Quality of life after surgical reduction for severe primary lymphoedema of the limbs and genitalia.

BACKGROUND: The aim was to assess the quality of life (QoL) of patients who had surgery for primary lymphoedema. METHODS: A QoL questionnaire was administered to patients who had surgery between 1981 and 2003 (retrospective group) and between 2003 and 2006 (prospective group). RESULTS: The response rate was 70.3 per cent (109 of 155 patients): 88 patients had limb reduction (78, retrospective; ten, prospective) and 21 had genital reduction (13, retrospective; eight, prospective). Forty-nine patients (63 per cent) who had limb reduction studied retrospectively reported satisfaction with the procedure and most of these would opt for surgery again. In the prospectively studied group, nine of ten patients reported improved limbs, and seven would opt for surgery again. Nineteen of 21 patients who had genital reduction would choose to have surgery again if needed (11 of the retrospectively assessed group and all of the prospective group). Patients' perception that surgery was worthwhile was greater in both of the prospectively assessed groups (P = 0.013). CONCLUSION: Surgery for severe lymphoedema improved QoL at early assessment. This, however, may not be sustained. Genital reduction appeared to provide greater benefit than limb reduction.

Hereditary palmoplantar keratoderma associated with primary (congenital) lymphedema.

The palmoplantar keratodermas are a heterogenous group of hereditary disorders of keratinization. They are characterized by epidermal thickening and a yellow waxy appearance of the palms and soles. Genetic studies have linked various forms of palmoplantar keratoderma to markers on chromosomes one, twelve, and seventeen, and several genes have been identified. Primary lymphedema is occasionally present at birth (congenital lymphedema or Milroy's disease), but more commonly develops at puberty (lymphedema praecox). Genetic studies have linked various autosomal dominant forms of primary lymphedema (Milroy's disease and lymphedema distichiasis), to genes on chromosomes five and sixteen respectively. We report a case of palmoplantar keratoderma in a child with congenital lymphedema. To our knowledge, this has not been previously described and may represent a new phenotype for future genetic study.

Giant mature cyst formation following mesh repair of hernias: an underreported complication?

BACKGROUND: Numerous complications have been described following the implantation of synthetic meshes during hernia repair; one of the rarest, with only three reported cases, is giant mature fibrous cyst formation. Our clinical experience with this complication has led us to believe that it may be more common than previously thought. METHODS: Surgical operation notes and pathology archives were reviewed for the period January 1998-January 2002 to determine the prevalence of mature cyst formation following mesh repair of hernias. RESULTS: Out of 1100 hernia repair operations involving the use of synthetic meshes in our institution during the period of study, five developed histologically confirmed mature fibrous cysts giving a prevalence of 0.45% for this complication. CONCLUSION: The formation of a giant mature cyst following mesh repair of hernias is an underreported complication.

Is lymphatic endoglin expression a risk marker for breast cancer metastasis? Results of a pilot study.

BACKGROUND: Studies have identified endoglin as a biological marker that is overexpressed on the microvessels of certain solid cancers (breast, colorectal cancer, and head and neck squamous cell cancers). There is, at present, no immunohistochemical marker that can discriminate between lymph node-negative and or lymph node-positive breast cancer tissue. METHODS: The expression of endoglin was quantified by immunohistochemistry and assessment of microvessel density in 53 surgical specimens. These were comprised of breast tumor tissue that had not spread to the regional lymph nodes (lymph node-negative breast tumor tissue: 20 specimens), breast tumor tissue had spread to regional lymph nodes (lymph node-positive breast tumor tissue: 21 specimens), and normal breast tissue as a control (12 specimens). RESULTS: Significant difference was observed between the expression of endoglin on microvessels of lymph node-negative and lymph node-positive breast cancer tissue (p<0.05). This significant difference was shown to be due to endoglin expression on lymphatic vessels (p<0.02), rather than on blood vessels (p>0.05). CONCLUSIONS: These findings are the first to suggest that endoglin expression on breast tumor lymphatic vessels may have diagnostic potential as a discriminator between lymph node-negative and lymph node-positive breast cancer. Further studies would be required to confirm this.

Internal carotid artery occlusion: its natural history including recanalization and subsequent neurological events.

OBJECTIVE: To determine the incidence of recanalization of the occluded internal carotid artery (ICA) and establish its natural history. METHODS: Patients with duplex-confirmed ICA occlusions were identified, and a subgroup offered repeat scanning. The antemortem condition and cause of death of patients who died were recorded. RESULTS: Of 153 patients identified, 77 underwent follow-up at a median of 35 months (interquartile range [IQR]: 14-61).In all, 8 (10.3%) demonstrated recanalization at a median of 53 months (IQR: 35-114). Of 8, 7 underwent carotid endarterectomy with histopathological confirmation of recanalization. Of the 153 patients, 45 (29%) had further neurological events, and 38 (25%) were within the territory of the occluded ICA. In all, 76 patients died, and of the 53 with a confirmed cause of death, 12 (23%) were attributed to a cerebrovascular accident corresponding to the territory of the occluded artery. CONCLUSION: Recanalization of ICA occlusion is common and leads to significant neurological events. Duplex ultrasound follow-up appears mandatory.