Straight line repair of unilateral cleft lip: new operative method based on 25 years experience.

The resultant scar in the primary repair of unilateral cleft lip should ideally be straight and the mirror image of the philtrum on the non-cleft side. In 1993, we reported a new operative technique for unilateral cleft lip, in which we designed a straight line for the incision on the white lip. In order to produce the nostril floor, we used the white lip tissue in the area between the alar base and alveolus at the cleft side as a flap. We also used a small triangular flap above the white skin roll to prevent Cupid's peak from being drawn up. Unlike the rotation-advancement method, our technique does not leave a transverse scar at the alar base. Instead, it leaves a scar only along the line coincident with the natural philtral ridge. However, during observations of our patients, we noticed that the small triangular flap designed to be 1.5mm tended to become a conspicuous angular scar as the patients grew older. In addition, drooping of Cupid's peak on the cleft side was often observed with this small triangular flap. To make it less conspicuous, we made some modifications to the small flap above the white skin roll. With this new technique, we designed a semi-circular flap (1.5 x 3mm) above the white skin roll, instead of the small triangular flap. The suture line of our refined procedure draws a gentle curve, which looks almost straight because of skin elasticity. Moreover, the semi-circular flap causes less drooping of the upper lip than the triangular flap. We believe that revising the shape of the small flap on the white skin roll greatly improves patients' appearance. In this report, we present our refined techniques of primary repair of unilateral cleft lip.

The change in the appearance of the vermilion border region caused by the difference of the design in symmetric bilateral incomplete cleft lip repair.

In the widely performed methods for repair of bilateral cleft lips, such as Mulliken and Trott methods, the formation of the median tubercle is done with the mucocutaneous flaps from the lateral lips. By this manoeuver, a visible horizontal scar just above the white skin roll of the philtral region tends to result, especially among Asian patients. To avoid the formation of this visible scar, we changed our design in such a way that mucosal flaps, instead of mucocutaneous flaps, are elevated from the lateral lips, and the prolabial white skin roll is preserved to become the final philtral white skin roll. By this change in design, the horizontal scar is shifted to the inferior edge of the vermilion border. In the past 8 years, we have performed this refined method along with the conventional one. In the present study, the five cases of symmetric bilateral incomplete cleft lip from each of the two groups are evaluated: one group with the mucocutaneous flaps and the other with the mucosal flaps. From the review of the cases, it was noted that when the philtral region is formed with the mucocutaneous flaps, the horizontal scar tends to be visible. On the other hand, when it is formed with the mucosal flaps, the scar is less conspicuous, although the white skin roll sometimes becomes less-defined. The refined method involving mucosal flaps from the lateral lips produces a better-accepted appearance concerning the prolabial horizontal scar in the symmetric bilateral incomplete cleft lip repair.

Capillary gas chromatographic behaviour of tert.-hydroxylated steroids by trialkylsilylation.

Capillary gas chromatographic behaviour was studied for a variety of structurally different bile acids and sterols having one to two tert.-hydroxy groups, together with several sec.-hydroxy groups, at positions C-3, -5, -7, -12, -14, -17, -20, -24, and/or -25. The tert.-hydroxylated steroids were subjected to trimethylsilylation with hexamethyldisilazane/trimethylchlorosilane /pyridine and N,O-bis(trimethylsilyl)acetamide/N-trimethylsilylimidazole/trimethylchlorosilane, and dimethylethylsilylation with N,N,-dimethylethylsilylimidazole. The methylene unit values of the resulting trialkylsilylation products were used for determining their structures of partially and/or fully derivatised ethers. The reactivity of the trialkylsilylation of tert.-hydroxy groups was found to be significantly dependent not only on the derivatisation reagents and conditions used, but also on the position and steric factor of the tert.-hydroxy groups. The following general order of the decreasing reactivity of tert.-hydroxy groups in steroids by trialkylsilyl etherification was observed: 25>20, 24>5beta>17alpha>>14alpha.

Effect of angiotensin-converting enzyme inhibitor on cardiopulmonary baroreflex sensitivity in patients with acute myocardial infarction.

We evaluated the effect of angiotensin-converting enzyme inhibition (quinapril) on cardiopulmonary baroreflex sensitivity in 30 patients with uncomplicated myocardial infarction (quinapril group, 15 patients; placebo group, 15 patients) at 5 and 10 days after the onset of myocardial infarction. This study indicates that quinapril improved cardiopulmonary baroreflex and thus reduced sympathetic outflow in patients with acute myocardial infarction.

Importance of intrinsic calf vasodilator capacity in determining distribution of skeletal muscle perfusion during supine bicycle exercise in patients with left ventricular dysfunction.

BACKGROUND: Distribution of skeletal muscle perfusion during exercise is an important factor in determining exercise capacity and is markedly impaired in patients with cardiac disease. This study examined the importance of intrinsic calf vasodilator capacity in determining distribution of skeletal muscle perfusion during supine bicycle exercise in patients with left ventricular dysfunction. METHODS: We studied 19 patients with left ventricular dysfunction (left ventricular ejection fraction <45%) after myocardial infarction. All the patients underwent cardiopulmonary exercise testing with measurements of central hemodynamics, leg blood flow (LBF), and the percentage of cardiac output distributed to both legs (%LBF). Calf reactive hyperemic flow (RH) was measured by venous occlusive plethysmography at supine rest. RESULTS: LBF at peak exercise was closely related to peak cardiac output and RH. Furthermore, %LBF at peak exercise had modest correlation with peak cardiac output and good correlation with RH. Although peak cardiac output and RH were independent determinants of LBF at peak exercise by multiple regression analysis, RH had higher correlation with %LBF at peak exercise than peak cardiac output. Despite marked changes in other hemodynamic variables, nonleg blood flow during exercise was constantly maintained at a level identical to resting value. CONCLUSIONS: Calf vasodilator capacity, which was the major determinant of distribution of skeletal muscle perfusion during exercise, may have contributed to maintaining perfusion of important nonexercising regions during exercise in patients with left ventricular dysfunction.

Effects of left ventricular peak filling rate on exercise capacity 3 to 6 weeks after acute myocardial infarction.

STUDY OBJECTIVES: To investigate the importance of early left ventricular (LV) diastolic filling on maintenance of exercise performance, we examined peak filling rate and its relation to exercise capacity during upright bicycle exercise in patients with recent myocardial infarction. DESIGN: Retrospective analysis of data of cardiopulmonary exercise testing characteristics in patients with recent myocardial infarction. SETTING: Coronary care unit in a university hospital. PATIENTS: Fifty-one patients 3 to 6 weeks after acute myocardial infarction. INTERVENTIONS: Upright bicycle exercise using a symptom-limited graded exercise protocol. MEASUREMENTS AND RESULTS: Peak filling rate increased significantly from 1.55 +/- 0.52 at rest to 3.43 +/- 1.1 end-diastolic volume per second at peak exercise. Despite no significant relation between peak filling rate at rest and peak oxygen consumption, peak filling rate at peak exercise correlated significantly with peak oxygen consumption (r = 0.50; p < 0.002), stroke volume (r = 0.51; p < 0.002), and cardiac output (r = 0.56; p < 0.002) at peak exercise. Although both end-systolic and end-diastolic volumes increased from rest to peak exercise, the increases in end-systolic volume correlated inversely with the changes in peak filling rate during exercise (r = -0.45; p < 0.02), but the increases in end-diastolic volume did not. CONCLUSIONS: During maximal upright bicycle exercise, exercise capacity and exercise hemodynamic responses were mainly dependent on early LV diastolic filling, and preserved LV systolic contraction, resulting in a cardiac suction effect following early diastole, seemed to have an important role in the enhancement of early LV diastolic filling in patients with recent myocardial infarction.

Importance of left ventricular diastolic function on maintenance of exercise capacity in patients with systolic dysfunction after anterior myocardial infarction.

To investigate the role of left ventricular (LV) diastolic function in the maintenance of exercise capacity in patients with systolic dysfunction, symptom-limited cardiopulmonary exercise testing combined with radionuclide ventriculography was performed in 24 patients with an LV ejection fraction < 35% after anterior myocardial infarction. The ratio of pulmonary artery wedge pressure (PAWP) to LV end-diastolic volume (EDV), an index of global diastolic function, correlated significantly with peak oxygen consumption at peak exercise (r = -0.55; p = 0.006), whereas ejection fraction at peak exercise did not. The change in PAWP/EDV ratio from rest to peak exercise was related to the increases in stroke volume (r = -0.54; p = 0.006) and cardiac output (r = -0.51; p = 0.01) during exercise, but the change in ejection fraction was not. Resting hemodynamics did not differ between patients with preserved exercise capacity (group 1, n = 8) and those with exercise impairment (group 2, n = 16). At peak exercise, stroke volume, cardiac output, and EDV were significantly higher, and PAWP and PAWP/EDV ratio were significantly lower in group 1 than in group 2, but ejection fraction and end-systolic volume were similar in both groups. Although the incidences of hypertension, LV hypertrophy, and infarct-related coronary artery lesions did not differ between the two groups, group 2 had a significantly higher incidence of non-infarct-related coronary artery lesions than group 1 (p < 0.05). Thus in patients with LV systolic dysfunction after anterior myocardial infarction, the major cause of exercise impairment and failure to increase LV performance during exercise was diastolic dysfunction associated with the presence of non-infarct-related coronary artery lesions with the potential for exercise-induced ischemia of the noninfarcted areas.

Skeletal muscle hypoperfusion during recovery from maximal supine bicycle exercise in patients with heart failure.

Leg blood flow (LBF) and its relation to central hemodynamics were examined during recovery following maximal supine bicycle exercise in 11 patients with heart failure and 20 patients with normal exercise capacity after myocardial infarction. The results indicate that LBF was markedly reduced in patients with heart failure, and that decreased cardiac output response and enhanced peripheral vasoconstriction, which functioned to prevent hypoperfusion in the nonexercising vital regions and to maintain arterial blood pressure, were responsible for the reduced LBF during recovery in heart failure.

Influence of intrinsic limb vasodilator capacity on exercise tolerance in patients with recent myocardial infarction.

To investigate the influence of intrinsic limb vasodilator capacity on exercise performance, limb reactive hyperemic flows and their relations to exercise capacity during upright bicycle exercise were examined in 52 patients with recent myocardial infarction. Reactive hyperemic flow was measured in the forearm and the calf by venous occlusive plethysmography after 5 min of arterial occlusion. Calf reactive hyperemic flow correlated significantly with cardiac output, systemic vascular resistance, and oxygen consumption at peak exercise, whereas flow in the forearm did not. In patients with preserved exercise capacity (group 1, n = 20) compared with those with exercise impairment (group 2, n = 32), calf reactive hyperemic flow was significantly augmented, but forearm flow was similar in the two groups. There were no significant differences in hemodynamic parameters at rest between the two groups. At peak exercise, however, cardiac output was lower and systemic vascular resistance was higher in group 2 than in group 1, whereas arterial blood pressure was maintained identically in the two groups. Thus, intrinsic calf but not forearm vasodilator capacity was linked to exercise hemodynamic responses and exercise capacity in patients with recent myocardial infarction. In addition, reduced calf vasodilation and concomitant enhanced vascular tone seemed to be useful for preserving arterial blood pressure in the setting of decreased cardiac output response to exercise in patients with exercise impairment.

Sustained ventricular tachycardias associated with myotonic dystrophy.

Patients with myotonic dystrophy are reported to have a higher frequency of sudden death than the general population. Although causes of sudden death in myotonic dystrophy are suggested to be due to conduction of defects progressing, the HV interval cannot predict whether conduction system disease would develop or progress. We report two cases of myotonic dystrophy complicated with sustained monomorphic ventricular tachycardias (VT), which can cause sudden death. In Case No. 1, although the patient was treated successfully for sustained VT with verapamil in electrophysiologic studies, another sustained VT was confirmed 2 years later. In Case No. 2, the patient showed decreased left ventricular ejection fraction and late potentials, and induced sustained VT that was identical to clinically documented VT. Although VT is believed to be rare in patients with myotonic dystrophy, these cases suggest that VT is a possible cause of sudden death.

A case of iatrogenic growth retardation induced by a corticosteroid-containing anti-allergic drug.

A nine-year old boy developed reduced growth velocity at the age of seven. The peak plasma growth hormone (GH) response to 3,4-dihydroxyphenylalanine, GH-releasing factor and insulin was 10.2, 8.1 and 7.6 micrograms/l, respectively, suggesting that the GH reserve was slightly reduced. Serum cortisol was undetectable and urinary excretion of 17-hydroxycorticosteroid was low (0.22-0.31 mg/day), but there were no physical or biochemical signs of adrenocortical insufficiency. He had taken an anti-allergic drug containing 0.25 mg of betamethasone and 2 mg of d-chlorpheniramine maleate per tablet for about 2 years to treat allergic rhinitis. Catch-up growth occurred when this drug was stopped. The present case suggests that daily administration of 0.25 mg of betamethasone can induce growth retardation and that ingestion of corticosteroid-containing preparations needs to be excluded in children who develop short stature without other symptoms.