A unique defect - persistent posterior cloaca: An example of staged genito-urinary and digestive tract reconstruction with an alternative vaginal creation using the urinary bladder wall.

We present a case of a girl with an extremely rare, posterior type of persistent cloaca, which was associated with other abnormalities, including an undeveloped vulva and vagina, agenesis of the right kidney, secondary obstructive megaureter, unicornate uterus, persisted tailgut, sacral bone hypoplasia, and pubic symphysis hypertrophy. An operative approach was as follows: (i) colostomy and ureterocutaneostomy; (ii) creation of an ileal conduit with antirefluxing uretero-ileal anastomosis, and then creation of a continent catheterizable ileal reservoir; (iii) anastomosis of sigmoid colon to rectal stump; and (iv) vaginal and external genital reconstruction. Because of abnormal anatomical conditions where the uterus was situated adjacent to the open, incompetent bladder neck, we decided to create a vagina using the bladder wall instead of the bowel segment.

Reorganization of nutritional therapy can markedly reduce the rate of catheter-related blood stream infections in pediatric patients receiving parenteral nutrition - a 7-year prospective follow-up study.

BACKGROUND: Implementation of hygienic measures and simple changes in the structure of medical team may considerably reduce the rate of catheter-related bloodstream infections (CRBSIs) in parenterally nourished patients. AIM: To analyze the effects of organizational changes in parenteral nutrition services on the CRBSI rates in pediatric patients. METHODS: We compared the CRBSI rates documented prior to, during and after the implementation of the organizational changes (introduction of a nutritional support team and related procedures, medical staff training). FINDINGS: A total of 260 courses of parenteral nutrition were offered to 141 pediatric patients during the analyzed period. Thirty CRBSIs were documented during this period. The most frequent etiological factors were staphylococci (21/30), followed by Klebsiella pneumoniae, Escherichia coli and Candida albicans (2/30 each). The reorganization was reflected by more than 8-fold reduction of the CRBSI incidence rate: from the initial value of 10.14 to 6.89 per 1000 catheter days and 1.17 per 1000 catheter days during and after the reorganization, respectively. CONCLUSION: Introduction of a nutritional support team, accompanied by extensive training of medical staff, can result in a marked reduction of CRBSI rate in pediatric patients nourished parenterally in a hospital setting.

Antecedentes: La implementación de medidas higiénicas y cambios sencillos en la estructura del personal médico puede reducir considerablemente la tasa de bacteriemia asociada al catéter (BAC) en pacientes que reciben nutrición parenteral. Objetivo: Analizar el impacto de los cambios organizacionales dentro de los servicios de nutrición parenteral sobre las tasas de BAC en pacientes pediátricos. Métodos: Hemos comparado las tasas de BAC documentadas antes, durante y después de la implementación de los cambios organizacionales (introducción de un grupo de apoyo nutricional y los procedimientos relacionados, formación del personal médico). Descubrimientos: Un total de 260 series de nutrición parenteral fueron ofrecidos a 141 pacientes pediátricos durante el periodo analizado. Se documentaron treinta BAC durante este periodo. Los factores etiológicos más frecuentes eran staphylococci (21/30), seguidos por Klebsiella pneumoniae, Escherichia coli y Candida albicans (2/30 cada uno). Los cambios organizacionales fueron reflejados en una reducción de la incidencia de BAC en más de 8 veces: el valor inicial disminuyó desde 10.14 hasta 6.89 por 1000 días-catéter y hasta 1.17 por 1000 días-catéter durante y después de la reorganización, respectivamente. Conclusión: La introducción de un grupo de apoyo nutricional, acompañada de una extensa formación del personal médico puede resultar en una reducción considera ble de la tasa de BAC en pacientes pediátricos que reciben nutrición parenteral en en un entorno hospitalario.

[Functionality determinants of the integrated systems dedicated to health care institutions]. / Determinanty funkcjonalnosci zintegrowanych systemów dedykowanych podmiotom leczniczym.

The Authors of the hereby dissertation aimed at presenting the functionality concept of the integrated e-service platform dedicated to health care institutions. In the form of a problem repository the research results allowing the identification of the functionality determinants were presented. Finally, the study presents the system's structure as well as analyzing the needs for e-services in terms of society and their usefulness evaluated by branch experts.

Hybrid approach for closure of muscular ventricular septal defects.

BACKGROUND: The complexity of ventricular septal defects in early infancy led to development of new mini-invasive techniques based on collaboration of cardiac surgeons with interventional cardiologists, called hybrid procedures. Hybrid therapies aim to combine the advantages of surgical and interventional techniques in an effort to reduce the invasiveness. The aim of this study was to present our approach with mVSD patients and initial results in the development of a mini-invasive hybrid procedure in the Gdansk Hybrid Heartlink Programme (GHHP) at the Department of Pediatric Cardiac Surgery, Pomeranian Centre of Traumatology in Gdansk, Poland. MATERIAL AND METHODS: The group of 11 children with mVSDs was enrolled in GHHP and 6 were finally qualified to hybrid trans-ventricular mVSD device closure. Mean age at time of hybrid procedure was 8.22 months (range: from 2.7 to 17.8 months, SD=5.1) and mean body weight was 6.3 kg (range: from 3.4 to 7.5 kg, SD=1.5). RESULTS: The implants of choice were Amplatzer VSD Occluder and Amplatzer Duct Occluder II (AGA Med. Corp, USA). The position of the implants was checked carefully before releasing the device with both transesophageal echocardiography and epicardial echocardiography. All patients survived and their general condition improved. No complications occurred. The closure of mVSD was complete in all children. CONCLUSIONS: Hybrid procedures of periventricular muscular VSD closure appear feasible and effective for patients with septal defects with morphology unsuitable for classic surgical or interventional procedures. The modern strategy of joint cardiac surgical and interventional techniques provides the benefits of close cooperation between cardiac surgeon and interventional cardiologist for selected patients in difficult clinical settings.

Bloody pericardial tamponade in a child treated for pneumonia mimicking a lung tumor and infiltration of the heart.

We present the dramatic course of a female 5-year-old child with pneumonia and symptoms similar to local compression of the pericardium by a tumorous mass originating from the left lung. The child was treated with antibiotics for pneumonia with bilateral pleural effusions that required chest drainage. On the 10(th) day of therapy there was sudden anemia observed with the echocardiographic finding of acute cardiac tamponade. The child was referred for emergency life-saving surgical intervention. The chest was opened via a minimally invasive mini-incision in the area of the xiphoid process and bloody tension pericardial effusion was evacuated. The laboratory and histopathology investigations were not specific for neoplastic disease or tuberculosis infection. In the further observation the girl recovered and was discharged home two weeks after tamponade drainage. Fortunately our initial suspicion of neoplastic disease was not proved; nevertheless we would like to emphasize the need for oncologic vigilance in similar cases.

The Foker technique (FT) and Kimura advancement (KA) for the treatment of children with long-gap esophageal atresia (LGEA): lessons learned at two European centers.

INTRODUCTION: We present the experiences from two European centers performing the Foker technique (FT) of esophageal lengthening by axial traction and the Kimura advancement (KA) method of lengthening the upper pouch by extrathoracic resiting a spit fistula (SF) in children with long-gap esophageal atresia (LGEA, gap length > 5 cm). MATERIALS AND METHODS: A total of 15 children were treated (8 pure EA, 6 lower tracheoesophageal fistula [TEF], and 1 upper TEF). Gaps ranged from 5 to 14 cm. Nine children already had a SF. Patients were grouped according to the presence of a SF and the subsequent surgical strategy: Group A (no SF, n = 6) received FT on both pouches. Group B (with SF, n = 6) received KA of SF and FT of the lower pouch. Group C (with SF, n = 3) received closure of the SF and subsequent Foker traction (CSFT) on both pouches. RESULTS: Group A: Primary repairs for all six children (mean age 3 months, gap length 6.5 cm) after a mean traction time of 3 weeks and a mean of 2.1 thoracotomies (range 2 to 3). Dilations were required in three out of six for anastomotic strictures with one perforation during the second dilation. Group B: All six children (mean age 16.4 months, gap length 9.5 cm) had a primary anastomosis, although for two it was significantly delayed (48 and 143 weeks traction time) because of infections. The number of thoracotomies ranged from 2 to 8 (mean 3.6). Leaks occurred in five out of six anastomoses (responsive to conservative management). Two children developed severe strictures, which required the anastomosis to be redone. In group C (mean age 10.6 months, gap length 6.5 cm), several major complications occurred. The three SF closures leaked (one iatrogenic) causing severe mediastinitis. CSFT was successful in only one case and the other two children had an esophageal replacement (stomach, jejunum). No deaths occurred in the series. CONCLUSION: FT of both pouches (group A) resulted in primary repairs of all six LGEA patients. The combination of KA and FT (group B) resulted in an equivalent rate of primary repairs, but with an increased number of thoracotomies and rate of complications compared with group A. CSFT (group C) resulted in a high failure rate. More data are needed (we propose a multicenter registry) to elucidate the safety and efficacy of each elongation technique and to establish an algorithm with clearer inclusion and exclusion criteria.

Laparoscopic management of giant ovarian cysts in adolescents.

A laparoscopic approach to giant ovarian cysts in girls, when the cyst's size exceeds the umbilicus, may be difficult regarding the risk of cyst rupture and limited working space. We present a series of three 12-year-old girls with giant ovarian cysts treated by percutaneous drainage under ultrasonographic guidance followed by laparoscopic resection. Clinical, laboratory and radiological diagnosis showed no signs of malignancy. After induction of general endotracheal anaesthesia, under ultrasonographic guidance, a 10 F vesicostomy catheter was placed into the cyst. Two to 4 l of serous (in one case mucinous) fluid were drained from the cyst. During laparoscopy, the abdominal cavity was inspected by the scope and no signs of malignancy were found. The laparoscopic procedure was completed with excision of the cyst and keeping some ovarian tissue in all cases. No intraoperative complications were observed. The mean operation time was 73 min. Pathological examination revealed a mature cystic teratoma in two cases and mucinous cystadenoma in one. The postoperative recovery was uneventful in all girls and they were discharged home on postoperative day 2-4 with minimal pain. The ultrasound scans and tumour markers were normal on follow-up after 3 and 6 months. Laparoscopic excision of giant ovarian cysts after ultrasound-guided drainage seems to be a safe and applicable treatment modality in pre-menarchal girls with a normal tumour marker profile and benign features of the cyst, excluding the possibility of malignancy.

Cardiovascular hybrid procedure in severe congenital diaphragmatic hernia with significant left heart hypoplasia.

The authors discuss an early hybrid cardiovascular intervention in a neonate with left-sided congenital diaphragmatic hernia and significant left heart hypoplasia. The operation included persistent ductus arteriosus stenting and right pulmonary artery calibrated banding (3.5 mm) to increase blood flow in the aorta and to decrease right ventricle overload and decrease blood pressure and overflow in pulmonary circulation. The operation improved the child's general condition and gained time for left ventricle growth and restoration of its function.

Candidemia in children after complex congenital heart defects surgery treated with caspofungin--our own experience and a review of literature.

BACKGROUND: Invasive fungal infections due to Candida species constitute an increasing clinical problem. There are no guidelines for the management of candidemia in children undergoing surgical procedures for congenital heart defects (CHD). The aim of the study was to draw attention to the problem of candidemia in children who are operated on due to congenital heart defects. MATERIAL/METHODS: We retrospectively analyzed medical documentation of 307 children with congenital heart defects treated in 1 clinical centre in Poland, from whom we selected those diagnosed with candidemia during the postoperative period. Next, we analyzed in detail the clinical course of invasive candidiasis in each individual and we performed an analysis of risk factors that lead to candidemia. RESULTS: In the analyzed period, among 307 children who underwent surgical procedures, we observed 2 cases of candidemia (0.65%) which were effectively cured with caspofungin. No adverse effects were observed after treatment with the drug. CONCLUSIONS: Candidemia in children who undergo surgical treatment of congenital heart defects is an important factor that can influence final clinical results. Caspofungin may be an effective therapeutic option when treating candidemia in children after extensive cardiosurgical procedures.

[Intraoperative imaging of hybrid procedure for muscular ventricular septal defects closure with Amplatzer Duct Occluder II]. / Sródoperacyjne obrazowanie hybrydowego zabiegu zamkniecia ubytku w przegrodzie miedzykomorowej za pomoca Amplatzer Duct Occluder II.

Hybrid approach becomes a promising conception of an alternative treatment for muscular ventricular septal defects (VSD) closure in infants. In this paper we report epicardial echocardiography imaging that enabled successful hybrid procedure performed in premature infant referred for surgical correction of a large perimembraneous VSD with simultaneous perventricular approach for concomitant muscular VSD.

Laparoscopic splenectomy in children--a multicenter experience.

PURPOSE: Splenectomy is frequently required in children for various hematologic pathologic findings. Because of progress in minimally invasive techniques, laparoscopic splenectomy (LS) has become feasible. The objective of this report is to present a multicenter experience of 159 laparoscopic splenectomies. METHODS: Records of 159 children, who underwent LS in 3 pediatric surgery centers between 1996 and 2006, were reviewed retrospectively with a special questionnaire. The indication for splenectomy were hematologic disorders (147), esophageal varices (6), splenic cyst (5), and tumor of the tail of the pancreas (1). The LS was performed using standard laparoscopic technique and instrumentation. RESULTS: There were 75 boys and 84 girls. Median age was 12.5 years (range, 2-19.4 years). Laparoscopic splenectomy alone was performed in 118 cases and LS with cholecystectomy or cholecystotomy in 36. In 5 cases, LS was performed together with fundoplication. Eight LS required conversion to an open procedure for following reasons: severe bleeding (3), massive splenomegaly (1), anatomical (2), and technical aspects (2). Accessory spleens were identified in 20 cases (12.6%). There was no mortality. Complications consisted of 8 conversions and postoperatively of mild generalized infection in 3 children and abdominal bleeding that resulted in re-laparoscopy in 1 case. CONCLUSIONS: Laparoscopic splenectomy can be safely and effectively performed in children.