Outcomes of kidney transplantations in children weighing 15 kilograms or less: a retrospective cohort study.

Kidney transplantation (KT) is often delayed in small children because of fear of postoperative complications. We report early- and long-term outcomes in children transplanted at ≤15 kg in the two largest Belgian pediatric transplant centers. Outcomes before (period 1) and since the introduction of basiliximab and mycophenolate-mofetil in 2000 (period 2) were compared. Seventy-two KTs were realized between 1978 and 2016: 38 in period 1 and 34 in period 2. Organs came from deceased donors in 48 (67%) cases. Surgical complications occurred in 25 KTs (35%) with no significant difference between the two periods. At least one acute rejection (AR) occurred in 24 (33%) KTs with significantly less patients experiencing AR during period 2: 53% and 12% in period 1 and, period 2 respectively (P < 0.001). Graft survival free of AR improved significantly in period 2 compared with period 1: 97% vs. 50% at 1 year; 87% vs. 50% at 10 years post-KT (P = 0.003). Graft survival tended to increase over time (period 1: 74% and 63% at 1 and 5 years; period 2: 94% and 86% at 1 and 5 years; P = 0.07), as well as patient survival. Kidney transplantation in children ≤15 kg remains a challenging procedure with 35% of surgical complications. However, outcomes improved and are nowadays excellent in terms of prevention of AR, patient and graft survival.

Does ultrasound guidance improve the efficacy of dorsal penile nerve block in children?

BACKGROUND: Penile nerve block (PNB) is a well-established technique used for circumcision; it requires the injection of local anesthetics close to the dorsal nerve of the penis. The goal of this study was to compare the efficacy of ultrasound-guided PNB versus the classical landmark-based technique (LBT) in children undergoing circumcision. METHODS: Forty boys, aged 1-14 years old that were scheduled for circumcision were randomly assigned to ultrasound-guided and landmark-based groups. All patients were placed under standard anesthesia with sevoflurane. In each group, patients received the penile block with ropivacaine, 0.75 mg·kg(-1) per side, and 0.05 mg·kg(-1) at the penis base. Groups were compared for intraoperative failure rate of the block, anesthesia time, postoperative pain scale, time of first required dose of paracetamol, time to first micturition, and average duration of stay in the postanesthesia care unit. RESULTS: The failure rate of dorsal PNB was not statistically different between groups (P = 0.5). Ultrasound guidance improved the efficacy of the PNB compared to the LBT in terms of postoperative pain scores on arrival in the PACU (P < 0.01) and after 30 min (P < 0.01). The ultrasound-guided technique also delayed the time to the first paracetamol dose administration (P < 0.0001), but the duration of the procedure, defined as the time between anesthesia induction and the end of surgery, was increased by 10 min in the US-guided group (P = 0.001). CONCLUSION: Ultrasound-guided PNB improved the efficacy of the block compared with the LBT in terms of the postoperative pain during the first postoperative hour and the time to the first requirement for postoperative analgesia.

Management of congenital and posttraumatic splenic cysts in children.

Splenic cysts are rarely observed in young patients. We report three cases of splenic cysts. The management and the surgical procedure, if needed, are discussed. If the splenic cyst is symptomatic or if its size reaches a diameter of 4-6cm, it requires surgical management. This treatment would be performed by laparoscopic approach and is intended to spare splenic tissue as much as possible. A prophylactic anti-pneumococcal vaccination is recommended before surgical treatment.

Prepubertal Vulvar Fibroma: Neoplasm or Physiological Condition?

BACKGROUND: Several authors have previously reconsidered vulvar fibroma, a rare prepubertal neoplasm, as a physiological labial asymmetry of early puberty, recommending a conservative approach for all cases, although some required further clarification. CASE: A fibrous mass found in the left labium majus of a 4-year-old girl was surgically removed. Imaging confirmed a relapse after 10 months and a second surgery was performed due to extremely rapid growth. No subsequent relapse occurred after 2 years of follow-up. Diagnosis confirmed prepubertal vulvar fibroma, with positive CD34 and lack of actin, desmin, S100, and estrogen receptors. SUMMARY AND CONCLUSION: A diagnosis of prepubertal labial asymmetry should prompt careful consideration of several variables, such as age, and radiologic and histologic features, in order to differentiate between a physiological condition and a neoplasm.

Neonatal hemochromatosis and Martinez-Frias syndrome of intestinal atresia and diabetes mellitus in a consanguineous newborn.

Neonatal hemochromatosis is a heterogeneous disorder of iron metabolism characterized by hepatic failure and marked iron accumulation in liver and extrahepatic tissues. Autosomal recessive transmission is found in most cases. Neonatal hemochromatosis shares cellular features with the adult disease but is clinically and genetically distinct, the causal gene(s) being presently unknown. We report on a newborn from consanguineous parents who presented with multiple congenital anomalies and neonatal hemochromatosis. The syndrome consisted of intra-uterine growth retardation, intestinal atresia, gallbladder aplasia and diabetes mellitus, and fitted with the diagnosis of Martinez-Frias syndrome, a very rare autosomal recessive phenotype, the gene of which remains to be identified. We suggest that neonatal hemochromatosis may be part of the Martinez-Frias syndrome. Molecular analyses in this and other reported patients with the Martinez-Frias syndrome should shed light on gut development and iron metabolism.

An unusual presentation of a cystic duplication of the sigmoid colon entirely lined with squamous epithelium.

Alimentary tract duplications are rare congenital malformations that occur most commonly in the jejunoileal part of the gastrointestinal tract. Management of this pathologic condition is usually drawn up. We report a case of descending colonic communicating duplication in which clinical presentation and anatomopathologic results were unexpected. A slightly echogenic abdominal mass reaching 72 x 36 mm in the left flank was diagnosed in a female fetus during the third trimester ultrasound examination. At birth, volume of the mass rapidly evolved, and despite no intestinal obstruction was observed by compression of the adjacent gastrointestinal tract, abdomen was distended. Abdominal plain film showed a large air collection, and the barium enema demonstrated a slight leak of contrast in the aerated mass, suggesting a communication with the sigmoid colon. No other abnormalities were seen. The patient underwent surgery in emergency. The mass was then totally excised through an antimesenteric resection of the tubular tract joining cystic mass and sigmoid colon. A lateral suture of the colon was subsequently performed. The wall of the duplication is usually composed of a smooth muscle layer covered by an epithelium, mostly of intestinal type. Herein, we describe a descending colonic duplication completely lined with nonkeratinizing squamous epithelium. Therefore, the association of a colonic mucosa (of endodermic origin) and a squamous epithelium (derived from the ectoderm) in our case is an interesting finding and is not explained by the various theories. Furthermore, the clinical characteristics, diagnosis, and treatment of intestinal duplications are discussed with regard to literature.

Sonographic prenatal diagnosis of malpositioned stomach as a feature of uncomplicated intestinal malrotation.

Intestinal malrotation is a developmental anomaly affecting the position and peritoneal attachments of the small and large intestines during fetal life. Most often the diagnosis is established in the first year of life on the basis of abdominal pain and bile-stained vomiting secondary to bowel obstruction. The antenatal diagnosis can be suggested by identification of the complications such as bowel dilatation, ascites or meconium peritonitis. We describe two cases of isolated antenatal gastric malposition without any other associated anomaly that were confirmed after birth to be due to intestinal malrotation. We suggest that such an antenatal finding should alert the paediatrician to close clinical follow-up and prompt the diagnosis and surgical treatment in case of abdominal pain and/or bilious vomiting.

Evaluation of da Vinci Nissen fundoplication clinical results and cost minimization.

BACKGROUND: A new technical tool was developed and introduced into the therapeutic field of videoscopic surgery-robotic telemanipulation surgery. The aim of this study is to investigate in a prospective randomized trial the feasibility of the Nissen procedure using the da Vinci and to evaluate the benefits and the costs of this new technique compared with the conventional laparoscopic approach. MATERIALS AND METHODS: Twenty patients with gastro-esophageal reflux disease (GERD) were randomized into laparoscopic Nissen versus robot-assisted Nissen fundoplication. All the patients signed an informed consent document. The time data of the procedure, the efficacy of the instruments, the intra-operative incidents, postoperative morbidity, and cost minimization are presented. RESULTS: Nine patients were assigned to the robot, and 11 to the laparoscopic procedure. Both groups were similar in age, male/female ratio, and body mass index. The robot procedure time was significantly longer. The hospital stay and the alimentation day were similar. The number of postoperative complaints was similar after the 1st, 6th, and 12th postoperative months. However, on the 3rd postoperative month, the number of complaints was significantly higher in the robot group. The robot procedure was more expensive with regard to the instrumentation and reusable material, the nursing costs, the investment costs, and the maintenance costs. CONCLUSIONS: No clear advantage of using robotics in the Nissen procedure was observed. The procedure seems to be feasible and safe. The technique is limited because of unadapted instruments. The disadvantages are the high costs and prolonged operative time.

New considerations for an overall approach to treat hepatocellular carcinoma in cirrhotic patients.

BACKGROUND: Increasing numbers of cases and organ shortage justify reconsidering the global therapeutic approach for hepatocelluar carcinoma in cirrhotic patients. METHODS: Recent literature was reviewed, focused on new therapeutic technologies such as radiofrequency. RESULTS: For small tumors, liver transplantation offers theoretically the best chance for cure. However, organ shortage may eliminate this advantage, because of tumor progression while waiting for a graft. For small tumors, arising on compensated cirrhosis, resection or radiofrequency ablation may provide efficient local tumor control without precluding subsequent transplantation in case of tumor recurrence and/or cirrhosis decompensation. CONCLUSIONS: For small tumors and compensated cirrhosis, resection or radiofrequency could represent acceptable first line treatments. In addition to permit safe and immediate tumor control, this strategy would allow a preferential redistribution of grafts to patients with decompensated cirrhosis in whom transplantation is the only possibility.

Aminopyrine breath test compared to the MELD and Child-Pugh scores for predicting mortality among cirrhotic patients awaiting liver transplantation.

Better tools for predicting the risk of death while awaiting transplantation are urgently needed because organ shortage is increasing the numbers on transplantation waiting lists. The aminopyrine breath test (ABT), model for end-stage liver disease (MELD), and Child-Pugh (C-P) score were compared as predictors of this risk in 137 cirrhotic candidates for liver transplantation. Eighty-three were transplanted within 3 months of registration, 35 others survived, 13 died before transplantation, and 6 were removed from the list. By univariate analysis, the continuous variables significantly associated with death while awaiting transplantation were: history of infected ascites, C-P score, ABT, and international normalized ratio or prothrombin time. Receiver operating characteristic curves for quantitative variables showed that the area under the curve was greatest for ABT (0.858 +/- 0.067). By Youden curve analysis, the best cut-off points for identifying cirrhotic patients at high risk of death while on the waiting list were: > 10, > 16, and < 0.7% for the C-P score, MELD score, and ABT, respectively. These results show that ABT is as good as the MELD and C-P scores, or better, as a predictor of death among cirrhotic patients awaiting liver transplantation.