Esophageal hypermotility: cause or effect?

Nutcracker esophagus (NE), Jackhammer esophagus (JHE), distal esophageal spasm (DES), and hypertensive lower esophageal sphincter (HTLES) are defined by esophageal manometric findings. Some patients with these esophageal motility disorders also have abnormal gastroesophageal reflux. It is unclear to what extent these patients' symptoms are caused by the motility disorder, the acid reflux, or both. The aim of this study was to determine the effectiveness of laparoscopic Nissen fundoplication (LNF) on esophageal motility disorders, gastroesophageal reflux, and patient symptoms. Between 2007 and 2013, we performed high-resolution esophageal manometry on 3400 patients, and 221 patients were found to have a spastic esophageal motility disorder. The medical records of these patients were reviewed to determine the manometric abnormality, presence of gastroesophageal symptoms, and amount of esophageal acid exposure. In those patients that underwent LNF, we compared pre- and postoperative esophageal motility, gastroesophageal symptom severity, and esophageal acid exposure. Of the 221 patients with spastic motility disorders, 77 had NE, 2 had JHE, 30 had DES, and 112 had HTLES. The most frequently reported primary and secondary symptoms among all patients were: heartburn and/or regurgitation, 69.2%; respiratory, 39.8%; dysphagia, 35.7%; and chest pain, 22.6%. Of the 221 patients, 192 underwent 24-hour pH monitoring, and 103 demonstrated abnormal distal esophageal acid exposure. Abnormal 24-hour pH monitoring was detected in 62% of patients with heartburn and regurgitation, 49% of patients with respiratory symptoms, 36.8 % of patients with dysphagia, and 32.6% of patients with chest pain. Sixty-six of the 103 patients with abnormal 24-hour pH monitoring underwent LNF. Thirty-eight (13NE, 2JHE, 6 DES, and 17 HTLES) of these 66 patients had a minimum of 6-month postoperative follow-up that included clinical evaluation, esophageal manometry, and 24-hour pH monitoring. Postoperatively, all 38 patients had normal distal esophageal acid exposure. Of these 38 patients, symptoms resolved in 28 and improved in 10. Of six patients (one with NE, two JHE, and three with HTLES) that underwent postoperative esophageal manometry, five exhibited normal motility. Typical reflux symptoms are common among patients with esophageal hypermotility disorders. Abnormal 24-hour pH monitoring is present in the majority of patients with who report typical reflux symptoms and almost half of patients who report respiratory symptoms. Conversely, the majority of patients who report dysphagia or chest pain have normal distal esophageal acid exposure. Based on a small number of patients in this study, it also appears that motility disorders often improve after LNF. LNF is associated with resolution or improvement in reflux related symptoms and esophageal motility parameters in patients exhibiting abnormal esophageal acid exposure. This suggests that patient symptoms are due to abnormal acid exposure and not the motility disorder.

The frequency of body dysmorphic disorder in dermatology and cosmetic dermatology clinics: a study from Turkey.

BACKGROUND: Body dysmorphic disorder (BDD) is a distressing and impairing preoccupation with a slight or imagined defect in appearance. There are few reports on the prevalence of BDD in the Turkish population. AIM: To investigate the frequency of BDD in dermatology settings, and to compare the results from cosmetic dermatology with those from general dermatology settings. METHODS: This cross-sectional study recruited 400 patients from cosmetic dermatology (CD) (n = 200) and general dermatology (GD) clinics (n = 200). A mini-survey was used to collect demographic and clinical characteristics, and the dermatology version of a brief self-report BDD screening questionnaire was administered. A five-point Likert scale was used for objective scoring of the stated concern, which was performed by dermatologists, and patients who scored ≥ 3 were excluded from the study. RESULTS: In total, 318 patients (151 in the CD group and 167 in the GD group) completed the study, and of these, 20 were diagnosed with BDD. The CD group had a higher rate of BDD (8.6%) than the GD group (4.2%) but this was not significant (P = 0.082). The major concern focused on body and weight (40.0%), followed by acne (25.0%). CONCLUSIONS: The number of cosmetic procedures in dermatology practices is increasing Therefore, it is becoming more important to recognize patients with BDD. Although the rates of BDD found in the present study are in agreement with the literature data, population-based differences still exist between this study and previous studies.

Carotid artery Doppler flow pattern after deep hypothermic circulatory arrest in neonatal piglets.

The mechanisms of cerebral injury after cardiac surgery in neonates are not clear. The aim of the study was the analysis of flow changes in the carotid artery of neonatal piglets after deep hypothermic circulatory arrest (DHCA). Eight neonatal piglets were connected to cardiopulmonary bypass (CPB) and underwent (i) cooling to 18°C core temperature within 30 min, (ii) DHCA for 90 min, and finally (iii) rewarming to 37°C after cross-clamp release (60 min of reperfusion). The blood flow was measured in the left carotid artery by an ultrasonic flow probe before CPB (baseline; T0 ), immediately after termination of reperfusion on CPB (T1 ), 30 min later (T2 ), and 60 min later (T3 ). Additionally, the pulsatility index and the resistance index were calculated and compared. Finally, the relationship between the carotid artery flow and the corresponding pressure at each time-point was compared. After termination of CPB (T1 ), the mean carotid artery flow was reduced from 45.26 ± 2.58 mL/min at baseline to 23.29 ± 2.58 mL/min (P < 0.001) and remained reduced 30 and 60 min later (P < 0.001 vs. baseline). Both the pulsatility index and the resistance index were increased after termination of reperfusion, with the maximum occurring 30 min after CPB end. In conclusion, the carotid artery Doppler flow in neonatal piglets was reduced after DHCA, while the indices of pulsatility and resistance increased.

Aortic surgery after previous procedure of congenital aortic stenosis.

Irrespective of previous procedure in congenital aortic stenosis, aortic surgery later in life may be indicated. The aim of the present study was the analysis of indications, risks, and outcomes of aortic surgery after previous aortic valve procedure. The data of patients who underwent aortic surgery after previous treatment of congenital aortic stenosis in a 10-year period (from 2000 to 2009) were retrospectively analyzed. Thirty-two patients (23 male and 9 female) underwent redo aortic surgery. The mean age at surgery was 13.5 ± 11.3 years. Seventeen patients had undergone initial aortic balloon valvuloplasty (BVP) and 15 patients open commissurotomy (COM). Nine cases had undergone the primary procedure at neonatal age and two patients had undergone cardiac surgery before the initial aortic valve procedure. Seven of the patients with previous COM (43.8%) had undergone concomitant surgery along with initial commissurotomy. A reintervention within the first year after the primary procedure was performed in seven patients (seven after BVP and none after COM; P < 0.05). The interval between the last intervention and the first redo aortic surgery was 7.5 ± 9.5 years (3.1 ± 3.5 years after BVP vs. 12.5 ± 11.7 years after COM; P < 0.05). A second redo surgery was performed in nine patients (four after initial BVP and five after COM). Congenital aortic stenosis is very often presented in combination with additional pathologies. These concomitant diseases along with the underlying disease give the indication for reoperation. Reinterventions are more often indicated after primary BVP. Long follow-up in specialized centers is mandatory.

Carotid Doppler flow after cardiopulmonary bypass and mild hypothermia in neonatal piglets.

Although the mechanisms of neurological disorders after cardiac surgery in neonates are still not fully understood, alterations in blood flow after cardiopulmonary bypass (CPB) may lead to cerebral injury. The aim of the study was the analysis of flow changes in the carotid artery of neonatal piglets after CPB. Ten neonatal piglets (younger than 7 days) were connected to the CPB and further management underwent three steps: (i) cooling to 32°C core temperature within 30 min; (ii) cardiac arrest under cardioplegic myocardial protection for 90 min; and (iii) rewarming to 37°C after cross-clamp release (60 min of reperfusion). In summary, piglets were separated from CPB after a total duration time of 180 min. The blood flow was measured in the left carotid artery by an ultrasonic flow probe before CPB (baseline), immediately after CPB, 30 min, and 60 min after CPB. Additionally, the pulsatility index and the resistance index were calculated and compared. Finally, the relation of the carotid artery flow data with the corresponding pressure data at each time point was compared. After termination of CPB, the carotid artery mean flow was reduced from 28.34 ± 13.79 mL/min at baseline to 20.91 ± 10.61 mL/min and remained reduced 30 and 60 min after CPB termination (19.71 ± 11.11 and 17.64 ± 15.31 mL/min, respectively). Both the pulsatility and the resistance index were reduced immediately after CPB termination and increased thereafter. Nevertheless, values did not reach statistical significance. In conclusion, the carotid Doppler flow immediately after CPB and mild hypothermia in neonatal piglets was lower than before CPB due to reduced vascular resistance. Additionally, the pressure-flow relation revealed that immediately after CPB, a higher pressure is required to obtain adequate flow.

Natural behaviour and surgical treatment of appendiceal carcinoids: an analysis of 2,376 consecutive emergency appendectomies.

OBJECTIVE: Carcinoid tumor of appendix is a rare condition. Its rarity may give rise to variances in its management. We aimed to demonstrate the occurrence and form of presentation of carcinoid tumor of appendix, as well as variations in its treatment. METHODS: All appendicectomies that took place between 2000 and 2008 were considered for study. RESULTS: A total of 2,376 appendicectomies were performed during this period while carcinoid tumours were diagnosed in 27 patients (1.13%). The mean age of patients with carcinoid tumor (26.7 years) was almost the same as that of patients with non-carcinoid pathology (28.1 years). The incidence of male patients was higher than that of females amongst the carcinoid tumor group (female/male ratio: 1/4). None of the carcinoid tumors were identified at operation. One patient (3.7 %) required right hemicolectomy. This patient was followed-up in an inconsistent manner. CONCLUSIONS: Carcinoid tumour of the appendix remains an incidental diagnosis. Patients with carcinoid were significantly younger than those with non-carcinoid conditions in the study. Re-operation rate was low. The interval to definitive surgery was very short and only one patient was followed up. No consideration as to whether the surgery was complete or not was done in the study (Tab. 1, Fig. 1, Ref. 29).

Myocardial contractile function in survived neonatal piglets after cardiopulmonary bypass.

BACKGROUND: Hemodynamic function may be depressed in the early postoperative stages after cardiac surgery. The aim of this study was the analysis of the myocardial contractility in neonates after cardiopulmonary bypass (CPB) and mild hypothermia. METHODS: Three indices of left ventricular myocardial contractile function (dP/dt, (dP/dt)/P, and wall thickening) were studied up to 6 hours after CPB in neonatal piglets (CPB group; n = 4). The contractility data were analysed and then compared to the data of newborn piglets who also underwent median thoracotomy and instrumentation for the same time intervals but without CPB (non-CPB group; n = 3). RESULTS: Left ventricular dP/dtmax and (dP/dtmax)/P remained stable in CPB group, while dP/dtmax decreased in non-CPB group 5 hours postoperatively (1761 ± 205 mmHg/s at baseline vs. 1170 ± 205 mmHg/s after 5 h; p < 0.05). However, with regard to dP/dtmax and (dP/dtmax)/P there were no statistically significant differences between the two groups. Comparably, although myocardial thickening decreased in the non-CPB group the differences between the two groups were not statistically significant. CONCLUSIONS: The myocardial contractile function in survived neonatal piglets remained stable 6 hours after cardiopulmonary bypass and mild hypothermia probably due to regional hypercontractility.

Extracorporeal life support in pediatric patients with congenital heart diseases: outcome of a single centre.

AIM: In pediatric patients with congenital heart disease low cardiac output (LCO) is the principal complication after corrective heart surgery. In LCO refractory to all therapeutic options, mechanical circulatory support is the final method to keep these patients alive. In this present study the authors reviewed the outcome of pediatric patients who required mechanical circulatory support after corrective surgery with extracorporeal membrane oxygenation or ventricle assisted devices (VAD). METHODS: A retrospective single centre consecutive cohort study was carried out in children who required different mechanical circulatory support indicated by postcardiotomy low output syndrome between 1991 and 2004. A total of 20 patients received extracorporeal life support. The indications for surgery were: 12 transposition of great arteries, 1 Bland-White-Garland syndrome, 3 tetralogy of Fallot, 1 hypoplasia of aortic arch, 1 total anomalous pulmonary vein connection, and 2 ventricle septum defect. RESULTS: Mean age was 1.29 years. Mean duration of assist was 8.87 days. Seven patients out of 20 survived, six could be discharged after myocardial recovery from LCO and one could be discharged after successful heart transplantation. The overall mortality in patients with extracorporeal life support was 65%. The causes of death were multiorgan failure and bleeding in one case was a VAD related complication. CONCLUSION: The use of extracorporeal life support (ECLS) shows a high mortality rate. However, ECLS can still help to keep some of those patients alive. Mechanical support devices are the ultimate chance to save time, to increase survival and to bridge the time until heart transplantation.

Clinical relevance of eNOS T-786C polymorphism for hospital mortality and morbidity in cardiac surgical patients.

AIM: The endothelial nitric oxide (eNOS) gene T-786C polymorphism may influence as a genetic risk factor cardiovascular diseases and shows association with cardiovascular mortality. We hypothesized that this polymorphism may lead to increase mortality and morbidity after cardiac surgery with cardiopulmonary bypass (CPB). METHODS: In 500 patients who underwent cardiac surgery with CPB we investigated the eNOS T-786C polymorphism by DNA-sequencing. The patients were grouped according to their genotype in three groups (TT, TC, and CC). RESULTS: The overall genotype distribution of T-786C polymorphism was TT=41.6%, TC=51.2%, and CC=7.2% respectively. The groups did not differ in age and gender. No significance was shown in preoperative risk factors, excluding peripheral disease (P=0.03). No difference was shown in Euroscore, APACHE II, and SAPS II. The usage of norepinephrine (P=0.03) and nitroglycerine (P=0.01) was significant higher in TC allele carrier. The mortality was quite uniform across elective and urgent subgroup. However, we found a significant difference concerning mortality and emergency cardiac procedures in homozygous C-allele carrier (P=0.014). CONCLUSION: The present study demonstrates that this polymorphism contributes to a higher prevalence of postoperative mortality after emergency cardiac surgery. Thus, the eNOS T-786C polymorphism could serve as a possibility to differentiate high risk subgroups in heterogeneous population of individuals with cardiac diseases who need cardiac surgery with CPB.

Reoperations in adults after correction of tetralogy of Fallot.

Adults with congenital heart diseases are an increasing patient population. Since the first successful palliative procedure in 1945 (Blalock and Tausing, JAMA 128: 189-194, 1945) and first corrective operation in 1955 (Lillehei et al., Ann Surg 142: 418, 1955) patients with Tetralogy of Fallot, which is the most common cyanotic CHD, have had excellent long term results and good quality of life. Over the long term, these patients are under risk of sudden death because of arrhythmias and congestive heart disease caused by tricuspid regurgitation, pulmonary regurgitation, ventricular septal defect, right ventricular outflow tract obstruction.

Ischemia-reperfusion injury in the liver during renal transplantation: does perfusion solution play any role?

The effect of ischemia-reperfusion (I/R) injury within a transplanted kidney has not been reported on the liver as a remote organ. One hypothesis is that there is no difference between kidney perfusion solutions regarding antioxidants in liver after an I/R injury. We used four pigs with Ringer's lactate (RL); four with university of Wisconsin (UW); and two in a control (C) group. A liver parenchymal biopsy was obtained before renal artery/vein solution clamping for 20 minutes. Either RL or UW solutions were infused through arterial cannulas for 20 minutes as previously described elsewhere. For the sham group, we used 0.9% NaCl. After reperfusion for 20 minutes, we obtained a second liver parenchymal biopsy. Measurements of superoxide dismutase (SOD), glutathione peroxidase (GP-x), and malondialdehyde (MDA) levels were compared using paired student t tests within groups and analysis of variance between groups. The results were expressed as mean values +/- SEM with P < .05 accepted as significant. Although GP-x, SOD, and MDA decreased after ischemia-perfusion-reperfusion injuries in all groups, except MDA in UW and SOD, and MDA in C groups; only the MDA for C was significant (P = .04) Comparing the groups, GP-x (P = .01) and MDA (P = .003) levels after ischemia-perfusion-reperfusion were significant while changes in SOD levels did not show any difference (P > .05). In a kidney transplantation model, the liver was affected during the ischemia-perfusion-reperfusion process as evidenced by antioxidant enzymes. The pathophysiology and clinical importance of this phenomenon requires further study. Comparing the perfusion solutions, no difference was found between RL and UW regarding their effects to decrease renal I/R injury on the liver in pigs.

Cardiac surgery after heart transplantation: coronary artery bypass grafting and heart valve replacement.

INTRODUCTION: Due to increasing need for and a shortage of donor organs, therapeutic procedures such as heart valve replacement for valve insufficiency and coronary artery bypass grafting (CABG) for graft vasculopathy (GVP) must be performed to improve allograft function to avoid retransplantation. METHODS: We performed a retrospective analysis of patients who underwent surgical procedures after orthotopic heart transplantation. Since 1989, we have performed more than 1400 heart transplantation procedures. Valve replacement was necessary in 8 patients and CABG was necessary in 3 patients. Five patients received valve prostheses (3 bioprostheses and 2 mechanical valves) at the tricuspid position. Three patients received a Hancock bioprosthesis at the mitral position. One of the 3 received the valve 3 years after heart transplantation while suffering from mitral regurgitation grade IV, and another patient received the valve 1 year following heart transplantation while suffering from mitral insufficiency grade III due to infective endocarditis. Three patients underwent coronary artery revascularization, 2 patients underwent the procedure 1 and 7 years after heart transplantation because of GVP, 1 patient underwent the procedure simultaneously with heart transplantation because of donor coronary artery disease. One patient received concomitant CABG with heart transplantation because of 75% left anterior descending stenoses in the donor organ, and one patient received CABG 1 year after heart transplantation because of rapidly progressive GVP in the left anterior descending artery. The third patient had 3-vessel disease with 95% left stem and 75% ramus circumflex, ramus marginalis, and ramus diagonalis. RESULTS: Two patients who underwent CABG and 4 patients who underwent valve replacement are still alive and maintain good clinical performance. One patient with a graft at the mitral position died 9 years after heart transplantation and 6 years after mitral valve replacement. Two patients with a graft at the tricuspid position died 17 and 4 years after heart transplantation (6 and 3 years after valve replacement, respectively). One patient with a bioprostheses at the tricuspid position had to be retransplanted 2 years following valve replacement while suffering from a paravalvular leakage grade III. CONCLUSION: Cardiac surgical procedures can be safely performed after heart transplantation. To improve graft and patient survival, such procedures must be carefully performed after heart transplantation to avoid retransplantation. The shortage of donor organs will and must lead to an increase in the number of conventional procedures performed to improve allograft function in transplanted hearts.

Pediatric patients with Kawasaki disease and a case report of Kitamura operation.

Kawasaki disease (KD), also called mucocutaneous lymph node syndrome, is an acute, self-limiting, small-vessel vasculitis with an unknown cause that affects children between the ages of 6 months and 5 years. It is the most common cause of acquired coronary artery disease in childhood. Acute myocardial infarction and coronary artery aneurysm are major complications. We present a cohort of patients with KD who were followed up and treated in the Heart Center, North Rhine-Westphalia. Included is a review of important relevant items common to cases of KD, such as clinical data and management, including medical management of the acute condition and the diagnosis and management of coronary vasculitis and aneurysms as well as the application of coronary artery bypass grafting (CABG) in those conditions. Between January 2002 and January 2006, we evaluated the findings and characteristics of 18 pediatric patients with a history of KD and their long-term outcome. The acute illness occurred between the ages of 4 months and 14 years of age. Anomalies of the coronary arteries were found in 6 patients ranging in age from 5 months to 10 years. One patient had acute myocardial infarction; another underwent CABG after 5 years from disease onset at the age of 15 years. Kitamura operation was performed successfully. The other patients are still under observation.Coronary artery aneurysms and stenosis requiring surgery are rare in KD; nevertheless, CABG is the standard therapy when myocardial ischemia is detected. Kitamura operation provides good growth potential and long-term graft patency.

Effects of perfusion solutions on kidney ischemia-reperfusion injury in pigs.

During ischemia-reperfusion, free oxygen radicals which directly affect renal cells may cause delayed graft function. We investigated whether there was a difference regarding antioxidant enzyme content between use of Ringer's lactate (RL) versus University of Wisconsin (UW) perfusion solutions in kidney transplantation. Ischemia was achieved by clamping the renal pedicle for 20 minutes followed by perfusion with either solution for 20 minutes and reperfusion for another 20 minutes. A parenchymal biopsy was taken before and after the ischemia, perfusion, and reperfusion (IPR) process. The levels of superoxide dismutase (SOD), glutathione peroxidase (GPx), and malondialdehyde (MDA) were investigated in the biopsy specimens. We used paired t tests within groups and t tests for comparisons between groups. The results were expressed as mean values +/- SEM with P < .05 accepted as statistically significant. After IPR, SOD, GPx, and MDA were decreased in all groups: only GPx (P = .001) and MDA (P = .04) for the RL group and SOD (P = .001) and MDA (P = .05) for the UW group were statistically significant. In the control group, we did not observe any difference (P > .05). Comparisons between groups did not reveal differences (P > .05). In our study, no difference was observed between RL and UW regarding their effects on antioxidant enzymes following renal I/R injury in pigs. More investigations are needed to evaluate graft function in this setting.

Management of tight filum terminale syndrome with special emphasis on normal level conus medullaris (NLCM).

BACKGROUND: Tethered cord syndrome is classically defined as having the tip of the conus medullaris below the body of L2, instead of at the normal L1-2 disc space level. The syndrome presents most commonly with urinary incontinence. In a patient with urinary incontinence and a low-lying conus medullaris, tethered cord syndrome can be diagnosed without difficulty. However, in a patient with urinary incontinence and a hyperreflexive type of neurogenic bladder, in whom the conus medullaris is found to be at the normal level on magnetic resonance imaging, diagnosing a tethered cord can be challenging. We hypothesized that patients with hyperreflexive neurogenic bladders have a neurologic basis for their bladder dysfunction, probably secondary to tethering by a tight filum terminale, despite having a normally positioned conus medullaris. METHODS: We investigated 13 patients with tethered cord syndrome with urinary incontinence. All patients underwent urodynamic tests, somatosensory-evoked potentials (SSEPs), and magnetic resonance imaging. All had normal neurologic exams including lower extremity motor, sensory, and reflex functions. To minimize disturbance of the normal anatomy, we used a flavotomy approach and transected the tight filum terminale to release the tethered conus medullaris. RESULTS: We show that urodynamic studies appear to be more predictive of a tight filum terminale than SSEPs. However, pathological SSEP results seem to correlate with poor surgical outcomes. Ninety-three percent of operated patients were continent by the first post-surgical day. However, disappointingly, almost half lost continence within the first month after surgery. CONCLUSION: In cases of incontinence with a normal level conus medullaris, but urodynamic studies showing a hyperreflexic neurogenic bladder, we believe that sectioning of the filum terminale often improves continence.