SMASAC Short Life Working Group on Lymphoedema - Lymphoedema Care in Scotland, Achieving Equity and Quality

Appendix 3: Case Histories

Case history 1

The patient is a 55 year old woman, obese and diabetic, referred by her General Practitioner (GP) because of persistent leg swelling failing to respond to diuretics. The GP has not undertaken any investigations other than routine urea and electrolytes. The patient was referred with a diagnosis of resistant congestive cardiac failure.

When seen at the clinic the patient was noted to be very obese with a BMI of 41. She is very overweight and is of very short stature.

Examination revealed that there was no rise in the jugular venous pulse, that there was no hepatomegaly, her lung bases were clear but she had significant oedema of both ankles. This oedema was pitting.

Further investigation revealed normal renal function and normal serum proteins. An echocardiogram was performed which again was completely normal with normal right and left ventricular function and dimensions with no evidence of pulmonary hypertension.

Clinical Diagnosis: Dependent oedema, at risk of lymphatic overload development of lymphoedema.

Gold Standard Recommendations: Compression therapy and weight reduction, plus education on her risk of lymphoedema and preventative strategies of activity, skin care, breathing exercises and leg elevation when seated. Ongoing management in primary care.

Management: The patient was advised to wear compression stockings. She was advised also to continue with low dose diuretic. She was referred to the dietician for dietary advice to lose weight.

The patient was reviewed in the clinic six weeks later with improvement in the leg oedema due to the use of compression stockings. Her weight, however, had not significantly changed. She was discharged from the clinic with advice to be followed up by the dieticians on a regular basis and for her GP to consider the use of weight reducing adjuncts such as appropriate drug therapy.

Case history 2

The patient, a 75 year old man, was referred by his GP to the outpatient clinic because of chronic leg swelling. The GP described that his legs were inflamed and occasionally weeping. These had not improved despite 80 mg of furosemide. The GP noted a past history of congestive cardiac failure and was concerned that the patient's leg oedema had not improved despite an enhanced diuretic dosage.

When seen at the clinic on examination the lower limbs were very heavy and oedematous with excoriation, scaling skin and redness over both shins. In addition the patient had evidence of right heart failure with raised JVP and some hepatomegaly.

Other examination was unremarkable.

Investigations included an echocardiogram which showed impaired right and left heart function. There was a moderate degree of pulmonary hypertension. Routine biochemical investigations showed a mild degree of renal impairment. Liver function tests were mildly abnormal in keeping with hepatic congestion but the serum proteins were normal.

Clinical diagnosis: chronic congestive cardiac failure (CCF). His legs, whilst showing some degree of pitting oedema, probably evidenced some development of chronic lymphoedema consequent to severe heart failure in the long term.

Management: As the patient had failed to respond to enhanced oral diuretic therapy he was offered admission for intensive intravenous diuretics. Following his admission with intensive diuretic therapy the leg swelling subsided. The patient was also treated with intravenous antibiotics with flucloxacillin as there was an element of cellulitis.

There was a mild degree of renal impairment which worsened with the intensive diuretic therapy but this again subsided on withdrawing the intravenous therapy and maintaining the patient on an enhanced dose of diuretic compared with prior to his admission.

The leg swelling persisted to some small degree suggesting a mild degree of chronic lymphoedema.

To reduce this patient's risk of worsening lymphoedema, the following care and interventions were required:

• Measurement for an appropriate compression garment and assessment of the patient's ability to apply a garment or availability of carers to assist with this
• Education on use and care of the garment
• Remeasurement and replacement of garment 4-6-monthly
• Verbal and written information on use and care of garment, skin care, physical activity, breathing exercises, diet, importance of prevention of infection, including tinea pedis/trauma and early treatment with antibiotics if signs of cellulitis develop (see BLS Consensus on Cellulitis).

Case history 3

The patient, a 54 year old woman, was referred by her GP to the outpatient clinic because of swelling of her left arm which had gradually increased over a period of 6-8 weeks. There was no associated discomfort.

There was no past history of note and in particular she had no past history of breast carcinoma.

On examination in the clinic she was noted to have a warm hand which was slightly dusky in colour. The pulses in the arm were normal and in particular the radial pulse was normal with good capillary refill.

The arm was swollen throughout its length and pitted only a little.

Other examination was unremarkable. In particular there was nothing to suggest underlying malignancy.

Clinical diagnosis: lymphoedema with a differential of possible axillary vein thrombosis.

Investigations with Doppler ultrasound and CT angiography demonstrated that the arterial supply to the arm was normal but that there was occlusion of the axillary vein.

The cause of this was uncertain. The patient had further investigations which revealed that she had a thrombophilia, i.e. an enhanced clotting state due to an inherited familial disorder.

Management: She was treated with anticoagulation initially with heparin and switched to warfarin. The arm swelling failed to improve with anticoagulation and the axillary vein remained occluded. She thus has secondary lymphoedema consequent to the axillary vein thrombosis and was subsequently referred to a lymphoedema specialist.

Case history 4

The patient, a 35 year old man, was referred by his GP to the outpatient department because of chronic leg swelling. The GP also noted that the patient appeared slightly jaundiced.

When seen at the clinic a history of leg swelling developing over a three to four month period was noted as well as some abdominal swelling with the patient describing that his trousers had become tight at the waistband.

Physical examination revealed that the patient was mildly jaundiced, that there was significant hepatomegaly, with a small degree of the splenic enlargement and that there was significant ascites.

The lower limbs were swollen and the swelling was mildly pitting.

A social history was taken and it was discovered that the patient drank excess alcohol in the region of 60 units per week, primarily cider and fortified wine.

Abdominal ultrasound revealed a degree of hepatomegaly and significant ascites.

A Doppler ultrasound of the legs was performed and this was normal with no evidence of deep venous thrombosis.

Routine biochemistry revealed a very low albumin level and abnormal liver function tests with a raised gamma GT. The patient was also mildly anaemic with a raised mean corpuscular volume (MCV). These biochemical parameters were in keeping with alcoholic liver disease.

Diagnosis: Advanced alcoholic liver disease

Management: The patient's management was palliative. His ascites improved with the use of diuretics with a combination of loop diuretic and spironolactone.

The leg swelling persisted due to persistent low albumin level. Skin care and palliative lymphoedema bandaging has a role in maintaining mobility, preventing and managing lymphorrhoea and maintaining comfort and mobility as long as possible.

The patient's progress was poor in that he went on to develop progressive portal hypertension and had recurrent admissions with worsening ascites and peripheral oedema and eventually succumbed three years later from hepatic failure as he had continued to consume very large quantities of alcohol despite advice to the contrary.

Case history 5

A 62-year-old man, known to have metastatic carcinoma of the prostate with bone metastases and enlarged abdominal lymph nodes, required a left ureteric stent. He had received palliative radiotherapy to the pelvis (10 fractions). He presented to his GP with leg and genital oedema and was referred on to a lymphoedema specialist with lymphoedema secondary to metastatic cancer.

Specialist Assessment:
Skin: Intact
Subcutaneous Tissues: Stemmer's Negative
Site: Swelling from digits to trunk
Shape: No distortion evident on measurement
Size: 26% excess limb volume

Diagnosis: Moderate lymphoedema secondary to metastatic cancer, complicated by extension to trunk and digits.

Gold Standard Recommendations: Intensive treatment, including lymphoedema compression bandaging and education and support for on-going self management.

Management:

• Discuss diagnosis, treatment options, implications and expected outcomes with patient
• Assess patient's expectations, goals, level of understanding and his own or his carer's ability to manage on-going treatment
• Class II RAL standard compression tights
• Whittaker Pouch for scrotal oedema
• Patient taught penile bandaging
• ETO custom-made compression shorts
• Kinesiotape to trunk to facilitate drainage of truncal swelling
• Regular monitoring and renewal of garments until able to self-manage with bilateral Farrow Wrap, skin care, movement and breathing exercises

Case history 6

A 63 year old woman with leg swelling was referred to the vascular clinic. A lymphogram demonstrated abnormal lymphatics in the calf, preventing knee contrast reaching the thigh. She was referred to a lymphoedema specialist with primary lymphoedema.

Specialist Assessment:
Skin: Very dry but intact
Subcutaneous Tissues: Significant non-pitting fibrosis below knee, severe around ankle; Stemmer's Positive
Site: Swelling from digits to root of limb but not involving trunk
Shape: Distortion present on measurement
Size: 63% excess limb volume

Diagnosis: Severe primary lymphoedema complicated by fibrosis, distortion and digit swelling

Gold Standard Recommendations: Intensive treatment, including lymphoedema compression bandaging, MLD, exercise and skin care with education and support for on-going self management.

Management: 3 episodes of intensive treatment of 3 weeks each. Maintaining reduction between treatments was difficult, with excess volume fluctuating between 25 - 35%. Was referred for liposuction, which reduced excess volume to 9.9%, decreasing to -4%. Patient currently wears two thigh length stockings - a class I under a class IV RAL standard - to maintain reduction alongside standard maintenance self-care of exercise, skin care and simple lymphatic drainage/breathing exercise. She attends annual review.

Case history 7

A 58 year old woman developed right arm swelling. She had had a renal transplant and brachio-cephalic arterio-venous fistula which had occluded. Vascular investigations identified a narrowing of the subclavian vein. Subcutaneous tissue changes indicated a lymphatic problem and referral was made to a lymphoedema specialist. The patient had also developed a malignant melanoma of the left lower leg, which had been removed. She was under the care of the skin cancer clinic.

The long-term management of lymphoedema focused on limiting further deterioration of swelling, enhancing limb function and gaining long-term control of the condition. Support, education and encouragement are key to helping patients adjust to living with a long-term condition and maximising their ability to self-manage and achieve a sense of control.