91/525. Prenatal ultrasonographic diagnosis of atypical Nonne-Milroy lymphedema. Nonne-Milroy lymphedema is a relatively rare disorder characterized by firm edema of the lower extremities either on the whole leg or limited to the feet or toes. We report a case of atypical Nonne-Milroy syndrome that presented prenatally with bilateral leg edema, bilateral hydrothorax and lung hypoplasia at 22 weeks' gestation. The differential diagnoses are discussed. ( info) |
92/525. Verruciform xanthoma in association with milroy disease and leaky capillary syndrome. An 18-year-old Caucasian boy with Milroy disease involving the right leg presented with erythematous, smooth-topped, waxy papules on the dorsum of his right foot. A 12-year-old Caucasian girl with leaky capillary syndrome presented with hemorrhagic verrucous papules on the dorsum of the toes of both feet. Histopathologic analysis revealed changes consistent with xanthoma. Both patients were treated with leg compression, curettage, and electrodesiccation. Although similar papules have been described in the setting of lymphedema from other causes, this is the first report of verruciform xanthoma associated with Milroy disease or leaky capillary syndrome. ( info) |
| We report a man aged 68 years old with pneumothorax and chronic bilateral pleural effusion in association with a history of yellow nails. The diagnosis of yellow nail syndrome based on yellow nails, lymphedema, chronic pleural effusion and intestinal lymphangiectasia. ( info) |
94/525. Radiological features of late-onset lymphoedema in Noonan's syndrome. Noonan's syndrome is a multiple congenital anomaly syndrome with diverse manifestations. lymphatic abnormalities occur in less than 20% of patients. We report a 14-year-old boy who presented with swollen lower limbs and dysmorphic features characteristic of Noonan's syndrome. The radiological features of this unusual case of late-onset lymphoedema in association with Noonan's syndrome are presented. ( info) |
95/525. Follow-up study of upper limb lymphedema patients treated by microsurgical lymphaticovenous implantation (MLVI) combined with compression therapy. We present a follow-up study of 18 patients with upper limb lymphedema treated by microsurgical lymphaticovenous implantation (MLVI) combined with compression therapy. This combined technique provides increased lymphatic flow through newly created lymphaticovenous bypasses by the MLVI surgery, with assistance for pumping function in the lymphatics by compression therapy. Preoperative assessment of the affected limb was performed by the average enlargement of edema circumference (AEEC), comparing the lymphedema limb and normal limb circumferences. Objective improvement was analyzed by the percent reduction of edema circumference (%REC) at two levels of the lymphedema limb. With an average follow-up of 24 months, 77.8% of patients presented excellent or good results, with %REC >50% at either the distal or proximal site of the treated limb. This combined treatment can be expected to provide favorable long-term results, even for patients with AEEC >8 cm. ( info) |
96/525. Aortic regurgitation and pigmentation - unusual features of noonan syndrome. A patient with typical features of Noonan's syndrome showed aortic regurgitation and widespread diffuse hyperpigmentation, features not previously associated with this syndrome. Detailed endocrine and dermatological studies failed to delineate the cause of hyperpigmentation. In addition to mild aortic regurgitation, cardiac catheterization revealed mild left ventricular dysfunction, probably due to primary myocardial disease. Other interesting findings included lymphedema and sexual infantilism despite normal testosterone levels. ( info) |
| arm edema occurs in 20 to 30 per cent of patients who undergo axillary lymph node dissection (ALND) for carcinoma of the breast. sentinel lymph node biopsy (SLNB) in lieu of ALND for staging of breast cancer significantly lowers this morbidity. We hypothesized that SLNB would have a lower lymphedema rate than conventional axillary dissection. patients who underwent SLNB were compared with those who underwent level I and II axillary node dissection. A total of 125 patients were evaluated with 77 patients who underwent SLNB and 48 patients who underwent ALND. The arm circumference 10 cm above and 10 cm below the olecranon process was measured on both arms. In this series a difference in arm circumference greater than 3 cm between the operated and nonoperated side was defined as significant for lymphedema. lymphedema was seen in two of 77 (2.6%) patients in the SLNB group as compared with 13 of 48 (27%) ALND patients. Given the above data patients who underwent sentinel lymph node biopsy show a significantly lower rate of lymphedema than those who had axillary lymph node dissection. This has an important impact on long-term postoperative management of patients with breast cancer. ( info) |
| lymphedema-cholestasis syndrome (LCS, Aagenaes syndrome) is the only known form of hereditary lymphedema associated with cholestasis. A locus, LCS1, has recently been mapped to chromosome 15q in a Norwegian kindred. In a consanguine Serbian Romani family with a neonate who had a combination of lymphedema and cholestasis with features atypical for Norwegian LCS, haplotype and linkage analysis of markers spanning the LCS1 region argue that a second LCS locus may exist. The infant may represent an instance of a previously undescribed lymphedema-cholestasis syndrome. ( info) |
99/525. Yellow-nail syndrome: report of three cases. The yellow nail syndrome, a combination of yellow discolouration of and dystrophic changes in the nails, pleural effusions and lymphedema, is thought to be relatively rare; to date 44 cases have been reported. Of a further three patients with this syndrome, one had all three features, one had the yellow nails alone and the other had pleural effusions and lymphedema without classic nail changes. Each had recurrent lower respiratory tract infections; and of all 47, chronic pulmonary infections occurred in approximately one quarter and were frequently associated with chronic sinus infections. The underlying abnormality is presumed to be a congenital defect of the lymphatics, but so far this has not been demonstrated to be the cause of the nail changes, the pathogenesis of which remains obscure. ( info) |
100/525. Hennekam syndrome presenting as nonimmune hydrops fetalis, congenital chylothorax, and congenital pulmonary lymphangiectasia. We report a female infant with congenital lymphedema, facial anomalies, intestinal lymphangiectasia consistent with a diagnosis of Hennekam syndrome. At birth the patient presented with severe respiratory distress due to nonimmune hydrops fetalis, a congenital chylothorax (CC), and pulmonary lymphangiectasia. hydrops fetalis may be present in newborns with the Hennekam syndrome. lymphoscintigraphy can be useful in explaining pleural-pulmonary involvement of this generalized lymph vessel malformation syndrome. ( info) |