Yves Aigrain

Laparoscopic renal surgery via a retroperitoneal approach in children

PURPOSE Laparoscopic procedures are still not widely performed by pediatric urologists due to operative time and costs as well as the lack of indications and, thus, the lack of sufficient surgeon experience. We report our experience with the retroperitoneal approach using special inexpensive methods for various indications in children. MATERIALS AND METHODS From 1994 to 1997 we performed 42 retroperitoneal laparoscopic procedures in 41 children with a mean age of 4 years, including 31 nephrectomies, 8 partial nephrectomies, 2 renal cystectomies and 1 pyelolithotomy. A total of 13 patients were younger than 1 year. Followup ranged from 6 months to 3 years. RESULTS Mean operative time was 104 minutes (range 35 to 150) for nephrectomy, 153 (range 90 to 210) for upper pole nephroureterectomy, 135 for pyelolithotomy and 60 for renal cystectomy. Average postoperative stay was 2 days. Conversion was required in 2 cases of partial nephrectomy due to unidentified polar vessels, including 1 involving duodenal perforation. CONCLUSIONS The indications for retroperitoneal laparoscopy are expanding with the experience of the surgical team. The retroperitoneal approach provides a technique comparable to that of conventional renal surgery.

Effect of Amnioinfusion on the Outcome of Prenatally Diagnosed Gastroschisis

Objective: Following recent data showing that an inflammatory response exists in the amniotic fluid of gastroschisis-affected fetuses, we hypothesized that amniotic fluid exchange or amnioinfusion would improve the prognosis of prenatally diagnosed gastroschisis. Methods: We compared the outcome of prenatally amnioinfused fetuses with gastroschisis to non-amnioinfused fetuses with gastroschisis. 10 patients undergoing this procedure were matched with 10 patients of our previous study. Comparisons were done on data including surgical procedure, follow-up in the NICU and the gastro-pediatric unit. Results: Our results show that gastroschisis-affected fetuses undergoing amnioinfusion had a lower duration of curarization after surgery (2.2 ± 1.9 vs. 6.8 ± 6.9 days, p = 0.019), a shorter delay before full oral feeding (49.7 ± 21.5 vs. 72.3 ± 56.6 days, NS), and a shorter overall length of hospitalization (59.5 ± 19.7 vs. 88.5 ± 73.6 days, NS). We confirmed our previous data showing that amniotic fluid displays a chronic inflammation profile. Conclusion: Our data suggest that amnioinfusion could improve the outcome of gastroschisis affected fetuses. The hypothesis by which this improvement could be due to a reduction of an inflammatory response remains to be proved.

OUTCOME OF POSTERIOR URETHRAL VALVES: TO WHAT EXTENT IS IT IMPROVED BY PRENATAL DIAGNOSIS?

PURPOSE To assess the impact of prenatal diagnosis and evaluation on the outcome of posterior urethral valves we studied all cases of valves detected prenatally, including cases of pregnancy termination due to posterior urethral valves. MATERIALS AND METHODS Between 1989 and 1996, 30 neonates with prenatally detected posterior urethral valves were treated at our hospital. The prenatal parameters analyzed were age of gestation at diagnosis, ultrasonographic appearance of renal parenchyma and amniotic fluid volume. Fetal urine was analyzed in 9 cases. We reviewed the outcome of 10 neonates treated for posterior urethral valves which were not diagnosed prenatally during the same period. RESULTS Of the 30 neonatal survivors 6 (20%) had renal failure, including end stage renal disease in 2, after a mean followup of 4 years. Renal failure developed in 2 of 5 cases detected before 24 weeks of gestation, in 1 of 6 with oligohydramnios and in 2 of 5 with abnormal parenchymal renal ultrasound. Normal parenchymal ultrasound and amniotic volume could not predict for good outcome. Renal failure developed in 2 of 7 cases predicted by fetal urinalysis as good prognosis and in 1 of 2 cases predicted as poor prognosis. Pregnancy was terminated for posterior urethral valves in 5 cases based on prenatal criteria of severe renal impairment. Considering these cases as poor outcome, the rate of poor prognosis increased from 20 to 31%. Among the 10 neonates without a prenatal diagnosis of posterior urethral valves renal failure developed in 2 (20%), including end stage renal disease in 1. CONCLUSIONS When negative parameters were absent and/or fetal urine predicted good outcome there were no cases of end stage renal disease in early infancy, which was a significant help in parent counseling. The predictive value of the currently available prenatal parameters needs to be updated with larger series specifically dealing with posterior urethral valves. According to the current data, the outcome of posterior urethral valves is not yet significantly improved by prenatal diagnosis.

Highly differentiated teratoma and fetus-in-fetu: A single pathology?

A case of sacrococcygeal teratoma is presented with characteristics of fetus-in-fetu. This pseudo-fetus presented a rudimentary single cavity heart, which beat at a different rate to that of the affected infant. X-ray examination showed no spinal column. This case confirms that fetus in fetu can be a remarkably complex, well-differentiated, highly organized teratoma.

New perspectives for children with microvillous inclusion disease: early small bowel transplantation.

Background. Microvillous inclusion disease (MVID) is a congenital intestinal epithelial cell disorder leading to lifelong intestinal failure. Despite long-term total parenteral nutrition, life expectancy is extremely reduced because of metabolic or septic complications or liver failure. Methods. Twelve patients with early-onset MVID were evaluated between 1995 and 2002 for the possibility of small bowel transplantation (SbTx). Three patients died before they could be placed on the waiting list for SbTx, and one patient is still awaiting SbTx. SbTx was contraindicated in one patient. Results. Seven of 12 patients (six boys and one girl) underwent transplantation (three SbTxs and four combined liver-SbTxs). Actuarial survival rates were 100% and 75% in the SbTx and combined liver-SbTx groups, respectively, with a mean follow-up of 3 years (1.1–8.5 years). In contrast, the survival rate was only 40% in the subgroup of five patients who did not undergo transplantation. After transplantation, all patients were weaned from parenteral nutrition: the five patients with an additional colon graft were weaned within 36 days as opposed to the others without colonic transplant who obtained full intestinal autonomy several months after transplantation. The only two surviving patients who did not undergo SbTx remain highly dependent on total parenteral nutrition, which is complicated by repeated episodes of metabolic decompensation. Conclusions. SbTx alone or in combination with the liver is highly successful in children with MVID, offering them a long-term perspective for the first time. Associated colon grafting markedly improves the outcome and quality of life after SbTx in patients with MVID.

Tracheobronchial ruptures from blunt thoracic trauma in children

BACKGROUND/PURPOSE Tracheobronchial ruptures in blunt thoracic trauma in children are rare. The aim of this study was to suggest the means of an early diagnosis and a conservative management as often as possible. METHODS Sixteen cases of tracheobronchial ruptures by blunt thoracic trauma were observed over 26 years in 9 regional pediatric centers. RESULTS There were 12 boys and 4 girls, from ages 1 hour to 17 years. Nine children presented with associated lesions. Fibroscopy established the following diagnosis: 8 tracheal wounds and 8 bronchial wounds. Six children were operated on within 18 hours (on average) after installation of a thoracic drainage. Two lobectomies, 3 ideal tracheal sutures, and 1 bronchial suture were performed. Seven children were treated exclusively by thoracic drainage. Two of them were intubated through the lesion, leading to a transitory endoprothesis accompanied or not by an external thoracic drainage. One infant recovered spontaneously. There were no deaths in this series. Two recurrent postoperative nerve injuries were noted, one of which was a transitory spontaneously resolutive scar bud and one a granuloma treated by laser. Three times, a stenosis occurred after a conservative management. Two were operated on. CONCLUSIONS Tracheobronchial ruptures in children are rare. An early fibroscopy holds an important place in the approach of this pathology. Treatment is variable, based on thoracic lesions, their tolerance by the child, and associated lesions. Surgery is not the only therapy because conservative treatment by simple thoracic drainage or lesion intubation has proved effective.

Interleukin-6 and interleukin-1β production in a pediatric plasma cell granuloma of the lung

Plasma cell granuloma (PCG) is a pseudotumor of unknown origin. It is frequently accompanied by acute-phase clinical and biological signs that resume after complete surgical removal, suggesting production of soluble mediators. We therefore investigated the role of cytokines in a previously healthy 10-year-old boy with a PCG of the lung and systemic symptoms. In this case, very high serum levels of interleukin-1 beta (IL-1 beta) and interleukin-6 (IL-6) were found before tumor excision, associated with inflammatory signs including major hyper-gamma-globulinemia. Pathologic analysis of the tumor showed an accumulation of fibroblasts and plasma cells producing immunoglobulins. Local production of IL-1 beta and IL-6 could be demonstrated at the messenger RNA (mRNA) level by the reverse transcriptase polymerase chain reaction and could be attributed to inflammatory cells by in situ hybridization and immunohistochemistry, whereas plasma cells exhibited membrane expression of the IL-6 receptor. Postsurgery follow-up showed rapid normalization of serum IL-1 beta and IL-6, whereas inflammatory protein levels decreased. This confirms the local production of cytokine within the PCG and raises the question of whether a dysregulation of cytokine production initiates the disease.

Expression of TLR-2, TLR-4, NOD2 and pNF-κB in a Neonatal Rat Model of Necrotizing Enterocolitis

Background The etiology of necrotizing enterocolitis (NEC) results from a combination of several risk factors that act synergistically and occurs in the same circumstances as those which lead to innate immunity activation. Pattern recognition molecules could be an important player in the initiation of an exaggerated inflammatory response leading to intestinal injury in NEC. Methodology/Principal Findings We specifically evaluated intestinal epithelial cell (IEC) expression of Toll-like receptor 2 (TLR-2), TLR-4, NOD2 and phosphorylated NF-κB (pNF-κB) after mucosal injury in a rat model of NEC induced by prematurity, systemic hypoxia, and a rich protein formula. In the control group (group 1), neonatal rats were full-term and breast-fed; in the experimental groups, rat pups were preterm at day 21 of gestation and rat-milk fed (group 2) or hand-gavaged with a protein rich formula after a hypoxia–reoxygenation procedure (group 3). Morphological mucosal changes in the small bowel were scored on hematoxylin- and eosin-stained sections. Immunohistochemistry was performed on frozen tissue sections using anti TLR-2 and active pNF-κB p65 antibodies. Real-time RT-PCR was performed to assess mRNA expression of NOD2, TLR-2 and TLR-4. Proliferation and apoptosis were studied in paraffin sections using anti Ki-67 and caspase-3 antibodies, respectively. The combination of immaturity, protein rich formula and a hypoxia–reoxygenation procedure induces pathological mucosal damage consistent with NEC. There was an overexpression of TLR-2, and pNF-κB in IECs that was correlated with the severity of mucosal damage, together with an increase of apoptotic IECs and markedly impaired proliferation. In addition, these immunological alterations appeared before severe mucosal damage. TLR-2 mRNA were also increased in NEC together with TLR-4 mRNA using real-time RT-PCR whereas NOD2 expression was unchanged. Conclusions/Significance These results show that this rat model of NEC induced mucosal injury, leading to a highly responsive IEC phenotype and suggesting that alterations in the innate immune system participates in the pathogenesis of NEC and are enhanced by prematurity.

Outcome of Urological Abnormalities Prenatally Diagnosed by Ultrasound

In order to evaluate the efficiency of antenatal diagnosis and postnatal management of urinary malformations, we reviewed 142 cases from October 1988 to December 1992 with abnormalities detected by routine ultrasound. A routine karyotype analysis showed 7 fetuses with chromosomal defects. Nine cases with isolated bilateral urinary tract dilatation underwent pelvic fetal urine sampling in order to assess antenatal renal function. The 142 cases comprised 107 children born alive (group I) and 35 intrauterine fetal deaths (group II). In group I, 79 kidneys had a transient pyelectasis, and 11 kidneys had a hydronephrosis which disappeared at postnatal examination. Among 103 pyelectasis cases detected during the second trimester of pregnancy, 22 kidneys were pathological after birth (positive predictive value (PPV) = 21.4%) with 17 ureteropelvic junction obstructions. The PPV was 66% when ultrasound showed hydronephrosis at the end of pregnancy. In that group, 15 cases had vesicoureteric reflux, with 5 cases where the homolateral upper urinary tract was morphologically normal in utero. Group II comprised 27 fetuses with multiple defects in other systems, and 8 cases with urological defects only 7 of which had urethral anomalies. This study confirmed the benefit of antenatal diagnosis for uropathies, and the advantage of prenatal consultation between pediatric surgeon and parents in order to explain what anomalies are involved and to program postnatal follow-up.

Laparoscopic retroperitoneal nephrectomy in high risk children.

PURPOSE Nephrectomy may be indicated in children with end stage renal disease before transplantation. We studied the feasibility and results of nephrectomy performed via a retroperitoneal laparoscopic approach in these high risk children. MATERIALS AND METHODS We performed 12 nephrectomies in 9 children with end stage renal disease and a mean age of 7 years (range 7 months to 13 years) through a 3 trocar retroperitoneal laparoscopic approach. Cases were classified as American Society of Anesthesiologists grade III and presented with end stage renal disease, hypertension, thrombocytopenia and/or the nephrotic syndrome. The renal artery and vein were ligated separately with endocorporeal knots and clips. Mean size of the kidney was 8 cm. (range 5 to 12). Bilateral nephrectomy was performed simultaneously in 2 patients 7 and 12 months old, respectively. Cardiorespiratory changes related to retroperitoneal gas insufflation were assessed prospectively. To compare laparoscopic versus open nephrectomy we retrospectively analyzed the data of 12 open nephrectomies performed in 9 children with similar nephrological indications. RESULTS The procedure was feasible in all cases without conversion to open surgery, and no intraoperative incident occurred. Mean operative time of laparoscopic nephrectomy was 2 hours (range 1 hour 20 minutes to 3 hours 10 minutes). After retroperitoneal carbon dioxide insufflation systolic arterial pressure and end-tidal carbon dioxide were significantly increased without the need for specific measure to correct these modifications. Hemodialysis began 1 day postoperatively and feeding began 2 days postoperatively. Mean hospital stay was 5.2 days (range 3 to 7). The comparative study of the open nephrectomy group showed no significant difference in mean operating time (p = 0.07), and hospital stay was significantly shorter for the laparoscopic group (p <0.001). CONCLUSIONS Retroperitoneal laparoscopic nephrectomy is safe and feasible for high risk children. The relatively long operating time is necessary for hemostasis in these children at risk for hemorrhagic complications.