Aortic aneurysm in a four-year-old child with tuberous sclerosis

We present a case of aortic aneurysm in a four-year-old child complicated with tuberous sclerosis. We used the same general principles as for adult patients and successfully managed our patient. Our methods included the use of isoflurane plus epidural anaesthesia, dopamine to maintain blood pressure, and induced mild hypothermia to reduce brain metabolism and to prevent spinal cord damage during aortic cross-clamping. Intensive monitoring including EEG was beneficial to the anaesthetic management.     

Prediction of Optimal Atrioventricular Delay in Patients with Implanted DDD Pacemakers

In patients with an implanted DDD pacemaker (PM), the atrial contribution may be interrupted by too short an atrioventricular (AV) delay, and filling time may be shortened by too long an AV delay. The AV delay at which the end of the A wave on transmitral flow coincides with complete closure of the mitral valve may be optimal. The subjects were 15 patients [70.3+/-12.3 (SD) years old] with an implanted DDD PM. Cardiac output (CO) and pulmonary capillary wedge pressure (PCWP) were measured by Swan-Ganz catheter. Transmitral flow was recorded by pulsed Doppler echocardiography. AV delay was prolonged stepwise by 25 msc. When the AV delay was set at 155+/-26 ms, the end of the A wave coincided with complete closure of the mitral valve. When the AV delay was prolonged 25, 50, 75, and 100 ms from this AV delay, the interval between the end of the A wave and complete closure of mitral the valve was prolonged 16+/-5, 39+/-6, 65+/-4 and 88+/-5 ms, respectively (r = 0.97, P<0.0001) and diastolic mitral regurgitation was observed during this period. Thus, the optimal AV delay may be predicted as follows: the slightly prolonged AV delay minus the interval between the end of the A wave and complete closure of the mitral valve. When the AV delay was set at 215 ms, there was a significant positive correlation between the predicted optimal AV delay (166+/-23 ms) and the optimal AV delay (CO: 161+/-26 msec, r = 0.93, P<0.0001, PCWP: 161+/-28 msec, r = 0.95, P<0.0001). In conclusion, optimal AV delay can be predicted by this simple formula: slightly prolonged AV delay minus the interval between end of A wave and complete closure of mitral valve at the AV delay setting.     

PAPULAR MUCINOSIS (SCLEROMYXOEDEMA) COMPLICATING DIFFUSE SYSTEMIC SCLEROSIS: CLINICAL FEATURES AND ELECTRON MICROSCOPE OBSERVATIONS

Papular mucinosis (scleromyxoedema) is an uncommon disordercharacterized by generalized papular eruption and cuta neousinduration, which may be associated with a variety of extracutaneousmanifestations. Although scleroderma and papular mucinosis sharemany features, they are clinically and histologically distinctentities. We report here a patient with diffuse sclerodermawho developed superimposed papular mucinosis. Degranulatingmast cells were a prominent ultra- structural finding in theinvolved skin. The occurrence of scleroderma and papular mucinosis,two uncommon cutaneous indurative diseases, in the same patienthas not been described previously. KEY WORDS: Papular mucinosis, Scleromyxoedema, Systemic sclerosis, Cutaneous mucinosis     

Successful laparoscopic management of chylous ascites following laparoscopic radical nephrectomy

A 44-year-old woman developed chylous ascites following laparoscopic transperitoneal left radical nephrectomy with para-aortic lymph node dissection. Because conservative managements failed to stop the lymphatic leakage, laparoscopic lymphostasis was performed. Drinking milk 6 h prior to the operation enabled visualization of chylous ascites. Although a definite fistula was hard to identify, most of the chylous leak disappeared after ligation of the para-aortic tissues at the distal and proximal ends of the previous lymph node dissection. Laparoscopic ligation of the para-aortic bundle of lymph ducts was effective in managing long-standing postoperative chylous ascites.