Pyloric atresia associated with epidermolysis bullosa: report of two cases and review of the literature

The coexistence of pyloric atresia (PA) and epidermolysis bullosa (EB) is a rare but well-known surgical emergency in neonates. PA/EB is described by the association of atresia of the pylorus and bullous lesions on the skin. Ninety one cases have been reported in the literature to date. We present two new cases and evaluate the association of PA/ EB, its etiopathogenesis and the clinical properties. Case 1: A three-day-old female presented with nonbilious vomiting and bullous lesions 2-3 cm in diameter on the extremities. Abdominal X-ray showed a single air-fluid level in the left upper quadrant. At laparotomy, we found PA and performed a pyloro-pylorostomy. The patient died due to sepsis complication of EB two months after surgery. Case 2: A two-day-old male presented with severe dermal bullous lesions on the trunk, neck and extremities. His stomach was dilated and there was no gas distally. We found PA and performed gastroduodenostomy. Initially, he tolerated the feeding well, but he died due to severe sepsis on the postoperative 23rd day. Almost all neonates born with the PA/EB result in a fatal outcome in the first few years. The complications related to EB are usually the cause of death. Even after successful repair of PA, skin lesions lead to death due to infection.     

Use of continuous horizontal mattress suture techniques in microsurgery: an experimental study in rats

PURPOSE: The purpose of this study was to determine whether the continuous horizontal mattress suture technique can replace the continuous simple suture technique and to compare the results with other microvascular suture procedures. METHODS: Sixty-four femoral arteries of 32 Sprague-Dawley rats were used in this study. The animals were divided equally into 4 groups with 16 anastomoses in each group. The arteries (0.8-1.0 mm diameter) were anastomosed by using the continuous horizontal mattress suture technique in group I, interrupted horizontal mattress suture technique in group II, simple interrupted suture technique in group III, and simple continuous suture technique in group IV. At the end of the anastomosis time, leakage, and patency were assessed and graded in all groups. On the 14th day after surgery the rats were killed and 5 patent specimens from each group were examined under light microscopy for histology. One specimen from each group was prepared for scanning of the endothelial surface under electron scanning microscopy. RESULTS: Group I anastomoses were performed the most quickly. Groups I and III anastomoses had 100% patency rates. Under light microscopy the edge eversion was apparent consistently and under electron microscopy all endothelial surfaces were intact and no suture material was seen in groups I and II. In group III some suture material was covered by endothelial cells and lumen surfaces were torn; endothelization also was rough compared with groups I and II. In group IV suture material was seen in the lumen because of a loose suture knot. The endothelium also was not regular. CONCLUSIONS: The horizontal mattress suturing technique is the only technique in which the suture material never contacts the lumen. Continuous horizontal mattress suture technique is superior to the other microvascular procedures and is the safest and fastest procedure for microvascular anastomosis in rats.     

Timing of surgical treatment for type III supracondylar humerus fractures in pediatric patients

Background  Type III supracondylar humeral fracture is a common cause of emergency hospitalization among children requiring surgical treatment. The configuration of the internal fixation material, surgical technique, and optimal timing of surgery (TS) have always been popular topics of debate. The TS in uncomplicated cases is usually determined by surgeons. Methods  In this study, we prospectively followed children with type III supracondylar fractures. We aimed to clarify the effects of injury side, gender, and post-injury delay on switching to open surgery and the ease of the reduction. Results  Based on our results, the probability of switching to open surgery increased by a factor of 4 every 5 h beginning 15 h after injury. Open surgery was necessary after 32 h. Conclusion  Reduction became technically more difficult as TS increased.     

MR fluoroscopy-guided transthoracic fine-needle aspiration biopsy: feasibility

The purpose of this study was to evaluate the feasibility of using an open-configuration magnetic resonance (MR) imaging system with MR fluoroscopic guidance to perform percutaneous transthoracic fine-needle aspiration biopsy in patients with lung masses. Percutaneous transthoracic aspiration biopsies were performed with MR fluoroscopic guidance in 14 patients. The masses were 2-7 cm in diameter (mean, 4.1 cm). The needle was positioned by using a free-hand technique with MR fluoroscopic guidance. The needle tip reached the target lesion, and biopsy was performed. Analysis of the biopsy specimens facilitated a specific diagnosis in all patients. Pneumothorax was noted in two patients (14%) with chronic obstructive pulmonary disease. Study results showed that the described MR fluoroscopy-guided transthoracic biopsy technique can be used safely and successfully for lung masses. MR fluoroscopy can be used to reach the target lesion easily and accurately.     

Value of Dual Energy Computed Tomography for detection of myocardial iron deposition in Thalassaemia patients: initial experience

Purpose

The aim of our study was to compare the value of cardiac DECT (cDECT) for detection of myocardial iron deposition to T2*w cardiac MRI (cMRI).

Material and methods

Nineteen patients with clinical history of Thalassaemia underwent T2*-weighted cardiac MRI (cMRI) with a 1.5 T MR scanner (MAGNETOM Symphony, Siemens Medical Solutions, Erlangen, Germany) and cardiac dual energy CT (cDECT) with a DSCT scanner (SOMATOM Definition, Siemens Medical Solutions, Erlangen, Germany) on the same day. HU values obtained from cDECT scans and T2*-values from cMRI were statistically correlated to calculate significance levels. Table times were measured for both cDECT and cMRI and compared. Patients were asked to grade their subjective comfort during the examination.

Results

In all patients cDECT scans were successfully acquired.HU values of septal muscle correlated strongly with T2*-values, whereas no correlation was found for paraspinal muscle.Table time was significantly shorter for cDECT compared to cMRI (mean: 3.7 min vs. 11.2 min) and subjective patient comfort was rated comfortable for cDECT and average to poor for cMRI. Mean radiation dose was 0.71 mSv.

Conclusion

cDECT scans seem to be possible for evaluation of myocardial iron load in pediatric Thalassaemia patients.     

The relationship between the MEFV genotype, clinical features, and cytokine-inflammatory activities in patients with familial mediterranean fever

Familial Mediterranean Fever (FMF) is an autosomal recessive disease characterized by periodic attacks of fever and polyserositis. The effects of the MEFV genotype differences on clinical picture and inflammatory activity have not been well documented. The aim of this study was to investigate levels of conventional inflammation markers, procalcitonin, interleukin levels, TNF-alpha, and C5a levels in patients with FMF who had different MEFV genotypes and compare them with those of healthy subjects. The study consisted of 41 patients with FMF (F/M: 23/18), and 31 healthy subjects (F/M: 18/13). Tests were performed during the attack-free period. White-blood cell count, CRP and IL-8 levels were higher in patients with FMF than in healthy subjects (p < 0.05) and also higher in M680I carriers than in the patients with M694V allele carriers. However, ESR, fibrinogen, procalcitonin, IL-6, C5a, TNF-alpha, and IgD levels were not significantly different between patients and healthy subjects (p > 0.05). Arthralgia or arthritis was significantly higher in M694V carriers than in non-M694V carriers (p < 0.05). It is concluded that the clinical features and inflammatory-cytokine activities were higher in patients with FMF during the attack-free period than in healthy subjects, and the different genotype might be related to different clinical pictures.     

Stereological evaluation of liver volume in living donor liver transplantation using MDCT via the Cavalieri method.

In living donor liver transplantation (LDLT), obtaining the precise volume of the graft is very important to decrease volume-related postoperative complications, especially in cases with suspected small-for size grafts. We used stereology based on the Cavalieri method (CM), a new method to measure liver graft volume, and compared the results with those obtained through intraoperative measurement (IOM) and through multidetector computed tomography (MDCT) measurement. Liver volumes estimated using the 3 methods were well-correlated with each other (r(2) = 0.94 and P < 0.001 for IOM and CM; r(2) = 0.91 and P < 0.001 for IOM and MDCT, and r(2) = 0.95 and P < 0.001 for CM and MDCT); however, they were different from each other (in descending order, 908 +/- 124 cm(2), 861 +/- 121 cm(2), and 777 +/- 168 cm(2) for MDCT, CM, and IOM, respectively). Although MDCT and CM overestimated the volumes, the results of CM were almost similar to those obtained via IOM. In conclusion, our results suggest that CM measured the liver graft volume more reliably. Thus, its use, particularly in cases with suspected small-for-size graft, may prove useful.     

Live donor liver transplantation for acute liver failure

BACKGROUND: Acute liver failure (ALF) carries a high mortality unless urgent orthotopic liver transplantation (OLT) is performed on time. Live donors are utilized to treat this irreversible condition first in pediatric cases and then in adults. Herein, we aimed to report our experience with live donors for ALF in a country of a deceased donor organ donation rate is only 1.5 per million people. METHODS: Among the 245 live donor liver transplantations (LDLT) performed from June 1999 to December 2005, 14 of them (6%) were performed for ALF in 8 pediatric and 6 adult cases. Right lobes were harvested for the adult cases whereas left lateral segments were harvested for pediatric cases, except one child transplanted with a right lobe graft. The etiology of the disease was; acute hepatitis B in four cases, hepatitis A in three cases, Wilson disease two cases, autoimmune hepatitis in two cases, and was unknown in three cases. RESULTS: Three-year graft and patient survival is 79% for these series. Five of the six adult patients and six of the eight pediatric cases survived after transplantation. There was not any donor mortality or major morbidity. CONCLUSIONS: LDLT offers a safe and effective modality of treatment for ALF for both pediatric and adult patients to overcome the problem of organ shortage especially in countries where the chance of receiving an organ from a deceased donor is low.     

Course of serum lipoprotein(a) and acute phase protein levels in patients undergoing open heart surgery

BACKGROUND: Lipoprotein(a) [Lp(a)] is an important risk factor in the pathogenesis of coronary artery disease because of its thrombogenic and atherogenic properties. Lp(a) also displays another property by acting as an acute phase reactant. METHODS: In this work, the study group consisted of 20 male patients having coronary artery bypass under cardiopulmonary bypass (CPB). Preoperative and postoperative levels of plasma total cholesterol, triglyceride, apolipoprotein A1 (Apo A1), apolipoprotein B (Apo B), alpha-1 antitrypsin (a1-AT), alpha-2 macroglobulin (alpha 2-MG), alpha-1 acid glycoprotein (alpha 1-AG), Lp(a) were measured in all patients one day before and after the 1st, 2nd, 4th, and 10th days of CPB. RESULTS: It was observed that the levels of Lp(a) levels gradually reached the preoperative levels at the 10th postoperative day period. Observed change of the Lp(a) levels was similar to that of the other acute phase proteins which are synthesized and released from liver. In contrast, alpha 2-MG has shown different behaviour in terms of operative values. The changes observed for all these 3 parameters were found to be statistically significant (p<0.01). CONCLUSIONS: The data has indicated that Lp(a) levels show similar progress with alpha 2-MG levels. It can be concluded that serum levels of Lp(a) after coronary arterial bypass decrease depending upon several factors and reach basal levels at the end of a 10 day-period of postoperation. The main cause for this decrease might result from the contact of blood with foreign surfaces of the heart-lung machine.