12th State of Georgia Lymphedema Awareness Program

Ophthalmological findings in microcephaly-lymphoedema-chorioretinal dysplasia syndrome

Ophthalmological findings in microcephaly-lymphoedema-chorioretinal dysplasia syndrome

Klin Monatsbl Augenheilkd. 2009 Apr

Hatt Brupbacher SC, Job O, Senn P, Dedes W.

Augenklinik, Luzerner Kantonsspital, Luzern, Schweiz. salome.hatt@triemli.stzh.ch

BACKGROUND: Microcephaly-lymphoedema-chorioretinal dysplasia (MLCRD) is a rare syndrome characterized by microcephaly, chorioretinal dysplasia, lymphoedema and a characteristic facial phenotype. The exact mode of inheritance is uncertain, autosomal dominant, recessive and X-chromosomal cases have been reported.

HISTORY AND SIGNS: A three-month-old boy with intrauterine growth retardation and microcephaly was referred to our clinic. The ophthalmic examination revealed a left eye with a persistent hyperplastic primary vitreous. On funduscopy of the right eye pale optic disc, chorioretinal dysplasia with pigmentary and atrophic changes and falciform folds were noted. General morphological changes and ophthalmological findings led to the diagnosis of MLCRD-syndrome.

THERAPY AND OUTCOME: Eye examinations of the parents and the grandparents did not show any retinal changes, therefore an autosomal dominant inheritance was excluded.

CONCLUSIONS: An ophthalmological examination in children with microcephaly and facial dysmorphies is essential. Parents and grandparents should also be considered for eye examination if a child has chorioretinal dysplasia and microcephaly.

Thieme Connect

Physical and psychological impairments of women with upper limb lymphedema following breast cancer t

Physical and psychological impairments of women with upper limb lymphedema following breast cancer treatment.

Psychooncology. 2009 Apr 27

Department of Internal Medicine, Wroclaw Medical University, Wroclaw, Poland.

Objective: The aim of the study was to identify factors associated with worse physical and emotional functioning of breast cancer survivors with upper extremity lymphedema.Methods: 1250 sets of questionnaires consisting of WHO-DAS II, EORTC QLQ-C30, EORTC QLQ-BR23 and GHQ-30 were mailed to women who underwent a breast cancer surgery at the Lower Silesian Oncology Center in Poland between January 1998 and December 2005.Results and conclusions: The response rate was 33.47%. 117 women were included into a lymphedema group and 211 into a group without this complication. Women with lymphedema were more disabled (overall disability (DAS) score 39.78 versus 34.67; p<0.001), reported symptoms from the upper extremity (shoulder or arm pain and difficulties in arm movement) and from the operated breast (pain and swelling) 2-3 times more often, experienced poorer quality of life (global quality of life (QOL) score 0.50 versus 0.57; p=0.005) and higher psychological distress (GHQ score 10.61 versus 8.01; p=0.007) in comparison to breast cancer survivors without lymphedema. The factors associated with higher DAS score, higher GHQ score and lower QOL score in women with lymphedema were as following: pain in the upper limb (mainly shoulder and arm), pain in operated breast, difficulties with arm movement, localization of lymphedema within the hand or in operated breast, a history of dermatolymphangitis and of receiving chemotherapy. Severity of lymphedema, younger age, BMI and localization of lymphedema within the dominant limb were not considerably related to worse outcomes in these women. 2009

John Wiley & Sons, Ltd.

Lymphedema in Patients With Melanoma

Melanoma is a growing public health problem with an estimated 132,000 cases diagnosed annually worldwide (World Health Organization, 2004). It is the sixth most common malignancy among Americans (American Cancer Society, 2004) and accounts for more than 79% of all skin cancer-related deaths (Geller & Annas, 2003; Trask et al., 2001). Fortunately, the majority of patients diagnosed with melanoma present with early-stage disease that is cured by surgical excision alone. For others, presenting with regional or distant metastatic disease, the prognosis is not as favorable. The most critical factors for determining the prognosis of patients with melanoma are primary tumor thickness and the status of regional lymph nodes. Both of these elements are incorporated into the American Joint Committee on Cancer staging system for melanoma (American Joint Committee on Cancer, 2002).

Surgical Treatment
The most important information for determining surgical treatment of melanoma is the thickness (Breslow thickness), which is measured in millimeters and is determined by pathologic examination of a biopsy of the primary tumor. Current surgical guidelines (National Comprehensive Cancer Network, 2004) include a wide local excision (WLE) of the primary tumor. Regional lymph nodes are the most common first site of melanoma metastases. Over the last decade, sentinel lymph node (SLN) biopsy has emerged as a reliable technique for identifying micrometastatic disease in clinically negative regional lymph node basins. SLN biopsy is a highly accurate, minimally invasive surgical procedure based on the theory that lymphatic metastases associated with melanoma follow an orderly progression through lymph channels from the primary tumor to a particular lymph node (designated the “sentinel” lymph node) before spreading into other regional (“nonsentinel”) lymph nodes (Reintgen et al., 1994).

Current guidelines outlined by the National Comprehensive Cancer Network for the treatment of patients with melanoma recommend SLN biopsy for all patients with primary melanomas greater than 1 mm thickness and subsets of patients with high-risk thin (< 1 mm) melanomas (Ak, Stokkel, Bergman, & Pauwels, 2000). In patients with an SLN that tests negative for presence of cancer, no further surgical therapy is recommended. In patients with metastases identified in the SLN, a complete lymph node dissection is performed. This combination of surgical treatments, a WLE of a primary tumor along with SLN biopsy and possible subsequent complete lymph node dissection, results in multiple sites of lymphatic disruption.

Unlike axillary (“armpit”) node dissection for patients with breast cancer, which includes dissection of level I and II lymph nodes, axillary node dissection for patients with melanoma of the upper extremity or trunk includes the routine dissection of level I, II, and III lymph nodes. Level III lymph nodes are the highest axillary nodes (apical). A standard lymph node dissection for patients with lower extremity or truncal melanomas draining to the inguinal region includes the removal of lymph nodes located in the inguinofemoral (groin) region. In addition, subsets of patients with melanoma with bulky nodal disease undergo an additional deep pelvic dissection.

The surgical treatment of melanoma results in varying degrees of lymphatic disruption resulting in a lifetime risk for developing lymphedema. It is postulated that lymphedema may occur more frequently in patients with melanoma because of: (1) multiple surgical sites (e.g., primary tumor excision on an extremity in conjunction with lymph node biopsy or dissection), (2) extent of nodal dissections, and (3) anatomic sites, particularly the lower extremity, which may be at increased risk due to physiologic reasons.

Review of the Literature
The data estimating the incidence of lymphedema in patients with melanoma are far from complete. We have systematically reviewed the literature and found a total of 25 studies performed between 1972 and 2004 pertaining to melanoma and lymphedema. Of these 25 studies, 12 (48%) were retrospective, 10 (40%) prospective, 2 (8%) clinical trials, and 1 (4%) a case series. As with the reported incidence of postsurgical lymphedema for patients with breast cancer (Passik & McDonald, 1998; Petrek & Heelan, 1998), the reported incidence of lymphedema in patients with melanoma varies widely, with reported rates ranging from 2% to 67%. The disparity likely relates to the heterogeneity of the reports, which will be discussed below.

Measurement Methods
Volume measurement using water displacement historically has been regarded as the most sensitive and accurate measure; however, clinicians rarely use this cumbersome approach (Petlund, 1991; Tierney, Aslam, Rennie, & Grace, 1996). Two of the 25 studies used water displacement alone (James, 1982) or in combination with circumference measurements (Baas et al., 1992). Circumference measurements were utilized in five of the studies (Holmes et al., 1977; Karakousis, Heiser, & Moore, 1983; Lawton, Rasque, & Ariyan, 2002; Papachristou & Fortner, 1977; Urist, Maddox, Kennedy, & Balch, 1983). The remaining 15 studies reported on lymphedema in patients with melanoma using clinical definitions with no objective measurement criteria.

Lymphedema Classification
In one study in which significant lymphedema was defined as greater than 1 inch difference (measured at the ankle or midcalf) between the affected and unaffected limb, lymphedema was reported in 80% of patients five years after surgery (Papachristou & Fortner, 1977). Two other studies reported rates of 21%–26% when 2–4 cm (Urist et al., 1983) or 3–4.5 cm (Karakousis et al., 1983) differences were used as criteria.

Surgical Procedures
In 235 patients who underwent SLN biopsy in the axillary or inguinofemoral region, only 5 patients (2%) were reported to have lymphedema on an average of six weeks after surgery (range of 2–10 weeks) (Wrone et al., 200). Three studies reported lymphedema in 20%–45% of patients undergoing inguinofemoral dissections, with no difference noted between superficial dissections alone or in combination with deep pelvic dissections (Baas et al., 1992; Karakousis & Driscoll, 1994; Stevens et al., 2000).

Postoperative wound complications often are associated with extensive nodal dissections, particularly in the inguinal region. Seven studies reported postoperative wound complications, including cellulitis in 6%–33% of patients (Baas et al., 1992; Ingvar, Erichsen, & Jonsson, 1984; Karakousis & Driscoll, 1994; Lawton et al., 2002; Serpell, Carne, & Bailey, 2003; Strobbe, Jonk, Hart, Nieweg, & Kroon, 1999; Urist et al., 1983).

Anatomic Variation—Upper Versus Lower Extremity
It is difficult to make direct comparisons of rates of lymphedema at various anatomic sites among studies given the heterogeneity of the reports. Of the studies that examined lymphedema associated with axillary versus inguinofemoral nodal dissections, similar trends were noted. The reported range of upper extremity lymphedema was 5%–8% (Shaw & Koea, 1992; Urist et al., 1983) compared to 14%–29% for lower extremity lymphedema (Lawton et al., 2002; Serpell et al., 2003). It appears from these reports that the incidence of lower extremity lymphedema may be twice as high as upper extremity lymphedema.

Time Horizon
It has been noted that breast cancer lymphedema studies with the shortest follow-up (12 months) report the lowest incidence (6%) (Petrek & Heelan, 1998), whereas studies with the longest follow-up have the highest incidence. Similar trends are noted in this review, with the lowest lymphedema rates (upper and lower extremity) noted in studies that examined patients six months postoperatively (Wrone et al., 2000), whereas the highest incidence (67%, lower extremity lymphedema) had the longest follow-up (20 years) (Papachristou & Fortner, 1977). High rates (44%) also were noted when lymphedema was defined as swelling lasting more than six months (Beitch & Balch, 1992).

Discussion
All people diagnosed with melanoma are at a lifetime risk of developing lymphedema because of the extensive lymphatic disruption associated with surgical treatment. Lymphedema and its sequelae are much-understudied morbidities associated with cancer treatment, particularly in patients with melanoma. In patients with breast cancer, lymphedema has been described as an overlooked, underdiagnosed, and undertreated condition, which, other than tumor recurrence, is the most feared effect of treatment (Disa & Petrek (2001)). The same likely can be said of melanoma and lymphedema.

Lymphedema is a significant problem. In addition to the symptoms and risks, the associated challenges also may lead to post-treatment psychosocial distress. Although researchers have documented the psychological sequelae of breast cancer treatment (Tobin, Lacey, Meyer, & Mortimer, 1993), we know little about such issues in patients with melanoma, especially those that may involve lymphedema of the lower extremity. A conclusion by Maunsell, Brisson, and Deschenes (1993) still stands: “The impact of lymphedema problems on patient quality of life has not been quantitatively assessed.” This research has been hampered by the traditional view that quality of life is less important than the eradication of cancer and detection of recurrence. Unfortunately, lack of attention to lymphedema by health professionals has not only meant that many people go undiagnosed and fail to receive basic preventive information (Maunsell et al.), but this lack of attention also has hindered the development of effective psychosocial and physiological therapeutic interventions.

Early detection and intervention hold the greatest promise of reducing this widespread condition (Petrek, Pressman, & Smith, 2000; Rockson, 1998). The range of findings in the literature reflects inconsistent criteria for defining lymphedema, small studies, retrospective analyses, and short follow-up. We must clearly identify epidemiological and clinical factors associated with risk and incidence to build a foundation for preventive interventions.

Note. This article originally appeared in volume 17, Issue 1, 2005, of LymphLink. Reprinted with permission.

References

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American Cancer Society. (2004). Cancer facts and figures, 2004. Atlanta, GA: Author.

American Joint Committee on Cancer. (2002). Cancer staging handbook from the AJCC cancer staging manual. Heidelberg, NY: Springer.

Baas, P.C., Schraffordt Koops, H., Hoekstra, H.J., van Bruggen, J.J., van der Weele, L.T., & Oldhoff, J. (1992). Groin dissection in the treatment of lower-extremity melanoma. Short-term and long-term morbidity. Archives of Surgery, 127, 281–286.

Beitch, P., & Balch, C. (1992). Operative morbidity and risk factor assessment in melanoma patients undergoing inguinal lymph node dissection. American Journal of Surgery, 164, 462–466.

Disa, J., & Petrek, J. (2001). Rehabilitation after treatment for cancer of the breast. In V. Devita, S. Hellman, & S. Rosenberg (Eds.), Cancer: Principles and practice of oncology (pp. 1717–1725). Philadelphia: Lippincott Williams and Wilkins.

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Holmes, E.C., Moseley, H.S., Morton, D.L., Clark, W., Robinson D., & Urist, M.M. (1977). A rational approach to the surgical management of melanoma. Annals of Surgery, 186, 481–490.

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Lymphedema Management Special Interest Group Newsletter  

The role of operative management of varicose veins in patients with lymphedema and/or lipedema of th

The role of operative management of varicose veins in patients with lymphedema and/or lipedema of the legs.

Lymphology. 2000 Dec

Földi M, Idiazabal G.

Földiclinic for Lymphology, Hinterzarten, Germany.

The role of operative management of "symptomatic" varicose veins in patients with lower extremity lymphedema or lipedema is controversial. We reviewed the clinical outcome of 261 patients between 1989-1997 at the Földiclinic with lower extremity lymphedema (68 patients), lipo-lymphedema or lympho-lipedema (103 patients) or lipedema (90 patients) who had undergone operation for varicose veins. In each group, the results were dismal as leg swelling worsened or was unchanged in greater than 90% whereas symptoms such as heaviness, fatigue, cramps (termed varicogenic symptomatology) were improved in less than 10%. These findings support that operations for varicose veins in the legs of patients with lymphedema, lipedema, or combinations of these disorders should be undertaken only if there is an absolute indication present (ascending phlebitis and/or bleeding). Otherwise, complete decongestive physiotherapy is still the best treatment approach for these groups of patients.

PMID: 11191657 [PubMed - indexed for MEDLINE]