[Slipped capital femoral epiphysis in an ambulant patient with spastic cerebral palsy. A long-term evolution].

We report the case of a boy aged 13 years and 9 months, with predominantly right-sided spastic tetraparesis, who could walk with assistance, GMFCS III, phenotype consistent with adiposogenital syndrome. He presented a 4-week history of left-sided limp without pain, radiologically classified as a stable, chronic slipped capital femoral epiphysis (SCFE) with mild slippage (<30º) on the Southwick classification. In situ fixation of the hip was performed using a full-headed screw, followed by six weeks of rest. Twelve years since the intervention, the patient remains asymptomatic with no clinical or radiological changes. SCFE in patients with cerebral palsy, while highly unusual, is possible. A high level of suspicion is required for diagnosing it. We would suggest ruling out the appearance of SCFE during surveillance screening of patients with cerebral palsy, =?10 years-old, particularly in obese individuals with or without adiposogenital phenotype and limited ability to communicate verbally.

Resting transmembrane potential difference of skeletal muscle in normal subjects and severely ill patients.

The resting membrane potential difference (Em) of skeletal muscle was measured in 26 normal human subjects, 7 patients with mild illness, and 21 patients with severe, debilitating medical disorders. A closed transcutaneous approach to the muscle was made by needle puncture and the Em was measured utilizing standard Ling electrodes. Measurements revealed an Em of -88 +/-3.8 mv in healthy subjects and -89 +/-2.1 mv in patients hospitalized for minor medical problems. The mean Em in 21 in-hospital patients, judged to be severely ill clinically from a variety of causes, was -66.3 +/-9.0 mv. Open deltoid muscle biopsies were performed in 7 of the healthy subjects and in 13 of the severely ill group. Estimation of the intra-extracellular water partition was made by calculating the chloride space from the previously measured Em. Analysis of the muscle samples revealed no significant difference in the intra-extracellular potassium ratios of the two groups biopsied. Intracellular Na(+) concentrations were uniformly increased in the muscle samples of the severely ill subjects and averaged 42.3% higher than those of the normal subjects. The mechanisms which might account for the elevation of intracellular Na(+) and a depression of Em independent of changes in intra-extracellular K(+) ratios are discussed and it is suggested that this defect may be a generalized cellular abnormality which is a common quality of serious illnesses.

Surgical therapy of a dissecting aortic aneurysm involving a right-sided aortic arch.

Dissecting aortic aneurysm involving a right-sided aortic arch is apparently quite rare. A patient with this unusual entity is described. By performing an extraanatomic bypass, the aneurysm was excluded between stainless steel staples. The patient was discharged from the hospital 21 days postoperatively. Although the reasons for the extreme rarity of this entity are not clear, precise anatomic definition is required for successful surgical therapy.

Definition of the safe lower limits of aortic resection during surgical procedures on the thoracoabdominal aorta: use of somatosensory evoked potentials.

The technique of intraoperative monitoring of somatosensory evoked potentials was applied to a canine model of spinal cord ischemia in an attempt to determine the safe lower limits of aortic resection during thoracic aortic surgery. Fifteen animals underwent left thoracotomy with institution of partial left atrial/femoral artery bypass for maintenance of distal aortic perfusion after proximal descending thoracic aortic exclusion. In Group I animals (n = 6, control), no further interventions were performed so that the effect of exclusion of vessels noncritical to spinal cord blood supply could be assessed by measurements of spinal cord blood flow and somatosensory evoked potentials. In Group II animals (n = 8), the level of distal aortic exclusion was progressively lowered until loss of somatosensory evoked potential (critical vessel exclusion) occurred. The effect of critical vessel exclusion on spinal cord blood flow was then assessed. Exclusion of multiple vessels noncritical to spinal cord blood supply (Group I) had no effect on spinal cord blood flow or function (somatosensory evoked potentials). Exclusion of vessels critical to spinal cord blood supply resulted in significant spinal cord ischemia (83.4% flow reduction, probability [p] less than 0.05 versus baseline) and ischemic spinal cord dysfunction (loss of somatosensory evoked potential).(ABSTRACT TRUNCATED AT 250 WORDS)

Transfusion-associated-graft-vs-host disease in a presumed immunocompetent patient.

Since the advent of bone marrow transplantation, a vexing clinical problem is that of acute graft-vs-host disease (GVHD). A less well-recognized disorder is that of GVHD in patients receiving blood products containing immunocompetent lymphocytes. Transfusion-associated (TA)-GVHD has a lower incidence and higher mortality (greater than 90%) than bone marrow transplantation-GVHD and until now has been limited to patients with hereditary or acquired immunologic deficits and to patients immunocompromised by chemotherapy for malignant neoplasms. We presently describe a patient who underwent coronary artery bypass graft surgery and who suffered what we believe was TA-GVHD. This diagnosis is supported by considering the chronology of events (in particular, blood transfusion), clinical features (fever, rash, abnormal results of liver function tests, diarrhea, and pancytopenia), and a skin biopsy specimen that revealed basal cell vacuolation and lymphocyte satellitosis that are considered characteristic for this disorder. We believe TA-GVHD can occur in previously immunocompetent patients who receive transfusions of blood products containing functioning lymphocytes and that this awareness will lead to the discovery of additional cases and a better understanding of this disorder.

One-year monkey heart xenograft survival in cyclosporine-treated baboons. Suppression of the xenoantibody response with total-lymphoid irradiation.

The shortage of cadaveric human organs for transplantation could be alleviated by the use of xenografts. Long-term (> one-year) survival of xenografts in humans or experimental animals has not been achieved with previous immunosuppressive protocols. Poor results in xenotransplantation compared with allotransplantation have been attributed to a more potent antibody response rather than to cell-mediated responses. To investigate these issues a concordant heterotopic cardiac xenograft model was developed in conjunction with cyclosporine and/or total-lymphoid irradiation. Concordant models permit examination of xenoantigen induced antibody and cell-mediated responses since preformed humoral factors (in discordant models) do not cause hyperacute rejection. Four groups of baboon recipients received cervical heart transplants from cynomolgous monkeys. Group I (n = 2), untreated, mean survival (MS) = 6 days; group II (n = 5), CsA and methylprednisolone, MS = 25 days; group III (n = 3), preoperative TLI, MS = 29 days; group IV (n = 6), preoperative TLI and CsA+MP, MS = 255 days (> 77, > 108, > 142, 184, > 480, 540 days). Heart xenografts of CsA-MP-treated recipients appear to be destroyed predominantly by antibody (IgM)-mediated processes whereas cell-mediated rejection is likely the major reaction in TLI-treated recipients. CsA-MP-treated recipients had early immunohistochemical evidence of antibody and complement-mediated rejection (deposition of IgM and complement but not IgG on heart xenografts). In contrast IgM and complement deposits were not detected on heart xenografts in TLI- and TLI-CsA-treated recipients. IgG xenoantibodies were only detected on the two rejected heart grafts of TLI-CsA-treated recipients. CsA-MP-treated recipients rapidly developed high xenoantibody titers (1:256 to 1:512) that immediately preceded rejection. In contrast, TLI-treated animals developed lower levels of xenoantibody (< or = 1:8) and TLI-CsA-treated recipients had no detectable xenoantibody during the initial three months after transplantation (and titers no greater than 1:8 thereafter.) The lack of xenoantibody was likely not due to a generalized inhibitory effect of the immunosuppressants on B cell function since all classes of serum immunoglobulins were in the normal range. Intragraft cytolytic lymphocyte activity was detected in rejecting TLI- and TLI-CsA-treated recipients but could not be detected in xenografts of CsA-MP-treated recipients. One explanation for these data is that TLI (either directly or indirectly) induces a state of specific B cell unresponsiveness to monkey xenoantigens, thereby preventing IgM mediated rejection in the third week after transplantation.(ABSTRACT TRUNCATED AT 400 WORDS)

Monitoring of cardiac output by thermodilution after open-heart surgery.

One hundred twenty-five separate cardiac output determinations were obtained after open-heart surgery in 10 patients by simultaneous use of thermodilution and dye-dilution techniques. Mean thermodilution cardiac output was 1.6 per cent greater than mean dye-dilution cardiac output (5.24 versus 5.16 L. per minute). Reproducibility of thermodilution cardiac output (coefficient of variation, 8.6 per cent) was superior to that of dye-dilution cardiac output (coefficient of variation, 12.3 per cent) for outputs ranging from 2.5 to 8.7 L. per minute (p less than 0.001). Linear regression analysis revealed a correlation showing that COtd = 0.86 COdye + 0.80 (r = 0.9, p less than 0.001) and indicating a similarity between thermodilution and dye-dilution output figures except in extremely low output states. In such cases, thermodilution cardiac output becomes progressively larger than dye-dilution cardiac output. The results indicate that thermodilution cardiac output is a valid method for determining cardiac output in the rapidly changing clinical setting following cardiopulmonary bypass. Clinical applications of this technique include evaluation of the efficacy of inotropic agents, effectiveness of intra-aortic balloon counterpulsation, and status of the low output syndrome postoperatively. Routine use in patients with Class III or IV cardiac disease appears justified.

Postoperative ST-segment elevation in coronary artery bypass surgery.

Using Holter monitors, 50 patients were monitored for vasospasm following coronary artery bypass surgery. Transient 2 mm ST-segment elevation was considered to be diagnostic or coronary vasospasm. Four patients (8 percent) had evidence of coronary vasospasm. Over 30 variables, including preoperative demographic information and medication, intraoperative technique, and postoperative medication, were subjected to multiple stepwise regression analysis. This analysis failed to show any association between preoperative prophylaxis with either nifedipine or nitrates (or other variables) and the postoperative development of coronary vasospasm. We conclude that the incidence of coronary vasospasm is more common than previously thought, and that a nifedipine or nitrate withdrawal, in this study, was not associated with an increased incidence of postoperative coronary vasospasm.

Monitoring of somatosensory evoked potentials during surgical procedures on the thoracoabdominal aorta. IV. Clinical observations and results.

Thirty-three patients undergoing operations on the descending thoracic or thoracoabdominal aorta were monitored to evaluate causes and effects of spinal cord ischemia as manifested by changes in somatosensory evoked potentials. Maintenance of distal aortic perfusion pressure (greater than 60 mm Hg) by either shunt or bypass techniques in 17 patients resulted in preservation of somatosensory evoked potentials and a normal postoperative neurologic status, irrespective of the interval of thoracic cross-clamping (range 23 to 105 minutes). In 16 other patients in whom cross-clamp time ranged from 16 to 124 minutes, evoked potential loss was observed because of failure to provide distal perfusion (n = 8), inadequate maintenance of distal perfusion pressure (less than 60 mm Hg) despite shunt/bypass (n = 6), or interruption of critical intercostal arteries (n = 2). Incidence of paraplegia in the entire group was 15.1% (5/33) and was limited to only those patients in whom evoked potential loss occurred (5/16, 31.2%) (p = 0.02). Loss of somatosensory evoked potentials for more than 30 minutes resulted in a 71.2% (5/7) incidence of paraplegia, whereas no neurologic deficit was noted in patients (0/26) in whom evoked potential loss was either prevented or limited in duration to 30 minutes (p less than 0.001 versus loss for more than 30 minutes). Intraoperative monitoring of somatosensory evoked potentials is a sensitive indicator of spinal cord ischemia. Simple aortic cross-clamping, failure to maintain distal perfusion pressure above 60 mm Hg, and inability to reimplant critical intercostals in a timely fashion result in a high rate of paraplegia if duration of spinal cord ischemia as measured by somatosensory evoked potentials exceeds 30 minutes. Routine evoked potential monitoring during thoracoabdominal procedures appears useful in assessing the adequacy of spinal cord perfusion. Furthermore, it can alert the surgeon to the necessity for critical intercostal artery reimplantation as well as the need for adjustment or regulation of distal aortic perfusion.

Reperfusion ventricular fibrillation during coronary artery bypass operations and its association with postoperative enzyme release.

Elevated creatine kinase MB level is the most common marker of myocardial infarction in patients who have had a recent coronary bypass operation. This study was performed to determine whether there is any relationship between reperfusion rhythms, their treatment, and postoperative creatine kinase MB concentrations. Twenty patients were monitored during coronary bypass operations. Four patients had no reperfusion ventricular fibrillation and no elevation of creatine kinase MB postoperatively. Of the 16 remaining patients, all had ventricular fibrillation and 12 had an elevation of postoperative creatine kinase MB (p less than 0.015). There was also a 75% correlation between the time in ventricular fibrillation and postoperative creatine kinase MB level. There was no correlation between other measured parameters, such as cross-clamp time, bypass time, or the number of defibrillations. It is concluded that reperfusion ventricular fibrillation is associated with release of creatine kinase MB, and the time in ventricular fibrillation is correlated with the postoperative creatine kinase MB level.

Use of profound hypothermia and circulatory arrest for the calcified aorta.

This case report discusses one method of dealing with the extensively calcified aorta in patients undergoing open heart surgery. Profound hypothermia and circulatory arrest was used in a patient undergoing aortic valve replacement with severe calcification of the ascending aorta and transverse arch. This patient recovered from surgery and was discharged from the hospital with no neurologic deficits.

Myocardial protection during prolonged aortic cross-clamping. Comparison of blood and crystalloid cardioplegia.

We compared the ability of blood and crystalloid cardioplegia to protect the myocardium during prolonged arrest. Twelve dogs underwent 180 minutes of continuous arrest. Group I (six dogs) received 750 ml of blood cardioplegic solution (potassium chloride 30 mEq/L) initially and every 30 minutes. Group II (six dogs) received an identical amount of crystalloid cardioplegic solution (potassium chloride 30 mEq, methylprednisolone 1 gm, and 50% dextrose in water 16 ml/L of electrolyte solution). Temperature was 10 degrees C and pH 8.0 in both groups. Studies of myocardial biochemistry, physiology, and ultrastructure were completed before arrest and 30 minutes after normothermic reperfusion. Biopsy specimens for determination of adenosine triphosphate were obtained before, during, and after the arrest interval. Regional myocardial blood flow, total coronary blood flow, and myocardial oxygen consumption were statistically unchanged in Group I (p greater than 0.05). Total coronary blood flow rose 196% +/- 49% in Group II (p less than 0.005), and left ventricular endocardial/epicardial flow ratio fell significantly in this group from 1.51 +/- 0.18 to 0.8 +/- 0.09, p less than 0.01 (mean +/- standard error of the mean. The rise in myocardial oxygen consumption was not significant in this group (34% +/- 36%, p greater than 0.05). Ventricular function and compliance were statistically unchanged in both groups. In Group II, adenosine triphosphate fell 18% +/- 3.4% (p less than 0.005) after 30 minutes of reperfusion; it was unchanged in Group I. Ultrastructural appearance in both groups correlated with these changes. We conclude that blood cardioplegia offers several distinct advantages over crystalloid cardioplegia during prolonged arrest.

Monitoring of somatosensory evoked potentials during surgical procedures on the thoracoabdominal aorta. I. Relationship of aortic cross-clamp duration, changes in somatosensory evoked potentials, and incidence of neurologic dysfunction.

To determine if intraoperative monitoring of somatosensory evoked potentials detects spinal cord ischemia, we subjected 21 dogs to aortic cross-clamping distal to the left subclavian artery. Group I animals (short-term studies, n = 6) demonstrated decay and loss of somatosensory evoked potentials at 8.5 +/- 1.1 minutes after aortic cross-clamping. During loss of somatosensory evoked potentials, significant decreases in spinal cord blood flow occurred in cord segments below T6. Significant reactive hyperemia occurred without normalization of somatosensory evoked potentials after reperfusion. Fifteen Group II animals (long-term studies) were studied to determine the relationship between duration of spinal cord ischemia (evoked potential loss) and subsequent incidence of paraplegia. Extension of aortic cross-clamping for 5 minutes after loss of somatosensory evoked potentials in six dogs resulted in no paraplegia (mean cross-clamp time 12.7 +/- 0.6 minutes). Prolongation of aortic cross-clamping for 10 minutes after evoked potential loss in nine dogs (mean cross-clamp time 17.6 +/- 0.6 minutes) resulted in a 67% (6/9) incidence of paraplegia 7 days postoperatively (p = 0.02 versus 10 minutes of aortic cross-clamping). These findings demonstrate that simple aortic cross-clamping uniformly results in spinal cord ischemia and that such ischemia is detectable by monitoring of somatosensory evoked potentials. Duration of ischemia, as measured by the time of evoked potential loss during the cross-clamp interval, is related to the incidence of postoperative neurologic injury.

Monitoring of somatosensory evoked potentials during surgical procedures on the thoracoabdominal aorta. II. Use of somatosensory evoked potentials to assess adequacy of distal aortic bypass and perfusion after thoracic aortic cross-clamping.

Pulsatile left atrial-femoral artery bypass was instituted after aortic cross-clamping distal to the left subclavian artery in a canine experimental model to determine the relationship of distal aortic perfusion pressure with spinal cord blood flow and somatosensory evoked potentials. In six animals (Group I) distal aortic perfusion pressure was maintained at 100 mm Hg throughout a 1 hour interval of aortic cross-clamping. During this period, somatosensory evoked potentials and spinal cord blood flow (radioactive microspheres) showed no significant change from baseline. In six other dogs (Group II) distal aortic perfusion pressure was initially maintained at 100 mm Hg after aortic cross-clamping and then progressively decreased to 70, 40, and 25 mm Hg. Somatosensory evoked potentials and spinal cord blood flow were preserved at baseline levels for all distal perfusion pressures greater than 70 mm Hg. At 40 mm Hg, abnormalities in amplitude of the somatosensory evoked potentials were noted in all animals with progression to complete loss of evoked potential activity at lower perfusion pressures. Maintenance of adequate somatosensory spinal cord conduction after thoracic aortic cross-clamping is dependent on a critical level of distal aortic perfusion that can be accomplished by use of an adjunct such as pulsatile left atrial-femoral artery bypass. The critical level of distal aortic perfusion pressure to maintain normal somatosensory evoked potentials and spinal cord blood flow in this canine experimental study was 70 mm Hg or greater. Because inadequate distal aortic perfusion can be easily detected by monitoring of somatosensory evoked potentials, these techniques should prove helpful in evaluating the effectiveness of distal perfusion techniques during clinical aortic cross-clamping for procedures on the thoracoabdominal aorta.

Preservation of myocardial ATP. Comparison of blood vs crystalloid cardioplegia.

Preservation of myocardial high-energy phosphates correlates with the heart's ability to resume normal function following aortic crossclamping (AXC). The ability of the canine myocardium to synthesize and maintain ATP during 180 minutes of AXC was evaluated in 12 hearts subjected to either blood or crystalloid cardioplegic arrest. Group 1 hearts were arrested by infusion of 750 ml of blood potassium cardioplegia (BKC) solution into the aortic root initially and every 30 minutes, as were group 2 (six) hearts but with a crystalloid cardioplegia (CC) solution. Transmural left ventricular biopsy specimens were obtained for ATP analysis prior to AXC (control), before and after cardioplegia injections 2, 4, and 6, prior to unclamping (180 minutes of AXC), and 30 minutes following reperfusion. ATP levels increased significantly above control (p less than 0.005) during the 180 minutes of AXC immediately following infusion of BKC. At the end of 180 minutes of AXC and following 30 minutes of reperfusion, ATP was noted to be normal in this group (p = NS). In contrast, ATP levels fell significantly (p less than 0.005) during the period of aortic cross-clamping in the crystalloid cardioplegia group and did not return to normal even after 30 minutes of reperfusion (p less than 0.005). We concluded that BKC, by presenting the arrested myocyte with adequate oxygen and substrate, allows for synthesis and preservation of myocardial ATP during periods of AXC as long as three hours. In this respect, it should be regarded as superior to CC, which permits a statistically significant depletion of ATP (p less than 0.005) uncorrected, even after 30 minutes of reperfusion in the beating, nonworking state.

Constant-pressure aortic root perfusion versus cardioplegia and hypothermia. Comparison of methods of myocardial protection.

Both coronary perfusion and hypothermic cardioplegia are widely used methods of myocardial protection during aortic valve replacement. A theoretical objection to coronary perfusion is that it is not synchronized with cardiac contractions. Accordingly, a special pump was designed to provide perfusion at a constant range of pressure. Twenty dogs were studied during 4 hours of bypass. In six dogs no manipulations were carried out and hearts were allowed to beat in a nonworking state. Seven dogs underwent 2 hours of aortic cross-clamping and constant-pressure aortic root perfusion. Seven dogs underwent 2 hours of uninterrupted aortic occlusion with myocardial protection being maintained by cold potassium-induced arrest, Contractility did not change significantly in any of the three groups. All animals demonstrated significant hyperemia after bypass but normal endocardial/epicardial flow ratios. Although compliance deteriorated in all groups, the most striking changes were seen following 4 hours of bypass alone or constant-pressure aortic root perfusion. Hypothermic potassium arrest, in contrast, provided a slightly greater degree of myocardial protection, perhaps both by limiting the degree of ischemic injury directly and by excluding the heart from the circulating blood and the pump oxygenator system.

Pseudocoarctation and mid-arch aortic coarctation.

A 21-year-old woman was found to have a mid-arch aortic coarctation in combination with pseudocoarctation. The angiographic disgnosis was established by left atrial injection after transseptal puncture.

Coronary artery bypass with freeze-preserved saphenous vein allografts.

Over the past 5 years, 13 patients had coronary artery bypass performed with freeze-preserved saphenous vein allografts. There were no operative deaths or significant morbidity. Six patients were studied postoperatively at 42, 37, 10, 7, 5, and 1 months. Six of 8 grafts were patent with good flow. There were four late deaths; two of these occurred in patients who had concomitant resection of a ventricular aneurysm. Of the 9 surviving patients, 6 (6/9) are asymptomatic and 2 (2/9) have occasional chest pains; the condition of 1 patient (1/9) is unchanged. This experience suggests that free-preserved saphenous vein allografts may be used successfully for coronary bypass when autologous veins and internal mammary arteries are unavailable or insufficient for multiple bypass.

Influence of primary closure of the pericardium after open-heart surgery on the frequency of tamponade, postcardiotomy syndrome, and pulmonary complications.

Experiences with primary closure of the pericardium in a series of 100 patients undergoing open-heart operations are described. The pericardium was kept under tension during the operation to minimize shrinkage and permit closure at the end of the procedure. In 28 patients one pleural space was opened for drainage, whereas in 72 patients intra- and extrapericardial sumps alone were used for drainage. Measurements of sump drainage revealed that most postoperative bleeding originates from outside the pericardium. There were no instances of cardiac tamponade although 19 patients lost more than 1 L. of blood after operation and 5 required reoperation for hemorrhage. Transpleural drainage tubes were shown to be ineffective and in addition were associated with a fourfold increase in postcardiotomy syndrome and a significantly greater frequency of pleural effusion and atelectasis when compared to the use of mediastinal sump drainage alone. We have concluded that closing the pericardium and using mediastinal sump drainage minimizes the risk of cardiac tamponade and allows early localization of the site of postoperative bledding. Another advantage of pericardial closure and drainage is that postoperative adhesions and postcardiotomy syndrome will be less significant. As a consequence the danger of injuring the heart in a subsequent operation is lessened.

Paraplegia following percutaneous insertion of an intra-aortic balloon.

Paraplegia following insertion of an intra-aortic balloon is an extremely rare and unusual complication with only one previous report in the literature. We recently encountered this problem in a man with severe coronary disease and unstable angina. The etiology of this complication, although never established in our patient, was most likely a critical occlusion of a spinal cord artery as a result of either a small dissection or an arterial embolus.