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Waardenburg syndrome is a genetic disease characterized by hearing loss and pigmentation abnormalities. Waardenburg syndrome type 4 is very rare, and children with Waardenburg syndrome type 4 present with intestinal aganglionosis. The associated findings and severity of Waardenburg syndrome type 4 may also differ significantly between cases. Intestinal insufficiency is probable and creates difficulties in terms of treatment; intestinal transplant may be required. In this case report, we present 4 cases of patients with Waardenburg syndrome who have intestinal issues, 2 of whom underwent small bowel transplant. Appropriate surgical and nutritional management should be provided for patients with Waardenburg syndrome type 4 who have gastrointestinal manifestations.

Penetrating injuries of the carotid and vertebral arteries are rare, but life-threatening conditions. There are still challenges in the first intervention management and patient treatment. Deciding which methods to apply in the first intervention, whether to perform imaging, and the preferred appropriate treatment for the patient (open surgery or endovascular intervention) plays a main role in the patient's survival without neurological deficit. The general management of trauma in penetrating neck injuries and the knowledge of special approaches to carotid artery injury are important for pediatric trauma centers. In this case report, we presented a case of penetrating carotid artery injury in a child who has recovered with no disability after a successful first intervention, surgical repair, and proper postoperative care.

WOS: 000463369400008, PubMed ID: 30666970, Background/Alms: Pediatric intestinal pseudo-obstruction (PIPO) is a severe disorder of gut motility. In this rare and difficult-to-manage disease, complex treatment method, such as intestinal transplantation, is sometimes needed. This study evaluated the management and follow-up results of patients with PIPO who received treatment at our center. Materials and Methods: The cases of 13 patients with PIPO were reviewed retrospectively. Demographic data, clinical features, etiologies, pharmacological and surgical treatments, nutritional support, anthropometric findings, small bowel transplantation (SBT), and survival rates were assessed. Results: Two of the patients were diagnosed at 1 and 5 years of age, while other patients were diagnosed during neonatal period. The etiological cause could not be identified for 5 patients. Pharmacological treatment response was observed in 38.4% of patients. Post-pyloric feeding was applied in 4 patients, but no response was observed. Gastrostomy decreased the clinical symptoms in 3 patients during the abdominal distension period. Total oral nutrition was achieved in 38.4% of the total-parenteral-nutrition (TPN)-dependent patients. It was observed that anthropometric findings improved in patients with total oral nutrition. Liver cirrhosis developed in 1 patient. Venous thrombosis developed in 4 patients. The SBT was performed on 3 patients. One of these patients has been followed up for the last 4 years. Conclusion: Pediatric intestinal pseudo-obstruction is a rare disease that can present with a wide range of clinical symptoms. While some patients require intestinal transplantation, supportive care may be sufficient in others. For this reason, patients with PIPO should be managed individually.

WOS: 000378158900002, Background: In this study, we report our short-and mid-term surgical results in patients with discrete subaortic stenosis. Methods: A total of 72 patients (43 males, 29 females; mean age 4.6 +/- 4.5 years; range, 6 months to 11 years) who were surgically treated for discrete subaortic stenosis in our clinic between September 2000 and January 2012 were retrospectively analyzed. Limited myectomy in addition to the transaortic excision of the stenotic structure was performed in all patients. Left ventricular outflow tract gradient and aortic valve regurgitation were evaluated by echocardiography following the procedure. Results: The mean follow-up was 4.9 +/- 2.7 (range, 1 to 10) years. No mortality occurred during the early and late postoperative period. Heart block which necessitated the placement of a permanent pacemaker developed in one pediatric patient. The mean preoperative left ventricle systolic gradient which was measured as 48.4 +/- 13.7 mmHg (range, 32 to 70 mmHg), diminished to 8.6 +/- 5.6 mmHg (range, 4 to 15 mmHg) in the postoperative period. Reoperation was necessary due to recurrent stenosis in two patients (2.7%) during follow-up. Conclusion: Our study results show that limited myectomy in addition to membranectomy results in good mid-term results without increasing morbidity and mortality.

Objectives: In this study, we aimed to compare early results of the modified eversion carotid endarterectomy technique with the conventional carotid endarterectomy technique. The modified eversion carotid endarterectomy technique consisted of a longitudinal arteriotomy that was begun from the common carotid artery proximally to the origin of external carotid artery. We also avoided applying a carotid shunt during surgery in both techniques. Methods: Each patient was evaluated for coronary artery stenosis and valve replacement indications. Diagnosis of significant coronary artery stenosis or valve disease directed us to a combination of CEA and cardiac surgery (CABG/Valvular). We generally had a tendency to perform these two surgical procedures separately. Patients which had surgery for both at the same session were excluded in this study. As a result, our study included 120 patients and 137 carotid interventions. We performed the modified eversion carotid endarterectomy technique in 61 patients (68 carotid interventions) (Group A) and the conventional CEA technique in 59 patients (69 carotid interventions) (Group B). At follow-up, the patients were evaluated by physical examination and color Doppler USG. Results: There was no significant difference between the groups in terms of demographic data. Statistically significant difference was observed in one operative parameter between Group A and Group B; patchplasty requirements during surgery (12 carotid interventions in group B and 4 carotid interventions in group A, p = 0.036). We recorded temporary tongue deviation in five cases, facial asymmetry in eight cases, hoarseness in four cases, neurocognitive impairment in three cases and transient neurologic in two cases with no significant difference between the groups. There was in one case of permanent neurologic deficit (1 in group B). There were two postoperative deaths (1 in group A and 1 in group B). The death in group A occurred because of subarachnoid haemorrhage and the death in group B occurred because of myocardial infarction. After 6 month follow-up, no restenosis occurred in group A. Restenosis occurred in three patients of group B. Conclusion: The modified eversion technique for carotid endarterectomy decrease the incidence of patchplasty applications and postoperative restenosis by avoiding internal carotid artery manipulation and sewing. Besides, it is easy and possible to remove plaques completely from internal carotid artery via the modified arteriotomy line.

Background: This study evaluated the possible hypothesis that high altitude might be a reason of excessive postoperative drainage after coronary artery bypass graft operations (CABG). Methods: 212 patients were operated by the same surgical team and included to the study with retrospective data scan on postoperative bleeding after elective CABG with cardiopulmonary bypass (CPB). Study was derived from two different altitude centers: Group A patients (n = 107) from the center at an altitude of 1985 meters and Group B patients (n = 105) from the center in approximately sea level with 150 meters altitude. Multiple preoperative, intraoperative and postoperative characteristics with postoperative bleeding amounts were reviewed and calculated to determine a result through the aim of our study. Results: Preoperative, operative and intraoperative parameters for both groups were almost identical. Statistically significance occurred on intensive care unit treatment period, total hospitalization days, usage of erythrocyte suspension and fresh frozen plasma among postoperative periods on behalf of Group B. Parameters such as CABG numbers, Cross clamp and CPB time were not significant. High altitude patients were considered to be with a higher outcome in preoperative hematocrit (Htc) levels which did not occur in the study group. Htc levels presented a slight elevation for Group A as in the preoperative values but a statistically significance was not calculated. Postoperative bleeding volumes and calculated mean values were statistically significantly different among both groups in days of 1, 2 and 3. Reoperations for hemorrhage occurred in 9 cases (8.4%) in Group A and 4 cases (3.8%) in Group B. Conclusions: We want to attract attention to the relation between high altitude and postoperative bleeding after CABG surgery. But yet uncertain and unidentified physiological and biochemical conditions can be a reason as an uncontrollable perioperative factor in this patients. Operation at high altitude can be a reason for postoperative excessively bleeding.

Background and Design: Treatment of chronic venous ulcers of the lower extremity is still an important difficulty. The principal treatment of these ulcers includes compression therapy, local wound care and surgery. Unresponsiveness to these standard treatments is a frequent situation with negative effects on life quality and reductions in personal productivity. Therefore, there is a need for new applications to increase the effectiveness of treatment in treatment-resistant cases. In the present study, we retrospectively evaluated the results of topical application of amelogenin extracellular matrix protein in resistant venous ulcers. Materials and Methods: We analyzed the records of patients with treatment-resistant venous ulceration who were treated with amelogenin extracellular matrix protein between June 2011 and December 2012.. Results: 26 patients (21 male and 5 female) with a total number of 28 ulcers (24 patients with 1 ulcer, 2 patients with two ulcers) were evaluated. The patients were treated with topically applied amelogenin extracellular matrix protein and regional four bandage compression. Bandages were changed weekly. Each cure continued for six weeks. In fourteen patients (15 ulcers), we observed a complete healing by the end of the first cure. In another twelve cases (13 ulcers), the same period resulted with a reduction in wound diameter. We continued to the second cure for these patients. By the end of the second cure, complete healing was achieved in five cases (6 ulcers). Conclusion: Topical application of amelogenin extracellular matrix protein may be considered as an effective therapeutic choice for refractory venous ulcers.

A 37-year-old man with symptoms of cardiovascular disease applied to our hospital. Seven years ago, he had undergone combined kidney and pancreas transplantation. Afterwards, 4 years ago, his renal transplantation was repeated because of rejection. Coronary artery angiography revealed two-vessel coronary artery stenosis. We performed coronary artery bypass grafting to treat the coronary artery disease. The post-operative period was uneventful, and he was discharged from the hospital without any complications. Coronary artery diseases in recipients of combined kidney and pancreas transplantation can be safely treated using coronary artery bypass grafting.

Introduction We aimed to investigate whether the temperature of tumescent anesthesia is important, if so, to establish an opinion about the ideal temperature. Materials and methods Endovenous laser ablations were performed in 72 patients; 35 patients (Group A) received tumescent anesthesia at +4℃, while other 37 patients (Group B) received tumescent anesthesia at room temperature. The groups were compared in terms of intraoperative pain, postoperative regional pain, ecchymosis, paresthesia, skin burns and necrosis. At month 1, great saphenous vein was evaluated for recanalization and patient satisfaction. Results The survey on intraoperative pain showed that patients receiving tumescent anesthesia at +4℃ experienced much less pain. Interestingly, statistical analysis showed that this difference was not significant ( p = 0.072). No skin burns or necrosis occurred in either group, whereas ecchymosis and paresthesia were the most frequently observed side effects in both groups, but no significant difference was found between the groups. There was no significant difference between pain levels on postoperative days and no significant difference between the groups in terms of satisfaction with endovenous laser ablation procedure and postoperative satisfaction. All venous segments treated with endovenous laser ablation in both groups were occluded. At month 1 no recanalization was observed. Conclusion We conclude that the temperature of tumescent anesthesia solution is not important, while the proper administration of tumescent solution in adequate amounts ensuring delivery of the fluid to all segments appears to be a more significant determinant for the success of the procedure.

Stenosis or occlusion of a large right coronary artery or its vein grafts in symptomatic patients who underwent previous bypass grafting procedure with patent left-sided grafts is mostly managed by percutaneous interventions. When percutaneous interventions fail, it is a difficult decision to reoperate on a such patient for a single-vessel disease considering the risk of resternotomy. We present our technique which involves small anterior thoracotomy and partial sternotomy. (J Card Surg 2011;26:148-150)