The reliability of the Lanthony Desaturated D-15 test.

PURPOSE: The Lanthony Desaturated Panel D-15 has been used to measure fine color discrimination for congenital and acquired color vision defects. This study investigated the test-retest reliability of the test using an intertest interval of approximately 1 month. METHODS: One hundred twenty-six color vision normals (mean age = 34.5 years) were administered several color vision tests, including the Lanthony Desaturated D-15. Normal color vision status was confirmed using the anomaloscope and HRR color plates. The color vision tests were readministered 3 to 6 weeks after initial testing. The results of the Lanthony test were expressed using the color confusion index of Bowman. The difference in Color Confusion Index (CCI) between the two administrations was calculated and used to determine the intraclass correlation coefficient. RESULTS: The overall mean CCI for the two administrations for these subjects was 1.11 +/- 0.136. The mean difference in CCI score between test administrations was -0.02 +/- 0.128. There was a strong correlation between the absolute value of the difference in CCI and the mean CCI for each subject (r = 0.51, p < 0.0001). The intraclass correlation coefficient was 0.56 (95% confidence interval, 0.43-0.67). CONCLUSIONS: Although the Lanthony Desaturated D-15 test can be used to assess fine color discrimination, there is considerable within-subject variability in test results. The intraclass correlation coefficient is less than that recommended for use in clinical testing or research. Clinicians should consider at least three administrations of the test at each sitting to ensure precision and we recommend taking the mean of those three tests.     

Lipofibromatous hamartoma of the digital nerve without macrodactyly: follow-up of an unusual case

Lipofibromatous hamartoma (LFH) of nerve is a rare benign, tumour-like fibro-fatty malformation of the epi- and perineurium. Major peripheral nerves of the upper extremity are mostly involved, most commonly the median nerve and its branches. Although there is a relatively large number of publications on LFH, involvement of digital nerves is rarely reported or described. A case of LFH involving the digital ulnar nerve of the index finger without macrodactyly is presented; the aetiology, pathogenesis, differential diagnosis and surgical management are described.     

Conventional industrial foam rubber as an alternative dressing for large skin grafts in plastic surgery

Industrially manufactured foam rubber (polyurethane) is clinically used as variable, hygienic mattresses and also serves as a positioning aid during surgery. It can be sterilized and used to cover large skin grafts; it conforms well to the anatomical topography. It is stapled together with the skin graft, this allows mobilization of patients with large skin grafts i.e. >10% body surface area.     

The scapular/parascapular flap in reconstruction of distal lower extremity soft tissue defects over the Achilles tendon

Combined defects of soft tissue and Achilles tendon are rare and are usually seen following repair of the tendon. Large size defects frequently cannot be reconstructed with local tissue. Various free flaps such as the radial forearm flap and the temporoparietal fascia flap have been described for reconstruction. In selected cases with concomitant Achilles tendon defect or loss of gliding tissue, the fasciocutaneous scapular/parascapular flap with an axial fascial extension offers considerable advantages. Three cases with soft tissue and Achilles tendon defects have been treated with a scapular/parascapular flap during an 18 months period. The defect size ranged from 8×9 or 6×15 cm. All flaps survived, donor site morbidity was not significant and primary donor site closure was possible in all cases. Achilles tendon function was good in two cases and fair in one case. One flap had to be revised to produce better contour, but the other flaps were aesthetically pleasing. The scapular/parascapular flap with fascial extension is a useful addition in reconstruction of combined soft tissue and Achilles tendon defects. The axial fascial part is versatile and can be wrapped around the tendon to provide tendon reinforcement, gliding tissue or both. The thickness of the flap is uniform and a custom tailored flap is possible. Received: 7 July 1997 / Accepted: 25 May 1998     

Phospho-STAT5 expression pattern with the MPL W515L mutation is similar to that seen in chronic myeloproliferative disorders with JAK2 V617F

Abnormal nuclear megakaryocytic staining for phospho-STAT5 (pSTAT5) correlates with JAK2 V617F mutational status in non-chronic myelogenous leukemia chronic myeloproliferative disorders. However, a proportion of wild-type JAK2 non-chronic myelogenous leukemia chronic myeloproliferative disorders cases also demonstrate this abnormal pSTAT5 expression pattern. We report a patient with a JAK2 V617F-negative myeloproliferative/myelodysplastic syndrome who had abnormal megakaryocytic pSTAT5 expression and a MPL W515L mutation. The patient was a 71-year-old man with anemia and thrombocythemia on laboratory examination. His peripheral blood smear demonstrated occasional dysplastic neutrophils. Bone marrow biopsy revealed hypercellular marrow with features consistent with myeloproliferative/myelodysplastic syndrome. Immunohistochemistry for pSTAT5 showed abnormal nuclear megakaryocyte positivity. Cytogenetic analysis revealed a normal karyotype, fluorescence in situ hybridization for BCR-ABL was negative, and JAK2 genotyping demonstrated wild-type JAK2. However, MPL genotyping showed a MPL W515L mutation. Abnormal nuclear megakaryocytic staining for pSTAT5 expression, previously associated with the JAK2 V617F mutation, is also associated with MPL W515L, likely reflecting activation of the JAK-STAT signaling pathway.     

Grafting of deeply burned problem zones in the lower extremity with a dermal substitute

Functional results after deep excision and split thickness skin grafts are often limited as a consequence of unstable grafted areas and contractures. In two patients with a full-thickness thermal injury to the lower extremities and one patient with a chronic unstable skin area over the knee, the Integra^TM bilayered membrane dermal substitute was applied. After uneventful dressing changes graft take was complete without infection or other complications. Complete wound closure was achieved in all three patients within 3–4 weeks after the initial operation. The functional range of motion of the involved joints and the skin quality and contour was superior to conventional grafted skin after excision down to fascia and rather more comparable to skin grafts over a tangentially excised eschar. After wound healing, the neodermis was histologically similar to normal dermis. Considering the high incidence of unstable skin and contractures after regular grafting of deeply excised burns, this concept may present a significant improvement not only for the primary but also for secondary reconstructive procedures, with respect to the long-term quality of life for burn patients. Received: 1 December 1997 / Accepted: 13 June 1998     

Identification of the JAK2 V617F mutation in chronic myeloproliferative disorders using FRET probes and melting curve analysis

We developed and validated a real-time polymerase chain reaction assay using fluorescent hybridization probes and melting curve analysis to identify the JAK2 V617F mutation, which is implicated in a substantial proportion of chronic myeloproliferative disorders (CMPDs). DNA from 161 samples was isolated from peripheral blood granulocytes and formalin-fixed bone marrow clot sections in patients with CMPDs and without myeloproliferative disorders previously genotyped for the JAK2 V617F (G-->T) mutation, which included 114 wild types (GG) and 47 mutants (GT and TT). Melting curve analysis of these samples yielded 114 wild types, 42 heterozygotes, and 5 homozygotes showing 100% concordance. Analytic sensitivity of the assay for mutant DNA was 5% for the LightTyper (Roche Applied Sciences, Indianapolis, IN) and 10% for the LightCycler (Roche Applied Sciences). Consistent with earlier reports, 78% of the non-chronic myelogenous leukemia CMPD patients and 8% of non-CMPD patients displayed this mutation. This study demonstrates that clinical genotyping of the JAK2 V617F mutation can be performed by melting analysis using both freshly isolated and formalin-fixed tissues.     

Increased risk of angiosarcoma secondary to cancer radiotherapy: Case series and review of the treatment options

Angiosarcomas (ASs) are aggressive tumors of vascular endothelial origin, occurring sporadically or in association with prior radiotherapy or chronic lymphedema. With only 1–5% of all sarcomas, the incidence seems low, but for the affected patient due to the extremely poor prognosis and the limited treatment options, the fate is often inevitable. Radiotherapy, chemotherapy, or “target therapy” have been used in the management of AS, but represent individual case decisions without lasting evidence. Over the past few years, breast‐conserving surgery followed by radiation therapy, known as breast‐conserving therapy (BCT), is being employed as a standard treatment for early‐stage breast cancer, but there has been an increase in reports of AS following BCT. We report two cases of AS following BCT and one case of primary AS involving the lower limb.