Treatment of Axillary Web Syndrome: A case report using manual techniques.

Jane Kepics MS PT CLT-LANA
University of Scranton
Final Case Report
Evidence Based Medicine
December 10, 2007

Jane Kepics MS PT CLT-LANA is a 1987 graduate of the Dr. Vodder School. She is a lymphedema
and cancer rehab specialist at Phoenixville Hospital in Phoenixville, PA.
Email her at Jane_Kepics@chs.net

Treatment of Axillary Web Syndrome: A case report using manual techniques.

Abstract:
Background and Purpose: Axillary web syndrome (AWS) is a painful and functionally limiting complication of breast cancer treatment most likely due to lymphatic thrombosis after lymph node resection. Patients describe cord-like structures in the axilla extending into the distal arm causing pain, limited shoulder ROM and functional compromise.  Medical literature claims that AWS is self limiting, resolving in 2-3 months regardless of treatment. Physical therapists challenge that claim, suggesting that when untreated, pain and disability can last for years. Early rehabilitation intervention is advocated to reduce pain, normalize ROM and return to premorbid function. Until recently, information regarding the signs and symptoms of AWS was limited and treatment documentation was virtually nonexistent. The purpose of this case report is to describe the clinical problems associated with AWS, to discuss the need for early rehabilitation intervention and to describe the use of manual treatment techniques to treat a patient with AWS after axillary lymph node surgery. 

Case Description: A physical therapy (PT) plan of care was developed for a 51-year-old female 4 weeks after a benign lymph node biopsy. Deficits included severe pain, axillary cording, limited shoulder mobility, and an inability to work. 

Discussion: This case provides evidence that early PT intervention may lead to a quicker resolution of symptoms and prevent unnecessary disability following axillary surgery.

Background and Purpose:
Axillary web syndrome (AWS) is a relatively new term for an old problem that may be seen in people who are treated for breast cancer. Identified for many years as lymphatic or axillary cording,1,2 AWS is described as a visible web of axillary skin overlying palpable cords of tissue that are made taut and painful by shoulder abduction.3

The etiology of AWS is most likely due to interruption of the axillary lymphatics during either sentinel lymph node biopsy (SLNB) or axillary lymph node dissection (ALND), two procedures that may be performed for the treatment of breast cancer.3-6 This phenomenon has also been observed in a patient following axillary staging for melanoma7 as well as a patient with breast cancer and axillary metastases who developed AWS without ever having surgery.3

Moskovitz et al coined the term Axillary Web Syndrome in 2001.3 Some practitioners suggest that “lymphatic cording” is the most accurate term for the syndrome since the condition is not limited to the axilla and some patients present with no apparent axillary involvement.1

The incidence of AWS is varied in the literature. Leidenius et al evaluated 85 patients pre-and postoperatively looking at shoulder range of motion (ROM) and the presence of AWS after SLNB or ALND.4  45% of  patients who underwent SLNB had limited shoulder movement and 20% of those demonstrated a visible axillary web. Arm motion was restricted in 86% of patients who had ALND and 72% had AWS. Moskovitz3 observed only a 6% incidence in AWS and Wyrick1 identified a 19% incidence. The variation in incidence may be related to the fact that the former study was done prospectively, while the latter 2 studies were performed retrospectively. In addition, varying diagnostic criteria may have contributed to this discrepancy.6

In a retrospective study of 44 patients with AWS, Moskovitz et al noted that the web was always present in the axilla but could extend into the medial ipsilateral arm, frequently down to the antecubital space and occasionally to the base of the thumb.4 Wyrick et al did a retrospective study of 31 patients with AWS.1 Cording in the axilla alone was seen in 17% of the patients. 21% of patients had no axillary component and presented with more distal cords. 
 
Tissue samplings taken from the webs in 4 patients with AWS proved to be sclerosed veins and lymphatic vessels with fibrosis.3 The principle surgeon in the research group speculated that the thrombosed lymphatics go through an inflammatory phase with thickening of the vessels and temporary shortening and tightening which later remits.8

Marcus et al described 5 cases of AWS also suggesting that the underlying pathology is lymphatic thrombosis.5 Disrupting the normal lymph drainage channels during ALND leads to lymph stasis. Often, this leads to lymphedema where the stasis gives rise to a protein rich exudate into the interstitium which can become fibrotic. The authors suggested that while the cording process is quite distinct from lymphedema, both seem to be a manifestation of the lymph stasis. They presented an interesting question that has yet to be answered. Why does lymph stasis, in some cases, lead to the development of lymphedema while other cases develop axillary cording?

Josenhans described a patient with a pronounced cord who developed a lump on the cord.2 Biopsy of the lump showed fibrotic lymph vessel surrounded by fat and granulation tissue. The lump apparently was a cystic enlargement of the lymph vessel. The surgeon postulated that when lymph nodes are removed, the adjacent lymph vessels, which are now non-functional, become fibrosed. They can attach to the axilla or chest via scar tissue. These hardened lymph vessels become apparent when the patient lifts her arm and stretches the tissue. Inflexibility in the lymph vessel can lead to significant pain and avoidance of movement. Adaptive postures can then lead to a loss of arm range of motion.

Josenhans also presented an excellent pictorial description of the variety found in axillary cords. It included single and double cords, axillary cords only vs. those extending beyond the elbow, soft tissue retraction in the breast scar and in the chest wall due to hardened lymph vessels, cording that develops after a single sentinel lymph node resection, and a cord that is present several years after surgery. 2

Treatment of AWS usually includes active and passive stretching, soft tissue and cord stretching techniques including myofascial release and scar releases.1,2,6,8 The use of manual techniques varies by patient acuity and presentation.  This author described a skin traction technique, which is found to be most beneficial.8 With the arm abducted to comfort level, 1 or 2 inch segments of the cord are stretched by the therapist’s thumb and index finger. This can be done along the length of the cord and onto the chest wall. Occasionally, the therapist can feel a pop or a snap in the cord where it actually breaks during the stretch.2,8  The patient usually feels no pain and gets an immediate increase in ROM. This is often felt with the thinner, piano-wire type cords that are more likely to be in the antecubital fossa and along the length of the cord rather than the thickened cords seen in the axilla. No harm seems to result from this breakage and patients are pleased with their newfound range of motion. In fact, this immediate improvement is why PT’s encourage early treatment.

Josenhans2 suggested using “hooking” manipulations in the axilla where the cord is anchored deep in the tissues progressing down the abducted arm. Her treatment is provided 2-3 times/ week for 30 minutes. Contraindications include: Reddening of the scars – avoid scar area; Radiation therapy: avoid manual techniques in the area being radiated until 2 weeks after radiation; and axillary metastases – avoid manual techniques in the axilla. Patients may also benefit from short periods of heat or cold prior to the manual techniques.8 Pain modalities must be used cautiously as patients may have sensory deficits after the axillary surgery. They are at risk for burns and the increased skin temperature potentially increases the risk of lymphedema.6 Manual lymph drainage may also be indicated to promote lymph flow and reduce lymphedema.  In this author’s experience, lightweight (15-20 mmHg) compression garments may provide mild pressure to the cords and lessen discomfort even in the absence of lymphedema.

Cheville and Tchou emphasize patient education regarding the syndrome and performance of a home exercise program 2x/day.6 Premedication with NSAIDs and opioids may be necessary to assist patients during treatment. Wyrick1 notes that in addition to AWS, patients may present with co-morbidities such as cancer fatigue, tissue tightness, lymphedema and muscle weakness consequent to their cancer treatment that need to be addressed as well. Therefore, identifying the single best treatment for cording is difficult.   She also discusses the fact that patients with cancer who are undergoing concurrent adjuvant therapy such as radiation or chemotherapy often have more PT appointment cancellations, which can increase the length of time in treatment. With so many factors impacting the patient, the treatment possibilities are numerous.

Moskovitz et al described AWS as self limiting, resolving in all cases within 2 to 3 months with or without any treatment including (undescribed) PT.3 Other medical researchers also cite this same retrospective study, lacking in formal pain or functional assessments, as universal fact without ever providing any additional evidence that it is true.4,7 Oncologic rehabilitation clinicians challenge this claim, as often, patients present months to years after primary breast cancer treatment with persistent symptoms of AWS.1,2,6,8  Without education regarding postoperative rehabilitation problems, patients tend to think that pain and functional loss are normal postoperative occurrences and over time, just learn to “live with them”.

The purpose of this case report is to describe the clinical problems associated with AWS, to discuss the need for early rehabilitation intervention and to describe the use of manual treatment techniques to treat a patient with AWS after axillary lymph node surgery. 

Case Description:
Subject/Case: Mrs. P is a 51-year-old female who had both a suspicious annual mammogram and a positive ultrasound for a left axillary mass. A biopsy of only one axillary lymph node showed marked non-specific reactive follicular hyperplasia, a benign and reversible enlargement of lymph tissue that most likely was idiopathic in origin.9 Given the benign nature of the lymph node, the patient was concerned when her incisional pain progressed to severe pain down the arm that was unremitting with over the counter pain medication. An ultrasound of the deep venous system of the arm proved negative for venous thrombus. Her surgeon told her to just massage the arm and it would go away, but the pain became too severe, limiting her ability to lift the arm away from her body. Most distressing was that she was unable to return to work and she had no sick time or medical benefits. She finally sought out a consult with an independent oncologist despite the fact that there was no cancer diagnosis. He referred her to PT with orders for evaluation and treatment of decreased shoulder ROM status post lymph node biopsy. 

Examination: Mrs. P was referred to PT 4 weeks after the biopsy. Her chief complaint was pain in the left axilla extending down the arm with overhead activities. She identified an acutely painful, fibrous cord from the axillary scar into her mid forearm with an inability to lift anything with the left hand or to reach overhead. Her goals were to eliminate her pain and to have to full functional use of the arm so she could return to work in a timely manner. 

 Mrs. P’s past medical history included GI bleeding, and gastro-esophageal reflux (GERD). There was no history of alcohol or substance abuse. Her past surgical history included a benign left breast biopsy 3 years prior to her recent surgery. Medications included prevacid for GERD and Tylenol for pain.

A review of Mrs. P’s social history revealed that she was single and she lived alone in a three-story house. She did clerical work full time prior to her biopsy, but had been unable to return to her job since surgery. This was a tremendous source of anxiety for her because she had no income during this time. Premorbidly, Mrs. P was independent in all ADL’s and ambulated independently without an assistive device. She often walked 2 miles a day, 3 times per week for exercise. She had not returned to this activity since her surgery.

Before the start of the initial evaluation, Mrs. P gave written consent to be evaluated, treated and photographed in PT.

Systems Review:
Cardiovascular/Pulmonary:
  Vital signs: resting blood pressure: 120/78; Heart rate: 72 bpm; respiratory rate 16/ minute. 

Integumentary:
The axillary incision was intact with no signs of drainage or wound dehiscence. There was a single thick axillary cord that was exquisitely painful to touch. See Figure 1. The cord extended as 3 piano wire like structures through the antecubital fossa before ending in the forearm. The cords retracted the arm and forearm skin when the patient attempted to abduct her arm with elbow extension. There was no pitting, heat, redness or signs of infection. Tissue texture was soft. There were no signs of lymphedema.

Cognitive: Mrs. P was alert and oriented times 3. She was very anxious whenever the therapist approached her left arm in anticipation of pain from movement. The patient was able to follow simple and complex commands. There were no learning barriers for PT to address.

Musculoskeletal: Mrs. P was right handed. She was 5’5” tall and weighed 150 pounds.
Gross ROM and strength testing of the right upper extremity (RUE) was within functional limits as tested through antigravity motions. She had an obvious loss of motion of the left upper extremity (LUE) as she held the limb protected close to her side. Her left scapula was protracted and depressed and there was tightness of the left pectoralis muscles.

Neuromuscular: Mrs. P reported numbness in the left axilla in the area of her scar. Gross movement patterns of the upper extremities were not well coordinated due to her protective posture.

Tests and Measures:
The choice and rationale behind the tests and measures performed were based on the patient’s overall presentation, findings from the gross systems review and experience of the PT.

ROM: Measurements for upper extremity (UE) ROM as described by Reese10 were obtained using a standard 360o goniometer. See Table 1. The use of a universal goniometer has been found reliable and valid for the measurement of ROM.11 The patient was unable to abduct the shoulder with the elbow extended due to pain and pulling on the cords.

Strength:
Manual muscle testing (MMT) in the form of break testing was performed for major UE muscle groups as described in Kendall.12 See Table 1. MMT is a reliable method to measure muscle strength if the therapist follows standardized testing procedures and if the patient is given clear instructions.13 No specific statistics regarding reliability or validity of MMT were found. Positions for testing shoulder motions had to be modified, because of axillary pain. Break tests were done in the patient’s available range of motion. The therapist gained the patient’s confidence by using a gentle touch and explaining the testing procedure thoroughly

Pain: A visual analog scale where 0/10 = no pain and 10/10 = severe pain as described by Bijur PE et al was used to measure pain.14 The VAS has proved reliable when accessing acute pain. Data was not documented for the validity of the VAS.14
Mrs. P rated pain in the LUE as 7/10 with active movement and 10/10 with passive movement of the shoulder, especially with abduction accompanied by elbow extension.

Limb Volume: Limb volume was measured for BUE using arm circumference measurements based on anatomical landmarks as described by Taylor R et al.15 Circumferential tape measurements were taken at each point using a thin, flexible plastic tape. Measurement sites included: the ulnar styloid,  the mid - forearm, the olecranon, the mid -  upper arm,  and a point 65% of the distance from the elbow (olecranon) to the shoulder tip (acromion). Total limb volume for the segment between the wrist and the 65% point was obtained by adding the volumes of the truncated cones between these points. The volume of a truncated cone is calculated as follows: 
                                                                    V=h(C12+C1C2+C22)/12π
where V is the volume of the segment, C1 and C2 are the circumferences at the ends of the segment, and h is the distance between them (segment length).16 Total limb volume for the segment between the wrist and the upper boundary was obtained by adding the volumes of the truncated cones between these points. Arm circumferential measurements in relation to anatomic landmarks are reliable and valid measurements of arm volumes (ICC≥ .98).16   The volume of the LUE was 2349 ml. The volume of the RUE was 2457 ml. The left, non-dominant arm was 108 ml or 5% smaller than the right arm. While there was no evidence of lymphedema, baseline volume measurements are important to track changes in volume that may occur as a result of treatment or at a later time.

Function: The Penn Shoulder Score (PSS) is a 100-point shoulder specific self-report questionnaire that measures pain, satisfaction with current level of function and functional ability.17 See Appendix I. The Penn Shoulder Score has proved to be reliable and valid with a test-retest ICC (2,1) of 0.94 (95% CI).18 Mrs. P scored 25/100 on the Penn Shoulder Score.

Evaluation:
Mrs. P was a fairly healthy 51-year-old female who presented with limited ROM of the left shoulder with axillary cording, pain, sensory impairment, and loss of function including an inability to work. It was hypothesized that these deficits were a direct result of AWS after axillary lymph node biopsy. The Guide to Physical Therapy Practice19 does not specifically mention axillary cording or AWS, nor is there a specific ICD- 9 code for the condition. Of the codes listed, AWS seems to most closely fall under category 457.9 - Unspecified noninfectious disorder of lymphatic channels. Since there was lymph node surgery the PT diagnosis appeared to fall under practice pattern 6H, Impaired Circulation and Anthropometric Dimensions Associated with Lymphatic System Disorders. Intervention under this practice pattern includes mobilization and manipulation of soft tissue but obviously, there was no information regarding prognosis for the condition.

Mrs. P would be seen 3 times per week for 2 weeks then 2 times per week for 2 weeks.

Anticipated goals and expected outcomes based on the Guide to PT Practice include: decreased pain, a reduction in soft tissue restriction, increased upper extremity ROM and strength, improved postural control and an increased ability to resume activities related to self care, home management and work.19 In addition, the patient should be independent in a home exercise program and be aware of strategies to decrease reoccurrence and prevent lymphedema.

     The proposed intervention includes:
     1. Patient education regarding the origin of axillary web syndrome as well as written instructions
         from the National Lymphedema Network regarding lymphedema precautions. 
          See Appendix II.
     2. Moist heat to the cord 8-10 minutes with extra padding and frequent visual inspection of the
         site to avoid burns due to sensory impairment.8
     3. Gentle passive or active assistive ROM of the arm, slowly bringing it into the painful range of
         abduction with elbow extension then backing off slightly. Instruct the patient to perform
         flexion and hyperextension of the wrist to provide maximum stretch and then release similar
         to nerve gliding techniques.8 Other exercises may include but are not limited to pendulum
         exercises, wall walking, a yoga child’s pose and corner stretch.
     4. Manual techniques including: the skin traction technique described earlier; myofascial
         release techniques such as the arm pull, and stretching of the pectorals, the intercostals,
         biceps and triceps and the diaphragm.21
     5. Manual lymph drainage if needed with a 15-20 mmHg compression sleeve and gauntlet to be
         worn during the day, should the patient develop lymphedema.
     6. Equipment such as reciprocal pulleys, exercise wands, Theraband® or light weights and
         UBE may also be used at the therapist’s discretion.

Discussion: The purpose of this case report was to describe the clinical problems associated with AWS, to discuss the need for early rehabilitation intervention and to describe the use of manual treatment techniques to treat a patient with AWS after axillary lymph node surgery.   Since the description of AWS is fairly new in medical literature3,4 and recommends no immediate treatment, many physicians are not aware of long term rehabilitation problems and do not readily refer patients to PT for the condition.  Treatment may be delayed for weeks, months, and even years. The PT literature has thus far been descriptive in nature8 but recent articles have tried to track trends such as incidence, presenting symptoms and treatment options.1,2,6

The patient chosen for this report presented with the typical signs and symptoms seen in AWS.  Her case was unusual in that she had a benign pathology report and should have had an uneventful postoperative course. Her plan of care was developed based on the rationale, guidelines and recommendations of current, evidence based research.1,2,6 The experienced therapist has seen quick reduction in symptoms with a gentle touch and slow passive ROM with manual pressure on the cord. It was hypothesized that with the implementation of PT focusing on patient education, pain modalities , manual techniques to stretch and possibly break the cords, manual lymph drainage with limb compression as needed, ROM and strengthening exercises  and a home exercise program,  the patient would achieve full shoulder ROM with minimal pain or lymphedema so that she could return to work.

The clinical problems seen in AWS are not only physical. Pain and limited ROM in the wake of a cancer scare take an emotional toll and can have an economic impact if the patient is unable to work. The patient perceives that the doctor, whose main goal is to cure the cancer, takes her complaints of pain and immobility too lightly. Anxiety ridden patients are finally referred to PT when the disability becomes quite severe or if the loss of ROM prevents them from achieving the overhead arm position necessary to start radiation therapy. PT literature suggests that resolution of symptoms, defined as elimination of pain, full ROM and full function, can be achieved in as few as 6 treatment sessions.2 More importantly, large gains in ROM can be achieved in a single therapy session.2,8 When patients are educated about the condition and slowly guided through the ROM, this therapist’s hunch is that they feel a sense of relief knowing that someone is listening to them, taking their complaints seriously, and actually giving them permission to move their arm . Prior to this, they did not know if they were hurting themselves by moving, therefore, they did not move. Cheville and Tchou note that prolonged shoulder immobility leading to altered movement patterns, and biomechanical and neuromuscular recruitment dysfunction, is the greatest threat posed by AWS.6

PT intervention for AWS has evolved over time, perhaps as more therapists began to specialize in the treatment of people with breast cancer and lymphedema. This author has often seen patients referred for lymphedema who also present with cords that pull so tightly they deform the tissue in the upper arm, making it appear edematous.8 Evaluation of some lymphedema patients also yields findings of functional adaptation to longstanding pain and ROM loss, perhaps a subacute version of the syndrome. In both cases, once treated with manual techniques, the cords and swelling may resolve.2,8  At times, the cord may still be visible, but the ROM is full and the pain eliminated. 

Cheville and Tchou note that the selection of treatment techniques for AWS evolves during treatment and is based on the individual patient’s progress or lack thereof.6 Rehabilitation interventions are multifactorial and a plan of care involves multiple modalities that change as the patient progresses through treatment. For this reason, double blind randomized controlled trials are difficult to perform in this patient arena.
 
Further research can better define the incidence and natural history of AWS, predisposing patient factors, if any, and surgical considerations that may lead to the development of AWS. Other questions include: 
     • What manual techniques work best to treat the cords?
     • What percentage of patients finds relief of symptoms with manual treatment?
     • Does manual treatment including breaking the cords have a lasting effect on patient mobility?
     • Are there some patients who cannot be helped by manual treatment?

References
1.  Wyrick SL, Waltke LJ, Ng AV. Physical therapy may promote resolution of lymphatic coding (sic)
     in breast cancer survivors. Rehabilitation Oncology. 2006; 24 (1); 29-34.
2.  Josenhans E. Physiotherapeutic treatment for axillary cord formation following breast cancer
     surgery. Pt_Zeitschrift für Physiotherapeuten. 2007; 59 (9): 868 – 878. English translation
     available at http://www.praxisjosenhans.de/Arbeit_Wissenschaftspreis.html
     Use the password: Berlin 2007.  Accessed November 24, 2007.
3.  Moskovitz AH, Anderson BO, Yeung RS, Byrd DR et al. Axillary web syndrome after axillary
     dissection. Am J Surg. 2001; 181: 434-439.
4.  Leidenius M, Leppanen E, Leppanen E, Krogerus L, von Smitten K. Motion restriction and
     axillary web syndrome after sentinel node biopsy and axillary clearance in breast cancer.
     Am J Surg. 2003; 185 (2): 127- 130.
5.  Marcus RT, Pawade J, Veli EJ. Painful lymphatic occlusion following axillary lymph node 
     surgery. British J. Surg. 1990; 77(6): 683.
6.  Cheville A, Tchou J. Barriers to rehabilitation following surgery for primary breast cancer.
     J Surg Onc. 2007; 95: 409- 418.
7.  Reedijk M, Boerner S et al. A case of axillary web syndrome with subcutaneous nodules
     following axillary surgery. The Breast. 2006; 15: 411-413.
8.  Kepics J. Physical therapy treatment of axillary web syndrome. Rehabilitation Oncology.
     2004; 22 (1): 21-22. 
9.  Innes DJ. University of Virginia Health System. Benign White Cell Disorders: Reactive
     Lymphadenopathy. Available at: 
     http://www.med-ed.virginia.edu/courses/path/innes/wcd/reactive.cfm.  
     Accessed December 2, 2007.
10. Reese NB, Bandy WD. Joint Range of Motion and Muscle Length Testing. Philadelphia: 
      Saunders Publishing.2002.
11. Mayerson NH, Milano RA. Goniometric Measurement Reliability in Physical Medicine. Arch Phys
      Med and Rehabil. Feb 1984; 65(2): 92-94.
12. Kendall FP, McCreary EK, Provance PG, Rodgers MM, Romani WA. Muscles: Testing and
      Function with Posture and Pain, 5th edition. Baltimore, MD: Lippincott Williams & Wilkins, 2005.
13. Palmer ML, Epler ME.  Fundamentals of Musculoskeletal Assessment Techniques 2nd Edition.
       Philadelphia: Lippincott; 1998. 
14. Bijur PE, Silver W, Gallagher EJ. Reliability of the Visual Analog Scale for Measurement of Acute
       Pain. Acad Emerg Med 2001; 8(12): 1153-1157.
15. Taylor R, Jayasinghe UW, Koelmeyer L, Ung O and Boyages J. Reliability and Validity of Arm
       Volume Measurements for Assessment of Lymphedema. Physical Therapy. February 2006;
       86(2): 205-214.
16. Casley-Smith JR, Casley-Smith JR. Modern treatment of lymphoedema, I: complex physical
       therapy—the first 200 Australian limbs. Australas J Dermatol 1992; 33:61–68.
17. Leggin BG, Shaffer MA, Neuman RM, Williams GR, Iannotti JP. Relationship of the Penn
      Shoulder Score with Measures of Range of Motion and Strength in Patients with Shoulder
      Disorders: A Preliminary Report. Univ PA Ortho J. 2003;16: 39- 44.
18. Leggin BG, Michener LA, Shaffer MA, Iannotti JP, Williams GR. The Penn Shoulder score:
       reliability and validity. J Orthop Sports Phys Ther. 2006; 36:138-151.
19. American Physical Therapy Association. The Guide to Physical Therapy Practice, second
       edition. Alexandria, VA: The American Physical Therapy Association; 2001.
20. Manheim C. The Myofascial Release Manual. 3rd edition. Thorofare, NJ: Slack, Inc; 2001.

                                                              Figure 1
                                                                  Single Axillary Cord

Axillary Cord

 Table 1
Upper Extremity ROM and Muscle Strength

Upper Extremity 

Appendix 1


Appendix 1



Appendix I

Penn Shoulder Score

Shoulder Appendix 1



Appendix II

Appendix II
Part II NLN