lymph nodes demonstrating filling of the lymphatic varix with contrast (black arrow) through feeding lymphatic vessel (arrowhead)

the neck and chest, which demonstrated a lymphatic varix in the left supraclavicular area adjacent to the distal part of the thoracic duct (Fig. 1). Given these findings, a left axillary nodal lymphangiogram was performed to better characterize the lymphatic varix. In the interventional radiology suite, the left axillary lymph nodes were accessed using a 25-G needle under ultrasound guidance, and *1 mL of Lipiodol (Guerbert, Villepinte, France) was injected using the technique described by Nadolski and Itkin [6]. Axillary nodal lymphangiography demonstrated extremely dilated left-sided ducts below the clavicle, leading to an exceedingly dilated supraclavicular lymphatic vessel (Fig. 2). It was assumed that this structure, due to inherent weakness in the vessel wall, was the cause of the swelling of the vessel and lymphatic leakage into the soft tissue. Embolization of the lymphatic varix, therefore, would divert lymphatic flow towards collaterals, preventing recurrence of the patient’s symptoms. Notably, this structure was in the center of the patient’s site of recurrent swelling. Two days after the lymphangiogram, the patient reported another episode of neck swelling. An axillary nodal lymphangiogram was repeated, again showing extremely dilated left-sided ducts below the clavicle, emptying into an exceptionally dilated transclavicular lymphatic vessel. The dilated lymphatic channel that led to the lymphatic varix was accessed using 21-G Chiba needle, a 0.018 Gold tip glidewire, followed by 3-French catheter. Contrast injection through the catheter demonstrated filling of the large supraclavicular lymphatic varix (Fig. 3). The contrast

Fig. 4 Postembolization image of the lymphatic varix packed with microcoils (white arrow). The coils were delivered through microcatheter (white arrowhead)

material then emptied into the left subclavian vein. The lymphatic varix was then embolized with multiple detachable Presidio microcoils (Codman & Shurtleff, Inc., Raynham, MA). Six coils were deployed; the coil sizes were: 12-mm 9 30-cm, 12-mm 9 40-cm, 13-mm 9 43cm, 11-mm 9 37-cm, 10-mm 9 16-cm, and 6-mm 9 15cm. In order to facilitate embolization of the varix, autologous blood was then sterilely injected through the catheter. Postembolization control injection was performed, demonstrating compete obliteration of the lymphatic varix, with the exception of its most inferior component where emptying into the subclavian vein was demonstrated

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(Fig. 4). The procedure was completed without complication, and the patient was discharged that afternoon. Shortly after the procedure, the patient developed mild left neck swelling, which rapidly resolved within 48 h without medical intervention. On 10-week follow-up, the patient reported no recurrences of neck swelling after the embolization procedure. On examination, she appeared well, with no swelling or tenderness. She had a full range of motion of her neck, and the coils were not visible or palpable through her skin. The patient reported no complications attributable to the lymphangiogram or embolization.

Discussion Differential diagnosis for neck swelling in an adult patient is broad, encompassing inflammatory, neoplastic, congenital, and posttraumatic etiologies. One of the rare causes of the left neck swelling is cystic dilation of the distal portion of the thoracic duct [1, 7]. Spontaneous rupture of the distal part of the thoracic duct at the neck is extremely rare with one recent report in the literature [8]. Conventional lymphangiography and cross sectional imaging are used to diagnose this condition [1]. The right upper extremity lymphatic ducts usually join the thoracic duct near its junction with subclavian vein. In certain cases, however, these lymphatic ducts drain separately into venous system in proximity to the distal part of the thoracic duct [9]. Due to the nonchylous features of the fluid, we suspected that the lymphatic varix that we observed on MRI was at the confluence of the cervical and upper extremity lymphatic trunks; this was confirmed using axillary lymphangiography. It was believed that inherent vessel wall weakness in this structure was the cause of its extreme dilation and subsequent lymphatic leakage into the soft tissue. Obliteration or removal of this lymphatic varix, therefore, would divert lymphatic flow towards collaterals and prevent recurrence of the patient’s symptoms. Direct access with the needle of the lymphatic varix was especially challenging due to its location behind the clavicle. Thus, we approached it by cannulating a feeding lymphatic vessel under fluoroscopic guidance. This technique was similar to fluoroscopy-guided thoracic duct cannulation, which is well-described in the literature [4]. There was significant lymphatic flow through the lymphatic varix and almost immediate evacuation of the contrast into the subclavian vein. Therefore, we decided that its

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obliteration with fluid agents would not be prudent, as it could result in potential nontarget embolization. For that reason, we embolized the lymphatic varix using endovascular embolization coils, in a manner similar to embolization of the brain aneurysms. Finally, the addition of sterile autologous blood likely facilitated clotting and caused a local inflammatory response, which may have aided in fibrosis.

Conclusions We believe that MR lymphangiography is an excellent modality to diagnose cystic lymphatic varices. Axillary intranodal lymphangiography followed by coil embolization and autologous blood patch is a safe and effective method of characterizing and treating this lesion and preventing recurrence of symptoms. Conflict of interest Anthony K Guzman, Brian A Pukenas, Yan Yan, Jason G Newman, and Maxim Itkin have no conflict of interest.

References 1. Kolbenstvedt A, Aanesen J (1986) Cystic dilatation of the thoracic duct presenting as a supraclavicular mass. Br J Radiol 59(708): 1228–1229 2. Barlow D, Gracey L (1965) Cystic dilatation of the thoracic duct. Br J Clin Pract 19:101–102 3. Gilony D, Schwartz M, Shpitzer T, Feinmesser R, Kornreich L, Raveh E (2012) Treatment of lymphatic malformations: a more conservative approach. J Pediatr Surg 47(10):1837–1842 4. Itkin M, Kucharczuk JC, Kwak A, Trerotola SO, Kaiser LR (2010) Nonoperative thoracic duct embolization for traumatic thoracic duct leak: experience in 109 patients. J Thorac Cardiovasc Surg 139(3):584–589; discussion 589–590 5. Okuda I, Udagawa H, Takahashi J, Yamase H, Kohno T, Nakajima Y (2009) Magnetic resonance-thoracic ductography: imaging aid for thoracic surgery and thoracic duct depiction based on embryological considerations. Gen Thorac Cardiovasc Surg 57(12):640–646 6. Nadolski GJ, Itkin M (2012) Feasibility of ultrasound-guided intranodal lymphangiogram for thoracic duct embolization. J Vasc Interv Radiol 23(5):613–616 7. Maruyama M, Kobayashi S, Kasuga Y, Fujimori M, Yokoyama S, Shingu K et al (1997) Thoracic duct cyst in supraclavicular region. Ulster Med J 66(2):140–143 8. Abi-Fadel F, Khan M, Apergis G, Fahmy S (2012) A 40-year-old female with an acute onset left lateral neck and supraclavicular swelling. Heart Lung Circ 21(12):815–816 9. Kinnaert P (1973) Anatomical variations of the cervical portion of the thoracic duct in man. J Anat 115(Pt 1):45–52

Embolization of the neck lymphatic varix, causing periodic neck swelling.

We report a case of a 44-year-old female patient, presented to us after years of recurrent intermittent episodes of unilateral left neck swelling. An ...
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